Radiology - AFIP - 2006-2007 Lecture Notes

Radiologic
Pathology
Fifth Edition
VOLUME 1
Chest, Gastrointestinal, and Genitourinary
Radiologic Pathology Correlation
2006
Editors
Angela D. Levy, COL, MC, USA

Chairman and Registrar
Chief, Gastrointestinal Radiology
2007
Ellen M. Chung, LTC, MC, USA
Chief, Pediatric Radiology
Jeffrey R. Galvin, MD
Chief, Chest Radiology
Kelly K. Koeller, MD
Chief, Neuroradiology
Mark D. Murphey, MD
Six Week Course Director
Chief, Musculoskeletal Radiology
Paula J. Woodward, MD Illustrators

Chief, Genitourinary Radiology Aletta A. Frazier, MD
Dianne D. Engelby, MAMS, RDMS
Associate Editor Heike Blum, MFA
Jean-Claude Kurdziel, MD
Department of Radiologic Pathology

Armed Forces Institute of Pathology
Washington DC, USA
American Registry of Pathology
Washington, DC
20306-6000
_____________________________________
© Copyright 2006 by the American Registry of Pathology.
All rights reserved. No part of this publication may be reproduced or transmitted in any form
or by any means: electronic, mechanical, photocopy, recording, or any other information
storage and retrieval system without written permission of the publisher.
Made in the United States of America

_____________________________________
Great care has been taken to guarantee the accuracy of the information contained in this
volume. However, neither the American Registry of Pathology, Armed Forces Institute of
Pathology, nor the editors and contributors can be held responsible for errors or for any
consequences arising from the use of the information contained herein.
The opinions and assertions contained herein are the private views of the authors and are
not to be construed as official nor as representing the views of the Departments of the Army,
Air Force, Navy, or Defense.
987654321
Library of Congress Cataloging-in-publication Data [in process]
ISBN 1-933477-00-8
Preface
The Armed Forces Institute of Pathology’s Radiologic Pathologic Correlation
course presented by the Department of Radiologic Pathology enters its 59th year
of educating radiology residents worldwide. For the fifth year, our staff and visiting
lecturers have contributed their lecture material and images to compile Radiologic
Pathology 2006 – 2007, continuing the tradition of presenting richly illustrated
material that teaches the pathologic basis of disease to improve our understanding
of the imaging appearance of disease. We hope the efforts of our authors and
editors have once again accomplished our goal of bringing the outstanding and
unique Radiologic Pathologic Correlation course to your fingertips.
Acknowledgements
The annual production of the Radiologic Pathologic Correlation course and

syllabus is made possible through the tremendous support, dedication, and
selfless service of countless individuals who work in the AFIP and the various
institutions and organizations throughout the world that believe in the importance
of teaching the principles of disease through radiologic pathologic correlation.
The Department of Radiologic Pathology of the Armed Forces Institute of

Pathology expresses our deepest appreciation and sincerest gratitude to:
- All radiologists and radiology residents who have contributed case material to
the Thompson Radiologic Pathologic Archive at the Armed Forces Institute of
Pathology,
- All pathologists in the AFIP who have donated their time and expertise to
radiologic pathologic correlation,
- All of our outstanding authors, illustrators, and department staff members who
make the course and the syllabus happen effortlessly year after year,
- And, to the extraordinary efforts of our production team, headed by Jean-
Claude Kurdziel, MD, who have tirelessly dedicated the spring and summer of
the last five years to the production of this syllabus.
iii
Faculty – VOLUME 1
Chest Radiology
Marc S. Levine, MD
Jeffrey R. Galvin, MD Professor of Radiology
Chief, Pulmonary and Mediastinal Radiology
Hospital of the University of Pennsylvania
Advisory Dean
University of Pennsylvania School of Medicine
Washington, DC
Philadelphia, PA
and
and
Professor of Radiology and Pulmonary Medicine
Former Distinguished Scientist
University of Maryland
Baltimore, MD
Gerald F. Abbott, MD Washington, DC
Director of Chest Radiology
Rhode Island Hospital Deborah Rubens, MD
and Professor and Associate Chair
Assistant Professor of Radiology Department of Imaging Sciences
Brown University School of Medicine University of Rochester Medical Center
Providence, RI Rochester, NY
and
Aletta A. Frazier, MD Distinguished Scientist
Staff Radiologist and Medical Illustrator Department of Radiologic Pathology
Department of Radiologic Pathology Armed Forces Institute of Pathology
Armed Forces Institute of Pathology Washington, DC
Washington, DC
and Francis J. Scholz, MD
Clinical Associate Professor of Radiology Staff Radiologist
University of Maryland School of Medicine Lahey Clinic Medical Center
Baltimore, MD Burlington, MA
and
Leonard M. Glassman, MD Clinical Professor of Radiology
Washington Radiology Associates, PC Tufts University School of Medicine
Washington, DC Boston, MA
and
Clinical Professor Robert K. Zeman, MD
Department of Radiology Chairman and Professor of Radiology
George Washington University Medical Center George Washington University
Washington, DC Washington, DC
Melissa L. Rosado de Christenson, MD, FACR
Clinical Professor of Radiology
Genitourinary Radiology
The Ohio State University Paula J. Woodward, MD
Columbus, OH Acting Chief, Genitourinary Radiology
and Department of Radiologic Pathology
Adjunct Professor of Radiology Armed Forces Institute of Pathology
Uniformed Services University of the Health Sciences Washington, DC
Bethesda, MD and
Adjunct Professor of Radiology
Rosita M. Shah, MD University of Utah School of Medicine
Clinical Associate Professor of Radiology
Salt Lake City UT
Hospital of the University of Pennsylvania
Philadelphia, PA
Peter L. Choyke, MD
Chief Molecular Imaging Program
Gastrointestinal Radiology National Cancer Institute
Angela D. Levy, COL, MC, USA Bethesda, MD
Chairman and Gastrointestinal Radiology Section Chief and
Department of Radiologic Pathology Professor of Radiology and Nuclear Medicine
Armed Forces Institute of Pathology Uniformed University of the Health Sciences
Washington, DC Bethesda, MD
and William D. Craig, MD
Associate Professor of Radiology and Nuclear Medicine Chief, Genitourinary Radiology
Uniformed Services University of the Health Sciences Department of Radiologic Pathology
Bethesda, MD Armed Forces Institute of Pathology
Bruce P. Brown, MD Washington, DC
Associate Professor of Radiology
University of Iowa
Iowa City, IA
iv
David S. Hartman, MD
Professor of Radiology
Department of Radiology
Pennsylvania State University
M. S. Hershey Medical Center
Hershey, PA
Deborah J. Rubens, MD
Professor and Associate Chair
Department of Imaging Sciences
University of Rochester Medical Center
Rochester, NY
and
Distinguished Scientist
Washington, DC
Brent J. Wagner, MD
Chairman, Department of Radiology
The Reading Hospital and Medical Center
West Reading Radiology Associates
West Reading, PA
Jade J. Wong-You-Cheong, MD
Associate Professor of Diagnostic Radiology
Director of Ultrasound
University of Maryland School of Medicine
Baltimore, MD
v
Table of Contents – VOLUME 1
Chest Radiology
An Approach to Diffuse Lung Disease, Sarcoidosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .3
The Idiopathic Interstitial Pneumonias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .14
Airways Disease: The Movement from Anatomic to Physiologic Assessment . . . . . . . . . . . . . . . . . . . . . . . .26
Inhalational Lung Disease (Asbestosis and Silicosis) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .44
Pulmonary Lymphoid Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .54
Angiitis and Granulomatosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .63
The Pulmonary Complications of Organ Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .75
The Diagnosis of Pulmonary Embolism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .82
Tuberculosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .93
Fungal Disease in the Thorax: Opportunistic and Primary Pathogens . . . . . . . . . . . . . . . . . . . . . . . . . . . . .100
Bronchogenic Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .110
Chest Seminar 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .121
Chest Seminar 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .126
Aletta A. Frazier, MD
Pulmonary Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .131
Pulmonary Metastasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .138
Differential Diagnosis of Mediastinal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .148
Chest Seminar: Where is the lesion? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .168
Chest Seminar: Differential Diagnosis of Mediastinal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .173
Rosita M. Shah, MD
Pneumonia: Usual and Unusual Organisms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .178
Gerald F. Abbott, MD
Uncommon Malignant Tumors of the Lung . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .192
Benign Tumors of the Lung and Tumor-like Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .199
Pleural Disease I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .205
Pleural Disease II and Chest Wall . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .213
Leonard M. Glassman, MD (Mammography)
Classic Breast Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .220
Basic Breast Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .229
Ductal Carcinoma in Situ (DCIS) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .238
Breast Abnormalities in Young Women . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .246
The Male Breast . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .257
Gastrointestinal Radiology
Benign Hepatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .267
Malignant Hepatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .275
Hepatic Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .284
Imaging of Chronic Liver Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .293
Benign Biliary Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .303
Biliary Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .313
Pancreatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .321
Gastric Malignancies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .332
Abdominal Non Hodgkin Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .344
Small Intestinal Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .353
Colorectal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .361
Mesenteric Masses and Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .372
Idiopathic Inflammatory Bowel Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .382
Approach to Inflammatory Diseases of the Colon . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .391
GI Seminar 1: Abdominal Gas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .400
GI Seminar 2: Nonneoplastic Disease of the Stomach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .405
GI Seminar 3: Pancreatic Duct . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .411
GI Seminar 4: Hepatic Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .417
GI Seminar 5: Complications of Meckel Diverticulum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .422
vi
GI Seminar 6: Beyond Appendicitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .427
GI Seminar 7: Tumors and Tumor-Like Lesions of the Gallbladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .432
Robert K. Zeman, MD
Cholelithiasis and Cholecystitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .438
Marc S. Levine, MD
Inflammatory Diseases of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .444
Tumors of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .450
Radiology of Peptic Ulcer Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .455
Bruce Brown, MD
Pancreatitis: Imaging Has Made a Difference . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .460
Gastrointestinal Bleeding In The Age of the Endoscope. What Does a Radiologist Have To Contribute? . .468
Francis J. Scholz, MD
Small Bowel Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .475
Acute Mesenteric Ischemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .487
Malabsorption . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .505
Familial Polyposis and Other Such . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .519
The Spleen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .531
Portal Venous Doppler . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .542
Paula J. Woodward, MD
Imaging of Uterine Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .551
Approach to Renal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .561
Urinary Tract Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .573
Retroperitoneum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .579
Radiologic Evaluation of the Scrotum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .585
First Trimester Ultrasound . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .594
Fetal CNS Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .602
Fetal Body Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .607
Peter L. Choyke, MD
Cystic Diseases of the Kidney . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .614
Imaging of Prostate Cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .620
William D. Craig, MD
Radiographic Evaluation of Urinary Stone Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .624
Testicular Torsion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .630
Brent J. Wagner, MD
Imaging of Ovarian Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .637
Adrenal Imaging in Adults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .645
Imaging of the Urinary Bladder and Urethra . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .649
Jade Wong You Cheong, MD
Non-Neoplastic Disorders Of The Ovary And Adnexae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .653
Imaging of Solid Organ Transplants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .664
The Neglected Nephrogram . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .674
Problem Renal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .681
GU Seminar 1: MSAFP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .691
GU Seminar 2: Renal Calcifications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .693
vii
Chest Radiology
An Approach to Diffuse Lung Disease,
Sarcoidosis
Describing Diffuse Lung Disease

• The Alveolar vs. Interstitial Problem
• Alveolar or Interstitial ?
An Approach to Diffuse Lung Disease Figure 1-1-2

• Radiograph
➢ Lung volumes
➢ Opacity
➢ Distribution
➢ Ancillary findings
• Computed tomography
➢ Opacity
➢ Distribution
Figure 1-1-1
Radiology and pathology form

a continuum of visualization
Fibrosis results in reduced lung volumes

Lung Volumes
• Reduced [Figure 1-1-2]
➢ Pathology distal to the airway Figure 1-1-3
➢ Fibrosis
➢ IPF, asbestosis, sarcoidosis, chronic
hypersensitivity pneumonitis
• Increased [Figure 1-1-3]
➢ Pathology of the airway
➢ Emphysema, asthma, bronchitis, constrictive
bronchiolitis, LAM
Airways disease results in increased

lung volumes
Chest Radiology 3 An Approach to Diffuse Lung Disease

Distribution: Upper vs Lower [Figure 1-1-4] Figure 1-1-4
Plain Film and CT Opacities

• Nodules
• Reticulation and Lines
• Ground glass
• Consolidation
• Cystic airspaces
Plain Film and CT Opacities

• Nodules
➢ Sarcoid
➢ Silicosis, coal-workers
➢ Hypersensitivity Pneumonitis
➢ Metastasis
• Reticulation and Lines
➢ Fibrosis
✧ IPF-lower, subpleural
✧ Asbestosis-lower, subpleural
✧ Sarcoidosis-peribronchovascular
✧ Chronic hypersensitivity pneumonitis-mid and
upper lung zone
• Ground glass [Shah and Miller AJR 2003]
➢ Non-specific
✧ Airspace, interstitial, combined
– DIP, NSIP, AIP, DAD (32%)
✧ Infection (32%)
✧ Drug toxicity (11%)
✧ Hemorrhage (3%)
✧ Ground glass with reticulation
– Fibrosis Chronic diseases tend to involve the upper lung
• Consolidation
➢ Organizing Pneumonia (BOOP)
➢ Chronic eosinophilic pneumonia
➢ Lymphoma
➢ Bronchoalveolar cell carcinoma
➢ Infection
➢ Hemorrhage
• Cystic airspaces
➢ Mimics reticulation on plain radiographs
➢ Fibrosis and honeycombing
✧ IPF-Lower, subpleural
✧ LAM-Diffuse
✧ LCH-Upper
Radiologic-Pathology Continuum
Anatomy - Secondary Lobule

• As defined by Miller
➢ Polygonal
➢ 1-2.5 cm
➢ Smallest unit demarcated by connective tissue septa
• Most useful diagnostically
➢ Readily identified on:
✧ HRCT
✧ Gross examination
✧ Histologic section
➢ Explains HRCT appearance
✧ Broad range of lung diseases
✧ Especially interstitial disease
An Approach to Diffuse Lung Disease 4 Chest Radiology

Anatomy - Secondary Lobule [Figure 1-1-5]
• Core structures
➢ Axial interstitium
➢ Bronchiole
➢ Pulmonary artery
➢ Lymphatics
• Septal structures
➢ Peripheral interstitium
➢ Pulmonary veins
➢ Lymphatics
• Parenchyma
➢ Alveolar interstitium
➢ Alveoli
➢ Pulmonary capillary bed
Figure 1-1-5
The secondary lobule with lymphatics in the

interloblular septa and along
the bronchovascular bundle
Figure 1-1-6
Septal pattern on HRCT and gross specimen

Abnormal Patterns Figure 1-1-7 Figure 1-1-8
• Bronchovascular
➢ Bronchus
✧ Asthma, CF, bronchitis,
bronchiectasis [Figure 1-1-7]
➢ Lymphatic
✧ CA, lymphoma, sarcoidosis
✧ Edema
• Centrilobular [Figure 1-1-8]
➢ Airway related
• Panlobular [Figure 1-1-9]
➢ Nonspecific
• Septal
➢ Lymphatic
✧ CA, lymphoma, sarcoidosis
[Figure 1-1-10and 1-1-11]
✧ Edema
• Random [Figure 1-1-12] Bronchovascular pattern Centrilobular pattern
➢ Hematogenous spread of tumor
➢ TB
Figure 1-1-9 Figure 1-1-10
Sarcoidosis
• Multisystem granulomatous disorder
• Unknown etiology
• Young and middle aged adults
• Bilateral hilar lymphadenopathy, pulmonary
infiltration, eye and skin lesions
• Clinical and radiologic findings supported
by evidence of noncaseating epithelioid
granulomas
• Exclusion of granulomas of unknown cause
and local sarcoid reactions
ATS Statement on Sarcoidosis 1999
Sarcoidosis: Epidemiology
• Worldwide
➢ both sexes, all races, all ages Panlobular pattern Septal pattern
• Predilection for adults
➢ under 40 years
➢ peak 20-29 years
• U.S. prevalence Figure 1-1-11 Figure 1-1-12
➢ 10 per 100,000 exams
• Highest disease
➢ African-American women
Sarcoidosis: Clinical Features

• Asymptomatic
➢ 15-50%
• Constitutional symptoms
➢ 33%
• Dyspnea, cough, chest pain
➢ 33-50%
• Palpable lymph nodes
➢ 33-75%
• Ocular involvement
➢ 11-83%
• Cutaneous involvement
➢ 20-30% Erythema nodosum, Lupus
pernio Combined septal and Random nodule pattern
bronchovascular pattern

Sarcoidosis: Laboratory Abnormalities Figure 1-1-13
• BAL
➢ ↑ macrophages, ↓ proportions; ↑ CD4 helper
cells
• Angiotensin-Converting Enzyme
➢ Nonspecific Produced by
granuloma/macrophage
➢ ↑ 33-90%
• Hypercalcemia 10%
• Hypercalciuria 30%
➢ Macrophage/granuloma extrarenal sources
of 1-25 Dihydroxyvitamin D
• Anergy
• Hypergammaglobulinemia
Sarcoidosis: Respiratory System

[Figure 1-1-13]
• 100% lung involvement
• Portal of entry
➢ Local lymph nodes
➢ Distant organs
• Disease distribution
➢ Alveolar wall
➢ Secondary lobule,
➢ Axial CT
➢ Radiograph
Non-Caseating Granuloma and Fibrosis

Sarcoidosis pathogenesis
Alveolar Distribution
Sarcoidosis and the Secondary Lobule [Figure 1-1-14]
Figure 1-1-14
Bronchovascular distribution of
granulomas in Sarcoidosis

Figure 1-1-15
Distribution of nodules in sarcoidosis Masses in Sarcoidosis
Ground glass in Sarcoidosis Conglomerate masses and fibrosis in sarcoidosis
Sarcoidosis: Computed Tomography [Figure 1-1-15]

• Nodules
• Masses
• Ground Glass
• Fibrosis
➢ Conglomeration
➢ Distortion
• Emphysema
• Bulla
• Honeycombing

Parenchymal Disease: Radiography Figure 1-1-16
• Bilateral
• Symmetrical
• Nodules
• Reticulonodular
• Masses
• Ground Glass
• Hilar Retraction
• Bulla
• Honeycombing
Sarcoidosis: Adenopathy [Figures 1-1-16 and 1-1-17]

• Node Group CXR CT
• Hilar 84 88
• R. Paratracheal 76 100
• A-P Window 72 92
• Subcarinal 12 64
• Ant. Med. 12 48
• Post. Med. 0 16
Lymph node involvement is a hallmark of sarciodosis
Sarcoidosis:
Staging based on Adenopathy and Parenchyma
Figure 1-1-17
Presentation Resolution
Stage 0 8 –
➢ Normal
Stage 1 51 65
➢ Adenopathy
Stage 2 29 49
➢ Adenopathy &
Parenchyma
Stage 3 12 20
➢ Parenchyma
20% develop fibrosis or Stage 4 disease
Sarcoidosis Stage I
Sarcoidosis Stage II
Sarcoidosis Stage III
Sarcoidosis Stage IV
Bilateral calcified lymph nodes are common
Sarcoidosis Progression
Sarcoidosis and the Parenchyma: Computed Tomography

• Thickened Bronchovascular Bundles
• Nodules
➢ Peribronchovascular
➢ Pleural, subpleural and septal
• Consolidation and Large Nodules
• Ground-Glass Opacities
• Fibrosis

Thickened Bronchovascular Bundles [Figure 1-1-18]
Figure 1-1-18
Peribronchovascular opacities
in sarcoidosis
Peribronchovascular Nodules
Peribronchovascular and Pleural Nodules
Septal Lines
Ground Glass Opacities
Consolidation and Large Nodules
Fibrosis
Bronchovascular Bundle Distortion [Figure 1-1-14]
Conglomerate Mass
Fibrosis and Emphysema
Fibrosis and Honeycombing
Sarcoidosis: Diagnosis
• Typical clinical and radiologic manifestations
• Non-caseating granulomas
• Transbronchial Bx
• Endobronchial Bx
Sarcoidosis: Differential Diagnosis

• Infection
➢ Tuberculosis, Fungal (Histoplasmosis)
• Pneumoconiosis
➢ Silica, Beryllium
• Hypersensitivity Pneumonitis
• Malignancy
➢ Lymphoma

Miliary Tuberculosis
Transbronchial Spread of Tuberculosis
Histoplasmosis
Silicosis
Berylliosis
Extrinsic Allergic Alveolitis
Sarcoidosis: Mortality
• Mortality range 5-10%
• Cor Pulmonale related to fibrosis
• Cardiac Arrhythmia
• Pulmonary Hemorrhage
➢ Aspergilloma
Cor Pulmonale
Sarcoidosis: Cardiac Involvement

• Clinical involvement 5%
➢ Heart block, arrhythmia, mitral regurgitation, CHF (dilated cardiomyopathy)
and sudden death
• Autopsy involvement 20-30%
• Localized wall motion abnormalities
➢ Anterior and apical
➢ MRI, Echocardiograph, Thallium-201
Vignaux AJR 184 Jan 2005
Cardiac Sarcoidosis [Figure 1-1-19]
Figure 1-1-19
Sarcoid infiltration on MRI is represented as focal zones of

increased signal on T2 and early gadolinium images
Dilated Cardiomyopathy

Sarcoidosis: Mycetoma Figure 1-1-20
• Present in 40-50% of cystic lesions
➢ Bullae, cavities or bronchiectasis
• Hemorrhage
• Steroids may convert to invasive process
Mycetoma [Figure 1-1-20]
Sarcoidosis: Therapy
• Cardiac, CNS, eye involvement
• Hypercalcemia
• Corticosteroids
➢ Relief of symptoms; resolution of radiologic abnormalities;
improved function
• Cytotoxic agents
➢ Methotrexate, Azathioprine
• Chlorambucil, cyclophosphamide, antimalarials
• Risk of recurrence
Sarcoidosis: Resolution
Sarcoidosis: Prognosis
• Favorable
➢ Acute onset, erythema nodosum,
➢ > 80% spontaneous remission
➢ Löfgren syndrome
➢ Low stage
• Poor
➢ Chronic course, Lupus pernio
➢ Older age at presentation
➢ Hypercalcemia/nephrocalcinosis
➢ Black race, Extrathoracic involvement
Mycetoma in a cystic space
Sarcoidosis Conclusion caused by sarcoidosis
References
General
1. Akira M, Hara H, Sakatani M. Interstitial lung disease in association with polymyositis- dermatomyositis: long-
term follow-up CT evaluation in seven patients. Radiology 1999; 210(2):333-8.
2. Bergin CJ, Muller NL. CT of interstitial lung disease: a diagnostic approach. American Jounal of Roentgenology
1987; 148:8-15.
3. Bergin C, Roggli V, Coblentz C, Chiles C. The secondary pulmonary lobule:normal and abnormal CT appearances.
American Journal of Roentgenology 1988; 15:21-25.
4. Epler GR, McLoud TC, Gaensler EA, Mikus JR Carrington CB. Normal chest roentgenograms in chronic diffuse
infiltrative lung disease. N Engl I Med 1978:298(17):934-9.
5. Epler GR. Chest films: underused tool in interstial lung disease. Journal of Respiratory Diseases 1987; 8(6):1 4-24.
6. Felson B. A new look at pattern recognition of diffuse pulmonary disease. American Journal of Roentgenology
1979; 133:183-189.
7. Calvin JR. Mon M, Stanford W. High-resolution computed tomography and diffuse lung disease. Curr Probl Diagn
Radiol 1992; 21(2):31-74.
8. Grenier P. Valeyre D, Cluze I R Brauner MW, Lenoir 5, Chastang C. Chronic diffuse interstitial lung disease:
diagnostic value of chest radiography and high- resolution CT. Radiology 1991; 179:123-132.
9. Gruden JF, Webb WR, Naidich DR, McGuinness G. Multinodular disease: anatomic localization at thin-section
CT—multireader evaluation of a simple algorithm. Radiology 1999; 210(3):711-20.
10. Gurney JW, Schroeder BA. Upper lobe lung disease: physiologic correlates. Radiology 1988; 167:359-366.
11. Heitzman ER. The lung. Second ed. St. Louis: C.V. Mosby, 1984.
12. Johkoh T, Muller NL, Cartier Y, Kavanagh PV, Hartman TE, Akira M, lchikado K, Ando M, Nakamura H.
Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology 1999; 211
(2):555-60.
13. Mathieson JR. Mayo JR. Staples CA, Muller NL. Chronic diffuse infiltrative lung disease: comparison of dianostic
accuracy of CT and chest radiography. Radiology 1989; 171:111-116.

14. Mayo JR. Webb WR, Gould R, Stein MG, Bass I, Gamsu G, Goldberg H. High- resolution CT of the lungs: an
optimal approach. Radiology 1987; 163:507-510.
15. McLoud TC, Carrington CB, Gaensler EA. Diffuse Infiltrative lung disease: a new scheme for description.
Radiology 1983; 149(2):353-363.
16. Muller NE, Miller RR. Computed tomography of chronic diffuse infiltrative lung disease. Part 2. Am Rev Respir
Dis 1990; 142(6 Pt 1 ):1440-8.
17. Muller NE, Miller RR. Computed tomography of chronic diffuse infiltrative lung disease. Part lAm Rev Respir Dis
1990; 142(5):1206-15.
18. Muller NE, Coiby TV. Idiopathic interstitial pneumonias: high-resolution CT and histologic findings.
Radiographics 1997; 17(4): 1016-22.
19. Murata K, Itoh H, Todo G, Kanaoka M, Noma 5, Itoh T, Furuta M, Asamoto H, Torizuka K. Centrilobular lesions
of the lung: demonstration by high-resolution CT and pathologic correlation. Radiology 1986; 161 :641-645.
20. Murata K, Khan A, Rojas KA, Herman PG. Optimization of computed tomography technique to demonstrate the
fine structure of the lung. Investigative Radiology 1988; 23:170-175.
21. Murata K, Khan A, Herman R Pulmonary parenchymal disease: evaluation with high-resolution CT. Radiology
1989; 170:629-635.
22. Muller NI, Miller RR. Computed tomography of chronic diffuse lung disease. American Review of Respiratory
Disease 1990; 142:1206-1215, 1440-1448.
23. Staples CA, Muller NE, Vedal S, Abboud R, Ostrow D, Miller RR. Usual interstitial Pneumonia: correlation of CT
with clinical, functional, and radiologic findings. Radiology 1987; 162:377-381.
24. Webb WR. High resolution CT of lung parenchyma. Radiologic Clinics of North America 1989; 27(6):1085-1097.
25. Weibel ER. Looking into the lung: what can it tell us? American Journal of Roentgenology 1979; 133:1021-1031.
26. Weibel ER, Bachofen H. The Fiber Scaffold of Lung Parenchyma. In: Crystal RG, West JB, eds. The Lung. New
York: Raven Press, 1991; 787-794.
27. Weibel ER, Crystal RG. Structural Organization of the Pulmonary Interstitium. In: Crystal RG, West JB, eds. The
Lung. New York: Raven Press, 1991; 369-380.
Sarcoidosis
1. Bergin CJ, Bell DY, Coblentz CL, Chiles C, Gamsu C, Maclntyre NR, Coleman RE, Putman CE. Sarcoidosis:
correlation of pulmonary parenchymal pattern at CT with results of pulmonary function tests. Radiology 1989;
171(3):619-24.
2. Gawne-Cain ML, Hansell CM. The pattern and distribution of calcified mediastinal lymph nodes in sarcoidosis
and tuberculosis: a CT study. Clin Radiol 1996; 51(4):263-7.
3. Gleeson FV, Traill ZC, Hansell CM. Evidence of expiratory CT scans of small- airway obstruction in sarcoidosis.
AJRAm J Roentgenol 1996; 166(5):1052-4.
4. Hansell DM, Milne DC, Wilsher ME, Wells AU. Pulmonary sarcoidosis:morphologic associations of airflow
obstruction at thin-section CT. Radiology 1998; 209(3):697-704.
5. Kuhlman JE, Fishman EK, Hamper UM, Knowles M, Siegelman SS. The computed tomographic spectrum of
thoracic sarcoidosis. Radiographics 1989; 9(3):449-66.
6. Miller WT Jr, Shah RM. Isolated diffuse ground-glass opacity in thoracic CT: causes and clinical presentations.
AJR Am J Roentgenol. 2005 Feb;184(2):613-22.
7. Muller NE, Kullnig P, Miller RR. The CT findings of pulmonary sarcoidosis: analysis of 25 patients. AJR Am J
Roentgenol 1989; 152(6):1179-82.
8. Muller NE, Mawson JB, Mathieson JR. Abboud R, Ostrow DN, Champion P Sarcoidosis: correlation of extent of
disease at CT with clinical, functional, and radiographic findings. Radiology 1989; 171 (3):61 3-8.
9. Murdoch J, Muller NE. Pulmonary sarcoidosis: changes on follow-up CT examination. AJR Am J Roentgenol
1992; 159(3):473-7.
10. Newman ES, Rose CS, Maier LA. Sarcoidosis [published erratum appears in N Engl J Med 1997 Jul
10;337(2):1391 [see comments]. N Engl J Med 1997; 336(17):1224-34.
11. Nishimura K, Itoh H, Kitaichi M, Nagai S, Izumi T. Pulmonary sarcoidosis: correlation of CT and histopathologic
findings [published erratum appears in Radiology 1994 Mar;190(3):907]. Radiology 1993; 189(1):105-9.
12. Nishimura K, Itoh H, Kitaichi M, Nagai S, Izumi T. CT and pathological correlation of pulmonary sarcoidosis.
Semin Ultrasound CT MR 1995; 16(5):361-70.
13. Padley SP, Padhani AR, Nicholson A, Hansell DM. Pulmonary sarcoidosis mimicking cryptogenic fibrosing
alveolitis on CT. Clin Radio! 1996; 51(11):807-10.
14. Rockoff SD, Rohatgi PK. Unusual manifestations of thoracic sarcoidosis. AJR Am J Roentgenol 1985; 144(3):513-
28.
15. Thomas PD, Hunninghake GW. Current concepts of the pathogenesis of sarcoidosis. Am Rev Respir Dis 1987;
135(3):747-60.
16. Vignaux O. Pictorial Essay: Cardiac sarcoidosis: spectrum of MRI features. AJR Am J Roentgenol 2005
Jan;184(1):249-54.
17. Winterbauer RH, Belic N, Moores KD. Clinical interpretation of bilateral hilar adenopathy. Ann Intern Med 1973;
78(1 ):65-71.

The Idiopathic Interstitial Pneumonias
Figure 1-2-1
Chronic Diffuse Lung Disease [Figure 1-2-1 and 1-2-2]
• Alveolar involvement
➢ Surrounding airways
➢ Fibrosis and/or cells
✧ Alveolar wall
✧ Alveolar space
• Restrictive physiology
• Decreased lung volumes
• Increased attenuation
• Subacute or chronic
➢ Weeks to months
Figure 1-2-2

involve the alveolar walls and spaces
The lung volumes are low and

there are areas of increased density

• Liebow 1975
• Supporting lung structures
➢ Inflammation
➢ Fibrosis
• Not confined to interstitium
• Initiated within the airspace
Liebow, Prog Reps Dis 1975

Current List-ATS/ERS Consensus Classification
• Idiopathic Pulmonary Fibrosis (IPF)
➢ Usual Interstitial Pneumonia (UIP)
• Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD)
• Desquamative Interstitial Pneumonia (DIP)
• Acute Interstitial Pneumonia (AIP)
• Cryptogenic Organizing Pneumonia (COP)
• NonSpecific Interstitial Pneumonia (NSIP)
Travis et al. Am J Respir Crit Care 2002
The Idiopathic Interstitial Pneumonias 14 Chest Radiology

Idiopathic Pulmonary Fibrosis
• Usual Interstitial Pneumonia: histologic pattern
• 5th-7th decade
➢ 66% > 60 years
➢ 7/100,000 women and 10/100,000 men
• Insidious onset of dyspnea
➢ 6 months before diagnosis
➢ Restrictive ventilatory defect
➢ Rales and clubbing
• Associations:
➢ Cigarette smoke
➢ Dusty environments: farming, wood dust, metal dust
➢ GE reflux
Figure 1-2-3
➢ Autoantibodies common (ANA, RA)
• Median survival 2.5-3.5 years
Usual Interstitial Pneumonia: Histology

• Geographic variation
• Temporal variation
➢ Fibroblast foci
➢ Mature fibrous tissue
• Extensive fibrosis
• Inflammation
➢ Minimal
➢ No correlation outcome
Abnormal wound healing
• Prognosis
➢ Fibroblast foci
✧ Presence and extent
Katzenstein, Am J Respir Crit Care Med 1998
Selman, Ann Int Med 2001
King, Am J Respir Crit Care Med 2001
Idiopathic Pulmonary Fibrosis Imaging [Figure 1-2-3]

• Radiograph abnormal-95%
The abnormalities are predominantly
➢ Volume loss
peripheral and lower lung field.
➢ Reticulonodular opacities
There is progressive volume loss
➢ Lower lobe
➢ Honeycombing
• Computed tomography Figure 1-2-4
➢ Peripheral and lower lobe
➢ Reticulation and ground glass
✧ Progress to honeycombing
➢ Ground glass in areas of
traction bronchiectasis
Hartman, Chest 1996
IPF-Progressive Volume Loss
Idiopathic Pulmonary Fibrosis [Figure 1-2-4]
IPF and Emphysema
Typical peripheral reticulation and honeycombing

and traction bronchiectasis in a patient with IPF
Chest Radiology 15 The Idiopathic Interstitial Pneumonias

Utilility of Biopsy for Diagnosis of IPF
• Prospective, multi-center study
➢ 91 patients suspected of IPF
• Clinical diagnosis
➢ Positive predictive value with a confident diagnosis-87%
• Imaging diagnosis
➢ Positive predictive value with a confident diagnosis-96%
➢ CT always abnormal in patients with proven IPF
• Histologic diagnosis
➢ Agreement regarding the presence or absence of IPF-85%
➢ Agreement in patients without IPF-48%
✧ Relevance to NSIP
Figure 1-2-5
• Uncertain diagnosis
• Discordant data
• Disease other than IPF
➢ Hypersensitivity pneumonitis
➢ Collagen-vascular disease
➢ Infection
Hunninghake, Am J Respir Crit Care Med 2001
IPF Rad-Path Discord
Smoking Related ILD

Interstitial Lung Disease [Figure 1-2-5]
• Respiratory bronchiolitis
➢ RB
• Respiratory bronchiolitis-interstitial lung disease
➢ RB-ILD
• Desquamative interstitial lung disease
➢ DIP
Smoking Related ILD

RB
• Clinical
➢ Cigarette smoke or equivalent Smoker’s macrophages
➢ Asymptomatic
• Pathology
➢ Peribronchiolar macrophages
➢ Peribronchiolar fibrosis
• Imaging
➢ Centrilobular nodules
✧ Poorly defined 2-3 mm
✧ Uper lobe predominance
➢ Ground glass opacity
➢ Bronchial wall thickening
➢ Decreased attenuation
➢ Emphysema
➢ Air trapping
➢ Reticulation
Niewoehner, NEJM 1974 ; Remy-Jardin, Radiology 1993

Smoking Related ILD [Figure 1-2-6] Figure 1-2-6
RB-ILD
• Clinical
➢ Cigarette smoke or equivalent
➢ Dyspnea
➢ Restrictive or mixed PFT’s
➢ Good prognosis
• Pathology
➢ Peribronchiolar macrophages
➢ Peribronchiolar fibrosis
• Imaging
✧ Poorly defined 2-3 mm
✧ Uper lobe predominance
➢ Ground glass opacity
➢ Decreased attenuation
➢ Emphysema
➢ Air trapping
➢ Reticulation
Meyers, Am Rev Respir Dis 1987
Park, J Comput Assist Tomogr 2002 Small centrilobular nodules with an
upper lobe predominance in RB-ILD
Smoking Related ILD
DIP
• Clinical
➢ Cigarette smoke
➢ 4th and 5th decade
➢ Uncommon
➢ 70% survival-10 years
➢ Steroids
• Pathology
➢ Pigmented macrophages
➢ Interstitial infiltrate
✧ Plasma cells and eosinophils
➢ Fibrosis
• Imaging
➢ Ground glass
✧ Symmetrical
✧ Basal predominance
➢ Reticulation
➢ Cysts
✧ Alveolar ducts
Figure 1-2-7
✧ Bronchioles
✧ Emphysematous spaces
Carrington, NEJM 1978 ; Hartman, Radiology
1993
Desquamative Interstitial
Pneumonia [Figure 1-2-7
Ground glass opacities in DIP

Dependent Density Figure 1-2-8
Desquamative Interstitial
Pneumonia
RB and DIP
Smoking Related ILD [Figure 1-2-8]
Acute Interstitial Pneumonia

AIP
• Hammon-Rich disease
• Rapidly progressive
• Days-weeks
• Antecedent flu-like syndrome
• Mean age 50 years
• 50% fatal at least
Vourlekis, Medicine 2000
Acute Interstitial Pneumonia Smoking related interstitial lung disease with upper lobe
Histology indistinct nodules, reticulation and well defined emphysematous
• Exudative phase spaces combined with lowerlobe ground glass
➢ Hyaline membranes Figure 1-2-9
➢ Edema
➢ Inflammation
• Collapse of alveoli
• Organizing phase
➢ Type II hyperplasia
➢ Loose fibrosis
• Diffuse Alveolar Damage
Katzenstein, Am J Pathol 1986 ; Ichikado, AJR 1997
Diffuse Alveolar Damage
Acute Interstitial Pneumonia

Radiography [Figure 1-2-9 and 1-2-10]
• Diffuse
• Airspace opacification Figure 1-2-10
• Costal sparing
• Mechanical ventilation
• Resembles ARDS
AIP involves all 5 lobes
Most patients are intubated with

diffuse opacities

Acute Interstitial Pneumonia Figure 1-2-11
Computed Tomography
• Exudative phase
➢ Consolidation
➢ Bilateral
➢ Focal sparing
• Organizing phase
➢ Distortion
➢ Traction bronchiectasis
➢ Ground glass
Johkoh, Radiology 1999;
Ichikado et al. Am J Respr Crit Care Med 2002
Acute Interstitial Pneumonia [Figure 1-2-11]
Cryptogenic Organizing Pneumonia

• Non-specific inflammatory response
• Pattern of repair
• Self-perpetuating
• Cryptogenic
• Secondary
➢ Connective tissues disease, hematologic
malignancy, drugs or organ transplantation
• Focal
➢ Bacteria, legionella, mycoplasma, mycobacterial,
or infarction
Lohr, Arch Int Med 1997
Focal areas of sparing are common in AIP
• Terminology problem
➢ Bronchiolitis obliterans OP (BOOP), bronchiolitis obliterans (BO),
bronchiolitis interstitial pneumonia (BIP)
• Subacute presentation (3 months)
• M=F
• Cough, dyspnea, weight loss, fever
• Restrictive PFT’s Figure 1-2-12
• Steroid responsive
• Relapse common
Epler, NEJM 1985

Histology
• Fibroblastic plugs in alveoli
• Fibrosis in the alveolar space
• May be airway centered
➢ Bronchiolitis
Cryptogenic Organizing Pneumonia [Figure 1-2-12]

Radiography
• Consolidation
➢ Unlateral or bilateral
• Small Nodules
➢ 10-50%
• Lung volumes
➢ normal in 75%
Cryptogenic organizing pneumonia is

characterized by focal areas of
consolidation more common in the
lower lung fields

Cryptogenic Organizing Pneumonia Figure 1-2-13
Computed Tomography
• Consolidation 90%
• Ground glass 75%
• Bronchial thickening and dilatation
• Small nodules along bronchvascular
bundles
• Large nodules (15%)
➢ Irregular margins
➢ Air bronchograms
• Reverse halo
Cryptogenic Organizing
Pneumonia [Figure 1-2-13 and 1-2-14]
Figure 1-2-14
Typical findings in COP with peripheral areas of consolidation.

The differential includes chronic eosinophilic pneumonia,
bronchoalveolar cell carcinoma, lymphoma and infection
Diffuse nodules may also be seen in

COP
Nonspecific Interstitial Pneumonia

• Katzenstein
➢ Described in 1994
• Does not fit definition of other IIPs
➢ UIP, RB-ILD, DIP, OP, AIP
• Represents a variety of etiologies
➢ Collagen vascular disease, drug reaction, inhaled antigen
➢ Inadequately sampled UIP or OP
• Median age 45
• Onset gradual with wide range
➢ 6 months to 3 years
• Better prognosis
Katzenstein, Am J Resp Crit Care 1994
Nicholson, Am J Respir Crit Care Med 2001

Nonspecific Interstitial Pneumonitis
Histology
• 3 categories
➢ Cellular
➢ Fibrosing
➢ Mixed
• Prognosis=fibrosis
• OP common
• Temporally uniform
Nonspecific Interstitial Pneumonitis

Imaging
• Few reports on chest radiography
• Wide variety of CT patterns
➢ Ground glass, consolidation, reticular and honeycombing
• Traction bronchiectasis=fibrosis
• CT pattern indistinguishable
➢ UIP 32%
➢ Hypersensitivity 20%
➢ OP 14%
➢ Other 12%
Hartman, Radiology 2000
NSIP ATS Consensus Conference

• Pathologists
• Radiologists
• Pulmonalogists
• 300 cases submitted
➢ 11 cases agreed to be NSIP by all pathologists
• Imaging
➢ Lower lobe
➢ Peribronchiolar reticulation and distortion
➢ Subpleural clearing
NSIP Current View
NSIP Fibrosis with IPF Imaging

• Areas of NSIP commonly found in proven cases of UIP
• NSIP and UIP
➢ Different severity of injury?
➢ Different mechanism of injury?
• Prognosis in these cases is driven by the imaging Figure 1-2-15
Katzenstein AA et al, Amer J of Surg Path 2002
Hypersensitivity Pneumonitis
NSIP in Cigarette Smokers
OP-NSIP [Figure 1-2-15]
OP-NSIP is peribronchovascular with UIP is peripheral

The Idiopathic Interstitial Pneumonias Figure 1-2-16
Current List
• Idiopathic Pulmonary Fibrosis (IPF)
➢ Usual Interstitial Pneumonia (UIP)
• Respiratory Bronchiolitis-Interstitial Lung Disease
(RB-ILD)
• Desquamative Interstitial Pneumonia (DIP)
• Acute Interstitial Pneumonia (AIP)
• Cryptogenic Organizing Pneumonia (COP)
• NonSpecific Interstitial Pneumonia (NSIP)

[Figure 1-2-16]
RB/RB-ILD [Figure 1-2-17]
RB-ILD/DIP [Figure 1-2-18]

IPF
Figure 1-2-17
Figure 1-2-18
RB-ILD
DIP
Acute Interstitial Pneumonia [Figure 1-2-19 and 1-2-20]
Figure 1-2-19
Figure 1-2-20
AIP early phase

AIP late phase with organization and fibrosis

Organizing Pneumonia [Figure 1-2-21] Figure 1-2-21
NSIP in the Literature
NSIP-IPF
NSIP-Cigarette Smokers [Figure 1-2-22]
Figure 1-2-22
Organizing pneumonia
Patients with smoking related fibrosis may have a

biopsy that demonstrates NSIP
NSIP-Hypersensitivity Pneumonitis
NSIP-Organizing Pneumonia [Figure 1-2-23]
Figure 1-2-23
Patients with organizing pneumonia may have a biopsy

that demonstrates NSIP

References
General
1. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification
of the Idiopathic Interstitial pneumonias. This joint statement of the American Thoracic Society (ATS), and the
European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS
Executive Committee, June 2001. Am J Respir Crit Care Med 2002; 165:277-304
2. Wittram C, Mark EJ, McLoud TC. CT-histologic correlation of the ATS/ERS 2002 classification of idiopathic
interstitial pneumonias. Radiographics. 2003 Sep-Oct;23(5):1057-71.
IPF/UIP
1. Hansell DM, Wells AU. CT evaluation of fibrosing alveolitis—applications and insights. J Thorac Imaging 1996;
11(4):231-49.
2. Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am U
Respir Crit Care Med 1998; 157(4 Pt 1):1301-15.
3. Kondoh Y, Taniguchi H, Kawabata Y, Yokoi T, Suzuki K, Takagi K. Acute exacerbation in idiopathic pulmonary
fibrosis. Analysis of clinical and pathologic findings in three cases. Chest 1993; 103(6):1808-12.
4. Liebow AA. Definition and classification of interstitial pneumonias in human pathology. Prog Resp Res 1975; 8:1-
33.
5. Tobin RW, Pope CE, 2nd, Pellegrini CA, Emond MJ, Sillery J, Raghu G. Increased prevalence of gastroesophageal
reflux in patients with idiopathic pulmonary fibrosis. Am U Respir Crit Care Med 1998; 158(6): 1804-8.
6. Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Polzleitner D, Burghuber OC, Tscholakoff D. Interstitial
lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology 1990; 176(755-
759).
7. Coxson HO, Hogg JC, Mayo JR, Behzad H, Whittall KP, Schwait DA, Hartley PC, Galvin JR, Wilson JS,
Hunninghake SW. Quantification of idiopathic pulmonary fibrosis using computed tomography and histology. Am
J Respir Crit Care Med 1997; 155(5):1649-56.
8. Gay SE, Kazerooni EA, Toews GB, Lynch UP, 3rd, Gross BH, Cascade PN, Spizarny DL, Flint A, Schork MA,
Whyte RI, Popovich U, Hyzy R, Martinez FJ. Idiopathic pulmonary fibrosis: predicting response to therapy and
survival. Am U Respir Crit Care Med 1998; 157(4 Pt 1):1063-72.
9. Bjoraker JA, Ryu JH, Edwin MK, Myers JL, Tazelaar Ho, Schroeder DR, Offord KR. Prognostic significance of
histopathologic subsets in idiopathic pulmonary fibrosis. Am U Respir Crit Care Med 1998; 157(1):1 99-203.
DIP
1. Gaensler EA, Goff AM, Prowse CM. Desquamative interstitial pneumonia. N Engl U Med 1966; 274(3)113-28.
2. Ryu JH, Myers JL, Capizzi SA, Douglas WW, Vassallo R, Decker PA.Desquamative interstitial pneumonia and
respiratory bronchiolitis-associated interstitial lung disease. Chest. 2005 Jan;127(1):178-84.
DAD/AIP
1. Bone RC. The ARDS lung. New insights from computed tomography [editorial; comment]. Jama 1993; 269(1
6):21 34-5.
2. Desai SR, Wells AU, Rubens MB, Evans TW, Hansell DM. Acute respiratory distress syndrome: CT abnormalities
at long-term follow-up. Radiology 1999; 210(1):29-35.
3. Greene R. Adult respiratory distress syndrome: acute alveolar damage. Radiology 1987; 163(1):57-66.
4. Ichikado K, Johkoh T, Ikezoe U, Takeuchi N, Kohno N, Arisawa U, Nakamura H, Nagareda T, Itoh H, Ando M.
Acute interstitial pneumonia: high-resolution CT findings correlated with pathology. AUR Am U Roentgenol 1997;
1 68(2):333-8.
5. Johkoh T, Muller NL, Taniguchi H, Kondoh Y, Akira M, Ichikado K, Ando M, Honda 0, Tomiyama N, Nakamura
H. Acute interstitial pneumonia: thin-section CT findings in 36 patients. Radiology 1999; 211(3):859-63.
6. Katzenstein AL, Myers UL, Mazur MT. Acute interstitial pneumonia. A clinicopathologic, ultrastructural, and cell
kinetic study. Am U Surg Pathol 1986; 10(4):256-67.
7. Olson U, Colby TV, Elliott CG. Hamman-Rich syndrome revisited [see comments]. Mayo Clin Proc 1990;
65(12):1538-48.
8. Primack SL, Hartman TE, Ikezoe U, Akira M, Sakatani M, Muller NL. Acute interstitial pneumonia: radiographic
and CT findings in nine patients [see comments]. Radiology 1993; 188(3):817-20.
NSIP
1. Cottin V, Donsbeck AV, Revel D, Loire R, Cordier JR Nonspecific interstitial pneumonia. Individualization of a
clinicopathologic entity in a series of 12 patients. Am J Respir Crit Care Med 1998; 158(4):1286-93.
2. Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical
significance. Am J Surg Pathol 1994; 18(2):136-47.

3. Kim TS, Lee KS, Chung MP, Han J, Park JS, Hwang JH, Kwon OJ, Rhee OH. Nonspecific interstitial pneumonia
with fibrosis: high-resolution CT and pathologic findings. AJR Am J Roentgenol 1998; 171(6): 1645-50.
BOOP/Organizing Pneumonia
1. Akira M, Yamamoto S, Sakatani M. Bronchiolitis obliterans organizing pneumonia manifesting as multiple large
nodules or masses. AJR Am J Roentgenol 1998; 170(2):291-5.
2. Carlson BA, Swensen SJ, O’Connell EJ, Edell ES. High-resolution computed tomography for obliterative
bronchiolitis. Mayo Clin Proc 1993; 68(3):307-8.
3. Chandler PW, Shin MS, Friedman SE, Myers JL, Katzenstein AL. Radiographic manifestations of bronchiolitis
obliterans with organizing pneumonia vs usual interstitial pneumonia. AJR Am J Roentgenol 1986; 147(5):899-
906.
4. Epler GR, Colby TV, McLoud TC, Carrington CB, Oaensler EA. Bronchiolitis obliterans organizing pneumonia. N
Engl J Med 1985; 312(3):152-8.
5. Gosink RB, Friedman Pd, Liebow AA. Bronchiolitis obliterans. Roentgenologic¬pathologic correlation. Am J
Roentgenol Radium Ther Nucl Med 1973; 11 7(4):81 6-32.
6. Haddock JA, Hansell DM. The radiology and terminology of cryptogenic organizing pneumonia. Br J Radiol 1992;
65(776):674-80.
7. Katzenstein AL, Myers JL, Prophet WD, Corley LS, 3rd, Shin MS. Bronchiolitis obliterans and usual interstitial
pneumonia. A comparative clinicopathologic study. Am J Surg Pathol 1986; 10(6):373-81.
8. Lau DM, Siegel MJ, Hildebolt CF, Cohen AH. Bronchiolitis obliterans syndrome: thin-section CT diagnosis of
obstructive changes in infants and young children after lung transplantation. Radiology 1998; 208(3):783-8.
9. Lee KS, Kullnig P, Hartman TE, Muller NL. Cryptogenic organizing pneumonia: CT findings in 43 patients. AJR
Am J Roentgenol 1994; 162(3):543-6.
10. Lohr RH, Boland BJ, Douglas WW, Dockrell DH, Colby TV, Swensen SJ, Wollan PC, Silverstein MD. Organizing
pneumonia. Features and prognosis of cryptogenic, secondary, and focal variants. Arch Intern Med 1997;
157(12):1323-9.
11. McLoud TC, Epler GR, Colby TV, Gaensler EA, Carrington CB. Bronchiolitis obliterans. Radiology 1986;
159(1)1-8.
12. Muller NL, Cuerry-Force ML, Staples CA, Wright JL, Wiggs B, Coppin C, Pare P, Hogg JC. Differential diagnosis
of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional, and
radiologic findings. Radiology 1987; 162(1 Pt 1):151-6.
13. Muller NL, Staples CA, Miller RR. Bronchiolitis obliterans organizing pneumonia: CT features in 14 patients. AJR
Am J Roentgenol 1990; 154(5):983-7.

Airways Disease: The Movement from
Anatomic to Physiologic Assessment
Assessment of Dyspnea
A Common Clinical Problem
• 55 million adult smokers
• 15 million meet criteria for bronchitis Figure 1-3-1
• 5 million with airway obstruction
• 10 million with asthma
Gordon Snyder
Differential Diagnosis of Airways Obstruction

• Common
➢ Emphysema, bronchitis, bronchiectasis, asthma
• Uncommon
➢ LAM, BO, panbronchiolitis, sarcoid,
alpha-1 deficiency, ABPA
Diseases with Obstructive Physiology
The Changing Role of Imaging

• Diagnosis
• Functional assessment
Why Pulmonary Functions are Insensitive

• PFT’s based on wide range of normal
➢ 80-120% predicted
• Diseases with opposing physiologic processes
• The “silent zone” of the lungs
Small airway tethered to
The “Silent Zone” of the Lungs the pleural surface by alveolar walls
[Figure 1-3-1 and 1-3-2]
“Small Airways”
• Peter Macklem Figure 1-3-2
➢ 1970’s
• No cartilage
➢ <2mm physiologists
➢ 1mm pathologists
• Tethered
➢ fiber skeleton
➢ pleura
Weibel
Mosaic attenuation on an expiratory CT in patient with

constrictive bronchiolitis
Airways Disease 26 Chest Radiology

Diffuse Lung Disease Figure 1-3-3 Figure 1-3-4
Airways [Figure 1-3-3]
• Airways involvement
• Obstructive physiology
• Increased lung volumes
• Decreased attenuation
Airways Disease
Direct Signs [Figure 1-3-4]
• Changes
➢ Airway wall
➢ Airway lumen
• Opacities
➢ Tubular
➢ Nodular
➢ Branching
Airways Disease
Indirect Signs
• Mosaic density
➢ Air trapping
• Subsegmental atelectasis
• Ground glass
Airways involvement at the level of “Tree-in-bud” in a patient with a
Airways Disease the secondary lobule respiratory infection
• Emphysema
• Emphysema and Fibrosis
• Alpha-1 deficiency
• Langherhans Cell Histiocytosis
• Bronchiectasis
• Asthma
• Allergic Bronchopulmonary Aspergillosis
• Sarcoidosis
• Diffuse Panbronchiolitis
• Bronchiolitis Obliterans
• Lymphangioleiomyomatosis
Figure 1-3-5
Emphysema
ATS Definition
• Permanent enlargement of airspaces distal to the terminal
bronchiole, accompanied by destruction of the walls without
obvious fibrosis
Emphysema
Emphysema
Classification [Figure 1-3-5]
• Proximal Acinar
➢ Centrilobular
➢ Resp bronchiole
➢ Cigarette smoke
➢ Upper lobes
• Panacinar
➢ Entire acinus
➢ Alpha-1 deficiency
➢ Lower lobes
• Distal Acinar
➢ Paraseptal
➢ Distal acinus
Cigarette smoke related
➢ Subpleura
emphysema is most severe in the
➢ Pneumothorax
upper lobes
Chest Radiology 27 Airways Disease

Emphysema Figure 1-3-6
Clinical Presentation
• Cough, dyspnea and sputum
production
• Hemoptysis rare
➢ R/O cancer
• Symptomatic air flow obstruction
➢ After age 50, 20-30 years of
smoking
• Cor pulmonale (late) related to
hypoxemia and loss of capillary bed
Emphysema and Cor Pulmonale
Emphysema
Pulmonary Functions
• Important to identify patients at risk
• Reduction in Fev1
➢ Most reproducible
• RV increases followed by TLC
• Volumes and flows Saber trachea
➢ Insensitive to early changes
• Diffusing capacity
➢ Sensitive but non-specific Figure 1-3-7
• Small airways tests
Emphysema
Radiographic Feature
• Hyperinflation
➢ Concave diaphragm
➢ Increased A-P diameter
➢ Retrosternal airspace
• Arterial deficiency pattern
• Bulla
➢ Cystic airspaces > 1cm
• Radiography is insensitive
➢ 41% of moderate disease
➢ 66% of severe disease
Saber Trachea [Figure 1-3-6]
Emphysema and Computed

Tomography [Figure 1-3-7] Typical low attenuation
lesions of emphysema
The Diagnosis of Mild Emphysema
Correlation of CT and Pathology Scores
• HRCT detects emphysema
➢ Before there is airflow
limitation on PFT’s
• HRCT excludes emphysema
➢ Patients with moderate
to severe airflow limitation
Kuwano et al, Am Rev Respir Dis 1990
Early Emphysema

Respiratory Bronchiolitis [Figure 1-3-8] Figure 1-3-8
“Smoker’s Bronchiolitis”
• Common change
➢ all smokers
• Pigmented macrophages
➢ In respiratory bronchioles
➢ Surrounding alveoli
• Upper lobe predominance
• Usually asymptomatic
➢ May cause symptoms
Relationship of RB and Emphysema

• Prospective study
• 111 subjects
➢ Followed for 5 years
✧ Imaged at inception TO and 5 years T1
➢ Smokers, nonsmokers and quitters
• Micronodules at TO predisposes to the development of
emphysema at T1
• Micronodules and emphysema at TO predicts more rapid decline
in lung function
Remy-Jardin, Radiology 2001
Relationship of RB and Emphysema Respiratory bronchiolitis

Remy-Jardin, Radiology 2001
Emphysema and Fibrosis

• 14 patients
• Scanning electron microscopy
• Thick and thin walls
➢ Both fibrotic Figure 1-3-9
Nagai & Thurlbeck, Am Rev Resp Dis 1985

• Normal lung volumes and
• Normal flow rates
• Reduced diffusing capacity
➢ Severe
• Minimal pulmonary reserve

• TLC 119%
• VC 126%
• RV 109%
• FEV1/FVC 88%
• D/Va 28%
Emphysema and Fibrosis [Figure 1-3-9]

Emphysema and fibrosis
Langerhans Cell Histiocytosis

• Almost exclusively cigarette smokers
• Slight male preponderance
• Cough and dyspnea most common
• May be asymptomatic
• Occasional bone lesion

Langerhans Cell Histiocytosis Figure 1-3-10
Histology [Figure 1-3-10]
• Nodular
➢ Interstitial lesions
➢ Located near bronchioles
• Histiocytes, eosinophils,
plasma cells and lymphocytes
• Diagnosis requires
Langerhans cells
➢ Large histiocytes
➢ Folded nuclei
➢ Eosinophilic cytoplasm
• Path DDX
➢ Eos pneumonia, DIP,UIP
Langherhans Cell Histiocytosis

[Figures 1-3-11 and 1-3-12]
The range of findings in Langerhans Cell Histiocytosis
Typical nodules in LCH Cystic lesions in LCH

Figure 1-3-13
Radiographic Features [Figure 1-3-13]
• Varies over time
• Upper lobe
➢ Predominance
• Nodules
➢ 0.5-1.0 cm in upper lobes
➢ Early
• Cysts replace nodules
➢ Later
• Honeycomb lung
• Pneumothorax 15%
• Adenopathy and effusion are unusual
LCH is characterized by low attenuation areas with bizarre shapes

EM and Immunohistochemistry
• Immunoperoxidase staining
➢ CD1a, S-100 protein
• Cells in clusters in interstitium
• EM
➢ X-bodies
➢ Langerhans cell granules Figure 1-3-14
➢ Birbeck granules

Clinical Course
• Clinical resolution
➢ Common
• Radiographic abnormalities
➢ Persist
• Occasional progression
➢ Fibrosis and honeycombing
• May be fatal
➢ Rapid progression
Alpha-1 Antitrypsin Deficiency

Pathophysiology
• 1-2% of emphysema in the US
• Alpha-1 antitrypsin inactivates neutrophil elastase
• Production controlled by 2 genes
• Level of antitrypsin dependent on allele
• ZZ homozygotes most severe
• Smoking accelerates the destruction

Imaging Features
• Radiograph may be normal
• Lower lobe predominance
• Panacinar emphysema
• CT
➢ Upper lobe involvement
➢ Bronchiectasis Alpha-1 antitrypsin deficiency
➢ Airway thickening common
• CT more sensitive
Alpha-1 Antitrypsin Deficiency [Figure 1-3-14]

Figure 1-3-15
Bronchiectasis
Pathophysiology
• Dilatation of bronchi
• Reversible form
➢ Infection
➢ Atelectasis
• Congenital
➢ tracheobronchomalacia
• Post-inflammatory
• Postobstructive
• Fibrotic
➢ IPF
➢ Sarcoid
Williams-Campbell [Figure 1-3-15]

Williams-Campbell

Mounier-Kuhn Syndrome Figure 1-3-16
Bronchiectasis
Postinflammatory
• Primary Ciliary Dyskinesia
➢ Kartagener’s
• Immunodeficiency
• Postinfectious
➢ TB, Measles, pertussis, viral
• Post-toxic bronchitis
➢ gastric acid aspiration
• Immunologic
➢ ABPA
Primary Ciliary Dyskinesia
Post Obstructive Bronchiectasis [Figure 1-3-16] Post obstructive bronchiectasis in a patient with
• Neoplasm mucoepidermoid carcinoma
• Foreign body
• Broncholith
• Lymph node enlargement Figure 1-3-17
Bronchiectasis
• Cough
• Purulent sputum
• Hemoptysis (50%)
• Dyspnea
• Rare
➢ clubbing, brain abscess, amyloidosis
Bronchiectasis
Radiographic Features Upper lobe smoking related
• Prominent markings emphysema
• Crowding of Vessels
• “Tram Tracks” Figure 1-3-18
• Loss of volume
• Cystic spaces
Bronchiectasis
CT Features
• Bronchi in the periphery
• “Signet Rings”
• “Tram Tracks”
• Sensitivity
➢ Collimation
RB-ILD
Emphysema [Figure 1-3-17]
RB/RB-ILD [Figure 1-3-18] Figure 1-3-19
Emphysema and Fibrosis [Figure 1-3-19]
Emphysema and fibrosis

Langherhans Cell Histiocytosis [Figures 1-3-20 to 1-3-22]
Alpha-1 Antitrypsin Deficiency [Figure 1-3-23]
Early LCH nodules Late LCH Cysts and nodules
End-stage LCH Lower lobe predominance in

Alpha-1 antitrypsin
Bronchiectasis
Diffuse Lung Disease

Airways
Asthma
ATS Definition
• Reversible airway disease
• Increased airway responsiveness
• Persistent airflow obstruction occurs in chronic asthmatic
➢ Why?
• 6% in the American population
➢ Rate has doubled in 20 years
➢ Higher incidence in large cities
Asthma
Extrinsic
• Family history atopy
• Early onset <30 years
• Seasonal symptoms
• Increased IGE
• Positive skin tests
• Often remits

Asthma Figure 1-3-24
Intrinsic
• No atopy
➢ Absence of external triggers
• Older age group
• Increased blood eosinophils
• Increased sputum eosinophils
• Fixed airway obstruction
➢ Progressive
Asthma
Pathology
• Airway smooth muscle
➢ Hypertrophy
• Airway wall
➢ Inflammation
➢ Edema
Airway thickening in asthma
• Airway plugging
➢ Mucus Figure 1-3-25
➢ Inflammatory exudate
Asthma
Radiographic Features [Figure 1-3-24]
• Chest roentgenogram
➢ Often normal
• Airway thickening
➢ Chronic disease
• Rapid attenuation of vessels
➢ hypoxemia
• Pneumomediastinum
➢ pneumothorax
• Hyperinflation
➢ Adaptive
➢ Later air trapping
Silva AJR 183 September 2004
Asthma-Hyperinflation [Figure 1-3-25]

Severe hyperinflation in which you
Asthma [Figure 1-3-26] can see the slips of the diaphragm
CT Features as it inverts
• More sensitive than CXR
Figure 1-3-26
• Reversible
➢ Consolidation
➢ Atelectasis
➢ Mucoid impaction
➢ Airway Narrowing
➢ Air Trapping
• Permanent
➢ Bronchiectasis
➢ Emphysema
Mucoid impaction in severe

asthma

Allergic Bronchopulmonary Aspergillosis Figure 1-3-27
Primary Criteria
• Asthma
• Eosinophilia
• Immediate skin test reactivity
• Precipitating antibodies (IgG)
• Elevated serum (IgE)
• Pulmonary infiltrates
• Central bronchiectasis
Allergic Bronchopulmonary Aspergillosis

Presentation & Pathology [Figure 1-3-27]
• Atopic individuals
➢ Most common
• Cystic fibrosis
• Airways filled
➢ Fungus
➢ Inspissated mucous
• Presentation with
➢ Cough, fever
➢ Hemoptysis
➢ Worsening asthma
• Seen in stable asthmatics
ABPA represents a non-invasive
• Good response to steroids
colonization of the airways
Imaging [Figures 1-3-28 and 1-3-29] Figure 1-3-28
• Bifurcating opacities
➢ “Gloved-finger”
➢ Mucous filled airways
• Central Bronchiectasis
• Fleeting infiltrates
• Pleural disease
➢ Uncommon
Sarcoidosis and the Airways

Computed Tomography
• Functional evidence of airways obstruction
➢ Obstructive PFT’s are common
• Endobronchial biopsies find granulomas
• Obstructive physiology correlates with
➢ Decreased attenuation on expiratory scans
(small airways) Mucoid impaction in ABPA
➢ Reticular pattern and advanced fibrotic disease
(large airways)
Hansell et al, Radiology 1998 Figure 1-3-29

Endobronchial Granulomas
Small Airway Distortion
Reticular Pattern and Fibrosis
Central bronchiectasis in ABPA

Diffuse Panbronchiolitis
• Japan most common
➢ Rarely: Korea, China, Europe and North America
➢ HLA BW54
➢ M-F 2:1
• Presentation
➢ Dyspnea
➢ Cough
• Obstructive PFT’s
• Slowly progressive
➢ 15 yr mean survival
• Erythromycin
➢ May not be an antibacterial effect
Pathology
• Discrete nodules
• Early infiltration Figure 1-3-30
➢ Interstitium
➢ Respiratory bronchioles
➢ Alveolar ducts
➢ Foamy histiocyte, lymphocyte and plasma cells
• Late secondary ectasia
➢ Proximal terminal bronchioles
Imaging Early
• Radiography
➢ Nodules 5mm
➢ Hyperinflation
• Computed Tomography
➢ Branching opacities
➢ Mosaic attenuation
Early Diffuse Panbronchiolitis represented by
widespread airways nodules
Diffuse Panbronchiolitis [Figure 1-3-30]
Diffuse Panbronchiolitis Figure 1-3-31

Imaging Late [Figure 1-3-31]
• Radiography
➢ Nodules
➢ Cysts and bulla
➢ Hyperinflation
• Computed Tomography
➢ Bronchiolectasis
➢ Bronchiectasis
Constrictive Bronchiolitis
Introduction
• Confusing Terminology
➢ Obliterative Bronchiolitis
➢ Bronchiolitis Obliterans
➢ Bronchiolitis Obliterans Organizing Pneumonia
✧ Different disease
✧ Cryptogenic organizing pneumonia
• Small Airways
➢ Fibrosis
➢ Inflammation
• Response to
Severe airway involvement in
➢ Inflammatory disorders
panbronchiolitis
➢ Infectious disorders

Constrictive Bronchiolitis Figure 1-3-32
• Cough, dyspnea and malaise
• History
➢ prior infection
➢ exposure
• Hypoxemia
• Airway obstruction
Classification
• Infection
➢ RSV, adenovirus and mycoplasma
• Toxic Inhalation
➢ Ammonia, acid and NO
• Aspiration: gastric acid
• Collagen Vascular: RA
• Organ Transplantation
• Unknown
• Obstruction
➢ Terminal bronchiole Constrictive bronchiolitis
➢ Respiratory bronchioles
• Polyps of fibrosis
• Cellular infiltration
➢ Lymphs
➢ Plasma cells
➢ Histiocytes
Imaging [Figures 1-3-33 and 1-3-34]
• Hyperinflation
➢ Localized
➢ Diffuse
• Discrete nodules Figure 1-3-34
➢ Airway associated
• Mosaic pattern
• Airway thickening
• Bronchiectasis
• Air trapping
Figure 1-3-33
Central bronchiectasis and mosaic

Mosaic attenuation in constrictive bronchiolitis attenuation in constrictive bronchiolitis

Swyer-James Syndrome [Figures 1-3-35 and 1-3-36]
Unilateral hyperlucent lung in a patient with

Swyer-James Swyer-James Syndrome
Swyer-James Syndrome-Adenovirus
Figure 1-3-37
Lymphangioleiomyomatosis
• Exclusively women
• Reproductive years
• Progressive dyspnea
• Chylous pleural effusions
• Hemoptysis
• Massive hemorrhage
Function
• Obstructive defect
• FEV1 is decreased
• TLC and RV increased
• DLCO reduced
• Hypoxemia
• Hypocapnia
Lymphangioleiomyomatosis Figure 1-3-38

Histology
• Smooth muscle proliferation
➢ Disorderly
Typical thin-walled cyst in
➢ Bronchioles, alveolar septa,
lymphangioleiomyomatosis
arteries, veins and lymphatics
• Small air filled cysts
➢ Air trapping
Gross Features [Figures 1-3-37 and 1-3-38]
• Cysts
➢ 0.2-2cm
• Diffuse involvement
• Enlarged thoracic duct
• Enlarged lymph nodes
The upper and lower lobes

are equally involved in LAM

Lymphangioleiomyomatosis Figure 1-3-39
Radiographic Features
• Reticulonodular opacities
➢ Basilar
• Lung volume
➢ Normal
➢ Increased
• Pleural effusion
➢ 60-75%
• Pneumothorax
• Honeycombing late
Thin-walled cysts and a
CT Features [Figure 1-3-39]
pneumothorax in patient
• Thin-walled cysts
with
➢ More sensitive than plain film
lymphangioleiomyomatosis
• Diffuse
• Bilateral involvement
• Adenopathy
Figure 1-3-40
Lymphangioleiomyomatosis [Figure 1-3-40]
Therapy and Prognosis
• Slowly progressive course
➢ Variable
• Progression
➢ Cor pulmonale
➢ Respiratory insufficiency
• 50-80% 5 year survival
➢ Average survival 10 years
• Hormonal therapy
➢ Oophorectomy, progesterone
Tuberous Sclerosis [Figure 1-3-41]

Minimal disease in LAM may be difficult may be
Emphysema difficult to separate from emphysema

Figure 1-3-41
Asthma [Figure 1-3-42]

Figure 1-3-42
LAM may represent partial expression of tuberous

sclerosis
Asthma

ABPA [Figure 1-3-43] Figure 1-3-43
Sarcoidosis [Figure 1-3-44]
Diffuse Panbronchiolitis [Figure 1-3-45]
Constrictive Bronchiolitis [Figure 1-3-46]
Swyer-James Syndrome
LAM [Figure 1-3-47]
Physiologic Measurement
An Integral Part of Imaging
• Imaging provides physiologic information
➢ not available from pulmonary functions
ABPA
• Air content and blood flow can be quantified
Airways Disease Figure 1-3-44

• Emphysema
• Emphysema and Fibrosis
• Alpha-1 deficiency
• Histiocytosis-X
• Bronchiectasis
• Asthma
• Allergic Bronchopulmonary Aspergillosis
• Sarcoidosis
• Diffuse Panbronchiolitis
• Bronchiolitis Obliterans
• Lymphangioleiomyomatosis
Sarcoidosis airways involvement

Diffuse Panbronchiolitis Mosaic attenuation in

constrictive
bronchiolitis
Typical findings in LAM

Figure 1-3-47

Diffuse Lung Disease
Airways
References
General
1. Hartman T, Primack 5, Lee K, Swensen S, Muller N. CT of bronchial and bronchiolar diseases. RadioGraphics 1994;
14:991-1003.
2. Hogg JO, Macklein PT, Thurlbeck WM. Site and nature of airway obstruction in chronic obstructive lung disease. N
Engl J Med 1968; 278(25):1355-60.
3. King GO, Muller NE, Pare PD. Evaluation of airways in obstructive pulmonary disease using high- resolution computed
tomography. Am J Respir Crit Care Med 1999; 159(3):992-1 004.
4 Lucidarme O, Coche E, Cluzel P, Mourey-Gerosa I, Howarth N, Grenier P. Expiratory CT scans for chronic airway
disease: correlation with pulmonary function test results. AJR Am J Roentgenol 1998; 170(2)101-7.
5. . Macklem PT. Obstruction in small airways—a challenge to medicine. Am J Med 1972; 52(6):721-4.
6. Muller NE, Miller RR. Diseases of the bronchioles: CT and histopathologic findings. Radiology 1995; 196(1):3-12.
7. Naidich D, McCauley Dl, Khouri NF, al e. Computed tomography of bronchiectasis. Journal of Computer Assisted
Tomography 1982; 6:437-444.
8. Neeld DA, Goodman LR, Gurney JW, Greenberger PA, Fink JN. Computerized tomography in the evaluation of
allergic bronchopulmonary aspergillosis. American Review of Respiratory Disease 1990; 142:1200-1205.
9. Snider GE. Distinguishing among asthma, chronic bronchitis, and emphysema. Chest 1985; 87(1, supplement):355-
39S.
10. Stern EJ, Swensen S, Hartman T, Frank M. Ct mosaic pattern of lung attenuation: distinguishing different causes.
American Journal of Roentgenology 1995; 165:813-816.
11. Teel G, Engeler C, Tahsijain J, duCret R. Imaging of small airways disease. RadioGraphics 1996; 16:27-41.
12. Weibel ER, Rachofen H. The Fiber Scaffold of Lung Parenchyma. In: Crystal RG, West JB, eds. The Lung. New York:
Raven Press, 1991; 787-794.
13. Weibel ER, Crystal RG. Structural Organization of the Pulmonary Interstitium. In: Crystal RG, West JB, eds. The
Lung. New York: Raven Press, 1991; 369-380.
14. Worthy SA, Muller NE, Hartman TE, Swensen S, Padley SF, Hansell CM. Mosaic attenuation pattern on thin-section
CT scans of the lung: differentiation among infiltrative lung, airway, and vascular diseases as a cause. Radiology
1997; 205(2):465-70.
Emphysema
1. Bankler AA De Maertelaer V, Keyzer C, Gevenois PA. Pulmonary emphysema: subjective visual grading versus
objective quantification with macroscopic morphometry and thin-section CT densitometry. Radiology 1999; 211(3):851-
8.
2. Coxson HO, Rogers RM, Whittall KP, D'Yachkova Y, Pare PD, Sciurba FC, Hogg JC. A quantification of the lung
surface area in emphysema using computed tomography. Am J Respir Crit Care Med 1999; 159(3):851-6.
3. Gelb AF, Hogg JC, Muller NE, Schein MJ, Kuei J, Tashkin DP, Epstein JD, Kollin J, Green Rh, Zamel N, Elliott WM,
Hadjiaghai E. Contribution of emphysema and small airways in COPD. Chest 1996; 109(2):353-9.
4. Kinsella M, Muller NE, Staples C, Vedal S, Chan-Yeung M. Hyperinflation in asthma and emphysema. Assessment
by pulmonary function testing and computed tomography. Chest 1988; 94(2):286-9.
5. Kinsella M, Muller NE, Abboud RT, Morrison NJ, DyBuncio A. Quantitation of emphysema by computed tomography
using a "density mask" program and correlation with pulmonary function tests. Chest 1990; 97:315-321.
6. Klein JS, Gamsu G, Webb WR, Golden JA, Muller NE. High-resolution CT diagnosis of emphysema in symptomatic
patients with normal chest radiographs and isolated low diffusing capacity. Radiology 1992; 182(3):817-21.
7. Kondoh Y, Taniguchi H, Yokoyama S, Taki F, Takagi K, Satake I Emphysematous change in chronic asthma in relation
to cigarette smoking: assessment by computed tomography. Chest 1990; 97:845-849.
8. Kuwano K, Matsuba K, Ikeda T, Murakami J, Araki A, Nishitani H, Ishida T, Yasumoto K, Shigematsu N. The diagnosis
of mild emphysema. Correlation of computed tomography and pathology scores. Am Rev Respir Dis 1990; 141(1):169-
78.
9. Miller RR, Muller NE, Vedal S, Morrison NJ, Staples CA. Limitations of computed tomography in the assessment
of emphysema. American Review of Respiratory Disease 1989; 139:980-983.
10. Muller NE, Thurlbeck WM. Thin-section CT, emphysema, air trapping, and airway obstruction [editorial;comment].
Radiology 1996; 1 99(3):621 -2.
11.Muller NE, Staples CA, Miller RR, Abboud RT. Density Mask” An objective method to quantitate emphysema using
computed tomography. Chest 1988; 94:782-787.

12. Nagao M, Murase K, Yasuhara Y, Ikezoe J. Quantitative analysis of pulmonary emphysema: three-dimensional fractal
analysis of single-photon emission computed tomography images obtained with a carbon particle radioaerosol. AJR
Am J Roentgenol 1998; 171(6):1657-63.
13. Park KJ, Bergin CJ, Clausen JE. Quantitation of emphysema with three-dimensional CT densitometry: comparison
with two-dimensional analysis, visual emphysema scores, and pulmonary function test results. Radiology 1999;
211(2):541-7
14. Remy-Jardin M, Remy J, Gosselin B, Becette V, Edme J. Lung parenchymal changes secondary to cigarette smoking:
pathologic-ct correlations. Radiology 1993; 186:643-651.
15. Snider GE, Kleinerman J, Thurlbeck WM, Bengali Zl-i. The definition of emphysema. Report of the National Heart,
Blood and Eung Institute, Division of Eung Diseases Workshop. American Review of Respiratory Diseases 1985;
132:182-1 85.
16. Sutinen S, Christoforidis AJ, Klugh GA, Pratt PC. Roentgenologic criteria for the recognitiion of nonsymptomatic
pulmonary emphysema. American Review of Respiratory Disease 1965; 91:69-76.
17. Uppaluri R, Mitsa T, Sonka M, Hoffman EA, McLennan G. Quantification of pulmonary emphysema from lung
computed tomography images. Am J Respir Crit Care Med 1997; 156(1:248-54.
Alpha-1 Antitrypsin
1. Brantly ME, Paul ED, Miller BH, Falk RT, Wu M, Crystal RG, Clinical features and history of the destructive lung
disease associated with alpha-1-antitrypsin deficiency of adults with pulmonary symptoms. Am Rev Respir Dis 1988;
138(2):327-36.
2. Brantly M, Nukiwa T, Crystal RG. Molecular basis of alpha-1-antitrypsin deficiency. Am J Med 1988; 84(6A):13-
31.
3. Guest PJ, Hansell CM. High resolution computed tomography (HRCT) in emphysema associated with alpha-1-
antitrypsin deficiency. Clin Radiol 1992; 45(4):260-6.
4. Kueppers F, Black ER Alphal-antitrypsin and its deficiency. Am Rev Respir Dis 1974; 110(2):176-94.
Eosinophilic Granuloma
1. Brauner MW, Grenier P, Mouelhi MM, Mompoint D, Lenoir S. Pulmonary histiocytosis X: evaluation with high-
resolution CT. Radiology 1989; 172(1):255-8.
2. Friedman PJ, Liebow AA, Sokoloff J. Eosinophilic granuloma of lung. Clinical aspects of primary histiocytosis in
the adult. Medicine (Baltimore) 1981; 60(6):385-96.
3. Lacronique J, Roth C, Battesti JP, Basset F, Chretien J. Chest radiological features of pulmonary histiocytosis X: a
report based on 50 adult cases. Thorax 1982; 37(2):104-9.
4. Moore AD, Godwin JO, Muller NE, Naidich DP, Hammar SR Buschman DE, Takasugi JE, de Carvalho CR. Pulmonary
histiocytosis X: comparison of radiographic and CT findings. Radiology 1989; 172(1):249-54.
5. Stern EJ, Webb WR, Golden JA, Gamsu G. Cystic lung disease associated with eosinophilic granuloma and tuberous
sclerosis: air trapping at dynamic ultrafast high-resolution CT. Radiology 1992; 182(2):325-9.
Asthma
1. Backman KS, Greenberger PA, Patterson R. Airways obstruction in patients with long-term asthma consistent with
irreversible asthma’. Chest 1997; 112(5): 1234-40.
2. Brown RH, Herold CJ, Hirshman CA, Zerhouni EA, Mitzner W. In vivo measurements of airway reactivity using high-
resolution computed tomography. Am Rev RespirDis 1991; 144(1):208-12.
3. Haraguchi M, Shimura S, Shirato K. Morphometric analysis of bronchial cartilage in chronic obstructive pulmonary
disease and bronchial asthma. Am J Respir Crit Care Med 1999; 159(3):1005-13.
4. Kinsella M, Muller NE, Staples C, Vedal S, Chan-Yeung M. Hyperinflation in asthma and emphysema. Assessment
by pulmonary function testing and computed tomography. Chest 1988; 94(2):286-9.
5. Martin J, Powell E, Shore S, Emrich J, Engel EA. The role of respiratory muscles in the hyperinflation of bronchial
asthma. Am Rev Respir Dis 1980; 121(3):441-7.
6. Paganin F, Trussard V, Seneterre E, Chanez R Giron J, Godard R Senac JP, Michel FB, Bousquet J. Chest radiography
and high resolution computed tomography of the lungs in asthma. Am Rev Respir Dis 1992; 146(4):1084-7.
7. Silva CI, Colby TV, Muller NL. Asthma and associated conditions: high-resolution CT and pathologic findings. AJR
Am J Roentgenol. 2004 Sep;183(3):817-24.

1. Neeld CA, Goodman LR, Gurney JW, Greenberger PA, Fink JN. Computerized tomography in the evaluation of allergic
bronchopulmonary aspergillosis. Am Rev Respir Dis 1990; 142(5):1200-5.
1. Aberle DR, Hansell CM, Brown K, Tashkin DP. Lymphangiomyomatosis: CT, chest radiographic, and functional
correlations. Radiology 1990; 176(2):381-7.

2. Chu SC, Horiba K, Usuki J, Avila NA, Chen CC, Travis WD, Ferrans VJ, Moss J. Comprehensive evaluation of 35
patients with lymphangioleiomyomatosis. Chest 1999; 115(4):1041 -52.
3. Corrin B, Liebow PA, Friedman Pd. Pulmonary lymphangiomyomatosis. A review. Am J Pathol 1975; 79(2):348-82.
4. Lenoir 5, Grenier P, Brauner MW, Frija J, Remy-Jardin M, Revel D, Cordier J. Pulmonary lymphangiomyomatosis
and tuberous sclerosis: Comparison of radiographic and thin-section CT findings. Radiology 1990; 175:329-334.
5. Muller NE, Chiles C, Kullnig P. Pulmonary lymphangiomyomatosis: correlation of CT with radiographic and functional
findings. Radiology 1990; 175(2):335-9.
6. Sullivan Ed. Lymphangioleiomyomatosis a review. Chest 1998; 114(6):1689-703.

Inhalational Lung Disease
(Asbestosis and Silicosis)
Pneumonokoniosis
• “It will then be necessary to embrace under a single title all essentially
identical forms of disease
• …the pneumonokoniosis (from Konis, dust) recommends itself” Figure 1-4-1
Zenker 1866 Hematite Mining
Inorganic Dusts
• Silica
• Asbestos
• Coal
• Iron
• Beryllium
Pneumoconiosis
The accumulation of dust in the lungs and the tissue
reaction to its presence
• Dust macules
• Diffuse interstitial fibrosis
• Diffuse alveolar damage
• Alveolar proteinosis
• Giant cell (GIP)
• Granulomatous inflammation
Types and Sizes of Common Aerosols Particles less that 5 microns can be
deposited beyond the conducting
Particle Deposition airways in the alveolar spaces.
Inertial impaction, sedimentation and diffusion [Figure 1-
4-1]
• 10,000-20,000 liters/day
• Deposition related to particle size
• >10 microns deposit in nasopharynx and large airways (100%) Figure 1-4-2
• 1-5 micron particles deposit in lung parenchyma (20%)
Airway Velocity
Inertial impaction, sedimentation and
diffusion
Particle Clearance [Figure 1-4-2]

Cough, tracheobronchial and alveolar
transport
• Most important
➢ Deposition less critical
• Mucociliary escalator
➢ Outer gel, inner liquid sol
• 90% of particles removed within 2 hrs
• Alveolar transport
➢ Dissolution, engulfed by macrophages, removed
to lymphatics
Early Basal Deposition

Macrophages remove small particles to regional
lymph nodes.
Inhalational Lung Disease 44 Chest Radiology

Removal to Lymph Nodes
Physiologic Gradients-Airflow FRC [Figure 1-4-3]
Physiologic Gradients-Airflow TLC [Figure 1-4-4]

Alveoli in the bases are smaller than those in the The smaller alveoli in the bases enlarge to a
apex. greater degree than those in the apex. Therefore
most airflow is directed towards the bases
Physiologic Gradients-Blood Flow [Figure 1-4-5]
Physiologic Gradients-Lymphatic Flow [Figure 1-4-6]

There is increased blood flow and hydrostatic The lymphatics are driven by hydrostatic pressure.
pressure in the dependent vessels Therefore lymphatic flow is best in the dependent
lung.
Removal to Lymph Nodes [Figure 1-4-7] Figure 1-4-7
This explains the

tendency for chronic
diseases to be upper
lobe
Chest Radiology 45 Inhalational Lung Disease

Tuberculosis Figure 1-4-8
Silicosis
Mineralogy
• Silicon
➢ Element
• Silica (SiO2)
➢ Mineral
• Silicone
➢ Synthetic polymer
Figure 1-4-9
Adenopathy with peripheral calcification is a

hallmark of silicosis
Figure 1-4-10
Nodules with an upper lobe predominance is

typical
Figure 1-4-11
Silicoproteinosis is an acute lower lobe process
Silicosis predisposes a patient to having active

tuberculosis

Silicosis Figure 1-4-12
Epidemiology
• Occupational exposure predominates
➢ 3 million workers
• Mining, stonecutting, engraving and foundry work
• Males more commonly affected
• Degree of exposure underestimated
• Increased risk of neoplasia and scleroderma
Silicosis
Pathogenesis
• 5 million particles/cubic foot-lower threshold
• 100 million particles/cubic foot-100% affected
• > 5 micron particle removed in nares and upper airways
• 80% of particles removed in hours to days
• Retained particles consistently .5-.7 microns
Silicosis
Pathogenesis
• Macrophages and polys concentrate
• Macrophages generate oxygen-free radicals
• Macrophages generate fibrogenic proteins
• Immune related tissue damage
➢ Rheumatoid factor, ANA and gamma globulin The silicotic nodule is typical
response to inhaled silica
Silicosis [Figures 1-4-8 to 1-4-11]
Clinical manifestations
• Diagnosis
➢ Typical imaging pattern of adenopathy and nodules
➢ Exposure to high concentration of silica
➢ 10-20 years of exposure
Figure 1-4-13
• Simple silicosis
➢ Asymptomatic
• Symptoms with PMF
• Intense exposure
➢ Silicoproteinosis
• TB and cancer
Simple Silicosis
Pathology [Figure 1-4-12]
• Progress to mature nodules: 3 zones
➢ Central dense fibrosis
➢ Mid-zone concentric collagen
➢ Peripheral dust laden cells
Simple Silicosis
Imaging manifestations [Figure 1-4-13]
• Adenopathy common
• Calcification 10-20%
• Calcification 5-10%
➢ Eggshell pattern
Simple Silicosis
Imaging manifestations
• Well-circumscribed nodules
➢ 1-10 mm
• Upper lobe and posterior
➢ Lymphatic gradient
➢ CT more sensitive
• Pleural lesions
➢ Candle-wax or pseudoplaques
Eggshell calcification

Progressive Massive Fibrosis Figure 1-4-14
Pathology
• Conglomeration of nodular lesions
• Pathology definition
➢ 2 cm
• Radiology definition
➢ 1 cm
• Upper lung zones
➢ Posterior
Progressive Massive Fibrosis

Imaging manifestations [Figure 1-4-14]
• Progression after exposure
• May fill entire upper lobe
➢ Posterior
• Usually bilateral
• Carcinoma mimic Progressive massive fibrosis
➢ Solitary
➢ Lower lobe
Figure 1-4-15
• Associated emphysema
➢ Not always smoking related
➢ Scar emphysema
Silicotic Alveolar Proteinosis

• High concentration of particulate silica
• Acute onset
➢ Weeks-months
• Alveoli filled with PAS+ material
• Similar to surfactant
• Type II cell hyperplasia
Silicosis and Tuberculosis [Figure 1-4-16]
Silicosis
Computed tomographic technique
• Thick sections of value in nodular diseases
➢ Small nodules easier to differentiate from vessels
• Thin sections 1-2 mm collimation at 10 mm intervals or 3-5 selected Crazy paving pattern associated
images with prior thick section CT with alveolar proteinosis
• High spatial frequency algorithm
• Supine Figure 1-4-16
• No contrast
Silica and Lung Disease

• Adenopathy
• Nodules
• PMF
• Silicoproteinosis
• Tuberculosis
• Cancer
Tuberculosis in a patient with silicosis

Asbestos Figure 1-4-17 Figure 1-4-18
Introduction
• Group of naturally occurring mineral fibers
➢ Hydrated fibrous silicate
• Flexible and strong
• Corrosion, thermal and electrical resistance
• 500 tons - 3 million tons
➢ 60 years
• 9 million people exposed
➢ Primary (mining)
➢ Secondary (industrial)
➢ Nonoccupational (air)
Nonoccupational Exposure
Asbestos Bodies [Figures 1-4-17 and 1-4-18]

• Indicates exposure
• Transparent fiber core
• Iron and mucopolysaccharide coat
• Predominantly amphiboles
• Longer fibers are coated Asbestos bodies are Asbestos fibers are much
➢ < 20 microns not coated commonly found larger than macrophages
➢ Uncoated fibers are pathogenic in urban dwellers and therefore cannot be
➢ 7-5000 X’s more uncoated fibers removed to regional lymph
• Fibers cannot be removed nodes
• Lower posterior disease
Asbestos
Serpentine: chrysotile Figure 1-4-19 Figure 1-4-20
• 95% of commercial use
• Curly and pliable
• Textile manufacture
• Fragments easily
• Chemically unstable
➢ Dissolves easily
• Less pathogenic
Asbestos
Amphiboles: amosite,
crocidolite, anthophilite,
tremolite and actinolite
• 5% of commercial use
• Straight, broad fiber
• Do not fragment easily
Pleural effusions are the most Rounded atelectasis is usually
• Long fibers (>20 microns)
common early complication of preceded by a pleural effusion
➢ Not cleared
asbestos exposure
• More likely coated
• Higher carcinogenic potential
Figure 1-4-21
Asbestos Related Chest Disease
[Figures 1-4-19 to 1-4-21]
• Pleural effusions
• Pleural plaques
• Round atelectsis
• Pleural thickening
➢ Diffuse
• Mesothelioma
• Asbestosis
• Lung cancer
Asbestosis is a lower
lobe subpleural process

Pleural Plaques Figure 1-4-22
Pathology
• Common autopsy finding
➢ (50-10%)
• Dense bands of collagen
➢ “Basket weave”
• Asbestos bodies absent
• Uncoated fibers in dissolved lung tissue
• Dose response
➢ Between parenchymal asbestos
bodies and presence of plaques
• Pathogenesis uncertain
Pleural Plaques
• Postero-lateral parietal pleura
• Central diaphragm
• Absent
➢ Apices and costophrenic angles
• Almost always bilateral The visceral pleural stripe is best seen between the ribs
• Sharply demarcated
• Millimeters to 10 cm
• May calcify extensively
• Highly suggestive of asbestos exposure
Roberts, AJCP 1971
Figure 1-4-23
Pleural Anatomy [Figure 1-4-22]
Pleural Plaques
Imaging
• Radiography insensitive
➢ (8-40% of autopsy cases)
• Companion shadows
➢ Fat and muscle
• HRCT
➢ Best sensitivity and specificity
Pleural Fat [Figure 1-4-23]
Pleural Plaques [Figure 1-4-24]

Fat may mimic fibrotic pleural disease
Diffuse Pleural Thickening
• Smooth pleural density
➢ CXR: > 25% of the length of the chest wall
➢ CT: 3 mm thick, 8 cm wide, 5 cm craniocaudal
• Posteromedial lower lobes
• Involves costophrenic angle
Figure 1-4-24
• Mediastinal pleural involvement
➢ Rare
➢ Suggests mesothelioma
• Visceral and parietal pleura
➢ Adhesions
• Sequela of prior effusion?
Pleural Effusion
Definition
• History of exposure to asbestos
• Confirmation of effusion
➢ Imaging of thoracentesis
• Absence of other disease related to effusion
• Absence of malignant tumor for 3 years
Epler, JAMA 1982 Visceral pleural plaques

Pleural Effusion Figure 1-4-25
Clinical presentation
• Most common abnormality
➢ First 20 yrs
• 3% prevalence
➢ Asbestos exposed
• Small < 500 ml
• Serosanguinous
• Persist for weeks to 6 months
• 66% asymptomatic
• 28% recur
Pleural Effusion
Differential diagnosis
• Lung cancer
• Tuberculosis
• Benign asbestos effusion
• Mesothelioma
Round Atelectasis
• Described 1928 Loeschke
• Folded lung vs inflammatory reaction
• Associated conditions Round atelectasis is associated with
➢ Asbestos exposure, CHF, infarct, TB and histoplasmosis pleural effusion
• Preceded by effusion
Round Atelectasis
• Irregular fibrous thickening of the visceral pleura
• Extensive pleural folding beneath the fibrosis
• Layers of invaginated pleura bound by fibrous adhesions
• Surrounding lung collapsed or fibrotic
Figure 1-4-26
Menzies, AJSP 1987
Round Atelectasis
Imaging criteria [Figure 1-4-26]
• Well-circumscribed
• Round or oval opacity
• “Comet tail” sign
• Pleural thickening
• Volume loss
Asbestosis
Pathologic definition
• Interstitial fibrosis
➢ Associated with asbestos bodies
• Biopsy
➢ Not the standard of practice
Asbestosis
• Dose-response relationship
• Probable exposure threshold
• Latency period inversely proportional to exposure level
• Latency is several decades
• Cigarette smoke may act synergistically
Round atelectasis

Asbestosis
ATS criteria 1986
• Reliable history of exposure
• Latency of at least 10 years
• Restrictive pulmonary functions
➢ DLco and VC <80%
• Appropriate physical findings
➢ Inspiratory crackles, clubbing
• Chest radiographic abnormalities
➢ ILO perfusion > 1/0 (s, t or u)
Asbestosis
Histology
• Early
➢ Fibrosis of respiratory bronchioles
• Progression
➢ Terminal bronchioles, alveolar ducts and alveolar septa
• Minimum 2 asbestos bodies in area of fibrosis
Craighead, Arch Pathol Lab Med, 1982
Asbestosis
Chest radiography
• Lower lobe
➢ Irregular opacities
➢ Nonspecific
➢ Associated pleural disease
• Large inter-observer variation
➢ Low perfusion
• Normal in 26% of path proven cases
Asbestosis and Cigarette Smoking

Small irregular opacities
• Small opacities are related to
➢ Dust exposure, cigarette smoke, age, radiographic technique and obesity
• Cigarette smoke causes
➢ Interstitial fibrosis
➢ Emphysema
➢ Bronchiolar thickening
• Asbestos causes
➢ Interstitial fibrosis
• Asbestos workers who smoke
➢ Have more opacities
➢ Related to dust exposure and cigarettes
Figure 1-4-27
Asbestosis
High-resolution CT
• Lower lobe and posterior
➢ Parenchymal bands
➢ Curvilinear subpleural line
➢ Interstitial short lines
➢ Honeycombing
• High sensitivity
• Nonspecific
• Specificity increases with # of abnormalities
• Prone imaging is key!
Parenchymal Bands [Figure 1-4-27]

Parenchymal bands in a patient with
asbestosis

Reticulonodular Opacites
Curvilinear Subpleural Line
Short Lines
Honeycombing
Asbestosis vs UIP
• Asbestos exposure in the last 30 years is low
• Clinical asbestosis requires substantial exposure
• Asbestos exposed individuals can have other interstitial lung diseases
• Band like opacities merging with the pleura are rare in UIP
• Upper zone fibrosis and ground glass are rare in asbestosis
Gaensler, ARRD, 1991 – Al-Jarad, Thorax, 1992
Asbestosis
High-resolution CT
• Short lines and parenchymal bands are statistically most significant
• Strong association with diffuse pleural disease
• Multifocal
• HRCT finds asbestosis in exposed individuals with normal radiographs and
PFT’s
• Obstructive PFT’s correlate with emphysema
Aberle, AJR, 1988 – Aberle, Radiology, 1988 – Staples, ARRD, 1989
Asbestosis
Dependent density
• Posterior blood flow
➢ 5X’s greater
• Posterior alveoli
➢ Smaller or collapsed
➢ Less steep ventilatory gradient
➢ Closing volumes
(10-40% of VC)
Asbestosis
Computed tomographic technique
• 1.5-2 mm collimation
• 10 mm interval
• High spatial frequency algorithm
• Prone
• Thick section supine: CA screen?
Asbestos Related Chest Disease
Tuberculosis
Silicosis
Asbestosis
Particle Deposition and Clearance

Cough, tracheobronchial and alveolar transport

Pulmonary Lymphoid Disorders
The Pulmonary Lymphoid System

• Lymphatics
• Lymph nodes
• BALT
➢ Bronchus Associated Lymphoid Tissue Figure 1-5-1 Figure 1-5-2
• Lymphoid aggregates
• Lymphocytes
• Dendritic cells
• Langerhans cells
The Pulmonary Lymphoid

System
[Figures 1-5-1 and 1-5-2]
• Lymphatics
➢ Originate in the pleura
➢ Valves
➢ Drain towards hilum
➢ Follow interlobular septa
➢ Accompany blood vessels
• Lymph Nodes
• BALT
• Lymphocytes
• Dendritic cells
One set of lymphatics originate in the Lymphatic channels continue along the
The Pulmonary Lymphoid visceral pleura as demonstrated by the pulmonary veins to the hilum. A second
System lines on the surface of the lung. These set of lymphatics originates near the
[Figures 1-5-3 and 1-5-4] lymphatics enter the lung and follow the center of the secondary lobule and
• Lymphatics interloblular septa in the periphery of the follows the pulmonary arteries
• Lymph Nodes lung
➢ Encapsulated lymph nodes Figure 1-5-3 Figure 1-5-4
✧ Proximal bronchi
✧ Bifurcations
➢ Reactive lymph nodes
✧ Peripheral and septal
✧ Cigarettes or dust
• BALT
• Lymphocytes
• Dendritic cells
Reactive Lymph Nodes
The Pulmonary Lymphoid

System
• Lymphatics
• Lymph nodes
• BALT
➢ Bronchus Associated
Lymphoid Tissue
• Lymphoid aggregates Classic encapsulated intrapulmonary The majority of intrapulmonary lymph
• Lymphocytes lymph nodes are found at the nodes are probably not visible
• Dendritic cells bifurcations of the first 3-4 orders of radiographically. Almost all of these
• Langerhans cells bronchi and are demonstrated adhering lymph nodes are subpleural and
to the right main pulmonary inferior to the carina
Pulmonary Lymphoid Disorders 54 Chest Radiology

BALT – The organizing principle [Figure 1-5-5] Figure 1-5-5
• Lymphoid collections
➢ Bronchial epithelium
➢ Bifurcations
• Absent in the normal adult
➢ Absent at birth
➢ Common in young children
• Reappears with antigenic stimulation
➢ Cigarette smoke
➢ Connective tissue disease
➢ AIDS
• Basis of pulmonary lymphoid disorders
Pulmonary Lymphoid Disorders – Derivations of BALT

• Hyperplasias of BALT
➢ Follicular hyperplasia
✧ Follicular bronchitis
➢ Diffuse hyperplasia
✧ Lymphoid Interstitial Pneumonia
➢ Nodular Lymphoid Hyperplasia
✧ Pseudolymphoma
• Non-Hodgkin’s lymphomas Bronchus associated lymphoid
➢ Low-Grade B Cell lymphomas tissue or BALT is found along the
➢ Lymphomatoid granulomatois bronchiole, interlobular septa and
• Immune impairment pleura. It is normally found only in
➢ PTLD, AIDS and other young children.
Koss, Sem Diag Pathol 1995
Follicular Hyperplasias of BALT – Hyperplasia of BALT

[Figure 1-5-6]
• Follicular bronchitis and bronchiolitis Figure 1-5-6
• Pathologic features
➢ Antigenic stimulation of BALT
➢ Lymphoid aggregates
➢ Peribronchial
➢ Reactive follicles
➢ Minimal alveolar extension
• Clinical
Follicular Hyperplasias of BALT – Hyperplasia of

BALT
• Clinical
➢ Young adults (44 yrs.)
➢ Cough and dyspnea
✧ Fever and weight loss
➢ Immune deficiencies
✧ AIDS
✧ Congenital
➢ Collagen vascular diseases
✧ Sjogren’s
✧ Rheumatoid arthritis
➢ Uncertain Etiology Follicular bronchitis is characterized
✧ Hypersensitivity reactions? by hyperplastic lymphoid follicles with
reactive germinal centers distributed
along bronchioles and to a lesser
extent bronchi.
Chest Radiology 55 Pulmonary Lymphoid Disorders

Follicular Hyperplasias of BALT – Hyperplasia of BALT: Imaging Figure 1-5-7
[Figure 1-5-7]
• Radiography
➢ Diffuse
➢ Reticulonodular
• CT
➢ Nodules 3-12 mm
✧ Centrilobular
✧ Peribronchial
➢ Ground Glass
➢ Air Trapping
Follicular Hyperplasia – Differential Diagnosis Almost all patients with follicular

• Respiratory Bronchiolitis bronchitis have centrilobular nodes
• Hypersensitivity pneumonitis that are less than 3mm. These
• Diffuse panbronchiolitis nodules correlate with the
• Cystic Fibrosis peribronchiolar distribution of
• Primary ciliary dyskinesia hyperplastic lymphoid follicles shown
on the histology section to your left
Diffuse Hyperplasias of BALT – Hyperplasia of BALT
[Figure 1-5-8] Figure 1-5-8
• Lymphocytic Interstitial Pneumonia
• Pathologic Features
➢ Alveolar septal infiltration
✧ Lymphocytes (T-cells)
✧ Diffuse
➢ Lymphoid follicles (B-cells)
✧ Germinal centers
✧ Peribronchial distribution
✧ Spectrum with follicular hyperplasia of BALT (Follicular
Bronchitis)
• Clinical
Diffuse Hyperplasias of BALT – Lymphoid Interstitial

Pneumonia LIP- Hyperplasia of BALT
• Clinical
➢ Women>men
➢ 4th-6th decade
➢ Collagen vascular disease
✧ Sjogrens, RA, and SLE
➢ Bone marrow transplantation LIP is characterized by diffuse
➢ AIDS rare in adults infiltration of the alveolar septa
✧ Common in children
➢ Dysproteinemia
➢ Restrictive lung functions

Diffuse Hyperplasia of BALT – Hyperplasia of BALT: Imaging Figure 1-5-9
[Figures 1-5-9 and 1-5-10]
• Radiography
➢ Lower lung zone
➢ Reticulonodular
• CT
➢ Ground Glass
➢ Nodules
✧ Centrilobular
✧ Poorly defined
➢ Cystic air spaces
➢ Thickened BVB’s
➢ Adenopathy The lung windows demonstrate
diffuse hazy ground glass that
LIP vs Lymphoma correlates with diffuse alveolar wall
LIP Lymphoma thickening. The alveolar wall
Cysts 82% 2% thickening is primarily the result of
Consolidation 18% 66% lymphoid infiltation
Large Nodules 6% 41%
Effusions 0% 25%
Figure 1-5-10
Nodular Lymphoid Hyperplasia – Hyperplasia of BALT
[Figure 1-5-11]
• Pseudolymphoma
➢ Solitary, subpleural mass
➢ Lymphoid proliferation
✧ Interstitial
✧ Perivascular
✧ B and T cells
✧ Polyclonal pattern
– Benign
➢ Reactive germinal centers
➢ Difficult to separate from lymphoma Thin walled cysts are often found
• Clinical deep within the lung parenchyma.
Previous reports have suggested that
Nodular Lymphoid Hyperplasia – Hyperplasia of BALT airway narrowing or obliteration
• Pseudolymphoma results in these cystic lesions. The
• Pathologic features histology on the left demonstrates
• Clinical complete obliteration of the
➢ Rare entity bronchiole by lymphoid infiltration.
➢ Most cases were The accompanying arteriole is
lymphomas identified by its typical wall.
✧ Monoclonal B
cell proliferation
➢ Middle age
✧ Asymptomatic
➢ Autoimmune Figure 1-5-11
Diseases 15%
✧ Sjorgren’s Nodular lymphoid hyperplasia or
✧ SLE pseudolymphoma presents as a
✧ Transverse solitary subpleural mass of
myelitis lymphoid tissue with numerous
➢ Surgical excision reactive germinal centers
curative

Nodular Lymphoid Hyperplasia Figure 1-5-12
Hyperplasia of BALT: Imaging
[Figures 1-5-12 and 1-5-13]
• Radiography
➢ Solitary Nodule
➢ Focal Consolidation
• CT
➢ Air bronchograms
✧ 100%
➢ Indistinct margins
➢ Occasionally multiple
➢ Adenopathy and/or effusion suggests lymphoma
Pulmonary Lymphoid Disorders The CT demonstrates the typical sub-

Derivations of BALT pleural, solitary lesion with indistinct
• Hyperplasias of BALT margins. The bulk of the lesion
• Non-Hodgkin’s lymphomas consists of a mass of lymphoid tissue
➢ Low-Grade B Cell lymphomas with multiple reactive germinal
➢ Lymphomatoid granulomatosis centers.
• Immune impairment
Low-Grade B-Cell Lymphoma Figure 1-5-13

➢ Lymphocytic infiltration
➢ Small lymphocytes
✧ Alveolar wall
✧ Peribronchiolar
✧ Perivascular
➢ Immunologic evidence of malignancy
✧ Monoclonality
✧ B-cell markers CD20
➢ Germinal Centers
Low-Grade B-Cell Lymphoma Air bronchograms are universally

• Clinical present and the lymphoid infiltration
➢ Similar presentation to nodular lymphoid hyperplasia gradually diminishes resulting in the
➢ 5th-6th decade classical indistinct margin
➢ Male=Female
➢ Asymptomatic 50% Figure 1-5-14
➢ 5 year survival 85-95%
➢ Surgical resection
✧ Rare recurrence
Low-Grade B-Cell Lymphoma

[Figure 1-5-14
• Imaging
➢ Radiography
✧ Solitary nodule/mass
– Multiple
✧ Consolidation
✧ Air bronchogram
– 50% Grossly low grade B-cell lymphoma
✧ Slow Growth usually presents as a single white tan
➢ CT lesion that can be either well
✧ Consolidation circumscribed or indistinct. This is
✧ Air bronchograms well demonstrated by the gross
✧ Airway narrowing or “stretching” specimen on the left from the AFIP
archive. The disease can, however,
be multifocal as shown on the right
and has been reported as a primarily
endobronchial lesion.

Primary Tracheal Lymphoma Figure 1-5-15
• Extremely rare
• BALT derivative
• Extensive at diagnosis
• Potentially curable
Lymphomatoid Granulomatosis
[Figure 1-5-15]
➢ Majority of cases are B-cell lymphomas
➢ Reactive small T-cells
➢ Malignant B-cells
✧ Majority of infiltrate
➢ Epstein-Barr Virus
➢ Angiocentric infiltration
➢ Necrosis
✧ Peribronchovascular
✧ Peripheral
• Clinical
➢ 7-85 years (mean 48 yrs)
➢ Male:Female (2:1) Lymphomatoid Granulomatosis is an
➢ Malaise and weight loss angiocentric B-cell lymphoma which
➢ Lung involvement 100% often demonstrates areas of necrosis.
➢ Skin 39-53%
✧ Nodules, ulcers and rash Figure 1-5-16
➢ CNS 37-53%
➢ Renal 32-40%
➢ High mortality rate 53-90%
➢ Most proceed to lymphoma
• Imaging
➢ Nodules 80%
✧ Multiple
✧ Bilateral (80%)
➢ Mid and lower lobes
➢ Cavitation 20%
➢ Large masses Chest CT on the left demonstrates a
✧ Correspond to infarcts bronchovascular distribution of
➢ Diffuse reticulonodular opacities nodules that are shown to be areas of
➢ Hilar adenopathy 25% infarction on gross examination.
LYG [Figure 1-5-16]
Pulmonary Lymphoid Disorders – Derivations of BALT

• Non-Hodgkin’s lymphomas
➢ Posttransplantation Lymphoproliferative Disease (PTLD)
➢ AIDS
➢ Other forms of prolonged immune suppression

Lymphoma and Immune Impairment [Figure 1-5-17] Figure 1-5-17
➢ B-cell non-Hodgkin’s lymphoma
✧ Driven by Epstein-Barr Virus infection
✧ Diffuse polyclonal expansion
– Reduced T-cell control
✧ Malignant transformation
Lymphoma and Immune Impairment

• Clinical
➢ Spectrum of benign to malignant
✧ Infectious mono-like
✧ PTLD polymorphic
✧ PTLD monomorphic
➢ Cyclosporin shortens induction (<1 year)
➢ May respond to reduction in
immunosuppression, anti-virals and surgery
➢ Chemotherapy should be avoided
➢ Heart-lung up 20%

• Imaging
➢ Nodules
✧ May cavitate
✧ Halo
✧ Along bronchovascular bundles
➢ Lymph node
➢ Ground glass
➢ Septa thickening
➢ Consolidation
➢ Effusion Post transplant lymphomas are driven by Epstein-
Barr virus, reduced T-cell surveillance and
PTLD malignant transformation. Genetic mutation may
[Figure 1-15-18] eventually result in malignant transformation of
one of these clones, represented in purple.
Bone Marrow Transplant
Prolonged Chemotherapy
Figure 1-5-18
Pulmonary Lymphoid Disorders
Derivations of BALT
• Non-Hodgkin lymphomas
➢ Posttransplantation Lymphoproliferative Disease
PTLD
➢ AIDS
➢ Other
58 year old male with alpha 1 antitrypsin

deficiency who underwent a double lung transplant
7 years prior to presenting with shortness of
breath, cough, fever and chills for 2 months. The
CT reveals multiple multiple indistinct nodules in a
characteristic distribution along the
bronchovascular bundles

Follicular Hyperplasia [Figure 1-5-19] Figure 1-5-19
Diffuse Hyperplasia of BALT-LIP [Figure 1-5-20]
Nodular Lymphoid Hyperplasia [Figure 1-5-21]
Low-Grade B-Cell Lymphoma [Figure 1-5-22]
LYG [Figure 1-5-23]
Figure 1-5-20 Follicular Bronchitis.
Figure 1-5-21
Lymphocytic Interstitial Pneumonia (LIP).
Figure 1-5-22 Nodular Lymphoid Hyperplasia which was

formerly known as Pseudolymphoma.
Figure 1-5-23
Low grade B-cell Lymphoma

Immune Impairment-PTLD [Figure 1-5-24] Figure 1-5-24
BALT - The organizing principle

• Lymphoid collections
• Basis of pulmonary lymphoid disorders
Post Transplant Lymphoproliferative

Disorder
References
General
1. Koss MN. Pulmonary lymphoid disorders. Semin Diagn Pathol. 1995 May;12(2):158-71.
2. Travis WD, Galvin JR.Non-neoplastic pulmonary lymphoid lesions. Thorax. 2001 Dec;56(12):964-71.

Angiitis and Granulomatosis
Angiitis and Granulomatosis

• First characterized by Averill Liebow 1973
• Angiitis
➢ Cellular infiltration of blood vessel
• Granulomatosis
➢ Necrosis of lung parenchyma not related to blood vessel occlusion
Angiitis and Granulomatosis: Current List

• Wegener’s granulomatosis
• Churg-Strauss syndrome
➢ Allergic granulomatosis
• Necrotizing sarcoid granulomatosis
• Bronchocentric granulomatosis
• Lymphomatoid granulomatosis
Angiitis and Granulomatosis: General Concepts

• Etiology remains unknown
• Inflammatory vs. lymphoproliferative
• Clinical and laboratory findings key to Dx
• Adequate tissue samples are important
• Must R/O infection: mycobacterial or fungal
Pathogenesis of Vasculitis
Angiitis and Granulomatosis: Differential

Multiple vessel associated nodules [Figure 1-6-1]
• Metastatic disease
➢ Squamous
• Multifocal infection
Figure 1-6-1
➢ Fungus, TB, bacteria
• Septic emboli
• Multiple pulmonary infarcts
• Langerhans’ cell histiocytosis
• Rheumatoid nodules
Wegener’s Granulomatosis: Classic Pathology Triad

• Vasculitis described 1852
➢ Von Rokitansky
• Wegener described 1936
➢ Wegener’s
• Focal vasculitis of
➢ Arteries and veins
• Necrotising granulomas
➢ Upper and lower airways
• Necrotising glomerulitis
➢ Focal
Vasculitis in the lung fits into the

differential of vessel associated
nodules
Chest Radiology 63 Angiitis and Granulomatosis

Wegener’s Granulomatosis: Gross Pathology Figure 1-6-2
• Necrotic nodules
➢ With and without cavitation
• Parenchymal consolidation
• Massive hemorrhage
• Airway narrowing
Wegener’s Granulomatosis: Demographics

• Rare
➢ 3/100,000 in US
• 2nd-8th decades of life
• Average age-50 years
• Male=Female
➢ Slight male predominance (4:3)
• May occur in children
Wegener’s Granulomatosis: Limited

• Involvement of lungs alone
• Clinical sparing
➢ Kidneys
➢ Upper respiratory tract
• Biopsy positive
➢ When clinically normal
Solid and cavitary nodules often
coexist in patients with Wegener’s
granulomatosis
Wegener’s Granulomatosis: Clinical Presentation
• Classic triad
Figure 1-6-3
➢ Sinusitis
➢ Pulmonary symptoms
➢ Renal insufficiency
• Variable onset and course
• Chronic URI symptoms
➢ May persist for years before pulmonary disease
• Overwhelming vasculitis
➢ Diffuse
Upper Airway
• Chronic nasal obstruction
➢ Chronic discharge
• Destruction of cartilaginous nasal septum
• “Saddle nose deformity”
• Laryngeal involvement
➢ Subglottic stricture
• Eustachian tube obstruction
• Otitis media
• Cochlear nerve vasculitis
Pulmonary
• Most commonly affected (94%)
• Multiple bilateral nodules or masses
Airway narrowing is a common
• Cavitation common (30-50%)
complication
• Occasionally solitary mass or nodule
➢ Dx difficult
➢ All patients progress
• Less common
➢ Diffuse alveolar hemorrhage
• Pleural lesion and effusions are rare
Angiitis and Granulomatosis 64 Chest Radiology

Renal Figure 1-6-4
• Tempo: insidious to explosive
• Segmental necrotizing glomerulonephritis
• UA: erythrocyte casts and proteinuria
• Large vessel vasculitis
Wegener’s Granulomatosis: Other Organ Involvement

• Skin (50%)
➢ Symmetric papulonecrotic lesion of extremities
• Eye and orbit (30%)
➢ Scleritis, conjunctivitis, optic nerve and retro-orbital mass
• Nervous system (30%)
➢ Mononeuritis multiplex
• Joints
➢ Acute arthritis follows activity of disease (+RA latex)
Wegener’s Granulomatosis: Airway Involvement

• Endobronchial abnormalities
➢ 59% bronchoscopy
• Subglottic stenosis
• Tracheobronchitis
➢ Ulcerating
• Tracheal or bronchial stenosis
Focal airway narrowing is a common
• Often multifocal
complication in Wegener’s
➢ Variable length of involvement
• CT key for evaluation
➢ CXR often normal
Wegener’s Granulomatosis: Radiography

• Earliest lesions
➢ Bilateral reticulo-nodular opacities Figure 1-6-5
• Multifocal nodules
➢ Bilateral
➢ 5mm-10cm
• Sharply marginated
• Cavitation 20-50%
• Evolution
➢ Thick walls to thin walled cysts with treatment
• Airspace consolidation
Changing Presentation
Necrosis and Hemorrhage [Figure 1-6-6]

Figure 1-6-6
Collapse due to airway narrowing in

Wegener’s
Massive necrosis and

hemorrhage in Wegener’s

Evolution with Treatment [Figures 1-6-7 and 1-6-8]
Infections are a common complication
Nodules in varying stages Figure 1-6-9

Wegener’s Granulomatosis:
Computed Tomography [Figure 1-6-9]
• Feeding vessels
➢ 88%
• Cavitation
➢ Nodules greater than 2cm
• Subpleural location
➢ Predominant
• CT “halo sign”
• Pleural based lesions
➢ Mimic infarcts
• Reveals more nodules
Diffuse Pulmonary Hemorrhage: Capillaritis

[Figure 1-6-10]
• Common Figure 1-6-10
➢ Microscopic
polyangiitis
➢ Wegener’s
granulomatosis
➢ SLE
• Uncommon Nodules with feeding vessels are
➢ Goodpastures common in vasculitis
✧ Anti-GBM
➢ Collagen vascular
➢ Idiopathic pulmonary
hemorrhage
➢ Churg Strauss
syndrome
➢ Behcet’s syndrome
➢ IgA Nephropathy
Pulmonary hemorrhage in capillaritis

Microscopic Polyangiitis
[Figure 1-6-11]
• Microscopic polyarteritis nodosa
• Most common cause of pulmonary-renal syndrome
• 5th decade
• Male > Female
• Renal, muskuloskeletal, pulmonary, GI and cutaneous
Figure 1-6-11
Microscopic
polyangiitis
Wegener’s Granulomatosis: Laboratory
• ANCA
➢ Serum Antineutrophil Cytoplasmic Autoantibody
• c-ANCA cytoplasmic pattern
➢ Proteinase 3
➢ 99% specificity and 96% sensitivity in active disease
➢ Positivity drops to 30% in remission
• p-ANCA perinuclear pattern
➢ Reacts with myeloperoxidase
➢ positive in collagen vascular diseases
Wegener’s Granulomatosis: Treatment and Prognosis

• Universally fatal without treatment
• Trimethoprim/Sulfa effective in localized disease
• Steroids and cyclophosphamide
➢ Remission in 93%
• 5 year survival 90-95%
• Infectious complications
➢ Relapse and drug toxicity require close monitoring and follow-up imaging
• Relapse has different manifestations from presentation
Churg-Strauss Syndrome: Allergic Angiitis and Granulomatosis

• Described by Churg and Strauss
➢ 1951
• True systemic vasculitis
• Associated
➢ Asthma
➢ Allergic rhinitis
➢ Blood eosinophilia
• Hypersensitivity response to inhaled antigen?

Churg-Strauss Syndrome: Pathology
• Necrotizing vasculitis
• Eosinophilic tissue infiltration
• “Allergic granulomas”
➢ Extravascular
➢ Eosinophils
➢ Multinucleated giant cells
Churg-Strauss Syndrome: Demographics

• 2nd-4th decades
• 28 years mean age of onset
• Male=Female
• Excellent response to steroids
Churg-Strauss Syndrome: Background

• Late onset asthma
➢ 100%
• Precedes CSS by weeks to years (30)
• Severe rhinitis and sinusitis
➢ 70%
Churg-Strauss Syndrome: Prodromal Stage

• Infiltration of tissues with eosinophils
• Blood eosinophilia
• Elevated IgE
• + rheumatoid factor
• Progressive asthma, sinus pain, myocardial involvement
• Loffler’s like fleeting infiltrates
• Abdominal pain
➢ Diarrhea and eosinophilic peritonitis
• Myalgias and neuritis
Churg-Strauss Syndrome: Vasculitic Stage

• Increasingly severe and widespread symptoms
• Lung
➢ Eosinophilic consolidation, miliary to 2 cm nodules (without cavitation), and
diffuse hemorrhage
• Cardiac
➢ Coronary vasculitis and eosinophilic myocarditis (50% of mortality)
• GI
➢ Ulcerations, perforations and peritonitis
Churg-Strauss Syndrome: Computed Tomography

• Parenchymal opacification
➢ Predominantly peripheral 59%
➢ Effusions
• Nodules
➢ 12%
• Bronchial thickening
• Dilatation
➢ 12%
• Interlobular septal thickening
➢ 6%
Worthy et. Al. AJR Feb. 1998
Churg-Strauss Syndrome: Comparison with Wegener’s

• CSS
➢ High incidence of asthma
➢ High incidence of cardiac involvement (47%)
➢ Less severe renal and sinus disease
➢ Associated with P-ANCA

Churg-Strauss Syndrome: Therapy and Prognosis
• Prognosis relates to early diagnosis and therapy
• High dose steroids usually effective
• Cyclophosphamide in resistant cases
• Therapy stopped after 6-12 months of remission
Necrotizing Sarcoid Granulomatosis

How is this related to sarcoidosis?
• A distinct entity?
➢ Katzenstein
• Some reported cases are undiagnosed infections
• Those with extrapulmonary involvement
➢ Sarcoidosis
Necrotizing Sarcoid Granulomatosis: Demographics Figure 1-6-12

• 3rd to 7th decades
• Mean age 49 years
• Female:male
➢ 2.2:1
Necrotizing Sarcoid Granulomatosis: Pathology

• Non-caseating granulomas
➢ Similar to sarcoidosis
• Vasculitis
➢ Pulmonary arteries
➢ Pulmonary veins
➢ Found in areas away from parenchymal granulomas
• Coagulative necrosis
➢ Widespread
➢ Main distinction from sarcoidosis
Necrotizing Sarcoid Granulomatosis:

• 100% lung involvement
• Cough most common symptom
• Chest pain, fever and dyspnea
• Weight loss and fatigue
➢ 15-40%
• Rare extrapulmonary involvement
➢ 13%
• Aspergillus antigens in some patients
Koss et al, Human Pathology 1980
Necrotizing Sarcoid Granulomatosis: Imaging

[Figure 1-6-12]
• Hilar adenopathy
➢ Variable
➢ Up to 79%
• Nodules
➢ Cavitation is common
➢ Subpleural
➢ Perivascular
• Parenchymal opacities
➢ Same distribution
Necrotizing Sarcoid Granulomatosis:

Prognosis and Therapy
• May require no therapy Typical nodules in NSG
• Prompt response to steroids
• No reported deaths

Lymphomatoid Granulomatosis: Etiology and Demographics
• Majority of cases are B-cell lymphomas
• Epstein-Barr Virus
• Reactive small T-cells
➢ Majority of infiltrate
• Malignant B Cells
• Age range
➢ 7-85 years
• Mean age of onset
➢ 48 years
• Male:Female (2:1)
Lymphomatoid Granulomatosis: Pathology

• Angiocentric infiltration
➢ Mixed cell population
✧ Atypical lymphocytes, plasma cells, histiocytes
• Vascular invasion
• Vascular destruction
• Necrosis
➢ Peribronchovascular
➢ Peripheral
Lymphomatoid Granulomatosis: Clinical Presentation

• Lung involvement LYG is a large B-cell lymphoma
➢ 100%
• Skin Figure 1-6-14
➢ 39-53%
➢ Nodules, ulcers and rash
• CNS
➢ 37-53%
• Renal
➢ 32-40%
• Malaise and weight loss
➢ 35%
Lymphomatoid Granulomatosis: Imaging

[Figure 1-6-14]
• Nodules
➢ 80%
➢ Multiple
➢ Bilateral (80%)
• Mid and lower lobes
• Cavitation
➢ 20%
• Large masses
➢ Correspond to infarcts
• Diffuse reticulonodular opacities
➢ 25% Peripheral opacities in LYG
Lymphomatoid Granulomatosis: Treatment and Prognosis

• Mortality rate
➢ 53-90%
• Long term remissions reported
➢ Cyclophosphamide and steroids
• All who fail therapy proceed to develop lymphoma
➢ 12-47%

Bronchocentric Granulomatosis Figure 1-6-15
Clinical and Demographics – Asthmatics
• Average age 22 years
• Tissue manifestation of ABPA
• Dyspnea, cough, fever, malaise and hemoptysis
• Peripheral and tissue eosinophilia
• No extrapulmonary findings
Bronchocentric Granulomatosis
Clinical and Demographics – Non-Asthmatics
• Average age 50 years
• Males=Females
• Fungal infections
➢ Histo, blastomyces, aspergillus
• Mycobacterial infections
• Rheumatoid arthritis
• Idiopathic
Bronchocentric Granulomatosis: Pathology

• Nonspecific reaction
• Early invasion of mucosa
➢ Histiocytes
➢ Eosinophils
Mucoid impaction in patients with
✧ Asthmatics
BCG
➢ Neutrophils
✧ Non-asthmatics
• Secondary involvement of adjacent arteries
• Granulomatous destruction
➢ Bronchial walls
• Bronchopneumonia
➢ Distal to affected airways
Bronchocentric Granulomatosis: Imaging [Figure 1-6-15] Figure 1-6-16

• Most often unilateral
➢ 75%
• Multiple or solitary nodules
• Parenchymal consolidation
➢ Upper lobe predominance
• Associated findings of ABPA
➢ Bronchiectasis
➢ Mucoid impaction
BCG and Tuberculosis
BCG and Aspergillus [Figure 1-6-16]

Bronchocentric Granulomatosis
Treatment and Prognosis
• Asthmatics respond to steroids ABPA may be clinically unsuspected
• Some cases remit without treatment
• Must rule out treatable infection and Wegener’s granulomatosis
Angiitis and Granulomatosis: Differential

Multiple vessel associated nodules
➢ Squamous
• Multifocal infection
➢ Fungus, TB, bacteria
• Septic emboli
• Multiple pulmonary infarcts
• Langerhans’ cell histiocytosis
BCG?
Fungal Infection ?
Angiitis and Granulomatosis: Conclusion

• Churg-Strauss syndrome
➢ Allergic granulomatosis
• Necrotizing sarcoid granulomatosis
• Bronchocentric granulomatosis
• Lymphomatoid granulomatosis
“Until specific causes are found ………we must devise syndromes”

• Etiology
• Prognosis
• Therapy
References
1. Thurlbeck WM, Churg AM, eds. Pathology of the lung, second edition. New York: Thieme Medical Publishers, 1995;
401-435.
2. Godman GC, Churg J. Wegener’s granulomatosis: Pathology and review of the literature. A.M.A. Arch Pathol, 1954;
58(6): 533-553
3. Churg A, Brallas M, Cronin SR, Churg J. Formes frustes of Churg-Strauss syndrome. Chest 1995; 108(2):320-323.
4. Liebow AA. The J. Burns Amberson Lecture: pulmonary angiitis and granulomatosis. Am Rev Respir Dis 1973; 108:1-
18.
5. Travis WD, Fleming MV. Vasculitis of the lung. Pathology: State of the Art Reviews 1996; 4(1): 23-41.
6. Travis WD. Pathology of pulmonary granulomatous vasculitis. Sarcoidosis Vasc and Diffuse Lung Dis 1996; 13:14-
27.
7. Leavitt RY, Fauci AS. Pulmonary vasculitis. Am Rev Respir Dis 1986; 134:149-166.
8. Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener’s granulomatosis: Prospective clinical and therapeutic experience
with 85 patients for 21 years. Ann Intern Med 1983; 98:76-85.
9. Kornblut AD, Fauci AS. Conversations on allergy and immunology; Cutis 1985; 35:27-34.
10. McDonald TJ, DeRemee RA. Wegener’s granulomatosis. Laryngoscope 1983; 93: 220-231.
11. Cordier JF, Valeyre D, Guillevin L, Loire R, Brechot JM. Pulmonary Wegener’s granulomatosis: a clinical and imaging
study of 77 cases. Chest 1990; 97:906-912.
12. Daum TE, Specks U, Colby TV, et al. Tracheobronchial involvement in Wegener’s granulomatosis. Am J Respir Crit
Care Med 1995; 151: 522-526.
13. Aberle DR, Gamsu G, Lynch D. Thoracic manifestations of Wegener granulomatosis: diagnosis and course. Radiology
1990; 174:703-709.
14. Nölle B, Specks U, Lüdemann J, Rohrbach MS, DeRemee RA, Gross WL. Anticytoplasmic autoantibodies: Their
immunodiagnostic value in Wegener granulomatosis. Ann Intern Med 1989; 111:28-40.
15. Travis WD, Carpenter HA, Lie JT. Diffuse pulmonary hemorrhage: an uncommon manifestation of Wegener’s
granulomatosis. Am J Surg Pathol 1987; 11(9): 702-708.
16. Staples CA. Pulmonary angiitis and granulomatosis. Radiol Clin North Am 1991; 29(5): 973-982.
17. Kornblut AD, Wolff SM, DeFries HO, Fauci AS. Symposium on granulomatous disorders of the head and neck:
Wegener’s granulomatosis. Otol Clin North Am 1982; 15(3):673-683.
18. Allen NB, Bressler PB. Diagnosis and treatment of the systemic and cutaneous necrotizing vasculitis syndromes. Med
Clin North Am 1997; 81(1): 243-259.
19. Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS. Surgical pathology of the lung in Wegener’s granulomatosis:
review of 87 open lung biopsies from 67 patients. Am J Surg Pathol 1991; 15(4): 315-333.
20. Katzenstein AA, Locke WK. Solitary lung lesions in Wegener’s granulomatosis: Pathologic findings and clinical
significance in 25 cases. Am J Surg Pathol 1995; 19(5): 545-552.
21. Feigin DS. Vasculitis in the lung. J Thorac Imag 1988; 3(1):33-48.
22. Epstein DM, Gefter WB, Miller WT, Gohel V, Bonavita JA. Spontaneous pneumothorax: an uncommon manifestation
of Wegener granulomatosis. Radiol 1980; 135:327-328.
23. Fraser RS, Pare JAP, Fraser PD, eds. Synopsis of diseases of the chest, second edition. Philadelphia: WB Saunders
Company, 1994; 411-419.

24. Farrelly CA. Wegener’s granulomatosis: a radiological review of the pulmonary manifestations at initial presentation
and during relapse. Clin Radiol 1982; 33:545-551.
25. Lee SJ, Berry GJ, Husari AW. Wegener’s granulomatosis presenting as right middle lobe obstruction. Chest 1993;
103(5):1623-1624.
26. Travis WD, Colby TV, Lombard C, Carpenter HA. A clinicopathologic study of 34 cases of diffuse pulmonary
hemorrhage with lung biopsy confirmation. Am J Surg Pathol 1990; 14(12):1112-1136.
27. Wadsworth DT, Siegel MJ, Day DL. Wegener’s granulomatosis in children: chest radiographic manifestations. AJR
1994;163:901-904.
28. McHugh K. Wegener’s granulomatosis in children. [Letter] AJR 1995; 165(3):743.
29. Maguire R, Fauci AS, Doppman JL, Wolff SM. Unusual radiographic features of Wegener’s granulomatosis. AJR
1978; 141:233-238.
30. Maskell GF, Lockwood CM, Flower CDR. Computed tomography of the lung in Wegener’s granulomatosis. Clin
Radiol 1993; 48:377-380.
31. Papiris SA, Manoussakis MN, Drosos AA, Kontogiannis D, Constantopoulos SH, Moutsopoulos HM. Imaging of
thoracic Wegener’s granulomatosis: the computed tomographic appearance. Am J Med 1992; 93: 529-536.
32. Jaspan T, Davison AM, Walker WC. Spontaneous pneumothorax in Wegener’s granulomatosis. Thorax 1982; 37:774-
775
33. Grotz W, Mundinger A, Würtemberger G, Peter HH, Schollmeyer P. Radiographic course of pulmonary manifestations
in Wegener’s granulomatosis under immunosuppressive therapy. Chest 1994;105(2):509-513.
34. Weir IH, Muller NL, Chiles C, Godwin JD, Lee SH, Kullnig P. Wegener’s granulomatosis: findings from computed
tomography of the chest in 10 patients. Can Assoc Radiol J 1992; 43(1):31-34.
35. Kuhlman JE, Hruban RH, Fishman EK. Wegener granulomatosis: CT features of parenchymal lung disease. J Comput
Assist Tomogr 1991; 15(6):948-952.
36. Erzurum SC, Underwood GA, Hamilos DL, Waldron JA. Pleural effusion in Churg-Strauss syndrome. Chest 1989;
95(6):1357-1359.
37. Primack SL, Hartman TE, Lee KS, Müller NL. Pulmonary nodules and the CT halo sign. Radiol 1994; 190:513-515.
38. Connolly S, Manson D, Eberhard A, Laxer RM, Smith C. CT appearance of pulmonary vasculitis in children. AJR
1996; 167:901-904.
39. Foo SS, Weisbrod GL, Herman SJ, Chamberlain DW. Wegener granulomatosis presenting on CT with atypical
bronchovasocentric distribution. J Comput Assist Tomogr 1990; 14(6):1004-1006.
40. Stokes TC, McCann BG, Rees RT, Sims EH, Harrison BDW. Acute fulminating intrapulmonary haemorrhage in
Wegener’s granulomatosis. Thorax 1982; 37:315-316.
41. Dugowson CE, Aitken ML. Unusual presentation of recurrent Wegener’s granulomatosis. Chest 1991; 99(3):781-784.
42. Erzurum SC, Underwood GA, Hamilos DL, Waldron JA. Pleural effusion in Churg-Strauss syndrome. Chest 1989;
95 (6):1357-1359.
43. Amundson DF. Cavitary pulmonary cryptococcosis complicating Churg-Strauss vasculitis. Southern Med J 1992;
85(7):700-702.
44. Buschman DL, Waldron JA, King TE. Churg-Strauss pulmonary vasculitis: high-resolution computed tomography
scanning and pathologic findings. Am Rev Respir Dis 1990; 142:458-461.
45. Liebow AA, Carrington CRB, Friedman PJ. Lymphomatoid granulomatosis. Hum Pathol 1972; 3(4):457-558.
46. Katzenstein AA, Carrington CB, Liebow AA. Lymphomatoid granulomatosis: a clinicopathologic study of 152 cases.
Cancer 1979; 43:360-373.
47. Fauci AS, Haynes BF, Costa J, Katz P, Wolff SM. Lymphomatoid granulomatosis: prospective clinical and therapeutic
experience over 10 years. N Engl J Med 1982; 306(2):68-74.
48. Bragg DG, Chor PJ, Murray KA, Kjeldsberg CR. Lymphoproliferative disorders of the lung: histopathology, clinical
manifestations, and imaging features. AJR 1994; 163:273-281.
49. Fauci AS, Haynes BF, Katz P. The spectrum of vasculitis: clinical, pathologic, immunologic, and therapeutic
considerations. Ann Intern Med 1978; 89(1):660-676.
50. Dee PM, Arora NS, Innes DJ. The pulmonary manifestations of lymphomatoid granulomatosis. Radiol 1982; 143:
613-618.
51. Prenovault JMN, Weisbrod GL, Herman SJ. Lymphomatoid granulomatosis: a review of 12 cases. J Can Assoc Radiol
1988; 39:263-266.
52. Koss MN. Pulmonary lymphoid disorders. Semin Diag Pathol 1995; 12(2):158-171.
53. Guinee D, Jaffe E, Kingma D, Fishback N, et al. Pulmonary lymphomatoid granulomatosis: evidence for a proliferation
of Epstein-Barr virus infected B-lymphocytes with a prominent T-cell component and vasculitis. Am J Surg Pathol
1994; 18(8): 753-764.
54. Hicken R, Dobie JC, Frew E. The radiology of lymphomatoid granulomatosis in the lung. Clin Radiol 1979; 30: 661-
664.
55. Scully RE, Mark EJ, McNeely WF, Ebeling SH. Case records of the Massachusetts General Hospital. New Engl J
Med 1996; 335(20):1514-1521.

56. Niimi H, Hartman TE, Müller NL. Necrotizing sarcoid granulomatosis: computed tomography and pathologic findings.
J Comput Assist Tomogr 1995;19(6):920-923.
57. Warren J, Pitchenik AE, Saldana MJ. Granulomatous vasculitides of the lung: a clinicopathologic approach to diagnosis
and treatment. South Med J 1989; 82(4):481-491.
58. Chittock DR, Joseph MG, Paterson NAM, McFadden RG. Necrotizing sarcoid granulomatosis with pleural involvement:
clinical and radiographic features. Chest 1994; 106:672-676.
59. Weisbrod CL. Pulmonary angiitis and granulomatosis: a review . J Can Assoc Radiol 1989; 40:138-134.
60. Sadoun D, Kambouchner M, Tazi A, et al. Granulomatose necrosante sarcoid-like: à propos de 4 observations. Ann
Med Interne 1994; 145(4) 230-233.
61. Myers JL, Katzenstein AA. Granulomatous infection mimicking bronchocentric granulomatosis. Am J Surg Pathol
1986; 10(5):317-322.
62. Koss MN, Robinson RG, Hochholzer L. Bronchocentric granulomatosis. Hum Pathol 1981; 12(7):632-638.
63. Sulavik SB. Bronchocentric granulomatosis and allergic bronchopulmonary aspergillosis. Clin Chest Med 1988;
9(4):609-621.
64. Berendsen HH, Hofstee N, Kapsenberg PD, Siewertsz Van Reesema DR, Klein JJ. Bronchocentric granulomatosis
associated with seropositive polyarthritis. Thorax 1985; 40:396-397.
65. Clee MD, Lamb D, Urbaniak SJ, Clark RA. Progressive bronchocentric granulomatosis: case report. Thorax 1982;
37:947-949.
66. Felson B, Reeder MM. Gamuts in radiology, second edition. Cincinnati: Audiovisual Radiology of Cincinnati, Inc,
1967: 561-562.
67. Albelda SM, Gefter WB, Epstein DM, Miller WT. Diffuse pulmonary hemorrhage: a review and classification.
Radiology 1985; 154: 289-29

The Pulmonary Complications of
Organ Transplantation
Introduction
• Organ transplant first performed the 1960’s
• Solid organ for vital organ failure
• Hematopoietic stem cell (HSC)
➢ Standard therapy
✧ Malignant, hematologic, autoimmune and genetic diseases
• Not proven in breast cancer
Pulmonary Complications in 40-60%: Multifactorial Cause

• Underlying disease
• Therapy for underlying disease
• Graft-vs-host disease
• Conditioning regimen
➢ Chemotherapy and radiation
Solid Organ Transplant: Typical Schedule [Figure 1-7-1] Figure 1-7-1

Solid organ transplant complications begin predominantly
after the first month post-transplant
Bone Marrow Transplant: Typical Schedule [Figure 1-7-2] Figure 1-7-2

Complications are usually separated into those that
occur before and after there first 100 day
Chest Radiology 75 Pulmonary Complications of Organ Transplantation

Early Pulmonary Complications Figure 1-7-3
• Pulmonary edema
• Fungal infection
• Diffuse alveolar hemorrhage
• Bacterial infection
• Viral infection
➢ CMV and herpes
• Pneumocystis carinii
• Acute graft-vs-host disease
• Idiopathic pulmonary syndrome
➢ ARDS, DAD
Late Pulmonary Complications

• Chronic graft-vs-host
• Obstructive airways disease
➢ Bronchiolitis obliterans
• Organizing Pneumonia
➢ BOOP
• Restrictive ventilatory defect
• Late bacterial infections
➢ Sinopulmonary
• Herpes varicella zoster
Pretransplant Considerations Traditionally, allogeneic

• Residual tumor transplantation used bone marrow
• Occult infection grafts. From 1999-2002 there was a
steady increase in peripheral blood
Operative Technique [Figure 1-7-3] stem cell grafts
• Donor aspiration
➢ General anesthesia
• 150-200 aspirates
• Marrow strained
• Immunocompetent T-cells
➢ Depleted with monoclonal reagents
• Infusion of marrow Figure 1-7-4
➢ 400-800 ml
Immunologic Impact
• Profound neutropenia
• Prolonged depression
➢ Cellular function
➢ Humoral function
• Graft-vs-host
➢ Direct effect
➢ Steroids
Pulmonary Edema [Figure 1-7-4]

• Common complication
• 2nd-3rd week posttransplantation
• Rapid onset
➢ Dyspnea and hypoxemia
• Reticulo-nodular markings
• Fluid overload
➢ Blood products, antibiotics and TPN
• Cardiac
• Renal dysfunction
• Decreased albumin
• Often accompanied by fever
Pulmonary edema in a bone marrow
transplant patient demonstrating
interlobular septal thickening and
enlarged pulmonary veins
Pulmonary Complications of Organ Transplantation 76 Chest Radiology

Fungal Infections Figure 1-7-5
• Up to 45% of BMT patients Fungi normally
➢ 85% mortality invade the lung
• Aspergillus is most common via the airway
• Occurs in the first 30 days posttransplantation
• Symptoms:
➢ Fever, dyspnea, cough, chest pain and hemoptysis
• Predisposition
➢ Prolonged granulocytopenia
➢ Broad spectrum antibiotics
• Nodules
• Early “Halo-Sign”
• Late “Air Crescent”
Fungal Infections [Figures 1-7-5 to 1-7-7]
Invasive Aspergillosis: Diagnosis

• BAL (69%) Figure 1-7-6
• Tissue Biopsy (60%)
• Antigen (83%)
• Computed Tomography (92%)
Caillot, J Clin Oncol. 1997
Imaging and Survival: Invasive

Aspergillosis
Caillot, J Clin Oncol. 1997
Pulmonary Hemorrhage
• 21% of BMT patients
• 12th day posttransplantation
• Neutrophil recovery
• Sudden onset:
➢ Dyspnea, cough, fever and hypoxemia
• Rare hemoptysis Typical infarct with a halo of blood in an aspergillus
• Mortality 50-80% infection
• Radiographic abnormalities before symptoms
• Bilateral ground glass opacities
➢ May be localized
Figure 1-7-7
Air-crescent sign in a patient with recovering cell counts

Pulmonary Hemorrhage [Figure 1-7-8] Figure 1-7-8
Cytomegalovirus Pneumonia
• 10-40% of BMT patients
• 6-12 weeks posttransplantation
• Mortality rate of 85%
• Reactivation of latent virus in 70%
➢ Remainder infected by “CMV positive”
blood products
• Anti-viral therapy improves prognosis
Cytomegalovirus Pneumonia [Figure 1-7-9]

• Bilateral
• Nodules 59%
➢ Alone 3%
Franquet et al AJR Oct 2003
Aspergillus infection may be a cause of pulmonary
Pneumocystis Jiroveci Pneumonia hemorrhage
• <10% of BMT patients
• Effective prophylaxis
➢ Trimethoprim/sulfa
• Rapid progression
• Severe dyspnea
• Bilateral perihilar
• Ground-glass opacities Figure 1-7-9
Noninfectious Pulmonary Complication
• Late-onset Noninfectious Pulmonary Complications
➢ LONIPC’s
➢ After the first 3 months
➢ 10-23% of allogeneic grafts
• Idiopathic pulmonary syndrome
➢ Diffuse alveolar damage (DAD)
• Bronchiolitis obliterans
• Organizing pneumonia
➢ BOOP
• Associate with GVHD
➢ Sicca syndrome
➢ Acive donor T-cells
Sakaida, Blood Vol 102 2003
Typical lower lobe nodules of CMV
Idiopathic Pulmonary Syndrome
• Diffuse lung injury posttransplantation
• Histology
➢ Interstitial mononuclear infiltrate
➢ DAD
• 12% of allogenic BMT
• 40-80 days posttransplantation
• Risk factors
➢ GVHD
➢ Radiation
• Fever, cough and hypoxemia
• Mortality rate of 70%
• Diffuse Opacities

Idiopathic Pulmonary Syndrome Figure 1-7-10
[Figures 1-7-10 and 1-7-11]
Diffuse Alveolar Damage

• Infectious agents
➢ Legionella, mycoplasma, viruses
• Inhalants
➢ Ammonia, chlorine, HS
• Drugs
➢ Cytoxan, BCNU, Bleomycin
• Ingestants
➢ Kerosene, Paraquat
• Shock/trauma
• Sepsis
• Radiation
• Idiopathic
➢ Hammon-Rich or AIP Patients with IPS present with diffuse opacities
involving all 5 lobes
Viral Infection Figure 1-7-11
Idiopathic Pulmonary Syndrome
[Figure 1-7-12]
Graft-vs-Host Disease (GVHD) :

Donor T-lymphocytes recognize the
recipient’s tissue as foreign
• Acute GVHD
➢ 20-100 days posttransplantation
➢ 25-75% of patients
➢ skin, gut and liver dysfunction
➢ 10% mortality
• Chronic GVHD
➢ 1> 100 days posttransplantation
➢ 20-45% of patients
➢ Features of autoimmune diseases
➢ Sjogren’s, scleroderma, biliary
cirrhosis and airway obstruction
GVHD and IPS In the early phase (exudative) there is diffuse consolidation
and ground glass often with peripheral clearing
GVHD and Infection
Figure 1-7-12
GVHD and IPS
Radiation Pneumonitis
• Related to dose of TBI
• Presents within 90 days
• Cough, fever, and dyspnea
• Threshold lowered by chemotherapy
Mediastinal Emphysema
• Correlates
➢ Idiopathic interstitial pneumonia
• Increased likelihood with more radiation
• Not a serious complication by itself
• May be a harbinger of pneumothorax
The late phase of IPS demonstrates traction bronchiectasis

consistent with fibrosis

Secondary Malignancies Figure 1-7-13
• 0.02% incidence
• 7X’s increase
➢ Over the general population
• 1 year after transplantation
➢ Median
• Hodgkin’s 45%
• Leukemia 17%
• Solid tumors 38%

➢ B-cell non-Hodgkin ’s
✧ Driven by Epstein-Barr virus infection
✧ Diffuse polyclonal expansion
– Reduced T-cell control
✧ Malignant transformation
Bone Marrow Transplant
Bronchiolitis Obliterans
• 2-13% of BMT’s Bronchiolitis obliterans
• Low immunoglobulin level demonstrating mosaic
• Chronic GVHD attenuation
➢ Sicca syndrome
Figure 1-7-14
• 100 days posttransplantation
• Gradual deterioration of PFT’S
• Airflow obstruction
➢ Fixed
• Reduction in diffusing capacity
• Imaging
➢ Mosaic attenuation
➢ Expiratory accentuation
➢ Patchy consolidation
Bronchiolitis Obliterans [Figures 1-7-13 to 1-7-15]
Segmental or Lobar Consolidation

• Infection
Diffuse Opacities
• Pulmonary edema Bronchiolitis obliterans demonstrating indistinct
• Hemorrhage nodules and branching opacities
• Viral pneumonia Figure 1-7-15
• Pneumocystis pneumonia
Rapid Progression Over 24 Hours

• Bacterial pneumonia
• Pulmonary edema
• Hemorrhage
Progression Over Days

• Aspergillus
• Pneumocystis
• CMV
Bronchiolitis obliterans demonstrating

bronchiectasis

Diffuse Opacities
Fluid Overload
Organizing Pneumonia
BMTP: dyspnea and cough
BMTP Lymphangitic Spread
BMTP
BMTP Aspergillus
BMTP: dyspnea and cough
BMTP: Edema
Bone Marrow Transplant: Typical Schedule
References
General
1. Franquet T, Muller NL, Lee KS, Gimenez A, Flint JD.High-resolution CT and pathologic findings of noninfectious
pulmonary complications after hematopoietic stem cell transplantation. AJR Am J Roentgenol. 2005 Feb;184(2):629-
37
2. Kotloff RM, Ahya VN, Crawford SW.Pulmonary complications of solid organ and hematopoietic stem cell
transplantation. Am J Respir Crit Care Med. 2004 Jul 1;170(1):22-48. Epub 2004 Apr 7

The Diagnosis of Pulmonary Embolism
Pulmonary Embolus
• Frequent
• Potentially fatal
• Largely undiagnosed
Baglin, J Clin Path, 1997
Pulmonary Embolus: Epidemiology

• 5 million episodes of DVT
• 300,000 embolic events
• 50,000 deaths
• 100/100,000 new cases
The “Clinical Picture” of PE

• Predisposing factors
• Pathology
• Signs and symptoms
• Radiography
• Arterial blood gases
• V/Q scanning
• Computed tomography
• Arteriography
Pulmonary Embolism is a Complication of Deep Venous

Thrombosis
Hull, Annals of Internal Med 1983
Sources of Pulmonary Emboli

• Majority of clots
➢ Lower extremity veins
• Increasing number of clots
➢ Upper extremities, cardiac chambers and catheters
• A negative venous study
➢ Does not rule out PE
• < 50% of PE patients
➢ Positive lower extremity study
Kelly, Ann Int Med, 1991
Embolic Events: Predisposing Causes

• Stasis
• Trauma
• Hypercoagulable states
Predisposing Causes
• 1° thrombophlebitis 39%
• Bed rest 32%
• Recent surgery 31%
• Venous insufficiency 25%
• Recent fracture 15%
• Myocardial infarction 12%
• Malignancy 8%
• CHF 5%
• No Predisposition 6%
Pulmonary Embolism 82 Chest Radiology

PE and Malignancy: 10-15% of unexplained phlebitis
• Gastrointestinal
• Pulmonary
• Genitourinary
The History and Physical are Non-Specific
Symptoms in Patients with Non-Fatal PE

• Chest Pain 88%
• Dyspnea 84%
• Apprehension 59%
• Cough 53%
• Hemoptysis 30%
• Sweats 27%
• Syncope 13%
Signs in Patients with Non-Fatal PE

• RR> 16 92%
• Rales 58%
• HR> 100 44%
• T> 37.8°C 43%
• Diaphoresis 36%
Figure 1-8-1
• Gallop 34%
• Phlebitis 32%
• Murmur 23%
• Cyanosis 19%
PE and Underlying Lung Disease
The History and Physical are Insensitive
We do not know the Prevalence of PE.
Diagnostic Algorithm and Clinical

Suspicion [Figure 1-8-1]
“Among the various causes of an incorrect

diagnosis, most important are: the failure to
suspect PE and, the protean nature of the The clinical diagnosis of PE is unreliable. Many
disease.” patients are symptomatic
Morpurgo, Chest 1995
History and Physical
The Role of Clinical Suspicion

• Less than 35% of fatal emboli were diagnosed antemortem
Symptoms in Patients with Fatal PE

• Dyspnea 59%
• Syncope 27%
• Altered mentation 20%
• Apprehension 17%
• Chest pain 10%
• Sweatiness 9%
• Pleuritic Pain 8%
• Cough 3%
• Hemoptysis 3%
• Arrest 8%
Chest Radiology 83 Pulmonary Embolism

Signs in Patients with Fatal PE
• RR> 16 66%
• HR> 100 54%
• Rales 42%
• T> 37.8°C 30%
• Edema 26%
• Hypotension 20%
• Cyanosis 12%
• Gallop 10%
• Diaphoresis 10%
• Phlebitis 7%
Figure 1-8-2
The Chest X-Ray is Usually Abnormal
The Chest X-Ray in Pulmonary Embolism

• 84% had abnormal radiographs
PE(%) NoPE(%)
➢ Atelectasis/Infiltrate 68 48
➢ Pleural Effusion 48 31
➢ Pleural Opacity 35 21
➢ Elevated Diaphragm 24 19
➢ Decreased Vascularity 21 12
➢ Prominent PA 17 28
➢ Cardiomegaly 12 11
➢ Westermark’s Sign 7 2
➢ Pulmonary Edema 4 13
PIOPED
Common Radiographic Abnormalities

• Infiltrate 54%
• Pleural Effusion 51%
• Atelectasis 27%
• Diaphragm Up 17%
• 2 or More 44%
• CHF 17%
• Focal Oligemia 2%
• Normal 7%
Chest CT Findings
• Atelectasis 100%
• Hampton’s hump 50%
• Pleural Effusions 87%
• Mosaic attenuation
Truong, ARRS, 1998
Radiographic and CT Findings [Figure 1-8-2]
Peripheral opacities may raise

suspicion for clinically unsuspected
PE

Pathology [Figure 1-8-3] Figure 1-8-3
• Edema
• Hemorrhage
• Infarction
Normal Arterial Oxygenation Does Not Exclude

Pulmonary Embolism
Arterial Blood Gases

• 10-15% will have a PO2 >85mm HG
• A low arterial PO2 is non-specific
• A respiratory alkalosis is most common
The Physiology of Pulmonary Embolism

• V/Q abnormalities
➢ Variable
• Complete vascular occlusion
➢ Rare
• Complete shunt 2° to
➢ Atelectasis
➢ Hemorrhage
• Autoregulation
➢ Hypoxic vasoconstriction
➢ Hypocapnic bronchoconstriction Hemorrhage and edema are common
Levy, JAP, 1974 sequela of PE. Infarct is less common
Dantzker, Circulation Res, 1974 and is more likely to occur in patients
Dantzker, Chest Vol. 91 no. 5 with CHF
Physiologic Change with Heparinization

• Ventilation
➢ Returns more rapidly than perfusion
• Perfusion
➢ May return before ventilation
Santolicandro, Am J Res Crit Care Med, 1995
V/Q Physiology
Ventilation/Perfusion Scanning
Clinical Science Probability (%)
80-100 20-79 0-19 All Probabilities
• High 96% 88% 56% 87% (103/118)
• Intermediate 66% 28% 16% 30% (104/345)
• Low 40% 16% 4% 14% (40/296)
• Normal 0% 6% 2% 4% (5/128)
• Total 68% 30% 9% 28% (252/887)
PIOPED
The Basis of “Clinical Science Probability”

• PE(%) No PE(%)
• Dyspnea 73 72
• Pleuritic Pain 66 59
• Cough 37 36
• Leg Swelling 28 22
• Hemoptysis 13 8
• Palpitations 10 18
• Wheezing 9 11
• “Angina” 4 6
PIOPED

Traditional Approach [Figure 1-8-4]
Figure 1-8-4
The Low-Probability Lung Scan

• “There is an 8% mortality rate in patients with a “low probability” V/Q scan and
limited cardiopulmonary reserve.” Hull, Archives of Internal Medicine, 1995
• “There is a 25%-30% disagreement between expert readers in interpreting
INTERMEDIATE and LOW probability V/Q scans.” PIOPED, JAMA, 1990
• A Potentially Lethal Reading
➢ “Pulmonary embolism cannot be diagnosed on clinical grounds; it can only
be suspected.” Bone, Archives of Internal Medicine, 1993
The Goal of Imaging – Visualization of the clot

The Role of Pulmonary CT Angiography
• Initial screening
• Detection of unexpected emboli
• Detection of other pathology
CT Angiography
Pulmonary CT Angiography – Sensitivity and Specificity

• Accurately identifies emboli
➢ Main, lobar and segmental vessels
• Misses some subsegmental emboli
• Indeterminate 8-10%
• Constantly changing
➢ Related to collimation, scanner speed and prevalence
➢ Sensitivity 66-93%
➢ Specificity 89-97%
Eng, AJR 183; 2004
Pulmonary CT Angiography – Sensitivity and Specificity
• 3mm visualizes 40% of subsegmental arteries
• 3mm visualizes 75% of segmental arteries
• 1.25mm visualizes 75% of subsegmental arteries
• 1.23mm visualizes 90% of segmental arteries
Patel, Radiology, 2003
Significance of Small Emboli – Standard Angiography

• Good outcome
➢ Patients with “negative angio”
• 1.5% embolize when followed 1 year
➢ 691 patients
Novelline, Radiology, 1978

Distribution of Pulmonary Emboli
• Multiple locations
➢ > 55%
• Marked preference for
➢ Right lung and lower lobes
• Subsegmental only
➢ 6-30%
PIOPED, JAMA, 1990; Oser, Radiology, 1996; Morpurgo, Chest, 1995
Significance of Small Emboli - CTA

• Small emboli that need treatment
➢ Poor cardiopulmonary reserve
➢ Coexisting acute DVT
➢ Recurrent PE
• Small emboli that may not need treatment
➢ Subsegmental clots without evidence of DVT
➢ Indeterminate scan without evidence of DVT
➢ Normal cardiovascular status
➢ Follow-up DVT scan in 1 week
Goodman, Radiology 234; 2005
Pulmonary CT Angiography
Negative Predictive Value of a Normal CT
• No prospective, consecutive studies
• “The safety of withholding anticoagulants…is uncertain”
Negative Predictive Value of a Normal CT
n Follow-up NPV
Mayo 69 3m 97%
Feretti 109 3m 97%
Garg 78 6m 99%
Loomis 81 6m 100%
Goodmann 198 3m 99%
Remy-Jardin 71 3m 97%
Tillie-Leblond 185 12m 98%
Kavanagh 85 9m 99%
Pulmonary CT Angiography – Intra and Interobserver Variability

• Radiology’s Achilles’ Heel
• Related to clot size
• Exacerbated by poor exam
• Related to reader experience
Mayo, Radiology, 1997; Chartrand-Lefebre, AJR, 1999
• Alternate diagnoses
➢ 11-33%
• Unexpected emboli
➢ 1-4%
Storto, AJR:184 2005

Alternative Diagnoses [Figure 1-8-5 and 1-8-6]
Pulmonary infection is a common alternative

diagnosis is patients suspected of pulmonary
embolus Adenocarcinoma is an important predisposition for
hypercoaguability and PE
Pitfalls in Helical CT [Figure 1-8-7 and 1-8-8]

• Partial volume
➢ Obliquely oriented arteries
• Suboptimal contrast enhancement
• Breathing artifacts
• Lymph nodes
Breathing artifacts should be Adenopathy can mimic PE

assessed before reading a CTA for
PE

Technical Improvements
• Multi-channel CT
• Narrower collimation: 1mm
• Subsecond scanning
• Contrast timing Figure 1-8-9
➢ “Smart prep”
➢ Test bolus: peak + 5 sec
➢ 20 seconds normal cardiac output
➢ Caudal-cranial scanning
• Workstation viewing
➢ Cine Mode (PACS or Workstation)
➢ Adjust window and levels for each case
➢ Multi-planar reconstruction
➢ Breathing artifact-coronal lung windows
16 Channel CT [Figure 1-8-9] Coronal reconstrution helps separate

pulmonary arteries and veins
Paddlewheel Reformation
Simon, AJR:177 July 2001
Combined Pulmonary CTA and Venography

• Increases detection of thromboembolic disease by 20%
• Contiguous sections
• 1cm collimation
Cham, Radiology;234 2005
Combined Pulmonary CTA and Venography

Year N Sensitivity Specificity
Loud 00 150 97% 100%
Duwe 00 74 89% 94%
Garg 00 70 100% 97%
Cham 00 116 100% 96%
Peterson 01 136 71% 93%
A Diagnostic Algorithm for Pulmonary Embolism
Pulmonary Embolus and Prognosis

The prognosis in PE patients is closely related to the
presence and extent of clot in the peripheral veins
Chest Pain-Dyspnea Screening
Conclusion
• The clinical diagnosis of PE is unreliable
• The chest radiograph is usually abnormal
• V/Q readings restricted to “reliable categories”
• Small clots are a problem for all modalities
• Outcome studies are key
• CT angiography is the modality of choice

References
General
1. Robin E, D. Overdiagnosis and overtreatment of pulmonary embolism: the emperor may have no clothes. Annals of
Internal Medicine 1977; 87(6):775-781.
2. Morgenthaler TI, Ryu JH. Clinical characteristics of fatal pulmonary embolism in a referral hospital. Mayo Clinic
Proceedings 1995; 70(5):417-424.
3. Baglin TP, White K, Charles A. Fatal pulmonary embolism in hospitalised medical patients. J Clin Pathol 1997;
50(7):609-10.
4. Goldhaber SZ. Pulmonary embolism. N Engl J Med 1998; 339(2):93-104.
5. Huisman MV, Buller HR, ten Cate JW, van Royen EA, Vreeken J, Kersten M-J, Bakx R. Unexpected high
prevalence of silent pulmonary embolism in patients with deep venous thrombosis. Chest 1989; 95(3):498-502.
6. Patriquin L, Khorasani R, Polak JF. Correlation of diagnostic imaging and subsequent autopsy findings in patients
with pulmonary embolism [see comments]. AJR Am J Roentgenol 1998; 171(2):347-9.
7. Shatz DV. Statewide, population-based, time-series analysis of the frequency and outcome of pulmonary embolus in
318,554 trauma patients [letter; comment]. J Trauma 1998; 44(1):239.
Physiology
1. Dalen JE, Haffajee CI, Alpert JS, Howe JP, Ockene IS, Paraskos JA. Pulmonary embolism, pulmonary hemorrhage
and pulmonary infarction. New England Journal of Medicine 1977; 296(25):1431-1435.
2. Dantzker DR, Bower JS. Clinical significance of pulmonary function tests: alterations in gas exchange following
pulmonary thromboembolism. Chest 1982; 81(4):495-501.
3. Dantzker DR. Ventilation-perfusion inequality in lung disease. Chest 1987; 91:749-754.
4. Santolicandro A, Prediletto R, Fornai E, Formichi B, Begliomini E, Giannella-Neto A, Giuntini C. Mechanisms of
hypoxemia and hypocapnia in pulmonary embolism. Am J Respir Crit Care Med 1995; 152(1):336-47.
Radiography
1. Moses DC, Silver TM, Bookstein JJ. The complementary roles of chest radiography, lung scanning, and selective
pulmonary angiography in the diagnosis of pulmonary embolism. Circulation 1974:179-188.
2. Bynum LJ, Wilson JE. Radiographic features of pleural effusions in pulmonary embolism. American Review of
Respiratory Disease 1978; 117:829-834.
3. Buckner CB, Walker CW, Purnell GL. Pulmonary embolism: chest radiographic abnormalities. Journal of Thoracic
Imaging 1989; 4(4):23-27.
4. Sasahara AA, Hyers TM. The urokinase pulmonary embolus trial-A national cooperative study. Circulation 1973;
47(suppl 2):1-108.
Scintigraphy
1. Alderson PO, Rujanavech N, Secker-Walker RH, Mcknight RC. The role of 133Xe ventilation studies in the
scintigraphic detection of pulmonary embolism. Radiology 1976; 120(633-640).
2. Hirsh J. Diagnosis of venous thrombosis and pulmonary embolism. American Journal of Cardiology 1990; 65:45C-
49C.
3. Hull RD, Hirsh J, Carter CJ, Jay RM, Dodd PE, Ockelford PA, Coates G, Gill G, Turpie AG, Doyle DJ, Buller HR,
Raskob GE. Pulmonary angiography, ventilation lung scanning, and venography for clinically suspected
pulmonary embolism with abnormal perfusion lung scan. Annal of Internal Medicine 1983; 98(6):891-899.
4. Hull RD, Hirsh J, Carter CJ, Raskob GE, Gill GJ, Jay RM, Leclerc JR, David M, Coates G. Diagnostic Value of
ventilation-perfusion lung scanning in patients with suspected pulmonary embolism. Chest 1985; 88(6):819-828.
5. Hull R, Raskob G, Ginsberg J. A noninvasive strategy for the treatment of patients with supected pulmonary
embolism. Archives of Internal Medicine 1994; 154:289-97.
6. Hull RD, Raskob GE, Coates G, Panju AA. Clinical validity of a normal perfusion lung scan in patients with
suspected pulmonary embolism. Chest 1990; 97(1):23-26.
7. Hull R, Raskob G, Pineo G, Brant R. The low-probability lung scan: a need for change in the nomenclature. Archives
of Internal Medicine 1995; 155(1845-1851).
8. Schluger N, Henschke C, King T, Russo R, Binkert B, Rackson M, Hayt D. Diagnosis of pulmonary embolism at a
large teaching hospital. Journal of Thoracic Imaging 1994; 9:180-184.
9. Pioped Investigators. Value of the ventialtion/perfusion scan in acute pulmonary embolism. Journal of The American
Medical Association 1990; 263:2753-2759.
Angiography
1. Novelline R, Baltarowich O, Athanasoulis C, Greenfield A, McKusick K. The clinical course of patient with
suspected pulmonary embolism and a negative pulmonary angiogram. Radiology 1978; 126:561-567.
2. Quinn MF, Lundell CJ, Klotz TA, Finck EJ, Pentecost M, McGehee WG, Garnic JD. Reliability of selective
pulmonary arteriography in the diagnosis of pulmonary embolism. American Journal of Roentgenology 1987;
149:479-471.

3. Stein PD, Athanasoulis C, Alavi A, Greenspan RH, Hales CA, Saltzman HA, Vreim CE, Terrin ML, Weg JG.
Complications and validity of pulmonary angiography in acute pulmonary embolus. Circulation 1992; 85(462-
468).
4. Stein PD, Henry JW, Gottschalk A. Reassessment of pulmonary angiography for the diagnosis of pulmonary
embolism: relation of interpreter agreement to the order of the involved pulmonary arterial branch. Radiology
1999; 210(3):689-91.
Computed Tomography
1. Balakrishnan J, Meziane MA, Siegelman SS, Fishman EK. Pulmonary infarction: CT appearance with pathologic
correlation. Journal of Computer Assisted Tomography 1989; 13(6):941-945.
2. Beigelman C, Chartrand-Lefebvre C, Howarth N, Grenier P Pitfalls in diagnosis of pulmonary embolism with
helical CT angiography. AJR Am J Roentgenol; 1998; 171(3):579-85.
3. Cham MD, Yankelevitz DF, Henschke CI.Thromboembolic disease detection at indirect CT venography versus CT
pulmonary angiography. Radiology. 2005 Feb;234(2):591-4.
4. Coche EE, Muller NL, Kim KI, Wiggs BR, Mayo JR. Acute pulmonary embolism: ancillary findings at spiral CT.
Radiology 1998; 207(3):753-8.
5. Eng J, Krishnan JA, Segal JB, Bolger DT, Tamariz LJ, Streiff MB, Jenckes MW, Bass EB. Accuracy of CT in the
diagnosis of pulmonary embolism: a systematic literature review. AJR Am J Roentgenol. 2004 Dec;183(6):1819-
27.
6. Eyer BA, Goodman LR, Washington L. Clinicians' response to radiologists' reports of isolated subsegmental
pulmonary embolism or inconclusive interpretation of pulmonary embolism using MDCT. AJR Am J Roentgenol.
2005 Feb;184(2):623-8.
7. Falashci F, Palla A, Formichi B, Sbragia P Petruzzelli S, Guintini C, Bartolozzi C. CT evaluation of chronic
thromboembolic pulmonary hypertension. Journal of Computer Assisted Tomography 1992; 16:897-903.
8. Garg K, Welsh CH, Feyerabend AJ, Subber SW, Russ PD, Johnston RJ, Durham JD, Lynch DA. Pulmonary
embolism: diagnosis with spiral CT and ventilation-perfusion scanning—correlation with pulmonary angiographic
results or clinical outcome. Radiology 1998; 208(1):201-8.
9. Gefter W, Hatabu H, Holland G, Gupta K, Henschke C, Pavelsky H. Pulmonary Thromboembolism: recent
developments in diagnosis with CT and MR imaging. Radiology 1995; 197:561-574.
10. Geraghty JJ, Stanford W, Landas S, Galvin J. Ultrafast computed tomography in experimental pulmonary
embolism. Investigative Radiology 1991; 27:60-63.
11. Goodman LR. Small pulmonary emboli: what do we know? Radiology. 2005 Mar;234(3):654-8.
12. Goodman LR, Curtin JJ, Mewissen MW, Foley WD, Lipchik RJ, Crain MR, Sagar KB, Collier BD. Detection of
pulmonary embolism in patients with unresolved clinical and scintigraphic diagnosis: helical ct versus
angiography. American Journal of Roentgenology 1995; 164:1369-1 374.
13. Goodman LR, Lipchik RJ. Diagnosis of acute pulmonary embolism: time for a new approach. Radiology 1996;
199:25-27.
14. Goodman LR, Lipochik RJ, Kuzo RS. Acute pulmonary embolism: the role of computed tomographic imaging.
Journal of Thoracic Imaging 1997; 12(2):83-86.
15. Goodman LR. Helical CT for initial imaging of pulmonary embolus. AJR Am 3 Roentgenol 1998; 171(4):1153-4.
16. Gurney JW. No fooling around: direct visualization of pulmonary embolism. Radiology 1993; 188:618-619.
17. Kim KI, Muller NL, Mayo JR. Clinically suspected pulmonary embolism: utility of spiral CT. Radiology 1999;
210(3):693-7.
18. Mayo JR. Remy-Jardin M, Muller NL, Remy J, Worsley DF, Hossein-Foucher C, Kwong JS, Brown MJ.
Pulmonary embolism: prospective comparison of spiral CT with ventilation-perfusion scintigraphy. Radiology
1997; 205(2):447-52.
19. Remy-Jardin M, Remy J, Artaud D, Deschildre F, Fribourg M, Beregi JP. Spiral CT of pulmonary embolism:
technical considerations and interpretive pitfalls. J Thorac Imaging 1997; 12(2):103-17.
20. Remy-Jardin M, Remy J, Deschildre F, Artaud D, Beregi JP Hossien-Foucher C, Marchdise X, Duhamel A.
Diagnosis of pulmonary embolism with spiral ct:comparison with pulmonary angiography and scintigraphy.
Radiology 1996;200:699-706.
21. Remy-Jardin M, Remy J, Wattinne L, Giraud F. Central pulmonary thromboembolism: diagnosis with spiral
volumetric ct with single-breath-hold technique-comparison with pulmonary angiography. Radiology 1992;
185:381-387.
22. Ren H, Kuhlman JE, Hruban RH, Fishman EK, Wheeler PS, Hutchins GM. CT of inflation-fixed lungs: wedge-
shaped density and vascular sign in the diagnosis of infarction. Journal of Computer Assisted Tomography
1990;14(1):82-86.
23. Revel MP, Petrover D, Hernigou A, Lefort C, Meyer G, Frija G. Diagnosing pulmonary embolism with four-
detector row helical CT: prospective evaluation of 216 outpatients and inpatients. Radiology. 2005 Jan;234(1):265-
73.

24. Storto ML, Di Credico A, Guido F, Larici AR, Bonomo L.Incidental detection of pulmonary emboli on routine
MDCT of the chest. AJR Am J Roentgenol. 2005 Jan;184(1):264-7.
25. Tardivon AA, Musset D, Maitre S, Brenot F Dartevelle P, Simonneau G, Lobrune M. Role of ct in chronic
pulmonary embolism: comparison with pulmonary angiography. Journal of Computer Assisted Tomography
1993;17:345-351.
26. Teigen C, Maus TP, Sheedy PF, Johnson CM, Stanson AW, Welch TJ. Pulmonary embolism: diagnosis with
electon-beam ct. Radiology 1993;188:839-845.
27. Teigen CL, Maus TR Sheedy PR Stanson AW, Johnson CM. Preen JR Mckusick MA. Pulmonary embolism:
diagnosis with contrast-enhanced electron-beam ct and comparison with pulmonary angiography. Radiology 1995;
194:313-319.
28. Van Erkel A, van Possum A, Bloem J, Mali W, Pattynama P. Cost-effectiveness of the us of spiral CT angiography
to determine suspected pulmonary embolism. Radiology 1995; 197(P):303-304.
29. Van Rossum AB, Pattynama PMT, Tjin ER, Treurniet FE, Arndt J-W, van Eck B, Kieft GJ. Pulmonary embolism:
validation of spiral ct angiography in 149 patients. Radiology 1996; 201 :467-470.
30. Winer-Muram HT, Rydberg J, Johnson MS, Tarver RD, Williams MD, Shah H, Namyslowski J, Conces D,
Jennings SG, Ying J, Trerotola SO, Kopecky KK.Suspected acute pulmonary embolism: evaluation with multi-
detector row CT versus digital subtraction pulmonary arteriography.Radiology. 2004 Dec;233(3):806-15.
31. Winston C, Wechsler RJ, Salazar AM, Kurtz AB, Spirn PW. incidental pulmonary emboli detected at helical ct:
effect on patient care. Radiology 1996;201:23-27.
Magnetic Resonance
1. Hatabu H, Gaa J, Kim D, Li W, Prasad PV, Edelman R. Pulmonary perfusion and angiography: evaluation with
breath-hold enhanced three-dimensional fast imaging steady-state precession mr imaging with short tr and te. AJR
1996; 167:653-655.
2. Hatabu H, Gaa J, Kim D, Li W, Prasad P, Edelman RR. Pulmonary perfusion: qualitative assessment with dynamic
contrast-enhanced mri using ultra short TE and inversin recovery turbo FLASH. Magnetic Resonance in Medicine
1996; 36:503-508.
3. Gefter W, Hatabu H, Holland G, Gupta K, Henschke C, Pavelsky H. Pulmonary Thromboembolism: recent
developments in diagnosis with CT and MR imaging. Radiology 1995; 197:561-574.
4. Gefter WB, Hatabu H, Dinsmore BJ, Axel L, Palevsky H, Reichik N, Schiebler ML, Kressel HY. Pulmonary vascular
cine MR imaging: a noninvasive approach to dynamic imaging of the pulmonary circulation. Radiology 1990;
176(3):761-770.
5. Meaney JFM, Weg JG, Chenevert TL, Stafford-Johnson D, Hamilton BH, Prince MR. Diagnosis of pulmonary
embolism with magnetic resonance angiography. The New England Journal Resonance 1997; 336:1422-1427.

Tuberculosis
Tuberculosis
• Leading cause of death from infectious disease
• 8-10 million new cases/year
• 2-3 million deaths/year
• 1/3 of world population infected
• > 90% of new cases in developing countries
• 80% 15-59 years of age
• Highest incidence
➢ Southeast Asia: 247/100,000
➢ Sub-Saharan Africa: 191/100,000
✧ HIV co-infection: 60% of children, 70% of adults
Tuberculosis: History
• Ancient disease
• 1882: Robert Koch
➢ Isolation of M. tuberculosis
• 1944: streptomycin
• 1952: INH
Tuberculosis Pre-Antibiotic Era
Tuberculosis: United States

• 1953: 84,304 cases
➢ 19,707 deaths
• 1985: 22,201 cases
➢ 1,752 deaths
• 1986-1992: 20% increase in reported cases
➢ HIV
➢ Immigration
➢ Congregate settings
➢ Deteriorating TB services
➢ MDR-TB
➢ Decreasing TB research
Tuberculosis: United States

• 2002: 15,075 cases
➢ 5.2/100,000
➢ 43% decrease from 1992
➢ 4-6% of population infected
✧ 15 million people
• 51% Foreign-born
➢ Mexico, the Philippines, Vietnam, India and China
• U.S. -born
➢ African Americans 25% of all cases
➢ homeless, immunocompromised, elderly
• Urban areas, coastal states, states bordering Mexico
NMWR,March 21, 2003 Vol 52
Mycobacteria
• Tuberculosis complex
➢ M. tuberculosis, M. bovis, M. africnum, M. microti
• M. tuberculosis and M. bovis
➢ > 95% of pulmonary mycobacterioses
• Slow growth
• Person-to-person transmission
Chest Radiology 93 Tuberculosis

M. tuberculosis: Pathologic features
• Gram positive pleomorphic rod
• Acid fast:
➢ Resists decolorization with acid alcohol Figure 1-9-1
• Virulence related to cell wall
➢ No endotoxin or enzymes
• Caseous necrosis
• Caseating granuloma
➢ Central caseous necrosis
➢ Rim of histiocytes, giant cells
Caseous Necrosis [Figure 1-9-1]
Tuberculosis: Pathogenesis
• Inhaled bacteria [Figure 1-9-2]
➢ Mid to lower lung zones
➢ Ghon focus
Figure 1-9-2 The caseating granuloma is the

Even though we tend to hallmark of TB. The actively growing
think of TB as an upper bacilli reside in the macrophages in
lobe disease, we inhale the periphery
most bacteria into the
mid and lower lung
zones
Figure 1-9-3
Physiologic Gradients-Airflow FRC
Tuberculosis: Pathogenesis
• Inhaled bacteria
➢ Mid to lower lung zones
➢ Ghon focus
• Regional lymph node spread [Figures 1-9-3]
➢ Ranke complex
• Lymphatic/hematogenous dissemination
• Cell-mediated immunity In primary tuberculosis the ineffective
• Delayed hypersensitivity macrophages carry bacteria to
➢ Caseous necrosis regional lymph nodes where they
➢ 2-10 weeks proliferate and disseminate
• Healing
Tuberculosis 94 Chest Radiology

Tuberculosis: Pathogenesis Figure 1-9-4
• Latent TB infection
➢ +PPD
➢ No active signs of infection
• Survival of organisms [Figures 1-9-4]
➢ Apical/posterior upper lobe
➢ Superior segment lower lobe
✧ Oxygen gradient
✧ Lymphatic gradient
✧ Bucket handle rib motion
• Active TB infection [Figures 1-9-5]
➢ 5% within 2 years
➢ 5-10% lifetime risk
➢ HIV: 50% within 2 years
➢ Pulmonary fibrotic lesions, underweight, silicosis, DM, renal
failure, gastrectomy, jejunoileal bypass, transplantation, head
and neck cancer, prolonged immunosuppressive therapy
Tuberculosis: Clinical features The lymphatic gradient helps explain

• Primary TB
the upper lobe distribution of
• Postprimary TB
reactivation tuberculosis
• Disseminated TB
Primary Tuberculosis: Clinical features Figure 1-9-5

• Asymptomatic 65%
➢ Nonspecific symptoms when present
• Progressive primary complex
➢ Fever, cough, hemoptysis, weight loss
Primary Tuberculosis: Radiologic features

• Lymphadenopathy [Figure 1-9-6]
➢ Children 95%, young adults 43%, elderly 10%
➢ Right paratracheal, hilar
➢ Peripheral enhancement, central low-attenuation
• Atelectasis, overinflation [Figure 1-9-7]
➢ Children
➢ Anterior segements upper lobes
➢ Medial segment middle lobe
• Consolidation
➢ Unifocal 75% Figure 1-9-6
➢ Segmental, lobar,
multifocal
➢ Homogeneous, patchy,
linear, nodular
➢ Adults 38%, children
The lymphatic gradient helps
11%
explain the upper lobe distribution
Leung, Radiology 1999, Vol 210 of reactivation tuberculosis
Lymphadenopathy is hallmark
of primary TB and is more
common in children

Postprimary Tuberculosis: Clinical Features
• Reactivation Figure 1-9-7
➢ Fever, malaise, anorexia, weight loss, anorexia, night sweats
➢ Dyspnea, cough, chest pain, hemoptysis
• Active TB infection
➢ 5% within 2 years
➢ 5-10% lifetime risk
➢ HIV: 50% within 2 years
➢ Pulmonary fibrotic lesions, underweight, silicosis, DM, renal
failure, gastrectomy, jejunoileal bypass, transplantation, head
and neck cancer, prolonged immunosuppressive therapy
Postprimary Tuberculosis: Pathogenesis

• Delayed hypersensitivity
• Liquifaction
• Cavitation
➢ Airway
➢ Vessel
➢ Pleura
Figure 1-9-8
The lymph nodes which surround
airways may cause narrowing that
results in atelectasis
Figure 1-9-9
Postprimary TB implies
reactivation of dormant bacilli. It is
characterized by tissue destruction
Cavitation and necrosis enables spread via the airway,

blood stream or pleura
Postprimary Tuberculosis: Radiologic features

• Consolidation 50-70%
• Cavitation 40-45%
• Nodules

Postprimary Tuberculosis: Radiologic features Figure 1-9-10
• Consolidation 50-70%
➢ Heterogeneous, nodular, linear
➢ Apical, posterior 85%, Superior segments 14%
• Cavitation 40-45% [Figure 1-9-10]
➢ Thin or thick walls, air-fluid levels 20%
• Nodules
➢ Tuberculoma
✧ SPN: variable borders, satellite lesions,
upper lobes
➢ Endobronchial spread [Figures 1-9-11 to 1-9-13]
✧ Centrilobular, tree-in-bud, 100% by CT
➢ Hematogenous spread
✧ Miliary 1-3mm, random
➢ Bronchiectasis, bronchitis, airway narrowing
Cavitation implies a large number of bacilli speeds
Figure 1-9-11 the progression of disease
Figure 1-9-12
Endobronchial spread leads to airways nodules
Endobronchial spread leads to

airways nodules
Postprimary Tuberculosis-Cavitation Figure 1-9-13
Thoracoplasty
Oleothorax
Plumbage [Figure 1-9-14]
Postprimary Tuberculosis-Nodules
Postprimary Tuberculosis-Airways
Endobronchial spread leads to airways nodules

Postprimary Tuberculosis: Assessment of Activity Figure 1-9-14
• Cannot discern activity from a single film
• Inactive disease
➢ radiographic stability
➢ 6mos
• Negative cultures
• Suggestive of active disease
➢ Cavitation
➢ Consolidation
➢ Ground glass
➢ Centrilobular opacities
Lee et al, Chest, 1996
Tuberculosis: Complications
• End-stage disease
• Hemoptysis Images demonstrate broncho-esophageal fistula
➢ Bronchial arteries in chronic cavities which was a complication of plumbage
➢ Mycetoma
➢ Rassmussen (pulmonary artery) aneurysm
• Chest wall involvement
• Pericardial involvement
• Empyema
➢ BPF, empyema necessitatis
Hemoptysis-Bronchial Artery
Hemoptysis-Mycetoma
End-Stage Lung
Tuberculosis-Chest Wall
Tuberculosis-Pericardial
Tuberculosis: HIV/AIDS
• CD4>200
➢ Well formed granulomas
➢ Upper lobe cavities, consolidation and nodules
• CD4<200
➢ Poorly formed granulomas
➢ Adenopathy, consolidation and miliary disease
• CD4<60
➢ No hypersensitivity reaction
➢ Organisms spread from GI tract
➢ Miliary Disease
Tuberculosis and AIDS
Tuberculosis and AIDS-Low CD4
Tuberculosis: Diagnosis
• Conventional methods
➢ Acid-fast smear: 1 day
➢ Culture: 1-2 weeks
➢ Identification: 2-3 weeks
➢ Drug susceptibility testing: 3-4 weeks
• Radiometric methods
• Polymerase chain reaction (PCR)
• HPLC

Summary
• Primary TB
➢ Consolidation
➢ Ipsilateral lymphadenopathy
➢ Pleural effusion
• Postprimary TB
➢ Consolidation
➢ Cavitation
➢ Apical/posterior upper lobe nodules
➢ Tracheobronchial spread
Tuberculosis Pre-Antibiotic Era
References
General
1. Leung AN.Pulmonary tuberculosis: the essentials. Radiology. 1999 Feb;210(2):307-22

Fungal Disease in the Thorax:
Opportunistic and Primary Pathogens
Fungal Disease in the Thorax: Overview

• Opportunistic invaders
➢ Aspergillus species
➢ Candida
➢ Mucormycosis
• Primary pathogens
➢ Histoplasma capsulatum
➢ Blastomyces dermatitidis
➢ Coccidioides immitis
Opportunistic Invaders
• Immunocompromised host
➢ Mucosal disruption
➢ Reduced cellular and/or humoral immunity Figure 1-10-1
• Ubiquitous
• Lack dimorphism
• Multiple organisms may occur
Primary Pathogens
• May infect healthy individuals
• Dimorphism
➢ Saprophytes in the soil
➢ Spores via germination
• Most disease mild or subclinical
• Fulminant or chronic disease may occur
• Specific geographic regions
➢ Endemic
Coccidioidomycosis
Blastomycosis The mycelial form of Histoplasmosis is found in

soil that has been enriched with bird droppings.
Histoplasmosis The fungus then releases conidia or spores
Histoplasmosis: Epidemiology and Ecology

• Endemic fungal disease
➢ Ohio, Mississippi and St. Lawrence river valleys
➢ Reported worldwide but relatively rare outside of the United States
➢ Infection rate up to 95% in endemic areas
➢ Point sources associated with aerosolization
✧ Earth moving, bird husbandry and spelunking
• Dimorphic fungus
• Clinical
Histoplasmosis: Epidemiology and Ecology

• Endemic fungal disease
➢ Mycelial form in high nitrogen soil
✧ Guano from birds and bats
➢ Yeast within the infected host
• Clinica
Histoplasmosis [Figure 1-10-1]
Fungal Diseases 100 Chest Radiology

Histoplasmosis: Pathology [Figure 1-10-2] Figure 1-10-2
• Early sequence of infection The fungal
➢ Mycelia produce micronidia spores are able
➢ Micronidia reach alveolar spaces to reach the
✧ 2-5 microns alveolar level,
bypassing the
Histoplasmosis: Pathology [Figure 1-10-3 and 1-10-4] upper airway
• Early sequence of infection defenses
➢ Lymphocytes and macrophages replace polys because of
➢ Micronidia transform to conidia or spores their small size
➢ Spores transform into budding yeast which is less
➢ Macrophages phagocytose and kill yeast than 5 microns
• Late sequence of infection
➢ Lymphocyte-mediated cellular immunity
➢ Granulomatous inflammation
➢ Necrosis
➢ Fibrosis
Histoplasmosis: Pathology
• Distinction from tuberculosis
➢ Histoplasmosis relatively benign
➢ Immunity to histoplasmosis short lived Figure 1-10-3
✧ 20% lose immunity each year From three to 5
✧ Continuous reinfection days following
– Primary and postprimary not appropriate inhalation the
spores germinate
Histoplasmosis: Clinical and release
• Asymptomatic yeast forms. The
➢ 95-99% of infection in endemic areas yeast within the
➢ Parenchymal opacities in 10-25% alveoli are rapidly
➢ Small inoculum or prior infection (cellular phagacytosed by
macrophages
immunity) and moderate inoculum
• Symptomatic
➢ Acute
✧ Moderate vs large inoculum
➢ Chronic
➢ Disseminated
• Late complications
➢ Histoplasmoma
➢ Broncholithiasis
➢ Mediastinal granuloma
➢ Mediastinal fibrosis
Figure 1-10-4
Histoplasmosis: Acute Clinical Lymphocyte-
• Signs and symptoms
mediated
➢ “Flulike”: fever, chills, cough
cellular
➢ Retrosternal pain
immunity
✧ Mediastinal lymph node involvement
develops at 10-
➢ Erythema nodosum in women
14 days
➢ Arthralgia
controlling the
• Shorter incubation with prior exposure
infection
through a
Histoplasmosis: Acute Radiology necrotizing
• Poorly defined areas of consolidation
granulomatous
✧ Single or multiple
response
• Hilar lymph node enlargement
• Numerous discrete nodular shadows in heavy
exposure
✧ 3-4 mm
✧ Symptoms precede radiographic change
✧ Nodules change to punctate calcifications
Chest Radiology 101 Fungal Diseases

Acute Histoplasmosis [Figures 1-10-5] Figure 1-10-5
Acute Histoplasmosis – large inoculum
Acute Histoplasmosis [Figure 1-10-6]
Histoplasmosis: Chronic Pulmonary

Histoplasmosis
• Emphysema and bullous disease a common
predisposition
• Upper lobe predominance
• Two possible mechanisms
➢ Hypersensitivity reaction in preexisting
emphysematous space
✧ Few organisms
✧ Colonization or minimal invasion
✧ Thick walled bulla filled with fluid may
clear spontaneously
✧ Progressive loss of volume
➢ Similar to TB Acute histoplasmosis is associated with areas of
✧ Fibrosis, cavitation and granulomatous consolidation and ipsilateral hilar and mediastinal
inflammation enlargement
Chronic Histoplasmosis [Figure 1-10-7] Figure 1-10-6

Histoplasmosis
Disseminated
• Clinical
➢ Rare entity (1/100,000-1/500,000)
➢ Most patients immunocompromised
✧ 30% infants < 2 years
✧ 20% immunocompromised
✧ 50% apparently normal (transient
compromise)
➢ Reduced macrophage function
✧ Parasitization of macrophages
✧ Intracellular survival and multiplication
➢ Radiology
✧ Miliary nodules (1-3 mm)
✧ 50% of disease associated with AIDS
purely extrathoracic
– Normal radiograph
– Positive blood or bone marrow
biopsy Bilateral soft tissue nodules imply a large innoculum.
The nodules disappear over months leaving behind
small calcific densities

Disseminated Histoplasmosis [Figure 1-10-8] Figure 1-10-7
Histoplasmosis: Late Complications

• Histoplasmoma
• Broncholithiasis
• Mediastinal granuloma
• Mediastinal fibrosis
Histoplasmosis: Histoplasmoma
• Solitary nodule (.5-3 cm)
➢ Sharply defined
➢ Smaller satellite lesions
➢ Central or diffuse calcification
✧ Diagnostic of benign lesion if less that 3 cm
➢ May increase in size
✧ Similar reaction to fibrosing mediastinitis
• Hilar calcification common on ipsilateral side
• Fungal nodules account for 30% of all solitary
nodules
• 87% are less than 2.5 cm in diameter
Histoplasmoma [Figure 1-10-9] Chronic histoplasmosis resembles post-primary TB but

usually represents a hypersensitivity reaction in patients
Broncholith with emphysema
Figure 1-10-8
Histoplasmosis: Mediastinal granuloma
• Pathology
➢ Direct infection of hilar and mediastinal lymph
nodes
• Clinical
➢ Often asypmtomatic with discovery of a
mediastinal mass on chest radiograph
➢ SVC or esophageal obstruction less common
• Radiology
➢ Middle mediastinal mass
✧ Subcarinal or paratracheal
➢ Enhancing capsule with low attenuation center
➢ Mass may be low signal on T2 weighted MR
because of fibrous tissue or calcification
Disseminated Histoplasmosis presents with miliary

nodules and macrophages filled with organisms
Figure 1-10-9
Histoplasmomas are the residua of a prior area of

pneumonitis. They typically demonstrate concentric rings of
calcification but may remain uncalcified especially in older
individuals

Mediastinal Granuloma [Figure 1-10-10] Figure 1-10-10
Histoplasmosis: Fibrosing
Mediastinitis
• Pathology
➢ Proliferation of acellular collagen and
fibrous tissue within the mediastinum
➢ Most cases in the United States are an
immunological response to H. capsulatum
✧ Focal form: paratracheal and
subcarinal
✧ Calcification
➢ Idiopathic form
✧ Diffuse, infiltrating
✧ Noncalcified
✧ Multiple mediastinal compartments
• Clinical
➢ Signs and symptoms of obstruction to
mediastinal structures Mediastinal granuloma is the result of direct infection of
✧ Superior vena cava, pulmonary veins mediastinal lymph nodes. Acutely the lymph nodes
demonstrate low attenuation with an enhancing capsule
or arteries, central airway or
esophagus
Fibrosing Mediastinitis [Figure 1-10-11] Figure 1-10-11
Blastomycosis
Blastomycosis: Epidemiology and Ecology

• Ecological niche [Figure 1-10-12]
➢ Difficult to establish
➢ Saprophyte in an unidentified resevoir within
reach of man and dogs
➢ Survives only in wet soil with a high PH and high
organic content
➢ Soil probably contaminated rather than the
natural resevoir
➢ Point sources in dead and decaying material
near rivers, streams and swamps
Fibrosing mediastinitis due to h. capsulatum usually
➢ Mycelium in natural habitat
presents as a mediastinal mass associated with
➢ Releases spores (conidia) into the air calcification
➢ Budding yeast
✧ (8-15 microns) in vivo
✧ Broad based
• Clinical Figure 1-10-12
➢ Less common than Histoplasmosis
➢ High risk of symptomatic disease although most
cases are probably asymptomatic
➢ Males more commonly affected (3:1-15:1)
➢ Exposure in heavily wooded areas
➢ Variable course
✧ Symptoms of acute pneumonia
– Abrupt onset, fever, chills, cough and
pleuritic pain
– Occasional rapid progression
> Hematogenous dissemination: skin,
bone and genitourinary tract
> ARDS
✧ Chronic disease similar to tuberculosis
Blastomycosis is usually associated with activity in

damp, wooded areas

Blastomycosis: Pathology
• Initial inflammatory response is neutrophilic
➢ Small collections of cells to hundreds of milliliters of pus
• Rapidly followed by chronic inflammatory response
➢ Lymphocyte, histiocytes and plasma cells
➢ Langhans’ giant cells
➢ Granulomas
• Both responses may coexist
➢ Organisms more common in supperative area
• Progression
➢ Coalescence of patchy consolidation
➢ Airway perforation
➢ Cavitation
• Ulcerative bronchitis is common
Blastomycosis: Pathology
• Initial response is neutrophilic
• Chronic inflammatory response
• Both responses may coexist
• Progression
➢ Coalescence of patchy consolidation
➢ Airway perforation
➢ Cavitation
• Ulcerative bronchitis is common Figure 1-10-13
Blastomycosis: Radiologic Manifestations
• Consolidation most common
➢ Upper lobe 2:1
➢ Rounded, ill-defined
➢ Masslike opacities
✧ Central or paramediastinal
✧ Carinoma mimic
✧ Solitary nodules
➢ Air bronchograms (88% CT)
➢ Cavitation
• Nodules
➢ Intermediate size
➢ Remote from consolidation
➢ Satellite lesions
• Miliary disease Blastomycosis often with an upper lobe areas of mass-
➢ Hematogenous dissemination like consolidation
Asymptomatic Mass [Figure 1-10-13]
Consolidation Figure 1-10-14
Solitary Pulmonary Nodule
Similar to Postprimary TB
Disseminated Disease [Figure 1-10-14]
Mass and Dissemination
Blastomycosis: Treatment
• Pulmonary disease may be self-limited even if
extensive
• Extrapulmonary disease requires treatment
• Amphotericin B IV or oral Keotconazole
Miliary disease may complicate infection with
Blastomycosis

Coccidioidomycosis [Figure 1-10-15]
Coccidioidomycosis: Epidemiology
and Ecology Figure 1-10-15
• Ecological niche
• Clinical
➢ Acute Disease
✧ 100,000 new cases each year, essentially all
in the southwest
✧ No racial, sex or age predilection in acute
disease
✧ Most inhabitants of the endemic area
infected in the first year of exposure
✧ Incubation period 10-16 days
✧ 60% are asymptomatic
✧ Symptoms when present include
– Fever, pleuritic chest pain, cough
– Valley Fever: allergic form with erythema
nodosum or multiforme
✧ Severity of disease related to immune status Coccidioidomycosis is associated exclusively with the
and race desert southwest
– Filipinos, African Americans and
Hispanics more likely to suffer dissemination
➢ Chronic Disease (5%)
✧ Symptoms persist without dissemination
✧ May be mildly immunocompromised
➢ Dissemination
✧ Rare occurrence
– Immunocompromise
– Non-Caucasian (Filipino, African American and Hispanic)
– Early dissemination more common and carries a poor prognosis
– Mortality rate or 50% even with early treatment
Coccidioidomycosis: Pathology
• Lung the usual portal of entry
• Neutrophilic response early
➢ Especially in response to ruptured spherules
➢ Spherules ingested by macrophages
• Granulomatous and giant cell reaction follows
• Necrosis may occur
Coccidioidomycosis: Radiologic Manifestations

• Acute Disease
➢ Consolidation most common (75%)
✧ Usually unilateral, hilar or basal
✧ Segmental or lobar
➢ Multifocal nodular or patchy opacities
➢ Peribronchiolar thickening
➢ Hilar or mediastinal adenopathy (20%)
✧ Mediastinal adenopathy may herald dissemination
➢ Pleural effusion 20%
✧ Small, unilateral
• Coccidioidoma

Acute Disease
Coccidioidoma [Figure 1-10-16]
Coccidioidomycosis: Radiologic Manifestations

• Acute Disease
• Coccidioidoma Figure 1-10-16
➢ Area of prior consolidation
➢ Round and well circumscribed
➢ 1.5cm average (up to 6cm)
➢ Usually single
➢ Marked enhancement with contrast CT
➢ Caseating chronic granulomatous inflammation
• Chronic Disease
➢ Cavitation
✧ Occur in areas of consolidation
✧ May be thin or thick walled
✧ Pneumothorax or empyema may result
➢ Chronic progressive pneumonia
Chronic Coccidioidomycosis [Figure 1-10-17]

A coccidioidoma is the resdua of an area of infectious
Coccidioidomycosis: Radiologic consolidation
Manifestations
• Chronic Disease
➢ Cavitation
➢ Chronic progressive pneumonia
✧ Indolent course similar to TB Figure 1-10-17
✧ Biapical fibronodular lesions
✧ Hilar and mediastinal adenopathy
✧ Hilar retraction
✧ Persistently positive sputum
✧ High complement fixing antibody titer
✧ Non-Caucasian
• Disseminated Disease
➢ Miliary or reticular nodular pattern
✧ Less well circumscribed that TB
➢ Lymphadenopathy is common
➢ Pericardial effusion
➢ Skin, bone, meninges or upper genitourinary
tract
Thin-walled cavities are suggestive of chronic

coccidioidomycosis

Dissemination – Miliary Nodules
Histoplasmosis
Acute Histoplasmosis [Figure 1-10-18]
Histoplasmosis – Solitary Nodule

[Figures 1-10-19 and 1-10-20]
Histoplasmosis – large inoculum [Figure 1-10-21]
Histoplasmosis [Figure 1-10-22]
Disseminated Histoplasmosis [Figure 1-10-23]
Chronic Histoplasmosis [Figure 1-10-24]
Fibrosing Mediastinitis [Figure 1-10-25]
Blastomycosis
Coccidioidomycosis Figure 1-10-18
Fungal Disease in the Thorax: Overview

• Opportunistic invaders
➢ Aspergillus species
➢ Candida
➢ Mucormycosis
• Primary pathogens
➢ Histoplasma capsulatum
➢ Blastomyces dermatitidis
➢ Coccidioides immitis
➢ Paracoccidioides brasiliensis
Symptomatic patients with acute histoplasmosis may present

with solitary or multifocal areas of consolidation and associated
adenopathy
As the infection heals the inflammatory area rounds up and is The nodule may enlarge by adding fibrous tissue to the
surrounded by a fibrous capsule. Over a prolonged period periphery
calcification may develop in the nodule and regional lymph
nodes

Figure 1-10-21
Figure 1-10-22
If the patient inhales a large number of spores then numerous

patches of consolidation may round up into well circumscribed
nodules
Figure 1-10-23
As they heal the patient will be left with numerous calcifications
Figure 1-10-24
Patients with reduced immune function may present with

hematogenous spread of disease and miliary nodules
Figure 1-10-25
Patients with underlying emphysema may develop chronic

histoplasmosis which in most cases represents a
hypersensitivity reaction to a small number of organisms
Fibrosing mediastinitis represents an exuberant fibrous reaction

within the mediastinum which may result in damage to
mediastinal structures. Most focal cases of fibrosing
mediastintis in the United States are due to H. capsulatum

Bronchogenic Carcinoma
A 20th Century Disaster
Histological Classification of Tumors

• World Health Organization
• Lung tumor editions
➢ 1967
➢ 1981
➢ 1999
➢ 2004
• Improve communication
• Consistent treatment
• Basis for comparative studies
• Prognosis
Changes in the 1999/2004 WHO

• Subclasses of adenomas
• Preinvasive lesions
• Adenocarcinoma
• Definition of BAC
• Neuroendocrine tumors
• Biphasic and pleomorphic tumors
Incidence of Lung Cancer

Gazdar, Semin Oncol, 1988
Histological Typing of Lung Tumors

• Based on light microscopic criteria
• Classified by the best differentiated region
• Graded by the most poorly differentiated region
• Histologic heterogeneity is the “rule”
Histological Typing of Lung Tumors

• Prognosis: Small cell vs. non-small cell
• Stage determines prognosis in non small cell
• >95% of 1° lung tumors
➢ Adeno
➢ Squamous
➢ Large cell
➢ Small cell
➢ Combination of above
Lung Cancer Demographics

• Most common cancer in males world-wide
• Leading cause of cancer mortality in women and men (United States)
• Mortality rates in women began increasing in 1935 and surpassed breast ca in
1987
Age-Adjusted Cancer Death Rates – Males vs Females

Cancer 49: 1999
Bonchogenic Carcinoma 110 Chest Radiology

Lung Cancer Etiology – Cigarette smoking
• 85-90% of lung cancer deaths
• 25% of lung cancer in non-smokers attributed to passive smoke
• Risk related to:
➢ Number of cigarettes smoked
➢ Depth of inhalation
➢ Age at which smoking began
• Central tumors
➢ Cough
➢ Wheezing
➢ Hemoptysis
➢ Pneumonia
• Extrapulmonary invasion
➢ Pain
➢ Pancoast Syndrome
➢ SVC Syndrome
• Metastases
• Paraneoplastic Syndromes
• Asymptomatic 10%
Paraneoplastic Syndromes
• Cachexia, malaise and fever
• Ectopic hormone production
➢ ACTH
➢ ADH
➢ Hypercalcemia
➢ Clubbing and HPO
➢ Thrombotic endocarditis
➢ Non-bacterial
• Migratory thrombophlebitis
Lung Cancer and Clotting
Squamous Cell Carcinoma

• Terminology
➢ Squamous
✧ Flattened cells
➢ Epidermoid
✧ Mimics differentiation of the epidermis
• Rapid local growth
• Distant metastases later
• Strong association
➢ Cigarette smoking

• Pancoast Syndrome
• Hyperparathyroidism
➢ Parathyroid-like substance
• Most common to present as radiographically occult
Chest Radiology 111 Bronchogenic Carcinoma

Preinvasive Lesions: Squamous Dysplasia
• Similar to cervical Ca
• Squamous metaplasia Figure 1-11-1
• Progression
➢ Dysplastic epithelium
• Carcinoma in situ
➢ Full thickness dysplasia
• Precursor
➢ Invasive squamous cell Ca
Squamous Cell Carcinoma:

Microscopic features
• Individual cell keratinization
➢ Eosinophilia
• Keratin pearls
➢ Well differentiated tumors Figure 1-11-2
• Intercellular bridges

Gross Features [Figure 1-11-1]
• Central lesion
➢ Polypoid, endobronchial,
exophytic growth The majority of squamous cell
• Central necrosis common cancers are central lesions
• Bronchial wall invasion
➢ Common
➢ Positive cytology
• Proximal growth
➢ Along bronchial mucosa
Squamous Cell Carcinoma: Radiologic Features [Figure 1-11-2]

• Hilar or perihilar mass
• Bronchial wall thickening
➢ Often focal
• Consolidation
➢ Must clear completely Squamous cell cancers are
• Atelectasis predominantly central and
• Peripheral nodule or mass endobronchial as exemplified by this
➢ 30% tomogram
• Cavitation
Atelectasis [Figures 1-11-3 to 1-11-5]
Figure 1-11-3
Figure 1-11-4
Atelectasis in an adult smoker is lung cancer until

proven otherwise Typical central squamous cell carcinoma

Cavitation [Figure 1-11-6] Figure 1-11-5
Pancoast Tumor:
Superior Sulcus Tumor
• Characteristic pain
➢ 8th cervical
➢ 2nd thoracic trunk
• Horner’s Syndrome
• Destruction of bone
• Hand muscle atrophy
Pancoast, JAMA, 1992
Small Cell Lung Cancer
• Rapid growth
• Considered metastatic at presentation
• Poorest survival
• Strongest association with cigarette
smoking
Small Cell Lung Cancer Golden’s S sign

• Small cell carcinoma
➢ Pure histology
• Variant
➢ Combined
• Elimination
➢ Oat cell
➢ Intermediate type
WHO, 2004
Small Cell Lung Cancer: Microscopic Features

• Small, uniform cells
• Scant cytoplasm
• Necrosis is common
➢ Often extensive
• >10 mitosis per 10 HPF
➢ Average 60-70
• Neuroendocrine morphology
• Neuroendocrine markers
➢ 75%
• Light microscopy diagnosis
WHO, 2004 Figure 1-11-6
Cavitation is most common in squamous cell cancer

Small Cell Lung Cancer: Gross Features Figure 1-11-7
• Large
• Central mass (90%)
• Bronchial compression
• No endobronchial lesion
• Proximal growth
➢ Along submucosa
• Extensive necrosis
• Hemorrhage
Small Cell Lung Cancer: Radiologic

Features
[Figures 1-11-7 and 1-11-8]
• Hilar or perihilar mass
• Mediastinal adenopathy
• Primary tumor
➢ Rarely evident Small cell tends to spread along the
• Cavitation peribronchovascular lymphatics without endobronchial
➢ Extremely rare invasion
Small Cell Lung Cancer

• Cushing Syndrome
• SIADH
• Eaton Lambert Figure 1-11-8
• Most common cause
➢ SVC Syndrome
Small Cell Lung Cancer: Therapy

• Response to chemotherapy and radiotherapy
• Untreated: median survival 2-4 months
• Treated: median survival 9-18 months
• Limited stage – 15-25% survive 2 years
Large Cell Carcinoma

• Rapid growth
• Location
➢ Segmental
➢ Subsegmental
• Early metastases
• Poor prognosis
• Strong association with cigarette smoking
Small cell most commonly presents as a mediastinal mass
Large Cell Carcinoma: Microscopic
Features
• Large cells
• Prominent nucleoli
• Poorly differentiated
• Diagnosis of exclusion
• Neuroendocrine features
Large Cell Carcinoma: Gross Features

• Large and bulky
➢ Greater that 3 cm
• Soft
• Large areas of necrosis

Large Cell Carcinoma: Radiologic Features
• Usually peripheral
• 70% of tumors
➢ > 4 cm at presentation Figure 1-11-9
Large Cell Carcinoma [Figure 1-11-9]
Adenocarcinoma: Etiology
• Cigarette smoke causatively linked to lung
cancer
➢ 1950
➢ Squamous cell 18X’s Adeno
➢ Squamous cell: central
Adenocarcinoma: Etiology
[Figure 1-11-10]
• Cigarette smoke causatively linked to lung
cancer
• Adenocarcinoma most common
➢ Peripheral
• Filtered low-yield cigarettes
➢ Smaller particles
➢ Reduced nicotine Large cell cancers are commonly necrotic but rarely cavitate
➢ Greater depth of puffs
➢ Increased number of puffs
➢ N-nitrosamines Figure 1-11-10
• Other factors - 10%
➢ Passive smoke
➢ Particulates
➢ Cooking practices
Adenocarcinoma: Microscopic Features

• Glands
• Papillary structures
• Mucin
➢ Intracellular
➢ Extracellular
• Prominent nucleoli
• Moderate cytoplasm
• Desmoplastic reaction
• “Scar carcinoma”
➢ Rare!
Adenocarcinoma: Radiologic Features

• Peripheral (75%)
• Solitary mass or nodule
• Upper lobes 3:2
• Right lung 3:2 Adenocarinoma, the most common lung
• Lobulated cancer is predominantly
• Borders a peripheral lesion
➢ Ill-defined
➢ Well-defined
• Spiculated
• Obstructive pneumonitis (25%)

Spiculation and Retraction [Figure 1-11-11] Figure 1-11-11
Scar Carcinoma [Figure 1-11-12]
Necrosis
Air Bronchogram [Figure 1-11-13]
Adenocarinomas are commonly spiculated peripheral

nodules
Figure 1-11-12
In scar carcinomas the scar is usually a reaction to the

malignancy
Figure 1-11-13
Air bronchograms are commonly seen in adenocarcinomas

Slow Growth
Atypical Adenomatous Hyperplasia: Preinvasive lesion

[Figures 1-11-14 and 1-11-15]
Figure 1-11-14
• Atypical cuboidal epithelium
➢ Lining alveoli
➢ Lining bronchioles
• Found in lung cancer resection specimens
• Probable precursor
➢ BAC
➢ Invasive adenocarcinoma
• Patchy ground glass
• 5mm or less
Kitamura, AJCP, 1999
AAH
Bronchioloalveolar Carcinoma: Microscopic Features

• Lepidic growth pattern
• No evidence
➢ Stromal invasion
➢ Vascular invasion
➢ Pleural invasion
• Diagnosis cannot be made on a small biopsy
• Requires thorough sampling of resected specimen
WHO, 1999
The diagnosis of AAH may be difficult and
Bronchioloalveolar Carcinoma: Mucinous Type the differentiation form a small BAC may
• Alveolar spaces distended with mucin be problematic
• Aerogenous spread is common
• Multifocal consolidation
Figure 1-11-15
Bronchioloalveolar Carcinoma: Non-mucinous Type
• Alveoli lined with
➢ Clara cells
➢ Type II cells
• Central alveolar fibrosis
➢ Common
• Close association
➢ AAH
In AAH the architecture of the lung is not

disturbed

Bronchioloalveolar Carcinoma: Gross Features Figure 1-11-16
[Figure 1-11-16]
• Consolidation
➢ Focal
➢ Multifocal
• Architecture
➢ Preserved
Bronchioloalveolar Carcinoma: Radiologic Features

[Figure 1-11-17]
• Solitary nodule
➢ Excellent prognosis
➢ Resection
• Consolidation
➢ May be multifocal
• Ground glass
• Multiple nodules
• May cavitate?
Noguchi, Cancer 1995
Bronchioloalveolar Carcinoma
BAC Recurrence
BAC often presents as as area of
BAC vs Adenocarcinoma consolidation
BAC - Adenocarcinoma: CT, Histology and Doubling Time

• Type A Figure 1-11-17
• Ground glass
• Localized BAC
• Doubling time
➢ Mean: 880 days
➢ Range: 662-1486 days
Aoki et al, AJR, 2000
BAC - Adenocarcinoma: CT, Histology and Doubling

Time
• Type B
• Ground glass
• Focal increased attenuation
• Localized BAC
• Doubling time
➢ Mean: 880 days
BAC usually presents as an area of
BAC - Adenocarcinoma: CT, Histology and Doubling consolidation
Time
• Type C
• Solid attenuation
• Focal ground glass
• Spiculation
• Pleural tag
• Localized BAC
➢ Active fibroblastic proliferation
• Doubling time

BAC - Adenocarcinoma: CT, Histology and Doubling Time Figure 1-11-18
• Type D
• Solid attenuation only
• Spiculation
• Pleural tag
• Poorly differentiated adenocarcinoma
• Doubling time
➢ Mean: 252 days
AdenoCa Appearance and Prognosis

Adenocarcinoma [Figure 1-11-18]

One form of adenocarcinoma begins as an invasive
BAC process and presents with a solid nodule
AAH [Figure 1-11-19]
BAC [Figure 1-11-20]
The precursor lesion to BAC is AAH BAC demonstrates lepidic growth and presents as an
area of ground glass and/or consolidation
Adenocarcinoma - BAC Prognosis [Figure 1-11-21] Figure 1-11-21
Survival decreases with increasing amount of consolidation and less

ground glass opacity

References
General
1. Travis W, Colby T, Shimasato Y, Brambilla E. Histological Typing of Lung and Pleural Tumors., International
Classification of Tumors. Third ed. Berlin: Springer Verlag, 1999.
2. Colby T, Koss M, Travis W. Tumors of the Lower Respiratory Tract, Atlas of Tumor Pathology. Third ed. Washington,
DC: Armed Forces Institute of Pathology, 1999.
3. Travis WD, Brambilla E, et al: Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart (WHO
Classification of Tumours), IARC Press, 2004 (Oxford).
4. Patel AM, Peters SG. Clinical manifestations of lung cancer Mayo Clin Proc 1993; 68(3):273-7.
5. Davila DG, Williams DE. The etiology of lung cancer. Mayo Clin Proc 1993; 68(2): 170-82.
6. Travis WD, Lubin J, Ries L, Devesa S. United States lung carcinoma incidence trends: declining for most histologic
types among males, increasing among females. Cancer 1996; 77(12):2464-70.
7. Travis WD, Travis LB, Devesa SS. Lung cancer [published erratum appears in Cancer 1995 Jun 15;75(12):2979].
Cancer 1995; 75(1 Suppl):191-2O2.
8. Pisani RJ. Bronchogenic carcinoma: immunologic aspects. Mayo Clin Proc 1993; 68(4):386-92.
9. Whitesell PL, Drage CW. Occupational lung cancer Mayo Clin Proc 1993; 68(2):1 83-8.
10. Patel AM, Davila DG, Peters SG. Paraneoplastic syndromes associated with lung cancer [see comments]. Mayo Clin
Proc 1993; 68(3):278-87.
11. Morabia A, Wynder EL. Cigarette smoking and lung cancer cell types. Cancer 1991; 68(9):2074-8.
12. Ko YC, Lee CH, Chen MJ, Huang CC, Chang WY, Lin HJ, Wang HZ, Chang PY. Risk factors for primary lung cancer
among non-smoking women in Taiwan. Int J Epidemiol 1997; 26(1):24-31.
13. Kitamura H, Kameda Y, Ito T, Hayashi H. Atypical adenomatous hyperplasia of the lung. Implications for the
pathogenesis of peripheral lung adenocarcinoma [see comments]. Am J CIin Pathol 1999; 111(5):610-22.
14. Karsell PR, McDougall JC. Diagnostic tests for lung cancer. Mayo Clin Proc 1993; 68(3):288-96.
15. Dalager NA, Pickle LW, Mason TJ, Correa P, Fontham E, Stemhagen A, Buffler PA, Ziegler RG, Fraumeni JF, Jr. The
relation of passive smoking to lung cancer Cancer Res 1986; 46(9):4808-11.
16. Charloux A, Hedelin G, Dietemann A, Ifoundza T, Roeslin N, Pauli G, Quoix E. Prognostic value of histology in
patients with non-small cell lung cancer. Lung Cancer 1997; 17(1):123-34.
17. Charloux A, Ouoix E, Wolkove N, Small D, Pauli G, Kreisman H. The increasing incidence of lung adenocarcinoma:
reality or artefact? A review of the epidemiology of lung adenocarcinoma. Int J Epidemiol 1997; 26(1 ):14-23.
18. Muller NL, Miller RR. Neuroendocrine carcinomas of the lung. Semin Roentgenol 1990; 25(1 ):96-1 04.
19. Travis WD, Rush W, Flieder DB, Falk E=R, Fleming MV, Gal AA, Koss MN. Survival analysis of 200 pulmonary
neuroendocrine tumors with clarification of criteria for atypical carcinoid and its separation from typical carcinoid.
Am J Surg Pathol 1998; 22(8):934-44.
20. Hardy J, Smith I, Cherryman G, Vincent M, Judson I, Perren T, Williams M. The value of computed tomographic
(CT) scan surveillance in the detection and management of brain metastases in patients with small cell lung cancer
Br J Cancer 1990; 62(4):684-6.
21. Sone S, Takashima S, Li F, Yang Z, Honda T, Maruyama Y, Hasegawa M, Yamanda T, Kubo K, Hanamura K, Asakura
K. Mass screening for lung cancer with mobile spiral computed tomography scanner [see comments]. Lancet 1998;
351 (9111):1242-5.

Chest Seminar 1
Case 1: This 57 year old male with a long history of smoking

cigarettes.
He now complains of a chronic cough
Chest Radiology 121 Chest Seminar 1

Case 2: This 20 year old Caucasian female presented 7 years
prior to the current admission with sudden onset of shortness
of breath. The original chest radiograph revealed a
pneumothorax.
The patient now presents with increasing shortness of breath.
Chest Seminar 1 122 Chest Radiology

Case 3: This 58 year old Caucasion female presented with a one
month history of hemoptysis

Case 4: This 38 year old African American female presented
with a history of chronic asthma and increasing cough and
shortness of breath

Case 5: This 72 year old Caucasian female
presented with cough and occasional fever.
She was treated intermittently with antibiotics for 6 months.
Open biopsy was obtained because of progressive symptoms.

Chest Seminar 2
Case 1: 15 year old female was admitted to the ER with an

overdose of Nefazodone and other unknown pills. Activated
charcoal was administered after which she developed vomiting
and gagging. Respiratory distress required intubation.
Bronchial lavage was performed and immunosuppressants
were started. The patient developed progressive dyspnea and
obstructive pulmonary functions over the next 6 months.
Bilateral lung transplantation was done 19 months later.

Case 2: 59 year old Caucasian female with history of breast
cancer 12 years prior to admission and local recurrence treated
with radiation 7 years later. She presented with a right lung
mass.

Case 3: 46 year old Caucasian male with long standing year
history of shortness of breath. He presents with a 3 months of
worsening dyspnea.
He demonstrated a mild leukocytosis and an increase in serum

LDH.

Case 4: 57 year old Caucasian male with a new history of cough
and a new abnormality on chest radiograph.
A chest CT was done based on the abnormalities found on the

chest radiograph.
A bronchoscopy was performed.

Case 5: 61 year old female worked as a hospital storage room
manager.
She presented to the ER complaining of fatigue, increasing

shortness of breath, chest pain, and cough productive of blood
tinged sputum.

Pulmonary Hypertension
Aletta Ann Frazier, MD
Key Points
• Radiologic findings distinguish precapillary (arterial) from postcapillary
(venous) pulmonary hypertension
• Idiopathic and secondary conditions are included in the differential diagnosis
• Vascular histopathology and secondary cardiac changes are often reflected in
the radiology of pulmonary hypertension
Precapillary Pulmonary Circulation [Figure 1-14-1]
Figure 1-14-1
Precapillary (arterial) circulation and vascular anatomy. Arterial vessels

accompany the dichotomously branching airways of the lung
Postcapillary Pulmonary Circulation [Figure 1-14-2]
Figure 1-14-2
Postcapillary (venous) circulation drains the capillary beds of the alveoli. Veins and
venules course back to the left atrium within interlobular septa
Normal Pulmonary Circulation

• Low pressure system with high degree of capacitance (recruitment and
distension)
• Less than one tenth the resistance to flow in comparison to systemic
circulation (low vasomotor tone)
• Right ventricle expends minimal energy to perfuse the pulmonary vascular bed
Chest Radiology 131 Pulmonary Hypertension

Precapillary (Arterial) Pulmonary Hypertension
• Insidious: dyspnea, chest pain, syncope
• Right heart pressure overload develops late
• Imaging reveals pulmonary HTN & clues to etiology
• NIH Criteria (cardiac cath): Mean PA pressure > 25 mm Hg at rest (normal 10)
Figure 1-14-3
Cor Pulmonale… the best predictor of clinical outcome
• PV regurgitation
• RV hypertrophy & enlargement
• TV regurgitation & RAE
• Dilated IVC, hepatic veins
Normal Heart vs. Cor Pulmonale
American College of Chest Physicians: “Venice

Classification”
• PAH
• PH with left-sided heart dz
• PH with lung dz, hypoxemia
• PH due to thromboembolic dz
• Miscellaneous
Simonneau G. et al. J Am Coll Cardiol. 2004 Jun 16;43 (12 Suppl S):5S-12S.
Pulmonary Hypertension: Precapillary Etiologies

• Idiopathic
• Secondary
➢ Chronic thromboembolic disease Photomicrograph demonstrates a
➢ Sickle cell disease muscular artery (adjacent to airway)
➢ Eisenmenger physiology narrowed by medial hypertrophy and
➢ Mediastinal fibrosis obstructed by intravascular thrombus
➢ Connective tissue disease Figure 1-14-4
➢ Chronic hypoxia (COPD, IPF)
Pulmonary Arterial Hypertension: Histology [Figure 1-14-3]

• Medial hypertrophy
• Intimal proliferation
• Thrombosis
• Arteritis
Precapillary Hypertension: Imaging

• Dilated central arteries
Radiographic features of pulmonary
• Pruning of peripheral vessels
hypertension: enlarged main
• Mosaic perfusion
pulmonary artery, dilated central hilar
• Cor pulmonale
vessels, and peripheral oligemia
• PA atherosclerosis
Figure 1-14-6
Idiopathic Pulmonary Hypertension [Figures 1-14-4 to 1-14-6]
Figure 1-14-5
CT criteria for enlarged

main PA in precapillary
pulmonary
hypertension: CT manifestations of cor pulmonale:
transverse diameter dilated RA and RV, thickened anterior
exceeds 29 mm RV wall, and flattened interventricular
septum
Pulmonary Hypertension 132 Chest Radiology

Cor Pulmonale Figure 1-14-7
• Axial White Blood Cine
• Sagittal White Blood Cine
Courtesy Laura Heyneman, MD
Idiopathic Pulmonary Hypertension

• Mean age 45, F>M (3:1)
• 6% “familial”
➢ Autosomal dominant, incomplete penetrance
• Associations
➢ HIV infection
➢ Appetite suppressants
➢ Cocaine abuse Longstanding uncorrected ASD with acquired
➢ Chronic liver disease Eisenmenger physiology in 35 y.o. female
• Mean survival 2.8 years without treatment [Courtesy of Melissa Rosado de Christenson, MD]
Atrial Septal Defect [Figures 1-14-7 to 1-14-9]
Lung specimen radiograph

demonstrates calcified atherosclerotic
plaques in the main PA
[Courtesy of Melissa Rosado de Christenson, MD]
Vascular pruning pattern
[Courtesy of Melissa Rosado de Christenson, MD]
Eisenmenger Physiology
• Congenital L-to-R shunt
➢ VSD, ASD, PDA
➢ Endocardial Cushion Defect
• Shunt reversal (R-to-L) follows sustained elevation in PVR
• PAH irreversible & requires lung transplantation
Chronic Thromboembolic Disease Figure 1-14-10

[Figures 1-14-10 to 1-14-12]
Chronic Thromboembolic Disease

• Approx 4% of cases acute PE; presents within 2 years
• Symptomatic with >60% vascular bed occlusion
• 5-year survival rate <35% (without surgery)
• V/Q scan - high probability (helps to exclude IPH)
• CT
➢ Enlarged central PA
➢ Eccentric & linear filling defects +/- calcification
➢ Abrupt cut-offs (pruning)
➢ Bronchial arteries (50% of cases) Broad-based intravascular soft tissue
➢ Mosaic perfusion density in the right PA, combined with
➢ Pleural tags (healed infarcts) multiple bronchial arterial collateral
vessels, suggests CTEPH

Chronic Thromboembolic Disease Figure 1-14-11
• CT Angio
➢ 94-100% sensitivity, 96-98% specificity for CTEPH
➢ More sensitive than PA angio for proximal disease
➢ Non-invasive modality for pre- and post-op assessment
➢ Multiplanar & curved multiplanar reconstructions further
characterize disease extent
• Cardiac MR
➢ Cine imaging
✧ Right heart function
➢ Phase-contrast imaging
✧ low velocities in L & R PA’s
✧ shunt vol from bronchial arteries to pulmonary venous
circulation
✧ R-to-L shunt via patent foramen ovale
“Recanalized” Chronic Thrombus
“Organizing” Chronic Thrombus
Pulmonary Thromboendarterectomy
• 15-30% CTEPH patients are candidates
• Thrombi from main to segmental or subsegmental level
• Intima & superficial media removed w/ thrombi
• Operative mortality 8-23%
• Improved long term survival - 75% at six years
Chronic thromboembolic disease:
Other thromboembolic materials mural-based soft tissue masses and
calcium in the lumen of right main PA
Intravenous Talcosis
• Chronic IV injection of crushed tablets (Methadone, amphetamines)
• Thrombogenic pharmaceutical binding agent: magnesium silicate Figure 1-14-12
• Granulomas coalesce into birefringent particles
Intravenous Talcosis: Imaging

• EARLY
➢ Diffuse micronodular opacities
• LATE
➢ Fibrosis
➢ High density perihilar masses
➢ Emphysema
Postcapillary Pulmonary Circulation Mosaic attenuation reflects

geographic variations in blood flow
Pulmonary Hypertension: Postcapillary Etiologies
• Idiopathic
➢ Pulmonary veno-occlusive disease (PVOD)
➢ Pulmonary capillary hemangiomatosis (PCH)
• Secondary
➢ Mitral valve stenosis
➢ Left ventricular failure
➢ Mediastinal fibrosis
➢ Left atrial mass / thrombus
➢ Venous constriction / invasion by tumor

Pulmonary Venous Hypertension Figure 1-14-13
• Acute or chronic onset
• Elevated wedge pressures (with exceptions)
• PAH - secondary
• Venous dilatation
• Venous “arterialization”
• Septal edema, fibrosis
• + Interlobular septa
• Subpleural thickening
• Ground glass opacities
PCH/PVOD
• Capillary proliferation & dilatation
• Venous medial hypertrophy & intimal proliferation
• Recanalized thrombus in veins & venules
PCH/PVOD
• Young adults (M:F is 2:1)
• Pediatric in 1/3 of cases
• Fatal within 3-5 years
• Associations
➢ Chemotherapy
➢ Prior viremia
➢ HIV
➢ ? Toxic exposure PVOD, a rare cause of postcapillary
• Current Debate pulmonary hypertension: prominent
➢ Separate entities? interlobular septa and subpleural edema
➢ Contiguous spectrum of injury?
➢ Cause-effect scenario?
PCH/PVOD [Figure 1-14-13] Figure 1-4-14

• Difficult to discern from primary PAH clinically
• Vasodilators contraindicated: severe pulmonary edema
PVOD/PCH
• Difficult to discern from primary PAH clinically
• Vasodilators contraindicated: severe pulmonary edema
• CT clues
➢ Septal lines
➢ GG nodules (+/-)
➢ Normal left atrium
Mediastinal Fibrosis [Figure 1-14-14]
Mediastinal Fibrosis: Imaging

• Mediastinal contours abnormal
• Coarse calcium
• Soft tissue replaces mediastinal fat
• Constriction, encasement of mediastinal structures
Two coronal CT reconstructions show

mediastinal fibrosis constricting
pulmonary venous drainage at their
entrance to left atrium, thereby
creating unilateral postcapillary
pulmonary hypertension. (Note that
precapiillary hypertension is also
evident: the main PA is dilated and
there is extrinsic compression of the
right PA by mediastinal fibrosis)

Mitral Stenosis
Diagnostic Strategy - Recommended Imaging Studies by ACCP

• CXR
• Echocardiography with Doppler
• V/Q (if CTEPH suspected)
➢ PA gram if positive (resectability)
ACCP Evidence-based Clinical Practice Guidelines. Chest 2004;126:14S-34S
Diagnostic Strategy - Imaging Studies

• From the ACCP:
➢ CXR
➢ Echocardiography with Doppler
➢ V/Q
• We also suggest:
➢ Chest CT/CTA
✧ Precapillary vs. postcapillary origin
✧ Clues to underlying etiology
➢ MRI
✧ Cardiac anatomy/function
✧ Future: hemodynamics of lung perfusion
Go With the Flow ….

• Pulmonary Arterial Hypertension
➢ Enlarged central and hilar vessels
➢ Pruned peripheral vessels
➢ Mosaic perfusion
➢ Por pulmonale
➢ PA atherosclerosis
• Pulmonary Venous Hypertension
➢ Septal lines
➢ Smooth pleural thickening
➢ Ground glass opacities
References
1. Bergin CJ, Rios G, King MA, Belezzuoli E, Luna J, Auger WR. Accuracy of high-resolution CT in identifying
chronic pulmonary thromboembolic disease. AJR Am J Roentgenol 1996; 166:1371-1377.
2. Benjamin MS, Drucker EA, Mcloud TC, Shepard JO. Small pulmonary nodules: Detection at chest CT and
outcome. Radiology 2003; 226:489-493.
3. Botticelli JT, Schlueter DP, Lange RL. Pulmonary venous and arterial hypertension due to chronic fibrous
mediastinitis. Hemodynamics and pulmonary function. Circulation 1966; 33:862-871.
4. Burke AP, Virmani R. Mini-symposium: Pulmonary pathology: Evaluation of pulmonary hypertension in biopsies
of the lung. Current Diagnostic Pathology 1996; 3:14-26.
5. Jones AT, Hansell DM, Evans TW. Quantifying pulmonary perfusion in primary pulmonary hypertension using
electron-beam computed tomography. Eur Respir J 2004; 23:202-207.
6. King MA, Ysrael M, Bergin CJ. Chronic thromboembolic pulmonary hypertension: CT findings. AJR Am J
Roentgenol 1998; 170:955-960.
7. Krowka MJ. Pulmonary hypertension: diagnostics and therapeutics. Mayo Clin Proc 2000; 75:625-630.
8. McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial
hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126:14S-34S.
9. Maltby JD, Gouverne ML. CT findings in pulmonary venoocclusive disease. J Comput Assist Tomogr 1984;
8:758-761.
10. Ng CS, Wells AU, Padley SP. A CT sign of chronic pulmonary arterial hypertension: the ratio of main pulmonary
artery to aortic diameter. J Thorac Imaging 1999; 14:270-278.
11. Primack SL, Muller NL, Mayo JR, Remy-Jardin M, Remy J. Pulmonary parenchymal abnormalities of vascular
origin: high-resolution CT findings. Radiographics 1994; 14:739-746.
12. Randall PA, Heitzman ER, Bull MJ, et al. Pulmonary arterial hypertension: a contemporary review. Radiographics
1989; 9:905-927.

13. Remy-Jardin M, Remy J, Louvegny S, Artaud D, Deschildre F, Duhamel A. Airway changes in chronic pulmonary
embolism: CT findings in 33 patients. Radiology 1997; 203:355-360.
14. Resten A, Maitre S, Humbert M, et al. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive
disease. AJR Am J Roentgenol 2004; 183:65-70.
15. Schoepf UJ, Costello P. Multidetector-row CT imaging of pulmonary embolism. Semin Roentgenol 2003; 38:106-
114.
16. Sherrick AD, Swensen SJ, Hartman TE. Mosaic pattern of lung attenuation on CT scans: frequency among patients
with pulmonary artery hypertension of different causes. AJR Am J Roentgenol 1997; 169:79-82.
17. Simonneau G et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004 Jun 16;43 (12 Suppl
S): 5S-12S.
18. Tan RT, Kuzo R, Goodman LR, Siegel R, Haasler GB, Presberg KW. Utility of CT scan evaluation for predicting
pulmonary hypertension in patients with parenchymal lung disease. Medical College of Wisconsin Lung
Transplant Group. Chest 1998; 113:1250-1256.
19. Worthy SA, Muller NL, Hartman TE, Swensen SJ, Padley SP, Hansell DM. Mosaic attenuation pattern on thin-
section CT scans of the lung: differentiation among infiltrative lung, airway, and vascular diseases as a cause.
Radiology 1997; 205:465-470.

Pulmonary Metastases
Aletta Ann Frazier, MD
Key Points
• The pathogenesis of pulmonary metastases is complex
• The spectrum of radiological manifestations reflects pathways of spread
• Many extrathoracic malignancies produce characteristic radiologic patterns of
pulmonary metastases
Metastatic Disease to the Lung

• Most common lung neoplasm
• Incidence: 20-55% of patients dying from extrathoracic malignancy
• Lung is the ONLY site of metastatic disease in 15-25% of these patients
Metastatic Disease to the Lung:Routes of Spread

• Pulmonary & bronchial arteries
• Pulmonary & pleural lymphatics
• Thoracic duct
• Airways
• Transdiaphragmatic lymphatics
What are the most likely primary extrathoracic malignancies?

• Breast
• Colon
• Uterus
• Kidney
• Prostate
• Oropharynx
• Stomach
• Pancreas
Which malignancies are the most highly predisposed?

• Choriocarcinoma
• Osteosarcoma
• Testicular tumors
• Melanoma
• Ewing’s sarcoma
• Thyroid carcinoma
• Kaposi’s sarcoma
Principle: “Generalizing Sites”

• Certain tumors seed the lung directly, others first drain via another filtration
organ (bone or liver)
• Systemic venous drainage directly to lung
➢ Melanoma
➢ Sarcomas
➢ Choriocarcinoma
➢ Thyroid
➢ Kidney, Testes, Adrenal Gland
➢ Oropharynx
• Venous drainage via liver
➢ Colon
➢ Pancreas
➢ Stomach
• Venous drainage via bone
➢ Prostate
• Dual venous drainage (simultaneous seeding)
➢ Kidney, Bladder, Ureters
➢ Uterus, Cervix
Pulmonary Metastases 138 Chest Radiology

➢ Anus, Rectum
• Complex venous (and lymphatic) drainage
➢Breast
Pathogenesis of Hematogenous Metastases [Figure 1-15-1]

• Tumor cells penetrate draining venules
• Enter systemic venous circulation
• Filtered by pulmonary arterial circulation
Figure 1-15-1
Pathogenesis of Hematogenous Metastases
[Figure 1-15-2]
• Adherence, extravasation in distal arterioles
• Expansile growth in interstitium and alveoli
• Vascularization
➢ Pulmonary arteries
➢ Bronchial circulation
➢ Transpleural collaterals
Parenchymal Nodules: Histology

• Well-defined
• Homogeneous cell population
• Adjacent to arteries and arterioles
• Alveolar septa compressed or obliterated
Nodular Metastases
• Rounded, coalescent or multilobulated
• Multiple
• Peripheral, basilar
Hematogenous metastases arise from tumor cells
• Variable sizes
which penetrate vessels and lymphatics at the
• Mixed areas of viability, necrosis, hemorrhage
primary site, and are transported to the right
heart via the systemic venous circulation
Parenchymal Nodules:
Multidetector Chest CT
• High sensitivity
➢ 95% for nodules >1cm
➢ 91% for nodules .5-1cm
• Low specificity (60% in 40-65 y.o. adults) Figure 1-15-2
➢ intrapulmonary lymph nodes
➢ granulomatous diseases
✧ sarcoidosis
✧ silicosis
➢ amyloidosis
➢ infection
Bloodborne tumor cells arrest in distal arterioles

of the pulmonary circulation, extravasate into the
interstitium, and establish nodules by expansile
growth
Chest Radiology 139 Pulmonary Metastases

Parenchymal Nodular Metastases: [Figure 1-15-3]
HRCT-Pathologic Correlation: Nodules <1cm
• Peribronchovascular (12%)
• Periseptal (28%)
• Intermediate (68%)
Figure 1-15-3
• Angiocentric (76%)
➢ Directly-centered on feeding vessel (18%)
➢ Eccentric to feeding vessel (58%)
Hirakata et al. AJR 1993;161:37-43
Parenchymal Nodules: [Figure 1-15-4]

Imaging Features, Chest CT
• Multiple
• Peripheral, basilar
• Variable in size
• “Random” - eccentrically located between BVB & interlobular
septa
• Occasionally angiocentric
• Less commonly - “cannonball” or miliary
• Rarely
➢ cavitary
➢ calcified
➢ solitary
➢ ground glass halo (hemorrhagic) Secondary pulmonary lobule:
✧ angiosarcoma hematogenous metastases may be
✧ choriocarcinoma angiocentric but are random with
✧ post therapy respect to the secondary pulmonary
lobular architecture
Figure 1-15-4
Figure 1-15-5
Metastatic colon carcinoma in middle aged male:

variable-sized nodules are random, peripheral,
occasionally angiocentric in location
“Cannonball” Metastases [Figures 1-15-5 and 1-15-6]

• Colorectal carcinoma
• Renal cell carcinoma
• Sarcomas
• Melanoma
Cannonball metastases (gross lung)

in young adult male with soft tissue
sarcoma

Micronodular (Miliary) Metastases [Figure 1-15-7] Figure 1-15-6
• Malignancies
➢ Thyroid CA (papillary)
➢ Choriocarcinoma
• Opacities may persist post-treatment (“sterile”)
• DDX
➢ Miliary tuberculosis
➢ Viral pneumonia
➢ Sarcoidosis
Pulmonary Metastases:
Unusual Manifestations
• Cavitary, calcified or solitary pulmonary nodules
• Lymphangitic carcinomatosis
• Tumor thromboembolism
• Endobronchial metastases
• Pleural-based metastases
Cavitation in Metastases [Figure 1-15-8]

• Incidence 4% (vs. 9% of lung primaries)
• Malignancies
➢ Squamous cell neoplasms (head and neck; cervix) - 69%
➢ Adenocarcinomas (colon, breast) - 31%
➢ Sarcomas (bone) - spontaneous ptx
• Wall thickness NOT indicative of benignity
• DDX
➢ septic emboli
➢ vasculitis
➢ collagen vascular disease
Figure 1-15-7
Cannonball metastases in a young
adult male with a soft tissue sarcoma
(scout; axial lung and mediastinal CT
images)
Figure 1-15-8
Micronodular metastases in middle aged

female with thyroid cancer
Cavitary metastases in elderly male

with oropharyngeal cancer

Calcification in Metastases [Figure 1-15-9] Figure 1-15-9
• Malignancies
➢ Osteosarcoma, chondrosarcoma, and synovial sarcomas
➢ Papillary/mucinous adenocarcinomas (ovary, thyroid, GI)
• Post-chemotherapy or post-radiation
• Variable content
➢ Osteoid matrix
➢ Dystrophic calcification
➢ Psammoma body formation
Solitary Metastasis
• Unusual: 1-28% of all metastatic lesions
• 3-10% of all SPNs are solitary metastases
• Variable margins
➢ well-defined Calcified metastases in middle-aged
➢ multilobulated female with ovarian cancer
➢ spiculated
Solitary Metastasis vs. Lung Primary

• “In patients with known primary malignancies and single parenchymal nodules,
the overall incidence of second primary lung carcinoma is greater than that of
solitary metastases”
Coppage et al: J Thorac Imaging 1987; 2(4):24-37

• “The likelihood of a primary lung cancer versus a metastasis depends on the
histologic characteristics of the extrapulmonary neoplasm and the patient’s
smoking history”
Quint et al: Radiology 2000; 217: 257-61

• A SPN is more likely to be bronchogenic CA than a solitary met if the patient
has carcinoma of:
➢ Head and neck
➢ Bladder
➢ Esophagus
➢ Breast
➢ Cervix
➢ Bile Ducts
➢ Ovary
➢ Prostate
➢ Stomach

• The incidence is fairly equal in patients with carcinoma of:
➢ Kidney
➢ Colon
➢ Adrenal gland
➢ Uterus
➢ Salivary or parotid gland
➢ Thyroid gland
• SPN is more likely solitary metastasis in:
➢ Melanoma
➢ Sarcoma (soft tissue, bone)
➢ Testicular carcinoma
Solitary Metastasis

Lymphangitic Carcinomatosis Figure 1-15-10
• Adenocarcinomas in 80%:
➢ Lung
➢ Breast
➢ Stomach
➢ Pancreas
➢ Prostate
➢ Colon
• Incidence 6-55%
• Symptoms: gradual onset dyspnea, cough
• PFT’s: reduced lung compliance & diffusing capacity
• Diagnosis: bronchial lavage or TBB
Lymphangitic Carcinomatosis [Figure 1-15-10]

• Blood-borne tumor cells extravasate and invade lymphatic
channels
• Tumor also enters lymphatics “retrograde” via mediastinal, hilar
lymph nodes (25%)
• Lymphatics expand with tumorlets and edema
• Clusters or cords of tumor in lymphatics of the interlobular septa
Secondary pulmonary lobule:
and peribronchovascular interstitium
lymphangitic carcinomatosis
• Edema and desmoplastic reaction accentuate interstitial
produces smooth and nodular
thickening
expansion of bronchovascular bundle
• Pleural involvement: 2/3
sheaths and interlobular septa
• Nodal involvement: 1/3
Lymphangitic Carcinomatosis:
Imaging Features - Chest radiograph
• Normal (50%)
• Kerley B lines Figure 1-15-11
• Reticulonodular opacities
• Subpleural edema
• Pleural effusion (30-50%)
• Hilar,mediastinal lymphadenopathy (20-40%)
• Bilateral or unilateral findings
Lymphangitic Carcinomatosis
Imaging Features: Chest CT [Figure 1-15-11]
• Smooth or nodular thickening of
➢ Bronchovascular bundles
➢ Interlobular septa (Kerley’s lines; polygonal
arcades)
➢ Lobar fissures (subpleural edema)
• Ground glass opacities
Lymphangitic carcinomatosis in a middle aged
• Focal or unilateral distribution (50%)
female with breast cancer
➢ lung or breast CA
• Lymphadenopathy (up to 50%)
Tumor Embolism
• Lodges in distal arterioles (100-200 micron diameter)
• 26% cancer pts (at autopsy)
• <1% clinically significant
• Complications
➢ Cor pulmonale (PAH)
➢ Lung infarction
➢ Lung hemorrhage
• Parenchymal or lymphatic mets if extravasation

Tumor Embolism Figure 1-15-12
• Malignancies
➢ Stomach
➢ Lung (esp. adenoCA)
➢ Breast
➢ ChorioCA
➢ Ovary
➢ Prostate
➢ Liver
➢ Kidney
➢ Lymphoma
➢ Right atrial myxoma
Tumor Embolism [Figure 1-15-12]

• CXR
➢ typically normal
➢ if widespread: nodules, airspace opacities
• CT
➢ “beading” of peripheral pulmonary arteries Tumor thromboembolism may produce
➢ mosaic perfusion beading along peripheral
➢ wedge-shaped peripheral opacities bronchovascular bundles, as well as
➢ if extravasation: nodules, lymphangitic carcinomatosis pulmonary infarction
[Image courtesy of Mark Gosselin, MD]
Endobronchial Metastases
• Tumor arrives via bronchial arteries & peribronchial lymphatics Figure 1-15-13
• Rarely, via airways (BAC)
• 2-5% incidence in pts dying from metastases
• Malignancies
➢ Kidney
➢ Colon, Rectum
➢ Breast
➢ Melanoma
➢ Pancreas
• Mean interval from diagnosis of primary: 65 months
• Mean survival after discovery: 15.5 months
• Treatment options
➢ Radiation, Endobronchial metastases with left
➢ Chemotherapy upper lobe collapse (renal cell
➢ Surgery carcinoma)
➢ Interventional bronchoscopy (stenting, laser or mechanical
resection, brachytherapy, photodynamic therapy)
Chan et al. Curr Opin Pulm Med 2003;9:301-308
Endobronchial Metastases: Imaging Features [Figure 1-15-13] Figure 1-15-14

• Intraluminal soft tissue mass
• Atelectasis or post-obstructive pneumonia
• Serpiginous or nodular opacities (distal mucoid
impaction)
• Hilar mass (if adjacent mediastinal invasion)
• DDX: bronchogenic carcinoma
Pleural Metastases [Figures 1-15-14 to 1-15-16]

• Malignancies
➢ Lung
➢ Breast (50% of patients)
➢ Ovary
➢ Stomach
➢ Lymphoma
• Arise from lymphangitic or vascular invasion
Large pleural effusion and pleural-based nodule in

patient with breast cancer

• Typically manifest as exudative pleural effusion Figure 1-15-16
➢ Pleural nodules less common
• Scattered nodules on pleural surface
• Visceral & parietal pleura typically both involved
• Radiologic DDX
➢ asbestos exposure
➢ splenosis
• Rind-like or sheet-like pattern
• Radiologic DDX
➢ mesothelioma
➢ post-inflammatory fibrothorax
Figure 1-15-15
Pleural rind-like metastases in elderly female with

NSCLC
Large pleural effusion and pleural-based nodule in

patient with breast cancer
Do we impact the management of cancer

patients with metastatic disease to the lung?
Statement from the Fleischner Society:

Guidelines for Management of Pulmonary Nodules
MacMahon H et al. Radiology

2005;237:395-400

Parenchymal Nodules
Assessing Therapeutic Response
• Tumor doubling time
➢ Definition: 25% increase in tumor diameter separated by time (Tdt)
➢ Nodule growth rates vary widely
✧ according to histologic tumor cell type
✧ within the same patient
➢ Proposed reasons for differences
✧ Not all nodules are tumor (benign or inflammatory)
✧ Smaller mets grow at faster rates than larger mets
✧ Nodules represent different monoclonal cell populations (with variable
responses to treatment)
• Document changes in several nodules in the same patient for optimal
therapeutic assessment - impacts treatment strategy & participation in clinical
trials
Chojniak et al. Am J Clin Oncol 2003;26(4):374-377
Parenchymal Nodules
Indications for Metastasectomy
• To cure, but only if
➢ Complete resection possible
➢ No extrathoracic metastases (EXCEPT colon CA with liver mets)
➢ No therapeutic alternative (chemotherapy-insensitive tumors)
➢ Multiple nodules NOT necessarily a contraindication
• To prolong 5-year survival
➢ Colorectal cancer: up to 20-50%
➢ Osteogenic & soft tissue sarcomas: up to 40%
➢ Melanoma, renal cell, head & neck, female GU: up to 30%
➢ Thyroid, parathyroid: up to 61%
Yoneda et al. Curr Opin Pulm Med 2000;6:356-363.
“A Sarcoid-like Reaction”
• Rare but well-documented
• Follows resection or treatment
➢ Lymphoma (Hodgkin & Non-Hodgkin)
➢ AML
➢ Lung CA
➢ Testicular CA
➢ Gastric CA
➢ Renal CA
• Radiologic manifestations
➢ Mediastinal, hilar lymphadenopathy
➢ Pulmonary nodules or consolidations
➢ Systemic sarcoidosis absent
➢ Positive on FDG-PET: mimics recurrence
• Biopsy required for confirmation
• ? Local immunologic response to tumor cells
Patterns of Metastatic Disease to the Lung

Overview
• Parenchymal nodules
➢ Well-circumscribed, random or angiocentric, basilar>apical
➢ Unusual: cavitary, calcified, solitary
• Lymphangitic carcinomatosis
➢ Septal lines, nodular/thickened fissures, GGO
➢ Lymphadenopathy
• Tumor thromboembolism
➢ Beading of peripheral arteries
➢ Mosaic perfusion
➢ Pleural-based opacity (infarction)

• Endobronchial nodule
➢ Rounded defect in airway, or cut-off of airway lumen
➢ Post-obstructive atelectasis, pneumonia, mucoid impaction
• Pleural-based metastases
➢ Nodules on pleural surface
➢ Variation: rind-like pattern mimics mesothelioma
References
1. Chan AL, Yoneda KY, Allen RP, Albertson TE. Advances in the management of endobronchial lung malignancies.
Curr Opin Pulm Med 2003; 9:301-308.
2. Chojniak R, Younes RN. Pulmonary metastases doubling time: Assessment by computed tomography. Am J Clin
Oncol 2003; 26(4):374-377.
3. Coppage L, Shaw C, Curtis A. Metastatic disease to the chest in patients with extrathoracic malignancy. J Thorac
Imag 1987; 2(4):24-37.
4. David SD, Westcott J, Fleishon H, Gefter WB, Henschke CI, McLoud TC, Pugatch RD, Sostman HD, Tocino I, White
CD, Yankelevitz D, Bode FR. Screening for pulmonary metastases. American College of Radiology. ACR
Appropriateness Criteria. Radiology 2000; 215 Suppl:655-62.
5. Davis S. CT evaluation for pulmonary metastases in patients with extrathoracic malignancy. Radiology 1991; 180:1-
12.
6. Heffner JE, Milam MG. Sarcoid-like hilar and mediastinal lymphadenopathy in a patient with metastatic testicular
cancer. Cancer 1987; Oct 1;60(7):1545-7.
7. Hirakata K, Nakata H, Haratake J. Appearance of pulmonary metastases on high-resolution CT scans: comparison
with histopathologic findings on autopsy specimens. Am J Roentgenol 1993; 161:37-43.
8. Jungraithmayr W, Hasse J, Stoelben E. Completion pneumonectomy for lung metastases. EJSO 2004; 30:1113-1117.
9. Kozuka T, Johkoh T, Hamada S, Maito H, Tomiyama N et al. Detection of pulmonary metastases with multi-detector
row CT scans of 5-mm nominal section thickness: Autopsy lung study. Radiology 2003; 226:231-234.
10. Libshitz HI, North LB. Pulmonary metastases. Radiol Clin North Am 1982; 20:437-451.
11. MacMahon H et al. Guidelines for management of small pulmonary nodules detected on CT scans: A statement from
the Fleischner Society. Radiology 2005; 237:395-400.
12. Marglin S, Mortimer J, Castellino R. Radiologic investigation of thoracic metastases from unknown primary sites. J
Thorac Imag 1987; 2(4):38-43.
13. Milne EC, Zerhouni EA. Blood supply of pulmonary metastases. J Thorac Imag 1987; 2(4):15-23.
14. Murata K, Takahashi M, Mori M, Kawaguchi N et al. Pulmonary metastatic nodules: CT-pathologic correlation.
Radiology 1992; 182:331-335.
15. Parra ER et al. Pulmonary and mediastinal “sarcoidosis” following surgical resection of cancer. Pathol Res Pract
2004; 200(10);701-5.
16. Poste G, Fidler I. The pathogenesis of cancer metastasis. Nature 1980; 283:139-145.
17. Pugatch RD. Radiologic Evaluation in Chest Malignancies. Chest 1995; 107:294S-297S.
18. Quint L, Park C, Iannettoni M. Solitary pulmonary nodules in patients with extrapulmonary neoplasms. Radiology
2000; 217:257-261.
19. Seo JB, Im J, Goo JM, Chung MJ, Kim M. Atypical pulmonary metastases: spectrum of radiologic findings.
RadioGraphics 2001; 21:403-417.
20. Snyder BJ, Pugatch RD. Imaging characteristics of metastatic disease to the chest. Chest Surg Clin N Am 1998;
8(1):29-48.
21. Woodard PK, Dehdashti F, Putman CE. Radiologic diagnosis of extrathoracic metastases to the lung. Oncology 1998;
12(3): 441-444.
22. Yoneda KY, Louie S, Shelton D. Approach to pulmonary metastases. Current Opinion in Pulmonary Medicine 2000;
6(4):356-363.
23. Zhao B, Schwartz LH, Moskowitz CS et al. Pulmonary metastases: Effect of CT section thickness on measurement
– initial experience. Radiology 2005; 234:934-939.

Differential Diagnosis of Mediastinal Masses
Learning Objectives:
• To define the mediastinum and describe the mediastinal compartments
• To provide a classification for a practical approach to the imaging diagnosis of
mediastinal masses
• To list clinical and cross-sectional imaging features that allow a focused differential
diagnosis
• To describe lesions with pathognomonic imaging features
• To differentiate neoplastic from non-neoplastic conditions with emphasis on
management
Mediastinal Compartments [Figure 1-16-1] Figure 1-16-1

• Mediastinum - space between pleural surfaces and lungs
• Bound by sternum and vertebrae
• From thoracic inlet to diaphragm
• Thymus, lymph nodes, heart, great vessels,trachea, esophagus,
nerves and other soft tissues
• Arbitrary division into compartments - no anatomic boundaries
The Mediastinal Compartments [Figures 1-16-2 and 1-16-3]

• Anatomic - Superior, anterior, middle, posterior
Excludes paravertebral areas
• Surgical - Superior, anterior, middle, posterior
Includes paravertebral areas
• Radiographic (Felson) - Anterior, middle, posterior The mediastinum
• Radiographic (Fraser, Müller, Colman, Paré) - Anterior, middle-posterior,
paravertebral
Figure 1-16-2
Figure 1-16-3
Anatomic and Surgical Mediastinal Compartments

Radiographic Mediastinal
Compartments
Mediastinal Masses 148 Chest Radiology

Mediastinal Masses
• Patients are often asymptomatic
• 83% of asymptomatic masses are benign
• 57% of symptomatic masses are malignant
• Approximately 1/3 are malignant
• Approximately 1/10 are vascular
Mediastinal Masses – Mayo Clinic (N=1,064)

• Neurogenic tumor 20%
• Thymoma 19%
• Cysts 18%
• Lymphoma 30%
Teratoma
Granuloma
Mediastinal goiter
Wychulis et al. J. Thorac Cardiovasc Surg 1971
Approach to Mediastinal Masses

• Clinical
➢ Demographics (age, gender) / Symptoms
• Radiography
➢ Mediastinal compartment / Adjacent structures
➢ Focal mass vs. diffuse mediastinal enlargement
➢ Lesion contours / Density
• Cross-sectional Imaging
➢ Location / Relationship to normal structures
➢ Morphologic features / Associated findings
Mediastinal Masses
• Neoplasia
➢ Malignant (secondary / diffuse)
➢ Benign and malignant (primary / focal)
• Congenital cysts
• Glandular enlargement
• Vascular lesions
• Herniations / Esophageal abnormalities Figure 1-16-4
• Miscellaneous conditions
Malignant Neoplasia – Lung Cancer

• Small cell / poorly differentiated lung cancer
• Elderly smokers; males and females
• Cough, dyspnea, SVC syndrome, weight loss
• Mediastinal mass, lymphadenopathy, local invasion
• Primary neoplasm may not be evident
• Non-surgical lesion
Malignant Neoplasia - Metastases [Figure 1-16-4]

• Known malignancy
• Renal cell carcinoma
• Testicular carcinoma Metastatic renal cell carcinoma
• Head and neck cancer
• Breast carcinoma
• Melanoma
Chest Radiology 149 Mediastinal Masses

Malignant Neoplasia – Lymphoma Figure 1-16-5
• Hodgkin disease / Non-Hodgkin lymphoma
• All age groups (young patients); Males and females
• Palpable lymphadenopathy, constitutional symptoms
• Lobular / diffuse mediastinal enlargement
• Prevascular, paratracheal lymphadenopathy
➢ Nodal coalescence, local invasion
• Primary mediastinal lymphoma
• Non-surgical lesion
Lymphoma
• Non-Hodgkin lymphoma - 75% of all cases
• 50-70% of mediastinal lymphoma is Hodgkin disease
➢ 15-21% is non-Hodgkin lymphoma
• Hodgkin – 66% intrathoracic at presentation
➢ Non-Hodgkin – 37% intrathoracic at presentation
• Treatment: Radiotherapy, chemotherapy
Hodgkin Disease: Microscopic Features
Lymphoma: Clinical Features
• Hodgkin Disease Figure 1-16-6
➢ Males = Females (NSHD, 2 X more common in females)
➢ Bimodal distribution: 2nd to 3rd and > 5th decades
➢ Lymphadenopathy: cervical, supraclavicular
➢ 20-30%; fever, night sweats, wt. loss
• Non-Hodgkin lymphoma
➢ Systemic disease with constitutional symptoms: lymphadenopathy,
local invasion
➢ Lymphoblastic - male children / adolescents
➢ Diffuse large-B cell - young adult females
Lymphoma: Pathologic Features [Figures 1-16-5 and 1-16-6]

• Hodgkin Disease
➢ Nodal cellular infiltrate, collagenous connective tissue (NS), Reed-
Sternberg cell
➢ Lymphadenopathy, nodal coalescence, primary thymic
involvement, cystic change, hemorrhage, necrosis
➢ Local invasion (including chest wall), hemorrhage, necrosis
• Non-Hodgkin Lymphoma
➢ Lymphoblastic (precursor T-lymphoblastic) -lymphoblasts
➢ Diffuse large B-cell (primary mediastinal [thymic] large B-cell);
Hodgkin Disease: Gross Features
large cells, vesicular nuclei, prominent nucleoli
➢ Large, infiltrative, locally invasive mass, necrosis
Lymphoma: Imaging Features [Figures 1-16-7 to 1-16-9] Figure 1-16-7

• Lobulated unilateral/bilateraldiffuse mediastinal enlargement
• Hodgkin Disease
➢ Intrathoracic involvement in 85%
➢ Lymphadenopathy; prevascular, paratracheal
➢ Nodal coalescence (homogeneous or heterogeneous)
➢ Ca++; 1% - 1 year post-therapy, rare pre-therapy
• Non-Hodgkin
➢ Prevascular, paratracheal adenopathy
➢ Isolated involvement of other mediastinal lymph nodes
• Local invasion
• Primary mediastinal lymphoma
Hodgkin Disease: Imaging Features

Non-Hodgkin lymphoma – nodal coalescence with low

attenuation corresponding to necrosis
Hodgkin disease – prevascular and paratracheal

lymphadenopathy and left pleural effusion
Secondary Neoplasia
• Diffuse, bilateral mediastinal enlargement
• Lymphadenopathy
• Local invasion
• Metastases
• Other imaging features of malignancy
Non-Neoplastic Lymphadenopathy
• Infection
➢ Fungal: Mediastinal fibrosis; Calcification
➢ Other granulomatous infections
• Sarcoidosis
➢ Bilateral symmetric hilar lymphadenopathy
➢ Typical lung parenchymal involvement
• Castleman disease
➢ Enhancement / calcification (10%)
Mediastinal Fibrosis
• Granulomatous lymphadenopathy
• Young patients with signs and symptoms of obstruction Figure 1-16-10
➢ Trachea, bronchi, esophagus, vessels
• Mediastinal mass, circumscribed or locally invasive,
calcification
• Systemic antifungal agents, excision, dilatation,
bypass graft
• 30% mortality
Castleman Disease [Figure 1-16-10]

• Angiofollicular or giant lymph node hyperplasia
• Hyaline vascular type (> 90%) vs. plasma cell variant
• Localized vs. systemic
• Adult females (M:F - 4:1) may be asymptomatic
• Middle mediastinal / hilar mass
➢ Solitary mass
➢ Dominant mass with lymphadenopathy
➢ Multiple enlarged lymph nodes
➢ Enhancement, calcification (10%)
Castleman Disease: Enhancing mediastinal
lymphadenopathy

Primary Neoplasia Figure 1-16-11
• Thymus
➢ Thymoma
➢ Thymic malignancy
➢ Thymolipoma
➢ Germ cell neoplasm
• Neurogenic neoplasms
Thymoma
• Epithelial neoplasm, most common primary thymic neoplasm
• Slow growth, “benign” behavior
• M=F; 70% in the 5th and 6th decades
• Most patients asymptomatic
• 25-30% with symptoms of compression/invasion
• Associated parathymic syndromes:
➢ Myasthenia gravis
➢ Pure red cell aplasia
➢ Hypogammaglobulinemia
Thymoma and Myasthenia Gravis

• Myasthenia gravis (MG) – autoimmune neurological disorder
• 85% of patients with MG have follicular thymic hyperplasia
• 15% of patients with MG have a thymoma
Thymoma: Microscopic Features
• Of all patients with thymoma, 30-50% have MG
Thymoma: Pathologic Features [Figures 1-16-11 to 1-16-13] Figure 1-16-12

• Lymphocytes and epithelial cells in varying proportions
• WHO 1999 classification (morphology and lymphocyte-to-epithelial
cell ratios)
• Types A, AB, B1, B2, B3
• Tumor lobules compartmentalized by fibrous septa
• Encapsulated vs. Invasive
• Spherical mass, variable size, lobular contours, typically encapsulated
• Hemorrhage, necrosis, cystic change (mural nodules)
• Invasive thymoma - microscopic documentation of capsular invasion,
local invasion, tumor implants, metastases
Figure 1-16-13 Thymoma: pathologic features –

tumor lobules compartmentalized by
fibrous bands
Thymoma: Gross Features

Thymoma: Imaging Features [Figures 1-16-14 to 1-16-18] Figure 1-16-14
• Anterior mediastinal mass; lobular, unilateral,
variable size
• Normal radiographs in 25% (occult thymoma)
• Focal, spherical, homogeneous or heterogeneous
➢ Necrosis, cystic (mural nodules), calcification
(typically curvilinear and peripheral)
• No lymphadenopathy
• Exclude local invasion of fat, cardiovascular
structures, lung
• Pleural implants (may cause diffuse pleural
thickening)
Thymoma: Unilateral, lobular, left anterior
mediastinal mass
Figure 1-16-15
Figure 1-16-16
Occult Thymoma: Well-defined, lobular, unilateral,

prevascular mass
Thymoma: Well-defined right cardiophrenic angle mass

with irregular low attenuation corresponding to necrosis Figure 1-16-18
Figure 1-16-17
Cystic thymoma: Unilateral spherical cystic mass with

peripheral curvilinear calcification and mural nodules
Invasive thymoma: Direct invasion of left

brachiocephalic vein

Thymoma: Staging (Masaoka) Figure 1-16-19
(10 - year survival)
I Encapsulated / no microscopic capsular invasion (86-100%)
II Microscopic invasion into surrounding fat / mediastinal pleura,
microscopic capsular invasion (55-100%)
III Macroscopic invasion of adjacent organs, pericardium, heart,
great vessels, lung (47-60%)
IVa Pleural / pericardial dissemination (0-11%)
IVb Lymphatic / hematogenous dissemination
Thymoma: Therapy / Prognosis

• Encapsulated; complete excision
➢ Best prognosis
➢ Occasional local recurrence, distant metastases
• Post-operative radiation for invasive thymoma to decrease local Thymic Carcinoid: Mediastinal mass with
recurrence adjacent lymphadenopathy in a patient
• Chemotherapy for for progression after surgery and unresectable with ACTH production and MEN 1
lesions
Thymic Malignancy: Carcinoid / Carcinoma

• Rare malignant epithelial neoplasms
• Symptomatic patients
• Poor prognosis
Thymic Carcinoid
• Neuroendocrine neoplasm; atypical carcinoid (necrosis / mitoses / invasion) Figure 1-16-20
• Males > Females; 3:1; wide age range (average, 43 yrs)
• 50% functionally active
➢ ACTH – Cushing syndrome (33-40%)
• MEN type 1 – (Wermer syndrome) (19-25%)
➢ Hyperparathyroidism (90%), islet cell tumor of pancreas
(80%) pituitary adenoma (65%)
Thymic Carcinoma
• Male > Female; wide age range (mean: 5th decade)
• Several cell types identical to primary lung cancer; R/O
metastases
• WHO Type C thymoma
Thymic Carcinoid / Carcinoma: Imaging Features

[Figure 1-16-19]
• Large anterior mediastinal mass (R/O thymoma)
• R/O metastatic lung malignancy (histology)
• Lymphadenopathy
• Local invasion, pleural or pericardial effusion/implantation,
metastases
• Carcinoid
➢ Octreotide imaging for diagnosis for occult (non-specific -
metastases and other neoplasms) Thymolipoma, microscopic features:
Thymic tissue admixed with mature
Thymolipoma adipose tissue
• Rare benign thymic neoplasm
• M=F; wide age range (average age, 28 yrs)
• Asymptomatic patients: 50%
➢ Symptoms with large tumors
Thymolipoma: Pathologic Features [Figure 1-16-20]

• Encapsulated, soft, lobular, yellow
• Mature adipose tissue and thymic tissue in variable proportions

Thymolipoma: Imaging Features [Figure 1-16-21] Figure 1-16-21
• Well-defined anterior / inferior mediastinal mass
➢ Unilateral or bilateral, slow growth
• May conform to shape of structures
➢ R/O cardiac enlargement / diaphragmatic
elevation
➢ Positional change in shape
• Anatomic connection to the thymus (pedicle)
• Mixed fat and soft tissue attenuation/signal
Germ Cell Neoplasms

• Most common in the gonad
• Extragonadal germ cell neoplasms; midline
locations, most commonly the mediastinum
• Postulated origin in multipotential primitive germ
cells “misplaced” during embryogenesis
• Cell types:
➢ Teratoma (mature, immature [immature
Thymolipoma, imaging features – anterior mediastinal
neuroectoderm], “malignant” [mixed malignant
mass with anatomic connection with the thymus and
germ cell neoplasm])
mixture of fat and soft tissue attenuation
➢ Seminoma
➢ Non-seminomatous germ cell neoplasms Figure 1-16-22
Mature Teratoma
• 60-75% of mediastinal germ cell neoplasms
• Males=Females
• Children and young adults (< 40 yrs)
• Often asymptomatic
• Symptoms of compression or rupture
Mature Teratoma: Pathologic Features

[Figures 1-16-22 and 1-16-23]
• More than one embryonic germ cell layer
➢ Ectoderm – skin, dermal appendages
➢ Mesoderm – bone, cartilage, muscle
➢ Endoderm – GI, respiratory tissue, mucus glands
• Spherical, encapsulated, lobulated
• Multilocular or unilocular cyst
➢ Oily, sebaceous, gelatinous material (lipid)
➢ Focal solid areas: hair, teeth, bone
Teratoma, microscopic features:
cystic neoplasm with ectodermal,
Figure 1-16-23 mesodermal and endodermal
components
Teratoma, gross features: multilocular

cystic mass

Mature Teratoma: Imaging Features [Figure 1-16-24] Figure 1-16-24
• Unilateral anterior mediastinal mass
• Spherical, lobular contours, well-defined
• Multilocular cystic - 85%
• Attenuation:
➢ Fluid 89%, Fat 76%, Ca++ 53%
➢ Fat fluid level - 11%
➢ ST/FL/FAT/Ca++ 39%
➢ ST/FL/FAT 24%
➢ ST/FL 15%
Mature Teratoma: Therapy and Prognosis

• Complete excision is curative
• Excellent prognosis
➢ Near 100% five-year survival
Teratoma, imaging features: Unilateral,
Germ Cell Neoplasms anterior mediastinal, multilocular cystic
• Most common in the gonad mass with intrinsic fluid, soft tissue, fat
• Extragonadal germ cell neoplasms; midline locations, most and calcium
commonly the mediastinum
• Postulated origin in multipotential primitive germ cells “misplaced” during
embryogenenesis
• Cell types:
➢ Teratoma (mature, immature, “malignant”)
➢ Seminoma
➢ Non-seminomatous germ cell neoplasms Figure 1-16-25
Seminoma
• 40% of malignant germ cell neoplasms of a single histology
➢ Caucasian males, third to fourth decades
• Most patients are symptomatic
• Rounded cells with sharp borders, clear cytoplasm, fibrous
bands, lymphocytes, plasma cells, granulomas
• Homogeneous soft tissue mass
• Radiation therapy / Cisplatin-based chemotherapy
➢ 60-80% long-term survival
Seminoma: Imaging Features [Figure 1-16-25] Seminoma, imaging features: Diffuse

• Anterior mediastinal mass (both sides of midline) homogeneous anterior mediastinal mass
➢ Large, bulky, well-defined, lobulated, locally invasive with mass effect
• CT:
➢ Homogeneous soft tissue mass
➢ Mimics nodal coalescence
➢ Slight homogeneous contrast-enhancement
➢ Rarely necrosis / cystic change (8%)
Figure 1-16-26
Non-Seminomatous
Malignant Germ Cell Neoplasms
• Yolk sac (endodermal sinus) tumor
• Embryonal carcinoma
• Choriocarcinoma
• Mixed germ cell neoplasm
• Males, 90% symptomatic
➢ Klinefelter syndrome (20%); hematologic malignancy
• Alpha-fetoprotein (EST, EC)
➢ B-human chorionic gonadotropin (choriocarcinoma)
➢ LDH (60%) tumor burden
• Large, unencapsulated
Non-seminomatous malignant germ cell
• Hemorrhage, necrosis, “cyst” formation
neoplasm, imaging features: Large
• Cisplatin-based chemotherapy; excision of residual tumor
anterior mediastinal locally invasive
heterogeneous mass

Non-Seminomatous GCN: Imaging Features Figure 1-16-27
[Figure 1-16-26]
• Large, well or poorly-defined anterior mediastinal mass
➢ Extends to both sides of midline
• Heterogeneous
➢ Large areas of central low attenuation
➢ Frond-like peripheral soft tissue
• Loss of tissue planes
➢ Local invasion, lymphadenopathy
Neurogenic Neoplasms [Figure 1-16-27]

• 20% of primary mediastinal neoplasms
➢ 35% in children
• 70–80% benign
• Peripheral nerves Neurogenic neoplasms may arise from
➢ Schwannoma peripheral nerves or sympathetic
➢ Neurofibroma ganglia
➢ Malignant peripheral nerve sheath tumor
• Sympathetic ganglia Figure 1-16-28
➢ Ganglioneuroma
➢ Ganglioneuroblastoma
➢ Neuroblastoma
Schwannoma / Neurofibroma [Figure 1-16-28]

• Schwannoma – Most common mediastinal neurogenic neoplasm
➢ Spherical, encapsulated
➢ Cellular and less cellular areas (Antoni A / B)
• Neurofibroma – second most common mediastinal neurogenic
neoplasm
➢ Spherical/fusiform, unencapsulated
• Calcification, cystic change, hemorrhage
• Young adults; 3rd and 4th decades
• Most (65%) asymptomatic
• Symptoms and signs of compression
Schwannoma / Neurofibroma: Imaging Features Schwannoma, gross features:

[Figures 1-16-29 and 1-16-30] Heterogeneous spherical mass
• Spherical, smooth / lobular, well-defined paravertebral mass
• Osseous findings (50%): pressure erosion/deformity of ribs or vertebrae;
expanded neuroforamen
• Homogeneous/heterogeneous
➢ Heterogeneous enhancement; Ca++ in 10%
➢ Growth into spinal canal in 10%
• MR Imaging – R/O spinal involvement
➢ T1 – Low-to-intermediate signal
➢ T2 – Foci of high signal
Schwannoma, imaging features: Unilateral paravertebral

spherical mass Schwannoma, imaging features: Intraspinal extension

Neurofibromatosis (NF1)
• Multiple neoplasms (including ganglioneuroma)
• Plexiform neurofibroma
• Vagus nerve, sympathetic chain, phrenic nerve
• Diffuse enlargement of peripheral nerve
• Multiple masses along a nerve
Malignant Peripheral Nerve Sheath Tumor

• Most frequent in the paravertebral region
• Rare among neurogenic neoplasms
• Large (> 5 cm) spherical mass
➢ Central low attenuation – necrosis
➢ Calcification
➢ May exhibit local invasion
Peripheral Nerve Neoplasms: Therapy and Prognosis

• Excision
• Schwannoma/Neurofibroma
➢ Excellent prognosis
• Malignant peripheral nerve sheath tumor
➢ Solitary – 75% five-year survival
➢ Neurofibromatosis – 30% five-year survival
Thoracic Meningocele
• Intrathoracic extrusion of meninges and their fluid content
• Well-defined spherical paravertebral mass
• Enlarged neuroforamen, pressure erosion, sclerosis Figure 1-16-31
• Homogeneous, fluid attenuation / signal
Ganglioneuroma [Figure 1-16-31]

• Children, adolescents, young adults
➢ Asymptomatic patients
• De novo; maturation of neuroblastoma
• Benign paravertebral neoplasm
• Mature ganglion cells, Schwann cells, nerve fibers
• Encapsulated, elongate mass
➢ Gray / yellow with lobular surface
Ganglioneuroma: Imaging Features

• Well-defined, oblong paravertebral mass
• Osseous erosion / displacement
• Homogeneous or heterogeneous
➢ Calcification in 25%
Ganglioneuroma, gross features:
• MR: Homogeneous intermediate signal on T1 / T2
Elongate paravertebral mass
➢ R/O intraspinal extension
Ganglioneuroblastoma/Neuroblastoma
• Infants and young children
• Asymptomatic; chest wall pain, paraplegia, Horner syndrome, diarrhea,
hemothorax
• Elevation of urine catecholamines
➢ Elevation of urine/serum VMA (screening)
• Neuroblastoma – Elongate paravertebral mass
➢ 50% < 2 years
➢ 90% < 5 years
➢ May be congenital

Ganglioneuroblastoma/Neuroblastoma: Pathologic Features Figure 1-16-32
• Adrenal – most common location
➢ Paravertebral – second most common location
• Ganglioneuroblastoma: Neuroblasts and ganglion cells
➢ Well / poorly differentiated
• Neuroblastoma: Neuroblasts, Homer-Wright pseudorosettes
➢ Well / poorly differentiated
Neuroblastoma: Imaging Features [Figure 1-16-32]

• Well-defined large elongate paravertebral mass
• Radiographic evidence of Ca++ in 10%
• Osseous erosion
• R/O intraspinal growth
• Local soft tissue invasion
Sympathetic Ganglia Tumors

• Ganglioneuroma
➢ Excision is curative
• Ganglioneuroblastoma
➢ Five-year survival near 90%
• Neuroblastoma
➢ Five-year survival – 30%
➢ More favorable course with: age < 2 yrs, mediastinal
➢ Spontaneous maturation to ganglioneuroma
Paraganglioma
• Middle mediastinum: Aortopulmonary paraganglia
➢ Paravertebral: Aortico sympathetic paraganglia
➢ Heart
• Adults (average age 30-40 yrs)
➢ Males > Females; 2:1
➢ Asymptomatic; excess catecholamines Neuroblastoma, imaging features:
• Well-defined spherical mass Unilateral calcified paravertebral mass in
➢ Homogeneous/heterogeneous a neonate with intraspinal extension
• Marked contrast enhancement
➢ 90% uptake of I131 or I123 MIBG
Primary Neoplasia
• Benign
➢ Focal, unilateral mass
➢ No lymphadenopathy
Figure1-16-33
➢ No local invasion
• Malignant (invasive)
➢ Focal, unilateral mass
➢ Lymphadenopathy
➢ Local invasion
Bronchogenic Cyst [Figure 1-16-33]

• Most common congenital cyst of the mediastinum
• Abnormal ventral foregut bud
• Failure to induce mesenchymal development to lung
parenchyma
• Mediastinum (85%), pericardium, diaphragm, pleura and
lung
Bronchogenic Cyst: Clinical Features

• Rare in infants, infrequent in children
• Young adults
• Asymptomatic – incidental finding
• Symptomatic – chest pain, mass effect, obstruction,
infection Bronchogenic cyst: Typical subcarinal location
• Excision, observation, drainage, sterile alcohol ablation

Bronchogenic Cyst : Pathologic Features Figure 1-16-34
[Figures 1-16-34 and 1-16-35]
• Respiratory epithelium
• Wall: bronchial glands, cartilage, smooth muscle
• Closed foregut connection
• Spherical, ovoid, unilocular
• Thin wall
• Fluid variable: clear, turbid, hemorrhagic, serous, viscous
Bronchogenic Cyst: Imaging Features

[Figures 1-16-36 and 1-16-37]
• Well-defined, spherical, middle mediastinal mass
• Near trachea, carina, stem bronchi
• CT:
➢ Thin smooth wall (enhancement)
➢ Water (40%) or soft tissue (43%) attenuation
➢ Homogeneous / heterogeneous, non-enhancing contents
• MR:
➢ T1 - variable (slightly hyperintense to muscle)
➢ T2 - isointense or hyperintense to CSF
• Thin-walled pulmonary cyst; air, fluid, air-fluid level
Bronchogenic cyst, microscopic
features: respiratory epithelium with
Other Congenital Cysts cartilage and smooth muscle in wall
• Foregut cysts
• Esophageal - within esophageal; ectopic gastric mucosa Figure 1-16-35
• Neuroenteric - Associated spinal anomaly
• Pericardial - Cardiophrenic angle, imperceptible wall, fluid
attenuation; asymptomatic patients
Figure 1-16-36
Bronchogenic cyst, imaging features: Subcarinal

spherical mass with extension to the right
Bronchogenic cyst, gross features: Thin-
walled unilocular cyst
Figure 1-16-37
Bronchogenic cyst, imaging features: spherical subcarinal mass that may not exhibit
water attenuation

Thymic Cyst [Figures 1-16-38 and 1-16-39] Figure 1-16-38
• Uncommon (3% of mediastinal masses)
• Acquired vs. Congenital
• Children / young adults
• Association with neoplasia, AIDS
Diffuse infiltrative lymphocytosis syndrome (DILS)
• Epithelial lining and thymus in cyst wall
• Multilocular / unilocular
• R/O cystic neoplasm
Congenital Cysts
• Focal, spherical
• Unilocular
• Thin-walled
• No mural nodules
• Along foregut-derived structures
Thymic Hyperplasia
• Lymphoid hyperplasia (lymphofollicular / autoimmune thymitis) -
secondary follicles with germinal centers; may not produce
thymus enlargement
• Myasthenia gravis, hyperthyroidism, lupus, scleroderma, RA, Thymic cyst, gross features: multilocular
cirrhosis cyst
• True hyperplasia - global increase in the size and weight of the Figure 1-16-39
thymus
• Rebound hyperplasia - following chemotherapy (2 weeks to 14
months), steroids or severe insult
• Ant. mediastinal widening
• Homogeneous soft tissue
• Maximal thickness
➢ Under 20 years – 1.8 cm
➢ Over 20 years – 1.3 cm
• Follicular thymic hyperplasia – normal or mildly enlarged thymus
Mediastinal Goiter [Figure 1-16-40]

• 20% of cervical goiters
• Asymptomatic females: incidental finding Thymic cyst, imaging features:
➢ May produce symptoms by mass effect Multilocular cyst
• Adenomatous goiter; rarely malignancy or thyroiditis
• Fibrous capsule; nodules composed of thyroid follicles
• Hemorrhage, calcification, cystic change Figure 1-16-40
Mediastinal goiter, pathologic features: iodine content, well-defined

lobular soft tissue mass

Mediastinal Goiter: Imaging Features Figure 1-16-41
[Figures 1-16-41 and 1-16-42]
• Unilateral anterior mediastinal mass (80%)
➢ Other compartments also affected, R > L
• Well-defined lobular borders
• Cervico-thoracic sign
➢ Continuity with cervical thyroid
• Calcification - punctate, coarse, curvilinear
• Cystic change
• High attenuation
➢ Intense, sustained contrast enhancement
Figure 1-16-42
Mediastinal goiter, imaging features:

Large calcified unilateral mass with
cervicothoracic sign
Figure 1-16-43
Mediastinal goiter, imaging features: Continuity between cervical and

mediastinal portions of the mass, high attenuation and calcification
Parathyroid Adenoma
• Ectopic parathyroid glands: superior pole of thymus (39%),
mediastinum (2%), intrathyroid (0.2-3.5%)
• Primary hyperparathyroidism post surgical parathyroidectomy
• MEN I
• Imaging
➢ Tc99m / Tl201 subtraction imaging
➢ T123 / Tl201
➢ Tc99m - Sestamibi (mitochondria)
➢ Single radionuclide/Dual radionuclide
• CT/MRI correlation of mediastinal uptake
Glandular Enlargement
• Anatomically related to normal gland
• Continuity with normal gland
• Function similar to that of normal gland
Lymphangioma, microscopic features:
Interconnecting endothelial lined
Lymphangioma vascular channels
• Benign mesenchymal mediastinal tumor
• Proliferation of lymphatic vessels without communication with lymphatic tree
• Developmental vs. neoplasm vs. hamartoma
• Asymptomatic / symptoms of compression
• Mediastinal extension of cystic hygroma (10%), soft palpable mass; 90%
diagnosed in infancy
• Mediastinal mass in asymptomatic child / adult
Lymphangioma: Pathologic Features

[Figures 1-16-43 and 1-16-44]
• Intercommunicating spaces of variable size lined by endothelial cells
• Soft, cystic mass
• Cystic hygroma– large vascular spaces
• Cavernous lymphangioma – small vascular spaces

Lymphangioma: Imaging Features [Figures 1-16-45 and 1-16-46] Figure 1-16-44
• Anterosuperior mediastinum; other compartments affected
• Cervical / axillary / chest wall mass; mediastinal extension
• Spherical, lobular, well-defined borders
• Circumscribed mass / infiltrative mass
• Multilocular, cystic, heterogeneous
➢ Solid components, tissue septa
Figure 1-16-45
Lymphangioma, gross
features: Multilocular
cystic appearance due to
enlargement of vascular
Lymphangioma, imaging features: Multilocular cystic mediastinal mass with channels
extension into the axilla
Figure 1-16-46
Lymphangioma, imaging features: Infiltrative or localized multilocular cystic

mediastinal mass
Hemangioma [Figure 1-16-47]

• Rare vascular mediastinal tumor Figure 1-16-47
➢ Neoplasm vs. developmental
• Young patients; 75% < 35 yrs.
• Asymptomatic; 1/3-1/2 with
symptoms of compression
➢ Rendu-Osler-Weber
syndrome
• Communicating vascular spaces
➢ Endothelial lining, organized
thrombi, Ca++, phleboliths
• Anterior mediastinal mass (also in
other compartments)
• Spherical, well-defined, Ca++
28%, punctate, phleboliths
Hemangioma, imaging features: Anterior mediastinal mass with intrinsic
• Heterogeneous intense
phleboliths and intense heterogeneous enhancement
enhancement

Vascular Lesion - Aneurysm
• Abnormal mediastinal contour contiguous with vascular structures
• Saccular aneurysms may resemble other primary mediastinal masses
• Curvilinear peripheral calcification
• Contrast enhancement
• Continuity with vascular lumen
Vascular Lesion - Varices

• Esophageal / paraesophageal
• Severe liver disease and portal hypertension; Left gastric – portosystemic
collaterals
• Visible on radiography in 10%
• Middle-posterior-paravertebral cluster of serpiginous vessels with intense
enhancement
Figure 1-16-48
Vascular Lesions
• Focal vs. infiltrative
• Lymphatic
➢ Multilocular cystic
➢ Extramediastinal involvement
• Blood vessels
➢ Intense, heterogeneous / serpiginous enhancement
• Aneurysms
➢ Focal vascular enlargement
Herniations – Hiatus Hernia [Figure 1-16-48]

• Gastric herniation through enlarged esophageal hiatus
• Increased intra-abdominal pressure / Increased prevalence with
increasing age
• Asymptomatic; Reflux / bleeding
• Retrocardiac mass, homogeneous, air-filled, air-fluid Hiatus hernia, imaging features:
• Identification of abdominal contents in hernia sac herniation of abdominal contents through
esophageal hiatus
Herniation - Morgagni
• Developmental defect in right anteromedial hemidiaphragm
• Asymptomatic / Abdominal pain
• Right cardiophrenic angle mass
• Demonstration of internal fat (omentum), bowel loops or abdominal organs
(liver)
Herniations
• Intrathoracic extension of abdominal contents
➢ Bowel
➢ Omental fat
• Esophageal hiatus
• Morgagni hernias
Miscellaneous – Achalasia
• Absent peristalsis and incomplete relaxation of esophageal sphincter
• Primary – deficiency of ganglion cells in myenteric plexus
• Secondary – (pseudo achalasia) Chagas disease and primary or secondary
malignancy at the GE junction
• Esophageal dilatation with air-fluid levels
➢ Esophageal displacement to the right, mass effect on mediastinum,
pulmonary consolidation (aspiration)
Miscellaneous - EMH
• Extramedullary hematopoiesis
• Compensatory formation of blood elements outside osseous medulla
• Hemolytic anemia
• Unilateral or bilateral paravertebral mass; may exhibit internal fat attenuation
• Adjacent medullary expansion

Miscellaneous – Acute Mediastinitis
• Surgery, instrumentation with esophageal perforation
• Ill patients with fever, chills and chest pain
• Focal or diffuse mediastinal widening, pneumomediastinum, pleural effusion,
pneumothorax
• Abscess, abnormal mediastinal air, extraluminal ingested contrast, obliteration
of tissue planes
Mediastinal Masses: Pathognomonic

• Lateral thoracic meningocele
• Aneurysm
• Esophageal varices
• Teratoma
• Lipomatosis
• Congenital cyst (BC, PC)
• Mediastinal goiter
Mediastinal Masses: Cystic

• Thymoma (mural nodules)
• Congenital cysts (unilocular, middle, posterior mediastinum)
• Neurogenic neoplasm (associated osseous erosion)
➢ Meningocele (NF1, continuity with spinal canal, homogeneous water
attenuation / signal)
• Mature teratoma (multilocular cystic mass with internal fat)
• Lymphoma (lymphadenopathy)
• Lymphangioma (multilocular cysts - vascular channels)
• Esophageal enlargement (achalasia)
• Mediastinal goiter (high attenuation, continuity with thyroid)
Mediastinal Masses: Fat

• Lipomatosis (diffuse, no mass effect)
• Lipoma
• Thymolipoma (fat / soft tissue connecting to thymus)
• Mature teratoma (cystic)
• Morgagni hernia (CPA, right, continuous with abdominal fat)
Mediastinal Masses: Intense Enhancement

• Mediastinal goiter (continuity with cervical thyroid)
• Hemangioma (phleboliths, follows vascular enhancement)
• Castleman disease (enhancing lymphadenopathy)
• Paraganglioma (catecholamine production)
• Aneurysm / Varices
References
General
1. Aquino SL, Duncan G, Taber KH, Sharma A, Hayman LA. Reconciliation of the anatomic, surgical, and
radiographic classifications of the mediastinum. J Comput Assist Tomogr 2001; 25: 489-492.
2. Armstrong P. Mediastinal and hilar disorders. In: Armstrong P, Wilson AG, Dee P, Hansell DM, eds. Imaging of
Diseases of the Chest. Third edition. London: Mosby, 2000; 789-892.
3. Felson B. Chest Roentgenology. Philadelphia: Saunders, 1973: 380-420.
4. Fraser RS, Müller NL, Colman N, Paré PD. Masses situated predominantly in the anterior compartment. In: Fraser
RS, Müller NL, Colman N, Paré PD, eds. Fraser and Paré's Diagnosis of Diseases of the Chest, Fourth edition.
Philadelphia: Saunders, 1999: 2875-2937.
5. Fraser RS, Müller NL, Colman N, Paré PD. Masses situated predominantly in the middle-posterior mediastinal
compartment. In: Fraser RS, Müller NL, Colman N, Paré PD, eds. Fraser and Paré’s Diagnosis of Diseases of the
Chest. Fourth edition. Philadelphia: Saunders, 1999; 2938-2973.
6. Fraser RS, Müller NL, Colman N, Paré PD. Masses situated predominantly in the paravertebral region. In: Fraser
RS, Müller NL, Colman N, Paré PD, eds. Fraser and Paré’s Diagnosis of Diseases of the Chest. Fourth edition.
Philadelphia: Saunders, 1999; 2974-2983.

7. Jeung M-Y, Gasser B, Gangi A, et al. Imaging of cystic masses of the mediastinum. RadioGraphics 2002; 22: S79-
S93.
8. Rosado de Christenson ML. Abnormalities and Diseases of the Mediastinum. In: Parker MS, Rosado de Christenson
ML, Abbott GF. Teaching Atlas of Chest Imaging. New York: Thieme 2006; 621-702.
9. Rosai J, Sobin LH. Histological Typing of Tumours of the Thymus. International Histological Classification of
Tumours, Second edition. New York: Springer 1999.
10. Shimosato Y, Mukai K. Tumors of the thymus and related lesions. In: Rosai J, ed. Atlas of Tumor Pathology:
Tumors of the Mediastinum, fasc 21, ser 3. Washington, DC: American Registry of Pathology and Armed Forces
Institute of Pathology, 1997: 33-247.
11. Shimosato Y, Mukai K. Tumors of the mediastinum excluding the thymus, heart and great vessels. In: Shimosato Y,
Mukai K, eds. Atlas of Tumor Pathology: Tumors of the Mediastinum, fasc 21, ser 3. Washington, DC: Armed
Forces Institute of Pathology, 1997; 249-273.
12. Strollo DC, Rosado-de-Christenson ML. Tumors of the thymus. J Thorac Imag 1999; 14: 152-171.
13. Woodburne RT, Burkel WE. Essentials of Human Anatomy, Ninth edition. New York: Oxford University Press,
1994: 370-371.
Thymoma
1. Rosado-de-Christenson ML, Galobardes J, Moran CA. Thymoma: Radiologic-Pathologic Correlation.
RadioGraphics 1992; 12: 151-168.
2. Thomas CR, Wright CD, Loehrer PJ, Sr. Thymoma: state of the art. J Clin Oncol 1999; 17: 2280-2289.
3. Tomiyama N, Müller NL, Ellis SJ, et al. Invasive and noninvasive thymoma: distinctive CT features. J Comput
Assist Tomogr 2001; 25: 388-393.
Thymic Malignancy
1. Jung K-J, Lee KS, Han J, Kim J, Kim TS, Kim EA. Malignant thymic epithelial tumors: CT-pathologic correlation.
AJR 2001: 176: 433-439.
2. Rosado-de-Christenson ML, Abbott GF, Kirejczyk WM, Galvin JR, Travis WD. Thoracic carcinoids: Radiologic-
pathologic correlation. RadioGraphics 1999; 19: 707-736.
Thymolipoma
1. Rosado-de-Christenson ML, Pugatch RD, Moran CA, Galobardes J. Thymolipoma: analysis of 27 cases. Radiology
1994; 193: 121-126.
Thymic Hyperplasia
1. Budavari AI, Whitaker MD, Helmers RA. Thymic hyperplasia presenting as anterior mediastinal mass in 2 patients
with Graves disease. Mayo Clin Proc 2002; 77: 495-499.
2. Hara M, McAdams HP, Vredenburgh JJ, Herndon JE, Patz EF Jr. Thymic hyperplasia after high-dose chemotherapy
and autologous stem cell transplantation: incidence and significance in patients with breast cancer. AJR 1999; 173:
1341-1344.
Germ Cell Neoplasms

1. Choi S-J, Lee JS, Song KS, Lim T-H. Mediastinal teratoma: CT differentiation of ruptured and unruptured tumors.
AJR 1998; 171: 591-594.
2. Moeller KH, Rosado-de-Christenson ML, Templeton PA. Mediastinal mature teratoma: imaging features. AJR 1997;
169: 985-990.
3. Strollo DC, Rosado-de-Christenson ML. Primary mediastinal malignant germ cell neoplasms: imaging features.
Chest Surg Clin N Am 2003; 12: 645-658.
Lymphoma
1. Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal
from the international lymphoma study group. Blood 1994; 84: 1361-1392.
2. Fraser RS, Müller NL, Colman N, Paré PD. Lymphoproliferative disorders and leukemia. In: Fraser RS, Müller NL,
Colman N, Paré PD, eds. Fraser and Paré’s Diagnosis of Diseases of the Chest. Fourth edition. Philadelphia:
Saunders, 1999; 1269-1330.
Non-Neoplastic Lymphadenopathy
1. Atasoy C, Fitoz S, Erguvan B, Akyar S. Tuberculous fibrosing mediastinitis: CT and MRI findings. J Thorac Imag
2001; 16: 191-193.
2. McAdams HP, Rosado de Christenson ML, Fishback NF, Templeton PA. Castleman disease of the thorax: radiologic
features with clinical and histopathologic correlation. Radiology 1998; 209: 221-228.
3. Rossi SE, McAdams HP, Rosado-de-Christenson ML, Franks TJ, Galvin JR. Fibrosing mediastinitis.

Mediastinal Cysts
1. Choi YW, McAdams HP, Jeon SC, et al. Idiopathic multilocular thymic cyst: CT features with clinical and
histopathologic correlation. AJR 2001; 177: 881-885.
2. Jeung M-Y, Gasser B, Gangi A, et al. Imaging of cystic masses of the mediastinum. RadioGraphics 2002; 22:
S79-S93.
3. McAdams HP, Kirejczyk WM, Rosado-de-Christenson ML, Matsumoto S. Bronchogenic cyst: imaging features
with clinical and histopathologic correlation. Radiology 2000; 217: 441-446
Neurogenic Neoplasms
1. Ichikawa T, Ohtomo K, Araki T, et al. Ganglioneuroma: computed tomography and magnetic resonance features.
Br J Radiol 1996; 69: 114-121.
2. Marchevsky AM. Mediastinal tumors of peripheral nervous system origin. Semin Diagn Pathol 1999; 16: 65-78.
3. Moon WK, Im J-G, Han MC. Malignant schwannomas of the thorax: CT findings. J Comput Assist Tomogr 1993;
17: 274-276.
4. Rossi SE, Erasmus JJ, McAdams HP, Donnelly LF. Thoracic manifestations of neurofibromatosis-I. AJR 1999;
173: 1631-1638.
Endocrine Lesions
1. Buckley JA, Stark P. Intrathoracic mediastinal thyroid goiter: imaging manifestations. AJR 1999; 173: 471-475.
2. Fraser RS, Müller NL, Colman N, Paré PD. Masses situated predominantly in the anterior mediastinal
compartment. In: Fraser RS, Müller NL, Colman N, Paré PD, eds.
3. Fraser and Paré’s Diagnosis of Diseases of the Chest. Fourth edition. Philadelphia: Saunders, 1999; 2875-2937.
4. Hopkins CR, Reading CC. Thyroid and parathyroid imaging. Semin US CT MRI 1995; 16: 279-295.
Vascular Lesions
1. Charruau L, Parrens M, Jougon J, et al. Mediastinal lymphangioma in adults: CT and MR imaging features. Eur
Radiol 2000; 10: 1310-1314.
2. Miyake H, Shiga M, Takaki H, Hata H, Osini R, Mori H. Mediastinal lymphangiomas in adults: CT findings. J
Thorac Imaging 1996; 11: 83-85.
3. McAdams HP, Rosado-de-Christenson ML, Moran CA. Mediastinal hemangioma: radiographic and CT features
in 14 patients. Radiology 1994; 193: 399-402.
4. Shaffer K, Rosado-de-Christenson ML, Patz EF Jr, Young S, Farver CF. Thoracic lymphangioma in adults: CT
and MR imaging features. AJR 1994; 162:283-29-89.
5. Henseler KP, Pozniak MA, Lee FT Jr, Winter TC III. Three-dimensional CT angiography of spontaneous
portosystemic shunts. RadioGraphics 2001; 21: 691-704.
6. Ibukuro K, Tsukiyama T, Mori K, Inoue Y. Preaortic esophageal veins: CT appearance. AJR 1998; 170: 1535-
1538.
7. Ibukuro K, Tsukiyama T, Mori K, Inoue Y. Precaval draining vein from paraesophageal varices: Radiologic-
anatomic correlation. AJR 1999; 172: 651-654.
8. Kim M-J, Mitchell DG, Ito K. Portosystemic collaterals of the upper abdomen: Review of anatomy and
demonstration on MR imaging. Abdom Imaging 2000; 25: 462-470.
9. Lee SJ, Lee KS, Kim SA, Kim TS, Hwang JH, Lim JH. Computed radiography of the chest in patients with
paraesophageal varices: Diagnostic accuracy and characteristic findings. AJR 1998; 170: 1527-1531.
Miscellaneous Lesions
1. Fraser RS, Müller NL, Colman N, Paré PD. The diaphragm. In: Fraser RS, Müller NL, Colman N, Paré PD, eds.
Fraser and Paré’s Diagnosis of Diseases of the Chest. Fourth edition. Philadelphia: Saunders, 1999; 2987-3010.
2. Mueller CF, Klecker RJ, King MA. Case 3. Achalasia. AJR 2000; 175: 867; 870-871
3. Woodfield CA, Levine MS, Rubesin SE, Langlotz CP, Laufer I. Diagnosis of primary versus secondary achalasia.
Reassessment of clinical and radiographic criteria. AJR 2000; 175: 727-731.
4. Dunnick NR. Image interpretation session: 1999. Extramedullary hematopoiesis in a patient with beta
thalassemia. RadioGraphics 2000; 20: 266-268.
5. Gilkeson RC, Basile V, Sands MJ, Hsu JT. Chest case of the day. Extramedullary hematopoiesis (EMH). AJR
1997; 169: 267, 270-273.
6. Moellers M-C, Bader JB, Alexander C, Samnick S, Kirsch C-M. Localization of extramedullary hematopoiesis
with Tc-99m-labeled monoclonal antibodies (BW 250/183). Clin Nuc Med 2002; 27: 354-357.

Chest Seminar: Where is the Lesion?
Learning Objectives
• To review the radiologic features of thoracic radiologic abnormalities based on
location
• To enumerate the radiologic characteristics that allow lesion localization and
the formulation of a focused radiologic differential diagnosis
Case 1: 38-year-old woman with cough

• Location
• Differential diagnosis
• Next best study
• Diagnosis:
Solitary Lung Mass

• Lung cancer
➢ Size / frequency
➢ Stage ?
• Carcinoid tumor
➢ Borders / bronchus
• Solitary metastasis
➢ Lower lobe location / shape
• Hamartoma / Infection
➢ Borders
Solitary Lung Mass

• Young, relatively asymptomatic woman
• Mass with well-defined lobular borders
• Lower lobe location
• Abutting bronchus
Chest Seminar: Where is the Lesion 168 Chest Radiology

Case 2: 16-year-old girl with cough
• Location
• Next best study
• Diagnosis:
Anterior Mediastinal Mass

• Young girl, relatively asymptomatic
• Well-defined, unilateral mass with peripheral calcification and lobular borders
• Central water attenuation
• Low attenuation mural nodule
Anterior Mediastinal Mass

• Mature teratoma
➢ Fluid / fat / Ca++
• Thymic cyst
➢ Fluid / Ca++
• Lymphoma
➢ Age group … but no lymphadenopathy
• Thymoma
➢ Fluid, calcium, mural nodule … but fat
Chest Radiology 169 Chest Seminar: Where is the Lesion

Case 3: 58-year-old man with chest pain and hemoptysis
• Location
• Next best study
• Diagnosis:
Lung Mass with Cavitation and Chest Wall Involvement

• Symptomatic older male
• Chest wall invasion (rib destruction)
• Upper lobe location
• Cavitation
Chest Wall Mass / Cavitation

• Bronchogenic carcinoma
➢ Chest wall invasion
➢ Stage?
• Infection
➢ Actinomycosis, tuberculosis, fungus
• Primary chest wall tumor / Metastasis
• Other

Case 4: Asymptomatic 40-year-old male; pre-operative
radiograph
• Location
• Next best study
• Diagnosis:
Multifocal Pleural Nodules

• Asymptomatic patient
• No known malignancy
• Well-defined peripheral pleural-based nodules
• Associated findings - Abdominal abnormalities?
Multifocal Pleural Nodules

• Splenosis
➢ Where is the spleen?
• Metastases
• Malignant pleural mesothelioma
• Other
Splenosis
• Auto-transplantation of splenic tissue typically following splenic rupture
• Most common manifestation: Multiple peritoneal nodules
• Thoracic splenosis:
➢ Multiple pleural-based nodules
➢ May be missed on radiography
➢ 99mTC-tagged heated RBC scintigraphy
➢ Liver-spleen scan
Chest Radiology 171 Chest Seminar: Where is the Lesion

Case 5: 34-year-old man with left chest pain for many years
• Location
• Next best study
• Diagnosis:
Multifocal Chest Wall and Mediastinal Masses

• Chronic lesions with minimal symptoms
• Unilateral or bilateral?
• Benign pressure erosion
• Pulmonary involvement
• Other chest wall / mediastinal involvement
Multifocal Chest Wall and Mediastinal Masses

• Neurofibromatosis
➢ Malignant potential
• Metastases
• Other

Chest Seminar: Differential Diagnosis of
Mediastinal Masses
Learning Objectives
• To review concepts of differential diagnosis of mediastinal masses
• To emphasize importance of demographics, location and morphology in the
formulation of a focused differential diagnosis
Case 1: Elderly man with chest pain

• Location
• Characterization
• Next study
• Biopsy
Heterogeneous Middle Mediastinal Mass; Rim CA++

• Neoplasia
➢ Carcinoma
➢ Lymphoma
• Congenital Cyst … but heterogeneous
• Vascular lesion
➢ Aneurysm – rim CA++
• Other
Chest Radiology 173 Chest Seminar: Differential Diagnosis of Mediastinal Masses

Case 2: Asymptomatic 52-year-old man
• Next best study
Spherical Paravertebral Mass with Pressure Erosion

• Neurogenic Neoplasm
➢ Next study?
• Lateral Thoracic Meningocele
➢ History?
• Other
Chest Seminar: Differential Diagnosis of Mediastinal Masses 174 Chest Radiology

Case 3: 40-year-old woman with difficulty swallowing
• Next best study
Unilateral Cystic Anterior Mediastinal Mass; Mural CA++

• Cystic Thymoma
➢ Mural nodule
• Cystic Teratoma
➢ No fat no calcium
• Thymic Cyst / Pericardial Cyst
➢ but mural nodule
• Cystic Lymphoma
➢ but no lymphadenopathy
Unilateral Cystic Anterior Mediastinal Mass; Mural CA++ and

mural nodule
• Symptomatic woman
➢ Symptoms related to function of voluntary musculature
• Age over 40
• Pattern of enhancement
➢ Mural nodule

Case 4: 24-year-old man with chronic abdominal discomfort
• Diagnosis
• Should the lesion be excised?
Right Cardiophrenic Angle Mass of Fat and Soft Tissue

Attenuation
• Thymolipoma
➢ Fat / soft tissue
➢ Does not conform to adjacent structures / thymus?
• Lipoma
➢ Fat / soft tissue
• Mature Teratoma
➢ But...No fluid
• Morgagni Hernia
➢ Continuity with abdominal fat
Chest Seminar: Differential Diagnosis of Mediastinal Masses 176 Chest Radiology

Case 5: 29-year-old woman with fatigue and cough
• Diagnosis
• Should the lesion be excised?
➢ Biopsied?
Anterior Mediastinal Mass (Cystic change, Ca++)

• Lymphoma:
➢ Age, local invasion, lymphadenopathy
➢ Cystic change, Ca++ ?
• Thymoma
➢ Cystic change, Ca++,local invasion
➢ But…lymphadenopathy
• Teratoma
➢ But…lymphadenopathy, local invasion and soft tissue predominant
• Malignant GCN
➢ But…wrong gender

Pneumonia: Usual and Unusual
Organisms
Rosita M. Shah, MD
Classification of Pulmonary Infection

• Community-acquired Pneumonia
➢ S. pneumoniae LOBAR
➢ Mycoplasma LOBULAR
➢ Influenzae INTERSTITIAL
• Nosocomial Pneumonia
• Unusual Pulmonary Infections
Pulmonary Infection: Classification: Morphology

• 3 radiographic and pathologic patterns
➢ Lobar
➢ Lobular (bronchopneumonia)
➢ Interstitial
Pulmonary Infection: Classification

• Lobar and lobular pneumonias both produce air space filling
• Significant differences include:
➢ Site of initial inflammation
➢ Degree of lobular opacification
➢ Radiographic pattern
➢ Etiologic agents
Alveolar Filling Pneumonias

• Site of initial infection varies
➢ Alveolar level in lobar pneumonia
➢ Bronchiolar level in bronchopneumonia

• Degree of opacification of secondary lobule is different
➢ Complete in lobar pneumonia
➢ Incomplete in bronchopneumonia

• Radiographic pattern will vary
➢ Lobar pattern
➢ Bronchopneumonia pattern

• Etiologic agent may vary
Lobar Pneumonia Bronchopneumonia
S.pneumoniae Gram –’s, anaerobes

K.pneumoniae Legionella
also seen with Actinomycosis
Legionella Nocardia
Mycoplasma Mycoplasma
H.influenzae Typical, atypical TB
Parasites
Pneumonia 178 Chest Radiology

• Accurate pattern recognition depends on:
➢ Early imaging
➢ Normal lung structure
• Organisms may produce more than one pattern
• Basic pattern differentiation may be difficult
➢ Interstitial vs bronchopneumonia
Community-acquired Pneumonia: Epidemiology

• 2–10 /1000 annual incidence
• 22-50% hospitalization rate
➢ Outpatient mortality 1-5%
➢ Inpatient mortality 25%
Community-acquired Pneumonia: Etiology

• In up to 50%, no definitive organism isolated
• Most common isolates:
➢ S. pneumoniae
➢ M. pneumoniae
➢ K. pneumoniae
➢ H. influenzae
➢ L. pneumophila
➢ Respiratory viruses
S. pneumoniae: Demographics
• S. pneumoniae most frequent isolate in CAP
➢ 8-76% incidence
• Recognized risk factors
➢ alcoholism, splenic dysfunction, viral pneumonia, congenital and acquired
immune deficiencies
S. pneumoniae: Demographics
• 25% incidence of bacteremia
• 25-40% mortality, unchanged >30y
➢ Age >65
➢ CHF,DM
➢ Alcoholism
➢ Thrombocytopenia
➢ Renal dysfunction
➢ Number of lobes
Chest 1993; 103:1152-56
S. pneumoniae: Pathology
• Aspiration to peripheral air spaces
• Alveolus represents site of initial inflammatory lesion
• Spread occurs by contignous involvement of adjacent alveoli
• 3 pathologic stages
S. pneumoniae: Pathology
• ACUTE RESPONSE
➢ Increased capillary permeability
➢ Protein rich edema
➢ Contiguous alveolar filling via Pores of Kohn and Canals of Lambert
• RED HEPATIZATION
➢ PMN infiltration and intra-alveolar hemorrhage
• GRAY HEPATIZATION
➢ Macrophage infiltration and uptake of blood products
Chest Radiology 179 Pneumonia

S. pneumoniae: Radiology [Figure 1-19-1]
• LOBAR pattern
➢ Homogeneous, confluent density
➢ Nonsegmental distributions
S. pneumoniae: Radiology
• Spread at alveolar level results in nonsegmental distributions characteristic of Figure 1-19-1
early lobar pneumonia
• Round pneumonia
➢ Manifestation of nonsegmental distribution
➢ Most common in pediatric infection with S.pneumoniae
• LOBAR pattern
➢ Prominent air bronchograms
➢ Preserved volume
• 48% of consecutive hospitalized pts demonstrated focal lobar
patterns
• 33%, multifocal lobar patterns
• 16% lobular pattern
• Dominant pattern did not vary with immune status or disease
severity Lobar pattern consolidation due to S.
AJR 2000;175:1533 pneumoniae
• Small pleural effusions up to 60%
• Infrequent cavitation
➢ Associated with serotype 3
• Most frequent organism in pulmonary gangrene
➢ Vascular thrombosis from severe necrosis
➢ Intracavitary mass (sloughed lung)
M. pneumoniae: Demographics
• 15-35% of CAP
➢ 50% of CAP during summer months
• Peak age 5-25 yo
• Self limited
➢ Few fatal cases associated with ARDS
➢ Increased severity in sickle cell anemia
• Most frequent etiology in Atypical Pneumonia Syndrome
➢ Atypical radiographic features
➢ Prominent extrapulmonary complaints
M. pneumoniae: Pathology
• Eaton agent-1944
➢ Gram -- filamentous rod
➢ Absent cell wall
• Acute cellular bronchiolitis
➢ Superficial inflammation involving luminal surface of bronchi, bronchioles
➢ Associated interstitial infiltrates

M. pneumoniae: Radiology [Figure 1-19-2] Figure 1-19-2
• LOBULAR pattern Bronchopneumonia
• Heterogeneous, patchy consolidation
➢ Minimal exudate into centrilobular alveoli
• Segmental distribution
➢ Spread at bronchiolar level
• Volume loss
• Minimal air bronchograms
➢ Peribronchial thickening
M. pneumoniae: Radiology
• CT Findings [Figure 1-19-3]
➢ 86% centrilobular nodules
➢ 82% bronchovascular thickening
➢ 59% consolidation with lobular distribution
Reittner, AJR 2000; 174:37
Bronchopneumonia pattern due to
Respiratory Viruses M. pneumoniae
• Influenzae A,B,C
• Para-influenzae
• Respiratory syncytial virus
• Adenovirus Figure 1-19-3
• Herpes viruses
• SARS
Influenzae A: Demographics
• 10-20% CAP
• 10,000-40,000 deaths/ influenzae epidemic
• Peak incidence
➢ Pediatric population
• Highest mortality-adult and aged
➢ Superinfection
➢ S.aureus
➢ S.pneumoniae
Influenzae A: Pathology
• St 1 infection of epithelial cells, proliferation and necrosis
• St 2 bronchial and alveolar wall edema,hemorrhage HRCT of M. pneumoniae
• Ulceration, bacterial infection
Influenzae A: Radiology
• INTERSTITIAL pattern
➢ Reticular Figure 1-19-4
➢ Nodular
➢ Peribronchial thickening
➢ Subpleural edema
➢ Hilar haze
[Figure 1-19-4]
• Bilateral, parahilar, lower
lobe
• Air trapping
• Prominent GGO
(left) CXR
(right) HRCT Influenzae pneumonia mimicking edema

• Pleural effusions, cavitation uncommon without bacterial superinfection
• Rapid deterioration should suggest superinfection
Adenovirus [Figure 1-19-5]

• Interstitial pneumonia with prominent necrotizing bronchiolitis
Figure 1-19-5
• Potential infection in immune competent and suppressed hosts with high
mortality
➢ Military epidemics
➢ Transplant recipients
• Swyer James, Macleod’s syndrome
➢ Bronchiolitis obliterans following viral infection in early
childhood
Respiratory Herpesviruses
• HSV-1, HSV-2, VZV, EBV, CMV
➢ Primary infection, latency, reactivation
➢ Up to 40% mortality
• Risk factors Swyer James Syndrome due to
➢ Immune-suppression, lung transplantation, airway pediatric viral pneumonia
management, pregnancy
Varicella Pneumonia
• Complication of adult chickenpox Figure 1-19-6
➢ 5-50% incidence
• Prominent acinar opacities
➢ 5-10mm nodules, coalescence
➢ Patchy GGO
Kim AJR 1999;172:113
• May heal with miliary calcifications
Varicella Pneumonia [Figure 1-19-6]

• Prominent acinar opacities
Severe Acute Respiratory Syndrome

• SARS-CoV (corona virus)
• Initial cases Nov 2002-June 2003, rapid spread from
Asia
• 20-50% require mechanical ventilation
• 10% mortality, age dependant
• Severe DAD
Severe Acute Respiratory Syndrome

• Predominant consolidation 1-2weeks Acinar nodules in varicella pneumonia
➢ Focal (39%), multifocal (28%), diffuse (14%)
• Ground glass opacity
• Reticulation
• Bronchiolar dilation
• Residual changes in 50% at 4wks
Ooi GC. Radiology 2004;230:836; Paul NS. AJR 2003;182:493
Severe Community-acquired Pneumonia: Definition

• Impending respiratory failure
• Hemodynamic instability
• Radiographic assessment
➢ Bilateral or multilobar involvemnt
➢ 50% increase in size of opacity within 48hr

Severe Community-acquired Pneumonia: Etiology
• S. pneumoniae
• L. pneumophila
• S. aureus
• P. aeruginosa in patients with bronchiectasis
L. pneumophila: Demographics
• 15% of CAP
➢ Epidemic and sporadic forms
➢ Legionnaire’s disease= pneumonic form
➢ Peak summer
• Aerobic Gram -- bacillus
• Proliferates in warm, humid environments
Figure 1-19-7
L. pneumophila: Pathology
• Bronchocentric inflammation
L. pneumophila: Demographics
• Acute onset
• Prominent extrapulmonary symptoms
➢ Neurologic manifestations, diarrhea, renal insufficiency
• 10% mechanical ventilation
• 15% mortality in cases requiring hospitalization
L. pneumophila: Radiology [Figure 1-19-7]

• Bronchopneumonia pattern
• Pleural effusions in 2/3
• Bilateral and multifocal in 50%
• May produce lobar or mass-like consolidation
• Cavitation uncommon without immunosupression
• Delayed resolution
K. pneumoniae: Demographics
• Nosocomial or community acquired
• 5-10% lobar pneumonias
• 25% bacteremic, 50% mortality
• Males, >60yo
• Risk factors: alcoholism, COPD, DM
K. pneumoniae: Pathology
• Gram -- bacillus
➢ Abundant PMN infiltration of alveoli, edema
➢ Lobar expansion - Friedlander’s pneumonia
➢ Massive necrosis HRCT in Legionella pneumonia
➢ Common association with gangrene demonstrating bronchocentric
nodules and pleural effusion
K. pneumoniae: Radiology
• Lobar pattern
➢ Bulging fissures
• Abscess 30-50%
• Necrotizing pneumonia at CT
➢ Low density areas with small cavities
Moon JCAT 1995;19:176

S.aureus: Demographics Figure 1-19-8
• 30-50% colonization rates in healthy adults
➢ DM
➢ IVDA
➢ HIV
➢ Surgical pts
• Methicillin resistance 1944
➢ Increasing incidence of resistant community-acq infection
• Antecedant viral pneumonia
• Frequent cause of nosocomial infection
• Extremes of age
➢ Nursing home population
• Risk factors
➢ Debilitated states, mechanical ventilation, burns, indwelling
catheters, IVDA
S. aureus: Radiology
• Aerogenous infection [Figures 1-19-8 and 1-19-9]
➢ Multifocal Broncho-pneumonia
• Hematogenous infection [Figure 1-19-10]
➢ Multifocal, discrete nodular or wedge shaped abnormality with
normal intervening lung
• Cavitation / abscess (25-75%)
• Pneumatoceles (60% ped infection)
• Pleural effusions / empyema (50%)
CXR and HRCT bronchopneumonia

pattern due to S. aureus
Figure 1-19-9
Necrotizing bronchopneumonia due to S. aureus

P. aeruginosa in Cystic Fibrosis Figure 1-19-10
• Chronic colonization with P.aeruginosa
➢ Mucoid variant
➢ ABX resistance
➢ Elastase production
➢ Bronchiectasis
The Practical Points

• S.pneumoniae and K.pneumoniae most commonly associated
with lobar pattern and pulmonary gangrene
• M.pneumoniae, L.pneumophilus most commonly associated with
broncho-pneumonia pattern and atypical pneumonia syndrome
• Viral pneumonias associated with interstitial pattern
• Pathologic in immune-competent and suppressed hosts
• Prominent bronchiolitis seen with mycoplasma, adeno and other
respiratory viruses Septic emboli
Nosocomial Pneumonia
• Rising incidence parallels usage of antibiotics
• Gram negative infections
➢ 40-50% increase 1950-60
➢ 55-65% nosocomial infections
➢ 50% nosocomial pneumonia
➢ 75% ICU pneumonia
Nosocomial Pneumonia: Definition

• Pneumonia developing >48hr sp admission, intubation or discharge
Diagnosis
• Quantitative cultures
➢ Tracheal aspirate 10 5-6 cfu/ml
➢ BAL 10 4 cfu/ml
➢ Protected specimen brush 10 3 cfu/ml
➢ False negative and false positive rates 20-30%
Baughman Chest 2000
Pathophysiology
• Direct inhalation
• Hematogenous spread
• Aspiration
➢ 45% incidence in sleep
➢ Altered gag reflex, consciousness, GI motility
➢ NG / ET
Pathophysiology
• Abnormal gram negative airway colonization
➢ 25% 24hr
➢ 40% 7d
• Gastric alkalinization
• Serious illness
• Antibiotic TX
Johanson Ann Intern Med 1972
Pathophysiology
• Repetitive aspiration leads to
• Bronchiolitis
• Lobular (broncho) pneumonia
➢ Peribronchiolar neutrophilic infiltrate (104 cfu/g)

Nosocomial Pneumonia in the ICU
• Ventilator-associated Pneumonia (VAP)
• Most common nosocomial infection in ICU
➢ 3-21x greater incidence in intubated patients
✧ > 1 intubation, >3 days
➢ 10-65% ICU patients acquire VAP
➢ 1/3-3/4 ARDS patients acquire VAP
• 20-80% mortality rate
Figure 1-19-11
Ventilator-associated Pneumonia
• Prognosis depends:
➢ Organism
✧ Highest mortality: P.aeruginosa, MRSA
➢ Population
✧ Highest mortality: medical ICU
✧ 10-20% mortality: trauma ICU
➢ Late onset
✧ MDR
Nosocomial Pneumonia and Aspiration [Figure 1-19-11]

• Pneumonitis (Mendelson Syndrome)
➢ pH < 2.5, >0.3 ml/kg
➢ Biphasic inflammatory response
✧ 1-2 hr permeability edema
✧ 4-6 hr acute inflammation
➢ 30% mortality
➢ Rapid clearing
Nosocomial Pneumonia and Aspiration

• Sterile
vs
• Normal flora (<5d)
vs Dependent aspiration pneumonia
• Gram – bacilli(>5d) complicated by ARDS
• Anaerobes seen in late aspiration
• Aspiration accounts for upto 15% of CAP
Marik NEJM 2001
Microbiology
• Normal flora
• Gram – bacilli
• S. aureus
• Anaerobes
• Legionella
• Respiratory viruses
Microbiology
• Early <5d
➢ H. influenzae
➢ S. pneumoniae
➢ S. aureus
• Late >5d
➢ S. aureus
➢ P. aeruginosa
➢ Enterobacteriaceae
➢ Acinetobacter spp.
➢ Stenotrophomonas maltophilia

P. aeruginosa
• Most common ICU isolate
• 70% TX failure rate
• Distal airway colonization, hematogenous dissemination
• Increased severity in neutropenia, bacteremia
P. aeruginosa: Pathology
• Micro-abscesses
• Necrotic vasculits
➢ Sm-med pulmonary arteries
• Hemorrhage
P. aeruginosa: Radiology
• Bronchopneumonia pattern
➢ Discrete nodules may be indicative of vasculitis
• Frequent cavitation
• Pleural effusions/empyema
Nosocomial Viral Pneumonia

• Rate of infection assoc with length of hospitalization
• Hospital worker as carrier
• Frequently unsuspected
• High mortality rates
➢ RSV 30-100%
➢ Parainfluenzae 15-30%
Nodular or Mass-like Consolidations

• Nonsegmental distribution
➢ ‘round’ pneumonia
• Granulomatous infection
➢ M. tuberculosis
➢ Fungi
➢ Actinomycosis
➢ Nocardia
A. Israelii; Demographics
• Normal oral flora Figure 1-19-12
• Sites of infection:
➢ Cervicofacial 55%
➢ Abdomen 20%
➢ Pulmonary 25%
• Risk factors: poor oral hygiene, aspiration
Smego RA. Clin Infec Dis 1998;26:1255
A. Israelii: Pathology
• Multifocal abscesses
• Interconnecting sinus tracts
• Sulphur granule
➢ Spoke-wheel arrangement of neutrophils surrounding Pneumonia with chest wall
filamentous organism involvement due to A. israelli
A. Israelii: Radiology [Figure 1-19-12]

• Consolidation
➢ Mass-like
➢ Cavitary
• Pleural, chest wall and osseous involvement
➢ Up to 50%

N. Asteroides: Demographics
• Ubiquitous distribution
• 50% of patients are immunocompetent
• Risk factors:
➢ Neutropenia
✧ Steroids, late HIV, hemetologic malignancy, alveolar proteinosis
N. Asteroides: Pathology
• Peribronchial abscesses, granulomatous inflammation
• Extensive necrosis
➢ May mimic M.TB or fungal infection
N. Asteroides: Radiology
• Extrapulmonary disease 50% with 40-90% mortality
➢ CNS 25%
➢ Skin and subcutaneous abscesses
N. Asteroides: Radiology
• Consolidation
➢ Mass-like
➢ Cavitary
• Pleural and chest wall involvement 30-50%
• Adenopathy 40%
Cavitary Pneumonia in AIDS

• N. asteroides
Alveolar Proteinosis and N. asteroides
Parasitic Infection
• Pulmonary involvement due to hypersensitivity or direct invasion
➢ Echinococcosis
➢ Paragonimiasis
➢ Ascariasis Figure 1-19-13
➢ Strongyloidiasis
Parasitic Infection
• Radiographic findings may overlap with other
infections
➢ Fleeting, patchy infiltrates
➢ Bronchopneumonia
➢ Atelectasis
Echinococcus granulosus
• Cestode (tapeworm), endemic to S.America,
Australia, Middle East, Africa and Mediterranean
• Definitive host - dog,wolf
Intermediate host - sheep, cow, deer, moose
• Duodenum - portal venous system liver
➢ 45-75% isolated liver involvement
➢ 15-35% pulmonary involvement
Intact (right lung) and ruptured (left lung)
echinococcal cysts
Echinococcus granulosus [Figure 1-19-13]
• Pulmonary cysts acquired in childhood
• Diagnosis 30-40yo
• Intact cyst - asymptomatic
• Eosinophilia 25-40%

Echinococcus granulosus: Pathology
• Hydatid cyst consists of 3 layers
➢ Pericyst – host inflammatory cells
➢ Exocyst – acellular laminated membrane
➢ Endocyst – fluid-filled germinal center, daughter cysts
Echinococcus granulosus: Radiology

• Intact cyst
➢ Well demarcated, homogeneous mass
➢ Spherical when central, ovoid when peripheral
➢ Multiple 20-30%
➢ Lower lobes 60%
Echinococcus granulosus: Radiology

• Impending Rupture
➢ Crescent sign - air between pericyst and laminated membrane
• Ruptured cyst
➢ Water lily sign – rupture of endocyst
Paragonimiasis westermani
• Trematode (lung fluke)
➢ endemic to Asia
➢ Contaminated freshwater crab
• Jejunum – peritoneal cavity – diaphragm – pleura – lung
• Chronic granulomatous reaction
Paragonimiasis westermani: Radiology

• Pulmonary findings dependant on stage of infection
➢ PTX and pleural infection during pleural penetration by juvenile worms
➢ Transient, patchy consolidation and linear tracts during larval migration
• Peribronchial cysts associated with mature worm
Ascariasis lumbricoides
• Roundworm infection
• Most common parasitic infection
➢ Endemic worldwide
➢ 25-95% prevalence
➢ Highest incidence in children
• Large iingestion associated with pneumonitis
• Small bowel – systemic circulation – alveoli - trachea – small bowel
Strongyloides stercoralis
• Round worm
• Skin – systemic circulation – alveoli – trachea – small bowel
Ascariasis Strongyloides: Radiology

• Bronchopneumonia
• Patchy, transient consolidation
• Eosinophilic pneumonia
B. Anthracis: Anthrax
• Gram+ spore forming rod
➢ Dormant spores are virulent
• Infection typical in livestock
• Exotoxin production associated with hemorrhagic mediastinitis, edema and
pleuritis
Earls Radiology:222:305, 2001

Complications of Pneumonia [Figure 1-19-14] Figure 1-19-14
• Pleural Infection
• Empyema
➢ Purulent exudate
➢ WBC>25,000
➢ pH<7.0
➢ + organisms
S.mitis Empyema in 51yo male with IVDA hx
Complications of Pneumonia Right empyema and LUL septic

• Cavitation embolus due to S. aureus
➢ Cavitary pneumonia
➢ Lung abscess
➢ Pneumatocele
➢ Gangrene
➢ DDX bronchopleural fitula
Complications of Pneumonia
• Pneumatocele
➢ Ball-valve mechanism
➢ Rapid evolution
➢ No lung destruction
• Most common with S.aureus
Figure 1-19-15
➢ 60% of peds infection
Complications of Pneumonia [Figure 1-19-15]

• Pulmonary Gangrene
➢ Lung necrosis due to vascular thrombosis
• Most common with S.pneumoniae K.pneumoniae
• Bronchiectasis
➢ Irreversible dilation
✧ Should not be diagnosed < 4 m of acute
infection
➢ Colonization with atypical TB, aspergillus
CXR and CT pulmonary gangrene due to
➢ Advanced course in HIV
K.pneumoniae
➢ +/- antecedant infection
A.fumigatus complicating post-infectious bronchiectasis
The Role of Imaging in Pneumonia

• Diagnosis of infection
➢ Presence of centrilobular nodules in acute parenchymal disease favors
pneumonia
Tomiyama N. AJR 2000;174:1745
➢ Thin section CT allows earlier diagnosis of pneumonia in
immunosuppressed pts (5 days)
Heussel CP. AJR 1997;169:1347
• Recognition of complications
➢ Decreased enhancement in pneumonia indicates severe necrosis
Donnelly LF. Radiology 1997;205:817
The Practical Points

• Organisms may produce more than one pattern
• Bacterial, viral and fungal pneumonia have similar CT findings post lung
transplantation
Collins AJR 2000;175:811
• Consider clinical setting

References
1. Kim JS, Ryu CW, Lee SI, Sung DW, Park CK. High-resolution CT findings of varicella-zoster pneumonia. AJR
Am J Roentgenol. 1999 Jan;172(1):113-6.
2. Moon WK, Im JG, Yeon KM, Han MC. Complications of Klebsiella pneumonia: CT evaluation. J Comput Assist
Tomogr. 1995 Mar-Apr;19(2):176-81.
3. Ooi GC, Khong PL, Muller NL, Yiu WC, Zhou LJ, Ho JC, Lam B, Nicolaou S, Tsang KW. Severe acute
respiratory syndrome: temporal lung changes at thin-section CT in 30 patients. Radiology. 2004 Mar;230(3):836-
44.
4. Paul NS, Chung T, Konen E, Roberts HC, Rao TN, Gold WL, Mehta S, Tomlinson GA, Boylan CE, Grossman H,
Hong HH, Weisbrod GL. Prognostic significance of the radiographic pattern of disease in patients with severe
acute respiratory syndrome. AJR Am J Roentgenol. 2004 Feb;182(2):493-8.
5. Paul NS, Roberts H, Butany J, Chung T, Gold W, Mehta S, Konen E, Rao A, Provost Y, Hong HH, Zelovitsky L,
Weisbrod GL. Radiologic pattern of disease in patients with severe acute respiratory syndrome: the Toronto
experience. Radiographics. 2004 Mar-Apr;24(2):553-63. Review.
6. Reittner P, Muller NL, Heyneman L, Johkoh T, Park JS, Lee KS, Honda O, Tomiyama N. Mycoplasma
pneumoniae pneumonia: radiographic and high-resolution CT features in 28 patients. AJR Am J Roentgenol. 2000
Jan;174(1):37-41.
7. Shah RM, Gupta S, Angeid-Backman E, O'Donnell J. Pneumococcal pneumonia in patients requiring
hospitalization: effects of bacteremia and HIV seropositivity on radiographic appearance. AJR Am J Roentgenol.
2000 Dec;175(6):1533-6.
8. Watanakunakorn C, Greifenstein A, Stroh K, Jarjoura DG, Blend D, Cugino A, Ognibene. AJ. Pneumococcal
bacteremia in three community teaching hospitals from 1980 to 1989.Chest. 1993 Apr;103(4):1152-6.
9. Collins J, Muller NL, Kazerooni EA, Paciocco G. CT findings of pneumonia after lung transplantation. AJR Am J
Roentgenol. 2000 Sep;175(3):811-8.
10. Donnelly LF, Klosterman LA. Pneumonia in children: decreased parenchymal contrast enhancement--CT sign of
intense illness and impending cavitary necrosis. Radiology. 1997 Dec;205(3):817-20.
11. Earls JP, Cerva D Jr, Berman E, Rosenthal J, Fatteh N, Wolfe PP, Clayton R, Murphy C, Pauze D, Mayer T,
Bersoff-Matcha S, Urban B. Inhalational anthrax after bioterrorism exposure: spectrum of imaging findings in two
surviving patients. Radiology. 2002 Feb;222(2):305-12.
12. Fartoukh M, Azoulay E, Galliot R, Le Gall JR, Baud F, Chevret S, Schlemmer B. Clinically documented pleural
effusions in medical ICU patients: how useful is routine thoracentesis? Chest. 2002 Jan;121(1):178-84.
13. Heussel CP, Kauczor HU, Heussel G, Fischer B, Mildenberger P, Thelen M. Early detection of pneumonia in
febrile neutropenic patients: use of thin-section CT. AJR Am J Roentgenol. 1997 Nov;169(5):1347-53.
14. Johanson WG Jr, Pierce AK, Sanford JP, Thomas GD. Nosocomial respiratory infections with gram-negative
bacilli. The significance of colonization of the respiratory tract. Ann Intern Med. 1972 Nov;77(5):701-6.
15. Smego RA Jr, Foglia G. Actinomycosis. Clin Infect Dis. 1998 Jun;26(6):1255-61; quiz 1262-3. Review.
16. Tomiyama N, Muller NL, Johkoh T, Honda O, Mihara N, Kozuka T, Hamada S, Nakamura H, Akira M, Ichikado
K. Acute parenchymal lung disease in immunocompetent patients: diagnostic accuracy of high-resolution CT. AJR
Am J Roentgenol. 2000 Jun;174(6):1745-50.

Uncommon Malignant Tumors of the Lung
Uncommon Malignant Tumors of the Lung:

• Bronchial Carcinoid - most common “Uncommon”
• Adenoid Cystic Carcinoma ----> Salivary Gland Tumors
• Mucoepidermoid Carcinoma----> Salivary Gland Tumors
• Carcinosarcoma ----> Mixed Tumors
• Pulmonary Blastoma ----> Mixed Tumors
“Bronchial Adenoma”: History Lesson

• Term formerly referred to:
➢ Bronchial Carcinoid
➢ Adenoid Cystic Carcinoma
➢ Mucoepidermoid Carcinoma
• A misnomer
• These tumors are not benign
Carcinoids
• Gastrointestinal tract 90%
• Lung
• Thymus
• Biliary tract
• Ovarian teratomas
Bronchial Carcinoid
• Typical carcinoid
➢ Low-grade malignancy
• Atypical carcinoid
➢ Moderate-grade malignancy
Typical Carcinoid
• 0.6-2.4% of all pulmonary neoplasms
• Low grade malignancy
• Good prognosis
➢ 95% five-year survival
• Not associated with smoking
Typical Carcinoid: Demographics

• Males = Females
• Wide age range. Median age: 50 years
• Symptoms: cough, hemoptysis, dyspnea
Typical Carcinoid: Microscopy

• Uniform cells
• Forming nests, ribbons, rosettes, trabeculae
• Stroma highly vascular
• May exhibit calcification or osseous metaplasia
• Polygonal cells, pale cytoplasm, stippled nuclear chromatin
• Rare mitoses
• Ultrastructure: Neurosecretory granules
Neuroendocrine Cells and Tumors: Electron microscopy

• Cytoplasmic neurosecretory granules
• Central or eccentric dense core
• Thin lucent halo
• May contain biologically active peptides
Uncommon Malignant Tumors 192 Chest Radiology

Neuroendocrine Markers: Immunohistochemistry
• Chromogranin
• Synaptophysin
• Neural cell adhesion molecules (NCAM)
Carcinoid: Relationship to Small Cell Carcinoma

• Similarities:
➢ Neurosecretory granules
➢ Rosette and trabecula formation
• Differences:
➢ Fewer granules in Small Cell Carcinoma
➢ Carcinoid not associated with smoking
Tumors with NE Morphology

• A spectrum by light micropscopy
• Low-grade Typical Carcinoid

• Intermediate Atypical Carcinoid
• High-grade Small cell lung carcinoma
Large cell neuroendocrine carcinoma
Neuroendocrine Tumors:
World Health Organization criteria (1999) [Figures1-20-1]
• Typical carcinoid: <2 mitoses per 10 HPF
• Atypical carcinoid: 2-10 mitoses per 10 HPF
• Large cell neuroendocrine ca: 11 or more mitoses per 10 HPF (median 70)
• Small cell ca: 11 or more mitoses per 10 HPF (median 80)
Figure 1-20-1 NE Tumors: Metastases
Atypical Carcinoid: Histopathologic criteria

• Poor architectural organization
• Cellular pleomorphism
• Focal necrosis
• Increased mitotic activity
Arrigoni J Thorac Cardiovasc Surg 1972
Atypical Carcinoid
• Morphology between Typical Carcinoid and Small Cell Ca
• Tend to be larger, more invasive, peripheral
• Age: Decade older than Typical
• Symptoms: similar to Typical
• Imaging: similar to Typical
Chest Radiology 193 Uncommon Malignant Tumors

Atypical Carcinoid
• 10% of bronchial carcinoid
• Peripheral
• Increased mitoses
• Aggressive behavior; early metastases
• Osteoblastic bone metastases
• Pathology DDx: Small Cell Carcinoma
Bronchial Carcinoid: Gross Pathology

[Figure 1-20-2]
• Usually seen at bronchoscopy
• Soft, fleshy, endobronchial mass
• Sessile. May be pedunculated
• Often extend through wall
Figure 1-20-2
Growth patterns of bronchial carcinoid tumors. Partially endobronchial tumors

(“iceberg” configuration) most common
Bronchial Carcinoid: Central

• Bronchiectasis
• Mucoid impaction
• Obstructive pneumonia
Bronchial Carcinoid: Peripheral

• Late presentation
• Discovered incidentally

Bronchial Carcinoid: Radiologic Findings Figure 1-20-3
[Figures 1-20-3 and 1-20-4]
• Central tumors in 80%
• Lobar, segmental, subsegmental bronchi
• Consolidation, atelectasis
Figure 1-20-4
Bronchial carcinoid manifesting as a

Small endobronchial carcinoid tumor in distal left mainstem bronchus central, well-defined left perihilar mass
Bronchial Carcinoid: CT Features [Figures 1-20-5 to 1-20-7] Figure 1-20-5

• Bronchial relationship in 83%
• Partially endobronchial
• Completely endobronchial
• Abutting a bronchus
• Sharply marginated, lobulated mass
• May enhance or demonstrate Ca++
• Atelectasis, consolidation, bronchiectasis, mucoid
impaction
• Lymphadenopathy
Figure 1-20-6
Bronchial carcinoid. Partially endobronchial (‘iceberg”)
tumor in left upper lobe
Figure 1-20-7
Bronchial carcinoid obstructing left upper lobe bronchus

with distal atelectasis, pneumonitis and associated left
hilar lymphadenopathy
Bronchial carcinoid in a 34-year-old male. Unenhanced

CT (left) demonstrates central tumor and distal,
peripheral consolidation. Contrast enhanced CT (right)
shows diffuse contrast enhancement

Bronchial Gland Tumors Figure 1-20-8
• Adenoid Cystic Carcinoma
• Mucoepidermoid Carcinoma
• Equivalent to salivary gland tumors of same name
Adenoid Cystic Carcinoma [Figure 1-20-8]

• Synonym - Cylindroma
• 80% of bronchial gland tumors
• 20 to 35% of all tracheal tumors
• Second most common tracheal malignancy (after
Squamous cell carcinoma)
Adenoid Cystic Carcinoma

• Guarded prognosis
• Common local recurrence Adenoidcystic carcinoma partially occluding the trachea
• Occasional metastases to regional nodes and extending into the adjacent soft tissues of the
• Rarely extrathoracic spread mediastinum
Adenoid Cystic Carcinoma: Demographics

• Males = Females
• Wide age range - Average age: 40 - 50
• Symptomatic patients: cough, wheezing, dyspnea, hemoptysis
Adenoid Cystic Carcinoma: Microscopy Figure 1-20-9

• Mucin-containing cysts
• Varying in caliber
• Within larger tumor tubules
• Surrounds, invades nerves
• Encases vessels, infiltrates bronchi
• Few mitoses
Adenoid Cystic Carcinoma: Pathologists’

Pitfalls
• Misdiagnosis
➢ Adenocaracinoma
➢ Pleomorphic adenoma
➢ Small cell carcinoma
• Metastatic salivary gland tumor
• Solid pattern on small biopsy
Adenoid Cystic Carcinoma: Gross

• Endobronchial mass
➢ Trachea and main bronchi
• Sessile, polypoid, annular growth
• Proximal & distal spread
• Extension into mediastinum
Adenoid Cystic Carcinoma: Radiology [Figure

1-20-9] Adenoidcystic carcinoma. Coronal (left) and saggital
• Central: trachea and main bronchi (right) surface-rendered CT images of the trachea
• Intraluminal nodule or mass demonstrate both nodular and circumferential deformity
• Constriction of tracheal/bronchial lumen of the air-column in the lower trachea
• 10 - 15% in lung periphery
• CT / MRI: length of involvement; mediastinal involvement
Mucoepidermoid Carcinoma: Demographics and Prognosis

• Male=Female. Wide age range
• Cough, fever, hemoptysis, pneumonia, atelectasis
• Low-grade: excellent prognosis
• High-grade: better prognosis than Bronchogenic Ca

Mucoepidermoid Carcinoma [Figure 1-20-10] Figure 1-20-10
Mucoepidermoid Carcinoma: Microscopy

• Low grade:
➢ Mucinous cysts
➢ Solid collections of squamous cells
• High grade:
➢ Solid sheets of tumor
➢ Mitoses and necrosis
Mucoepidermoid Carcinoma: Gross

• Submucosal, smooth surfaced
• Endobronchial, exophytic, polypoid
• High grade may have ragged invasive appearance
Mucoepidermoid Carcinoma: Mucoepidermoid carcinoma arising in the right

Radiologic Findings [Figures 1-20-11 and 1-20-12] mainstem bronchus at the origin of the right upper lobe
• Solitary nodule or mass bronchus
• Most in main or lobar bronchi
• Few in trachea Figure 1-20-11
• Distal effects:
➢ Atelectasis, pneumonia
• Central lesions
• Atelectasis, pneumonia
Mixed Tumors: Neoplasms with malignant epithelial

and mesenchymal components
• Carcinosarcoma
• Pulmonary Blastoma
Carcinosarcoma
• Rare - 0.3% of all lung neoplasms
• Middle aged and elderly males
• Poor prognosis
• Aggressive: local invasion, widespread metastases, and rapid
death
Carcinosarcoma: Microscopy Mucoepidermoid carcinoma manifesting as a

• Epithelial component: solitary pulmonary nodule on chest
➢ Squamous Cell Carcinoma radiography
➢ Adenocarcinoma
➢ Undifferentiated Carcinoma
• Mesenchymal component:
➢ Usually dominant Figure 1-20-12
➢ Spindle cell (common)
➢ Chondrosarcoma
➢ Osteosarcoma
➢ Rhabdomyosarcoma
Carcinosarcoma: Gross
• Peripheral
➢ Large mass
➢ Average diameter 6 cms.
➢ Frequent necrosis and hemorrhage
• Central
➢ Endobronchial growth
➢ May extend to adjacent parenchyma
➢ Tumor-distended bronchi may resemble mucus plugs
Central mucoepidermoid carcinoma manifesting as mild

prominence of the suprerior aspect of the right hilum
with associated atelectasis of the right upper lobe

Carcinosarcoma: Imaging [Figure 1-20-13] Figure 1-20-13
• Peripheral
➢ Large
➢ Well-circumscribed mass
• Central
➢ Atelectasis, pneumonia
➢ Tumor “mucus plugs”
➢ Upper lobe predominance
➢ Direct extension to pleura, chest wall, and
mediastinum
Pulmonary Blastoma
• Primary lung tumor
• Mix of epithelial and mesenchymal components
• Both components blastomatous and immature
• Morphologic mimic of embryonal lung
• ? a variant of carcinosarcoma
Pulmonary Blastoma: Demographics and Carcinosarcoma. Contrast enhanced CT demonstrates

Prognosis a peripheral mass with irregular and lobulated borders in
• Predominantly males the right upper lobe
• Biphasic age distribution: first and seventh decades
• Symptoms: cough, hemoptysis, dyspnea, chest pain
• Poor survival
Figure 1-20-14
Pulmonary Blastoma: Microscopy

• Mixture:
➢ Epithelial-lined tubules
➢ Primitive stroma
• Resembles embryonal lung
• Metastases: mesenchymal, epithelial, or mixed
Pulmonary Blastoma: Gross

• Large mass
• Unencapsulated and soft
• Abundant central necrosis and hemorrhage Pulmonary blastoma manifesting as a
large, heterogeneous mass in the left
Pulmonary Blastoma: Radiology [Figure 1-20-14] lower lobe
• Large peripheral mass
• May show pleural invasion
• May metastasize
Endobronchial Tumors: Malignant

• Squamous cell ca
• Adenocarcinoma
• Small cell ca (rare)
• Carcinoid
• Adenoid cystic ca
• Mucoepidermoid ca
• Carcinosarcoma
• Pulmonary blastoma
• Sarcoma (10%)
• Endobronchial metastasis
• Lymphoid malignancies (NHL>HD)

Benign Tumors of the Lung and
Tumor-like Lesions
Benign Tumors and Tumor-like lesions

• Hamartoma
• Papilloma / Papillomatosis
• Inflammatory pseudotumor
• Granuloma
“Hamartoma”
• Albrecht, 1904
• Tumor-like malformation
• Tissues normal to location
• In excess or disarray (disorganized)
• “Adult”, “Classic”, “Local” hamartoma
Hamartoma
• Acquired lesion
• Disorganized growth of tissues normally found in lung
• Benign neoplastic proliferation
• Probably derived from bronchial wall mesenchymal cell
(“benign mesenchymoma”)
Hamartoma
• Most common benign tumor of lung
• 77% of benign lung tumors
• 8% of SPNs
• 3% of all lung tumors
Hamartoma: Evidence of Acquired Lesion

• Onset in adult life
• Often adults with previously normal CXR
• Almost never seen in infants
• Histology: passive entrapement of epithelium
• Cytogenetics: Chromosome 12: abnormal q13-q15 regions
(as in other benign soft-tissue neoplasms)
Hamartoma: Demographics
• Age range: 30-70 years
• Peak incidence: 6th decade
• Female: Male = 3:2 (1:1 for endobronchial hamartoma)
• Asymptomatic in 90%
< 8% obstructive symptoms
Hamartoma: Clinical
• Most are peripheral and asymptomatic
• If symptomatic: hemoptysis
• If bronchial obstruction: pneumonitis
• Fever, cough, expectoration, chest pain
Hamartoma: Microscopic
• Cartilage nests (lobules) in 95%
• Surrounded by fibrous tissue
• Mature fat cells
• Cleft-like invaginations of entrapped respiratory epithelium
Chest Radiology 199 Benign Tumors

Hamartoma: Gross Figure 1-21-1
• Solitary
• 1 – 3 cm (rarely “Giant”)
• Rounded, well-circumscribed, lobulated
• Firm lesions. Usually cartilaginous
• May see areas of fat
• Easily “shelled-out”
Hamartoma: Distribution
• Peripheral > Central
• 80 – 90 % Peripheral
• No lobar predilection
Hamartoma: Radiographic
• Sharply defined, lobulated subpleural Hamartoma. Unenhanced chest CT demonstrates a
• Most < 3 cm peripheral solitary nodule with focal fat attenuation
• Calcification on CXR (10-15% )
• Rarely see fat on CXR Figure 1-21-2
• May enlarge on serial CXRs
• Up to 3 to 5 mm per year
Hamartoma: Calcification
• 10 – 15% speckled or “Popcorn”
• “Popcorn” less frequent than once thought
• Diagnostic when present
➢ Nodular growths within lesion
➢ Protrude in different directions
Hamartoma: Computed Tomography

• Distinguishes fat and cartilage
• Most are 2.5 cm or less
• Smooth edge
• No fat / Focal fat alone / Fat with areas of
calcification Hamartoma. Unenhanced chest CT demonstrates a
• Cavitation: rare central mass in the left lower lobe with a lobular area of
“popcorn” calcification
Hamartoma: Computed Tomography [Figures 1-21-1 to 1-21-3]
• Thin sections (2mm)
• Smoothly contoured nodule
• = or < 2.5 cm diameter
• Focal fat in 8 voxels or more
• Or fat with calcification
Siegelman. Radiology 1986; 160:313-317. Figure 1-21-3
Hamartoma: Computed Tomography

• 36% no fat or calcification
• 4% diffuse calcification
• 38% areas of fat
• 21% calcium and fat
• Occasionally: focal calcification, no fat
“Carney’s Triad”
• Gastric smooth muscle tumors
• Extra-adrenal paraganglioma
• Pulmonary chondroma
• Association unclear
• Young females < 20 years
• May have only 2/3 of the triad Hamartoma. Unenhanced chest CT demonstrates
Carney JA. Cancer 1979 speckled calcification in a central endobronchial tumor
with associated loss of volume in the left lung
Benign Tumors 200 Chest Radiology

Chondroma
• Rare
• Benign cartilaginous tissue
• Parenchymal or endobronchial
• Lack epithelial-lined clefts seen in hamartomas
• In young female
• Search for Carney’s Triad
Hamartoma: Endobronchial
• Morphologically identical to parenchymal
• Often polypoid. Sessile or thin pedicle
• Manifest by airway obstruction
• Micro: more fat, lack clefts, cartilage scant or absent
Hamartoma: Treatment and Prognosis

• Benign
• Surgical excision = Cure
• Exceptional cases: additional hamartomas
Papillomas
• Branching or coarsely lobulated tumor
• Arise from and project above an epithelial surface
• Rare pulmonary tumors
• Solitary (rare) or Multiple (papillomatosis)
• Proximal or peripheral
Solitary Papillomas
• Rare
• Usually in adults
• Papillary exophytic growth
• Trachea, main or lobar bronchi
• Males >40 years of age
• Post-obstructive pneumonia, bronchiectasis
Juvenile Laryngeal Papillomatosis

• Children 18 months to 3 years of age
• Majority remain localized, disappear spontaneously
• May spread distally and obstruct airways
➢ 5% Spread remains limited to trachea
➢ 1% Develop lung disease - 10 years after laryngeal disease
(extension to bronchi, bronchioles, alveolar airspaces)
Laryngeal Papillomatosis: Demographics and Etiology

• Human papilloma virus - HPV types 6 and 11
• 0.1% of infants develop LP. Predilection for first-born infants
• 50% of their mothers have genital tract involvement
• HPV spread transvaginally at birth
• Infects oropharyngeal secretions of child
Papillomas: Microscopic
• Non-keratinizing squamous cells
• Fibrovascular core
• Form papillomatous projections
Papillomatosis: Gross
• Cauliflower-like excresences
• Protrude into bronchial lumens
• May extend into parcenchyma as nodules or cavities

Laryngeal Papillomatosis
• Majority remain localized
• 5% spread to trachea and distal airways
Tracheobronchial Papillomatosis
• Many remain limited to trachea
• 1% Develop lung disease
• Patients with lung disease may develop
• Squamous cell carcinoma
Tracheobronchial Papillomatosis: Pathogenesis of lower

respiratory tract involvement:
• Implantation of inhaled fragments from larynx?
• Multifocal viral infection?
• Trauma-induced by tracheostomy? Figure 1-21-4
• In children, papillomas in bronchi and lung associated with multiple papillomas
of trachea or larynx
Papillomatosis: Imaging
• Multiple nodules
• Cavities, 2-3 mm thick walls
• Air-fluid levels
Papillomatosis: Imaging [Figures 1-21-4 and 1-21-5]

• Multiple, well-defined nodules
• Perihilar, posterior thorax
• Grow to several centimeters
• Cavitate, 2-3 mm thick walls
• Air-fluid levels may develop
• Cavities may represent:
➢ Papillomatosis
➢ Squamous cell carcinoma
➢ Abscess (obstructive pneumonitis)
Papillomatosis. Chest CT demonstrates nodular and
Papillomatosis: Treatment and Prognosis cystic opacities that predominantly involve the dorsal
• Multiple recurrences aspects of both lungs
• Multiple excisions
• Tracheostomy Figure 1-21-5
• 37.5% mortality if spread to lungs
• Worse if malignant degeneration occurs
Inflammatory Pseudotumor: Synonyms

• Plasma cell granuloma
• Histiocytoma
• Fibroxanthoma, Xanthoma
• Myofibroblastic tumor
• Mast cell granuloma
Inflammatory Pseudotumor
• Uncommon. Reactive or neoplastic process?
• May begin as organizing pneumonia
• May have aggressive features:
➢ Vertebral destruction
➢ Recurrence
Papillomatosis and squamous cell

carcinoma. Contrast-enhanced Chest CT
demonstrates central squamous cell
carcinoma in the left lower lobe with distal
pneumonitis

• WHO 1999: histologic spectrum of
fibroblastic and myofibroblastic proliferations
• With varying infiltrate of inflammatory cells
• Usually solitary, tumefactive lesion
• Destroys underlying lung architecture
• Reactive or Neoplastic ?
Inflammatory Pseudotumor: Demographics

• Males = Females
• Wide age range: 1 to 77 years. Average: 29.5 years
• 60% <40 years
➢ Children: peak 6-7 years
o
➢ Most common 1 lung mass in children
• Many patients have history of respiratory infection
Inflammatory Pseudotumor: Microscopic

• Variable.
• A continuum from plasma cell granuloma to fibrohistiocytic
• Mixture of collagen, fibroblasts, myofibroblasts, and chronic inflammatory cells
Inflammatory Pseudotumor: Gross

• SPN or Mass
• Well-defined. Firm. Lobulated
• Lack a capsule
• Cut-surface: whorled, heterogeneous
• 1-10cm, 4.4 cm mean
• Solitary nodule or mass in 70%
• Well-defined
• May manifest as consolidation
• May mimic neoplasm
• Endobronchial lesions occur in 10% Figure 1-21-6
Inflammatory Pseudotumor: Radiographic

• Solitary, well-defined nodule or mass 70%
• Endobronchial lesions occur 10%
• May extend into mediastinum 5%
• Parenchymal consolidation 6%
• Calcification, cavitation infrequent
• May mimic malignant neoplasm
• Usually no or slow growth. May regress
Inflammatory Pseudotumor: CT [Figure 1-21-6]

• Solitary nodule or mass
• Sharply circumscribed
• Lobulated
• Heterogeneous or homogeneous Inflammatory pseudotumor. Contrast-enhanced chest
• Enhancement: / calcification variable, nonspecific CT demonstrates an irregular, heterogeneous mass in
• Calcification: variable the left upper lobe
• Endobronchial lesions occur
Inflammatory Pseudotumor: Therapy and Prognosis

• Diagnosed and treated by surgical excision
• Excellent prognosis after resection
• Recurrence in 5%
➢ Especially if mediastinal or chest wall involvement
• DDx: fibrous histiocytoma, sarcomatoid carcinoma

Granulomas
• Infectious
• Sarcoid (necrotizing granuolomatosis)
• Hypersensitivity pneumonitis
Infectious Granulomas
• Mycobacterial 64%
• Fungal 30%
• Parasitic 6%
Granuloma: Infectious
• Tuberculoma or Histoplasmoma
• Satellite lesions common
• Usually small, smooth
• Often calcified when healed
Granuloma – Well-defined Pulmonary Nodule

Multiple Ill-defined Pulmonary Nodules
• TB
• Histoplasmosis
• Coccidioidomycosis
• Cryptococcosis
• Aspergillosis
Granulomas – Tiny nodules

• <5 mm, micronodular, military
• Histoplasmosis
• Blastomycosis
• Cryptococcosis
Solitary Pulmonary Nodule

(n = 955)
• Malignant 49%
➢ Primary carcinoma 38%
➢ Metastases 9%
➢ Other 1° malignancy 2%
• Benign 51%
➢ Non-neoplastic lesion 37%
➢ Tumor 14%
( Hamartoma 8% )
Toomes H. The coin lesion of the lung. Cancer 1983.

Pleural Disease I
Pleural Disease I and II: Objectives

• Anatomy and physiology
• Non-neoplastic and neoplastic pleural disease
• Chest wall disease
• Radiologic-Pathologic correlation
Figure 1-22-1
Pleural Disease I
• Normal anatomy
➢ Standard fissures
➢ Accessory fissures
• Non-neoplastic pleural disease
➢ Effusions
➢ Fibrosis
➢ Pneumothoraces
Standard (solid lines)
Pleural Anatomy and accessory (dashed lines) fissures
• Parietal Pleura
➢ Covers non-pulmonary surfaces
➢ Systemic supply/drainage
➢ Lymphatics communicate with pleural space
Figure 1-22-2
➢ Pain fibers
➢ 5-15 ml of pleural fluid
• Visceral Pleura [Figure 1-22-1]
➢ Covers lung surface
➢ Dual supply/drainage
➢ Vagus nerve/sympathetic trunks
➢ Lymphatics do not communicate with pleural
space
Pleural Imaging
• Radiography / CT
➢ Inconspicuous
➢ Visceral + Parietal = 0.2 mm
• Thin-collimation
➢ 1-2 mm thick line
➢ Intercostal regions
➢ Normal fluid
➢ Endothoracic fascia Pneumothorax in a supine patient manifesting as a
➢ Innermost intercostal m. “deep sulcus” and hyperlucency overlying the left
hemidiaphragm
Pleural Anatomy [Figure 1-22-2]
Caudal limit of pleura lower than lung
Costal and diaphragmatic pleura contact to form costophrenic recess
Pleural Anatomy
• Junction Lines
• Apposition of layers of pleura
➢ Anterior
➢ Posterior
Pleural Anatomy - Fissures

• Visceral pleura
• Variable depth into parenchyma
• Complete
• Incomplete
Chest Radiology 205 Pleural Disease I

Incomplete Major Fissure: CT
• More frequent Right
• RUL / RLL 70%
• RML / RLL 47%
• LUL / LLL 40%
• Lingula / LLL 46%
Major Fissure: Radiography

• Major (oblique) fissures
• Best seen on lateral CXR
• Origin: T4 Left, T5 Right
• Right fissure more oblique Figure 1-22-3
Major Fissure: CT
• 80-90% of standard CT
➢ Lucent band
➢ Line
➢ Dense band
Major Fissure: CT
Propeller-like morphology [Figure 1-22-3]
• Upper thorax
➢ Anterior concave
➢ Lateral-facing
• Inferior thorax
➢ Anterior convex Chest CT (lung window) demonstrates right upper
➢ Medial-facing lobe loss-of-volume manifested by displacement of
the right major fissure. An endobronchial carcinoid
Standard Fissures: Radiography obstructs the origin of the right upper lobe
• Minor fissure bronchus
➢ “Lights up” in CHF
➢ Interstitial edema
(Subpleural interstitium)
Figure 1-22-4
Minor Fissure: CT
• Lucent area
• Devoid of vasculature
• 44% triangular
• 8% round / ovoid
• Ground glass attenuation
Incomplete Minor Fissure: HRCT

• Curvilinear line or band
• Increased attenuation
• C-shaped
• Fusion between RUL / RML (60-90%)
Accessory Fissures: Radiography [Figure 1-22-4]

• 10% CXR / 20% CT (50% of anatomic specimens)
➢ Azygos, Superior, Inferior, Left minor
Accessory Fissures: Azygos

• Abnormal migration of posterior cardinal vein
• Four layers of pleural
• 1% population Diagram illustrates position of
• 2M : 1F inferior (I), superior (S) and azygos
(az) accessory fissures
Pleural Disease I 206 Chest Radiology

Accessory Fissures: CT Azygos [Figure 1-22-5]
Accessory Fissures: Inferior Accessory [Figures 1-22-6 and 1-22-7]

• Separates medial basal segment from remaining basilar segments
• Most common
Figure 1-22-5
• 30 - 45% anatomic specimens
• CXR: 5 - 10%
• 80% Right-sided
• CT: 15%
Indirect Signs of Atelectasis:

Juxtaphrenic Peak
• Common in RUL or LUL atelectasis
➢ Less common in RML
• Seen post upper lobectomy
• Small triangular opacity
• Projects upward from diaphragm
• Related to inferior accessory fissure
Chest CT demonstrates an azygos fissure forming
Accessory Fissures: Superior Accessory the lateral margin of an azygos lobe
• Separates superior segment from basilar segments
• 6% anatomic specimens
• Right > Left Figure 1-22-6
• Horizontal course
➢ Inferior to minor fissure
Accessory Fissures: Left Minor Fissure

• Separates lingula from remainder of upper lobe
• 8-18% anatomic specimens
• 1.5% of chest radiographs
• Oblique course
• More cephalad
Pulmonary Ligament
• Formed by Parietal & Visceral pleura
• Courses inferiorly & posteriorly
• Contains bronchial veins, lymphatics, nodes
Figure 1-22-7
Coned-down view of right lung demonstrates

an inferior accessory fissure separating the
medial basal segment from the remaining
basal segments of the right lower lobe
Chest CT demonstrates an
inferior accessory fissure
(arrow).

Pulmonary Ligament: Imaging [Figure 1-22-8] Figure 1-22-8
• CXR: not visualized
• CT: 60%-70%
Pleural Effusion
Cardiac Decompensation [Figure 1-22-9]
• Most common cause
• Increased hydrostatic pressure
• Bilateral >80%
• Unilateral = right-sided
• Pseudotumor
Figure 1-22-9 Chest CT demonstrates inferior accessory fissure
Lateral chest (arrowhead) and right and left pulmonary
radiograph ligaments (arrows)
demonstrates
lenticular opacity of
fluid accumulation in
the minor fissure
(pseudotumor)
Figure 1-22-10
Pleural Effusion: Bacterial Pneumonia

• Parapneumonic effusions 40%
• Exudate
• 10% require drainage
• Complications Ultrasound demonstrates multiple septations
➢ Loculation within a loculated fluid collection representing
➢ Empyema empyema
Pleural Effusion: Empyema [Figure 1-22-10] Figure 1-22-11

• Three phases
➢ Exudative
➢ Fibrinopurulent
➢ Organizing
Pleural Effusion: Empyema [Figure 1-22-11]

• Lenticular shape
• Obtuse margins
• Compress lung Contrast
• Split pleura sign enhanced chest
CT demonstrates
Pleural Effusion: smoothly
Lung abscess or Empyema ? thickened
parietal and
Pleural Effusion: Empyema visceral pleura
• Lenticular shape enclosing a fluid
• Obtuse margins collection of
• Compress lung empyema (“split
• Split pleura sign pleura sign”)
• Disparity in length of air-fluid level

Pleural Effusion: Lung abscess
• Round shape
• Does not compress lung
• Equal length of air-fluid level
Pleural Effusion: Empyema

• Treatment
➢ Tube thoracostomy
➢ Fibrinolytics
➢ Decortication
Pleural Effusion: Empyema necessitatis

• Inadequate treatment
• Drainage into chest wall
• Tuberculosis 73%
• Bacterial / Fungal
v Malignancy
• Immunocompromised patients
Pleural Effusion: Tuberculosis

• Exudate
➢ ↑ lymphocyte count
➢ ↓ glucose level
• Unilateral
• Small to moderate
Pleural Effusion: Subpulmonic

• Fluid accumulates between lung base and diaphragm
• Shifts apex of diaphragm laterally
• Usually transudate
➢ Cardiac decompensation
➢ Renal failure
➢ Cirrhosis with ascites
Pleural Effusion: Subpulmonic

• Imaging
➢ Apparent elevation diaphragm
➢ Ill-defined costophrenic angle
➢ Diaphragmatic spur
➢ Mobile fluid
➢ Displace gastric bubble
➢“Rock of Gibraltar” on lateral
Pleural Effusion and Ascites

• CT Features
➢ Effusion = outside of hemidiaphragm
➢ Ascites = inside of hemidiaphragm
Pleural Effusion: Connective Tissue Disease

• Rheumatoid arthritis
• Most common thoracic manifestation
• Middle aged males
• Antedates clinical disease
• Exudate / chyliform / low glucose
• Imaging
➢ Unilateral
➢ Chronic
➢ Transient / relapse
➢ Fibrothorax / decortication

Pleural Effusion: Asbestos Exposure
• Occupational exposure
• No malignancy within 3 yrs
• 10 yrs post-exposure
• Exudate
• 1/3 patients have chest pain
• Recurrent 15 - 30%
• Small (<500 ml)
Pleural Effusion - Asbestos Exposure

• Associated with diffuse pleural thickening
➢ Involves C-P angle
• Implicated in formation of Rounded Atelectasis
Round Atelectasis: CXR

• Peripheral mass
• Abuts thickened pleura
• 3.5 to 7 cm Figure 1-22-12
• Posterior lower lobe most common
➢ Other lobe, diaphragms
• Bronchovascular bundles converge, forming “comet
tail”
Round Atelectasis: CT
• Rounded subpleural mass
• Broadly abuts contiguous pleural thickening
• Air-bronchogram hilar aspect
• Bronchovascular “comet tail”
• Loss of volume in same lobe
Round Atelectasis: Required CT Findings

[Figure 1-22-12]
Chest CT (mediastinal and lung windows) demonstrates
• Subpleural mass
the CT findings of round atelectasis
• Thickened pleura
• Loss of volume
• Comet tail
Round Atelectasis: Pathogenesis

• Asbestos exposure
• Atelectasis
• Pleural adherence
• Effusion subsides
• Lung re-expands
• Pleural fibrosis
• Contraction
Pleural Fibrosis
• 2nd most common pleural abnormality
• Result of many primary diseases of the pleura
• Complication of inflammatory disease
• Most localized to single area
• Less often diffuse
➢ May have functional abnormalities
Pleural Fibrosis: Focal

• Healed Pleuritis
• Bacterial pleuritis/trauma
• Imaging
➢ Blunt posteriolateral CP sulci
➢ Rule-out small effusion

Pleural Fibrosis: Focal [Figure 1-22-13] Figure 1-22-13
• Pleural Plaques
• Serpentine (chrysotile) asbestos
• Dense hyalinized collagen
• Parietal pleural surface
• Asbestos exposure
• Asymptomatic
Pleural Fibrosis: Focal

• Pleural Plaques
• 50% of exposed individuals
• Visible plaques
➢ 15 years non-calcified
➢ 20 years calcified
Pleural Fibrosis: Imaging

• Bilateral (80%) Chest CT (lung window) demonstrates multiple
• Lateral chest wall bilateral pleural plaques
• 4th to 8th ribs
• Tendinous diaphragm
• Spares apices and CPAs
Figure 1-22-14
• En face “Holly leaf”
Pleural Fibrosis: Diffuse Fibrothorax Figure 1-22-14]

• Fibrous obliteration of normal pleural space
➢ Tuberculosis/bacterial empyema
➢ Hemothorax
➢ Asbestos-related pleural effusions
➢ Rheumatoid effusions
• Volume loss/restrictive disease
Pleural Fibrosis: Diffuse Fibrothorax

• Radiographic definition
➢ Smooth/uninterrupted
➢ 25% or more of chest wall
➢ May obliterate c-p suclus
➢ ≤ 2.0 cm thickness
➢ +/- calcification
Pleural Fibrosis: Diffuse Fibrothorax Pleural fibrosis. Chest radiograph demonstrates

• Imaging CT pleural thickening and calcification in the right
➢ Extends > 8.0 cm cranio-caudal hemithorax
➢ Pleura > 3 mm thick
➢ Extrapleural fat hypertrophy Figure 1-22-15
➢ +/- Pleural calcification
➢ Mediastinal pleura spared
Pneumothorax
• Air within the pleural space
• Spontaneous
➢ Primary
➢ Secondary
• Traumatic
Pneumothorax: Primary Spontaneous [Figure 1-22-15]

• M:F=5:1
• 3rd - 4th decade
• Right-side predominance
• 30% ipsilateral recurrence
• 10% contralateral recurrence Chest CT demonstrates left pneumothorax
• Rupture of apical bleb/bulla and a bleb along the visceral pleural
surface of the collapsed lung

Pneumothorax: Secondary Spontaneous
• COPD
• Most common concurrent condition
• 0.5% per year
• 45-65 years of age
• Peripheral emphysematous lung
• Mortality rate ~3%

• Pneumocystis Jiroveci Pneumonia (PCP)
• Destruction of alveolar septa → bulla
• Subpleural necrosis → cystic degeneration / bulla

• Pneumocystis Jiroveci Pneumonia (PCP)
• Complicates in 12%
• Refractory “air-leaks”
• Poor prognosis
• Death in 8 weeks (<57%)

• Lymphangioleiomyomatosis (LAM)
• Women child-bearing age
• Proliferation of immature smooth muscle
• Bronchiolar obstruction
➢ Cysts → PTX
• Recurrence ~40%

• Langerhans Cell Histiocytosis
• Smokers
• Cysts rupture
• Recurrent ptx (25%)

Pleural Disease II and Chest Wall
Malignant Pleural Effusion

• Most common manifestation of metastatic involvement
• Exudative effusion
• Lung Ca 36%
• Breast Ca 25%
• Lymphoma 10%
• Ovarian 5%
• Gastric Ca 2%
TNM Staging of Lung Cancer

• Malignant Effusion = T4
N0 N1 N2 N3
T1 IA IIA IIIA IIIB
T2 IB IIB IIIA IIIB
T3 IIB IIIA IIIA IIIB
T4 IIIB IIIB IIIB IIIB
MI=IV
Malignant Pleural Effusion:

Diagnosis and Prognosis
• Combined pleural cytology and pleural biopsy
• Multiple thoracenteses / pleural biopsies
• Poor prognosis
• Lung Carcinoma - mean survival 2 to 3 months
• Breast Carcinoma - mean survival 7 to 15 months
Pleural / Chest Wall Mass

• Discrepant margins on orthogonal views
• Elliptical shapes
• Obtuse angles
• Cross boundaries
Pleural Neoplasms
• Primary
• Localized fibrous tumor
• Malignant mesothelioma
• Secondary
• Pleural metastases
➢ Bronchogenic carcinoma
➢ Other carcinomas
➢ Lymphoma
➢ Invasive thymoma
Localized Fibrous Tumor

• Rare (< 5% of pleural neoplasms)
• Not related to asbestos
• M=F
• Mean age: 50 years
• Symptoms in 54%
• Cough, chest pain, dyspnea
• HPO 0 - 35%
• Hypoglycemia 4%
Chest Radiology 213 Pleural Disease II

Localized Fibrous Tumor: Microscopic Figure 1-23-1
• Variable patterns
• Disorderly arrangement
• Spindle cells and collagen
• High mitotic activity suggests malignancy 20%
Localized Fibrous Tumor: Gross

• 2 - 40 cm
• 80% visceral / 20% parietal
• Lobular, encapsulated
• Pedicle: good prognosis
• Cut-surface: whorled, nodular, fibrous hemorrhage, necrosis,
cysts
Localized Fibrous Tumor: CXR [Figures 1-23-1 to 1-23-3]

• Incidental finding
• Solitary rounded, lobular mass
• Mid to inferior thorax
• Obtuse or acute angles at interfaces Localized fibrous tumor. PA chest radiograph
• Pedunculated tumors demonstrates a pleural mass a peripheral
➢ Positional mobility mass in the right lower hemithorax with
➢ Pathognomonic incomplete borders
Localized fibrous tumor. Chest CT

(lung windows) demonstrates a
pleural mass that forms obtuse
Localized fibrous tumor. PA chest radiograph
angles at its interface with the chest
demonstrates a large mass that opacifies most of
wall
the right hemithorax and produces mass effect
with contralateral shift of the mediastinum
Localized Fibrous Tumor: CT [Figure 1-23-4] Figure 1-23-4
• Well-defined, smooth, lobular
• Abutting pleural surface
• Elongated, lenticular
• Heterogeneity:
➢ hemorrhage, necrosis, cysts
Localized fibrous tumor.

Contrast enhanced
chest CT demonstrates
a large heterogeneous
mass in the right lower
hemithorax with
lobulated contours
Pleural Disease II 214 Chest Radiology

Localized Fibrous Tumor
• Treatment of choice: complete excision
• 90% cure rate
• Symptoms usually resolve post-op
• Recur with tumor recurrence
• Recurrence in 10% of patients
Malignant Mesothelioma
• Most common primary pleural neoplasm
• 2,000 to 3,000 cases / year in USA
• 10% of exposed individuals
• Shipyards / asbestos plants
• Sixth to eighth decades
• Male : Female 3-6 : 1
• Amphiboles most tumorigenic
• Latency: 30-40 years
Malignant Mesothelioma: Clinical

• Insidious onset of symptoms
• 6-8 months prior to Dx
Figure 1-23-5
• Dyspnea, chest pain, cough, weight loss
• Rarely: SVC Syndrome, Horner Syndrome, dysphagia,
vocal cord paralysis, HPO, clubbing, hypoglycemia
Malignant Mesothelioma - Microscopic

• Epithelioid 50 %
• Sarcomatous 15 %
• Biphasic 25 %
• Interobserver agreement 50%
Malignant Mesothelioma: Gross

• Sheets, plaques, masses
• Parietal > Visceral
• Bulk in inferior hemithorax
• Lung encasement
• Fissural growth Malignant mesothelioma. PA chest
• Parenchymal involvement radiograph demonstrates circumferential,
• Mediastinal, chest wall, diaphragmatic invasion nodular and contiguous pleural masses
throughout the left hemithorax
Malignant Mesothelioma: Radiographic
[Figure 1-23-5]
Figure 1-23-6
• Pleural masses
• Circumferential
• Mediastinal shift
• Pleural plaques 25%
Malignant Mesothelioma: Radiographic

• Malignant mesothelioma cannot be reliably differentiated
from pleural metastases
Malignant Mesothelioma: CT [Figure 1-23-6]

• Pleural thickening 92%
• Fissural thickening 86%
• Pleural effusion 74%
• Ipsilateral ↓ volume 42%
• Pleural calcification 20% Chest CT demonstrates circumferential,
• Ipsilateral ↑ volume 14% nodular pleural thickening in the left
hemithorax that extends into the major
Kawashima AJR 1990
interlobar fissure. Calcified pleural plaques
are demonstrated bilaterally

Malignant Mesothelioma: DX
• Video-Assisted-Thoracoscopic-Surgery: Sensitivity 98%
➢ Complication: tumor seeding along entry ports
• Open biopsy: when adhesions preclude VATS
• Cytology and FNA Bx of limited value
Malignant Mesothelioma: MR
• Staging
• Comparable / superior to CT
• Tumor enhancement
• Increased signal intensity
Malignant Mesothelioma: Treatment and Prognosis

• Median survival: 10 months
• Best prognosis:
➢ 25-30% 5-year survival
➢ Negative margins
➢ Epithelial cell type
➢ No metastases
• Extrapleural pneumonectomy
➢ High mortality / morbidity
Pleural Metastases
Most common pleural neoplasm
• Common
➢ Adenocarcinoma
✧ Lung, breast, ovary, stomach
• Less common: Figure 1-23-7
➢ Lymphoma, Thymoma
• Imaging
➢ Pleural masses
➢ Or both
Pleural Metastases
• Hematogenous / Lymphatic
• Direct extension
➢ Lung ca, breast ca
• Drop metastases
➢ Invasive thymoma
• May be bilateral
Pleural Thickening
Malignant Pleural Thickening [Figure 1-23-7]

• Circumferential
• Nodular
• > 1 cm
• Mediastinal pleura
Leung et al AJR 1990 Pleural metastases. Chest CT demonstrates
adenocarcinoma arising from a right upper lobe
Chest Wall bronchus and pleural metastases that are nodular,
• Congenital and developmental anomalies circumferential, and involve the mediastinal pleura
• Inflammatory and infectious diseases
• Non-neoplastic conditions
• Neoplasia: benign and malignant

Chest Wall: Congenital / Developmental Anomalies Figure 1-23-8
[Figures 1-23-8 and 1-23-9]
• Pectus deformities
• Anomalous ribs
• Cleidocranial dysostosis
• Poland syndrome
Chest Wall: Inflammatory / Infectious Diseases

• Hematogenous
• Pyogenic infection: S. aureus, P. aeruginosa
• Imaging
➢ Osseous destruction if advanced
➢ CT / MR for better delineation
➢ CT for biopsy and/or drainage
Chest Wall:Tuberculosis [Figure 1-23-10]

• Uncommon
• Hematogenous spread Lateral chest radiograph
• Contiguous spread demonstrates pectus carinatum
• Abscess / sinus tract 25%
• Imaging Figure 1-23-9
➢ Bone / cartilage destruction
➢ Soft-tissue mass
➢ Calcification
➢ Peripheral enhancement
Chest Wall: Inflammatory / Infectious

Diseases
Chest Wall: Actinomycosis [Figure 1-23-11]

• Actinomyces israelii
• Anaerobic gram-positive
• Lung → Pleura → Chest Wall
• Proteolysis → Fistulas
• Diagnosis: anaerobic cultures
• Imaging: soft-tissue mass draining sinus, periostitis
Poland syndrome. Chest CT demonstrates
Figure 1-23-11 congenital absence of the right pectoralis muscles
Figure 1-23-10
Actinomycosis. Contrast enhanced chest CT Tuberculosis. Contrast enhanced chest CT

demonstrates peripheral consolidation in the left demonstrates an anterior chest wall
upper lobe with contiguous soft-tissue density abscess with subtle peripheral
extending into the adjacent mediastinum and enhancement and right pleural effusion
anterior chest wall and pleural thickening

Chest Wall: Neoplasms Figure 1-23-12
• Adults
• Benign:
➢ Lipoma
➢ Other mesenchymal neoplasms
• Malignant:
➢ Fibrosarcoma
➢ Malignant fibrous histiocytoma
➢ Other mesenchymal neoplasms
➢ Lymphoma
Chest Wall Neoplasms: Lipoma

• Common
• Subcutaneous
• Intrathoracic
• Both
• Diagnostic CT number Fibrosarcoma. Chest CT demonstrates post-
surgical changes of right mastectomy and a soft-
Chest Wall Neoplasms: tissue mass in the left chest wall. The patient
Desmoid (Fibromatosis) received radiation therapy through ports that
• Aggressive fibromatosis included the left posterolateral chest wall.
• Most: second to fourth decades
• Shoulder, chest wall
• Soft tissue mass
• Frequent recurrence
Chest Wall Neoplasms: Soft Tissue Involvement [Figure 1-23-12]

• Adults
• Malignant:
➢ Fibrosarcoma
➢ Malignant fibrous histiocytoma
Chest Wall Neoplasms: Osseous Involvement [Figure 1-23-13]

• Rib expansion
➢ Fibrous Dysplasia
➢ Enchondroma
• Pressure erosion
➢ Neurogenic (slow growth)
• Rib destruction:
➢ Metastatic or Primary
➢ Inflammatory Figure 1-23-13
Chest Wall Neoplasms: Osseous Destruction

• Adult
➢ Metastatic disease (Lung, Breast, Prostate, etc.)
➢ Multiple myeloma
➢ Chondrosarcoma
• Child
➢ Ewing sarcoma
➢ Neuroblastoma
➢ Lymphoma
➢ Askin tumor (PPNET)
Chest Wall Neoplasms: Myeloma

• Males > Females Fibrous dysplasia. Chest CT
• 5th - 7th decades demonstrates an expansile lesion
• Axial skeleton with intact cortical margins nvolving
• Multiple or solitary a right rib
• Imaging:
➢ Osseous destruction

Chest Wall Neoplasms: Chondrosarcoma
• Adults
• Painful, palpable mass
• Costochondral junction, rib, sternum
• Imaging:
➢ Expansile, destructive
➢ Chondroid calcification
PNET “Askin Tumor”: Primitive Neuroectodermal Tumor

• Malignant small round cell tumor
• Children, adolescents
• Female: Male = 3:1
• Unilateral
• Rib destruction 2/3
• Poor prognosis

Classic Breast Lesions
Leonard M. Glassman, MD
Figure 1-24-1
Most Lesions are Non-specific [Figure 1-24-1]
• Differentials can be given
• High likelihood diagnoses can be made
• Is this a cyst or a solid mass?
Normal Variants
• Pectoralis major
• Lymph nodes
Pectoralis Major [Figure 1-24-2]
Figure 1-24-2
CC view showing a
rounded density medially,
the medial end of the
Non specific lobulated mass which could be a
pectoralis major muscle
cyst, benign solid mass or a carcinoma
Figure 1-24-3
Sharply marginated medial density,

Sternalis [Figure 1-24-3] smaller than the pectoralis major,
is the sternalis muscle
Intramammary Lymph Nodes

• Normal finding
• 28% of breasts
• May enlarge and shrink in size
• Circumscribed with hilar notch or fatty hilum
• Usually less than 15 mm in size
• Not related to the usual lymphatic drainage patterns Figure 1-24-4
• Usually upper outer quadrant
Intramammary Lymph Nodes

Figures 1-24-4 to 1-24-6]
Left: Medial lymph node Right: Typical lymph

node with fatty hilum
Classic Breast Lesions 220 Chest Radiology
Left: Lymph node with hilar notch Six examples of benign lymph nodes.
Right: Core biopsy specimen of normal lymph The top three are with traditional real time scanning
node and the bottom three with compound scanning
Congenital Anomalies Figure 1-24-7

• Polythelia
➢ Accessory nipples
➢ 2.4% of neonates
• Polymastia
➢ 2-3% of women
➢ Axillary breast tissue most common
➢ Inframammary fold and labia next Figure 1-24-8
most common
Polythelia [Figures 1-24-7 and 1-24-8]
Polymastia [Figures 1-24-9 and 1-24-10]

• Can be palpable or visible
Benign Abnormalities Accessory nipple seen

• Fatty lesions medially
• Gynecomastia
• Fibrocystic changes
• Foreign bodies
Fatty Lesions
• Hamartoma Accessory nipple in a patient at
• Lipoma the inframammary crease
• Fat necrosis
• Galactocele Figure 1-24-9
All Lesions that Contain Fat are Benign Except

• Very rare hamartomas
• 1 Phyllodes with liposarcoma
Figure 1-24-10
Palpable axillary
accessory breast Visible bilateral axillary accessory
tissue breast tissue
Chest Radiology 221 Classic Breast Lesions

Hamartoma [Figures 1-24-11 to 1-24-14]
• Fibroadenolipoma
• Palpable mass or mammographic finding
➢ Can be large and not palpable
• Encapsulated normal breast elements Figure 1-24-14
Figure 1-24-11 Figure 1-24-12 Figure 1-24-13
Large hamartoma Small hamartoma Hamartoma showing

Hamartoma presenting
containing fat, glandular mixed echogenicity
as an intermediate
tissue and fibrous tissue
density mass without
visible fat
Lipoma [Figure 1-24-15] Figure 1-24-15

• Benign tumor
• Usually not palpable because it is soft
• Liposarcoma usually water density
Liposarcoma [Figure 1-24-16] Figure 1-24-16
Lipoma in the axillary

region
Left: Liposarcoma is water density, not fatty

Right: Water density liposarcoma
Fat Necrosis
• 50% have history of trauma
➢ Including surgery & XRT
• Oil cyst
• Partially calcified lesion
• Can be spiculated
• Can progress from fatty to spiculated
Oil Cyst [Figures 1-24-17 to 1-24-19]
Oil cyst Oil cyst with scarring Heavily calcified oil

and dystrophic cyst
calcification
Fat Necrosis - Progression [Figure 1-24-20] Figure 1-24-21
Figure 1-24-20
Left: Oil cyst with scarring

Right: Several years later the scarring
predominates and
the fatty part of the lesion disappears
Galactocele [Figure 1-24-21]

• Pregnant or breast feeding
• Cystic lesion Two galactoceles with layering of
• Fat fluid level on horizontal beam film milk
• Aspiration usually curative (fat rising and calcium dropping)
Fibrocystic Changes [Figures 1-24-22 and 1-24-23] Figure 1-24-22 Figure 1-24-23
• Exaggerated physiologic phenomenon
➢ Cysts
➢ Apocrine metaplasia and hyperplasia
➢ Stromal alterations
➢ Mild epithelial hyperplasia
➢ Mild adenosis
Apocrine Metaplasia [Figure 1-24-24]
Cyst [Figure 1-24-25]

• Cystic lobular involution Specimen showing
• Anechoic with sharp back wall Multiple small
multiple small cysts with hypoechoic cysts and
• Enhanced thru-transmission of sound a characteristic blue color
• 10% atypical textural irregularity of
(blue domed cysts) fibrocystic change
• Diagnosable on ultrasound or aspiration
Figure 1-24-26
Microcystic nodule of
apocrine metaplasia
Anechoic mass with sharp

borders and enhanced
Pneumocystography [Figure 1-24-26] transmission of sound, a
classic cyst
Pneumocystogram of a
benign cyst

Foreign Body Figure 1-24-27 Figure 1-24-28
• Silicone or paraffin
➢ Free injection
➢ Leakage from implants
• Surgical drain
• Wire fragments
Free Silicone (Implant Rupture)

[Figure 1-24-27]
Free Silicone or Paraffin [Figure 1-24-28]
Free Silicone [Figure 1-24-29]

Silicone in the tissue
from implant rupture Diffuse fibrosis and
Figure 1-24-29 calcification from
injected silicone or
paraffin for augmentation
Left: Multiple high density globules of injected

silicone for augmentation
Right: Low power view of “holes” in the tissue from
Wire fragment left
silicone
Penrose drain remaining behind after breast
after biopsy biopsy
Surgical Drain [Figure 1-24-30]
Wire Fragment [Figure 1-24-31]

Thickened Skin Pattern Figure 1-24-32
• Edema
• Mastitis
• Inflammatory carcinoma
• Post-radiation change
• Obstruction to lymphatic drainage in the axilla or
superior mediastinum
• Lymphoma
Thickened Skin Pattern – Mastitis

[Figure 1-24-32]
Prominent thickened skin pattern as compared to

the normal side

Thickened Skin Pattern – Radiation Therapy Figure 1-24-33
[Figure 1-24-33]
Inflammatory Carcinoma [Figure 1-24-34]

• Clinical findings
➢ Heavy firm breast
➢ Red skin
➢ Warm skin
➢ Peau d’orange
• Can not differentiate from acute mastitis
• Far advanced local disease
➢ Usually poorly differentiated ductal carcinoma
• Radiographic findings
➢ Obstruction of dermal lymphatics
✧ Can diagnose with a skin biopsy
➢ Diffuse lymphatic invasion within the breast
➢ Increased density
➢ Trabecular thickening
• 50% five year survival
➢ Pre-op chemo, mastectomy and radiation Extensive edema and
increased density after
Mammographic Findings radiation therapy
• Skin thickening
• Diffuse increased density
• Trabecular thickening Figure 1-24-34
• Adenopathy
• Signs of carcinoma
➢ Mass, calcification, asymmetry, distortion

• Axillary nodes
• Supraclavicular node
Figure 1-24-35
Far advanced inflammatory breast

cancer with skin necrosis
Left: Enlarged abnormal axillary nodes in

inflammatory breast cancer
Right: Abnormal supraclavicular node
Figure 1-24-36
Classically Benign Calcifications

• Lobular
• Sutural
• Fibroadenoma
• Skin
• Vascular
Left: Extremely dense breast in inflammatory
• Secretory
breast cancer. Right: Inflammatory breast cancer
• Lucent centered
with enlargement, and extensive skin thickening
• Egg shell

Lobular Calcifications [Figure 1-24-37] Figure 1-24-37
• Tightly clustered
• Round
• Fit together like a jigsaw puzzle
Sutural Calcifications [Figure 1-24-38]

• Look like sutures
• Usually post radiation therapy
Figure 1-24-38
Calcified Fibroadenoma
• Coarse or “popcorn-like”
• Calcification generally peripheral Tight cluster of smooth
calcifications, a benign
Peripheral Calcification lobular cluster
[Figure 1-24-39]
Figure 1-24-39
Dystrophic calcification with

calcified suture
Figure 1-24-40
Surface calcification in a
fibroadenoma
Calcified Fibroadenoma
[Figures 1-24-40 and 1-24-41]
Skin Calcifications
• Faint peripheral clusters with lucent centers
• Tangent view Left: Dense calcification (popcorn like) in a
fibroadenoma
Dermal Localization [Figure 1-24-42] Right: Sclerotic fibroadenoma with calcification
Figure 1-24-41
Figure 1-24-42
Left: Marker over cluster for dermal localization

Extremely dense calcification in a
Right: Tangential film showing cluster in skin
fibroadenoma

Vascular Calcifications Figure 1-24-43
• Parallel tracks associated with blood vessels
• Calcifications are on the outside of the tube
• Diabetes ?
• Heart Disease ?
Vascular / Ductal [Figure 1-24-43]
Secretory Calcifications
• Large rods
➢ Luminal calcifications
➢ Oriented toward nipple
➢ Relatively smooth surface Left: Calcification in artery
➢ May branch Right: Calcification in duct
Loa Loa [Figure 1-24-44 Figure 1-24-44 Figure 1-24-45
• Also called eye worm
• Found in rain forest and swamps of West
Africa, especially Cameroon
• Transmitted by day biting Chrysops flies
• Loa loa filarial nematode
• Larvae develop over 1 year
• Mature worms up to 3-6 cm x 0.5 cm
Osteosarcoma
• Primary in the breast
• 27 to 89 years old
• Median 64.5 years
• Highly aggressive tumors
Calcified loa loa worm
Primary Osteosarcoma [Figure 1-24-45]
Mass with dense osteoid
National Flower of the Radiologist is the Hedge matrix
Some Diagnoses Can be Made

• Make them when you can
References
1. Adler DD, Jeffries DO, Helvie MA. Sonographic features of breast hamartomas. J Ultrasound Med 1990; 9:85-90.
2. Ahern V, Brennan M, Ung O, Kefford R. Locally advanced and inflammatory breast cancer. Aust Fam Physician
2005; 34:1027-1032.
3. Bassett LW, Gold RH, Cove HC. Mammographic spectrum of traumatic fat necrosis: the fallibility of
"pathognomonic" signs of carcinoma. AJR Am J Roentgenol 1978; 130:119-122.
4. Berg WA, Campassi CI, Ioffe OB. Cystic lesions of the breast: sonographic-pathologic correlation. Radiology
2003; 227:183-191.
5. Bosch X. Unique features of inflammatory breast carcinoma. Lancet Oncol 2005; 6:549.
6. Dershaw DD, Moore MP, Liberman L, Deutch BM. Inflammatory breast carcinoma: mammographic findings.
Radiology 1994; 190:831-834.
7. Ellis DL, Teitelbaum SL. Inflammatory carcinoma of the breast. A pathologic definition. Cancer 1974; 33:1045-
1047.
8. Erguvan-Dogan B, Yang WT. Direct injection of paraffin into the breast: mammographic, sonographic, and MRI
features of early complications. AJR Am J Roentgenol 2006; 186:888-894.
9. Ganott MA, Harris KM, Ilkhanipour ZS, Costa-Greco MA. Augmentation mammoplasty: normal and abnormal
findings with mammography and US. Radiographics 1992; 12:281-295.
10. Hance KW, Anderson WF, Devesa SS, Young HA, Levine PH. Trends in inflammatory breast carcinoma incidence
and survival: the surveillance, epidemiology, and end results program at the National Cancer Institute. J Natl
Cancer Inst 2005; 97:966-975.
11. Helvie MA, Adler DD, Rebner M, Oberman HA. Breast hamartomas: variable mammographic appearance.
Radiology 1989; 170:417-421.

12. Hogge JP, Robinson RE, Magnant CM, Zuurbier RA. The mammographic spectrum of fat necrosis of the breast.
Radiographics 1995; 15:1347-1356.
13. Kushwaha AC, O'Toole M, Sneige N, Stelling CB, Dryden MJ. Mammographic-pathologic correlation of apocrine
metaplasia diagnosed using vacuum-assisted stereotactic core-needle biopsy: our 4-year experience. AJR Am J
Roentgenol 2003; 180:795-798.
14. Levitan LH, Witten DM, Harrison EG, Jr. Calcification In Breast Disease Mammographic-Pathologic Correlation.
Am J Roentgenol Radium Ther Nucl Med 1964; 92:29-39.
15. Mester J, Simmons RM, Vazquez MF, Rosenblatt R. In situ and infiltrating ductal carcinoma arising in a breast
hamartoma. AJR Am J Roentgenol 2000; 175:64-66.
16. Sheppard DG, Whitman GJ, Huynh PT, Sahin AA, Fornage BD, Stelling CB. Tubular carcinoma of the breast:
mammographic and sonographic features. AJR Am J Roentgenol 2000; 174:253-257.
17. Soo MS, Kornguth PJ, Hertzberg BS. Fat necrosis in the breast: sonographic features. Radiology 1998; 206:261-
269.
18. Stacey-Clear A, McCarthy KA, Hall DA, et al. Calcified suture material in the breast after radiation therapy.
Radiology 1992; 183:207-208.
19. Svane G, Franzen S. Radiologic appearance of nonpalpable intramammary lymph nodes. Acta Radiol 1993;
34:577-580.

Basic Breast Imaging
There are Two Diseases

• Cancer and no cancer Figure 1-25-1
Cancer has Two Predominant Signs
• Mass and calcification
You Have the Opposite Side for Comparison

• Anatomy is simple
• Physiology is almost irrelevant
What You Need to Remember

• 90% of cancers present as calcification, mass or both
• 10% present as
➢ Focal asymmetry, developing or neodensities
➢ Dilated duct
(left) Ductogram showing normal ducts;
➢ Thickened skin pattern
(right) : Diffuse tubular parenchymal pattern
➢ Architectural distortion
caused by periductal fibrosis not ductal
➢ Paget’s disease
dilatation
Rad Path Correlation: What You Need to Remember
• The mass edge interface with the surrounding tissue reflects the
aggressiveness of the underlying lesion
• Benign masses tend to be less aggressive than malignant masses
• The shape of the calcification represents a cast of an underling anatomic or
pathologic space
• Benign processes often cause smooth spaces
• Necrosis (benign or malignant) causes irregular spaces
Figure 1-25-2
Normal Anatomy
• Skin
• Nipple and areola
• Subcutaneous fat
• Premammary fascia
Normal Anatomy
• Glandular cone
➢ Breast disease occurs here
• Retromammary fascia
• Retromammary fat (left) Normal ductal distribution from nipple to
• Muscle lobules;
• Ribs (right) : Normal duct with single cell epithelial
layer and clear lumen
Segmental Anatomy
• 15 - 20 lobes or segments
Figure 1-25-3
Normal Ducts [Figures 1-25-1 and 1-25-2]
Terminal Duct Lobular Unit (TDLU) [Figure 1-25-3]

• Short segment of terminal duct and a cluster of
ductules (acini)
• Functional unit of the breast
• Ductal and lobular cancers begin here
• Explains mixed ductal & lobular features in the same
neoplastic lesion
(left) Drawing of TDLU
(right) Microscopic view of TDLU
Chest Radiology 229 Basic Breast Imaging

Embryology
• Milk ridges
➢ Ventral ectodermal thickenings from the axillary to the inguinal region
➢ Usually limited to the pectoral regions by the ninth week of embryonic life
Congenital Anomalies
• Athelia
➢ Rarest anomaly of the breast
➢ Absence of the nipple
• Amastia
➢ Agenesis of breast & nipple
➢ Associated with hypoplasia of the ipsilateral pectoral muscles in 90%
➢ Can be iatrogenic Figure 1-25-4
• Polythelia
• Polymastia
Pregnancy Changes [Figure 1-25-4]

• Increased estrogen & progesterone
➢ Estrogen promotes ductal growth
➢ Progesterone promotes lobular growth and breast secretion
• Hyperplasia and hypertrophy
• Extremely dense breast pattern
• Can still see calcifications on mammography
• Can see masses on sonography
Mastitis
• 3% of primary diagnoses at biopsy
• Many different types Very dense parenchyma in a
➢ Infection normal pregnant patient
➢ Systemic
➢ Antigen-antibody reaction Figure 1-25-5
➢ Idiopathic
Mastitis [Figures 1-25-5 and 1-25-6]

• Acute mastitis
➢ Usually in lactating women with a cracked nipple
➢ Can go on to abscess
• Chronic mastitis
Chronic Mastitis
• Chronic infection
➢ TB Focal parenchymal
➢ Fungus density from acute
• Immunologic bacterial mastitis
➢ Diabetes
➢ Amyloid
➢ Wegener granulomatosis Figure 1-25-6
➢ Sarcoid
➢ Churg – Strauss
• Idiopathic
➢ Necrobiotic xanthogranulomatosis
➢ Granulomatous mastitis
Most Common Benign Neoplasms

• Fibroadenoma (left) Early abscess with accumulation of pus in
➢ Biphasic tumor small spaces.
• Intraductal papilloma (right) Later stage with larger area of pus
• Hamartoma
Basic Breast Imaging 230 Chest Radiology

Biphasic Tumors Figure 1-25-7
• Epithelial & stromal elements
➢ Fibroadenoma
❖ Benign epithelial and stromal elements
➢ Phyllodes tumor
❖ Benign epithelial & hyperplastic or
sarcomatous stroma
➢ Carcinosarcoma
❖ Both elements malignant
Fibroadenoma [Figure 1-25-7]

• Begins in TDLU (left) Circumscribed mass (fibroadenoma) showing
• Response to unopposed estrogen in young women sharp border since there is no tissue invasion.
• Oval or round circumscribed nodule (right) Fibroadenoma showing surface devoid of
• May have coarse calcification, especially in periphery adherent surrounding tissue
• Growth pushes surrounding tissue without invasion
• 7-16% of patients have multiple tumors Figure 1-25-9
• Polyclonal cell population
➢ Begins as local fibroadenomatiod change which coalesces into a
fibroadenoma
Figure 1-25-8
Fibroadenoma [Figures 1-25-8 to 1-25-10]
• Fibroadenoma
• Carcinoma
Carcinoma Arising in a
Fibroadenoma
• Rare
• Most often lobular neoplasia (LCIS) or (left) Fibroadenoma showing pushing
DCIS but not invasion of surrounding tissue. Fibroadenoma showing
• Invasive carcinoma very rare (right) Invasive carcinoma without the sharp borders and
➢ Usually grows in from outside sharp border because of invasion, not enhanced thru transmission
pushing. of sound
Fibroadenoma Phyllodes
Phyllodes Tumor [Figures 1-25-11 and 1-25-12]

• Benign epithelial elements and cellular spindle cell stroma
Figure 1-25-10
• Can act malignant
➢ Local recurrence
➢ Distant blood born metastases
➢ Lymph node enlargement reactive usually
• Well circumscribed lobulated mass
• Similar appearance on sonography
➢ May have cystic spaces
Figure 1-25-11
(left) Densely calcified fibroadenomas.

(right) Irregular calcification and ill defined density
from degenerated fibroadenoma
(left) Phyllodes tumor as a

macrolobulated
circumscribed mass similar
to a fibroadenoma.
(right) Phyllodes tumor
similar to a fibroadenoma
except note the small cystic
clefts.

(left) Mass with nonvascular cystic clefts in this

Phyllodes tumor.
Ill defined subareolar
(right) Phyllodes tumor showing clefts on low
and UOQ mass in this
power microscopy.
carcinosarcoma
Figure 1-25-14
Carcinosarcoma [Figure 1-25-13]
Papilloma [Figures 1-25-14 to 1-25-16]

• Papillary growth pattern supported by a fibrovascular
stalk
➢ Arises centrally
➢ Usually solitary
• Papillomatosis
➢ Arises peripherally in the TDLU
➢ Usually multiple
Figure 1-25-15 (left) Filling defect in duct from papilloma

(right) Specimen of papilloma in a dilated duct.
Figure 1-25-16
(left) Unusual presentation of a papilloma as a

discrete mass.
(right) Filling defect in a dilated duct from a
papilloma (left) Lobulated mass representing a dilated duct.
(right) Dilated duct with papilloma showing
prominent blood flow
Lobular Neoplasia
• No mammographic signs usually
• Incidental finding on biopsy
• Includes atypical lobular hyperplasia and LCIS
Lobular Neoplasia
• High incidence of bilaterality and multifocality
➢ Consider it a bilateral disease
• High risk marker for the development of invasive carcinoma
➢ Up to 15% in either breast equally within 20 years
➢ Lobular or ductal features
• If found on core biopsy 19% upgraded to carcinoma on excision
➢ Usually LN2 or LN3
• Pleomorphic and florid LCIS (subset of LN3) diagnosis carries highest risk

➢ Pleomorphic type causes necrosis and can present as irregular Figure 1-25-17
calcifications
Invasive Ductal Cancer [Figures 1-25-17 to 1-25-25]

• NOS (not otherwise specified)
➢ 50 to 75% of invasive cancers
• Medullary
• Papillary Figure 1-25-18
• Colloid (Mucinous)
• Tubular
• Metaplastic
• Cribriform Diffusely infiltrative invasive
• Adenoid cystic ductal carcinoma
• Paget's disease
• Inflammatory
(left) Spiculated mass in this invasive ductal

carcinoma
(right) Specimen of spiculated invasive ductal
carcinoma
(left) Irregular calcifications in invasive ductal (left) Irregular calcification and spiculation in this
carcinoma invasive ductal carcinoma
(right) Specimen showing irregular (right) Specimen of this case showing irregular
calcifications in irregular ductal lumens in this calcifications in irregular lumens and necrotic
invasive ductal carcinoma tissue spaces
(left) Mass with indistinct borders in invasive ductal

MR showing enhancing carcinoma
mass in invasive ductal (right) Spiculated mass in this invasive ductal
carcinoma carcinoma

Irregular shaped mass with

lobulations and an acute angle on (left) Invasive ductal carcinoma presenting as a
the right side in this invasive thick walled cyst (the “cyst” is central necrosis)
ductal carcinoma (right) Dense shadowing by invasive ductal
carcinoma
Figure 1-25-25
Paget’s Disease [Figures 1-25-26 and 1-25-27]
• Red nipple and areola
• Scaling eczematoid reaction
• 50% have a palpable mass
• Must have Paget’s cells in skin
• Usually has underlying carcinoma
Figure 1-25-26
Many irregular calcifications in this

invasive ductal carcinoma
Figure 1-25-27
(left) Paget’s disease with red moist nipple

(right) Paget’s disease with dry scaly nipple
Types of Invasive Ductal Carcinoma With

Rounded Expansile Periphery
• Medullary
• Papillary
• Cribriform
• Colloid
(left) Invasive ductal carcinoma calcifications
behind nipple in Paget's disease
Types of Invasive Ductal Carcinoma with (right) Specimen confirming calcifications in
Improved Prognosis invasive ductal carcinoma. Paget’s cells in skin
• Medullary
• Papillary
• Cribriform Figure 1-25-28
• Colloid
• Tubular
• Adenoid cystic
Medullary Carcinoma [Figure 1-25-28]
(left) Medullary carcinoma with slightly more

indistinct borders than fibroadenoma
(right) Minimal invasion of surrounding tissue
(bottom right) by medullary carcinoma

Papillary Carcinoma [Figure 1-25-29] Figure 1-25-29
Tubular Carcinoma [Figures 1-25-30 and 1-25-31]

• Typically spiculated
• Must have 75 - 100% tubular formation
• Less than 75% acts like usual invasive carcinoma
Figure 1-25-30
Circumscribed
macrolobulated mass
was a papillary
carcinoma
Figure 1-25-31
Very spiculated mass is typical

tubular carcinoma
Adenoid Cystic Carcinoma

[Figure 1-25-32] (left) Tubule formation in tubular carcinoma
(right) Spiculations in tubular carcinoma
Invasive Lobular Cancer
• Prognosis similar to invasive ductal cancer Figure 1-25-32
• Most commonly a spiculated mass
• Some are more difficult to see as they are diffusely infiltrating
➢ Present as asymmetric density
Invasive Lobular Cancer [Figures 1-25-33 to 1-25-35]

• Invasive lobular
• Invasive ductal
Figure 1-25-33
Figure 1-25-34
Typical adenoid cystic

carcinoma with
lobulations but no
spiculation
(left) Tumor cells of invasive lobular carcinoma in
single file
(right) Tumor cells of invasive ductal carcinoma in
enlarged thick walled ducts
Spiculkated mass is the
most common presentation
of invasive lobular
carcinoma

Sarcoma Figure 1-25-35
• 1% of breast malignant tumors
• Breast contains fat, fibrous tissue, blood vessels, etc.
➢ Angiosarcoma, malignant fibrous hystiocytoma, chondrosarcoma,
rhabdomyosarcoma etc.
• Metaplasia can occur
• Malignant transformation can occur
• Often after chest or breast irradiation
Fibrosarcoma [Figure 1-25-36]
Spindle Cell Sarcoma [Figure 1-25-37]
Angiosarcoma [Figure 1-25-38]
What You Need to Remember

• The mass edge represents the aggressiveness of the underlying
abnormality Focal asymmetric density in
• The shape of the calcification represents a cast of an underlying space invasive lobular carcinoma
often causes misdiagnosis
Figure 1-25-36
Figure 1-25-37
(left) Non specific mass in fibrosarcoma.

(right) Specimen of fibrosarcoma
(left) Irregular non specific mass in spindle cell
Figure 1-25-38 sarcoma.
(right) Specimen of spindle cell sarcoma
Angiosarcoma

References
1. Alleva DQ, Smetherman DH, Farr GH, Jr., Cederbom GJ. Radial scar of the breast: radiologic-pathologic correlation
in 22 cases. Radiographics 1999; 19 Spec No:S27-35; discussion S36-37.
2. Bartella L, Liberman L, Morris EA, Dershaw DD. Nonpalpable mammographically occult invasive breast cancers
detected by MRI. AJR Am J Roentgenol 2006; 186:865-870.
3. Bassett LW. Imaging of breast masses. Radiol Clin North Am 2000; 38:669-691.
4. Chao TC, Lo YF, Chen SC, Chen MF. Phyllodes tumors of the breast. Eur Radiol 2003; 13:88-93.
5. Chen SC, Cheung YC, Su CH, Chen MF, Hwang TL, Hsueh S. Analysis of sonographic features for the differentiation
of benign and malignant breast tumors of different sizes. Ultrasound Obstet Gynecol 2004; 23:188-193.
6. Espinosa LA, Daniel BL, Vidarsson L, Zakhour M, Ikeda DM, Herfkens RJ. The lactating breast: contrast-enhanced
MR imaging of normal tissue and cancer. Radiology 2005; 237:429-436.
7. Feder JM, de Paredes ES, Hogge JP, Wilken JJ. Unusual breast lesions: radiologic-pathologic correlation. Radiographics
1999; 19 Spec No:S11-26.
8. Goel NB, Knight TE, Pandey S, Riddick-Young M, de Paredes ES, Trivedi A. Fibrous lesions of the breast: imaging-
pathologic correlation. Radiographics 2005; 25:1547-1559.
9. Jackson VP, Bassett LW. Breast sonography. Breast Dis 1998; 10:55-66.
10. Kolb TM, Lichy J, Newhouse JH. Comparison of the performance of screening mammography, physical examination,
and breast US and evaluation of factors that influence them: an analysis of 27,825 patient evaluations. Radiology
2002; 225:165-175.
11. Kriege M, Brekelmans CT, Boetes C, et al. Efficacy of MRI and mammography for breast-cancer screening in women
with a familial or genetic predisposition. N Engl J Med 2004; 351:427-437.
12. Lehman CD, Blume JD, Weatherall P, et al. Screening women at high risk for breast cancer with mammography and
magnetic resonance imaging. Cancer 2005; 103:1898-1905.
13. Liberman L, Drotman M, Morris EA, et al. Imaging-histologic discordance at percutaneous breast biopsy. Cancer
2000; 89:2538-2546.
14. Liberman L, Mason G, Morris EA, Dershaw DD. Does size matter? Positive predictive value of MRI-detected breast
lesions as a function of lesion size. AJR Am J Roentgenol 2006; 186:426-430.
15. Mercado CL, Hamele-Bena D, Oken SM, Singer CI, Cangiarella J. Papillary lesions of the breast at percutaneous
core-needle biopsy. Radiology 2006; 238:801-808.
16. Park JM, Han BK, Moon WK, Choe YH, Ahn SH, Gong G. Metaplastic carcinoma of the breast: mammographic and
sonographic findings. J Clin Ultrasound 2000; 28:179-186.
17. Pisano ED, Parham CA. Digital mammography, sestamibi breast scintigraphy, and positron emission tomography
breast imaging. Radiol Clin North Am 2000; 38:861-869.
18. Sabate JM, Clotet M, Gomez A, De Las Heras P, Torrubia S, Salinas T. Radiologic evaluation of uncommon
inflammatory and reactive breast disorders. Radiographics 2005; 25:411-424.
19. Samardar P, de Paredes ES, Grimes MM, Wilson JD. Focal asymmetric densities seen at mammography: US and
20. Schnall MD, Blume J, Bluemke DA, et al. Diagnostic architectural and dynamic features at breast MR imaging:
multicenter study. Radiology 2006; 238:42-53.
21. Sewell CW. Pathology of benign and malignant breast disorders. Radiol Clin North Am 1995; 33:1067-1080.
22. Sheppard DG, Whitman GJ, Huynh PT, Sahin AA, Fornage BD, Stelling CB. Tubular carcinoma of the breast:
mammographic and sonographic features. AJR Am J Roentgenol 2000; 174:253-257.
23. Shetty MK, Shah YP, Sharman RS. Prospective evaluation of the value of combined mammographic and sonographic
assessment in patients with palpable abnormalities of the breast. J Ultrasound Med 2003; 22:263-268; quiz 269-270.
24. Sickles EA. Management of probably benign breast lesions. Radiol Clin North Am 1995; 33:1123-1130.
25. Sickles EA. Probably benign breast lesions: when should follow-up be recommended and what is the optimal follow-
up protocol? Radiology 1999; 213:11-14.
26. Slawson SH, Johnson BA. Ductography: how to and what if? Radiographics 2001; 21:133-150.
27. Soo MS, Williford ME, Walsh R, Bentley RC, Kornguth PJ. Papillary carcinoma of the breast: imaging findings. AJR
1995; 164:321-326.
28. Stavros AT, Thickman D, Rapp CL, Dennis MA, Parker SH, Sisney GA. Solid breast nodules: use of sonography to
distinguish between benign and malignant lesions. Radiology 1995; 196:123-134.
29. Stavros T, Rapp CL, Parker SH. Sonography of mammary implants. Ultrasound Q 2004; 20:217-260.
30. van den Bosch MA, Daniel BL, Mariano MN, et al. Magnetic resonance imaging characteristics of fibrocystic change
of the breast. Invest Radiol 2005; 40:436-441

Ductal Carcinoma in Situ (DCIS)
Ductal Carcinoma in Situ (DCIS)

• Also called intraductal carcinoma
➢ Not invasive ductal carcinoma
➢ DCIS is “benign”
❖ Disease is confined to the breast
Figure 1-26-1
❖ Patients die from metastatic disease
secondary to invasive carcinoma
Relative Risk of Invasive Carcinoma

(Biopsy Findings)
• Mild intraductal hyperplasia (IDH)
➢ No increased risk
• Moderate or florid IDH
➢ Slight increased risk
• Atypical hyperplasia (ductal or lobular) with no family
history and/or postmenopausal
➢ Mild to moderate increased risk
• Atypical hyperplasia (ductal or lobular) with positive
family history and/or premenopausal
➢ High risk
• Ductal carcinoma in situ (left) Thickened duct walls with necrosis and comedo
➢ High risk secretion in the lumen.
• Lobular carcinoma in situ (top right) Stretching and thinning if duct lumen by early
➢ High risk DCIS
(bottom right) Dilated duct with wall irregularity in DCIS
Confined to the Duct [Figure 1-26-1]
• No spread to blood or lymph nodes
• Less than 1% positive axillary nodes
➢ Probably unrecognized invasion
➢ Most likely in large lesions and palpable lesions
The Problems
• How big is the problem?
• How do we classify the disease?
• How do we diagnose it?
• What is adequate treatment?
How Big is the Problem?

• USA data
➢ 50% eligible women get screening annually after 40
➢ 240,000 new breast cancers annually
❖ Includes invasive and intraductal
• 1980 5% of new breast cancers were DCIS
➢ Usually a palpable lump or nipple discharge
• 2000 20% of new breast cancers
➢ Usually microcalcifications on mammography
• Age-adjusted incidence increasing
➢ 2.4 per 100,000 in 1973
➢ 15.8 per 100,000 in 1992
Is It Malignant?
• 30 to 60% of underdiagnosed DCIS becomes invasive cancer within 25 years
• Usually in the same breast and near the biopsy site
Ductal Carcinoma in Situ 238 Chest Radiology

Invasive Cancer after DCIS
• NASBP B17 (790 women) at 5 years
➢ Women treated with breast conservation
➢ 115 recurrent cancers
❖ 80% same breast
❖ 15.7% opposite breast
❖ 4.3% regional or distant metastases
➢ 12.1% recurrence with lumpectomy + radiation (36% invasive)
➢ 26.8% recurrence with lumpectomy alone (51% invasive)
Indicators of Recurrence after Conservative Treatment

• Tumor size
• Nuclear grade
• Necrosis
• Margin status
• Multifocality
• Lymphocytic infiltrate
Epidemiology
• Same risks as for invasive cancer
➢ Increasing age
➢ Early menarche
➢ Family history
➢ Previous breast biopsy
➢ Nulliparity or late age at first birth
Pathologic Classification of DCIS

• No uniform agreement on single scheme
• Interobserver agreement between schemes is poor
Classifications of DCIS
• DIN
• European Commission Working Group
• Lagios
• Modified Lagios
• Nottingham
• UKNBCSP
• Van Nuys
Architectural Classification of DCIS

• Comedo
➢ Needs comedonecrosis and high nuclear grade
• Non-comedo
➢ Cribriform
➢ Micropapillary
➢ Papillary
➢ Solid
• Special type
➢ Apocrine
➢ Clear cell
➢ Signet ring cell
➢ Small cell
➢ Endocrine
➢ Spindle cell
Intraductal Carcinoma (DCIS)

• Histology may be able to predict recurrence risk
➢ High grade, large cell, comedo have higher recurrence
➢ Low grade, small cell, noncomedo (cribriform, micropapillary)
• Poor correlation of calcification type and extent with grade and extent of tumor
Chest Radiology 239 Ductal Carcinoma in Situ

Is DCIS One Disease?
Low Grade High Grade
• ER +++ +
• PR +++ +
• HER-2/neu + +++
• p53 + ++
• bcl-2 + –
Is DCIS One Disease? Figure 1-26-2

• Associated invasive carcinoma shows marker phenotype like precursor DCIS
• Low grade DCIS yields low grade invasive tumors
• High grade DCIS yields high grade invasive tumors
• Theory: Low grade and high grade DCIS are different from the start
Diagnosis of DCIS
• Mass
• Mammographic calcifications
➢ Can’t distinguish from invasive carcinoma
➢ Associated mass usually invasive disease
Mass
• Rare today
• Usually small
Small indistinct mass
DCIS Mass Close-up [Figure 1-26-2] represents DCIS
DCIS Mass [Figures 1-26-3 to 1-26-5] Figure 1-26-3
Intraductal Carcinoma (DCIS)

• Microcalcification usually without mass
• Particles < 1 mm
• Varying size shape and density
• Clustered
• May coexist with benign calcifications
Calcification
• Size (left) Large obscured mass was entirely DCIS.
• Number (right) Small vertically oriented solid mass
• Distribution represents DCIS
• Shape
• Change over time Figure 1-26-5
Size of Particles
• < 1 mm
➢ Evaluate malignant potential by smallest particles in the
abnormality
Number
• Cluster is 5 particles or more in 1 cubic cm.
Figure 1-26-4
Irregular filling defect in duct represents

DCIS
MRI with large area of enhancement was DCIS on
biopsy

Distribution [Figure 1-26-6] Figure 1-26-6
• Cluster
• Linear
➢ DCIS involves a duct
➢ Linear distribution toward nipple
➢ High grade is continuous involvement
➢ Low grade has skip areas
• Segmental
➢ Involvement of an entire ductal system
Segmental Intraductal Carcinoma

[Figure 1-26-7]
(left) Cluster of irregular calcifications was DCIS
Shape is Most Important on biopsy
• Irregular (right) Broken rod shaped calcification in a linear
➢ Not smooth round or hollow distribution represents DCIS
• Heterogeneous or pleomorphic
➢ Not all the same
Figure 1-26-7
Pleomorphic [Figure 1-26-8]
Calcifications represent the caste of a space

• Irregular duct
• Necrotic tissue space
Intraductal Carcinoma [Figure 1-26-9]
Figure 1-26-9
Segmental distribution of
granular calcifications was
DCIS
Left: Small calcifications filling the residual lumen Figure 1-26-8

made irregular by wall thickening of DCIS
Right: Duct calcification shape is related to the
contour of the duct wall
Shapes of DCIS Calcification

• Granular
• Irregular rods
• Casting
• Irregular
➢ Branching
➢ Comma shaped
➢ Arrow shaped or pointed
Multiple irregular calcifications in
biopsy proven DCIS

Granular [Figure 1-26-10] Figure 1-26-10
• Very small (<0.5 mm)
• Need magnifying glass to evaluate
• Too small to see true shape
• “Grains of sand”
Irregular Rods
• Made up of many tiny pieces on magnification
• Not solid large rods
➢ Secretory disease
• Often branch
Rods [Figure 1-26-11] Extremely small calcifications

• Regular represent a finding of DCIS
• Irregular
Casting
• Granular calcifications filling the lumen of an irregular duct Figure 1-26-11
• Often branch
Irregular [Figure 1-26-12]

• Castes of necrotic spaces
• Branching
• Comma shaped
• Arrow shaped or pointed
Figure 1-26-12 (left) Smooth rods of secretory disease

(right) Broken rods of DCIS
Figure 1-26-13
(left) Diffuse microcalcifications of DCIS.

(right) Ultrasound shows calcifications and
parenchymal change in DCIS
Small irregular clustered calcifications were

biopsied yielding DCIS Figure 1-26-14
Intraductal Carcinoma [Figures 1-26-13 to 1-26-15]

Figure 1-26-15
(left) Clustered irregular calcifications.

(right) Calcification in necrotic wall of duct involved Diffuse amorphous
with DCIS calcifications of DCIS

Change Over Time
• Benign processes can change
• Malignant processes almost always change within 3 years
• Short interval follow-up
➢ Probably benign findings
➢ 6 months unilateral, annual bilateral for 3 years
❖ No scientific basis
Biopsy
• Imaging guided biopsy with specimen radiography
➢ Usually stereotactic
• Wire guided excision with specimen radiography
Pathologic Findings Needed in Any Report

• Nuclear grade
➢ Low, intermediate or high
• Necrosis
➢ Comedo or punctate
• Architectural pattern
• Lesion size
• Margin assessment
• Specimen processing
➢ Report should include
❖ Presence of calcification
❖ Correlation with specimen radiograph and/or mammogram
Extent of Calcification Does Not Correspond

to the Extent of the Tumor
• Best correlation is with comedo type but not good enough
➢ Poor correlation with cribriform and micropapillary
• Must use histologic margins to define true extent
Treatment
• Simple mastectomy without axillary dissection
➢ 25% of patients choose this option
➢ Large lesions in small breasts
➢ Multiple lesions
➢ No radiation
❖ Unavailability
❖ Prior radiation
❖ Collagen vascular disease
➢ Patient preference
• Reconstruction

Breast Conservation
• Wide local excision without axillary dissection
➢ Sentinel node when large or palpable lesions
• Post -excision mammogram with magnification views of biopsy site
➢ May be done 2 or 3 months after excision
➢ Not necessary if specimen radiograph shows complete excision with
10 mm margin
• Radiation is standard
➢ Helps in all patients
➢ Benefit may be small in a subset of patients
❖ Small lesions
❖ Low grade histology
❖ Wide clear margins
• Local excision alone without radiation
➢ Controversial
➢ Less than 2 - 3 cm lesion
➢ Margins should be 10 mm or greater
➢ Nuclear grade low or intermediate
• Recurrence risk 1% per year
What is a Clear Margin?

• Relative risk of recurrence after excision and radiation
➢ 10 mm or greater 1.14
➢ 1 – 9 mm 1.49
➢ <1 mm 2.54
Radiation Therapy
• 1.8 to 2.0 Gy fractions Monday through Friday
• 45 to 50 Gy total dose
• Boost 10 to 20 Gy to surgical bed
• No axillary radiation
Chemotherapy
• No cytotoxic drugs
• Tamoxifen 20 mg daily for 5 years
➢ Newer drugs possible with fewer side effects
➢ Decreases invasive recurrences
➢ No change in survival
❖ Survival is over 90% without chemotherapy
Treatment of Recurrence
• DCIS
➢ Mastectomy if radiation given previously
➢ Mastectomy or wide excision with radiation
• Invasive carcinoma
➢ Treat like any invasive cancer
➢ Can not give radiation twice
Follow-up
• Lifetime
• Annual mammography
➢ First exam 6 months after completion of treatment
➢ Every 6 months for the first two years?
➢ Use of magnification views common
❖ Most common in first exam after treatment
Summary
• DCIS is carcinoma without the ability to spread YET
• It is detected on mammography as calcification
• Adequate detection and treatment decreases the incidence of invasive cancer
and therefore death

References
1. Consensus Conference on the classification of ductal carcinoma in situ. The Consensus Conference Committee.
Cancer 1997; 80:1798-1802.
2. Cornfield DB, Palazzo JP, Schwartz GF, et al. The prognostic significance of multiple morphologic features and
biologic markers in ductal carcinoma in situ of the breast: a study of a large cohort of patients treated with surgery
alone. Cancer 2004; 100:2317-2327.
3. Hashimoto BE, Kramer DJ, Picozzi VJ. High detection rate of breast ductal carcinoma in situ calcifications on
mammographically directed high-resolution sonography. J Ultrasound Med 2001; 20:501-508.
4. Hermann G, Keller RJ, Drossman S, et al. Mammographic pattern of microcalcifications in the preoperative diagnosis
of comedo ductal carcinoma in situ: histopathologic correlation. Can Assoc Radiol J 1999; 50:235-240.
5. Hermann G, Keller RJ, Halton K, Schwartz I, Rabinowitz JG, Tartter P. Nonpalpable ductal carcinoma in situ versus
infiltrating carcinoma of the breast--can they be differentiated by mammography? Can Assoc Radiol J 1991; 42:219-
222.
6. Holland R, Hendriks JH. Microcalcifications associated with ductal carcinoma in situ: mammographic-pathologic
correlation. Semin Diagn Pathol 1994; 11:181-192.
7. Ikeda DM, Birdwell RL, Daniel BL. Potential role of magnetic resonance imaging and other modalities in ductal
carcinoma in situ detection. Magn Reson Imaging Clin N Am 2001; 9:345-356, vii.
8. Moon WK, Myung JS, Lee YJ, Park IA, Noh DY, Im JG. US of ductal carcinoma in situ. Radiographics 2002; 22:269-
280; discussion 280-281.
9. Morris EA, Liberman L, Ballon DJ, et al. MRI of occult breast carcinoma in a high-risk population. AJR Am J
Roentgenol 2003; 181:619-626.
10. Page DL, Lagios MD. Pathology and clinical evolution of ductal carcinoma in situ (DCIS) of the breast. Cancer Lett
1994; 86:1-4.
11. Schwartz GF, Solin LJ, Olivotto IA, Ernster VL, Pressman PI. Consensus Conference on the Treatment of In Situ
Ductal Carcinoma of the Breast, April 22-25, 1999. Cancer 2000; 88:946-954.
12. Sewell CW. Pathology of high-risk breast lesions and ductal carcinoma in situ. Radiol Clin North Am 2004; 42:821-
830.
13. Silverstein MJ, Lagios MD, Groshen S, et al. The influence of margin width on local control of ductal carcinoma in
situ of the breast. N Engl J Med 1999; 340:1455-1461.
14. Stomper PC, Connolly JL, Meyer JE, Harris JR. Clinically occult ductal carcinoma in situ detected with mammography:
analysis of 100 cases with radiologic-pathologic correlation. Radiology 1989; 172:235-241.
15. Waldman FM, DeVries S, Chew KL, Moore DH, 2nd, Kerlikowske K, Ljung BM. Chromosomal alterations in ductal
carcinomas in situ and their in situ recurrences. J Natl Cancer Inst 2000; 92:313-320.
16. Yen TW, Hunt KK, Mirza NQ, et al. Physician recommendations regarding tamoxifen and patient utilization of
tamoxifen after surgery for ductal carcinoma in situ. Cancer 2004; 100:942-949.

Breast Abnormalities in Young Women
Lesions in Young Women are Rare

• Very few patients seen by the average radiologist
• Women 21 years of age and younger
➢ Fibroadenoma (up to 95%)
➢ Juvenile hypertrophy
➢ Abscess and mastitis
➢ Phyllodes tumor
➢ Malignancy
❖ Primary
❖ Metastatic
➢ Cysts are rare
Lesions in Young Women are Rare

• Women over 21 years of age
➢ Fibroadenoma
➢ Abscess and mastitis
➢ Phyllodes tumor
➢ Cysts
❖ More common as age approaches 35
➢ Malignancy
Diagnosis in Young Women

• Ultrasound is primary modality
➢ Breasts are dense after puberty
➢ Radiation has a small risk
• Mammography is used in select older patients
➢ High risk screening
➢ Masses in patients over 30
➢ Malignant looking lesions
Breast Cancer Incidence in Young Women

• % of all breast cancers
➢ Age <20
❖ 0%
➢ Age <30
❖ 0.3%
➢ Age 20 – 34
❖ 2% Invasive
❖ 1% DCIS
➢ Age 40 – 49
❖ 19%
High Risk Screening

• Multiple first degree family members
➢ Begin mammography 10 years before the earliest affected relative
❖ Interval uncertain but every 1-2 years usual
• BRCA 1 and 2
➢ Begin at 25
❖ Interval uncertain but annual is common
• Ultrasound can be useful
• MRI can be useful
➢ More sensitive than mammography in high risk groups
Breast Abnormalities in Young Women 246 Chest Radiology

Ultrasound in Young Women
• Good test in dense breasts BUT
➢ Many benign non palpable masses in young women
➢ Very few cancers in young women
Breast MRI in Young Women

• Diagnosis
➢ Proven cancer
❖ Multifocality
➢ Difficult imaging
❖ Is biopsy needed?
• Screening
➢ Strong family history
➢ Gene positive BRCA 1 BRCA 2
Presenting Signs & Symptoms

• Mass
• Pain
• Screening
➢ BRCA 1
➢ BRCA 2
➢ Family history
Benign Lesions Occurring Multiple Times

• Fibroadenoma
➢ Juvenile
➢ Giant
• Phyllodes low grade
• Granular cell tumor
• Lactating adenoma
• Hamartoma
• Normal breast
• Fibrocystic change
• Intraductal papilloma
• Juvenile papillomatosis
• Vascular
• Mastitis
• Juvenile hypertrophy
• Diabetic mastopathy
Benign Lesions Occurring Once

• PASH
• Granulomatous mastitis
• Fibromatosis
• Adenosis
• Intraductal papilloma
• Fibroadenomatoid hyperplasia
• Fibrosis
• Mondor’s disease
• Varix
• Rosai Dorfman disease (Sinus histiocytosis with lymphadenopathy)
32 Fibroadenoma
• Age 0-35
➢ 3 Age 5-9
➢ 8 Age 10-14
➢ 3 Age 15-19
Chest Radiology 247 Breast Abnormalities in Young Women

Fibroadenoma
• Second most common breast lesion
➢ Fibrocystic change is first
• Begins in TDLU
➢ Caused by unopposed estrogenic stimulation
➢ Rare in men Figure 1-27-1
❖ Must have hyperestrogenic state
Fibroadenoma
• Multiple in 16 – 25% of patients clinically
• Found in 25% of breasts examined microscopically
• Can undergo myxoid change or hyalinization
➢ Gelatinous nodule
➢ Calcification
Fibroadenoma
• Originate in lobules (TDLU)
• Stages of development
➢ Proliferation of epithelial and stromal elements in (left) Two intermediate density circumscribed
multiple lobules fibroadenomas.
➢ Confluence of the hyperplastic lobules (right) Sharply bordered horizontally oriented oval
➢ Formation of fibroadenomatous nodules solid mass representing a fibroadenoma
➢ Nodules coalesce to form FA
Fibroadenoma (32) [Figure 1-27-1]
Fibroadenoma vs Giant Fibroadenoma

vs Juvenile Fibroadenoma
• Fibroadenoma
• Giant fibroadenoma
➢ Large lesion usually > 10 cm
• Juvenile fibroadenoma
➢ Age 20 years or younger
➢ Typically rapid growth and large size
➢ Usually pericanicular type with cellular stroma
Giant Fibroadenoma (32) [Figure 1-27-2] Figure 1-27-2

Juvenile Fibroadenoma (14) [Figure 1-27-3]
Figure 1-27-3
(left) Large circumscribed intermediate density

mass which is a giant fibroadenoma.
(upper right) Same lesion on ultrasound. It is
sharply bordered and homogeneous
(lower right) Surgical specimen without adherent
surrounding tissue
(left) Large solid sharply bordered mass in a 14
year old, a juvenile fibroadenoma.
(right) Gross specimen of a juvenile fibroadenoma

Phyllodes Tumor
• Benign epithelial elements and cellular spindle cell stroma
• Can act malignant
➢ Local recurrence Figure 1-27-4
➢ Distant blood born metastases
➢ Lymph node enlargement reactive usually
• Well circumscribed lobulated mass
• Similar appearance on sonography to fibroadenoma
➢ May have cystic spaces
Phyllodes Tumor
• Low grade
➢ Pushing margins
➢ Mild atypia (left) CT scan showing left breast mass with areas
➢ May recur locally of liquefaction necrosis.
➢ Rare metastases (right) Gross specimen of low grade Phyllodes
• High grade with areas of necrosis
➢ Invasive margin
➢ Moderate to severe atypia Figure 1-27-5
➢ Common local recurrence
➢ Hematogenous metastases
Phyllodes Tumor
• Treatment
➢ Wide local excision
6 Phyllodes Low Grade

• Age 25-35
➢ 3 Age 25-29
➢ 3 Age 30-35
• Can occur in girls under 10 years old
• Usually older than 10 years
• Tendency to recur but not metastasize
• Pushing margins without invasion
(left) Partially obscured large non calcified mass in
Phyllodes Low Grade (34) [Figure 1-27-4] the upper breast.
(right) Specimen shows mass with only minimal
Phyllodes Low Grade (31) [Figure 1-27-5] adherent tissue
6 Granular Cell Tumor

• Age 15-35 Figure 1-27-6
➢ 1 Age 15-19
➢ 1 Age 20-24
➢ 2 Age 25-29
➢ 2 Age 30-35
• Neural cell origin
➢ First described in tongue
➢ 6% in the breast
➢ 1/1000 incidence of invasive ductal carcinoma
• Wide age range (17-75 years)
➢ Average age 30’s
• Discrete round mass or spiculated mass
➢ Push or invasive margin
• Rare metastasis to axillary nodes
➢ One case in literature of lung metastases
Granular Cell Tumor (33) [Figure 1-27-6]

Circumscribed mass was
granular cell tumor on
biopsy

Granular Cell Tumor (35) [Figure 1-27-7] Figure 1-27-7
Lactating Adenoma [Figures 1-27-8 and 1-27-9]

• Young women
• Pregnant or lactating women
• Circumscribed lobulated masses
Figure 1-27-8
(left) Spiculated granular cell tumor.

(right) Irregular solid markedly hypoechoic mass
with microlobulations and spiculation in this
granular cell tumor
Figure 1-27-9
(left) Lobulated solid mass is a biopsy proven

lactating adenoma.
(right) Lactating adenoma presenting as a smooth
intermediate density non-calcified mass
Hamartoma
• Fibroadenolipoma
• Palpable mass or mammographic finding
➢ Can be large and not palpable
• Encapsulated normal breast elements
Hamartoma (34) [Figure 1-27-10]

Solid mass with an angular border on the right
Juvenile Papillomatosis side which was proven to be a lactating adenoma
• Firm discrete mass
➢ Localized cystically dilated ducts with intraductal proliferation
• 2/3 less than 20 years old Figure 1-27-10
• Association with family history of breast carcinoma
➢ 10% develop carcinoma within 10 years
• Treat with excisional biopsy
Juvenile Papillomatosis (14) [Figure 1-27-11]
Figure 1-27-11
(left) Hamartoma as a smooth large non-

calcified mass with fat in it.
(right) Lobulated mildly inhomogeneous solid
mass corresponding to the hamartoma on the
last figure
(left) Multicystic mass with many small cystic
spaces represents classic juvenile papillomatosis
in a 14 year old.
(right) Pathologic specimen of juvenile
papillomatosis

Juvenile Hypertrophy [Figure 1-27-12] Figure 1-27-12
• Usually age 11-14
• Usually coincides with first menses
• Usually lasts 3-6 months
• Unilateral or bilateral palpable mass
• Iatrogenic amastia if removed
Diabetic Mastopathy
• Focal fibrosis in the breast
• Diabetes mellitus type 1 since childhood
➢ Poorly controlled
➢ Complications from vasculitis elsewhere
• Occurs in young to middle age
Diabetic Mastopathy (33) [Figure 1-27-13] Figure 1-27-13

Diabetic Mastopathy (28) [Figure 1-27-14]
Figure 1-27-14
Increased tissue behind

the nipple in this 12 year
old girl was juvenile
hypertrophy
Very irregular hypoechoic mass with

shadowing was also diabetic mastopathy in
a 28 year old
Dense mass in a 33 year old

PASH (Pseudoangiomatous Stromal Type 1 diabetic was diabetic
Hyperplasia) mastopathy
• Wide age range
• Focal lesion usually
• Histologically shows slit-like separation of stromal cells
• Exaggerated stromal response to hormone stimulation
PASH (35) [Figure 1-27-15] Figure 1-27-15
(left) Circumscribed mass in PASH in a 35 year old.

(center) Large cystic spaces in PASH
(right) Specimen showing large cystic spaces in PASH

Granulomatous Mastitis [Figure 1-27-16] Figure 1-27-16
• Usually in reproductive age
• Often within 3 years of pregnancy
• Idiopathic
• Specific causes must be excluded
➢ TB or other bacteria
➢ Sarcoid
➢ Fat necrosis
➢ Foreign body
Granulomatous Mastitis (26) [Figures 1-27-17 and 1-27-18]
Figure 1-27-17
Swelling and redness in

granulomatous mastitis
Figure 1-27-18
Spiculated mass in granulomatous

Irregular hypoechoic mass was
mastitis
granulomatous mastitis
Malignant
• No malignant lesion under age 15 in our series
• Invasive ductal carcinoma
➢ DCIS Figure 1-27-19
• Sarcoma
➢ Angiosarcoma most common
• High grade phyllodes
• Lymphoma
• Metastasis
Ductal Carcinoma
• Age 15-35
➢ 2 age 15-19
➢ 2 Age 20-24
➢ 4 Age 25-29
➢ 18 Age 30-35
• 2 Secretory carcinoma
Invasive Ductal Carcinoma [Figure 1-27-19]

• Most common carcinoma
• Youngest patient 6 years (not in this series)
• Signs similar to older patients Clustered amorphous calcifications
of invasive ductal carcinoma in a
30 year old

Invasive Ductal Carcinoma (28) [Figure 1-27-20] Figure 1-27-20
Medullary Carcinoma (24) Figure 1-27-21

[Figure 1-27-21]
Medullary Carcinoma (17)

[Figure 1-27-22]
Figure 1-27-22
Microlobulated mass with

spiculations in a 28 year old
represents invasive ductal
carcinoma
Seventeen year old patient with
medullary carcinoma
Secretory Carcinoma Irregular shaped mass with

• Previously called juvenile spiculations in a 24 year old patient
carcinoma with medullary carcinoma
• Initial report age 3-15
➢ Oldest patient 87
• Limited aggressiveness in younger patients
Figure 1-27-23
Secretory Carcinoma (23) [Figure 1-27-23]
DCIS (26) [Figure 1-27-24]

• Found on screening mammography in high risk
patients
• Found as mass rarely or nipple discharge
Figure 1-27-24
Slightly irregular mass of secretory carcinoma in

a 23 year old
Amorphous calcifications of DCIS in a

26 year old
Sarcoma
• Malignant mesenchymal tumors
• 1% of malignant tumors in all ages
➢ Higher % in young women
• After radiation therapy 2-15 years
• Many histological subtypes

Sarcoma
• Age 15-35
➢ 2 Age 15-19 Figure 1-27-25
➢ 2 Age 20-24
➢ 2 Age 25-29
➢ 4 Age 30-35
• 7 Angiosarcoma
• 2 Granulocytic sarcoma
• 1 Myosarcoma
Angiosarcoma
• 14 – 82 years
➢ Mean of 35
• Lobulated mass
• Highly aggressive lesion
➢ Axillary metastasis rare
➢ Hematogenous metastasis
usual
(left) Large mass replacing the entire breast in angiosarcoma
Angiosarcoma (34) [Figure 1-27- (right) Angiosarcoma
25]
Phyllodes High Grade

• Age 20-35 Figure 1-27-26
➢ 2 Age 20-24
➢ 1 Age 25-30
➢ 2 Age 30-35
• Usually older than 10 years
• Tendency to recur and metastasize
• Invasive margins
• Axillary adenopathy usually reactive
• Metastases hematogenous
Phyllodes High Grade (31) [Figure 1-27-26]
Lymphoma
• Primary or secondary (left) Lobulated mass representing a high grade
• Focal mass or diffuse process phyllodes tumor
(right) Lobulated edge of high grade phyllodes
Lymphoma (27) [Figure 1-27-27] tumor well seen on ultrasound
Metastatic Disease
• Neurofibrosarcoma
• Medulloblastoma Figure 1-27-27
• In adults (male and female)
➢ Melanoma
➢ Lung
➢ Prostate
➢ Lymphoma
Irregular mass of primary lymphoma in a

27 year old

Metastatic Disease (29) [Figure 1-27-28] Figure 1-27-28
• Neurofibrosarcoma
Metastatic Disease (35) [Figure 1-27-29]

• Medulloblastoma
Figure 1-27-29
(left) Well marginated oval solid mass in a

metastatic neurofibrosarcoma
(right) Specimen shows sharply marginated mass
without invasion of surrounding tissue
(left) Partially obscured noncalcified mass in

metastatic medulloblastoma
(right) Well marginated lobulated metastatic
medulloblastoma
Conclusions
• Ultrasound is the primary modality in this age group
• Mammography is reserved for screening, likely malignant lesions and the older
patients in this group
• MRI indications are evolving
• Cysts are rare especially in the younger age groups
• Most solid lesions are benign
➢ Fibroadenoma most common
• Juvenile hypertrophy and juvenile papillomatosis are unique to this age group
and have specific appearances on imaging
• Malignant lesions occur and look like malignant lesions in older women
➢ Invasive ductal carcinoma most common
References
1. Bock K, et.al. Pathologic Breast Conditions in Childhood and Adolescence. Evaluation by Sonographic Diagnosis.
J Ultrasound Med 2005; 24:1347-1354.
2. Chateil JF, Arboucalot F, Perel Y, Brun M, Boisserie-Lacroix M, Diard F. Breast metastases in adolescent girls: US
findings. Pediatr Radiol 1998; 28:832-835.
3. Ciftci AO, Tanyel FC, Buyukpamukcu N, Hicsonmez A. Female breast masses during childhood: a 25-year review. Eur
J Pediatr Surg 1998; 8:67-70.
4. Elsheikh A, Keramopoulos A, Lazaris D, Ambela C, Louvrou N, Michalas S. Breast tumors during adolescence. Eur
J Gynaecol Oncol 2000; 21:408-410.
5. El-Tamer MB, Song M, Wait RB. Breast masses in African American teenage girls. J Pediatr Surg 1999; 34:1401-
1404.
6. Green I, Dorfman RF, Rosai J. Breast involvement by extranodal Rosai-Dorfman disease: report of seven cases. Am
J Surg Pathol 1997; 21:664-668.
7. Greydanus DE, Parks DS, Farrell EG. Breast disorders in children and adolescents. Pediatr Clin North Am 1989;
36:601-638.
8. Harris VJ, Jackson VP. Indications for breast imaging in women under age 35 years. Radiology 1989; 172:445-448.
9. Karl SR, Ballantine TV, Zaino R. Juvenile secretory carcinoma of the breast. J Pediatr Surg 1985; 20:368-371.
10. Kronemer KA, Rhee K, Siegel MJ, Sievert L, Hildebolt CF. Gray scale sonography of breast masses in adolescent
girls. J Ultrasound Med 2001; 20:491-496; quiz 498.
11. Murphy JJ, Morzaria S, Gow KW, Magee JF. Breast cancer in a 6-year-old child. J Pediatr Surg 2000; 35:765-767.
12. Pettinato G, Manivel JC, Kelly DR, Wold LE, Dehner LP. Lesions of the breast in children exclusive of typical
fibroadenoma and gynecomastia. A clinicopathologic study of 113 cases. Pathol Annu 1989; 24 Pt 2:296-328.

13. Raganoonan C, Fairbairn JK, Williams S, Hughes LE. Giant breast tumours of adolescence. Aust N Z J Surg 1987;
57:243-247.
14. Raju GC, Jankey N, Naraynsingh V. Breast disease in young West Indian women: an analysis of 1051 consecutive
cases. Postgrad Med J 1985; 61:977-978.
15. Rogers DA, Lobe TE, Rao BN, et al. Breast malignancy in children. J Pediatr Surg 1994; 29:48-51.
16. Rosen PP, Holmes G, Lesser ML, Kinne DW, Beattie EJ. Juvenile papillomatosis and breast carcinoma. Cancer 1985;
55:1345-1352.
17. Simmons PS. Diagnostic considerations in breast disorders of children and adolescents. Obstet Gynecol Clin North Am
1992; 19:91-102.
18. Simmons PS. Breast disorders in adolescent females. Curr Opin Obstet Gynecol 2001; 13:459-461.
19. Squire R, Bianchi A, Jakate SM. Radiation-induced sarcoma of the breast in a female adolescent. Case report with
histologic and therapeutic considerations. Cancer 1988; 61:2444-2447.
20. Templeman C, Hertweck SP. Breast disorders in the pediatric and adolescent patient. Obstet Gynecol Clin North Am
2000; 27:19-34.
21. Weinstein SP, Conant EF, Orel SG, Zuckerman JA, Bellah R. Spectrum of US findings in pediatric and adolescent
patients with palpable breast masses. Radiographics 2000; 20:1613-1621.

The Male Breast
Figure 1-28-1
Development
• Birth to puberty same as female
Anatomy [Figure 1-28-1]

• Major ducts with little branching
• Connective tissue and fat
• Almost no lobules
Imaging
• Less than 1% of breast imaging
➢ Mammography
➢ Ultrasound
➢ MRI
➢ CT
Normal Male Mammogram [Figures 1-28-2 to 1-28-5] Normal male with no

tissue seen except
Figure 1-28-2 Figure 1-28-3 Figure 1-28-4 fat
Figure 1-28-5
Normal male with Normal male with Normal male with

minimal subareolar small focus of intramammary node
tissue subareolar tissue Large amount of
subareolar tissue in
an asymptomatic
Male Breast Disease male
• Presents as mass, swelling or pain
• Presents as nipple discharge
• Can be benign or malignant
Benign Disease
• Gynecomastia
• Pseudogynecomastia
• Papilloma
• Adenoma
• Myofibroblastoma
➢ More common in men than women
• Fibrocystic change
Chest Radiology 257 The Male Breast

Benign Disease
• Diabetic mastopathy
• Epitheal inclusion cyst
• Cystic Lymphangioma
• Pleomorphic hyalinizing angioectatic tumor of soft parts
• Varix
• Leiomyoma
• Lipoma
Benign Disease
• No lactating adenomas
➢ No pregnancy
• Rare lobular tumors
➢ No lobules without progesterone
➢ Rare invasive lobular carcinomas reported
Benign Disease
• Rare biphasic tumors
➢ Fibroadenoma, phyllodes, carcinosarcoma
➢ Lesions begin in TDLU (lobules)
✧ Lobular development rare in men
Gynecomastia
• Potentially reversible enlargement of the male breast
• Presents as soft mobile tender subareolar mass
• Simultaneous proliferation ducts and stroma without encapsulation
➢ Florid (early) phase
✧ Begins as increased number of ducts and epithelial proliferation with
edema and cellular fibroblastic stroma
✧ Reversible phase
➢ Fibrotic (late) stage
✧ Progresses to dilated ducts, moderate epithelial proliferation and
fibrosis
Gynecomastia
• 2cm or more of subareolar tissue in non obese male
• Common “normal” finding
➢ 55% of men at autopsy
➢ Peak incidence 60 – 69 years
➢ Significant if new or symptomatic
• Palpable unilateral or bilateral subareolar mass
➢ Often conical shape
• 65% of breast lesions in elderly males
➢ 25% Carcinoma
➢ 10% Other lesions
Gynecomastia
• Response to hyperestrogenism or estrogen like response
• Absolute increase in estrogen HCG or estrogen precursors
➢ Secretion by tumors
✧ Leydig cell tumor
✧ Germ cell tumors
✧ Hepatoma
✧ Adrenal cortical tumors
✧ Pituitary tumors
Gynecomastia
• Absolute increase in estrogen HCG or estrogen precursors
➢ Estrogen therapy
✧ Prostate carcinoma
✧ Topical estradiol to scalp
The Male Breast 258 Chest Radiology

➢ Increase in estrogen precursors Figure 1-28-6
✧ Cirrhosis
✧ Hyperthyroidism
Gynecomastia
• Relative increase in estrogen
➢ Testicular failure or atrophy
✧ Idiopathic
✧ Cytotoxic chemotherapy
➢ Puberty and senescence
✧ Transient in puberty (1 – 2 years)
➢ Klinefelter’s syndrome (XXY)
➢ Testicular feminization syndrome
Gynecomastia
• Hyperthyroidism
➢ Reverses when the patient is euthyroid
Symptomatic male with prominent
• Refeeding after malnutrition or starvation
subareolar tissue
• Onset of hemodialysis
Gynecomastia Figure 1-28-7 Figure 1-28-8

• Drugs (partial list)
➢ Spironolactone
➢ Reserpine
➢ Digitalis
➢ Ergot
➢ Thyroid extract
➢ Dilantin
➢ Thiazide diuretics
➢ Cimetadine
➢ Marijuana
Gynecomastia
• Mammographic patterns
➢ Nodular glandular (florid phase)
➢ Dendritic (fibrotic phase) Irregular dense Symptomatic male
➢ Diffuse glandular (very high estrogen levels) tissue behind the with diffusely dense
nipple pattern
Nodular Pattern [Figure 1-28-6]
• Fan shaped density radiating from the nipple
➢ May be more prominent in UOQ
➢ Blends into surrounding fat
Dendritic Pattern [Figure 1-28-7] Figure 1-28-9

• Subareolar density with prominent extensions into fat
➢ Density smaller than nodular pattern
Diffuse Pattern [Figure 1-28-8]

• Small heterogeneously dense breast
Pseudogynecomastia [Figure 1-28-9]

• No palpable mass
• Excessive fat deposition in breast area
➢ Normal variant
➢ Obesity
➢ Neurifibromatosis
Male with enlarged

fatty breast

Myofibroblastoma [Figures 1-28-10 and 1-28-11] Figure 1-28-10
• Solitary palpable firm mass
➢ Rarely bilateral
➢ No calcifications
• Freely moveable
• More common in men than women
• Mean age late 50’s
• Circumscribed lobulated mass without calcification
• Treated with local excision
Granular Cell Tumor [Figures 1-28-12 and 1-28-13]

• Benign tumor of neural origin
• 6% in breast
• Typical age is 30’s Oval solid mass proven to be
• Bimodal appearance myofibroblastoma
➢ Spiculated or circumscribed
✧ Usually circumscribed in males Figure 1-28-13
Granular cell tumor with smooth

margins
Typical appearance Granular cell tumor
of a with irregular
myofibroblastoma margins
Figure 1-28-14
Epidermal Inclusion Cyst [Figure 1-28-14]
• Skin lesion
• Round well circumscribed dense mass
Cystic Lymphangioma Figure 1-28-15

[Figure 1-28-15]
Left: Smooth circumscribed superficial mass proven

to be an epidermal inclusion cyst.
Right: Ultrasound shows epidermal origin of the
mass
Non specific
lobulated mass
proven to be a
cystic
lymphangioma

Granulomatous Mastitis Figure 1-28-16
• Idiopathic
• Specific causes must be excluded
➢ TB or other bacteria
➢ Sarcoid
➢ Fat necrosis
➢ Foreign body
Pleomorphic Hyalinizing
Angioectatic Tumor of Soft Parts [Figure 1-28-16]
Varix [Figure 1-28-17]
Leiomyoma [Figure 1-28-18]

• Circumscribed mass Complex mass on MRI with non
• No mitotic activity specific appearance proven to be a
➢ >2 mitoses/hpf is leiomyosarcoma pleomorphic hyalinizing angioectatic
tumor of soft parts
Malignant Disease
• Carcinoma Figure 1-28-17
• Metastasis
• Lymphoma
• Sarcoma
Male Breast Cancer

• 1690 new cases estimated in 2005 in USA
➢ 460 men will die of disease in 2005
• 1% of all invasive mammary cancers
➢ Less than 0.1% of male cancers
➢ Higher incidence in China and Africa
✧ High incidence of hyperestrogenism secondary to
parasitic liver disease
Male Breast Cancer Large vascular structure with venous

• Occurs in 60’s (10 years after women) flow (not shown)
➢ Reported in ages 5 - 93
• Unilateral painless subareolar mass
➢ Bilateral in 2% of cases Figure 1-28-18
➢ Can present as bloody nipple discharge
• Most invasive ductal cancer including special types
➢ Invasive lobular cancer very rare
➢ DCIS rare (no screening)
➢ 80% ER positive
Male Breast Cancer

• Frequently located subareolar
➢ Most common presentation is a painless subareolar mass
➢ Mass usually eccentric to the nipple
➢ Mass round, oval or irregular
➢ Calcifications rare and coarser than in women
Male Breast Cancer

• Paget’s disease and skin ulceration more common than in women
Leiomyoma with
• Axillary metastases similar to women at same stage
typical appearance
• Found at later stage than women
as a circumscribed
non specific mass
Male Breast Cancer
• Infiltrating ductal carcinoma including special types
➢ 93.7% invasive ductal (usually NOS)
➢ 2.6 % papillary
➢ 1.8% colloid
➢ 1.5% lobular

Male Breast Cancer
• DCIS Figure 1-28-19
➢ 10% of cases
➢ Usually papillary type
➢ Comedo type very rare
• Liposarcoma
• Lymphoblastic lymphoma
• Metastasis
Carcinoma Risk Factors

• Advanced age
• Family history
• Jewish heritage
• Chest wall irradiation
• Hyperestrogenism
• Hyperthyroidism
• Exposure to hepatotoxins Irregular lobulations
• Occupational exposure to high heat in a subareolar
invasive ductal
Carcinoma Risk Factors carcinoma
• BRCA 2 in 4 – 16% of cancer patients
➢ 40% in Iceland Figure 1-28-20
• Undescended testes
• Orchiectomy and orchitis
• Klinefelter’s syndrome
➢ 47, XXY
➢ 6% of male breast cancer
➢ 3% lifetime risk
Invasive Ductal Carcinoma [Figures 1-28-19 to 1-28-21]
Papillary Carcinoma [Figure 1-28-22] Figure 1-28-21
Irregular mass with spiculations is

proven invasive ductal carcinoma
Figure 1-28-22
Ultrasound of irregular lobulated

vascular mass typical of invasive
ductal carcinoma
Metastasis
• Prostate most common in males
• Hematogenous spread from primary
• Usually in patients with widespread disease
➢ Occasionally solitary
• Usually round or oval circumscribed lobulated non-calcified mass
Ductal involvement by a papillary

carcinoma

Metastasis Small Cell Carcinoma Lung [Figure 1-28-23] Figure 1-28-23
Lymphoma
• Primary or secondary
• Usually a unilateral mass
➢ Can be diffuse thickening rarely
➢ No calcification or retraction
Liposarcoma [Figures 1-28-24 and 1-28-25]

• Very rare sarcoma
• Slowly enlarging painful mass
Gynecomastia Carcinoma
• Age 60’s Age 60’s
• Soft Soft or hard
• Mobile Mobile or fixed Typical rounded
• Tender usually Tender or painless masses in
• Subareolar Subareolar metastatic disease
➢ Central Eccentric usually
• Unilateral or bilateral Unilateral usually
• Nodular, fibrotic or diffuse Mass, large or small Figure 1-28-24
Gynecomastia Carcinoma
• Nodular Large mass
➢ Fan shaped Lobulated border
• Fibrotic Small mass
➢ Subareolar density with Spiculations
extensions into fat
Gynecomastia / Carcinoma [Figures 1-28-26]
Conclusion
• Disease presents as mass pain or nipple discharge
• Gynecomastia and invasive ductal cancer are the most common lesions in the
male breast
➢ There are other rarer benign and malignant lesions Large water density
• Gynecomastia and carcinoma can look similar mass in a male
➢ Biopsy is sometimes necessary to separate gynecomastia from carcinoma breast with a
• All lesions eccentric to the nipple need biopsy unless they are preexisting lipoma
characteristically benign
➢ Contain fat Figure 1-28-25
➢ Lymph node
Figure 1-28-26
CT scan showing water density mass

Left: Gynecomastia. in this male patient
Right: Carcinoma

References
1. Appelbaum AH, Evans GF, Levy KR, Amirkhan RH, Schumpert TD. Mammographic appearances of male breast disease.
Radiographics 1999; 19:559-568.
2. Braunstein GD, Glassman HA. Gynecomastia. Curr Ther Endocrinol Metab 1997; 6:401-404.
3. Chantra PK, So GJ, Wollman JS, Bassett LW. Mammography of the male breast. AJR Am J Roentgenol 1995; 164:853-858.
4. Dershaw DD, Borgen PI, Deutch BM, Liberman L. Mammographic findings in men with breast cancer. AJR Am J Roentgenol
1993; 160:267-270.
5. Evans GF, Anthony T, Turnage RH, et al. The diagnostic accuracy of mammography in the evaluation of male breast disease.
Am J Surg 2001; 181:96-100.
6. Fentiman IS, Fourquet A, Hortobagyi GN. Male breast cancer. Lancet 2006; 367:595-604.
7. Giordano SH. A review of the diagnosis and management of male breast cancer. Oncologist 2005; 10:471-479.
8. Giordano SH, Cohen DS, Buzdar AU, Perkins G, Hortobagyi GN. Breast carcinoma in men: a population-based study. Cancer
2004; 101:51-57.
9. Gunhan-Bilgen I, Bozkaya H, Ustun EE, Memis A. Male breast disease: clinical, mammographic, and ultrasonographic
features. Eur J Radiol 2002; 43:246-255.
10. Haraldsson K, Loman N, Zhang QX, Johannsson O, Olsson H, Borg A. BRCA2 germ-line mutations are frequent in male breast
cancer patients without a family history of the disease. Cancer Res 1998; 58:1367-1371.
11. Hill TD, Khamis HJ, Tyczynski JE, Berkel HJ. Comparison of male and female breast cancer incidence trends, tumor
characteristics, and survival. Ann Epidemiol 2005; 15:773-780.
12. Hodgson NC, Button JH, Franceschi D, Moffat FL, Livingstone AS. Male breast cancer: is the incidence increasing? Ann Surg
Oncol 2004; 11:751-755.
13. Iredale R, Brain K, Williams B, France E, Gray J. The experiences of men with breast cancer in the United Kingdom. Eur J
Cancer 2006; 42:334-341.
14. Jellici E, Malago R, Remo A, Bonetti F, Pozzi Mucelli R. Imaging of the male breast. Radiol Med (Torino) 2005; 110:574-588.
15. Michels LG, Gold RH, Arndt RD. Radiography of gynecomastia and other disorders of the male breast. Radiology 1977;
122:117-122.
16. Pappo I, Wasserman I, Halevy A. Ductal carcinoma in situ of the breast in men: a review. Clin Breast Cancer 2005; 6:310-314.
17. Shi AA, Georgian-Smith D, Cornell LD, et al. Radiological reasoning: male breast mass with calcifications. AJR Am J
Roentgenol 2005; 185:S205-210.

Gastrointestinal Radiology
266 Gastrointestinal Radiology
Benign Hepatic Neoplasms
AFIP Classification - Tumors of the Liver and Intrahepatic Bile Ducts

• Hepatocellular origin
➢ Hepatocellular adenoma, focal nodular hyperplasia, nodular regenerative
hyperplasia
➢ Hepatocellular carcinoma, fibrolamellar carcinoma, hepatoblastoma
• Cholangiocellular origin
➢ Bile duct cyst, biliary cystadenoma, bile duct adenoma
➢ Cholangiocarcinoma, biliary cystadenocarcinoma
• Mesenchymal origin
➢ Hemangioma, angiomyolipoma, myelolipoma, mesenchymal hamartoma
➢ Angiosarcoma, epithelioid hemangioendothelioma
Benign Hepatic Neoplasms - Objectives

• Benign neoplasms
➢ Hemangioma
➢ Focal nodular hyperplasia (FNH)
➢ Hepatocellular adenoma
➢ Bile duct cyst
➢ Biliary cystadenoma/cystadenocarcinoma
➢ Lipomatous tumors
• Surgical vs. nonsurgical neoplasms
Hemangioma
• Most common benign hepatic tumor
➢ Likely a hamartoma rather than true neoplasm
• 1% to 7% of the population
➢ Most common in adult women
➢ Least common in pediatric population
• More common in women, 5:1
➢ Estrogen influences
➢ May enlarge during pregnancy
• Symptoms
➢ 85% asymptomatic
➢ Pain
➢ Palpable mass
➢ Rupture
Hemangioma
• Kasabach-Merritt syndrome
➢ Hemolytic anemia and consumptive coagulopathy
• Erythropoietin secretion
➢ Erythrocytosis
• Associations
➢ Focal nodular hyperplasia
➢ Tuberous sclerosis
Hemangioma - Pathology
• Peripheral feeding vessels
• Blood filled spaces
• Endothelial lining
• Fibrosis from
➢ Slow flowing blood
➢ Thrombosis
➢ Hyalinization
➢ Scar formation
Gastrointestinal Radiology 267 Benign Hepatic Neoplasms

Hemangioma - Sonography [Figure 2-1-1] Figure 2-1-1
• Homogeneous, hyperechoic
• Minimal posterior acoustic
enhancement
• Atypical features
➢ Hypoechoic center
➢ Echogenic border
➢ Scalloped borders
➢ Heterogeneous hypoechoic
Hemangioma - Hypoechoic Hepatic hemangioma on sonography shows a well-defined mass that

Foci is homogenously hyperechoic. Histologically, the tumor is composed
of multiple blood filled spaces that provide interfaces to produce an
Hemangioma - Scalloped, echogenic mass on sonography
echogenic border
Figure 2-1-2
Hemangioma -
Heterogeneous, hypoechoic
Hemangioma - CT and MR
• Peripheral globular enhancement
in arterial phase
• Slow centripetal filling during
portal venous/equilibrium
• Rapid enhancement pattern
➢ Capillary hemangiomas
➢ "Flash fill" phenomenon Classic appearance of hemangioma on CT.
• MR There is discontinuous, nodular, peripheral enhancement and gradual
➢ Homogenous hyperintense contrast filling in the lesion
T2
➢ Progressive hyperintensity Figure 2-1-3
as TE increases
➢ "Light bulb" phenomenon
Hemangioma - CT [Figure 2-1-2]
Hemangioma - MR
Hemangioma - Pedunculated
Hemangioma - Edematous Atypical hemangioma on MR due to large size and central

scar hyalinization. Tagged-RBC nuclear medicine imaging is positive
confirming the diagnosis of hemangioma
Hemangioma -
Heterogeneous with Fibrosis [Figure 2-1-3] Figure 2-1-4
Hemangioma - Multiplicity
[Figure 2-1-4]
Focal Nodular Hyperplasia

• Second most common benign liver
neoplasm
• 80% to 95% occur in women
➢ Peak age, 20 to 40 years
• Associations
➢ Hepatic hemangiomas
➢ Intracranial aneurysms
➢ Dysplastic system arteries
➢ Intracranial neoplasms:
meningioma, astrocytoma Multiple hemangiomas shown on MR
Benign Hepatic Neoplasms 268 Gastrointestinal Radiology

• Pathogenesis
➢ Hyperplastic response to a vascular malformation
➢ Central artery Figure 2-1-5
➢ Central scar
• Gross Pathology
➢ Central scar
➢ Nodular with fibrous septa
➢ No hemorrhage or necrosis
➢ No capsule

• Histology
➢ Fibrous septa FNH is often isoechoic to normal liver on sonography and may show
➢ Large arteries marked flow on color doppler of power doppler imaging
➢ Normal hepatocytes
➢ Kupffer cells
➢ No portal tracts or central veins
Focal Nodular Hyperplasia - Sonography [Figure 2-1-5]

• Subtle
➢ Similar texture to normal liver
➢ “Stealth lesion”
• Scar is hypoechoic
• Doppler
➢ Peripheral and central vessels
Focal Nodular Hyperplasia - CT Figure 2-1-6

• Noncontrast
➢ Iso- or hypodense
➢ Hypodense scar
• Arterial
➢ Rapid enhancement
➢ Hypodense scar
• Portal venous
➢ Iso- or hypo- or hyperdense
➢ Delayed enhancement of
scar FNH shows contrast enhancement during the arterial phase and near
➢ Peripheral capsule-like isoattenuation during the portal venous phase
vessels
FNH [Figures 2-1-6 and 2-1-7] Figure 2-1-7

FNH - Sulfur Colloid
• Normal uptake 60%
• Defect 30%
• Increased uptake 10%
FNH - MR
• T1 isointense
➢ Low signal scar
• T2 iso or slightly hyperintense
➢ High signal scar
• Gd-DTPA FNH on CT showing late enhancement of the central scar and
➢ Rapid homogeneous peripheral vessels
enhancement
➢ May have flash enhancement
➢ Delayed enhancement of the scar
➢ Rim-like enhancement late
• T2 with ferumoxide
➢ Lesion decreases signal
➢ Except scar

FNH [Figure 2-1-8]
FNH - Flash Enhancement
FNH - Ferumoxide-enhanced MR
Paley MR, et al. AJR 2000; 175:1: 159-63
FNH - Atypical Imaging Features Figure 2-1-8

• Multiplicity
• Absent scar
• Very large scar
• Fat
• Hemorrhage
• Calcification (very rare)
Atypical FNH - Absent Scar
Atypical FNH - Hemorrhage Typical appearance of FNH on MR
Hepatocellular Adenoma
• Third most common benign liver tumor
• Composed of benign hepatocytes
• Almost always occur in women
➢ Mean age, 30 years
➢ History of oral contraceptive use
➢ Declining incidence
• Surgical resection
➢ Risk of hemorrhage
➢ Small risk of malignant transformation to HCC
• Hepatocyte proliferation
➢ Exogenous estrogens
➢ Ovarian tumors
➢ Anabolic steroids
➢ Antiestrogens
➢ Glycogenosis, type Ia and III
➢ Hurler syndrome
Hepatocellular Adenoma - Clinical Features

• Acute abdominal pain 40%
➢ Hemorrhage within tumor
➢ Intraperitoneal hemorrhage
• Palpable mass 35%
• Incidental 10%
Hepatocellular Adenoma - Pathologic Features

• Histology
➢ Benign hepatocytes
➢ Rich in glycogen
➢ Kupffer cells
• Gross
➢ Solitary
➢ Multiple (up to 50%)
➢ Capsule (25%)
➢ Peripheral vessels
➢ Central fat
➢ Necrosis, infarcts, hemorrhage

Hepatocellular Adenoma - CT and MR
• Capsule Figure 2-1-9
• Heterogeneous
• Hemorrhage (25% to 50%)
➢ Acute, high density on
unenhanced CT
➢ Chronic, hemosiderin rings
on MR
• Focal fat
• Enhancement
➢ Variable
• Intracellular glycogen/fat
➢ Diffuse low attenuation on CT Hemorrhagic hepatocellular adenoma
➢ Loss of signal on out-of-
phase MR
Hepatocellular Adenoma Acute Hemorrhage Figure 2-1-10

[Figure 2-1-9]
Hemosiderin Rings
Focal Fat and Capsule
[Figure 2-1-10]
Hepatocellular Adenoma Hepatocellular adenoma with focal fat and a capsule on CT with the
Diffuse Low Attenuation corresponding gross specimen
[Figure 2-1-11]
Hepatocellular Adenoma - Out-of-Phase MR Figure 2-1-11

Hepatocellular Adenoma -
Fat Suppression
Imaging Difficulties
• Nonhemorrhagic
• Fibrosis/scar formation
• Multiple
➢ Glycogenosis
➢ Hepatocellular adenomatosis Diffuse low attenuation in hepatocellular adenoma due to intracellular
glycogen
Multifocality
• Multiple estrogen-associated adenomas
• Hepatocellular adenomatosis
Hepatocellular Adenomatosis [Figure 2-1-12]

• Affects men and women
• Unrelated to estrogens Figure 2-1-12
• Abnormal LFT's
• Biopsy for diagnosis
• Treated symptomatically
Hepatocellular adenomatosis

Bile Duct (Hepatic) Cyst
• Common
• Congenital/developmental origin
➢ Lined by a single layer of columnar cells
• Affect all age groups
➢ Majority occur in 4th to 6th decades of life
➢ Rare in children

• Asymptomatic
➢ Majority of cases
➢ Incidental discovery
• Symptomatic
➢ Large size
➢ Secondary hemorrhage or infection
➢ Treated with drainage, sclerotherapy, or excision
• Imaging
➢ Unilocular, simple cyst
➢ Septations, debris when complicated by infection or hemorrhage

• Complex cyst differential
➢ Echinococcal cyst
➢ Simple cyst with hemorrhage/infection
➢ Post-traumatic cyst
➢ Abscess
➢ Ciliated hepatic foregut cyst
➢ Peliosis
➢ Biliary cystadenoma
➢ Biliary cystadenocarcinoma
➢ Cystic metastasis
➢ Teratoma
Biliary Cystadenoma
• Benign tumor, but
➢ May recur after excision
➢ May develop into cystadenocarcinoma
• Middle-aged women
➢ 42 - 55 years
➢ Ovarian stroma histologically
• Cystic neoplasms
➢ Unilocular or multilocular
➢ Septations
➢ Mural nodules
➢ Calcification
Biliary Cystadenoma - Imaging Features

➢ Unilocular or multilocular
➢ Cyst fluid variable composition
• Septations
• Mural nodules
➢ May enhance
• Calcification
➢ Punctate or linear
• May communicate or extend into biliary system

Biliary Cystadenoma [Figure 2- 1-13] Figure 2-1-13
Lipomatous Tumors
• Angiomyolipoma
➢ Benign
➢ Composed of adipose, smooth muscle, and blood vessels
➢ Most cases sporadic
➢ Tuberous sclerosis in 6%
• Myelolipoma
➢ Rare
➢ Benign
➢ Composed of myeloid, adipose, and blood vessels
Angiomyolipoma [Figures 2-1-14 and 2-1-15]
Myelolipoma
Summary - Benign Hepatic Neoplasms

• Nonsurgical lesions
➢ Hemangioma
➢ Focal nodular hyperplasia
Biliary cystadenoma
• Surgical lesions
➢ Hepatocellular adenoma Figure 2-1-14
Summary - Hemangioma
• Sonography
➢ Homogenous
➢ Hyperechoic
• CT/MR
➢ Peripheral nodular enhancement
• Tagged-RBC
Summary - FNH
• CT/MR
➢ Homogenous tumor
➢ Hypodense/intense scar
➢ Delayed scar enhancement
➢ Delayed peripheral enhancement
• Sulfur colloid
Summary - HCA
• For imaging diagnosis Echogenic angiomyolipomas on
➢ Female patient sonography
➢ Oral contraceptive use
➢ Evidence of hemorrhage
• Suggest HCA Figure 2-1-15
➢ Diffuse low attenuation
➢ Diffuse fat on MR
➢ Appropriate patient
• BIOPSY !
Summary - Biliary
Cystadenoma
• Cystic neoplasm
➢ Septations
➢ Nodules
Multiple hepatic angiomyolipomas in a patient with tuberous sclerosis
➢ Calcification
who has angiomyolipomas in the right kidney and a history of left
• Most common in middle-aged
nephrectomy for a hemorrhagic angiomyolipoma
women

References
Hemangioma
1. Freeny PC, Marks WM. Patterns of contrast enhancement of benign and malignant hepatic neoplasms during bolus
dynamic and delayed CT. Radiology 1986; 160:613-618.
2. Birnbaum BA, Noz ME, Chapnick J, et al. Hepatic hemangiomas: diagnosis with fusion of MR, CT, and Tc-99m-
labeled red blood cell SPECT images. Radiology 1991; 181:469-474.
3. Quinn SF, Benjamin GG. Hepatic cavernous hemangiomas: simple diagnostic sign with dynamic bolus CT.
Radiology 1992; 182:545-548.
4. Yamashita Y, Ogata I, Urata J, Takahashi M. Cavernous hemangioma of the liver: pathologic correlation with
dynamic CT findings. Radiology 1997; 203:121-125.
5. Kim T, Federle MP, Baron RL, Peterson MS, Kawamori Y. Discrimination of small hepatic hemangiomas from
hypervascular malignant tumors smaller than 3 cm with three-phase helical CT. Radiology 2001; 219:699-706.

1. Mattison GR, Glazer GM, Quint LE, Francis IR, Bree RL, Ensminger WD. MR imaging of hepatic focal nodular
hyperplasia: characterization and distinction from primary malignant hepatic tumors. AJR Am J Roentgenol 1987;
148:711-715.
2. Rummeny E, Weissleder R, Sironi S, et al. Central scars in primary liver tumors: MR features, specificity, and
pathologic correlation. Radiology 1989; 171:323-326.
3. Buetow PC, Pantongrag-Brown L, Buck JL, Ros PR, Goodman ZD. Focal nodular hyperplasia of the liver:
radiologic-pathologic correlation. RadioGraphics 1996; 16:369-388.
4. Paley MR, Mergo PJ, Torres GM, Ros PR. Characterization of focal hepatic lesions with ferumoxides-enhanced
T2-weighted MR imaging. AJR Am J Roentgenol 2000; 175:159-163.
5. Brancatelli G, Federle MP, Grazioli L, Blachar A, Peterson MS, Thaete L. Focal nodular hyperplasia: CT findings
with emphasis on multiphasic helical CT in 78 patients. Radiology 2001; 219:61-68.
6. Ruppert-Kohlmayr AJ, Uggowitzer MM, Kugler C, Zebedin D, Schaffler G, Ruppert GS. Focal nodular
hyperplasia and hepatocellular adenoma of the liver: differentiation with multiphasic helical CT. AJR Am J
Roentgenol 2001; 176:1493-1498.
7. Hussain SM, Terkivatan T, Zondervan PE, et al. Focal nodular hyperplasia: findings at state-of-the-art MR
imaging, US, CT, and pathologic analysis. Radiographics 2004; 24:3-17; discussion 18-19.
1. al-Otaibi L, Whitman GJ, Chew FS. Hepatocellular adenoma. AJR Am J Roentgenol 1995; 165:1426.
2. Casillas VJ, Amendola MA, Gascue A, Pinnar N, Levi JU, Perez JM. Imaging of nontraumatic hemorrhagic
hepatic lesions. Radiographics 2000; 20:367-378.
3. Grazioli L, Federle MP, Ichikawa T, Balzano E, Nalesnik M, Madariaga J. Liver adenomatosis: clinical,
histopathologic, and imaging findings in 15 patients. Radiology 2000; 216:395-402.
4. Ichikawa T, Federle MP, Grazioli L, Nalesnik M. Hepatocellular adenoma: multiphasic CT and histopathologic
findings in 25 patients. Radiology 2000; 214:861-868.
Biliary Cystadenoma
1. Palacios E, Shannon M, Solomon C, Guzman M. Biliary cystadenoma: ultrasound, CT, and MRI. Gastrointest
Radiol 1990; 15:313-316.
2. Buetow PC, Buck JL, Pantongrag-Brown L, et al. Biliary cystadenoma and cystadenocarcinoma: clinical-imaging-
pathologic correlations with emphasis on the importance of ovarian stroma. Radiology 1995; 196:805-810.
3. Levy AD, Murakata LA, Abbott RM, Rohrmann CA, Jr. From the archives of the AFIP. Benign tumors and
tumorlike lesions of the gallbladder and extrahepatic bile ducts: radiologic-pathologic correlation. Armed Forces
Institute of Pathology. Radiographics 2002; 22:387-413.

Malignant Hepatic Neoplasms
Malignant Hepatic Neoplasms - Objectives

• Malignant neoplasms
➢ Hepatocellular carcinoma (HCC)
➢ Fibrolamellar carcinoma (FLC)
➢ Intrahepatic cholangiocarcinoma
➢ Angiosarcoma
➢ Epithelioid hemangioendothelioma
• Approach to the incidentally discovered liver mass
Hepatocellular Carcinoma
• Neoplasm composed of malignant hepatocytes
• Fifth most common cancer worldwide
Hepatocellular Carcinoma - Geographic Variation

• High incidence areas
➢ Sub-Saharan Africa, Asia
➢ 30 to 45 years old
➢ Hepatitis B and C, aflatoxins
➢ Aggressive
• Low incidence areas
➢ Western hemisphere
➢ 70 to 80 years old
➢ Alcoholic cirrhosis, hepatitis C, hemochromatosis
➢ Insidious
Hepatocellular Carcinoma - Etiology

• Strong association with chronic liver disease
➢ Cirrhosis
➢ Hepatitis B
➢ Hepatitis C
Hepatocellular Carcinoma - Other Etiologies

• Aflatoxin B1
• Metabolic Diseases
➢ Hemochromatosis (25%)
➢ Hereditary tyrosinemia (20%)
➢ Alpha-1-antitrypsin deficiency (15%)
• Anabolic steroids
Hepatocellular Carcinoma - Clinical Features

• More common in men
➢ 2:1 to 5:1
• Elevated alpha-fetoprotein (AFP)
➢ Elevated in 70%-90%
• Paraneoplastic syndromes
➢ Hypoglycemia
➢ Erythrocytosis
➢ Hypercholesterolemia
➢ Rare, hypercalcemia, precocious puberty, gynecomastia, carcinoid
syndrome, osteoporosis, hypertrophic pulmonary osteoarthropathy
Gastrointestinal Radiology 275 Malignant Hepatic Neoplasms

Hepatocellular Carcinoma - Pathophysiology Figure 2-2-1
• Key feature relevant to imaging
➢ Angiogenesis
• Normal liver blood supply
➢ ~80% portal venous
➢ ~20% hepatic artery
• HCC blood supply
➢ ~100% hepatic artery
➢ Rarely, hypovascular
Gross Pathology
• Key features relevant to imaging
➢ Capsule
➢ Necrosis/hemorrhage/fibrosis
➢ Macroscopic fat
➢ No calcification when HCC occurs in chronic liver disease
Hepatocellular Carcinoma - Gross Pathology

• Solitary and Encapsulated
Sonographic appearance of a small
• Macroscopic fat
HCC. The lesion is well defined and
• Hemorrhage and necrosis
hypoechoic. On CT, the mass is
• Multifocal
hypervascular in the arterial phase of
contrast enhancement
Hepatocellular Carcinoma - Histologic Features
• Trabecular growth
• Occasional Kupffer cells Figure 2-2-2
Hepatocellular Carcinoma -
Sonographic Features
• Variable and nonspecific
➢ Small lesions, hypoechoic
and uniform
➢ Large lesions, focal and
heterogeneous
➢ Diffuse, multinodular pattern
• Suggestive features Mosaic appearance of HCC on sonography, CT, and gross pathology
➢ High velocity arterial flow
➢ Peripheral hypoechoic rim
Figure 2-2-3
Hepatocellular Carcinoma Hypoechoic [Figure 2-2-1]
Hepatocellular Carcinoma - Peripheral Hypoechoic

Rim
Hepatocellular Carcinoma - Mosaic Pattern [Figure 2-2-2]
Hepatocellular Carcinoma - Multifocal with Portal Vein

Invasion [Figure 2-2-3]
Multifocal HCC with portal vein

invasion on sonography
Malignant Hepatic Neoplasms 276 Gastrointestinal Radiology

Hepatocellular Carcinoma - CT and MR Features Figure 2-2-4
• Arterial phase
➢ Rapid enhancement in
small HCC
➢ Late arterial phase
better than early
arterial phase
• Portal venous phase
➢ Heterogeneous,
"mosaic pattern"
• Suggestive features
➢ Capsular Hypervascular HCC in cirrhosis
enhancement
➢ Central fibrosis Figure 2-2-5
➢ Fatty change
➢ Arterioportal shunting
Hepatocellular
Carcinoma
Small HCC in Cirrhosis
[Figure 2-2-4]
Hepatocellular
Carcinoma HCC with capsule and macroscopic fat
Capsule and
Macroscopic Fat [Figure 2-2-5] Figure 2-2-6
Hepatocellular Carcinoma - Mosaic Pattern
Mosaic Pattern with Capsular Enhancement [Figure 2-2-6]
Mosaic Pattern
Fibrosis
Multifocal with Portal Vein Invasion
Solitary and Portal Vein Invasion
Hepatocellular Carcinoma Hepatic Vein/IVC Invasion

[Figure 2-2-7]
HCC with IVC

HCC with a mosaic pattern and
Figure 2-2-7 invasion on MDCT
capsular enhancement

Hepatocellular Carcinoma - MR Imaging in Cirrhosis Figure 2-2-8
Hepatocellular Carcinoma - Noncirrhotic Liver

• Large, solitary masses
• Heterogeneous
• Capsule
• Fat (10%)
• Calcification (25%)
Hepatocellular Carcinoma - Noncirrhotic Liver
Fibrolamellar Carcinoma [Figure 2-2-8]

• Variant of HCC
➢ Bands of fibrous lamellae
➢ Tumor cells have "oncocytic" cytoplasm
• Young patients
➢ No cirrhosis
➢ AFP usually normal
Fibrolamellar Carcinoma - Gross Pathology Histology and gross pathology of

• Central scar fibrolamellar carcinoma
➢ Radiating septa
➢ Calcification
• Lobulated contour
• Bile staining
Fibrolamellar Carcinoma - CT Features[Figures 2-2-9 and 2-2-10]

• Lobulated, well defined margins
• Heterogeneous mass
➢ Arterial phase enhancement
• Central scar
➢ Hypodense in all phases of enhancement
➢ Calcification in 40%
Figure 2-2-9
Fibrolamellar Carcinoma -
MR Features
• Lobulated margins
• Heterogeneous signal mass
➢ Dark T1
➢ Bright T2
• Hypointense central scar
➢ Dark T1
➢ Dark T2
➢ No enhancement
Fibrolamellar carcinoma
Fibrolamellar Carcinoma
How can I differentiate FLC from FNH?

• Tumor heterogeneous in FLC
Figure 2-2-10
➢ Homogeneous in FNH
• Scar nonenhancing in FLC
➢ Delayed enhancement in
FNH
• Scar dark T2 signal in FLC
➢ Scar bright T2 in FNH

Intrahepatic Cholangiocarcinoma (ICC)
• Adenocarcinoma arising from intrahepatic bile ducts
➢ 10% of bile duct adenocarcinomas Figure 2-2-11
• Synonyms
➢ Peripheral cholangiocarcinoma, cholangiocellular
carcinoma,
intrahepatic bile duct carcinoma
• Geographic incidence variation
➢ 10 times more common in Japan compared to
U.S.
Intrahepatic Cholangiocarcinoma - Etiology

• Majority of cases
➢ Unknown etiology
➢ Noncirrhotic liver
Intrahepatic Cholangiocarcinoma - Etiologic

Associations
• Chronic cholestatic disease
➢ Primary sclerosing cholangitis
Intrahepatic cholangiocarcinoma
➢ Primary biliary cirrhosis
➢ Caroli disease/congenital hepatic fibrosis
• Chronic biliary inflammation
➢ Recurrent pyogenic cholangitis
Figure 2-2-12
➢ Parasitic infection
➢ Hepatolithiasis
• Hepatitis B and C
• ETOH abuse
• Radiation
Intrahepatic Cholangiocarcinoma -
Pathologic Features [Figure 2-2-11]
• Morphology
➢ Solitary
➢ Multifocal Intrahepatic cholangiocarcinoma showing
➢ Diffuse capsular contraction and
• Satellite nodules biliary dilatation peripheral to the mass
• Marked fibrosis
• No capsule
• Rare
➢ Hemorrhage and necrosis Figure 2-2-13
➢ Calcification
Intrahepatic
Cholangiocarcinoma
CT and MR Features
• Irregular borders
➢ Infiltrative
• Enhancement pattern
➢ Due to
fibrosis/hypovascularity
➢ Delayed peripheral to central Intrahepatic cholangiocarcinoma on MR showing central to peripheral
• Biliary dilatation peripheral to the enhancement on gadolinium enhanced T1-weighted images.
tumor The gross photograph shows
• Capsular contraction characteristic fibrosis within the tumor
Intrahepatic Cholangiocarcinoma
[Figures 2-2-12 and 2-2-13]

How can I differentiate ICC from HCC?
• Difficult
➢ HCC has variable morphology
➢ HCC occurs more commonly
➢ HCC associated with cirrhosis and hepatitis
➢ But, HCC may occur in normal livers
• Ultimately
➢ Biopsy is needed for diagnosis
How can I differentiate ICC from HCC?

• Enhancement
➢ Delayed, peripheral to central favors ICC
➢ Rapid filling favors HCC
➢ Marked heterogeneity (mosaic) favors HCC
• Tumor margins
➢ Lobulated, irregular favors ICC
➢ Capsule favors HCC
• Capsular contraction Figure 2-2-14
➢ More common in ICC
• Biliary dilatation peripheral to the
tumor
➢ More common in ICC
Angiosarcoma
• Malignant neoplasm of
endothelial cells
• Rare
➢ But, most common hepatic Angiosarcoma on CT and MR showing central hemorrhage that is
sarcoma fluid attenuation on CT and high signal on T1-and T2-weighted MR
• Etiologic associations
➢ Vinyl chloride
➢ Arsenical compounds
➢ Radiation therapy
➢ Anabolic steroids
Angiosarcoma
• More common in men, 3:1
• Clinical presentation Figure 2-2-15
➢ Variable
➢ Hemoperitoneum
➢ Metastasis in 60%, spleen, lung
Angiosarcoma - Imaging Features

• Solitary or multifocal
• Evidence of hemorrhage
• Enhancement
➢ Peripheral or heterogeneous
➢ Spleen and lung
Angiosarcoma [Figure 2- 2-14]
Epithelioid Hemangioendothelioma
[Figure 2- 2-15]
• Rare malignancy of endothelial origin
➢ Contains dense fibrous stroma
• Imaging
➢ Multifocal, lesions coalesce over time
➢ Peripheral enhancement Epithelioid hemangioendothelioma
➢ Central fibrous stroma showing multifocality and capsular
➢ Retracted liver capsule contraction
➢ May calcify

Approach to the incidentally discovered liver mass?
• Does the mass meet the criteria for a benign, nonsurgical lesion?
➢ Bile duct cyst
➢ Hemangioma
➢ FNH
• Are there equivocal features of hemangioma or FNH on CT and/or MR?
➢ Consider scintigraphy
• Is there clinical history that will suggest the etiology?
➢ History of primary malignancy
➢ History of chronic liver disease
➢ History or exogenous estrogens
• Are there features that suggest HCC?
➢ Capsule
➢ Fat
➢ Mosaic pattern
• Are there features that suggest hepatocellular adenoma?
➢ Clinical/demographic history
➢ Capsule
➢ Fat
➢ Diffuse low attenuation
➢ Hemorrhage
• Are there features that suggest cholangiocarcinoma?
➢ No capsule
➢ Ill-defined margins
➢ Biliary dilatation
➢ Capsular contraction
• If the answer is NO to all the above, and the finding is a small, focal area of
arterial enhancment on MDCT, the possibilities are:
➢ Hemangioma
➢ FNH
➢ Small adenoma
➢ Small HCC
➢ Hypervascular met
➢ AVM
➢ THAD
Summary Hepatocellular Carcinoma

• Most common primary hepatic malignancy
• Strong association with chronic liver disease
• Variable imaging features
➢ Capsule
➢ Mosaic pattern
➢ Focal fat
Summary Fibrolamellar Carcinoma

• Variant of HCC
• Young patients
• Otherwise normal liver
• Key features
➢ Lobular tumor
➢ Central scar
➢ Heterogeneous mass
Summary Intrahepatic Cholangiocarcinoma

• Arise from bile duct epithelium
• Uncommon
• Key features
➢ Delayed central enhancement

➢ Biliary dilatation peripheral to tumor
Summary Angiosarcoma
• Rare
• Key features
➢ Evidence of hemorrhage
➢ Splenic metastasis at presentation
References
1. Winter TC, 3rd, Takayasu K, Muramatsu Y, et al. Early advanced hepatocellular carcinoma: evaluation of CT and
MR appearance with pathologic correlation. Radiology 1994; 192:379-387.
2. Takayasu K, Furukawa H, Wakao F, et al. CT diagnosis of early hepatocellular carcinoma: sensitivity, findings, and
CT-pathologic correlation. AJR Am J Roentgenol 1995; 164:885-890.
3. Baron RL, Oliver JH, 3rd, Dodd GD, 3rd, Nalesnik M, Holbert BL, Carr B. Hepatocellular carcinoma: evaluation
with biphasic, contrast-enhanced, helical CT. Radiology 1996; 199:505-511.
4. Kelekis NL, Semelka RC, Worawattanakul S, et al. Hepatocellular carcinoma in North America: a
multiinstitutional study of appearance on T1-weighted, T2-weighted, and serial gadolinium-enhanced gradient-
echo images. AJR Am J Roentgenol 1998; 170:1005-1013.
5. Loyer EM, Chin H, DuBrow RA, David CL, Eftekhari F, Charnsangavej C. Hepatocellular carcinoma and
intrahepatic peripheral cholangiocarcinoma: enhancement patterns with quadruple phase helical CT--a comparative
study. Radiology 1999; 212:866-875.
6. Winston CB, Schwartz LH, Fong Y, Blumgart LH, Panicek DM. Hepatocellular carcinoma: MR imaging findings
in cirrhotic livers and noncirrhotic livers. Radiology 1999; 210:75-79.
7. Ward J, Guthrie JA, Scott DJ, et al. Hepatocellular carcinoma in the cirrhotic liver: double-contrast MR imaging
for diagnosis. Radiology 2000; 216:154-162.
8. Murakami T, Kim T, Takamura M, et al. Hypervascular hepatocellular carcinoma: detection with double arterial
phase multi-detector row helical CT. Radiology 2001; 218:763-767.
9. Brancatelli G, Federle MP, Grazioli L, Carr BI. Hepatocellular carcinoma in noncirrhotic liver: CT, clinical, and
pathologic findings in 39 U.S. residents. Radiology 2002; 222:89-94.
10. Iannaccone R, Laghi A, Catalano C, et al. Hepatocellular carcinoma: role of unenhanced and delayed phase multi-
detector row helical CT in patients with cirrhosis. Radiology 2005; 234:460-467.
1. Ichikawa T, Federle MP, Grazioli L, Madariaga J, Nalesnik M, Marsh W. Fibrolamellar hepatocellular carcinoma:
imaging and pathologic findings in 31 recent cases. Radiology 1999; 213:352-361.
2. McLarney JK, Rucker PT, Bender GN, Goodman ZD, Kashitani N, Ros PR. Fibrolamellar carcinoma of the liver:
3. Soyer P, Roche A, Levesque M, Legmann P. CT of fibrolamellar hepatocellular carcinoma. J Comput Assist
Tomogr 1991; 15:533-538.
4. Titelbaum DS, Hatabu H, Schiebler ML, Kressel HY, Burke DR, Saul SH. Fibrolamellar hepatocellular carcinoma:
MR appearance. J Comput Assist Tomogr 1988; 12:588-591.
5. Titelbaum DS, Burke DR, Meranze SG, Saul SH. Fibrolamellar hepatocellular carcinoma: pitfalls in nonoperative
diagnosis. Radiology 1988; 167:25-30.
6. Blachar A, Federle MP, Ferris JV, et al. Radiologists' performance in the diagnosis of liver tumors with central
scars by using specific CT criteria. Radiology 2002; 223:532-539.
1. Choi BI, Park JH, Kim YI, et al. Peripheral cholangiocarcinoma and clonorchiasis: CT findings. Radiology 1988;
169:149-153.
2. Tani K, Kubota Y, Yamaguchi T, et al. MR imaging of peripheral cholangiocarcinoma. J Comput Assist Tomogr
1991; 15:975-978.
3. Kim TK, Choi BI, Han JK, Jang HJ, Cho SG, Han MC. Peripheral cholangiocarcinoma of the liver: two-phase
spiral CT findings. Radiology 1997; 204:539-543.
4. Loyer EM, Chin H, DuBrow RA, David CL, Eftekhari F, Charnsangavej C. Hepatocellular carcinoma and
intrahepatic peripheral cholangiocarcinoma: enhancement patterns with quadruple phase helical CT--a comparative
study. Radiology 1999; 212:866-875.
5. Zhang Y, Uchida M, Abe T, Nishimura H, Hayabuchi N, Nakashima Y. Intrahepatic peripheral
cholangiocarcinoma: comparison of dynamic CT and dynamic MRI. J Comput Assist Tomogr 1999; 23:670-677.

6. Vitellas KM, Keogan MT, Freed KS, et al. Radiologic manifestations of sclerosing cholangitis with emphasis on
MR cholangiopancreatography. RadioGraphics 2000; 20:959-975; quiz 1108-1109, 1112.
7. Han JK, Choi BI, Kim AY, et al. Cholangiocarcinoma: pictorial essay of CT and cholangiographic findings.
8. Levy AD. Malignant liver tumors. Clin Liver Dis 2002; 6:147-164.
Angiosarcoma
1. Peterson MS, Baron RL, Rankin SC. Hepatic angiosarcoma: findings on multiphasic contrast-enhanced helical CT
do not mimic hepatic hemangioma. AJR Am J Roentgenol 2000; 175:165-170.
2. Koyama T, Fletcher JG, Johnson CD, Kuo MS, Notohara K, Burgart LJ. Primary hepatic angiosarcoma: findings at
CT and MR imaging. Radiology 2002; 222:667-673.
3. Levy AD. Malignant liver tumors. Clin Liver Dis 2002; 6:147-164.
4. Thompson WM, Levy AD, Aguilera NS, Gorospe L, Abbott RM. Angiosarcoma of the Spleen: Imaging
Characteristics in 12 Patients. Radiology 2005. In Press
1. Ishak KG, Sesterhenn IA, Goodman ZD, Rabin L, Stromeyer FW. Epithelioid hemangioendothelioma of the liver:
a clinicopathologic and follow-up study of 32 cases. Hum Pathol 1984; 15:839-852.
2. Miller WJ, Dodd GD, Federle MP, Baron RL. Epithelioid hemangioendothelioma of the liver: imaging findings
with pathologic correlation [see comments]. AJR Am J Roentgenol 1992; 159:53-57.
3. Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study
of 137 cases. Cancer 1999; 85:562-582.
4. Mermuys K, Vanhoenacker PK, Roskams T, D'Haenens P, Van Hoe L. Epithelioid hemangioendothelioma of the
liver: radiologic-pathologic correlation. Abdom Imaging 2004; 29:221-223.

Hepatic Infections
Hepatic Infections
• Pyogenic Abscess
• Amebic Abscess
• Echinococcal Infections
• Schistosomiasis
• Clonorchiasis
• Infections in the Immunocompromised host
➢ Candidasis
➢ Pneumocystis
Pyogenic Hepatic Abscess

• Polymicrobial infections
• Variable clinical presentation
➢ Septicemia, pain, fever, indolent symptoms
➢ Tender hepatomegaly
Pyogenic Hepatic Abscess: Pathogenesis

• Biliary
➢ MOST COMMON ETIOLOGY
➢ Cholangitis, biliary obstruction
➢ Multiple and bilateral
• Portal vein
➢ Pylephlebitis
➢ Solitary, 65% right lobe
• Hepatic artery
• Traumatic-blunt or penetrating trauma
• Necrotic tumor

• Mortality rate <10%
• Effectively treated with percutaneous drainage
➢ 8% failure rate
➢ 8% recurrence rate
Pyogenic Hepatic Abscess: Sonography

• Variable echogenicity
➢ Anechoic (50%)
➢ Hyperechoic (25%)
➢ Hypoechoic (25%)
• Ill-defined margins
• Internal character
➢ Irregular wall
➢ Septations
➢ Fluid-fluid levels
➢ Debris
➢ Reverberation artifact if gas is present
• Posterior acoustic enhancement
Hepatic Infection 284 Gastrointestinal Radiology

Pyogenic Hepatic Abscess: CT Figure 2-3-1
• Singe best imaging method
➢ Sensitivity 97%
➢ Intravenous contrast essential
• Hypoattenuating
➢ 0 to 45 H.U.
• Helpful CT features
➢ Rim-enhancement
➢ Transition zone
➢ Cluster sign
➢ Gas (<20%) Pyogenic hepatic abscess shows
➢ Air/fluid or debris/fluid level cluster sign and transition zone
❖ Suspect GI communication
Pyogenic Hepatic Abscess CT
Pyogenic Hepatic Abscess: Cluster Sign
Pyogenic Hepatic Abscess: Cluster Sign/ Transition zone

[Figure 2-3-1]
Pyogenic Hepatic Abscess: Intrahepatic Gas
Pyogenic Hepatic Abscess: Imaging Guided Drainage

• Unilocular and liquefied
• Multilocular or multiple
➢ Multiple catheters
• Multiple, small (<1 cm)
➢ Aspiration for diagnosis
➢ Treatment: antibiotics or aspiration + antibiotics
Amebic Liver Abscess

• Most common extra-intestinal manifestation of amebiasis
➢ 3%-7% of patients with amebic infection
➢ Fever, RUQ pain
• Route of spread
➢ Portal venous (most common)
➢ Lymphatic
➢ Direct extension from colon
Amebic Abscess: Sonography

• Round or oval shape
➢ Absent wall echoes
➢ Homogenous low level internal echoes
• Location
➢ Near or touching the liver capsule
Figure 2-3-2
➢ 85% solitary
➢ 72% right lobe
• Enhanced through transmission
Amebic Abscess [Figure 2-3-2]
Amebic Abscess: CT
• Enhancing wall (3-15 mm)
➢ Round
➢ Smooth or irregular
➢ Peripheral zone of edema Amebic abscess
• Low attenuation/complex fluid
➢ Septations
➢ Fluid/debris level
• Extrahepatic extension
Gastrointestinal Radiology 285 Hepatic Infection

Amebic Abscess Figure 2-3-3
Amebic vs. Pyogenic Abscess

• Cannot reliably differentiate by imaging
• Patients with amebic abscess
➢ More likely to have hepatomegaly
and diarrhea
➢ History of recent travel or inhabitant
of high prevalence areas
➢ Serologic tests positive in >90%
Amebic Abscess: Therapy

• Medical therapy
• Percutaneous biopsy of abscess wall Worldwide distribution of E. granulosus
➢ If serology does not confirm
diagnosis and clinical suspicion is high
• Percutaneous drainage if Figure 2-3-4
➢ Large, >5 cm abscess
➢ Left lobe
➢ Biliary communication
➢ Pregnancy
➢ Perforation
➢ Poor response to drug therapy
Amebic Abscess / Pyogenic

Abscess
Echinococcus: E. granulosus and

E. multilocularis Worldwide distribution of E. multilocularis
• Nomenclature
➢ Hydatidosis is the infection by the larval tapeworm of the genus
Echinococcus
• Endemic worldwide
➢ Humans accidental host
➢ Infection usually acquired during childhood
E. granulosus [Figure 2-3-3] Figure 2-3-5

E. multilocularis [Figure 2-3-4]
Echinococcus
E. granulosus and
E. multilocularis
• Symptoms occur during adulthood
➢ Cyst enlargement
➢ Erosion of cyst into peritoneal or
pleural cavity
➢ Development of biliary
communication
• Serology confirms diagnosis
➢ Positive >80% of cases
Echinococcus [Figure 2-3-5]
Echinococcus: E. granulosus
Echinococcus: E. multilocularis Echinococcus lifecycle

E. granulosus: Imaging Features Figure 2-3-6
• Unilocular or multilocular cyst
➢ Calcification in cyst wall
➢ Internal debris (hydatid sand)
• Complex cyst
➢ Internal daughter cysts
➢ Undulating membrane
(water lily sign)
➢ Fibrous and avascular walls and
membranes
Daughter cysts of E. granulosus
❖ Low MR signal
❖ No enhancement
E. granulosus
E. granulosus: Figure 2-3-7

Daughter cysts [Figure 2-3-6]
E. granulosus:
Water Lily Sign [Figures 2-3-7 and 2-3-8]
E. granulosus:
Complications and Treatment
• Cyst rupture
➢ Anaphylaxis Laminated membranes and water lily sign of E. granulosus
➢ Biliary tract, peritoneal cavity
➢ Pleural, pericardial cavity
• Treatment Figure 2-3-8
➢ Surgical excision
➢ Laparoscopic excision
➢ Percutaneous drainage
+ sclerosing scolicidal agents
E. multilocularis:
Pathologic Features
• Alveolar hydatid disease
• Propagation by external budding
• Invade surrounding tissue
➢ Infiltrative mass
➢ No limiting host tissue
➢ Resembles neoplasm
E. multilocularis:
Imaging Features
• Ultrasound
➢ Echogenic
➢ Single or multiple
➢ Ill-defined walls Water lily sign of E. granulosus
➢ Partially calcified
• CT
➢ Geographic Figure 2-3-9
➢ Infiltrating lesions
➢ Amorphous calcification
E. multilocularis [Figure 2-3-9]
E. multilocularis

Schistosomasis (Bilharziasis) Figure 2-3-10
• Trematode (fluke)
➢ S. Japonicum, S. mansoni,
S. hematobium
• Humans are definitive host
➢ Mature in the portal venules
• Migrate to deposit eggs
➢ Intestine (S. japonicum, S.
mansoni)
➢ Bladder (S. hematobium)
Schistosomasis: S. japonicum
Schistosomasis: S. mansoni
Schistosomasis: S. hematobium Lifecycle of Schistosomiasis
Schistosomasis [Figure 2-3-10] Figure 2-3-11

Schistosomasis [Figure 2-3-11]
• Granulomatous inflammation
• Fibrosis
➢ Symmers' fibrosis
➢ Turtle back liver
• Progressive portal vein occlusion
• Presinusoidal portal hypertension
Schistosomasis: Imaging
Features
• S. japonicum
➢ Hepatic calcification
➢ “Turtle back” configuration
• S. mansoni
➢ Low attenuation, rounded foci
➢ Low attenuation, linear branching
Symmers' fibrosis
bands
Schistosomiasis japonicum Figure 2-3-12

[Figure 2-3-12]
Biliary Parasites
• Parasites that invade bile ducts
➢ Trematodes
❖ Clonorchis sinensis
❖ Fasciola gigantica, Fasciola
hepatica
Schistosomiasis japonicum on CT
❖ Opisthorchis viverrini
❖ Opisthorchis felineus
➢ Nematodes
❖ Ascariasis lumbricoides
➢ Cestodes
❖ Taenia saginata

Clonorchis sinensis [Figures 2-3-13 and 2-3-14] Figure 2-3-13
• Peripheral intrahepatic bile ducts
➢ Dilatation of small intrahepatic ducts
➢ Periductal fibrosis
• Complications
➢ Cholangitis
➢ Cholangiohepatitis
➢ Liver abscess
➢ Cholangiocarcinoma
Figure 2-3-14
Lifecycle of Clonorchis sinensis
Cholangiogram shows a
filling defect, peripheral
intrahepatic strictures,
and dilatation due to
infestation of Clonorchis
sinensis
Fasciola Hepatica
Hepatic Infections in the Immunocompromised Host

• Candidiasis
• Pneumocystis Carinii
• Herpes Simplex Virus
• Liver Abscess
➢ Pyogenic
➢ Multiorganism
Disseminated Candidiasis
• Synonym: hepatosplenic candidiasis
• Pathogenesis
➢ Prolonged neutropenia
➢ Mucosal damage to the GI tract
➢ Local invasion of candida with entry into the hepatosplenic circulation
• Clinical manifestations
➢ Neutropenic with fever
➢ Return of neutrophil count
• Organ Involvement
➢ Spleen 94%, liver 75%, kidney 69%
Hepatosplenic Candidiasis: Pathology

• Necrosis with minimal inflammation
• Microabscesses with severe inflammation
• Collagen formation/fibrosis
• Granuloma formation

Hepatosplenic Candidiasis: Sonography [Figures 2-3-15]
• Type 1 lesion
➢ “wheel-within-a-wheel”
• Type 2 lesion
➢ “bull’s-eye”
• Type 3 lesion-most common
➢ hypoechoic nodule
• Type 4 lesion
➢ hyperechoic nodule
Figure 2-3-15
I
II
III
IV
Figure 2-3-16
Hepatosplenic Candidiasis:
CT Features [Figure 2-3-16]
• Concentric rings
• Hypodense nodules
• Punctate calcification
Hepatosplenic Candidiasis:
MR Features
• Low T1, high T2
• Fat-suppressed T2 improves detection Hepatic candidiasis on MDCT
• Gd-FLASH most sensitive
• Splenic gamna-gandy bodies false positive T1

Hepatosplenic Candidiasis: Imaging Management
• High index of suspicion
• Imaging during neutropenia is often negative
➢ Follow up studies if clinical suspicion high and prophylactic therapy
contraindicated
• Prophylactic therapy
• Biopsy
• Lesions change morphology with healing
Pneumocystis jiroveci
• Previously classified as Pneumocystis carinii
• Now considered a fungus
• Opportunistic infection
➢ AIDS
➢ Organ transplant recipients
Pneumocystis jiroveci (carinii): Imaging Features Figure 2-3-17]

• Sonography
➢ Nonshadowing hyperechoic nodules
➢ Shadowing echogenic clumps of calcification
• CT scan
➢ Hypodense nodules with progressive calcification
➢ Renal and lymph node calcification Figure 2-3-17
Summary: Pyogenic Abscess
• Transition zone
• Cluster sign
• Percutaneous drainage
Summary: Amebic Abscess

• Cannot reliably distinguish from
pyogenic abscess on imaging
• Percutaneous biopsy if necessary
• Viable organisms in wall
• Drainage if necessary
Summary: Echinococcus
• E. granulosus
➢ Daughter cysts
➢ Water-lily sign Sonogram and CT of disseminated pneumocystis
➢ Rim-like calcification
• E. multilocularis
➢ Infiltrating mass
➢ Calcification
Summary: Immunocompromised Hosts

• Candidiasis
➢ Neutropenics
➢ Imaging negative during neutropenia
➢ Imaging positive during WBC rebound
• Pneumocystis carinii
➢ Hyperechoic nodules
➢ +/- shadowing
➢ Hypodense on CT with progressive calcification
➢ Renal and lymph node calcification

References
Pyogenic and Amebic Hepatic Abscess

1. Halvorsen RA, Korobkin M, Foster WL, Silverman PM, Thompson WM. The variable CT appearance of hepatic
abscesses. AJR Am J Roentgenol 1984; 142:941-946.
2. Mathieu D, Vasile N, Fagniez PL, Segui S, Grably D, Larde D. Dynamic CT features of hepatic abscesses.
Radiology 1985; 154:749-752.
3. Jeffrey RB, Jr., Tolentino CS, Chang FC, Federle MP. CT of small pyogenic hepatic abscesses: the cluster sign.
AJR Am J Roentgenol 1988; 151:487-489.
4. Radin DR, Ralls PW, Colletti PM, Halls JM. CT of amebic liver abscess. AJR Am J Roentgenol 1988; 150:1297-
1301.
5. Juimo AG, Gervez F, Angwafo FF. Extraintestinal amebiasis. Radiology 1992; 182:181-183.
6. Gabata T, Kadoya M, Matsui O, et al. Dynamic CT of hepatic abscesses: significance of transient segmental
enhancement. AJR Am J Roentgenol 2001; 176:675-679.
7. Mortele KJ, Segatto E, Ros PR. The infected liver: radiologic-pathologic correlation. Radiographics 2004; 24:937-
955.
8. Yang DM, Kim HN, Kang JH, Seo TS, Park CH, Kim HS. Complications of pyogenic hepatic abscess: computed
tomography and clinical features. J Comput Assist Tomogr 2004; 28:311-317.
Hydatid Disease (Echinococcus)

1. de Diego Choliz J, Lecumberri Olaverri FJ, Franquet Casas T, Ostiz Zubieta S. Computed tomography in hepatic
echinococcosis. AJR Am J Roentgenol 1982; 139:699-702.
2. Pandolfo I, Blandino G, Scribano E, Longo M, Certo A, Chirico G. CT findings in hepatic involvement by
Echinococcus granulosus. J Comput Assist Tomogr 1984; 8:839-845.
3. Claudon M, Bessieres M, Regent D, et al. Alveolar echinococcosis of the liver: MR findings. J Comput Assist
Tomogr 1990; 14:608-614.
4. Acunas B, Rozanes I, Acunas G, Celik L, Alper A, Gokmen E. Hydatid cyst of the liver: identification of detached
cyst lining on CT scans obtained after cyst puncture. AJR Am J Roentgenol 1991; 156:751-752.
5. Taourel P, Marty-Ane B, Charasset S, Mattei M, Devred P, Bruel JM. Hydatid cyst of the liver: comparison of CT
and MRI. J Comput Assist Tomogr 1993; 17:80-85.
6. Ustunsoz B, Akhan O, Kamiloglu MA, Somuncu I, Ugurel MS, Cetiner S. Percutaneous treatment of hydatid cysts
of the liver: long-term results. AJR Am J Roentgenol 1999; 172:91-96.
Schistosomiasis
1. Araki T, Hayakawa K, Okada J, Hayashi S, Uchiyama G, Yamada K. Hepatic schistosomiasis japonica identified
by CT. Radiology 1985; 157:757-760.
2. Fataar S, Bassiony H, Satyanath S, et al. CT of hepatic schistosomiasis mansoni. AJR Am J Roentgenol 1985;
145:63-66.
3. Monzawa S, Uchiyama G, Ohtomo K, Araki T. Schistosomiasis japonica of the liver: contrast-enhanced CT
findings in 113 patients. AJR Am J Roentgenol 1993; 161:323-327.
4. Lee RC, Chiang JH, Chou YH, et al. Intestinal schistosomiasis japonica: CT-pathologic correlation. Radiology
1994; 193:539-542.
5. Willemsen UF, Pfluger T, Zoller WG, Kueffer G, Hahn K. MRI of hepatic schistosomiasis mansoni. J Comput
Assist Tomogr 1995; 19:811-813.
6. Cheung H, Lai YM, Loke TK, et al. The imaging diagnosis of hepatic schistosomiasis japonicum sequelae. Clin
Radiol 1996; 51:51-55.
7. Mortele KJ, Ros PR. Imaging of diffuse liver disease. Semin Liver Dis 2001; 21:195-212.
Hepatosplenic Candidiasis
1. Ho B, Cooperberg PL, Li DK, Mack L, Naiman SC, Grossman L. Ultrasonography and computed tomography of
hepatic candidiasis in immunosuppressed patients. J Ultrasound Med 1982; 1:157-159.
2. Shirkhoda A. CT findings in hepatosplenic and renal candidiasis. J Comput Assist Tomogr 1987; 11:795-798.
3. Pastakia B, Shawker TH, Thaler M, O'Leary T, Pizzo PA. Hepatosplenic candidiasis: wheels within wheels.
Radiology 1988; 166:417-421.
4. Thaler M, Pastakia B, Shawker TH, O'Leary T, Pizzo PA. Hepatic candidiasis in cancer patients: the evolving
picture of the syndrome. Ann Intern Med 1988; 108:88-100.
5. Gorg C, Weide R, Schwerk WB, Koppler H, Havemann K. Ultrasound evaluation of hepatic and splenic
microabscesses in the immunocompromised patient: sonographic patterns, differential diagnosis, and follow-up. J
Clin Ultrasound 1994; 22:525-529.
6. Rudolph J, Rodenwaldt J, Ruhnke M, Hamm B, Kopka L. Unusual enhancement pattern of liver lesions in
hepatosplenic candidiasis. Acta Radiol 2004; 45:499-503.

Imaging of Chronic Liver Disease
Chronic Liver Disease: Objectives

• Cirrhosis
• Steatosis and steatohepatitis
• Budd-Chiari
• Disorders of Iron Deposition
➢ Hemosiderosis
➢ Hemochromatosis
Cirrhosis: Definition
• Endpoint of chronic liver disease
Cirrhosis: Pathology
• Hepatocyte injury and loss
• Fibrosis
• Nodule formation
• Architectural reorganization
• Nodules
➢ Micronodular (<3mm)
➢ Macronodular (>3mm)
➢ Mixed
• Liver volume
➢ Early, hepatomegaly from inflammation
➢ Late, small liver from fibrosis
Cirrhosis: Segmental Alterations in Volume

• Common feature
• Not fully understood
➢ Alteration in blood flow
• Atrophy
➢ Segments IV, VI, VIII
• Hypertrophy
➢ Segments I, II, III
Cirrhosis: Imaging
• Cannot assess severity
• Role of imaging
➢ Assess disease complications
➢ Evaluation of portal hypertension
➢ HCC surveillance
Cirrhosis: Sonography
• Fibrosis
➢ Increased parenchymal echogenicity
➢ Decreased penetration of the ultrasound beam
➢ Poor visualization of hepatic vasculature
➢ Loss of triphasic hepatic vein doppler
➢ Increased pulsatility of portal vein doppler
• Nodules
• Volume redistribution
• Portal hypertension
Gastrointestinal Radiology 293 Imaging of Chronic Liver Disease

Cirrhosis: CT Figure 2-4-1
• Morphologic changes
➢ Volume redistribution
➢ Nodules
• Fibrosis
➢ Prominent porta and fissures
➢ Focal confluent fibrosis
➢ Decreased parenchymal
enhancement
• Mesenteric changes
➢ Lymphadenopathy Cirrhosis with fibrosis, altered enhancement and nodules on CT
➢ Increased mesenteric attenuation
Cirrhosis: Volume redistribution and nodules Figure 2-4-2
Cirrhosis: Volume redistribution
Cirrhosis: Fibrosis, altered

enhancement, nodules [Figure 2-4-1]
Cirrhosis: Mesenteric Changes,

Adenopathy [Figure 2-4-2]
Chronic hepatitis B cirrhosis with nodules, mesenteric changes,
Cirrhosis: Nodules and adenopathy
• Regenerative nodule
➢ Benign
➢ Proliferation of hepatocytes
➢ Precursor to dysplastic nodule and HCC Figure 2-4-4
• Dysplastic nodule
➢ Premalignant
• Hepatocellular carcinoma
Cirrhosis: Regenerative Nodule

• Benign proliferation of hepatocytes
• Hemosiderin deposition
➢ "Siderotic nodule"
➢ Noncontrast scans helpful for detection
• CT
➢ Isodense with and without contrast
➢ Hyperdense on noncontrast (siderotic nodule)
• MR
➢ Dark T1, T2, gradient echo
➢ Bright T1 (rare), Dark T2
➢ Best seen on GRE and T2 images
Cirrhosis: Regenerative Nodules on CT [Figures 2-4-3 and

2-4-4]
Figure 2-4-3
Cirrhosis with high attenuation regenerating nodules (siderotic Cirrhosis with regenerating nodules
nodules) on noncontrast CT on CT
Imaging of Chronic Liver Disease 294 Gastrointestinal Radiology

Cirrhosis: Regenerative Nodules and HCC on MR Figure 2-4-5
Cirrhosis: Regenerative Nodules on MR [Figure 2-4-5]
Cirrhosis: Dysplastic Nodule

• Premalignant nodule
➢ Nodule with histologic evidence of dysplasia
• Very common
➢ Most undetectable on CT and MR
➢ Rarely seen on preoperative imaging
• Imaging appearance variable
• Detection of malignant transformation depends upon evidence
of angiogenesis
➢ Arterial enhancment
➢ Nodule in a nodule
➢ MR bright T1 with central dark signal
➢ MR dark T2 with central high signal
Cirrhosis: Dysplastic Nodule on MR
Cirrhosis: Hepatocellular Carcinoma [Figure 2-4-6] Cirrhosis with regenerating nodules

• Incidence in cirrhosis on T2 MR and gross photography
➢ 20% of hepatitis B and C cirrhosis
➢ 10% of alcoholic cirrhosis
• CT and MR equally accurate Figure 2-4-6
➢ 70%-75% of patients
➢ 35%-40% of lesions
• Late arterial phase imaging is key
➢ Maximum contrast volume
➢ 4 to 5 ml/sec injection rate
Cirrhosis: HCC Detection

• False positives
➢ Transient hepatic attenuation (intensity) difference
(THAD/THID)
➢ Focal confluent fibrosis (look for associated atrophy)
➢ Enhancing regenerative nodule
➢ Flash filling hemangioma
➢ Early enhancing pseudolesions (arterioportal shunting)
Cirrhosis: Transient Hepatic Attenuation (Intensity)

Difference
• Causes
➢ Portal vein obstruction
➢ Hepatic venous outflow obstruction
➢ Adjacent benign or malignant mass
➢ Arterioportal shunting
➢ Aberrant venous drainage
• Imaging features HCC in cirrhosis
➢ Typical locations
❖ Subcapsular
❖ Adjacent to falciform ligament
➢ No mass effect
➢ Straight margins
➢ Wedge shape

Cirrhosis: Focal Confluent Fibrosis
• Massive areas of fibrosis Figure 2-4-7
➢ Present in up to 30% of cirrhotic livers
• Typical location
➢ Anterior segment right lobe
➢ Medial segment left lobe
• Imaging
➢ Focal mass
➢ Wedge shape, radiating from
porta hepatis
➢ Capsular retraction
➢ Low density on noncontrast
CT
➢ Isodense with contrast or
irregular enhancement
➢ MR: low signal T1, high
signal T2
Cirrhosis: Hemangioma
Primary Biliary Cirrhosis Primary biliary cirrhosis

• Chronic cholestasis
➢ Probably immune mediated
• Middle-aged women
➢ Median age 50
➢ Female to male ratio 9:1
Primary Biliary Cirrhosis: CT [Figure 2-4-7]

• Global or segmental atrophy
• Nodules
• Fibrosis
➢ Lace-like pattern
➢ Segmental
➢ Focal confluent
➢ Often present before morphologic changes
• At risk for HCC
Fatty Liver Diseases

• Steatosis
➢ Alcohol-associated
➢ Nonalcoholic fatty liver disease (NAFLD)
• Steatohepatitis
➢ Alcoholic steatohepatitis
➢ Nonalcoholic steatohepatitis (NASH)
Steatosis
• Nomenclature
➢ Fatty infiltration, fatty change, nonalcoholic fatty liver disease (NAFLD)
• Very common
• Pathogenesis
➢ Abnormal fatty acid metabolism
➢ Insulin/glucagon imbalance
➢ Shift to lipogenesis

Steatosis: Etiology Figure 2-4-8
• ETOH
• Obesity, diabetes
• Malnutrition
• Parenteral nutrition
• Hepatitis, hepatotoxins,
chemotherapy, hyperlipidemia, drugs
• Malabsorption syndromes
• Idiopathic
Sonography of diffuse fatty infiltration
Steatosis and Steatohepatitis:
Clinical Features
• Asymptomatic
• Mild RUQ pain
• Mild hepatomegaly and/or tenderness on exam
• Mild transaminase elevation
Figure 2-4-9
Steatosis: Sonography
• Diffuse
➢ Echogenic parenchyma
➢ Poor visualization hepatic
vasculature
➢ Absorption of sound
• Focal
➢ Focal fat
➢ Focal sparing
Diffuse Steatosis: Sonography

[Figure 2-4-8]
Focal Steatosis: Sonography
Focal Fatty Sparing: Sonography

Focal fatty infiltration
Steatosis: CT
• Normal liver noncontrast CT
➢ 30 to 60 HU
➢ 8 to 10 HU > spleen
• Fatty liver noncontrast CT
➢ 10 HU < spleen
• Fatty liver contrast CT
➢ 25 HU < spleen
Steatosis in Celiac Disease
Nonalcoholic Steatohepatitis (NASH): CT
NASH with Cirrhosis: CT
Focal Steatosis: CT [Figure 2-4-9]

• Features of focal fat
➢ No mass effect
➢ Straight line margin
➢ No contour abnormality
• Often transient
• Common locations
➢ Falciform ligament
➢ Subcapsular
➢ Adjacent to porta hepatis
➢ Adjacent to gallbladder fossa

Focal Steatosis: Common Locations Figure 2-4-10
Focal Steatosis
vs. Focal Sparing
Focal Sparing [Figure 2-4-10]
Focal Steatosis: Transient
Steatosis: MR Focal fatty sparing

• Conventional spin echo typically
insensitive to fat deposition
• Chemical shift imaging
➢ Fat and water signal additive in-phase
➢ Fat signal subtracted from water signal out-of-phase
Figure 2-4-11
Steatosis: 1.5T MR [Figure 2-4-11]
Steatosis: MR [Figure 2-4-12]
Multifocal Steatosis: Pseudometastatic Disease
Budd-Chiari Syndrome
• Hepatic venous outflow obstruction
• Primary
➢ Membranous (web) obstruction of hepatic veins 1.5 T MR fat and water proton signal
• Secondary intensity
➢ Hypercoaguable states, infections, neoplasms, trauma
Budd-Chiari Syndrome: Clinical

• Acute fulminant disease
➢ Total obstruction
➢ Rare
• Subacute/Chronic
➢ Vague illness, 6 months duration
➢ Ascites
➢ Triad of hepatomegaly, ascites, pain Figure 2-4-12
Budd-Chiari Syndrome:
Pathophysiology
• Sinusoidal dilatation
• Increase sinusoidal pressure
• Centrolobular hepatocyte necrosis
• Centrolobular fibrosis
➢ Lobular collapse
➢ Nodular regeneration Focal fat on in-phase images and out-of-phase images
Budd-Chiari Syndrome: Pathology

• Acute
➢ Hepatomegaly
➢ Sinusoidal dilatation
➢ Hemorrhagic necrosis
• Chronic
➢ Fibrosis
➢ Cirrhosis

Budd Chiari Syndrome: Imaging Figure 2-4-13
• Vascular changes
➢ Hepatic vein stenosis
➢ Intravascular thrombus
➢ Web-like stenosis or narrowed IVC
➢ Intrahepatic collaterals
• Parenchymal changes
➢ Nonvisible hepatic veins
➢ Cirrhosis
➢ Nodular regenerative hyperplasia
Hepatic Venous Waveforms [Figure 2-4-13]
Budd-Chiari Syndrome:
Enhancement Patterns
• Noncontrast
➢ Heterogeneous hypodensity
❖ Hepatic parenchymal congestion
➢ Hyperdense thrombi
➢ Patchy enhancement
➢ Normal central hepatic, left lobe, and caudate lobe Normal hepatic vein (upper image)
enhancement and Budd Chiari (lower image)
➢ Late peripheral enhancement
Figure 2-4-14
[Figure 2-4-14]
Budd Chiari Syndrome

[Figure 2-4-15]
Budd Chiari Syndrome: MR

[Figure 2-4-16]
• Narrowed veins
• Intraluminal thrombus
Budd Chiari
• Collaterals
Figure 2-4-15
Figure 2-4-16
Budd-Chiari Budd Chiari

Budd Chiari from IVC Web
Budd Chiari and Nodular Regenerative Hyperplasia
Disorders of Iron Deposition

• Functional Classification
➢ Hemosiderosis
❖ Iron accumulation in the reticuloendothelial system
❖ Iron in the liver with no organ damage
➢ Hemochromatosis
❖ Iron in hepatocytes with eventual fibrosis and cirrhosis
❖ Two types
Hereditary hemochromatosis
Secondary hemochromatosis
❖ High risk for HCC
Hemosiderosis in Sickle Cell Anemia [Figure 2-4-17]
Hemochromatosis
• Hereditary hemochromatosis
➢ Increased intestinal absorption of iron
➢ Iron predominantly within hepatocytes
➢ Highest incidence of cirrhosis and HCC (14%)
• Secondary hemochromatosis
➢ Multiple transfusions
➢ Iron predominantly in the reticuloendothelial system
Hereditary Hemochromatosis (HHC): Clinical Features Figure 2-4-17

• Hyperpigmentation
• Diabetes mellitus (bronze diabetes)
• Hepatomegaly
• Chondrocalcinosis/osteoarthritis
• Cardiomyopathy
Hereditary Hemochromatosis
(HHC): Pathology
Hemosiderosis
Hemochromatosis: Increased CT
Attenuation (75-135 HU)
Figure 2-4-18
Hereditary Hemochromatosis
Increased Hepatic CT
Attenuation
• Differential Diagnosis
➢ Iron deposition
➢ Glycogen storage disease
➢ Amiodarone
➢ Wilson’s disease Hereditary Hemochromatosis
➢ Chronic arsenic poisoning
Hemochromatosis: MR
• T2*-gradient echo imaging is most sensitive
➢ Quantitate with liver:muscle ratio
• Decrease signal on T2-weighted images
➢ Hereditary = iron in liver and pancreas
➢ Secondary = iron in liver and spleen
Hereditary Hemochromatosis [Figure 2-4-18]

Secondary Hemochromatosis
Summary: Cirrhosis
• Endpoint of chronic liver disease
• Nodules
➢ Regenerative
➢ Dysplastic
➢ HCC
• HCC false positives
Summary: Fatty Infiltration

• Steatosis
• Steatohepatitis
Summary: Focal Steatosis

• No mass effect
• Straight line margins
• Typical Locations
➢ Subcapsular
➢ Falciform ligament
• Chemical shift MR
➢ Signal loss on out-of-phase images
Summary: Budd-Chiari Syndrome

• Hepatic venous outflow obstruction
• Sonography
• CT enhancement
➢ Early central
➢ Late peripheral
• MR
Summary: Disorders of Iron Overload

• Hemosiderosis
• Hemochromatosis
➢ Hereditary
➢ Secondary
References
Cirrhosis
1. Baron RL, Peterson MS. From the RSNA refresher courses: screening the cirrhotic liver for hepatocellular carcinoma
with CT and MR imaging: opportunities and pitfalls. Radiographics 2001; 21 Spec No:S117-132.
2. Brancatelli G, Baron RL, Peterson MS, Marsh W. Helical CT screening for hepatocellular carcinoma in patients with
cirrhosis: frequency and causes of false-positive interpretation. AJR Am J Roentgenol 2003; 180(4):1007-1014.
3. Dodd GD, 3rd, Baron RL, Oliver JH, 3rd, Federle MP. Spectrum of imaging findings of the liver in end-stage cirrhosis:
Part II, focal abnormalities. AJR Am J Roentgenol 1999; 173(5):1185-1192.
4. Dodd GD, 3rd, Baron RL, Oliver JH, 3rd, Federle MP. Spectrum of imaging findings of the liver in end-stage cirrhosis:
part I, gross morphology and diffuse abnormalities. AJR Am J Roentgenol 1999; 173(4):1031-1036.
5. Hussain HK, Syed I, Nghiem HV, et al. T2-weighted MR imaging in the assessment of cirrhotic liver. Radiology
2004; 230(3):637-644.
6. Ohtomo K, Baron RL, Dodd GD, 3rd, et al. Confluent hepatic fibrosis in advanced cirrhosis: appearance at CT.
Radiology 1993; 188(1):31-35.
7. Ohtomo K, Itai Y, Ohtomo Y, Shiga J, Iio M. Regenerating nodules of liver cirrhosis: MR imaging with pathologic
correlation. AJR Am J Roentgenol 1990; 154(3):505-507.
8. Shimizu A, Ito K, Koike S, Fujita T, Shimizu K, Matsunaga N. Cirrhosis or chronic hepatitis: evaluation of small
(<or=2-cm) early-enhancing hepatic lesions with serial contrast-enhanced dynamic MR imaging. Radiology 2003;
226(2):550-555.

Steatosis/Steatohepatitis
1. Yoshikawa J, Matsui O, Takashima T, et al. Focal fatty change of the liver adjacent to the falciform ligament: CT and
sonographic findings in five surgically confirmed cases. AJR Am J Roentgenol 1987; 149:491-494.
2. Jain KA, McGahan JP. Spectrum of CT and sonographic appearance of fatty infiltration of the liver. Clin Imaging
1993; 17:162-168.
3. Siegelman ES. MR imaging of diffuse liver disease. Hepatic fat and iron. Magn Reson Imaging Clin N Am 1997;
5:347-365.
4. Jacobs JE, Birnbaum BA, Shapiro MA, et al. Diagnostic criteria for fatty infiltration of the liver on contrast-enhanced
helical CT. AJR Am J Roentgenol 1998; 171:659-664.
5. Kroncke TJ, Taupitz M, Kivelitz D, et al. Multifocal nodular fatty infiltration of the liver mimicking metastatic disease
on CT: imaging findings and diagnosis using MR imaging. Eur Radiol 2000; 10:1095-1100.
6. Brunt EM. Nonalcoholic steatohepatitis: definition and pathology. Semin Liver Dis 2001; 21:3-16.
7. Siegelman ES, Rosen MA. Imaging of hepatic steatosis. Semin Liver Dis 2001; 21:71-80.
8. Kemper J, Jung G, Poll LW, Jonkmanns C, Luthen R, Moedder U. CT and MRI findings of multifocal hepatic steatosis
mimicking malignancy. Abdom Imaging 2002; 27:708-710.
1. Noone TC, Semelka RC, Siegelman ES, et al. Budd-Chiari syndrome: spectrum of appearances of acute, subacute,
and chronic disease with magnetic resonance imaging. J Magn Reson Imaging 2000; 11:44-50.
4. Brancatelli G, Federle MP, Grazioli L, Golfieri R, Lencioni R. Large regenerative nodules in Budd-Chiari syndrome
and other vascular disorders of the liver: CT and MR imaging findings with clinicopathologic correlation. AJR Am
J Roentgenol 2002; 178:877-883.
5. Maetani Y, Itoh K, Egawa H, et al. Benign hepatic nodules in Budd-Chiari syndrome: radiologic-pathologic correlation
with emphasis on the central scar. AJR Am J Roentgenol 2002; 178:869-875.
Disorders of Iron Deposition

1. Bonkovsky HL: Disorders of iron overload. In Bloomer JR, Goodman ZD, Ishak KG (eds): Clinical and pathologoical
correlations in liver disease: approaching the next millennium. Washington, DC: Armed Forces Institute of Pathology,
1998
2. Gandon Y: Iron, liver, and MRI. http://www.radio.univ-rennes1.fr/Sources/EN/Hemo.html, 2001
3. Siegelman ES, Mitchell DG, Semelka RC: Abdominal iron deposition: metabolism, MR findings, and clinical
importance. Radiology 199:13, 1996

Benign Biliary Disease
Objectives
• Congenital Disorders
➢ Caroli disease
➢ Choledochal cyst
➢ Polycystic Liver Disease
• Inflammatory Disorders
➢ AIDS-related cholangiopathy
➢ Recurrent Pyogenic Cholangitis
➢ Acute Pyogenic Cholangitis
Differential Diagnosis
• Obstructive biliary dilatation
• Caroli disease
• Choledochal cyst
• Polycystic liver disease
• Cholangitis
➢ RPC, Pyogenic Figure 2-5-1
41-year-old male with history of renal
stones and diagnosis of medullary
sponge kidney presents with abdominal
pain, sepsis, elevated LFTs [Figure 2-5-1]
Obstructive Biliary Dilatation

• Tubular dilatation
• Diffuse dilatation proximal to the obstruction
• Abrupt termination at level of obstruction
Congenital Disorders
• Caroli disease
• Polycystic liver disease
Saccular and fusiform biliary dilatation in
Caroli Disease Caroli disease
• Autosomal recessive
• Secondary to ductal plate malformation (DPM)
• Associated with renal disorders
➢ ARPCKD, ADPCKD
➢ Medullary sponge kidney
➢ Medullary cystic disease
Ductal Plate
• Embryologic precursor of intrahepatic bile ducts
Ductal Plate Malformation

• Abnormal development of intrahepatic bile ducts
• Lack of ductal plate remodeling
➢ Persistence of embryonic structures (DPM)
➢ Segmental dilatation
➢ Destructive inflammation/fibrosis
• Spectrum of diseases
➢ Small interlobular ducts: Congenital hepatic fibrosis (CHF)
➢ Large intrahepatic ducts: Caroli disease
➢ All ducts: Caroli syndrome (CHF and Caroli disease)
Gastrointestinal Radiology 303 Benign Biliary Disease

Intrahepatic Duct Embryology: Ductal Plate
Single layer of ductal Double layer of Fusion and

plate cells ductal plate cells absorption of ductal
plate cells
Normal ductal plate development of the intrahepatic bile ducts
Caroli Disease: Clinical Features

• Presentation at any age (mean age 37 years)
• Pain, fever, jaundice
➢ Recurrent cholangitis
➢ Stone formation
• Liver failure
➢ Fibrosis
Caroli Disease: Complications Normal ductal plate remodeling

• Recurrent biliary stones results in intercommunicating
• Recurrent cholangitis intrahepatic bile ducts
• Hepatic abscess surrounding a normal portal
• Fibrosis/cirrhosis tract.
• Cholangiocarcinoma (7%) Ductal plate malformation
results in biliary duct ectasia
Caroli Disease: Radiologic Features and fibrosis surrounding the
• Intrahepatic duct dilatation portal tract
➢ Distribution: segmental (83%) or diffuse (17%)
➢ Shape: saccular (76%) or fusiform (24%)
➢ Central dot sign
• Extrahepatic duct dilatation
➢ 53% of cases Figure 2-5-4
➢ Secondary to cholangitis and stone passage
• Cirrhosis
Caroli Disease: “Central Dot Sign” [Figure 2-5-4]
Caroli disease showing saccular

biliary dilatation and
the central dot sign
Benign Biliary Disease 304 Gastrointestinal Radiology

Caroli Syndrome Cirrhosis, Portal Hypertension Figure 2-5-5
[Figure 2-5-5]
Caroli Disease Cholangiography
Caroli Disease: Segmental
Caroli Disease: Hepatic Abscess
Caroli Disease: Intrahepatic Lithiasis
Caroli Disease: Intraductal Cholangiocarcinoma
Caroli Disease: Cholangiocarcinoma

• Enhancing intraductal masses
• Focal strictures
➢ Shoulders
➢ Irregular tapering
• Infiltrating masses
Caroli disease in two different patients
Choledochal Cyst showing diffuse fusiform and saccular
• Congenital dilatation of the bile ducts dilatation
• Association with anomalous pancreatico-biliary junction (APBJ)
Choledochal Cyst: Figure 2-5-6

Clinical Features
• Presentation at any age
(mean, 17 years)
➢ F>M
• Clinical presentation
➢ Pain, jaundice, palpable
mass
• Complications
➢ Stones
➢ Cholangitis
➢ Malignancy
Normal pancreaticobiliary junction showing union of the ducts in the
Choledochal Cyst: sphincter complex, which is located in the duodenal wall. There may be a
Etiology [Figures 2-5-6 and 2-5-7] common channel (ampulla) or not
• Normal Pancreaticobiliary
Junction
➢ Sphincter complex encircles distal CBD and PD
Figure 2-5-7
➢ 80% to 90% have a common channel (4-5 mm
• Anomalous Junction (APBJ)
➢ Union of CBD and PD outside of duodenum and sphincter
complex
➢ Reflux of pancreatic enzymes into CBD)
Choledochal Cyst: Pathologic Features

• Extrahepatic duct dilatation
• Mural thickening
• Normal epithelium
Anomalous pancreaticobiliary
junction showing union of the
common bile duct and pancreatic
duct proximal to the duodenal wall
and sphincter complex

Choledochal Cyst: Todani Classification [Figure 2-5-8] Figure 2-5-8
Todani Type I
Todani Type II: Diverticulum
Todani Type III:

Choledochocele
Tubulovillous Adenoma
Todani Type IV [Figure 2-5-9]
Todani Type V: Caroli Disease
Is Todani Type V Caroli

Disease?
➢ Congenital, not inherited
➢ Extrahepatic bile duct
dilatation with varying degrees Todani classification of choledochal cysts
of proximal dilatation
➢ Surgical therapy with biliary reconstruction
• Caroli disease Figure 2-5-9
➢ Congenital, inherited
➢ Intrahepatic +/- extrahepatic dilatation
➢ Liver biopsy shows DPM
➢ Medical therapy (surgery for complications)
Malignancy and Choledochal Cyst

• Adenocarcinoma most common
• Locations
➢ Within the choledochal cyst
➢ Gallbladder
➢ Biliary tract
Malignancy and Choledochal Cyst
Polycystic Liver Disease

Todani Type IV choledochal cyst showing central
• Two forms
intrahepatic and extrahepatic duct dilatation on CT.
➢ Isolated to the liver
The cholangiogram shows an anomalous
➢ Occurring with ADPCKD
pancreaticobiliary junction as well as the extent of
• Bile duct cysts
duct dilatation
➢ Secondary to von Meyenberg complexes
➢ Von Meyenberg complex is DPM at the lowest level
Polycystic Liver Disease - Radiologic Features

• Multiple liver cysts
➢ May have internal hemorrhage
• Normal bile ducts
• Associated feature of ADPCKD
➢ Renal cysts
➢ Pancreatic cysts
➢ Thyroid cysts
➢ Seminal vesicle cysts in males

Polycystic Liver Disease Figure 2-5-10
Polycystic Liver Disease in ADPCKD [Figure 2-5-10]
Polycystic Liver Disease without ADPCKD

• Bile ducts
➢ Displaced, but normal
➢ Rare, mucosal irregularity
41-year-old male with history of renal stones and

diagnosis of medullary sponge kidney presents with
abdominal pain, sepsis, elevated LFT’s
• Obstructive biliary dilatation
• Caroli disease
• Polycystic liver disease Polycystic liver disease occurring in
• Cholangitis ADPCKD
➢ RPC, Pyogenic
Caroli Disease Figure 2-5-11
Summary: Congenital
Disorders
• Exclude obstructive
dilatation
• Congenital disorders
➢ Caroli disease
❖ Intrahepatic
➢ Choledochal cyst
❖ Extrahepatic
➢ Polycystic liver disease
❖ Noncommunicating Primary sclerosing cholangitis
cysts
40-year-old woman with elevated LFT’s

[Figure 2-5-11]
Figure 2-5-12
• Cholangitis
➢ Primary sclerosing
➢ AIDS-related
➢ Recurrent pyogenic
➢ Acute pyogenic cholangitis
• Neoplasm
Inflammatory Disorders
• Primary sclerosing cholangitis
• AIDS-related cholangiopathy
• Recurrent pyogenic cholangitis
• Acute pyogenic cholangitis
Primary Sclerosing Cholangitis [Figure 2-5-12]

• Cholestatic liver disease
• Fibrosing inflammation Illustration showing disease distribution, mural
• Diagnosis thickening, and inflammatory changes of primary
➢ Liver biopsy sclerosing cholangitis
➢ Cholangiogram

PSC - Imaging Figure 2-5-13
• Thickened duct wall
➢ Asymmetric or circumferential
➢ 2 to 5 mm
• Hepatic parenchymal changes
➢ Cirrhosis
➢ Periportal fibrosis
➢ Discontinuous duct dilatation
➢ Portal hypertension
• Distribution
➢ Intrahepatic 100%
CT of primary sclerosing cholangitis showing discontinuous bile
➢ Extrahepatic 70%
duct dilatation, mural thickening of the common hepatic duct,
➢ Cystic duct 30%
and hepatoduodenal ligament adenopathy
➢ Pancreatic duct (rare)
• Other features
➢ Gallbladder disease (40%) Figure 2-5-14
➢ Ductal stones (8%)
➢ Adenopathy
➢ Neoplasm
PSC: Sonographic Features
PSC: CT Features [Figure 2-5-13]
PSC: Cholangiography [Figure 2-5-14]

• Beading
• Pruned-tree
• Mural irregularity
• Diverticula
MRCP
PSC: Cholangiocarcinoma
• Stricture (90%)
➢ Long strictures (>1cm) ERCP of primary sclerosing cholangitis showing beading and
➢ Completely obstructing strictures pruning of the intrahepatic bile ducts. The extrahepatic bile
➢ Associated mass duct shows mural irregularity with focal stricture formation
• Multicentric (10%)
• Polypoid mass
AIDS Cholangiopathy [Figure 2-5-15] Figure 2-5-15

• Group of disorders
➢ Sclerosing cholangitis
➢ Papillary stenosis
➢ Acalculous cholecystitis
➢ Cryptosporidium
➢ Cytomegalovirus
• Declining incidence
➢ HAART therapy
AIDS Cholangiopathy
• Cholangiographic features
➢ Beading
➢ Pruning
➢ Mural irregularity
➢ Filling defects (granulation tissue)
➢ Papillary stenosis (papillitis)
➢ No EHD stenosis or diverticula
Illustration of AIDS cholangiopathy showing
disease distribution

AIDS Cholangiopathy [Figure 2-5-16] Figure 2-5-16
AIDS Cholangiopathy
• Sonographic features
➢ Gallbladder wall thickening
❖ Acalculous cholecystitis
➢ Bile duct wall thickening
➢ Hyperechoic nodule in distal
CBD (papillitis)
AIDS Cholangiopathy
Acalculous cholecystitis
[Figure 2-5-17]
Recurrent Pyogenic
Cholangitis (RPC) [Figure 2-5-18]
• Clinical syndrome
➢ Pigmented stones
➢ Recurrent infection
➢ Biliary parasites
➢ Malnutrition AIDS cholangiopathy with papillary stenosis
➢ Portal bacteremia
• Complications Figure 2-5-17
➢ Biliary cirrhosis
➢ Cholangiocarcinoma
RPC: Imaging [Figure 2-5-19]

• Stones, sludge
• Duct dilatation
• Left lobe predominant
• Parenchymal changes
➢ Atrophy
➢ Fatty change Acalculous cholecystitis in AIDS
➢ Altered enhancement
➢ Abscess Figure 2-5-18
RPC
Acute Pyogenic Cholangitis [Figure 2-5-20]

• Almost always post-obstructive
• Polymicrobial
• Etiology
➢ Stones
➢ Anastomotic stricture
➢ Papillary stenosis
➢ Carcinoma
➢ Underlying biliary disease
Acute Pyogenic Cholangitis

• Imaging Features
➢ Duct dilatation
➢ Obstructive lesion
➢ Echogenic bile
➢ Mural irregularity
➢ Hepatic abscess Illustration of recurrent pyogenic cholangitis
showing typical disease distribution

Figure 2-5-19
Recurrent pyogenic cholangitis with intrahepatic lithiasis and duct dilatation
Figure 2-5-20
Acute Pyogenic Cholangitis with

Microabscesses
40-year-old woman with elevated LFT’s
• Cholangitis
➢ Primary sclerosing
➢ AIDS-related
➢ Recurrent pyogenic
➢ Acute pyogenic cholangitis
• Neoplasm
Primary Sclerosing Cholangitis
Summary
• PSC
➢ Fibrosis
• AIDS cholangiopathy
➢ Papillary stenosis Illustration showing typical manifestations of acute
➢ Acalculous cholecystitis pyogenic cholangitis
• RPC
➢ Stones
➢ Focal dilatation
• Pyogenic cholangitis
➢ Obstruction
References
Caroli Disease
1. Choi BI, Yeon KM, Kim SH, et al: Caroli disease: central dot sign in CT. Radiology 174:161, 1990
2. Desmet VJ: Ludwig symposium on biliary disorders--part I. Pathogenesis of ductal plate abnormalities. Mayo
Clinic Proceedings 73:80, 1998
3. Krause D, Cercueil JP, Dranssart M, et al: MRI for evaluating congenital bile duct abnormalities. J Comput Assist
Tomogr 26:541, 2002
4. Levy AD, Rohrmann CA, Jr., Murakata LA, et al: Caroli's disease: radiologic spectrum with pathologic correlation.
AJR 179:1053, 2002
5. Marchal GJ, Desmet VJ, Proesmans WC, et al: Caroli disease: high-frequency US and pathologic findings.
Radiology 158:507, 1986

6. Miller WJ, Sechtin AG, Campbell WL, et al: Imaging findings in Caroli's disease. AJR 165:333, 1995
7. Pavone P, Laghi A, Catalano C, et al: Caroli's disease: evaluation with MR cholangiopancreatography (MRCP).
Abdom Imaging 21:117, 1996
8. Pavone P, Laghi A, Catalano C, et al: Caroli's disease: evaluation with MR cholangiography. AJR 166:216, 1996
Choledochal Cyst
1. Babbitt DP, Starshak RJ, Clemett AR: Choledochal cyst: a concept of etiology. AJR 119:57, 1973
2. Govil S, Justus A, Korah I, et al: Choledochal cysts: evaluation with MR cholangiography. Abdom Imaging
23:616, 1998
3. Levy AD, Rohrmann CA, Jr.: Biliary cystic disease. Curr Probl Diagn Radiol 32:233, 2003
4. Liu CL, Fan ST, Lo CM, et al: Choledochal cysts in adults. Arch Surg 137:465, 2002
5. O'Neill JA, Jr.: Choledochal cyst. Curr Probl Surg 29:361, 1992
6. Savader SJ, Benenati JF, Venbrux AC, et al: Choledochal cysts: classification and cholangiographic appearance.
AJR 156:327, 1991
7. Savader SJ, Venbrux AC, Benenati JF, et al: Choledochal cysts: role of noninvasive imaging, percutaneous
transhepatic cholangiography, and percutaneous biliary drainage in diagnosis and treatment. J Vasc Interv Radiol
2:379, 1991
8. Todani T, Watanabe Y, Fujii T, et al: Anomalous arrangement of the pancreatobiliary ductal system in patients with
a choledochal cyst. Am J Surg 147:672, 1984
9. Todani T, Watanabe Y, Narusue M, et al: Congenital bile duct cysts: Classification, operative procedures, and
review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134:263, 1977
10. Wearn FG, Wiot JF: Choledochocele: not a form of choledochal cyst. J Can Assoc Radiol 33:110, 1982

1. Dranssart M, Cognet F, Mousson C, et al: MR cholangiography in the evaluation of hepatic and biliary
abnormalities in autosomal dominant polycystic kidney disease: study of 93 patients. J Comput Assist Tomogr
26:237, 2002
2. Grunfeld JP, Albouze G, Jungers P, et al: Liver changes and complications in adult polycystic kidney disease. Adv
Nephrol Necker Hosp 14:1, 1985
3. Gupta S, Seith A, Dhiman RK, et al: CT of liver cysts in patients with autosomal dominant polycystic kidney
disease. Acta Radiol 40:444, 1999
4. Itai Y, Ebihara R, Eguchi N, et al: Hepatobiliary cysts in patients with autosomal dominant polycystic kidney
disease: prevalence and CT findings. AJR 164:339, 1995
5. Pirson Y, Lannoy N, Peters D, et al: Isolated polycystic liver disease as a distinct genetic disease, unlinked to
polycystic kidney disease 1 and polycystic kidney disease 2. Hepatology 23:249, 1996
6. Segal AJ, Spataro RF: Computed tomography of adult polycystic disease. J Comput Assist Tomogr 6:777, 1982
Primary Sclerosing Cholangitis

1. Ament AE, Haaga JR, Wiedenmann SD, et al: Primary sclerosing cholangitis: CT findings. J Comput Assist
Tomogr 7:795, 1983
2. Brandt DJ, MacCarty RL, Charboneau JW, et al: Gallbladder disease in patients with primary sclerosing
cholangitis. AJR Am J Roentgenol 150:571, 1988
3. Campbell WL, Ferris JV, Holbert BL, et al: Biliary tract carcinoma complicating primary sclerosing cholangitis:
evaluation with CT, cholangiography, US, and MR imaging. Radiology 207:41, 1998
4. Campbell WL, Peterson MS, Federle MP, et al: Using CT and cholangiography to diagnose biliary tract carcinoma
complicating primary sclerosing cholangitis. AJR Am J Roentgenol 177:1095, 2001
5. Dodd GD, 3rd, Baron RL, Oliver JH, 3rd, et al: End-stage primary sclerosing cholangitis: CT findings of hepatic
morphology in 36 patients. Radiology 211:357, 1999
6. Fulcher AS, Turner MA, Franklin KJ, et al: Primary sclerosing cholangitis: evaluation with MR cholangiography-a
case-control study. Radiology 215:71, 2000
7. Gulliver DJ, Baker ME, Putnam W, et al: Bile duct diverticula and webs: nonspecific cholangiographic features of
primary sclerosing cholangitis. AJR Am J Roentgenol 157:281, 1991
8. Ito K, Mitchell DG, Outwater EK, et al: Primary sclerosing cholangitis: MR imaging features. AJR Am J
Roentgenol 172:1527, 1999

9. Lumsden AB, Alspaugh JP: Cholangiocarcinoma complicating primary sclerosing cholangitis: cholangiographic
appearances. Radiology 158:856, 1986
10. MacCarty RL, LaRusso NF, Wiesner RH, et al: Primary sclerosing cholangitis: findings on cholangiography and
pancreatography. Radiology 149:39, 1983
11. Majoie CB, Smits NJ, Phoa SS, et al: Primary sclerosing cholangitis: sonographic findings. Abdom Imaging
20:109, 1995
12. May GR, Bender CE, LaRusso NF, et al: Nonoperative dilatation of dominant strictures in primary sclerosing
cholangitis. AJR Am J Roentgenol 145:1061, 1985
13. Olsson R, Danielsson A, Jarnerot G, et al: Prevalence of primary sclerosing cholangitis in patients with ulcerative
colitis. Gastroenterology 100:1319, 1991
14. Teefey SA, Baron RL, Rohrmann CA, et al: Sclerosing cholangitis: CT findings. Radiology 169:635, 1988
15. Teefey SA, Baron RL, Schulte SJ, et al: Patterns of intrahepatic bile duct dilatation at CT: correlation with
obstructive disease processes. Radiology 182:139, 1992
16. Williams SM, Harned RK: Hepatobiliary complications of inflammatory bowel disease. Radiol Clin North Am
25:175, 1987
AIDS Cholangiopathy
1. Chen XM, LaRusso NF: Cryptosporidiosis and the pathogenesis of AIDS-cholangiopathy. Semin Liver Dis 22:277,
2002
2. Collins CD, Forbes A, Harcourt-Webster JN, et al: Radiological and pathological features of AIDS-related
polypoid cholangitis. Clin Radiol 48:307, 1993
3. Da Silva F, Boudghene F, Lecomte I, et al: Sonography in AIDS-related cholangitis: prevalence and cause of an
echogenic nodule in the distal end of the common bile duct. AJR Am J Roentgenol 160:1205, 1993
4. Defalque D, Menu Y, Girard PM, et al: Sonographic diagnosis of cholangitis in AIDS patients. Gastrointest Radiol
14:143, 1989
5. Dolmatch BL, Laing FC, Ferderle MP, et al: AIDS-related cholangitis: radiographic findings in nine patients.
Radiology 163:313, 1987
Recurrent Pyogenic Cholangitis

1. Chan FL, Man SW, Leong LL, et al: Evaluation of recurrent pyogenic cholangitis with CT: analysis of 50 patients.
Radiology 170:165, 1989
2. Federle MP, Cello JP, Laing FC, et al: Recurrent pyogenic cholangitis in Asian immigrants. Use of
ultrasonography, computed tomography, and cholangiography. Radiology 143:151, 1982
3. Jeyarajah DR: Recurrent Pyogenic Cholangitis. Curr Treat Options Gastroenterol 7:91, 2004
4. Kim MJ, Cha SW, Mitchell DG, et al: MR imaging findings in recurrent pyogenic cholangitis. AJR Am J
5. Okuno WT, Whitman GJ, Chew FS: Recurrent pyogenic cholangiohepatitis. AJR Am J Roentgenol 167:484, 1996
6. Park MS, Yu JS, Kim KW, et al: Recurrent pyogenic cholangitis: comparison between MR cholangiography and
direct cholangiography. Radiology 220:677, 2001

Biliary Neoplasms
Objectives
• Biliary adenocarcinoma
➢ Intrahepatic cholangiocarcinoma
➢ Intraductal cholangiocarcinoma
➢ Hilar (Klatskin) cholangiocarcinoma
➢ Extrahepatic duct adenocarcinoma
➢ Benign strictures
➢ Other neoplasms
Biliary Adenocarcinoma
• Incidence in U.S.
➢ ~2000 to 2500 cases per year
• Incidence worldwide
➢ Up to 10 times greater in Asian countries
➢ 2:1
• High risk groups
➢ Autoimmune diseases
❖ PSC, ulcerative colitis, primary biliary cirrhosis
➢ Congenital anatomic anomalies
❖ Caroli, choledochal cyst, anomalous pancreaticobiliary junction
❖ Abnormal tumor suppressor genes, FAP, NF1
➢ Infection
❖ Biliary parasites, recurrent pyogenic cholangitis
➢ Jaundice
➢ Pain
➢ Fever if secondary cholangitis
• Moderately to well differentiated
• Desmoplastic stroma
• Infiltrative margins
➢ NO CAPSULE
• Dismal 5-year survival
• < 20% resectable at diagnosis
• Curative resection
➢ Tumor free margins
➢ “No touch” technique
• Intrahepatic Cholangiocarcinoma
• Intraductal Cholangiocarcinoma
• Hilar Cholangiocarcinoma
Gastrointestinal Radiology 313 Biliary Neoplasms

Biliary Adenocarcinoma Figure 2-6-1
• Extrahepatic bile duct adenocarcinoma
Intrahepatic Cholangiocarcinoma:
Pathologic Features
• Solitary, large mass
• No capsule
• Dense fibrous stroma
• No necrosis or hemorrhage
• Multifocal mass
• Satellite lesions
• Intrahepatic metastasis
Intrahepatic Cholangiocarcinoma [Figure 2-6-1]

• Delayed enhancement
• Peripheral biliary dilatation
• Capsular contraction
➢ Metastasis
➢ HCC
➢ Gallbladder adenocarcinoma
➢ Rare, sarcoma
• Identifying key features of ICC
➢ Evidence of fibrous stroma
➢ Contrast enhancement pattern
Intraductal Cholangiocarcinoma [Figure 2-6-2] Intrahepatic cholangiocarcinoma with

• Rare delayed enhancement and capsular
• Intrabiliary mass contraction
• Biliary diliatation peripheral to the mass
Hilar Cholangiocarcinoma: Klatskin Tumor

• Tumors arising at or near the confluence of the right and left hepatic ducts
• Most common site of biliary adenocarcioma
• Aggressive biologic behavior Figure 2-6-2
Hilar Cholangiocarcinoma: Klatskin Tumor
• Imaging features
• Anatomic location
Hilar Cholangiocarcinoma: “Klatskin Tumor”

➢ Duct dilatation
➢ Ill-defined mass
➢ Lobar atrophy
Hilar Cholangiocarcinoma: Imaging Features [Figure 2-6-3]

• Dilated ducts
➢ Discontinuous ducts
• Poorly defined mass
➢ Poor visibility of tumor mass
➢ Minimal tumor enhancement on CT (50% of cases)
➢ More likely to enhance on MR Intraductal cholangiocarcinoma.
➢ Parenchymal invasion (segment IV) 30% of cases There is an intrabiliary mass on the
• Lobar or segmental atrophy CT and corresponding gross
➢ Secondary to vascular compromise photograph
Biliary Neoplasms 314 Gastrointestinal Radiology

Hilar Cholangiocarcinoma [Figure 2-6-4] Figure 2-6-3
Hilar Cholangiocarcinoma [Figure 2-6-5]

• Cholangiographic features of malignant strictures
➢ Evidence of mass effect
➢ Irregular or abrupt tapering at obstruction
• Limitations of MRCP
➢ Spatial resolution
➢ Evaluation of secondary ducts
Hilar Cholangiocarcinoma
Role of Preoperative Imaging
• Determination of resectablility
• Surgical planning
➢ Bismuth-Corlette classification1
➢ Define extent of duct involvement
1Bismuth H, Corlette MB. Surg Gynecol Obstet 1975, 140: 170-178.
Classic sonographic appearance
Hilar Cholangiocarcinoma: Unresectability of hilar cholangiocarcinoma
• Bilateral tumor extension showing biliary dilatation and an
➢ Into secondary ducts ill-defined hilar mass
➢ Into hepatic parenchyma
➢ Hepatic artery or portal vein
• Occlusion main portal vein Figure 2-6-4
• N2 nodes
• Distant mets
• Medically unfit patients
Bismuth-Corlette: Type I
• Tumor below confluence
Bismuth-Corlette: Type II Hilar cholangiocarcinoma. Ill-defined mass adjacent to bile duct stent
[Figure 2-6-8] and extending into hepatoduodenal ligament
• Tumor at confluence
Bismuth-Corlette: Type IIIa and IIIb [Figure 2-6-9] Figure 2-6-5

• Tumor extends to right or left
hepatic ducts
• Hemiliver resection
➢ Preoperative PV embolism
Bismuth-Corlette: Type IIIa
Bismuth-Corlette: Type IV
[Figures 2-6-10 and 2-6-11]
• Tumor in bilateral ducts
Hilar cholangiocarcinoma on MRCP and percutaneous transhepatic
cholangiography

Bismuth Corlette Type I hilar Bismuth Corlette Type II hilar

cholangiocarcinoma. Tumor is cholangiocarcinoma. Tumor involves
below the confluence of the right the confluence
and left hepatic ducts
Bismuth Corlette Type I
Figure 2-6-10
Figure 2-6-9
Bismuth Colette Type IV hilar

cholangiocarcinoma. Tumor involves
bilateral intrahepatic ducts
Figure 2-6-11
Bismuth Colette Type IIIa (upper)

and IIIb (lower) hilar
cholangiocarcinoma. Tumor involves
the right (IIIa) or left (IIIb) hepatic
duct
Bismuth Corlette Type IV

EHBD Adenocarcinoma [Figure 2-6-12] Figure 2-6-12
• Variable morphology
➢ Diffusely infiltrating
➢ Polypoid
➢ Nodular
➢ Constricting (scirrhous)
EHBD Adenocarcinoma: Imaging

• Biliary obstruction
• Tumor
➢ Intraluminal mass
➢ Stenosis
➢ Complete obstruction
Papillary Adenocarcinoma [Figure 2-6-13]
EHBD Adenocarcinoma vs. Benign Stricture

• Malignant
➢ Duct abruptly terminates at stricture
• Benign
➢ Duct tapers to stricture
Baron, RL. Radiol Clin N Am
Morphologic types of extrahepatic
1991. 29:1237. Figure 2-6-13 duct adenocarcinoma
Papillary adenocarcinoma of the extrahepatic bile duct
Malignant Stricture [Figures 2-6-14 and 2-6-15]
EHBD Adenocarcinoma
Figure 2-6-14
Figure 2-6-15
Cholangiographic and CT features of a malignant

Malignant stricture stricture

Benign Stricture [Figures 2-6-16 and 2-6-17] Figure 2-6-16
Pancreatitis with Stricture
• Post-inflammatory benign strictures
➢ Pancreatitis
➢ Post radiation or chemotherapy
• Inflammatory strictures
➢ AIDS cholangiopathy
➢ Recurrent pyogenic cholangitis
➢ Biliary parasites
• Other neoplasms
➢ Gallbladder adenocarcinoma
➢ Pancreatic adenocarcinoma
➢ Metastasis
➢ Granular cell tumor
➢ Biliary papillomatosis
Granular Cell Tumor [Figure 2-6-18]

• Benign tumors of Schwann cell origin
• 90% of patients are females, mean age 34 years
• Location: Benign stricture
➢ CBD (50%)
➢ Cystic duct (37%)
➢ CHD (11%)
➢ Gallbladder (4%) Figure 2-6-17
➢ Intrahepatic ducts (4%)
• Infiltrative lesions that produce short annular
strictures
Figure 2-6-18
Cholangiographic and CT features of a benign

stricture
Granular cell tumor

Biliary Papillomatosis
• Multiple and recurrent adenomas of the biliary tract
• Men and women in the 6th and 7th decades
• Clinical presentation: biliary obstruction, cholangitis
• Local recurrence and malignant transformation common
Summary
• Biliary Adenocarcinomas
➢ Uncommon
• Peripheral intrahepatic cholangiocarcinoma
➢ Mass forming tumors
➢ Delayed, patchy enhancement
➢ Look for imaging evidence of fibrosis
• Intraductal cholangiocarcinoma
➢ Rare
➢ Intraductal masses
➢ Biliary obstruction
• Hilar cholangiocarcinoma
➢ Most common subtype
➢ Look for discontinuous biliary dilatation
➢ Determination of resectablility
• Extrahepatic duct adenocarcinoma
➢ Must differentiate from a benign stricture
Summary: Benign vs. Malignant

• Malignant stricture
➢ Abrupt change
• Benign stricture
➢ Tapering
Klatskin / PSC
Summary: Granular Cell Tumor

• Benign Neoplasm
➢ Young, women
➢ True mimic for carcinoma
References
1. Choi BI, Lee JM, Han JK: Imaging of intrahepatic and hilar cholangiocarcinoma. Abdom Imaging 29:548, 2004
2. Ishak KG, Goodman ZD, Stocker JT: Tumors of the Liver and Intrahepatic Bile Ducts. Washington, D.C.: Armed
Forces Institute of Pathology under the auspices of Universities Associated for Research and Education in
Pathology For sale by the Armed Forces Institute of Pathology, 2001
3. Kim TK, Choi BI, Han JK, et al: Peripheral cholangiocarcinoma of the liver: two-phase spiral CT findings.
Radiology 204:539, 1997
4. Lim JH: Cholangiocarcinoma: morphologic classification according to growth pattern and imaging findings. AJR
Am J Roentgenol 181:819, 2003
5. Tani K, Kubota Y, Yamaguchi T, et al: MR imaging of peripheral cholangiocarcinoma. J Comput Assist Tomogr
15:975, 1991
6. Vilgrain V, Van Beers BE, Flejou JF, et al: Intrahepatic cholangiocarcinoma: MRI and pathologic correlation in 14
patients. J Comput Assist Tomogr 21:59, 1997
7. Worawattanakul S, Semelka RC, Noone TC, et al: Cholangiocarcinoma: spectrum of appearances on MR images
using current techniques. Magn Reson Imaging 16:993, 1998
8. Yalcin S: Diagnosis and management of cholangiocarcinomas: a comprehensive review. Hepatogastroenterology
51:43, 2004
9. Zhang Y, Uchida M, Abe T, et al: Intrahepatic peripheral cholangiocarcinoma: comparison of dynamic CT and
dynamic MRI. J Comput Assist Tomogr 23:670, 1999

Hilar Cholangiocarcinoma
1. Arepally A, Georgiades C, Hofmann LV, et al: Hilar cholangiocarcinoma: staging with intrabiliary MRI. AJR Am J
2. Bold RJ, Goodnight JE, Jr.: Hilar cholangiocarcinoma: surgical and endoscopic approaches. Surg Clin North Am
84:525, 2004
3. Koea J, Holden A, Chau K, et al: Differential diagnosis of stenosing lesions at the hepatic hilus. World J Surg
28:466, 2004
4. Manfredi R, Masselli G, Maresca G, et al: MR imaging and MRCP of hilar cholangiocarcinoma. Abdom Imaging
28:319, 2003
5. Principe A, Ercolani G, Bassi F, et al: Diagnostic dilemmas in biliary strictures mimicking cholangiocarcinoma.
Hepatogastroenterology 50:1246, 2003
6. Soyer P, Bluemke DA, Reichle R, et al: Imaging of intrahepatic cholangiocarcinoma: 2. Hilar cholangiocarcinoma.
AJR Am J Roentgenol 165:1433, 1995
Extrahepatic Bile Duct Adenocarcinoma

1. Albores-Saavedra J, Henson DE, Klimstra DS: Tumors of the gallbladder and extrahepatic bile ducts, and ampulla
of vater. Washington, D.C.: Armed Forces Institute of Pathology under the auspices of Universities Associated for
Research and Education in Pathology For sale by the Armed Forces Institute of Pathology, 2000
2. Park MS, Kim TK, Kim KW, et al: Differentiation of extrahepatic bile duct cholangiocarcinoma from benign
stricture: findings at MRCP versus ERCP. Radiology 233:234, 2004
3. Stroszczynski C, Hunerbein M: Malignant biliary obstruction: value of imaging findings. Abdom Imaging 30:314,
2005
4. Uchida M, Ishibashi M, Tomita N, et al: Hilar and suprapancreatic cholangiocarcinoma: value of 3D angiography
and multiphase fusion images using MDCT. AJR Am J Roentgenol 184:1572, 2005

Pancreatic Neoplasms
Classification of Pancreatic Tumors

• Tumors of the Exocrine Pancreas
➢ Ductal adenocarcinoma
➢ Acinar cell carcinoma
➢ Solid-pseudopapillary neoplasm
➢ Intraductal papillary mucinous neoplasm
➢ Mucinous cystic neoplasm
➢ Microcystic adenoma
➢ Pancreatoblastoma
➢ Mature cystic teratoma
• Tumors of the Endocrine Pancreas
➢ Islet cell tumors (neuroendocrine tumors)
➢ Small cell carcinoma
• Non-epithelial tumors
➢ Soft tissue tumors
➢ Lymphoma
• Secondary Tumors
• Tumor like lesions
➢ Pancreatitis
➢ Lymphoepithelial cyst
➢ Pseudocyst
Objectives
• Adenocarcinoma
➢ Mucinous noncystic adenocarcinoma
• Intraductal papillary mucinous neoplasm
➢ Intraductal papillary mucinous neoplasm
➢ Solid and pseudopapillary epithelial neoplasm
• Endocrine neoplasms
• Metastasis
Pancreatic Adenocarcinoma: Epidemiology

• 85% to 90% of pancreatic neoplasms
• Second most common GI tract malignancy in the U.S.
➢ US: colorectal, pancreas, stomach, liver, esophagus, gallbladder
➢ Worldwide: stomach, colorectal, liver, esophagus, pancreas, gallbladder
Pancreatic Adenocarcinoma: Epidemiology

• Death:incidence ratio of .99
➢ 5-year survival 4%, overall
➢ 5-year survival 17%, early stage
• More common in men than women
• 80% of cases occur between 60 to 80 years of age
Pancreatic Adenocarcinoma: Risk Factors

• Cigarette smoking (2-3 fold relative risk)
• Hereditary risk factors
➢ Hereditary nonpolyposis colon cancer (HNPCC)
➢ Familial breast cancer (BRCA2)
➢ Familial adenomatous polyposis
➢ Peutz-Jeghers
Gastrointestinal Radiology 321 Pancreatic Neoplasms

➢ Peutz-Jeghers
➢ Ataxia telangiectasia
➢ Familial atypical multiple mole-melanoma
➢ Familial pancreatitis
Figure 2-7-1
Pancreatic Adenocarcinoma:
Clinical Features
• Symptoms
➢ Pain most common
➢ Unexplained weight loss
➢ Jaundice in 50% tumors in the head of
the pancreas
➢ Diabetes in 25% to 50%
• Distribution
➢ 60% head
➢ 20% body
➢ 10% tail
➢ 5% to 10% entire gland
Pancreatic Adenocarcinoma:
• Microscopy
➢ Moderately to well differentiated
Pathology of ductal adenocarcinoma of the pancreas
➢ Desmoplastic stromal reaction
• Gross Pathology
➢ Fibrotic
➢ Infiltration and invasion of adjacent structures
➢ Hemorrhage and necrosis uncommon
Pancreatic Adenocarcinoma: MDCT

• Dual Phase Technique
➢ Rapid bolus, 3 ml/sec
➢ Pancreatic phase, 40 sec after injection
➢ Portal venous phase
➢ Thin reformations with overlap, 1.25 mm
Fletcher JG, Wiersema MJ, Farrell MA, et al. Pancreatic malignancy: value of arterial,
pancreatic, and hepatic phase imaging with multi-detector row CT. Radiology 2003;
229(1):81-90.
Pancreatic Adenocarcinoma: MDCT

• Volume rendering
• Maximum intensity projection (MIP)
• Curved planar reformations
➢ Additional information for local extension
➢ Secondary signs in iso-attenuating carcinomas
Pancreatic Adenocarcinoma: CT Features

• Pancreatic and CBD duct dilatation
➢ "Double duct sign”
➢ Abrupt tapering of the CBD
• Atrophy of the distal gland
➢ Focal soft tissue density in a fatty gland
• Alterations in morphology
➢ Spherical enlargement of the pancreatic head
➢ Rounded borders of the uncinate process
• Extension to adjacent organs
• Vascular encasement
Pancreatic Neoplasms 322 Gastrointestinal Radiology

Double Duct Sign [Figure 2-7-2] Figure 2-7-2
Spherical Enlargement of the

Pancreatic Head
Rounded Borders of the Uncinate

Process [Figure 2-7-3]
Atrophic Changes in the Distal

Pancreas [Figure 2-7-4] Double duct sign of ductal adenocarcinoma of the pancreas
Pancreatic Adenocarcinoma: MR Figure 2-7-3
• Problem solving
➢ Equivocal CT
➢ Small tumors
• T1-weighted images
➢ Low signal tumor on
unenhanced images
➢ Subtraction images from in
and out of phase images
Nonresectability
• Invasion of adjacent organs, Ductal adenocarcinoma of the pancreas showing a rounded contour
except duodenum to the uncinate process
• Tumor diameter > 5 cm
• Encasement or occlusion of vessels Figure 2-7-4
➢ SMA, SMV, portal vein
➢ Celiac trunk and major
branches
➢ +/- isolated focal involvement
of PV or SMV
➢ Accuracy of CT 88%-90%
➢ 3D CT angiography
• Distant nodal metastasis
• Liver metastasis
Ductal adenocarcinoma of the pancreas showing atrophy of the distal
Resectable? No, stomach
pancreas
and vascular invasion
Resectable? No, SMA Encasement Figure 2-7-5
Resectable? No, SMA

Encasement and Liver Mets
[Figure 2-7-5]
Resectable? YES
Nonresectable adenocarcinoma of the pancreas
Mucinous Noncystic
Adenocarcinoma
(Infiltrating Colloid Carcinoma)[Figure 2-7-6]
• Rare variant of adenocarcinoma Figure 2-7-6
• Marked extracellular mucin
• Signet rings cells
• Imaging
➢ Large tumors
➢ Well-defined hypoattenuating
mass
➢ May have calcification
Mucinous noncystic adenocarcinoma of the pancreas

Mucinous Noncystic Adenocarcinoma Figure 2-7-7
(Infiltrating Colloid Carcinoma)
➢ Intraductal papillary mucinous Intraductal papillary mucinous
neoplasm neoplasm, diffuse main duct
➢ Microcystic (Oligocystic) adenoma pattern
➢ Pancreatic pseudocyst
Intraductal Papillary Mucinous

Neoplasm (IPMN)
• Intraductal papillary neoplasm
➢ Produces mucin
• All have malignant potential
➢ May or may not have an invasive
component
• Synonyms
➢ Intraductal mucin-hypersecreting neoplasm
➢ Ductectatic type of pancreatic ductal carcinoma
➢ Mucinous ductal ectasia
➢ Mucin-producing tumor (or carcinoma)
➢ Mucin-hypersecreting tumor Figure 2-7-8
Intraductal Papillary Mucinous Neoplasm (IPMN)

• Most common in men, 7th decade
• Clinical symptoms similar to chronic pancreatitis
➢ Pain
➢ Malabsorption
➢ Diabetes
➢ Prior episodes of pancreatitis
IPMN: Histopathology
• Neoplastic papillary epithelium
• Mucin production
• Duct dilatation
IPMN: Imaging Features Intraductal papillary mucinous

• Duct dilatation neoplasm, focal main duct pattern
➢ Main duct or side branch
➢ Diffuse or focal (cystic appearing)
• Pancreatic glandular atrophy
• Calcification Figure 2-7-9
• Bulging duodenal papilla
IPMN: Diffuse Involvement of Main Pancreatic Duct

[Figure 2-7-7]
IPMN: Focal Involvement of Main Pancreatic Duct

[Figure 2-7-8]
IPMN: Focal Involvement of Main Pancreatic Duct

• Show communication with duct structures
➢ MRCP
➢ ERCP/EUS
• Show intraductal masses
IPMN: Focal Involvement of a Side Branch Duct

[Figure 2-7-9]
Intraductal papillary mucinous
neoplasm, focal side branch pattern

Side Branch IPMN Figure 2-7-10
IPMN: Diffuse Involvement of a Side Branch Duct [Figure 2-7-10]
Bulging Papilla [Figure 2-7-11]
IPMN: Diagnostic Difficulties

• MR/MRCP
➢ Target imaging for ductal communication
IPMN: MR/MRCP
IPMN: Diagnostic Difficulties

• Must differentiate from chronic pancreatitis
• Side branch variant may mimic
➢ Pseudocyst
Intraductal papillary mucinous
neoplasms, diffuse side branch
• ERCP
pattern
➢ Definitive imaging technique
Solid and Pseudopapillary Tumor (SPT)

• Neoplasm of young women
• Benign or low-grade malignancy
• Good prognosis
• Synonyms
➢ Solid-cystic tumor
➢ Papillary cystic tumor
➢ Solid and pseudopapillary epithelial neoplasm
Solid and Pseudopapillary Tumor (SPT)

• Clinical features
➢ Usually incidentally discovered
➢ Abdominal discomfort, pain
➢ Jaundice or duct obstruction is rare
SPT: Pathology Features

➢ Capsule Figure 2-7-11
➢ Hemorrhage, necrosis, cystic
areas
➢ Solid areas
➢ May calcify
• Histopathology
➢ Highly cellular areas
➢ Pseudopapillary areas
➢ Hemorrhage
➢ Sclerosis
Bulging papilla in IPMN
SPEN: Imaging Features
• Circumscribed
➢ Capsule
➢ Early peripheral enhancement
• Cystic change
➢ Hemorrhage
➢ Fluid-fluid levels
• Calcification
• Rare features
➢ Biliary/pancreatic duct dilatation
➢ Adjacent organ invasion

SPT [Figures 2-7-12 and 2-7-13] Figure 2-7-12
SPT: Diagnostic Difficulties

• Older patient age
• Male patient
• May mimic other cystic lesions
➢ Oligocystic adenoma
• Supportive CT and MR features for diagnosis
➢ Evidence of capsule
➢ Evidence of blood products
Mucinous Cystic Neoplasm (MCN)

• Mucin-secreting cystic neoplasm
➢ Mucinous cystadenoma
➢ Mucinous cystadenocarcinoma
• Most common in women
➢ 80% occur in women
➢ Mean age 49 years
• Less common in men
Solid and pseudopapillary tumor
➢ Older age, mean 70 years
Figure 2-7-13
Mucinous Cystic Neoplasm (MCN)
➢ Variable
➢ Dependent upon tumor size
• Jaundice and CBD obstruction are rare
➢ Mucinous cystadenoma
MCN: Histopathology
• Columnar cell lining
• May have ovarian-type stroma
• Mucin
• Hemorrhage MR showing early capsular enhancement in SPT
• Calcification
MCN: Gross Pathology

• Majority in tail of pancreas
➢ 70% to 95%
• Large size at diagnosis
➢ Mean diameter, 6 to 10 cm
• Multilocular cysts
➢ Septations
➢ Solid mural nodules
➢ Occasional calcifications
➢ Unilocular, rare
MCN: Imaging
• Well-circumscribed cystic mass
➢ Cannot differentiate benign from malignant
• Enhancement
➢ Cyst wall, septations, mural nodules
• Calcifications
➢ Cyst wall, septations, mural nodules
• Cyst fluid
➢ Variable CT attenuation/MR signal intensity
➢ Mucin
➢ Hemorrhage
➢ Proteinaceous fluid

Mucinous Cystic Neoplasm [Figure 2-7-14] Figure 2-7-14
MCN: Diagnostic Difficulties

• Small lesions
• Lesions in the head of the pancreas
• Lesions without septations and mural nodules
➢ Pseudocyst
➢ Solid and pseudopapillary tumor
➢ Rare, congenital cysts
Microcystic Adenoma
• Benign
• Synonyms
➢ Serous cystadenoma
➢ Glycogen-rich cystadenoma
• Variants
Microcystic Adenoma Mucinous cystic neoplasm

➢ 70% in women
➢ Incidental
➢ Pain, nausea, vomiting, weight loss Figure 2-7-15
➢ Jaundice is unusual
Microcystic Adenoma: Gross Pathology

• Small cysts arranged around a central scar
➢ “Honeycomb” or “sponge” appearance
➢ Thin fibrous septae
➢ Central stellate scar
➢ Occasional hemorrhage
Microcystic Adenoma: Histopathology

• Histology
➢ Small cysts
➢ Cuboidal cells
➢ High glycogen content
Microcystic Adenoma: Pathology
Microcystic Adenoma: Imaging features

[Figures 2-7-15 and 2-7-16]
• Sharp, circumscribed margins
➢ Lobular margins
• Honeycomb appearance
Microcystic adenoma
➢ Multiple small cysts
• Central scar
➢ +/- calcification Figure 2-7-16
Microcystic adenoma

Oligocystic Adenoma [Figure 2-7-17] Figure 2-7-17
• Uncommon variant of microcystic
adenoma
• Few, very large cysts
• ? association with von Hippel
Lindau syndrome
• Synonyms
➢ Macrocystic serous
cystadenoma
➢ Serous oligocystic adenoma
Oligocystic adenoma
Microcystic Adenoma: Diagnostic Difficulties
• Oligocystic variant
➢ Differential diagnosis
❖ Mucinous cystic neoplasm
❖ Pseudocyst
• Small lesions
➢ Difficult to identify central scar/septations
Islet Cell Tumors (Neuroendocrine Tumors)

• Tumors of the pancreatic islets
• Occur in all age groups
• Variable biologic behavior
➢ Benign or malignant Figure 2-7-18
• Clinical evidence of hormone production
➢ 65% to 85%
• Clinically silent
➢ 15% to 35%
Insulinoma [Figure 2-7-18]

• Somatostatin receptor
scintigraphy
➢ Positive in only 60-70% of
cases
Insulinoma
Zollinger-Ellison Syndrome - Pancreatic Gastrinoma
• Somatostatin receptor scintigraphy
➢ Sensitivity for primary tumor, 58%-75%
➢ Sensitivity for metastatic disease, 100%
Glucagonoma (DM-Dermatitis Syndrome)
MEN I Syndrome (Wermer’s Syndrome)

• 3P’s
➢ Pituitary
➢ Pancreas
➢ Parathyroid
➢ Other: thymus, thyroid, adrenal gland, GI tract
• Autosomal dominant
➢ Long arm chromosome 11
Clinically-Silent Islet Cell Tumors [Figures 2-7-19 and 2-7-20]

• Occur at any age
• Typically large at time of diagnosis
➢ Range, 6 to 20 cm
• Nonspecific imaging appearance
➢ Necrosis and cystic degeneration common
➢ 25% calcify
➢ Liver metastases are common

Metastatic Disease Figure 2-7-19
• Mets to pancreas
➢ Lung, breast
➢ Melanoma
➢ Renal cell
➢ Lymphoma-adjacent nodal
disease
• Mimic primary pancreatic
neoplasms
➢ Islet cell
Clinically-silent islet cell tumor
• Biliary obstruction in 30%
Summary: Adenocarcinoma Figure 2-7-20

• Most common pancreatic
neoplasm
• MDCT
➢ Dual phase
➢ Thin reformations
➢ Overlapping images
• RESECTABLILITY
➢ Vascular encasement
➢ Liver mets
Summary: IMPN
• Main duct or side branch
• Imaging
➢ Duct dilatation
➢ Intraductal masses
➢ Bulging papilla
Renal cell metastatic to the pancreas
Summary: Solid
Pseudopapillary Tumor
• Young women
➢ Capsule
➢ Solid and cystic
➢ Hemorrhage
Summary: Mucinous Cystic Neoplasm

• Mucinous cystadenoma
• Mucinous cystadenocarcinoma
• Complex cyst
➢ Middle-aged women
➢ Tail of the pancreas
➢ Septations, nodules, calcification
Summary: Microcystic Adenoma

• Older women
• Benign neoplasm
➢ Central scar
➢ Honeycomb pattern of cysts
➢ Lobulated margins

References
Adenocarcinoma
1. Vargas R, Nino-Murcia M, Trueblood W, Jeffrey RB, Jr. MDCT in Pancreatic adenocarcinoma: prediction of
vascular invasion and resectability using a multiphasic technique with curved planar reformations. AJR Am J
Roentgenol 2004; 182:419-425.
2. Bronstein YL, Loyer EM, Kaur H, et al. Detection of small pancreatic tumors with multiphasic helical CT. AJR
Am J Roentgenol 2004; 182:619-623.
3. Roche CJ, Hughes ML, Garvey CJ, et al. CT and pathologic assessment of prospective nodal staging in patients
with ductal adenocarcinoma of the head of the pancreas. AJR Am J Roentgenol 2003; 180:475-480.
4. Fletcher JG, Wiersema MJ, Farrell MA, et al. Pancreatic malignancy: value of arterial, pancreatic, and hepatic
phase imaging with multi-detector row CT. Radiology 2003; 229:81-90.
5. Prokesch RW, Chow LC, Beaulieu CF, et al. Local staging of pancreatic carcinoma with multi-detector row CT:
use of curved planar reformations initial experience. Radiology 2002; 225:759-765.
6. Prokesch RW, Chow LC, Beaulieu CF, Bammer R, Jeffrey RB, Jr. Isoattenuating pancreatic adenocarcinoma at
multi-detector row CT: secondary signs. Radiology 2002; 224:764-768.
7. Imbriaco M, Megibow AJ, Camera L, et al. Dual-phase versus single-phase helical CT to detect and assess
resectability of pancreatic carcinoma. AJR Am J Roentgenol 2002; 178:1473-1479.
8. Horton KM, Fishman EK. Adenocarcinoma of the pancreas: CT imaging. Radiol Clin North Am 2002; 40:1263-
1272.
9. McNulty NJ, Francis IR, Platt JF, Cohan RH, Korobkin M, Gebremariam A. Multi--detector row helical CT of the
pancreas: effect of contrast-enhanced multiphasic imaging on enhancement of the pancreas, peripancreatic
vasculature, and pancreatic adenocarcinoma. Radiology 2001; 220:97-102.
10. Tabuchi T, Itoh K, Ohshio G, et al. Tumor staging of pancreatic adenocarcinoma using early- and late-phase helical
CT. AJR Am J Roentgenol 1999; 173:375-380.
11. O'Malley ME, Boland GW, Wood BJ, Fernandez-del Castillo C, Warshaw AL, Mueller PR. Adenocarcinoma of the
head of the pancreas: determination of surgical unresectability with thin-section pancreatic-phase helical CT. AJR
Am J Roentgenol 1999; 173:1513-1518.
12. Hough TJ, Raptopoulos V, Siewert B, Matthews JB. Teardrop superior mesenteric vein: CT sign for unresectable
carcinoma of the pancreas. AJR Am J Roentgenol 1999; 173:1509-1512.
13. Keogan MT, Tyler D, Clark L, et al. Diagnosis of pancreatic carcinoma: role of FDG PET. AJR Am J Roentgenol
1998; 171:1565-1570.
14. Zeman RK, Cooper C, Zeiberg AS, et al. TNM staging of pancreatic carcinoma using helical CT. AJR Am J
Roentgenol 1997; 169:459-464.
15. Raptopoulos V, Steer ML, Sheiman RG, Vrachliotis TG, Gougoutas CA, Movson JS. The use of helical CT and CT
angiography to predict vascular involvement from pancreatic cancer: correlation with findings at surgery. AJR Am
J Roentgenol 1997; 168:971-977.
16. Lu DS, Reber HA, Krasny RM, Kadell BM, Sayre J. Local staging of pancreatic cancer: criteria for unresectability
of major vessels as revealed by pancreatic-phase, thin-section helical CT. AJR Am J Roentgenol 1997; 168:1439-
1443.
17. Lu DS, Vedantham S, Krasny RM, Kadell B, Berger WL, Reber HA. Two-phase helical CT for pancreatic tumors:
pancreatic versus hepatic phase enhancement of tumor, pancreas, and vascular structures. Radiology 1996;
199:697-701.
18. Megibow AJ, Zhou XH, Rotterdam H, et al. Pancreatic adenocarcinoma: CT versus MR imaging in the evaluation
of resectability--report of the Radiology Diagnostic Oncology Group. Radiology 1995; 195:327-332.
19. Megibow AJ. Pancreatic adenocarcinoma: designing the examination to evaluate the clinical questions. Radiology
1992; 183:297-303.
Intraductal Papillary Mucinous Neoplasm

1. Cellier C, Cuillerier E, Palazzo L, et al: Intraductal papillary and mucinous tumors of the pancreas: accuracy of
preoperative computed tomography, endoscopic retrograde pancreatography and endoscopic ultrasonography, and
long-term outcome in a large surgical series. Gastrointest Endosc 47:42, 1998
2. Fukukura Y, Fujiyoshi F, Sasaki M, et al: Intraductal papillary mucinous tumors of the pancreas: thin-section
helical CT findings. AJR Am J Roentgenol 174:441, 2000
3. Itai Y, Kokubo T, Atomi Y, et al: Mucin-hypersecreting carcinoma of the pancreas. Radiology 165:51, 1987
4. Lim JH, Lee G, Oh YL: Radiologic spectrum of intraductal papillary mucinous tumor of the pancreas.
Radiographics 21:323, 2001
5. Prasad SR, Sahani D, Nasser S, et al: Intraductal papillary mucinous tumors of the pancreas. Abdom Imaging
28:357, 2003
6. Procacci C, Graziani R, Bicego E, et al: Intraductal mucin-producing tumors of the pancreas: imaging findings.
Radiology 198:249, 1996

7. Procacci C, Megibow AJ, Carbognin G, et al: Intraductal papillary mucinous tumor of the pancreas: a pictorial
essay. Radiographics 19:1447, 1999
8. Taouli B, Vilgrain V, Vullierme MP, et al: Intraductal papillary mucinous tumors of the pancreas: helical CT with
histopathologic correlation. Radiology 217:757, 2000
Mucinous Cystic Neoplasm

1. Buetow PC, Rao P, Thompson LD: From the Archives of the AFIP. Mucinous cystic neoplasms of the pancreas:
radiologic-pathologic correlation. Radiographics 18:433, 1998
2. Procacci C, Carbognin G, Accordini S, et al: CT features of malignant mucinous cystic tumors of the pancreas. Eur
Radiol 11:1626, 2001
3. Thompson LD, Becker RC, Przygodzki RM, et al: Mucinous cystic neoplasm (mucinous cystadenocarcinoma of
low-grade malignant potential) of the pancreas: a clinicopathologic study of 130 cases. Am J Surg Pathol 23:1,
1999
Solid and Pseudopapillary Tumor

1. Buetow PC, Buck JL, Pantongrag-Brown L, et al: Solid and papillary epithelial neoplasm of the pancreas:
imaging-pathologic correlation on 56 cases. Radiology 199:707, 1996
2. Cantisani V, Mortele KJ, Levy AD, et al: MR imaging features of solid pseudopapillary tumor of the pancreas in
adult and pediatric patients. AJR Am J Roentgenol 181:395, 2003
3. Coleman KM, Doherty MC, Bigler SA: Solid-pseudopapillary tumor of the pancreas. Radiographics 23:1644, 2003
4. Friedman AC, Lichtenstein JE, Fishman EK, et al: Solid and papillary epithelial neoplasm of the pancreas.
Radiology 154:333, 1985
Microcystic Adenoma
1. Buck JL, Hayes WS: From the Archives of the AFIP. Microcystic adenoma of the pancreas. Radiographics 10:313,
1990
2. Healy JC, Davies SE, Reznek RH: CT of microcystic (serous) pancreatic adenoma. J Comput Assist Tomogr
18:146, 1994
3. Hough DM, Stephens DH, Johnson CD, et al: Pancreatic lesions in von Hippel-Lindau disease: prevalence, clinical
significance, and CT findings. AJR Am J Roentgenol 162:1091, 1994
4. Itai Y, Ohhashi K, Furui S, et al: Microcystic adenoma of the pancreas: spectrum of computed tomographic
findings. J Comput Assist Tomogr 12:797, 1988
5. Khurana B, Mortele KJ, Glickman J, et al: Macrocystic serous adenoma of the pancreas: radiologic-pathologic
correlation. AJR Am J Roentgenol 181:119, 2003
6. Minami M, Itai Y, Ohtomo K, et al: Cystic neoplasms of the pancreas: comparison of MR imaging with CT.
Radiology 171:53, 1989
7. Yeh HC, Stancato-Pasik A, Shapiro RS: Microcystic features at US: a nonspecific sign for microcystic adenomas
of the pancreas. Radiographics 21:1455, 2001
Islet Cell Tumors

1. Buetow PC, Miller DL, Parrino TV, et al: Islet cell tumors of the pancreas: clinical, radiologic, and pathologic
correlation in diagnosis and localization. Radiographics 17:453, 1997
2. Buetow PC, Parrino TV, Buck JL, et al: Islet cell tumors of the pancreas: pathologic-imaging correlation among
size, necrosis and cysts, calcification, malignant behavior, and functional status. AJR Am J Roentgenol 165:1175,
1995
3. Ichikawa T, Peterson MS, Federle MP, et al: Islet cell tumor of the pancreas: biphasic CT versus MR imaging in
tumor detection. Radiology 216:163, 2000
4. Semelka RC, Cumming MJ, Shoenut JP, et al: Islet cell tumors: comparison of dynamic contrast-enhanced CT and
MR imaging with dynamic gadolinium enhancement and fat suppression. Radiology 186:799, 1993
5. Sheth S, Hruban RK, Fishman EK: Helical CT of islet cell tumors of the pancreas: typical and atypical
manifestations. AJR Am J Roentgenol 179:725, 2002
6. Stafford Johnson DB, Francis IR, Eckhauser FE, et al: Dual-phase helical CT of nonfunctioning islet cell tumors. J
Comput Assist Tomogr 22:59, 1998
7. Van Hoe L, Gryspeerdt S, Marchal G, et al: Helical CT for the preoperative localization of islet cell tumors of the
pancreas: value of arterial and parenchymal phase images. AJR Am J Roentgenol 165:1437, 1995
Metastases
1. Klein KA, Stephens DH, Welch TJ: CT characteristics of metastatic disease of the pancreas. Radiographics 18:369,
1998
2. Ng CS, Loyer EM, Iyer RB, et al: Metastases to the pancreas from renal cell carcinoma: findings on three-phase
contrast-enhanced helical CT. AJR Am J Roentgenol 172:1555, 1999

Gastric Malignancies
Gastric Malignancies
• Adenocarcinoma
• Lymphoma
• Gastrointestinal Stromal Tumors
• Carcinoid
• Kaposi Sarcoma
• Metastases
Gastric Adenocarcinoma
• Fourth most common cancer worldwide1
➢ Lung, breast, colorectum, stomach, liver
1Steward BW and Kleihues P (eds). World Cancer Report. IARC Press. Lyon
2003.
Gastric Adenocarcinoma: Geographic Variation

• High incidence areas
➢ China, Japan, South America, Eastern Europe
• Low incidence areas
➢ North America and Northern Africa
Gastric Adenocarcinoma: Clinical

• Peak incidence 50 to 70 years of age
➢ 2:1
• Early disease
➢ Asymptomatic
• Advanced disease
➢ Epigastric pain, bloating, nausea
➢ Early satiety, anorexia, vomiting
➢ Upper GI bleeding
Figure 2-8-1
Gastric Adenocarcinoma:
Etiology [Figure 2-8-1]
• Atrophic Gastritis
➢ Helicobacter pylori
(80% of cases)
➢ Pernicious Anemia
➢ Partial Gastrectomy
• Adenomatous Polyps
➢ Polyposis syndromes
• HNPCC-Hereditary
Nonpolyposis Colon
Cancer Syndromes Normal Gastritis Atrophic Gastritis Intestinal Metaplasia
(Lynch Syndromes)
Dysplasia Intramucosal Carcinoma Invasive Carcinoma
Pathogenesis of gastric adenocarcinoma
Gastric Malignancies 332 Gastrointestinal Radiology

WHO Classification of Gastric Adenocarcinoma Figure 2-8-2
• Signet Ring [Figure 2-8-2]
• Papillary [Figure 2-8-3]
• Mucinous [Figure 2-8-4]
Figure 2-8-3
Signet ring cell adenocarcinoma
produces "linitis plastica"
Figure 2-8-4
Papillary adenocarcinoma produces
intraluminal polypoid masses
Early Gastric Carcinoma

• Japanese Research Society for
Gastric Carcinoma
• Carcinoma limited to the mucosa and
submucosa, irrespective of nodal Mucinous adenocarcinoma produces tumor calcifications
metastases
Advanced Gastric Carcinoma [Figure 2-8-5] Figure 2-8-5

• Borrmann Classification
• Most common gastric cancer in the U.S.
• Tumor penetrating the muscularis propria
Advanced Gastric Carcinoma

• Morphology
➢ Polypoid
➢ Ulcerating
➢ Infiltrating
➢ Scirrhous
Gastric Carcinoma: Polypoid [Figure 2-8-6]
Figure 2-8-6
Advanced gastric carcinoma

(Borrmann) classification
Polypoid gastric adenocarcinoma
Gastrointestinal Radiology 333 Gastric Malignancies

Figure 2-8-7
Ulcerated - Bulk of tumor mass

has been replaced by ulceration
Ulcerated Carcinoma
Lesser Curvature
Carmen Meniscus Sign

[Figure 2-8-7]
Ulcerated gastric
Gastric Carcinoma: Ulcerated adenocarcinoma showing the
Carmen meniscus sign
Gastric Carcinoma: Infiltrating
[Figure 2-8-8]
Scirrhous Carcinoma [Figure 2-8-9]

• Infiltrating tumors with desmoplasia
➢ Signet ring cell carcinomas Figure 2-8-8
• Radiologic Features
➢ Irregular narrowing
➢ Decreased gastric capacity
➢ Rigidity, “linitis plastica”
➢ Most common in the antrum
➢ Rarely-nodular,distorted folds
when the tumor is proximal
Carcinoma of the Cardia

• One-third of all gastric
carcinomas in the U.S. Infiltrating gastric adenocarcinoma
• Compared to other gastric
carcinomas:
➢ Male predominance, 6:1
Figure 2-8-9
➢ 40% associated with hiatal hernia
➢ Atrophic gastritis and signet
ring cell types are uncommon
➢ Association with smoking and
alcohol
• Early lesions are difficult to detect
• Difficult to differentiate from
Barrett’s adenocarcinoma
• Pseudoachalasia
• Pitfalls on CT:
➢ GE junction pseudomass Scirrhous carcinoma
➢ Hiatal hernias
Normal Gastric Cardia Figure 2-8-10
Carcinoma of the Cardia

[Figure 2-8-10]
Carcinoma of the cardia

Staging Figure 2-8-11
• Endoscopic Ultrasound
➢ Depth of tumor invasion
❖ T stage accuracy 85%
➢ Perigastric nodes
❖ Sensitivity 55%-80%
• CT
➢ Presence and extent of extragastric spread
➢ Peritoneal
➢ Lymph nodes
➢ Distant metastasis
Staging - Extragastric Spread [Figure 2-8-11 and 2-8-12]

• Anatomic Pathways
➢ Lesser omentum
➢ Greater omentum
➢ Transverse mesocolon Routes of extragastric spread of
➢ Lesser sac carcinoma
➢ Lower esophagus
• CT features
➢ Soft tissue stranding Figure 2-8-12
➢ Soft tissue nodules
Staging - Lymphatic Spread

Staging - Krukenberg Metastasis
Staging - Adjacent Organ Invasion

• Contiguous tumor
• Loss of fat planes
• Focal enlargement of the adjacent organ
Direct Extension and Adjacent Organ Invasion
Gastric Lymphoma
• Increasing incidence
• Up to 10% of gastric malignancies
• Most common site of extranodal lymphoma
• Most common site of GI lymphomas
Gastric Lymphoma: Clinical

• Low grade
➢ Dyspepsia, nausea, vomiting
• High grade:
➢ Bleeding, pain, early satiety, weight loss
Mucosa-Associated Lymphoid Tissue (MALT)

• Organized lymphoid tissue located in mucosal sites Gastric adenocarcinoma extending into
• Native MALT the greater omentum and lesser sac
➢ Intraepithelial lymphocytes
➢ Plasma cells, B and T lymphocytes in the lamina propria
➢ Mesenteric lymph nodes
➢ Peyer’s patches
• Acquired MALT
➢ Arises as a result of antigenic stimulation (H. pylori infection)
➢ Accumulates before the development of B cell lymphomas

Primary Gastric Lymphoma Figure 2-8-13
• MALT lymphoma
➢ Arises from acquired MALT Normal mucosa
➢ H. pylori is invariably present
➢ Good clinical prognosis
• High grade B cell lymphoma
➢ Probably arises from MALT lymphoma
Primary Gastric Lymphoma: Etiology and

Pathogenesis Figure 2-8-13]
• H. pylori infection
H. pylori infection
H. pylori gastritis
formation of acquired MALT
low-grade MALT lymphoma
high-grade B-cell lymphoma
Evolution of gastric lymphoma

Low Grade Malt Lymphoma [Figure 2-8-14] Figure 2-8-14
• Clinical
➢ Dyspepsia, nausea, vomiting
➢ Good prognosis
• Imaging
➢ Nodules
➢ Ulcers
➢ Erosions
➢ Thick rugal folds
Low Grade Malt Lymphoma: CT Features

• Homogeneous mural thickening
• Perigastric adenopathy
High Grade B Cell Lymphoma

• Clinical
➢ Bleeding, pain,
➢ Early satiety, weight loss
• Imaging
➢ Mural thickening
➢ Adenopathy
High Grade B Cell Lymphoma:Typical CT Features

[Figure 2-8-15]
• Homogeneous mural thickening
• Perigastric adenopathy
High Grade B Cell Lymphoma:Typical CT Features Low grade MALT lymphoma

• Cavitation
High Grade B Cell Lymphoma: Atypical CT Features Figure 2-8-15

• Heterogeneous attenuation
• Enhancement
• Low attenuation necrosis
CT features of Gastric Lymphoma

• Wall thickening1
➢ Tends to be greater (mean, 4 cm) than that of
adenocarcinoma
➢ Tends to be homogeneous attenuation
• Ulceration
• Polypoid masses
• Regional adenopathy
1Buy J, Moss A. AJR 138:859-865, 1982
CT features differentiating Gastric Adenocarcinoma

and Lymphoma1
• Gastric wall thickening in lymphoma (mean 4 cm) is typically more
impressive than adenocarcinoma (mean 1.8 cm)
• Wall thickening is more homogeneous in lymphoma
• Perigastric fat is more likely to be preserved in lymphoma
• Regional adenopathy is common in both High grade B cell lymphoma
• Adenopathy in lymphoma tends to be bulky and may extend
below the level of the renal veins
1Buy J, Moss A. AJR 138:859-865, 1982
Fork FT, Haglund U, Hogstrom H. Endoscopy 17:5-7, 1985

Gastric Lymphoma
Mesenchymal Neoplasm of the Stomach

• Gastrointestinal Stromal Tumor
• Leiomyoma
• Leiomyosarcoma
• Schwannoma
• Neurofibroma
• Ganglioneuroma
• Paraganglioma
• Lipoma, liposarcoma
• Fibrous lesions
• Tumors of blood vessels
Gastrointestinal Stromal Tumors (GIST)

• Most common mesenchymal neoplasm of the GI tract
• Arise from the muscularis propria
• Cellular origin
➢ Primitive "stem cell” like cell
➢ Interstitial cell of Cajal (gut pacemaker cell)
GIST: Clinical Features

• Uncommon tumors
• Prevalence in the U.S.
➢ 5000 to 6000 new cases per year1
• Increased incidence
➢ Neurofibromatosis (NF-1)
➢ KIT germline mutations
1Fletcher CD, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal
tumors: A consensus approach. Hum Pathol 2002. 33:459-465
GIST: Clinical Features

• Median age 50-60 years
• 60% occur in the stomach
• Benign and malignant
• Defined by KIT expression
➢ Initial diagnosis
➢ Therapy (Gleevac)
What is KIT?
• KIT-tyrosine kinase growth factor
• KIT-tyrosine kinase growth factor receptor
• CD117 binds to KIT receptors
• Normally expressed
➢ Hematopoietic stem cells
➢ Germ cells
➢ Interstitial cell of Cajal (gut pacemaker cell)
• KIT-inhibitor therapy
➢ STI-571, Imatinib [Gleevac]
What Happened to Leiomyomas and Leiomyosarcomas?

• Very rare
• Except,
➢ Leiomyomas are the most common benign tumor of the ESOPHAGUS
➢ Leiomyosarcomas of the RETROPERITONEUM
GIST
• Most common sites
➢ Stomach
➢ Small bowel

➢ Anorectum Figure 2-8-16
• Unusual sites
➢ Esophagus
➢ Colon
➢ Mesentery/omentum
GIST
• Spindle Cell GIST
• Epithelioid GIST
GIST: CD117 (KIT) Positive
GIST [Figure 2-8-16]

• Mural Origin
➢ Mural mass
➢ Exophytic mass
➢ Mural and exophytic
GIST: Mural Origin [Figure 2-8-17]

Figure 2-8-17
Imaging and morphologic patterns of

gastrointestinal stromal tumors: intramural
polypoid mass, exophytic mass, or intramural and
exophytic mass
Gastrointestinal stromal tumor

GIST: Internal Hemorrhage and Necrosis
GIST: Cyst Formation
GIST: Cavity Formation
GIST: Calcification
GIST: Metastatic Spread

• Direct invasion
• Peritoneal
• Hematogenous
➢ Liver
Management of Metastatic and Recurrent Disease

• Kit-inhibitor therapy
➢ Gleevac
➢ Clinical trials of other kit-inhibitors
• Imaging features of treated metastasis
➢ Cystic transformation Figure 2-8-19
➢ Pet important to determine residual functional
tumor
Imaging of Recurrent Disease

Choi H, Charnsangavej C, de Castro Faria S, et al. CT
evaluation of the response of gastrointestinal stromal
tumors after imatinib mesylate treatment: a quantitative
analysis correlated with FDG PET findings. AJR Am J
Roentgenol 2004; 183:1619-1628.
Gastric GIST vs. Adenocarcinoma
[Figures 2- 7-19 and 2-7-20]
Gastric Carcinoid
• Type I: autoimmune chronic atrophic gastritis
➢ Hypergastrinemia
➢ Multiple, small
➢ Benign biologic behavior
• Type II: MEN I and Zollinger Ellison syndrome
➢ Hypergastrinemia
➢ Multiple, small
➢ Benign biologic behavior
• Type III: sporadic
➢ Single
➢ Aggressive biologic behavior GIST vs. adenocarcinoma

Carcinoid: Imaging Features Figure 2-8-20
• Submucosal mass
• Central ulceration-”bull’s eye”
• Pedunculated polypoid lesions, rarely
• Large ulcerative masses
• Thick, rugal folds if hypergastrinemia is present
Carcinoid: “Bull′′s Eye Lesion”
Carcinoid: Pedunculated Polyps
Kaposi Sarcoma [Figure 2-8-21]

• AIDS patients
• Cutaneous KS usually
• Stomach, duodenum, and small bowel most
common GI locations
• Radiologic features
➢ Submucosal masses
➢ “Bull’s-eye”appearance
➢ Polypoid masses
➢ Infiltrating variant, rare
Metastases
• Melanoma, breast, lung
➢ Ulcerating masses
➢ Polyps
GIST vs. lymphoma
➢ Infiltrating
➢ “Linitis Plastica”
Figure 2-8-21
Summary: Adenocarcinoma
• H. pylori
• Chronic atrophic gastritis
• Primary tumor morphology
➢ Polypoid
➢ Ulcerating
➢ Infiltrating
➢ Schirrous
• CT: extragastric spread
Summary: Lymphoma
• H. pylori
• Low grade MALT to high grade B cell
• Compared to adenocarcinoma
➢ Greater wall thickening
➢ Bulky, more extensive adenopathy
Summary: GIST
• Most common mesenchymal neoplasm
• KIT reactivity
➢ Diagnosis
➢ Gleevac therapy
• Classic mural masses on barium
• May have extensive extragastric growth
Bull’s eye lesions from Kaposi Sarcoma.

Endoscopy shows hemorrhagic masses

Summary: Bull’s Eye Lesions
• Carcinoid
• Metastasis
➢ (Breast, Lung, Melanoma)
• Kaposi’s Sarcoma
• Lymphoma
• Adenocarcinoma
• Ectopic Pancreas
References
Gastric Carcinoma
1. Balthazar EJ, Siegel SE, Megibow AJ, et al: CT in patients with scirrhous carcinoma of the GI tract: imaging
findings and value for tumor detection and staging. AJR 165:839, 1995
2. Gore RM: Gastric cancer. Clinical and pathologic features. Radiol Clin North Am 35:295, 1997
3. Gore RM, Levine MS, Ghahremani GG, et al: Gastric cancer. Radiologic diagnosis. Radiol Clin North Am 35:311,
1997
4. Levine MS, Kong V, Rubesin SE, et al: Scirrhous carcinoma of the stomach: radiologic and endoscopic diagnosis.
Radiology 175:151, 1990
5. Longmire WP, Jr.: A current view of gastric cancer in the US. Ann Surg 218:579, 1993
6. Miller FH, Kochman ML, Talamonti MS, et al: Gastric cancer. Radiologic staging. Radiol Clin North Am 35:331,
1997
7. Morales TG: Adenocarcinoma of the gastric cardia. Dig Dis 15:346, 1997
8. Parsonnet J: Helicobacter pylori and gastric cancer. Gastroenterol Clin North Am 22:89, 1993
9. Parsonnet J, Friedman GD, Vandersteen DP, et al: Helicobacter pylori infection and the risk of gastric carcinoma.
N Engl J Med 325:1127, 1991
10. Sipponen P, Marshall BJ: Gastritis and gastric cancer. Western countries. Gastroenterol Clin North Am 29:579,
2000
Gastric Lymphoma
1. An SK, Han JK, Kim YH, et al: Gastric mucosa-associated lymphoid tissue lymphoma: spectrum of findings at
double-contrast gastrointestinal examination with pathologic correlation. Radiographics 21:1491, 2001
2. Buy JN, Moss AA: Computed tomography of gastric lymphoma. AJR 138:859, 1982
3. Choi D, Lim HK, Lee SJ, et al: Gastric mucosa-associated lymphoid tissue lymphoma: helical CT findings and
pathologic correlation. AJR 178:1117, 2002
4. Jaffe ES, Harris NL, Stein H, et al (eds): World Health Organization Classification of Tumours: Pathology and
Genetics of Tumours of Haematopoietic and Lymphoid Tissues), Lyon: IARC Press, 2001
5. Kim YH, Lim HK, Han JK, et al: Low-grade gastric mucosa-associated lymphoid tissue lymphoma: correlation of
radiographic and pathologic findings. Radiology 212:241, 1999
6. Levine MS, Elmas N, Furth EE, et al: Helicobacter pylori and gastric MALT lymphoma. AJR Am J Roentgenol
166:85, 1996
7. Levine MS, Rubesin SE, Pantongrag-Brown L, et al: Non-Hodgkin's lymphoma of the gastrointestinal tract:
radiographic findings. AJR Am J Roentgenol 168:165, 1997
8. Megibow AJ, Balthazar EJ, Naidich DP, et al: Computed tomography of gastrointestinal lymphoma. AJR 141:541,
1983
9. Parsonnet J, Hansen S, Rodriguez L, et al: Helicobacter pylori infection and gastric lymphoma. N Engl J Med
330:1267, 1994
10. Wotherspoon AC, Doglioni C, de Boni M, et al: Antibiotic treatment for low-grade gastric MALT lymphoma.
Lancet 343:1503, 1994
11. Yoo CC, Levine MS, Furth EE, et al: Gastric mucosa-associated lymphoid tissue lymphoma: radiographic findings
in six patients. Radiology 208:239, 1998
Gastrointestinal Stromal Tumor (GIST)

1. Burkill GJ, Badran M, Al-Muderis O, et al: Malignant gastrointestinal stromal tumor: distribution, imaging
features, and pattern of metastatic spread. Radiology 226:527, 2003
2. Chen MY, Bechtold RE, Savage PD: Cystic changes in hepatic metastases from gastrointestinal stromal tumors
(GISTs) treated with Gleevec (imatinib mesylate). AJR 179:1059, 2002
3. Dematteo RP, Heinrich MC, El-Rifai WM, et al: Clinical management of gastrointestinal stromal tumors: before
and after STI-571. Hum Pathol 33:466, 2002
4. Fletcher CD: Clinicopathologic correlations in gastrointestinal stromal tumors. Hum Pathol 33:455, 2002

5. Fletcher CD, Berman JJ, Corless C, et al: Diagnosis of gastrointestinal stromal tumors: A consensus approach.
Hum Pathol 33:459, 2002
6. Levy AD, Remotti HE, Thompson WM, et al: From the Archives of the AFIP: Gastrointestinal Stromal Tumors:
Radiologic Features with Pathologic Correlation. RadioGraphics 23:283, 2003
7. Miettinen M, El-Rifai W, Sobin LH, et al: Evaluation of malignancy and prognosis of gastrointestinal stromal
tumors: a review. Hum Pathol 33:478, 2002
8. Miettinen M, Lasota J: Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical,
and molecular genetic features and differential diagnosis. Virchows Arch 438:1, 2001
9. Nishida T, Kumano S, Sugiura T, et al: Multidetector CT of high-risk patients with occult gastrointestinal stromal
tumors. AJR Am J Roentgenol 180:185, 2003
10. Sharp RM, Ansel HJ, Keel SB: Best cases from the AFIP: gastrointestinal stromal tumor. Armed Forces Institute of
Pathology. RadioGraphics 21:1557, 2001
Gastric Carcinoid
1. Balthazar EJ, Megibow A, Bryk D, et al: Gastric carcinoid tumors: radiographic features in eight cases. AJR Am J
2. Berger MW, Stephens DH: Gastric carcinoid tumors associated with chronic hypergastrinemia in a patient with
Zollinger-Ellison syndrome. Radiology 201:371, 1996
3. Binstock AJ, Johnson CD, Stephens DH, et al: Carcinoid tumors of the stomach: a clinical and radiographic study.
AJR 176:947, 2001
4. Borch K, Renvall H, Kullman E, et al: Gastric carcinoid associated with the syndrome of hypergastrinemic
atrophic gastritis. A prospective analysis of 11 cases. Am J Surg Pathol 11:435, 1987
5. Ho AC, Horton KM, Fishman EK: Gastric carcinoid tumors as a consequence of chronic hypergastrinemia: spiral
CT findings. Clin Imaging 24:200, 2000

Abdominal Non Hodgkin Lymphoma
Objectives
• Definition
• Patterns of disease
➢ NHL Adenopathy
➢ Gastrointestinal Lymphoma
• Immunodeficiency-related lymphomas
➢ Post-transplantation Lymphoproliferative Disorder (PTLD)
➢ AIDS-related Lymphomas
Lymphoid Neoplams
• 2001 WHO classification of Hematological Malignancies
• Three major categories
➢ B cell, T and NK (natural killer) cell, Hodgkin lymphoma
• NHL
➢ Large group of diverse diseases
➢ Indolent, aggressive, and very aggressive
Non-Hodgkin Lymphoma (NHL)

• 4% of all cancers
➢ 5th most common cancer
➢ 5th leading cause of cancer death
• 4 times more common than Hodgkin lymphoma
• Male to female ratio: 1.3 to 1
• Median age 55 years
• Third most common cancer mortality in children under age 15
Non-Hodgkin Lymphoma
• Rising incidence
➢ True increase in incidence
➢ Improved identification and understanding
➢ HIV infection
➢ Organ transplants
• Immunodeficiency increases risk
➢ Wiskott-Aldrich syndrome
➢ Ataxia telangiectasia
➢ Long-term immunosuppressive therapy
Role of Imaging in Newly Diagnosed NHL

• Clinical Staging:
➢ Ann Arbor Staging Classification
➢ Tumor bulk has important prognostic significance in intermediate and high
grade NHL
• Identification of nodal and extranodal sites
➢ Mesenteric adenopathy
➢ GI tract
➢ Liver
➢ Spleen
Abdominal Non-Hodgkin Lymphoma 344 Gastrointestinal Radiology

Role of Imaging in Management of NHL Figure 2-9-1
• CT and PET complimentary
• CT imaging
➢ Define anatomy
➢ Response to therapy
• Limitations of CT
➢ Decrease in node size is not a
reliable indicator of response
➢ Cannot differentiate residual tumor
vs. fibrosis vs. necrosis Well-defined mesenteric nodes in NHL
Raanani P, Shasha Y, Perry C, et al. Is CT
scan still necessary for staging in Hodgkin and non-Hodgkin lymphoma patients in
the PET/CT era? Ann Oncol 2005.
PET/CT in Management of NHL

• Initial staging
➢ Equivalent or better to CT alone Figure 2-9-2
• Prognosis
➢ Persistent uptake after
chemotherapy predicts treatment
failure/early recurrence
Kumar R et al. Utility of fluorodeoxyglucose-
PET imaging in the management of patients
with Hodgkin’s and non-Hodgkin’s
lymphomas. RCNA 2004. 42:1083-1100
Sandwich sign of the mesentery in NHL
NHL: Abdomimal Adenopathy
• Mesentery
• Retroperitoneum
• CT Patterns
➢ Discrete rounded nodes
➢ Confluent nodes
➢ Ill-defined masses Figure 2-9-3
➢ Mesenteric caking
➢ Stellate mesentery
• CT attenuation at diagnosis
➢ Homogeneous in most cases
➢ Heterogeneous in cases with
aggressive histology
• CT attenuation during treatment
➢ Heterogeneous from necrosis
➢ Calcification may occur
Confluent retroperitoneal nodes in NHL
NHL: Discrete Nodes [Figure 2-9-1]
NHL: Sandwich Sign [Figure 2-9-2]

Figure 2-9-4
NHL: Confluent Nodes [Figure 2-9-3]
NHL: Confluent Nodes in AIDS-

Associated NHL [Figure 2-9-4]
NHL: Mesenteric Caking in AIDS-

Associated NHL
Confluent nodes in AIDS-associated NHL. There is extensive

necrosis and heterogeneous enhancement
Gastrointestinal Radiology 345 Abdominal Non-Hodgkin Lymphoma

Differential Diagnosis: Mesenteric Masses
• Lymphoma
• Metastasis
• Carcinoid
• Castleman disease
• Mesenteric fibromatosis
• Gastrointestinal Stromal Tumor (GIST)
• Granulomatous disease
➢ Tuberculosis
➢ Histoplasmosis
➢ Sarcoid
• Sprue
• Whipple disease
• Inflammatory Pseudotumor
Castleman Disease
• Hyperenhancing masses
➢ Homogeneous or heterogeneous
• May calcify
Mesenteric Fibromatosis (Desmoid Tumor)

• Homogeneous attenuation
➢ Soft tissue or low attenuation myxoid stroma
➢ Foci of low attenuation myxoid stroma
• May infiltrate adjacent bowel
Carcinoid
• Primary lesion in bowel
• Mesenteric metastasis
➢ Spiculations/tethering of mesentery
➢ May calcifiy

• Mesenteric masses of GIST
➢ Primary to small bowel or mesentery
➢ Metastatic disease
➢ Low attenuation hemorrhage/necrosis
Sarcoid
• Small, discrete nodes
• Retrocural nodes atypical in sarcoid
Gastrointestinal Lymphoma
• Lymphoma that presents with GI disease and no other major site of
involvement
• Most common extranodal site of NHL
➢ 4.4% of all lymphomas
➢ 25% of all extranodal lymphomas
• Almost exclusively NHL
• Stomach is the most common site in US and Western Europe
• Small bowel is the most common site in the Mediterranean, Northern Africa,
Middle East
• B-cell lymphomas
➢ MALT lymphomas
❖ Immunoproliferative small intestinal disease, “alpha-heavy chain
disease”
➢ Mantle cell lymphoma (multiple lymphomatous polyposis)
➢ Burkitt and Burkitt-type lymphoma
➢ Nodal equivalents (diffuse large B-cell lymphomas, follicular, etc)

• T-cell lymphoma
➢ Enteropathy-type T-cell lymphoma (ETTL)
Figure 2-9-5
NHL: Small Intestine
• Approximately 25% of all primary small bowel malignancies
• Male predominance, mean age 60 years
• Clinical presentation:
➢ Weight loss, pain, bleeding
➢ Intussusception, obstruction, perforation
• Ileum most common location and duodenum least common
• Multiple lesions in 10 to 25% of cases
NHL Small Bowel: Radiologic Patterns

• Mural infiltration
➢ Fold thickening
➢ Circumferential wall thickening
➢ Luminal dilatation
• Polypoid nodules
➢ Solitary
➢ Multiple (lymphomatous polyposis)
• Cavities
• Mesenteric disease
NHL Small Intestine: Tumor Morphology [Figure 2-9-5]
NHL Small Intestine: Mural Infiltration

[Figures 2-9-6 to 2-9-8]
Figure 2-9-7
Lymphoma histology shows tumor
extension from mucosa to serosa
Figure 2-9-6
Ileal lymphoma showing mural infiltration, ulceration,

and nodularity
Ileal lymphoma extending

into small bowel mesentery

NHL Small Intestine: Cavitary Mass [Figure 2-9-9] Figure 2-9-8
Figure 2-9-9
Cavitary mass
NHL Small Intestine: Adjacent Mesenteric Disease

[Figure 2-9-10]
Figure 2-9-10
Mesenteric mass engulfing small intestine
Burkitt Lymphoma
• High grade B-cell lymphoma
• More common in males
• Endemic Mural infiltration with luminal
➢ African Burkitt, related to EBV dilatation
➢ Head and neck disease
• Sporadic
➢ Western countries, not related to EBV
➢ ileocecal region of children
➢ Intestinal obstruction
➢ Intussusception
Mantle Cell Lymphoma [Figure 2-9-11]
Mantle Cell Lymphoma (Multiple Lymphomatous Polyposis)

• Histologically resembles the mantle zone of the lymph follicle
• Median age 65, male predominance
➢ Abdominal pain and bloody stools Figure 2-9-11
• Imaging
➢ Multiple polyps, 0.5 to 2.0 cm
➢ Solitary polyp
➢ Most common in the ileocecal
region
Polypoid masses of mantle cell lymphoma

Enteropathy-Type T-cell Lymphoma (ETTL) [Figure 2-9-12]
• Associated with celiac disease (Sprue)
Figure 2-9-12
➢ Sixth to seventh decade of life
• Most common site jejunum
• Gross pathology
➢ Ulcerated plaques
➢ Strictures
• Poor prognosis
NHL Small Intestine:

• Adenocarcinoma Enteropathy type T-cell lymphoma
• GIST
• Carcinoid
• Metastases
• Crohn disease Figure 2-9-13
• Tuberculosis
• Causes of fold thickening
➢ Sprue
➢ Hemorrhage
➢ Edema
➢ Ischemia
Malignant Melanoma Metastases

Gastrointestinal Stromal Tumor
Jejunal Adenocarcinoma
Tuberculosis / Lymphoma
Post-transplantation Lymphoproliferative
Disorder (PTLD)
• Spectrum of benign and malignant disorders
• Variable incidence
➢ 1% renal transplants
➢ 10% combined heart/lung
➢ 10% of patients on cyclosporine and OKT3
• Association with EBV infection
• Lung, GI tract
PTLD [Figures 2- 9-13 and 2-9-14]

➢ Driven by Epstein-Barr Virus infection
➢ Diffuse polyclonal expansion Pathogenesis of post-transplantation
➢ Reduced T-cell control lymphoproliferative disorder
➢ Malignant transformation
• Clinical
➢ May respond to reducing immunosuppression, anti-virals, surgery
Figure 2-9-14
AIDS-Related Lymphoma
• Second most common neoplasm in HIV
infection
• AIDS defining illness
• Incidence is 4% to 10% in the AIDS
population
• Three categories
➢ Systemic (nodal and/or extranodal)
➢ Primary CNS
Colonic lymphoma in a patient with a renal transplant

➢ Body cavity-based (primary effusion) lymphomas
• Major histologic subtypes
➢ Burkitt lymphoma
➢ Burkitt-like lymphoma
➢ Large cell lymphoma
➢ Large cell immunoblastic lymphoma
AIDS-Related Lymphoma
• 25% have GI tract disease
• Higher incidence of mesenteric disease than non-AIDS lymphomas
• Aggressive histology and biologic behavior
➢ Atypical radiologic features
➢ Hemorrhage
➢ Necrosis
• Unique subtypes
➢ Body cavity-based lymphoma (Kaposi’s sarcoma-associated herpes virus
(KSHV))
➢ Anorectal lymphoma Figure 2-9-15
Primary Peritoneal Lymphoma

[Figure 2-9-15]
Colonic Lymphoma
Anorectal Lymphoma [Figure 2-9-16]
Summary
Primary peritoneal lymphoma in AIDS
• Spectrum of Adenopathy
• ➢ GI lymphomas are predominantly
NHL
➢ Unique subtypes involve the bowel
➢ Various patterns: infiltrating masses, luminal dilatation, polyps, cavitary
masses, mesenteric masses
• AIDS-related
➢ Aggressive behavior
➢ Unusual sites, unusual manifestations
• PTLD Figure 2-9-16
➢ Colon, liver
Patterns of Adenopathy
Patterns of Small Bowel Disease
1-Mural Infiltration
2-Polyps
Anorectal lymphoma in AIDS
3-Cavitary Masses
4-Mesenteric Masses
AIDS-Related Lymphomas
Post Transplantation Lymphoproliferative Disorder (PTLD)

References
Ann Arbor Staging of Gastrointestinal Lymphomas

Stage IE: Confined to the wall of the stomach or bowel
Stage II1E: Regional lymph nodes contiguous to primary site
Stage II2E: Regional lymph nodes not contiguous to primary site
Stage III: Lymph nodes on both sides of the diaphragm, spleen (IIIS), or both (IIIE&S)
Stage IV: Bone marrow or other non-hematolymphoid organ
World Health Organization Classification of B-Lymphoid Neoplasms

Precursor B-cell Neoplasms
Precursor B-lymphoblastic leukemia/lymphoma
Mature (peripheral) B-cell Neoplasms
B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma
B-cell prolymphocytic leukemia
Lymphoplasmacytic lymphoma
Splenic marginal zone B-cell lymphoma
Hairy cell leukemia
Plasma cell myeloma/plasmacytoma
Extranodal marginal zone B-cell lymphoma of MALT type
Nodal marginal zone B-cell lymphoma
Follicular lymphoma
Mantle-cell lymphoma
Diffuse large B-cell lymphoma
Burkitt lymphoma
World Health Organization Classification of T- and NK-Lymphoid Neoplasms

Precursor T-cell Neoplasms
Precursor T-lymphoblastic lymphoma/leukemia
Mature (peripheral) T-cell Neoplasms
T-cell prolymphocytic leukemia
T-cell granular lymphocytic leukemia
Aggressive NK-cell leukemia
Adult t-cell lymphoma/leukemia
Extranodal NK/T-cell lymphoma
Enteropathy-type T-cell lymphoma
Hepatosplenic gamma-delta T-cell lymphoma
Subcutaneous panniculitis-like T-cell lymphoma
Mycosis fungoides/Sezary syndrome
Peripheral T-cell lymphoma, not otherwise characterized
Angioimmunoblastic T-cell lymphoma
Anaplastic large-cell lymphoma
Lymphoma Classification
1. Harris NL, Jaffe ES, Diebold J, et al: The World Health Organization classification of neoplasms of the hematopoietic
and lymphoid tissues: report of the Clinical Advisory Committee meeting--Airlie House, Virginia, November, 1997.
Hematol J 1:53, 2000
2. Jaffe ES, Harris NL, Stein H, et al (eds): World Health Organization Classification of Tumours: Pathology and
Genetics of Tumours of Haematopoietic and Lymphoid Tissues), Lyon: IARC Press, 2001
Imaging of non Hodgkin lymphoma

1. Byun JH, Ha HK, Kim AY, et al: CT Findings in Peripheral T-Cell Lymphoma Involving the Gastrointestinal Tract.
Radiology 227:59, 2003
2. Choi D, Lim HK, Lee SJ, et al: Gastric mucosa-associated lymphoid tissue lymphoma: helical CT findings and
pathologic correlation. AJR 178:1117, 2002
3. Crump M, Gospodarowicz M, Shepherd FA: Lymphoma of the gastrointestinal tract. Semin Oncol 26:324, 1999
4. Gossios K, Katsimbri P, Tsianos E: CT features of gastric lymphoma. Eur Radiol 10:425, 2000
5. Isaacson PG: Gastrointestinal lymphoma. Hum Pathol 25:1020, 1994
6. Isaacson PG: Gastrointestinal lymphomas of T- and B-cell types. Mod Pathol 12:151, 1999
7. Isaacson PG: Intestinal lymphoma and enteropathy. J Pathol 177:111, 1995

8. Isaacson PG: Mucosa-associated lymphoid tissue lymphoma. Semin Hematol 36:139, 1999
9. Isaacson PG, MacLennan KA, Subbuswamy SG: Multiple lymphomatous polyposis of the gastrointestinal tract.
Histopathology 8:641, 1984
10. Kessar P, Norton A, Rohatiner AZ, et al: CT appearances of mucosa-associated lymphoid tissue (MALT) lymphoma.
Eur Radiol 9:693, 1999
11. Levine MS, Elmas N, Furth EE, et al: Helicobacter pylori and gastric MALT lymphoma. AJR Am J Roentgenol
166:85, 1996
12. Levine MS, Rubesin SE, Pantongrag-Brown L, et al: Non-Hodgkin's lymphoma of the gastrointestinal tract:
radiographic findings. AJR Am J Roentgenol 168:165, 1997
13. Megibow AJ, Balthazar EJ, Naidich DP, et al: Computed tomography of gastrointestinal lymphoma. AJR 141:541,
1983
14. Park MS, Kim KW, Yu JS, et al: Radiographic findings of primary B-cell lymphoma of the stomach: low-grade versus
high-grade malignancy in relation to the mucosa-associated lymphoid tissue concept. AJR 179:1297, 2002
15. Rodallec M, Guermazi A, Brice P, et al: Imaging of MALT lymphomas. Eur Radiol 12:348, 2002
16. Sheth S, Horton KM, Garland MR, et al: Mesenteric Neoplasms: CT Appearances of Primary and Secondary Tumors
and Differential Diagnosis. Radiographics 23:457, 2003
AIDS-related lymphomas
1. Albin J, Lewis E, Eftekhari F, et al: Computed tomography of rectal and perirectal disease in AIDS patients. Gastrointest
Radiol 12:67, 1987
2. Brar HS, Gottesman L, Surawicz C: Anorectal pathology in AIDS. Gastrointest Endosc Clin N Am 8:913, 1998
3. Burkes RL, Meyer PR, Gill PS, et al: Rectal lymphoma in homosexual men. Arch Intern Med 146:913, 1986
4. Ferrozzi F, Tognini G, Mulonzia NW, et al: Primary effusion lymphomas in AIDS: CT findings in two cases. Eur
Radiol 11:623, 2001
5. Gottlieb CA, Meiri E, Maeda KM: Rectal non-Hodgkin's lymphoma: a clinicopathologic study and review. Henry
Ford Hosp Med J 38:255, 1990
6. Ioachimm HL, Antonescu C, Giancotti F, et al: EBV-associated anorectal lymphomas in patients with acquired immune
deficiency syndrome. Am J Surg Pathol 21:997, 1997?
7. Munn S: Imaging HIV/AIDS. Burkitt's lymphoma. AIDS Patient Care STDS 16:395, 2002
Post-transplantation lymphoproliferative disorder

1. Meador TL, Krebs TL, Cheong JJ, et al: Imaging features of posttransplantation lymphoproliferative disorder in
pancreas transplant recipients. AJR 174:121, 2000
2. Pickhardt PJ, Siegel MJ: Abdominal manifestations of posttransplantation lymphoproliferative disorder. AJR Am J
3. Pickhardt PJ, Siegel MJ: Posttransplantation lymphoproliferative disorder of the abdomen: CT evaluation in 51
patients. Radiology 213:73, 1999
4. Pickhardt PJ, Siegel MJ, Hayashi RJ, et al: Posttransplantation lymphoproliferative disorder in children: clinical,
histopathologic, and imaging features. Radiology 217:16, 2000
5. Tubman DE, Frick MP, Hanto DW: Lymphoma after organ transplantation: radiologic manifestations in the central
nervous system, thorax, and abdomen. Radiology 149:625, 1983
6. Vrachliotis TG, Vaswani KK, Davies EA, et al: CT findings in posttransplantation lymphoproliferative disorder of
renal transplants. AJR Am J Roentgenol 175:183, 2000
7. Wu L, Rappaport DC, Hanbidge A, et al: Lymphoproliferative disorders after liver transplantation: imaging features.
Abdom Imaging 26:200, 2001

Small Intestinal Neoplasms
Small Intestinal Neoplasms: Introduction

• Small bowel neoplasms
➢ Uncommon
➢ Spectrum from hamartoma to benign to malignant
➢ Majority of malignant small bowel tumors are metastatic
➢ Primary small bowel tumors account for 1% to 2% of GI malignancies
• Most common benign small bowel tumors
➢ Adenoma
➢ GIST (may be benign or malignant)
➢ Lipoma
• Location of benign tumors
➢ Least common in duodenum
➢ Most common in ileum
• Primary malignant small bowel tumors
➢ Strong associations with chronic inflammation: sprue, Crohn disease
➢ Association with precursor conditions: FAP, MEN, NF1, Peutz-Jegher
• Trend in distribution of malignant tumors is relative to histology
➢ Adenocarcinoma most common in duodenum
➢ Carcinoid most common in ileum
➢ Lymphoma most common in ileum
➢ GISTs, even distribution throughout small bowel
➢ Many occult and asymtomatic until advanced disease
➢ Symptoms dependent upon location and tumor morphology
➢ Symptoms are similar in benign, malignant, primary or secondary
• Diagnostic approach
➢ Differential diagnosis based upon location, morphology, and associated
conditions
Small Intestinal Neoplasms: Objectives

• Case based approach
➢ Tumors of proximal duodenum
➢ Periampullary tumors
➢ Polypoid jejunal/ileal tumors
➢ Annular tumors of jejunum and ileum
➢ Tumors associated with adjacent disease in the mesentery
➢ Tumors associated with NF1
• Case based approach
➢ Brunner gland lesions
➢ Adenomas
➢ Adenocarcinomas
➢ Carcinoid
➢ GISTs
38-year-old man with recent onset of abdominal pain Figure 2-10-1

[Figure 2-10-1]
Brunner gland hamartoma
Gastrointestinal Radiology 353 Small Intestinal Neoplasms

Differential Diagnosis: Proximal Duodenal Polyp
• Nonneoplastic
➢ Brunner gland hamartoma
➢ Heterotopia
➢ Prolapsed antral mucosa
➢ Peutz-Jegher hamartoma
• Neoplastic
➢ Adenoma
➢ Adenocarcinoma
➢ GIST
➢ Carcinoid
➢ Prolapsed gastric neoplasm
Brunner Gland Hamartoma [Figure 2-10-1]

• Solitary hamartoma
➢ Brunner glands, muscular, and fatty elements
➢ Heterotopic pancreatic acini and ducts
• Synonym: Brunner gland adenoma
• Most common in duodenal bulb
• Clinical
➢ Peak incidence, 4th to 6th decade
➢ Asymptomatic or rarely, obstruction or bleeding
• Treatment
➢ Resection
Brunner Gland Hamartoma

➢ Solitary
➢ Sharply circumscribed polyp
➢ Proximal duodenum
• Homogenous CT attenuation
➢ Composed of mostly glandular elements
• Heterogeneous CT attenuation
➢ Abundant fat, smooth muscle, and cystic change
Brunner Gland Hyperplasia

• Smaller, multifocal version of Brunner hamartoma
• Clinical associations
➢ Duodenal ulcers Figure 2-10-2
➢ Gastric hypersecretory states
• Treatment
➢ None
Brunner Gland Hyperplasia [Figure 2-10-2]

➢ Brunner gland hyperplasia
➢ Lymphoid hyperplasia
➢ Duodenitis
➢ Adenomas in FAP
➢ Hamartomas in Peutz-Jegher
➢ Heterotopia
Brunner gland hyperplasia
Small Intestinal Neoplasms 354 Gastrointestinal Radiology

68-year-old woman with recurrent pancreatitis
[Figure 2-10-3]
Differential Diagnosis Figure 2-10-3

Periampullary Duodenal Mass
• Nonneoplastic
➢ Choledochocele
➢ Duplication cyst
➢ Peutz-Jegher hamartoma
• Neoplastic
➢ Adenoma/adenocarcinoma
➢ Carcinoid/NF1
➢ GIST Periampullary tubulovillous adenoma
➢ Adjacent pancreatic or ampullary adenocarcioma
Figure 2-10-4
Tubulovillous Adenoma
Adenoma
• Benign intraepithelial neoplasm composed of
dysplastic cells
➢ Tubular, villous, or tubulovillous histology
➢ May progress to adenocarcinoma
• Locations
➢ 80% are periampullary
• Increased incidence
➢ Familial adenomatous polyposis, FAP
➢ Hereditary nonpolyposis colon carcinoma,
HNPCC
Periampullary Adenocarcinoma
Periampullary adenocarcinoma
➢ Biliary obstruction
➢ Duodenal mural thickening or polypoid mass
➢ May extend into adjacent pancreas and/ampulla Figure 2-10-5
Adenocarcinoma Duodenum:
Ampullary/Periampullary
[Figures 2-10-4 and 2-10-5]
• May arise from periampullary duodenal mucosa
• May arise from ampulla
• May be mixed location
➢ Origin not clear
Small Bowel Adenocarcinoma

• More common in proximal small intestine1
➢ 55% periampullary/ampullary
➢ 10% duodenum
➢ 25% jejunum
➢ 10% ileum
• Association with colonic adenocarcinoma Ampullary adenocarcinoma
➢ APC gene
➢ Mismatch repair gene
1Riddel RH, Petras RE, Williams GT, Sobin LH. Atlas of Tumor Pathology:Tumors
of the Intestines. AFIP 2003

Small Bowel Adenocarcinoma
• Most patients between 50 and 60 years
• Predisposing conditions
➢ Inherited syndromes: FAP, HNPCC, Peutz-Jegher, NF1
➢ Chronic inflammation: sprue, Crohn disease, ileostomies, ileal pouches,
bypassed bowel
Differential Diagnosis: Small Bowel, Intussuscepting Mass

• Benign
➢ Adenoma
➢ Peutz Jegher polyp
➢ Lipoma
➢ Uncommon
❖ Neurofibroma
❖ Schwannoma
❖ Inflammatory fibroid polyp
Figure 2-10-6
❖ Heterotopia
• Malignant
➢ Metastasis
➢ Adenocarcinoma
➢ Lymphoma
➢ GIST
➢ Carcinoid
Jejunal Adenocarcinoma: Annular Adenocarcinoma of the jejunum with annular morphology

[Figure 2-10-6]
• CT features
➢ Focal, annular mural thickening
➢ Spiculated or irregular margins
➢ +/- mesenteric adenopathy Figure 2-10-7
Ileal Adenocarcinoma: Annular
and Infiltrating [Figure 2-10-7]
Differential Diagnosis: Jejunal or

Ileal Stricture
• Neoplastic
➢ Adenocarcinoma
➢ Carcinoid Adenocarcinoma of the ileum with annular and infiltrating
➢ Lymphoma morphology
➢ Metastasis
• Nonneoplastic
➢ Crohn disease
➢ Celiac disease
➢ NSAID (tend to be web-like)
➢ Ischemia
➢ Tuberculosis
➢ Heterotopia Figure 2-10-8
➢ Radiation
Ileal Adenocarcinoma:
Cavitary Mass [Figure 2-10-8]
• Unusual presentation for
adenocarcinoma
• More aggressive histology
➢ Poorly differentiated
➢ Endocrine features mixed
with adenocarcinoma Adenocarcinoma of the ileum manifesting as a cavitary mass

Differential Diagnosis: Jejunal or Ileal Cavitary Mass Figure 2-10-9
• Lymphoma
➢ Look for homogenous attenuation tumor
• GIST
• Adenocarcinoma
50-year-old man with abdominal pain and

diarrhea
[Figures 2-10-9 and 2-10-10]
• CT findings
➢ Fixed segment of ileum
➢ Adjacent spiculated mesenteric mass
Carcinoid
Carcinoid
• Well-differentiated endocrine neoplasms
• Classification
➢ Foregut, stomach and proximal duodenum
➢ Midgut (60% to 80%), distal duodenum, jejunum, ileum, appendix,
ascending colon, proximal transverse colon Figure 2-10-10
➢ Hindgut, distal transverse colon,
descending colon, rectum
• Spectrum of clinical/imaging features
➢ Population and type of endocrine
cell changes throughout the bowel
➢ Variety of hormones produced
➢ Biologic behavior ranges from
benign to malignant
Carcinoid
Duodenal Carcinoid
• Most common in first and second portion
➢ Low-grade malignancies
➢ Gastrin or somatostatin production most common
➢ Periampullary tumors = somatostatin producing and NF1 association
• Associations
➢ Zollinger-Ellison syndrome
➢ Multiple endocrine neoplasia (MEN 1)
➢ Neurofibromatosis type 1 (NF1) Figure 2-10-11
Duodenal Carcinoid [Figure 2-10-11]
➢ Solitary or multifocal polyps
➢ Intramural mass
Jejunal and Ileal Carcinoid

• Aggressive biologic behavior
• Serotonin production
➢ Desmoplasia
➢ Kinking of bowel Duodenal carcinoid
➢ Spiculation of mesentery
➢ Ischemia from "elastic vascular sclerosis”
➢ May have carcinoid syndrome with liver mets

Jejunal and Ileal Carcinoid: Imaging Features
• Discrete mass in wall of bowel Figure 2-10-12
➢ Mural mass
➢ Polypoid mass
➢ Multiple masses, less
common
• Extensive wall abnormalities
➢ Luminal narrowing
➢ Thick, spiculated folds
• Local nodal mesenteric
metastasis often most prominent
feature
➢ Spiculated, fibrotic mass
adjacent to bowel
➢ Sunburst pattern of vessels
on angiogram
➢ May calcify
Carcinoid Ileum [Figure 2-10-12]
Carcinoid Ileum
• ”Elastic vascular sclerosis”
• Sunburst pattern of mesenteric Ileal carcinoid
vessels
• Multifocal nodules
• Kinking of bowel
• Rigid segment of bowel
Somatostatin Receptor Scintigraphy:

Octreotide Scans (111In-Pentetreotide) [Figure 2-10-13]
Carcinoid Syndrome
• 10% of patients with carcinoids
Figure 2-10-13
• Most common with ileal
carcinoids
• Hepatic metastasis are usually
present
➢ Serotonin and metabolites in
systemic circulation
• Classic syndrome
➢ Paroxysms of sweating,
flushing, cyanosis, wheezing,
abdominal colic, right-sided Metastatic carcinoid shown on CT and 111In-pentetreotide
heart failure, diarrhea scintigraphy
➢ Symptoms precipitated by
ETOH intake, stress, exercise
• Carcinoid heart disease
➢ Right sided valvular dysfunction
➢ Congestive heart failure
Carcinoid: Differential Diagnosis

Figure 2-10-14
• Lymphoma
• Granulomatous infection
• Sclerosing mesenteritis
77-year-old asymptomatic man

[Figure 2-10-14]

Differential Diagnosis: Figure 2-10-15
Small Bowel Polypoid Mass
• Benign
➢ Adenoma
➢ Peutz Jegher polyp
➢ Inflammatory fibroid polyp
➢ Inflammatory pseudotumor
• Malignant
➢ Adenocarcinoma
➢ Lymphoma
➢ GIST
➢ Carcinoid

• Small bowel second most common site
• Variable biologic behavior
GIST: Small Bowel, polypoid Figure 2-10-16
GIST: Small Bowel, mural

[Figure 2-10-15]
GIST: Small Bowel, polypoid

and exophytic
GIST: Small Bowel, exophytic

[Figure 2-10-16]
GIST: Small Bowel, cavitary
52-year-old man with NF-1 complains of abdominal pain
Gastrointestinal Neoplasms in NF-1

• Neurofibroma
• Carcinoid
➢ Duodenal
➢ Somatostatinoma
• Gastrointestinal stromal tumors
➢ Small intestine, multiple
• Ganglioneuroma
• Leiomyoma, leiomyosarcoma
• Adenocarcinoma
Metastatic Disease
• Most common site for metastasis in GI tract
• Metastasis are more common than primary malignancies in the small bowel
• Widespread metastatic disease usually present
➢ Melanoma
➢ Lung
➢ Breast
➢ Kidney
• Intraperitoneal, direct extension, lymphatic spread
➢ Tumors of GI origin
➢ Ovarian and endometrial carcinoma
• Imaging patterns
➢ Identical to primary neoplasms
➢ Polyps, mural masses, annular strictures, cavitary lesions, association with
mesenteric nodal masses

Metastatic Disease: Renal Cell Carcinoma [Figure 2-10-17] Figure 2-10-17
Metastatic Disease:
Melanoma [Figure 2-10-18]
Summary: Brunner Gland

Lesions
• Brunner gland hamartoma
➢ Solitary mass
• Brunner gland hyperplasia
Renal cell carcinoma metastatic to the small bowel
➢ Multiple nodules
Summary: Adenoma
• Uncommon
• Most periampullary
• Association Figure 2-10-18
➢ FAP
➢ HNPCC
Summary: Adenocarcinoma
• Periampullary location most
common
• Morphology
➢ Polypoid
➢ Annular
➢ Infiltrating
➢ Cavitary Melanoma metastatic to the small bowel
Summary: Carcinoid
• Endocrine neoplasms
• Midgut most common
➢ Serotonin production
➢ Octreotide scintigraphy
• Key imaging features
➢ Mural wall thickening
➢ Fixation of bowel
➢ Mesenteric mass
➢ Mesenteric retraction
Summary: GIST
➢ KIT positive
• Mural masses
➢ Intraluminal polyp
➢ Exophytic component
➢ Hemorrhage
➢ Cyst formation
➢ Cavitation
Summary: Metastatic Disease

• Most common malignancy in the small bowel

Colorectal Carcinoma
Colorectal Carcinoma: Objectives

• Epidemiology/pathogenesis
• Screening
• Detection
➢ Preoperative assessment
• Staging
➢ Rectal carcinoma
Colorectal Carcinoma
• Third most frequent cancer in the U.S.1
➢ ~150,000 new cases per year
➢ 11% of cancers in men and women
➢ 10% of cancer deaths
1Jemal A et al. CA Cancer J Clin 2005; 55:10-30
Colorectal Carcinoma: Risk Factors

• Lifetime risk 6%
• Incidence increases after age 50
• Familial risk
➢ 2 to 4 fold increase risk with a single first degree relative
➢ 3 to 6 fold increase risk with two first degree relatives
• Increased risk
➢ Familial adenomatous polyposis syndrome (FAP)
➢ Inflammatory bowel disease
Colorectal Carcinoma: Pathogenesis

• Adenoma-Carcinoma Sequence
➢ Slow evolution to cancer, average 10 years
➢ Adenoma detection and removal = cure
• Exception to adenoma-carcinoma sequence
➢ Carcinomas in inflammatory bowel disease
Adenoma-Carcinoma Sequence [Figures 2-11-1]
Colorectal Carcinoma: Role of Radiology

• Screening
➢ ACBE Figure 2-11-1
➢ CT colonography
• Detection
➢ Symptomatic patients
• Preoperative screening
➢ Primary disease complications
➢ Preoperative staging
• Recurrent disease
Adenoma to carcinoma sequence progressive from normal

mucosa, unicryptal adenoma, polypoid adenoma, dysplasia,
high-grade dysplasia, carcinoma in-situ, to invasive carcinoma
Gastrointestinal Radiology 361 Colorectal Carcinoma

Colorectal Carcinoma: American Cancer Society Screening
Recommendations
• Average risk adults begin screening at age 50
➢ Annual fecal occult blood (FOBT) or fecal immunochemical test (FIT)
➢ Sigmoidoscopy every 5 years
➢ Annual FOBT or FIT + Sigmoidoscopy every 5 years
➢ Colonoscopy every 10 years
➢ DCBE every 5 years
Colorectal Carcinoma: American College of Gastroenterology

Polyp guidelines1
• Colonoscopy every 3 years, high risk for metachronous adenomas
➢ >2, >1cm, villous histology or high-grade dysplasia
• Colonoscopy every 5 years, low risk for metachronous adenomas
➢ 1-2 tubular adenomas, no family history
1Bond JH. Am J Gastroenterology 2000. 95(11): 3053-3063
Colorectal Carcinoma: Screening

• Air contrast barium enema
➢ Accuracy 90% for polyps >1 cm
➢ Pitfalls
❖ Anatomic difficulties (overlapping segments)
❖ Diverticular disease
❖ Perceptive errors
• Colonoscopy
➢ Accuracy 90%
➢ Invasive, requiring sedation
➢ Perforation rate .1% to .5%
➢ Pitfalls
❖ Failure to reach cecum
❖ Blind spots
Colorectal Carcinoma: Screening

• Virtual colonography
➢ Sensitivity 73% to 93% for >10mm polyps
➢ Prone and supine imaging improves sensitivity
➢ Difficult lesions
❖ Poor bowel preparation
❖ Flat adenomas
❖ Adenomas on folds
❖ Adenomas seen in only one position
Colorectal Polyps: Histologic Spectrum

• Hyperplastic
➢ Most common
➢ Usually <5 mm, descending colon and rectum
➢ NOT neoplastic
• Adenoma
➢ Tubular, 75% are <1 cm, most pedunculated
➢ Villous, 60% are > 2 cm, most sessile
➢ Mixed
• Juvenile
• Peutz-Jeghers
• Inflammatory/post-inflammatory
Tubular Adenoma
Villous Adenoma
Colorectal Carcinoma 362 Gastrointestinal Radiology

Adenoma Figure 2-11-2
• Size
➢ < 5 mm, benign
➢ 5 mm to 1 cm, 1% are carcinoma
➢ 1 - 2 cm, 10% are carcinoma
➢ > 2 cm, 30% to 50% are carcinoma
• Synchronous adenomas
➢ 40% to 50%
• Recurrence
➢ 20% to 60% recurrence rate
➢ Majority recur within 2 years
Adenoma: Barium Features

• Filling defect in barium pool
• Protrusion into the lumen
➢ “Innies not Outies”
➢ Bowler hat sign
➢ Sessile or pedunculated
• Carpet lesions
➢ Sessile lesions
➢ Bubbly or nodular contour
➢ Villous change
Bowler Hat Sign [Figure 2-11-2]
Virtual Colonography
Villous Adenoma
• Higher rate of malignancy
• Recurrence rate 9.3%
• Three types Sessile adenomatous polyp showing
➢ Flat, carpet-like the Bowler Hat sign
➢ Sessile, lobulated
➢ Pedunculated
• Histology
➢ Nonbranching finger-like fronds
Villous Adenoma: Pathology [Figure 2-11-3]
Figure 2-11-3
Villous adenoma of the cecum showing a bubbly, carpet-like

appearance

Villous Adenoma: CT Features [Figure 2-11-4] Figure 2-11-4
➢ Sessile
➢ Eccentric
➢ Stalk
➢ Expands lumen
• Irregular luminal margin
➢ Low attenuation luminal margin
➢ High mucin content
Villous adenoma of the rectum showing low attenuation along
Colonic Adenocarcinoma the luminal margin
Colorectal Carcinoma: Distribution [Figure 2-11-5]
Colorectal Carcinoma: Clinical Presentation Figure 2-11-5

• Minimal or absent symptoms in up to 12% of patients
• Bleeding
➢ Initial complaint in 50%
• Weight loss, malaise
• Pain
• Change in bowel habits
• Right vs. left sided lesions
• Symptoms from complications
➢ Obstruction, ischemia, perforation, peritonitis,
fistula
Colorectal Carcinoma: Morphologic Patterns

• Polypoid
➢ Intraluminal masses
➢ Bulky, fungating masses in cecum and ascending
colon
• Infiltrating/annular constricting
➢ Transverse, descending, and sigmoid colon
➢ Encircle the bowel
➢ “Apple core” Distribution of colorectal carcinoma
➢ Diffuse infiltration (linitis plastica) uncommon
• Ulcerating
➢ Deeply invade colonic wall
➢ Edge of tumor slightly elevated above normal mucosa
• Flat plaques
➢ Carcinomas from flat adenomas
➢ Carcinomas in inflammatory bowel disease
Colorectal Carcinoma: Computed Tomography

• Primary Tumor
➢ Discrete mass
• Extension beyond the bowel
➢ Irregular outer margin
➢ Soft-tissue stranding in pericolonic fat
• Adjacent organ/muscle invasion
➢ Loss of fat planes
➢ Tumor mass in adjacent organ or muscle
• Lymph node metastasis

Polypoid Adenocarcinoma Figure 2-11-6
[Figure 2-11-6]
Annular Adenocarcinoma
[Figure 2-11-7]
Infiltrating Adenocarcinoma
[Figure 2-11-8]
Pericolonic Extension and

Adenopathy Polypoid adenocarcinoma of the cecum showing pericolonic
extension
Adjacent Organ Invasion: Figure 2-11-7
Contiguous Soft Tissue
Attenuation [Figure 2-11-9]
Adjacent Organ Invasion
Coloduodenal Fistula
Multiple Carcinomas
• Synchronous carcinomas
➢ Diagnosed within 6 months of each Annular adenocarcinoma of the distal transverse colon
other
➢ Incidence 1.5% to 12% Figure 2-11-8
➢ Most are >5 cm away from each
other
• Metachronous carcinomas
➢ Incidence 0.6% to 9.1%
➢ Time interval to second lesion
discovery
❖ 64% within 5 years
❖ 45% within 3 years
❖ 20% within 1 year
Infiltrating adenocarcinoma of the sigmoid colon with
• 8% to 20% of patients with colorectal
pericolonic extension and pericolonic adenopathy
carcinomas have malignancies in other
organs
Figure 2-11-9
Synchronous Carcinomas
[Figure 2-11-10]
Rectal adenocarcinoma (T4) showing contiguous soft tissue

attenuation into the pelvic side walls consistent with adjacent
organ invasion
Figure 2-11-10
Synchronous adenocarcinomas of the hepatic flexure and

descending colon

Colonic Adenocarcinoma in Inflammatory Bowel Disease: Figure 2-11-11
[Figure 2-11-11]
• Ulcerative colitis
➢ Highest incidence
• Crohn disease
➢ Large and small intestinal
adenocarcinoma
• Features of carcinoma in IBD
➢ Typically do not arise in pre-existing
adenomas
➢ Arise in flat mucosa
➢ Carcinomas may be long and flat
Adenocarcinoma in Ulcerative
Colitis
Colorectal Carcinoma:
Complications Adenocarcinoma in ulcerative colitis
• Bleeding
➢ Occult
➢ Chronic anemia
➢ Massive bleeding, unusual Figure 2-11-12
• Obstruction
➢ Occlusion of the colonic lumen
➢ Colocolic intussusception
• Perforation
➢ Abscess
➢ Fistula
➢ Differential diagnosis, diverticulitis
CT of Obstructing Colon
Colonic ischemia in an obstructing carcinoma of the
Carcinomas
descending colon
• IV contrast
• Identify obstructing lesion Figure 2-11-13
➢ Infiltration of adjacent fat
• Evaluate bowel integrity
➢ Obstructive colitis (1% to 7%)
➢ Ischemic changes
➢ Pneumatosis
• Stage
➢ Local extension
➢ Lymph node mets
➢ Liver mets Perforated adenocarcinoma of the transverse colon with
abscess formation
CT of Obstructing Colon Carcinomas
Ischemia in Obstructive Cancers [Figure 2-11-12]
Carcinoma with Perforation and Abscess [Figure 2-11-13]
Colorectal Carcinoma:Role of Preoperative Imaging

• Tumors proximal to the rectum are staged surgically
➢ Preoperatively image patients with clinical evidence of advanced disease
• Preoperative imaging rectal tumors
➢ EUS and CT
➢ MR

Colorectal Carcinoma: Preoperative CT
• Local tumor extension
➢ Early rim enhancement, followed by hyperdensity
➢ Hypodense in the portal venous phase
➢ Isodense in the equilibrium phase
• Lymphatic Spread
Rectal Adenocarcinoma: Preoperative EUS

• Endoscopic Ultrasound (EUS)*
➢ 360 degree probe
➢ Normal 5-layer rectal wall
➢ T stage accuracy 69% to 97%
➢ Nodal accuracy 70% to 80%
• EUS limitations
➢ EUS best at early stage tumors
➢ Limited assessment because of location or bulk occurs
➢ May overstage (fibrosis vs. tumor vs. inflammation)
➢ Intraobserver variability
Wolfman NT, Ott DJ. Endoscopic Ultrasonography. Semin Roentgenol 1996. 31(2):
154-161.
Beets-Tan RGH, Beets GL. Rectal cancer: review with emphasis on MR imaging.
Radiology 2004. 232: 335-346
Rectal Adenocarcinoma: Preoperative MR

• Endoluminal MR
➢ Equal accuracy for early stage tumors to EUS
➢ T stage accuracy, 71% to 91%
• Phased array MR
➢ High spatial resolution
➢ Large field of view
➢ Limitations differentiating T2 and T3 lesions
Colorectal Carcinoma: Role of PET

• No role in screening/diagnosis
• Preoperative staging
➢ Highly sensitive for liver mets
➢ Not sensitive for T staging and nodal mets
• Detection of recurrent disease
➢ Following liver met resection/treatment
➢ Scar vs. recurrent tumor at resection margin
Hustinx R. PET imaging in assessing gastrointestinal tumors. RCNA 2004. 112 (6)
1123-1139.
Rectal Adenocarcinoma: Management

• High T1 or T2 lesion
➢ Lesions 5 to 6 cm above dentate line or at peritoneal reflection
➢ Primary resection and anastomosis (LAR)
• Low T1 or T2 lesion
➢ APR (Miles procedure), LAR, coloanal anastomosis with J-pouch, local or
transanal excision, total mesorectal excision, posterior proctotomy
• T3 or T4
➢ Downstage with preoperative neoadjuvant chemoradiation
➢ APR and post operative XRT, adjuvant chemotherapy

TNM Staging [Figure 2-11-14] Figure 2-11-14
• T-Primary tumor
➢ T1 invades submucosa
➢ T2 invades muscularis propria
➢ T3 through muscularis propria or
into nonperitonealized pericolic fat
➢ T4 perforates visceral peritoneum or
directly invades adjacent organs or
structures
• N-Regional nodes
• M-Distant metastasis
EUS Layer 1: Hyperechoic

superficial mucosa
EUS Layer 2: Hypoechoic deep

mucosa
EUS Layer 3: Hyperechoic

submucosa
EUS Layer 4: Hypoechoic

TNM Staging for colorectal carcinoma
muscularis propria
EUS Layer 5: Hyperechoic perirectal fat [Figure 2-11-15]
T1 N0 M0
T2 N0 M0 Figure 2-11-15
T3 N2 M0 [Figure 2-11-16]
T3 N10 M0
T3 N8 M1
T4
T4: Extension to pelvic side

wall
T4: Extension to labia Normal anatomy of the colon wall

with endoscopic ultrasound
Figure 2-11-16
T3N2M0 rectal adenocarcinoma

Colorectal Carcinoma: Figure 2-11-17
Lymphatic Spread [Figure 2-11-17]
• Pericolonic nodes
➢ Paracolic
➢ Epiploic
• Mesenteric Nodes
➢ Intermediate nodes
• Principal nodes
➢ SMA
➢ IMA
Pericolonic Nodes
Intermediate
Principal
Rectal Adenocarcinoma:
Lymphatic Drainage [Figures 2-11-18 and 2-11-
19]
• Pararectal nodes
• Internal iliac nodes
➢ Tumors above dentate line
• Inguinal nodes
➢ Tumors below dentate line
Distribution of
Figure 2-11-18 lymphatic spread for
colon carcinoma
Distribution of lymphatic spread for rectal carcinoma

Figure 2-11-19
Inguinal lymph nodes in a rectal adenocarcinoma that

extended below the dental line

Summary: Adenoma
• 40% - 50% synchronous
• 20% - 60% recur
• BE features
➢ Filling defect
➢ Bowler hat
➢ Sessile
➢ Pedunculated
Summary: Villous Adenoma

• Carpet lesions
• Bubbly appearance
• Expand lumen
• Low attenuation on luminal surface
Summary: Primary Tumor

• Morphology
➢ Polypoid
➢ Infiltrating/annular
➢ Ulcerating
➢ Flat plaques
• Synchronous carcinomas
• CT
➢ Local extent
Summary: Complications
• Bleeding
➢ Usually chronic blood loss
➢ Massive GI bleed, unusual
• Obstruction
➢ CT
➢ Identify lesion and bowel wall integrity
• Perforation
➢ Abscess
➢ Fistula
➢ Differential diagnosis inflammatory disorders
Summary: Role of Imaging

• Preoperative CT
➢ Local tumor extent
➢ Liver metastasis
➢ Lymphatic spread
Summary: Rectal Adenocarcinoma

• Preoperative staging
➢ EUS and CT, MR
• T3 lesions
➢ Through muscularis propria
➢ Spiculated outer margin on CT
➢ Perirectal adenopathy

References:
Virtual Colonography
1. Fidler JL, Johnson CD, MacCarty RL, et al: Detection of flat lesions in the colon with CT colonography. Abdom
Imaging 27:292, 2002
2. Fletcher JG, Johnson CD, MacCarty RL, et al: CT colonography: potential pitfalls and problem-solving techniques.
AJR Am J Roentgenol 172:1271, 1999
3. Fletcher JG, Johnson CD, Welch TJ, et al: Optimization of CT colonography technique: prospective trial in 180
patients. Radiology 216:704, 20
4. Gluecker TM, Fletcher JG, Welch TJ, et al: Characterization of Lesions Missed on Interpretation of CT Colonography
Using a 2D Search Method. AJR Am J Roentgenol 182:881, 2004
5. Gluecker TM, Johnson CD, Harmsen WS, et al: Colorectal cancer screening with CT colonography, colonoscopy,
and double-contrast barium enema examination: prospective assessment of patient perceptions and preferences.
Radiology 227:378, 2003
6. Johnson CD, Ahlquist DA: Computed tomography colonography (virtual colonoscopy): a new method for colorectal
screening. Gut 44:301, 1999
7. Johnson CD, Harmsen WS, Wilson LA, et al: Prospective blinded evaluation of computed tomographic colonography
for screen detection of colorectal polyps. Gastroenterology 125:311, 2003
8. Johnson CD, Toledano AY, Herman BA, et al: Computerized tomographic colonography: performance evaluation in
a retrospective multicenter setting. Gastroenterology 125:688, 2003
9. Macari M: Virtual colonoscopy: clinical results. Semin Ultrasound CT MR 22:432, 2001
10. Pescatore P, Glucker T, Delarive J, et al: Diagnostic accuracy and interobserver agreement of CT colonography (virtual
colonoscopy). Gut 47:126, 2000
11. Pickhardt PJ: Three-dimensional endoluminal CT colonography (virtual colonoscopy): comparison of three
commercially available systems. AJR Am J Roentgenol 181:1599, 2003
12. Pickhardt PJ, Choi JR, Hwang I, et al: Computed tomographic virtual colonoscopy to screen for colorectal neoplasia
in asymptomatic adults. N Engl J Med 349:2191, 2003
13. Royster AP, Fenlon HM, Clarke PD, et al: CT colonoscopy of colorectal neoplasms: two-dimensional and three-
dimensional virtual-reality techniques with colonoscopic correlation. AJR Am J Roentgenol 169:1237, 1997
14. Spinzi G, Belloni G, Martegani A, et al: Computed tomographic colonography and conventional colonoscopy for
colon diseases: a prospective, blinded study. Am J Gastroenterol 96:394, 2001
15. Taylor SA, Halligan S, Bartram CI: CT colonography: methods, pathology and pitfalls. Clin Radiol 58:179, 2003
16. Taylor SA, Halligan S, Bartram CI, et al: Multi-detector row CT colonography: effect of collimation, pitch, and
orientation on polyp detection in a human colectomy specimen. Radiology 229:109, 2003
17. Taylor SA, Halligan S, Goh V, et al: Optimizing bowel preparation for multidetector row CT colonography: effect of
Citramag and Picolax. Clin Radiol 58:723, 2003
18. Taylor SA, Halligan S, Goh V, et al: Optimizing colonic distention for multi-detector row CT colonography: effect
of hyoscine butylbromide and rectal balloon catheter. Radiology 229:99, 2003

Mesenteric Masses and Cysts
Mesenteric Masses and Cysts: Objectives

• Definitions
➢ Review mesenteric anatomy
• Case based approach to differential diagnosis
➢ Mesenteric and omental cysts
➢ Mesothelioma
➢ Mesenteric Fibromatosis
➢ Sclerosing mesenteritis Figure 2-12-1
➢ Inflammatory myofibroblastic pseudotumor
➢ Extrapleural solitary fibrous tumor
MesentericAnatomy: Definitions
• Mesentery
➢ Double fold of peritoneum
➢ Connects an organ to the
abdominal wall
• Omentum
➢ Specialized mesentery
extending from stomach
to an adjacent organ
Anatomy Mesentery
[Figure 2-12-1]
• Transverse mesocolon
• Small bowel mesentery
• Sigmoid mesentery
• Mesoappendix
Anatomy: Omentum
[Figure 2-12-2]
• Greater omentum Normal posterior attachments of the
➢ Gastrocolic ligament mesentery in sagittal and AP planes
➢ Gastrosplenic ligament
➢ Gastrophrenic ligament
• Lesser omentum
➢ Gastrohepatic ligament
Figure 2-12-2
➢ Hepatoduodenal ligament
Mesenteric and Omental Cyst

• Descriptive term
• 5 histologic subtypes
➢ Defined by internal lining
Mesenteric and Omental Cyst

• Lymphangioma
➢ Endothelial lining
• Enteric duplication cyst
➢ Enteric lining with muscular wall
• Enteric cyst
➢ Enteric lining with a fibrous wall
• Mesothelial cyst
➢ Mesothelial lining
• Nonpancreatic pseudocyst
➢ No lining
Normal anatomy of greater and lesser omentum
Mesenteric Masses and Cysts 372 Gastrointestinal Radiology

35-year-old woman with increasing abdominal girth [Figure 2-12-3]
Differential Diagnosis: Cystic Mesenteric Mass

• Mesenteric cyst Figure 2-12-3
➢ Lymphangioma
➢ Enteric duplication cyst
➢ Enteric cyst
➢ Mesothelial cyst
➢ Nonpancreatic pseudocyst
• Cystic neoplasm
➢ Teratoma
➢ Cystic malignant mesothelioma
➢ Benign multicystic mesothelioma Lymphangioma
➢ Cystic soft tissue primary
➢ Pseudomyxoma peritonei
• Complex ascites
➢ Infectious, neoplastic
• Pseudocyst
➢ Internal hemorrhage, abscess
Lymphangioma Figure 2-12-4

• Benign
• Vascular origin
• Affect all ages
• Many anatomic sites
➢ 95% neck, axilla
➢ 5% mesentery
➢ Lymphangiomatosis
Abdominal Lymphangioma:
Lymphangioma of the greater omentum
Pathology
• Interconnecting cysts
• Endothelial lining
• Dilated lymphatic spaces
➢ Proteinaceous fluid
➢ Chyle, low attenuation
➢ Hemorrhage
Abdominal Lymphangioma: Imaging Features [Figures 2-12-4 and 2-12-5]

• Mesenteric, omental or retroperitoneal location
• Complex cyst
➢ Multilocular
➢ Enhancing septations
➢ Internal debris
• Closely associated with small bowel
• Lack features of free fluid Figure 2-12-5
➢ Mass effect
➢ Septations
➢ No fluid in dependent spaces
peritoneum
• Infiltration/insinuation
➢ Within mesentery and bowel
• Complications
➢ Small bowel obstruction
➢ Volvulus Mesenteric lymphangioma showing low attenuation
➢ Infection and insinuating growth
Gastrointestinal Radiology 373 Mesenteric Masses and Cysts

71-year-old woman with abdominal pain [Figure 2-12-6] Figure 2-12-6
• Pancreatic cystic neoplasm
• Pancreatic pseudocyst
• Mesenteric cyst
➢ Lymphangioma Enteric duplication cyst
➢ Enteric cyst
➢ Mesothelial cyst
• Cystic mesenteric neoplasm
➢ Cystic mesothelioma
Enteric Duplication Cyst
Enteric Cyst and Mesothelial Cyst

• Enteric cyst
➢ Variant of enteric duplication, does not contain muscular wall
➢ Rare
➢ Fusion failure of visceral/parietal peritoneum
• Nonspecific imaging features
• Similar appearance compared to enteric duplication cyst
Nonpancreatic Pseudocyst [Figure 2-12-7] Figure 2-12-7

• Old hematoma, abscess
• No histologic lining
• Imaging
➢ Thick walled
➢ Internal debris
55-year-old man, former shipyard

worker, with worsening abdominal
pain [Figure 2-12-8] Nonpancreatic pseudocyst
• Primary neoplasms
➢ Diffuse malignant mesothelioma
Figure 2-12-8
➢ Serous papillary carcinoma
➢ Intra-abdominal desmoplastic round
cell tumor
➢ Leiomyomatosis peritonealis
disseminata
• Diffuse Infection
➢ Tuberculosis
➢ Histoplasmosis
Diffuse Malignant Mesothelioma Diffuse malignant mesothelioma

• Malignancy of mesothelial origin
• Association with asbestos
• Variants
➢ Diffuse peritoneal malignant mesothelioma
➢ Cystic malignant mesothelioma

Diffuse Malignant Mesothelioma
• Gross Pathology
➢ Nodules, masses, caking
➢ Bowel encasement
➢ Thick, nodular peritoneum
➢ Ascites
• Histopathologic variants
➢ Desmoplastic
➢ Lymphohistiocytoid
➢ Small cell
➢ Papillary
Diffuse Peritoneal Malignant Mesothelioma

➢ Peritoneal soft tissue nodules
➢ Omental and mesenteric masses, nodules
➢ Ascites
➢ Bowel wall thickening
➢ Fixation of small bowel
Diffuse Peritoneal Malignant Mesothelioma: Peritoneal, Omental

Nodules and Masses Figure 2-12-9
Diffuse Malignant Mesothelioma:
Small Bowel Fixation
Cystic Malignant Mesothelioma
[Figure 2-12-9]
Benign Multicystic Mesothelioma

[Figure 2-12-10]
• Rare Cystic malignant mesothelioma
➢ Arises from pelvic peritoneum
• Unrelated to asbestos
• Unrelated to malignant mesothelioma
• Synonym Figure 2-12-10
➢ Multilocular peritoneal inclusion cyst
• Clinical symptoms
➢ Chronic pelvic pain
Benign Multicystic Mesothelioma

➢ Multicystic pelvic mass
Benign multicystic mesothelioma
➢ Enhancing septa
➢ Peritoneal surfaces of uterus, bladder
➢ May extend into upper abdomen
Benign Multicystic Mesothelioma: Differential Diagnosis

• Metastasis
➢ Mucinous adenocarcinoma
➢ Serous papillary carcinoma of ovary
• Cystic malignant mesothelioma
• Primary serous papillary carcinoma of peritoneum
• Infection with complex ascites
➢ Tuberculosis

31-year-old man complained of abdominal fullness and early
satiety. Physical exam revealed a palpable mass
[Figure 2-12-11] Figure 2-12-11
Differential Diagnosis:
Solid Mesenteric Mass
• Malignant
➢ Soft tissue sarcoma
➢ Lymphoma
➢ Gastrointestinal stromal tumor
• Benign Mesenteric fibromatosis
➢ Mesenteric fibromatosis
➢ Sclerosing mesenteritis
➢ Inflammatory pseudotumor Figure 2-12-12
Mesenteric Fibromatosis:
(Intraabdominal Fibromatosis or
Abdominal Desmoid)
• Classified as a deep fibromatosis
➢ Mesenteric, pelvic, retroperitoneal
➢ Abdominal wall
➢ Extraabdominal
• Benign proliferative process Mesenteric fibromatosis with low CT attenuation, located in the
➢ Locally aggressive greater omentum
➢ Recurs following excision
➢ Does not metastasize
➢ No gender predilection
➢ Most cases sporadic
➢ 13% associated with FAP
• Abdominal fibromatosis
➢ Most common in young women, 20-30 years of age
Mesenteric Fibromatosis: Pathologic Features

• Gross pathology
➢ Well-defined or infiltrative margins
• Histology
➢ “Melting insinuating” and “tentacular growth”
➢ Microscopic tumor infiltration into bowel
➢ Collagenous and/or myxoid stroma
Mesenteric Fibromatosis: Imaging Figure 2-12-13

• Homogeneous
➢ Collagenous stroma
➢ Myxoid stroma (low attenuation
CT/high signal T2 MR)
• Heterogeneous
➢ Bands of myxoid stroma “whorls”
Homogeneous Attenuation Mesenteric fibromatosis
Mesenteric Fibromatosis: Low CT Attenuation Myxoid Stroma

[Figure 2-12-12]
Mesenteric Fibromatosis : High T2 Signal [Figure 2-12-13]

Mesenteric Fibromatosis: MR Enhancement
Mesenteric Fibromatosis in FAP [Figure 2-12-14]

Figure 2-12-14
Mesenteric Fibromatosis
• Complications
➢ Fistula formation
➢ Perforation
➢ GI bleeding
Infiltrates small bowel wall Mesenteric fibromatosis in a patient with FAP. The myxoid
stroma creates a “whorled” pattern in this example
Mesenteric Fibromatosis in FAP
• Almost always post operative
➢ Occurs at operative sites
➢ Usually within 4 years of surgery
• Unusual manifestations
➢ Multiplicity
➢ May occur with abdominal wall fibromatosis
➢ Diffuse form may involve mesentery, pelvis, and retroperitoneum
Mesenteric, Pelvic, and Retroperitoneal: Fibromatosis in FAP

Differential Diagnosis: Solid Mesenteric Mass
• Malignant
➢ Soft tissue sarcoma
➢ Lymphoma
➢ Gastrointestinal stromal tumor
• Benign
➢ Mesenteric fibromatosis
➢ Sclerosing mesenteritis
➢ Inflammatory pseudotumor
Metastatic Disease: Metastatic Lung Carcinoma
Soft Tissue Sarcoma: Synovial Sarcoma
Lymphoma
Gastrointestinal Stromal Tumor: Small Bowel Primary
Gastrointestinal Stromal Tumor: Primary to the Mesentery
Gastrointestinal Stromal Tumor: Retroperitoneal
Mesenteric Fibromatosis: Management

• Controversial
➢ Wide excision, antiestrogens, chemotherapy, radiation therapy
➢ Complications and recurrence common
• Sporadic cases
➢ Surgery often curative
• FAP
➢ Higher recurrence rate
➢ Higher morbidity
➢ Nonsurgical therapy more commonly used

Mesenteric Fibromatosis: Postoperative Recurrence
Sclerosing Mesenteritis
• Rare
• Idiopathic, nonneoplastic
• Chronic inflammation
• Synonyms represent histologic spectrum
➢ Mesenteric panniculitis
➢ Fibrosing mesenteritis
➢ Mesenteric lipodystrophy
Sclerosing Mesenteritis: Clinical Features

• Twice as common in men
• Mean age, 60 years
• Symptoms Figure 2-12-15
➢ Pain
➢ Palpable mass
➢ Bowel complications
➢ Incidental
Sclerosing Mesenteritis:
Pathologic Features
• Pathologic spectrum
➢ Loose myxomatous to dense
sclerosis Sclerosing mesenteritis
• Histologic features
➢ Sclerosing fibrosis
➢ Fat necrosis
➢ Lipid-laden macrophages
➢ Chronic inflammation
➢ Focal calcification
Sclerosing Mesenteritis: Imaging Features [Figure 2-12-15] Figure 2-12-16

• Mesenteric mass
➢ Mixed fat and soft tissue
➢ Radiating fibrosis
➢ Fat-ring sign
➢ Calcifications
➢ Cystic appearance
• Small Bowel
➢ Kinking or fixation
Sclerosing mesenteritis with the “fat ring” sign on CT
[Figure 2-12-16]
• “Fat-ring Sign”
Sclerosing Mesenteritis [Figure 2-12-17]
Sclerosing Mesenteritis: Differential Diagnosis Figure 2-12-17

• Carcinoid metastasis
➢ Look for primary
➢ Somatostatin scintigraphy
Sclerosing mesenteritis with low CT attenuation from loose

myxomatous stroma shown on the accompanying histology
image

Sclerosing Mesenteritis: Somatostatin Receptor Scintigraphy
Sclerosing Mesenteritis: Management

• Biopsy may establish diagnosis
• Many cases self-limiting
• Asymptomatic or mild symptoms
➢ Observation
• Symptomatic
➢ Immunosuppresive therapy
➢ Surgical resection
Inflammatory Pseudotumor (Inflammatory Myofibroblastic

Tumors)
• Chronic inflammation
• Unclear pathogenesis
➢ Sequelae occult infection
➢ Minor trauma
➢ Post surgical
• Variable nomenclature
➢ Inflammatory myofibroblastic pseudotumor
➢ Plasma cell granuloma
➢ Plasma cell pseudotumor
➢ Inflammatory fibrosarcoma
Inflammatory Pseudotumor: Clinical Features

• Most common in pediatrics and young adults
• May occur in may anatomic locations
• Symptoms
➢ Fever
➢ Malaise
➢ Weight loss
➢ Pain
Inflammatory Pseudotumor [Figure 2-12-18]
Extrapleural Solitary Fibrous Tumor Figure 2-12-18

• Rare neoplasms
• Submesothelial origin
• Most commonly pleural origin
“Solitary Fibrous Tumor of the

Peritoneum”
• Few case reports
• Natural history unknown
Inflammatory myofibroblastic pseudotumor
➢ Pleural lesions may show
aggression
➢ Long-term follow up
Extrapleural Solitary Fibrous Tumor
Summary
• Mesenteric cysts
• Solid mesenteric masses
Summary: Mesenteric Cyst

• Lymphangioma
• Most common
• Imaging
➢ Multilocular
➢ Enhancing septa
➢ Insinuating growth

• Enteric duplication cyst
• Enteric cyst
• Histologic differentiation
• Identical imaging

• Nonspecific imaging appearance

• Nonpancreatic pseudocyst
• No histologic lining
• Old trauma/abscess
• Imaging
➢ Thick wall
➢ Internal debris
Summary: Mesothelioma
• Diffuse malignant mesothelioma
➢ Asbestos
➢ Nodules, masses
➢ Bowel encasement
➢ Bowel fixation
• Cystic malignant mesothelioma
➢ Variant of DMM
➢ Cystic masses
➢ Ascites
Summary: Benign Multicystic Mesothelioma

• Controversial
➢ AKA peritoneal inclusion cyst
➢ Unrelated to DMM
➢ Unrelated to asbestos
➢ Pelvic peritoneum
➢ Multicystic mass
Summary: Benign Fibrous Lesions

• Sclerosing mesenteritis
• Inflammatory myofibroblastic pseudotumor
• Solitary fibrous tumor of peritoneum
Summary: Mesenteric Fibromatosis

• Benign
➢ Locally aggressive
➢ Association with FAP
• Imaging
➢ Homogeneous
➢ Heterogeneous
• Myxoid stroma
➢ Low CT attenuation
➢ High T2 signal
➢ “whorled” pattern
Summary: Sclerosing Mesenteritis

• Idiopathic inflammation
• Imaging
➢ Mixed attenuation
➢ Bowel retraction
➢ May calcify
• Conservative treatment

Summary: Inflammatory Pseudotumor
• Inflammatory/fibrotic infiltrate
• Nonspecific imaging
Summary: Extrapleural Solitary Fibrous Tumor

• Few case reports
References
Lymphangioma
1. Kempson RL, Fletcher CDM, Evans HL, Hendrickson MR, Sibley RK. Tumors of the soft tissues: atlas of tumor
pathology, third series, fascicle 30. Washington, DC: Armed Forces Institute of Pathology; 2001
2. Levy AD, Cantisani V, Miettinen M. Abdominal Lymphangiomas: Imaging Features with Pathologic Correlation.
AJR 2004. 182: 1485-1491
3. Ros PR, Olmsted WW, Moser RP, Jr., Dachman AH, Hjermstad BH, Sobin LH. Mesenteric and omental cysts:
histologic classification with imaging correlation. Radiology 1987;164:327-332
Mesenteric Fibromatosis
1. Burke AP, Sobin LH, Shekitka KM, Federspiel BH, Helwig EB. Intra-abdominal fibromatosis. A pathologic analysis
of 130 tumors with comparison of clinical subgroups. Am J Surg Pathol 1990; 14(4):335-341.
2. Levy AD, Rimola J, Mehrotra AK, Sobin LH. Benign Fibrous Tumors and Tumor-like Lesions of the Mesentery:
Radiologic Pathologic Correlation. RadioGraphics 2006; 26: 245- 264
3. Magid D, Fishman EK, Jones B, Hoover HC, Feinstein R, Siegelman SS. Desmoid tumors in Gardner syndrome: use
of computed tomography. AJR Am J Roentgenol 1984; 142(6):1141-1145.
1. Emory TS, Monihan JM, Carr NJ, Sobin LH. Sclerosing mesenteritis, mesenteric panniculitis and mesenteric
lipodystrophy: a single entity? Am J Surg Pathol 1997; 21(4):392-398.
2. Sabate JM, Torrubia S, Maideu J, Franquet T, Monill JM, Perez C. Sclerosing mesenteritis: imaging findings in 17
patients. AJR Am J Roentgenol 1999; 172(3):625-629.
3. Valls C. Fat-ring sign in sclerosing mesenteritis. AJR Am J Roentgenol 2000; 174(1):259-260.

Idiopathic Inflammatory Bowel Disease
Idiopathic Inflammatory Bowel Disease: Objectives

• Ulcerative colitis (UC)
• Crohn disease
Idiopathic Inflammatory Bowel Disease: General Features

• Etiology unknown
➢ ? Genetic basis
➢ ? Immune related
➢ ? Infectious agent
• Incidence of IBD
➢ UC is more common than Crohn
IBD: Epidemiologic Comparison

• Ulcerative colitis • Crohn disease
➢ 35-100 cases/100,000 ➢ 10-70 cases/100,000
➢ Age range, 15-25 years, ➢ Age range, 15-25 years,
second peak, 50 to 80 years second peak, 50-80 years
➢ Urban dwellers ➢ Urban dwellers
➢ Developed countries ➢ Whites
➢ Whites, 2 to 5 times risk ➢ Jewish (8 fold increase)
➢ Jewish, 2 to 4 times risk ➢ Family history, 12 to 15 times
➢ Family history, 30 to 100 risk
times risk
IBD: Comparison of Clinical Features

➢ Diarrhea ➢ Diarrhea
➢ Obstruction rare ➢ Obstruction common
➢ Rectal bleeding usually ➢ Rectal bleeding, less
➢ Abdominal pain, common
predefecatory urgency ➢ Abdominal pain, post
➢ Chronic, low grade illness in prandial, colicky
most ➢ Abdominal mass
➢ Acute, fulminating in 15% ➢ Vomiting
➢ Perianal disease
➢ Alternating attacks and
remissions
IBD: Comparison of Disease Distribution
➢ Colon to anus, rarely TI ➢ Entire GI tract, mouth to anus
➢ Continuous disease or ➢ Asymmetric, skip lesions
➢ Ulcerative proctitis that may ➢ May extend beyond bowel
extend proximally
Idiopathic Inflammatory Bowel Disease 382 Gastrointestinal Radiology

IBD: Pathologic Features
➢ Mucosal and submucosal ➢ Transmural inflammation
inflammation ➢ Marked mural edema
➢ Minimal mural edema
IBD: Gross Pathologic Features [Figure 2-13-1]

➢ Fine ulceration ➢ Linear ulcers
➢ Granular mucosa ➢ Cobblestones
➢ Hyperemic ➢ Marked mural thickening
IBD: Gross Pathologic Features

➢ Shallow ulcers, granularity ➢ Linear ulceration
➢ Loss of haustra ➢ Nodules
IBD: Histologic Features

➢ Diffuse mucosal ulceration ➢ Aphthous ulcer
➢ Crypt abscesses ➢ Linear, serpiginous ulcers
➢ Inflammatory infiltrate ➢ Wide-based ulcers
➢ Pseudopolyps ➢ Cobblestones
➢ Fissures, fistulas, abscesses
➢ Strictures
➢ Pseudopolyps
IBD: Histologic Features of Active Disease

➢ Crypt abscesses, ➢ Ulcers, fissures,
lymphoplasmacytic transmural lymphoid
lamina propria infiltrate aggregates, granulomas
Figure 2-13-1

➢ Crypt destruction ➢ Aphthous ulcer
➢ Crypt abscess ➢ Lymphoid aggregates
➢ Hemorrhage

➢ Atrophic, distorted ➢ Fissures
mucosa ➢ Transmural lymphocytes
➢ Thick muscularis mucosa
➢ Fat within submucosa
Gross pathologic specimens show

ulcerative colitis with a hemorrhagic
ulcerated mucosa and mural thinning.
Crohn disease has marked mural
thickening, mucosal cobblestones,
and proliferation of adjacent
mesenteric fat
Gastrointestinal Radiology 383 Idiopathic Inflammatory Bowel Disease

Ulcerative Colitis: Imaging Features Figure 2-13-2
Acute Changes [Figure 2-13-2]
• Mucosal granularity
• Mucosal stippling
• Collar button ulcers
• Haustral thickening or loss
• Inflammatory polyps
• Confluent, contiguous, circumferential disease
Ulcerative Colitis: Imaging Features

Chronic Changes
• Haustra loss
• Luminal narrowing
• Loss of rectal valves
• Widened presacral space
• Backwash ileitis
• Post-inflammatory pseudopolyps
Ulcerative Colitis: Role of CT

• Early, acute disease
➢ Low diagnostic sensitivity
➢ Often normal
• Complementary to endoscopy to assess for complications
➢ Toxic megacolon
➢ Pneumatosis
➢ Perforation
Gore RM et al. AJR 1996: 167:3-15.
Ulcerative Colitis: CT Features [Figures 2-13-3 and 2-13-4]

• Severe, acute disease Acute ulcerative colitis with fine
➢ Mural thinning mucosal ulceration producing
➢ Pneumatosis granularity and stippling on barium
➢ Perforation evaluation
• Subacute and chronic disease Figure 2-13-4
➢ Luminal narrowing
➢ Proliferation of Figure 2-13-3
perirectal fat
➢ Assessment/detection
of carcinoma
Gore RM et al. AJR 1996:
167:3-15.
Chronic ulcerative colitis

with mural stratification Acute ulcerative colitis with mild
(target sign), submucosal mural thickening and pericolonic
fat in the rectum, and an hyperemia on CT
increase in the perirectal fat

Ulcerative Colitis
• Mural stratification and luminal narrowing in chronic UC
➢ Thickening of muscularis mucosa
➢ Edema and fat in submucosa
Ulcerative Colitis: Perirectal Fat Proliferation
Toxic Megacolon and IBD

➢ Fever, tachycardia, hypotension Figure 2-13-5
• Incidence
➢ 5% to 10% of UC
➢ 2% to 4% Crohn disease
➢ May be initial manifestation of IBD
• Other causes
➢ Pseudomembranous colitis
➢ Ischemia
➢ Infection
• Pathology
➢ Transmural inflammation
➢ Loss of normal tissue cohesion
➢ Thinned wall and areas of denuded mucosa
• Imaging
➢ Abdominal radiograph establishes diagnosis
➢ Marked colonic dilatation, 6 to 15 cm
➢ Transverse colon most often involved
➢ Nodular mucosa
➢ Loss of haustra
➢ Intraluminal fluid
Toxic Megacolon [Figure 2-13-5]

• Ulcerative colitis
➢ Colonic distension
➢ Pseudopolyps
Ulcerative Colitis
Differential CT Features
• CT features suggesting UC over Crohn disease
➢ Mural stratification, 61% UC vs. 8% Crohn
➢ Mural thickness less in UC compared to Crohn
➢ Outer colonic contour, smooth in UC and irregular in Crohn
Gore RM et al. AJR 1996: 167:3-15
Crohn Disease
Early Imaging Features
• Distribution
➢ Asymmetric
➢ Segmental
➢ Skip lesions
• Lymphoid hyperplasia
Ulcerative colitis with toxic
• Ulceration
megacolon
➢ Aphthous ulcerations
➢ Linear ulcers
➢ Deep ulcerations (fissuring)
• Cobblestoning
• Inflammatory pseudopolyps

Crohn Disease: Aphthous Ulcers [Figure 2-13-6]
Figure 2-13-6
Crohn Disease: Linear Ulceration and Nodules
Crohn Disease
• Rose thorn ulcers
Crohn Disease: Cobblestoning [Figure 2-13-7]
Crohn Disease [Figure 2-13-8]
Figure 2-13-7
Crohn disease with aphthous ulcers

Figure 2-13-8
Crohn disease with cobblestoning
Crohn disease with terminal ileal

nodularity and ulceration

Crohn Disease: Acute/Early CT Features Figure 2-13-9
➢ 1 to 2 cm
• Mural stratification
➢ Mural enhancement
➢ Target or double halo sign
Crohn Disease
• Mesenteric hyperemia
• Target sign
➢ Acute inflammation
Crohn Disease
• Linear ulceration
• Inflammatory polyps
Crohn Disease: Subacute to Chronic CT Features

➢ Homogeneous, nonenhancing = fibrosis or stricture
➢ Enhancing wall = reversible inflammatory disease
• Mesenteric changes
➢ Fibrofatty proliferation
➢ Lymphadenopathy
➢ Hypervascularity
➢ Inflammatory stranding
➢ Phelgmon/abscess
Crohn Disease Crohn disease with distal ileal

• Homogeneous mural thickening inflammation (target sign), fibrofatty
➢ Nonenhancing = irreversible fibrosis proliferation of the small bowel
• Loss of mural stratification mesentery, and mesenteric
lymphadenopathy
Crohn Disease

• Fibrofatty proliferation
➢ “creeping fat”
• Mesenteric lymphadenopathy,
3 to 8 mm

• Prominent, dilated vasa recta
➢ “comb sign”
Figure 2-13-10
Crohn disease with prominent

and engorged vasa recta (comb
sign)

Crohn Disease: Chronic Features
• Fissures, fistulas, and sinus tracts
• Haustral loss
• Strictures
• Sacculations
• Post-inflammatory pseudopolyps Figure 2-13-11
Crohn Disease: Strictures
Sacculations and Strictures

[Figure 2-13-11]
Crohn Disease: Stricture
Crohn Disease
• Complications
➢ Sinus tracts
➢ Fistula
➢ Abscess
➢ Carcinoma
Crohn Disease
Intramural Fistula
Crohn Disease
Fistulae [Figure 2-13-12]
Chronic Crohn disease with structuring and sacculations
Crohn Disease: Abscess
[Figure 2-13-13]
• Secondary to deep penetrating ulcers
Figure 2-13-12
➢ Sinus tracts
➢ Fistulas
➢ Perforation
• 15% to 20% of patients
• Most frequently associated with small bowel or ileocolic disease
Crohn Disease: Perirectal Sinus Tract
Figure 2-13-13
Crohn disease with a ileal-ileal fistula
Crohn disease with a psoas abscess

Crohn Disease: Extraintestinal Complications
• Hepatobiliary
➢ Hepatic steatosis, 20% to 50%
➢ Cholelithiasis, 30% to 50%
➢ PSC, 1% to 4%
➢ Hepatic abscess
• Pancreatic
➢ Pancreatitis
• Musculoskeletal
➢ Arthritis
➢ Sacroileitis-spondylitis
• GU tract
➢ Nephrolithiasis, 2% to 10%
Neoplasia in IBD
• Adenocarcinoma
➢ Ulcerative colitis, highest incidence
➢ Crohn disease, small and large intestine
• Lymphoma
➢ Increased incidence in Crohn disease
• Features of carcinoma in IBD
➢ Typically do not arise in pre-existing adenomas
➢ Arise in flat mucosa
➢ Carcinomas may be long and flat
➢ May arise in bypassed segments of bowel
Adenocarcinoma in Ulcerative Colitis
Adenocarcinoma in Crohn Disease
Additional Imaging Modalities: Sonography UC vs. Crohn

Disease
➢ Hypoechoic wall ➢ Hypoechoic wall
➢ Mural stratification ➢ Loss of mural stratification
➢ Loss of haustra ➢ Loss of haustration
➢ Loss of peristalsis ➢ Diminished compressibility
➢ Absent peristalsis
➢ Increased blood flow
Gore RM, Laufer I, Berlin, JW. Ulcerative and granulomatous colitis: idiopathic
inflammatory bowel disease. In: Gore RM, Levine MS (eds), Textbook of
Gastrointestinal Radiology. 2nd ed.
Sarrazin J, Wilson SR. Manifestations of crohn disease at US. RadioGraphics
1996. 16: 499-520.
Additional Imaging Modalities: MR Enterography of Crohn

Disease
• Evolving technique
• Assessment of active disease
➢ Mural thickening > 4mm
➢ Mural enhancement
➢ Increase in mesenteric vascularity
Koh DM et al. MR imaging evaluation of the activity of crohn’s disease. AJR 2001:
177(6) 1325-1332.

Establishing Diagnosis: UC vs. Crohn
• Clinical course
• Disease distribution
• Endoscopy findings
• Biopsy
UC vs. Crohn Disease: CT Features

➢ Greatest with Crohn disease
• Submucosal fat
➢ More commonly seen in UC
• Mesenteric fibrofatty proliferation
➢ Crohn disease
➢ But, perirectal fat may increase in UC
• Sinus tracts, fistulas, abscess
➢ Crohn disease
Differential Diagnosis of IBD

• Infectious colitis
• Ischemic colitis
• Radiation enteropathy and colitis
• Behçet disease
• Graft vs. host disease
• Diverticular disease
Summary
• UC vs. Crohn
➢ Similar demographics
➢ UC, contiguous colonic disease
➢ Crohn, entire GI tract with skip areas
➢ UC, mucosal and submucosal disease
➢ Crohn, transmural disease with extension into the mesentery

Approach to Inflammatory Disease of the
Colon
Objectives
• General approach
• Differential diagnosis of idiopathic IBD
➢ Pseudomembranous colitis
➢ Neutropenic colitis (typhlitis)
➢ Ischemic colitis
➢ Diverticulitis
➢ Infectious colitis
General Approach
• Disease location
➢ Small vs. large bowel
➢ Focal vs. multifocal vs. diffuse
➢ Ascites
• Degree and pattern of mural thickening
➢ How much mural thickening?
➢ Mural enhancement?
➢ Fat attenuation in the submucosa?
• Associated mesenteric disease
➢ Inflammation
➢ Phelgmon/abscess
➢ Mesenteric fat proliferation
• Clinical history
Helpful Features for Differential Diagnosis

• Location of disease
➢ Small bowel involvement
➢ Diffuse vs. focal vs. multifocal
• Degree of mural thickening
➢ Marked mural thickening favors PMC
➢ Antibiotics (PMC)
➢ Radiation therapy
➢ Neutropenia
➢ Travel history
Colitis Differential Diagnosis

• Right-sided disease • Diffuse disease
➢ Campylobacter ➢ Ischemia
➢ Yersinia ➢ PMC
➢ Salmonella ➢ E. Coli
➢ Typhlitis ➢ Shigella, campylobacter,
➢ Crohn disease salmonella, amebiasis
➢ TB ➢ CMV
➢ Amebiasis ➢ Inflammatory bowel
➢ Histoplasmosis disease
➢ Behçet syndrome
➢ Graft vs. host disease
(GVDH)
➢ Radiation
Gastrointestinal Radiology 391 Inflammatory Disease of the Colon

41-year-old man who developed diarrhea one month after Figure 2-14-1
hospitalization for pneumonia [Figure 2-14-1]
Pseudomembranous Colitis
• Features suggesting PMC
➢ Diffuse colonic involvement
➢ Marked low attenuation mural thickening
➢ Accordion sign
➢ Ascites
• Onset following antibiotic therapy
➢ Clostridium difficile toxin
➢ Symptoms within days or weeks following antibiotic therapy
➢ Copious watery diarrhea
➢ Abdominal pain
➢ Fever
➢ Leukocytosis
• Epithelial necrosis Pseudomembranous colitis
• Inflammatory infiltrate
• Crypt eruption
• Pseudomembranes Figure 2-14-2
Pseudomembranous Colitis: CT Features

➢ Low attenuation
➢ Accordion sign
➢ Target sign
• Intraluminal plaques or nodules
• Pericolonic inflammation
• Ascites
• Complications
➢ Luminal dilatation, toxic megacolon
➢ Perforation
Pseudomembranous Colitis:
Thumbprinting = Mural Thickening
Pseudomembranous Colitis:
Low Attenuation Wall with Accordion Sign
Pseudomembranous Colitis: Target Sign [Figure 2-14-2] Pseudomembranous colitis

• PMC typically has the greatest amount of mural thickening of the
compared to other colitis
• Presence of ascites favors acute colitis over IBD
➢ PMC, infectious, ischemia
65-year-old woman abdominal pain during chemotherapy for

leukemia [Figure 2-14-3]
Neutropenic Colitis (Typhlitis)

• Suggestive features
➢ Clinical history
➢ Right-sided involvement
➢ Children and adults
➢ Neutrophil counts <500 to 1000 cells/mm3
➢ Fever, diarrhea, pain, abdominal distension
Inflammatory Disease of the Colon 392 Gastrointestinal Radiology

• Pathogenesis Figure 2-14-3
➢ Cecal stasis
➢ Cytotoxic mucosal injury
➢ Bacterial invasion
• CT features
➢ Predominant right-sided disease
➢ Mural thickening, low attenuation
➢ Pericolonic inflammation
➢ Ascites
➢ Pneumatosis
70-year-old man with acute abdominal pain and

bloody diarrhea [Figure 2-14-4]
Ischemic Colitis
• Most common in elderly
➢ Underlying atherosclerosis, diabetes, hypertension
➢ Low flow states
➢ Occlusive disease
➢ Complicates infectious colitis, especially CMV
• Ischemic Injury
➢ Acute, fulminant
✧ Transmural necrosis and perforation
➢ Transient, reversible
✧ Confined to mucosa and submucosa
➢ Chronic
✧ Submucosal fibrosis
• Locations
➢ Diffuse
➢ SMA and IMA watershed vunerable
Neutropenic colitis
✧ Normal communication through Arc of Riolan (marginal
artery of Drummond)
✧ Absent in 5% Figure 2-14-4
✧ Splenic flexure and rectosigmoid
Ischemic Colitis
• Denuded mucosa
• Pseudomembranes, Hemorrhage
Ischemic Colitis- Imaging Features

• Mucosal ulceration
➢ Low attenuation
➢ Target sign
• Luminal dilatation
• Pericolonic inflammation
• Ascites
• Chronic changes
➢ Fibrosis, stricture
Ischemic Colitis- Acute [Figure 2-14-4 and 2-14-5]
Figure 2-14-5 Ischemic colitis
Ischemic colitis

Ischemic Enteritis - Pathophysiology Figure 2-14-6
• Blood supply reduced by >50%
➢ Arterial occlusive
➢ Venous occlusive
➢ Nonocclusive (low flow states)
Ischemic Enteritis
• Segmental
• Diffuse
• Segmental, necrotic, ulcerated mucosa
➢ Stack of coins
• Thick wall, engorged mesentery
➢ Target sign [Figure 2-14-6]
• Thin wall, infiltrated mesentery
➢ Infiltrated mesentery [Figure 2-14-7]
• Mucosal cast, intramural fistula
• Mucosal ulceration, pneumatosis
• Pneumatosis, mesenteric venous gas
Figure 2-14-7
Ischemic enteritis due to SMV
thrombosis
Figure 2-14-8
. Ischemic enteritis
Ischemic Colitis or Enteritis [Figure 2-14-8]

• Chronic
Chronic ischemic colitis in the
Imaging Findings Suggestive of Ischemia watershed region of the colon
• Ileus
➢ Dilated bowel
➢ Gasless abdomen
➢ Unchanging bowel
➢ Stack of coins
➢ Target sign
• Mucosal ulceration
➢ Ulceration
➢ Intramural fistulas
➢ Loss of folds
• Mucosal breakdown
➢ Intraluminal mucosal cast
➢ Pneumatosis
➢ Mesenteric or portal venous gas
➢ Intraperitoneal air

50-year-old woman with abdominal pain and fever [Figure 2-14-9] Figure 2-14-9
• Diverticulitis - Hepatic Flexure
Diverticular Disease
• Most common disease of the
colon
• Diverticulosis increases with age
➢ 33% to 50% of people over
50
➢ 50% of people over 80
Acute diverticulitis
Diverticular Disease
Etiology of Pulsion Diverticula
• Pressure gradient
➢ Between lumen and serosa
➢ Compartmentalized by haustra
➢ Highest in sigmoid
• Weakness in bowel wall
➢ Intramural vasa recta penetrate wall
➢ Between taenia mesocolica and taenia libera
➢ Between taenia mesocolica and taenia omentalis
Diverticular Disease - Pathologic Features

• False diverticula
➢ Mucosa and submucosa only
➢ 0.5 to 1.0 cm
• Myochosis
➢ Thickening of circular muscle
➢ Shortening of taenia
➢ Narrowing of the lumen
➢ Results in corrugated appearance
Diverticular Disease: CT Features

• Diverticular outpouchings
Diverticular Disease: Complications

• Diverticulitis
• Hemorrhage
• Giant sigmoid diverticulum
Diverticulitis
• Most common complication
➢ 10% to 20% of patients with diverticulosis
• Pathogenesis
➢ Stagnation of fecal material
➢ Inflammatory erosion of the mucosa
➢ Perforation
✧ Intramural abscess
✧ Extramural abscess
Diverticular Disease: Complications

• Diverticulitis
➢ Luminal obstruction
➢ Infection

Diverticulitis: CT Features Figure 2-14-10
• Diverticula
➢ May have hyperdense fecal material
• Inflammatory changes
➢ Pericolonic stranding
➢ Pericolonic phlegmon
➢ Intramural abscess
➢ Pericolonic abscess
• Circumferential mural thickening
➢ Usually < 1 cm
➢ Rarely exceeds 2-3 cm
• Tethered lumen
➢ Saw-tooth configuration
➢ Due to muscular spasm
• Pneumoperitoneum, abscess
Diverticulitis: Hyperdense Fecal Material
Diverticulitis: Intramural Fistula - Pneumatosis

[Figure 2-14-10]
Diverticulitis: Complications
• Hemorrhage 25%
• Muscular hypertrophy and obstruction 25%
• Pericolic abscess 20%
• Free perforation 18% Acute diverticulitis with intramural
➢ Debilitated patients fistula and pneumatosis
➢ Corticosteroid therapy
• Vesicocolic fistula 8%
• Small bowel obstruction
• Pyelophlebitis and liver abscess
Diverticulitis: Perforation
Diverticulitis: Pericolic Abscess
Diverticulitis: Colovesical Fistula
• Colon carcinoma
• Colitis
➢ Infectious
➢ Ischemic
➢ Crohn disease
• Foreign body perforation
• Epiploic appendagitis
Diverticulitis vs. Carcinoma

• Wall thickening
➢ Mild circumferential thickening in diverticulitis (4 to 5 mm)
➢ Carcinoma usually > 2 cm
• Zone of transition
➢ Abrupt change in lumen caliber favors carcinoma
➢ Lobulated soft-tissue favors carcinoma
➢ Tethered (saw-tooth) lumen favors diverticulitis
• Inflammatory changes
➢ Favors diverticulitis
• Regional adenopathy
➢ Favors carcinoma

Diverticulitis vs. Carcinoma Figure 2-14-11
Diverticulitis: CT Pitfalls
• Differential diagnosis of colon cancer
➢ Problematic in 10% of cases
➢ If immediate surgery not performed: mucosal evaluation
(endoscopy or BE) to exclude cancer
Diverticulitis: CT and Surgical Management

• Antibiotic therapy
➢ Mild Diverticulitis
• CT guided drainage
➢ Focal abscess
• Immediate Surgery
➢ Free perforation with peritonitis
➢ Severe hemorrhage
• Elective surgical resection
➢ Following successful abscess drainage
➢ Recurrent diverticulitis
➢ Persistent pain
➢ Bleeding
Diverticular Hemorrhage
• Most common cause of adult rectal bleeding
• Site of hemorrhage
➢ Single diverticulum Giant sigmoid diverticulum
➢ Right side of colon in 2/3 of cases
• Rupture of the vasa recta
Diverticular Hemorrhage
➢ Elderly patients
➢ Sudden onset
➢ Stops spontaneously in 80%
➢ Rebleeding in 25%
Cecal Diverticulitis
• Congenital or acquired diverticula
➢ Congenital are true diverticula
➢ Acquired are most common
• CT features
➢ Demonstration of diverticula
➢ Intramural or pericolonic abscess
➢ Normal appendix
Giant Sigmoid Diverticulum [Figure 2-14-11]

• Rare
• Etiology
➢ Subserosal perforation and inflammation
➢ Air trapping
➢ Ball-valve mechanism
• Clinical Features
➢ Chronic pain
➢ Palpable mass

Giant Sigmoid Diverticulum Figure 2-14-12
• CT features
➢ Mesenteric side of sigmoid colon
➢ Usually 7 cm or greater
➢ Thin wall
➢ Unilocular
30-year-old man with diarrhea and pain following

vacation in Mexico [Figure 2-14-12]
Campylobacter Colitis
➢ Infectious colitis
✧ Distribution
✧ Clinical
Summary
• General approach
➢ Location of disease
➢ Degree of mural thickening
➢ Ascites
➢ Mesenteric disease
Summary - Pseudomembranous Colitis

• History of antibiotics
➢ Clostridium difficile
• Accordion sign
• Pericolonic inflammation Campylobacter colitis
• Ascites
Summary - Neutropenic Colitis/Typhlitis

• Chemotherapy
➢ Low neutrophil counts
• Bacterial invasion
• Predominant right-sided disease
➢ Pneumatosis
➢ Ascites
Summary - Ischemic Colitis

• Elderly patients
➢ Diffuse
➢ Watershed regions
• Acute
➢ Mucosal ulceration
• Chronic
➢ Stenosis
Summary - Diverticulitis
• Focal disease
• Diverticula
➢ Air filled
➢ Hyperdense
• Adjacent inflammation

Summary - Diverticulitis vs. Carcinoma
• Favor Diverticulitis
➢ Lesser mural thickening
➢ Gradual zone of transition
➢ Tethered lumen
• Favor carcinoma
➢ Greater mural thickening
➢ Lobular mural thickening
➢ Sharp zone of transition
➢ Pericolonic adenopathy
Summary
• Infectious colitis
• Right-sided colitis
➢ Campylobacter, yersinia, salmonella
➢ TB
➢ Amebiasis
• Diffuse colitis
➢ PMC
➢ E. Coli
➢ Shigella, campylobacter, salmonella
➢ Amebiasis
➢ CMV

Gastrointestinal Seminar 1: Abdominal Gas
Case 1: 45 year old man with chronic pancreatitis and acute onset
of lower abdominal pain, distension, and constipation
Cecal volvulus
Marked Cecal Dilatation
➢ Cecal volvulus
➢ Cecal bascule
➢ Pseudoobstruction (Ogilvie
syndrome)
Cecal Volvulus
• Volvulus is an axial twist of at
least 90 degrees
➢ Abnormal fixation to posterior
parietal peritoneum
➢ Freely mobile cecum
• Mechanical obstruction
• Radiographic features
➢ Cecal dilatation
➢ Beak on contrast enema
➢ Whirl on CT
Cecal volvulus
Seminar 1: Abdominal Gas 400 Gastrointestinal Radiology

Cecal Bascule
• Anterior-cephalad fold
➢ May cause obstruction
• Volvulus:Bascule = 10:1
Ogilvie Syndrome
• Colonic pseudo-obstruction
• Marked cecal dilatation
Sigmoid Volvulus
Transverse Colon Volvulus
Cecal bascule
Sigmoid volvulus
Gastrointestinal Radiology 401 Seminar 1: Abdominal Gas

Case 2: 85 year old woman with abdominal pain, fever, and
shock
Intestinal ischemia with infarction and hepatic portal venous

gas
Intestinal Ischemia with Infarction and Hepatic Portal

Venous Gas
Hepatic Portal Venous Gas

• Branching radiolucencies extending to within two cm of the
hepatic capsule
• Must differentiate from pneumobilia
• Differential Diagnosis:
➢ Bowel Necrosis (75%)
➢ IBD (10%)
➢ Abscess
➢ Obstruction
➢ Ulcer
Pneumobilia
Pneumobilia Portal venous gas
Intestinal Infarction with Portal Venous Gas
Portal venous gas

Case 3: 60 year old man with progressive dyspepsia and acute,
severe upper abdominal pain
Pneumoperitoneum Gastric Ulcer

Perforation
Pneumoperitoneum Signs on the

Supine Abdominal Film
• Diaphragmatic Slips (Leaping Dolphins)
• Central Diaphragm (Cupola)
• Morison’s Pouch (Doge’s Cap)
• Lesser Sac
• Falciform Ligament
• Fissure of Ligamentum Teres
“Leaping Dolphin”
Cupola Sign
“Doge’s Cap” Pneumoperitoneum from gastric ulcer perforation
Case 4: 67 year old man with severe chest pain after vomiting
Boerhaave’s Syndrome
• Dr. Hermann Boerhaave
• Emetogenic rupture
➢ Distal esophagus or
➢ Gastric cardia
• Left posterolateral region
➢ 1.5 - 4 cm tear
➢ Reduced muscle fibers
➢ Entrance of nerves, vessels
• Radiographic features
➢ Mediastinal gas
➢ LLL infiltrate, atelectasis
➢ Left effusion
Causes of Esophageal Rupture

• Spontaneous
➢ Boerhaave
➢ Mallory-Weiss
• Iatrogenic
➢ Endoscopic
➢ Dilation
➢ Tube placement
• Other
➢ Caustic Ingestion
➢ Trauma
➢ Inflammatory
➢ Neoplastic
Boerhaave syndrome
Gastrointestinal Radiology 403 Seminar 1: Abdominal Gas

Case 5: 50 year old man with upper abdominal pain, epigastric
fullness, and constipation
Cecal Herniation through the

Foramen of Winslow
Foramen of Winslow
Foramen Of Winslow Hernia

• 8% of internal hernias
• Involved bowel
➢ Small intestine 70%
➢ Cecum 25%
• Cecal herniation
➢ Persistence of ascending
mesocolon
➢ Mobility
➢ Alterations in intraabdominal
pressure
• Radiographic features Cecal herniation through the foramen of Winslow
➢ Circumscribed gas collection LUQ
➢ Medial and posterior to stomach
➢ Stomach displaced left and anterior
➢ Gas in lesser sac from abscess or perforation
Cecal herniation through the foramen of Winslow

Gastrointestinal Seminar 2:
Nonneoplastic Disease of the Stomach
Case 1: 65-year-old woman presents with retching and the

production of little vomitus. The ER physician cannot pass a NG
tube into her stomach
Gastric Volvulus Mesenteroaxial volvulus

• Abnormal rotation of the
stomach
➢ Rare
➢ Children and adults
➢ Most are secondary to fixation defect
• Associated anomalies
➢ Diaphragmatic defects
➢ Malrotation
➢ Wandering spleen
➢ Asplenia
Gastric Volvulus
• Classic Clinical presentation
➢ Severe epigastric pain
➢ Violent retching with production of little vomitus
➢ Inability to pass NG tube into stomach
• Outcomes
➢ Recurrent
➢ Resolve spontaneously
➢ Resolve with NG placement
➢ Complete obstruction
Gastrointestinal Radiology 405 Seminar 2: Nonneoplastic Disease of the Stomach

• Organoaxial
➢ Rotation about a line extending from cardia to pylorus
• Mesenteroaxial
➢ Rotation about a line connecting middle of lesser curvature to middle of
greater curvature
• Mixed types occur
• 30% associated with hiatal hernia
Gastric Volvulus - Radiologic Features

• Double air-fluid level
• Inversion of stomach
➢ Greater curve above lesser curve
• Positioning of cardia and pylorus at the same level
• Downward pointing pylorus and duodenum
Organoaxial Volvulus
Mechanism for organoaxial volvulus
Mesenteroaxial Volvulus
Mechanism for mesenteroaxial volvulus
Seminar 2: Nonneoplastic Disease of the Stomach 406 Gastrointestinal Radiology

Case 2: 22-year-old woman developed epigastric pain when she
was dieting in preparation for her wedding
Gastric Bezoar
• Accumulated ingested material
• Trichobezoar
➢ Hair
• Phytobezoar
➢ Vegetable matter
• Pathophysiology
➢ Altered gastric motility
➢ Altered gastric anatomy Gastric Bezoar
➢ Trichotillomania

Case 3: 8-year-old girl with recurrent emesis and diarrhea
Imaging findings
• Thick gastric folds
• Thick duodenal wall
• Large, enhancing pancreatic mass
• Positive pentetreotide scan
Zollinger-Ellison Syndrome: Pancreatic

Pancreatic Gastrinoma gastrinoma
producing
Zollinger-Ellison Syndrome Zollinger Ellison
• Affects all ages, peak 3rd to 5th decade
syndrome
• Gastrin-secreting neuroendocrine tumor
(gastrinoma)
➢ Pancreas (75%)
➢ Duodenum (15%)
➢ Liver, ovary, lymph nodes
➢ 60% malignant
• Clinical Features
➢ One or more benign peptic ulcers
➢ Diarrhea from hypergastrinemia (30%)
➢ Elevated gastrin levels
• May occur in MEN I syndrome
Zollinger-Ellison Syndrome
➢ Multiple ulcers
➢ Increased gastric secretions
➢ Thick gastric folds
• Preoperative localization of gastrinoma
➢ CT
➢ MR
➢ Somatostatin receptor scintigraphy
47-year-old man with recurrent PUD
Ectopic Gastrinoma in Retroperitoneal Lymph Node
Thick Gastric Wall? Gastric Air-Fluid Sign*

*Hammerman AM, Mirowitz SA, Susman N. Gastrointestinal Radiology 14:109-
112. 1989
Differential Diagnosis: Thick Gastric Folds

• Hypertrophic Gastropathy
➢ Menetrier disease
➢ Zollinger-Ellison syndrome
• Gastritis
• Neoplasm
➢ Adenocarcinoma
➢ Lymphoma
➢ Metastasis
• Miscellaneous
➢ Amyloid
➢ Eosinophilic gastritis
➢ Adjacent inflammation

Case 4: 70-year-old man presents epigastric pain and pedal
edema
Menetrier Disease
Menetrier Disease : Adult Form

• Most common in men, 50 to 70 years
• Symptoms
➢ Epigastric pain
➢ Vomiting
➢ Weight loss
➢ Peripheral edema
• Hypoalbuminemia and hypochlorhydria
• Diffuse enlargement of gastric folds
➢ Proximal stomach
• Mucus hypersecretion
• Irreversible
Menetrier Disease : Pediatric Form

• Associated with CMV infection
• Allergic or autoimmune reaction
• Symptoms
➢ Periorbital and facial edema
➢ Vomiting
➢ Pain
• Self-limited
• Spontaneous resolution and reversal of protein loss
• Antrum more commonly involved
Menetrier Disease : Pathology

• Pathology
➢ Foveolar hyperplasia, glandular atrophy, cysts
➢ Enlarged folds (1-3 cm) resembling cerebral convolutions
➢ H. pylori?
Menetrier Disease : Radiology

• Thick folds
➢ Nonuniform
➢ Tortuous
• Spiculation of greater curvature
• Antral sparing
• Flocculation of contrast

Case 5: UGI images from two different patients that complained
of epigastric pain. Both patients had a history of diarrhea
Case 5A: Radiologic Findings Case 5A Case 5B

• Narrowed antrum
• Multiple filling defects
• Nodularity
• Ulceration?
• Effaced/nodular duodenal bulb
Case 5B: Radiologic Findings

• Multiple ulcers
➢ Aphthous ulcers
• Nodularity
Crohn disease
Case 5 : Differential Diagnosis
• Gastritis
➢ H. pylori
➢ Radiation
➢ Caustic ingestion
• Neoplasm
➢ Adenocarcinoma
➢ Lymphoma
➢ Mets
• Granulomatous disease
➢ Crohn
➢ Sarcoid
➢ Amyloid
➢ TB
Gastric Crohn Disease

• Histologically present in up to 33% of patients with Crohn disease
• 20% of patients with ileo-colic disease have abnormal UGI1
• Antrum and duodenum most often affected
1Levine MS. Crohn’s disease of the upper gastrointestinal tract. RCNA 1987
Gastric Crohn Disease

• Ulcers
➢ Aphthous lesions
➢ One or more large ulcers
• Nodules
➢ Focal nodules
➢ Cobblestone mucosa
• Abnormal gastric motility
• Fibrosis
➢ Tubular antrum (ram’s horn sign or shofar sign)
➢ Obliteration of the pylorus (pseudo-Billroth I sign)

Pancreatic Duct
Normal Pancreatic Embryology
18 days 22 days
Normal Anatomy
• Minor Papilla
➢ Accessory PD
➢ Duct of Santorini
• Major Papilla
➢ Main PD
➢ Duct of Wirsung
5 weeks 5 weeks
Normal pancreatic and biliary duct

anatomy Anatomic variants of the pancreatic duct
Normal pancreatic duct by ERCP and MRCP
Gastrointestinal Radiology 411 Seminar 3: Pancreatic duct

Case 1: 25-year-old woman with a long history of nausea,
vomiting, and abdominal distension
Annular Pancreas
• Bilobed ventral pancreatic bud
• Buds migrate in opposite directions
• Duodenal obstruction
Proposed mechanism for

annular pancreas
Annular pancreas
Seminar 3: Pancreatic duct 412 Gastrointestinal Radiology

Case 2: 17-year-old female with abdominal pain and elevated
LFT’s
Pancreatic divisum with focal chronic

pancreatitis
Pancreatic Divisum and Chronic Pancreatitis
Pancreatic Divisum
• Incomplete fusion of dorsal and ventral pancreas
• Body and tail drain through the duct of Santorini, minor
papilla
• Incidence
➢ 4 to 11% (autopsy)
➢ 3 to 4% (ERCP)
• Most asymptomatic
• 12-24% develop idiopathic recurrent pancreatitis
Choledocholithiasis in a patient with pancreatic

divisum by MRCP

Case 3: Two Different Patients with the same Disease
Chronic Pancreatitis
Chronic Pancreatitis: Ductal Features

• Ectasia
➢ Loss of normal tapering
• Contour irregularity
• Side branches
➢ Clubbing
➢ Stenosis
➢ Opacification of cavities
• Stenoses or occlusion
➢ “Chain of lakes”
• Intraductal calculi
Chronic pancreatitis by MRCP

Case 4: 50-year-old man with abdominal pain
Intraductal papillary mucinous neoplasm
Intraductal Papillary Mucinous Neoplasm
IPMN: Imaging
• Duct dilatation
➢ Focal or diffuse
➢ Main duct or side branch
• Intraductal masses
• Bulging duodenal papilla
• Glandular atrophy
Main Duct IMPN

Side Branch IPMN
Bulging Papilla

Case 5: 45-year-old man with chest pain and elevation of serum
amylase
Mediastinal pseudocyst from acute

pancreatitis
Mediastinal Pseudocyst
Fluid Collections and Pancreatitis

• 50% of patients
➢ Rupture of pancreatic duct
➢ Exudation of fluid from gland surface

Hepatic Imaging
Case 1: 50-year-old woman with

vague abdominal discomfort
Complex Hepatic Cyst
• Nonneoplastic
➢ Echinococcal cyst
➢ Simple cyst with
hemorrhage/infection Biliary cystadenoma
➢ Post-traumatic cyst
➢ Abscess
➢ Ciliated hepatic foregut cyst
• Neoplastic
➢ Biliary cystadenocarcinoma
➢ Cystic metastasis
➢ Peliosis
➢ Teratoma
Biliary Cystadenoma
Simple Cyst
Gastrointestinal Radiology 417 Seminar 4: Hepatic Imaging

Case 2: 10-year-old girl with right upper quadrant pain
Focal nodular hyperplasia
Differential Diagnosis: Hepatic Mass with a Scar

• Focal nodular hyperplasia
• Fibrolamellar carcinoma
• Hepatocellular adenoma
• Hemangioma
Hepatic Mass with a Scar
Hepatocellular Carcinoma (HCC)
Hepatocellular Adenoma (HCA) with Fibrosis
Hemangioma
Hemangioma: Tagged RBC Scan
Seminar 4: Hepatic Imaging 418 Gastrointestinal Radiology

Case 3: 54-year-old man with right upper quadrant pain and
jaundice
Intrahepatic cholangiocarcinoma
Rim-like Enhancement
• Hemangioma
• Intrahepatic cholangiocarcinoma
• Angiosarcoma
• Epithelioid hemangioendothelioma
Hemangioma

Case 4: 50-year-old male with vague abdominal pain
Hepatocellular carcinoma
Differential Diagnosis: Liver Mass with Fat

• Angiomyolipoma
• Myelolipoma
• Hepatocellular adenoma
• Metastasis
➢ Liposarcoma
• VERY RARE, Teratoma
Hepatocellular Adenoma: Focal Fat and Capsule
Hepatocellular Adenoma: Diffuse Hypodensity
Angiomyolipoma
Myelolipoma
Hepatic Teratoma
Seminar 4: Hepatic Imaging 420 Gastrointestinal Radiology

Case 5: 26-year-old woman with RUQ pain and mild elevation of
serum AST
Differential Diagnosis: Calcified Liver Mass

• Nonneoplastic
➢ Hematoma
➢ Simple cyst
➢ Parasitic infection
➢ Healed infection
• Benign neoplasm
➢ Hemangioma
➢ Teratoma
• Malignant neoplasm
➢ Fibrolamellar carcinoma
➢ Epithelioid hemangioendothelioma
➢ Hepatoblastoma (kids)
Colon Adenocarcinoma Metastases
Echinococcus multilocularis

Complications of Meckel Diverticulum
Cases 1-5
• All patients have the same disease
• The underlying disease is a congenital anomaly
• Each presents with a different manifestation Omphalomesenteric
(Vitelline) Duct Anomalies
Meckel Diverticulum
• Most common anomaly of the GI tract
• 2% - 3% of the population
• M=F
• Symptoms more common in males
• 60% of patients present before age 10
• Omphalomesenteric duct anomaly
➢ Improper closure and absorption
Omphalomesenteric (Vitelline) Duct

• Embryonic connection between yolk sac and midgut
• 10th week of embryogenesis
➢ Midgut returns to abdomen
➢ Duct is a thin fibrous band connecting midgut to umbilicus
➢ Disintegrate
➢ Absorption Umbilico-ileal fistula
Umbilical sinus Umbilical cyst Persistent fibrous cord
Meckel diverticula Meckel diverticula Meckel diverticula

with a fibrous attachment to the supported by a mesentery
umbilicus
Seminar 5: Meckel Diverticulum 422 Gastrointestinal Radiology

Omphalomesenteric (Vitelline) Duct Anomalies
• Umbilico-ileal fistula
• Umbilical sinus
• Umbilical cyst
• Persistent fibrous cord
• Meckel diverticulum
➢ With a fibrous cord
➢ With a portion of mesentery
Meckel Diverticulum: Pathology

• Antimesenteric side of distal ileum
➢ Within 100 cm of ileocecal valve
• True diverticulum
➢ Composed of all layers of the small bowel wall
• Heterotopic tissue
➢ 50% of resected diverticula
➢ Gastric most common (23% - 50%)
➢ Pancreas (5% to 16%)
➢ Rare, Brunner glands, colonic, biliary
Meckel Diverticulum: Heterotopic Gastric Mucosa
Meckel Diverticulum: Heterotopic Pancreatic Mucosa
Gastrointestinal Radiology 423 Seminar 5: Meckel Diverticulum

Case 1: 22-year-old man with fever and guaiac positive stools
Meckel diverticulitis located in the midline because of persistent

attachment to the umbilicus
• Inflammatory bowel disease
• Urachal remnant
• Colonic Diverticulitis
• Meckel diverticulitis
• Idiopathic ileal diverticula
Meckel Diverticulitis
Case 2: 22-year-old man with chronic abdominal pain and

anemia
Hemorrhagic Meckel diverticulum
• Neoplasm
• Ulcer
• Vascular ectasia
• Meckel diverticulum
Hemorrhagic Meckel Diverticulum
Angiographic Features of Meckel Diverticulum

• Vitellointestinal artery
➢ Arises from a distal ileal branch of the SMA
• Tubular shaped angiographic blush
• Intraluminal contrast if brisk bleeding
Hemorrhage in Meckel Diverticulum

• Most frequent complication
• Tc99-pertechnetate
➢ Localizes in ectopic gastric mucosa
➢ Modality of choice in pediatric population
➢ Sensitivity 85%, specificity 95% in kids
➢ Sensitivity 63%, specificity 2% in adults

Case 3: 61-year-old woman with intermittent abdominal pain
Inverted Meckel diverticulum
• Lipoma
• Inverted Meckel diverticula
Inverted Meckel Diverticulum
Inverted Meckel's Diverticulum

with Intussusception
Illustration of an inverted Meckel diverticulum
Case 4: 57-year-old man with abdominal pain and fever
Meckel diverticulitis with perforation and a stone
Meckel Diverticulitis with Perforation and a Stone
Meckel Diverticulitis with a Stone
Meckel Diverticulitis: Etiology

• Luminal obstruction
➢ Enterolith
➢ Foreign body
➢ Edema of orifice
• Peptic ulceration
• Torsion
Gastrointestinal Radiology 425 Seminar 5: Meckel Diverticulum

Meckel Diverticulitis
➢ Appendicitis
➢ Idiopathic ileal diverticula
• Helpful CT features
➢ Blind-ending pouch
➢ Mural contrast enhancement
➢ Connection to ileum
➢ Midline location
➢ Associated SBO
Case 5: 40-year-old man with pain and vomiting
Small bowel obstruction from Meckel diverticulitis
Inflamed Meckel
with Small Bowel Obstruction
Small Bowel Obstruction due to Meckel Diverticula

• Second most common complication of Meckel
• Etiology
➢ Inversion with intussusception
➢ Diverticulitis
➢ Volvulus from attachment to umbilicus
➢ Congenital mesodiverticular bands
➢ Foreign body impaction
➢ Inclusion of Meckel in a hernia (hernia of Littre)
➢ Neoplasm
➢ Inclusion of Meckel in a true knot
Summary: Complications of Meckel Diverticula

• Hemorrhage
• Obstruction
• Diverticulitis
• Inversion
➢ Intussusception
• Stones
• Torsion
• Neoplasm
Summary: Complications of Meckel Diverticula


Beyond Appendicitis
Angela D. Levy, LTC (P), MC, USA
Case 1: 23-year-old man with a 1-day history of left lower

quadrant pain and bilious emesis
Imaging Features
• Reversal of SMA and SMV
• Swirling vessels about SMA
• Absent colon right side of
abdomen
• Inflammatory process in LLQ
➢ Inflamed tubular structure
➢ Mesenteric inflammation
• Malrotation
• Inflammation
➢ Diverticulitis
➢ Meckel diverticulum
➢ Appendicitis
Malrotation with Left-sided

Appendicitis
Malrotation with left-sided appendicitis
Various locations of the cecum and appendix

within the abdomen
Gastrointestinal Radiology 427 Seminar 6: Beyond Appendicitis

Case 2: 47-year-old man complains of fever and right lower
quadrant pain
Appendiceal mucinous cystadenoma causing appendicitis
Appendiceal Mucinous Cystadenoma Causing Appendicitis
Appendiceal Neoplasms
• Uncommon
➢ <0.4% of intestinal tumors
• Histologic subtypes
➢ Carcinoid
➢ Mucinous cystadenoma/cystadenocarcinoma
➢ Adencarcinoma
➢ Non-mucin producing adenocarcinoma
Appendiceal Carcinoid
• Most common location for GI tract carcinoid
➢ 45% of gastrointestinal carcinoids
• Most common appendiceal tumor
➢ 50% to 85% of appendiceal tumors
• Majority benign clinical course
➢ 70% to 90% discovered incidentally
➢ >95% of appendiceal carcinoids have benign biologic behavior
Mucinous Cystadenoma/Cystadenocarcinoma
• Mucin producing epithelial neoplasm
➢ M=F
➢ 27 to 77 years of age
• Presentation
➢ Right lower quadrant pain, nausea, vomiting, abdominal swelling
• Complications
➢ Bowel obstruction, torsion, perforation, intussusception,
appendicitis
• 20% with a synchronous colonic adenocarcinoma
Mucinous Cystadenoma/Cystadenocarcinoma
• Radiologic Findings
➢ RLQ mass on plain film
➢ Mass effect medial cecal wall
➢ Nonfilled appendix on BE
• Cross-sectional imaging
➢ Fluid-filled, complex mass on CT or US
➢ Mass bulges into cecal lumen
➢ Short T1 and long T2 signal on MR
➢ May be the lead point for intussusception
Mucinous Cystadenoma
Mucinous cystadenoma of the

appendix
Seminar 6: Beyond Appendicitis 428 Gastrointestinal Radiology

Appendiceal Adenocarcinoma
• Non mucin producing
• Less common than mucinous
tumors
• Radiologically resembles colonic
adenocarcinoma
➢ Focal soft tissue mass
➢ Soft tissue mural infiltration
Neoplastic vs. Nonneoplastic

Appendicitis Appendiceal Adenocarcinoma
• CT findings suggestive of neoplasm
➢ Focal soft tissue mass
➢ Cystic dilatation of the appendix
➢ Nonspecific inflammatory changes may be seen in neoplasms of the
appendix
➢ 95% sensitivity for neoplasm if you combine morphologic changes with a
diameter > 15 mm1
1Pickhardt PJ, Levy AD, Rohrmann CA, Kende AI. Primary Neoplasms of the
Appendix Manifesting as Acute Appendicitis: CT Findings with Pathologic
Correlation. Radiology 2002. 224 (3): 775-781

Case 3: 65-year-old man with acute RLQ pain
Epiploic appendagitis
Epiploic Appendagitis
• Appendix epiploica
➢ Torsion
➢ Infarction
➢ Ischemia
• Clinical course
➢ Self limited
➢ Spontaneous resolution
Epiploic Appendagitis: Imaging Features

• Pericolonic fatty mass
➢ Peripheral inflammatory change
➢ Central high attenuation from vascular thrombosis
➢ May have mass effect on adjacent bowel
Epiploic Appendagitis
Case 4: 17-year-old man with RLQ pain and poor appetite
Mesenteric adenitis
Mesenteric Adenitis
• Inflammation of ileocecal nodes
➢ Coexistent inflammation of the TI and cecum may be present
• Children, young adults
• Self limited
Seminar 6: Beyond Appendicitis 430 Gastrointestinal Radiology

Case 5: 64-year-old man with RLQ pain that progressed to
involve the entire abdomen, fever, and vomiting
Cecal adenocarcinoma resulting in perforation, appendicitis, and

small bowel obstruction
Cecal Adenocarcinoma
Acute Appendicitis: Pathogenesis
• Luminal obstruction followed by infection
➢ Stones
➢ Food, mucus, adhesions
➢ Mucosal edema, lymphoid hyperplasia
➢ Parasites
➢ Tumors
➢ Endometriosis
Cecal Adenocarcinoma
➢ Eccentric or asymmetric
• Intraluminal mass
➢ Often near appendiceal orifice if patient presents with appendicitis
• Pericolic lymph nodes
• Peritoneal implants, distant mets
Non Hodgkin Lymphoma
Case 6: 42-year-old woman with RLQ pain and peritoneal signs

on physical exam
Omental Infarction
Omental Infarction
• Omental torsion
➢ Most commonly site free edge of the right lateral omentum
• CT features
➢ Focal inflammation of omental fat
➢ Normal appendix, colon, terminal ileum
➢ Fatty mass with concentric or swirling lines
Tumors and Tumor-like Lesions of the
Gallbladder
Angela D. Levy, LTC (P), MC, USA
Case 1: 45-year-old woman with RUQ pain
Adenomyomatous hyperplasia
Sonographic Findings
• Gallbladder wall thickening
• Reverberation artifact
Differential Diagnosis: Gallbladder Wall Thickening

• Infection/inflammation
➢ Acute cholecystitis
➢ Chronic cholecystitis
➢ Xanthogranulomatous cholecystitis
• Edema
➢ Cardiac, liver, renal failure
➢ Hepatitis
• Neoplasm
➢ Primary or secondary
• Tumor-like lesions
➢ Adenomyomatous hyperplasia
Seminar 7: Tumors and Tumor-like Lesions of the Gallbladder 432 Gastrointestinal Radiology
Reverberation (“comet-tail”) Artifact
• Gas in gallbladder wall
• Adenomyomatous hyperplasia
Coronal T2 MR Findings
• “String of pearls”
Adenomyomatous Hyperplasia
• “String of pearls”
Adenomyomatous Hyperplasia
• Common
➢ 9% cholecystectomy specimens
➢ More common in women than men
• Gallstones frequently present
• Three variants
➢ Diffuse
➢ Segmental
➢ Localized (fundic adenomyoma)
Fundal Adenomyomatous Hyperplasia: "Adenomyoma"
Gastrointestinal Radiology 433 Seminar 7: Tumors and Tumor-like Lesions of the Gallbladder
Case 2: 47-year-old man complains of fever and right lower
quadrant pain
Xanthogranulomatous cholecystitis
CT Findings
• Gallstone
• Hypodense nodules in gallbladder wall
• Ill-defined hepatic margin
• Inflammatory change
• Cholecystitis
➢ Acute
➢ Chronic
➢ Xanthogranulomatous
• Neoplasm
Xanthogranulomatous Cholecystitis
• Aggressive inflammatory process
• Pathophysiology
➢ Intermittent cystic duct obstruction
➢ Bile enters gallbladder wall
➢ RUQ pain, fever, tenderness
• Surgical treatment
➢ Reported association with gallbladder carcinoma
➢ Difficult to preoperative distinguish from carcinoma
➢ Involvement of adjacent organs
Xanthogranulomatous Cholecystitis: Pathology
Xanthogranulomatous Cholecystitis: Imaging

• Wall thickening
• Mural nodules or bands
➢ Hypoechoic on sonography
➢ Low-attenuation on CT
• Stones
• Pericholecystic fluid
• Adjacent invasion
• Lymphadenopathy
Case 3: 75-year-old woman with RUQ pain and weight loss
MR Findings
• Intraluminal gallbladder mass
• Hepatoduodenal ligament mass
• Gallbladder carcinoma
• Bile duct carcinoma
Gallbladder Carcinoma
• Sixth most common GI tract malignancy
➢ Worldwide: stomach, colorectal,
liver, esophagus, pancreas,
gallbladder
➢ US: colorectal, pancreas, stomach,
liver, esophagus, gallbladder
• More common in women (3:1)
➢ Mean age 72 years
Gallbladder Carcinoma:
Pathology
• Epithelial malignancies (98%)
➢ Adenocarcinoma (90%), squamous
cell, adenosquamous, small cell
carcinoma
• Other (2%)
➢ Sarcomas, lymphomas, carcinoid,
metastases
Gallbladder adenocarcinoma
Gallbladder Carcinoma
• Imaging patterns
➢ Intraluminal polypoid mass (15% to 25%)
➢ Focal or diffuse wall thickening (20% to 30%)
➢ Mass replacing the gallbladder (40% to 65%)
Adenocarcinoma: Diffuse Wall Thickening
Papillary Adenocarcinoma : Polypoid Mass
Adenocarcinoma: Mass Replacing the Gallbladder Fossa
Gallbladder Adenocarcinoma: Direct Extension to Liver
Gallbladder Adenocarcinoma: Direct Extension to

Hepatoduodenal Ligament
Case 4: 30-year-old man with RUQ pain
Metastatic melanoma
Differential Diagnosis: Polypoid Gallbladder Mass

• Cholesterol polyp
• Gallbladder adenoma
• Adenomyomatous hyperplasia
• Carcinoma
Metastatic Melanoma
Management of Gallbladder Polyps

• Size < 5 mm
➢ Do nothing
• Size 5 to 10 mm
➢ Follow
• Size >10 mm
➢ Remove
• Features suggesting malignancy
➢ Adjacent gallbladder wall thickening
➢ Abnormal gallbladder/liver interface
➢ Abnormal liver parenchyma
➢ Hepatoduodenal ligament adenopathy
Case 5: 35-year-old woman with RUQ pain
Cholesterol polyp
Sonographic Findings
• Small, nonshadowing echogenic mass
• Adherent to gallbladder wall
Cholesterol Polyp
• Common
➢ 50% of polypoid lesions in the gallbladder
• More common in women
➢ 3:1
Cholesterol Polyp
• Lipid laden macrophages
• Normal gallbladder epithelium
Cholesterol Polyp: Sonography

• Most <10 mm
• Small lesions
➢ Echogenic nodules
• Larger lesions
➢ Hypoechoic
➢ Internal echogenic foci
Summary
• Look for features to suggest a specific process tumor-like process
➢ Ring-down artifact, pearl necklace sign for adenomyomatous hyperplasia
➢ Focal mural nodules for XGC in the right clinical setting
• Look for features to suggest a malignancy
➢ Gallbladder wall thickening in association with a polypoid mass
➢ Abnormal gallbladder/liver interface
➢ Abnormal liver parenchyma
➢ Hepatoduodenal ligament mass or adenopathy
Cholelithiasis and Cholecystitis
Robert K. Zeman, MD
Outline/Objectives
• Detection of cholelithiasis
• Gravel versus sludge
• Acute cholecystitis
• Complications of acute cholecystitis
➢ Gangrenous cholecystitis
➢ Emphysematous cholecystitis
➢ Empyema of the gallbladder
➢ Gallbladder perforation
➢ Choledocholithiasis
Premise
• The radiologist plays a central role in identifying the cause of the patient’s
symptoms and…
• Detecting complications of cholecystitis (inflammatory and neoplastic) that will
dictate the therapeutic approach
Cholelithiasis
• 30 million American adults harbor stones
• Should “silent” stones be treated?
• 22% of patients with stones are symptomatic (Sirmione study)
• In symptomatic patients, 50% chance of colic in 1 year; 1–2% cumulative risk
of acute cholecystitis.
Cholelithiasis
• For symptomatic stones, recommend elective laparoscopic cholecystectomy
• For acute cholecystitis:
➢ Delayed surgery allows for better vizualization of surgical field
➢ Early surgery means less adhesions
US of Cholelithiasis [Figure 2-22-1] Figure 2-22-1

• 3 common appearances
➢ Solitary stone
➢ Gravel
➢ Double-arc (WES)
Solitary Gallstone
Gravel
Double-Arc Sign (WES)
How Sensitive is US?

• Remember the neck of the gallbladder
Solitary stone Gravel Double-arc (WES)
Sludge vs Gravel?
• Gravel represents small, discrete calculi
• Sludge is viscous, lithogenic bile
Cholelithiasis and Cholecystitis 438 Gastrointestinal Radiology

Tumefactive Sludge [Figure 2-22-2] Figure 2-22-2
• Baseline
• After walking
Is there any role for the OCG?

[Figure 2-22-3]
• No stones on OCG
• See 5mm stone on US
Acute Cholecystitis
• Uncomplicated vs complicated
• Treatment options if complications
• (do imaging findings influence
operative approach?)
• Cholescintigraphy vs US
Cholescintigraphy vs. Tumefactive sludge mimics GB mass-but changes shape with

Ultrasound change in patient position
• Both equally sensitive and specific
• Emergency availability is key
Figure 2-22-3
• Ultrasound screens for more non-biliary diseases
• If biliary obstruction present, scintigraphy does not identify cause
despite high sensitivity; US may see cause
• Scintigraphy great problem solver; can add EF when confusing
symptoms
Cholescintigraphy In AC
• The only reliable indicator of acute cholecystitis is non-visualization
of the gallbladder – remember the lateral
• High sensitivity, moderate specificity
Cholescintigraphy: Positive study for acute

cholecystitis
Potential Causes of False-Positive Scintigraphy For

Acute Cholecystitis Missed stones often “hide” in the
• Lack of adequate fasting gallbladder neck
• Chronic cholecystitis
• Failure to obtain delayed views
• Pancreatitis
• Hyperalimentation
• Biliary obstruction
• Prolonged fasting
• Intercurrent illness
• Alcoholism
• Overly conservative pathologic criteria of Acute Cholecystitis
• Trauma
• Gallbladder Neoplasm
Is There Anything That Can Reduce False-Positives?
Pharmacologic Enhancement of Cholescintigraphy

• Can dramatically reduce false-positives*
• Two approaches – CCK versus morphine (in setting of suspected AC)
• CCK to “pre-empty” GB
• Morphine (.04 mg/kg) given if GB fails to visualize by 30–50 minutes
*Kim et al, AJR 147:1177,1986
Gastrointestinal Radiology 439 Cholelithiasis and Cholecystitis

Use of Morphine Reduces False-Positives [Figure 2-22-4] Figure 2-22-4
• 35 minutes (pre-MS)
• 55 minutes (post-MS)
Sonographic Findings Associated

With Acute Cholecystitis
• Most useful signs
➢ Cholelithiasis
➢ Intramural sonolucency
➢ Sonographic Murphy Sign*
➢ GB wall hyperemia** 35 minutes (pre-MS) Normal scan after morphine
• Secondary signs 55 minutes (post-MS)
➢ Pericholecystic fluid
➢ GB distention
➢ Sludge
➢ Gas in the gallbladder wall or lumen
➢ Pericholecystic abscess
*Laing et al, Radiology 140:449, 1981
**Uggowitzer AJR 168:707, 1997
Intramural Sonolucency
• Described in 11 patients as first specific sign for acute cholecystitis*
• “Consists of a hypo-reflective or sonolucent band, continuous or interrupted,
within the hyper-reflective gallbladder wall” Figure 2-22-5
• Focal lucency or concentric rings (striate)
most suggestive of inflammation**
*Marchal et al, Radiology 133:429, 1979
**Cohen et al, Radiology 164:31, 1987
Acute Cholecystitis-Striate GB
Walls [Figure 2-22-5]
Acute Cholecystitis-Focal Lucency
Lucency in GB Wall is not always

Edema
• Gallbladder varices in portal hypertension
Which one has Varices?
Gallbladder Wall Thickening

• Failure to fast
• Acute cholecystitis
• Chronic cholecystitis
• Hypoalbuminemia Multiple patients
• Hepatitis
• Ascites Figure 2-22-6
• Varices
• AIDS
• Carcinoma
• Cholesterolosis
• Mononucleosis
• Beware of “sliver” of edema in some of

these entities
Gallbladder Wall Thickening

[Figure 2-22-6]
• Hepatitis Gallbladder wall thickening has many causes and is not as
• Ascites specific as a striate wall

Hyperemia of Acute Cholecystitis
Complications/Severity of Acute Cholecystitis
Options for Treatment of Acute Cholecystitis

• PCCL
• OC
• LC
• Temporize
Role of the Radiologist in Acute Cholecystitis

• Identify findings that make temporizing ill-advised or that would potentially
result in open cholecystectomy:
➢ Extreme striations
➢ Gangrenous cholecystitis
➢ Emphysematous cholecystitis
➢ Perforation
➢ Biliary obstruction / Mirizzi syndrome
➢ Incidental findings that preclude LC Figure 2-22-7
Incidental Finding
• Hemangioma next to GB neck
Gangrenous Cholecystitis
• Not always Clostridal infection
• Implies severe inflammation
• Sonography-may see desquamated
mucosa/membranes
• Scintigraphy-increased pericholecystic
activity* due to:
➢ delayed excretion from perihepatitis Two different patients show a band of tracer where the liver
➢ hyperemia with increased tracer abuts the inflamed gallbladder (arrow)
delivery
*Smith et al, Radiology 156:197, 1985
Gangrenous Cholecystitis-Rim Sign [Figure 2-22-7]

• Two different patients
Gangrenous Cholecystitis
• Sloughed membranes
Emphysematous Cholecystitis
• Elderly patients, 20–30% are diabetic
• Male predominance
• 1/3 infected with Clostridia welchii
• Perforation 5 times as common as for non-emphysematous cholecystitis
• “Dirty” shadowing and echogenic GB wall on sonography is suggestive
• Don’t forget plain film – differential diagnosis of RUQ air
Figure 2-22-8
• US-echogenic foci=gas
• KUB
[Figure 2-22-8]
• US-ring-down
• KUB
US-ring-down (reverb) KUB

Not all GB Walls with Ring-Down Contain Gas
• Diagnosis?
Not all GB Walls with Ring-Down Contain Gas

• Adenomyomatosis
Emphysematous Cholecystitis-CT
• Gas confined to GB wall Figure 2-22-9
• Gas in lumen, hepatic ducts
Gallbladder Empyema [Figure 2-22-9]

• Infection in obstructed, inflamed
gallbladder
• 25% incidence-rises to 80% if untreated
after 7 days*
• Results in marked GB distention in 38%
of patients**
• US, CT-Nonspecific. See distention,
bile/debris level, “snow storm”
*Goldman et al, Gastro 11:318, 1948
**Fry et al, Am J Surg 141:366, 1981
Perforation of the Gallbladder

• Seen both in the context of chronic cholecystitis (eg., gallstone ileus) and AC
• In older literature, occurs in 3–15% of patients with acute cholecystitis
• Patient feels transiently better and then develops peritoneal signs
• Cholescintigraphy – extravasated activity – maybe
• Sonography, CT-pericholecystic collection, non-specific
GB Perforation in AC [Figure 2-22-10]
GB Perforation
Figure 2-22-10
Choledocholithiasis
• May occur as primary duct stone (usually pigment),
secondary to gallstones, or following
cholecystectomy
• Most small stones will pass spontaneously. The duct
caliber and dynamics may rapidly change
• CT and US are approx. 70-80% sensitive for
detection of choledocholithiasis
• If you suspect CBD stones…options are MRCP,
ERCP, intraop cholangiogram
When to Perform MRCP

• Jaundice Contracted GB
• Dilated ducts on US
• Delayed egress on IDA
• Anatomic finding that suggests process that may result in altered duct
anatomy or make laparoscopic cholecystectomy risky
• Post-cholecystectomy complications
MRCP
• T2 wt TSE or FSE, thin sections or “slab”
• Extra sections as needed

RUQ Pain (789.01) and Fever: Approach
• “R/O Acute calculous cholecystitis”...IDA, US
• “R/O Stones”...US
• “R/O Acute acalculous cholecystitis”...?
• “R/O a reason to operate”... if suspect biliary do US, if suspect acute abdomen/GI disease do CT
• Remember MRCP
References
1. Zeman RK, Garra BS. Gallbladder Imaging: The State-of-the-art. Gastroent Clin N. Am 2:127, 1991.
2. Garra BS, Davros WJ, Lack EE, Horii SC, Zeman RK: Visibility of gallstone fragments at ultrasound and
fluoroscopy. Implications for monitoring of gallstone lithotripsy. Radiology 174:343, 1990.
3. Mathieson JR, So CB, Malone DE, Becker CD, Burhenne HJ: Accuracy of sonography for determining the number
and size of gallbladder stones before and after lithotripsy. AJR 153:977, 1989.
4. duPlessis DJ, Jersky J. Management of acute cholecystitis. Surg Clin North Am 53:1071, 1973.
5. Halasz NA. Counterfeit cholecystitis: A common diagnostic dilemma. Am J Surg 130:189, 1975.
6. Zeman RK, Burrell MI, Cahow CE, Caride V. Diagnostic utility of cholescintigraphy and ultrasonography in acute
cholecystitis. Am J Surg 141:446, 1981.
7. Weissmann HS, Badia J, Sugarman LA et al. Spectrum of 99m Tc-IDA cholescintigraphic patterns in acute
cholecystitis. Radiology 138:167, 1981.
8. Eikman EA, Cameron JL, Colman M et al. A test for patency of the cystic duct in acute cholecystitis. Ann Int Med
82:318, 1975.
9. Fonseca C, Greenberg D, Rosenthall L et al. Assessment of the utility of gallbladder imaging with 99m Tc-IDA.
Clin Nucl Med 3:437, 1978.
10. Freitas JE. Cholescintigraphy in acute and chronic cholecystitis. Semin Nucl Med 12:18, 1982.
11. Shuman WP, Gibbs P, Rudd TG et al. PIDIDA scintigraphy for cholecystitis: False positives in alcoholism and total
parenteral nutrition. AJR 138:1, 1982.
12. Kalff V, Froelich JW, Lloyd R et al. Predictive value of an abnormal hepatobiliary scan in patients with severe
intercurrent illness. Radiology 146:191, 1983.
13. Laing FE, Federle MP, Jeffrey RB et al. Ultrasonic evaluation of patients with acute right upper quadrant pain.
Radiology 140:449, 1981.
14. Ralls PW, Colletti PM, Lapin SA et al. Real-time sonography in suspected acute cholecystitis: Prospective
evaluation of primary and secondary signs. Radiology 155:767, 1985.
15. Cohan RH, Mahony BS, Bowie JD, Cooper C, Baker ME, Illescas FF: Striated intramural gallbladder lucencies on
US studies. Predictors of acute cholecystitis. Radiology 164:31–35, 1987.
16. Teefey SA, Baron RL, Bigler SA: Sonography of the gallbladder. Significance of striated (layered) thickening of
the gallbladder wall. AJR 156:945, 1991.
17. Shaler WJ, Leopold GR, Scheible FW: Sonography of the thickened gallbladder wall. A nonspecific finding. AJR
136:337, 1981.
18. West MS, Garra BS, Horii SC, Zeman RK et al. Gallbladder varices: Imaging findings in patients with portal
hypertension. Radiology 179:179, 1991.
19. Weissmann HS, Berkowitz D, Fox MS et al. The role of technetium-99m iminodiacetic acid (IDA)
cholescintigraphy in acute acalculous cholecystitis. Radiology 146:177, 1983.
20. Shuman WP, Rogers JV, Rudd TG et al. Low sensitivity of sonography and cholescintigraphy in acalculous
cholecystitis. Radiology 142:531, 1984.
21. Swayne LC. Acute calculous cholecystitis: Sensitivity in detection using technetium-99m iminodiacetic acid
cholescintigraphy. Radiology 160:33, 1986.
22. Mirvis SE, Vainright JR, Nelson AW, et al. The diagnosis of acute acalculous cholecystitis: A comparison of
sonography, scintigraphy, and CT. AJR 147:171, 1986.
23. Jeffrey RB, Laing FC, Wong W, Callen PW. Gangrenous cholecystitis: Diagnosis by ultrasound. Radiology
156:797, 1985.
24. Wales LR. Desquamated gallbladder mucosa: Unusual sign of cholecystitis. AJR 139:810, 1982.
25. Smith R, Rosen J, Gallo LN, Alderson PO. Pericholecystic hepatic activity in cholescintigraphy. Radiology
156:797, 1985.
26. Siskind B, Hawkins M, Cinti D, Zeman RK, Burrell MI. Perforation of the gallbladder: Radiologic-pathologic
correlation. J Clin Gastroenterol 9:670–78, 1987.
27. Clemett AR, Lowman RM. The roentgen features of the Mirizzi syndrome. AJR 94:480, 1965.
28. Weltman D, Zeman RK. Imaging of acute diseases of the gallbladder and bile ducts. Radiological Clinics of North
America 32:933-950, 1994.
29. Fulcher AS, Turner MA, Capps GW. Technical Advances and Clinical Applications. RAdiographics 19:25-41,
1999.

Inflammatory Diseases of the Esophagus
Marc S. Levine, MD
Technique Figure 2-23-1

• Double-contrast:
➢ Upright
➢ Right lateral cardia
• Single-contrast:
➢ Separate swallows
➢ Prone esophagus
Reflux Esophagitis
• Most common inflam condition
• Purpose of Ba study not simply to show HH/GER but to R/O morphologic
sequelae of GERD
Pathogenesis
• Frequency of GER
➢ Decreased LES tone
➢ Mult trans LES relaxations
• Duration of GER
➢ Abnormal motility
➢ (scleroderma)
Pathogenesis
• Acidity of refluxate
➢ ZES (increased acid)
➢ Billroth II (bile or panc)
• Resistance of mucosa
➢ Age
➢ Debilitation
Clinical Findings
• Heartburn and regurg Reflux esophagitis with
• Epigastric or RUQ pain granular mucosa
• Upper GI bleeding
• Dysphagia (peptic stx)
Figure 2-23-2
Hiatal Hernia and GER
• Occur independently
• Spont GER at fluoro:
➢ 30–60% in esophagitis
➢ 40–50% in volunteers
Reflux Esophagitis: Radiographic Findings

[Figures 2-23-1 and 2-23-2]
• Abnormal motility
• Granularity
• Thickened folds
• Inflammatory EG polyp
• Ulceration
Peptic Scarring: Radiographic Findings

• Radiating folds
• Deformity of wall
• Peptic stricture
• Sacculations
• Transverse folds Reflux esophagitis with
ulceration
Inflammatory Disease of the Esophagus 444 Gastrointestinal Radiology

Radiologic Dx of Esophagitis
Gr 1 Gr 2 Gr 3
• Koehler 13% 90% 100%
• Ott 22% 83% 95%
• Creteur 53% 93% 100%
Schatzki Ring
• Variant of peptic stx
• Episodic dysphagia (meat)
➢ (Steakhouse syndrome)
• Symm ringlike constriction
• Vertical height 2–4 mm
• Usually sx if < 13 mm diam
• Best seen on prone views
Barrett’s Esophagus
• Prog columnar metaplasia from GER and esophagitis
• Prevalence:
➢ 10% with esophagitis
➢ 40% with peptic stx
Clinical Findings
• Men > Women, W > B
• Reflux sx, dysphagia
• Tx of GER may not cause Barrett’s to regress
Histologic Findings
• Projections or islands of columnar epith separated by squam epith
• Foveolar epith > 3 cm above LES or intestinal metaplasia with goblet cells
Figure 2-23-3
Premalignant Condition
• Risk of adenocarcinoma
• Dysplasia-Ca sequence
• Endoscopic surveillance
Radiographic Findings [Figure 2-23-3]

• Classic: High stx or ulcer or reticular pattern
• Common: GER, hiatal hernia, reflux esophagitis, or peptic stricture
Dx of Barrett’s by D/C Tech

• 200 pts with reflux sx
• Classified at high, mod, or low risk for Barrett’s
AJR 150:97–102, 1988
Classification of Risk
• High: High stx or ulcer or reticular pattern
• Mod: Distal stx or reflux esophagitis
• Low: None of above
Radiologic vs Endoscopic Dx
Risk Endo
• High 10 9 (90%)
• Moderate 73 12 (16%)
• Low 117 1 (1%)
Barrett esophagus
Radiologic Diagnosis with mid esophageal stricture
• Less sensitive than endoscopy
and reticular pattern
• Most false negative exams in mild disease
• Vast majority do not have Barrett’s esophagus
Gastrointestinal Radiology 445 Inflammatory Disease of the Esophagus

Reflux Symptoms
Figure 2-23-4
Candida Esophagitis
• Most common type
• Immunocompromised (75%)
• Local esophageal stasis (25%)
➢ (achalasia, scleroderma)
Clinical Findings
• Dysphagia/odynophagia
• OP Candidiasis (50%)
• Marked clinical response to antifungal agents (ketoconazole)
Candida esophagitis
Radiographic Findings [Figures 2-23-4 and 2-23-5] with plaques
• Mucosal Plaques (90%)
➢ Linear Figure 2-23-5
➢ Etched in white
• “Shaggy” esophagus (AIDS)
➢ Plaques and membranes
➢ Superimposed ulcers
Herpes Esophagitis
• 2nd most common type
• Herpes simplex virus type 1
• Immunocompromised
• Viral Cx or Bx (intranuclear inclusions in cells adjacent to ulcers)
Clinical Findings
• Dysphagia/odynophagia
• Oropharyngeal herpes
• Marked clinical response to antiviral agents (acyclovir)

• Discrete ulcers in upper and midesophagus
• Ulcers and plaques (mimics Candida)
Herpes Esophagitis in Healthy Pts

• Young men (15–30 y/o)
• Sexual partners with OP herpes
• Flu-like prodrome (3–10 days)
• Severe odynophagia
• Multiple tiny ulcers
• Sx resolve in 3–14 days
Candida esophagitis
with shaggy esophagus

CMV Esophagitis
• Pts with AIDS
Figure 2-23-6
• Odynophagia
• Viral Cx or Bx (intranuclear inclusions in cells at ulcer base)
• Tx with ganciclovir (endo for confirmation)
Radiographic Findings [Figures 2-23-7]

• Nodular mucosa
• Small ulcers (mimics herpes)
• Giant ulcers
Herpes esophagitis
with tiny ulcers
CMV esophagitis HIV esophagitis

with giant ulcer with giant ulcer
HIV Esophagitis
• Odynophagia and giant ulcers
• Palatal ulcers
• Maculopapular rash
• Recent seroconversion
• Dx of exclusion (No CMV)
• Treatment with steroids

• Giant ulcers
➢ (mimics CMV)
• Satellite ulcers
Giant Ulcers in 21 HIV + Pts

Cause No Pts %
• HIV 16 76
• CMV 3 14
• Both 2 10
Radiology 194:447–451, 1995
Giant Ulcers in 21 HIV + Pts

• All had AIDS (CD4 ct < 200)
• Avg time from serodetect 2 yrs
• Only 1 pt had palatal ulcers or rash

Conclusions Figure 2-23-9
• Most giant ulcers in HIV+ Pts caused by HIV not CMV
• Impossible to diff by clin or rad criteria
• Endoscopy for definitive Dx
Drug-Induced Esophagitis [Figure 2-23-9]

• Contact esophagitis (doxycycline, tetracycline, KCl, quinidine, NSAIDs,
alendronate)
• Aortic arch or lt main bronchus
• Superficial ulcers
• Severe odynophagia but rapid clinical improvement after withdrawal of
offending agent
Radiation Esophagitis
• 2–5000 rad: self-limited esophagitis (1 – 2 weeks)
• 5000 or more rad: stx, progressive dysphagia (4–8 months)
• Adriamycin potentiates XRT (only 500 rad)
Radiographic Findings
• Acute
➢ Ulceration
➢ Granular mucosa
➢ Decreased distensibility Tetracycline-induced
• Chronic esophagitis with three ulcers
➢ Strictures
Caustic Esophagitis
• Strong acids or alkali (liquid lye)
• Three phases of injury: Figure 2-23-10
➢ Acute necrosis
➢ Ulceration and granulation
➢ Cicatrization
• Chest pain, odynophagia, hematemesis,
shock
Radiographic Findings Figure 2-23-11

• Acute
➢ Ulceration
➢ Perforation
• Chronic
➢ Strictures
➢ (1–3 months)
Esophageal Intramural
Pseudodiverticulosis
• Dilated excretory ducts
• Ductal obstruction
• Candida, diabetes, alcohol
• High strictures classic
• Peptic stx more common
[Figures 2-23-10 and 2-23-11]
• Flask-shaped outpouchings
• “Floating” outside wall
• Associated strictures
Diffuse esophageal Localized esophageal
➢ (especially peptic stx)
intramural intramural
pseudodiverticulosis with pseudodiverticulosis with
high stricture peptic stricture

Esophageal Varices
Location Cause
• Uphill Distal Portal HTN
• Downhill Mid SVC obst
Diffuse Nodules/Plaques
Cause Finding
• Reflux Granularity
• Candida Plaques
• Glycogenic acanthosis Nodules/plaques
Localized Nodules/Plaques
Cause Finding
• Candida Plaques
• Sup spr Ca Coalesce nodules
• Barrett’s Reticular pattern
Ulcers
Cause Finding
• Reflux Distal
• Herpes Small, mid
• Drugs Small, mid
• CMV Giant
• HIV Giant
Thickened Folds
• Esophagitis
• Varices
• Varicoid Ca
High Strictures
• Barrett’s esophagus
• Mediastinal irradiation
• Caustic ingestion
• Primary or metastatic tumor
Distal Strictures
• Peptic stricture
• Lower esoph ring
• Barrett’s Ca

Tumors of the Esophagus
Marc S. Levine, MD
Mucosal Lesions
• Squamous papilloma
• Adenoma
• Glycogenic acanthosis
Squamous Papilloma: Pathologic Findings

• Coral-like excrescence
• Fibrovascular core
• Hyperplastic squamous epithelium
Clinical Findings
• Malignant degeneration rare
• Multiple papillomas (papillomatosis)
Figure 2-24-1
• Small, sessile polyp
• Lobulated mass
• Bubbly appearance
• Diff Dx – early Ca
Glycogenic Acanthosis
• Accum of cytoplasmic glycogen
• White nodules/plaques
• Rarely causes esophageal sx
• No risk of malignant degeneration

• Round nodules/plaques
• 1–5 mm in diameter
• Predominantly midesophagus
• DDx – Candidiasis
Intramural Lesions
• Fibrovascular polyp
• Leiomyoma
• Duplication cyst
• Idiopathic varix
Leiomyoma: Pathologic Findings

• Most common benign tumor Glycogen acanthosis with nodules
• Bands of smooth muscle
• 60% DT, 30% MT, 10% PT
• Up to 20 cm in diameter
• Patterns – submucosal, exophytic, intraluminal, circumferential
Clinical Findings
• Most pts asx
• Dysphagia
• GI bleed rare
• Enucleation

• CXR – soft tissue mass, Ca++ rare
Tumors of the Esophagus 450 Gastrointestinal Radiology

• Ba – submucosal mass Figure 2-24-2
• CT – soft tissue mass
• DDx – fibroma, hemangioma, granular cell tumor, duplication cyst
Unusual Findings
• Annular lesion
• Giant intraluminal mass
• Gastric involvement
• Multiple lesions
• Leiomyomatosis
Leiomyomatosis
• Proliferation of smooth m.
• Children/adolescents
• Long-standing dysphagia
• Familial – autosomal dominant
• Alport’s syndrome (nephritis, deafness, ocular lesions)
Esophageal leiomyoma in
profile
• Ba – tapered narrowing of distal esophagus (1° achalasia?)
• Length > achalasia
• Symmetric fundal defects Figure 2-24-3
• CT – thickened wall (2° achalasia?)
Fibrovascular Polyp: Pathologic Findings

• Benign intraluminal tumor
• Fibrous/adipose/vascular tissue with nl squam epith
• Hamartoma/fibroma/lipoma/fibrolipoma/angiolipoma
• All classified as FVPs
• Malig degen rare
Pathologic Findings
• Arises in cervical esophagus
• Loose submucosal conn tiss
• Dragged inf by peristalsis
• Occas prolapses into fundus CXR with right superior mediastinal mass
• Pedicle in cervical esophagus caused by fibrovascular polyp
Clinical Findings Figure 2-24-4

• Dysphagia
• Resp sx – inspiratory stridor, choking, wheezing
• Regurgitation of fleshy mass
• Asphyxia/sudden death
Radiographic Findings [Figures 2-24-3 to 2-24-5]

• CXR
➢ Rt sup med mass
➢ Retrotracheal bowing
• Ba Figure 2-24-5
➢ Smooth, expansile
intraluminal mass
➢ Var size & location
➢ Lobulation common
➢ Prox pedicle rare
• DDx
Air bubble, achalasia,
malignant tumor
Fibrovascular polyp on barium
Radiographic Findings study
[Figures 2-24-5 and 2-24-6]
(same patient as Figure 2-22-3)
Path CT Fibrovascular polyp with fat
attenuation on CT
Gastrointestinal Radiology 451 Tumors of the Esophagus

• Adipose Lipid density [1] Figure 2-24-6
• Mixed Heterogeneous
• Fibrous Soft tissue density
[1] High-signal intensity on T1MR / High echo on endoscopic U/S
Duplication Cyst
• Abnl embryo development
• Sequest from prim foregut
• Ciliated columnar epith
• Most pts asymptomatic
• Occas bleeding/infection
Radiographic Findings Fibrovascular polyp with geographic

• CXR: Mediastinal mass areas of fat and soft tissue
• Ba: Submucosal mass attenuation on CT
• CT: Homogen low atten
• MR: High-signal on T2
Malignant Tumors
• Squamous cell carcinoma
• Adenocarcinoma
• Spindle cell carcinoma
• Leiomyosarcoma
• Kaposi’s sarcoma
• Malignant melanoma
• Lymphoma
• Metastases

• Epidemiological Factors
• Tobacco and alcohol
• Geographic variations (China, Iran, S Africa)
• Low molybdenum in soil (accum of nitrosamines)
Predisposing Factors
• Achalasia
• Lye strictures
• Head and neck tumors
• Celiac disease
• Plummer-Vinson
• Tylosis
Definitions
• Early: mucosa or submucosa without lymph node mets
• Superficial: mucosa or submucosa with or without lymph node mets
• Small: < 3.5 cm regardless of depth of invasion or lymph node mets
Routes of Spread
• Direct extension – trachea, bronchi, lungs, pericard, aorta, diaphragms
• Lymphatic spread – nodes in med, neck, upper abdomen (paracardiac, lesser
curv, celiac)
• Hematogenous – lungs, adrenals, liver
Clinical Findings
• Dysphagia and wt loss
• Odynophagia (if ulcerated)
• Chest pain (poor sign)
• Paroxysmal coughing (if TEF)
• 5-year survival < 10%

Early Squamous Cell Carcinoma: Radiographic Findings Figure 2-24-7
[Figure 2-24-7]
• Small, sessile polyp
• Plaquelike (central ulcer)
• Focal irregularity in wall
• Superficial spreading
Adv Squamous Cell Carcinoma [Figures 2-24-8 and 2-24-9]

• Plain Film
➢ Widened med
➢ Ant tracheal bowing
➢ Thick RT stripe
➢ A/F level in esoph
• Barium
➢ Infiltrating
➢ Polypoid
➢ Ulcerative
➢ Varicoid
Superficial spreading
Squamous Cell Carcinoma: Staging carcinoma with focal nodularity
• CT: Sens limited by adenopathy (mets in nl-sized nodes)
• MRI: Comparable to CT
• US: Depth of invasion & lymph node mets
Adenocarcinoma
• Arises in Barrett’s mucosa
• Dysplasia-Ca sequence (low-grade, high-grade, ca-in-situ, invasive)
• Comprises 20–50% of esoph Ca’s
• Predominantly in distal esophagus
• Often invades proximal stomach
Figure 2-28-9
• Prevalence 10% in Barrett’s esoph
• 30–40X greater risk than gen pop
• Dysphagia and weight loss
• Same prognosis as squamous Ca
• Endoscopic surveillance
Figure 2-28-8
Primary ulcerative carcinoma with Varicoid carcinoma with

giant meniscoid ulcer surrounded large submucosal defects
by rind of tumor in lower esophagus
Gastrointestinal Radiology 453 Tumors of the Esophagus

Radiographic Findings [Figures 2-24-10 and 2-24-11]
• Early: sessile polyp, plaque, sup spreading, stricture
• Adv: infiltrating, polypoid, ulcerative, varicoid (often invades cardia)
Adenocarcinoma in Barrett esophagus

invading gastric cardia
Early adenocarcinoma in Barrett’s

esophagus with plaque-like lesions
Spindle Cell Carcinoma Figure 2-24-12

• Carcinomatous and sarcomatous elements
• Spindle cell metaplasia
• Dysphagia and weight loss
• Same prognosis as squamous Ca

• Ba-polypoid intraluminal mass expanding lumen without obstruction
• CT-expansile esophageal mass
• DDx-malignant melanoma
Spindle cell carcinoma seen as

polypoid mass expanding
esophagus without causing
obstruction

Radiology of Peptic Ulcer Disease
Marc S. Levine, MD
Hypertrophic Gastritis [Figures 2-25-1 and 2-25-2]

• Glandular hyperplasia
• Increased acid secretion
• Thickened folds
• Diff Dx:
➢ Menetrier’s
➢ Lymphoma
Erosive gastritis with varioloform erosions in

antrum
Antral gastritis with

hypertrophied antral-
pyloric fold on lesser
curvature of distal antrum Figure 2-25-3
Antral Gastritis [Figure 2-25-1]
• Thickened antral folds
• Longitudinal or Transverse
• Crenulation of lessercurvature
• Hypertrophic antral-pyloric fold
Causes of Erosive Gastritis [Figure 2-25-3]

• PUD
• NSAIDs
• Alcohol
• Stress
• Trauma
• Crohn’s
Acute Aspirin Ingestion (2–8 tabs/day in nl pts)

Finding Time Span
• Erosions 8–24 hrs
• Max damage 1–3 days
• Healing 3–7 days
Duodenitis
• Spastic bulb NSAID-induced erosive gastritis with
• Thickened folds linear erosions clustered in gastric
• Nodules body near greater curvature
• Erosions
Gastrointestinal Radiology 455 Peptic Ulcer Disease

Gastric Ulcers
• Shape
• Size
• Location
• Morphology
Benign posterior wall gastric ulcer Anterior wall gastric ulcer seen as ring shadow on
double contrast view
Ulcer fills with barium on prone compression Giant NSAID greater curvature ulcer
Multiple Gastric Ulcers

• 2–30% of pts with GUs
• Association with aspirin in 80% Figure 2-25-8
• Each ulcer evaluated separately
Upper GI vs Endoscopy
• More than 95% GUs Dx in North America are benign
• 6–16% of benign-app GUs on S/C studies are
malignant
• Is endo always necessary?
Benign Gastric Ulcers [Figures 2-25-4 to 2-25-8-]

• En Face
➢ Round or ovoid crater
➢ Smooth mound of edema
➢ Symmetric radiating folds
• In Profile NSAID-induced greater curvature ulcer with
➢ Projection outside lumen gastrocolic fistula
➢ Hampton’s line or ulcer mound or collar
Peptic Ulcer Disease 456 Gastrointestinal Radiology

Malignant Gastric Ulcers [Figure 2-25-9] Figure 2-25-9
• En Face
➢ Irregular crater in mass
➢ Loss of areae gastricae
➢ Nodularity, clubbing, or fusion of radiating folds
• In Profile
➢ Projection of crater inside lumen within mass
➢ Acute angles of mass
Equivocal Ulcers
• Irregularity of ulcer shape
• Asymmetry of mass effect
• Nodularity, irregularity, or clubbing of radiating folds
• Enlarged areae gastricae
• Location on greater curve
Malignant lesser curvature ulcer with clubbed folds
abutting ulcer on prone compression
Radiologic Dx of Gastric Ulcers
Rad Dx No Pts Endo Final Dx
• Benign 191 164 All ben
• Equiv 69 63 56 ben / 7 malig
• Malig 72 68 2 ben / 66 malig
AJR 141:331–333, 1983
Radiologic Dx of Gastric Ulcers

Rad Dx No Pts Endo Final Dx
• Benign 68 24 All ben
• Equiv 37 33 All ben
• Malig 3 3 All malig
AJR 164:9–13, 1987
Gastric Ulcer Investigation
Advantages of Upper GI over Endoscopy

• Shorter procedure time
• Negligible risk
• Lower cost
Upper GI vs Endoscopy
Cost
• D/C upper GI $218
• Endoscopy procedure $540
• Pathology $180
• Hospital $102
• Total $822
Cost of Evaluating 1 Million GUs in United States

• UGI + endo $1 billion
• UGI + sel endo $490 million
• Diff in Cost $510 million

Ulcer Healing Figure 2-25-10
• Change in size and shape
• Avg pd for healing 8 wks
• Ulcer scar in 90%
Ulcer Scar
• Central pit or depression
• Radiating folds
• Retraction of adjacent wall
Duodenal Ulcers [Figure 2-25-10]

• 90% < 1 cm in size
• 50% on anterior wall
• 85% with deformed bulb
• 5% linear Duodenal bulbar ulcer
• 15% multiple
Giant Duodenal Ulcers

• Greater than 2 cm in size Figure 2-25-11
• Higher frequency of complix (bleeding, obst, perforation)
• Fixed configuration at fluoro
Postbulbar Duodenal Ulcers [Figure 2-25-11]

• 5% of all duodenal ulcers
• Medial wall of prox descending duodenum above papilla
• Indentation of lateral wall
• Notoriously difficult to Dx
• Can result in development of ring stricture
Investigation of Duodenal Ulcers
Post-bulbar duodenal ulcer with ulcer niche

in proximal descending duodenum
H. Pylori Figure 2-25-12

• Gram-negative bacillus
• Increases with age (50% of pop > age 60)
• Eradicated by antibiotics and antisecretory agents

• Thickened gastric folds (predom antrum and body)
• Polypoid gastritis with thickened, lobulated folds
• Enlarged areae gastricae
Association with Gastric Carcinoma

• Increased risk of gastric Ca
• Less than 1% develop Ca
• Not enough evidence to treat all pts with H.pylori
Polypoid H. pylori gastritis with markedly

thickened, lobulated folds in gastric body
Peptic Ulcer Disease 458 Gastrointestinal Radiology

H. Pylori & Gastric Lymphoma
• Stomach devoid of lymphoid tissue
• Development of lymph follicles with H. pylori (MALT)
• Low-grade MALT lymphoma (MALTOMA)
• Characteristic pathologic features
Gastric MALT Lymphoma

• Regress with antibiotics in 70-80%
• Precursor of high-grade lymphoma
Figure 2-25-13
• 50-72% of all gastric lymphomas
• More common than prev recognized

• Nodularity of mucosa (rounded 2-7 mm nodules)
• Diff Dx:
➢ Focal gastritis
➢ Intest metaplasia
➢ Enlarged areae gastricae
Risk of Ulcers
Prevalence
Gastric ulcer 60-80%
Duodenal ulcer 95-100% Gastric MALT lymphoma with confluent nodules in
gastric body
Detection of H.Pylori
• Endoscopic bx
• Urea breath test
• Serum Ab test

Pancreatitis: Imaging Has Made a
Difference
Bruce P. Brown, MD
“Is this heaven?” “No. It’s the anterior pararenal space.”
Normal Pancreas
Acute Pancreatitis
• (Marseilles 1985)
• Sudden onset abdominal pain
• Increased pancreatic enzymes, blood, urine
• Pancreatic edema, fat and gland necrosis, hemorrhage
• Variable involvement of regional or remote tissues (Atlanta 1992)
• (Marseilles 1985)
• Recurrent or persistent abdominal pain
• +/- increased enzymes
• Irreversible morphologic change in pancreas
➢ Fibrosis
➢ Acinar destruction
➢ Calcification
• Diffuse, Focal
• Loss of function
Acute Pancreatitis: Who Gets It?

• Biliary stones (45%)
• Alcohol (35%)
• Idiopathic (10–15%)
• Hypercalcemia
• Hypertriglyceridemia
• Drugs
• Post ercp
• Hereditary
• Trauma
• Infection
• Vasculitis
• Pancreatic cancer
• Pancreas divisum
• Sludge?
Acute Pancreatitis: Pathophysiology

• Alcohol
➢ Alters duct permeability -> protein precipitation in ductules
• Gallstones
➢ Common channel of bile and
pancreatic ducts -> bile reflux into
pancreatic duct
Acute Pancreatitis: A Cascade of

Events
Pancreatitis 460 Gastrointestinal Radiology

Acute Pancreatitis: Good and Bad
• Interstitial
➢ Edema
➢ Architecture preserved
➢ No hemorrhage
• Hemorrhagic
➢ Tissue necrosis, pancreas, fat
➢ Hemorrhage
➢ Vascular thrombosis & inflammation
Acute Pancreatitis: Clinical Dx

• Abdominal pain->back
• Nausea, vomiting
• DDx
➢ Perforated ulcer, bowel ischemia, cholecystitis
• Labs
➢ Hyperamylasemia
➢ Elevated lipase, more specific for pancreatitis
➢ Degree of enzyme elevation: no correlation w. severity
Acute Pancreatitis: Complications

• Early ( 2-3 days ) multi-organ failure
➢ Cardiovascular, pulmonary, renal
➢ Phospholipase A2, elastase, tumor necrosis factor, cytokines, IL-1,2,6,
trypsinogen activated peptide (TAP)
• Intermediate ( 2-5 weeks )
➢ Infection, pseudocyst, GI, biliary
• Late ( months – years )
➢ Vascular, pseudoaneurysm
Balthzar 2002 Radiol Clin
Acute Pancreatitis: Plain Films

• Chest film
➢ Pleural effusion 43% w. severe pancreatitis
➢ ARDS
➢ Pulmonary infarction
• Duodenum or colon distention
➢ Sentinal loop = focal dilation
➢ Colon cutoff = gas-filled colon -> abrupt cutoff at splenic flexure
Acute Pancreatitis: GI Contrast

• No primary role; may screw up CT
• Perigastritis, duodenitis, colitis -> Thick folds
• Mucosa intact
• Mass effect from pancreatic fluid
• Fundal varices from splenic vein thrombosis
Acute Pancreatitis: Ultrasound

• Of limited use in Dx
• Is pancreatitis associated w gallstones?
• Fluid collections?
• Vascular complications?
• Intervention
Gastrointestinal Radiology 461 Pancreatitis

Acute Pancreatitis: MRI
• Advantages
➢ Gadolinium easy on kidneys Figure 2-26-1
➢ Able to view biliary tract
• Sick Patients
➢ Motion artifacts
➢ Difficult to monitor
➢ Specialized equipment
➢ Intervention difficult
Acute Pancreatitis: CT
• Best overall modality
• Global view
• Prognosis & followup
• Understand widespread nature of pancreatitis
• Routes for intervention Large, well-encapsulated pseudocyst
adjacent to pancreatic tail
Acute Pancreatitis: Terminology
Atlanta Symposium 1992
• Confusion of terms
• Acute pancreatitis
➢ Mild = minimal organ involvement, uncomplicated recovery w. supportive
Rx
➢ Severe = organ failure or complications eg. pseudocyst, necrosis, infected
necrosis, abscess
Figure 2-26-2
Acute Pancreatitis: Acute Fluid Collections
• Extravasated pancreatic fluid
• Anterior pararenal space, lesser sac
• Not loculated
• No capsule
• 40% patients w. acute pancreatitis
• 50% resolve spontaneously
• May develop into pseudocyst
Acute Pancreatitis: Pseudocyst [Figure 2-26-1]

• Loculated collect. of panc. enzymes
• Non-epithelialized wall of fibrous or granulation
tissue
• 4-6 wks to develop
• Arise from acute fluid collect. (30-50%)
• > 5 cm less likely to resolve
Pseudocyst: Complications [Figure 2-26-2]

• Infection
• Bile duct or GI obstruction
• Perforation -> adjacent organs
• Vascular
➢ Venous stenosis,occlusion
➢ Gastric varices
➢ Pseudoaneurysm
➢ Hemorrhage
(top)Large gastric varices produced by splenic

vein thrombosis from pancreatic pseudocyst
adjacent to splenic hilum.
(bottom) Pseudocyst projecting from the
pancreatic tail to the splenic hilum with no
visualization of hilar splenic vein

Acute Pancreatitis : Necrosis
• Non-enhancing pancreas or peripancreatic tiss. – old “phlegmon”
• Non-viable tissue
• Poor prognosis
• Type determines Rx
➢ Sterile-trial of med Rx
➢ Infected-debridement
➢ If infected, mortality 15-50%
• Needle aspiration to Dx
Acute Pancreatitis: Abscess

• Circumscribed collection of pus
• Develops after several weeks
• Needle aspiration
• Percutaneous drainage
Acute Pancreatitis: Location

• Central location affords several routes for spreading disease
• Anterior pararenal space
➢ Pancreas
➢ Duodenum
➢ Colon
• Bare area = reflection of post parietal peritoneum to form the transverse
mesocolon
• Root of the small bowel mesentery contiguous w. transverse mesocolon
• Tail = intraperitoneal -> splenorenal ligament
• Posterior to the lesser sac
Barium Left Anterior Pararenal Space-o-Gram [Figure 2-26-3]
Figure 2-26-3
(top left) Extensive necrosis in the anterior

pararenal space on both sides with air anterior to
the left kidney mistaken for air in the colon.
(bottom left). Delayed views showing contrast
anterior to the left kidney.
Thought to be in the colon.
(right) Barium upper gi contrast study showing
erosion of the pancreatic inflammation into the
small bowel with barium contrast leaking
throughout the entire left anterior pararenal space
Acute Pancreatitis: Good and Bad

• Interstitial (Good) 80-90%
➢ < 10% organ failure
➢ 1-3% mortality
• Hemorrhagic (Bad) 10-20%
➢ Necrosis
➢ 50-60% organ failure
➢ 15-20% mortality
Banks. Gastro Endoscopy 2002

Acute Pancreatitis: Can we predict trouble?
• 75% acute pancreatitis resolve w/o complications
• 5–20% mortality
• Can′t biopsy
• Who is really sick?
• Clinical Predictors
➢ Ranson Criteria Sens = 57–85%; Spec = 68–85%
➢ APACHE II = 77% pos. pred. value on admit; 88% after 48 hrs
Clinical Assessment of Pancreatitis Severity

• RANSON
• Non-Gallstone pancreatitis
➢ Admission (any 3)
❖ > 55yrs old; WBC > 16K,
❖ Blood sugar >200
❖ LDH >350 ; AST > 250
➢ At 48 hrs (any 3),
❖ Hct decr >10
❖ Rise in BUN > 5
❖ Calcium < 8
❖ PO2 < 60
❖ Fluid deficit > 6L
❖ Base deficit > 4
• APACHE II
➢ Vitals signs
➢ PO2
➢ pH
➢ Electrolytes
➢ Creatinine
➢ HCT; WBC
➢ Glasgow coma score
Can Imaging Alone Predict Trouble? Yes

• CT grading (Balthazar 1985;1990)
➢ A = Normal
➢ B = Focal or diffuse enlargement
➢ C = Peripancreatic inflammation
➢ D = Single fluid collection outside gland
➢ E = 2 or more fluid collections or gas in or near panc.
➢ 83 PTS
❖ All A’s discharged w/o complications within 2 wks
❖ A or B -> no abscess
❖ D or E -> 5 of 6 deaths; 89% of abscesses
Imaging Predicts Trouble. Can we refine this further?

• Problem: After classifying patients as high-risk, fluid collections resolved in
54%
• Pancreatic necrosis
➢ Poor gland enhancement correlates w. degree of necrosis at surgery
(Kivisaari GI Radiol 1984)
➢ Gland necrosis correlates with development of complications (Balthazar
Radiol 1990)
❖ No necrosis = no mortality; 6% morbidity
❖ Necrosis = 23% mortality; 82% morbidity

Acute Pancreatitis: Can we predict trouble? [Figure 2-26-4]
• CT Severity Index (Balthazar Radiol: 1990) Figure 2-26-4
• CT anatomic changes
➢ A = 0, B = 1, C =2, D = 3, E = 4
• Gland necrosis
• < 30% = 2, 30-50% = 4, > 50 = 6
➢ 0-1 = no mortality or complications
➢ 2 = no mortality; 4% complications
➢ 7-10 = 17% mortality; 92% complications
Acute Pancreatitis: The Power of CT

• Suspected pancreatitis – Dx in doubt
• Severe pancreatitis suspected of complications
• Pancreatitis w/o improvement in 72 hrs of med. Rx
• Improving pancreatitis that deteriorates
• Severe pancreatitis w. initial scan D-E; CTSI 3–10 –
follow-up may detect asymptomatic complications
• Definition (Marseilles 1985)
➢ Recurrent or persistent abdominal pain
➢ May or may not see increase enzymes
➢ Irreversible morphological change in pancreas
❖ Fibrosis
❖ Acinar destruction
❖ Calcification, duct /parenchyma
➢ Focal, segmental, diffuse
➢ Progressive loss of exocrine/endocrine function
• Who Gets It?
➢ Chronic alcohol abuse (60–70%)
➢ Idiopathic (30%) Balthazar Classification of severity of acute
➢ Biliary tract disease pancreatitis
➢ Hereditary (top). Mild Pancreatitis: CTSI = 0-1. Small amount
➢ Hyperlipidemia of peripancreatic stranding. No fluid collections.
➢ Hyperparathyroid Entire gland enhances.
➢ Pancreas divisum
• Clinical (bottom). Severe Pancreatitis: CTSI = 8-9.
➢ Recurrent abdominal pain (95%), Pancreas outlines are obliterated with necrosis.
➢ Pancreatic insufficiency , No enhancement with contrast
❖ Malabsorption,
❖ Diabetes,
➢ Amylase/Lipase levels +/– abnormal
Chronic Pancreatitis: Pathophysiology

• Poorly understood
➢ Etoh increases ductal secretion ->
➢ Precipitation of protein plugs ->
➢ Calcification
❖ Chain of lakes / dilated duct
➢ Inflammatory infiltrate + fibrosis
Chronic Pancreatitis: Plain Films

• Pancreas Ca++ (75-90%)
➢ Most common in Etoh pancreatitis,
➢ Ductal or parenchymal
➢ May be focal
➢ Increase w. progression pancreatic dysfunction
➢ Also w. hereditary pancreatitis, cystic fibrosis

Chronic Pancreatitis: Barium [Figure 2-26-5]
• Inflammation/scar -> perigastritis
• Not primary disease of bowel
Figure 2-26-5
(left) Severe distortion of the gastric
contours on double-contrast barium study
from chronic pancreatitis with inflammatory
changes and scar in the perigastric tissues,
so-called "perigastritis." There is no primary
gastric disease.
(right) CT appearance of the same patient
showing changes of chronic pancreatitis
with parenchymal calcifications and gland
atrophy
Chronic Pancreatitis: Ultrasound

Heterogenous echotexture
➢ Hyperechoic foci = Ca++/ fibrosis,
➢ Bile &/or pancreatic duct dilation
➢ 40% focal mass DDx = cancer
➢ Complications
❖ Pseudocyst portal / splenic vein thrombosis
➢ Endoscopic ultrasound?
❖ 98% sensitivity / 90% specificity?
Chronic Pancreatitis: Endoscopic Ultrasound

• Difficult to establish a gold standard esp. for mild to moderate disease
• Few studies with histology
➢ Sens = ?87%; Spec = ?64%
Chronic Pancreatitis: CT
• Not as useful as in acute pancreatitis
• Gland enlargement (30%)
• Mass (30%) ? Cancer
• Atrophy (15%)
• Sens. 50-90%: Spec. 55-85%
• Acute + chronic w. exacerbation of disease
Chronic Pancreatitis: MRI

• Parenchymal enhancement
➢ T1 fat-supressed, pre & post Gd dynamic
➢ Decreased signal/Delay in peak vs. controls
➢ Sens = 79%; Spec = 75%
➢ Better than morphologic changes alone
• MRCP- ductal anatomy
➢ Highly T2 weighted, single breath-hold sequences
➢ 85-90% agreement w. ERP for duct caliber
➢ Limited ability to dx early chronic pancreatitis
➢ Functional exam w secretin not conclusive
Remer EM Radiol. Clin. of N. Am.2002
Chronic Pancreatitis: ERCP

• Cambridge Classification of chronic pancreatitis
➢ Mild = 3 side branches dilated; main duct 2–4 mm
➢ Moderate = small cysts, irregular duct
➢ Severe = any of above +
❖ Cyst >10mm, intraductal filling defect, calculi, main duct obstruction,
severe irregularity

Chronic Pancreatitis: Pancreas Divisum
• Dorsal & ventral ducts fail to fuse (5%)
• Minor papilla (Santorini) atretic
• Most of pancreas drained through atretic minor papilla
• Pancreatitis?
• Dx = ERCP
Pancreatitis Requests We Have Known and Loved
Pancreatitis: “Febrile. Please aspirate fluid.” What then?

• You are in charge of thinking ahead.
• Modality?
• Route?
➢ Transgastric? Not for diagnosis only
• What is the plan?
➢ Pus -> tube
➢ Indeterminate -> Gram stain +/- tube
➢ Clear fluid -> Gram stain, culture
➢ Solid stuff -> no flow -> saline -> culture. BX?
Pancreatic Pseudocyst
Pancreatic Fluid Collection: “I am happy to help, but what is the

indication for drainage? My staff wants it”
• Indication for access to evolving fluid collection or necrosis decided on full
evaluation of clinical, lab, and imaging
• Percutaneous drain useless if won′t flow through tube
➢ No tube for necrosis or hematoma
➢ Aspiration to dx infected necrosis
• Uninfected collections and small pseudocysts may resolve on their own
References
1. Topazian M, Gorelick GS. Acute Pancreatitis. In: Yamada T, Textbook of

Gastroenterology, Third Edition, Volume 2. Lippincott Williams and Wilkins, 1999,
2121-2150.
2. Owyang C. Chronic Pancreatitis. In: Yamada T, Textbook of Gastroenterology, Third
Edition, Volume 2, Lippincott Williams and Wilkins, 1999, 2151-2177.
3. Banks PA. Epidemiology, natural history, and predictors of disease outcome in acute
and chronic pancreatitis. Gastrointestinal Endoscopy 2002; 56 (6) S226-S230
4. Meyers MA. Dynamic Radiology of the Abdomen Normal and Pathologic Anatomy,
Fifth Edition.Springer, New York 2000.
5. Balthazar EJ. Staging of Acute Pancreatitis. Radiol. Clin. of N. Am. 2002;40:6, 1199-
1209.
6. Balthazar EJ. Complications of Acute Pancreatitis. Radiol. Clin. of N. Am 2002; 40:6,
1211-1227.
7. Remer EM, Baker ME. Imaging of Chronic Pancreatitis. Radiol. Clin of N. Am 2002;
40:6, 1229-1242.
8. Fulcher AS, Turner MA. MR Cholangiopancreatography. Radiol. Clin of N. Am 2002:
40:6, 1363-1376.
9. Strate T, Knoefel WT, Yekebas E, Isbicki JR. Chronic Pancreatitis: etiology,
pathogenesis, diagnosis, and treatment. Int. J Colorectal Dis. 2003; 18: 97-106,.
10. Chatizicostas C, Roussomoustakaki M, et al. Balthazar Computed Tomography
Severity Index Is Superior to Ranson Criteria and APACHE II and III Scoring Systems
in Predicting Acute Pancreatitis Outcome. J. Clin. Gastroenterol. 2003; 36: 3, 253-260.
11. Wiersema MJ, Hawes RH, et al. Prospective evaluation of endoscopic ultrasonography
and endoscopic retrograde cholangiopancreatography in patients with chronic
abdominal pain of suspected pancreatic origin. Endoscopy 1993;25:555-564.

Gastrointestinal Bleeding In The Age of
the Endoscope. What Does a Radiologist
Have To Contribute?
Bruce P. Brown, MD
GI Bleeding: Demographics
• Older
• Male
• Use alcohol, tobacco
• Aspirin, non-steroidal anti-inflammatory
• Anticoagulants
Peura et al, Am.J.Gastro. 1997
Gastrointestinal Bleeding: Presentation

• Hematemesis – Bloody vomitus, red, coffee grounds; indicates upper GI
bleeding
• Melena – Black, tarry stools; usually indicates upper GI bleeding
• Hematochezia – Red blood per rectum; lower GI bleed, large-volume upper GI
bleed (> 1000 cc)
Acute GI Bleeding: Demographics

• Upper GI 76%
➢ Duodenal & gastric ulcers >50%,
• Lower GI 24%
➢ Diverticular 30-50%
• 79% Anemia
• 31% Hypovolemia
• 59% Transfused
• 45% Endoscopic Rx
• 7% Surgery
• 2% Death
Peura et al, Am.J.Gastro. 1997
Gastrointestinal Bleeding: How Bad Is It?

• Hypovolemia - 30% of GI bleeders
➢ 5 L (10 Units) = normal volume
➢ Hct poor measure of acute bleeding
➢ 20% blood loss -> 10 mmHg drop BP w. standing
➢ 40% blood loss = Shock = resting supine tachycardia, hypotension, pallor,
agitation
➢ Massive GI bleed = > 6 units transfusion needed in 24 hours
Acute Gastrointestinal Bleeding: Diagnosis is NOT the first

priority
• Resuscitation
➢ Two BIG lines 18 gauge
➢ Fluids immediately
➢ Blood when available; 6 u typed & crossed
➢ ICU
Gastrointestinal Bleeding 468 Gastrointestinal Radiology

Gastrointestinal Bleeding: Where Is It?
• Upper GI
➢ Proximal to ligament of Treitz,
➢ Usually melena
➢ NG tube – 16% negative even w. UGI bleed
• Lower GI
➢ Distal to the ligament of Treitz
➢ Usually hematochezia
Upper GI Bleeding: Causes

• Diagnosis % of total
➢ Duodenal Ulcer 24
➢ Gastric Erosions 23
➢ Gastric Ulcer 21
➢ Varices 10
➢ Mallory-Weiss tear 7
➢ Esophagitis 6
➢ Neoplasm 3
➢ Other 11
Silverstein et al,Gastro.Endosc. 1981
Lower GI Bleeding: Causes

➢ Diverticulosis 43
➢ Vascular Ectasia 20
➢ Idiopathic 12
➢ Neoplasia 9
➢ Colitis
❖ Radiation 6
❖ Ischemia 2
❖ Ulcerative colitis 1
➢ Other 7
Reinus et al GI Clin NA 1990
GI Bleeding: Endoscopy
• First line procedure in UGI bleed
➢ 90–95% accurate Dx
➢ Useful for prognosis, treatment
• Performed immediately
➢ Alcoholics,
➢ Large volume loss
➢ Aorto-enteric fistula
• Performed more “electively”
➢ Young, no evidence of hypovolemia
Nuclear Scintigraphy
• Most sensitive non-invasive test
• Detects bleeding rates 0.1ml/min
• Two techniques
➢ Tc 99m sulfur colloid
➢ Tc 99m labeled red blood cells
• Used to
➢ Delineate obscure sources – small bowel, intermittent bleeding
➢ Enhance the efficacy of angiography
Gastrointestinal Radiology 469 Gastrointestinal Bleeding

Angiography
• Usually preceded by RBC study
• Detects 0.5 ml/min
• Upper GI bleeding
➢ When endoscopy inconclusive
➢ Anticipation of transcatheter intervention
• Lower GI bleeding
➢ Procedure of choice?
Upper GI Bleeding: Peptic Ulcer Disease

• Gastric, duodenal, stomal ulcers = 50% UGI bleeding
• Etiology: Non-steroidals, H. Pylori
• Anatomic risk factors
➢ High lesser curve
➢ Posterior-inferior duodenal bulb
➢ Giant gastric (>3 cm) & duodenal (>2 cm)
• Endoscopic risk factors
Risk of Rebleeding: Endoscopy

• Peptic ulcer disease rebleeding
➢ Clean fibrin base 5%
➢ Flat spot 10%
➢ Adherent clot 22%
➢ Visible vessel 43%
➢ Spurting vessel 90%
Laine NEJM; 717; 1994. UCLA-CURE studies.
Gastritis
• Hemorrhage, erythema, erosions
• Erosion = superficial break in mucosa w. punctate bleeding, fibrin base
• Causes
➢ Non-steroidals -> antral erosions, ulcer
❖ bleeding usually not severe, resolve w. D/C
➢ Alcohol ingestion
❖ Direct toxin? ->erythema
Gastritis
➢ Diffuse or patchy erythema, punctate bleeding, vascular ectasia
• Requires reduction of portal hypertension
• Stress Erosions
➢ ICU patients
➢ One or more bleeding erosions
❖ Bleeding may be severe
Acute Hemorrhagic Gastritis
Esophageal Varices
• 50% cirrhotics develop esoph. varices.
• 1/3 of these bleed
• Portal v. pressure >12 mmHg. above Hep.v
• At risk to bleed
➢ Large size
➢ Located near GE Junct.
➢ Vascular ectasia on the varices
• Rapid bleeding
• Emergent endoscopy
• 50% of cirrhotics w. bleed = non-variceal
• Poor prognosis
➢ 30–50% mortality for first bleed
➢ 2/3 die within one year

Esophageal Varices: Rx
• Vasopressin ( somatostatin/octreotide )
➢ 50% effective
• Sclerotherapy – 85–95% effective
➢ Probably improves survival; complications
• Band ligation
➢ As effective as sclero Rx; few complications
• Balloon tamponade
➢ 70–90% effective
➢ 30–50% rebleed after balloon down,
➢ 10–30% severe complications
• TIPS (Transjugular Intrahepatic Portosystemic Shunt)
➢ Expandable stent – hepatic to portal v.
➢ 95% technically successful
➢ As effective as sclero Rx
➢ 10–15% complications
➢ 10–25% encephalopathy
➢ 30–50% stenosis at 1 year
• Surgical porta-caval shunts
➢ 50–80% mortality for emergency shunt
➢ Elective shunts for endoscopic Rx failures
Gastric Varices Without Esophageal Varices
Mallory-Weiss Tear
• 5–10% GI bleeds
• Hx of retching; 40% no retching
• Non-penetrating linear tear(s) near GEJ
➢ 25% multiple lesions; 75% have o. pathol.
• Rx ->endo.oversewing
Gut Hemangioma
• Rare
• Described in young and old
• Esophagus, stomach, sm. bowel, colon
• Classification
➢ Capillary – collection of thin-walled vess.
➢ Cavernous – large, dilated channels w. thrombosis -> Ca++
❖ Tendency to bleed
➢ Angiomatosis – large area of hemangioma
Gut hemangioma
• Cavernous hemangioma
➢ Phleboliths on plain film
➢ UGI = Submucosal mass
• CT
➢ Thick wall
➢ Early enhancement – network of vessels & sinuses thickening the wall
➢ Late enhancement – confluent sinus fill-in
• Endoscopy
➢ Soft, submucosal mass or thickened folds, blue-red discoloration
Small Bowel Bleeding: Tough to Dx

• 3–5% GI bleeds occur in small bowel (2nd portion duod. to ileocec. valve)
• Bleeding is intermittent
• Most common causes are vascular
• Inaccessible
• Anatomy variable

Small Bowel Bleeding: Causes
➢ Angiodysplasia, hemangioma, AVM, vasculitis
• Small bowel tumors
➢ Leiomyoma/sarcoma, adenoma/carcinoma, lymphoma, mets
• Ulcers
➢ Crohn’s, Meckel’s diverticulum, ZE syndrome
• Diverticula
• Aortoenteric fistula
Small Bowel Bleeding: How Well Does Imaging Do?

• Small bowel series vs enteroclysis
➢ 71% lesions missed on small bowel series [1]
• Small bowel series for occult bleeding
➢ 5% yield for bleeding site [2]
• Enteroclysis
➢ 10 % yield for bleeding site [3]
• Enteroscopy
➢ Cumbersome, not generally available
[1] Maglinte, Radiol 144:737; 1982
[2] Rabe, Radiol. 140:47; 1981
[3] Rex, Gastro 58;89; 1997
Small Bowel Bleeding
Nuclear Scintigraphy
• Most sensitive non-invasive test
• Detects bleeding rates of 0.1 ml/min
• Two techniques
➢ Tc 99m sulfur colloid
➢ Tc 99m labeled RBC′s
• Used to
➢ Delineate obscure sources – small bowel, intermittent bleeding
➢ Enhance the efficacy of angiography
Technetium 99m Labeled RBC′′s

• New in vitro process (Ultratag) >95% eff.
• Continuous dynamic imaging
➢ Large FOV camera over abdomen
➢ 60 images q 15 min
➢ Stored for dynamic playback to detect labeled RBC’s outside normal blood
pool
Technetium 99m Labeled Red Blood Cells

• Disadvantages
➢ Origin of bleed unclear on delayed scans
➢ Vascular organs may interfere w. detection
➢ Loss of tag can produce false +/-
• Advantages
➢ Detects intermittent bleeding
• Labeled RBC′s
➢ Sensitivity = 85–95%; Specificity = 70–85%,
➢ Method of choice

Meckel’s Diverticulum
• Most common congenital GI tract anom.
• Vitelline duct fails to resorb
• True diverticulum –
➢ 2% of population
➢ 2 x more common in males
➢ 2 cm long ( 1–10 cm),
➢ 2 feet from ileocecal valve
• 50% ectopic gastric or pancreatic mucosa
• 25–40% symptomatic
• Complications
➢ Bleeding – usually in kids <5 yr,
➢ Intussusception – kids & adults
➢ Volvulus, diverticulitis, perforation
• Bleeding – ulceration of gastric mucosa
Aortoenteric Fistula
• Erosion of aorta into 3rd portion of duod,
• Dacron graft, atheroma, mycotic aneurysm
• “Herald bleed” stops spontaneously followed by exsanguinating bleed
• Preemptive surgery
Pill Endoscopy
• Ingestible capsule
• 7 hour recording
• 2 images per second
• Localizing surface antennae
• View in “real-time”
• Contraindicated w. obstruction
• 22% Capsule vs. 3% barium-positive in 52 pts w. occult gi bleed
Hara AK, Radiol 2004, 230: 260-265)
Lower GI Bleeding: Causes

➢ Diverticulosis 43
➢ Vascular Ectasia 20
➢ Idiopathic 12
➢ Neoplasia 9
➢ Colitis
❖ Radiation 6
❖ Ischemia 2
❖ Ulcerative colitis 1
➢ Other 7
Reinus et al GI Clin NA 1990
Colonic Diverticulosis
• Colon Diverticula = herniations of mucosa and submucosa through muscular
layers at site of penetration of vasa recta through bowel wall.
Colonic Diverticular Bleeding

• 35–50% prevalence of diverticula
• 15% pts. tics bleed
• 5% massively
• The major cause of lower GI bleed
• 75% of tics in left colon
• 70% of bleeding tics in right colon [1]
• Not asst′s w. diverticulitis
[1] Cassarella, NEJM 286:450;1972

Colonic Diverticular Bleeding: RX
• Colonoscopic vasoconstrictor injection, heater probe, laser – select patients
• Angiography
➢ Selective catheterization
❖ Vasopressin 50-90% success
❖ Embolo Rx – Gelfoam, coils
• Surgery
Angiodysplasia
• 20–40% acute LGI bleeding
• Vascular ectasia
➢ 2/3 in pts >70 yrs old
➢ Aortic valve disease
❖ Von Willebrand factor depletion?
• < 5mm vascular tufts
• Cecum & right colon
• Bleeding
➢ Not massive, intermittent
➢ Stop spontaneously, 85% bleed again
• Pathogenesis
➢ Increased tension in cecal wall
➢ Repeated, intermittent obstruction of submucosal veins -> dilation &
tortuosity
➢ Develop small A-V malformation
• Colonoscopy 80–90% sensitive
• Angiography
➢ early tangle of vessels
➢ early filling & slow emptying dilated veins
• Treatment
➢ Abnormal vessels – poor response to vasoconstrictors; may temporize
➢ Endoscopic electrocoagulation
➢ Embolo Rx
➢ Diffuse disease – estrogen-progesterone
➢ Surgery
References
1. Peura DA, Lanza FL, et al. The American College of Gastroenterology Bleeding Registry: Preliminary Findings.
Am J Gastroenterol. 1997, Jun: 92(6): 924-8.
2. Reinus JF, Brandt LJ. Upper and lower gastrointestinal bleeding in the elderly. Gastroenterology Clinics of North
America. 1990 Jun; 19(2): 293-318.
3. Mitros FA, Atlas of Gastrointestinal Pathology. Gower Medical Publishing.
4. Elta GH, Approach to the patient with gross gastrointestinal bleeding. In Textbook of Gastroenterology, Lippincott,
Williams and Wilkins. Philadelphia, 1999, Yamada T, et al eds.
5. Fritscher-Ravens A, Swain CP. The wireless capsule: new light in the darkness. Dig. Dis. 2002;20(2): 127-33.
6. Hara, AK et al. Small bowel: preliminary comparison of capsule endoscopy with barium study and CT
Radiology 2004, 230: 260-265.
7. Hara AK. Capsule endoscopy: the end of the barium small bowel examination?Abdom Imaging, 2005, Jan.

Small Bowel Obstruction
Figure 2-28-1
Small Bowel Obstruction
• “Impaired passage of contents thru SB.”
• Partial vs Complete (“High Grade”)
• Intermittent vs Continuous
• Mechanical vs Paralytic (“Ileus”)
SBO
• Review
➢ Mechanical
➢ Classic Acute “Complete” SBO
❖ Simple SBO
❖ Closed Loop Obstruction (CLO)
- Urgent Emergency !
➢ Classic Appearances
❖ Intermittent “Chronic” SBO
❖ Partial SBO
➢ Motility
❖ Common Ileus
❖ Unusual dysmotilities
Acute mechanical SB obstruction
SBO showing uniformly distended bowel
• Motility
➢ Paralytic Ileus
➢ Scleroderma “Collagen Vasc Disease”
➢ Sprue, MAB diseases Figure 2-28-2
➢ Radiation enteritis, earliest stage
➢ Hypothyroidism, metabolic
➢ Chronic Intestinal Pseudo-obstruction
➢ DYSMOTILITY is a FUNCTIONAL Obstruction !
➢ Slow passage acts / looks obstructive
Chronic vs Acute SBO:

Concept to help analyze SB in CT, KUB, SB Series
• Distention vs Dilatation: 2 variables
➢ Dilatation: SB diameter larger than expected
❖ A few loops or entire SB
❖ May or may not be Distended
➢ Distention: SB uniform appearance of maximum possible
diameter
❖ Like a sausage shaped balloon inflated to its capacity
❖ Appears tensely filled, to capacity
Simple SBO
• A tapered distension meandering back toward Treitz.
• A single transition point
Scleroderma SB shows dilated loops
Chronic Intermittent SBO
with segments whose diameter are
• Dilated but not distended
greater than last case of acute SBO.
Loops are not uniformly distended
Chronic vs Acute SBO [Figures 2-28-1 to 2-28-3]
with segments that are partly
• Distention vs Dilatation
collapsed
• Distended, not (XS) Dilated:
➢ Acute, initial SBO
• Dilated, not Distended:
➢ Chronic, intermittent SBO or
➢ DYSMOTILITIES !
Gastrointestinal Radiology 475 Small Bowell Obstruction

• Dilated and Distended: Figure 2-28-3
➢ Acute, recurrent SBO
Chronic Idiopathic Intestinal Pseudo-obstruction

“CIIPO”
• No cause (readily) apparent.
• Myopathic forms:
➢ More common
➢ Dilated,
➢ Atonic
➢ Conceptually like: Scleroderma
• Neuropathic forms:
➢ Spastic,
➢ Non propulsive peristalsis
➢ Pulsion divertics of SB
➢ Conceptually like: Diffuse Esophageal Spasm
Bloating, Obstruction
• Prior Colectomy for constipation with
• Ileo-rectal anastomosis
• Idiopathic Intestinal Pseudoobstruction [Figure 2-28-4]
• Myopathic type
MNGIE = POLIP
• Mitochondrial Neuro Gastro Intestinal Encephalopathy MNGIE
• Polyneuropathy, Ophthalmoplegia, Leukoencephalopathy,
Intestinal Pseudo-obstruction. P-O-L-IP
• Rarenth power (73 cases up to 2005)
• Familial, Autosomal Recessive
Blondon H, et al Digestive smooth muscle mitochondrial myopathy in
pts with mitochondrial-neuro-gastro-intestinal encephalomyopathy
(MNGIE), 3 cases & review of literature; Gastroenterologie 2005 Aug. SB appears both dramatically dilated
VOL 29 - N 8-9,p. 773 - 778 and uniformly distended.(double
arrow). This suggests acute
Simon et al, Polyneuropathy, Ophthalmoplegia, obstruction in a patient with chronic
Leukoencephalopathy, Pseudoobstruction: POLIP Syndrome; Ann recurring obstruction from Crohns
Neurol 1990;28:349-360 Disease (arrow)
Figure 2-28-4
48 Hr films shows barium in proximal SB.

Dysmotility diseases may produce massive
dilatation. Segments that do not propel act like
mechanical obstruction.
Diseases affecting nerves and muscles of the
bowel are multiple, infrequent, and require
extensive work-up. A history of prior partial
colon resection, SMA syndrome requiring
duodeno-jejunostomy, volvulus of colon in a
young pt, or recurrent obstructions without
apparent cause should raise suspicion
Small Bowell Obstruction 476 Gastrointestinal Radiology

Radiology of “POLIP” “MNGIE” [Figure 2-28-5] Figure 2-28-5
• Slow GI Transit
• Non-propulsive SB hypermotility
➢ LIKE DES Corkscrew esophagus
• SB Tics from segmental spasm
• Malabsorption pattern: wet, moulage,
delay
• MRI: White matter changes, high signal
on T2
• Transition Point: “Single Discordance”
• Tapering: Normal @ Treitz to max
obstruction
• SB Meanders to max allowed by
mesentery
CT: Acute SBO [Figure 2-28-6]

• Early (ER) CT:
➢ Reduce mortality & morbidity
➢ Cost effective
• Diagnose SBO Segmental non coordinated SB
➢ Grade Severity contractions of the small bowel may
❖ Simple produce PULSION DIVERTICULA
❖ Closed Loop (T) and marked delay in transit,
❖ Strangulating findings both evident on a 24 hr follow
❖ Dead Bowel up film with contrast still in stomach
➢ Etiology and proximal SB. This “neuropathic
intestinal pseudo-obstruction pattern”
CT: Acute SBO may be seen in other rare diseases of
• Holy Grail = Transition Point disordered mitochondrial activity
• Define Lesion: altering peristaltic neuromuscular
❖ Tumor, hernia bowel coordination
➢ No “Lesion” = “Adhesion”
➢ Study:
❖ Colon -?
Collapsed SBO
Fluid filled: Ileus, MAB
❖ Ileocecal Valve Figure 2-28-6
❖ Duodenal Crossing
❖ Mesenteric Vessels
Adhesion
• MAJOR CAUSE SBO:
• Benign Adhesions
➢ Surgical
➢ Inflammatory -itis
➢ Radiation
➢ Endometriosis
➢ Ischemia
• Neoplastic Adhesions
➢ (Carcinoid)
Transition point without discernable

mass or hernia indicates adhesion

Tethered Adhesion [Figure 2-28-7] Figure 2-28-7
Bezoar [Figure 2-28-8]

• Rarely sole cause SBO:
➢ THINK: motility dis.
❖ Radiation, scleroderma
➢ Nl person: Fiber binge
• More often part of SBO:
➢ Fibrous Food impaction just above
minor “lesion”. (adhesion) “SB
Feces sign”
➢ POINTS TO OBSTRUCTION Tenting or sharp
Mayo-Smith WW, Wittenberg, et al. CT SB angulation of a loop is
faeces sign: description & clinical suspicious for adhesion
significance. Clin Radiol. 1995 or entrapment of its
Nov;50(11):765-7 mesentery
Abdominal Hernias
• By Location
➢ External vs Internal
Figure 2-28-8
➢ Inguinal, Femoral, Sciatic, Hiatal, Spigelian, etc
• By Type
➢ Complete vs Partial (Richter)
• By Content
➢ Littre (pre-existing tic), Amyand (appendix)
• By Severity
➢ Reducible
➢ Non-Reducible or “Incarcerated”
➢ Ischemic or “Strangulated”
➢ Infarcted Figure 2-28-9
Obturator Hernia
[Figure 2-28-9]
• Elderly F 10 to 1 M
• R >> L
• Assoc w recent wt loss
• Not palpable
• SBO in Obturator
Pectineal Space
• Howship Romberg Sign
➢ Pain medial thigh +/-
50%
• Hannington Kiff
➢ Absent thigh adductor
reflex
Ijiri R, et al Oburator H:
usefulness of CT in DX.:
Surg.1996 Feb;119(2):137-40.
SB “Bezoars” are useful to point to a
site of obstruction. They are
composed of residual fibrous material
that begins fermentation. Carrots,
mushrooms, and other fibrous foods
Obturator Hernias are unusual may cause transient symptomatic
hernias difficult to detect by obstruction in normal patients when
simple physical examination. the bolus reaches the ileocecal valve.
Special maneuvers are Poor peristaltic tone may play a role
needed. Tiny barely visible in pts with dysmotility disorders
hernias may produce
significant obstruction

Spigelian Hernia SBO [Figure 2-28-10]
• Note hernia under extern obl muscle
Figure 2-28-10
Spigelian Hernias may

not be evident on
physical examination.
Classic location is either
lower quadrant lateral to
rectus muscle. The
hernia sac protrudes
through a normal weak
point where the
transversalis muscle
fascia changes from
posterior to anterior attachment to the rectus
sheath. Often the hernia stays under the external
oblique muscle
Figure 2-28-11
Incarcerated & Strangulated Parastomal

Hernia
[Figure 2-28-11]
• SBO upstream
➢ Efferent Limb collapsed
➢ Neck squeezed
• CLO
➢ Distended Hernia Loop
• Strangulation
➢ Hernia Sac Fluid
• Incarceration
➢ Compressed Abd Wall
Chronic incarceration, or non-

reducibility, may cause inward bowing
of the anterior abdominal wall.
Incarceration often is associated with
intermittent obstruction and
predisposed to vascular compromise

Gallstone Ileus [Figure 2-28-12] Figure 2-28-12
Zalcman M, et al Helical CT signs in Dx of
intestinal ischemia in SBO. AJR 2000
Dec;175(6):1601-7
Value of Combining 2 signs for Dx

of Ischemia in SBO
Sign Combination Sens% Spec %
Mural Thickening + 29.2 99.2

Mesen Fluid
Mural thickening + 29.2 93.3
Mesen Vasc Congest
Mural Thickening + 25 94.2
Ascites
Mesenteric Fluid + 50 94.2
Mesen Vasc Congest
Mesenteric Fluid + 66.7 94.2
Ascites
Mesenteric Vasc 41.7 94.2 ? ISCHEMIA
Congestion + Ascites Gallstone Ileus with compromised bowel. Note fistula to
duodenum, air in hepatic bile duct, and stone in distal SB. Top
Zalcman et al Helical CT Signs in Dx of right image shows a target sign loop (left of diamond) with white
Intestinal Ischemia in SBO; AJR internal mucosa and (right of diamond) adjacent loop which has
2000;175:1601-1607 less mucosal enhancement . The lower right arrow points to
the longitudinal equivalent of the target sign. Acute high grade
SB Series: Value of Tangent unrelieved obstruction causes edema from compression of
mucosal venous drainage. Although above findings are of
Value of Tangent…with Valvalsa obstructive edema, simple SBO may lead to bowel infarction
Parastomal Hernias: Tangent with Valsalva
Mesenteric Volvulus
• Assoc w
➢ Malrotation
❖ Left Colon
❖ Right SB
❖ “Weak” Treitz
➢ Internal Hernia
➢ External Hernia
➢ Post operative
• Short / bunched mesentery
Closed Loop Obstruction

• Lumen occluded at 2 adj. sites
➢ Adhesion,
➢ Hernia - Internal, External
➢ Tumor,
➢ Volvulus
• Obstructed loop fills w fluid,
• Distends, elongates
• Base narrows, loop twists
• Venous & Art Occlusion - Infarct may result or may be chronic intermittent
Maglinte, Herlinger, Nolan Rad of CLO: 25 confirmed cases. Radiology. 1991
May;179(2):383-7 (Chronic)

CT Closed Loop Obstruction (CLO) [Figure 2-28-13]
• Closed Loop “knot”
➢ Clustering of SB loops Figure 2-28-13
➢ Blocked at two ends
➢ Very distended
• Mesentery:
➢ Bunching of engorged vessels
• SBO above CLO
➢ Less distended
• Ascites
Single Discordance of Simple SBO

Double Discordance of CLO - SBO
SB CLO Ischemic & Hemorrhagic [Figure 2-28-14]

• Bowel wall changes
➢ Thickening,
➢ High attenuation I-, It is critical to
➢ Target sign, diagnose Closed
• Abnormalities in attached mesentery, Loop SB Obstruction.
• Absence of findings of ischemia or infarction in CLO With early CLO SBO,
does not rule out strangulation fluid will fill the lumen
Balthazar et al Closed-loop & strangulating intestinal of the closed loop.
obstruction: CT signs. Radiology. 1992 Dec Then the fluid heavy
loop will spin slightly
CLO SBO Problems in Dx around the vessels.
• Knot may be elongated This compromises venous outflow engorging
• Mesenteric engorgement the wall. Then the elevated hydrostatic
➢ Knot angle on slice may obscure pressure forces more fluid into the lumen
• Obst loops prox to CLO, and decomp loops distal may distending it to the maximum. Additional serum,
mingle with CLO plasma,and eventually blood may ooze into the
• Ascites may mask mes changes wall. This increases the weight of the loop and
• “BOW TIE” CLO SBO it tends to twist further. This progressive
• High Suspicion on all SBOs: twisting begins compromising arterial inflow.
➢ Diameter CLO big & uniform Rapid bowel infarction may then occur. This
➢ Criss Crossing: Vessels / SB may all happen before the attending radiologist
➢ Thick walls comes in the next morning
➢ No oral contrast gets in
Figure 2-28-14
A non-contrast CT shows high density in the wall of distended

loops of SB indicating intramural hemorrhage is occuring

CLO SBO in Camouflage [Figure 2-28-15] Figure 2-28-15
CLO SBO Dead Bowel
Criss-Crossing Vessels / Bowel

[Figure 2-28-16]
Bow Tie CLO SBO [Figure 2-28-17]
Gastric Bypass [Figure 2-28-18]
Gastric Bypass Simple

Obstruction
Gastric Bypass Closed Loop

Obstruction
CLO: Stomach to Roux [Figures 2-28-19

and 2-28-20]
Sandrasegaran K, Maglinte DD, et al CT of
acute bilio-pancreatic limb obst. AJR 2006 CLO SBO may be obscured by location of the “knot”, by small
Jan;186(1):104-9 or large size of knot, by the angle of the “knot” relative to the
Scheirey C, Scholz F, et al. Radiology Lap slice, by ascites obscuring the mesenteric bunching, and by
Roux-Y Gastric Bypass: Conceptualization intertwining of other loops of decompressed distal SB and
and Precise Interpretation Radiographics in simply-obstructed proximal SB among the loops of the CLO
press Sept 2006 SBO complex
Figure 2-28-16
Note that on sequential sections there is an abrupt

crisscrossing of bowel and vessels. This finding is suggestive
of entrapment of mesentery by adhesions or internal hernia

Obst post Roux Figure 2-28-17
Paraduodenal Hernia
• 50% IH = Paraduodenal
• Mortality pre-CT era (20%)
• Clinically:
➢ Asymptomatic,
➢ Pain,
➢ SBO,
• Left 3X > R; M > F
• SB entrapment =
➢ Congenital anomaly
J Comp. Assist. Tomo. 10:542, 1986
Figure 2-28-18
Bilio
Pancreatic Alimentary
Limb Limb
The “Bow Tie” CLO SBO

occurs when two or
more loops are involved
in the closed loop.
There will be the simple
SBO above the first
Common Limb to Cecum
closed loop. There will
Gastric bypass for morbid obesity offers many be two or more CLOs
opportunities for SBO and one unique situation for with each having uniform
CLO SBO but differing degrees of distension. CLO 1 in the
upper abdomen is shorter in length, more
distended, and shows thin perfusing walls. CLO2
Figure 2-28-19 involves a more distal longer limb with thicker
walls
Closed Loop SBO obstruction

may occur in Gastric Bypass
patients when the bypassed
Bilio-Pancreatic Limb obstructs
at the Roux Y anastomosis.
The Stomach has been stabled
shut so a closed loop
obstruction is created. While
there is not the risk of a
twisting of vessels, this
obstruction puts high pressure
on the closed gastric stump
staple line

Ascending Meso-colic H [Figure 2-28-21] Figure 2-28-20
• “Right PDH”
• Ascending Mesocolic H
• Absent lig. Treitz
• Normal Cecum
• Transverse colon not displaced caudally
• CT: Ascending Colon vessels anterior to
SB loops
Desc Meso-colon H
• “Left PDH”
• Descending mesocolic H
• Ligament of Treitz OK
• Cecum OK
• Stomach displaced to right
• Neck contains IMVein & Left Colic Art.
displaced anteriorly by hernia
• CT: IMV ant to SB loops
Treatment with percutaneous draininage will allow elective
surgery if the obstruction does not resolve
Desc Meso-colon Hernia
[Figure 2-28-22]
• 1. Ligament of Treitz OK Figure 2-28-21
• 2. IMV in front of SB
• 3. SMA, V’s into hernia
• 4. Bunched SB possible
• If you want to diagnose a Left PDH, look for the IMV
Figure 2-28-22
Right and left meso-colic hernias, or “PDHs”, can

be defined by the relationship of the right and left
colic vessels to the proximal small bowel.
Normally they pass behind the upper SB. Note the
anterior course of the right colic vessels anterior to
the SB (arrows) in a right “PDH”). Note the IMV (
(large pointers) in its normal position behind the
SB on the left
A Left PDH or Ascending Meso-colic hernia with

the Inferior Mesenteric Vein coursing anterior to
the entrapped proximal small bowel

Truth about “PDH”s [Figure 2-28-23] Figure 2-28-23
• PDH: Bad name.
• “Retro-mesocolic congenital hernia”
➢ Behind ascending, transverse, or descending mesocolon
(and colon).
➢ Space usually obliterated as embryo
➢ Mesocolon fuses to parietal peritoneum.
➢ Rotation and fixation anomaly
❖ SB does not get out of the way
❖ SB prevents fusion
Foramen of Winslow
• “Epiploic Foramen”
• SB above antrum
ascending
Cecal Volvulus into lesser sac
• SBO
➢ Potential in Cecal Volvulus
➢ SB follows IC Valve
Pre op DX: Infarcting Internal Hernia

• Assoc w prior bowel surg ( Clips )
• See
➢ Mesenteric:
❖ Bunching
❖ Engorgement transverse
❖ Twisting
➢ Criss-crossed vessels
SB Obstruction
• Common disease.
• Mechanical: Acute, Chronic, Internal, External
• Dysmotilities
• Critical
➢ Diagnose
➢ Stage
➢ Establish etiology
descending
SBO The Paraduodenal Hernia will
• Reviewed remain in our literature and our
➢ Mechanical Board Examinations even
❖ Classic Acute “Complete” SBO though they are not hernias but
Simple SBO errors of rotation in which the
Closed Loop Obstruction (CLO) SB is trapped behind the
Urgent Emergency !!!!!!!! returning meso-colon.
❖ Classic Appearances Because there is a wide arc of
Intermittent “Chronic” SBO returning colon, from the
Partial SBO ascending, the transverse,and
➢ Paralytic the descending colon, there will
❖ Common be variation in radiographic
❖ Unusual appearance depending on
where the SB is trapped.
Above one can see ascending,
transverse, and descending
retro-meso-colic entrapment

References
1. Balthazar et al Closed-loop & strangulating intestinal obstruction: CT signs. Radiology. 1992 Dec
2. Blondon H, et al Digestive smooth muscle mitochondrial myopathy in pts with mitochondrial-neuro-gastro-
intestinal encephalomyopathy (MNGIE), 3 cases & review of literature. Gastroenterologie 2005 Aug. 29N, 8-
9:773-778
3. Frager D, Medwid SW, et al . CT of SBO: Value in Establishing Diagnosis and Determining Degree and
Cause.AJR 1994;162: 37-41.
4. Ljiri R et al. Oburator H: usefulness of CT in DX.:Surg. 1996 Feb; 119(2): 137-40
5. Luedke, Scholz. Larsen CT of Spigelian H. Comp Med Imag Graph. 198812(2):123-9.
6. Maglinte, Herlinger, Nolan. Rad of CLO: 25 confirmed cases. Radiology. 1991 May;179(2):383-7 (Chronic)
7. Mayo-Smith WW, Wittenberg, et al. CT SB faeces sign: description & clinical significance. Clin Radiol. 1995
Nov;50(11):765-7.
8. Megibow A, Balthazar E, Cho K, et al. Bowel Obstruction: evaluation with CT. Rad 1991;180:313-318.
9. Passas V, Karavias D, Grilias D, Birbas A. Computed tomography of left paraduodenal hernia. J Comput Assist
Tomogr. 1986 May-Jun;10(3):542-3.
10. Sandrasegaran K, Maglinte DD, et al CT of acute bilio-pancreatic limb obst. AJR 2006 Jan;186(1):104-9
11. Scheirey C, Scholz F, et al. Radiology Lap Roux-Y Gastric Bypass: Conceptualization and Precise Interpretation
Radiographics in press Sept 2006.
12. Simon et al, Polyneuropathy, Ophthalmoplegia, Leukoencephalopathy, Pseudoobstruction: POLIP Syndrome; Ann
Neurol 1990;28:349-360
13. Zalcman et al Helical CT Signs in Dx of Intestinal Ischemia in SBO; AJR 2000;175:1601-1607
14. Zalcman M, et al Helical CT signs in Dx of intestinal ischemia in SBO. AJR 2000 Dec;175(6):1601-7.

Acute Mesenteric Ischemia
Bowel Ischemia
• Small Bowel or “Mesenteric” ischemia
➢ SMA distribution: SB and Right Colon
• ”Colonic Ischemia” - a different disease
➢ Watershed: Sigmoid, Splenic
• ESD: never: unless
➢ Surgery
➢ Radiation
➢ Vasculitis
The Radiology of Mesenteric Ischemia

• 1. Review classifications & pathophysiology
• 2. Rad Findings: Ischemia & Infarction
• 3. Clues to Etiology, emphasis on CT

3 categories
➢ Arterial Occlusive
❖ Without Reperfusion
❖ With Reperfusion
➢ Venous Occlusive
➢ Non-Occlusive Arterial - Low Flow
Pathophysiology of Ischemia
• Initial Damage to Endothelial cells of pre-capillary arteriole and capillaries.
• Blood vessels leak fluid, then cells
• Mucosa & submucosa
➢ Most sensitive, high metabolism
➢ Edema, hemorrhage, & slough
• Muscularis propia
➢ Initial spasm
➢ Then atonia
➢ Then perforation / death
(healing with stricture)
• Serosa
➢ Petechiae, Ascites
➢ With healing may see adhesions
Ischemia
• Rad Findings, Symptoms & Prognosis depend on:
➢ Duration
❖ Momentary to Permanent
➢ Degree
❖ 1%-100%
➢ Extent
❖ % of SB
Fast Ischemia
• Cell, Tissue, Organ & Organism death -24-48 H
➢ Eg. Embolus to SMA
➢ Eg. Hypotension: Profound & prolonged
Gastrointestinal Radiology 487 Mesenteric Ischemia

Slow or Minimal Ischemia
• Cellular & localized tissue death →
• Organ dysfunction
➢ Eg Radiation Enteritis
➢ Eg Scleroderma
➢ Eg Arteriosclerotic Abdominal Angina
Ischemia
• Chronic - recurrent - slow
• Acute - sudden - fast
• Often both
➢ Chronic for months then Acute
Wet vs Dry Ischemia

• Wet: Ischemia w arterial inflow p insult (“reperfusion”)
➢ SEE: Thickest wall, bleeding into wall, ascites.
➢ Eg: Venous occlusion, Transient hypotension, fleeting, partial embolism
• Dry: Ischemia w no arterial inflow. (“non-reperfused”)
➢ SEE: “Thinner” or normal wall, no / min ascites.
➢ Eg. Complete proximal SMA embolus, sudden thrombosis.
Chou C, CT Manifestations of Bowel Ischemia. AJR2002;178-87
Chou C, CT of SB ischemia. Abd Imaging 2004; 29:18-22 Figure 2-29-1
Wet vs Dry Ischemia : Personal experience

• Two extremes: Prune vs Plum
• Wet: Classic
➢ Radiologists overcall on CT
➢ Surgeons undercall at Surgery
• Dry: Puzzling SBO
➢ Radiologists undercall - miss completely
➢ Surgeons baffled by our stupidity
(SECRET: Study Mesenteric Vessels I+)
Most Specific Single Finding of Acute Mesenteric Absence of wall opacification is

Ischemia the most specific sign of wall
[Figure 2-29-1] ischemia. It is not sensitive in all
• No CT perfusion of bowel wall cases. Because of the length of
• 100% specific, 30%-50% sensitive the SB, small segments that are
• Diagnosis depends on not perfusing may not be
➢ History evident. In this image one
➢ Summation of findings segment is perfusing while
❖ Wall thickening another loop is not
❖ Mesenteric Edema
❖ Ascites

• WBC
• Elevated Lactic Acid
• History
➢ Suggestive History:
❖ Pain in excess of Physical Exam
➢ Risk Factors
High Risk Patients (Boley, Clark)

• Pt > 50 yrs with:
• Valvular or Atherosclerotic Ht Dis
• Longstanding CHF
• Arrythymia
• Hypovolemia or hypotension
• Dig or diuretic Rx
• Recent MI
Mesenteric Ischemia 488 Gastrointestinal Radiology

• Also: AAA w or wo repair Figure 2-29-2
• Also: Any Abd, Cardiac, Thoracic Surg
Dry Infarct [Figure 2-29-2]

• Tendency to:
➢ Thinner Wall
➢ Absent “Target”
➢ No intramural blood
➢ Ascites min /absent
➢ No perfusion
• Beware: “Ileus or SBO” in Sick Pt at high risk
Dry Infarct, Dead Bowel - CLO SBO
Spectrum: Ischemia to Infarction [Figure 2-29-3]

• Gasless abdomen
• Ileus
• Thick Folds
➢ Target - CT, US
➢ Stack of Coins - Films
• Loss of Folds in Unchanging Thick-walled Loop Four sequential images (A-D) of a patient with
• Focal ulcer infarction WITHOUT REPERFUSION. Thin walls
• Shaggy gas pattern without target sign or intramural blood. No ascitic
• Collar button ulcers fluid. Good example of a dry infarct with minimal
• Intramural fistulas or absent inflow of arterial blood. Dry infarcts may
• Intralum mucosal cast be due to a large central SMA embolus or smaller
• Mesenteric or portal vein gas embolic or thrombi with lack of adequate
• Intraperitoneal air collaterals to allow inflow. This form of ischemia
• Stricture and infarction is less common and is hard to
• Pseudodiverticulum diagnose unless mesenteric vessel contrast is
• Many findings possible in same pt studied carefully
Usually there is REPERFUSION or extensive
Remember the Law of Burps & Farts inflow via collaterals producing edema or
[Figure 2-29-4]
hemorrhage into the bowel wall and ascitic fluid
• Air rises & thins normal walls
(Also note engorged mesentery)
Figure 2-29-4
Figure 2-29-3
Image shows normal thin wall effaced by air

(arrows) while arrowheads show thick wall not
effaced by air. A loop with a target sign is also
noted (curved arrow).
The slightest amount of SB (or colon) air will rise
and thin wall of normal bowel. Walls thickened by
blood or tumor will not thin out. Understanding this
With reperfusion ischemia the wall thickens with fluid concept allows for easy detection of abnormal
and blood from leaking capillaries. Stack of coin bowel
appearance is due to fluid and blood in the valvulae
conniventes

The Law of Bowel Gas ll [Figure 2-29-5] Figure 2-29-5
• Air in lumen coalesces into one bubble.(unless
trapped in pneumatosis, blood, stool, or bezoar).
• Note dense blood layering
Regular Stack [Figure 2-29-6]

• Blood / Edema in Wall
• Suggests:
➢ Acute
➢ Recent
➢ Severe
• Not specific for ischemia
Irregular Stack [Figure 2-29-7]

• Blood / Edema in wall
• Suggests: Images show non-coalescent air bubble (small
➢ Chronicity arrow) suggesting either pneumatosis or air
➢ Recurrence trapped in viscous blood. There is a low density /
➢ Fibrosis high density fluid-fluid level (large arrow)
indicating bleeding into lumen
Loss of Folds [Figure 2-29-8]
Figure 2-29-7
Figure 2-29-6
An irregular stack of coins

suggests re-bleeding and
chronicity with fibrosis
Figure 2-29-8
A regular stack of coins with relatively
uniform appearance of folds is
suggestive of recent bleeding into
wall. A stack of coins appearance
can be due to blood or fluid. It is a
longitudinal image of cross sectional
“target sign”. Pts with coagulopathies
or with leaking capillaries due to
vasculitis will have a similar
appearance
Loss of folds in an unchanging loop is

an ominous sign for infarction. It
indicates extreme wall thickening and
loss of peristalsis

Thick Wall & Ulcers [Figure 2-29-9] Figure 2-29-9
Ischemic Pneumatosis
[Figure 2-29-10]
Intramural and
Intravenous Air [Figure 2-29-
11]
V Air & Lack of Wall

Perfusion [Figure 2-29-12]
CT Equivalent to stack of coins with arrows

pointing to collections of contrast in the wall
suggesting loss of mucosal integrity
Figure 2-29-11
Figure 2-29-10
Image A: Intramural and intravenous air (arrows),

evidence of a degree of bowel infarction. The
degree of infarction is difficult to determine and Two spot films(images A & B) from a water soluble
may not correlate with amount of air. A small contrast ileostomy enema shows multiple
focus of wall infarction may allow a large amount ulcerations filling with contrast or with air. The CT
of air to enter veins. Once air enters a vein it may scan slice (image C) shows pneumatosis
travel long distances. When SB obstruction is
present air will be under pressure and large Figure 2-29-12
amounts may enter veins through even tiny foci of
loss of mucosal integrity. With even the severest
mesenteric ischemia and infarction, the unaffected
stomach muscles will continue to peristalse fluid
and air into the SB creating SB distension
Look carefully for AIR in VESSELS

USE lung windows in Ischemic and in SBO cases
to look for venous air. Use narrow windows to
find subtle wall density changes indicating either
blood or lack of perfusion

Extreme Perfusion Variations [Figure 2-29-13]
Intrahepatic Portal Venous Air [Figure 2-29-14]

• Splanchnic air will go everywhere splanchnic. It doesn’t stay near its origin Figure 2-29-13
Figure 2-29-14
Loops show EXTREME variations in wall perfusion

from absent to HYPERperfusion. This pt with
infarction and perforation from vasculitis. Pt is
Air is seen in portal branches and within veins in being treated for hypotension and has dense
the otherwise normal stomach. (Arrow) Air in enhancement from “shock bowel” in unaffected
portal system may distribute anywhere within loops not involved with vasculitis
portal circulation by gravity. In prone position, air
in intra-splenic veins would likely be seen
Sloughed Mucosa / Serosa [Figure 2-29-15] Figure 2-29-15
Mucosal Cast [Figure 2-29-16]

• Inside Serosa
SBO Pearl [Figure 2-29-17]

• SBO makes isch / Infarxn look worse
• We overestimate Infarction
• Edema greater
• Air dissects great distances under pressure:
➢ Neck crepitus reported
• Pneumatosis not = Infarxn
Figure 2-29-16
The mucosa (long arrows) is seen sloughed in

lumen outlined by contrast reaching to smooth
as yet intact serosa (short arrows)
With infarction and diffuse loss of mucosal
integrity, there will be slough of mucosa. The
serosa is more resistant to ischemia (tough as
hot dog or sausage skins) and will be last to
perforate
Two CT images, A & B, show shaggy irregular

intramural air, a sign of a mucosal cast, a
sloughing of mucosa

Pneumatosis Intestinalis (PI) Figure 2-29-17
• Venous gas … not allow prediction of transmural infarction, …
observed with only partial … wall damage
• Outcome ..pneumatosis… depends … on underlying disease
➢ Wiesner W, et al . PI and portomesenteric venous gas in
intestinal ischemia: correlation of CT with severity of ischemia
and clinical outcome. Am J Roentgenol. 2001
Dec;177(6):1319-23.
• CT Dx of PI: Lactic Acid > 2.0 mmol/L at Dx assoc with > 80 %
mortality
➢ Hawn MT, et al Serum lactic acid determines outcomes of CT
Dx of pneumatosis of GI tract. Am Surg. 2004 Jan;70(1):19-
23;
• AIR GOES EVERYWHERE…LOOKS WORSE THAN IT IS
Ischemia Mimic: J Tube Jejunitis

• Rad Findings:
➢ Jejunal Edema
➢ Ascites
➢ Portal & SMV Gas
➢ Pneumatosis
➢ SB Necrosis
Two slices from a pt show a shaggy
• Clinical:
irregular mucosal cast (image A)
➢ RARE, 4 / 1460 pts - .021%
indicating mucosal slough (long
➢ J Tube Abd or ENT surg
arrow). Other loops of bowel have
➢ Post op feeding day 1
extensive pneumatosis (image B).
➢ Day 5 bloating, N & V
The length of bowel involved with
➢ Day 7 hypotension - death.
pneumatosis may greatly exceed
➢ “They suddenly crump”
amount of bowel that is infarcted
• CT Findings
because air travels long distance in
➢ Portal Venous Gas
veins and in areolar submucosal
➢ SMV Gas
tissue
➢ Pneumatosis
➢ Penrose, J Tubes
• OP:
➢ Normal Bowel, pink, no resection
Rare… but recognize early

• Carucci LR, Levine MS, Rubesin SE, Laufer I, Assad S, Herlinger H.
Evaluation of pts with jejunostomy tubes: imaging findings. Radiology. 2002
Apr;223(1):241-7
J Tube Jejunitis
• Schunn CD, Daly JM. SB necrosis associated w post-op jejunal tube feeding.
J Am Coll Surg. 1995 Apr;180(4):410-6.
• Lawlor DK, et al SB necrosis assoc w jejunal tube feeding. Can J Surg. 1998
Dec;41(6):459-62.
• Rai J, et al SB necrosis in assoc w jejunostomy tube feedings. Am Surg. 1996
Dec;62(12):1050-4.
• Munshi IA, et al.SB necrosis assoc with early post-op jejunal tube feeding in a
trauma pt.J Trauma. 2000 Jul;49(1):163-5.
• Schloerb PR, et al. Bowel necrosis caused by water in jejunal feeding. J
Parenter Enteral Nutr. 2004 Jan-Feb;28(1):27-9.
• Brenner DW, Schellhammer PF. Mortality assoc w feeding catheter
jejunostomy after radical cystectomy. Urology. 1987 Oct;30(4):337-40.
• Gaddy MC, et al . SB ischemia: consequence of feeding jejunostomy? South
Med J. 1986 Feb;79(2):180-2.
• Jorba R, et al. SB necrosis in assoc w early post-op enteral feeding after
pancreatic resection. Surgery. 2000 Jul;128(1):111-2.

“Luxury” Reperfusion Hyperemia [Figure 2-29-18] Figure 2-29-18
• Vessels lose auto-regulation
➢ Tiniest vessels in mucosa
❖ Dead, clotted,
❖ Mucosa non-perfused
➢ Bigger vessels
❖ Lose muscular tone
❖ Hyperemic bowel musculature
❖ Shunt to veins
Reperfusion Bleeding [Figure 2-29-19]
Ischemia may cause strictures [Figure 2-29-20]
Figure 2-29-19
Luxury
Reperfusion with
gray mucosa and
hyperemic
muscularis and
mesentery
Intra-luminal bleeding may be obscured or

overlooked if pt is given opaque contrast material.
This pt had intraluminal bleeding from diffuse
vasculitis of Degos Syndrome initially assumed to
be ingested contrast. The pt did not drink opaque Figure 2-29-20
material! Always look carefully at the density of
ascites. High density ascites indicates bleeding
into peritoneal cavity
A SB spot film (A) and a CT slice (B) show a

tapered stricture (arrows) and a long tubular
stricture (arrowheads). With recovery from a
severe ischemic insult, healing with permanent
stricturing can occur

Etiologies of Ischemia Figure 2-29-21
• Arterial Occlusive
➢ Emboli 40%-50%
➢ Atherosclerosis - Thrombosis 10%-20%
➢ Mechanical
❖ Closed Loop Obstruction
❖ Volvulus,
❖ Incarceration
❖ Avulsion
➢ Large and Small Vessel Vasculitides
• Venous Occlusive
• Arterial Non-Occlusive
Embolus [Figure 2-29-21]

• Atrial Fibrillation
• Valvular heart disease
• Sharp cut off
• Filling defect
Lateral aortogram shows a filling defect (arrow) in
Embolism [Figure 2-29-22] SMA, a sign of embolus. AP injection of SMA in
another pt. shows long filling defect of embolic clot
(two arrows)
Figure 2-29-22
White SMA (short

arrows) Then Gray SMA
(long arrows)
ANGIO shows cutoff and
clot.
Dead bowel at surgery
within 8 hrs of
symptoms.
Pt died 16 hrs after
symptoms

Straddle embolus vs. mural

thrombus extending into
SMA Reperfusion by
collaterals distally
Slow filling of an irregular faint SMA

(arrow) usually indicative of
atherosclerotic thrombotic disease
Figure 2-29-25
Figure 2-29-26
Non-opacification of the SMA proximally with contrast in SMA

distally indicating distal perfusion by collaterals, confirmed with
a coronal reconstruction
A small segment of distal SB shows stack of coins

appearance (arrows) on UGI series ( Image A).
An angiogram (Image B) shows tapered narrowing
of small peripheral branch of SMA (arrow). A
careful SB series or enteroclysis may be needed
to diagnose short segment disease caused by
segmental dis. in peripheral SMA branches

“Straddler”: Embolus Vs Clot [Figure 2-29-23] Figure 2-29-27
Thrombosis [Figures 2-29-24 and 2-29-25]
• Absent segment
• Slow filling distal vessel
• Large collaterals
• Reconstitution
• Vascular calcification
• Irregular lumen
SMA flow thru GD collaterals

• Thrombosis vs. Embolus appearance can be similar,
Hx AF or Ht Valve important
Peripheral focal lesion [Figure 2-29-26] A SB series (Image A) shows a stack of coins
appearance (within circle). An angiogram (Image
Ulcerating Plaque [Figure 2-29-27] B) shows a bulging segment of SMA (arrow), an
ulcerating plaque showering cholesterol emboli.
Dysplasia [Figure 2-29-28] (This could also be a mycotic aneurysm if patient
were septic.)
Becker Duodenal necrosis as presenting manifestation of
polyarteritis nodosa.
Clin Rheumatol. 2002 Aug;21(4):314-6 Chronic Radiation Enteritis
Degos Disease
• “Malignant atrophic papulosis”
• Porcelain-white, atrophic papules
➢ Peripheral erythema
➢ Telangiectases.
• Small vessel thromboses
• M3 X F, all ages
• Skin presentation
• May rarely remain dermal
• When systemic, 2-4 yr prognosis
Coskun B Benign Cutaneous Degos' Disease:
case report and review of literature. J Dermatol. 2004 Aug;31(8):666-70
Degos GI Path
• GI - Any portion, but SB predominant
• 60% Degos: GI perfs lead to death Figure 2-29-28
• Sub-serosal white or yellow plaque, transmural bowel
inflam., ulcers, hemorrhage, infarction
Etiologies of Ischemia
• Arterial Occlusive
➢ Atherosclerosis
➢ Embolus
• Venous Occlusive 5%-10%
➢ Proximal Obstruction
➢ Distal Disease
Etiology SMV Thrombosis

• Idiopathic 20%
• Recent Surgery, esp Colon An SMA angiogram (Image A) shows an irregular
• Hypercoagulable States abrupt tapered narrowing of SMA branch vessel
➢ Protein S, C defic, polycythemia, hematological (arrowhead), consistent with dysplasia. There is
• Cirrhosis also subtle corrugation of main trunk and small
• Portal Vein Thrombosis branches within circle. There is a clip (arrow)
• Pancreatic Inflamm / Neoplasm where right renal artery, resected previously for
• Pelvic Infectious Processes renal artery dysplasia, originated

Symptoms SMV Thrombosis [Figure 2-29-29] Figure 2-29-29
• Duration of symptoms
➢ 9.1 days, range 1-42 d
• Pain 84%, N & V 56%, Fever & Chills 56%
• Diarrhea 23%, Blood in Stools 23%
• Ischemia 21%
➢ Bowel Wall Thickening
➢ Mesenteric Congestion
• Mortality in 7%, (rapidly falling in MDCT era)
• (MDCT: Incr detection & Rx)
Warshauer DM, Lee JKT, Mauro MA, White GC; Superior
Mesenteric Vein Thrombosis w Radiologically Occult
Cause: Retrospective Study of 43 Cases;
AJR2001;177:837-841 Images A-C: CT images show thrombus (arrows)
in Portal Vein. Images D-F Images of mid
SB Infarction [Figure 2-29-30] abdomen show engorged mesenteric SB leaves
• Lymphoma of SB Mesentery and thickening of SB loops (arrows) consistent
• Nodes Compress Veins with ischemia. While wall and mesenteric
• Engorged Mesentery thickening may mimic Closed Loop SBO, absence
• Infarction / Slough of bunching, lack of bowel distension, and
preservation of transit distinguish the two.In
Etiologies of Ischemia absence of ongoing thrombotic coagulopathy,
• Arterial Occlusive venous thromboses often eventually resolve with
➢ Atherosclerosis or without therapy
➢ Embolus
• Venous Occlusive Figure 2-29-30
➢ Low Flow States
❖ Shock
❖ Steals
❖ Arterial Vasospasm
❖ SBO
Figure 2-29-31
Aortogram shows lush

perfusion and opacification of
even small peripheral
branches of upper abdomen
vessels with minimal Images A-C show bulky nodes in the
mesenteric vasculature SB mesentery (straight arrow). The
apparent. There is no IMA mesentery is engorged (arrowhead)
visible. The main SMA trunk indicating compression of mesenteric
stops abruptly (arrowhead). vessels. Shaggy irregular intramural
An embolus is possible but air (curved arrows) indicates
absence of visible filling defect infarction with mucosal slough.
in SMA and extensive vascular Compression of SB veins may lead to
disease makes thrombosis venous engorgement and bowel
more likely infarction

Abdominal Angina, a Clinical Syndrome Figure 2-29-32
• 1. Pain following eating
• 2. Weight loss
• 3. Diarrhea, rapid transit
• “Classic”
➢ Occlusion of 2 of 3: Celiac, SMA, IMA
➢ May be 1 vessel occlusion, part others
➢ May be absent with full 3 vessel occlusion
• Vasculitis, Radiation, Median Arcuate Ligament
Syndrome, Steal Syndromes, CA Pancreas
Vasculopath “Blood Thievery”[Figure 2-29-31]

• Lush vasculature in upper abdomen
• Abrupt SMA end.
• Paucity lower abdomen
Vasculopath Collateral Steal from SMA

[Figure 2-29-32]
• See steal from SMA to SMA, IMA and beyond
• Reflex Arterial Vasospasm in SMA
Three images from SMA injection (A-C) show
Abdominal Angina: Median Arcuate abrupt termination (arrow) and collateral flow filling
Ligament an enlarged marginal arcade vessel (arrowheads)
[Figure 2-29-33] and filling of IMA branches (curved arrow).
• Median Arcuate Ligament of diaphragm Chronic steal syndromes in vasculopaths have
• Compression / fibrosis of Celiac Artery (occ SMA too) variations in amount stolen depending on varying
• Collateral Steal from SMA demand. Walking may deplete visceral flow and
produce Reflex Arterial Vasopasm and abdominal
angina
Figure 2-29-33
Figure 2-29-34
Image A in a pt with chronic intermittent abdominal pain shows

a stack of coins appearance to the jejunum. Image B and a
detail from it, image C, shows a short segment narrowing of
Celiac Axis with a normal SMA just caudal to it. The
appearance allows diagnosis of median arcuate ligament
syndrome. The median arcuate ligament of diaphragm may
compress Celiac Axis. This forces a physiologic steal from The AP projection during an SMA
SMA which may be asymptomatic when bowel is at rest. injection shows collateral filling of
Following eating, classic abdominal angina may occur because Celiac vessels and reflex mesenteric
steal creates a functional mesenteric ischemia vasoconstriction of mid and distal
branches of SMA, placing them at risk
Expiration / Inspiration of thrombosis. Celiac artery occlusion
may be cause of mesenteric angina,
Median Arcuate Ligament ischemia, or infarction due to collateral
Collateral Steal from SMA [Figure 2-29-34] steal
• Large collaterals
➢ SMA -> Celiac
• Reflex Mesenteric Vasoconstriction

Reflex Mesenteric Vasoconstriction Figure 2-29-35
Shock Bowel
• Dense persistent enhancement:
➢ Bowel Wall, solid organs
• Delayed pyelogram
• Small aorta, IVC, Spleen
• Ascites
• Variable Distension - Wall Thickness
• Periportal extravasation of fluid
• Major trauma with
➢ Resuscitation
➢ Volume repletion
• 2° to Reflex vasoconstriction
Mirvis SE, et al Diffuse SB ischemia in hypotensive
adults after blunt trauma (shock bowel): CT findings &
clinical significance. AJR Am J Roentgenol. 1994
Dec;163(6):1375-9
Shock Bowel
• May develop:
➢ Ischemia
➢ Infarction
• SB = Low Flow State usually caught in time CT images (A-F) show wall thickening with fluid
density in pt with angioedema, a process where
Ischemia Mimic: Angioedema capillaries leak serum. It may be due to allergies
[Figure 2-29-35] to food, drugs, or other exogenous allergens. A
• Enhancement of mucosa hereditary form occurs without specific causation.
• Submucosa edema Angiotensin Converting Enzyme inhibitor drugs
• Fluid in lumen may produce this finding alone or in association
• Ascites with glottic or generalized edema. It may be dose
• Etio related or seen with certain ACE inhibitors. Those
➢ Allergic reaction, with bowel angioedema from ACE inhibitors may
➢ Hereditary, present with a rad and clin picture suggesting
➢ ACE inhibitors. mesenteric ischemia. Pts on hypertensive or
cardiac medications should be questioned about
DeBacker AI, et al; CT of Angioedema of the Small
antihypertensive medication to exclude this as an
Bowel, AJR 2001; 176: 649-52
etiology. Cessation of offending ACE inhibitor may
3 cases: 3 different etiologies
provide relief and rad return to normal within 24 to
1 case report of 1 pt
48 hrs
1 NEJM Images in Clinical Medicine
Ischemia Mimic: Angioedema

Serum Leak from Capillaries
• ACE Inhibitors
➢ Accupril
➢ Aceon
➢ Altace
➢ Capoten
➢ Captopril
➢ Lisinopril
➢ Lotensin
➢ Mavik
➢ Monopril
➢ Prinivil
➢ Univasc
➢ Vasotec
➢ Zestril

Ischemia Mimic: ACE I A E Figure 2-29-36
• 7 Female 1 Male
• Ascites > Bowel Change
• Preserved Transit
• ACE I stopped:
➢ < 24 Hr resolution
• Bowel changes mild enough to wait / watch, avoid surgery!!
• Stiff Arcs in 6 of 8 pts
Lahey experience
Ischemia Mimic: Stiff Arc Sign

• Serum leaks from capillaries
• Intact
➢ Arteries
➢ Capillaries
➢ Veins
➢ Blood Flow
➢ Oxygenation Note thumbprinting of colon and
• Wall stiffest per degree of thickness stack of coins appearance of SB.
• Long Arcs of erect SB possible Chronic high intra-luminal pressure
will affect the hemodynamics of
Ischemia Mimic: Edema Post Obstructive perfusion. Submucosal edema will
occur with severe obstruction. When
9 Days Earlier: SBO & LBO Diverticulitis obstruction is relieved, the edema
and altered perfusion dynamics may
Ischemia Mimic: Obstructive & Post Obst Edema persist, and the edema may become
[Figure 2-29-36] more prominent immediately after
• Drop in high IL pressure relief of obstruction
• Local Arteriolar Hypertension &
• Increase Vasc Permeability =
• Tissue Edema
Figure 2-29-37
Ischemia Mimic: Hemorrhage vs Ischemia [Figure 2-29-37]
• Some CT features overlap: target sign, hemoperitoneum
• Intramural Hemorrhage:
➢ Short segment < 15 cm
➢ Wall thicker = or > 1 cm
• Ischemia:
• Long > 30 cm
➢ Wall less thick < 1 cm
• 15 -30 cm overlap
Macari M, et al Intestinal ischemia versus intramural hemorrhage: CT
evaluation. AJR. 2003 Jan;180(1):177-84
Ischemia Mimic: ITP

• Immune Thrombocytopenic Purpura
• Bowel Hemorrhage
• Note: post splenectomy
• Ascites
• 2 groups: Age 2-4, Adult
• Causes
➢ Idiopathic A regular stack of coins with relatively
➢ Drug Induced uniform appearance of folds is
➢ SLE suggestive of recent bleeding into
➢ Infection wall. A stack of coins appearance
➢ Pregnancy can be due to blood or fluid. Pts with
• Rx coagulopathies or with leaking
➢ Immune Suppression capillaries due to vasculitis will have a
➢ Splenectomy similar appearance

Figure 2-29-38
Purpuras are a group of diseases that weep small

amounts of blood from many tiny vessels.
Henoch Schonlein is transient, often recurring
immune mediated vasculitis, usually affecting
children, can be seen in adults. Usually palpably
raised itchy red lesions (Arrows Image A) are
present and allow a diagnosis. Petechiae (arrows
in image B of ileal endoscopy) and purpuric
lesions also occur in bowel. Abd involvement is
seen in 50-75% of pts who present with dramatic
Figure 2-29-39 colicky abd pain and bleeding, which may be
massive in 1-2% of patients. Bleeding into wall of
bowel thickens it (Image C arrows) and gives a
stack of coins appearance (Image D arrows). This
intramural bleeding may cause obstruction, GI
bleeding, infarction, perforation, or intussusception
in distal SB. While no effective therapy, pts must
be monitored for complications until attack
subsides
The descriptive term

“target sign” has been
applied to a loop of bowel
with distinct demarcation
of circular layers of the
wall. It is seen whenever
fluid of some type, or fat,
or air enter submucosal
space between mucosa - muscularis mucosa and
muscularis propria-serosa.
Image A innermost gray is fluid in lumen. Then
there is a white line representing contrast opacified
mucosa and muscularis mucosa. Then there is a
gray circle which represents fluid in submucosa.
The outermost white ring represents contrast
opacified muscularis propria and serosa
Image B, the innermost white dot is ingested
opaque contrast material. Then a less opaque ring
represents mucosa and mucosal muscle. There is
a low density ring which is negative in Hounsfield
units indicating fat. The outermost layer is
muscularis propria-serosa. Fat prominence is seen
in pts with Crohns disease and for unknown
reasons may be seen in occasional normal pts in
the distal Ileum, possibly due to prior infectious
gastroenteritis

Ischemia Mimic: Henoch Schonlein Purpura
[Figure 2-29-38]
Target sign [Figure 2-29-39]

• Blood, Serum, Plasma, Interstitial Fluid, Fat, Air
• Ischemia
• Vasculitis
• Intramural Hemorrhage
• Crohns: edema (or fat)
• Angioedema
• Portal Hypertension
• NSAIDs Enteritis
• ANY ENTERITIS
➢ Chemo, Rad, Infect. etc
Bowel Damage Pathways

• Loss of Barrier Integrity
➢ Vascular Barrier
❖ Leak of serum, plasma, cells
❖ Edema
❖ Ischemia
❖ Loss of mucosal barrier
➢ Mucosal Barrier
❖ Inflow of excluded molecules
❖ Edema
❖ Loss of vascular barrier
❖ Vascular compromise
❖ Ischemia
Ischemia CT Mimics
• Vascular or Mucosal Barrier Interruption
➢ Ischemia
➢ Vasculitides HSP
➢ Coagulopathies
❖ Bleeders - Purpuras, anticoags
❖ Clotters - P Vera, S,C defic
➢ Angioedema - ACE inhibitors
➢ Regional Inflammation - tic appy itis
➢ Crohns
➢ Infectious Enteritis
➢ Neutropenic Enterocolitis
Mesenteric Ischemia
• Diagnosis
➢ Imperative in Acute & Chronic Ischemias
➢ Now earlier Dx by CT - study vessels
➢ Think of it in every abd pain CT.
• Physiological understanding is critical
• Remember Steals
• Surgeons undercall some, be brave, stay bold
• We undercall some, explain plums & prunes
References
General References
1. Becker Duodenal necrosis as presenting manifestation of polyarteritis nodosa. Clin Rheumatol. 2002 Aug;
21(4):314-6.
2. Carucci LR, Levine MS, Rubesin SE, Laufer I, Assad S, Herlinger H. Evaluation of pts with jejunostomy tubes:
imaging findings. Radiology. 2002 Apr; 223(1): 241-7.
3. Coskun B. Benign Cutaneous Degos' Disease: case report and review of literature. J Dermatol. 2004
Aug;31(8):666-70

4. DeBacker AI, et al; CT of Angioedema of the Small Bowel, AJR 2001; 176: 649-52
5. Mirvis SE, et al Diffuse SB ischemia in hypotensive adults after blunt trauma (shock bowel): CT findings &
clinical significance. AJR Am J Roentgenol. 1994 Dec; 163(6):1375-9.
6. Warshauer DM, Lee JKT, Mauro MA, White GC; Superior Mesenteric Vein Thrombosis w Radiologically Occult
Cause: Retrospective Study of 43 Cases; AJR 2001;177:837-841
Wet vs Dry Ischemia

1. Chou C, CT Manifestations of Bowel Ischemia. AJR2002;178-87
2. Chou C, CT of SB ischemia. Abd Imaging 2004; 29:18-22
Pneumatosis Intestinalis (PI)

1. Hawn MT, et al Serum lactic acid determines outcomes of CT Dx of pneumatosis of GI tract. Am Surg. 2004
Jan;70(1):19-23;
2. Wiesner W, et al . PI and portomesenteric venous gas in intestinal ischemia: correlation of CT with severity of
ischemia and clinical outcome. Am J Roentgenol. 2001 Dec;177(6):1319-23.
J Tube Jejunitis
1. Brenner DW, Schellhammer PF. Mortality assoc w feeding catheter jejunostomy after radical cystectomy. Urology.
1987 Oct;30(4):337-40.
2. Gaddy MC et al. SB ischemia: consequence of feeding jejunostomy? South Med J. 1986 Feb; 79(2):180-2.
3. Jorba R, et al. SB necrosis in assoc w early post-op enteral feeding after pancreatic resection. Surgery. 2000
Jul;128(1):111-2.
4. Lawlor DK, et al SB necrosis assoc w jejunal tube feeding. Can J Surg. 1998 Dec; 41(6):459-62.
5. Munshi IA, et al.SB necrosis assoc with early post-op jejunal tube feeding in a trauma pt.J Trauma. 2000 Jul;
49(1):163-5.
6. Rai J, et al SB necrosis in assoc w jejunostomy tube feedings. Am Surg. 1996 Dec; 62(12):1050-4.
7. Schloerb PR, et al. Bowel necrosis caused by water in jejunal feeding. J Parenter Enteral Nutr. 2004 Jan-
Feb;28(1):27-9.
8. Schunn CD, Daly JM. SB necrosis associated w post-op jejunal tube feeding. J Am Coll Surg. 1995 Apr;
180(4):410-6.

Malabsorption
The Radiology of Malabsorption (MAB)

• Review
➢ Celiac Disease “Sprue” in detail
➢ “MAB Pattern” Barium & CT
➢ Other Diseases of MAB
➢ CT Detection of MAB
Images from a Virtual Colonoscopy

75 F Asymptomatic
Celiac Disease
• Vessel cloaking nodes, fluid in pelvic SB loops
1991: Sprue Presentations

• Diarrhea 85%
• Weight loss 57%
• Abd distress 29%
• Edema 29%
• Bone pain 19%
• Tetany 10%
• Failure to grow, hematuria, foot drop, hypovolemic shock, each 2%
Trier J, Celiac Sprue NEJM 1991
2005: Sprue Presentations

• 50% of adult pts present w Fe Defic Anemia
➢ Farrell RJ, Kelly CP. Celiac Sprue.N Engl J Med. 2002 Jan 17;346(3):180-
8
• “Occult GI Bleeding (FOBT+)…detected in half of pts with Sprue”
➢ Fine KD, Prevalence of occult GI bleeding in Celiac Sprue, NEJM 1996
334:1163-7
Back & Leg Pain

• -> Primary Care MD ->
• Neurologist - back pelvis films ->
• Radiologist - “Osteomalacia” ->
➢ GI series ->
• Gastroenterologist: Biopsy: Sprue
The Physiologist’s MAB

• Maldigestion (no enzymes, no mixing)
➢ Biliary - panc insuff, ZE, bacterial overgrowth, SB diverticulosis “Luminal”
• Cellular MAB (Columnar Cell uptake failure)
➢ Sprue, ischemia, villous tip infiltration
• Malassimilation (Columnar Cell exit failure)
➢ lymphangiectasia, abetalipoproteinemia, mesenteric diseases “Mesenteric”
The Radiologist’s MAB: “Malabsorption Pattern”

• Dilution from XS intraluminal fluid
➢ Acute & Chronic Diseases
• Dilatation
• Delay
• “MP” → to enteric fluid overload, greater chronicity: > Dilatation, Delay
Gastrointestinal Radiology 505 Malabsorption

“Malabsorption Pattern” (MP) Figure 2-30-1
• “MP” = Dilution + Dilatation + Delay.
➢ Due to chronic enteric fluid overload
• Historically, radiologic MAB Pattern = Sprue
• Sprue is king of MAB pattern BUT
• Other diseases can cause MAB pattern
• Not all Sprue pts have “MAB pattern”
Sprue: Gold Standard Dx

• SB Biopsy
• Antiendomysial antibody (“EMA”) Image shows two jejunal biopsies, of similar
➢ IgA Ab to extracellular reticular fibers magnification, contrasting Celiac Sprue at top with
➢ 90% sensitive, 98% specific a normal biopsy at bottom. With Celiac Sprue
• Tissue Transglutaminase antibody (tTGab) there is loss of normal fingerlike villi (arrows) seen
➢ 86% sensitive; 84% specific below and Crypt Hyperplasia (compare thickness
• AntiGliadin IGA antibody of double arrowheads)
➢ 76% sensitive, 79% specific
Johnston SD, et al A comparison of antibodies to tissue transglutaminase with
Figure 2-30-2
conventional serological tests in the diagnosis of coeliac disease. Eur J
Gastroenterol Hepatol. 2003 Sep;15(9):1001-4.
Villous Atrophy & Crypt Hyperplasia

Normal Villi & Crypts [Figure 2-30-1]
Entero-Enteric Circulation
• Crypts secrete fluid into lumen
• Villi absorb fluid + nutrients from lumen
• Nutrients into portal veins, lymphatics
• Crypts recycle fluid back into lumen
Sprue – Pathophysiologic Sequence

• Mucosal Villous Atrophy + Crypt hypertrophy →
• Chronic Fluid Overload →
• Dilatation (SB Muscle exhausted- “CGF”) →
• Delay in Transit –>
➢ Increases Malabsorption
❖ Bacterial overgrowth
❖ Other Degradations The “Malabsorption Pattern” is
characterized by Dilution evident with
Sprue 1° Rad findings l [Figure 2-30-2] watery low density of barium (arrow)
• Dilution WET !! caused by fluid mixing with it,
• Dilatation WIDE !! Dilatation evident by wide diameter
• Delay in transit WAY LATE !! (double arrow head) and Delay
• Segmentation evident by a 7 hr marker without any
• Folds: normal –> nodular –> flat barium reaching colon
• MALABSORPTION PATTERN Figure 2-30-3
Jejunal Peristalsis
“Feathery Fishtails”
Ileal Peristalsis
“Esophageal”
[Figure 2-30-3]
The MAB pattern results in part from loss of normal peristalsis. Long
arrow in A is normal feathery or fish-tail appearance of jejunal
peristalsis. Short arrows in B show normal contractile pattern of
Ileum with parallel folds in tapered segments mimicking esophageal
contraction
Malabsorption 506 Gastrointestinal Radiology

Look at the difference in “tone”: diameter and peristalsis
[Figure 2-30-4]
Sprue 1° Rad Findings ll

• Proximal SB mucosal villous atrophy
➢ reversal of jejuno-ileal fold pattern
➢ toothpaste jejunum (moulage, < 4 folds/inch)
➢ “jejunization” of ileum (increased ileal folds)
➢ flattened bald duodenal mucosa
➢ foamy mucosal pattern “mosaic”
• Intussusceptions, momentary + non-obstructing (loss of wall thickness AND
tone allow for loops to slide in and out.)
Toothpaste – Reversal [Figure 2-30-5
Foamy, Thick, Bald [Figure 2-30-6] Figure 2-30-4

• The Jejunum looks like Ileum, the Ileum looks like
Jejunum, the Duodenum looks like hell
The MAB pattern is evident in A with dilution giving

Figure 2-30-5 gray watery barium ( short arrow) and dilated
loops(double arrowheads) with minimal peristaltic
events. The bowel looks baggy, flabby, like
chronically stretched tube socks. Contrast A with
a normal SB film in B. Numerous peristaltic events
(arrows) are apparent and there is a state of tonic
contraction allowing for visualization of the
mucosal detail
Figure 2-30-6
Image A shows a smooth fold-free segment of the
jejunum, called “toothpaste” or “moulage” caused
by atrophy of mucosal villi and thickening of the
wall by crypt hyperplasia.
Image B shows a bald jejunum in the LUQ and a
feathery abundant fold pattern in the ileum RLQ.
Chronic increase in the nutrient mix presented to
the ileum because of lack of jejunal absorption
cause compensatory hypertrophy of ileal mucosa,
hence “Reversal of Fold Pattern”
Image A shows a nodular lacy mucosal pattern

(arrow) in the duodenal bulb. This is due to
atrophy of the mucosa allowing the normal
submucosal glands to become apparent

Mosaic Pattern [Figure 2-30-7] Figure 2-30-7
Mucosal Atrophy [Figure 2-30-8]

• Fissures
• Pits
• Acid burns thinned mucosa
Acid Burns, Ulcerates, Strictures [Figure 2-30-9]
Ulcer [Figure 2-30-10]

• “Occult GI Bleeding (FOBT+)…detected in half of pts
with Sprue”
Fine KD, Prevalence of occult GI bleeding in celiac
sprue, NEJM 1996 334:1163-7
Jejunal Webs [Figure 2-30-11]

Mucosal and fold atrophy may create a lacy
Figure 2-30-8 granular or “mosaic” pattern in jejunum seen only
with double contrast or mucosal detail
compression images
Figure 2-30-9
Areas of narrowing ( arrows) may be

seen in the proximal jejunum due to
inflammation or scarring
Mucosal atrophy leaves the wall

susceptible to inflammation with
cracks, fissures (arrows A) , ulcers, Figure 2-30-10
and pitting from chronic inflammation
(Arrows B)
Figure 2-30-11
Ulcerations may heal with stricture formation
Recurrent ulceration and healing may lead to

multiple short segment web-like strictures (arrows)

Classic MAB Pattern [Figure 2-30-12]
Intussusception [Figure 2-30-13]

Figure 2-30-12
CT in Sprue
• SB
➢ Fluid filled pelvic SB
➢ Dilated, Non distended SB - BAGGY
➢ Dilution if O = Iodine
➢ Flocculation if O = BA
➢ Intussusceptions
➢ Fragmentation
• Colon
➢ Big, Gassy, Wet
➢ Foamy Feces (Stool Whip)
• Nodes, incr number
• Loss of body fat
• Small Spleen
• Fatty Liver Classic MAB pattern. Dilution is evident with watery
appearing barium (curved arrows). Dilatation is seen
Classic Appearance of SB (double arrowheads). Puddles of isolated barium
Intussusception [Figure 2-30-14] evident (red dots) which, if large, are called
“segmentation”, if small, “flocculation”. Fluid and poor
peristaltic activity in MAB causes segmentation and
flocculation. Image A (Arrowhead): A worm or
threadlike collection of barium is caused by a small
amount of barium settling out in a fluid filled length of
bowel that has not had enough peristalsis to keep the
barium and water mixed
Figure 2-30-13
Figure 2-30-14
Intussusceptions are usually transient with dilated

flaccid thin walled loops of bowel sliding easily into,
and out of, each other. Because the muscle is
weak and stretched out in Celiac Sprue, it cannot
pull the intussusception deeper. With normal
bowel grabbing a lead point, peristaltic muscle
contraction pulls it further and tighter until it is CT of Lower abdomen shows classic appearance
wedged of SB intussusception. The classic dotted crescent
of fat(arrow) represents mesenteric fat and
mesenteric vessels pulled in with the
intussuscepting loop

Dilated non-distented SB, Fluid filled distal SB loops, colon
Slow transit of contrast, Fluid filling Colon [Figures 2-30-15 and 2-30-16] Figure 2-30-15
Figure 2-30-16
Image A shows fluid and gas in the right and left

colon (arrows). Image B show fluid filled mildly
dilated loops of SB in pelvis (arrow). Both show
minimal body fat. The fluid absorbing ability of
colon is preserved in Celiac Sprue. With severe
chronic malabsorption, the volume of fluid
delivered to colon may overwhelm its ability to dry
out wet foamy stool. The colon in severe cases
will be fluid filled and pt will have prominent
diarrhea
Large amount of stool (arrow), lack of body fat in
subcutaneous tissues (curved arrow) and in
peritoneal cavity, and dilated flaccid appearing SB
loops are consistent with MAB. Sprue should be
suggested if the history is appropriate
Reactive Lymphadenopathy Figure 2-30-17

[Figure 2-30-17]
Nutritional Collapse
• Hypoproteinemia
➢ Hypoalbulminemia
➢ Ascites
• Vitamin deficiencies
➢ K (Coagulation defects)
• Iron Deficiency Anemia
➢ Jejunum absorbs Fe
➢ Slowwww bleeding
• Electolyte Disturbances
➢ Tetany
➢ Seizures Multiple small and moderate sized lymph nodes
are seen in the SB mesentery (arrows)
Sprue = “Immune Disease” surrounding mesenteric vessels. They are
• Lymphatic activity reactive lymph nodes chronically stimulated by the
➢ Reactive mesenteric nodes low grade SB autoimmune inflammatory process.
➢ Para-aortic LNs They are notable for number but rarely for size
➢ Large cavitating nodes = poor prognosis
➢ Peripheral lymphadenopathy
➢ Splenic atrophy & clinical hyposplenism
• Antibody Tests define “Autoimmunity”
“Genetic Disease”
• Unique histo-compatability complex in 80% of sprue HLA B8 , DR3
➢ (vs 20% of “normal” population)
➢ Increased prevalence of Sprue in families
➢ 10% latent Sprue in 1st order relatives

“Allergic Disease”
• Wheat, rye, barley
• Alpha-gliadin component of Gluten
• “Grass Allergy”
Sprue: Immune Sequence

• Genetic susceptibility
• ?Viral exposure → immune memory
• Gluten + endothelium → antigen
• Lymphocytes flood villous tips
• Antibodies destroy villi
Determinants of Severity
• Genetic “Dose” +
• Gluten “Dose” +
• Time +
• Other Factors
Adapted from Marsh, Gastroenterology 1992: 102:330-54
Marsh Biopsy Categories

• 0. Normal (Latent)
• 1. Intraepithelial lymphocytes increased
• 2. Infiltration with lymphocytes
• 3. Early villous atropy
• 4. Severe villous atrophy and crypt hyperplasia
Marsh M N, Gluten, major histocompatibility complex, and SB. A molecular and
immunobiologic approach to spectrum of gluten sensitivity ('celiac sprue')
Gastroenterology 1992 Jan;102(1):330-54)
Sprue Associated Auto-immunities

• Skin
➢ Dermatitis Herpetiformis
• Pancreas
➢ Autoimmune Pancreatitis, Macroamylasemia
• Kidney
➢ IGA mesangial glomuleronephritis
• Insulin dependant diabetes
➢ 3 X increase incidence of Sprue
• Hair
➢ Alopecia areata
• MULTIPLE emerging associated auto-immunities
Dermatitis Herpetiformis
• Pruritic papulovesicular lesions
• IG A deposits - dermal-epidermal junction
• Goes away with gluten restriction
CD in High Risk Patients

• General Population 0.4%-2% 1%
• Superfamily DR 3 / 3 33 %
• 1° Relative of Celiac 4%-10%
• Trisomy 21, Turners,
Williams Synd 5%-10%
• Type 1 Diabetic 4%
• Autoimmune Thyroid 3%
• 1° Relative of Diabetic 2%
• IG A Deficiency 2%

Nodules !! In Celiac Sprue ?? [Figure 2-30-18] Figure 2-30-18
Healing Sprue
• Folds slowly return, first
• Diameter slowly shrinks
• May take 3-4 years
Sprue
• Occult Marsh 1
➢ Nl SB exam, Asympt,
➢ bx +, labs +
➢ Intraepithelial lymphocytes
➢ 1° relatives of sympt pts: 5%-15 %
• Nodular Marsh 2
➢ Sandy Nodules, irritable
• Classic MAB pattern Marsh 3, 4
➢ Wet, wide, way late
• Non-responsive
➢ Diet errors, misdiagnosis
➢ Recalcitrant 5%-20% - Bact Overgr; Panc Insuffic
➢ Lymphoma
Recalcitrant Sprue
• Responsive to initial Rx 8+yrs
• Loss of responsiveness
• Smoldering symptoms
• Thick folds
• “Ulcerative Jejunitis”
• High incidence Lymphoma
“Recalcitrant” “Relapsing” Sprue

• Misty Mesentery One phase of Sprue is an early
• Perivascular Cloaking transient phase in which lymphoid
infiltration occurs. This will produce
Lymphoma in Sprue prominent or even nodular folds in
• Loss of response to gluten restriction. SB, not typical appearance of Sprue.
• Rising Ig A, Sepsis. The stage is not often radiographed
• Increasing lymphadenopathy. and it may be a fleeting phase of this
• Thickened bowel loops. disease
• Mesenteric Perivascular Cloaking ???
• 1 yr lymphoma survival - 31 %, 5 yr - 11% Figure 2-30-19
➢ Survival improves every yr, better
CT Dx, Rx
• 3.4% malignancy in CD (Lymphoma,
CA Esophagus, other)
Think Physiologically
[Figures 2-30-19 and 2-30-20]
• Maldigestion (no enzymes, no mixing)
➢ Biliary - panc insuff, ZE, bacterial
overgrowth, SB diverticulosis
“Luminal”
• Cellular MAB (columnar C uptake
failure)
➢ Sprue, ischemia, villous tip
infiltration
• Malassimilation (columnar cell exit
failure) A patient with bloating, cramps, gas, indigestion, occasional
➢ Lymphangiectasia, diarrhea for yrs. CT Scan shows a big gassy colon, fluid filled
abetalipoproteinemia, mesenteric loops of pelvic small bowel, and baggy proximal SB. The
diseases “Mesenteric” diagnosis was made because of these findings and because of
obvious findings in upper slices evident in the first two images

Luminal (Lumenal) [Figure 2-30-21] Figure 2-30-20
• Pancreatic Insufficiency
College Freshman, Wt Loss,

Diarrhea
[Figure 2-30-22]
• Fluid
• Colon Gas
• Minimal Fat
Luminal MAB: Shwachman -

Diamond Syndrome [Figure 2-30-23]
• Exocrine Pancreatic Insufficiency
➢ 50% outgrow in adolescence
• Neutropenia
• Chronic Infections
• Myeloid Leukemia
• Metaphyseal Chondrodysplasia
➢ Dwarfism Pancreatic calcifications. Pancreatic insufficiency can cause
malabsorption. The patient may have no other symptoms other
than abdominal bloating, discomfort, and diarrrhea
Figure 2-30-21
Figure 2-30-23
The pancreas is absent in this pt with MAB due to

pancreatic insufficiency. Only fat is seen (arrows)
where pancreas should be
Figure 2-30-22
CT of upper abdomen shows no

glandular tissue with fat in the
pancreatic bed (arrows). Pt had been
diagnosed with Shwachman Diamond
previously and had stopped
replacement therapy at the start of
college
A patient with diarrhea shows fluid filled loops of

SB (arrow) in the pelvis indicating MAB and slow
transit

Luminal MAB: ZE Syndrome [Figures 2-30-24 and 2-30-25] Figure 2-30-24
• MAB X-Ray pattern 2° to
➢ Increased gastric fluid
➢ Decreased pH
➢ > Enzyme non-activation
➢ > Poor digestion
➢ > Hypermotility
➢ > Edema/hyperemic folds
• Clinical MAB variable
Figure 2-30-25
Patients with ZE will have

radiographic features of MAB. SB
series shows dilated loops (double
headed arrow) with dilution and with
some fragmentation (curved arrows).
The proximal SB and stomach shows
See dilution, dilatation from ZE syndrome fold thickening due to edema of folds(
arrows) from large volumes of acid
reaching the proximal SB
ZE
• Excess fluid Figure 2-30-26
• Thick folds
• Hyperemic mucosa
Luminal MAB: Gastric Surgery [Figure 2-30-26]

• MAB pattern 2° to
➢ Vagotomy
➢ Loss of pylorus –> bolus into SB
➢ > Poorly mixed food
➢ > Lack of acid digestion
➢ > Poor enzyme synchronization
• Clinical MAB not common 1yr p Surg
➢ Absorption occurs distally
➢ Pt changes eating patterns
Cellular Villous Dysfunction

• Sprue
• Cong / Acq Enzyme Deficiencies
➢ Sugar splitting enzymes (Lactase) With a Bilroth L, II,
• Bacterial / Viral Toxins gastrojejunostomy, or other surgery
➢ Crypts Hypersecrete to increase gastric emptying, the
➢ Capillaries Leak proximal SB will become dilated due
➢ Enterocytes Malfunction to surges of fluid and poor
➢ Lymphatic Congestion synchronization of the digestive
• Cellular Poisons - Drugs - Chemo - Rad Rx process. Absorption equilibrates
and occurs in distal SB and pts
usually become asymptomatic as
they modify their dietary habits

Malabsorption from Bacterial Overgrowth [Figure 2-30-27] Figure 2-30-27
• Motility Diseases, eg Scleroderma, Chronic Narcotics
• SB Diverticulosis
Weight loss and diarrhea [Figure 2-30-28]
Wt Loss, episodic Diarrhea 30 F, MD, Wife of

MD,
2 m in US from India for training [Figure 2-30-29]
Bacterial infections affect absorption in a number

of ways. Invasive bacteria may stun or destroy
absorptive endothelial cells, may impair small
capillary or lymphatic vessel drainage, compete
for nutrients, or degrade critical enzymes
Figure 2-30-28 MAB pattern is apparent with dilated loops (double
arrow in A) and diluted barium (arrows A & B).
Transit delay evident image B at 285 minutes.
Numerous diverticula (red dots) shelter bacteria
from peristaltic cleansing, allowing them to
multiply to such a degree that they degrade or
utilize nutrients and enzymes. Note how more
diverticula are apparent in image B, diverticula
may be difficult to assess, hiding amid dilated
loops. Pts with numerous large SB diverticula
may develop Megaloblastic anemia from B12 and
folate deficiency
Weight loss and diarrhea. There are dozens of large SB

diverticula not apparent. Easily overlooked unless you
very very very carefully scroll. The MAB pattern with
baggy SB loops, fluid levels, foamy feces should make
you think hard and look for subtle causes of MAB
Figure 2-30-29
Marcha MAB Tropical

SB Fluid - Dilution with FLOCCULATION !! [Figure 2-30-30] Figure 2-30-30
Tropical Sprue
• Villous and Crypt Atrophy
• Malabsorption
• Glossitis, wt loss, diarrhea, skin changes
• Folate & B12 deficiency prominent
➢ Rx folate, B12 improves partly
• Antibiotic Rx cures
• Relapses common in tropics
Westergaard H.Tropical Sprue Curr Treat Options
Gastroenterol. 2004 Feb;7(1):7-11.
Haghighi P, Wolf PL. Tropical Sprue and subclinical
enteropathy.. Crit Rev Clin Lab Sci. 1997 Aug; 34(4):
313-41.
Scleroderma [Figure 2-30-31]

• Scleroderma always Dilated Delayed Dry
• Scleroderma WET =
BACTERIAL OVERGROWTH Marcha MAB Tropical Fluid
Giardiasis - Campylobacter [Figure 2-30-32] Figure 2-30-31

Chemotherapy Enteritis
MAB: 2° Villus Blockage by

• Lymph cells (immune disease, lymphoma, Crohn
Disease)
• PMNs (infections)
• Eosinophils (eosinophilic gastroenteritis)
• Mast cells (mastocytosis)
• Macrophages (Whipples)
• Amyloid (amyloidosis)
“Giardia” on CT Req [Figure 2-30-33] In image A, typical changes of Scleroderma

involving SB are apparent with dilated loops,
Villus Dysfunction hidebound appearance and pseudo-sacculations.
• Engorged Veins & Lymphatics Scleroderma creates a motility disturbance without
• Blocked Arteries (ischemia) affecting absorption, creating a dry pattern without
• Paraplegic with diarrhea dilution. However, with severe Scleroderma and
• MAB pattern dysmotility, patients may have episodes of
• Villous atrophy on Bx bacterial overgrowth which then produce MAB as
• No response to gluten restricted diet seen in the pt in image B with dilution apparent
• Physical Exam -> NO LEG PULSES
Figure 2-30-32
Arterial Insufficiency [Figure 2-30-34]
Lymphangiectasia [Figure 2-30-35]
Malabsorption
• Radiologists may still be first on scene in pts with
Sprue & MAB diseases
• MAB major radiographic pattern
• Fluid is the hallmark of MAB pattern
➢ Increased production +/or
➢ Decreased absorption
Image A shows MAB associated with Giardiasis.

Image B is a patient with Mab from Campylobacter

Figure 2-30-33
Figure 2-30-34
The oral contrast type determines the appearance of the fluid

filled loops of pelvic SB. If barium is given, flocculation may be
seen. If water soluble oral contrast is given, dilution may be
seen. Acute and subacute infectious diseases may cause SB
fluid to increase but may not be chronic enough to cause
distention and delay. This patient has dilute water soluble oral Arterial insufficiency may
contrast in the colon. Clips from a lymph node biopsy are cause dysfunction of all cells
present of the small bowel creating
dysmotility and malabsorption.
Note dilatation (double arrow)
Figure 2-30-35 and dilution (curved arrow).
Usually these bowel
symptoms are accompanied
by abdominal pain leading to a
correct diagnosis. This
paraplegic patient had an
element of sensory
denervation which caused the
diagnosis of aortic thrombosis
(image B arrow) to be missed
initially
Lymphangiectasia may cause degrees of clinical

and radiographic MAB due to engorged
lymphatics and mucosal edema which will impede
absorption. Usually folds remain prominent

Sprue is King of Rad MAB
• Nodular Phase
➢ Rarely seen, ? short phase
• MAB Phase
➢ Commonest for Radiology, Classic
• Recalcitrant
➢ Dietary indiscretions, edema, nodes, MALIG Bacterial Overgrowth,
Pancreatic Insuffic
• Lymphoma
➢ Nodes, weight loss, loss of gluten response
References
1. Farrell RJ, Kelly CP. Celiac Sprue. N Engl J Med. 2002 Jan 17;346(3):180-8
2. Fine KD, Prevalence of occult GI bleeding in Celiac Sprue. NEJM. 1996 334: 1163-7
3. Haghighi P, Wolf PL. Tropical Sprue and subclinical enteropathy. Crit Rev Clin Lab Sci. 1997 Aug; 34(4): 313-41.
4. Johnston SD et al. A comparison of antibodies to tissue transglutaminase with conventional serological tests in the
diagnosis of coeliac disease. Eur J Gastroenterol Hepatol. 2003 Sep; 15 (9): 1001-4.
5. Jones B, Bayless TM, Hamilton SR, Yardley JH. "Bubbly" duodenal bulb in celiac disease: radiologic-pathologic
correlation. Am J Roentgenol. 1984 Jan; 142(1): 119-22
6. Lomoschitz F et al. Enteroclysis in adult celiac disease: diagnostic value of specific radiographic features. Eur Radiol.
2003 Apr;13(4):890-6.
7. Lomoschitz F et al. Enteroclysis in adult celiac disease: diagnostic value of specific radiographic features. Eur Radiol.
2003 Apr;13(4):890-6.*
8. Marsh MN. Gluten, major histocompatibility complex, and small intestine. A molecular and immunobiologic approach
to spectrum of gluten sensitivity ('celiac sprue'). Gastroenterology 1992 Jan;102(1):330-54)*
9. Marsh M N, Gluten, major histocompatibility complex, and the small intestine. A molecular and immunobiologic
approach to the spectrum of gluten sensitivity ('celiac sprue'). Gastroenterology 1992 Jan;102(1):330-54)
10. Rubesin SE, Herlinger H, Furth EE." Bubbly" duodenal bulb in clinically unsuspected or refractory adult celiac
disease. Abdom Imaging. 1998 Jul-Aug; 23 (4): 449-52.
11. Schweiger GD, Murray JA. Postbulbar duodenal ulceration and stenosis associated with celiac disease. Abdom
Imaging. 1998 Jul-Aug; 23(4):347-9.
12. Tomei E et al. CT of SB in adult celiac disease: jejunoileal fold pattern reversal. Eur Radiol. 2000; 10(1):119-22.*
13. Trier J. Celiac Sprue. NEJM. 1991
14. Westergaard H. Tropical Sprue. Curr Treat Options Gastroenterol. 2004 Feb; 7(1): 7-11.

Things that go bump in the bowel:
Familial Polyposis and Other Such
54 y/o M, Vomiting
• 2003: Vomiting 2-3 hrs p meals
• Liquid diet wks
• 30 lb wt loss - 4 mos
• Mother deceased, colon CA.
• 1996 Proctocolectomy, end-ileostomy
• 2001 Laparotomy, gastrotomy: 3 big gastric polyps removed
Figure 2-31-1
2 yrs earlier
Endoscopy
• Many gastric polyps, Large obstructing duodenal
polyp(s)
• Gastrectomy:
➢ Stomach: inflamm, mucosal papillary
hyperplasia with atypia.
• Duodenum : Hyperplastic mucosa, foci suggestive of
adenomatous change
FAP
• Usually:
➢ Dx known clinically ~75%
➢ Colonoscopic Dx, not BE, not CT
➢ Not Dx challenge except: Only a small percentage of Colorectal Cancers are
❖ ~25% de novo mutation related to FAP
❖ Professor’s quiz
➢ Differential important:
➢ More things look like FAP than are FAP
Figure 2-31-2
Colorectal Cancer CRC Perspective
[Figure 2-31-1]
• 150,000 new CR CA / yr, only ~ 1% = FAP
FAP = Colonic Polyposis [Figure 2-31-2]

• Onset of polyps
➢ Age 10 -- 15%
➢ Age 20 -- 75%
➢ Age 30 -- 90%
• Risk of CA nearly 100% by age 40 yrs
FAP Genotypes & Phenotypes

• VARIABILITY IN:
➢ Age onset
➢ Polyp #: 100 - 1000s
➢ Bone, Eye, Skin, Brain, Thyroid, Desmoid There are many components possible in the
• APC is BIG gene, 300+ variable mutations patient with Familial Adenomatous Polyposis
➢ “Gardner’s Syndrome”
➢ “Turcotte”, etc
• Early non-colonic changes may help early Dx
Gastrointestinal Radiology 519 Familial Polyposis

Uncountable Figure 2-31-3
Barely Countable
Countable, Pedunculated
Variable size [Figure 2-31-3]
1,000s of Small Early Polyps
FAP Colon Polyps = Adenomas

No matter what size
100s of Bigger “Older” Polyps 1,000s of Small Polyps

Polyp number, size, and age of
FAP UGI Polyps [Figure 2-31-4] appearance vary in FAP
• Fundal gland hyperplastic polyps 100s of Bigger “Older” Polyps patients
• Adenomatous change in hyperplastic
polyps in 100% of FAP pts. eventually.
• 5%-8% develop duodenal or ampullary CA, stomach, SB
• LIFETIME ENDO SURVEILL
Fundal gland hyperplastic polyps

“Fundal” refers to normal location of nl type of gland Figure 2-31-4
FAP UGI
• Start Flat
• Fundal first
• If you look you will find them.
• Follow colon polyps, occ precede.
Fundal gland hyperplastic polyps
Duodenal: Hyperplastic vs Adenoma vs CA ? [Figure 2-31-5]

• Lifetime Surveillance Needed
Figure 2-31-5 While they

occur first
and most
prominently
in the
gastric fundus, the term Fundal
Gland refers to the histologic
characteristics of the polyp.
“Fundal Gland” hyperplastic
polyps in FAP can occur
throughout the stomach and
duodenum
While they start as hyperplastic polyps, they may

undergo adenomatous change and then may
undergo malignant degeneration. We cannot
differentiate the three types of polyps:
Hyperplastic, Adenoma, Adenocarcinoma.
Lifetime endoscopy is required
Familial Polyposis 520 Gastrointestinal Radiology

FAP Duodenal AdenoCa [Figure 2-31-6] Figure 2-31-6
FAP Ampullary Tumor (Clue: Stoma)

[Figure 2-31-7]
Figure 2-31-7
Obvious Duodenal Adenocarcinoma
Figure 2-31-8
Ampullary tumor and stoma: a link worthy of being
an Aunt Minnie
FAP Duo - Ampulla [Figure 2-31-8]

• 1. Cystic Glandular Hyperplasia
• 2. Adenomas
• 3. Carcinoma
• Jaundice, abn LFTs
• Pancreatitis
FAP - 33 yr p Colectomy [Figure 2-31-9]
Relative Risk: Upper GI CA in FAP

Site No of Carcinoma Rel Risk
Duodenum 7 331
Ampulla 4 124
Gastric 2 2.4
Non-duodenal 1 12.7
F/u 1391 pts 18,679 pt - yrs Ampullary Polyp and absent colon.
Offerhaus GJA, et al. Gastroenterology 1992; 102:1980 Another linkup creating an almost
Aunt Minnie
Osteomas [Figure 2-31-10]
• Sinuses Figure 2-31-9
• Mandible
• Anywhere
Osteomas “Benign Bone Islands”

• Note absent colon gas
Mandibular Osteomas
“Exostotic Osteomas” [Figure 2-31-11]
Diffuse Cortical Thickening
Cortical Endo & Exos [Figure 2-31-12]

Ileostomy and SB tumor. With history of remote
total colectomy, another almost Aunt Minnie.
Without history, a great differential

Osteomas growing out from the angle of the

mandible, a favorite place for these rare
manifestations to occur in FAP
Figure 2-31-12
Osteomas may be “innie” or

“outie” osteomas
Figure 2-31-13
Osteomas of paranasal sinuses
FAP Dental ~80% [Figure 2-31-13]

• Fused roots 1st & 2nd molars
• Missing
• Impacted
• Supernumerary
• Long tapered roots post teeth
• Mal-erupted
Carl, W. Herrera, L. Dental and bone abnormalities in patients with Dental anomalies, mild, moderate, or
familial polyposis coli; Semin Surg Oncol 73-83, 1987 severe, occur in FAP
“CHRPE”: Congenital Hypertrophy of Retinal Pigment

Epithelium
• Evident in subset of FAP
• Screening marker for subset of certain families
• ~Earliest clinical marker
FAP Skin
• Sebaceous Cysts
• Pigment Changes
Desmoids
• Rare benign “tumors”,
• Never metastasize, invade locally,
• FAP associated, occ spontaneous
• In FAP pts:

➢ 50% abd wall (surg excision) Figure 2-31-14
➢ 50% intra-abdom,
❖ 85%-100% are mesenteric:
-SBO or ischemia,
-Hydronephrosis.
❖ Rx: NSAID in comb. w tamoxifen
Knudsen AL, Bulow S. Desmoid tumour in familial adenomatous polyposis. A
review of literature. Fam Cancer. 2001;1(2):113-21
Desmoid Radiology [Figure 2-31-14]

• 1. Desmoid “Tumor”
➢ Geometric: muscle or intraperitoneum
• 2. Mesenteric Fibromatosis
➢ Infiltration: mesentery, retroperitoneum
• Both can occur in same pt
Desmoid
NOTE: Colectomy, Hazy Dense Mesentery Desmoid tumors may be
geometric or infiltrative
NON FAP DESMOID: TUMORAL & INFILTRATIVE with
Ischemia
Infiltrative / Tumoral
FAP: Causes of death

Cause Number Mean age Yrs after Colectomy
Desmoid tumor 11 (31%) 35 7 Figure 2-31-15
Periampullary cancer 8 (22%) 49 23
Trauma/accident 3 ( 8%) 31 5
Perioperative death 3 ( 8%) 37 11
Rectal cancer 3 ( 8%) 42 13
Other 8 (22%) 50 11
DCR 1990, 33:639
Peritoneal Inclusion Cysts: “PIC” [Figure 2-31-15]

• F, usually premenopausal
• Prior proctocolectomy
➢ (Endometriosis, PID)
• Large pelvic cyst(s), multilocular
• Ovary trapped in pelvic loculation.
• Cysts lined by mesothelial cells.
• May see ovary on wall
Peritoneal Inclusion Cyst [Figure 2-31-16]

• Entrapped Ovary Syndrome
➢ Ovary secretes more fluid than can be absorbed by peritoneal
locule.
• Mimic Ovarian Cystadenoma or CA
• Hx important.
• Rx Cycle suppression
PIC [Figure 2-31-17]
FAP Mimics
• Numerically, more things resemble FAP than the cases of FAP that
we see.
• More common entities mimic classic polyposis syndromes
Peritoneal Inclusion Cysts are a
complication produced by pelvic
peritoneal adhesions in
premenopausal women.

Classification of Hereditary GI Figure 2-31-16
Polyposis Syndromes (Gene)
• Familial adenomatous polyposes
➢ Adenomatous polyposis coli (APC)
incl: Gardner, Turcot, Attenuated (APC)
• Hamartomatous polyposes
➢ Peutz Jeghers syndrome 1/8 Less common
➢ Familial juvenile polyposis rarer still
➢ Cowden’s disease
➢ Intestinal ganglioneuromatosis
➢ Ruvalcaba-Myrhe-Smith syndrome
Classification of Hereditary GI
Polyposis Syndromes (Gene)
• Familial adenomatous polyposes
➢ Born Adenomas -> Malignant transformation
• Hamartomatous polyposes HPs
➢ Born Hamartomas of varying types
➢ Some undergo epithelial atypia from “overwork”
➢ Some adenomas may develop, at risk CA
➢ Bowel CA can occur in all HPs
✧ More than general population
✧ Far less than FAP
Non-Hereditary GI Polyposes
• Inflammatory and post inflammatory
➢ CUC, Crohns, Infectious colitides Peritoneal Inclusions Cyst (s) progressively
• Lymphoid enlarge unless cycle suppression therapy is
➢ Reactive nodular lymphoid hyperplasia instituted. Reoccurrence may occur after lysis of
➢ Lymphoma adhesions
• Pneumatosis cystoides intestinalis
• Lipomatosis
• Angiomatosis Figure 2-31-17
• Leiomyomatosis
• Cronkhite-Canada syndrome
Peutz Jeghers [Figure 2-31-18]

• 3 SB Hamartomas
Figure 2-31-18
Ovary is evident in the wall of the PIC. A cystic

lesion with septations and a mural nodule may
simulate ovarian carcinoma
Peutz Jeghers with three large polyps in SB.

Polyps may be large, medium, small,
endoscopically visible, or so teeny weeny to be
visible only by microscopic biopsy. All sizes
may be encountered in the same patient

Peutz Jeghers [Figure 2-31-19] Figure 2-31-19
• Episodic Pain due to intussusceptions
Peutz Jeghers
• One 5 X 8 mm, many 1-2 mm polyps
Peutz-Jeghers [Figure 2-31-20]
Cowden’s Syndrome [Figure 2-31-21]

• Autosomal Dominant - rare
• “Multiple Hamartoma Synd.”
• Age onset: 1st to 3rd decade Peutz-Jeghers pts may
• Hamartomatous GI polyps, have painful episodes
• Non GI manifestations. due to intussusception
• CA, Breast~ 50% of F requiring surgery
➢ Thyroid
➢ Colon
Figure 2-31-20
Cowden’s Syndrome
• Papillomas / fibromas mucosa, tongue, (cobblestone
appearance)
• Acral keratosis
• Tricholemoma of face
• Also: angiomas, lipomas, skin vitiligo
Juvenile Polyposis Syndrome [Figure 2-31-22]

• The polyps are juvenile hamartomas
• Syndrome can be adult presentation.
• Anywhere
Peutz-Jeghers hamartomatous polyps in the

stomach and duodenum
Figure 2-31-21
Figure 2-31-22
Cowdens Syndrome
Gastric Juvenile Polyposis refers to the type of

polyp defined by microscopy, not the age of
presentation

SB Juvenile Polyps [Figure 2-31-23] Figure 2-31-23
Ruvalcaba-Myhre-Smith
• “Bannayan-Riley-Ruvalcaba syndrome (BRRS)”
• “Riley - Smith syndrome”
• “Bannayan - Zonana syndrome”
• “R M S” is best
• “Macrocephaly hamartoma papilledema syn”
RMS [Figure 2-31-24]
RMS Syndrome
• Rare2, autosomal dominant ? (80% male)
• Men more common
• Ileal & Colonic polyps in 45%
• Other:
➢ Colonic tumors
➢ GI tumor/polyp/hemangioma
➢ Lobulated tongue (including hamartomas)
➢ Skin
❖ Abnormal genital pigmentation
Large Juvenile Polyps in the SB
❖ Acanthosis nigricans
❖ Cafe au lait spots
❖ Nevi or lentigines Figure 2-31-24
❖ Lipomata
❖ Capillary hemangioma
❖ Cavernous hemangioma
❖ Other tumors of skin
❖ Supernumerary nipples
➢ Bones
❖ Delayed bone age
❖ Asymmetric limbs
❖ Joint laxity
❖ Muscle weakness/myopathy
➢ Brain
❖ Tumors/cysts
❖ Intra-cranial calcification
❖ Vascular malformations of brain Ruvalcabre Myhre Smith in SB and Colon
❖ Macrocephaly
❖ Papilledema
➢ Eye
❖ Anterior chamber abnormalities, Figure 2-31-25
❖ Visible nerve fibers on cornea
❖ Palpebral fissures slant down
➢ High birth wt (> 90th %centile)
Cronkite-Canada syndrome [Figure 2-31-25]

• Hyperpigmentation
• Alopecia
• Glossitis
• Dystrophic nails
• GI manifestations
➢ Harmatomatous polyps
➢ Exocrine pancreatic insufficiency)
➢ Diarrhoea
❖ Pancreatic insufficiency
Cronkhite-Canada Syndrome
❖ Protein losing enteropathy, low protein

CCS [Figure 2-31-26] Figure 2-31-26
Lymphoma
[Figure s 2-31-27 and 2-31-28]
• Nodularity one form of
Lymphoma.
• Remember L is a “PoLyposis+
Nodular Lymphoid
Hyperplasia Colon [Figure 2-31-29]
• Can be:
➢ Related to GI infection
➢ Assoc w
hypogammaglobulinem
➢ Assoc w Giardia
➢ Diff to histo diff from
Lymphoma
➢ TI most frequent Cronkhite Canada Syndrome
➢ Anywhere in GI tract
Lymphoma probably produces diffuse

polyposis of the colon, and other
organs, more frequently than does
any one of the more famous Polyp
Syndromes. Remember Lymphoma
!!!!! In your differential for polyposis
cases
Diffuse Colonic Polyposis. One of the most frequent Figure 2-31-29

causes of diffuse polyposis is not due to a syndrome
Figure 2-31-30
Nodular Lymphoid
Hyperplasia
Small Bowel [Figure 2-31-30]
• Radiology
➢ Uniform, 1-4 mm
➢ May be umbilicated
➢ Diffuse
➢ Regional
➢ Clustered -”patches”
Nodular Lymphoid
Hyperplasia
Colon
• Radiology
➢ Uniform, 1-4 mm
➢ May be umbilicated
➢ Diffuse Nodular Lymphoid Hyperplasia
➢ Regional may mimic a polyposis syndrome
➢ Clustered or Lymphoma
NLH of SB

Mastocytosis [Figure 2-31-31] Figure 2-31-31
• Secret:
• Small nodules differential:
• Think:
• White Blood Cell Differential:
➢ PMN
➢ Lymph
➢ Eo
➢ Mast
➢ Macroph
Hyperplastic Polyps CUC

[Figure 2-31-32]
NLH of SB Nodules in the SB are usually caused by cellular
Filiform Polyposis [Figure 2-31-33] infiltrates. To ease your differential brain pain, think of a WBC
• Crohns smear and all the types of WBCs seen. So nodularity in the SB
• CUC from ordinary PMNs caused by infections, acute or chronic -
eg Whipples Lymph cells: Lymphoma, Nodular Lymphoid
CUC Hyperplasia, Lymph Cell Granulomas of Crohns or TBC,
Immunoproliferative Small Intestinal Disease (IPSID)
Figure 2-31-32 Eosinophils: Eosinophilic Gastroenteritis
Mast Cells: Mastocystosis
Macrophages: Chronic infections: IPSID, Tropical Sprue,
Amyloid, Whipples
Figure 2-31-33
Polyps in CUC may be due to islands

of residual mucosa persisting after
slough of diseased mucosa or may
due to inflammatory polyps
developing as the mucosa attempts
regeneration. Note ahaustral colon
Hyperplastic polyps often have random shapes including
Giant Hyperplastic Polyposis wormlike or “filiform” polyps
[Figure 2-31-34]
• Seen in
➢ CUC
➢ Crohns
➢ Infect. Colitis
Figure 2-31-34
Hyperplastic polyps may be massive. Imagine polyps so long, so numerous that

they fill the colon like hamburger stuffing the colon like a sausage. Any colitis that
sloughs the mucosa may create localized of diffuse inflammatory polyposis. Each
polyp weeps serum creating hypoproteinemia. Pts may have inactive colitis and
present with CHF, peripheral edema, or anasarca

All Granulomatous Disease may give bumps !! Figure 2-31-35
• Granulomatous change in ….
Crohn’s Stomach & Colon
Crohns Stomach [Figure 2-31-35]
Hyperplastic Polyps [Figure 2-31-36]

• Benign
• Assoc w Atrophic Gastritis
• Atrophic Gastritis assoc w Gastric CA
• HP - AG - CA !!
• ? Assoc w Acid Suppression therapy: Crohns of the Stomach!!
• “Purple Pill Polyps”
Declich P, et.al. Fundic gland polyps under omeprazole treatment. Am J Clin
Pathol. 1999
Carcinoid Hyperplasia [Figure 2-31-37]

• IN:
➢ Atrophic Gastritis
➢ Zollinger-Ellison Figure 2-31-36
• 2° to:
➢ Absent Acid
➢ Acid Suppression
➢ Elevated Gastrin
• Cause:
➢ Carcinoid gland stimulation
Solcia E et al Morphology & pathogenesis of endocrine hyperplasias,
precarcinoid lesions, & carcinoids arising in chronic atrophic gastritis.
Scand J Gastroenterol Suppl. 1991;180:146-59
Figure 2-31-37
“Hyperplastic polyps” are the most

common polyp of stomach. 1 to 2 cm
size, PLUS multiple, PLUS mucosal,
PLUS fundal and body location,
PLUS approximately equal in size
add up to allow statistical call of
benign hyperplastic polypsl They are
Carcinoid Glandular Hyperplasia in themselves truly benign but are
associated with degrees of atrophic
gastritis which has risk for developing
cancer. I call these the “purple pill”
polyps because of suggested
association with Acid Suppressive
therapies. In your lifetime the
argument will be settled

Pneumatosis Cystoides Coli [Figure 2-31-38] Figure 2-31-38
Familial Polyposis
• Protean manifestations
• Multiple mimics
• Worthy of further study:
➢ For itself
➢ As starting point for Bumps of the
Bowel Pneumatosis Cystoides Coli may fool the unwary
References
1. Carl, W. Herrera, L. Dental and bone abnormalities in patients with familial polyposis coli; Semin Surg Oncol
73-83, 1987
2. Knudsen AL, Bulow S. Desmoid tumour in familial adenomatous polyposis. A review of literature. Fam Cancer.
2001;1(2):113-21.
3. Offerhaus GJA et al. Gastroenterology 1992;102:1980

The Spleen
Embryology
• Formed from the mesenchymal cells between the layers of the dorsal
mesentery, which lies between the stomach and pancreas
• Rotates to the left pulling the mesentery with it and forming the lesser sac
between the stomach and pancreas. Left side of the dorsal mesentery fuses
with the parietal peritoneum covering the left kidney and adrenal to form
Gerota’s fascia. This fusion brings the splenic vessels and the pancreas into
the retroperitoneum.
• Hilum of the spleen is retroperitoneal, while most of the spleen is
intraperitoneal, with a bare area similar to that of the liver, along posterior
surface adjacent to the left kidney
Anatomy
• Crescent shaped, convex toward the diaphragm and concave medially, located
in the LUQ
• Bounded by ribs, stomach, left kidney and splenic flexure of the colon.
• Splenic hilum contains splenic vessels and tail of pancreas (retroperitoneal)
➢ Splenic artery-tortuous, often containing aneursyms
➢ Splenic vein-straight. Confluence with SMV forms the portal vein. Splenic
vein often enlarges with splenomegaly. Upper normal is 1.5cm
Important Connections
• Splenorenal ligament (retroperitoneal)
➢ Connects the splenic hilum to the left kidney
➢ Contains the pancreatic tail, and splenic artery and vein
• Gastrosplenic ligament (peritoneal)
➢ Peritoneal fusion of the lesser and greater sacs, connecting the splenic
hilum to the stomach
• Phrenicocolic ligament (peritoneal)
➢ Connects the lower pole of the spleen to the splenic flexure of the colon
and to the diaphragm
Histopathology
• Stroma supports functional red and white pulp
• White pulp: the functional cells of the spleen; lymphocytes, plasma cells and
macrophages.
• Red pulp: surrounds white pulp and is comprised of arteries and sinuses filled
with blood. Also contains chords which slowly filter the blood, removing aging
cells
Splenic Function
• Adult: filtration of aged rbc’s, sequesters platelets, removes foreign particles
with macrophages.
• Childhood: adult functions plus production of lymphocytes and monocytes.
• Fetal hematopoesis
• Maintains immunity against bacterial pathogens (streptococcus D)
Normal Size
• Long axis from diaphragm to inferior pole
• Usual size (US and CT and MRI) length 12cm, width 7cm and thickness 3-
4cm. (you can allow greater length if the spleen is thinner)
• Spleen may be horizontal or longitudinal in orientation
Gastrointestinal Radiology 531 The Spleen

Normal Appearance (CT) [Figure 2-32-1] Figure 2-32-1
• Normally less dense than liver by 15 HU pre-
contrast, if greater than liver, then liver is fatty, or
spleen contains iron. Normal is 40-60 HU.
• Arterial phase imaging (30 sec p/injection)
➢ Heterogenous enhancement with a serpentine,
zebra-like pattern.
• Portal phase imaging (70 sec p/injection)
➢ Homogenous enhancement of 100-150 HU,
generally 25 HU greater than the liver. If more
than that, then liver is fatty
Normal Spleen. Normal arterial phase (left) and
Normal Spleen (US) [Figure 2-32-2] portal venous phase (right) CT images of the
• Measures 12 cm cranial-caudal spleen. Note the striped pattern on the arterial
• Normally does not extend beyond the left kidney phase at 30 seconds post injection has become
• Homogeneous mid-level echoes, slightly greater than uniform by the 70 second delay portal scan
liver and equal to or greater than left kidney.
• Image in longitudinal and transverse planes, often easier to see in expiration
Vos PM, Mathieson JR, Cooperberg PL, The Spleen: in Diagnostic Ultrasound;
Rumack CM, Wilson SR, and Charbonneau JW eds. .Elsevier Mosby 2005, St
Louis, pp 147-170
Figure 2-32-3
Figure 2-32-2
Normal US. Longitudinal image between the ribs

(left) and transverse image (right) shows the
uniform medium level echoes of the spleen with
the concave hilum containing the anechoic splenic Normal signal intensity of the spleen on T1 (top
vessels in the pancreatic tail left) T2 fat suppressed (lower left), Early arterial
T1 Gadolinium enhanced (top right) and portal
phase Gadolinium T1 enhanced (lower right) MRI
Normal MR Appearance [Figure 2-32-3] scans. The early arterial phase in MRI shows
• T1-low signal intensity, similar to muscle, lower than
heterogeneous enhancement which becomes
liver.
uniform in the portal phase
• T2 - high signal intensity, greater than liver.
• Gradient echo: in and out of phase
• Post contrast appearance: Arciform with serpiginous bands of low signal Figure 2-32-4
intensity separating larger regions of intense
enhancement, similar to that seen on CT
Normal Variants
• The spleen is formed from multiple cell aggregates
which coalesce.
• This gives rise to:
➢ Accessory spleens
➢ Clefts Normal accessory spleen
➢ Splenosis Left: normal spleen in left upper quadrant
➢ Splenic rests Right: Isoattenuating 2cm enhancing nodule
lateral to the left kidney and adjacent to the splenic
Accessory Spleens [Figure 2-32-4] flexure is a normal accessory spleen
• 30%-40% incidence in normal population and 10% of
patients have >1 focus
• Frequently (75%) in splenic hilum
The Spleen 532 Gastrointestinal Radiology

• Remainder usually adjacent to splenic poles or along Figure 2-32-5
splenic artery or in pancreatic tail
• Size ranges from microscopic to 2-3cm. May
hypertrophy following splenectomy up to 5cm.
• Importance is not to mistake for other soft tissue
pathology, usually lymphadenopathy
Accessory Spleen: Appearance [Figure 2-32-5]

• Isoattenating to spleen on CT, isointense on MRI,
and isoechoic on US.
• Ultimate diagnostic test is Tc99mSC nuclear
medicine scan (macrophages take up
radiopharmaceutical, differentiating accessory
spleen from lymphatic tissue)
Spleen Cleft [Figure 2-32-6]

• These are the residual spaces between partially
fused lobules. Enlarged spleen with accessory spleen (arrows)
• These are sharp and well defined. They may be as on US (top left), CT (lower left) and MRI T1
deep as 2-3cm weighted ( right top), T2 weighted ( right middle)
• Most commonly they occur along the lateral margin and Gadolinium enhanced (right lower) images.
of the spleen and on the superior diaphragmatic The image characteristics of the accessory spleen
portion. match those of the adjacent prinicipal splenic
• They may mimic splenic lacerations tissue on all imaging modalities
Splenosis [Figures 2-32-7 and 2-32-8]

• Residual splenic tissue following splenectomy. Fairly large (up to 5cm
diameter) multiple nodules may occur, but typically small, multiple and Figure 2-32-6
enhancing.
• Location: most frequently in the LUQ,
but may be anywhere in the abdomen
along the peritoneal surfaces and/or
mesentery.
• May involve the diapharagm and pleura
• DDX: endometriosis, mesothelioma
• Dx with Tc99mSC or RBC study Splenic clefts. Left: fine shallow clefts are noted at the superior
and posterior margins of the spleen adjacent to the diaphragm.
Note the lack of any fluid, and the sharp margins. The superior
cleft extends from the medial to the lateral surface.
Center: a deeper cleft in the posterior spleen, adjacent to the
diaphragm.
Right: An unmistakable cleft at the junction of 2 lobules, not a
splenic tumor. Note the normal enhancement similar to the rest
of the spleen
Figure 2-32-7
Splenosis. Left: multiple splenules in the left upper quadrant

after splenectomy. Middle CT show splenules adjacent to the
gallbladder and in the hepatorenal fossa (right) as well as in
the left splenic and renal fossae

Splenic Gonadal Fusion Figure 2-32-8
• Splenic tissue contained within epididymis, spermatic
cord, or testis
• Male: female ratio is 17:1
• Mimics tumor
• Believed to arise from adhesion between the
gonadal primordial tissue and the spleen prior to
gonadal descent.
• A fibrous band between gonad and spleen contains
additional splenules in 50% and is associated with
other congenital anomalies (cardiac or limb defects,
hernias, undescended testes, micrognathia)
• Unconnected gonadal rests not associated with other
anomalies
Warshaer DM. Spleen; in Computed Body Tomography
with MRI Correlation, Lee JK, Sagel SS, Stanley RJ and
Heiken JP, eds. 4th Edition, Lippincott Williams and
Wilkins, Philadelphia, 2006: 973-1006 Splenosis.Top left: 2 cm enhancing round mass
anterior to the aortic arch. Lower left: Round soft
Polysplenia tissue mass posterior to the left atrium. Top right:
• Bilateral left-sidedness with multiple other organ Bowel fills the left upper quadrant in the splenic
system manifestations fossa. Lower right: Technesium 99m sulfur colloid
➢ ie 2 left lungs or left sided azygous or interrupted study shows the normal liver, and enhancing
IVC, biliary atresia, absent gallbladder, GI nodules in the chest at the level of the aortic arch
malrotation and the heart
➢ Associated with cardiac abnormalities including
VSD, ASD, right sided arch, partial anomalous pulmonary venous return
(PAPVR), transposition of the great vessels
Asplenia
• Bilateral right-sidedness
➢ 2 right lungs in 2/3
➢ Midline liver
➢ More complex cardiac anomalies including single AV valve, pulmonary
stenosis or atresia, TAPVR, transposition of the great vessels, ASD, single
ventricle
➢ Mortality is as high as 80% in first year.
• Impaired immune response due to asplenia
➢ May present with serious bacterial infections
“Wandering Spleen”
• Long mesentery if dorsal mesentery fails to fuse with the posterior peritoneum.
• Diagnosis made by US, CT or MR showing classic splenic tissue in abnormal
location
• May torse and lead to acute or chronic abdominal pain. Lack of enhancement
is present in complete infarction.
• Chronic torsion may lead to hypersplenism, splenomegaly, or gastric varices
Enlarged Spleen
• Moderately large
➢ Portal hypertension most common (check for cirrhosis and collaterals)
➢ Anemia
➢ Infection
➢ AIDS
• Very large (17 cm or more)
➢ Leukemia or lymphoma
➢ Infectious mononucleosis
➢ Myelofibrosis

Splenomegaly [Figure 2-32-9] Figure 2-32-9
Splenomegaly with Spontaneous

Rupture
• 81 yo female with polycythemia
presented acutely with abdominal pain.
Note massively enlarged spleen with
anterior disruptions and
hemoperitoneum. Splenomegaly. Right: transverse US shows an enlarged spleen
anterior to the kidney. Center and right: massive splenomegaly
Benign Focal Lesions in a patient with portal hypertension
• Cysts
• Hemangiomas Figure 2-32-10
• Granulomas
• Abscesses
• Infarcts
• Trauma
Simple Splenic Cysts [Figure 2-32-10]

• Most commonly post traumatic in origin and lack an
epithelial lining, thus are really pseudocysts.
• Appearance:
➢ US: anechoic with imperceptible wall and
posterior acoustic enhancement.
➢ MR: low on T1, bright on T2 Splenic Cyst by CT (left) is often calcified post
➢ CT: water attenuation without enhancement trauma. Post-traumatic splenic cyst in a different
• Splenic cysts may rarely contain debris (cholesterol patient. Sagittal ultrasound (right) shows a largely
crystals, post trauma) anechoic mass with some near field echoes but a
• No enhancement on CT or MR after contrast sharp back wall, and posterior acoustic
enhancement. This one also has some rim
Epidermoid Cysts calcifications
• True congenital lesions discovered incidentally
• Often with trabeculations or septations and occasional peripheral calcification
Figure 2-32-11
Other Cysts
• Echinococcal cysts
➢ Extensive wall calcification
• Pancreatic pseudocysts may arise within the spleen.
Check for accompanying features of pancreatitis.
Pseudocysts may contain debris or hemorrhage
• Abscesses may mimic cysts by US, but on CT or MR
should have an enhancing rim. On US they may
contain gas or debris, differentiating them from
simple cysts Echinococcal cyst. Left: CT shows a heterogenous
cystic and solid lesion replacing the splenic tissue.
Echinococcal Cyst [Figure 2-32-11] The lesion contains central soft tissue and a
• 38-year-old Russian woman with persistent left flank spoke-wheel appearance of multiple cysts.
pain Right: Gross pathology shows multiple cysts in an
encapsulated mass within the spleen
Pancreatic Pseudocyst [Figure 2-32-12]
Figure 2-32-12
36 yo female with 3 days of LUQ and flank
pain. Cysts occupy the spleen (left and
center) and the gastric wall and supcapsular
regions of the spleen (right). Surgery
revealed multiple pseudocysts. The
pseudocyst in the gastric wall is a clue to
the origin of the cysts

Hemangioma [Figures 2-32-13 and 2-32-14] Figure 2-32-13
• Most common benign splenic neoplasm
• Echogenic on ultrasound
• Low signal on T1 and high on T2 (MRI)
• Low attenuation with early phase enhancement on MR or CT,
often lacking the nodules and centripetal fill-in seen with liver
hemangiomas, especially if <2cm
Other Benign Lesions

• Lymphangiomas-cystic with septations, may be septated and/or
calcified
• Hamartomas-normal splenic tissue, predominantly red pulp,
single or multiple, variable size (1-15cm)-slow and prolonged Sagittal US shows an echogenic well
enhancement noted. circumscribed mass on this
• Littoral Cell angioma-vascular tumor unique to spleen, multiple assymptomatic patient presenting for
lesions of low attenuation on CT .2-9cm in size renal ultrasound
Malignant Lesions
• Lymphoma Figure 2-32-14
• Metastases
• Angiosarcoma
Lymphoma
• Most common splenic malignancy
• Rarely as isolated lesion, usually as part
of diffuse disease
• Low grade lymphomas usually diffuse
enlargement
• Hodgkins and higher grade NHL cause Splenic hemangiomas. Left: early phase dynamic CT shows
discrete low attenuation/echogenicity markedly enhancing smooth round nodules. Right: Delayed CT
nodules. image at the same level shows delayed washout, characteristic
• Accuracy of CT prediction of splenic of a benign lesion. On CT splenic hemangiomas often show
involvement ranges from 30%-70%. diffuse bright enhancement, more than the puddling peripheral
Marked splenomegaly the best predictor enhancement seen in hepatic hemangiomas
of involvement. FDG PET reported
98%-100% accuracy in predicting splenic lymphoma
Warshauer, D. Spleen: Computed Body Tomography with MRI Correlation. Lee
JKT, Sagel SS, Stanley RJ, Heiken JP eds. Lippincott, Williams and Wilkins.
Philadelphia, PA. 2006 pp 973-1006
Lymphoma
• Often presenting with splenic enlargement, LUQ pain or fever, weight loss,
malaise.
• US: nodules are hypoechoic
• CT or MR, nodules usually not seen without contrast, but can be low signal on
T2w MRI.
Figure 2-32-15
• Post contrast (CT or MR) are less intense than normal spleen but fill in quickly
(2 min).
• Look for adjacent adenopathy
Lymphoma [Figure 2-32-15]

• A 53 yr old female with hepatitis
C,splenomegaly and thrombocytopenia
is evaluated for portal hypertension
Focal Non-Hodgkins lymphoma. A 53 yo female with hepatitis
C, splenomegaly and thrombocytopenia is evaluated for portal
hypertension. Left US shows a well circumscribed hypoechoic
lesion. On Doppler imaging (center) it is vascularized. On CT
(right ) there are multiple ill defined low attenuation lesions
which are non-specific, and could be tumor, infarct or infection

Lymphoma [Figures 2-32-16 and 2-32-17] Figure 2-32-16
Hodgkins Lymphoma
• A 35 yr old man with past left seminoma presents
with new lymphadenopathy and focal splenic lesions.
Lymph node biopsy yielded Hodgkins lymphoma
Metastases
• Common at autopsy difficult to image except on early
arterial phase contrast imaging (CT or MR)
Diffuse Non-Hodgkins lymphoma. 22 yo male with
• Sources include islet cell tumors, melanoma, breast
fever and LUQ mass. Left: Longitudinal US shows
carcinoma and lung carcinoma
massive splenomegaly with multiple diffuse
hypoechoic lesions. Right: US guided biopsy of a
Angiosarcoma [Figure 2-32-18]
focal lesion returned non-Hodgkins lymphoma.
• Most common primary nonlymphomatous splenic
The patient died within 2 weeks of splenic rupture
malignancy
and hemorrhage
• Single or multifocal
• Aggressive growth with hemorrhage and necrosis.
• Very vascular and enhance intensely with contrast in arterial phase Figure 2-32-17
Figure 2-32-18
Initial CT (left) shows isolated hypoechoic lesion of

44-year-old woman with abdominal pain, nausea, Non Hodgkins Lymphoma. One year later (right)
vomiting, chills. Patient underwent US, CT, US the lesion has nearly resolved
biopsy, laparoscopic biopsy with hemorrhage;
exploratory laparotomy to rebiopsy, and evacuate
hemoperitoneum, continued bleeding and dies 2
days later. CT (left) shows a low density lesion
with peripheral nodular enhancement and multiple
mixed attenuation partially enhancing lesions in Figure 2-32-19
the liver as well as ascites. Ultrasound (right)
shows heterogenous echogenic lesions in the
liver, consistent with highly vascular lesions with
multiple interfaces
Infection [Figure 2-32-19]

• Bacterial
➢ Aerobic from GI tract, sepsis
➢ TB
• Viral
➢ CMV, mononucleosis
• Fungal
➢ Candida 40yo male with AIDS presented with marked
splenomegaly. CT with contrast showed
inumerable tiny low attenuation lesions. US (upper
right) shows a 20 cm spleen with multiple tiny
hypoechoic lesions. Core biopsy (lower right) with
18 g needle revealed granulomas, eventually
proven to be TB

5 Years Later [Figure 2-32-20] Figure 2-32-20
Crohns Disease [Figure 2-32-21]

• 27 yo male with Crohns and ileal
perforation presents 3 weeks
postoperatively (ileocecectomy) with
LUQ pain and fever. What is the cause
of the splenic lesion?
Abscess Evolution
• 2 weeks later
• 6 weeks later TB in the spleen. Left: original US
Right: repeat US 5 years later shows persistant splenomegaly,
Abscess now with inumerable calcifications. Patient was treated
• What caused the abscess? successfully and was assymptomatic
• How did it get to the spleen?
• Dx: Diverticulitis extending along the phrenicocolic ligament
• What else do you see? Figure 2-32-21
• Splenic artery aneurysm
Sarcoidosis
• Splenic involvement common on biopsy
(24%-59%)
• Most patients asymptomatic
• May show splenomegaly, or diffuse
hypoattenuating nodules on CT and MR
which lack peripheral enhancement Splenic abscess. 27 yo male with Crohn’s disease presents 3
• Associated abdominal weeks status post ileal and cecal resection with LUQ pain and
lymphadenopathy common fever. Left CT shows portal vein thrombus. Center CT shows
splenic vein thrombosis. Right CT shows typical rosette shaped
Trauma abscess, in this case from septic thrombophlebitis from the GI
• Spleen most frequently affected organ tract
in blunt abdominal trauma.
• Highly associated with left lower rib fractures.
• Four appearances:
➢ Lacerations (check for splenic hilar involvement) are decreased
attenuation on contrast enhanced CT-perisplenic blood or clot often more
apparent than the laceration.
➢ Intrasplenic hematoma (contusion) may be low attenuation or contain
higher attenuation clot
➢ Subcapsular hematoma: non-enhancing fluid with crescentic compression
of underlying splenic tissue.
➢ Infarcts-non-enhancing wedge shaped areas extending to the capsule.
• Severe trauma shatters the spleen into fragments.
• Active bleeding is identified as focal extravascular enhancement similar in
intensity to the aorta
Trauma
• Clinically important injury is accompanied by hemoperitoneum
Active bleeding identified as area of contrast enhancement with arterial intensity.
Surgical intervention is based on clinical stability/hypotension, lacerations involving
the hilum, and presence of pseudoaneurysms or arteriovenous fistulae
Molina PL, Quinn MT, Bouchard EW, Lee JKT. Computed Tomography of
Thoracoabdominal Trauma; Computed Body Tomography with MRI Correlation,
4th ed, Lippincott, Williams and Wilkins, Philadelphia 2006, pp1440-1429
Trauma
• Massive splenic trauma with fragmentation of the spleen, active arterial
extravasation of contrast and hemoperitoneum

Splenic Trauma? Figure 2-32-22
• 23 yo snowboarder with injury. Initial arterial phase
images (left) show multiple possible contusions,
without perisplenic hematoma. Portal venous phase
images on repeat exam (right) show a normal spleen
Trauma [Figures 2-32-22 and 2-32-23]

• Lesion usually < 1cm
• Signal void on all pulse sequences
• Susceptibility artifact on GE images seen as
blooming artifact
Splenic Angiosarcoma
• Exceedingly rare
• Most common nonlymphoid primary malignant tumor
of the spleen
• More common in patients with thorotrast exposure Trauma. Initial CT shows tiny low attenuation
• Splenomegaly with well defined nodules or diffuse lesion (top left ) without fluid, and subtle low
involvement attenuation lesions (lower right) in an otherwise
normal spleen (lower left and upper right
Delayed Splenic Rupture
• Not predicted by degree of splenic injury
• Actual incidence unknown, but not uncommon.
• May occur days to weeks after initial injury Figure 2-32-23
Molina PL, Quinn MT, Bouchard EW, Lee JKT.
Computed Tomography of Thoracoabdominal Trauma.
Computed Body Tomography with MRI Correlation, Lee
JK, Sagel SS, Stanley RJ and Heiken JP, eds. 4th
Edition, Lippincott Williams and Wilkins, Philadelphia,
2006: 1417-1480
Sequelae of Trauma
• Injuries (lacerations, infarcts and hematomas) may
take months to a year to heal and may leave scars,
deformed splenic margins, or splenic pseudocysts.
• Uncommon injuries requiring intervention include
pseudoaneurysms or arteriovenous fistulae
Trauma. Eleven days later patient presents with
Blunt Splenic Trauma diffuse abdominal pain. Top left CT shows medial
• Initial CT for blunt trauma shows a bright area of contusion and perisplenic blood and ascites
extravasation superiorly (left) and multiple around the liver. Lower left and upper right show
lacerations more caudally (right). The patient was additional lesions. Lower right CT in the pelvis
discharged shows marked hemoperitoneum (high attenuation
ascites)
Splenic Trauma
Vascular Abnormalities
➢ Enlarged splenic vein and/or collaterals
• Splenic vein thrombosis
➢ Infection (Crohn’s, diverticulitis)
➢ Pancreatitis
• Splenic artery thrombosis (embolic)
• Splenic artery aneurysms
• Pseudoaneurysms ((trauma, pancreatitis)

Portal Hypertension Figure 2-32-24
• Splenomegaly-often up to 20cm c-c
• Collaterals
➢ Located at the spenic hilum and
most commonly directed into the
renal vein (a spontaneous spleno-
renal shunt)
➢ Venous flow in the spleen is never
Gamma Gandy Bodies. Spin echo T1 (top left) and T2
reversed even if it is reversed in the
weighted series (center), show signal voids at the iron foci.
splenic vein.
Gradient echo T1 images (Right) show the blooming effect of
• Gamma gandy bodies
iron due to magnetic susceptibility (pathognomonic)
Portal Hypertension
• 53 yo woman with cirrhosis and portal hypertension has massively enlarged
liver on ultrasound and LUQ collaterals (US and CT)
Gamma Gandy Bodies Figure 2-32-25

• Focal iron deposition
• Common in patients with cirrhosis and
portal hypertension due to
microhemorrhages
• Generally mm in size (less than 1cm)
• Signal void on all MR images with
pathognomonic blooming on gradient
echo images due to susceptibility 10 year old with sickle cell disease. Nn-contrast CT (left) shows
artifact high attenuation spleen, greater than the liver.(normally should
be less dense than liver pre contrast). MR T1 weighted (center)
Gamma Gandy Bodies [Figure 2-32-24] and T2 weighted (right) show very low signal intensity in the
• Spin echo T1, T2, show signal voids at spleen, which is normally equal to muscle on T1 and much
the iron foci. Gradient echo T1 images brighter than liver on T2. Note also the marked splenomegaly
show blooming effect of iron due to and the gallstones (T2)
magnetic susceptibility (pathognomonic)
Hemosiderosis [Figure 2-32-25]

• Hematoma
➢ Sucapsular
➢ Intrasplenic
➢ Perisplenic
• Look for sharp margin b/w spleen and peritoneal fluid
• Fluid will accumulate in dependent areas
Infarcts
• Common due to arterial emboli
• Common in lymphomatous spleens which outgrow their blood supply
• Autoinfarction with sickle cell disease
Infarct Appearance
• Focal wedge-shaped peripheral lesions
• Invisible on US
• Decreased attenuation on contrast enhanced CT and low signal on T1
weighted post-Gd enhanced MRI
• May rupture with peripsplenic hematoma
Splenic Infarcts [Figure 2-32-26]

• 53 yo man with MDS presents with increasing LUQ pain. Initial US (left) shows
large heterogeneous avascular cystic lesion in an enlarged spleen.
2 weeks later the lesion has retracted slightly. The patient underwent
splenectomy which showed hemorrhagic infarcts

Infarcts [Figure 2-32-27] Figure 2-32-26
• Patient with sarcoidosis and multiple infarcts.
Healing Infarcts
• Several months later the splenic contour is
diminished and the areas of infarction still lack
enhancement
What Is Your DX?

• Dx: Aortic valve vegetation with splenic infarcts
• 80 yo with abdominal pain, “R/O aortic dissection or
aneurrysm”
12 Hours Later
Conclusion
• The spleen is easily evaluated on cross-sectional
imaging modalities.MRI is most sensitive for iron and
for small lesions such as diffuse Candidiasis. Round, hemoorhagic splenic infarcts. Top left US
• Anatomic variants (splenules, clefts) are common shows a large heterogenous mass with through
and should not be mistaken for pathologic transmission. The mass is avascular (upper right).
processes. Lower left Ultrasound 2 weeks later shows less
• Splenomegaly is non-specific, but usually related to fluid in the lesion with smaller diameter, indicating
hematologic abnormalities or portal hypertension. some clot retraction. Pathology specimen (lower
• Infection is acquired by hematogenous or direct right) shows 2 infarcts, the larger one
spread (splenic vein, colon, pancreas) corresponding to the US images
• Splenic infarction is often the sequelae to other
disease processes and should encourage you to
search harder to make the diagnosis
Figure 2-32-27
Classical infarcts going from superior spleen (left) to hilum (center) to inferior spleen (right)
show varying shapes of infarcts which all extend to the periphery of the spleen and lack
enhancement
References
1. Vos PM, Mathieson JR, Cooperberg PL. The Spleen In: Diagnostic Ultrasound; Rumack CM, Wilson SR, and
Charbonneau JW eds.Elsevier Mosby 2005, St Louis, pp 147-170
2. Kelekis NL, Burdeny DA, and Semelka RC. Spleen In: MRI of the Abdomen and Pelvis. Semlka RC, Ascher SM
and ReinholdC, eds. 1997, Wiley-Liss New York, NY. Pp 239-256
3. Warshaer DM. Spleen; In: Computed Body Tomography with MRI Correlation, Lee JK, Sagel SS, Stanley RJ and
Heiken JP, eds. 4th Edition, Lippincott Williams and Wilkins, Philadelphia, 2006: 973-1006
4. Molina PL, Quinn MT, Bouchard EW, Lee JKT. Computed Tomography of Thoracoabdominal Trauma; Computed
Body Tomography with MRI Correlation, 4th ed, Lippincott, Williams and Wilkins, Philadelphia 2006, pp1440-1429.
5. Molina PL, Quinn MT, Bouchard EW, Lee JKT. Computed Tomography of Thoracoabdominal Trauma. Computed
Body Tomography with MRI Correlation, Lee JK, Sagel SS, Stanley RJ and Heiken JP, eds. 4th Edition, Lippincott
Williams and Wilkins, Philadelphia, 2006: 1417-1480

Portal Venous Doppler
WHY DO WE USE DOPPLER?

• To identify vascular from non-vascular structures
• To find vessels invisible on gray scale
• To make diagnoses based on arterial or venous spectral or color flow patterns
SCANNING PARAMETERS
• 3MHz transducer-small footprint often preferred for intercostal approaches
• Doppler gain as high as possible without image or spectral noise
• Wall filter as low as possible to avoid false diagnosis of thrombosis
• Scale (PRF) as low as possible to localize vessels quickly with color, then
sample and angle correct for spectral Doppler
WHICH DOPPLER TO USE?

• Spectral Doppler most sensitive to flow
➢ Inefficient for quick overview of flow direction, requires precise gate
placement, suspended respiration.
➢ Use wide gate and search for small hepatic arteries not seen with color or
power, and turn off color.
• Color Doppler good for flow direction (portal and hepatic veins)
➢ Quickly localizes hepatic arteries for spectral sampling.
➢ Aliasing identifies areas of stenosis (HA’s, TIPS)
• Power Doppler has best flow sensitivity
➢ Limited by motion (flash) artifact
➢ Best used in TIPS (which often have poor Doppler angles) and in portal
vessels with slow flow
COLOR DOPPLER ALIASING IN TIPS

• Color Doppler shows direction of flow and mean velocity. Spectral Doppler
shows angle-corrected true velocity
POWER vs. COLOR DOPPLER

• Partial thrombosis suggested. Spectral Doppler implies PV flow.
• Note HA visualized on power Doppler as well as intrahepatic vessels
THE NORMAL PORTAL VEIN

• Monotonous continuous waveform normally directed into the liver
(hepatopedal)
• Color doppler should fill out the entire vessel to exclude portal vein thrombosis
(may be anechoic)
• Spectral doppler velocities are typically low, 15-40cm/sec and may even be bi-
directional due to swirling flow in a large slow-flowing vein.
• Portal vein flow reverses (out of the liver, hepatofugal) in portal hypertension,
and in patent, well functioning porto-systemic shunts
PORTAL HYPERTENSION
• Most commonly caused by cirrhosis, but may also be caused by diffuse
metastatic disease and by venous outflow obstruction.
• Color flow Doppler and spectral doppler are used in the evaluation of portal
hypertension to detect portal vein thrombosis, the presence of collaterals and
to assess bypass shunts (portocaval, splenorenal, mesocaval, and TIPS) for
patency.
• A positive diagnosis can be made by reversed (hepatofugal) flow on Doppler
or by presence of porto-systemic collaterals
eMedicine - Portal Hypertension : Article by Ali Nawaz Khan, MBBS ...
Wilson SR, Withers CE. The Liver in Diagnostic Ultrasound, Rumack, Wilson and
Charboneau eds.2005, Mosby, Inc. pp 77-146
Gastrointestinal Radiology 542 Seminar: Portal Venous Doppler

CASE 1: 55 YO WOMAN WITH ABNORMAL LFT’S
• A transverse image of the right lobe of the liver shows 2 equal-sized vessels
adjacent to the gallbladder
CASE 1 What is your diagnosis?

• DX: Portal hypertension with hepatofugal flow
CASE 1: 55 year old woman with abnormal liver

function tests. (below) A transverse image of the
right lobe of the liver shows 2 equal sized vessels
to the right of the gallbladder
CASE 1: (below) Spectral display of the hepatic

artery (left) and portal vein (right). What is your
diagnosis?
PORTAL HYPERTENSION WITH HEPATOFUGAL FLOW

• US offers information re: flow direction; into or out of the liver, which is
unavailable on CT.
• Flow reversal out of the liver is a late, but diagnostic sign of advanced portal
hypertension.
• Quick clues are the opposing colors and opposing spectral flow directions of
the hepatic artery and portal vein, which should normally both flow in the same
direction
CASE 2
• Patient with AML and rising liver function tests. Baseline examination
CASE 2: 3 days later. What has happened and what is your

diagnosis?
• Dx: Veno-occlusive disease with reversed portal flow
CASE 2: Three days later the patient returns with

CASE 2: Patient with Acute myelogenous abnormal liver function tests
leukemia and rising liver function tests. (below) What has happened and what is your
Baseline examination shows normal portal Doppler diagnosis?
waveform (right)
Seminar: Portal Venous Doppler 543 Gastrointestinal Radiology

VENO-OCCLUSIVE DISEASE WITH REVERSED PORTAL FLOW
• Hepatic veno-occlusive disease involves hepatic venules in bone marrow
transplant patients.
• Major veins appear normal – the diagnosis is confirmed by liver biopsy.
• Rapid development of portal vein hepatofugal flow has been reported with
veno-occlusive disease* and when present is diagnostic
*Brown BP, Abu-Yousef M, Farner R, La Brecque D, Gingrick R. AJR
1990;154:721-4
CASE 3 :Explain the spectral tracings in the right and left lobes
• Dx: Liver metastases with localized left-sided portal hypertension
ATYPICAL PORTAL
HYPERTENSION
• While portal hypertension is most
commonly the sequelae of cirrhosis, it
can be caused by any process which
obstructs the sinusoids including Veno-
occlusive disease, Budd Chiari
syndrome, and metastases.
• Focal flow reversal should prompt a CASE 3: 61 year old female with rising liver function tests and
search for localized disease, including jaundice. Prior Doppler ultrasound was normal.
analysis of the gray scale images Left: Doppler tracing of the right HA and PV.
Center: Gray scale transverse image of the left lobe
PORTOSYSTEMIC COLLATERALS Right: Spectral Doppler tracing of the left HA and PV. Explain
• These are diagnostic of portal the difference between the left and right lobe.
hypertension Diagnosis: Metastatic breast carcinoma with localized left portal
• Frequent locations include the hypertension
gastroesophageal junction,
paraumbilical vein in the falciform ligament, splenorenal and gastrorenal in the
left upper quadrant, intestinal veins in the retroperitoneum, and hemorrhoidal
veins in the pelvis. Visualization of the paraumbilical vein is specific for the
diagnosis of portal hypertension.
• Visualization of collaterals requires slow flow settings, and a good sonographic
window, unobscured by bowel gas
CASE 4: 31 year old male with ascites
CASE 4: Can you account for the alternate flow directions in the
left and right portal veins? What is your diagnosis?
• Portal hypertension with a patent umbilical collateral vein
CASE 4: Can you account for the alternate flow

directions in the left and right portal veins? What is
CASE 4: 31 year old male with ascites your diagnosis?

PORTAL HYPERTENSION WITH COLLATERALS
• Multiple collateral pathways carry portal blood around the liver into the
systemic circulation
• The most common is the coronary vein, however this is not specific for portal
hypertension
• Another common pathway is the patent umbilical vein, seen in up to 20% of
patients and 100% specific for portal hypertension.
• A patent umbilical vein collateral will preserve forward (hepatopetal) flow in the
left portal vein while the flow in the right portal vein is reversed (hepatofugal).
CASE 5: What is happening in the splenic vein?
CASE 5: What is your diagnosis now?

• Portal hypertension with spontaneous
splenorenal shunt formation
CASE 5: Portal hypertension with spontaneous Case 5: A 68 year old woman

splenorenal shunt formation seen on sequential presents in liver failure.
CT images and corresponding transverse US Top: Midline transverse flow in the
image (bottom right) splenic vein is toward the transducer
(toward the spleen). Bottom: Flow in
CASE 6: A 30 yr old woman presents with acute the splenic vein is away from the
epigastric pain. spleen at the hilum
What is the diagnosis and possible etiology?
• Dx: Splenic and portal vein thrombosis, patient taking birth control pills
Portal Vein Thrombosis

• Bland thrombus is a frequent sequelae to slow flow and portal hypertension.
Other etiologies include hypercoagulability, pancreatitis, pyelophlebitis from
diverticulitis or Crohn’s disease or
cholangitis, and portocaval shunts.
Thrombus may be partial or complete,
involving main or branch vessels.
• Early thrombus is hypoechoic, and may
require color Doppler to detect.
• Older thrombus is hyperechoic and
recanalizes and/or forms collaterals,
which have typical portal spectral
waveforms. CASE 6: A 30 yr old woman presents with acute epigastric
• Tumor thrombus (HCCA) contains pain. What is the diagnosis and possible etiology?
hepatic arterial waveforms and may be Left: US shows enlarged anechoic portal vein with normal color
biopsied for staging.* flow in the IVC posteriorly. Right: Transverse image of
*Dodd GD, Memel DS, Baron RL, Eichner L, noncontrast CT corresponds to US on the left.
Santaguida LA. AJR 1995;165:573-577 Dx: Splenic and portal vein thrombosis in a patient taking birth
control pills

CASE 7: A 64 year old man is being considered for liver
transplant.
• Dx: Cavernous transformation of the portal vein
CAVERNOUS TRANSFORMATION OF THE

PORTAL VEIN
• This refers to numerous collateral vessels at the
porta due to acute or longstanding thrombosis.
(Cavernous transformation may occur as soon as a
week after occlusion) CASE 7: A 64 year old man is being considered
• The absence of an adequate portal vein for for liver transplant
anastamosis precludes successful liver
transplantation
De Gaetano AM, Lafortune M, Patriquin H, De Franco A, Aubin B, Paradis K.
Cavernous transformation of the portal vein: patterns of intrahepatic and
splanchnic collateral circulation detected with Doppler sonography. AJR 165,
1151-1155,
CASE 8: Is this normal or abnormal flow?

• Right and left portal vein flow reversal in normally
functioning TIPS
PORTO-SYSTEMIC SHUNTS
• These are used to decompress portal hypertension
to control bleeding
• Color Doppler is useful to assess shunt patency,
providing an adequate acoustic window is present
(may be limited in mesocaval and splenorenal
shunts)
• Intrahepatic portal venous flow should be hepatofugal
if the shunt is working properly
CASE 8: TIPS shunt. Is the portal vein flow normal
PORTOSYSTEMIC SHUNTS: TIPS or abnormal?
• These are the most common portosystemic shunt now used.
• The entrance is percutaneous from the jugular vein into the right hepatic vein
through the liver to the main portal vein.
• Spectral Doppler TIPS peak velocity is obtained in the proximal (portal venous
end), mid and distal (hepatic end) shunt. Absent flow indicates shunt occlusion
• Color flow in the intrahepatic portal veins should be toward the TIPS, and the
involved hepatic vein flow should be toward the IVC
TIPS – DOPPLER SURVEILLANCE

• Monitoring is recommended post procedure, then q3 months and as clinically
indicated
• Normal velocity is between 90 and 190cm/s in the mid and distal shunt.*
(although the original lower limit of normal was reported to be 50-60 cm/sec,**
this has been unreliable in the proximal shunt due to portal mixing)
• A change in peak stent velocity by >50cm/sec over time, reversed flow in the
draining hepatic vein, and focal shunt stenosis are also useful ***
*Kanterman RY, Darcy MD, Middleton WD, Sterling KM, Teefey SA, Pilgram TK.
AJR 1997;168:467-472.
**Foshager MC, Ferral H, Nazarian GK, Castaneda-Zuniga WR, Letournea JG.
AJR 1995;165:1-7.
***Dodd GD, Zajko AB, Orons PD, Martin MS, Eichner LS, Santaguida LA. AJR
1995;164:1119-1124.

PROXIMAL TIPS SHUNT VELOCITY
• Doppler signal from the proximal end of the TIPS shunt. Flow
velocity increases as the cursor is moved from the main portal
vein (less than 40 cm/sec) into the shunt (69 cm/sec)
NORMAL TIPS
ABNORMAL TIPS
• 28mm gradient between TIPS and right atrium due to tight
stenosis at the hepatic venous end which was balloon
angioplastied. Note monophasic portal wave form
Proximal TIPS Shunt Velocity
TIPS Shunt Stenosis Doppler signal from the proximal end
• Increased shunt velocity at the distal end of the shunt indicated by of the TIPS shunt. Note the increase
color Doppler aliasing in flow velocity as the cursor is
• Spectral Doppler signal demonstrates the focal distal shunt moved from the main portal vein into
stenosis, velocity is 256.7 cm/sec the shunt
Abnormal US, Normal Angiogram

• US abnormal by strict velocity criteria, but note
normal pulsatility
TIPS Monitoring: Low Velocities

• Subsequent venogram showed severe stenosis
throughout the stent, which was replaced with an
intrastent following balloon angioplasty
CONCLUSION
• Portal venous Doppler US is an effective way to
assess the patency and function of the hepatic portal
TIPS Shunt Stenosis. (Right) Increased shunt
system.
velocity at the distal end of the shunt indicated by
• It permits rapid identification of portal flow direction
color Doppler aliasing. (Left)Spectral Doppler
and can identify collaterals, specific to portal
signal demonstrates the focal distal shunt
hypertension.
stenosis, velocity is 256.7 cm/sec
• It is particularly useful to serially monitor TIPS shunt
function
Wilson SR, Withers CE. The Liver in Diagnostic Ultrasound, Rumack, Wilson and Charboneau eds.2005,
Mosby, Inc. pp 77-146.
References
1. Brown BP, Abu-Yousef M, Farner R, La Brecque D, Gingrick R. AJR 1990; 154:721-4.

2. De Gaetano AM, Lafortune M, Patriquin H, De Franco A, Aubin B, Paradis K. Cavernous transformation of the
portal vein: patterns of intrahepatic and splanchnic collateral circulation detected with Doppler sonography. AJR
165, 1151-1155,
3. Dodd GD, Memel DS, Baron RL, Eichner L, Santaguida LA. AJR 1995;165:573-577.
4. Dodd GD, Zajko AB, Orons PD, Martin MS, Eichner LS, Santaguida LA. AJR 1995; 164:1119-1124.
5. eMedicine - Portal Hypertensions: Article by Ali Nawaz Khan, MBBS.
http://www.emedicine.com/radio/topic571.htm
6. Foshager MC, Ferral H, Nazarian GK, Castaneda-Zuniga WR, Letournea JG. AJR 1995;165:1-7.
7. Kanterman RY, Darcy MD, Middleton WD, Sterling KM, Teefey SA, Pilgram TK. AJR 1997;168:467-472.
8. Wilson SR, Withers CE. The Liver in Diagnostic Ultrasound, Rumack, Wilson and Charboneau eds.2005, Mosby,
Inc. pp 77-146.

548 Genitourinary Radiology
Genitourinary Radiology 549

550 Genitourinary Radiology
Imaging of Uterine Disorders
Overview Figure 3-1-1

• Normal Anatomy
• Imaging Techniques
• Congenital Anomalies
• Benign Lesions
• Malignancies
Uterus
• Fundus
• Corpus
• Cervix
Uterine Corpus
• Serosa
➢ peritoneal reflection
• Myometrium
➢ involuntary smooth muscle
• Endometrium
➢ stratum basalis
➢ stratum functionalis
Normal cervix
Cervix [Figure 3-1-1]
• Internal os
• Endocervical canal
➢ columnar epithelium
➢ plicae palmatae
➢ surrounded by fibrous stroma and muscular layer
• External os Figure 3-1-2
➢ squamocolumnar junction
Uterine Ligaments
• Broad ligaments
➢ double sheet of peritoneum
• Cardinal ligaments
• Uterosacral ligaments
• Uterovesical ligaments
• Round ligaments
Blood Supply [Figure 3-1-2]

• Uterine artery
➢ branch of internal iliac
➢ passes superficial to the ureter
➢ enters myometrium at internal os
• Ovarian artery
➢ branch of the aorta
➢ anastomosis with uterine artery
Dual blood supply to uterus
Imaging Techniques
• Ultrasound
• Hysterosalpingography
• Sonohysterography
• MRI
Genitourinary Radiology 551 Imaging of Uterine Disorders

Ultrasound Figure 3-1-3
• Transabdominal
➢ full baldder
➢ 2.5–5.0 MHz transducer
• Transvaginal
➢ empty bladder
➢ 5.0–7.5 MHz transducer
Myometrium [Figure 3-1-3]

• Homogeneous intermediate echogenicity
• Can sometimes see hypoechoic inner and outer layers
• Blood supply
➢ uterine
➢ arcuate
➢ radial
➢ spiral (endometrium) The uterine arteries give rise to the
arcuate arteries located in outer
Endometrium [Figures 3-1-4 and 3-1-5] third of myometrium. Radial arteries
• Early proliferative phase course through the myometrium and
➢ thin echogenic line terminate as spiral arteries in the
• Late proliferative phase endometrium
➢ hypoechoic thickening, 4–8mm
• Secretory phase
➢ hyperechoic thickening, 7–14mm
• Menstrual phase Figure 3-1-4
➢ thin broken echogenic line
Figure 3-1-5a
Endometrium, proliferative phase Before ovulation (days 1 – 14), the ovary is in the
follicular phase and the endometrium is in the
proliferative phase. After ovulation (days 14-28)
Figure 3-1-5b the ovary is in the luteal phase and the
endometrium is in the secretory phase
Endometrium, secretory phase
Hysterosalpingography: HSG
• First ten days of menstrual cycle
• Active PID contraindication
• Radiation dose 75–750 mrad
• Only visualizes internal contour
• Primary use tubal patency
Imaging of Uterine Disorders 552 Genitourinary Radiology

Pelvic MRI Figure 3-1-6
• Phased array coils
• Fast T2WI images
Pelvic Protocol
• Pelvic Coil
• Coronal localizer – FMSPGR
➢ Always include kidneys
• FSE T2 – sagittal, axial,
coronal, oblique
➢ TR 4,000–5,000
➢ TE 90–130
➢ ETL 16
➢ FOV 20–24 cm
Normal Uterus: Sagittal T2WI and HSG view
➢ Thickness 4–5 mm, 1
mm gap
Figure 3-1-7
➢ Matrix 256x256
➢ 2–4 NEX
• T1 SE
➢ Axial
➢ TR 300–500
➢ TE min
• T1 Fat Sat with Gd
Uterus
• T1 – uniform intermediate
signal
• T2 – zonal anatomy
➢ Endometrium – high
signal Normal Cervix: sagittal and donut view
➢ Junctional zone – low
signal Figure 3-1-8
➢ Myometrium – intermediate signal
Normal Uterus
[Figure 3-1-6]
Normal Cervix
[Figure 3-1-7]
Embryology
Figure 3-1-9
Embryologic paramesonephric ducts.

Metanephrosis (kidney) with concurrent
development
Uterus forms from fused paramesonephric

ducts. Distal vagina forms from urogenital
sinus

Mullerian Duct Anomalies Figure 3-1-10
[Figure 3-1-10]
• Class Description
• I Agenesis or hypoplasia
• II Unicornuate
• III Didelphys
• IV Bicornuate
• V Septate
• VI VIDES-related
Unicornuate / Didelphys
[Figure 3-1-11]
• Low rate of pregnancy loss
• Limited surgical options
• Unicornuate – highest rate of renal
agenesis
• Didelphys – 75% have vaginal
septum
Bicornuate Classification of Mullerian duct anomalies

[Figures 3-1-12 and 3-1-13]
• Partial fusion of ducts
• Concave external contour
• Bicollis or unicollis
Figure 3-1-12
Figure 3-1-11
Bicornuate bicollis (2 cervices)

and
Unicornuate and didelphys bicornuate unicollis (1 cervix)
Figure 3-1-13
Bicornuate, unicollis

Septate Figure 3-1-14
[Figures 3-1-14 and 3-1-15]
• Most common uterine malformation
• Highest spontaneous abortion rate
• Septum may be complete or partial
• Septum may be fibrous or composed of myometrium
Figure 3-1-15
Complete and partial septum
Septate uterus with a complete fibrous

septum through the cervix
Bicornuate vs. Septate

[Figure 3-1-16]
Bicornuate Septate
Angle between horns >90º <90º

External morphology concave normal
Complications abnormall lie increased spontaneous
premature labor abortion rate
Treatment metroplasty hysteroscopic resection
Arcuate Uterus
[Figure 3-1-17]
Figure 3-1-16
Figure 3-1-17
At least 1 cm of remaining
myometrium should be present for
hysteroscopic resection
Arcuate uterus is a normal variant. 1 -
1.5 cm indentation

Diethylstilbestrol: DES – Related Figure 3-1-18
[Figure 3-1-18]
• 1–1.5 million female progeny exposed
• 50% have a uterine anomaly
• Associated with clear cell carcinoma of the vagina
• No associated urinary tract abnormality
Mullerian Duct Anomalies

• Form a continuum
• Renal anomalies in 25%
• Obstructions are common – risk for endometriosis and adenomyosis
• Septate has highest spontaneous abortion rate
• DES exposure risk factor for clear cell carcinoma of the vagina
Benign Uterine Masses

• Leiomyomas
• Adenomyosis
Leiomyoma
• Benign smooth muscle tumor
• Dysmenorrhea, hypermenorrhea, fertility problems
• 25% of premenopausal women
DES exposure
Leiomyoma
[Figure 3-1-19]
• Submucosal, intramural, subserosal
• Well circumscribed
• US – generally hypoechoic
• MRI – low signal on T1 and T2 unless they have
undergone degeneration
Figure 3-1-19
US, MRI, and hysteroscopic image of a submucosal fibroid
Leiomyoma Degeneration [Figure 3-1-20] Figure 3-1-20

• Hyaline
• Myxomatous
• Cystic
• Hemorrhagic (carneous)
• Sarcomatous
Cystic and hemorrhagic degenerated

fibroid

Indications for MRI Figure 3-1-21
• Pre-myomectomy
• Rapidly growing fibroid
• When US is confusing
Adenomyosis
[Figures 3-1-21 and 3-1-22]
• Heterotopic implants of endometrium within the
myometrium
• Dysmenorrhea, hypermenorrhea
• 25% of hysterectomy specimens
Adenomyosis [Figure 3-1-23]

• May be diffuse or focal (adenomyoma)
• MRI
➢ junctional zone > 1 cm
➢ low signal on T1 and T2 Diffuse adenomyosis
➢ punctate areas of high signal
➢ irregular borders
Adenomyosis Figure 3-1-22

• Ultrasound
➢ Enlarged heterogeneous uterus
➢ Focal form often confused for fibroids
Endometrial Thickness
• < 15 mm in premenopausal patient (secretory phase)
• ≤ 8mm in asymptomatic post-menopausal patient (if on hormones
scan after withdrawl bleeding)
• ≤ 4mm postmenopausal and bleeding
Abnormal Uterine Bleeding

• Polyps
• Submucosal fibroids
• Hyperplasia Adenomyosis
• Carcinoma
• Atrophy
➢ most common cause of post-menopausal bleeding
Figure 3-1-23
Sonohysterography
[Figure 3-1-24]
Figure 3-1-24
Focal adenomyosis (adenomyoma)
Sonohysterography. Saline is infused while

scanning

Endometrial Polyps
[Figure 3-1-25]
• Focal overgrowth of endometrial tissue
• Pedunculated or sessile
• 20% are multiple
• May be cystic
Figure 3-1-25
Endometrial polyp Submucosal fibroid
Endometrial Hyperplasia [Figure 3-1-26] Figure 3-1-26

• Increased estrogen stimulation
➢ hormone replacement (unopposed estrogen)
➢ tamoxifen
➢ anovulatory cycles, polycystic ovarian
disease
➢ obesity
➢ estrogen producing tumors (granulosa cell,
thecoma)
• Risk factor for carcinoma
Tamoxifen [Figure 3-1-27]

• Has an antiestrogen effect on the breast
but weak estrogen effect on the uterus
• Increased risk of endometrial carcinoma,
hyperplasia, and polyps
• Cystic changes often present
Endometrial hyperplasia
Postmenopausal Bleeding
• ≤ 4mm – atrophy
Figure 3-1-27
• > 4mm – sonohysterogram
➢ diffuse thickening – random bx or D&C
➢ focal thickening – hysteroscopy
Endometrial Carcinoma
• Most common GYN malignancy- 33,000
cases/year
• Risk factors:
➢ unopposed estrogens, tamoxifen
➢ nulliparous
➢ diabetes
➢ obesity
Cystic endometrium from tamoxifen

Endometrial Carcinoma
• Histology
➢ adenocarcinoma (80%–90%)
➢ adenosquamous
➢ papillary serous **
➢ clear cell carcinoma **
• Grade
➢ I – well differentiated
➢ II – moderately well differentiated
➢ III – poorly differentiated
Endometrial Carcinoma: FIGO – Staging

Stage Description
0 Carcinoma in situ
Ia Limited to endometrium
Ib Less than 1/2 myometrium
Ic Greater than 1/2 myometrium
II Invades cervix but not beyond uterus
III Beyond uterus but not outside pelvis
IVa Outside true pelvis / bladder / bowel
IVb Distant metastases
MRI Findings
• Intermediate signal mass Figure 3-1-28
• Expands endometrial cavity
• Enhances less than myometrium
• Not for screening
MRI Staging [Figure 3-1-28]

• Disruption of junctional zone
• Depth of myometrial invasion
• Extension into cervix
• Extension beyond uterus
• Adenopathy
Prognostic Factors
• Histology
• Tumor grade
• Depth of myometrial invasion
• Lymph node involvement
Endometrial carcinoma extending to serosa
Cervical Carcinoma
• 14,000 cases/year, 4,900 deaths/year
• Begins at squamocolumnar junction
• 90% are squamous cell
• Association with papilloma virus, herpes, and HIV
Cervical Carcinoma
Stage Description
0 Carcinoma in situ
I Confined to cervix
II Invades beyond cervix but not to pelvic sidewall or lower third of vagina
IIa No parametrial invasion
IIb Parametrial invasion
III Extension to pelvic sidewall / lower third of vagina / causes
hydronephrosis
IVa Invasion into bladder / rectum
IVb Distant metastases

Staging
• Clinical
➢ errors in 32% for stage IB (greater than 5mm deep and 7mm wide)
➢ 62% for II-IV
• MRI
➢ 93% accuracy for tumor size within 5mm
➢ Staging accuracy 87%- 92% Figure 3-1-29
Prognosis
• Tumor size
• Depth of invasion
• Parametrial extension
• Lymph node involvement
MRI
• Intermediate signal T2WI
• Check list
➢ tumor size
➢ depth of stromal invasion
➢ parametrial invasion
➢ hydronephrosis
➢ lymphadenopathy
Cervical Carcinoma Stage I

[Figure 3-1-29] Stage I cervical carcinoma. The tumor remains confined to the
cervix
Cervical Carcinoma Stage II
Stage IIB cervical carcinoma with obvious parametrial invasion
References
1. Bazot M, Cortez A, Darai E, et al. Ultrasonography compared with magnetic resonance imaging for the diagnosis
of adenomyosis: correlation with histopathology. Hum Reprod 2001; 16:2427-2433.
2. Davis PC, O'Neill MJ, Yoder IC, Lee SI, Mueller PR. Sonohysterographic findings of endometrial and
subendometrial conditions. Radiographics 2002; 22:803-816.
3. Nicolet V, Carignan L, Bourdon F, Prosmanne O. MR imaging of cervical carcinoma: a practical staging approach.
Radiographics 2000; 20:1539-1549.
4. Reinhold C, Khalili I. Postmenopausal bleeding: value of imaging. Radiol Clin North Am 2002; 40:527-562.
5. Reinhold C, Tafazoli F, Mehio A, et al. Uterine adenomyosis: endovaginal US and MR imaging features with
histopathologic correlation. Radiographics 1999; 19 Spec No:S147-160.
6. Scheidler J, Heuck AF. Imaging of cancer of the cervix. Radiol Clin North Am 2002; 40:577-590, vii.
7. Troiano RN, McCarthy SM. Mullerian duct anomalies: imaging and clinical issues. Radiology 2004; 233:19-34.
8. Ueda H, Togashi K, Konishi I, et al. Unusual appearances of uterine leiomyomas: MR imaging findings and their
histopathologic backgrounds. Radiographics 1999; 19 Spec No:S131-145.

Approach to Renal Masses
Approach to Renal Masses: 4 Questions

• Cyst vs Solid
• Infiltrative vs Expansile
• Fatty vs Soft Tissue
• Solitary vs Multiple
Tumor Growth: Ball vs. Bean

• Expansile – “Ball”
• Infiltrative – “Bean”
Expansile Renal Mass

• Spherical, exophytic, frequently encapsulated
• DDx
➢ Malignant – adenocarcinoma, metastases, lymphoma
➢ Benign – cyst, angiomyolipoma, oncocytoma, etc.
Infiltrative Renal Masses

• Invades parenchyma, preserves renal contour, poorly marginated
• DDx
➢ Malignant – transitional cell, squamous cell, lymphoma, atypical
adenocarcinoma
➢ Benign – pyelonephritis, XGP, TB
Intravenous Urography
• Only good for expansile masses
• Misses 1/3 of masses <3cm
• All lesions require further work up
➢ US, CT, MRI
Ultrasound
• Cyst
➢ anechoic
➢ acoustic enhancement
➢ sharp posterior wall
• RCCA
➢ can be hypo, iso, or hyperechoic
Computed Tomography
• 94% sensitive for lesions 3 cm or less
• 90%-95% accuracy in staging
• Pre and post contrast of lesion
• Scan in both corticomedullary and nephrographic phase
Genitourinary Radiology 561 Renal Neoplasms

Volume Averaging – All Tissues in the Slice Figure 3-2-1
Volume are Averaged
[Figures 3-2-1 to 3-2-3]
Phases of Excretion
• Corticomedullary phase 25 –80 sec
• Nephrographic phase 90–120 sec
• Excretory phase 3 –5 min
➢ Varies with injection rate, cardiac output, and renal
function
Corticomedullary Phase (CMP)

• Cortex and medulla > 100 HU difference Volume averaging
• Best for metastases and vascular invasion
• Pitfalls: Can miss hyperdense cortical masses and hypodense Figure 3-2-2
medullary masses, pseudotumor in the IVC
Nephrographic Phase
• Renal lesions are best seen in the nephrographic phase
• 1.1 > 2.4 more masses detected Contiguous vs. overlapping
reconstruction
Venous extension
Excretory Phase
• Decreasing density of nephrograms
• Worsening streak artifact especially with non-ionic contrast
Excretory Phase
• Decreasing density of nephrograms
• Worsening streak artifact especially with non-ionic contrast
• New role in CT urography
Affect of volume averaging

Figure 3-2-4 on an AML
Surgical approach is based on extent of venous invasion
Enhancement
• < 10 HU no enhancement
• > 15 HU enhancement
• 10–15 HU gray zone
Renal Neoplasms 562 Genitourinary Radiology

De-enhancement
• Decrease 15 HU at 15 minutes
CT Technique for Renal Mass Characterization

• Scan kidneys both pre and post contrast
• Slice thickness of 5–7mm
• Scan during corticomedullary and nephrograhic phase
• Perform overlapping reconstruction if the lesion is small
• Delayed scans
Magnetic Resonance Imaging

• Equivalent to CT in accuracy with Gd
• Calcification difficult to detect
• Excellent for vascular invasion
Magnetic Resonance Imaging

• Cysts
➢ low-signal T1WI
➢ high-signal T2WI
➢ no enhancement
• Solid
➢ 15% enhancement with Gd
Calculating % Enhancement
• (Post SI – Pre SI) / Pre SI x 100 = % enhancement
Malignant Neoplasms
• Adenocarcinoma
• Uroepithelial tumors
➢ Transitional cell
➢ Squamous cell
• Lymphoma
• Metastases
Renal Cell Carcinoma

• 25,000 –30,000 new cases
• 12,000 deaths per year
• Peak incidence – sixth and seventh decade
• M:F (2–3:1)
• Bilateral 2%, multicentric 15%

• Expansile cortical mass
➢ 90% originate from proximal convoluted tubule

• Slow growing low grade malignancies may be encapsulated
• Can have areas of necrosis, cyst formation, hemorrhage, or calcification
Histology
• Clear cell – 70%–80%
➢ Deletion on chromosome 3p
➢ Lipid rich
• Papillary – 10%–15%
➢ Slower growing, less vascular, calcification more common, often
encapsulated, better prognosis
• Other – chromophobe, sarcomatoid, medullary, etc.

Renal Cell Carcinoma - Risk factors Figure 3-2-5
• Dialysis [Figure 3-2-5]
• von Hipple Lindau [Figure 3-2-6]
➢ often multiple RCCAs
➢ multiple renal cysts
➢ affects other abdominal organs
• Tuberous sclerosis (much less common)
Renal Cell Carcinoma: Presentation

• “Classic triad” – hematuria, flank pain, mass < 50%
• Paraneoplastic – hypertension, erythrocytosis,
hypercalcemia
• Other – fever, weight loss, anemia, varicocele
• 30% present with metastases
Calcification Cystic disease of dialysis with RCCA

• 20%–30% of RCCA
• 1%–2% of benign cysts
• Rim calcification – 80% benign Figure 3-2-6
• Central calcification – 87% malignant
“Cystic” Changes
• 15–25% of RCCA
➢ Necrosis 75%
➢ Cystic 25% – often papillary histology
❖ Mural nodule or septations [Figure 3-2-7]
❖ Malignant cell lining (VHL)
Benign Lesions
• Simple Cyst
➢ Water density
➢ Thin (1-2 mm) wall
➢ No enhancement
• Minimally Complicated Cyst VHL with multiple RCCA
➢ High density cysts (protein or blood)
➢ Thin septations
➢ Thin curvilinear calcifications Figure 3-2-7
Surgical Lesions
• Enhancing lesions
• Nodularity
• Thick wall (>2mm)
• Thick septations
• Irregular or central calcifications
➢ Less important
Spontaneous Renal Hemorrhage

• RCAA (men)
• AML (women)
• Infarction Cystic RCCA
• Infection
• AV malformation
• Vasculitis
• Glomerulonephritis
Infiltrating Renal Cell

• 7% of adenocarcinomas
• Arise from the renal medulla – difficult to differentiate from
invasive uroepithelial tumor
• Medullary carcinoma, collecting duct carcinoma, sarcomatoid neoplasms
• Poor prognosis

Medullary Carcinoma [Figure 3-2-8] Figure 3-2-8
• Young Black male with sickle cell
trait
• From epithelium of papilla or distal
collecting duct
• Survival < 4 mos.
Robson Staging [Figure 3-2-9]

• I – Confined to kidney
• II – Within Gerota’s fascia
• III A – Renal vein or IVC invasion
• III B – Lymph nodes
• III C – Vascular invasion plus nodes
• IV A – Direct organ invasion
• IV B – Distant metastases
Medullary carcinoma with infiltrative pattern
TNM Staging
• T1 – < 7cm
• T2 – > 7 cm Figure 3-2-9
• T3a – local invasion not beyond Gerota’s fascia
• T3b – venous invasion below diaphragm
• T3c – venous invasion above diaphragm
• T4 – extension beyond Gerota’s fascia
• N0 – no regional lymph nodes
• N1 – metastasis in a single regional lymph node
• N2 – metastasis in more than one regional lymph
node
• M0 – no distant metastasis
• M1 – distant metastasis
• Stage I
➢ T1,N0,M0
• Stage II
➢ T2,N0,M0
• Stage III
➢ T1,N1,M0
➢ T2,N1,M0
➢ T3a,N1,M0
➢ T3b,N0,M0 Stage 1 Stage II with invasion
➢ T3b,N1,M0 into Gerota’s fascia
➢ T3c,N0,M0
➢ T3c,N1,M0
• Stage IV
➢ T4,N0,M0
➢ T4,N1,M0
➢ Any T,N2,M0
➢ Any T,any N,M1 Figure 3-2-10
American Joint Commitee on Cancer
Nephron-sparing Surgery
• Margins of at least 5 mm normal tissue
• < 4cm
• Away from renal hilum (polar, cortical)
• Survival rates comparable to radical nephrectomy
• RF ablation non-surgical alternative
Stage II vs. Stage I

[Figure 3-2-10]
• Nodule in perinephric space most specific
but present < 50%
• Perinephric stranding unreliable
Stage II with nodule in perirenal space

Stage III [Figure 3-2-11]
Figure 3-2-11
Stage III
Stage III Renal Carcinoma: Imaging

[Figure 3-2-12]
Figure 3-2-12
Vascular invasion extending to the right atrium

Stage IV [Figure 3-2-13]
Figure 3-2-13
Stage IV
Stage IV renal carcinoma: Imaging

Renal Cell Carcinoma: Metastases
• Lung 69%
• Bone 43%
• Liver 34%
• Nodes 22%
• Brain 5%
• Adrenal 4%
Abdominal CT Checklist
• Renal vein, IVC
• Regional lymph nodes
• Adrenal glands
• Contralateral kidney
• Review lung and bone windows Stage IV with invasion into the descending colon
Uroepithelial Neoplasms
• 5%–10% of all urinary tract malignancies
• Transitional cell 85%– 95%
• Squamous cell carcinoma 5%–10%
• Rare – adenocarcinoma, sarcoma, metastases
Transitional Cell Carcinoma

• 50–70 yo
• Males > females (3:1)
• Risk factors
➢ Smoking
➢ Aniline dyes
➢ Benzene
➢ Analgesic nephropathy (phenacetin)
➢ Balkan nephropathy
Transitonal Cell Carcinoma

• Hematuria 75%
• Multicentric 30%–50%
• Bilateral 10%
• Incidence by location:
➢ Bladder 92%
➢ Pelvis 6%
➢ Ureter 2%

Transitional Cell Carcinoma
[Figure 3-2-15]
• Small, hypovascular masses Figure 3-2-15
• Majority papillary with endophytic growth
• Renal invasion in 25%
• Imaging
➢ Retrogrades, IVP with compression
➢ CT urography
➢ US and conventional CT poor

[Figure 3-2-16]
• Squamous metaplasia from chronic irritation
• Associated with stones (>50%)
• Aggressive behavior, commonly infiltrative
• Survival < 1 yr
Renal Lymphoma Invasive transitional cell carcinoma presenting as

[Figures 3-2-17 to 3-2-19]
a renal mass
• Common in widespread disease
• Primary lymphoma very rare
• Hematogenous spread or direct invasion
• 50-70% bilateral Figure 3-2-16
• Homogeneous
• Multiple masses 50%
• Infiltrating hilar
mass 25%
• Perirenal 10%
• Renal enlargement 10%
• Solitary mass 5%
Figure 3-2-17
Sqaumous cell carcinoma with a staghorn calculus and large soft tissue
mass. SCCA often has overlapping features with XGP
Figure 3-2-18
Renal lymphatics are

located around the
capsule and renal hilum
Figure 3-2-19
Renal lymphoma with homogeneous

expansile and infiltrative masses
Perirenal
lymphoma with
capsular
invasion

Metastases Figure 3-2-20
• 7%–20% of autopsy cases
• Generally asymptomatic
• Primaries
➢ Lung
➢ Breast
➢ GI (esp colon)
➢ Melanoma
Metastates [Figure 3-2-20]

• Spread
➢ Hematogenous – usually cortical
➢ Lymphatic – perirenal
➢ Direct invasion
• Expansile or infiltrative pattern
• Solitary or multiple masses Metastases with adenopathy
Benign Renal Neoplasms Figure 3-2-21

• Cystic
➢ Multilocular cystic nephroma
• Solid
➢ Parenchymal
❖ Oncocytoma
❖ Juxtaglomerular tumor
❖ Leiomyoma (capsuloma)
➢ Mesenchymal
❖ Angiomyolipoma
Multilocular Cystic Nephroma

[Figure 3-2-21]
• Bimodal age distribution
➢ < 2 yo (M:F, 3:1)
➢ > 40 yo (F:M, 9:1)
• Multiple well-defined cysts with enhancing septa, no hemorrhage
• Can herniate into renal pelvis
• DDx: cystic renal cell carcinoma, MCDK complicated benign cyst,
abscess
Oncocytoma
[Figure 3-2-22]
• Oncocyte – Greek “swollen cell”
• Large epithelial cells with granular eosinophilic cytoplasm (abundant
mitochondria)
• Found in kidney, salivary glands, thyroid, parathyroid, and pancreas
Multilocular cystic nephroma
Oncocytoma with herniation into the renal
• Usually asymptomatic pelvis
• Large – 7 cm avg. at detection Figure 3-2-22
• Older males
• Solid exophytic enhancing mass
• Can not distinguish from RCCA
Oncocytoma
• Helpful features – all non-specific
➢ Central scar
➢ “Spoke wheel” angio appearance
➢ Necrosis, hemorrhage, calcification rare
➢ No adenopathy or metastases
• Gross – tan/brown tumor with pale central scar
Oncocytoma with a central scar

Juxtaglomerular Cell Tumor Figure 3-2-23
• Renin producing tumor
• Rare cause of hypertension, may also have headache
and muscle weakness
• Young adults (F:M, 2:1)
• Hypovascular mass – imaging non-specific
Leiomyomas [Figure 3-2-23]

• Small solid masses on renal surface (capsuloma)
• Usually incidental finding
• Imaging non-specific
➢ Low signal on T2-weighted MRI is suggestive
Angiomyolipoma: AML
• Renal hamartoma with blood vessels, smooth muscle, and fat
• Prevalence 0.3%–3%
• 80% sporadic
➢ Females 30–50 yo
➢ Usually solitary
• 20% tuberous sclerosis
Low-signal, non-enhancing
Angiomyolipoma: Imaging leiomyoma (capsuloma)
• Ultrasound non-specific [Figure 3-2-24]
• AML
➢ Shadowing Figure 3-2-24
➢ Markedly hyperechoic
• RCCA
➢ Hypoechoic rim
➢ Cystic spaces
• Must prove with CT or MRI
Angiomyolipoma: Imaging
• CT
➢ HU < –10 will detect 85% of AMLs
➢ No calcifications
➢ Vascular phase imaging can detect aneurysms
• MRI
➢ fat bright on T1 and T2
➢ fat saturation sequence
• Angiography
➢ Tortuous, abnormal vessels with small
aneurysms
➢ Embolization AML with shadowing
Tuberous Sclerosis [Figure 3-2-25]

• Clinical triad – seizures, adenoma sebaceum, mental retardation Figure 3-2-25
• Multiple hamartomatous lesions including: retinal hamartoma,
cortical tubers, subependymal nodules, ungual fibroma,
angiofibroma, pulmonary lymphangiomyomatosis, cardiac
rhabdomyoma
Tuberous Sclerosis
[Figure 3-2-25]
• Renal involvement
➢ Approx 3/4 will have AML
❖ 75% multiple
❖ 50% bilateral
➢ Cysts can also be seen especially in children
➢ 1%–2% develop RCCA Tuberous sclerosis with large bilateral
AMLs

Angiomyolipoma: Presentation Figure 3-2-26
[Figure 3-2-26]
➢ Usually < 4 cm
• Hemorrhage
➢ Usually > 4cm
➢ May be spontaneous or minor trauma
• Bleeding may be life-threatening in up to 25% of cases
• Vessels thick walled with decreased elastin
• Predisposition for aneurysm formation
RCCA with Fat

[Figure 3-2-27]
Angiomyolipoma with perinephric
• Osseous metaplasia – calcification
hemorrhage
• Lipid necrosis – large necrotic masses
• Engulfed perirenal or sinus fat – large masses, irregular
Figure 3-2-27
invasive appearance
RCCA with osseous metaplasia

Helpful Tips
• No enhancement (etc.)
➢ Benign cyst
• Fat
➢ AML
• Multiple AMLs
• Infiltrative + expansile
➢ Lymphoma
• Herniation into renal pelvis + female
• Cysts + solid masses
➢ VHL or dialysis
• Central scar + no adenopathy or vein invasion
➢ Oncocytoma
References
1. Catalano C, Fraioli F, Laghi A, et al. High-resolution multidetector CT in the preoperative evaluation of patients
with renal cell carcinoma. AJR Am J Roentgenol 2003; 180:1271-1277.
2. Choyke PL, Glenn GM, Walther MM, Zbar B, Linehan WM. Hereditary renal cancers. Radiology 2003; 226:33-
46.
3. Khan A, Thomas N, Costello B, et al. Renal medullary carcinoma: sonographic, computed tomography, magnetic
resonance and angiographic findings. Eur J Radiol 2000; 35:1-7.
4. Rendon RA, Stanietzky N, Panzarella T, et al. The natural history of small renal masses. J Urol 2000; 164:1143-
1147.
5. Sheth S, Scatarige JC, Horton KM, Corl FM, Fishman EK. Current concepts in the diagnosis and management of
renal cell carcinoma: role of multidetector ct and three-dimensional CT. Radiographics 2001; 21 Spec No:S237-
254.
6. Agrons GA, Wagner BJ, Davidson AJ, Suarez ES. Multilocular cystic renal tumor in children: radiologic-
7. Israel GM, Bosniak MA, Slywotzky CM, Rosen RJ. CT differentiation of large exophytic renal angiomyolipomas
and perirenal liposarcomas. AJR Am J Roentgenol 2002; 179:769-773.
8. Urban BA, Fishman EK. Renal lymphoma: CT patterns with emphasis on helical CT. Radiographics 2000; 20:197-
212.
9. Wong-You-Cheong JJ, Wagner BJ, Davis CJ, Jr. Transitional cell carcinoma of the urinary tract: radiologic-
pathologic correlation. Radiographics 1998; 18:123-142; quiz 148.
10. Yamakado K, Tanaka N, Nakagawa T, Kobayashi S, Yanagawa M, Takeda K. Renal angiomyolipoma: relationships
between tumor size, aneurysm formation, and rupture. Radiology 2002; 225:78-82.

Urinary Tract Trauma
GU Trauma
• Evaluate lower tract before upper tract if both may be injured Figure 3-3-1
• Males always perform retrograde urethrogram before foley is inserted if there
is blood at the meatus or pubic rami fx/diastasis
Male Urethra [Figure 3-3-1]

• Posterior
➢ Prostatic
➢ Membranous
• Anterior
➢ Bulbous
➢ Penile
Retrograde Urethrogram: RUG

[Figure 3-3-2]
• Inflate pediatric foley (3–5cc) in fossa navicularis
• 50 cc of 30%–60% contrast
• Inject 20–30 cc and take film while continuing to inject
• Oblique if possible or gently move penis laterally
• May perform pericatheter RUG if foley is in place
Figure 3-3-2
Normal urethral anatomy
Figure 3-3-3
Intact urethra
Urethral Trauma
[Figures 3-3-3 to 3-3-8]
Type 1 - Stretch injury
• Posterior – pelvic fractures
I – stretch
II – rupture above UGD Figure 3-3-4 Figure 3-3-5
➢ retropubic extravasation
III – rupture above and below
UGD
➢ perineal/scrotal
extravasation
IV – bladder neck and urethra
Type II – rupture above UGD
Genitourinary Radiology 573 Urinary Tract Trauma

Type III – rupture

above and below
UGD
Figure 3-3-8
Type V – Anterior urethral trauma [Figures 3-3-9 and 3-3-10]
• Bony injury uncommon
• Partial/complete
➢ Corpora/venous extravasation
• Associated scrotal trauma
Type IV – Bladder neck and

urethra
Type V – Anterior urethral trauma (straddle injury)
Complications
• Strictures
• Fistulas
Bladder Trauma
• Blunt or penetrating
• 5%–10% of pubic rami fx
• Pelvic fractures in 80% of ruptures
➢ 83% of extraperitoneal
➢ 62% of intraperitoneal
Bladder Trauma Evaluation

• Standard cystogram
➢ 300–500cc
incomplete filling may miss leak
➢ 15%–30% I concentration
➢ AP, obliques
➢ post drainage important for small leaks
• CT
➢ clamp foley
➢ delayed images, post drain
Urinary Tract Trauma 574 Genitourinary Radiology

Extraperitoneal Bladder
Rupture Figure 3-3-11 Figure 3-3-12
[Figures 3-3-11 and 3-3-12]
• 60%
• focal extravasation,
“flame-shaped”
• conservative therapy
Intraperitoneal Bladder
Rupture
[Figures 3-3-13 and 3-3-14]
• 40%
• free flowing extravasation,
outlines intraperitoneal
organs
• surgical therapy Extaperitoneal bladder rupture
Bladder Trauma Evaluation

[Figure 3-3-15 and 3-3-16]
• CT cystogram
➢ perform routine CT Figure 3-3-13 Figure 3-3-14
➢ drain bladder
➢ refill with 2%–3% I
solution (300 cc)
➢ scan full and post
drain
Intraperitoneal bladder rupture
Figure 3-3-15
Figure 3-3-16
Intraperitoneal bladder rupture

on CT cystogram
Ureteral Injury
• Least common site of injury (<3%) Extraperitoneal bladder rupture on CT cystogram
• Penetrating trauma – anywhere
• UPJ disruption

Renal Injuries Figure 3-3-17
[Figures 3-3-17 to 3-3-21]
• Category I - Minor (85%) Figure 3-3-18
➢ contusion
➢ intrarenal hematoma
➢ small subcapsular/perirenal hematoma
➢ segmental infarction
➢ superficial laceration
• Conservative management
Figure 3-3-19
Hematoma
Figure 3-3-20 Subcapsular hematoma has

an abrupt start and stop
point and deforms renal
parenchyma
Subcapsular hematoma with

delayed nephrogram Figure 3-3-21
Renal Injuries [Figures 3-3-22 and 3-3-23]

• Category II - Serious (10%)
➢ deep lacerations
➢ laceration through the collecting
system
➢ large perinephric/subcapsular
hematoma
• Conservative management vs. surgery
Figure 3-3-22
Laceration breaks through
renal capsule and causes a
perinephric hematoma
Figure 3-3-23
Segmental infarction
Laceration into the collecting Laceration into the collecting

system system with urine leak

Renal Injuries [Figures 3-3-24 to 3-3-30] Figure 3-3-24 Figure 3-3-25
• Category III - Catastrophic
➢ fractured/shattered kidney
➢ renal artery occlusion/avulsion
➢ renal vein occlusion/avulsion
➢ UPJ avulsion
• Often surgical treatment
Figure 3-3-26
Shattered kidney Vascular avulsion
Figure 3-3-29
Vascular avulsion with contrast

extravasation
Figure 3-3-27
Figure 3-3-28
Acute arterial thrombosis with

subsequent development
of a rim sign
Figure 3-3-30
Renal artery thrombosis.

Non-functioning normal sized
kidney
Rim sign: collateral

circulation forming after
thrombosis
Renal vein thrombosis. Kidney

will be enlarged

UPJ disruption [Figures 3-3-31 and 3-3-32]
• Deceleration injury
• 3:1, children:adults
• 3:1, R:L
• Stent, nephrostomy, surgery
Figure 3-3-31
Figure 3-3-32
UPJ disruption. Leak obvious on

delayed films
UPJ disruption
Conclusion
• If there is blood at the meatus, perform urethrogram first
• A normal bladder on CT does not rule out a leak
• Post-drainage films are key for small leaks
• Don’t forget delayed images
References
1. Ali M, Safriel Y, Sclafani SJ, Schulze R. CT signs of urethral injury. Radiographics 2003; 23:951-963; discussion
963-956.
2. Blankenship B, Earls JP, Talner LB. Renal vein thrombosis after vascular pedicle injury[clin conference]. AJR Am
J Roentgenol 1997; 168:1574.
3. Fishman EK, Horton KM. CT evaluation of bladder trauma: a critical look. Acad Radiol 2000; 7:309-310.
4. Goldman SM, Sandler CM, Corriere JN, Jr., McGuire EJ. Blunt urethral trauma: a unified, anatomical mechanical
classification. J Urol 1997; 157:85-89.
5. Herschorn S, Radomski SB, Shoskes DA, Mahoney J, Hirshberg E, Klotz L. Evaluation and treatment of blunt
renal trauma. J Urol 1991; 146:274-276; discussion 276-277.
6. Kamel IR, Berkowitz JF. Assessment of the cortical rim sign in posttraumatic renal infarction. J Comput Assist
Tomogr 1996; 20:803-806.
7. Kawashima A, Sandler CM, Corriere JN, Jr., Rodgers BM, Goldman SM. Ureteropelvic junction injuries
secondary to blunt abdominal trauma. Radiology 1997; 205:487-492.
8. Kawashima A, Sandler CM, Corl FM, et al. Imaging of renal trauma: a comprehensive review. Radiographics
2001; 21:557-574.
9. McAndrew JD, Corriere JN, Jr. Radiographic evaluation of renal trauma: evaluation of 1103 consecutive patients.
Br J Urol 1994; 73:352-354.
10. Nunez D, Jr., Becerra JL, Fuentes D, Pagson S. Traumatic occlusion of the renal artery: helical CT diagnosis. AJR
Am J Roentgenol 1996; 167:777-780.
11. Roberts JL. CT of abdominal and pelvic trauma. Semin Ultrasound CT MR 1996; 17:142-169.

Retroperitoneum
Figure 3-4-1
Retroperitoneum
• Non-neoplastic
➢ Fluid collections
❖ Pancreatic, urinoma, hematoma, abscess
➢ Retroperitoneal fibrosis
➢ Extramedullary hematopoiesis
• Lymphadenopathy
➢ Inflammatory/infectious
➢ Castleman disease
➢ Lymphoma
➢ Metastatic adenopathy
• Organs
➢ Pancreas, colon, duodenum
➢ Kidneys, adrenal, ureters
• Primary ( > 100 benign and malignant tumors) Retroperitoneal spaces
➢ Neurogenic
❖ Nerve sheath, ganglioneuroma,
ganglioneuroblastoma, neuroblastoma Figure 3-4-2
❖ Paraganglioma
➢ Mesenchymal
❖ Lipoma/sarcoma, leiomyoma/sarcoma, malignant
fibrous histiocytoma (MFH), lymphangioma,
hemangioma, hemangiopericytoma, angiosarcoma
➢ Germ cell
❖ Teratoma (benign and malignant)
Anatomy: 3 spaces [Figure 3-4-1]

• Anterior pararenal
• Perirenal
• Posterior pararenal
Updated view of the perirenal space with
Anterior Pararenal Space (the GI space) complex fascial boundaries
• Colon (ascending and descending)
• Pancreas Figure 3-4-3
• Duodenum (2nd and 3rd portions)
Perirenal Space (the GU space) [Figure 3-4-2]

• Kidneys
• Adrenal glands
• Upper portion of ureters
Posterior Pararenal Space (the nothing space)

• No solid organs
• Fat, connective tissue, nerves
Borders [Figure 3-4-3]

• Parietal peritoneum separates peritoneal space from APRS
• Anterior renal fascia separates APRS from perirenal space (Gerota’s
fascia)
• Posterior renal fascia separates PPRS from perirenal space
(Zuckerkandl’s fascia)
• Lateral conal fascia demarcates the lateral
Sagittal view of the
extent of the APRS
retroperitoneum. All 3
• Separates the APRS from the PPRS
compartments
• All spaces communicate inferiorly
communicate inferiorly
Genitourinary Radiology 579 Retroperitoneum

Retroperitoneal Fibrosis
• Microscopically: collagen, fibroblasts, and inflammatory cells
Figure 3-4-4
• Typical distribution: below kidneys to bifurcation
• 40% may have atypical distribution
• 8%–10% malignant
➢ Desmoplastic reaction to infiltrating metastases
➢ Breast, lung, colon, prostate, cervix
➢ Prognosis poor (3-6 months)
Retroperitoneal Fibrosis: Etiology

• 2/3 idiopathic (Ormand’s Disease)
• Methysergide toxicity
• Aortic aneurysm
• Surgery
• Hemorrhage
• Inflammatory bowel disease
• Collagen vascular disease
• Radiation/surgery
• Fibrosing conditions elsewhere
Retroperitoneal Fibrosis [Figure 3-4-4]

• IVP, retrogrades
➢ Medial deviation of ureters
➢ Hydronephrosis
Retroperitoneal Fibrosis [Figure 3-4-5]

• CT
➢ Wispy plaque-like deposits to confluent masses RPF with medial deviation of the ureters and
➢ Enhancement variable hydronephrosis
➢ Aorta is encased but not deviated
• MR
➢ Fibrotic phase
Figure 3-4-5
❖ Low on T1 and T2
❖ No enhancement
➢ Active phase
❖ High on T2
❖ Enhancement
❖ Can not rule out malignancy
❖ Must biopsy
Treatment
• Stents
• Steroids
• Immunosuppression
• Surgery
Neurogenic Tumors
• Nerve sheath
➢ Schwannoma (neurilemmoma), neurofibroma,
malignant nerve sheath tumor
• Ganglionic
➢ Ganglioneuroma, ganglioneuroblastoma,
neuroblastoma
• Paraganglionic
➢ Paraganglioma (pheochromocytoma)
Neurogenic Tumors
• Paraspinal masses
• Mass often elongated and well-defined
• Smooth or mildly lobular RPF with low signal on T2WI
• Generally benign
• Rapid growth, increased vascularity, poorly circumscribed suggest malignancy
Retroperitoneum 580 Genitourinary Radiology

Neurogenic Tumors Figure 3-4-6
• Low density on CT
• Low signal on T1WI
• May be hyperintense on T2WI (myxoid matrix)
• May have calcifications
Nerve sheath tumors [Figure 3-4-6]

• Often appears as psoas mass
• Look at neuroforamen
• May have intraspinal (extradural) extension
• Multiple consider neurofibromatosis
Ganglion Cell Tumors [Figure 3-4-7]

• Form from primitive neural crest cells
• Sympathoblast
➢ Neuroblastoma
➢ Ganglioneuroma
• Pheochromoblast Nerve sheath tumor presenting as a psoas mass
➢ Paraganglioma
Figure 3-4-7
Ganglioneuroma
• Benign
• More common in mediastinum
• Generally asymptomatic
• Elongated low-density masses
• Maybe be hyperintense on T2WI
Paraganglioma
(Extra-adrenal pheochromocytoma)
• About 10% of pheochromocytomas
• Most (60%–80%) have known catecholamine excess
➢ Hypertension, palpitations, sweating, tremor, diarrhea,
nausea
• More commonly malignant than adrenal pheochromocytomas
Paraganglioma [Figure 3-4-8]

• Organs of Zuckerkandl
• CT non-specific
➢ Enhance avidly
➢ Contrast contraindicated
• High signal on T2 is suggestive but is not universally seen
• Uptake on MIBG scan
Figure 3-4-8 Sympathetic chain
Paraganglioma with high signal on T2WI

Leiomyosarcoma [Figure 3-4-9]
• More commonly necrotic than other tumors
• May have intravascular invasion
• May arise in the wall of the IVC
Figure 3-4-9
Leiomyosarcoma with IVC invasion
MFH / Malignant Fibrous Histiocytoma [Figure 3-4-10] Figure 3-4-10

• Generally large masses
• Necrosis less common
• T2WI helpful – “Bowl of fruit sign”
➢ Mosaic of low and high signal
❖ Fibrous tissue – low
❖ Myxoid stroma – hyperintense
❖ Soft tissue - intermediate
Lymphangioma [Figure 3-4-11]

• Benign
• Fluid-filled
• Uni-multiloculated
• Insinuates itself around organs
• Can be huge
Figure 3-4-11
MFH with “bowl of fruit” on T2WI
Lymphangioma

Liposarcoma [Figure 3-4-12] Figure 3-4-12
• The most common primary retroperitoneal tumor
• 85% have fat detected by CT or MR
• Well-differentiated, pleomorphic, myxoid, de-differentiated
• Poorly differentiated tumors have no detectable fat by
imaging studies
Liposarcoma [Figure 3-4-13]

➢ Often present late
➢ Weight gain
• Infiltrative margins
• Complete surgical excision may be difficult
• Local recurrence common
Liposarcoma
Hyperintense T2WI: Things with a myxoid matrix
• Neurogenic tumors
• Malignant fibrous histiocytoma Figure 3-4-13
• Myxoid liposarcoma
Germ Cell Tumors

• Teratoma
➢ Mature
➢ Immature
• Malignant germ cell tumors
Teratoma
• Most are mature (benign) and are cured by surgery
• Children (less than 6 months) and young adults
(15–25 years)
• Female:male = 3:1
Teratoma [Figures 3-4-14 and 3-4-15]

• Fat (sebum or adipose tissue)
• Calcification in 90% (may be clump-like)
• Cystic portion in 75%
Figure 3-4-14
Myxoid liposarcoma with high signal on T2WI
Figure 3-4-15
Embryo with germ cells in the

retroperitoneum Retroperitoneal teratoma

Malignant germ cell tumors are more common in males and are
much more likely to be secondary to a testicular tumor (rather
than primary retroperitoneal)
Fat containing retroperitoneal masses

• Liposarcoma: large, heterogeneous
• Teratoma: young patients, calcification, cystic area
• Myelolipoma: usually arising from adrenal
• Angiomyolipoma: arises from kidney, but attachment may be hard to find
References
1. Engelken JD, Ros PR. Retroperitoneal MR imaging. Magn Reson Imaging Clin N Am 1997; 5:165-178.
2. Granstrom P, Unger E. MR imaging of the retroperitoneum. Magn Reson Imaging Clin N Am 1995; 3:121-142.
3. Kim T, Murakami T, Oi H, et al. CT and MR imaging of abdominal liposarcoma. AJR Am J Roentgenol 1996; 166:829-
833.
4. Nishimura H, Zhang Y, Ohkuma K, Uchida M, Hayabuchi N, Sun S. MR imaging of soft-tissue masses of the
extraperitoneal spaces. Radiographics 2001; 21:1141-1154.
5. Nishino M, Hayakawa K, Minami M, Yamamoto A, Ueda H, Takasu K. Primary retroperitoneal neoplasms: CT and
MR imaging findings with anatomic and pathologic diagnostic clues. Radiographics 2003; 23:45-57.
6. Morton A. Meyers. Dynamic Radiology of the Abdomen. Springer-Verlag. A must read book in your residency

Radiologic Evaluation of the Scrotum
Embryology
• Sex is chromosomally determined at fertilization
• No morphologic differentiation until week 7 (“indifferent stage”) Figure 3-5-1
• Testis determining factor on short arm of Y chromosome
➢ induces formation of seminiferous tubules
3 Components of Gonad [Figure 3-5-1 to 3-5-4]

• Germ cells
➢ arise from yolk sac
➢ migrate to genital ridges
• Mesothelium
➢ primitive sex cord
➢ Sertoli cells
• Mesenchyme
➢ Interstium
➢ Leydig cells
Migration of germ cells along the
hindgut to the genital ridges
Normal seminiferous tubules
Embryologic formation of the testes Figure 3-5-4
• Leydig cells secrete testosterone

➢ stimulate mesonephric ducts
• Sertoli cells secrete mullerian-inhibiting factor
Sex organs at indeterminate stage
Genitourinary Radiology 585 Radiologic Evaluation of the Scrotum

Mesonephric (Wolffian) Ducts
• Epididymis
• Vas deferens Figure 3-5-5
• Ejaculatory ducts
• Seminal vesicles
Embryologic Remnants
• Mullerian
➢ appendix testis
• Wolffian
➢ appendix epididymis
Testis [Figure 3-5-5]

• 200-300 lobules
• Seminiferous tubules (300-980 meters)
• Efferent ductules (15-20) converge at mediastinum
Epididymis
• Form single convoluted tubule in head (600 cm)
• Tail loosely attached inferiorly
• Exits as single tube Normal testis
Spermatic cord [Figure 3-5-6]

• Vas deferns Figure 3-5-6
• Testicular, deferential, cremasteric arteries
• Pampiniform plexus
• Nerves, lymphatics
Ultrasound
• Testes
➢ homogeneous low level echoes
➢ linear echogenic mediastinum testis
• Epididymis
➢ globus major (head), body, tail
➢ iso- to slightly hyperechoic
MRI
• T1WI - homogeneous intermediate signal
• T2WI - high signal with low signal mediastinum testis and linear
septations
MRI [Figure 3-5-7]

• Tumors are low signal masses
Spermatic cord
Goals of Ultrasound
• Intra-testicular vs. extra-testicular
• Cyst vs. solid
Figure 3-5-7
Testicular Neoplasms
• Germ Cell Neoplasms (95%)
• Sex cord, Stromal Tumors
➢ Sertoli cell
➢ Leydig cell
• Lymphoma
• Metastases
• Epidermoid Cysts
Testicular tumors are low signal on T2WI
Radiologic Evaluation of the Scrotum 586 Genitourinary Radiology

Germ Cell Neoplasms Figure 3-5-8
• Seminoma (most common “pure” tumor)
• Embryonal Cell Carcinoma
• Yolk Sac Tumor (Endodermal Sinus Tumor)
• Teratoma
• Choriocarcinoma
• MGCT - Mixed Germ Cell Tumor (most common
overall)
Seminoma [Figure 3-5-8]

• Homogeneous, well-defined
• May be lobular and multifocal
• Bilateral 2%
• Peak age 30-40 years
• Radiosensitive
• Good prognosis
Seminoma
Non Seminomatous Germ Cell Tumor [Figure 3-5-9] Figure 3-5-9
• Embryonal
➢ Rare in pure form
➢ 87% of MGCT
• Yolk Sac (endodermal sinus tumor)
➢ Most common childhood tumor
➢ 44% of MGCT
• Teratoma
➢ Mature and immature (always malignant in
adults)
➢ Cysts/calcifications common features
• Choriocarcinoma
➢ Very rare
➢ Dismal prognosis
Non Seminomatous Germ Cell Tumor MGCT with large amount of teratoma
• Mixed germ cell tumors much more common than
any pure tumor
• Heterogeneous, ill-defined
• Peak age 20’s
• Not radiosensitive
• Often higher stage and less favorable prognosis than seminoma
Germ Cell Tumors: Modes of Spread

• Lymphatic
➢ ipsilateral renal hilum
• Hematogeous
➢ common with choriocarcinoma
➢ lung, liver, brain
Tumor Markers
• Alpha-fetoprotein (AFP)
➢ from fetal liver, GI tract, and yolk sac
➢ elevated in tumors with yolk sac elements
• Human chorionic gonadotropin (HCG)
➢ produced by syncytiotrophoblasts from developing placenta
➢ elevated in tumors with choriocarcinoma (occasional seminoma)
• LDH
➢ Non-specific, correlates with bulk of disease
• Elevated in 80% of non-seminomatous tumors

Burned-Out Germ Cell Tumor Figure 3-5-10
• Presents with metastases (often extensive)
• The primary may not contain any active tumor and
may be difficult to identify
• Orchiectomy performed if any suspicious area seen
Testicular Microlithiasis
• 0.6% in general population
• Present in 40% of germ cell tumors
• Consider annual screening
Risk Factors for Testicular Carcinoma

• Prior testicular tumor
• Cryptorchidism
• Infertility Bilateral undescended testes
• Family history
• Intersex syndormes (hermaphrodite) Figure 3-5-11
• ??? microcalcifications
Cryptorchidism [Figure 3-5-10]

• 5% testicular agenesis
• 65% migratory testis
• 30% undescended
• Increased incidence of malignancy
• Risk is also increased in the contralateral testis
Sex Cord, Stromal Tumors [Figure 3-5-11]

• Sertoli (sex cord) and Leydig (stromal)
• 90% benign
• More common in pediatric age group
• May be be hormonally active Sertoli cell tumor with calcification
➢ precocious puberty, gynecomastia
➢ more common with Leydig Figure 3-5-12
• Sertoli cell tumors may be bilateral and calcified
Testicular Lymphoma − Presentation

• Most common testis tumor > 60 yo
• 5% of testicular neoplasms
• < 1% of patients with lymphoma
• Often presents as the site of recurrent disease because of
“blood-testis barrier” (Sertoli cells)
Testicular Lymphoma − Imaging [Figure 3-5-12]

• Most common bilateral tumor
• Homogeneous
• Epididymis and spermatic cord often involved Lymphoma with bilateral testicular
masses
Epidermoid Cyst [Figure 3-5-13]
• Benign Figure 3-5-13
• ? germ cell tumor
• Filled with keratin
• Well-defined
• Ringed-appearance
• Can not differentiate from a malignant neoplasm
➢ May do focal resection rather than orchiectomy
Concentric rings in a
epidermoid cyst

Tumors Summary
• Children
➢ Yolk sac tumor Figure 3-5-14
➢ Sertoli, Leydig cell
• Younger men (20’s)
➢ Mixed germ cell tumor
➢ Heterogeneous, poorly defined
• Somewhat older (30s)
➢ Seminoma
➢ Homogeneous
• Older males (> 60 yo)
➢ Lymphoma
➢ Bilateral
➢ May involve paratesticular structures
Non-neoplastic Testicular Masses

• Tubular ectasia
• Cysts
• Sarcoidosis
• Adrenal rests Tubular ectasia with an intratesticular cyst
• Acute scrotum
➢ infection
➢ infarction
➢ trauma
Figure 3-5-15
Tubular Ectasia
• Dilatation of the rete testis
• Often bilateral
• Associated with a spermatocele
• Tubular US appearance
• Iso- to hyperintense on T2WI
Testicular Cysts [Figure 3-5-14]

• Peripheral
➢ Tunica albuginea cyst
• Central
➢ Must be careful to differentiate from cystic
neoplasm
➢ Can not have any solid component
➢ Often associated with dilated rete testis
Sarcoidosis [Figure 3-5-15]

Sarcoidosis with multiple testicular masses
• Multisystem chronic granulomatous disorder
• 5% will have genital involvement Figure 3-5-16
• Epididymis more commonly involved
• More common in Blacks (testicular tumors are rare)
Adrenal rest hypertrophy secondary to

congenital adrenal hyperplasia [Figure 3-5-16]
• Adrenal rests in 7.5-15% of newborns, 1.6% adults
• Hypertrophy when exposed to elevated ACTH
• Bilateral, multiple, eccentric
• Tx – glucocorticoids not orchiectomy
Bilateral Testicular Masses

• Lymphoma
• Seminoma (rarely)
• Metastases
• Sarcoidosis
• Adrenal rests Developing adrenals. Adrenal tissue may become
entrapped within the developing testis

Extratesticular Scrotal Masses [FigureS 3-5-17 and 3-5-18]
Developing scrotum. Testicular descent is aided by Adult scrotum. Processus vaginalis

the processus vaginalis closes to form tunica vaginalis
Hydrocele
• Fluid between the parietal and visceral layers of the tunica vaginalis
• Small amount is normal
Hydrocele Figure 3-5-19

• Congenital
➢ Patent processus vaginalis, may have an
inguinal hernia
• Acquired
➢ Infection, infarction, trauma, tumor
Scrotal Calculi
• Torsion of appendix or inflammatory deposits
• Repeated microtrauma
➢ bikers
• Variable size and calcification
• Mobile
Epididymal Masses
• Cyst, Spermatocele [Figure 3-5-19]
• Infection Epididymal cyst
➢ Bacterial (acute)
➢ TB (chronic)
• Tumors
Figure 3-5-20
➢ Adenomatoid tumor
➢ Papillary cystadenoma (von Hippel-Lindau)
➢ Lymphoma
• Sarcoidosis
Adenomatoid Tumor [Figure 3-5-20]

• Benign
• Most common epididymal tumor
• Solid, small, well-circumscribed
Papillary Cystadenoma
• Associated with VHL (70%)
• 40% bilateral
• Benign
Adenomatoid tumor

Epididymal and Testicular Mass Figure 3-5-21
• Lymphoma
➢ Testicular involvement greater than epididymis
• Sarcoidosis [Figure 3-5-21]
➢ Epididymal involvement greater than testis
➢ More common in Blacks
• Infection
➢ Bacterial (acute)
➢ TB (chronic)
Tuberculosis
• Epididymis primary site with testis secondarily
involved Sarcoidosis with marked epididymal enlargement
• 30% bilateral
• 50% will have abscess or fistulas
Acute Scrotum
• Trauma
• Epididymitis/orchitis
• Torsion
Acute Epididymitis
• Bacterial infection from lower urinary tract - chlamydia, gonococcus, E coli
• US findings - enlarged, hypoechoic, hyperemia, hydrocele, skin thickening
• 20% have associated orchitis
Orchitis
• Usually secondary to epididymitis
• May rarely be focal
• US findings - enlarged, heterogeneous echogenicity, hyperemia
• May lead to focal ischemia/infarction
Fournier Gangrene
• Diabetics or other immunosuppression
• Scrotal abscess with necrotizing infection of the perineum
• Surgical emergency
Torsion
• Gray scale US may be normal early
• Decreased or absent flow with Doppler
➢ Compare with normal side
➢ Venous compromise occurs first
• Look for mass in inguinal canal
• Testis becomes enlarged and hypoechoic with time
Torsion
• < 6 hrs at diagnosis salvage rate 80%-100%
• 12 hr salvage rate 20%
Paratesticular masses
• Varicocele
• Fibrous pseudotumors
• Polyorchidism
• Neoplasms
➢ Lipomas
✧ Half of all spermatic cord tumors
➢ Liposarcoma
➢ Rhabdosarcoma, leiomyosarcoma, MFH
➢ Mesothelioma

Varicocele [Figure 3-5-22] Figure 3-5-22
• > 3mm
• Idiopathic
➢ incompetent valves
➢ more common on left (bilateral 10%)
✧ longer course
✧ more perpendicular insertion
✧ “nutcracker” effect of left renal vein under SMA
• Secondary to abdominal mass
Varicocele
• 15% of general population
• 40% of men with infertility
• ? increased temperature
• Improved pregnancy rates (35%-50%) with repair even if
subclinical
Large varicocele
Fibrous Pseudotumor [Figure 3-5-23]
• Hylanized collagen and granulation tissue Figure 3-5-23
• Attached to tunica albuginea
• US non-specific
• MRI low signal intensity
Polyorchidism
• Abnormal division of genital ridge
• Often abnormal spermatogenesis
• Increased risk of torsion
Paratesticular Neoplasms
• Lipomas [Figure 3-5-24]
• Liposarcoma [Figure 3-5-25]
• Rhabdosarcoma
• Leiomyosarcoma
• MFH
• Mesothelioma
Lipoma [Figure 3-5-24]

• Most common extratesticular neoplasm
• Half of all cord tumors
• Variable by ultrasound Fibrous pseudotumor. Round low-
➢ may be homogenously hypoechoic signal mass involving the tunica
• MR with fat suppression helpful albuginea
Figure 3-5-24
Figure 3-5-25
Hypoechoic lipoma Liposarcoma

Mesothelioma [Figure 3-5-26] Figure 3-5-26
• Tunica vaginalis lined with mesothelial cells
• Much less common then pleural or peritoneal
• Benign and malignant
• Often present with hydrocele
Mesothelioma with nodules and

hydrocele
References
1. Black JA, Patel A. Sonography of the abnormal extratesticular space. AJR Am J Roentgenol 1996; 167:507-511.
2. Black JA, Patel A. Sonography of the normal extratesticular space. AJR Am J Roentgenol 1996; 167:503-506.
3. Bostwick DG. Spermatic cord and testicular adnexa. In: Bostwick DG, Eble JN, eds. Urologic surgcial pathology.
St. Louis: Mosby, 1997; 647-674.
4. Chung JJ, Kim MJ, Lee T, Yoo HS, Lee JT. Sonographic findings in tuberculous epididymitis and epididymo-
orchitis. J Clin Ultrasound 1997; 25:390-394.
5. Cramer BM, Schlegel EA, Thueroff JW. MR imaging in the differential diagnosis of scrotal and testicular disease.
Radiographics 1991; 11:9-21.
6. Doherty FJ. Ultrasound of the nonacute scrotum. Semin Ultrasound CT MR 1991; 12:131-156.
7. Feuer A, Dewire DM, Foley WD. Ultrasonographic characteristics of testicular adenomatoid tumors. J Urol 1996;
155:174-175.
8. Frates MC, Benson CB, DiSalvo DN, Brown DL, Laing FC, Doubilet PM. Solid extratesticular masses evaluated
with sonography: pathologic correlation. Radiology 1997; 204:43-46.
9. Geraghty MJ, Lee FT, Jr., Bernsten SA, Gilchrist K, Pozniak MA, Yandow DJ. Sonography of testicular tumors
and tumor-like conditions: a radiologic-pathologic correlation. Crit Rev Diagn Imaging 1998; 39:1-63.
10. Grebenc ML, Gorman JD, Sumida FK. Fibrous pseudotumor of the tunica vaginalis testis: imaging appearance.
Abdom Imaging 1995; 20:379-380.
11. Heaton ND, Hogan B, Michell M, Thompson P, Yates-Bell AJ. Tuberculous epididymo-orchitis: clinical and
ultrasound observations. Br J Urol 1989; 64:305-309.
12. Horstman WG, Middleton WD, Melson GL. Scrotal inflammatory disease: color Doppler US findings. Radiology
1991; 179:55-59.
13. Kassis A. Testicular adenomatoid tumours: clinical and ultrasonographic characteristics. BJU Int 2000; 85:302-
304.
14. Kim ED, Lipshultz LI. Role of ultrasound in the assessment of male infertility. J Clin Ultrasound 1996; 24:437-
453.
15. Kutchera WA, Bluth EI, Guice SL. Sonographic findings of a spermatic cord lipoma. Case report and review of the
literature. J Ultrasound Med 1987; 6:457-460.
16. Mattrey RF. Magnetic resonance imaging of the scrotum. Semin Ultrasound CT MR 1991; 12:95-108.
17. Ragheb D, Higgins JL, Jr. Ultrasonography of the scrotum: technique, anatomy, and pathologic entities. J
Ultrasound Med 2002; 21:171-185.
18. Sudakoff GS, Quiroz F, Karcaaltincaba M, Foley WD. Scrotal ultrasonography with emphasis on the
extratesticular space: anatomy, embryology, and pathology. Ultrasound Q 2002; 18:255-273.
19. Tessler FN, Tublin ME, Rifkin MD. Ultrasound assessment of testicular and paratesticular masses. J Clin
Ultrasound 1996; 24:423-436.
20. Woodward PJ, Schwab CM, Sesterhenn IA. From the archives of the AFIP: extratesticular scrotal masses:
radiologic-pathologic correlation. Radiographics 2003; 23:215-240.
21. Woodward PJ, Sohaey R, O'Donoghue MJ, Green DE. From the Archives of the AFIP: Tumors and Tumorlike
Lesions of the Testis: Radiologic-Pathologic Correlation. Radiographics 2002; 22:189-216.

First Trimester Ultrasound
Ovarian Period: (Weeks 1–2)

• Ovarian follicle matures
• Ovulation
• Corpus luteum formation Figure 3-6-1
Conceptus Period: (Week 3–5)
• Fertilization
• Morula (16 cells)
• Blastocyst
• Trilaminar embryo
Embryonic Period: (Weeks 6–10)

• C-shaped embryo
• Major organs develop
• Yolk sac detaches
Intradecidual sac sign
Fetal Period: (Weeks 11–12)
• Fetal growth
• Amniotic and chorionic membranes approach each other
Gestational Sac
• Visualized as early as 4 - 4.5 wks (TV)
• Intradecidual sac sign [Figure 3-6-1]
• Double decidual sac sign [Figure 3-6-2]
➢ Basalis [DB]
➢ Capsularis [DC]
➢ Parietalis [DP]
Gestational Sac = Chorionic Sac

The sac is eccentrically located with
• Chorionic laeve = Smooth chorion = Chorionic membrane respect to the endometrial cavity
• Chorionic frondosum + Decidua basalis = Placenta
Figure 3-6-2
Figure 3-6-3
Double decidual sac sign
Chorionic sac with chorionic

frondosum and laeve (smooth
chorion)
First Trimester Ultrasound 594 Genitourinary Radiology

Figure 3-6-4
Series of 5 illustrations showing
normal 1st trimester development
with expansion of the amnion and
detachment of the yolk sac
a b
c d
Genitourinary Radiology 595 First Trimester Ultrasound

Yolk sac [Figure 3-6-5 ] Figure 3-6-5
• Visualized at 5 – 5.5 weeks
Embryo [Figure 3-6-6 ]

• Visualized 6 – 6.5 wks
Major First Trimester Landmarks [Figures 3-6-7 and 3-6-8]

• Gestational sac 4.5 wks
• Yolk sac 5.5 wks
• Embryo 6.5 wks
Figure 3-6-6
Multiple Gestations Normal yolk sac at 5.5 wks
• Types of Twinning
➢ Dizygotic (70%)
✧ 2 eggs Figure 3-6-7
➢ Monozygotic (70%)
✧ single egg
Multiple Gestations
• # of chorions equals
# of placentas
➢ sharing is bad
➢ risk for twin/twin Normal 6.5 week embryo with
transfusion “double bleb” or “diamond ring” sign
• # of amnions equals
# of separate sacs 8 week embryo surrounded by
➢ sharing is really bad amnion
➢ risk for cord accidents
Dizygotic Twins [Figure 3-6-9] Figure 3-6-8

• Dizygotic must be dichorionic (2 placentas)
and diamniotic (2 sacs)
Figure 3-6-9
Normal first trimester US including rhombencephalon

and bowel herniation
Dichorionic, diamniotic twins

Monozygotic Twins Figure 3-6-10
• 1/3 are Dichorionic/Diamniotic [Figure 3-6-10]
➢ cleavage by day 3
• 2/3 are Monochorionic/Diamniotic [Figures 3-6-11 and 3-6-12]
➢ cleavage day 4-8
• Rare (approx 1%) Monochorionic/Monoamniotic [Figure 3-6-13]]
➢ cleavage > 8 days
• Conjoined twins
➢ cleavage > 14 days
Figures 3-6-11 and 3-6-12
Dichorionic, diamniotic twins
Figure 3-6-13
Monochorionic, monoamniotic twins
Monochorionic, diamniotic twins
AIUM Guidelines: First Trimester

• Gestational sac
➢ Location
➢ Mean Sac Diameter (MSD)
MSD = (L+W+D)/3
➢ Yolk sac
• Embryo
➢ Crown rump length
➢ Cardiac activity
• Fetal number
➢ Chorionicity/Amnionicity
• Uterus, adnexa, cul-de-sac
• Threshold Level - the size at which a finding may be seen
• Discriminatory Level - the size at which a finding must be seen

Threshold Discriminatory
Level – MSD Level – MSD
• Gestational Sac 2 mm 5 mm
• Yolk Sac TV 4 mm TV10 mm
TA 20 mm
• Embryo TV 8 mm TV 18 mm
TA 25 mm
• Heartbeat 2 mm (CRL) 5 mm (CRL)
Major: Discriminators
• MSD > 10 mm must have a yolk sac
• MSD > 18 mm must have an embryo
• CRL > 5mm must have a heartbeat
Cardiac Activity
• Must be present if embryo is > 5 mm
• 5–6 weeks 100–110 bpm
• 8–9 weeks 150–170 bpm
Abnormal Frist Trimester

• 25% threatened abortion
• Embryonic demise
• Bradycardia
• Anembryonic pregnancy [Figure 3-6-14]
• Perigestational hemorrhage [Figure 3-6-15]
• Abnormal yolk sac
• Poor growth Figure 3-6-14
Anembryonic Pregnancy [Figure 3-6-14]

• Major discriminators
➢ MSD > 10 mm without a yolk sac
➢ MSD > 18 mm without a fetal pole
• Minor discriminators
➢ weak decidual reaction
➢ abnormal shape or location
➢ empty amnion
Yolk Sac
• First landmark in gestational sac
• In the chorionic cavity
• Abnormal findings: Anembryonic pregnancy
➢ >6mm with empty amnion
➢ irregular shape
➢ calcifications
➢ multiple yolk sacs
Growth Figure 3-6-15

• Normal growth rate 1 mm per day
Ectopic pregnancy
• Tubal 95%
• Unusual locations 5%
➢ Interstitial
➢ Cervix
➢ Ovary
➢ Abdominal
• 1:50-1:200 live births
• Risks factors: IUD, prior ectopic, PID, tubal surgery,
infertility treatment
Perigestational hemorrhage

Ectopic pregnancy: Uterine Findings Figure 3-6-16
[Figures 3-6-16 and 3-6-17]
• No gestational sac
• Thickened endometrium
• Pseudogestional sac
• Discriminatory hCG levels
➢ hCG >1,000 IU/L (2nd IS)
➢ hCG >2,000 IU/L (3rd IRP)
Figure 3-6-17
Double decidual sac sign vs. pseudosac
Figure 3-6-18
Ectopic with echogenic ring

Pseudosac and blood in the cul-de-sac
Ectopic Pregnancy: Adnexal Findings
[Figure 3-6-18]
• Living extrauterine embryo
• Echogenic ring +/- yolk sac
➢ “ring of fire”
• Adnexal mass (clot)
• Cul-de-sac blood Figure 3-6-19
• Normal adnexa
Heterotopic Pregnancy
• 1 in 30,000 spontaneous pregnancies
• 1 in 4,000 assisted pregnancies
Interstitial Pregnancy [Figure 3-6-19]

• Isthmus
• Rupture later – catastrophic bleeding
• Eccentric
• Lack of encircling myometrium
• Interstitial line sign
Cornual ectopic with interstitial line sign

Management of Ectopic Pregnancy
• Surgical
➢ Salpingectomy
➢ Salpingostomy
• Medical
➢ Systemic methotrexate
➢ Intragestational methotrexate
➢ Intragestational KCI
Systemic Methotrexate
• Preserves fallopian tube
• Non-invasive
• Outpatient
• Criteria
➢ Mass < 4cm
➢ No bleeding Figure 3-6-20
➢ hCG <3,000 IU/L (2IS)
➢ No formed fetal parts
Cutting Edge
• Sonoembryology
• Early diagnosis of major malformations
• Screen for aneuploidy
➢ nuchal translucency
➢ hypoplastic nasal bone
➢ abnormal flow in ductus venosus
Nuchal Translucency
Screen for Trisomy 21 [Figure 3-6-20]
• Accredited lab
• 11-14 weeks
Increased nuchal translucency in
• >3mm abnormal
Down syndrome
• Risk assessment based on age, NT, serum screen
• High detection rates (75%-90%)
References
1. Ackerman TE, Levi CS, Dashefsky SM, Holt SC, Lindsay DJ. Interstitial line: sonographic finding in interstitial
(cornual) ectopic pregnancy. Radiology 1993; 189:83-87.
2. Brown DL, Emerson DS, Felker RE, Cartier MS, Smith WC. Diagnosis of early embryonic demise by endovaginal
sonography. J Ultrasound Med 1990; 9:631-636.
3. Brown DL, Doubilet PM. Transvaginal sonography for diagnosing ectopic pregnancy: positivity criteria and
performance characteristics. J Ultrasound Med 1994; 13:259-266.
4. Dickey RP, Olar TT, Curole DN, Taylor SN, Matulich EM. Relationship of first-trimester subchorionic bleeding
detected by color Doppler ultrasound to subchorionic fluid, clinical bleeding, and pregnancy outcome. Obstet Gynecol
1992; 80:415-420.
5. Frates MC, Brown DL, Doubilet PM, Hornstein MD. Tubal rupture in patients with ectopic pregnancy: diagnosis
with transvaginal US. Radiology 1994; 191:769-772.
6. Frates MC, Benson CB, Doubilet PM, et al. Cervical ectopic pregnancy: results of conservative treatment. Radiology
1994; 191:773-775.
7. Frates MC, Laing FC. Sonographic evaluation of ectopic pregnancy: an update. AJR Am J Roentgenol 1995; 165:251-
259.
8. Jarjour L, Kletzky OA. Reliability of transvaginal ultrasound in detecting first trimester pregnancy abnormalities.
Fertil Steril 1991; 56:202-207.
9. Jurkovic D, Gruboeck K, Campbell S. Ultrasound features of normal early pregnancy development. Curr Opin Obstet
Gynecol 1995; 7:493-504.
10. Nyberg DA, Mack LA, Laing FC, Patten RM. Distinguishing normal from abnormal gestational sac growth in early
pregnancy. J Ultrasound Med 1987; 6:23-27.

11. Nyberg DA, Filly RA, Laing FC, Mack LA, Zarutskie PW. Ectopic pregnancy. Diagnosis by sonography correlated
with quantitative HCG levels. J Ultrasound Med 1987; 6:145-150.
12. Oh JS, Wright G, Coulam CB. Gestational sac diameter in very early pregnancy as a predictor of fetal outcome.
Ultrasound Obstet Gynecol 2002; 20:267-269.
13. Rempen A. Diagnosis of viability in early pregnancy with vaginal sonography. J Ultrasound Med 1990; 9:711-716.
14. Sohaey R, Woodward P, Zwiebel WJ. First-trimester ultrasound: the essentials. Semin Ultrasound CT MR 1996; 17:2-
14.
15. van Leeuwen I, Branch DW, Scott JR. First-trimester ultrasonography findings in women with a history of recurrent
pregnancy loss. Am J Obstet Gynecol 1993; 168:111-114.

Fetal CNS Malformations
AIUM: Fetal Brain [Figure 3-7-1]

• Ventricular Plane Figure 3-7-1
➢ atrium and choroid
• BPD Plane
➢ thalami
➢ third ventricle
➢ cavum septi pellucidi
• Posterior Fossa
➢ cerebellum
➢ cisterna magna
➢ nuchal skin thickeness
Fetal MRI
• Fast T2WI (SSFSE, HASTE)
• Safety issues
➢ No known deleterious effects
➢ Do not perform in the first trimester
➢ Do not give gadolinium
➢ Obtain informed consent
Congenital CNS Malformations

• Dorsal Induction
➢ anencephaly 3 required images of the fetal brain
➢ encephalocele
➢ spina bifida
• Ventral Induction
➢ holoprosencephaly
➢ Dandy-Walker malformation
• Neuronal Proliferation
➢ microcephaly
➢ macrocephaly
➢ tumors
• Migration
➢ agenesis of corpus callosum
There is too much fluid in there

• Hydrocephalus
• Holoprosencephaly
• Hydranencephaly
Hydranencephaly
• Absent cerebral hemispheres
• Occlusion of ICA -? etiology
➢ infection
➢ vasculitis
➢ emboli
• Falx
• Normal facial development
Fetal CNS Malformations 602 Genitourinary Radiology

Holoprosencephaly [Figures 3-7-2 to 3-7-4] Figure 3-7-2
• Spectrum of arrested development
➢ alobar
➢ semilobar
➢ lobar
• Absent cavum, absent falx, fused thalami, dorsal sac
• Midline facial defects
➢ proboscis
➢ cyclopia
➢ midline cleft
• Trisomy 13
Figure 3-7-3
Alobar, semilobar lobar
holoprosencephaly
compared to normal.
Alobar holoprosencephaly with single ventricle Figure 3-7-4
Hydrocephalus
• Dilated ventricles and enlarged head
Ventriculomegaly
• Dilated ventricles
Signs
• Lateral ventricle > 10mm
• Medial ventricular wall to choroid > 3mm
• Dangling choroid
Hydrocephalus: Differential Semilobar holoprosencephaly with a dorsal sac.

• Aqueductal Stenosis Face shows a midline cleft.
• Dandy-Walker Malformation
• Chiari II Figure 3-7-5
• Communicating Hydrocephalus
Aqueductal Stenosis [Figures 3-7-5]

• Block at aqueduct of Sylvius
• Most common cause of hydrocephalus
• Male predominance (X-linked form)
Aqueductal stenosis with dilatation

of the lateral and third ventricles.
Genitourinary Radiology 603 Fetal CNS Malformations

Dandy-Walker Malformation [Figures 3-7-6 and 3-7-7]
• Communicating PF cyst
• Hydrocephalus +/-
• 50 have accociated abnormality
Figure 3-7-6
Figure 3-7-7
Dandy-Walker Malformation with

Dandy-Walker enlargement of the 4th ventricle and
Malformation posterior fossa cyst.
Chiari II [Figures 3-7-8 to 3-7-10]

• Downward herniation of the 4th ventricle and vermis
• Myelomeningocele
• Hydrocephalus Figure
• “Lemon” and “Banana” sign Normal Chiari II
3-7-8
3-7-9
3-7-10
Normal vs. Chiari II

Communicating Hydrocephalus [Figure 3-7-11] Figure 3-7-11
• Dilatation of all ventricles and subarachnoid space
• Etiology
➢ hemorrhage
➢ ? abnormal arachnoid granulations
➢ ? abnormal superior sagittal sinus
Hydrocephalus: Differential
• Aqueductal Stenosis
• Dandy-Walker Malformation
• Chiari II
• Communicating Hydrocephalus
Agenesis of the Corpus Callosum [Figure 3-7-12]

• Tear-dropped shaped ventricules (Colpocephaly)
• Absent cavum septi pellucidi
• Associated with lipomas and arachnoid cysts
• Often missed or confused with mild ventriculomegaly
Communicating Hydrocephalus
Neural Tube Defects
• Anencephaly
• Spina Bifida Figure 3-7-12
• Encephalocele
• Acrania
Anencephaly [Figure 3-7-13]

• Absent cranium and cerebral hemispheres
• Area cerebrovasculosa
Figure 3-7-13
Agenesis of the CC with colpocephaly

and arachnoid cyst
Figure 3-7-14
1st trimester anencephaly
Encephalocele [Figure 3-7-14]

• 75% occipital
• Frontal – Southeast Asia
• Evaluate brain tissue
• 80% have associated malformations
Choroid Plexus Cysts

• 30% of trisomy 18
• 1%–2% of normals
• 1/500 chance of trisomy 18
Occipital encephalocele
Genitourinary Radiology 605 Fetal CNS Malformations

Trisomy 18 [Figure 3-7-15]
• Overlapping Fingers
• Cardiac Defects
• Omphalocele/Diaphragmatic Hernia
• Choroid plexus cysts
Figure 3-7-15
Trisomy 18 with overlapping fingers
References
1. Chang MC, Russell SA, Callen PW, Filly RA, Goldstein RB. Sonographic detection of inferior vermian agenesis in
Dandy-Walker malformations: prognostic implications. Radiology 1994; 193:765-770.
2. Chatzipapas IK, Whitlow BJ, Economides DL. The 'Mickey Mouse' sign and the diagnosis of anencephaly in early
pregnancy. Ultrasound Obstet Gynecol 1999; 13:196-199.
3. Coleman BG, Adzick NS, Crombleholme TM, et al. Fetal therapy: state of the art. J Ultrasound Med 2002; 21:1257-
1288.
4. d'Ercole C, Girard N, Cravello L, et al. Prenatal diagnosis of fetal corpus callosum agenesis by ultrasonography and
magnetic resonance imaging. Prenat Diagn 1998; 18:247-253.
5. Ghidini A, Strobelt N, Locatelli A, Mariani E, Piccoli MG, Vergani P. Isolated fetal choroid plexus cysts: role of
ultrasonography in establishment of the risk of trisomy 18. Am J Obstet Gynecol 2000; 182:972-977.
6. Goldstein RB, LaPidus AS, Filly RA. Fetal cephaloceles: diagnosis with US. Radiology 1991; 180:803-808.
7. Johnson SP, Sebire NJ, Snijders RJ, Tunkel S, Nicolaides KH. Ultrasound screening for anencephaly at 10-14 weeks
of gestation. Ultrasound Obstet Gynecol 1997; 9:14-16.
8. Levitsky DB, Mack LA, Nyberg DA, et al. Fetal aqueductal stenosis diagnosed sonographically: how grave is the
prognosis? AJR Am J Roentgenol 1995; 164:725-730.
9. McGahan JP, Nyberg DA, Mack LA. Sonography of facial features of alobar and semilobar holoprosencephaly. AJR
Am J Roentgenol 1990; 154:143-148.
10. Pilu G, Romero R, Rizzo N, Jeanty P, Bovicelli L, Hobbins JC. Criteria for the prenatal diagnosis of holoprosencephaly.
Am J Perinatol 1987; 4:41-49.
11. Ulm B, Ulm MR, Deutinger J, Bernaschek G. Dandy-Walker malformation diagnosed before 21 weeks of gestation:
associated malformations and chromosomal abnormalities. Ultrasound Obstet Gynecol 1997; 10:167-170.
12. Vergani P, Ghidini A, Strobelt N, et al. Prognostic indicators in the prenatal diagnosis of agenesis of corpus callosum.
Am J Obstet Gynecol 1994; 170:753-758.

Fetal Body Anomalies
Paula J. Woodward, M.D.
Neck Masses
• Neural Tube Defects Figure 3-8-1
• Cystic Hygroma
• Teratoma (Epignathus)
• Thyroid
Cystic Hygroma [Figure 3-8-1]

• Lymphangioma
• Chromosomal Abnormalities
➢ Turners Syndrome XO
➢ Trisomy 21 (2nd trimester nuchal thickening) Sagittal and transverse images through the fetal
• Often associated with hydrops neck show a cystic hygroma
Iniencephaly [Figure 3-8-2]

• Fixed hyperextension of neck (“stargazer”
position) Figure 3-8-2 Figure 3-8-3
• Rachischisis
• Cephalocele
• Shortened or absent vertebral bodies
• First trimester
➢ head appears large
➢ CRL less than expected
AIUM: Chest [Figures 3-8-4 and 3-8-5]

• Four chamber heart
➢ side (stomach and heart both on left)
➢ axis ~35-45° First trimester scan of Epignathus (teratoma)
➢ equal chamber size iniencephaly shows a
➢ excludes 90% of cardiac defect hyperextended head and
• LVOT, RVOT if feasible short body
Figure 3-8-5
Figure 3-8-4
Four-chamber view of normal heart Left ventricular outflow tract and

right ventricular outflow tract
Genitourinary Radiology 607 Fetal Anomalies

Hypoplastic Left Heart [Figure 3-8-6] Figure 3-8-6
• Lethal in neonate if untreated
➢ Norwood or transplant
• Small or invisible LV
• Hypoplastic aortic arch
• RA < LA
• Consider Turner syndrome in female fetuses
Chest Masses
• Congenital Diaphragmatic Hernia
• Cystic Adenomatoid Malformation
• Extralobar Sequestration
• Teratoma
Congenital Diaphragmatic Hernia [Figure 3-8-7]

• 90% left-sided through foramen of Bochdalek
• 50% have other anomalies
• Pulmonary hypoplasia Hypoplastic left ventricle
• “Liver-up” poor prognosis
Cystic Adenomatoid Malformation [Figure 3-8-8] Figure 3-8-7

• Lung Hamartoma
• Types I – III
• Fetal CCAMs classified as micro- or macro cystic
• Arterial supply from pulmonary artery
• May spontaneously regress
• In utero surgery for hydrops
Figure 3-8-8
Congenital diaphragmatic hernia

with deviation of the heart
Figure 3-8-9
Sagittal scan of the fetal chest and neonatal CXR
showing type II CCAM
Extralobar Sequestration [Figure 3-8-9]

• Non-communicating (sequestered) lung segment
• Arterial supply from aorta
• 90% left sided
• 10% below diaphragm
AIUM: Abdomen
• Stomach
• Kidneys
• Bladder
• UC insertion site Extralobar sequestration with feeding vessel from
• Umbilical cord vessel number the aorta.
Amniotic Fluid Balance

Production Removal
Fetal/ Embryo plasma Intramembranous
volume Transmembranous
Uterine Perfusion Metanephros (>10 wks) Swallowing
Lungs Lungs
Fetal Anomalies 608 Genitourinary Radiology

Polyhydramnios Figure 3-8-10
• 2/3 idiopathic
• 1/3 definable cause
➢ macroscomia
➢ GI obstruction
➢ CNS malformation
➢ hydrops
Oligohydramnios
• Never normal
• A “DRIP” of fluid
➢ Demise Double bubble, oblique view confirms
➢ Renal, also bladder duodenal atresia
often anhydramnios
➢ IUGR Figure 3-8-11
➢ PROM, post dates
Fetal GI Tract
• Atresias
• Abdominal Wall Detect
Gastroschisis
Atresias
• Esophageal
• Duodenal
• Small Bowel
• Anorectal
Esophageal Atresia
• Stomach may be present but small
• Polyhydramnios after 20 wks Figure 3-8-12
• IUGR common
➢ ingested fluid important for nutrition
Double Bubble [Figure 3-8-10]

• Duodenal Atresia
➢ 30% have trisomy 21
➢ 50% have other structural abnormalities
• Ladd’s bands, annular pancreas usually do not present in utero
Jejunal/Ileal Atresia
• 1/3 have cystic fibrosis
• 5%-10% may perforate Omphalocele
• Meconium peritonitis
➢ ascites Figure 3-8-13
➢ calcifications
➢ pseudocyst formation
Bowel
• Normal bowel herniation at 8 weeks
• Rotates counterclockwise 270°
• Returns to abdomen in 12 weeks
Abdominal Wall Defects

• Gastroschisis [Figure 3-8-11]
• Omphalocele [Figure 3-8-12 and 3-8-13]
• Limb-body-wall defect
Gastroschisis Omphalocele
• Location Right Central
• Membrane No Yes
• Cord Insertion NL On sac
• Associated Anomalies No 50-75%
Omphalocele

Limb-Body-Wall Defect (Body Stalk Anomaly) Figure 3-8-14
• Fetus attached to placenta
• Absent or short umbilical cord
• Severe (lethal) malformation
• Scoliosis common
Renal Anomalies [Figure 3-8-14]

• Agenesis
• Renal Cystic Disease
• Hydronephrosis
• Masses
Renal Cystic Disease

• Autosomal recessive polycystic kidney disease [Figure 3-8-15]
a) Normal vs. b) renal agenesis
• Multicystic dysplastic kidneys [Figures 3-8-16 and 3-8-17]
• Cystic dysplasia due to obstruction Figure 3-8-15
• Autosomal dominant polycystic kidney disease
Associations
• VACTERL Syndrome
➢ Vertebral, anal atresia, cardiac, TE fistula, renal, limb
• Inherited Disorders
➢ Meckel-Gruber (renal cystic dysplasia, encephalocele,
polydactyly)
• Chromosomal Abnormalities
➢ Trisomy 13
Autosomal recessive polycystic
Hydronephrosis kidney disease
• UPJ Obstruction
• UVJ Obstruction
• Duplications Figure 3-8-16
• PUV, Urethral Atresia
• Reflux
Hydronephrosis [Figure 3-8-18]

• Renal Pelvis
➢ > 4 mm before 33 weeks
➢ > 7 mm after 33 weeks
• AP pelvis diam/AP kidney diam >50%
• Calyceal Dilatation
• Any degree of dilatation when accompanied by cystic renal
changes
Multicystic dysplastic kidney
Figure 3-8-17
Figure 3-8-18
Bilateral MCDK UPJ obstruction

Retroperitoneal Masses
• Renal Cystic Disease
• Renal Tumors
➢ Mesoblastic Nephroma
➢ Wilms Tumor
• Adrenal
Figure 3-8-19
➢ Neuroblastoma
➢ Hemorrhage
• Extralobar Sequestration
Cystic Abdominal/Pelvic Collections

• Bladder Obstruction
• Dilated Bowel
• Cysts
➢ Ovarian
➢ Duplication
➢ Mesenteric
➢ Choledochal
➢ Meconium pseudocyst
Posterior Urethral Valves [Figures 3-8-19 and 3-8-20]

• Bladder “funnels” into dilated posterior urethra Posterior urethral valves
• Oligohydramnios common
• Renal dysplasia (echogenic cystic kidneys) bad prognostic sign
Figure 3-8-20
Figure 3-8-21
Severe posterior urethral valves
with oligohydramnios
Ovarian Cyst [Figure 3-8-21]

• Most common cyst in 3rd trimester
• Anywhere in abdomen
• Complexity suggests torsion or hemorrhage
• Resolve by 6 mos
Sacral Mass
• Sacrococcygeal teratoma
• Myelomeningocele
Ovarian cyst

Sacrococcygeal Teratoma [Figures 3-8-22 and 3-8-23] Figure 3-8-23
• Solid, cystic, or mixed
• Location
➢ Type 1: completely external
➢ Type 2: external and internal into pelvis
➢ Type 3: external and internal into abdomen
➢ Type 4: completely internal
Figure 3-8-22
Sacrococcygeal teratoma
Solid sacrococcygeal teratoma

with marked growth
References
1. Leung JW, Coakley FV, Hricak H, et al. Prenatal MR imaging of congenital diaphragmatic hernia. AJR Am J
Roentgenol 2000; 174:1607-1612.
2. Coleman BG, Adzick NS, Crombleholme TM, et al. Fetal therapy: state of the art. J Ultrasound Med 2002;
21:1257-1288.
3. Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ. Fetal lung lesions: management and
outcome. Am J Obstet Gynecol 1998; 179:884-889.
4. Lopoo JB, Goldstein RB, Lipshutz GS, Goldberg JD, Harrison MR, Albanese CT. Fetal pulmonary sequestration: a
favorable congenital lung lesion. Obstet Gynecol 1999; 94:567-571.
5. Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA. Intestinal atresia and stenosis: a
25-year experience with 277 cases. Arch Surg 1998; 133:490-496; discussion 496-497.
6. Nyberg DA, Resta RG, Luthy DA, Hickok DE, Mahony BS, Hirsch JH. Prenatal sonographic findings of Down
syndrome: review of 94 cases. Obstet Gynecol 1990; 76:370-377.
7. Corteville JE, Gray DL, Langer JC. Bowel abnormalities in the fetus--correlation of prenatal ultrasonographic
findings with outcome. Am J Obstet Gynecol 1996; 175:724-729.
8. Stringer MD, McKenna KM, Goldstein RB, Filly RA, Adzick NS, Harrison MR. Prenatal diagnosis of esophageal
atresia. J Pediatr Surg 1995; 30:1258-1263.
9. Meizner I, Levy A, Katz M, Maresh AJ, Glezerman M. Fetal ovarian cysts: prenatal ultrasonographic detection and
postnatal evaluation and treatment. Am J Obstet Gynecol 1991; 164:874-878.
10. Muller-Leisse C, Bick U, Paulussen K, et al. Ovarian cysts in the fetus and neonate--changes in sonographic
pattern in the follow-up and their management. Pediatr Radiol 1992; 22:395-400.
11. Hutton KA, Thomas DF, Davies BW. Prenatally detected posterior urethral valves: qualitative assessment of
second trimester scans and prediction of outcome. J Urol 1997; 158:1022-1025.
12. James CA, Watson AR, Twining P, Rance CH. Antenatally detected urinary tract abnormalities: changing incidence
and management. Eur J Pediatr 1998; 157:508-511.
13. Abuhamad AZ, Horton CE, Jr., Horton SH, Evans AT. Renal duplication anomalies in the fetus: clues for prenatal
diagnosis. Ultrasound Obstet Gynecol 1996; 7:174-177.

14. Pryde PG, Bardicef M, Treadwell MC, Klein M, Isada NB, Evans MI. Gastroschisis: can antenatal ultrasound
predict infant outcomes? Obstet Gynecol 1994; 84:505-510.
15. Luton D, De Lagausie P, Guibourdenche J, et al. Prognostic factors of prenatally diagnosed gastroschisis. Fetal
Diagn Ther 1997; 12:7-14.
16. Getachew MM, Goldstein RB, Edge V, Goldberg JD, Filly RA. Correlation between omphalocele contents and
karyotypic abnormalities: sonographic study in 37 cases. AJR Am J Roentgenol 1992; 158:133-136.
17. Salihu HM, Boos R, Schmidt W. Omphalocele and gastrochisis. J Obstet Gynaecol 2002; 22:489-492.

Cystic Diseases of the Kidney
Peter L.Choyke, MD
Cystic Disease of the Kidney

• Autosomal Dominant Polycystic Kidney Disease (ADPKD)
• Autosomal Recessive Polycystic Kidney (ARPKD)
• Tuberous Sclerosis Complex (TS or TSC)
• Von Hippel-Lindau Disease (VHL)
• Acquired Cystic Kidney Disease (ACKD) Figure 3-9-1
ADPKD
• Occurs in 1:1000 Individuals
• Genetics: Autosomal Dominant
• ESRD in 50%
• Risk of Cancer = Not increased
Types of ADPKD Illustration of the microvilli

• PKD 1 (85-95%) on the surface of lumenal
➢ 16p13.3 cells that are abnormal in
➢ Polycystin I ADPKD
➢ Mean age of ESRD=55y
• PKD 2 (~5% )
➢ 4q21–23
➢ Polycystin II
➢ Mean age of ESRD= 71.5y
• PKD3? PKD4?
Mechanism [Figure 3-9-1]
Clinical Manifestations
• Pain
• Hypertension
• Infection (Women > Men)
• Stones
• Loss of Renal Function
• Renal Failure
Imaging [Figures 3-9-2 to 3-9-5]
Figure 3-9-2
Figure 3-9-3
Retrograde pyelogram in Montage of ultrasounds at different stages of

ADPKD life. From upper left clockwise: 14 years, mid
twenties, mid fifies, mid sixties
Cystic Diseases of the Kidney 614 Genitourinary Radiology

Montage of CT scans of different patients at different stages of

their disease
MRI of ADPKD
Complications [Figure 3-9-6] Figure 3-9-6
• Acute Infection
Manifestations of ADPKD
• Intracranial Aneurysms
• Cardiovascular Disease: Mitral, Aortic
valve, aortic aneurysm
• Cysts: Hepatic, Pancreatic, Spleen
• Diverticula: Colon
Intracranial Aneurysms [Figure 3-9-7] Gas forming organism requiring percutaneous drainage
• ICA
➢ 18–26% of ADPKD
Figure 3-9-7
➢ Rupture ~2–11%
➢ 46–61% Mortality Rate
➢ Mean age 39–47 years of rupture
• Screening (MRA) performed periodically in patients
with ADPKD
Extrarenal Cysts
• Occur in 70–75% of ADPKD
• Complications (liver):
➢ Pain
➢ Biliary Obstruction
➢ IVC compression
Dissecting aneurysm of the abdominal aorta in
Screening ADPKD
• US Screening begins in teenage years
• ~ 2/3 of affected children will show cysts Figure 3-9-8
between 11–20
• ~ 95% by age 30
Localized PKD [Figure 3-9-8]

• Unilateral, segmental
• Non Hereditary
➢ Possible “mosaic” form of ADPKD
Two cases of unilateral PKD
Genitourinary Radiology 615 Cystic Diseases of the Kidney

Autosomal Recessive Polycystic Kidney Disease (ARPKD) Figure 3-9-9
[Figures 3-9-9 and 3-9-10]
• Prevalence: Variable 1:6000-50,000
• Genetics: Autosomal Recessive
• ESRD: >50%
• Cancer: None
• Unrelated to ADPKD
• Congenital Hepatic Fibrosis
• Caroli’s, Biliary Cystic Disease
• Renal ductal ectasia, abnormal cilia
➢ Enlarged echogenic kidneys
➢ Infantile Form: Renal Failure leading to Oligohydramnios,
Pulmonary dysplasia
➢ Childhood form: Portal Hypertension, Caroli’s, Renal
enlargement, Late renal failure
Mechanism: Disease of Microvilli
Tuberous Sclerosis
• Prevalence: 1:10,000
• Genetics: Autosomal Dominant**
• Produces hamartomas throughout the body:
• ESRD: 15% (cystic/AML bleeding)
• Risk of Cancer: 1-2% (slight increase)
➢ ** mostly new mutations; not hereditary
Types of TSC
• TSC 1
➢ 9q34
➢ ~1/3 TSC
➢ “Hamartin”
➢ Assoc with severe MR
• TSC 2
➢ 16p 13.3 !!
➢ 2/3 of TSC Typical appearance of increased
➢ “Tuberin” echogencity within the kidneys and
➢ Assoc with worse renal disease increased signal on T2W MRI in
ARPKD
Pathogenesis
• Tuberous= nodular, Sclerosis= hard
➢ Skin: Adenoma Sebaceum, Angiofibromas
➢ CNS: Tubers, Subependymal nodules, Giant Cell Astrocytoma
➢ Kidneys: Cysts, Angiomyolipomas
➢ Heart: Rhabdomyomas
➢ Lungs: Lymphangiomyomatosis (LAM)
➢ Bone: Islands
Figure 3-9-10
Biliary cystic dilation in ARPKD

Cyst Predominant Forms of TSC Figure 3-9-11
[Figure 3-9-11]
Renal Involvement
[Figure 3-9-12]
• Angiomyolipoma predominant
➢ Mild to severe
➢ Risk of Hemorrhage
➢ Treat conservatively
❖ Partial nephrectomy
❖ Angioembolization
❖ Radiofrequency ablation
Non Fatty Angiomyolipoma [Figure 3-9-13]
Renal Manifestations
• Carcinoma of the Kidney
➢ 1–2% of TS patients Cystic predominant form of TSC
➢ Heterogenous solid lesions
➢ Faster growing than AMLs Figure 3-9-12
➢ No screening recommendations
Von Hippel Lindau Disease

[Figures 3-9-14 to 3-9-17]
• Prevalence ~ 1:35,000 to 1:45,000
• Genetics: Autosomal Dominant
• ESRD: < 5% Usually due to nephrectomy
• Risk of Cancer: 30-40%
• Target Organs
➢ CNS, Retina --Hemangioblastomas
➢ Kidney--Cysts and Cancers
➢ Pancreas-- Cysts and
Neuroendocrine tumors
➢ Adrenal--Pheochromocytomas
➢ Epididymis/ Broad Ligament–
Cystadenomas
Renal Manifestations Examples of angiogmyolipomas in TSC at differing degress of

• Multiple Cysts severity
➢ Virtually all will have neoplastic clear
cell lining
• Cysts containing tumors
• Solid (Clear Cell) tumors
Management
Risk of Metastases - 3 cm rule - Risk of Renal Failure
Figure 3-9-13
Illustrates features of non fatty angiomyolipomas; hyperdense lesions that enhance uniformly

CNS hemangioblastomas in VHL
Figure 3-9-15
Solid islet cell or pancreatic

neuroendocrine tumors in VHL
Figure 3-9-17
Cystic lesions of the pancreas in VHL
Radiofrequency Ablation [Figure 3-9-18]
Acquired Cystic Kidney Disease

• Rate: up to 100% of dialysis pts
• Genetics: None
• ESRD: 100%
• Risk of Cancer: Increases with duration
Pathogenesis
• Theory 1: Dialysis Toxin
• Theory 2: Uremic Milieu Bilateral pheochromocytomas
➢ Mutations which lead to cysts, adenomas, tumors and
metastatic cancers
Figure 3-9-18
Successful treatment with RFA in patient with VHL

Renal Cancer in ACKD [Figure 3-9-19] Figure 3-9-19
• 10–50 Fold Risk of RCC
➢ Mean dialysis duration 8 yrs
➢ Multifocal & Bilateral
➢ 17% Risk of Metastases
Screening
• “Screening is not routinely justified”
➢ Relatively low risk of cancer
➢ High risk of dying from other causes
➢ Reserve screening for pts with good long term prognosis
Levine E, Abdom Imaging 1995 20:569-71
ACKD-RCC After Transplant

• Transplantation
➢ Cysts Regress
➢ New Tumor Formation Decreases
➢ Increased Risk of Metastases from Existing RCC –
Immunosuppression
Take Home Points

• When confronted with a “polycystic kidney”
➢ Pure cysts ? Aneurysms ? ADPKD Examples of tumors in patients on
➢ Enlarged echogenic kidneys? ARPKD dialysis due to ACKD
➢ Cysts and AMLs? Brain, skin? TSC
➢ Cysts and RCCs? VHL
➢ Renal failure on dialysis? ACKD
• Genetic Testing and Screening:
➢ PKD1, PKD2
➢ TSC1, TSC2
➢ VHL
• Risk of Renal Cancer:
➢ ADPKD None known
➢ ARPKD None known
➢ TSC ~ 1-2%
➢ VHL ~ 35%
➢ ACKD ~ 50% (after 8 years of dialysis)
References
1. Witzgall R. New developments in the field of cystic kidney diseases. Curr Mol Med 2005; 5:455-465.
2. Tahvanainen E, Tahvanainen P, Kaariainen H, Hockerstedt K. Polycystic liver and kidney diseases. Ann Med 2005;
37:546-555.
3. Adeva M, El-Youssef M, Rossetti S, et al. Clinical and molecular characterization defines a broadened spectrum of
autosomal recessive polycystic kidney disease (ARPKD). Medicine (Baltimore) 2006; 85:1-21.
4. Okumura M, Bunduki V, Shiang C, Schultz R, Zugaib M. Unusual sonographic features of ARPKD. Prenat Diagn
2006.
5. Choyke PL, Glenn GM, Walther MM, Zbar B, Linehan WM. Hereditary renal cancers. Radiology 2003; 226:33-
46.
6. Sessa A, Righetti M, Battini G. Autosomal recessive and dominant polycystic kidney diseases. Minerva Urol
Nefrol 2004; 56:329-338.
7. Herring JC, Enquist EG, Chernoff A, Linehan WM, Choyke PL, Walther MM. Parenchymal sparing surgery in
patients with hereditary renal cell carcinoma: 10-year experience. J Urol 2001; 165:777-781.
8. Seizinger BR, Smith DI, Filling-Katz MR, et al. Genetic flanking markers refine diagnostic criteria and provide
insights into the genetics of Von Hippel Lindau disease. Proc Natl Acad Sci U S A 1991; 88:2864-2868.
9. Choyke PL, Glenn GM, Walther MM, Patronas NJ, Linehan WM, Zbar B. von Hippel-Lindau disease: genetic,
clinical, and imaging features. Radiology 1995; 194:629-642.
10. Ishikawa I, Saito Y, Asaka M, et al. Twenty-year follow-up of acquired renal cystic disease. Clin Nephrol 2003;
59:153-159.

Imaging of Prostate Cancer
Peter L.Choyke, MD
Prostate Cancer
• Diagnosis
• Staging
• Image guided Therapy
Epidemiology
• ~220,000 new diagnoses per year
➢ ~29,000 cancer deaths (USA)
➢ 2nd most common cause of cancer deaths in males
➢ 21% decrease in cancer deaths in the PSA era
Only a small fraction of prostate cancers cause death

• We are overdiagnosing and overtreating prostate cancer
Grading Prostate Cancer

• Gleason Grading System
➢ Two predominant cell types
➢ Add together for score from 2-10
Screening and Detection

• Recommendations:
➢ For men > 50 years or > 40 in African Americans or with Family history :
❖ Annual Digital Rectal*
❖ Annual PSA
Figure 3-10-1
Prostate Specific Antigen
• Ranges of PSA
➢ 0–4ng/ml Normal (PPV=5%)
➢ 4–10ng/ml Indeterminate (PPV=22%)
➢ > 10 ng/ml Abnormal (PPV=67%)
Detecting Prostate Cancer

• Elevated PSA or Abnormal Rectal Exam
• Transrectal Ultrasound Guided Biopsies
• Biopsy Mapping and Grading
Anatomy
• Zonal Anatomy of Prostate
➢ Peripheral Zone
❖ Glandular
❖ 70% of Cancers
➢ Transitional Zone
❖ Stromal/Glandular
❖ 25% of Cancers, 90% of Hyperplastic nodules
➢ Central Zone
Distribution of Prostate Cancers
Prostate Ultrasound [Figure 3-10-1]

• Transrectal Ultrasound-History Normal ultrasound of the prostate
➢ The Chair (Watanabe 1968) showing echogenic peripheral zone
➢ Zonal Anatomy (Stamey 1980) and ejaculatory duct on sagittal view
➢ Screening (Lee 1983)
➢ TRUS Guided Biopsy (1985-)
➢ Color Doppler (1995-)
➢ Contrast Enhanced US (2000-)
Imaging of Prostate Cancer 620 Genitourinary Radiology

The TRUS Examination [Figure 3-10-2] Figure 3-10-2
• Examine the PZ for nodules
• Examine the TZ for asymmetry
• Examine Seminal Vesicles
• Determine the Volume
TRUS Guided Biopsy

• Prep:
➢ Antibiotics before and after (Cipro)
➢ Enema (Fleets)
• Core Biopsies with Automatic Cutting Needle
➢ Directed Biopsies at sites of abnormality
➢ Label all specimens; send separately
Doppler power ultrasound can be
Tumor Mapping with Biopsy used to detect prostate cancer
vascularity
Staging Prostate Cancer
Figure 3-10-3
Staging (TNM)
• Non palpable A1, 2 T1a, b
• Detected by Bx ** T1c
• Palpable B1, 2 T2a, b
• Extracapsular C1 T3a, b, c(sv)
• Fixed, invasive C2 T4
• Regional Nodes D1 Tx, N+
• Distant Mets D2 Tx, Nx, M+
Treatment
• A1, 2 T1 a-c Surg/XRT/WW
• B1, 2 T2 a,b Surg/XRT/WW
• C1, 2 T3 a-c XRT/WW/Hormonal
• D1 T4 XRT/WW/Hormonal
• D2 Tx, N+, M+ Hormonal/Chemotherapy
Staging with Imaging

• Local Staging (Extracapsular) [Figure 3-10-3]
➢ Ultrasound
➢ MRI
• Key Structures:
➢ Neurovascular Bundles
➢ Seminal Vesicles
➢ Apex
➢ Periprostatic Venous Plexus Extracapsular extension on
ultrasound
TRUS Staging
• Sensitivity
➢ ~40–50%
• Exceptions:
➢ Seminal Vesicles Figure 3-10-4
➢ Neurovascular Bundle
Endorectal Coil MRI [Figure 3-10-4]

• Sensitivity:
➢ Early Studies ~85–90%
➢ Multi institutional Trial ~60–70%
❖ Motion
❖ Microscopic Disease
❖ Operator dependent
❖ Observer dependent Extracapsular extension on endorectal coil MRI
Genitourinary Radiology 621 Imaging of Prostate Cancer

Endorectal Coil MRI Figure 3-10-5
• Improvements
➢ Dynamic contrast enhancement
➢ MR Spectroscopy
Dynamic Enhanced MRI of the Protaste
MRI and 1H-MRSI- Prostate: Metabolic

Interrogation
Nodal Staging [Figures 3-10-5 and 3-10-6]

• CT/MRI only ~ 36% sensitive
➢ Size threshold ~8mm + biopsy of nodes
➢ Yield improves for high risk pts
➢ (PSA >20 ng/ml)
• Prostascint SPECT Prostascint images demonstrate positive node despite
• USPIO (Combidex) MR Lymphography negative CT
Staging for Distant Metastases

• Bone Metastases
➢ Bone Scan Figure 3-10-6
❖ Yield increases
after PSA >10
❖ **Superscan**
❖ Quantitation/
Confirmation
Radioactive Ablation
• Strontium-89 (Metastron)
➢ ~80% response rate
➢ Up to 6 months relief of Examples of nodal metastasis due to prostate cancer. Percutaneous biopsy
bone pain can be performed to determine if a node is positive
➢ Samarium and
Rhenium
Figure 3-10-7
Image Guided Treatment
• Brachytherapy
• Cryotherapy
Brachytherapy [Figure 3-10-7]

• Interstitial Radioactive Seeds
(Afterloaded)
➢ Iodine, Iridium, Palladium, Gold
• Introducers are placed within prostate at
regular spacing via:
➢ CT
➢ US Examples of brachytherapy seeds immediately after seed
➢ MRI placement (left) and several years after placement (right)
Cryotherapy [Figure 3-10-8]

• Liquid Nitrogen instilled via cannulas placed within Prostate under TRUS
• Not enough Data Figure 3-10-8
➢ High rate of impotence
➢ Steep learning curve
Cryotherapy of the prostate monitored by ultrasound
Imaging of Prostate Cancer 622 Genitourinary Radiology

Take Home Points
• Imaging currently plays minor role in prostate cancer detection:
➢ MR spectroscopy, Dynamic MRI may change this
• Staging depends on PSA/Grade
➢ MRI for local staging
➢ CT/MRI (USPIO) for nodal staging
➢ Bone Scan/CT/MR for distant staging
• Image Guided therapy is an important trend in treatment
References
1. Adusumilli S, Pretorius ES. Magnetic resonance imaging of prostate cancer. Semin Urol Oncol 2002; 20:192-210.
2. Campbell T, Blasko J, Crawford ED, et al. Clinical staging of prostate cancer: reproducibility and clarification of
issues. Int J Cancer 2001; 96:198-209.
3. el-Gabry EA, Halpern EJ, Strup SE, Gomella LG. Imaging prostate cancer: current and future applications. Oncology
(Huntingt) 2001; 15:325-336; discussion 339-342.
4. Engelbrecht MR, Barentsz JO, Jager GJ, et al. Prostate cancer staging using imaging. BJU Int 2000; 86 Suppl 1:123-
134.
5. Harisinghani MG, Barentsz J, Hahn PF, et al. Noninvasive detection of clinically occult lymph-node metastases in
prostate cancer. N Engl J Med 2003; 348:2491-2499.
6. Hocht S, Wiegel T, Bottke D, et al. Computed tomogram prior to prostatectomy. Advantage in defining planning target
volumes for postoperative adjuvant radiotherapy in patients with stage C prostate cancer? Strahlenther Onkol 2002;
178:134-138.
7. Hernandez J, Thompson IM. Prostate-specific antigen: a review of the validation of the most commonly used cancer
biomarker. Cancer 2004; 101:894-904.
8. Konety BR, Bird VY, Deorah S, Dahmoush L. Comparison of the incidence of latent prostate cancer detected at
autopsy before and after the prostate specific antigen era. J Urol 2005; 174:1785-1788; discussion 1788.
9. Karakiewicz PI, Eastham JA, Graefen M, et al. Prognostic impact of positive surgical margins in surgically treated
prostate cancer: multi-institutional assessment of 5831 patients. Urology 2005; 66:1245-1250.
10. Kumar R, Zhuang H, Alavi A. PET in the management of urologic malignancies. Radiol Clin North Am 2004; 42:1141-
1153, ix.
11. Mathews D, Oz OK. Positron emission tomography in prostate and renal cell carcinoma. Curr Opin Urol 2002; 12:381-
385.
12. Ravery V, Boccon-Gibod L. T3 prostate cancer: how reliable is clinical staging? Semin Urol Oncol 1997; 15:202-
206.
13. Raja J, Ramachandran N, Munneke G, Patel U. Current status of transrectal ultrasound-guided prostate biopsy in the
diagnosis of prostate cancer. Clin Radiol 2006; 61:142-153.
14. Sanchez-Chapado M, Angulo JC, Ibarburen C, et al. Comparison of digital rectal examination, transrectal
ultrasonography, and multicoil magnetic resonance imaging for preoperative evaluation of prostate cancer. Eur Urol
1997; 32:140-149.
15. Sodee DB, Nelson AD, Faulhaber PF, Maclennan GT, Resnick MI, Bakale G. Update on fused capromab pendetide
imaging of prostate cancer. Clin Prostate Cancer 2005; 3:230-238.
Genitourinary Radiology 623 Imaging of Prostate Cancer

Radiographic Evaluation of Urinary Stone
Disease
William D. Craig, MD
Learning Objectives
• Pathogenesis of renal stone disease
• Highlight CT as the modality of choice
• Alternative modalities
• Reinforce the critical role of Radiology
AFIP
• Limited Rad-Pathology
• Stone Dz
➢ Major nuisance
➢ Med/Urologic Advances
➢ Previously Debilitating
• Annual 2-3% incidence
• White male LTR is 1 in 3-8
➢ 14% @ 1yr
➢ 35% @ 5yr
➢ 52% @ 10yr
• Multi Billion $$ Cost
Genetics
• Family Hx (3 X)
• M : F : 3: 1
• Recognized D/O
➢ Familial RTA
➢ Mutations in CLCN5 gene
Extrinsic
• Climate
• Water
• Diet
• Occupation
• Stress
Predisposing Factors
• Family Hx
• Bone/GI Dz
• Gout
• Chronic UTI
• Nephrocalcinosis
• Stasis
Stone Makeup
• Composition Percent of all stones
• Ca Oxalate/phosphate 75
• Struvite/matrix 10-15
• Uric Acid 6
• Cystine 1-2
• Other (incl indinavir) <5
Heterogeneous Nucleation
• Epithelial cells
• Urinary Casts
• RBC’s
• Homogeneous Nuc---
Radiographic Evaluation of Urinary Stone Disease 624 Genitourinary Radiology

Inhibitors
• Organic Molecule
• Magnesium
• Pyrophosphate
• Citrate
• Mucoproteins
• RNA Fragmets
Promoters
• Glycosaminoglycans
• Tamm-Horsfall
Aggregation
• Free crystals need to grow (2-5 min transit fm glomerulus to nephron)
• Anatomic Abnl
➢ UPJ
➢ MSK
• Lack of inhibitors Figure 3-11-1
➢ Light chain proteins
Formation Product
• Real question?
• Why don’t we all form stones
• Kf is 7-11 X Ksp
Calcium Stones [Figure 3-11-1]

• Hypercalcuria
➢ Idiopathic
➢ Steroid Use
Immobilizaion
• Hypocitrinuria
• Hypomagnesuria Left Ureteral Stone
Calcium Stones
• Hyperoxaluria
➢ Crohn disease
➢ Celiac sprue
➢ Pancreatic insufficiency Figure 3-11-2
➢ Small intestinal bypass surgery for obesity
• CaPhos
➢ PTH or RTA
Struvite Stone MgNH4 PO4 · 6H2 O

Magnesium Ammonium Phos [Figure 3-11-2]
• (NH2 )2 --CO ? H2 O + 2NH3 + CO2
• Urine pH increases because ammonia hydrolyzes
• Urease
➢ Proteus
➢ Klebsiella
Struvite Staghorn Calculus

• Low Urine Volumes
• Infection
• F to M: 3 to 1
• Sx
➢ Malaise
➢ Fever
➢ Wt Loss
Genitourinary Radiology 625 Radiographic Evaluation of Urinary Stone Disease

Uric Acid Stones
• Hyperuricosuria
➢ Gout 35% if Uric Acid > 700mg/24 hr urine
➢ Newly Dx’ed Gout 1 per 114 with stones
➢ Hereditary (Lesch-Nyhan)
➢ Idiopathic
• Dehydration
• Markedly acid urine (< pH of 6.2)
➢ UC is classic example
❖ Ileostomy w/water and bicarb loss
Cystine Stone
• Hereditary Cystinuria
➢ Three Types
➢ Auto Recessive
➢ Abnormal renal tubule transport
➢ Large amounts of cystine are excreted in the urine (10 X normal)
➢ Younger Patients
Presentation
• Autonomic System
➢ Celiac ganglion
➢ Confusion about source
➢ Diaphragm to testicle
➢ GI sx
❖ N/V
❖ Diarrhea
❖ Ileus
Figure 3-11-3
Films
• Visualize
• Characterize
• Sens 45%
• Spec 50%
IVP
• Sensitivity: 64-97%
• Specificity: 92-94%
• Still 10-15% false negative rate
CT Distal left ureteral stone demonstrating a Rim Sign

• 1995
• Sens of > 90%
• Spec of nearly 100%
• Quick
• No contrast Figure 3-11-4
• Non-urologic Abnl
CT-Radiation
4 by 2.5, 120 KVP, 120mAs,1-1.5
• 500 mrem
• 150-350 mrem for full IVP
• 13 mrem for one image
Rim Sign [Figure 3-11-3]
Comet Tail Sign [Figure 3-11-4] Calcifications in right pelvis demonstrating the
Comet Tail Sign

Secondary Signs
Hydronephrosis/ Hydroureter [Figure 3-11-5]
Figure 3-11-5
Secondary Signs
Unilateral renal enlargement [Figure 3-11-6]
Secondary Signs
Perinephric/ureteral edema [Figure 3-11-7]
Secondary Signs
Unilateral absence of the white pyramid
[Figures 3-11-8 to 3-11-10]
Figure 3-11-6
Asymmetric Right Hydronephrosis
Figure 3-11-7
Asymmetric left renal enlargement
Figure 3-11-8
Left Perinephric stranding
Figure 3-11-9
Normal hyperdense pyramids
Figure 3-11-10
Unilateral absence of right

hyperdense pyramids
Obstructed left system with lower parenchymal

attenuation

Conclusions
• If H.U. Discrepancy > 5
• Sens = 61%
• Spec = 100%
• Accur = 79%
Urologist
• Size
• Number
• Location
• Complications
Spontaneous Passage Rate of Ureteral Calculi as a Function of

Stone Size
Stone Size (mm) No of Stones Passage Rate (%)
1 15 87
2 43 72
3 23 83
4 18 72
5 15 60
6 18 72
7 17 47
8 9 56
9 3 33
10 11 27
Figure 3-11-11
Expectant Treatment
• Non-infected
• Two kidneys and normal renal function
• Small Stones
• <4mm 90% pass spontaneously
Distal Stone Protocol

• Steroids
• Calcium channel blockers
• Fluids
• Pain meds
Non-invasive treatment: ESWL

• Extracorporeal Shock wave lithotripsy
• < 2 cm
• Ca Stones
• Upper/Mid Pole Calyx
Invasive treatment: Ureteroscopy

• Mechanical extraction Shadowing stone in central collecting system
• Homium Laser
Invasive Therapy: PCNL Figure 3-11-12

• Percutaneous Nephrolithotomy
• Not amenable to ESWL or ureteroscopic approaches
• 1-2 day hospitalization
Alternative Modalities [Figure 3-11-11]
Quantifying Obstruction: Ultrasound [Figure 3-11-12]

Hydronephrosis
Examine ureteral jets
Potential use of resistive index (>.7)
Hydronephrotic Kidney

MR [Figure 3-11-13]
Figure 3-11-13
MR Urography shows distal ureteral stone
References
1. Walsh : Campbell's Urology, 8th ed. 2002 W. B. Saunders Company.

2. Tamm EP, Silverman PM, Shuman WP. Evaluation of the patient with flank pain and possible
ureteral calculus. Radiology. 2003 Aug;228(2):319-29. Epub 2003 Jun 20. Review.
3. Guest AR, Cohan RH, Korobkin M, Platt JF, Bundschu CC, Francis IR, Gebramarium A, Murray
UM. Assessment of the Clinical Utility of the Rim and Comet-Tail Signs in Differentiating Ureteral
Stones from Phleboliths AJR 2001;177:1285-1291.

Testicular Torsion - Case Based Review
Testicular Torsion
• Defined as a twist of the spermatic cord or of the testis itself on its
attachments.
• Degree of ischemia relative to the amount of twisting, beginning with venous
compromise, and progressing to arterial occlusion. A 360 degree twist may still
have arterial inflow.
• Torsion most common in puberty (ages 12-18), but also occurs in neonates
and adults
Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the scrotum.
Radiology 2003;227:18-36
Types of Testicular Torsion

• Extravaginal (Neonatal)
• Intravaginal
➢ Bell-Clapper deformity
Scrotal Anatomy and Torsion

• Normal Tunica Vaginalis
➢ Inner visceral layer covers the testis and epididymis and cord
➢ Outer parietal layer lines the scrotum except posterolaterally where it fuses
with the visceral layer and the scrotal wall to form the bare area.
➢ Hydroceles occur between these 2 layers
Scrotal Anatomy and Torsion

• Bell-Clapper Deformity of the Tunica Vaginalis
➢ Failure of fusion of the visceral and parietal layers to the scrotal wall, so
the space completely encircles the epididymis, distal spermatic cord and
the testis rather than attaching to the posterolateral aspect of the scrotum
to form the normal bare area.
➢ It is bilateral in most cases
➢ 12% incidence found in one autopsy series
Dogra VS, Gottlieb RH, Oka M, Rubens DJ.
Sonography of the scrotum. Radiology 2003;227:18-36 Figure 3-12-1
Scrotal Anatomy Tunica
[Figure 3-12-1] Vaginalis
Bell-Clapper Deformity
Sagittal line drawings of bell-clapper (left) and

normal (right) testis. Tunica vaginalis is the
outermost layer (arrows).
Testicular Torsion 630 Genitourinary Radiology

Testicular Torsion
• Clinical presentation includes:
➢ Acute onset of scrotal pain
➢ Anorexia, nausea and/or vomiting
➢ Lack of urinary symptoms or fever
• As many as 35-50% of patients experience gradual onset of pain, similar to
epididymitis
• Pain may be intermittent (detorsion)
Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the scrotum.
Radiology 2003;227:18-36
Testicular Torsion: Clinical Significance

• Time from onset of symptoms to surgery associated with salvage rate:*
➢ 5-6 hours, 80-100%
➢ 6-12 hours 70%
➢ After 12 hours 20%
• If non salvageable, the necrotic testis is removed to decrease risk of
autoimmune reaction to the residual testis
Donohue RE, Utley WL. Urology 1978 11:33
Testicular Torsion: Grayscale Patterns

• Acute torsion with viable testis: normal
• Acute torsion with infarction: hypoechoic pattern which may be
total, or partial in the case of a partial infarct Figure 3-12-2
• Acute torsion with hemorrhagic infarction: hyperechoic and
heterogeneous pattern.
• Chronic: hypoechoic with small testis
Middleton WD, Middleton MA, Dierks M, et al. Sonographic prediction
of viability in testicular torsion: preliminary observation. J Ultrasound
Med. 1997;16:23–27.
Testicular Torsion: Doppler Patterns

• Absent arterial and venous flow
• Increased Resistive Index on affected side (diminished or reversed
diastolic flow)
• Decreased flow velocity difficult to measure due to small
vessels/angle correction, but may be subjectively inferred by
relative difficulty in finding small low amplitude flow on symptomatic
side
Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the
scrotum. Radiology 2003;227:18-36
Dogra VS, Sessions A, Mevorach R, Rubens DJ Reversal of diastolic
plateau in partial testicular torsion. J Clin Ultrasound 2001; 29:105-108
CASE 1 [Figure 3-12-2]

• Sudden right sided pain
• Diagnosis: Acute right testicular torsion with viable testis.
Dx: Acute torsion with viable testis

• Important imaging findings:
➢ Note grayscale symmetry on transverse images
➢ Color Doppler flow is absent in the affected testis, but not in the
epididymis
❖ The epididymis has alternate blood supply and may be
perfused even if the testis is not
CASE 1: 21 year old man with

sudden right-sided testicular pain
Genitourinary Radiology 631 Testicular Torsion

CASE 2 [Figures 3-12-3 and 3-12-4]
• A seven year old presents with acute symptoms. Which side is abnormal?
• Minimal right sided flow
• Right testis is hypoechoic
Figure 3-12-3
Figure 3-12-4
CASE 2: A seven year old presents

with acute symptoms. Which side is CASE 2: Minimal right sided flow. Right testis is hypoechoic
abnormal?
• Left sided Doppler findings. What is your diagnosis?
• Chronic left testicular torsion with hemorrhagic non-viable testis
From: Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of Testicular
Torsion Ultrasound Clin 2006; 1:55-66 with permission.
Figure 3-12-5
Chronic Torsion with
Hemorrhagic
Non-Viable Testis
• In young children the testis may be
small and hypoechoic. Flow is often
minimal, but note the normal spectral
waveform pattern
• The abnormal side has markedly
increased Doppler flow, but it is around
the testis, not within it. It is important
always to image in 2 planes and to
document flow within the actual testis. CASE 2: Left sided Doppler findings in long axis (left) and in
• Hemorrhage creates additional tissue transverse (right) plane. What is your diagnosis?
planes and is hyperechoic and
heterogeneous. Hemorrhage is a result
of infarction and indicates a non-viable testis
Figure 3-12-6
CASE 3 [Figure 3-12-6] RT LT
• Right sided pain, nausea and vomiting.
Is this torsion or epidydimitis?
• Diagnosis:
Torsion with 360 degree twist of the
spermatic cord
CASE 3 Right sided pain, nausea and vomiting in a 15 year

old. Is this torsion or epidydimitis?

Torsion with preservation of Doppler flow
• Torsion is not an all or none phenomenon
• Venous obstruction occurs first and is indicated by a high-resistance arterial
spectral Doppler waveform.
• Flow may still be present even if the testis is twisted up to 720 degrees.
• More flow will be detected with power Doppler and probably also with US
contrast.
• THE PRESENCE OF FLOW DOES NOT EXCLUDE TORSION!
Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of Testicular Torsion
Ultrasound Clin 2006; 1:55-66
Figure 3-12-7
• 2 hours of left sided symptoms
• Can this be torsion?
• DX: Torsion/detorsion with increased flow post torsion
From: Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of
Testicular Torsion Ultrasound Clin 2006; 1:55-66 with permission.
TORSION/DETORSION
• History is critical-classically that of intermittent acute and sharp pain
with long symptom-free intervals
• Know which side hurts and if it still hurts during the examination.
• If scanned immediately after detorsion, the affected testis may show
increased blood flow

• Left sided symptoms progressing over several days. Prior US exam
showed an enlarged epididymis with increased flow to the
epididymis
• Dx: Torsion/detorsion with focal infarction
Figure 3-12-8
CASE 5:Left sided CASE 4: 2 hours of left sided

symptoms symptoms. Can this be torsion?
progressing over From: Dogra VS, Bhatt S, Rubens,
several days. Prior DJ. Sonographic Evaluation of
US exam showed an Testicular Torsion
enlarged epididymis Ultrasound Clin 2006; 1:55-66 with
with increased flow permission
to the epididymis
Torsion/detorsion with infarction

• Focal hypoechoic areas with concave margins are typical of infarcts.
• Focal infarcts when associated with normal or increased flow should alert you
to the possibility of intermittent torsion.
• Frequently the epididymis is enlarged and hyperemic in torsion/detorsion and
can be mistaken for epididymitis.
• Careful surveillance of the testis for focal infarction may lead to the correct
diagnosis

• A 2 month old had pain 1 month ago with a normal US exam,
Now he has new pain and right scrotal swelling for 18 hours Figure 3-12-9

• What is the finding adjacent to the testis.
Is this torsion?
• Dx: Inguinal hernia with obstructed flow to
the spermatic cord
Figure 3-12-10
CASE 6: A 2 month old had pain 1 month ago with a

normal US exam, Now he has new pain and right scrotal
swelling for 18 hours
CASE 6: Note fluid filled

structure superior to the right
testis (top) with color Doppler
flow in the wall (bottom).
A hydrocele surrounds the
right testis. Is this torsion?
Inguinal Hernia With Obstructed Testicular Perfusion

• Multiple etiologies of altered testicular perfusion may occur, including mass
lesions obstructing the spermatic cord.
• Even in a newborn, flow should be obtainable in the testis Figure 3-12-12

• 4 day old with large left hydrocele. Is there left-sided torsion?

• Repeat scan 10 hours later
Figure 3-12-11
CASE 7: Repeat scans 10

hours later
CASE 7: A 4 day old presents with a large left hydrocele. Is Right testes (top)
there left-sided torsion? Left testes (bottom)

• Repeat spectral Doppler exam 10 hours later. What is your diagnosis?
• Dx: Normal testes Figure 3-12-13
Doppler Technical Considerations

• Always use the highest frequency Doppler which will yield a signal
without attenuation
• Initial examination was performed at Doppler frequency of 5MHz,
repeat examination at 10 MHz.
• Always confirm a true arterial spectral waveform. The waveform on
the initial examination was only noise, and could have been
interpreted as no flow

• Acute left sided pain
• Patient is s/p Lt orchiectomy and s/p Rt Orchiopexy
• Now with right sided pain. Repeat scan done 2 days later
[Figure 3-12-15]
• Dx: Bilateral infarction due to polyarteritis nodosa
Figure 3-12-14
RT LT CASE 7: Repeat spectral Doppler
exam 10 hours later. What is your
diagnosis?
Top: Bilateral normal testes with
inadequate Doppler on initial
examination
Figure 3-12-15
Acute left sided pain

No flow in the left testis CASE 8: 2 days later the patient presents with
Testicular Ischemia right sided pain. He is post left orchiectomy and
Causes for ischemia other than torsion include: right orchiopexy.
• Vasculitis Gray scale image (left) is unremarkable.
➢ Polyarteritis Nodosa Doppler image (right) shows a noise spectrum.
➢ Systemic Lupus Erythematosum Dx: Bilateral infarction due to polyarteritis nodosa
• Severe edema from infection
➢ Uncontrolled or unresponsive epididymo-orchitis
• Venous thromboses (ie hypercoagulable patients)
Imaging Methods
• Doppler examination is 86% sensitive, 100% specific and 97% accurate* when
using absent flow in the symptomatic side as the single diagnostic criteria. If
assymmetric abnormal spectral tracing were also used for diagnosis,
sensitivity would improve
• In children, power Doppler is more sensitive than color Doppler to detect
normal flow, with rates of 97% vs 88% respectively **
*Burks DD, Markey BJ, Burkhard TK, Balsara ZN Haluszka MM, Canning DA.
Suspected testicular torsion and ischemia: evaluation with color Doppler
sonography. Radiology 1990;175:815-21
** Barth RA, Shortliffe LD. Normal pediatric testis: comparison of power Doppler
and color Doppler US in the detection of blood flow. Radiology 1997;204:389-93

Torsion/Detorsion
• Classic history of intermittent symptoms
• If scanned when asymptomatic or after detorsed, will see increased flow in the
affected testis, which may suggest epididymitis
• Testis may be enlarged, and focal infarcts may or may not be present
Torsion Mimics/Variants
• Infarction may present with pain which mimics torsion
• Partial infarction may occur from torsion/detorsion, from vasculitis, or from
variant arterial anatomy. In some patients, the epidydimal artery, a branch of
the testicular artery, supplies the superior and anterior pole of the testis. If this
artery is hypoplastic, a small twist may result in focal infarction involving the
superior pole
• Total infarction is more unusual, but should be suspected in patients with
underlying vasculitides such as polyarteritis nodosa and systemic lupus
Artery to Epididymis
• In some patients, the epidydimal artery, a branch of the testicular artery,
supplies the superior and anterior pole of the testis. If this artery is hypoplastic,
a small twist may result in focal infarction
Torsion Take Home Messages

• Torsion may be present despite testicular flow.
• Diminished or high resistance flow should suggest torsion in the proper
setting.
• A history of intermittent symptoms should suggest detorsion, and
corresponding hyperperfusion should not be confused with epididymo-orchitis.
• Other rare causes of decreased testicular perfusion include vasculitis, and if
torsion is not present, appropriate medical therapy should be pursued
References
1. Barth RA, Shortliffe LD. Normal pediatric testis: comparison of power Doppler and color Doppler US in the
detection of blood flow. Radiology 1997;204:389-93.
2. Burks DD, Markey BJ, Burkhard TK, Balsara ZN Haluszka MM, Canning DA. Suspected testicular torsion and
ischemia: evaluation with color Doppler sonography. Radiology 1990;175:815-21
3. Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of Testicular Torsion. Ultrasound Clin 2006; 1:55-66 with
permission.
4. Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the Scrotum. Radiology 2003;227:18-36
5. Dogra VS, Sessions A, Mevorach R, Rubens DJ Reversal of diastolic plateau in partial testicular torsion. J Clin
Ultrasound 2001; 29:105-108
6. Donohue RE, Utley WL. Urology 1978 11:33
7. Middleton WD, Middleton MA, Dierks M, et al. Sonographic prediction of viability in testicular torsion:
preliminary observation. J Ultrasound Med. 1997;16:23–27.

Imaging of Ovarian Masses
Brent J. Wagner, MD
Ovarian Masses
• Non-neoplastic
➢ physiologic cyst, endometriosis, etc.
• Neoplastic
➢ epithelial tumors 65%
➢ germ cell tumors 25%
➢ sex-cord stromal tumors 5%
➢ secondary malignancies 5%
➢ gonadoblastoma <1%
Relative Incidence of Ovarian Neoplasms [Figure 3-13-1]

Figure 3-13-1
Major Ovarian Tumor Types
• Epithelial
➢ Serous
➢ Mucinous
➢ Endometrioid
➢ Clear Cell
➢ Brenner
➢ (others)
• Germ Cell
➢ Mature teratoma
➢ Dysgerminoma Relative incidence of
➢ Immature teratoma ovarian neoplasms
➢ (others)
• Sex Cord – Stromal
➢ Fibrothecoma
➢ Granulosa cell
➢ Sertoli-Leydig
➢ (others)
Common Ovarian Epithelial Tumors: Classification

• Serous
• Mucinous
• Endometrioid
• Clear Cell
• Brenner
• (others, including mixed, undifferentiated, etc.)
Ovarian Epithelial Tumors

• 65% of ovarian neoplasms
• 85% of ovarian malignancies
• 60% of epithelial tumors are benign
• 35% of epithelial tumors are malignant
• 5% of epithelial tumors are borderline, low malignant potential
Ovarian Epithelial Tumors

• major risk factor: “incessant ovulation” (Lancet, 1973)
➢ infertility
➢ celibacy
➢ nulliparity
➢ family history
➢ high socioeconomic status
➢ breast cancer
➢ endometrial cancer
➢ lack of oral contraceptive use
Genitourinary Radiology 637 Imaging of Ovarian Masses

CA-125 Figure 3-13-2
• abnormally elevated in 85% of ovarian cancer patients
• false negative in 50% of Stage I disease
• false negative in 30% of mucinous tumors
• false positives occur (especially in pre-menopausal patients)
with benign neoplasms, endometriosis, etc.
• most commonly used to follow known disease for remission and
recurrence
• rarely of use in deciding on surgical vs. non-surgical
management at the time of initial presentation of a pelvis mass
Ovarian Epithelial Tumors Serous cystadenoma (bilateral)

• benign
• low malignant potential (LMP)
➢ “borderline” tumors
Figure 3-13-3
➢ based on histologic appearance of primary
➢ may be a heterogeneous group (but histologic features
overlap)
➢ 95% five year survival overall•
❖ But the patients who have metastasis or recurrence are
clinically similar to patients with (true) ovarian cancer
• malignant
Epithelial Tumors: Terminology

• adenoma or adenocarcinoma
• add prefix “cyst-” if cystic Serous cystadenoma (LMP)
• add suffix “-fibroma” if more than 50% fibrous
➢ (e.g. “cystadenofibroma” or “fibrous cystadenocarcinoma”)
➢ do not confuse with a true fibroma (one of the sex-cord stromal tumors) Figure 3-13-4
Epithelial Ovarian Neoplasms: Serous

[Figures 3-13-2 to 3-13-6]
• also known as “papillary” tumors
• 25% of benign neoplasms
• 50% of malignant neoplasms
• 63% benign, 30% malignant, 7% LMP
• strongest association with CA-125
• thin-walled cyst, usually unilocular
• papillary soft tissue projections often seen
• psammomatous calcification is more common than with other
ovarian neoplasms
• solid or bilateral tumors suggest malignancy
Figure 3-13-5
Serous
cystadenocarcinoma Serous cystadenoma (LMP)
Imaging of Ovarian Masses 638 Genitourinary Radiology

Epithelial Ovarian Neoplasms: Mucinous Figure 3-13-6
• mucin-containing cells (cyst content varies)
• up to 20% of benign ovarian neoplasms
• 10% of carcinomas
• 73% benign, 16% malignant, 11% LMP
• serum marker (CA-125) is less reliable (falsely negative) with
mucinous tumors
Epithelial Ovarian Neoplasms: Mucinous [Figure 3-13-7]

• thin-walled cyst, usually multilocular
• often large, may be enormous
• occasionally, linear calcifications (but calcifications are LESS
frequent than with mucinous tumors of colonic origin)
• solid elements suggest malignancy
• LMP tumors are associated with pseudomyxoma peritonei
➢ cause / effect relationship is often unclear
Pseudomyxoma peritonei [Figure 3-13-8]

• usually arises from appendix
• often difficult to determine whether the process originated from
the appendix, ovary, or both (synchronous)
• prolonged, uncomfortable survival (limited treatment options) Serous cystadenocarcinoma
• low density, “scalloping” seen on CT
• “Pseudomyxoma peritonei is a poorly understood condition and Figure 3-13-7
it is unclear whether its continous production of gelatinous mucin
is due to peritoneal implantation of neoplastic mucinous cells or
to metaplasia of peritoneal cells into mucinous epithelium,
induced by mucin.” [1]
[1] Tropé CG et al. Surgery for borderline tumor of the ovary.
Seminars in Surgical Oncology 2000; 19:69–75.
Epithelial Ovarian Neoplasms: Endometrioid

• mimics endometrial ca, but is primary to ovary
• may have malignant stroma (“carcinosarcoma” or “malignant
mixed mesodermal tumor” = MMMT)
• almost all are malignant
• 10–15% of ovarian cancers Mucinous cystadenoma (LMP?)
• 25% of patients have an associated uterine abnormality
➢ endometrial carcinoma (separate primary malignancy) Figure 3-13-8
➢ endometrial hyperplasia
• 15% of patients have coexistent endometriosis (cause/effect
unclear)
Epithelial Ovarian Tumors: Clear Cell [Figure 3-13-9]

• mimics clear cell cancer of the vagina, but no association with in
utero DES exposure
• 5% of ovarian cancers (all clear cell tumors are malignant)
• gross appearance is variable:
➢ unilocular cyst with a mural nodule
➢ multilocular
➢ solid, etc. Figure 3-13-9 Pseudomyxoma peritonei
Clear cell carcinoma

Ovarian Carcinoma Staging: Local disease (30%) Figure 3-13-10
• Stage I
➢ limited to ovary [subtypes]
• Stage II
➢ extra-ovarian pelvic extension [subtypes]
Ovarian Carcinoma Staging: Advanced Disease

(70%)
• Stage III
➢ tumor within the peritoneum (outside the pelvis) [or]
retroperitoneal lymph nodes [or] surface of the liver [or] small
bowel/omentum (within the pelvis)
• Stage IV
➢ distant spread
❖ hepatic parenchyma
❖ lung
❖ etc.
Typical Ovarian Cancer Therapy

• Surgical: hysterectomy, oophorectomy, appendectomy,
omentectomy, removal of peritoneal masses
• Medical: 6–8 (monthly) cycles of chemotherapy (a platinum-
based agent, plus taxol)
• Second look surgery? (only as part of a structured research
protocol)
Ovarian Masses
• Non-neoplastic
➢ Physiologic cyst, endometriosis, etc.
• Neoplastic
➢ Epithelial tumors 65%
➢ Germ cell tumors 25%
➢ Sex-cord stromal tumors 5%
➢ Secondary malignancies 5%
➢ Gonadoblastoma <1%
Ovarian Germ Cell Neoplasms

• most are mature teratomas:
➢ the most common mature teratomas are mature cystic
teratomas (commonly referred to as “dermoid cysts”)
➢ less common mature teratomas include carcinoid tumors,
struma ovarii, etc.
Mature teratoma
in the ovary . . .
• all dermoid cysts are mature teratomas (and most, but not all,
mature teratomas are dermoid cysts)
Figure 3-13-11
Mature Cystic Teratoma [Figures 3-13-10 and 3-13-11]
• unilocular cyst
➢ cyst fluid is nearly sonolucent
➢ posterior acoustic enhancement (fluid at body temperature)
➢ images as “fat” (lipid) by CT, MRI – but it is NOT adipose
tissue
• Rokitansky nodule
➢ contains various tissues (cartilage, gastrointestinal
epithelium, etc)
➢ echogenic
Malignant transformation of
mature teratoma

• child-bearing years Figure 3-13-12
• may undergo:
➢ torsion
➢ rupture
➢ malignant transformation (very rare)
• often discovered as an incidental finding
• 12% bilateral
Ovarian Malignant Germ Cell Tumors

• dysgerminoma (similar to seminoma)
• embryonal carcinoma
• endodermal sinus tumor
• immature teratoma
• choriocarcinoma Dysgerminoma
• mixed germ cell tumor (much less common than testicular mixed Figure 3-13-13
GCT)
Malignant Germ Cell Tumors [Figure 3-13-12]

• in general:
➢ younger age group (15–30 years) than epithelial tumors
➢ solid / heterogeneous
➢ highly aggressive
➢ differentiation among the various types is difficult (but
immature teratomas are the most likely to have fat,
calcification)
➢ may have elevated markers (AFP, HCG)
Fibroma / fibrothecoma
Sex-cord stromal tumors Figure 3-13-14
• many are very low grade malignancies
• generally diagnosed at Stage I (and therefore surgery is often
curative)
• 5–8% of ovarian neoplasms
• hormonal manifestations include:
➢ estrogenic effects: pseudoprecocious puberty, endometrial
stimulation
➢ virilization (less common)
Sex-cord stromal tumors

• fibrothecoma
➢ 50% of all sex-cord stromal tumors Hemorrhagic infarction of fibroma
➢ more common than either pure thecoma or pure fibroma
• granulosa cell tumors Figure 3-13-15
➢ including juvenile variety
• Sertoli-Leydig
➢ more common than either pure Sertoli or Leydig cell tumors
➢ rare, but the most common virilizing tumor of the ovary
Fibrothecoma [Figures 3-13-13 and 3-13-14]

• thecoma component produces estrogen
• fibroma component accounts for low signal on T2-weighted MRI
• sonographically, they tend to be homogeneously hypoechoic but
sound-attenuating
Granulosa cell tumors [Figure 3-13-15]

• “sponge-like” appearance on imaging
• multicystic lesion with hemorrhage in a patient under 30
suggests juvenile granulosa cell tumor (but these account for
only 5% of granulosa cell tumors overall)
Granulosa cell tumor

Sex-cord stromal tumors
• low signal on T2 suggests fibroma
• hypoechoic sound-attenuating lesion suggests fibroma
• diagnosis of this and other sex-cord stromal tumors may be possible if clinical
factors are taken into consideration (morphology is generally solid and non-
specific)
• “. . . difficult to suggest a simple algorithm for evaluation of women with
ovarian masses” [1]
• “[Doppler U/S, CT, and MRI] yielded similar [results] for discrimination between
benign disease and cancer . . . Although differentiation of benign from
malignant disease is obviously clinically important and these detection rates
are higher than those previously reported, they are likely still not high enough
for surgery to be avoided in most cases.” [1]
• “Whatever the modality used, it is hoped that correct staging of advanced
disease will lead to appropriate referral to a specialist in gynecologic
oncology.” [1]
[1] Kurtz A et al Radiology 1999 Jul;212(1):19–27
Scoring systems for ovarian tumors

• wall thickness
• nodularity
• septations
• echogenicity
• ascites?
• size?
Ovarian Masses: Sonographic scoring

• wall irregularities
➢ smooth --> papillary projections
• wall thickness
➢ thin --> thick (< or > 3 mm)
• septa
➢ none --> thin--> thick
• echogenicity
➢ low --> high
• ascites? size?
Doppler sonography*
• ideally, should allow more specificity and sensitivity for malignancy
• based on low resistance flow (high diastolic flow) in malignant neovascularity
• significant overlap with benign processes, especially in pre-menopausal
women
* = controversial
Doppler sonography of ovarian masses*

• it should work:
➢ in a large series of patients, the presence of high diastolic flow is predictive
of malignancy
• however, it is of limited usefulness:
➢ specificity is limited, especially in pre-menopausal patients
➢ there is considerable overlap of benign vs. malignant
* = controversial

Doppler sonography of ovarian masses* [Figure 3-13-16] Figure 3-13-16
• proposed threshold values:
➢ Resistive index (RI) = .45 (or .50)
➢ Pulsatility index (PI) = 1.0
• below these values: suggests malignancy
• corpus luteum may give false positive
• incomplete sampling may give false negative
* = controversial
Doppler sonography of ovarian masses*

• do not use the RI or PI values at the exclusion of the
sonographic morphology Use of resistive index in assessment
• color / amplitude Doppler may be of use in characterizing areas of ovarian masses
that may be confusing or indeterminate morphologically (for
example, clot vs. tissue)
* = controversial
Is pre-operative staging of ovarian cancer important? Maybe

not, because . . .
• “all” patients go to surgery (cytoreduction)
• in most centers, staging laparotomies are performed by a gynecologic
oncologist
Is prediction of malignancy in a neoplastic mass important?

• May determine the surgical approach
• May determine who the surgeon is:
➢ If “probably benign”, general gynecologist
➢ If “probably malignant”, gynecologic oncologist
Is the differentiation of a neoplasm from a non-neoplastic

ovarian mass important?
• Yes (and it′s usually accomplished sonography)
Pre-menopausal (ovulating) patient

• Acute symptoms?
➢ check pregnancy test
➢ check for fever, elevated white blood cell count
➢ if severe acute pain, consider torsion
Pre-menopausal (ovulating) patient

• If sub-acute or mild symptoms:
➢ simple cyst < 30 mm, no follow-up
➢ “hemorrhagic cyst” < 25 mm, no follow-up
➢ simple cyst 30–60 mm, follow-up in 6–10 weeks
➢ “hemorrhagic cyst” 25–60 mm, follow-up in 6–10 weeks
➢ any appearance > 60 mm, consider surgery
➢ any soft tissue component (septation, etc), consider surgery
Post-menopausal patient
• simple cyst <16 mm: ignore
• simple cyst 16–50 mm: follow-up 4 months
➢ presumed serous inclusion cyst vs. benign neoplasm
• simple cyst > 50 mm OR any complex lesion: consider surgery

References
1. Gajewski W, Legare RD. Ovarian cancer. Surg Onc Clin N Am 1998; 7:317.
2. Hricak H, Chen M, Coakley FV. Complex adnexal masses: detection and characterization with MR imaging --
multivariate analysis. Radiology 2000; 214:39.
3. Jung SE, Lee JM, Rha SE, Byun JY, et al. CT and MR Imaging of Ovarian Tumors with Emphasis on Differential
Diagnosis. Radiographics 2002; 22:1305.
4. Kawamoto S, Urban BA, Fishman EK. CT of epithelial ovarian tumors. Radiographics 1999; 19:S85.
5. Kinkel K, Lu Y, Mehdizade A, et al. Indeterminate ovarian mass at US: incremental value of second imaging test
for characterization – meta-analysis and Bayesian analysis. Radiology 2005; 236:85-94.
6. Koonings PP, Campbell K, Mishell DJ, Grimes DA. Relative frequency of primary ovarian neoplasms: a 10-year
review. Obstet Gynecol 1989; 74:921-926.
7. Kurtz AB, et al. Diagnosis and Staging of Ovarian Cancer: Comparative Values of Doppler and Conventional US,
CT, and MR Imaging Correlated with Surgery and Histopathologic Analysis—Report of the Radiology Diagnostic
Oncology Group. Radiology 1999; 212:19.
8. Outwater EK, Wagner BJ, Mannion C, McLarney JK, Kim B. Sex-cord stromal and steroid cell tumors of the ovary.
RadioGraphics 1998; 18:1523.
9. Patel MD, Feldstein VA, Lipson SD, Chen DC, and Filly RA. Cystic teratomas of the ovary: diagnostic value of
sonography. Am J Roentgenol 1998; 171:1061-1065.
10. Siegelman ES, Outwater, EK. Tissue Characterization in the Female Pelvis by Means of MR Imaging. Radiology
1999; 212:5.
11. Sironi S, Messa C, Mangili G, Zangheri B, et al. Integrated FDG PET/CT in Patients with Persistent Ovarian Cancer:
Correlation with Histologic Findings. Radiology 2004; 233:433.
12. Tanaka YO, Tsunoda H, Kitagawa Y, Ueno T, et al. Functioning Ovarian Tumors: Direct and Indirect Findings at
MR Imaging. RadioGraphics 2004; 24:S147.
13. Wagner BJ, Buck JL, Seidman JD, McCabe KM. Epithelial Neoplasms of the Ovary: Radiologic-Pathologic
Correlation. RadioGraphics 1994; 14:1351.
14. Woodward PJ, Hosseinzadeh K, Saenger JS. Radiologic Staging of Ovarian Carcinoma with Pathologic Correlation.
RadioGraphics 2004; 24:225.

Adrenal Imaging in Adults
Brent J. Wagner, MD
Neoplastic
• Adenoma
• Metastasis
• Lymphoma
• Pheochromocytoma
• Adrenocortical Carcinoma
• Myelolipoma
• Hemangioma (rare)
Non-neoplastic
• Hemorrhage
• Inflammation
• Hyperplasia
• Cyst
• Pseudocyst*
*may be secondary to neoplasm (adenoma)
Clinical manifestations of adrenal tumors

• Aldosteronism (hypertension, hypokalemia)
➢ 80% due to adenoma (Conn’s syndrome)
➢ 20% due to hyperplasia
➢ < 1% due to adrenal cortical carcinoma
• Virilization
➢ most due to hyperplasia
➢ 15% due to adenoma
➢ 5% due to carcinoma
• Cushing’s syndrome (hypertension, obesity, diabetes, etc) may be due to:
➢ exogenous steroids (most common)
➢ pituitary adenoma → ACTH production → bilateral adrenal hyperplasia
➢ non-pituitary tumor → ACTH production → bilateral adrenal hyperplasia
➢ 20% due to adrenal adenoma
➢ 10% due to carcinoma
• Catecholamine excess (hypertension, tachycardia, flushing, etc.)
➢ pheochromocytoma
Adenoma [Figure 3-14-1]

• (3% of the general population)
➢ Non-hyperfunctional (vast majority)
➢ Hyperfunctional (imaging features are the same as non-hyperfunctional)
Adenoma microscopic pathology Figure 3-14-1

• clear cells (high lipid content)
• cords of fibrovascular tissue
Adenoma (typical) macroscopic pathology

• well-circumscribed
• homogeneous
• small (usually less than 3 cm)
Adrenal adenoma
Genitourinary Radiology 645 Adrenal Imaging in Adults

Adenoma with degeneration (atypical) [Figure 3-14-2] Figure 3-14-2
• heterogeneous
• hemorrhagic
• cystic / necrotic
• calcifications
• (gross and radiologic appearance mimics carcinoma)
Adenoma radiology
• CT findings (NCCT):
➢ small, homogeneous
➢ hypodense due to lipid content (< 18HU?, <15HU?,
<10HU?) Degenerating adenoma
• CT findings (CECT):
➢ decreased enhancement compared to metastasis, etc.
➢ rapid wash-out of contrast? Figure 3-14-3
• Some adenomas are “lipid poor”
• A mass that does not satisfy the density requirements for an
adenoma may still be an adenoma (biopsy or washout study
required?)
• A mass that does not decrease in signal on an opposed phase
image may still be an adenoma
• MR (opposed phase imaging) [Figure 3-14-3]
➢ spleen used as internal reference
➢ visual assessment is generally adequate, although signal
intensity ratios of lesion:spleen may be used
• Opposed phase MRI operates on the same principle (lipid
content) as non-contrast CT, therefore will generally add little to
the patient work-up* (i.e. an indeterminate lesion by CT will likely
be indeterminate on opposed phase MRI).
* controversial
Adrenal Carcinoma [Figure 3-14-4]

• rare
• heterogeneous
• large ( mean >10 cm)
• necrotic
• percutaneous biopsy unreliable
• more than 1/3 are calcified
• half are hyperfunctional (these are generally smaller)
Adrenal adenoma, including opposed

phase imaging
Figure 3-14-4 Adrenocortical carcinoma with extension to inferior vena cava
Adrenal Imaging in Adults 646 Genitourinary Radiology

Pheochromocytoma [Figures 3-14-5 and 3-14-6] Figure 3-14-5
• almost all are abdominal
depends on definition – perhaps all are “adrenal”
• 90% are adrenal (the remainder are “paragangliomas”)
• 90% are unilateral
• 90% are “benign” — benignity established by clinical follow-up
• elevated catecholamines
• imaging generally performed for localization, not diagnosis
• CT:
➢ 3 - 6 cm mass
➢ heterogeneous when large (often cystic)
➢ calcification < 5%
• MRI:
➢ historically, characterized as very high signal on T2
➢ not totally specific, but normally needed only for localization
• MIBG (iodine-131-meta-iodobenzylguanidine):
➢ high sensitivity and specificity
➢ (but generally not needed and availability is limited)
Myelolipoma [Figures 3-14-7 and 3-14-8] Pheochromocytoma

• marrow elements: blood precursors and fat Figure 3-14-6
• benign — no malignant potential
• small lesions very unlikely to bleed
• usually incidental findings, but may present with hemorrhage
• may be diagnosed by needle biopsy (often not needed)
• rarely extra-adrenal (differential diagnosis — liposarcoma)
• most are predominantly fat attenuation (CT) or SI (MR)
• one third have calcification
• occasionally, associated with hormonal activity
Adrenal Mass Evaluation: Helpful features

• Mass is very large: favor adrenocortical carcinoma Pheochromocytoma
• Mass is between 2- 6cm in patient with hypertension: hyperfunctioning
adenoma vs. pheochromocytoma
• Mass contains a cystic portion and is less than 6 cm: pheochromocytoma
• Mass is primarily very high signal on T2: suggests pheochromocytoma
• Calcification: inflammatory, old hemorrhage, adrenocortical ca [unlikely: mets,
pheo]
• Small, homogeneous, hypodense = adenoma
Myelolipoma Myelolipoma
Genitourinary Radiology 647 Adrenal Imaging in Adults

Absolute washout calculation
• “percentage of enhancement washout”
• (HUdyn – HUdelayed) / (HUdynamic – HUpre)
• if greater than 60%, = adenoma
Relative washout calculation

• (HUdyn – HUdelayed) ?(HUdynamic)
• if > 40%, = adenoma
Algorithm
• NCCT:
➢ If less than 10 HU, it’s an adenoma [STOP]
➢ If more than 10 HU, proceed to:
• CECT: (dynamic and 15* minute delay)
❖ If less than 30* HU on delayed scan = adenoma ?
❖ If more than 30 HU on delayed scan, what is washout value
* = controversial
• Opposed phase MRI (OPMRI)
➢ decrease in signal relative to spleen = adenoma → no further evaluation
needed
➢ no decrease → biopsy
➢ indeterminate → consider CT evaluation [will probably need
“washout”/delay scans because the lipid content is probably too small to
make the lesion sufficiently hypodense]
References
1. Blake MA, Kalra MK, Sweeney AT, et al. Distinguishing beinign from malignant adrenal masses: multi-detector
row CT protocol with 10-minute delay. Radiology 2005; 238:578-85.
2. Blake MA, Slattery JMA, Kalra MK, et al. Adrenal lesions: characterization with fused PET/CT image in patients
with proved or suspected malignancy – initial experience. Radiology 2006; 238:970-77.
3. Caoili EM, Korobkin M, Francis IR, et al. Adrenal masses: characterization with combined unenhanced and delayed
enhanced CT. Radiology 2002; 222:629-33.
4. Elsayes KM, Narra VR, Leyendecker JR, et al. MRI of adrenal and extraadrenal pheochromocytoma. Am J Roentgenol
2005; 184:860-67.
5. Haider MA, Ghai S, Jhaveri K, Lockwood G. Chemical shift MR imaging of hyperattenuating (>10 HU) adrenal
masses: does it still have a role? Radiology 2004; 231:711.
6. Kenney PJ, Wagner BJ, Rao P, Heffess CS. Myelolipoma: CT and pathologic features. Radiology 1998; 208: 87-
95.
7. Korobkin M. CT characterization of adrenal masses: the time has come. Radiology 2000; 217:629.
8. Mayo-Smith WW. CT characterization of adrenal masses (letter). Radiology 2003; 226:289.
9. Savci G, Yazici Z, Sahin N, et al. Value of chemical shift subtraction MRI in characterization of adrenal masses. Am
J Roentgenol 2006; 186:130-53.
Adrenal Imaging in Adults 648 Genitourinary Radiology

Imaging of the Urinary Bladder and
Urethra
Brent J. Wagner, MD
Outline
• Bladder Figure 3-15-1
➢ filling defects
➢ wall thickening (+/– calcification)
➢ abnormal contour
• Urethra
➢ anatomy
➢ filling defects
➢ obstructive processes (strictures, valves)
Bladder: Filling defects

• neoplasm
• calculus
• clot
• fungus ball
• ureterocele
• endometriosis
• schistosomiasis
• (prostate)
Bladder: Types of Neoplasms

• transitional cell ca (TCC) (urothelial)
• squamous cell ca
• adenocarcinoma
• leiomyoma/sarcoma
• hemangioma
• metastasis
➢ invasion
➢ other
• embryonal rhabdomyosarcoma (child)
Bladder Neoplasms: (TCC), urothelial carcinoma

[Figure 3-15-1]
• males > females
• 80% over age 50 Urothelial carcinoma
• typically, projects into lumen
• papilloma = low grade TCC Figure 3-15-2
• irregular surface, “papillary”
• occasionally (30% ?) multifocal
• CT to assess extraluminal extent
• enhances on early CT scan; filling defect on delayed scan
Bladder neoplasms: Differential features

[Figures 3-15-2 and 3-15-3
• TCC = common
• squamous cell carcinoma = look for associated stones, history of
infection (Schistosomiasis?), or chronic indwelling catheter
• adenocarcinoma = often of urachal origin; look for calcified
anterior midline mass with prominent extracystic growth
Urachal Anomalies
• patent urachus
• umbilical-urachal sinus
Urachal carcinoma
• vesico-urachal diverticulum
• urachal cyst
Genitourinary Radiology 649 Imaging of the Urinary Bladder and Urethra
Filling Defects: (may be mobile) Figure 3-15-3
• clot
➢ often smooth
• stones
➢ shadowing on U/S, midline on supine radiograph
➢ occasionally radiolucent (or obscured) post-contrast
➢ history of infection (and/or)
➢ evidence for bladder outlet obstruction
❖ trabeculation
❖ hydroureter
❖ prostate impression
• fungus ball
➢ laminated, gas-containing
Filling Defects: Miscellaneous: [Figures 3-15-4 and 3-15-5]

• ureterocele
➢ smooth
• prostate Bladder leiomyoma
➢ midline, generally smooth
• endometriosis
➢ can look like anything Figure 3-15-4
• gastrointestinal inflammation
➢ Crohn’s
➢ diverticulitis
Wall thickening [Figure 3-15-6]

• I. cystitis and variants
➢ infection
❖ TB*
❖ Schistosomiasis*
❖ malakoplakia
❖ cystitis cystica (lobulated, diffuse)
Prostate carcinoma
➢ radiation*
➢ post-cytoxan*
• II. Neoplasm (TCC)
• III. Bladder outlet obstruction Figure 3-15-5
• IV. Inflammation/invasion
*may calcify
Malakoplakia
• most common in females with recurrent infection
• mimics infiltrating carcinoma
➢ cysto/bx to diagnose
• Michaelis-Gutman bodies
Cystitis cystica et glandularis

• etiology/significance is controversial (? inflammatory) Endometriosis
• regenerative / reparative
• “proliferative cystitis”
• may result in wall thickening, but typically there are no imaging Figure 3-15-6
findings
• prominent dilated glandular lumina
Tuberculosis
Imaging of the Urinary Bladder and Urethra 650 Genitourinary Radiology

Cystitis glandularis – is it pre-malignant?
• “Cystitis glandularis is so common that it may be considered a normal feature
of the vesical mucosa.”
• There are 2 types of cystitis glandularis
➢ “typical”
➢ “intestinal” (less common)
• “Diffuse cystitis glandularis of the intestinal type is termed intestinal metaplasia
and usually occurs in chronically irritated bladders such as those of
paraplegics or in patients with stones or long term catheterization . . . it is
associated with an increased risk of bladder carcinoma.”
• “It is only the intestinal type of cystitis glandularis that is associated with
adenocarcinoma.”
Young RH, Eble JN. Non-neoplastic disorders of the urinary bladder. In: Urologic
Surgical Pathology. Mosby 1997. pp 174–5.
Schistosomiasis
• calcification in 50%
• calcification is rare in transitional / urothelial carcinoma
• Schistosomiasis is a risk factor for squamous cell ca of the bladder
Regional enteritis (Crohn’s) or other gastrointestinal disease

• combination of regional wall thickening and invasion
• diverticulitis more common than regional enteritis
• associated findings with regional enteritis Figure 3-15-7
➢ calculi
➢ anterior/right (posterior/left for diverticulitis)
• progression:
➢ impression/thickening
➢ invasion
➢ fistula
Emphysematous cystitis
• urinary tract combined with uncontrolled diabetes mellitus
• gas may be intraluminal* as well as intramural
➢ linear, lucent streaks
• non-surgical condition
• treatment: antibiotics and insulin
• * if gas is only intraluminal, consider fistula
Abnormal contour
• smooth narrowing:
➢ pelvic lipomatosis
➢ pelvic hematoma
➢ (irregular narrowing = lymphoma, other mass?)
• focal outpouching (diverticula):
➢ bladder outlet obstruction
➢ stones/tumors/bleeding
➢ reflux/ureteral obstruction
❖ (especially in children)
Urethra: Anatomy
• posterior:
➢ prostatic
➢ membranous
• anterior:
➢ bulbous
➢ penile
Condyloma acuminata
Genitourinary Radiology 651 Imaging of the Urinary Bladder and Urethra

Urethrography: Technique Figure 3-15-8
• Clamp vs. catheter
• Fluoroscopic guidance
• Hand injection
• Usually, dilute (30%) contrast
Urethra: Masses/filling defects

• urethral carcinoma
➢ most are squamous (if proximal, consider
transitional/urothelial carcinoma) Acute and subacute
➢ 70% of cases in males are associated with postinflammatory gonococcal urethritis
stricture
➢ filling defect or irregular stricturing
• condyloma acuminata [Figure 3-15-7] Figure 3-15-9
➢ urethral disease in only 5% of pts with external lesions
➢ viral
Urethra: Strictures [Figure 3-15-8]

• post-inflammatory
➢ especially gonococcal (40% of strictures in the U.S.)
• post-traumatic Urethral diverticulum (male)
➢ includes iatrogenic
• may be associated with perineal fistula
Figure 3-15-10
Urethra: Obstructive processes [Figure 3-15-9]
• posterior urethral valve
• anterior urethral valve
➢ (vs. diverticulum)
➢ acquired or congenital
➢ may obstruct, or develop calculi
Urethra: Diverticulum of the female urethra [Figure 3-15-10

• outpouching of contrast
➢ may require double-balloon technique
• fluid-filled mass on CT, MR, or sonography
• associated with carcinoma (usually squamous)
Urethral diverticulum (female)

References
1. Beer A, Saar B, Rummeny EJ. Tumors of the urinary bladder: technique, current use, and
perspectives of MR and CT cystography. Abdom Imaging 2003; 28:868.
2. Hahn WY, Israel GM, Lee VS. MRI of female urethral and periurethral disorders. Am J
Roentgenol 2004; 182:677-82.
3. Pavlica P, Menchi I, Barozzi L. New imaging of the anterior male urethra. Abdom Imaging 2003;
28:180.
Yu J-S, Kim KW, Lee H-J, Lee Y-J, et al. Urachal remnant diseases: spectrum of CT and US findings.
RadioGraphics 2001; 21: 451.
Imaging of the Urinary Bladder and Urethra 652 Genitourinary Radiology

Non-Neoplastic Disorders Of The Ovary
And Adnexae And GTD
Outline
• Clinical and imaging characteristics of common non neoplastic ovarian and
adnexal pathology
• Role of CT / MR
• Gestational trophoblastic disease
Essential Clinical Information

• Age of patient
• Symptoms e.g fever, discharge
• Menstrual status and time in cycle
• Pregnancy status
• Previous surgery and medical history
• Drugs, e.g HRT, ovulation stimulation
Functional Ovarian Cysts

• Very common incidental findings occurring during normal ovarian cycle
• Failure of ovulation or development of fluid in corpus luteum
• Most regress spontaneously
Functional Ovarian Cysts

• Follicular
• Corpus luteal
• Theca lutein
Follicular Cyst [Figure 3-16-1]

• Failure of mature follicle to rupture or regress
• Usually 3–8 cm
• Unilocular simple cyst
• Well defined thin smooth wall
• Regress spontaneously (if <5 cm) or may respond to hormonal suppression
• Clinical or sonographic follow-up in 6–8 weeks
Figure 3-16-1
Follicular cyst of left ovary with resolution one month later. a. initial
transabdominal ultrasound. b. initial transvaginal ultrasound. Echoes
are artefactual. c. Follow up.
Genitourinary Radiology 653 Non-Neoplastic Disorders Of The Ovary And Adnexae

Normal Ovaries-MR [Figure 3-16-2] Figure 3-16-2
MR of normal ovaries. a. T1-w image: left ovary (arrow) is isointense to muscle. On T2-w (b
and c) images the right and left ovaries (arrows) contain multiple high signal follicles
Follicular Cyst [Figure 3-16-3] Figure 3-16-3

Corpus Luteum Cyst
[Figure 3-16-4]
• Persistence of corpus luteum or
bleeding into it
• >3 cm
• Unilocular
• Thick vascular wall
• Wall slightly echogenic
• CL cyst of pregancy regresses by
16 wk
Corpora Lutea
Symptomatic functional a. Sagittal and b. coronal T2-w MRI showing a right sided follicular
cysts cyst (arrow) with surrounding ovarian parenchyma
• Internal hemorrhage
• Rupture
➢ May rupture and bleed into peritoneal cavity with peritoneal
Figure 3-16-4
signs and hypotension
➢ Or rupture with simple free fluid
Hemorrhagic Functional Cysts [Figure 3-16-5]

• Hemorrage occurs into existing cyst
• Acute pain or asymptomatic
• More common in luteal cysts
• Imaging spectrum depends on age
• Rapid change
• Thin linear fibrin strands “reticular, fish net or lacy”
• Retracting hyperechoic clot
• Fluid – debris level
• Mildly thickened wall
• Diffuse low level echoes with acoustic enhancement “ground
glass”
➢ Rare, usually a feature of endometriosis
Figure 3-16-5
Thick walled unilocular cyst with low

resistance arterial flow in wall
Interval development of hemorrhage into a functional cyst.
Note fine lacy linear echoes (arrow)
Non-Neoplastic Disorders Of The Ovary And Adnexae 654 Genitourinary Radiology
Hemorrhagic Cyst [Figure 3-16-6] Figure 3-16-7
Figure 3-16-6
Acute left pelvic pain. US and CT of left ovarian hemorrhagic

cyst. Note fishnet appearance on US and hematocrit level on
CT (arrow)
MR
• Blood products Acute pain from cyst rupture.Note free
➢ High on T1 fluid and crenated cyst (bottom)
➢ Usually high on T2
Cyst Rupture [Figure 3-16-7]
Cyst Rupture with Hemorrhage [Figure 3-16-8]
Theca Lutein Cysts

• Gestational trophoblastic disease
➢ Associated with high levels of HCG Figure 3-16-8
• Ovarian hyperstimulation syndrome
➢ Secondary to infertility drugs
➢ Abdominal pain, distension, nausea,
vomiting
Hyperstimulation Cysts
• Bilateral enlarged ovaries
• Multiple large cysts
• May bleed, rupture or torse
• OHSS associated with ascites, pleural
effusion, hemorrhage, DIC
OHSS [Figure 3-16-9]
Endometriosis
• Functioning ectopic endometrium
• Pelvic peritoneum, ovary, tube
Endometriosis Acute pain and pelvic guarding. Bleeding from left corpus
• Symptoms luteum cyst * into peritoneal cavity. U= uterus
➢ Dysmenorrhea
➢ Dyspareunia
➢ Pelvic pain
• Cyclic pain with menses
• Associated with infertility
➢ Prevalence 25%

Figure 3-16-9
Ascites and bilateral enlarged ovaries with multiple cysts

Endometrioma - US Figure 3-16-10
[Figures 3-16-10 and 3-16-11]
• Thick walled cystic lesion
• “Ground glass” homogeneous low
level echoes (highly suggestive)
• Unilocular or multilocular with
septations
• Mural reflectors
• Rarely fluid-fluid level
Endometriosis:
MR Technique
• Axial T1-w SE Ground glass appearance of endometriotic cyst. a. Transabdominal
• *Axial fat-suppressed T1-w SE to and b. transvaginal ultrasound. Note homogeneous internal echoes
distinguish fat from blood* and posterior acoustic enhancement
• Axial/sagittal/coronal T2-w FSE
• Dynamic enhanced T1-w (optional)
Endometrioma: MR [Figure 3-16-12]

• Highly accurate, sensitive and specific (90-96%) Figure 3-16-11
• Thick walled cystic lesion
• Hyperintense on T1-w
• Hypointense on T2-w with “shading”
Endometrioma: MR
• Less specific signs
➢ Multiple homogeneous hyperintense lesions on
T1-w and T2-w
➢ Low signal hemosiderin ring
➢ Enhancement of cyst wall/peritoneum Homogeneous endometriomas (*) with mural
reflectors (arrows)
Endometrioma
Endometrioma with hematosalpinx
Rectus Endometriosis
Diffuse Endometriosis
• More common Figure 3-16-12
• Associated with fibrosis and adhesions
• Laparoscopy is gold standard allows
staging and treatment
• MR may be useful for inaccessible
sites or for evaluation of response to
medical treatment
High T1 signal ovarian endometrioma with no suppression on
fat sat. Low signal “shading” on FSE T2

Endometriosis
Figure 3-16-13
Endometrioma or Hemorrhagic Cyst?
• Sequential imaging with US
• MR
➢ Less bright on T1-w
➢ No shading on T2-w
➢ Single
“Rule Out Ovarian Torsion”
Ovarian (adnexal) Torsion [Figure 3-16-13]

• 3% of gynecologic emergencies
• Usually premenopausal
• 20% pregnant
• 80% associated mass
• Acute pain, nausea, vomiting
• Previous self limiting episodes
Ovarian Torsion - US
• Gray scale non specific, depends on cause
➢ Most suggestive: ipsilateral enlarged hypoechoic ovary (+/-
peripheral follicles)
➢ Mass e.g. teratoma, functional cyst
➢ Hemorrhagic infarction
➢ Associated thickened fallopian tube
Ovarian Torsion: Enlarged Ovary [Figure 3-16-14]

Absence of flow in torsed left ovary
Adnexal Torsion (bottom)
Ovarian Torsion - US Figure 3-16-14

• Doppler is extremely useful
➢ Absence of arterial and venous flow
➢ High resistance arterial flow
➢ Loss of venous flow
➢ Twisted vascular pedicle
➢ Corkscrew vessels
Ovarian Torsion [Figure 3-16-15]
Figure 3-16-15
Asymmetric ovarian sizes in torsion
A-CT of enlarged right ovary with hemorrhagic infarction.

Paraovarian cyst (arrow) caused the torsion.
B-Low signal ovary on T2 with folllicles.
C-T1 shows high signal from hemorrhage.
D-Post contrast sat sat T1 shows no enhancement in
right ovary

Torsed Teratoma [Figure 3-16-16] Figure 3-16-16
Adnexal Torsion: CT/MR

• Deviation of uterus to affected side
• Obliteration of fat planes
• Enlarged displaced ovary
• Beak sign with congested vessels
• Lack of enhancement
Torsion of Cystadenoma*
Ovarian Torsion Twisted Pedicle

[Figure 3-16-17]
Twisted vascular pedicle on color Doppler of a torsed teratoma
Ovarian torsion
caused by para-ovarian cyst
Figure 3-16-17
Ovarian Torsion
• Diagnostic difficulties
➢ Dual ovarian arterial supply
➢ Incomplete and intermittent torsion
➢ False positives
❖ Technical
❖ Pathologic
• High index of suspicion if symptomatic ovary enlarged
• Rescan early
Hydrosalpinx [Figure 3-16-18]

• Tubular fluid filled structure
• Folding mimics multilocular lesion
• Sequela of PID, endometriosis, surgery
Figure 3-16-18
Twisted pedicle (arrow): intra

operative image and CT
Dilated thin walled fallopian tube: US and CT
Hydrosalpinx/Pyosalpinx
Hydrosalpinx

Pelvic Inflammatory Disease Figure 3-16-19
• Imaging for:
➢ Complications
➢ Failure to respond to first line
treatment
➢ Alternative diagnosis
• US first line
• CT or MR for difficult / severe cases
• Thick walled tube
• Cog wheel
• Internal echoes
• Tuboovarian complex or abscess
➢ Complex adnexal mass with
pyosalpinx
Acute Salpingitis
Pyosalpinx [Figure 3-16-19]
Tuboovarian Abscess Aspiration
Tuboovarian abscesses a. Coronal and b. longitudinal ultrasound of a dilated

thickened tube with internal debris and endosalpingeal fold
Ovarian Vein Thrombophlebitis thickening (arrow). c. longitudinal scan of a thickened dilated
• Septic thrombosis in ovarian veins tube. d. pathologic specimen showing endosalpingeal folds
• Post partum or post surgery/pelvic (arrow)
inflammatory disease
• Pain, fever and leucocytosis Figure 3-16-20
• My be occult
• Treated with antibiotics and anticoagulants
• Distended ovarian vein
• Thrombus
• Perivenous inflammatory changes
• Edematous adnexa
Post Partum Thrombophlebitis [Figure 3-16-20]
Peritoneal Inclusion Cysts [Figures 3-16-21 and 3-16-22]

“Benign cystic mesothelioma, multilocular peritoneal cyst”
• Loculated peritoneal fluid within adhesions
• Previous pelvic surgery/endometrosis/PID
• Pre or post menopausal
• Treatment
➢ Surgical (30–50% recurrence)
➢ OCP +/– TV US guided aspiration
• May mimic a cystic ovarian neoplasm
• Septated cystic peritoneal lesion surrounding normal ovary
• Ovary suspended by adhesions “spider in a web”
• Flow may be present in septations
Figure 3-16-21
MR Venogram. Distended right

ovarian vein with intra luminal
thrombus (arrow). Uterus (*) is
enlarged with central fluid/clot
Multilocular cystic lesion surrounding normal ovary (o)

Polycystic Ovary Syndrome Figure 3-16-22
• Infertility and hormonal disturbance
• 3–7% of women
• “Stein Leventhal Syndrome”
➢ Amenorrhea, Infertility, Hirsutism
• Absence of mid cycle LH surge
• Increased LH: FSH
• Suspended follicular development
• Androgen production
Polycystic Ovary Syndrome

[Figures 3-16-23 and 3-16-24]
• Enlarged ovaries (>10–12 cm3)
• Multiple (>10) small (<8–10 mm)
peripheral follicles
• Echogenic stroma
• Normal ovaries (30%)
Figure 3-16-23
Large peritoneal inclusion cyst status post bilateral renal

transplants. The uterus (arrow) is displaced and compressed
by a large pocket of fluid which herniates into the perineum
Figure 3-16-24
Hirsutism and amenorrhea. Enlarged

ovaries with multiple peripheral
follicles
Enlarged ovaries with multiple small
follicles

Paraovarian/Paratubal Cysts
• 10–20% of adnexal masses
• Arise in broad ligament from mesothelial and paramesonephric remnants
• Any age (3rd–4th decades most common)
• Complicated by
➢ Hemorrhage
➢ Torsion
➢ Rupture
➢ Neoplasm
Paraovarian Cysts
• Simple unilocular adnexal cyst
• Separate from ovary
• Lack of change with time
• Rarely bilateral or multiple or complex
Serous Inclusion Cysts

• 17% of asymptomatic post menopausal women
• <3 cm, thin walled unilocular cysts
• Cyclic variation
• Secondary to remote ovulation with trapping of surface epithelium in ovarian
cortex
• Majority resolve, follow-up sonography
Epithelial Inclusion Cysts
Gestational Trophoblastic Disease

• Heterogenous group of disorders
• Abnormal proliferation of chorionic tissues
• Varying propensity to invade and metastasize
• Elevated beta HCG
➢ Hyperemesis, toxemia, bleeding

• Benign
➢ Hydatidiform mole
• Malignant
➢ Invasive mole
➢ Choriocarcinoma
➢ Placental site trophoblastic disease

• Chorionic villi of blighted ovum persist
• Hydropic change in placenta
Benign Hydatidiform Mole

• Most common (80%)
• 1 in 1200–2000 pregnancies (US)
• Risk factors
➢ Extremes of reproductive life
➢ Previous mole
➢ Risk of recurrence 1% after 1 mole
➢ 23% after 2 molar pregnancies
Benign Mole
Classic “complete” mole Partial mole
➢ 80% Triploid
➢ 46 XX 69 XXY 80%
➢ Complete molar change 69 XXX
➢ No fetal tissue Hydropic placenta
➢ Nuclear DNA paternal 0.5% malignant change
➢ 10% malignant change

Complete Mole - Sonography Figure 3-16-25
• Enlarged uterus
• Echogenic mass in
endometrial cavity
• Small cystic spaces
• Low impedance flow
• Theca lutein cysts
(20–50%)
• May mimic incomplete Transabdominal and transvaginal US of cystic endometrial mass.(left and
abortion, hydropic placenta center) Theca lutein cysts in right ovary (right)
Hydatidiform Mole [Figure 3-16-25]
Complete Hydatidiform Mole [Figure 3-16-26]

Figure 3-16-26
Theca Lutein Cysts
Partial Mole
• Triploid fetus
➢ IUGR, anomalies
• Hydropic placenta
• Spontaneous abortion
Gestational Thickened endometrium with myometrial hypervascularity (*).

Trophoblastic Disease Arrows: theca lutein cysts
• Management
➢ D&C
➢ Monitoring of beta HCG levels
❖ Exponential drop (near zero by 10–12 weeks)
➢ US to exclude pregnancy
➢ Invasive mole 10% → Chemotherapy
➢ Choriocarcinoma 5% → Chemotherapy
Recurrent Complete Mole
Malignant GTD
• Invasive mole
➢ Locally invasive, non metastatic, <10%
➢ Vesicular chorionic villi with myometrial invasion
• Choriocarcinoma Figure 3-14-28
➢ 5%, hematogenous metastases to lungs, brain, liver, etc.
➢ May not necessary follow a gestation
➢ No villous structure
Choriocarcinoma [Figures 3-14-27 and 3-16-28]
Figure 3-16-27
Infiltrating cystic mass in endometrial Infiltrating myometrial mass (top)

cavity and myometrium Lung metastases (bottom)

Summary
• Characteristic sonographic features allow diagnosis of most benign adnexal
masses
• MR useful for indeterminate adnexal mass
• HCG and ultrasound for diagnosis and follow up of GTD
References
1. Albayram F, Hamper UM. Ovarian and adnexal torsion: spectrum of sonographic findings with pathologic
correlation. J Ultrasound Med 2001;20:1083-1089.
2. Bennett GL, Slywotzky CM, Giovanniello G. Gynecologic causes of acute pelvic pain: spectrum of CT findings.
Radiographics 2002;22:785-801.
3. Christensen JT, Boldsen JL, Westergaard JG. Functional ovarian cysts in premenopausal and gynecologically
healthy women. Contraception 2002;66:153-157.
4. Descargues G, Tinlot-Mauger F, Gravier A, Lemoine JP, Marpeau L. Adnexal torsion: a report on forty-five cases.
Eur J Obstet Gynecol Reprod Biol 2001;98:91-96.
5. Green CL, Angtuaco TL, Shah HR, Parmley TH. Gestational trophoblastic disease: a spectrum of radiologic
diagnosis. Radiographics 1996;16:1371-1384.
6. Hertzberg BS, Kliewer MA, Paulson EK. Ovarian cyst rupture causing hemoperitoneum: imaging features and the
potential for misdiagnosis. Abdom Imaging 1999;24:304-308.
7. Jain KA. Imaging of peritoneal inclusion cysts. AJR Am J Roentgenol 2000;174:1559-1563.
8. Lee EJ, Kwon HC, Joo HJ, Suh JH, Fleischer AC. Diagnosis of ovarian torsion with color Doppler sonography:
depiction of twisted vascular pedicle. J Ultrasound Med 1998;17:83-89.
9. Levine D, Gosink BB, Wolf SI, Feldesman MR, Pretorius DH. Simple adnexal cysts: the natural history in
postmenopausal women. Radiology 1992;184:653-659.
10. Okai T, Kobayashi K, Ryo E, Kagawa H, Kozuma S, Taketani Y. Transvaginal sonographic appearance of
hemorrhagic functional ovarian cysts and their spontaneous regression. Int J Gynaecol Obstet 1994;44:47-52.
11. Pache TD, Wladimiroff JW, Hop WC, Fauser BC. How to discriminate between normal and polycystic ovaries:
transvaginal US study. Radiology 1992;183:421-423.
12. Patel MD, Feldstein VA, Chen DC, Lipson SD, Filly RA. Endometriomas: diagnostic performance of US.
Radiology 1999;210:739-745.
13. Rha SE, Byun JY, Jung SE, et al. CT and MR imaging features of adnexal torsion. Radiographics 2002;22:283-
294.
14. Sam JW, Jacobs JE, Birnbaum BA. Spectrum of CT findings in acute pyogenic pelvic inflammatory disease.
Radiographics 2002;22:1327-1334.
15. Siegelman ES, Outwater EK. Tissue characterization in the female pelvis by means of MR imaging. Radiology
1999;212:5-18.
16. Sohaey R, Gardner TL, Woodward PJ, Peterson CM. Sonographic diagnosis of peritoneal inclusion cysts. J
Ultrasound Med 1995;14:913-917.
17. Sugimura K, Okizuka H, Imaoka I, et al. Pelvic endometriosis: detection and diagnosis with chemical shift MR
imaging. Radiology 1993;188:435-438.
18. Togashi K, Nishimura K, Kimura I, et al. Endometrial cysts: diagnosis with MR imaging. Radiology 1991;180:73-
78.
19. Wagner BJ, Woodward PJ, Dickey GE. From the archives of the AFIP. Gestational trophoblastic disease:
radiologic-pathologic correlation. Radiographics 1996;16:131-148.
20. Woodward PJ, Sohaey R, Mezzetti TP, Jr. Endometriosis: radiologic-pathologic correlation. Radiographics
2001;21:193-216; questionnaire 288-194.

Imaging of Solid Organ Transplants
Transplantation
• Higher success rates with better anti rejection therapy, patient selection and
surgical techniques
• Rejection remains major cause of graft loss
• Immunosuppression predisposes to infection and neoplasm
• Symptoms and signs of infection subtle
Types of Transplants
• Renal
• Pancreas
• Liver
Post-transplantation Imaging
• Ultrasound -- primary modality
➢ Parenchymal echotexture
➢ Blood supply
➢ Specific complications
➢ Guidance for interventional procedures
• CT
➢ Collections
➢ Infection
➢ Surgical Complications
➢ Neoplasm
➢ Guidance for procedures
• MRI and MRA
➢ Parenchyma
➢ Vascular complications
➢ Masses
➢ Rejection?
• Other
➢ Scintigraphy
➢ Cystography
➢ Cholangiography
➢ Arteriography and intervention
Renal Transplants
• CRT: Cadaveric renal transplant
• LRT: Living related renal transplant
• LNRT: Living non-related renal transplant
• Dual: “En bloc” pediatric or two adult
• SPK: Simultaneous pancreas-kidney
Imaging of Solid Organ Transplants 664 Genitourinary Radiology

Renal Transplantation: Surgical Technique
• Iliac fossa extraperitoneal placement
• Arterial anastomosis
➢ End to side to external iliac artery
• Venous anastomosis
➢ End to side to external iliac vein
• Ureteral anastomosis very variable
Renal Transplants: Sonography

• Hydronephrosis, echotexture, collections
• Color Doppler: identify vessels
• Duplex Doppler of
➢ MRA, MRV
➢ Segmental, interlobar and arcuate arteries
➢ Measure RI – x3
• Real time guidance for biopsy
Normal Renal Transplant
Resistive Index
RI= (PSV–EDV)
PSV
Complications
• Perinephric fluid collections (50%)
• Rejection – acute and chronic
• Obstruction (1–10%)
• Vascular Complications (10%)
• Acute tubular necrosis (DGF)
• Cyclosporine toxicity
• PTLD (1%)
• Torsion
Perinephric Fluid Collections Figure 3-17-1

• Early
➢ Hematomas 9%
➢ Seromas
➢ Urinary leak 18% (1–2
weeks)
• Later
➢ Abscess 30%
➢ Lymphoceles 43% (4–8
weeks) Sub acute perinephric hematoma (Left).
• Aspirate for diagnosis Acute subcaspular hemorrhage with bleeding vessel post biopsy
(Center).
Hematoma [Figure 3-17-1] Organizing hematoma (Right)
• Appearance depends on age
• Acute hyperechoic
• Sub acute mixed Figure 3-17-2
• Chronic hypoechoic/anechoic
• Contained or free
Hemorrhage [Figure 3-17-2]
Acute perinephric high density hematoma.(Left). Free

intraperitoneal hemorrhage (Right)
Genitourinary Radiology 665 Imaging of Solid Organ Transplants

Urinoma [Figure 3-17-3] Figure 3-17-3
Lymphocele [Figure 3-17-4]
Abscess
Hydronephrosis
• Early: edema of UVJ
• Late:
Compression by fluid
collections
➢ Denervation (non
obstructive)
➢ Full bladder (repeat with
empty bladder)
➢ Ureteric ischemia,
surgical technique Cystogram showing extra luminal contrast (arrow) from ureteral leak.(Left)
(kinks) Renogram showing collection of radioactivity (arrow) inferomedial to
➢ Rejection transplant and non visualization of bladder (Right)
➢ Intraluminal clot or
calculi
Figure 3-17-5
Ureteral Stricture [Figure 3-17-5]
Hydronephrosis Figure 3-17-4

• Dilatation does not equal
obstruction
• RI not reliable in
differentiating dilatation
from obstruction
Echoes Within
Collecting System
• Hemonephrosis
➢ Low level echoes
➢ Move with patient
➢ Hematuria
➢ Post biopsy: look for
AVF
➢ Urinary infection
Hemonephrosis
[Figure 3-17-6]
Simple fluid collection causing some Hydonephrosis and hydroureter

mass effect on the transplant (Top) (arrow) (top)
Lymphocele (arrow) medial to Antegrade pyelogram showing distal
transplant (Bottom) ureteral stricture (arrow) (bottom)
Figure 3-17-6
a. Hydronephrosis with clot in collecting system. b. Clot in bladder. c. CT showing high density
blood in left transplant ureter (arrow) and d. clot in bladder (arrow) as well as Foley catheter

Candidiasis
• Fungus balls
➢ Highly echogenic, weakly shadowing
➢ Candida in urine
Echoes Within Collecting System

• Calculi or nepohrocalcinosis
➢ Echogenic structures with acoustic shadowing
Nephrocalcinosis
Gas Figure 3-17-7

• Emphysematous pyelonephritis
• Reflux from catheterization
Gas/Stent
Rejection
• Non specific elevation of creatinine
• Fever, white count, pain over transplant
• Decreased urine outpout
• Acute (> 5 days) reversible with treament
• Chronic (months to years) irreversible
Acute Rejection Gray Scale Edematous kidney with prominent pyramids

• Non specific (arrow), increased cortical echogenicity and
➢ Enlargement thickened urothelium (*)
➢ Increased cortical echogenicity
➢ Decreased echogenicity of central sinus
➢ Loss of corticomedullary differentiation
➢ Prominent pyramids
➢ Thickening of collecting system Figure 3-17-8
Acute Rejection
[Figure 3-17-7]
Thick Urothelium
[Figure 3-17-8]
Acute Rejection
[Figure 3-17-9] Circumferentially thickened renal pelvis (arrow) in acute rejection
• Vascular rejection results in
increased resistance with increase in resistive index
• Correlation highly variable
• Threshold? 0.7 or 0.9 Figure 3-17-9
Acute rejection
• BIOPSY - only reliable method to
determine cause of renal dysfunction
Chronic Rejection
• Small allograft
• Echogenic from fibrosis
• Fatty replacement
• Calcification High resistance arterial waveforms with reversal of diastolic
• Decreased blood flow flow in main renal artery and absence of diastolic flow in
interlobar arteries

Vascular Complications Figure 3-17-10
• Early (Surgical emergencies)
➢ Renal vein thrombosis
➢ Renal artery occlusion
• Later
➢ Renal artery stenosis (10%)
➢ Post biopsy complications (AVF,
PSA)
➢ Renal vein stenosis
Renal Vein Thrombosis [Figure 3-17-10]

• Gray-scale: swollen hypoechoic
• Doppler:
➢ Absent venous flow
➢ Reversed plateauing of diastole
➢ High resistance a. Power Doppler with minimal flow.b. Reversed arterial flow in
diastole. c. Absent venous flow. Beware of noise
Renal Artery Occlusion
• Gray-scale: swollen kidney
• Doppler
➢ Absent intrarenal arterial
➢ High resistance, high PSV, no
Figure 3-17-11
diastolic flow
➢ Spiked preocclusive wave form
• NB: Severe acute rejection can cause
diminished flow
Renal Artery Stenosis

• Hypertension, graft dysfunction and
bruit
• Conventional angiography
➢ Reference standard (invasive
contrast)
➢ Allows angioplasty
a. MRA of normal transplant renal artery. b. MRA showing
• Sonography for screening
diffuse atherosclerosis with a mild stenosis (arrow) of the
• MR Angiography
proximal renal artery. c. MRA of a high grade iliac stenosis
(arrow) above a normal transplant renal artery
MRA [Figure 3-17-11]
Sonographic Criteria
• PSV >2 m/s
• Velocity gradient >2:1
• Post stenotic spectral broadening
• Pulsus tardus-parvus( prolonged early acceleration, diminished amplitude
SAT >0.07s, AI <3 m/s2 , RI <0.56
Main Renal Artery Origin
Tardus Parvus
Intrarenal Arteriovenous Fistulae/Pseudoaneurysms

• Secondary to percutaneous biopsy
• Most clinically insignificant and resolve
• Treated conservatively if small and asymptomatic
• Embolized if large or causing ischemia and severe hematuria

Intrarenal Arteriovenous Fistulae
• Gray scale
➢ Usually invisible
• Color Doppler
➢ Flurry/perivascular bleeding
➢ Feeding artery – draining vein if large Figure 3-17-12
➢ Aliasing
• Duplex
➢ High velocity/low resistance
➢ Arterialized venous flow
Arteriovenous Fistula
[Figure 3-17-12]
Arteriovenous Fistula
Embolization [Figure 3-17-13]
Pseudoaneurysms
[Figure 3-17-14]
• Gray scale
➢ Simple or complex cyst
• Doppler
➢ Yin yang swirling disorganized flow
➢ To and fro (neck)
• May rupture a. Color; b. Power Doppler of perivascular thrill.
c. duplex of artery and d. of draining arterialized vein
Renal Vein Stenosis
• Perivascular fibrosis
• Compression by fluid collections
• Doppler Figure 3-17-13
➢ Aliasing
➢ Velocity increase (x3–4)
Pancreas Transplants
• SPK: Simultaneous pancreas-kidney
• PAK: Pancreas after kidney
• PTA: Pancreas transplant alone
Pancreatic Transplantation:
Surgical Technique Selective renal arteriography. a. abnormal distal arterial branch
• Endocrine Drainage (Venous) with early venous filling (arrow). b. Prompt filling of renal and
➢ Systemic (iliac vein) iliac veins. c. Post embolization, the av fistula is no longer
➢ Portal vein visualized
• Exocrine Drainage
➢ Bladder
➢ Enteric
• Arterial supply from common iliac artery Figure 3-17-14
SPK
• Systemic bladder drainage
• Portal enteric drainage
Pancreatic Transplant
Complications
• Rejection Acute and Chronic
• Surgical complications a. Gray scale; b. color and c. duplex Doppler of
➢ Infection pseudoaneurysm.
➢ Anastomotic Leak Note to and fro flow in neck of pseudoaneurysm (arrow)
• Vascular thrombosis Arterial / Venous
• Pancreatitis

Normal Pancreas Transplant [Figure 3-17-15] Figure 3-17-15
Pancreas Transplant CT
[Figure 3-17-16]
Pancreatic Transplant MR
Pancreas Transplant MRA
Peripancreatic Collections
• 2-10%
• Hematoma, seroma, anastomotic leak,
abscess
• Nonspecific appearance
• Aspiration needed for diagnosis
Anastomotic Leak with Abscess

[Figure 3-17-17]
Pancreatic Transplant Rejection

• 40% of graft loss
Gray scale, color and duplex of
• Enlargement and heterogeneity of gland
normal pancreas transplant.
• US Doppler RI – no correlation
Arrow on pancreatic duct
• Diagnosed by percutaneous US guided
biopsy
Pancreatic Transplant Vascular Thrombosis

• 6–10% of graft loss Figure 3-17-16
• Venous more common than arterial
• US and MRA most useful
• Swollen heterogenous gland
• No flow or enhancement
• Thrombosed vessels
Pancreatic Thrombosis
[Figure 3-17-18]
Liver Transplantation
• Established or fulminant liver failure
(hepatitis C, PBC, PSC, alcolhol,
cryptogenic cirrhosis, etc.)
• Cadaveric
• Living or cadaveric split liver (right lobe)
Reformatted coronal CT showing pancreas transplant * and
Liver Transplantation vessels (arrows)
• Gray scale evaluation includes
➢ Free fluid (ascites or bile) Figure 3-17-17
➢ Biliary dilatation – choledochojejunostomy or
choledocholedochostomy
➢ Parenchyma
Liver transplantation
• Doppler evaluation includes
➢ MPV, LPV, RPV
➢ CHA, LHA, RHA
➢ HV x 3
➢ IVC above and below anastomosis
Ultrasound (a and b) and CT(c) of a peripancreatic

fluid collection containing gas (arrow). P pancreas

Complications Figure 3-17-18
• Rejection
• Vascular thrombosis or stenosis
• Biliary obstruction or leak
• Recurrent hepatitis
• Fatty infiltration
• Neoplasm
Hepatic Artery Thrombosis

[Figure 3-17-19]
MRA
• Normal
• Hepatic artery thrombosis
Hepatic Artery Thrombosis

[Figure 3-17-20]
a. Absence of color flow on color Doppler. b. Swollen pancreas

transplant. c. Non enhancement of pancreas.following contrast
d. Stump of graft artery
Figure 3-17-19
Figure 3-17-20
High resistance arterial flow secondary to thrombosis just distal

to site of sample. Proximal hepatic artery (arrow) with high
resistance pattern of flow
Digital subtraction arteriogram

demonstrating hepatic thrombosis.
The splenic artery (arrow) is patent.
Percutaneous cholangiogram shows
diffuse abnormality of the bile ducts
with strictures and filling defects
(arrow) resulting from ischemic bile
ducts

Bilomas after Arterial Thrombosis [Figure 3-17-21] Figure 3-17-21
Portal Vein Thrombosis [Figure 3-17-22]
Cavernous Transformation
Biliary Tree
Collections
Post Transplant Lymphoproliferative

Disorder
• Related to Epstein Barr virus
• Any time (mean = 15 months)
• Spectrum
➢ Polyclonal diffuse B cell proliferation Despite revascularization after hepatic artery thrombosis,
➢ Malignant monoclonal lymphoma multiple bilomas (*) have developed in the liver on CT
• Treatment and MR. Hepatic artery
➢ Decreased immunosuppression
➢ Antiviral agents Figure 3-17-22
➢ Chemotherapy
• Radiology
➢ Lymphadenopathy
➢ Solid/hollow visceral
involvement
❖ Liver
❖ Lungs
❖ Spleen
❖ Bowel
Portal vein thrombosis after liver transplant
Post Transplant a and b. Coronal reformatted CT showing thrombus (arrow) within the
Lymphoproliferative superior mesenteric and portal veins.
Disorder [Figure 3-17-23] c. Color Doppler ultrasound showing echoes in the portal vein with lack of
color flow. The adjacent hepatic artery is patent
PTLD SBO [Figure 3-17-24]
Figure 3-17-24
Figure 3-17-23
Multiple hepatic lesions on CT (a) and enhanced MR (b).

Mass in the transplant kidney (c) which was biopsied (d)
under ultrasound guidance
Soft tissue mass (arrows) causing

small bowel obstruction
(arrowheads)

PTLD Post Liver Transplant
Post Transplant Malignancy

• Kaposi’s sarcoma x 400–500
• Lymphoma x 20–350
• Vulva/perineum x 100
• Lip x 29
• Skin (squamous) x 7–40
• Cervix x 4–14
Lymphadenopathy
Summary
• Ultrasound with color and duplex Doppler is an ideal first line modality for
renal, pancreas and liver transplants
• Sensitive for vascular complications, fluid collections and hydronephrosis
• Biopsy needed for diagnosis of rejection
• CT for infection, fluid collections, procedures, malignancy
• MR for evaluation of vascular and parenchymal abnormalities
References
1. Baxter GM. Ultrasound of renal transplantation. Clin Radiol 2001; 56:802-818.

2. Boeve WJ, Kok T, Tegzess AM, et al. Comparison of contrast enhanced MR-angiography-MRI and digital subtraction
angiography in the evaluation of pancreas and/or kidney transplantation patients: initial experience. Magn Reson
Imaging 2001; 19:595-607.
3. Crossin JD, Muradali D, Wilson SR. US of liver transplants: normal and abnormal. Radiographics 2003; 23:1093-
1114.
4. Dachman AH, Newmark GM, Thistlethwaite JR, Jr., Oto A, Bruce DS, Newell KA. Imaging of pancreatic
transplantation using portal venous and enteric exocrine drainage. AJR Am J Roentgenol 1998; 171:157-163.
5. Hohenwalter MD, Skowlund CJ, Erickson SJ, et al. Renal transplant evaluation with MR angiography and MR
imaging. Radiographics 2001; 21:1505-1517.
6. Kaushik S, Fulcher AS, Frable WJ, May DA. Posttransplantation lymphoproliferative disorder: osseous and hepatic
involvement. AJR Am J Roentgenol 2001; 177:1057-1059.
7. Krebs TL, Daly B, Wong JJ, Chow CC, Bartlett ST. Vascular complications of pancreatic transplantation: MR
evaluation. Radiology 1995; 196:793-798.
8. Linkowski GD, Warvariv V, Filly RA, Vincenti F. Sonography in the diagnosis of acute renal allograft rejection and
cyclosporine nephrotoxicity. AJR Am J Roentgenol 1987; 148:291-295.
9. Middleton WD, Erickson S, Melson GL. Perivascular color artifact: pathologic significance and appearance on color
Doppler US images. Radiology 1989; 171:647-652.
10. Middleton WD, Kellman GM, Melson GL, Madrazo BL. Postbiopsy renal transplant arteriovenous fistulas: color
Doppler US characteristics. Radiology 1989; 171:253-257.
11. Sebastia C, Quiroga S, Boye R, Cantarell C, Fernandez-Planas M, Alvarez A. Helical CT in renal transplantation:
normal findings and early and late complications. Radiographics 2001; 21:1103-1117.
12. Tobben PJ, Zajko AB, Sumkin JH, et al. Pseudoaneurysms complicating organ transplantation: roles of CT, duplex
sonography, and angiography. Radiology 1988; 169:65-70.
13. Vrachliotis TG, Vaswani KK, Davies EA, Elkahammas EA, Bennett WF, Bova JG. CT findings in posttransplantation
lymphoproliferative disorder of renal transplants. AJR Am J Roentgenol 2000; 175:183-188.
14. Wong JJ, Krebs TL, Klassen DK, et al. Sonographic evaluation of acute pancreatic transplant rejection: morphology-
Doppler analysis versus guided percutaneous biopsy. AJR Am J Roentgenol 1996; 166:803-807.

The Neglected Nephrogram
Goals
• Review normal nephrographic physiology
• Present 6 abnormal patterns with the respective differential diagnosis
Urogram Does Not Equal Nephrogram
Nephrogram Figure 3-18-1

• Excretory Urography
• CT
• MR
• Nuclear Medicine
• Angiography
• ? Ultrasound
Normal - Nephrographic
- Physiology
Renal Vascularity Normal CT enhancement. Left, enhanced CT in the vascular

• Most flow is to the cortex
(corticomedullary phase); middle, nephrographic phase; right, pyelographic
• Vasa recta to medulla
phase
• Capsular vessels may
supply peripheral nephrons
• #2,#3 important for rim NG
and reverse NG
Normal Nephrographic Pysiology

• The main driving force for urine production is filtration pressure (blood
pressure)
• Contrast is filtered. It is not excreted or reabsorbed by the tubules
• Contrast which gets into the nephron will eventually get to the collecting
system
Density of the Nephrogram (3 Factors)

• Iodine concentration Figure 3-18-2
• GFR
• Transit time
Normal Nephrogram requires

• Kidney
• Blood in
• Blood out
• Urine out
• Nephrons
Normal Pyelogram
[Figures 3-18-1 and 3-18-2]
• Symmetric
• 3 minute film
• Delayed side is the diseased side
Normal MR enhancement. Enhanced T1-weighted with fat

suppression dynamic scan of the left kidney
The Neglected Nephrogram 674 Genitourinary Radiology

6 Nephrographic Patterns
• Absent NG Figure 3-18-3
• Unilateral delayed pyelogram
• Bilateral persistent NG
• Rim NG
• Reverse rim NG
• Striated NG
Pattern #1 Absent [Figure 3-18-3]

• Diagnosis:Renal agenesis with seminal
vesicle cyst
Renal agenesis with ipsilateral seminal vesicle cyst. Left,
Pertinent Embryology enhanced CT: there is no left kidney in the left renal fossa
• Ureteral bud comes off of the which is filled by large bowel. Right, CT at the level of the
mesonephric duct bladder shows extrinsic impression of the left posterior bladder
• Close association of mesonephric duct by a cystic mass
and ureter
Following nephrectomy the small bowel fills the renal fossa

[Figure 3-18-4]
17 Yo, Trauma: Global Infarction [Figure 3-18-5]
Nonfunction of the nonobstructed kidney

equals vascular occlusion Figure 3-18-4
No Blood In
20 YO Woman, Rt flank pain, hematuria,

facial rash [Figure 3-18-6]
No Blood Out
Bowel in the left renal fossa. CT scans of 2

different patients. Left: congenital absence or
ectopia of the left kidney with large intestine filling
Figure 3-18-5 the renal fossa. Right: acquired absence of the
kidney (nephrectomy) with small bowel filling the
left renal fossa
Figure 3-18-6
Global infarction. Enhanced CT.

There is an absent left nephrogram
due to disruption of the left renal
artery
Right renal vein thrombosis. Left:

enhanced CT shows absent
nephrogram in the right kidney which
is enlarged. Right: IV cavogram
shows thrombus in the IVC which
had extended into the right renal vein
Genitourinary Radiology 675 The Neglected Nephrogram

74 Year Old Man, BPH [Figure 3-18-7]
• There was no dilated right ureter on lower images
• Right UPJ with post obstructive atrophy
Figure 3-18-7
No Urine Out
22 YO man, Hematuria [Figure 3-18-8]

• Multicystic dysplastic kidney
32 year old woman, RUTI’S

[Figure 3-18-9]
Absent Left Nephrogram

[Figure 3-18-10]
• Renal TB
Long standing right UPJ
Figure 3-18-10 obstruction with post
obstructive atrophy. Right,
IVP with non visualization of
the right kidney. Left,
enhanced CT shows a left
dilated pelvis and calyces
Figure 3-18-8
Multicystic dysplastic kidney.

Left, KUB with multiple
peripheral calcifications in the
left renal fossa. Right, IVP
shows absent function on the left
Autonephrectmy from
renal tuberculosis. Top,
KUB shows multiple ill- Figure 3-18-9
defined calcifications in
the left kidney. Bottom,
IVP shows no function
No Nephrons Xanthogranulomatous pyelonephritis. Upper left, KUB shows a

• Congenital (MCK) left staghorn caclculus. Upper right, IVP shows left
• Acquired (XGP, TB) nonfunction. Bottom, CT demonstrates replacement of the left
kidney by and inflammatory mass which extends into the
perirenal space

Pseudo-Absent Nephrogram [Figure 3-18-11] Figure 3-18-12
• Pelvic kidney
Figure 3-18-11
Pelvic kidney. Left, CT through the kidneys shows an absent

right kidney with colon in the right renal fossa. Right, lower
section shows a nonrotated right pelvic kidney (arrows)
Pattern #1 Absent Nephrogram
• No kidney
• No blood in
• No blood out
• No urine out
• No nephrons
Pattern #2 Unilateral Delayed Pyelogram [Figure 3-18-12]

• TCC left UVJ (slow urine out)
Slow Urine Out
10 year old boy hypertension [Figure 3-18-13]
Renal Artery Stenosis (Slow Blood In)

• Delayed pyelogram
• Small kidney
• Hyperdense pyelogram
Slow Blood In TCC left UVJ. Upper left, IVP: there

is delay of the left pyelogram. Upper
Diagnosis: Compression of Renal Vein by Pancreatic right, left kidney: There is
Carcinoma (Slow Blood Out) [Figure 3-18-14] hydronephrosis. Lower right,
bladder near the UVJ: there is a soft
tissue mass (arrow)
Figure 3-18-13
Figure 3-18-14
Renal vascular hypertension

(Takaysu’s). Left, axial CT: Upper, axial CT: There is a mass (M) ventral to
the left kidney is small and the the left kidney. The left kidney is in the
pyelogram is delayed. There corticomedullary phase while the right kidney is in
is soft tissue around the aorta. the nephrographic phase. Lower, axial CT at the
Right, sagital MR: there is level of the left renal vein. The left renal vein
narrowing of the aorta (arrows) arrow) is compressed by the mass

Slow Blood Out Figure 3-18-15
75 Yo Woman Left Flank Pain

[Figure 3-18-15]
• Acute pyelonephritis
• Poor nephron function
Poor Nephron Function
Pattern #2 Delayed Pyelogram (Unilateral)

Acute pyelonephritis. Axial CT: the
• Slow urine out (OBST uropathy)
left kidney has a diminished
• Slow blood in (RA stenosis)
nephrogram and delayed pyelogram
• Slow blood out (RV compress)
• Poor nephron function (pyelonephritis)
In Cases with A Delayed Pyelogram There May Be An Ipsilateral

Hyperdense Nephrogram Figure 3-18-16
• Slow urine out
• Slow blood in
• Slow blood out
22 YO man, flank pain, hematuria [Figure 3-18-16]

• Obstruction of renal pelvis by blood clot
Pyelonephritis
• Rarely produces a unilateral hyperdense nephrogram (unless
there is tubular or pelvic obstruction with pus)
Pattern #3 Persistent Bilateral NG [Figure 3-18-17]

• Diagnosis:Hypotension
Figure 3-18-17 Hyperdense nephrogram from pelvic

obstruction by clot. Upper, noncon
CT: there is hyperdense blood in the
right renal pelvis. Lower, enhanced
CT: there is a delayed right
pyelogram and a hyperdense right
nephrogram
Hypotension. Left, 10 min IVP: there are bilateral persistent

nephrograms with delayed pyelograms. Right, 20 min IVP with
correction of the hypotension: normal examination Figure 3-18-18
Contrast 3 days ago [Figure 3-18-18]

• Acute tubular necrosis (ATN)
ATN
• Tubular damage and obstruction
• Decrease blood flow
• “Acute vasomotor nephropathy”
Renogram
• ATN
• Bilateral ureteral obsruction
• Bilateral renal artery stenosis Acute tubular necrosis. Axial CT
(contrast 3 days ago): There are
persistent bilateral nephrograms with
delayed pyelograms

Pattern #3 Persistent Bilateral Nephrogram
• Hypotension
• Intra renal obstruction Figure 3-18-19
➢ ATN
➢ Urate
➢ Protein
➢ Myoglobin
• Less likely
➢ Bilateral ureteral obstruction
➢ Bilateral renal Artery stenosis
➢ Bilateral renal vein thrombosis
Pattern #4 Rim Nephrogram [Figure 3-18-19 Global infarction with rim

• 21 yo man trauma 10 days ago nephrogram. Enhanced CT: The
right kidney is normal. The left
Pattern #5 Striated Nephrogram [Figure 3-18-20] kidney shows enhancement near the
coriticomedullary junction and in the
5 Patterns subcapsular area resulting in a “rim”
• Absent NG of enhancement. This is the same
• Unilateral delayed pyelogram case as figure 5, 10 days later
• Rim NG
• Striated NG Figure 3-18-20
Acute Cortical Necrosis

• Decreased blood flow to the cortex
• Continued perfusion to the
subcapsular and juxtamedullary
cortex
• Leads to renal failure
• Late cortical nephrocalcinosis
#6 Striated Nephrogram
[Figure 3-18-21]
• ARPCK
• Acute Pyelo
• Obstruction
• RVT
• Contusion
• Hypotension
• Tubular Obst
• Normal
Figure 3-18-21 Acute cortical necrosis. Enhanced CT scans: There is no

cortical enhancement with selective enhancement of the
medulla
Case courtesy Dr. Parvi Ramchandai University of Pennsylvania
Acute pyelonephritis. The striated

nephrogram shows alternating bands
of density and lucency

6 Patterns Figure 3-18-22
• Absent NG
• Unilateral delayed pyelogram
• Rim NG
• Reverse rim NG
• Striated NG
Each Pattern May Be

Segmental or Subsegmental
[Figure 3-18-22]
• Tubular obstruction
• Shock
• Pyelo
• Infarct
Segmental abnormal nephrograms, 4 different cases. Upper left:

tubular obstruction, upper right: hypotension, lower left:
pyelonephritis, lower right: lobar infarction
References
1. Davidson AJ, Hartman DS, Choyke PL, Wagner BJ. Davidson’s Radiology of the Kidney & Genitourinary Tract 3rd
Edition, W.B. Saunders Philadelphia 1999.

Problem Renal Masses
Learning Objective
• To use radiological imaging for the characterization and management of the
problematic renal mass
Centennial Sounding Board

Personal Refelection on Growth of Diagnostic Imaging
• “As we accurately image and inspect the human body with thinner and more
detailed sections, we approach the 1–2 mm serial sections of the pathologist,
who can find evidence of “disease” in almost every organ and everyone.”
• “The radiologist of the future will need to understand the implications of their
findings and know the natural history of each disease detected.”
Robert J. Stanley, AJR 2000;174:609

• Principles of Neoplasia
• Small Renal Mass
• Cystic Renal Mass
Principles of Neoplasia (4 Arbitrary Steps)

• Carcinoma In Situ (CIS)
• Angiogenesis
• Metastasis
Cell Cycle
• Proliferation
• Programmed death (Apoptosis)
Normally Cell Proliferation and Apoptosis are Activated in

Parallel
• Controlled by genes
Neoplasia
• Results from disequilibrium of proliferation and cell death
Chromosomal Instability Pathway

• Multi-step process
• Numerous genetic events
• Can stop at any point
Carcinoma In Situ
• Confined by basement membrane
• Stops expanding after reaching diffusion limit of the nearest vessel
• “No” metastatic potential
• Very, very common
Carcinoma In Situ
• Most human tumors exist as in situ lesions
➢ 0.2– 2 mm
• Renal CIS is found in 22% of autopsies
Angiogenic Phenotype
• Ability to recruit host blood supply
• Penetrate basement membrane
• May enlarge to become macroscopic
Genitourinary Radiology 681 Problem Renal Masses

Virtually All Solid Tumors Which are Visible Are Angiogenesis
Dependent
Vascular Invasion
• Tumor shedding and vascular invasion may occur relatively early
• In animal models, tumors shed 3-6 million cells per Gram per 24 hours
• Most cells which are shed do not progress to viable metastases
Metastasis
• Very imprecise at knowing which, where and when RCC will metastasize
• Mets require activation of genes
• Each metastasis must become angiogenic to grow
Nonangiogenic Metastases May Remain Microscopic and

Dormant for Many Years
Figure 3-19-1
• Principles of neoplasia
• Small renal mass
• Cystic renal mass
Rationale For Management Decisions
Best Way to Treat RCC Excision or Ablation

• Chemotherapy, radiation and immunotherapy are
less effective
Critical Feature of RCC Noncon Enhanced

• Metastases
Cyst, AML and solid nonfatty mass in the right
Renal Tumors <3 cm Uncommonly Have kidney. Left, nonenhanced and right, enhanced
Detectable Metastases axial CT through the kidney. A is a 1.8 cm cyst. B
• The renal tumor doesn’t “know” how large it is is a 2 cm AML. C is a 1.7 cm nonfatty mass which
• The larger the renal tumor, the more undifferentiated most likely represents a renal epithelial tumor with
it may be a low probability of metastasis
• The more undifferentiated, the greater the liklihood that a metastasis can
become angiogenic
Pathology / Radiology
The Small (< 3 cm) Renal Mass

• 1.5 - 3.0 cm
A . Cyst
B . Cystic
C . Solid
• < 1.5 cm (often TSTC)
The Small Solid Renal Mass [Figure 3-19-1]

• Fat = AML
• No Fat
A . Renal Epithelial Tumor with low metastatic potential
B. Cannot diagnose Adenoma (Oncocytoma)
Management Options
• Excise
• Ablation
• Follow
• Biopsy
• Nephrectomy
• Ignore
Problem Renal Masses 682 Genitourinary Radiology

The Small (< 3 cm) Renal Mass
• 1.5 - 3.0 cm
A . cyst Figure 3-19-2
B . cystic
C . solid
• < 1.5 cm (often TSTC)
Too Small To Characterize

• No single algorithm for every case
• Risks and benefits of any strategy
Always Consider
• Pretest probability Enhanced CT scans of 2 different patients both of
• Patient’s ability to tolerate uncertainty which have a lesion which is too small to
• Your ability to tolerate uncertainty characterize. Left, renal cell carcinoma; right,
renal cyst
How Should The TSTC Mass Be Managed?
• There is no large, prospective, pathologically proven series which indicates
correct management
RCC / Cyst [Figure 3-19-2]
Too Small to Characterize (< 1.5 cm)

• 1. Ignore
• 2. Follow
• 3. Get another study
How Often Should Small Lesions Be Followed?

• The smaller the lesion, the longer the followup interval
• If following a lesion, compare oldest comparable study available Figure 3-19-3
How Is Doubling Time Calculated?
http://www.chestx-ray.com/index.html
Small Lesion Considered Aggressive

• Size >3cm
• Doubling time faster than 6 months
Too Small to Characterize (< 1.5 cm)

• 1. Ignore
• 2. Follow
• 3. Get another study
T2 Fat Sat: Simple Cyst [Figure 3-19-3]

• Value of getting a T2 fat sat MR when the CT is
equivocal
Get the Referring Doctor Involved

Simple cyst confirmed on MR. Upper CT scans
Get the Patient Involved (unenhanced, corticomedullary and pyelographic
phases): the mass (arrow) is too small to
Problem Renal Masses characterize. Below, T2 fat sat MR: the lesion is
• Principles of neoplasia homogeneously bright
• Small renal mass
• Cystic renal mass

Teaching Goal
• To allow you to suggest the appropriate management for cystic renal
masses:
➢ Ignore
➢ Excise
➢ Follow
Rationale For Management

• 10% of cases of renal carcinoma present as a fluid-filled mass
• The simple cyst can be confidently diagnosed by ultrasound CT or MR
• Rarely simple cysts become complicated
➢ Hemorrhage
➢ Infection
➢ Ischemia
• On gross examination, the complicated cyst may be indistinguishable from
cystic renal cell carcinoma
• Differentiation is based upon histological diagnosis
The most effective treatment for renal cell carcinoma

• Surgery or Ablation
A cyst not simple if it has any of the following
Renal Carcinoma Can Grow As A Fluid Filled Mass

• Calcification
• Hyperdense/high signal
• Septations
• Multiple locules
• Enhancement
• Nodularity
• Thick wall
Although microscopic evaluation is required for precise

diagnosis, there are certain radiological findings which are
reliable in differentiating complicated cyst from cystic renal cell
carcinoma
Caveats
• The portion of the cystic mass which is most worrisome should be used in
deciding appropriate management.
• In cases with discordant imaging findings utilizing different radiological
examinations, the lesion should be managed based upon the most aggressive
imaging findings
What About Biopsy?

• If there is a strong clinical suspicion that the mass is inflammatory, careful
puncture is acceptable. If there is evidence of infection, treat (antibiotics,
drainage, etc.) and follow
• Renal neoplasia very rarely presents with infection
Guidelines
• Ignore
• Excise
• Follow
Calcification
• Ignore
➢ “Small” amount
➢ Smooth
➢ Septal
➢ Milk of calcium
➢ No enhancement

• Excise Figure 3-19-4
➢ Enhancement
➢ Wall thickening
➢ Nodularity
• Follow
➢ Thick
➢ Nodularity
➢ No enhancement
Hyperdense/High Signal [Figure 3-19-4]

• CT: > 20 HU on unenhanced CT
Hyperdense/High Signal [Figure 3-19-5]

• MR: higher signal intensity than water on T1-weighted images
Most Hyperdense Masses Are Cystic Hyperdense renal masses.

• Blood Unenhanced CT: there are two
• Protein masses (arrows) which are denser
• Colloid than water. Without contrast, it is
impossible to state if they are cystic
Hyperdense Masses May Be Solid [Figure 3-19-6] or solid
• Lymphoma
• RCC (papillary)
• AML (small amt of fat) Figure 3-19-6
• Mets
Figure 3-19-5
Renal lymphoma (solid).

High signal renal mass. T1-weighted Unenhanced CT scan shows several
axial MR of the left kidney: the signal hyperdense masses some of which
of the mass is higher that that of are confluent (arrows)
water
Hyperdense/High Signal
• Ignore
➢ Sharp margin
➢ < 3 cm
➢ Not completely intrarenal
➢ Homogeneous or hematocrit
➢ No enhancement
➢ US: cyst or cystic
Significant Enhancement
• CT
➢ <10 H.U.=Beam hardening
➢ 10-15 H.U.=Indeterminate
➢ >15 H.U.=Vascularity

• MR
➢ <15% relative enhancement = Benign
➢ >15% relative enhancement =
➢ Surgical
Hyperdense/High Signal
• Excise
➢ Poorly defined
➢ Heterogeneous
➢ Enhancement
➢ US: solid
• Follow
➢ Totally intrarenal
➢ > 3 cm
Septation
• Ignore
➢ Thin (< 1 mm)
➢ Smooth
➢ May calcify
➢ No enhancement
• Excise
➢ Thick, irregular
➢ Nodular
➢ Enhancement
• Follow
➢ Only slightly “greater than hairline”
A cyst not simple if it has any of the following Figure 3-19-7

• Calcification
• Hyperdense/high signal
• Septations
• Multiple locules
• Enhancement
• Nodularity
• Thick wall
With More Than 3 or 4 Septa,

Multiloculated [Figure 3-19-7]
Multiloculated Masses Three multiloculated masses. Left: ultrasound; middle: enhanced CT;
• Excise right: T2-weighted axial MR
➢ All multiloculated masses
ML-RCC
Multilocular Cystic Nephroma (MLCN)
MLCN ML-RCC
• Female Male
• No Blood Blood
• Pelvic Herniation Venous Invasion
• Usually Benign Always Malignant
Wall Thickening, Enhancement, Nodularity: Cannot Tell Benign

From Surgery
Enhancement
• Excise
➢ All enhancing masses

Nodularity Figure 3-19-8
• Excise
➢ All masses with enhancing nodularity
• Follow
➢ Very small nonenhancing nodules
Wall Thickening
• Excise
➢ All noninfected masses with wall thickening
“The Current Radiological Approach To Renal Cysts”

Morton A. Bosniak, M.D., Radiology January 1986
Bosniak Classification - Ignore, Excise, Follow

I Simple cyst Ignore
II Min complic Ignore
IIF Probably benign Follow
III Benign or malignant Excise Calcified cystic renal mass that can
IV Clearly malignant Excise be ignored. Top, unenhanced CT:
there is a small amount of
• Communication is crucial! calcification at the 11 0’clock position
as well as milk of calcium at the 6
Examples o’clock position. Bottom, enhanced
• Calcification that can be ignored [Figure 3-19-8] CT: there is no enhancement of the
➢ Small amt of peripheral Ca++ mass
➢ Milk of calcium
➢ No enhancement
• Surgical Calcification [Figure 3-19-9]
➢ Septal Figure 3-19-9
➢ Thick, irregular, nodular
➢ Enhancement
• Uncomplicated Cyst [Figure 3-19-10]
Figure 3-19-10
Cystic renal mass which should be

excised. Top, unenhanced CT:
There is a thin septation which is
calcified (horizontal arrow). There is
NON CON NEPHROGRAPHIC EXCRETORY an irregular nodular calcification at
Calcific cystic mass which should be followed. Left, the 1 o’clock position. Bottom,
unenhanced CT, middle early phase of enhancement, right, enhanced CT shows an area of
excretory phase of enhancement. The mass is sharply enhancement (vertical arrow)
marginated and does not enhanced adjacent to the calcific nodule

• Follow-up Calcification [Figure 3-19-11] Figure 3-19-11
➢ Nodular
➢ No enhancement
• Hyperdense that can be ignored
[Figure 3-19-12]
➢ Well defined, homogeneous
➢ < 3 cm
➢ No enhancement
• Homogeneous with 7 OR 13.5 HU of
enhancement [Figure 3-19-13]
• Surgical Hyperdense [Figure 3-19-14]
➢ Papillary RCC
➢ Solid on US
• Follow-up Hyperdense [Figure 3-19-15] NON CON NEPHROGRAPHIC EXCRETORY
➢ Homogenous Higher CT sections show several irregular nodules of
➢ No enhancement calcification. There is no enhancement
➢ Cystic
➢ > 3 cm Figure 3-19-12
Figure 3-19-13
Hyperdense cyst that can be ignored. Left,

unenhanced CT: The mass is hyperdense (34
HU), homogeneous, well-defined and less than 3
cm. Right, enhanced CT: the mass does not
enhance (31 HU) and remains homogeneous
Figure 3-19-14
Because the enhancement was equivocal,

ultrasound was performed showing a solid mass
which was a papillary renal cell carcinoma
Figure 3-19-15
Minimally enhancing
hyperdense papillary renal cell
carcinoma. Top, unenhanced
CT scan: the mass measures
Hyperdense cyst which should be followed. Left, unenhanced
29 HU. Center, enhanced CT
CT: the 6 cm mass is homogeneous and measures 68 HU.
scan, nephrographic phase:
Center, enhanced CT, the mass remains homogeneous and
the mass enhanced 13.5 HU to
does not enhance. Right, ultrasound: the mass is cystic with a
42.5 Bottom, enhanced CT,
thin septation. Because the mass is greater than 3cm it should
pyelogram: the mass de-
be followed
enhances to 36 HU

• Septations that can be ignored [Figure 3-19-16]
➢ Thin <2mm, no nodularity
➢ No enhancement
➢ May calcify Figure 3-19-16
• Surgical Septations [Figure 3-19-17]
➢ Thick > 2mm
➢ Associated nodularity
➢ Associated nodularity
➢ Complicated cyst
➢ Cystic RCC
• MLCN / MLRCC [Figure 3-19-18]
• Surgical [Figure 3-19-19]
➢ Enhancing thick wall
Septations which can be ignored. Left, ultrasound: the septum
• Surgical nodularity [Figure 3-19-20]
is thin without nodularity. Right, CT: the septum is thin and
➢ Enhancing nodule
smooth without apparent enhancement
• Case 10 [Figure 3-19-21]
➢ Very small nonenhancing nodule
But follow very, very carefully [Figure 3-19-22]

• Baseline / 1 year later
Figure 3-19-17
Figure 3-19-18
Septations which should be excised. Left, ultrasound: the

septation is thick and has associated nodularity (horizontal
arrow). Right, CT: the septation is irregular and enhancing
Left: complicated cyst. Right: cystic renal cell carcinoma
Figure 3-19-19
2 different multiloculated
masses. Top: multilocular
cystic nephroma. Bottom:
multiloculated renal cell
carcinoma
Cystic renal cell carcinoma with an enhancing thick wall. Left,

unenhanced CT: the thick wall is difficult to appreciate. Right,
enhanced CT: the thick enhancing wall is easily visualized

2 different cases of cystic renal cell carcinoma. Enhancing

nodule (arrow) in the wall of each is clearly visualized
Figure 3-19-22
Very small nonenhancing nodule

which could be followed. Top,
ultrasound: there is a very small
Cystic renal cell carcinoma with a small nodule which was nodule (vertical arrow). Bottom,
followed. Left, baseline T2 fat sat MR shows a small nodule in enhanced CT: there is a very small
the 8 o'clock position (arrow). Right, follow-up T2 fat sat MR nonenhancing nodule (horizontal
one year later shows increased nodularity and thickening arrow)
References
1. Hartman DS, Choyke PL, Hartman MS.A practical approach to cystic renal masses.RadioGraphics 2004;24: S101-
S115.

Genitourinary Seminar 1: MSAFP
All of the following scans were ordered following a routine

blood test.
• What was the test?
• Was it high or low?
Elevated Maternal Serum Alpha-Fetoprotein (MSAFP)
Fetal Alpha-fetoprotein
• Glycoprotein produced by fetal liver, GI tract, and yolk sac
• Excreted through the urinary tract into the amniotic fluid
• Peaks at 14–16 wks
• Small amounts leak into maternal circulation
Maternal Serum Alpha-fetoprotein (MSAFP)

• Screening in second trimester (16–18 weeks)
• Elevated if 2.5 MOM (multiples-of-the-median)
• 10–15% risk of open neural tube defect
Case 1
Case 2
Elevated MSAFP
• Incorrect dates
• Twins
• Fetal death
• Open neural tube defect
• Abdominal wall defect
• Subchorionic hemorrhage
Genitourinary Radiology 691 Seminar 1: MSAFP

Case 3 Case 4 Case 5
Placental hemorrhages
Elevated MSAFP
• Can perform amniocetesis and measure direct AFP and ACE
• Acetylcholinesterase (ACE) – neural tissue specific
Elevated MSAFP
• Inc AFP, inc ACE – ONTD
• Inc AFP, nl ACE – abdominal wall defect
• Nl AFP, nl ACE – prior bleed
Decreased MSAFP
• Trisomy 21,18
• Combine with human chorionic gonadotropin (hCG) and estriol (uE3) for
increased specificity – triple screen
Seminar 1: MSAFP 692 Genitourinary Radiology

Genitourinary Seminar 2: Renal Calcifications
Renal Calcifications
• Dystrophic calcification
• Nephrocalcinosis
➢ cortical
➢ medullary
• Nephrolithiasis
Dystrophic Calcification
• Calcification of abnormal tissue
• DDx
➢ tumor
➢ inflammatory mass (TB)
➢ hematoma
➢ cysts
66 yo with hematuria
Renal Tuberculosis
• Bacilli lodge in
corticomedullary jct.
• Progress along nephron
into pelvo-calyceal
system
• 75% of active TB only in
one kidney
Symptoms
• Asymptomatic
• Frequency
• Hematuria
• “Sterile” pyuria
Papillary necrosis
Granuloma formation
Genitourinary Radiology 693 Seminar 2: Renal Calcifications

Radiologic Findings
• 10% normal
• Papillary irregularity
• Papillary necrosis
Radiologic Findings
• Infundibular stenosis
• Amputated calyx
• Parenchymal scarring
• Tuberculomas
Calcifications
• Present in 30–50%
• Variable appearance
➢ punctate – healed granulomas
➢ amorphous – granulomatous masses
➢ extensive reniform – autonephrectomy (putty kidney)
• Ureter and bladder may also be involved
Diffuse renal calcification
45 yo woman from Mexico with pyuria
Medullary Nephrocalcinosis
• Metastatic calcification – calcification in normal tissue
• Triangular deposition conforming to pyramids
• Renal function usually not impaired
• Often associated with nephrolithiasis
• Hypercalcemic states
➢ hyperparathyroidism, paraneoplastic, sarcoidosis, milk-alkali syndrome,
hyper-vitaminosis D
• Medullary sponge kidney (renal tubular ectasia)
➢ may be unilateral or focal
• Renal tubular acidosis – Type I (distal)
➢ distal tubule can not secrete hydrogen ion, urine becomes alkaline
➢ symmetric
• Oxalosis
➢ primary (children) -severe may also see cortical calcification
➢ secondary – distal small bowel resection
Seminar 2: Renal Calcifications 694 Genitourinary Radiology

27 yo man with a history of stone 5 yo boy with an inherited disorder
disease
45 yo female with microscopic

hematuria and intermittent flank pain
Cortical Nephrocalcinosis
• Egg-shell calcification
• Generally small kidneys
• Renal function usually impaired
Cortical Nephrocalcinosis
• Chronic glomerulonephritis
• Acute cortical necrosis
➢ pregnancy, sepsis, trauma, nephrotoxins (ethylene glycol)
• Chronic transplant rejection
• Alport’s syndrome
➢ nephritis, nerve deafness, hematuria, ocular abnormalities
Genitourinary Radiology 695 Seminar 2: Renal Calcifications

33 yo male in an aircraft accident with 47 yo male admitted for an abdominal
severe chest and skeletal trauma abscess. What is the renal disease?
Seminar 2: Renal Calcifications 696 Genitourinary Radiology

Radiologic Pathology 2006-2007 - Volume 1 - Index
A. Israelii 187 Alveolar Filling Pneumonias 178

Abdominal Angina 499 Amebic Abscess (hepatic) 285
Abdominal Wall Defects 609 Anembryonic Pregnancy 598
Abscess (Acute Pancreatitis) 463 Anencephaly 605
Abscess (Crohn Disease) 388 Aneurysm (mediastinum) 164
Abscess (tuboovarian) 659 Angiodysplasia (GI Bleeding) 474
Absent Nephrogram 677 Angiomyolipoma (Kidney) 570
Accessory Spleen 532 Angiosarcoma (intrahepatic) 280
ACCP 136 Angiosarcoma (Spleen) 539
Achalasia 164 Anorectal Lymphoma 350
Actinomycosis 178 Anthrax 189
Acute Cholecystitis 439, 441 Appendagitis 430
Acute Epididymitis 591 Appendiceal Neoplasms 428
Acute Interstitial Pneumonia AIP 18 Appendicitis 427
Hammon-Rich 18 ARPKD 616
Acute Mediastinitis 165 Arteriovenous Fistulae/Pseudoaneurysms (post kidney
Acute Mesenteric Ischemia 488 transplantation) 668
Acute Pancreatitis 460 Asbestos 49
Acute Rejection (Kidney Transplants) 667 Asbestosis and Cigarette Smoking 52
Acute Scrotum 591 Mesothelioma 50
Adenitis (Mesenteric) 430 Pleural Effusion 50
Adenocarcinoma (bile ducts) 317 Pleural Plaques 50
Adenocarcinoma (Gastric) 332 Round Atelectasis 51
Advanced Gastric Carcinoma 333 Rounded atelectasis 49
Carmen Meniscus Sign 334 Ascariasis 188
Early Gastric Carcinoma 333 Ascariasis lumbricoides 189
WHO Classification 333 Aspergillus 77
Adenocarcinoma (lung) 115 Air Crescent 77
Adenocarcinoma (small intestine) 356 Halo-Sign 77
Adenoid Cystic Carcinoma 196 Aspiration (Nosocomial Pneumonia and) 186
Adenoma (male Breast) 257 Asplenia (Ivemark Syndrome) 534
Adenoma (Oligocystic) 328 Asthma 33
Adenoma (parathyroid) 162 Atresia 609
Adenovirus 182 Esophageal 609
Adhesion (GI) 477 Jejunal/Ileal 609
Adnexae (non neoplastic disorders) 653 Atrial Septal Defect 133
Adnexal Torsion 658 Atypical Adenomatous Hyperplasia 117
ADPCKD 306 B. Anthracis: Anthrax 189
ADPKD 614 BCG 71
Adrenal Imaging 645 Benign Hepatic Neoplasms 267
Adrenal Tumors 645 Bile Duct (Hepatic) Cyst 272
Adenoma 645 Biliary Cystadenoma 272
Carcinoma 646 Focal Nodular Hyperplasia 268
Myelolipoma 647 Hemangioma 267
Pheochromocytoma 647 Hepatocellular Adenoma 270
Washout Calculation 648 Hepatocellular Adenomatosis 271
AIDS Cholangiopathy 308 Lipomatous Tumors 273
AIDS-Related Lymphoma (GI) 350 Bilharziasis (see Schistosomasis) 288
Airways Disease 26 Biliary Disease (benign) 303
Allergic Bronchopulmonary Aspergillosis 27 Acute Pyogenic Cholangitis 309
Diffuse Panbronchiolitis 27 AIDS Cholangiopathy 308
Langherhans Cell Histiocytosis 27 Caroli Disease 304
Lymphangioleiomyomatosis 27 Choledochal Cyst 305
Mosaic density 27 Polycystic Liver Disease 306
AIUM Guidelines: First Trimester 597 Primary Sclerosing Cholangitis 307
Allergic Bronchopulmonary Aspergillosis 35 Recurrent Pyogenic Cholangitis 309
Alpha-1 Antitrypsin Deficiency 31 Biliary Parasites 288
Alpha-fetoprotein (Fetal) 691 Clonorchis sinensis 289
I1
Bilomas (liver transplantation) 672 Calcifications (benign - breast) 225
Bladder Neoplasms 649 Fibroadenoma 226
Blastomycosis 104 Loa Loa 227
Pathology 105 Lobular 226
Radiologic Manifestations 105 Secretory 227
Bleeding - Gastrointestinal 468 Skin 226
Boerhaave’s Syndrome 403 Sutural 226
Bowel Disease (Idiopathic Inflammatory) 382 Vascular 227
Bowel Ischemia 487 Cancer (Male Breast) 261
Brachytherapy (Prostate Cancer) 622 Carcinoid
Breast 229 Atypical 193
Angiosarcoma 236 Typical 192
Congenital Anomalies 230 Duodenal, Jejunal, Ileal 358
Fibroadenoma 231 Gastric 341
Fibrosarcoma 236 Thymic 154
Invasive Ductal Cancer 233 Syndrome 358
Invasive Ductal Carcinoma 234 Carcinoma - Adrenal 646
Invasive Lobular Cancer 235 Carcinoma (Male Breast) 262
Lobular Neoplasia 232 Invasive Ductal Carcinoma 262
Mastitis 230 Lymphoma (Male Breast) 263
Medullary Carcinoma 234 Metastasis (Male Breast) 262
Normal Anatomy 229 Papillary Carcinoma 262
Paget’s Disease 234 Carcinoma (Scirrhous - Stomach) 334
Papilloma 232 Carcinoma Colon, Rectum (see Colorectal Carcinoma) 361
Phyllodes Tumor 231 Carcinosarcoma 197
Pregnancy Changes 230 Cardia (Carcinoma) 334
Sarcoma 236 Carney’s Triad 200
Spindle Cell Sarcoma 236 Caroli Disease 304
Tubular Carcinoma 235 Castleman Disease 151, 346
Breast (Male) 257 Cavitary pneumonia 190
Breast (Young Women) 246 Cavitation 190
Angiosarcoma 254 Cecal Volvulus 400
Benign Lesions 247 Cervix Carcinoma 559
Diabetic Mastopathy 251 Chest Wall 216
Fibroadenoma 247 Neoplasms 218
Granular Cell Tumor 249 Chiari II 604
Granulomatous Mastitis 252 Cholangiocarcinoma (intrahepatic) 279
Hamartoma 250 Cholangitis 309
Invasive Ductal Carcinoma 252 Acute Pyogenic 309
Juvenile Hypertrophy 251 Recurrent Pyogenic 309
Juvenile Papillomatosis 250 Cholecystitis 438, 441
Lactating Adenoma 250 Choledocholithiasis 442
Lymphoma 254 Cholelithiasis 438
Medullary Carcinoma 253 Cholescintigraphy 439
Phyllodes High Grade 254 Chondroma 201
Phyllodes Tumor 249 Chorionic Sac 594
Pseudoangiomatous Stromal Hyperplasia (PASH) 251 Choroid Plexus Cysts 605
Sarcoma 253 Chronic Idiopathic Intestinal Pseudo-obstruction 476
Secretory Carcinoma 253 Chronic Liver Disease 293
Bronchial Adenoma 192 Budd-Chiari Syndrome 298
Bronchial Carcinoid 194 Cirrhosis 293
Bronchiectasis 190 Hemochromatosis 300
Bronchiolitis Obliterans 80 Hemosiderosis 300
Bronchioloalveolar Carcinoma 117 Hepatocellular Carcinoma 295
Bronchocentric Granulomatosis 71 Nonalcoholic Steatohepatitis 297
Bronchogenic Cyst 159 Primary Biliary Cirrhosis 296
Brunner Gland 354 Steatosis 296
Hamartoma 354 Chronic Pancreatitis 460, 465
Hyperplasia 354 Chronic Thromboembolic Disease 133
Budd-Chiari Syndrome 298 Churg-Strauss Syndrome 67
Burkitt Lymphoma 348 Cirrhosis 293
CA-125 638 CNS Malformations (Fetal) 602
I2
Coccidioidomycosis 106 Diffuse Alveolar Damage 79
Coccidioidoma 107 Diffuse Lung Disease 3
Pathology 106 AIP 4
Radiologic Manifestations 106 Asbestosis 3
Colitis Asthma 3
Ischemic 398 Bronchiectasis 6
Colon (Inflammatory Disease) 391 Bronchoalveolar cell carcinoma 4
Cecal Diverticulitis 397 Constrictive bronchiolitis 3
Diverticular Hemorrhage 397 Cor Pulmonale 11
Diverticulitis 395 DAD 4
Diverticulitis vs. Carcinoma 396 DIP 4
Giant Sigmoid Diverticulum 397 Edema 6
Ischemic Colitis 398 Emphysema 3
Colonic Lymphoma 350 Granuloma 7
Colonic Polyposis 519 Hypersensitivity pneumonitis 3
Colorectal Carcinoma 361 LAM 3
Adenoma 363 LCH 4
Complications 366 Löfgren syndrome 12
EUS 368 Lymphoma 4
Inflammatory Bowel Disease 366 NSIP 4
Lymphatic Spread 369 Organizing Pneumonia (BOOP) 4
Multiple 365 Sarcoidosis 3, 6, 7
Obstructing 366 Differential Diagnosis 10
Rectal Adenocarcinoma: Lymphatic Drainage 369 Mycetoma 12
Screening 362 Diffuse Panbronchiolitis 36
Synchronous 365 Diverticulitis 396
Villous Adenoma 363 Cecal 397
Community-acquired Pneumonia 179 Colovesical Fistula 396
Congenital CNS Malformations 602 CT 397
Congenital Diaphragmatic Hernia 608 Differential Diagnosis 396
Constrictive Bronchiolitis 36 Pericolic Abscess 396
Cor Pulmonale 133 Ductal Carcinoma in Situ 238
Corpus Callosum - Agenesis 605 Biopsy 243
Cowden’s Syndrome 525 Calcification 240
Crohn disease 382, 386 Classification 239
Gastric 410 Ductal Plate 303
Cronkite-Canada syndrome 526 Duodenal Carcinoid 357
Cryotherapy (Prostate Cancer) 622 Duodenitis 455
Cryptorchidism 588 Echinococcosis 188
Cyst - Bile Duct (Hepatic) 272 Echinococcus granulosus 188, 286
Cyst (Breast) 223 Echinococcus multilocularis 286
Pneumocystography 223 Ectopic pregnancy 598
Thickened Skin Pattern (breast) 224 Eisenmenger Physiology 133
Cyst (Bronchogenic) 159 Emphysema 27
Cyst (thymic) 161 Emphysematous Cholecystitis 441
Cystadenoma (Biliary) 272 Emphysematous Cystitis 651
Cystic Abdominal/Pelvic Collections 611 Encephalocele 605
Cystic Adenomatoid Malformation 608 Endometrial Carcinoma 558
Cystic Disease of the Kidney 614 FIGO – Staging 559
Cystic Fibrosis (P. aeruginosa) 185 Endometrioma 656
Cystic Hygroma 607 Endometriosis 655, 656
Cystic Kidney Disease - Acquired 618 Endorectal Coil MRI 622
Cystic Lymphangioma (Male Breast) 258 Enteric Cyst 374
Cystic Nephroma 686 Enteritis - Chronic Radiation 497
Cystitis 650, 651 Enterography (MR - Crohn Disease) 389
Cysts (Congenital - Mediastinum) 160 Epidermal Inclusion Cyst (Male Breast) 260
Cytomegalovirus 78 Epidermoid Cyst(Testis) 588
Cytomegalovirus Pneumonia 78 Epididymal Masses 590
Dandy-Walker Malformation 604 Adenomatoid Tumor 590
Daughter cysts (E. granulosus) 287 Lipoma (Paratesticular) 592
Delayed Pyelogram 677, 678 Papillary Cystadenoma 590
Diabetic mastopathy 258 Polyorchidism 592
I3
Epididymis 586, 636 Gastritis 470
Epididymitis 591 Lower GI 469
Epiploic Appendagitis 430 Nuclear Scintigraphy 469
Epithelial Inclusion Cysts 661 Risk of Rebleeding 470
Esophageal Rupture (Causes) 403 Upper GI 469
Esophageal Varices 470 Gastrosplenic ligament 531
Esophagus - Inflammatory Diseases 444 Germ Cell Neoplasms 155 (Chest)
Barrett’s Esophagus 445 Germ Cell Tumors (Retroperitoneum) 583
Candida Esophagitis 446 Gestation 596
CMV Esophagitis 447 Gestational Sac 594
Drug-Induced Esophagitis 448 Gestational Trophoblastic Disease 661
Herpes Esophagitis 446 GIST (Gastrointestinal Stromal Tumors) 338
HIV Esophagitis 447 Glucagonoma 328
Intramural Pseudodiverticulosis 448 Goiter (mediastinal) 161
Reflux Esophagitis 444 Graft-vs-Host Disease 79
Esophagus - Malignant Tumors 452 Granular Cell Tumor (Male Breast) 260
Adenocarcinoma 453 Granuloma (lung) 205
Early Squamous Cell Carcinoma 453 Granulomatous Mastitis 261
Spindle Cell Carcinoma 454 Gut Hemangioma 471
Squamous Cell Carcinoma 452 Gynecomastia 257, 258
Esophagus Tumors 450 H.influenzae 178
Duplication Cyst 452 H.Pylori 459
Leiomyoma 450 Hamartoma 199
Leiomyomatosis 451 Hamartoma (Breast) 222
Squamous Papilloma 450 Hemangioma 268
Extralobar Sequestration 608 Hemangioma (Gut) 471
Extramedullary hematopoiesis 164 Hemangioma (Mediastinum) 163
Extrarenal Cysts 615 Hemochromatosis 300
Fetal Anomalies 607 Hereditary 300
Fibrolamellar Carcinoma 278 Secondary 301
Fibrous Pseudotumor 592 Hemosiderosis 300
Foramen Of Winslow Hernia 404 Hepatic Artery Thrombosis (liver transplantation) 671
Fournier Gangrene 591 Hepatic Cyst (Complex) - Differential Diagnosis 417
Galactocele 223 Hepatic Mass with a Scar - Differential Diagnosis 418
Gallbladder and Biliary Neoplasms 313 Hepatic Neoplasms 267
Gallbladder Empyema 442 Hepatic Portal Venous Gas 402
Gallbladder Wall Thickening 440 Hepatocellular Adenomatosis (see Benign Hepatic
Gallstone 438 Neoplasms) 271
Ganglion Cell Tumors 581 Hepatocellular Carcinoma 275
Ganglioneuroblastoma 158 Hereditary GI Polyposis Syndromes 524
Ganglioneuroma 158, 581 Herniations 164
Gangrenous Cholecystitis 441 Hiatus Hernia 164
Gastric Lymphoma 335 Morgagni 164
Gastric Malignancies 332 Herpes viruses (respiratory) 182
Carcinoid 340 Heterotopic Pregnancy 599
Carcinoma of the Cardia 334 Histoplasmosis 100
Gastric Adenocarcinoma 332 Acute Radiology 101
Gastrointestinal Stromal Tumors (GIST) 338 Chronic 102
Kaposi Sarcoma 341 Disseminated 102
Lymphoma 335 Fibrosing Mediastinitis 104
Mesenchymal Neoplasm 338 Histoplasmoma 103
Metastases 341 Mediastinal granuloma 103
Mucosa-Associated Lymphoid Tissue 335 Pathology 101
Scirrhous Carcinoma 334 Hodgkin Disease - Mediastinum 150
Gastric Ulcer Investigation 457 Holoprosencephaly 603
Gastric Volvulus 405 Hydranencephaly 602
Bezoar (Gastric) 407 Hydrocele 590
Zollinger-Ellison Syndrome 408 Hydrocephalus 603
Gastritis 455 Hydronephrosis 610
Gastrointestinal Bleeding 468 Hydronephrosis (post transplantation) 666
Angiography 470 Hydrosalpinx 658
Endoscopy 469 Hypoplastic Left Heart 608
I4
Idiopathic Inflammatory Bowel Disease 382 Cigarette smoking 111
Immunocompromised Host (Hepatic Infections) 289 Clinical Presentation 111
Candidiasis 289 Paraneoplastic Syndromes 111
Hepatosplenic Candidiasis 290 Lymphangioleiomyomatosis 38
Pneumocystis jiroveci 291 Lymphangioma
Infiltrating Colloid Carcinoma 323 Mediastinum 162
Inflammatory Carcinoma (breast) 225 Lymphangioma
Inflammatory Myofibroblastic Tumors 379 Retroperitoneum 582
Inflammatory Pseudotumor (lung) 202 Mesentery
Influenzae 178 Differential Diagnosis 374
Influenzae A 181 Lymphangitic Carcinomatosis 143
Inguinal Hernia 634 Imaging Features 143
Iniencephaly 607 Lymphocele (post kidney transplantation) 666
Interstitial Pneumonia 182 Lymphoma 150
Interstitial Pneumonias 14 Lymphoma
Acute Interstitial Pneumonia (AIP) 14 Burkitt) 348
Cryptogenic Organizing Pneumonia (COP) 14 Lymphoma
Desquamative Interstitial Pneumonia (DIP) 14 Primary Gastric 336
Idiopathic Pulmonary Fibrosis (IPF) 14 Lymphoma
NonSpecific Interstitial Pneumonia (NSIP) 14 Testicular 588
Respiratory Bronchiolitis-Interstitial Lung Disease (RB- Lymphomatoid Granulomatosis 70
ILD) 14 Epstein-Barr Virus 70
Usual Interstitial Pneumonia (UIP) 14 M. pneumoniae 180
Interstitial Pregnancy 599 Malabsorption 505
Intracranial Aneurysms 615 Malakoplakia 650
Intraductal Papillary Mucinous Neoplasm 324 Male Breast 257
Intrahepatic Portal Venous Air 492 Cancer 261
Intravenous Talcosis 134 Malignant Fibrous Histiocytoma 582
Ischemia Mimic (Mesenteric) 500 Malignant Germ Cell Neoplasms (Non-Seminomatous) 156
Jejunal and Ileal Carcinoid 357 Malignant Hepatic Neoplasms 275
Juvenile Laryngeal Papillomatosis 201 Angiosarcoma 280
Juvenile Polyposis: Syndrome 525 Epithelioid Hemangioendothelioma 280
K. pneumoniae 178, 183 Fibrolamellar Carcinoma 278
Kaposi Sarcoma (Gastric) 341 Hepatocellular Carcinoma 275
Kidney Intrahepatic Cholangiocarcinoma 279
Cystic Diseases 614 Malignant Neoplasia (Chest)149
Neoplasms 561 Malignant Peripheral Nerve Sheath Tumor 158
Transplants 664 Mallory-Weiss Tear 471
Trauma 576 MALT 335
KIT 338 Masaoka (Thymoma: Staging) 154
Krukenberg Metastasis 335 Mastitis 224
L. pneumophila 183 Mastitis (Granulomatous - Male Breast) 261
Langerhans Cell Histiocytosis 29 Mature Teratoma 155
Large Cell Carcinoma 114 Meckel Diverticulum (complications) 422
Legionella 178 Diverticulitis 424
Leiomyoma 258, 261 Diverticulitis with a Stone 425
Leiomyosarcoma (retroperitoneum) 582 Hemorrhage 424
Limb-Body-Wall Defect 610 Heterotopic Gastric Mucosa 423
Lipoma 258 Heterotopic Pancreatic Mucosa 423
Lipoma (breast) 222 Inverted 425
Lipomatous Tumors (Liver) Meckel’s Diverticulum 473
Angiomyolipoma 273 Mediastinal
Myelolipoma 273 Compartments 148
Liposarcoma (breast) 222 Fibrosis 135, 151
Liposarcoma (retroperitoneum) 583 Goiter 161
Liver Disease (chronic) 293 Masses 148
Liver Mass with Fat - Differential Diagnosis 420 Mediastinitis 165
Liver Neoplasms 267, 275 Mediastinum 148
Liver Transplantation 670 Medullary Carcinoma (Kidney) 565
Complications 671 Menetrier Disease 409
Liver Transplants 664 Meningocele (Thoracic) 158
Lung Cancer 111, 149 Mesenchymal Neoplasm of the Stomach 338
I5
Mesenteric Adenitis 430 Necrotizing Sarcoid Granulomatosis 69
Mesenteric Cyst 372 Neoplasms (Germ Cell) 155
Mesenteric Fibromatosis (Desmoid Tumor) 346, 376 Neoplasms (Neurogenic) 157
Mesenteric Ischemia 487 Nephrocalcinosis 694
Mesenteric Ischemia - Etiologies 495 Nephrogram 674
Embolus 495 Nephroma (Multilocular Cystic) 569
Thrombosis 497 Nerve Sheath Tumor 158 (Chest)
Mesenteric Masses (Differential Diagnosis) 346 Nerve sheath Tumors (Retroperitoneum) 581
Mesenteric Masses and Cysts 372 Neural Tube Defects 605
Benign Multicystic Mesothelioma 375 Neuroblastoma (Mediastinal) 158
Diffuse Peritoneal Malignant Mesothelioma 375 Neuroendocrine Tumors (pancreas) 328
Enteric Cyst and Mesothelial Cyst 374 Neurofibroma 157
Inflammatory Myofibroblastic Tumors (Inflammatory Neurofibromatosis (NF1) 158
Pseudotumor) 379 Neurogenic Neoplasms 157
Lymphangioma 373 Neurogenic Tumors (Retroperitoneum) 580
Nonpancreatic Pseudocyst 374 NF-1 (GI Neoplasms) 359
Sclerosing Mesenteritis 378 Nocardia 178
Mesothelioma Nodes (NHL - Gastrointestinal) 345
Malignant 215 Nodular Lymphoid Hyperplasia Colon 527
Mesenteric 374 Non Hodgkin Lymphoma (abdominal) 344
Benign Multicystic 375 Adenopathy 345
Cystic Malignant 375 AIDS-Related Lymphoma 349
Diffuse Malignant 375 Burkitt Lymphoma 348
Scrotal 593 Differential Diagnosis 346
Metastases (pleural) 216 Enteropathy-Type T-cell Lymphoma 349
Metastases (pulmonary) 138 Gastrointestinal Lymphoma 346
Calcification 142 Mantle Cell Lymphoma 348
Cannonball 140 Small Intestine 347
Cavitation 141 Small Intestine: Differential Diagnosis 349
Endobronchial 144 Non Hodgkin lymphoma - Mediastinum 150
Lymphangitic Carcinomatosis 143 Non-Hereditary GI Polyposes 524
Micronodular 141 Non-Neoplastic Lymphadenopathy - Mediastinum 151
Parenchymal Nodules 139 Non-Seminomatous Malignant Germ Cell Neoplasms 156
Pathogenesis of Hematogenous Metastases 139 Nosocomial Pneumonia 185
Pleural 144 Nosocomial Pneumonia and Aspiration 186
Solitary 142 Ogilvie Syndrome 401
Tumor Embolism 143 Oligohydramnios 609
Metastases Breast Young Women 254 Omental Infarction 431
Microcystic Adenoma 327 Oncocytoma 569
Microscopic Polyangiitis 67 Orchitis 591
Mole 661 Organ Transplantation 75
Benign Hydatidiform 661 CMV 76
Choriocarcinoma 662 Fungal Infections 77
Complete 662 Graft-vs-Host 76
Partial 662 Pneumocystis carinii 76
Morgagni (Herniation) 164 Ovarian Cyst 611
Mounier-Kuhn Syndrome 32 Ovarian Cysts 653
MR Enterography 389 Corpus Luteum 654
MRSA 186 Follicular 653
Mucinous Cystadenoma/Cystadenocarcinoma (Appendiceal) Functional 653
428 Hemorrhagic 654, 655
Mucinous Cystic Neoplasm 326 Hyperstimulation 655
Mucinous Noncystic Adenocarcinoma 324 Rupture 655
Mucoepidermoid Carcinoma 196 Theca Lutein 655
Multilocular Cystic Nephroma 686 Ovarian Neoplasms 637
Multiple Lymphomatous Polyposis (Mantle Cell Lymphoma) Epithelial Ovarian Neoplasms: Endometrioid 639
348 Epithelial Ovarian Neoplasms: Mucinous 639
Myasthenia Gravis (Thymoma and) 152 Epithelial Ovarian Neoplasms: Serous 638
Mycoplasma 178 Epithelial Ovarian Tumors: Clear Cell 639
Myelolipoma 647 Epithelial Tumors: Classification 637
Myofibroblastoma (Male Breast) 257, 260 Epithelial Tumors: Terminology 638
N. Asteroides 188 Mature Cystic Teratoma 640
I6
Ovarian Germ Cell Neoplasms 640 Malignant 213
Ovarian Malignant Germ Cell Tumors 641 Pleural Neoplasms 213
Sex-cord stromal tumors 641 Localized Fibrous Tumor 213
Ovarian Torsion 657 Mesothelioma 215
Ovary Pneumatocele 190
non neoplastic disorders 653 Pneumatosis Intestinalis 493
polycystic 660 Pneumocystis Jiroveci 78
Masses 637 Pneumocystis Jiroveci Pneumonia 78
Pancoast Tumor 113 Pneumonia 178
Pancreas 412 Pneumoperitoneum 403
Annular 412 Pneumothorax 211
Divisum 413 Polycystic Kidney Disease (Autosomal Recessive) 616
Intraductal Papillary Mucinous Neoplasm 415 Polycystic Liver Disease 306
Pancreatitis, Chronic 414 Polycystic Ovary Syndrome 660
Pancreas (Neoplasms) 321 Polyhydramnios 609
Adenocarcinoma 321 Polymastia 221
Intraductal Papillary Mucinous Neoplasm 324 Polyposis - Familial 519
Islet Cell Tumors 328 Polysplenia 534
Metastatic 329 Polythelia 221
Microcystic Adenoma 327 Post Transplant Lymphoproliferative Disorder 672
Mucinous Cystic Neoplasm 326 Post Transplant Malignancy 673
Mucinous Noncystic Adenocarcinoma 323 Posterior Urethral Valves 611
Oligocystic Adenoma 328 Post-transplantation Imaging 664
Solid and Pseudopapillary Epithelial Neoplasm 325 Post-transplantation Lymphoproliferative Disorder (GI) 349
Pancreas Transplants 664, 669 Pregnancy 598
Complications 669 Primary Ciliary Dyskinesia 32
Rejection 670 Prostate Cancer 620
Vascular Thrombosis 670 Grading 620
Pancreatic Adenocarcinoma 321 Screening 620
Resectability 323 Prostate Specific Antigen 620
Pancreatic Duct 411 Pseudoaneurysms (kidney transplantation) 669
Pancreatitis 460 Pseudocyst (Nonpancreatic) 374
Papilloma 201 Pseudocyst (Pancreatitis) 462
Papilloma (Male Breast) 257 Pseudogynecomastia 257, 259
Papillomatosis (Biliary) 319 Pseudopapillary Epithelial Neoplasm (Solid and) 325
Paraganglioma 159 Pulmonary Blastoma 198
Paraganglioma (Extra-adrenal pheochromocytoma) 581 Pulmonary Circulation 131
Paragonimiasis westermani 189 Idiopathic 132
Paraovarian Cysts 661 Pulmonary Embolism 82
Parathyroid Adenoma 162 Arterial Blood Gases 85
Pelvic Inflammatory Disease 659 Chest X-Ray 84
Pelvic MRI 553 Clinical Science Probability 85
Peptic Ulcer Disease 455 Combined Pulmonary CTA and Venography 89
Peribronchial abscesses 188 Common Radiographic Abnormalities 84
Perinephric Fluid Collections 665 CT Angiography 86, 87
Peritoneal Inclusion Cysts 523, 659 CT Findings 84
Peritoneal Lymphoma (Primary) 350 Diagnostic Algorithm 89
Persistent Bilateral Nephrogram 679 Pitfalls 88
Peutz Jeghers 525 Small Emboli 86
Pheochromocytoma 647 Ventilation/Perfusion Scanning 85
Pleural Disease 204 Pulmonary Gangrene 190
Bacterial Pneumonia 208 Pulmonary Hypertension 131
Empyema 208 Pulmonary Lymphoid Disorders 55
Pleural Effusion 208 B-Cell Lymphoma 58
Pleural Effusion: Asbestos Exposure 210 Follicular bronchitis 55
Pleural Effusion: Subpulmonic 209 Follicular Hyperplasia 55
Pleural Effusion: Tuberculosis 209 Lymphoid Interstitial Pneumonia LIP 56
Pleural Fibrosis 211 Lymphomatoid Granulomatosis 59
Pneumothorax 211 Nodular Lymphoid Hyperplasia 57
Pulmonary Ligament 207 Posttransplantation Lymphoproliferative Disease 60
Round Atelectasis 210 Pseudolymphoma 57
Pleural Effusion 213 Pulmonary Lymphoid System 54
I7
BALT 54 Cholangiocarcinoma 308
Bronchus Associated Lymphoid Tissue 54 Sclerosing Mesenteritis 378
Pulmonary Thromboendarterectomy 134 Scrotal Anatomy 630
Pulmonary Venous Hypertension 135 Scrotal Calculi 590
Pyelogram 674 Scrotum 585
Pyelonephritis 678 Seminoma 156
Pyogenic Hepatic Abscess 284 Sequestration 608
Pyosalpinx 659 Serous Inclusion Cysts 661
Radioactive Ablation (Prostate Cancer) 622 Serum Alpha-Fetoprotein (Elevated Maternal) 691
Rejection (kidney transplants) 667 Severe Acute Respiratory Syndrome 182
Renal Anomalies 610 Shock Bowel 500
Renal Artery (kidney transplantation) 668 Shwachman - Diamond Syndrome 513
Renal Calcifications 693 Sigmoid Volvulus 401
Dystrophic 693 Silicosis 46
Medullary 694 Adenopathy 47
Renal Cancer in ACKD 619 Alveolar Proteinosis 48
Renal Cell Carcinoma 563 Calcification 47
Renal Injuries 576 Conglomeration 48
Renal Masses 681 Scar emphysema 48
Carcinoma In Situ 681 Small Bowel Bleeding 472
follow up interval 683 Small Bowel Lymphoma 347
Multilocular Cystic Nephroma 686 Small Bowel Obstruction 475
Small renal mass 682 Small Cell Lung Cancer 113
Renal Neoplasms 561 Small Intestinal Benign Neoplasms 353
Angiomyolipoma 570 Adenoma 355
Infiltrating Renal Cell 564 Brunner Gland Hamartoma 354
Juxtaglomerular Cell Tumor 570 Brunner Gland Hyperplasia 354
Medullary Carcinoma 565 Differential Diagnosis: Duodenal Polyp 354
Metastases 569 Periampullary Adenocarcinoma 355
Multilocular Cystic Nephroma 569 Periampullary Duodenal Mass 355
Oncocytoma 569 Tubulovillous Adenoma 355
Renal Cell Carcinoma 563 Small Intestine 347
Renal Lymphoma 568 Adjacent Mesenteric Disease 348
Robson Staging 565 Cavitary Mass 348
Spontaneous Renal Hemorrhage 564 Mural Infiltration 347
Squamous Cell Carcinoma 568 Small Intestine Malignant Neoplasms
TNM Staging 565 Adenocarcinoma 355
Transitional Cell Carcinoma 567 Carcinoid 357
Tuberous Sclerosis 570 Carcinoid Syndrome 358
Uroepithelial Neoplasms 567 Differential Diagnosis 356, 359
Venous extension 562 Metastatic Disease 359
Renal Transplants 664 Small Intestine NHL - Differential Diagnosis 349
Renal Vein Thrombosis (kidney transplantation) 668 Smoking Related ILD 16
Respiratory Bronchiolitis 29 Macrophages 16
Respiratory Viruses 181 RB 16
Retroperitoneal Fibrosis 580 Respiratory bronchiolitis 16
Retroperitoneal Masses 611 Spermatic cord 586
Retroperitoneal Masses (Fat containing) 584 Spleen 531
Retroperitoneum 579 Splenorenal ligament 531
Ruvalcaba-Myhre-Smith 526 Splenosis 533
S. pneumoniae 178, 179 Sprue 505
S.aureus 184 Squamous Cell Carcinoma 111
Saber Trachea 28 Steatosis 296
Sacrococcygeal Teratoma 612 Stomach Malignancies 332
Salpingitis 659 Stone Urinary 624
Sandwich Sign (NHL) 345 Striated Nephrogram 679
SARS 182 Strongyloides stercoralis 189
Scar Carcinoma 116 Strongyloidiasis 188
Schistosomasis (Bilharziasis) 288 Swyer James Syndrome 38, 182
Schistosomiasis (Urinary) 651 Sympathetic Ganglia Tumors 159
Schwannoma 157 Tamoxifen 558
Sclerosing Cholangitis (primary) 307 T-cell Lymphoma (Enteropathy-Type T-cell Lymphoma) 349
I8
Teratoma (Mature) 155 Urinary Tract Trauma 573
Teratoma (Retroperitoneum) 583 Uroepithelial Neoplasms 567
Testicular Carcinoma (Risk Factors) 588 Urothelial carcinoma 649
Testicular Cysts 589 Usual Interstitial Pneumonia: Histology 15
Testicular Ischemia 635 Fibroblast foci 15
Testicular Masses (Bilateral) 589 Uterine Disorders 551
Testicular Neoplasms 586 Arcuate 555
Germ Cell Neoplasms 587 Bicornuate 554
Non Seminomatous Germ Cell Tumor 587 Diethylstilbestrol: DES – Related 556
Seminoma 587 Mullerian Duct Anomalies 554
Testicular Torsion 630 Septate 555
Testis 586 Unicornuate / Didelphys 554
Testis (torsion) 632 Uterine Masses - Benign 556
Thymic Carcinoid 154 Abnormal Uterine Bleeding 557
Thymic Cyst 161 Adenomyosis 557
Thymic Hyperplasia 161 Endometrial Hyperplasia 558
Thymolipoma 154 Endometrial Polyps 558
Thymoma 152 Leiomyoma 556
Torsion (Ovary) 657 Postmenopausal Bleeding 558
Torsion (Testicle) 591 VACTERL Syndrome 610
Transplants (Solid Organs) 664 Varicella Pneumonia 182
Transverse Colon Volvulus 401 Varices (Esophageal) 164
Trauma (Urinary Tract) (see Urinary Tract Trauma) 573 Varicocele 592
Trisomy 18 606 Ventilator-associated Pneumonia 186
Trisomy 21 600 Viruses - Respiratory 181
TRUS 621 Von Hippel Lindau 617
Tuberculosis 93 Water Lily Sign (E. granulosus) 287
Clinical features 95, 96 Wegener’s Granulomatosis 63
Hemoptysis 98 halo sign 66
Lymphatic gradient 95 Williams-Campbell 31
M. tuberculosis 93 Yolk sac 596
Mycetoma 98 Zollinger-Ellison Syndrome 328
Mycobacteria 93
Pathogenesis 94
Radiologic features 95, 96
Rassmussen (pulmonary artery) aneurysm 98
Tuberculoma 97
Tuberculosis (renal) 693
Tuberculosis (scrotal) 591
Tuberous Sclerosis 39, 616
Renal 570
Tubular Ectasia (Testis) 589
Twins 596
Dizygotic 596
Monozygotic 597
Ulcerative Colitis 382, 384
CT Features 384
Imaging Features 384
Toxic Megacolon 385
Ulcers - Duodenal 458
Ulcers - Gastric 456
Benign 456
Equivocal 457
Malignant 457
UPJ disruption 578
Urachal Anomalies 649
Ureteral Injury 575
Urethra 649, 652
Urethral Trauma 573
Urethrography 652
Urinary Bladder 649
Urinary Stone Disease 624
I9
Radiologic
Pathology
Fifth Edition
VOLUME 2
Musculoskeletal
2006
Editors

2007
Mark D. Murphey, MD


Washington DC, USA
Washington, DC
20306-6000
_____________________________________

_____________________________________
987654321
ISBN 1-933477-00-8
Preface
Acknowledgements


Pathology,
iii
Musculoskeletal Radiology
Mark E. Schweitzer, MD
Mark D. Murphey, MD Professor of Radiology and Orthopedic Surgery
Chief, Musculoskeletal Radiology Chief of Radiology - Hospital for Joint Diseases
Department of Radiologic Pathology Director, Musculoskeletal Radiology
Armed Forces Institute of Pathology New York University
Washington, DC New York, NY
Christopher G. Fielding, COL, DC, USA

Department of Oral Maxillofacial Pathology
Washington, DC
Mark W. Anderson, MD
Associate Professor of Radiology and Orthopedic Surgery
Division Head, Division of Musculoskeletal Radiology
University of Virginia Health System
Charlottesville, VA
Donald J. Flemming, CAPT, MC, USN
G. Victor Rohrer Professor of Radiology Education
Penn State Hershey Medical Center
Hershey, PA
Mark J. Kransdorf, MD
Mayo Clinic College of Medicine
Rochester, MN
and
Consultant, Musculoskeletal Radiology
Mayo Clinic
Jacksonville, FL
William B. Morrison, MD
Director, Division of Musculoskeletal
and General Diagnostic Radiology
Thomas Jefferson University Hospital
Philadelphia, PA
Michael Mulligan, MD
Associate Professor of Diagnostic Radiology
Baltimore, MD
Thomas L. Pope, MD
Clinical Professor of Radiology/Orthopedics
Medical University of South Carolina
Charleston, SC
and
Former Distinguished Scientist
Washington, DC
Charles S. Resnik, MD
Professor of Diagnostic Radiology
Director, Section of Musculoskeletal Radiology
Director, Residency Program
Baltimore, MD
Timothy Sanders, MD
Assistant Professor of Radiology
Uniformed Services University of the Health Sciences
Bethesda, MD
iv
Mark D. Murphey, MD
Radiologic Assessment of Joint Replacement and Imaging of Bone Grafts . . . . . . . . . . . . . . . . . . . . . . . . .699
Musculoskeletal Manifestations of Chronic Renal Insufficiency . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .711
Fundamental Concepts of Musculoskeletal Neoplasm: Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .720
Fundamental Concepts of Musculoskeletal Neoplasm: CT and MR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .733
Osteoid Lesions of Bone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .743
Cartilaginous Lesions of Bone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .757
Fibrous Lesions of the Musculoskeletal System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .771
Alphabet Soup and Cystic Lesions of Bone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .784
Juxtaarticular Soft Tissue Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .792
Musculoskeletal Angiomatous Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .805
Paget Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .812
Musculoskeletal Infection I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .820
Musculoskeletal Infection II . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .829
Imaging of Cervical Spine Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .839
Christopher J. Fielding, COL, DC, USA
Radiographic Differential Diagnosis of the Jaws . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .849
Mark W. Anderson, MD
MRI of the Knee: Part 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .858
MRI of the Knee: Part 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .865
MRI of the Wrist . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .872
MRI of the Ankle and Foot . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .879
Osseous Lesions: Unknown Histogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .887
Soft Tissue Lipomatous Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .893
Metabolic Bone Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .900
Osteonecrosis and Related Conditions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .906
Donald J. Flemming, CAPT, MC, USN
Approach to the Inflammatory Arthropathies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .912
MRI of the Rotator Cuff . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .925
Timothy Sanders, MD
MR Arthrography of Glenohumeral Instability . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .932
Imaging of Upper Extremity Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .941
Crystal Deposition Diseases and Neuropathic Osteoarthropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .949
Mark Schweitzer, MD / William Morrison, MD
MRI of the Elbow . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .955
Michael Mulligan, MD
Skeletal Metastases, Myeloma, Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .961
Thomas Lee Pope, MD
Imaging of Hematologic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .968
Generalized Musculoskeletal Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .976
Osseous Musculoskeletal Stress Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .987
Pelvis and Lower Extremity Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .995
Mark D. Murphey, MD
Musculoskeletal Seminar I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1005
Musculoskeletal Seminar II . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1009
Musculoskeletal Seminar III . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1013
Musculoskeletal Seminar IV . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1018
Musculoskeletal Seminar V . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1025
v
697
698
Radiologic Assessment of Joint Replacement and Bone Grafts
Mark D. Murphey, MD
Current Materials For Total Joint Replacement

• Metal components
• Ultra-High Molecular Weight Polyethylene
Metallic Components
• Cobalt-chromium-molybdenum alloy
• Cobalt-chromium-tungsten alloy
• Titanium-aluminum-vanadium alloy
Ultra-High Molecular Weight Polyethylene Component Figure 4-1-1

• Allows articulation of metallic components
• Lowers friction and prolongs wear
• Allows some plastic deformity improving joint
congruity
• Radiolucent
Complications of Joint Arthroplasty

• I. Loosening and/or infection
• II. Small particle disease (osteolysis)
• III. Dislocation and abnormal alignment
• IV. Fractures and nonunion
• V. Heterotopic bone formation
• VI. Cement extrusion
• VII. Polyethylene liner displacement and metal
arthropathy
Loosening and/or Infection Aseptic loosening of both acetabular and femoral components
• Most common complication historically of a total hip arthroplasty with bone cement (arrows), and
• Difficult to differentiate cement-metal lucency (arrowheads) that progresses over
• 4–13% hip replacement several years (right image). Cement fracture (open arrow) and
• 7–10% knee arthroplasty lateral migration of the femoral stem (curved arrow) are also
apparent
Radiographic Signs: Loosening-Infection Cemented Arthroplasty [Figure 4-1-1]
• Cement-bone lucency or cement metal lucency >2mm
• Progressive widening of interfaces post-op
• Component migration
• Fracture of metal or cement
• Periosteal reaction Figure 4-1-2
• Smooth endosteal scalloping with cement lucency
• Air in soft tissues or joint
Bone Ingrowth (Porous Coated)

Joint Arthroplasty
• Improved longevity
• Beads sintered onto metal surface
• Bone ingrowth into irregular surface (biologic
fixation)
• Technically demanding
• No motion to promote bone ingrowth
Normal Radiographic Appearance Ingrowth

Arthroplasty
[Figure 4-1-2]
• Resorption of medial femoral cortex
• Thin lucent rim with sclerotic margin about metal
(< 2 mm)
• Endosteal sclerosis
• Prosthetic subsidence (< 10 mm) Normal appearance of ingrowth hip arthroplasty with
superolateral lucency (arrowheads) (<2mm) and surrounding
• Periosteal reaction and cortical thickening
sclerosis and resorption of medial cortex (arrow)
• Not progress after 9 – 12 months post-op
Musculoskeletal Radiology 699 Joint Replacement and Bone Grafts

Loosening/Infection Radiographic Findings Figure 4-1-3
With Ingrowth Arthroplasty
• Prominent prosthetic subsidence (>8mm)
• Bone destruction
• Component migration or motion
• Prominent lucent zone about metal (>2mm)
• Increasing number of displaced beads
Infection of Total Joint Arthroplasty

• The major long-term complication (1%-4%)
➢ 33% first 3 months post-op
➢ 33% 3 months – 12 months post-op
➢ 33% > 12 months post-op
• Usually associated loosening
• Difficult to differentiate from aseptic loosening
Radiographic Signs Most Suspicious for

• Extensive bone destruction Aseptic loosening of femoral component ingrowth hip
➢ 47% sensitive; 96% specific replacement with prominent lucency, lateral migration of
femoral stem (arrow), and subsidence medially (arrowhead)
• Air in soft tissue and/or joint
• Extensive or aggressive periosteal reaction
➢ 25% sensitive; 92% specific
• Wide or irregular lucent zone Figure 4-1-4
Radionuclide Evaluation of Total Joint

Arthroplasty
• Bone scintigraphy
• Gallium scan
• Indium WBC scan
• PET (combined with bone scan)
Bone Scintigraphy
• Normal increased activity post-op (6–9 months)
• Increased activity subsequently suspicious for
loosening/infection
• Nonspecific
• Overall accuracy 50%-70%
Gallium (Ga-67) Scanning Aseptic loosening of acetabular component ingrowth hip

• In conjunction with bone scan replacement with prominent lucency (arrows) and bead
• Incongruence with increased gallium uptake vs. bone sheading (circles)
scan suspicious for infection
• Not as accurate as bone scan WBC combination
Figure 4-1-5
Infection of total knee arthroplasty with early prominent

development of lucency and bone destruction (arrows) about
both the femoral and tibial components soon following surgery
(two months- previous normal post-operative radiographs not
shown)
Joint Replacement and Bone Grafts 700 Musculoskeletal Radiology

Indium-111 WBC Scanning Figure 4-1-6
• Increased sensitivity (50%-100%) and specificity (45%-100%) for
infection of TJR
• Increased activity at tip of metal components can be normal for
up to 2 years post-op
• Used in conjunction with bone and bone marrow scans
incongruity with more uptake on WBC scan > 90% accuracy
• Sensitivity and the ability to correctly localize infection decreases
➢ Infection becomes more chronic
➢ Anatomic location more central
Arthrography of Joint Arthroplasty

• Purpose
➢ Obtain fluid for culture/sensitivity
➢ Document intra-articular location
➢ Confirm loosening
➢ Detect other causes of pain
Hip Arthrography Technique Digital subtraction arthrogram with contrast in

• Preliminary fluoroscopy and radiographs the bone remnant interface of the acetabular
component (zone 2-arrowhead) and below the
• Anterolateral approach to metal at head neck junction 20 gauge
intertrochanteric line (arrows) representing
spinal needle aseptic loosening of both components
• Aspiration for culture
• If no aspirate inject saline reaspirate
• Contrast injection with subtraction technique
• Radionuclide injection
• Pre and post exercise radiographs
Arthrographic Criteria for Loosening/Infection

[Figure 4-1-6]
• Acetabular Component
➢ Contrast in bone – cement or metal-cement interface all zones (90%)
➢ Contrast in bone – cement or metal-cement interface zones I & II or zones II
& III (90%)
➢ Contrast in zones I & III with medium or large pseudocapsule bursa (57%)
➢ Rim of contrast >2mm thick any zone (95%)
➢ Lymphatic filling (?)
• Femoral Component
➢ Contrast in cement-bone interface distal to intertrochanteric line (98%)
➢ Contrast in bone-metal interface below intertrochanteric line (98%)
➢ Contrast at or below mid component – long stemmed prosthesis (98%)
Knee Arthrography Technique Figure 4-1-7

• Preliminary fluoroscopy and radiographs
• Lateral patellofemoral or anterior intercondylar approach 20
gauge spinal needle
• Aspirate for culture
• If no aspirate inject saline reaspirate
• Contrast injection-subtraction technique
• Pre and post exercise radiographs
Arthrography and Bursa

[Figure 4-1-7]
• Greater trochanteric – 50%
• Supraacetabular – 33%
• Iliopsoas – 17%
• Can reduce accuracy of arthrography
II. Small Particle Disease

• Granulomatous pseudotumor/histiocytic reaction/osteolysis
• Previously unusual late sequelae of arthroplasty Septic loosening of the femoral component on
• Now may be most common cause failure arthrography of a total hip replacement with
• Large lobulated lucencies with cortical thinning synovial nodularity in the joint (arrows) and
• Prosthesis loosening supraacetabular bursa formation (arrowhead).
Contrast extends below intertrochanteric line

Small particle disease (osteolysis or granulomatous

pseudotumor) as a cause for loosening of the tibial component
of a total knee replacement with large mass-like
radiolucency/low attenuation in the proximal tibia (arrowheads)
with associated fracture (arrow)
Figure 4-1-10
Small particle disease as a cause for
loosening of femoral and acetabular
components of a total hip replacement
with multiple largely intracortical areas of
radiolucency (arrowheads)
Dislocation / Abnormal Alignment

Normal Alignment
• Acetabular angle: 40 degrees (+ or – 10 degrees) –
AP view
• Acetabular anteversion
➢ 0–30 degrees – lateral view
Knee:
• Tibial plateau component parallel to floor
• Tibia 5–7 degrees valgus
Abnormal Alignment
Predisposing to Subluxation Dislocated noncemented total hip replacement, both femoral
[Figure 4-1-10] (arrowhead) and acetabular components (arrows), with
increased inclination of the acetabular component as a
• Varus position of knee is unacceptable
predisposition to this complication
• Acetabular angle > 50/55 degrees – AP view
• Acetabular anteversion < 0 degrees or > 30 degrees lateral view
• Exceeding extremes of motion
• Interposed material
• Greater trochanteric separation Figure 4-1-11
• Joint effusion
• Loss of soft tissue support or imbalance (knee)
IV. Fractures and Nonunion Associated with

Arthroplasty
[Figures 4-1-11 and 4-1-12]
• Bone
• Metal
• Cement
• Polyethylene
Greater Trochanteric Nonunion After Total

Hip Arthroplasty
[Figure 4-1-11]
• Improves exposure at surgery Fracture of greater trochanteric wire mesh on follow up
• Osseous union normally 6 – 12 weeks radiograph (right image) with retraction due to the pull of the
• Nonunion results in lack of abductor support abductors (arrow) about the total hip replacement.
• Bursitis predisposes to dislocation

Heterotopic Bone Formation After Total Joint Arthroplasty Figure 4-1-12
• Not infrequent – 3 weeks post-op
2 years to mature
• Hip: 21%–40%; Knee: 10% anterior to femur
• Predisposing conditions – Ankylosing Spondylitis, DISH, prior
occurrence
• Treatment – radiation, steroids, diphosphonates, surgery, indocin
Brooker Classification
Heterotopic Bone
• After hip replacement
➢ Class I: Small islands of bone
➢ Class II: Bone projection from acetabulum or femur with >1 cm
between osseous surfaces
➢ Class III: <1cm between opposing bridge surfaces
➢ Class IV: Osseous ankylosis bridging joint
Cement Extrusion
• Usually clinically insignificant
• Vein or lymphatic
• Rarely nerve, vascular, bowel or bladder injury
Polyethylene Liner Displacement & Metal Arthropathy

[Figure 4-1-13]
• Allows metal-metal friction
• Inflammation and aseptic synovitis
• Abnormal component position
• Visualize radiolucent polyethylene Ingrowth total hip replacement with fracture
• Metal – line sign and debris (arrow) at the tip of the femoral component
• Prevented by early recognition transfixed by cerclage wires
VIII. Silicone Arthroplasty Figure 4-1-13

• Complications
➢ Fracture
➢ Dislocation
➢ Infection
➢ Silicone arthropathy
Displacement of polyethylene liner on follow-up radiograph of

total hip replacement (right image). Note widened medial joint
space compared to initial post-op radiograph (left image)
“metal-line” sign (arrowhead) and radiolucent rotated
polyethylene liner (*)
References
Joint Replacement
1. Bauer TW, Schils J. The pathology of total joint arthroplasty. I. Mechanisms of implant fixation. Skeletal Radiol.
1999 Aug;28(8):423-32. Review.
2. Keogh CF, Munk PL, Gee R, Chan LP, Marchinkow LO. Imaging of the painful hip arthroplasty. AJR Am J
Roentgenol. 2003 Jan;180(1):115-20.
3. Manaster BJ. From the RSNA refresher courses. Total hip arthroplasty: radiographic evaluation. Radiographics.
1996 May;16(3):645-60. Review.
4. Oswald SG, Van Nostrand D, Savory CG, Callaghan JJ. Three-phase bone scan and indium white blood cell
scintigraphy following porous coated hip arthroplasty: a prospective study of the prosthetic tip. J Nucl Med. 1989
Aug;30(8):1321-31.

History and Importance of Bone Graft Procedures Figure 4-1-14
• First performed 1688
• Second most frequently transplanted tissue
• Vital for orthopedic management
Radiologic Assessment: Important for

Patient Management
• Normal bone graft incorporation
• Abnormal alterations
Imaging Modalities
• Radiographs
• Conventional Tomography
• Scintigraphy
• Computed Tomography (CT)
• Magnetic Resonance Imaging (MRI)
Bone Graft Indications

• Delayed or nonunion
• Pseudarthrosis
• Fill osseous defects or cavities
• Arthrodesis Pictorial representation of onlay (A) and inlay (B and insert)
• Stabilize spinal segments bone graft procedures
• Bone stock in arthroplasty
• Restore function in diseased articulations Figure 4-1-15
Classification of Bone Graft by Origin

• Autograft
• Allograft (homograft)
• Xenograft (heterograft)
• Bone graft substitute
Classification of Bone Graft by Structure

• Cancellous
• Cortical
• Combination
Classification of Bone Graft by Technique [Figure 4-1-14]

• Onlay
• Inlay
• Dowel
• Muscle pedicle
• Strut
• Vascularized
• Clothespin (H)
Radiographic Evaluation of the Donor Site

[Figures 4-1-15 and 4-1-16]
• Iliac
• Fibula
• Rib
• Distal radius
• Calvarium
• Femoral head
• Tibia
• Greater trochanter
• Posterior spinal elements
Dowel graft placed across a scaphoid nonunion

(arrows) with progressive healing at both the
bone grafted site (arrows) and bone graft donor
site (arrowheads) on radiographs 3 months apart

Normal Radiographic Appearance Of Donor Site Figure 4-1-16
[Figure 4-1-17]
• Wedge or oval defect
• Irregular margins after surgery
• Initial increase in ill-defined margins
• Subsequent marginal sclerosis
• Complete regeneration
Donor Site Complications

• Pain
• Failure to recognize (radiologists)
• Infection
• Muscle herniation
• Involvement of sacroiliac joint
• Fracture
Normal Bone Graft: Repair/Incorporation CT of normal iliac bone graft donor site with outer shell of bone
[Figures 4-1-18 and 4-1-19] retrieved (arrowheads) and no violation of the SI joint (arrow)
• Cancellous autograft
• Cortical autograft
• Vascularized autograft Figure 4-1-17
• Allograft
Figure 4-1-18
Fibula resection for use as bone graft with

regeneration over time on three sequential
radiographs
1 month post-op 2 months post-op Figure 4-1-19
4 months post-op 6 months post-op

Progressive normal incorporation of cancellous Normal incorporation (arrows) of fibular cortical graft replacing a
autograft (arrows) about post-traumatic site in the humeral resection for Ewing sarcoma reconstruction. Three sequential
lower leg on radiographs with individual osseous radiographs show progressive osseous bridging between fibular graft
fragments coalescing into a large ossific mass and native humerus (arrowhead)

Important Terminology: Bone Graft Healing
• Osteoconduction: Tissue ingrowth with prominent vascular and mesenchymal
components
• Osteoinduction: Mesenchymal tissue differentiation into tissue capable of
osteogenesis
• Osteogenesis: Bone formation
Vascularized Bone Autograft

• Rib
• Iliac
• Fibula
Indications: Vascularized Autograft

• Intercalary defects
• Composite defects
• Mandible reconstruction
• Tumor resection
• Conventional failure
• Congenital pseudarthrosis
Normal Autograft Healing: Cancellous
Osseous union 1-1.5 years:

allografts successful in this pattern 70-80%
Normal Autograft Healing: Cortical
necrotic
osteoclastic

Characteristics Fibula Rib Iliac Crest
Bone Length (Max) 22-26 cm 30 cm 10 cm

Shape Straight Curved Slight Curve
Structure Cortico-Cancellous Membranous Cortico-Cancellous
Vessels
Artery Peroneal Posterior Intercostal Deep circumflex-iliac
or Superficial
circumflex iliac
Vein 2 Venae Comitnantes 1 Intercostal 1 Vena comitante
Vascular Stalk 1.0-7.0 cm 3.0-5.0 cm 1.5-5.0 cm

• Advantages
➢ Graft remains viable
➢ Promotes healing
➢ Participates in osteogenesis
➢ Improved strength
• Disadvantages
➢ Microvascular surgery expertise required
➢ Increased time for surgery
➢ Two surgical sites
Autograft Limitations
• Insufficient volume
• Postoperative morbidity risk
• Inability to mold for function
Allograft
• Particulate
• Intercalary
• Osteoarticular
Allograft Healing Depends on the Recognized Immunologic

Disparity
Allograft Source and Pretreatment

• Trauma 15–45 years of age
• No history neoplasm or infection
• No steroids or respirator
• Freeze or freeze-drying
Osteoarticular Allograft
• Osteochondral shell
• Half-joint
• Whole joint

Normal Allograft Healing
Histology Radiographic Appearance
Prominent vascular and granulation

tissue ingrowth
Short initial osteoclastic phase (several Graft resorption with ill-defined and
weeks) irregular margins
Improved osteoinduction and Further gradual decrease in graft
osteogenesis owing to osteoprogenitor density and volume (first 1 month) until
cells improved survival (endosteum and bone production exceeds resorption
marrow elements)
Further healing Gradual hematopoietic tissue ingrowth
w/graft density increasing to normal,
loss of margin between native bone and
graft w/trabecular continuity and
formation of cortex (2-6 months)
Initial increase in strength due to
osteoblastic activity
Osteoarticular Allograft
• Low ratio bone: cartilage requires less pretreatment
• Cartilage immuno-privileged tissue
• Success depends on osseous component
Bone Graft Complications

• Infection
• Nonunion or pseudarthrosis
• Fracture
• Graft resorption
• Joint instability
Infection of Bone Graft

• Persistent tissue swelling
• Periosteal reaction
• Progressive bone destruction
• Lucency about fixation and failure
• Indium WBC scan may add specificity
Figure 4-1-20
Infection of Bone Graft
[Figure 4-1-20].
• Autograft
➢ Clinical evidence usually present
• Vascularized Autograft
➢ 5%
• Allograft
➢ 5–13% soft tissue swelling (beyond 6 months
post-op)
➢ Increasing bone resorption (beyond 10 weeks
post-op)
Nonunion and Pseudarthrosis

• Persistent lucency graft/host junction
• Sclerosis at margins
• Rounded osseous margins
• Fracture or loosening of adjacent fixation
• Stress views helpful Allograft infection with progressive lack of osseous bridging
(arrowheads) and ultimately loosening and fracture of the
fixation device (arrow) on three sequential radiographs

Nonunion and Pseudarthrosis
• Autograft
➢ Failure to heal by 12 months
➢ 14% in segmental cortical bone grafts
➢ 7% questionable graft viability
➢ Bone scan – 1 week
• Allograft
➢ 11% preventable with adequate osseous contact at host/graft junction
➢ Treated with regrafting and/or fixation change
Fracture
• Linear lucency through graft
• Callus
• Stress views helpful
Fracture
• Autograft
➢ Not infrequent
➢ Often after healing with stress (6–8 months)
➢ More common in longer grafts
➢ Decreased incidence 3.5% due to improved strength
• Allograft
➢ 16.5% most at weak points
➢ Affected by pretreatment method
Bone Graft Resorption

• Progressive graft loss
• Graft decreases in size
• Graft decreases in density
• Difficult to distinguish from infection
Bone Graft Resorption

• Autograft
➢ Unopposed osteoclastic activity
➢ Same as autograft
• Allograft
➢ Acute or chronic rejection
➢ Graft replaced by fibrous tissue
Osteochondral Allograft: Joint Instability

• 2.9%–5.5 % incidence
• Causes: articular incongruity; lack of innervation and cartilage viability
• AVN, neuropathic joint or rejection
• Can be difficult to distinguish from infection or rejection
Joint Instability: Radiographic Findings

• Joint narrowing and sclerosis
• Osteophytes and subchondral cysts
• Fragmentation with debris
• Fracture and migration of fixation
• Weight bearing views helpful
Xenograft
• Supply-demand limitations of other graft
• Calf and ox-bone
• Treated to prevent rejection
• Used as spacer prevents soft tissue ingrowth
• Other graft material in combination

Bone Graft Substitutes
• Hydroxyapatite (Ca10 [PO4] 6 [(OH) 2])
• Tricalcium phosphate (Ca3 [Po4] 2) Figure 4-1-21
• Dense or porous ceramics
• Osteoconductive but not osteoinductive
Porous Ceramics
• Goniopora-cancellous bone
• Porites-cortical bone
• Approved human studies 1982
• Initially weak mechanically
• Strength increases after incorporation
• More dense than native bone
• Lucent peripheral band obliterated with ingrowth
• Complications: Fractures, Implant failure, Infection
Spinal Bone Graft Assessment

• Causes of Failed Back Surgery Syndrome (FBSS)
➢ Recurrent disk
➢ Arachnoiditis
➢ Epidural scar Pseudoarthrosis of posterolateral
➢ Infection lumbar spine graft with horizontal
➢ Facet subluxation radiolucent clefts (arrows) and
➢ Spinal stenosis surrounding sclerosis on conventional
tomography
➢ Pseudarthrosis
➢ Spine Bone Autograft: Normal Healing/Incorporation Figure 4-1-22
➢ Cervical - 3-4 months
➢ Lumbar - by 9 months
Spine Bone Autografts: Normal Healing/Incorporation

• Cervical – 3–4 months
• Lumbar – by 9 months
Anterior Vertebral Interbody Fusion

• Cervical and lumbar spine
• Rib, iliac or fibular graft
• Initial discrete graft-host junction obliterated
Radiologic Evaluation: Spine Pseudarthosis

[Figures 4-1-21 and 4-1-22]
• Radiographs
➢ Oblique views best; Radiolucent defect; Motion with bending increase
spine curve
• Bone Scintigraphy
➢ Wide range specificity and sensitivity Normal uptake <6 months post-op
➢ Abnormal if increased uptake beyond 6 months post-op; Improved with
SPECT imaging; Asymptomatic patients may have focal uptake
• Conventional Tomography
➢ AP optimal plane (2–5 mm) best reported method – 96% polydirectional
best; Limited availability and technically demanding
Interbody Fusion Complications

• Nonunion
• Graft fracture
• Extrusion of graft fragments
Sagittal T1 (upper image) and
• Infection T2-weighted (lower image) MR
images showing horizontal
References clefts (arrowheads) in
posterolateral lumbar bone
Bone Graft graft with high signal on long
1. Murphey MD, Sartoris DJ, Bramble JM: "Radiographic Assessment of Bone Grafts" TR image representing two
In: Bone Grafts from Basic Science to Clinical Application, Habal MB, Reddi AH, sites of pseudoarthrosis
Editors. Philadelphia: W. B. Saunders, 1992, p. 9-36.

Musculoskeletal Manifestations of Chronic
Renal Insufficiency
Mark D. Murphey, MD
Hyperparathyroidism (HPT)
• Primary
• Secondary
• Tertiary
Primary Hyperparathyroidism: Etiology

• Adenoma 80%–90%
• Hyperplasia 10%–15%
• Carcinoma 2%–4%
• Nonparathyroid tumor
• MEN Syndromes
Tertiary Hyperparathyroidism: Etiology

• Autonomous gland function
End-Stage Renal Disease: Secondary Hyperparathyroidism

• Multiple Causes
• Most common cause glomerulonephritis
• 0.01% U.S. population
• 85,000 hemodialysis patients/year
• 8,000 renal transplantations/year
• Involves all organ systems
• Musculoskeletal manifestations common and increasingly recognized
Secondary Hyperparathyroidism: Etiology

• Inability of renal excretion of phosphate
• Resultant hyperphosphatemia
• Hyperplasia of parathyroid chief cells and increased parathormone (PTH)
• Also reduced degradation of PTH
Effects of PTH on Bone

• Development of osteoclasts, osteoblasts, osteocytes
• Osseous resorption
• Brown tumors
Renal Osteodystrophy
• Secondary hyperparathyroidism
• Osteosclerosis
• Osteoporosis
• Osteomalacia
• Soft tissue and vascular calcification
Bone Resorption in Chronic Renal Insufficiency (CRI)

• Caused by osteoclastic activity
• 10% early stages; 50%–70% long-standing disease
• Subperiosteal
• Cortical
• Subchondral
• Trabecular
• Endosteal
• Subligamentous/Subtendinous
Musculoskeletal Radiology 711 Musculoskeletal Manifestations of Chronic Renal Insufficiency

Subperiosteal Resorption Figure 4-2-1
[Figures 4-2-1 to 4-2-3]
• Initially described by Camp and Ochsner
• Pathognomonic-hyperparathyroidism
• Lacelike irregularity of cortical margin progress to
scalloping and spiculation
• Earliest involvement middle phalanges/ terminal tufts-hand
• Additional sites-upper medial tibia, femur and humerus,
ribs, lamina dura
Figure 4-2-2
Subperiosteal resorption involving the middle, proximal

and terminal phalanges (arrows) resulting from
secondary hyperparathyroidism and renal failure.
Similar features are noted on the clinical photograph as
well as clubbing
Figure 4-2-3
Figure 4-2-4
Subperiosteal resorption involving the

middle and terminal phalanges (solid
arrows) resulting from secondary
Subperiosteal resorption involving
hyperparathyroidism and renal failure.
the lamina dura (arrows) caused
The terminal phalanx also shows
by secondary
band-like acroosteolysis (open arrow)
hyperparathyroidism and renal
resulting from subperiosteal resorption
failure
Cortical Resorption
[Figure 4-2-4]
• Caused by osteoclastic activity within haversian canal Intracortical resorption with areas of intracortical
• Radiographs-intracortical tunneling with increased lucent tunneling (arrows) resulting from hyperparathyroidism
striations in cortex on radiography and matched histologic macrosection
• Nonspecific finding showing resorption along preexisting Haversian canals
(arrowheads)
Endosteal Resorption
• Causes scalloping of endosteum- hands
• Osteopenia with loss of trabecular sharpness
• Calvarium – “salt and pepper” appearance with loss of distinction of
tables
Endosteal resorption in the skull on CT

causing loss of distinction of inner and
outer table cortices (salt and pepper
appearance-arrowheads) in secondary
hyperparathyroidism
Musculoskeletal Manifestations of Chronic Renal Insufficiency 712 Musculoskeletal Radiology

Subchondral resorption of the distal clavicle and

acromion (arrows and arrowhead) from secondary
hyperparathyroidism on radiography
Subchondral resorption of the metacarpal heads

(arrows) from secondary hyperparathyroidism on
radiography simulating an erosive arthropathy
Figure 4-2-8
Subchondral Resorption
[Figures 4-2-6 to 4-2-8]
• Common in appendicular and axial skeleton
• Often in hands-single DIP joint (4th or 5th) also MCP and
PIP joints
• More recently polyarticular involvement 40% of patients on
long-term hemodialysis IP and first CMC joints with
symmetry
• Simulates erosions, often progress, 50% symptomatic
• Other frequent sites-distal clavicle, AC joint (20%), SI joint,
SC joint, symphysis pubis, posterior patella Brown tumor (*), diffuse sclerosis and subchondral
• Pathologically-collapsed cortical bone and overlying resorption (arrows) about the SI joints are seen on this
cartilage CT of the pelvis in a patient with chronic renal failure
• Initiate an osteogenic synovitis
• Accentuated by mechanical stress, joint incongruity and
intraarticular debris
Subligamentous/Subtendinous Resorption
[Figure 4-2-9]
• Patients usually asymptomatic
• Radiographs-smooth and scalloped or irregular
• Common sites Figure 4-2-9
➢ Inferior calcaneus
➢ Greater and lesser trochanters
➢ Anterior inferior iliac spine
➢ Humeral greater tuberosity
➢ Ischial tuberosity
➢ Elbow
Brown Tumors (Osteoclastomas)

• Caused by localized bone replacement by vascularized
fibrous tissue
• May become cystic after necrosis and liquefaction
(osteitis fibrosa cystica) higher incidence in primary
hyperparathyroidism; 1.5%–1.7% in secondary
Subligamentous/subtendinosis resorption in the pelvis
on radiography at the ischial tuberosity and anterior
inferior iliac spine (arrowheads) resulting from secondary
hyperparathyroidism and renal failure

Brown Tumors: Radiographic Findings Figure 4-2-10
[Figures 4-2-8 and 4-2-10]
• Often solitary; may be multiple
• Well defined lytic lesions
• Frequently eccentric or cortical (long bones)
• May cause scalloping and bone expansion
• Sites-ribs, pelvis, facial bones and femora, axial skeleton
can be involved
• May heal after treatment with calcification, sclerosis and
lesion disappearance
Periosteal New Bone: Formation

• Caused by osteoblastic activity
• Prevalence 8%–25% often with severe disease
• Linear often with radiolucent zone separating it from cortex
• Can be laminated and chronically thicken cortex
• Most common-humeri, femora, tibiae, radii, ulnae,
metacarpals, metatarsals and phalanges
Osteosclerosis
[Figure 4-2-11] Brown tumor of hyperparathyroidism involving the tibia
• Cause unknown with pathologic fracture (arrows) on radiography and
• 9%–34% coronal macrosection. Cyst formation (*) is seen on
• Predilection for axial skeleton the macrosection
• “Rugger Jersey” spine
• Other sites-pelvis, ribs and clavicles Figure 4-2-11
• Metaphyses and epiphyses can be involved
• After renal transplant osteosclerosis may regress but more common to
further increase
Osteopenia
• Accumulated effect of osteomalacia, bone resorption and osteoporosis
• Contributory factors-acidosis, poor nutrition, azotemia, steroids,
hyperparathyroidism, and reduced vitamin D
• After renal transplant osteopenia may worsen or bone mineral content
may increase
• Predisposed to fractures (5%–25%): vertebral body, pubic rami and ribs
• Fracture healing-normal but delayed
Osteomalacia
• Decreased active form of vitamin D
• Renal tissue hydroxylates vitamin D to active form
• Additional factors-hypocalcemia, inhibitors to calcification in uremia,
aluminum toxicity, hepatic dysfunction
Rickets Diffuse sclerosis of the cervical spine

• Common in children with chronic renal insufficiency on radiography in a patient with
• Normal vessels that invade zone of provisional calcification fail to secondary hyperparathyroidism
develop
• Result-disorganized cartilage zone columns
Figure 4-2-12
Radiographic Findings in Rickets

[Figure 4-2-12]
• Widened growth plate
• Epiphyseal irregularity; metaphyseal fraying and
cupping
• Delayed bone age and osteopenia
• Bowed long bones and scoliosis
• Concave vertebral endplates
• Basilar invagination
• Triradiate pelvis and acetabuli protrusio
• Rachitic rosary
• Slipped epiphyses
Rickets in the distal femur resulting from renal failure on
radiography and coronal macrosection with metaphyseal
widening and cupping (arrows) caused by growth plate
disorganization (arrowheads)

Slipped Epiphyses in CRI Induced Rickets Figure 4-2-13
• Not uncommon -10%
• Proximal femur and humerus, distal femur and radius,
metacarpal and metatarsal heads
• Greatest risk-adolescent boys, uremia > 2 years,
treatment close to onset of puberty
• Usually bilateral in chronic renal insufficiency (CRI)
and often asymptomatic initially
Radiographic Findings: SCFE in CRI

[Figure 4-2-13]
• Medial femur subperiosteal resorption
• Increase epiphyseal plate width
• Decrease neck-shaft angle
• Typical findings of slipped capital femoral epiphysis
(SCFE)
Rickets of the proximal femora and bilateral slipped capital
femoral epiphyses (arrowheads) in a renal failure patient on
Radiographic Findings: Osteomalacia in pelvic radiograph with hips abducted
Adults
• Osteopenia with ill defined trabeculae unlike
osteoporosis Figure 4-2-14
• Looser zones-pseudofractures
➢ Uncommon - 1% CRI patients
➢ Pubic rami, medial femoral neck, scapulae, ribs,
long bones, lesser trochanters, ischial tuberosity
Soft Tissue and Vascular Calcification

• Increases calcium - phosphate product > 75mg/dL
• Contributory factor - local tissue damage and
alkalosis (calcium salt precipitation)
• Common sites - ocular tissue, arteries,
subcutaneous, periarticular and visceral
Periarticular Calcification
[Figures 4-2-14 and 4-2-15]
• Asymptomatic or pain and joint limitation
• Prevalence
➢ 7% after 1 year hemodialysis
➢ 55% after more than 4 years hemodialysis
• Often regresses with treatment
• Often multifocal and symmetric
• Dense and cloudlike on radiographs
• Hydroxyapatite - chalky paste-like material
• Can extend into tenosynovial tissue and joints
• Sites - phalangeal joints, wrists, elbows, shoulders,
hips, knees, ankles
Figure 4-2-15
Heterogeneous large soft tissue mass (*) about the right

shoulder on coronal STIR MR images in a patient with renal
failure initially sent to radiology for biopsy of suspected
sarcoma. Subsequent radiograph shows subtle calcification
and vascular catheters in the central vessels for hemodialysis.
Diagnosis of periarticular calcification related to renal failure
and no biopsy was necessary
Periarticular calcification about the left hip on CT with several

calcium fluid levels (arrows) in a patient with renal failure

Arterial Calcification
• Occur in media and intimal tissue
• Pipestem appearance radiographically
• Can make shunts or fistula for hemodialysis difficult
• Initially - dorsalis pedis artery also leg, hand and forearm
• Prevalence – 27% < 1 year therapy
➢ 83% with 8 years or more of therapy
➢ Rare in children
• Second type - nodular with luminal encroachment, obstruction with ulceration,
gangrene and cardiac failure
• Calcification in shunt aneurysms
Visceral Calcification
• Usually not apparent on radiographs
• May detect on bone scintigraphy (poor looking bone scan)
• Sites - heart, lungs, stomach, kidneys
• Prevalence - 79%
• In myocardial tissue important, can cause conduction defects and death
Musculoskeletal Abnormalities More Common After Treatment

• Aluminum toxicity
• Amyloidosis
• Tendon rupture
• Crystal deposition
• Infection
• Avascular necrosis (AVN)
Aluminum Toxicity
• Prevalence 1%–30% (rare today)
• Results in osteomalacia previously responsible for most osseous abnormalities
in patients on long-term hemodialysis
• Unknown mechanism
• Clinically - low PTH, serum aluminum > 100ng/mL
• Cause - ingestion of aluminum salts in phosphate - binding antacids to control
hyperphosphatemia
• Cannot excrete alumina
• Toxic effects
➢ Cerebral (Encephalopathy)
➢ Osseous system
Aluminum Toxicity: Radiographic Findings

[Figure 4-2-16]
• Osteomalacia - osteopenia, looser zones, fractures, rickets
• More than 3 atraumatic fractures (86%) - ribs, vertebrae, hips, pelvis, sternum,
clavicles, extremities
• AVN
• Lack of osteosclerosis
• Limited subperiosteal resorption
• Bone biopsy - histochemical stain for aluminum
Figure 4-2-16
Amyloidosis
• Secondary due to chronic disease
• B2 – microglobulin
• Areas of deposition – bone, tenosynovium,
intervertebral disk, cartilage, capsule, ligament,
muscle
• Stains with Congo red, characteristic under
polarized microscopy and immunoperoxidase
methods
Amyloidosis
• Musculoskeletal Involvement
➢ Carpal tunnel syndrome
➢ Osseous and intraarticular deposition Osteomalacia due to aluminum toxicity in a renal failure patient
➢ Destructive spondyloarthropathy with radiograph showing multiple nontraumatic fractures
(arrows) and acetabulae protrusio (curved arrows)

Carpal Tunnel Syndrome in CRI Figure 4-2-17
• Long-term hemodialysis prevalence 2%–31 %
• Equal sex distribution
• No prevalence for dominant hand
• Amyloid primary cause of median nerve compression
• Other causes - venous stasis and edema related to
treatment fistula
Osseous and Intraarticular Involvement in

CRI
[Figures 4-2-17 and 4-2-18]
• Focal lytic areas or less well defined intramedullary
lytic lesions
• Most common - carpus - scaphoid, lunate and
capitate - can enlarge
• Other sites - humeral head, knee (patella) and about Amyloidosis in a patient with chronic renal insufficiency
causing multiple punched out lytic lesions in the wrist
hip
(arrowheads)
• Endosteal scalloping, fractures, extrinsic erosion
from soft tissue mass, neuropathic appearance Figure 4-2-18
• Intraarticular deposition - common in hip, knee,
shoulder-low intensity on T2W images
Spontaneous Hemorrhage Associated with

CRI
• In hemodialysis patients likely related to heparin
• Most frequent in thigh
• MRI best for evaluation; appearance varies with
hemorrhage age
Destructive Spondyloarthropathy
• Described 1984 by Kuntz and colleagues
• Usually in patients on long-term hemodialysis
(2–19 years)
• Prevalence - 15%; symptoms - pain
• Cervical and lumbar spine
• Multiple levels >50% of patients Amyloid deposition in the hip joint of a renal failure patient on
hemodialysis on coronal T1 and T2-weighted MR images
• Rapid progression 33%; simulates infection
showing low signal intensity material in the joint (*) with
• Initial postulated etiology - crystal and noncrystal extrinsic bone erosion (arrow)
deposition, neuropathic and hyperparathyroidism
• Amyloid now considered offending agent Figure 4-2-19
Destructive Spondyloarthropathy: Radiographic
Findings
[Figure 4-2-19]
• Discovertebral erosions with sclerosis
• Vertebral body compression
• Disk space narrowing with Schmorl nodes
• Lack of osteophytes
• Facet involvement with subluxation
Destructive spondyloarthropathy at L5-S1

caused by amyloid deposition related to renal
failure with disc narrowing and destruction
(arrows) on radiograph simulating
infectious spondylodiskitis

Distinction of Infection vs. Destructive Spondyloarthropathy
[Figure 4-2-20]
Figure 4-2-20
• Clinical symptoms / laboratory evidence lacking
• Multilevel involvement unusual for bacterial infection T1 T2
• Limited uptake on scintigraphy
• CT - lack of paravertebral soft tissue mass (also
MRI)
• MRI - disc / endplate marrow replaced T1W
No prominent increased intensity on T2W
Tendon Rupture or Avulsion in CRI

• Spontaneous; in patients on long- term dialysis
• Solitary or multiple
• Tendon sites - quadriceps, patellar, triceps, flexors
and extensors of fingers
• Cause - PTH excess - increased joint laxity
➢ Tendon calcification Destructive spondyloarthropathy related to amyloid deposition
➢ Chronic acidosis (same patient as previous radiograph) with marrow and disk
• Result - decrease tendon tensile strength and replacement remaining predominantly low signal intensity on all
accelerated degeneration pulse sequences (*)
Radiologic Findings in Tendon Rupture in CRI

[Figure 4-2-21]
• Before rupture - may see subtendonous bone resorption
• After rupture - focal soft tissue swelling, effusion, subluxation
• CT, MRI or sonography to evaluate tendon integrity and disruption site
Crystal Deposition Disease in CRI Figure 4-2-21

• Calcium hydroxyapatite, CPPD, monosodium urate, calcium oxalate
• Hemodialysis elbow - olecranon bursitis
• Calcium hydroxyapatite - EM or X-ray diffraction
• CPPD - chondrocalcinosis not common - knee, wrist, hip, shoulders
and symphysis
• CPPD arthropathy rare in CRI
• Gout - infrequent in CRI, radiographic findings same as those in
primary podagra except distribution
• Oxalosis - chondrocalcinosis, calcified joints, disks and
periarticular, diffuse osseous sclerosis
Predisposition to Infection in CRI

• Depressed host responsiveness
• Steroids and immunosuppressive treatment
• Entry site via arteriovenous fistula for hemodialysis
• Secondary infection in osteonecrosis
Infection Associated with CRI

• Osteomyelitis and septic arthritis Spontaneous rupture of the quadriceps
• Bacterial or fungal tendon (arrows) in a renal failure patient on
• Radiographic findings - deep soft tissue swelling, periosteal sagittal T2-weighted MR image
reaction, bone destruction, and joint space narrowing - same as
other situations
• Unusual syndrome - progressive peripheral ischemic ulcers usually after
treatment and secondary infection
Bone Scintigraphy in CRI

• Diffuse increased activity “Super bone scan”
• May use as index of severity
• Cause of increased activity is combination of vitamin D deficiency and
hyperparathyroidism
➢ Increase rate bone turnover and collagen metabolism
➢ Excess immature collagen
➢ High enzyme activity
➢ Increased osseous surface area for binding
• Increased focal or diffuse soft tissue uptake

Avascular Necrosis in CRI
• Up to 40% of CRI patients after renal transplantation
• Additional factors - structural weakening, fracture and collapse, excess PTH,
graft-host reaction
• Most common site - femoral head
• Other sites - humeral head, about knee, talar dome, humeral condyles, cuboid,
carpal bones, long bone diaphyses
Avascular Necrosis in CRI

• Radiologic appearance identical to other causes AVN
• MRI most sensitive however only 50% show early changes
• Osseous malignancy can complicate osteonecrosis and general increased
malignancy rate after renal transplant
Hyperparathyroidism: Primary vs. Secondary

1 2
• Brown Tumors +++ ++

• Osteosclerosis Rare +++
• Chondrocalcinosis + Rare
• Periostitis Rare +
References
1. Camacho CR, Talegon Melendez A, Valenzuela A, Gonzalez Guirao MA, Gomez Benitez S, Gil L, palma Alvarez A,
Mateos Aguilar J. Radiological findings of amyloid arthropathy in long-term haemodialysis. European Radiology.
1992; 2:305-309.
2. Leone A, Sundaram M, Cerase A, Magnavita N, Tazza L, Marano P.. Destructive spondyloarthropathy of the cervical
spine in long-term hemodialyzed patients: a five-year clinical radiological prospective study. Skeletal Radiol. 2001
Aug;30(8):431-41.
3. Murphey MD, Sartoris DJ, Quale JL, Pathria MN, Martin NL. Musculoskeletal manifestations of chronic renal
insufficiency. Radiographics. 1993 Mar;13(2):357-79.
4. Slavotinek JP, Coates PT, McDonald SP, Disney AP, Sage MR.. Shoulder appearances at MR imaging in long-term
dialysis recipients. Radiology. 2000 Nov;217(2):539-43.

Fundamental Concepts of Musculoskeletal
Neoplasm: Radiographs
Mark D. Murphey, MD
Tumors are classified by their pattern of differentiation
Tumors are graded on their degree of anaplasia
Skeletal Components
(Derived from Embryonal Mesenchyme)
• Bone and cartilage progenitor cells
• Periosteal cells
• Hematopoietic cells
• Lipocytes
• Nerve and Schwann cells
• Fibroblasts
• Osteoclasts and Osteoclast-like cells
• Endothelial cells
• Perithelial cells
• Notochordal cells (rests) HISTOGENIC CLASSIFICATION
• Histiocytic cells OSTEOID BONE TUMORS
• Epithelial cells (rests)
BENIGN MALIGNANT
ENOSTOSIS OSTEOSARCOMA
OSTEOID OSTEOMA
OSTEOMA
OSTEOBLASTOMA
HISTOGENIC CLASSIFICATION
CARTILAGE BONE TUMORS
BENIGN MALIGNANT
CHONDROBLASTOMA CHONDROSARCOMA
CHONDROMYXOID FIBROMA
ENCHONDROMA
JUXTACORTICAL CHONDROMA
OSTEOCHONDROMA
MARROW BONE TUMORS
BENIGN MALIGNANT
LIPOMA LIPOSARCOMA
LYMPHOMA
MYELOMA/PLASMACYTOMA
Fundamental Concepts of MSK Neoplasm: Radiographs 720 Musculoskeletal Radiology

FIBROUS BONE TUMORS
BENIGN MALIGNANT
DESMOPLASTIC FIBROMA FIBROSARCOMA
HISTIOCYTIC TUMORS
BENIGN MALIGNANT
EOSINOPHILIC GRANULOMA MALIGNANT FIBROUS HISTIOCYTOMA
NOTOCHORD BONE TUMORS
BENIGN MALIGNANT
CHORDOMA (HISTOLOGICALLY BENIGN) CHORDOMA
VASCULAR TUMORS
BENIGN LOW GRADE MALIGNANT MALIGNANT
GLOMUS HEMANGIOENDOTHELIOMA ANGIOSARCOMA
HEMANGIOMA HEMANGIOPERICYTOMA
LYMPHANGIOMA
UNKNOWN ORIGIN TUMORS
BENIGN MALIGNANT
GIANT CELL TUMOR MALIGNANT GIANT CELL TUMOR
MALIGNANT FIBROUS HISTIOCYTOMA
EWING SARCOMA
ADAMANTINOMA
Musculoskeletal Radiology 721 Fundamental Concepts of MSK Neoplasm: Radiographs

Incidence of Bone Tumors
[Figure 4-3-1]
• Approximately 1 individual in 75000 develops a primary bone tumor that leads
to biopsy
• About 4000 new cases per year
Incidence of Bone Tumors

[Figures 4-3-2 to 4-3-4]
• Of biopsied primary bone tumors: malignant tumors are three times more
common as benign lesions
• Metastatic lesions are biopsied about 35 times more frequently than primary
tumors
Primary Benign Bone Tumors

[Figure 4-3-3]
Primary Malignant Bone Tumors

[Figure 4-3-4]
15
5

Important Factors in the Diagnosis of Bone Tumors
• Patient age and sex
• Bone involved
• Location in bone
• Lesion margin
• Matrix formation
These radiologic characteristics reflect the pathologic process and its biologic
activity.
Primary Benign Bone Tumors: Age Distribution by Decade

[Figure 4-3-5]
Figure 4-3-5
Primary Malignant Bone Tumors: Age Distribution by Decade

[Figure 4-3-6]
Figure 4-3-6

“The site frequency, peak age of incidence, and numerical frequency of bone
tumor indicate that they are not completely autonomous, but are subject
to the laws of field behavior and developmental anatomy of normal
bone...”
Johnson L. 1953 Figure 4-3-7
Location in Bone: Longitudinal

• Epiphysis
• Metaphysis
• Diaphysis
Figure 4-3-8
Chondroblastoma with lytic lesion in the

epiphysis
Ewing sarcoma involving the femoral diaphysis on radiograph, T1-weighted MR

and gross specimen
Location in Bone: Axial

[Figures 4-3-9 to 4-3-10]
• Central
• Eccentric
• Cortical Figure 4-3-10
• Juxtacortical
• Soft Tissue
Figure 4-3-9

Pattern of Bone Destruction and Lesion Margin Figure 4-3-11
[Figure 4-3-11]
• Type I: Geographic
➢ A: Well-defined, sclerosis
➢ B: Well-defined, no sclerosis
➢ C: Ill-defined
• Type II: Motheaten
• Type III: Permeative
• Transition Zone
Margin Reflects Biologic Activity

Figure 4-3-12
Aggressive versus Nonaggressive
Biologic Activity
Margin Growth Rate
Geographic IA Slow
Geographic IB Slow to Intermediate
Geographic IC Intermediate
Motheaten Intermediate
Permeative Fast
1A Margin
[Figure 4-3-12]
• Geographic
• Well-Defined
• Sclerosis
Geographic 1A
Geographic 1A: Differential Diagnosis
• Bone cyst
• Brodie abscess [Figure 4-3-14]
• Cartilage lesions
➢ Chondroblastoma
➢ Chondromyxoid Fibroma
➢ Enchondroma
• Fibroxanthoma
• Fibrous Dysplasia
Nonossifying Fibroma /Fibroxanthoma with geographic 1A margin on

radiograph, gross specimen and macrosection
Figure 4-3-14
Brodie abscess with geographic 1A margin.

Note the channel like extension (arrow) representing a sinus
tract inferiorly on the conventional tomogram (right image)

1B Margin Figure 4-3-15
[Figure 4-3-15]
• Geographic
• Well-Defined
• No Sclerosis
Geographic IB: Differential Diagnosis

• Giant Cell Tumor [Figure 4-3-16]
• Bone Cyst
• Cartilage Lesions
➢ Chondroblastoma
➢ Chondromyxoid Fibroma
➢ Enchondroma
• Myeloma/Metastasis
Figure 4-3-16
Geographic 1B
Figure 4-3-17
Giant cell tumor of the distal radius with geographic 1B margin

on radiograph and macrosection extending to subchondral bone
1C Margin
[Figure 4-3-17]
• Geographic Geographic 1C
• Ill-Defined
Figure 4-3-18
Geographic IC: Differential Diagnosis
• Chondrosarcoma
• Enchondroma (Active)
• MFH/Fibrosarcoma [Figure 4-3-18]
• Giant Cell Tumor
• Osteosarcoma
• Metastasis/Myeloma
Tumor Margin
• Motheaten [Figure 4-3-19]
• Permeative[Figures 4-3-20 and 4-3-21]
Fibrosarcoma

Motheaten Permeative
Motheaten: Differential Diagnosis

• Ewing sarcoma
• Round cell tumors
• Malignant fibrous histiocytoma/Fibrosarcoma
• Osteomyelitis
• Osteosarcoma
• Langerhans cell histiocytosis (LCH)
• Metastasis/Myeloma
Permeative: Differential Diagnosis Permeative

• Ewing sarcoma
• Round cell tumors
• Malignant fibrous histiocytoma/Fibrosarcoma
• Metabolic disorders [Figure 4-3-22]
• Osteomyelitis (acute) [Figure 4-3-23]
• Osteosarcoma
• LCH
• Myeloma/Metastasis
Figure 4-3-22
Figure 4-3-23
Hyperparathyroidism with variation of the permeative pattern of

bone lysis in the cortex on radiograph and macrosection
(multiple areas of resorption along Haversion canals – arrows)
Multifocal acute bacterial osteomyelitis with motheaten to

permeative destructive pattern of bone lysis on radiographs
involving the tibia and femur.
Macrosection
(right image shows pus on either side of cortex-arrows)

Lytic Patterns
[Figure 4-3-24]
Figure 4-3-24
Invisible Margin
[Figure 4-3-25: Lymphoma]
Figure 4-3-25
Lymphoma (same patient) with extensive marrow replacement (*) on T1 weighted MR, not seen on
radiograph images.

Changing Margin [Figure 4-3-26]
• Increased Biologic Activity
Figure 4-3-26
Changing Margin Osteonecrosis (*) with malignant

Osteonecrosis (*) with malignant transformation to MFH on CT (same
transformation to malignant fibrous patient as previous radiograph) with
histiocytoma (MFH) showing new lysis new cortical destruction laterally and
(arrow) at periphery on specimen soft tissue mass (arrow)
Matrix Formation radiograph representing the changing
• I. Mineralized margin
➢ Chondroid - rings, arcs, Coronally
honeycomb[Figure 4-3-27] sectioned gross
specimen and
➢ Osteoid - ivory or cloudlike
macrosection
[Figure 4-3-28]
showing
• II. Nonmineralized osteonecrosis (*)
➢ Fluid and MFH arising at
➢ Soft Tissue periphery (arrows)
➢ Fat (same patient as
previous
radiograph and CT)
Figure 4-3-27
Figure 4-3-28
Chondrosarcoma of the fibula on specimen

radiograph and gross specimen show “ring and Osteosarcoma of the tibia with dense cloud-like matrix
arc” matrix mineralization (arrows) mineralization (arrows)

Periosteal Reaction: Nonaggressive
[Figure 4-3-29]
• Solid (a)
• Buttressing (b)
• Expansion (c)
• Septation (d)
Figure 4-3-29
(a) (b)
(c) (d)

Periosteal Reaction: Aggressive
[Figures 4-3-30 and 4-3-31]
• Codman triangle (a)
• Sunburst (b)
• Hair-On-End (c)
• Laminated (d)
Figure 4-3-30
(a) (b)
(c) (d)

Polyostotic vs. Monostotic Figure 4-3-31
Holes in Bone
Polyostotic Lesions: Benign

• Langerhans Cell Histiocytosis (LCH) [Figure 4-3-32]
• Enchondromatosis
• Hereditary multiple exostoses (HME)
• Osteomyelitis
• Paget disease
• Neurofibromatosis (type 1)
• Angiomatous lesions
Polyostotic Lesions: Malignant

• Metastases
• Multiple Myeloma [Figure 4-3-33]
• Hemangioendothelioma
Osteosarcoma with aggressive “hair-

on- end” periosteal reaction (arrows)
Langerhans cell histiocytosis with areas of calvarial lysis in the Multiple myeloma on lateral skull radiograph
frontal and occipital areas (arrows) with multiple areas of bone lysis
References
1. "General Considerations". In: Bone Tumors, ed Dorfman HD, Czerniak B, Mosby: St.
Louis 1998. p. 1-33.
2. Ghelman B. Radiology of bone tumors. Orthop Clin North Am. 1989 Jul;20(3):287-
312. Review.
3. Lodwick GS, Wilson AJ, Farrell C, Virtama P, Dittrich F. Determining growth rates of
focal lesions of bone from radiographs. Radiology. 1980 Mar;134(3):577-83.
4. Madewell JE, Ragsdale BD, Sweet DE. Radiologic and pathologic analysis of solitary
bone lesions. Part I: internal margins. Radiol Clin North Am. 1981 Dec;19(4):715-48.
5. Ragsdale BD, Madewell JE, Sweet DE. Radiologic and pathologic analysis of solitary
bone lesions. Part II: periosteal reactions. Radiol Clin North Am. 1981 Dec;19(4):749-
83.
6. Sweet DE, Madewell JE, Ragsdale BD. Radiologic and pathologic analysis of solitary
bone lesions. Part III: matrix patterns. Radiol Clin North Am. 1981 Dec;19(4):785-
814.

Fundamental Concepts of Musculoskeletal
Neoplasm: CT and MRI
Mark D. Murphey, MD
Important Features in Evaluation of Musculoskeletal Masses

• Preoperative assessment and staging
Osseous Neoplasm [Figure 4-5-1]

• Differential diagnosis of primary skeletal neoplasms is best determined by
radiographs!!
But...
• MRI and/or CT are vital for delineating and staging osseous neoplasms prior to
surgery
Figure 4-5-1
Chondrosarcoma (same patient as previous radiograph) on MR due to

associated cortical destruction and soft tissue mass (arrows)
Enchondroma vs.Chondrosarcoma on
radiograph due to chondroid
mineralization (arrow)
Soft Tissue Neoplasm

• Radiographs only occasionally helpful
• CT and more often MRI can be tissue-specific
But...
• MRI and/or CT are again vital for defining extent, staging and preoperative
evaluation. Clinical and radiologic characteristic often limit differential
diagnosis
Causes of Tissue Specific Diagnosis on CT/MRI in Evaluating

Soft Tissue Masses
• 20%–50% cases
• Contrast resolution, MRI > CT
• Multiplanar imaging, MRI > CT
• Location of mass
• Growth pattern and history
Soft Tissue Masses Diagnosed with Imaging Alone

• Lipomatous lesions
• Neurogenic tumors
• Elastofibroma and fibromatosis
• PVNS and ganglion
Musculoskeletal Radiology 733 Fundamental Concepts MSK Neoplasm: CT and MRI

Figure 4-5-2
T1 T2
Lipoma (coronal T1 and T2-weighted images) isointense to fat
on all pulse sequences (*) with single thin septation (arrows)
Figure 4-5-3
Malignant peripheral nerve sheath tumor (arrow) in patient with

neurofibromatosis type 1 (note second small subcutaneous
neurofibroma- curved arrow)
Staging of Musculoskeletal Tumors: Benign (G-O) [Figure 4-5-4]

• Stage 1 – Unchanged or healing lesion; well-encapsulated; indolent clinical
course
• Stage 2 – Active growth; symptomatic, remains intracapsular but may be
deforming
• Stage 3 – Aggressive local growth; may penetrate cortex or compartment;
higher recurrence rate
Staging of Musculoskeletal Tumors: Malignant [Figure 4-5-5]

• Stage 1 (G1): Low Grade, well differentiated, few mitoses; tend to recur locally
➢ 1A – Intraosseous / Intracompartmental
➢ 1B – Extraosseous / Extracompartmental
• Stage 2 (G2) – High Grade, poorly differentiated, many mitoses; high
incidence of metastases
➢ 2A – Intraosseous / Intracompartmental
➢ 2B – Extraosseous / Extracompartmental
• Stage 3 – Metastases; regional or remote (visceral, lymphatic or osseous)
Fundamental Concepts MSK Neoplasm: CT and MRI 734 Musculoskeletal Radiology

Figure 4-5-4
Staging of Musculoskeletal Neoplasm: Histologically Benign (G-O)
STAGE Bone Soft Tissue
II
III

Figure 4-5-5
Staging of Musculoskeletal Neoplasm: Histologically Malignant
Low grade histo: IA Low grade histo: IB

Bone
Soft Tissue
High grade histo: IIA High grade histo: IIB Stage III
BONE BONE
SOFT TISSUE SOFT TISSUE

American Joint Commission Staging
Protocol for Sarcoma of Soft Tissue
• Histologic grade (G)
➢ G1 well differentiated
➢ G2 moderately well differentiated
➢ G3-4 poorly differentiated, undifferentiated
• Primary Tumor (T)
➢ T1 tumor 5cm or less in greatest dimension
➢ T2 tumor more than 5cm in greatest dimension
• Regional lymph nodes (N)
➢ N0 no regional lymph node metastasis
➢ N1 regional lymph node metastasis
• Distant metastasis (M)
➢ M0 no distant metastasis
➢ M1 distant metastasis
Staging of Musculoskeletal Neoplasm Has Figure 4-5-6

Implication on Surgical Treatment
• Intracapsular excision
• Marginal excision
• Wide excision
• Radical resection
• Amputation
Figure 4-5-7
LIMB SALVAGE PROCEDURES

AMPUTATIONS

Important Factors on Imaging for Staging Figure 4-5-8
Musculoskeletal Neoplasm
• Intramedullary extent
• Extent of soft tissue component
• Lesion matrix
• Cortical involvement
• Neurovascular involvement
• Joint involvement
Intramedullary and Soft Tissue: Extent of

Musculoskeletal Neoplasm [Figures 4-5-8 and 4-5-9]
• MRI superior to CT
➢ Superior contrast resolution
➢ Multiplanar imaging capability
• Regional Metastases – osseous/lymph node
• Can be helpful to direct biopsy Osteosarcoma with spread across physeal plate (arrows) not
➢ Always perform in consultation with orthopod seen on radiograph
➢ Done in institution of definitive procedure
• Give orthopod anatomic landmarks ! Figure 4-5-9
MRI may overestimate musculoskeletal

neoplasm extent because of surrounding
edema (reactive zone)
Musculoskeletal Neoplasm: Lesion Matrix

Evaluation
• I. Mineralized – CT > MRI
➢ A. Chondroid – rings and arcs [Figure 4-5-10]
➢ B. Osteoid – cloudlike, ivory-like
➢ C. Other calcification – phlebolith, synovial sarcoma
• II. Nonmineralized – MRI > CT
➢ A. Fluid, necrosis, hemorrhage [Figure 4-5-11]
➢ B. Fat T1 T2
➢ C. Soft tissue – nonspecific
Ewing sarcoma following chemotherapy with prominent
reactive zone (*) around the low signal intensity
pseudocapsule (arrows)
Figure 4-5-10
Figure 4-5-11
Chondrosarcoma with chondroid matrix mineralization

not seen on radiographs or MRI Aneurysmal bone cyst with fluid levels on T2-weighted
MRI reflecting cystic spaces on gross specimen

Musculoskeletal Neoplasm: Cortical Figure 4-5-12
Involvement [Figure 4-5-12]
• CT > MRI (my opinion)
• MRI=CT (literature)
• CT better spatial resolution
• Important in differential diagnosis of osseous lesions
• Important for surgical resection/staging
Musculoskeletal Neoplasm: Neurovascular

Involvement
[Figures 4-5-13 and 4-5-14]
• Vital information for surgical resection
• MRI > CT (post-contrast if use CT)
• Improved contrast resolution
• Multiplanar MR images often helpful
• Axial plane usually best
• Look for intact fat plane Osteoid osteoma (arrow) and lesion was difficult to detect on
➢ Best on T1W images MRI (right image) compared to CT (left image)
• If fat plane lost cannot exclude involvement
• Soft tissue mass encase vessels – definite involvement
Figure 4-5-13
Figure 4-5-14
Osteosarcoma with displaced but nonencased

neurovascular bundle (arrows)
Musculoskeletal Neoplasm: Ligament and

Tendon Involvement Osteosarcoma with encased neurovascular bundle (arrows)
• Important for surgical reconstruction with tumor replacing normal fat seen about vessels
• MRI > CT; best on T2W image
• Tendons/ligaments low intensity vs. tumor high signal
• On CT tendon/ligament similar to tumor attenuation
• Also multiplanar imaging of MR helpful
Figure 4-5-15
Musculoskeletal Neoplasm: Joint
Involvement
[Figure 4-5-15]
• Dramatically changes surgery from:
➢ Limb salvage; intraarticular resection
➢ Extraarticular limb salvage/amputation
• MRI superior to CT – multiplanar imaging
➢ Coronal or sagittal plane best
• Three routes of spread into joint
➢ Through bone/cartilage (transarticular)
➢ Around joint margin (periarticular)
➢ Along ligaments/tendons, or hematogenous
• Presence of joint effusion – suggestive
• Absence of joint effusion – excludes
Osteosarcoma invading the knee joint with effusion (arrows)

and tumor (*) along ACL (arrowheads) on sagittal T2 MR
and gross specimen

Overall Delineation of Musculoskeletal Masses: All Features
(56 Cases, N=189)
• MRI > CT – 60%
• MRI = CT – 16%
• CT > MRI – 24%
MRI (N=56) CT (N=56)
Intramedullary Extent 81% 5%
Soft Tissue Mass 89% 0%
Mineralized Matrix 0% 92%
Cortical Involvement 7% 72%
Neurovascular Involv. 78% 0%
Joint Involvement 73% 3%
CT Indications
• Cannot perform MRI
• Matrix producing neoplasm not adequately evaluated on radiographs
• Unusual location
➢ Ribs, sternoclavicular region, scapula
➢ Abdominal/chest wall
➢ Fibula
Imaging Characteristics Suggesting Benign Soft Tissue Mass

• Small size
• Well marginated
• Homogeneous signal intensity
• No neurovascular encasement
• Enhancement pattern dynamic MRI (late, slow, diffuse/none)
Imaging Characteristics Suggesting Malignant Soft Tissue Mass

• Large size
• Poor margin definition with edema
• Heterogeneous signal intensity
• Neurovascular encasement
• Enhancement pattern dynamic MRI (early, rapid, peripheral)
Distinction of Benign vs. Malignant Soft Tissue Mass by MRI

• Totty – Radiology 1986; 160:135–141 (N=32)
• Sundaram – MRI 1988; 6:237–248 (N=53)
• Kransdorf – AJR 1989; 153:541–547 (N=112)
• Berquist – AJR 1990; 155:1251–1255 (N=95)
• Crim – Radiology 1992; 185:581–586 (N=83)

Soft Tissue Masses Misinterpreted on MRI: Figure 4-5-16
Benign vs. Malignant [Figures 4-5-16 to 4-5-18]
• Diabetic muscle ischemia
• Hematoma
• Fibromatosis
• Reactive lymph node, abscess, bursitis
• Myositis ossificans
• Synovial sarcoma
• Myxoid liposarcoma
General consensus is that in an individual case, MRI is not accurate
enough to predict whether a nonspecific solid soft tissue mass is
benign or malignant
Musculoskeletal Neoplasm Follow-Up Myositis ossificans with aggressive

• Pre-operative-post therapy appearance on coronal T2-weighted MRI
• Post-operative-recurrence
• MRI superior to CT Figure 4-5-17
Post Therapy Imaging
• Increasing ossification – Osteosarcoma, Ewing sarcoma –
Radiographs/CT
• Change in size and extent – MRI
• Increasing peritumoral edema
• Tumor necrosis and hemorrhage
➢ > 90% required for a pathologic good response
Post-Operative Imaging: Radiographs

• Comparison to previous studies
• Findings of recurrence
➢ New bone destruction
Myositis ossificans with early rim of ossification
➢ New areas matrix formation (arrow) on CT (same patient as previous MRI)
Post-Operative Imaging: MRI/CT: Normal

• MRI > CT – improved contrast resolution
• Comparison to baseline study (first 2-3 months) Figure 4-5-18
• Recognize normal changes
➢ Post-op edema/myositis
➢ Radiation necrosis
➢ Muscle flap
➢ Fluid collections – subfascial, lymphocele/seroma
Post-Operative Imaging: MRI/CT: Abnormal

[Figures 4-5-19 and 4-5-20]
• New bone destruction/marrow replacement
• Any recurrent or residual nodular region
➢ Tumor until proven otherwise
➢ Texture sign Synovial sarcoma with homogeneous appearance and
➢ Regardless of signal characteristics unless low all defined margins suggesting an indolent lesion. Intrinsically
sequences representing fibrosis or fluid collection the lesion has nonspecific features of a solid mass
• Contrast studies can be helpful
Figure 4-5-19
Post operative lymphocoele/seroma (*)

in patient with previous MFH resection
with homogeneous low (T1) and high
(T2) signal intensity as expected for a
fluid collection

Musculoskeletal Neoplasm: Use of MRI with Figure 4-5-20
Gadolinium
[Figure 4-5-21]
• Increase lesion conspicuity (usually not needed)
• Tumor, edema, inflammation and fibrosis all
enhance
• Help differentiate cyst/hemorrhage
• Helpful in post-op cases to show nodular
enhancement with recurrence
• Vanel/Bloem - dynamic subtraction MRI early
enhancement of recurrent tumor and response to
therapy
Musculoskeletal Masses: Imaging Goals

• Delineate precise extent of lesion
• Diagnosis/exclude metastases
• Give most likely tissue type and differential Recurrent MFH (arrows) adjacent to residual
diagnosis lymphocoele/seroma (*) (same patient as previous MRI but 2
years later). Note the tumor staining on angiogram (far right
image-arrowhead) versus vessels draped about fluid collection
(curved arrow)
Figure 4-5-21
Myxoid MFH with enhancing peripheral solid nodular tissue

(arrows). These enhancing areas represent viable tumor
regions and biopsy should be directed toward these regions as
they harbor diagnostic tissue and were only detected after
contrast administration
References
1. Berquist TH. Magnetic resonance imaging of musculoskeletal neoplasms. Clin Orthop Relat Res. 1989
Jul;(244):101-18. Review.
2. Sundaram M, McGuire MH. Computed tomography or magnetic resonance for evaluating the solitary tumor or
tumor-like lesion of bone? Skeletal Radiol. 1988;17(6):393-401.
3. Enneking WF. A system of staging musculoskeletal neoplasms. Clin Orthop Relat Res. 1986 Mar;(204):9-24.
4. Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin
Orthop Relat Res. 1980 Nov-Dec;(153):106-20.
5. Stacy SG, Mahal RS, Peabody TD. Staging of Bone Tumors: A Review with Illustrative Examples. Am. J.
Roentgenol., Apr 2006; 186: 967 - 976.
6. Murphy WA Jr. Imaging bone tumors in the 1990s. Cancer. 1991 Feb 15; 67(4 Suppl):1169-76. Review.

Osteoid Lesions of Bone
Mark D. Murphey, MD
Figure 4-5-1
Osteoid Lesions of Bone
• Enostosis and associated conditions
• Osteoma
• Osteoid osteoma
• Osteoblastoma
• Osteosarcoma
Enostosis (Bone Island): Clinical

Characteristics
• Stieda 1905 - “Kompakten Knochenkerne”
• Usually incidental finding
• Patient asymptomatic
• Common lesions-unknown frequency
(0.43%–14% rib, pelvis and spine)
• Rare in children
Enostosis histologically with pink cortical bone (*) and irregular
thorn-like medullary margins (arrows)
Enostosis (Bone Island): Histology [Figure 4-5-1]
• Intramedullary location
• Composed of normal appearing compact lamellar bone with haversian Figure 4-5-2
canals
• Blends with surrounding trabecular bone creating
irregular margin
• Likely developmental - can be considered a
hamartoma
Enostosis (Bone Island): Radiology

• Round to oval (0.2–2.0 cm) osteoblastic area
• Often epiphyseal or metaphyseal
• Thorny, radiating spicules at margin but well
defined
• Vast majority (>95%) no need for further radiologic
evaluation following radiograph
• Bone scan (if needed) usually normal (may show
minimal activity) Enostosis with thorn-like margins (arrow) in the fifth
• May slowly increase or decrease in size metacarpal head
• Differential diagnosis: osteoblastic metastasis, osteoma, osteoid osteoma, low
grade osteosarcoma
• Follow-up 1, 3, 6 and 12 months Figure 4-5-3
• Biopsy if increase in size too rapidly
➢ > 25% over 6 months
Giant Bone Island [Figure 4-5-4]

• > 2-3 cm in size
• Most often in pelvis
• More likely to have increased activity (25%) on bone scan
(but usually mild; < ant. iliac crest)
• Most difficult to differentiate from sclerosing low grade
intramedullary osteosarcoma (on histology look for
entrapped host lamellar bone)
• Identical on histology to other bone islands
Enostosis with thorn-like margins (arrow)
Musculoskeletal Radiology 743 Osteoid Lesions of Bone

Possible Diseases Related to Enostosis Figure 4-5-4
• Osteopoikilosis
• Osteopathia striata
• Melorheostosis
Osteopoikilosis (Osteopathia Disseminata)

Clinical Characteristics
• Autosomal dominant inheritance; asymptomatic
• Dermatofibrosis lenticularis disseminata
• Keloid formation
• May have mild arthralgias (15%-20%)
Osteopoikilosis: Giant bone island with irregular thorn-like

Radiology and Pathology margins (*)
• Numerous circular or ovoid radiodensities
• Often symmetric - no increased activity on bone scan
Figure 4-5-5
• Predilection epiphyses and metaphyses
• Also carpus, tarsus, pelvis
• May increase or decrease in size
• Pathology - same as solitary enostosis
Osteopathia Striata (Voorhoeve Disease):

• Osteopoikilosis variant - 1924
• Autosomal dominant?
• Usually asymptomatic or mild arthralgia’s
• Associated syndromes - Goltz syndrome, cranial sclerosis
Osteopathia Striata: Radiology [Figures 4-5-5 to 4-5-7] Mixture of osteopoikilosis (circular areas of
• Linear bands of sclerosis from metaphysis in long bones sclerosis-arrow) and osteopathia striata (linear
• Fan-like bands of sclerosis in flat bones (iliac) areas of sclerosis-arrowhead)
➢ Could simulate heavy metal poisoning
• Sometimes associated with osteopoikilosis Figure 4-5-6
• Bone scan-normal
Melorheostosis: Clinical Data

• Described 1922: Leri and Joanny
• Only 50% evident before age of 20 years
• Equal sex distribution
• Often symptomatic - pain, decreased range of motion (ROM),
contractures; limb swelling/length discrepancy and bowing
• Scleroderma like skin lesions over osseous changes
Melorheostosis: Pathology
• Thickened and enlarged cortical Figure 4-5-7
bone
• Haversian canals normal with
irregular arrangement
• Marrow space may show
increased cellularity
• Soft tissue may contain mass of
fibrous tissue with or without Mixture of osteopoikilosis (arrowhead)
and osteopathia striata (arrow) showing
ossification low signal intensity on MRI
Osteopathia striata with linear bands of

sclerosis in the tibia and femur
Osteoid Lesions of Bone 744 Musculoskeletal Radiology

Melorheostosis: Radiology [Figures 4-5-8 to 4-5-10] Figure 4-5-8
• Distribution - single limb - more common lower extremity
• One or more bones–sclerotome (skeleton supplied by spinal sensory
nerves) pattern
• Osseous excrescences often exuberant and lobulated along bone
surface -“candle wax”
• Also endosteal involvement may extend into marrow space
• Can extend into soft tissue with ossification, often periarticular
• May lead to joint ankylosis
• Intense activity on bone scan Figure 4-5-10
Figure 4-5-9
Melorheostosis
involving foot
with increased
uptake on bone
Melorheostosis with mineralized
scan (same
inguinal soft tissue mass
patient as
(arrowhead)
previous two
images)
Figure 4-5-11
Melorheostosis classic “dripping candle wax”

appearance (arrow)
Osteoma: Clinical
Characteristics
• A benign, slow growing tumor, composed of osteoid tissue
• Found in cranial vault, sinuses, mandible and (rarely) long bones
• Represent protruding mass of dense periosteal intramembranous bone
on surface of host bone
• Signs/symptoms depend on size/location
• Sinus lesions may lead to sinusitis, headache, or can grow into cranial
vault
• Orbital lesion may cause exophthalmos, displacement of globe, diplopia
• 0.42% patients with sinus radiographs
Osteoma: Pathology
• Nodules of dense osseous tissue Calvarial osteoma (*)
• Differences from bone island:
➢ Often a mix of woven & lamellar bone Figure 4-5-12
➢ May/may not contain haversian system
➢ Arises from cortex rather than intramedullary
➢ Do not blend with trabecular bone
➢ Most frequent in the skull
• In craniofacial area often in spectrum of fibrosseous
lesions
Osteoma: Radiology [Figure 4-5-11 and 4-5-12]

• Sharply defined, homogeneous, bone mass arising
from surface of bone
• Most frequently diagnosed incidentally on radiographs
• Frontoethmoid sinus region - 75%
• Sphenoid 1%–4%
Gross specimen calvarial osteoma (*) on surface of outer
table (arrow) but not extending into the diploic space (DS)

Osteoma: Gardner Syndrome
• Multiple osteomas are almost always associated with Gardner syndrome
➢ Familial autosomal dominant
➢ Intestinal polyposis
➢ Multiple osteomas
➢ Dental lesions
➢ Soft tissue desmoid tumors
➢ Skin lesions
➢ Sebaceous cysts and fibromas
Osteoid Osteoma: History

• Described in 1935 by Jaffe as an osteoblastic tumor composed of osteoid and
atypical bone
• Established by Jaffe as a distinct clinical and pathologic entity
• Controversy remains as to true nature: inflammatory, traumatic, vascular, viral
Osteoid Osteoma: Clinical Characteristics

• 11% of all bone lesions that come to biopsy (3% of primary bone tumors)
• Spinal lesions commonly present with painful scoliosis, without neurologic
dysfunction
• Intraarticular lesions often present with nonspecific vague joint pain
• Young patients usually 10–25 years
• Male : Female approximately 2–3:1
• Rare in blacks
• Pain almost invariably present complaint (1.6% painless - 50% of these in the
hand)
• Pain relief with aspirin/nonsteroidals
➢ Inhibit prostaglandin E2, aggravated by ETOH
Figure 4-5-13
Osteoid Osteoma: Skeletal Distribution [Figure 4-5-13]
• Femur/tibia - 50% - 60% of lesions
• Most frequent in long bone diaphysis (70% - 80%)
• Spine - 10% (90% posterior elements; 10% vertebral body)
• Hand/foot - 10% - 20% - proximal phalanx, metacarpal, scaphoid,
navicular, calcaneus
• Epiphyseal lesions - rare
Osteoid Osteoma: Pathology

• The “nidus” is the lesion-yellowish to red pea
• Composed of osteoid and woven bone with interconnected
trabeculae
• Background and rim of highly vascularized fibrous connective tissue
• Extensive reactive bone may surround the lesion
• No malignant potential
Osteoid Osteoma: Classification

• Cortical (70% - 75%): long bone shaft; intense fusiform sclerosis; central nidus
• Cancellous (25%) intermediate frequency; usually femoral neck, hand/foot;
often limited surrounding sclerosis
• Intraarticular lesions (cortical/cancellous may have limited sclerosis/periosteal
reaction and can be distant from nidus
• Subperiosteal; rare, arises adjacent to bone; usually femoral neck, talar neck,
hand, foot; bone may show pressure erosion

Osteoid Osteoma: Radiology: Cortical Lesions Figure 4-5-14
[Figures 4-5-14 to 4-5-19]
• Dense fusiform sclerosis
• Periosteal bone solid, rarely lamilated
• Nidus usually central, rarely > 1.5 - 2cm
• “Hot” on scintigraphy - double density sign
• Nidus well-defined on CT with a smooth peripheral
margin, +/- central mineralization
Figure 4-5-15
CT of osteoid osteoma with central

focus of calcification Humeral osteoid osteoma in the cortex with bone scan showing
“double uptake sign”
Figure 4-5-16
Figure 4-5-17
MR imaging of osteoid osteoma with intermediate signal

intensity on T1 [left] and T2 [right] -weighting (arrow).
The surrounding marrow edema is prominent (*) Osteoid osteoma specimen radiograph shows central
and can obscure the nidus calcification and entire nidus within
the bivalved gross specimen
Osteoid Osteoma: MR Imaging
• Low to intermediate intensity T1-images
• Intermediate to high intensity T2-images
• Low intensity all pulse sequences if totally Figure 4-5-18
mineralized nidus
• May detect synovitis/joint effusion/soft tissue
edema that can be very prominent/confusing
imaging feature
• Reactive marrow edema may obscure lesion
• CT more helpful in majority, however, MRI may be
very helpful in difficult cases
Figure 4-5-19
Radiograph and bone scan of spinal osteoid osteoma in facet

with a sclerotic pedicle (arrow) and increased uptake on the
radionuclide bone scan (arrowhead)
CT of spinal osteoid osteoma in facet

with central calcification

Differential Diagnosis of Osseous Lesions with Sequestra-Like
Appearance
• Osteomyelitis-pyogenic
• Subacute osteomyelitis or unusual organism
• Osteoid osteoma/osteoblastoma
• Langerhans cell histiocytosis
• Metastases
• Fibrosarcoma/MFH
• Lymphoma Figure 4-5-20
Cortical Osteoid Osteoma: Differential
Diagnosis
• Brodie abscess
• Stress fracture (linear not circular)
Osteoid Osteoma Radiology:

Intraarticular/Cancellous Lesions
• Reactive osteosclerosis/periosteal reaction often
mild/absent and may be distant from lesion
• Associated joint effusion/lymphofollicular synovitis
• Regional osteoporosis-disuse
• May have associated periostitis
• May be diffusely “hot” on scintigraphy
Subtle intraarticular osteoid osteoma (arrow) with central
• Subperiosteal lesions present as juxtacortical calcification causing effusion and hip joint widening simulating
masses septic arthritis
Intraarticular Osteoid Osteoma: Figure 4-5-21

[Figures 4-5-20 and 4-5-21]
• Rheumatoid arthritis, JRA
• Tuberculous arthritis
• Nonspecific synovitis
• Septic arthritis
• Osteoblastoma (especially spine)
Osteoid Osteoma: Classic Treatment

• Surgical excision - curative with complete nidus removal - post op biopsy
radiographs
• Dramatic relief of symptoms
• Recurrence due to incomplete excision can then have multiple nidi
• Problems
➢ Locating lesion at surgery
➢ Tetracycline and radionuclide labeling
Osteoid Osteoma: Other Treatment Methods

• Medical- spontaneous healing
• Percutaneous removal
• Percutaneous ablation (radiofrequency)
• Embolization single feeding artery (potentially) ?
Osteoblastoma
• Rare benign osteoid producing tumor characterized by osteoid and woven
bone production
• Synonyms include: giant osteoid osteoma and osteogenic fibroma Subtle intraarticular osteoid osteoma
• About 1.0% of excised primary osseous tumors (arrow) with central calcification
causing effusion and hip joint widening
• Osteosarcoma 20x more common, osteoid osteoma 4x more frequent simulating septic arthritis on CT and
MR. The CT shows typical nidus with
central calcification (arrow) that is
difficult to perceive on the MR

Osteoblastoma: Clinical Features
• Patients are young, median age 18, 80% are between 10–30 years
• Males : Females ratio 2–3:1 Figure 4-5-22
• Pain is most common symptom, less severe than osteoid osteoma
• Less often at night and may or may not be relieved by aspirin
Osteoblastoma: Skeletal Distribution

• Spine, (40%) equally distributed cervical through sacrum
• About 30% occur in long bones, most commonly femur and tibia
• Skull, mandible, maxilla (15%)
• Also small bones of hand & feet (10%) and pelvis (5%)
Osteoblastoma: Pathology
• Micro: large number of osteoblasts producing trabeculae, osteoid or
bone
• Virtually indistinguishable from osteoid osteoma on high-power
histologic examination
• At times minor microscopic differences from osteoid osteoma but may
rely on radiology
Osteoblastoma: Radiology - General

• Described as having 3 radiologic patterns
➢ 1) Similar to but larger than osteoid osteoma (>2cm) Osteoblastoma of C3 spinous process
➢ 2) Expansile lytic lesion with mineralization and lamina
➢ 3) More aggressive appearance – marked bone destruction, scattered
calcification and soft tissue mass
Osteoblastoma: Radiology - Specific

• Radiologic features are not always distinctive
• Lytic lesion with varying bone production
• Cortex thinned with expanded contour, may be destroyed, and have a soft
tissue mass
• Mineralization may appear like chondroid tissue -“arcs and rings” but no Figure 4-5-23
chondroid tissue pathologically
• May have surrounding edema but less common than osteoid osteoma
• Up to 16% secondary ABC (Aneurysmal Bone Cyst) component
• Solid elements often low/intermediate signal T2 MR
Osteoblastoma: Radiology – Spine [Figures 4-5-22 and 4-5-23]

• Posterior elements alone most common (>60%)
• Posterior elements extending into vertebral body (25%)
• Vertebral body alone (<15%)
• More likely to contain ossification and soft tissue mass
• Less likely to elicit sclerosis
• Scoliosis variably present less characteristic than osteoid osteoma
Osteoid Osteoma/Osteoblastoma
Differential Diagnosis: Spine
• Osteoblastoma
➢ Size > 1.5–2.0cm
➢ Growth and soft tissue mass
➢ Matrix - multifocal - noncentral
➢ Scoliosis and classic symptoms absent
• Pedicle sclerosis - lymphoma, mets, spondylolysis, congenital CT of C3 osteoblastoma with extensive
absence/ hypoplasia posterior elements, malaligned apophyseal joints, mineralization
unusual infection

Osteoblastoma Radiology – Long Bones Figure 4-5-24
[Figure 4-5-24]
• Usually eccentric, metaphyseal (25%)
or diaphyseal (75%)
• Intramedullary or cortical, rarely subperiosteal
• Solid periostitis (50%)
• May appear as a blister lesion in hand or foot, may
also have osteoporosis
• Correct diagnosis usually not suggested
prospectively
Osteoblastoma: Natural History

• The lesion usually grows slowly
• Treatment is curettage or excision
• Recurrence rate is 10%–15%
Aggressive “Malignant” Osteoblastoma Osteoblastoma of proximal femur with calcification on CT

• Initially described 1967- Mayer
• Considerable controversy and definition not clearly established
• Problems in distinction from osteoblastoma like osteosarcoma and rare reports
of osteoblastoma recurring as osteosarcoma
Figure 4-5-25
Aggressive Osteoblastoma: Pathology
• Similar to conventional osteoblastoma
• Wider more irregular trabeculae
• Lace-like osteoid
• Variable mitoses but no atypical figures
• Epithelioid osteoblast
Aggressive Osteoblastoma:
Clinical and Radiologic Characteristics
[Figure 4-5-25]
• Older patients average 33 years of age
• Similar locations
• Larger more aggressive on radiologic examination
with soft tissue mass Aggressive osteoblastoma with large soft tissue mass (arrow)
• Local recurrence rate up to 50% and multilevel involvement
• Usually no metastases
Osteosarcoma: Definitions
• A mesenchymal malignancy that differentiates to produce osteoid
• “...If only 1% of a tumor manifests osteoid and/or bone production by
malignant cells, it is by general convention an osteosarcoma”
• “No matter how meager the osseous component”
• Only true for intraosseous neoplasm
Mirra “Bone Tumors”. Lee & Febiger 1989
Osteosarcoma is the second most common primary malignant

bone tumor - 15% of all biopsied primary bone tumors
Osteosarcoma (OS): Additional Definitions

• Primary OS: lesion in absence of a benign precursor lesion or treatment
• Secondary OS: lesion that has a benign precursor or is metastatic from
primary OS
• Synchronous OS: lesions discovered within 6 months of each other
• Metachronous OS: lesions discovered more than 6 months apart
Osteosarcoma: Pathology-General
• Osteoid and/or immature bone production by tumor cells
• Malignant stromal cells graded on their degree of anaplasia I-IV

Osteosarcoma: Histologic Pattern Figure 4-5-26
• Types
Osteoblastic (mixed and sclerosing) 82%
Fibroblastic
(Fibrosarcoma and MFH like) 7%
Chondroblastic 5%
Telangiectatic 3%
Small Cell 1%
Other 2%
• Symptoms usually pain and swelling
Primary Osteosarcoma: Classification

• Intramedullary-high grade 75%
• Juxtacortical 7%-10%
• Gnathic 6%
• Low grade sclerosing 4% - 5%
• Soft tissue 4%
• Osteosarcomatosis - multifocal 1% - 2%
• Intracortical 0.2%
Intramedullary osteosarcoma with osteoid matrix (arrows) and
Secondary Osteosarcoma: Classification aggressive bone lysis (arrowheads)
• Paget disease (67% - 90%)
• Radiation induced (6% - 22%) Figure 4-5-27
• Osteonecrosis
• Others - fibrous dysplasia, prosthesis, osteogenesis imperfecta, chronic
osteomyelitis, retinoblastoma
• About half of osteosarcoma over age 50 are secondary, 67% over age 60
Osteosarcoma: Primary, High-Grade, Intramedullary

• About 75% of all osteosarcomas
• Most patients are between 15 and 25 years, rare younger than 6 or older than
60 years
• Male > Females 1.5–2:1
• About 70% are in the long bones, more than 50% about the bones of the knee
• 90% are metaphyseal, 5%–10% diaphyseal
Osteosarcoma Intramedullary: Radiology

[Figures 4-5-26 to 4-5-29]
• Usually mixed sclerosis and lysis
• Soft tissue mass (80%) Bone scan of intramedullary osteosarcoma of
• Periosteal reaction (80%) - Codman triangle, lamination, tibia with intense radionuclide uptake. The
femur and ankle also show increased uptake
perpendicular (“sunburst”, “hair on end”) (arrowheads) from hypermia and disuse
• Osteoid matrix “fluffy”/ “cloud-like” (90%)
• Extend across epiphyseal plate Figure 4-5-28
(75% - 90%)
• MRI/CT essential for staging and preoperative
planning
Osteosarcoma Telangiectatic
• Tumor largely composed of cystic cavities containing
necrosis and hemorrhage ( > 90%)
• ABC like – misdiagnosed on radiographs
• Distribution similar to other intramedullary
osteosarcomas
➢ Femur, tibia, humerus
➢ Metaphyseal (90%), diaphyseal (10%)
Coronal STIR MR image and gross specimen of tibial

intramedullary osteosarcoma show identical lesion extent (*)

Osteosarcoma Telangiectatic Figure 4-5-29
[Figures 4-5-30 to 4-5-33]
• Largely osteolytic and expansile
• Look for small areas of osteoid (58% X-rays, 85%
CT)
• Fluid-fluid levels (CT 48% / MRI 89%)
• Pathologic fracture (25% - 61%)
• Donut sign on bone scan (65%)
• Previously worse prognosis, now may actually be
better than other intramedullary osteosarcomas
(68% 5 year survival)
Juxtacortical Osteosarcoma
• Parosteal (65%)
• Periosteal (25%)
• High grade surface (10%)
• Prognosis varies with grade and extent
Intramedullary osteosarcoma with skip metastases (*) on
coronal STIR MR image and gross specimen with distal
primary lesion (arrow) and intervening normal marrow (M)
Telangiectatic osteosarcoma of
scapula with thick peripheral
mineralization (arrow) Telangiectatic osteosarcoma of scapula with thick enhancing
nodular wall containing calcification (arrowheads) and central
hemorrhage/necrosis (*) on CT
Figure 4-5-33
Figure 4-5-32
Telangiectatic osteosarcoma of distal femur on sagittal T1-

weighted post-contrast MR image and gross specimen showing
Gross specimen of scapular telangiectatic
thick nodular enhancement peripherally in viable tumor
osteosarcoma with cystic spaces (*) surrounded
(arrowheads) correlating to gross specimen with central
by solid viable tissue (arrowheads)
nonenhancing cystic/necrotic regions (*)

Parosteal Osteosarcoma: Clinical and Pathology Figure 4-5-34
• Arise from outer layer of periosteum
• Usually a low grade tumor – fibroblastic stroma and
streamers of woven bone
• Patients about a decade older than intramedullary
osteosarcoma; F>M
• Location - femur (65%), humerus (15%), tibia (10%),
fibula (3%), forearm (3%)
• Most common posterior distal femur metaphysis
Parosteal Osteosarcoma: Radiology

[Figures 4-5-34 to 4-5-37]
• Initially an exophytic sclerotic mass
• Cauliflower-like with lucent cleavage plane between
lesion and cortex
• Radiodense centrally with growth may reattach to cortex
and obliterate cleavage plane
• MRI/CT to evaluate intramedullary invasion important for
surgical resection
• Long term survival 80%–90% Parosteal osteosarcoma with specimen radiograph
and gross specimen showing surface mass with
central dense stalk of attachment to the cortex (*)
Periosteal Osteosarcoma: Clinical and Pathology and lucent cleavage plane (arrows)
• Periosteal sarcoma is usually chondroblastic (>90% of
tumor) and intermediate grade
• Arise from inner layer of periosteum
• More than 85% are in the diaphysis of the femur and tibia; ulna and humerus
(10%)
• Similar age to conventional osteosarcoma and sex distribution (M>F)
• Better prognosis but 15% rate of metastasis
Figure 4-5-.37
Parosteal osteosarcoma of the

distal femoral metaphysis with
radiograph, CT and gross
specimen and specimen
radiograph showing surface
mass (*), lucent cleavage plane
(arrows) and medullary
backgrowth (arrowhead)
Parosteal osteosarcoma of the distal femoral metaphysis with

radiograph, CT and gross specimen and specimen radiograph
showing surface mass (*), lucent cleavage plane (arrows) and
medullary backgrowth (arrowhead)

Periosteal Osteosarcoma: Radiology Figure 4-5-38
[Figure 4-5-38]
• Saucerized cortex with chondroblastic soft tissue
mass on imaging in area of erosion
• Cortical thickening at margins of erosion (40%)
• May have Codman triangle
• Spiculated periosteal reaction
• Only rarely intramedullary invasion reported
Osteosarcoma: High - Grade Surface

[Figures 4-5-39 and 4-5-40]
• Histology high-grade similar to a conventional
osteosarcoma as is prognosis with same potential for
metastasis
• Sites – femur (45%), humerus (26%), fibula (10%),
ulna (6%)
• Radiologic changes: broad based lesion with osteoid Periosteal osteosarcoma or radiograph and coronal CT
arising on osseous surface reconstruction with diaphyseal broad based soft tissue
• Similar appearance to periosteal osteosarcoma but mass causing erosion of uderlying thickened cortex and
often more aggressive “hair-on-end” periosteal rection
High-grade surface osteosarcoma

High-grade surface osteosarcoma (*) on sagittal T1-weighted
MR and sagittally sectioned gross specimen. Note the surface
Gnathic Osteosarcoma [Figure 4-5-41] location and sparing of the medullary canal (M)
• About 6% of all osteosarcomas
• Patients are usually older
• Lesions are usually lower grade
• About half are chondroblastic Figure 4-5-41
• Prognosis is better – don’t tend to metastasize but
locally invasion
Osteosarcoma: Low Grade Intramedullary

• Described in 1977 by Unni (27 cases)
• Also called sclerosing osteosarcoma – being recognized with
increasing frequency
• Patients average about a decade older than conventional
osteosarcoma
• Most patients present with pain (85%), or swelling (15%), 5%
are incidental findings
• Four histologic patterns simulating FD (50%), NOF (25%),
chondroblastoma (15%), CMF (10%) Gnathic osteosarcoma with an aggressive
mixed lytic and sclerotic (arrows) lesion
destroying the alveolar ridge (arrowhead)

Osteosarcoma: Low Grade Intramedullary Figure 4-5-42
[Figures 4-5-42 and 4-5-43]
• Not uncommonly metaepiphyseal
• Location same as intramedullary conventional
• Central sclerosis with expansile remodeling
• Ground glass density and trabeculation within
• Not as aggressive appearance on radiographs
and lack soft tissue mass
➢ ISS 2003 (Skel Rad 2004, 33:373-379)
MR/CT all 17 cases had a soft tissue mass
• Better prognosis with 10% or less metastatic rate
Osteosarcoma: Soft Tissue

(Extraskeletal)
• Rare – same histology
• Middle aged to older patients (mean 55 years)
• Location-deep soft tissues of extremities-thighs
and shoulders also retroperitoneum
• Not uncommonly history of trauma (10%–15%)
Low-grade intramedullary osteosarcoma
• Relationship to myositis ossificans? simulating fibrous dyplasia on radiograph and CT.
• Radiology – soft tissue mass with calcification or Note soft tissue mass medially (*)
ossification
Osteosarcomatosis: Figure 4-5-43

Multifocal Osteosarcoma [Figures 4-5-44 and 4-5-45]
• Rare
• Classified into types in 1969 by Amstutz:
➢ 1-Synchronous, young patients < 18 years of age
➢ 2-Synchronous, adults
➢ 3-Metachronous
• Type 3 likely represents metastatic disease
• Types 1 and 2 usually demonstrate a radiologically dominant lesion
Amstutz. Cancer 1969;24:923
Low-grade intramedullary
osteosarcoma simulating fibrous
dyplasia on radiograph and CT.
Note soft tissue mass medially (*)
Figure 4-5-44
Figure 4-5-45
Osteosarcomatosis with multifocal areas of metaohyseal

sclerosis (*)
with primary dominant sclerotic focus in the tibia ( *)
[Figure 4-5-45]

Osteosarcoma : Intracortical Figure 4-5-46
[Figure 4-5-46]
• Very rare, only a handful of cases
• Almost all in the femur and tibia Intracortical osteosarcoma with matrix
diaphysis mineralization and location within the
• The lesion is dominantly or exclusively cortex (arrow) on radiograph and CT
intracortical with no
(or only minimal) intramedullary
involvement
• Usually lytic/surrounding sclerosis
Osteosarcoma: Treatment and

Prognosis
• Complete removal of the primary tumor
– limb salvage
• Preoperative chemotherapy – look for >
90% tumor necrosis – most important
predictor of prognosis (90% 5 year
survival; 14% <90% necrosis)
• Post operative chemotherapy
• Overall 5 year survival 41%–64%
• 5 year survival 60%-70% no metastases
at presentation and surgical resection
References
1. Greenspan A, Stadalnik RC.. Bone island: scintigraphic findings and their clinical application. Can Assoc Radiol J. 1995
Oct;46(5):368-79.
2. Judkiewicz AM, Murphey MD, Resnik CS, Newberg AH, Temple HT, Smith WS. Advanced imaging of melorheostosis
with emphasis on MRI. Skeletal Radiol. 2001 Aug;30(8):447-53.
3. Klein MH, Shankman S. Osteoid osteoma: radiologic and pathologic correlation.
Skeletal Radiol. 1992;21(1):23-31. Review.
4. Kroon HM, Schurmans J. Osteoblastoma: clinical and radiologic findings in 98 new cases. Radiology. 1990
Jun;175(3):783-90.
5. Murphey MD, Robbin MR, McRae GA, Flemming DJ, Temple HT, Kransdorf MJ. The many faces of osteosarcoma.
Radiographics. 1997 Sep-Oct;17(5):1205-31.
6. Sundaram M, Falbo S, McDonald D, Janney C. Surface osteomas of the appendicular skeleton. AJR Am J Roentgenol.
1996 Dec;167(6):1529-33.

Cartilaginous Lesions of Bone
Mark D. Murphey, MD
Figure 4-6-1
Cartilaginous Lesions
• Osteochondroma
• Enchondroma
• Juxtacortical chondroma
• Chondromyxoid fibroma (CMF)
• Chondroblastoma
• Chondrosarcoma
Osteochondroma
• The most common benign neoplasm of bone that
leads to biopsy
Osteochondroma: Types Osteochondromas with marrow (*) and cortical (arrows)

• Solitary osteocartilaginous exostosis continuity on radiography and histology. Note undertubulation
• Hereditary multiple exostoses (HME) on macrosection in patent with hereditary multiple exostoses
➢ Diaphyseal aclasis (HME) with other small lesions identified by
➢ Multiple osteochondromas hyaline cartilage caps (arrowheads)
➢ Osteochondromatosis
Figure 4-6-2
Osteochondroma: Radiographic Subtypes
• Pedunculated
• Sessile
Osteochondroma: Clinical Data

• No sex predilection
• Young patients - 75% < 20 years old
• Present as a mass: responsible for symptoms
• Mechanical, cosmetic, affect on adjacent
structures (tendon, muscles, nerve, vessel),
fracture
• Location: femur (30%), tibia (20%), humerus
(20%), hand and foot (10%), pelvis (5%), scapula
(4%)
• Symptoms dependent on size/location
➢ Bursa formation Sessile and pedunculated osteochondromas with marrow (*)
and cortical (arrows) continuity
• Malignant transformation
➢ Solitary < 1%
• Treatment-Individualized-Resection Figure 4-6-3
➢ Dependent on symptoms/size/location
Osteochondroma: Pathology
• Medullary and cortical continuity with underlying
bone
• Hyaline cartilage cap
• Cartilage cap involutes after growth (skeletal
maturity)
• Only benign skeletal neoplasm associated with
radiation
• Can be induced by implanting epiphyseal tissue
• Traumatic osteochondroma
Pelvic osteochondroma revealing only sclerosis on radiograph

with cortical and medullary continuity revealed on CT (arrow)
and gross specimen (*)
Musculoskeletal Radiology 757 Cartilaginous Lesions of Bone

Osteochondroma: Imaging [Figures 4-6-1 to 4-6-3] Figure 4-6-4
• Mature bone-cortex and marrow
• Hyaline cartilage cap-calcification
• Cortical and marrow continuity with underlying bone
• Long bones – radiographs to diagnose
➢ Metaphyseal
➢ Grows away from epiphysis
➢ May be associated with failure of tubulation (particularly
HME)
• Flat bones – often need CT/MR to diagnose
➢ Tend to be larger and sessile Osteochondroma on CT with marrow and
cortical continuity
➢ More variable appearance
Figure 4-6-5
Osteochondroma
• The cartilage cap deserves the most consideration in
radiologic interpretation
Osteochondroma: Cartilage Cap

[Figures 4-6-4 to 4-6-9]
• Radiographs - chondroid calcification
➢ Increasing destruction or change in appearance
➢ Worrisome for malignancy
• Ultrasound - good for cap and bursae
• CT - soft tissue with calcification
➢ Can be difficult to distinguish from muscle
• BS - increased uptake Osteochondroma on radiograph and sagittal T1-
weighted and axial T2-weighted MR images with thin
• MRI - Intermediate signal T1W images cartilage cap showing high signal on long TR MR
➢ High signal T2W images (arrowhead)
• Cap thickness - benign vs malignant
➢ Benign < 1.5 cm (0.1- 3.0 cm; ave. 0.6-0.9 cm)
Figure 4-6-8
➢ Malignant > 1.5 cm (1.5 -12 cm; ave. 6 cm)
➢ Depends on skeletal maturity
Figure 4-6-6
Gross specimen and macrosection of resected Axial T1-weighted MR images show cortical and marrow
osteochondroma with thin bluish cartilage cap (*) correlating ((arrowheads) continuity and thick cartilage cap (*) (15 year old
with the imaging (same patient as 4-6-5) boy; same patient as previous radiographs)
12 years old 15 years old

Lateral ankle radiographs at 3 year interval shows growth Sagittal STIR MR and gross specimen of resected
osteochondroma with high signal, thick cartilage cap (15 year
of the osteochondroma in this 12 year old boy. The same
old boy with thick cartilage cap simulating malignancy but only
radiographic appearance would represent malignant represented growth due to young age; same patient as
transformation in an adult previous radiographs and axial T1-weighted MR)
Cartilaginous Lesions of Bone 758 Musculoskeletal Radiology

Subungual Exostosis: Dupuytren Exostosis Figure 4-6-11
[Figure 4-6-10]
• Osteochondroma variant
• Females > Males (2:1)
• Often painful and associated with trauma and infection
• Great toe (77%-80%); Fingers (10%-14%)
• Fibrocartilage cap
• Located away from physis
Figure 4-6-10
Dysplasia epiphysealis hemimelica

(Trevor disease) with early genu varus
Subungual exostosis (arrow) deformity caused by the epiphyseal
with clinical photograph osteochondroma (arrow)
Dysplasia Epiphysealis Figure 4-6-12

Hemimelica: Trevor Disease
[Figures 4-6-11 to 4-6-13]
• Male predominance (3:1)
• Rare
• Swelling, pain and deformity
• Usually lower extremity, unilateral
• 65% multiple bone involvement talus, distal femur,
tibia
• Ankle and knee most common
• Medial joint 2x lateral
• Lobular epiphyseal mass
• Histologically identical to an osteochondroma
• May produce deformity and secondary
osteoarthritis
Figure 4-6-13 Dysplasia epiphysealis hemimelica (Trevor disease) with the

epiphyseal osteochondroma (*) arising from the posterior
femur on lateral radiograph, and sagittal T2-weighted MR and
coronal 3D CT reconstruction
Dysplasia epiphysealis hemimelica (Trevor

disease) with the epiphyseal osteochondroma
(*) arising from the posterior femur on lateral
radiograph, sagittal T2-weighted MR and
coronal 3D CT reconstruction
(same patient as 4-6-12)

Hereditary Multiple Exostoses: Clinical Data Figure 4-6-14
[Figures 4-6-14 to 4-6-16]
• Male predominance (3:1)
• Autosomal dominant inheritance
• Variability in size and number
• Any portion of the skeleton preformed in cartilage may be
involved
• Present in childhood
• May be bilaterally symmetric
• One side may predominate
• Increased incidence of malignant transformation
(10%–20%)
• Newer literature 3%–5% Hereditary multiple exostoses (HME) with associated
undertubulation of bone (Erlenmeyer flask deformity)
Figure 4-6-15
Figure 4-6-16
HME with typical pelvic and proximal femoral deformity
Enchondroma: The most common tumor

encountered in the phalanx
Enchondroma: Types
• Solitary enchondroma
• Multiple enchondromatosis
• Ollier disease
• Maffucci syndrome
Enchondroma: Clinical Data HME with typical pelvic and proximal femoral deformity. Bone
• 3%–5% all biopsied primary bone lesions; 1% all scan shows a left pelvic lesion to reveal more intense
bone tumors radionuclide uptake(*) and this area demonstartes a very thick
hyaline cartilage cap (>3 cm) and soft tissue mass with
• No sex predilection chondroid mineralization (rings and arcs) on radiograph, CT
• Peak incidence 3rd decade (10–30 years old) and T1/T2 weighted MR images (arrows) resulting from
• Hands and feet (40%–65%), long tubular bones malignant transformation to chondrosarcoma. This is also
(25%) shown on the gross specimen
• Phalanges and metacarpals most common locations
• May be incidental finding or present with pathologic fracture
Enchondroma: Pathology
• Rests of hyaline cartilage
• Hyaline cartilage often with myxoid areas
• Variable amorphous calcification and enchondral ossification
• May cause expansile remodeling and cortical thinning

Enchondroma: Imaging [Figures 4-6-17 to 4-6-19] Figure 4-6-17
• Geographic lytic lesion IA-IB
• Central often metaphyseal
• Expansile remodeling with prominent thinned
cortex (short tubular bones only)
• Chondroid matrix in majority – 17% limited or none
(radiographs); all by CT
• MRI – Lobulated margin
➢ Marked increase intensity T2W images
➢ Calcified chondroid-low intensity
Figure 4-6-18
Enchondroma of the phalanx with typical ring and arc

chondroid mineralization and deep endosteal scalloping
Figure 4-6-19
Enchondroma of tibia on coronal T1-weighted (left) and

T2-weighted (right) MR images. Note lobular margin
and no endosteal scalloping and high signal on long TR
image resulting from high water content of
nonmineralized hyaline cartilage
Multiple Enchondromatosis: Clinical Data

• Variable severity
• May be predominantly unilateral
(Ollier disease-1899)
• May become stable at puberty
• Increase malignant transformation to
chondrosarcoma (5%–50%)
• Marked skeletal deformity
Enchondroma of the distal femur on radiograph, sagittal T1-
• Not hereditary weighted and T2-weighted MR images and histology. Note
• Mild male predilection lobular margin (arrows), ring and arc mineralization
• Presents in childhood (arrowhead), no endosteal scalloping and high signal on long
TR image resulting from high water content of hyaline cartilage
Maffucci Syndrome
• Described in 1881 by Maffucci as enchondromatosis with hemangiomas
Figure 4-6-20
Maffucci Syndrome: Clinical Data

• Multiple enchondromas
• Cavernous hemangiomas
• Very rare; nonhereditary
• Mild male predilection
• Hands and feet greatest involvement
• Complications of hemangiomas
➢ Chondrosarcoma (15%–20%)
➢ Vascular sarcoma (3%–5%)
➢ Ovarian malignancy
➢ Glioma and carcinoma
Enchondromatosis on radiograph and coronal T2* (GRE)

MR image which show diagnostic columns of cartilage
extending into metaphysis from epiphyseal plate (arrows)

Maffucci and Ollier Syndromes: Imaging Characteristics Figure 4-6-21
[Figures 4-6-20 and 4-6-21]
• Lesions seen in infancy
• Lytic columns from epiphyseal plate extending into
the metaphysis
• Later typical enchondromata
➢ Geographic IA-IB margin with expansion
• Chondroid matrix calcification
• Growth disturbance and bowing
• Enchondroma growth slows after plate closure
• Soft tissue masses with phleboliths
• Chondrosarcomatous transformation
➢ New bone destruction with ST mass
➢ New periosteal reaction
➢ Disorganized or destroyed matrix calcification
Juxtacortical Chondroma
• Arise adjacent to cortex beneath periosteum
• Metaphyseal
• Proximal humerus (50%), femur and tibia also
hands and feet (25%)
• < 30 years old, M > F (2:1)
• Often more cellular than enchondroma
Juxtacortical Chondroma: Imaging

[Figure 4-6-22]
• Cortical saucerization (1–3 cm)
• Variable sclerosis/periosteal reaction
• Soft tissue mass with chondroid calcification
(50%) Enchondromatosis on bone scan and extensive deformity of
• High intensity T2W MR images the upper extremity on radiograph. Note predominance on one
• Difficult to differentiate chondrosarcoma side of the body on bone scan. MR, CT and gross specimen
reveal malignant transformation to chondrosarcoma with small
associated soft tissue mass laterally (arrows)
Figure 4-6-22
Juxtacortical chondroma with extrinsic erosion or

saucerization (arrowheads) of metacarpal
Chondromyxoid Fibroma (CMF): Fibromyxoid Chondroma

• Rare, least common cartilage tumor
• Usually lower extremity
➢ 55% around knee, 20%–25% in foot
• Young adults, 60% < 30 years old
• Rarely malignant transformation

CMF: Pathology Figure 4-6-23
• Myxoid, fibrous and chondroid tissue in various
proportions
• Myxoid areas-central in lobules
• Cellular areas peripheral in lobules
• Foci of calcification 5%–27%
• Lobulated tumor mass
CMF: Radiology [Figure 4-6-23]

• Geographic lytic lesion IA -IC
• Eccentric metaphyseal location; often cortical (long
bone lesions)
• Expansile remodeling simulate cortical permeation
• Flat/short tubular bone lesions central
• Rare matrix mineralization (CT/tomography)
• MRI – similar to slightly lower intensity than muscle
T1W images Chondromyxoid fibroma with intracortical location in the
tibia and outer margin appearing aggressive on
➢ Very high signal intensity T2W images radiograph (arrowhead) but intact on CT (arrow) and low
attenuation (*) resulting from high water content hyaline
Chondroblastoma: Codman Tumor - History cartilage (note the lack of matrix mineralization)
• Cartilage containing GCT
➢ Kolodney 1927
• Calcifying GCT Figure 4-6-24
➢ Ewing 1928
• Epiphyseal chondromatous GCT
➢ Codman 1931
Chondroblastoma: Clinical Data

• Uncommon; 1%–2% all bone tumors
• Male > Female (2:1)
• Children and young adults; 90% between ages 5
and 25 years
Chondroblastoma: Location
• Epiphysis/apophysis only 40%
• Epiphysis and metaphysis 55%
• Metaphysis only 4%
Epiphyseal/Apophyseal Lesions: Chondroblastoma centered in the epiphysis but extending into

the metaphysis, matrix mineralization and periosteal reaction
Differential Diagnosis extending into the diaphysis (arrowhead) is also seen
• Chondroblastoma
• GCT
Figure 4-6-25
• Subchondral cyst/intraosseous ganglion
• Infection
• Clear cell chondrosarcoma
Chondroblastoma: Location
• Proximal femur 23%
➢ Head and neck 16%
➢ Trochanter 7%
• Distal femur 20%
• Proximal tibia 17%
• Proximal humerus 17%
• Hands and feet 10%
Chondroblastoma on coronal T1-weighted and T2-weighted

MR image with the lesion showing low to intermediate signal
intensity on long TR image (arrowheads) and extensive
surrounding marrow edema (*)
(same patient as previous image)

Chondroblastoma: Histology Figure 4-6-26
• Chondroblasts - can be confused with
chondro/osteosarcoma
• Multinucleated giant cells
• Chondroid 1% - 15%
• ABC component 5% - 15%
• Initially cellular; later necrosis, fibrosis, maturation
Chondroblastoma: Imaging
[Figures 4-6-24 to 4-6-28]
• Geographic lytic lesion IA / IB
• Eccentric > central; rarely expansile
• Calcified chondroid matrix 30% - 50%
• Periosteal reaction 30% - 50%
➢ Adjacent diaphysis/metaphysis Chondroblastoma of greater trochanter (apophysis =
• CT/MRI-fluid/fluid levels epiphyseal equivalent) on coronal T2-weighted MR image
• MRI – not typical chondroid characteristics with typical low signal intensity lesion (arrow)
➢ BEWARE!! - low/intermediate T2W ( 95%) and surrounding edema (*)
➢ Extensive surrounding edema
➢ Joint effusion (30% - 50%)
Figure 4-6-27
Chondroblastoma: Treatment
• Curettage and cryosurgery or en bloc resection
and bone graft
• Radiofrequency ablation
• Local recurrence 5%-10%
• Malignant chondroblastoma very rare
Chondrosarcoma Types: Primary

• Intramedullary
• Periosteal/juxtacortical
• Clear Cell
• Mesenchymal
• Myxoid
• Extraskeletal
• Dedifferentiated
Chondrosarcoma Types: Secondary

• Enchondroma
• Osteochondroma
• Paget Disease
• Radiation induced
• Miscellaneous
Figure 4-6-28
Chondroblastoma with ABC component in the patella
presenting as a pathologic fracture. Radiograph and sagitally
sectioned gross specimen and macrosection show the lytic
expansile lesion with fracture, largely composed of cystic areas
(*) and small solid component of chondroblastoma inferiorly
(arrow)
Chondroblastoma with ABC component in the greater

trochanter. Multiple MR image show the lesion largely
composed of cystic areas (*) and small solid component of
chondrobstoma medially (arrows)

Chondrosarcoma: Pathology Figure 4-6-29
• Malignant tumor of cartilage often with myxoid
changes
• Grades I (30%), II (40%), III (30%)
• Grade I difficult to differentiate from benign
• Diagnosis based on histologic and growth
features, symptoms and tumor size/location
Intramedullary Chondrosarcoma:
Clinical Data
• Symptoms — pain (95%-99%) and mass (82%)
• Male > Female (3:2)
• Average age 40–45 years; metaphysis
• Location — femur (25%), pelvis (30%), shoulder
(15%), ribs/sternum (10%), vertebrae (7%),
scapula (5%)
• 8%-17% all biopsied primary bone tumors Low grade chondrosarcoma of the humerus with typical
features on multiple imaging modalities. Radiograph shows
typical ring and arc mineralization of a chondroid lesion (white
Intramedullary Chondrosarcoma: Imaging arrows) with deep endosteal scallop (black arrow).
[Figures 4-6-29 to 4-6-37] Bone scan reveals marked increased radionuclide uptake
• Geographic IA -IC to permeative
➢ Often predominantly sclerotic
• Deep endosteal scalloping
Figure 4-6-30
• Cortical thickening/periosteal reaction
• Expansile remodeling
• Soft tissue mass (20% - 76%)
• Chondroid matrix (78% by X-ray; 94% by CT)
• CT/conventional tomography if matrix subtle
• MRI - similar to muscle T1W images
➢ Lobulated high intensity T2W images
➢ Matrix calcification low intensity
➢ Peripheral/Septal contrast enhancement
Low grade chondrosarcoma of the humerus with typical features

on multiple imaging modalities. CT and axial MR images show
Figure 4-6-31 matrix mineralization on the CT (white arrows) and typical septal
and peripheral enhancement of cartilage lesions on the post
contrast MR (white arrows)
(same patient as previous and next images)
Figure 4-6-32
Low grade chondrosarcoma of the humerus with typical

features on multiple imaging modalities. Coronal T2-weighted
MR and coronally sectioned gross specimen reveal marrow
replacement (large white arrows) and deep scalloping with
early extension into the soft tissues (small white arrows)
Intramedullary
chondrosarcoma of femur with
chondroid mineralization
superiorly (arrow) and deep
area of scalloping laterally
(arrowhead)

Axial proton density MR images show cortical

breakthrough and soft tissue mass (arrowheads) in this
femoral intramedullary chondrosarcoma
(same patient as previous radiograph)
Acetabular intramedullary
Figure 4-6-35 chondrosarcoma shows subtle bone
destruction (arrow) and matrix
mineralization in this
complex area of anatomy
Figure 4-6-36
CT of acetabular intramedullary chondrosarcoma shows

matrix mineralization (arrowhead)
and large associated soft tissue mass (*)
Figure 4-6-37
Coronal T2-weighted MR image of acetabular intramedullary
chondrosarcoma shows large high signal intensity mass (*)
(same patient as previous CT)
Intramedullary chondrosarcoma of anterior rib on CT with low

attenuation mass and matrix mineralization (arrow)

Juxtacortical/Periosteal: Chondrosarcoma Figure 4-6-38
[Figures 4-6-38 and 4-6-39]
• Similar to juxtacortical chondroma
• Periosteal lesion – cortical erosion
• Similar in appearance to periosteal OGS (but no
hair on end periosteal reaction)
• Chondroid matrix calcification
• Larger size than juxtacortical chondroma
(>3–4cm)
• Intramedullary canal spared
Clear Cell Chondrosarcoma: Clinical Data

• 2% of chondrosarcomas
• Slightly younger age
• 75%–80% lesions proximal femur or humerus
• Flat bones involved – 10%
• Propensity for epiphysis, > 90%
• Better prognosis Juxtacortical chondrosarcoma seen on multiple imaging
modalities. Radiograph, CT and axial T1-weighted MR show
Clear Cell Chondrosarcoma: Pathology the juxtacortical mass (M) with chondroid matrix mineralization
(rings and arcs). There is extrinsic erosion of the fibular cortex
• Clear cell chondrocytes
(black arrows on radiograph/CT and curved arrow on MR) and
• Osteoblastoma like osseous metaplasia low attenuation of the nonmineralized components on CT (*)
• Areas of conventional chondrosarcoma – 50%
• Chondroblastoma like areas
• Osteoclastic giant cells Figure 4-6-39
Clear Cell Chondrosarcoma: Imaging

[Figures 4-6-40 and 4-6-41]
• Geographic lysis IA to IC
• Totally lytic (50%); Calcified chondroid matrix 33%
• Rind of sclerosis (20%) simulates nonaggressive
lesion
• Soft-tissue mass less common – 10%
• MRI – often high signal T2W images but may have
areas of low signal as well
Figure 4-6-40 Juxtacortical chondrosarcoma seen on multiple imaging

modalities. Sagittal T2-weighted MR shows high signal
intensity of the mass (arrows and black M). The coronally
sectioned gross specimen reveals the lobular chondroid growth
(C), extrinsic erosion of the cortex (arrows), Normal marrow
space (white M) and the periosteal elevation (P)
(same patient as figure 4-6-38)
Figure 4-6-41
Clear cell chondrosarcoma of humerus extending to

subchondral region simulating a giant cell tumor
Clear cell chondrosarcoma with high signal intensity (unlike

giant cell tumor) on coronal STIR MR images

Mesenchymal Chondrosarcoma: Clinical Data Figure 4-6-42
• Less than 10% chondrosarcoma’s
• Younger age – averages 25 years
• Males = Females
• Osseous location – femur (15%), ribs (15%), spine
(10%), craniofacial (20%), pelvis (10%)
Extraskeletal Chondrosarcoma:
Mesenchymal Type
• Young patients 23 – 44 years
• 20%–30% soft tissue
• Location – meninges and thigh
• High grade malignancy
• Mesenchymal cells with islands of cartilage
• Large soft tissue masses – may have chondroid Mesenchymal chondrosarcoma showing very aggressive bone
matrix calcification destruction with large soft tissue mass and chondroid matrix
• Metastases – lymph node, lung mineralization (arrows) on radiograph and CT
Figure 4-6-43
Mesenchymal Chondrosarcoma: Pathology
• Undifferentiated mesenchymal cells
• Multifocal islands of malignant cartilage
• Hemangiopericytoma like areas
• Aggressive high grade lesions
• Poor prognosis
Mesenchymal Chondrosarcoma: Imaging

[Figures 4-6-42 and 4-6-43]
• Aggressive osseous destruction motheaten to
permeative
• Chondroid matrix calcification less prominent – small Mesenchymal chondrosarcoma on MR images shows marrow
foci (60%–70%) replacement (M) and diffuse contrast enhancement as well as
• Soft tissue mass (near 100%) small serpentine vessels (arrows). This is not the enhancement
• Masses show lower water content (CT/MR) and pattern of conventional chondrosarcoma (peripheral/septal)
enhance diffusely; may see high flow vessels (MR)
Myxoid Chondrosarcoma
• Rare in bone – 12% chondrosarcomas
• More aggressive radiologic appearance
• Worse prognosis
• Look for myxoid areas
➢ Low attenuation or signal intensity T1W
images; may show areas of
hemorrhage
➢ Very high signal intensity T2W images
➢ Contrast enhancement — Peripheral /
diffuse
Extraskeletal Chondrosarcoma: Myxoid chondrosarcoma of Axial T2-weighted MR image

proximal femur shows bone reveals marked high signal
Myxoid Type (Chordotic) destruction with intramedullary intensity resulting from very
[Figures 4-6-44 and 4-6-45]
chondroid mineralization high water content of myxoid
• Middle aged patients – average age 50 years (arrowhead) and large posterior chondrosarcoma (same patient
• Deep musculature tissue extremities soft tissue mass with marked low as previous image)
• Thigh/Popliteal fossa (70%) attenuation (*)
• Low grade malignancy – may recur late
• Hemorrhage and myxoid areas can be seen with imaging
• Typical chondroid regions – radiographs
• Metastases – lymph node, lung

Dedifferentiated Chondrosarcoma: Clinical Data Figure 4-6-46
• Older patients – average 60 years
• 10%–20% chondrosarcomas
• Often associated with secondary chondrosarcoma
(> 50%)
• Location – femur (20%), humerus (15%), pelvis
(30%), ribs and scapula (12%)
Dedifferentiated Chondrosarcoma:
Pathology
• Low grade chondrosarcoma
• Small foci higher grade chondrosarcoma
• Spindle cell component
• MFH/ fibrosarcoma, osteosarcoma,
rhabdomyosarcoma, GCT
• Collision of two tumors
Dedifferentiated chondrosarcoma with radiographs showing
typical chondroid mineralization (rings and arcs-arrows). There
Dedifferentiated Chondrosarcoma: Imaging is anterior cortical destruction with a small soft tissue mass
[Figures 4-6-46 and 4-6-47] (arrowheads)
• Radiology emulates pathology: beware the dual
characteristic
➢ One region chondrosarcoma
➢ Second area aggressive bone destruction
• Cortical permeation and soft tissue mass (70%)
• Biopsy of anaplastic region – confusing
• Dedifferentiated component compared to chondroid component Figure 4-6-47
➢ Different intrinsic characteristics
➢ Different contrast enhancement (diffuse)
Radiologic Differential of
Chondrosarcomatous Lesions
• Aggressive chondroid lesion with soft tissue mass
➢ Higher grade conventional chondrosarcoma
➢ Dedifferentiated chondrosarcoma
➢ Mesenchymal chondrosarcoma
• Large fluid component bone or soft tissue
➢ Myxoid chondrosarcoma
• Change in appearance or foci of more aggressive
nature
Dedifferentiated chondrosarcoma with post contrast fat
suppressed T1-weighted MR image showing typical peripheral
Low-Grade Chondroid Lesion: and septal enhancement in the cartilaginous portion of the
Differential Diagnosis lesion (arrows) and diffuse enhancement in the dedifferentiated
• Enchondroma anterior soft tissue component (*) correlating with the sagitally
• Low-grade chondrosarcoma sectioned gross specimen
• Bone infarct
Bone Infarct: Osteonecrosis [Figure: 4-6-48 and 4-6-49]

• Ischemic area may undergo mineralization
• Can have chondroid-like matrix
• Look for peripheral rim of calcification
• No cortical thickening
• Prominent areas of endosteal scalloping or mass exclude osteonecrosis
➢ Except malignant degeneration
Diagnostic Dilemma Long Bone:

Enchondroma vs. Chondrosarcoma
• Enchondroma
➢ Common in hand/foot
➢ Rare in axial skeleton
➢ Common in long bones (1.7% distal femur)

• Chondrosarcoma Figure 4-6-48
➢ Common in axial skeleton
➢ Common in long bones
➢ Rare in hand/foot
Enchondroma vs. Low-Grade Chondrosarcoma:

Clinical Data
• Pain (95%–99%) and mass (20%–76%) favor chondrosarcoma
• Pain in enchondroma – 40%
➢ Often related to activity
➢ Stress microfracture
➢ Vague longer duration
• Is pain referable to lesion?
• Radiologic consultation
Enchondroma vs. Low-Grade Chondrosarcoma:

Pathology
• Permeation of chondroid tissue
• Permeation of cortex
• Fibrous bands separating cartilage
• Invasion of marrow fat
Long Bone Enchondroma : Imaging

• Size < 6–7cm (X-ray); < 5cm (CT/MRI)
• Bone scan =/< AIC* 79% (70% homogeneous)
• Majority in diaphysis
• Endosteal scalloping depth < 2/3 cortex (90% - 95%) Multiple areas of osteonecrosis with serpentine
• No cortical thickening (17%); periosteal reaction (3%) peripheral calcification (arrows) and simulating
• No cortical destruction/soft tissue mass chondroid (ring and arc) mineralization
• MRI peripheral enhancement?
Murphey, Radiographics 98; 18: 1213 * *AIC = Anterior Iliac Crest Figure 4-6-49
Long Bone Chondrosarcoma: Imaging
• Majority in the metaphysis
• Size > 6–7cm (X-ray); > 5cm (CT/MRI)
• Bone scan =/ > AIC* 82% (63% heterogeneous)
• Endosteal scalloping depth > 2/3 cortex (75% -
90%)
• Cortical thickening (47%); periosteal reaction
(51%)
• MRI peripheral and septal enhancement?
CT of osteonecrosis shows peripheral rim of serpentine
Murphey, Radiographics 98; 18: 1213 * *AIC = calcification. Simulation of chondroid mineralization is an
Anterior Iliac Crest artifact of radiographs in looking at a three dimensional
structure with a one dimensional image
References
1. Bloem JL, Mulder JD. Radiol. 1985;14(1):1-9. Chondroblastoma: a clinical and radiological study of 104 cases.
Skeletal Radiol. 1985;14(1):1-9.
2. Murphey MD, Choi JJ, Kransdorf MJ, Flemming DJ, Gannon FH. Imaging of osteochondroma: variants and
complications with radiologic-pathologic correlation. Radiographics. 2000 Sep-Oct;20(5):1407-34. Review.
3. Murphey MD, Flemming DJ, Boyea SR, Bojescul JA, Sweet DE, Temple HT. Enchondroma versus chondrosarcoma
in the appendicular skeleton: differentiating features. Radiographics. 1998 Sep-Oct;18(5):1213-37; quiz 1244-5.
4. Murphey MD, Walker EA, Wilson AJ, Kransdorf MJ, Temple HT, Gannon FH. From the archives of the AFIP:
imaging of primary chondrosarcoma: radiologic-pathologic correlation. Radiographics. 2003 Sep-Oct;23(5):1245-
78. Review.
5. Robinson P, White LM, Sundaram M, Kandel R, Wunder J, McDonald DJ, Janney C, Bell RS. Periosteal chondroid
tumors: radiologic evaluation with pathologic correlation. AJR Am J Roentgenol. 2001 Nov;177(5):1183-8.
6. Wilson AJ, Kyriakos M, Ackerman LV. Chondromyxoid fibroma: radiographic appearance in 38 cases and in a review
of the literature. Radiology. 1991 May;179(2):513-8. Review. Erratum in: Radiology 1991 Aug;180(2):586.

Fibrous Lesions of the Musculoskeletal System
Mark D. Murphey, MD
Fibrous Lesions Figure 4-7-1

• Fibroxanthoma (Nonossifying fibroma)
• Fibrous dysplasia
• Osteofibrous dysplasia / Adamantinoma
• Desmoplastic fibroma
• Fibromatosis
• Malignant fibrous fistiocytoma / fibrosarcoma
• Dermatofibrosarcoma Protuberans (DFSP)
Fibroxanthoma: Other Terms

• Fibrous cortical defect
• Nonossifying fibroma (NOF)
• Fibrous medullary defect
• Nonosteogenic fibroma
Fibroxanthoma [Figures 4-7-1 and 4-7-2]

• ...If the lesion has attained a fairly large size and has penetrated into
and continues to grow in the medullary cavity, it ceases to be a mere
fibrous cortical defect and is then known as a nonossifying fibroma
(Jaffe 1958) Typical nonossifying
fibroma/fibroxanthoma in the tibia
Fibroxanthoma (arrow)
• Very common 20% F, 50% M, older than 2 years of age
• Children and adolescents; M > F
• Usually asymptomatic: only 2% of biopsied primary bone tumors
Figure 4-7-2
• Heal spontaneously with average “life span” 29 months
Fibroxanthoma: Pathology
• Whorls/bundles of fibrous tissue
• Variable cellularity
• Giant cells
• Foam or xanthoma cells
• Areas hemorrhage/hemosiderin
Fibroxanthoma: Skeletal Location

• Metaphyseal origin can migrate to diaphysis
• Long tubular bones – 90%
• Lesions around knee – 55%
• Tibia – 43%, femur – 38%, fibula – 8%
• Upper extremity uncommon – 8%, humerus – 5%
Fibroxanthoma: Radiology [Figures 4-7-3 to 4-7-5]

• Eccentric cortically based lesion
• Longitudinal growth pattern Nonossifying fibroma/fibroxanthoma
• Can extend or primarily involve medullary cavity with healing by extensive ossification
• Lobulated contour (arrow)
• Expansile remodeling with trabeculation
• Cortex may appear permeated focally but no soft tissue mass
• Usually a rim of sclerosis
• Bone scan – minimal to mild uptake
• MR – can be low or high intensity on T2W images
Musculoskeletal Radiology 771 Fibrous Lesions of the Musculoskeletal System

Nonossifying fibroma/fibroxanthoma in the distal femur on radiograph, gross

specimen and histology
Figure 4-7-4
Nonossifying
fibroma/fibroxanthoma in the
fibula with medullary location
(arrow) as is typical for lesions
in this location
Figure 4-7-6
Nonossifying fibroma/fibroxanthoma in the distal femur on radiograph and

multiple MR images. Note intracortical location, heterogeneous signal intensity
on T2 with areas of high signal and enhancement
Interval growth of nonossifying

fibromas/fibroxanthomas and multiple lesions (arrows)
between two radiographs
Figure 4-7-7
Fibroxanthoma: Natural History
• Often heal with residual sclerosis start from diaphyseal
side
• May persist or grow
• Pathologic fracture – greater likelihood in lesions > 3
cm and with >50% bone width involved and weight
bearing bones
Fractures through three nonossifying

fibromas/fibroxanthomas
Fibrous Lesions of the Musculoskeletal System 772 Musculoskeletal Radiology

Fibroxanthoma: Types and Associations [Figure 4-7-8] Figure 4-7-8
• Solitary or multiple
• Multiple with neurofibromatosis type 1
• Multiple with cafe-au-lait spots
Jaffe-Campanacci syndrome
• Oncogenic osteomalacia
Benign Fibrous Histiocytoma [Figure 4-7-9]

• Use of this terminology controversial
• Patient often symptomatic
• Radiographic appearance – larger lesions, more
expansion, medullary involvement, older patient
• Pathology identical to fibroxanthoma
Fibrous Dysplasia: Clinical

Characteristics Multiple nonossifying fibromas/fibroxanthomas associated with
• Developmental anomaly of bone formation neurofibromatosis 1
• Osteoblasts fail to develop
• Marrow replaced by fibrosseous tissue Figure 4-7-9
• Usually diagnosed < age 30 but > age 2 years
• Males and females equally affected
• Monostotic (70%–80%)
• Polyostotic (15%–30%)
• Cafe-au-lait spots – irregular serrated borders
(coast of Maine)
• 1% of biopsied primary bone tumors
Fibrous Dysplasia: Monostotic

• Smaller sized lesions
• Often asymptomatic
• Cafe-au-lait spots less common
• Distribution – femur (35%–40%), tibia (20%),
skull and facial bones (10%–25%), ribs (10%)
• Uncommon sites – hands and feet, spine,
clavicle
Fibrous Dysplasia: Polyostotic

• Larger lesions, symptomatic at earlier age
• 70% present before age 10 – limp, pain, fracture or
deformity
• Cafe-au-lait spots >50% patients
• Involvement variable – two to >75% skeleton; propensity to
involve one side of body more extensively
• Common sites – skull and facial bone (>50%), long bones,
ribs, pelvis
Fibrous Dysplasia Polyostotic: Associations

• Endocrinopathies: 2%–3% patients
• McCune Albright syndrome – bone lesions, cutaneous
pigmentation, precocious puberty – 20%–50% of females Benign fibrous histiocytoma in the tibia with prominent
(only 1 in 30 to 40 have complete triad) sclerotic margin (unusual for GCT).
On MR, prominent low signal intensity on both pulse
• Others – hyperthyroidism, hyperparathyroidism,
sequences
acromegaly, diabetes m., Cushing syndrome This lesion is identical pathologically to nonossifying
• Soft tissue myxoma – Mazabraud syndrome fibroma/fibroxanthoma but patients are symptomatic
and older age at presentation
Fibrous Dysplasia: Pathologic Characteristics
• Fibrosseous metaplasia
• Stroma may have cystic or myxoid elements
• Trabeculae are pure woven bone with “alphabet soup” appearance
• Occasional osteoblastic rimming and chondroid foci
• May have ABC component

Fibrous Dysplasia
Radiology: Appendicular Skeleton [Figures 4-7-10 to 4-7-12]
• Medullary diaphyseal lesions Figure 4-7-10
• Radiolucent with woven bone in marrow
creating “ground glass appearance”
• Expansile remodeling
• Usually well defined and may have sclerotic rind
(monostotic lesions)
• May have “multiloculated appearance” caused
by subperiosteal reinforcement
• Areas of sclerosis (most common in skull)
• Skeletal deformity – fracture, bowing
(Shepherd’s Crook), growth disturbance (more
common polyostotic disease)
Figure 4-7-11
Typical "ground glass" appearance in several patents with fibrous

dysplasia
Figure 4-7-12
Typical monostotic fibrous dysplasia in

intertrochanteric femur with thick rind of
sclerosis (arrow) Fibrous dysplasia of rib on radiograph and macrosection showing
elongated involvement (*) of a prominent osseous extent
Fibrous Dysplasia Radiology:
Craniofacial Skeleton [Figures 4-7-13 to 4-7-15]
• Commonly involved – frontal, sphenoid, ethmoid, maxilla, zygoma, parietal,
occipital and temporal
• Often mixed lucency and sclerosis
• Sclerosis often marked at skull base – can impinge on cranial nerves
• Calvarium expanded with greater involvement
outer table
Figure 4-7-13
Fibrous dysplasia on T2-weighted

Fibrous dysplasia of the calvarium with skull base Coronal CT reconstruction of fibrous MR with prominent low signal
sclerosis (*) and expansion of the occipital outer table dysplasia shows mixed lysis and intensity in the frontal bone with
(arrow) sclerosis and outer table expansion expansile remodeling (*)

Fibrous Dysplasia Radiology: Other Studies Figure 4-7-16
[Figures 4-7-16 and 4-7-17]
• Bone scan – usually increased activity probably more
variable than recognized
• CT – especially helpful in skull
• MRI
➢ 20% low intensity T2W images
➢ 20% same as fat T2W images
➢ 60% high intensity T2W images
Figure 4-7-17a
Fibrous dysplasia of the humerus with typical intense

uptake of radionuclide bone scan
Figure 4-7-17b
Fibrous dysplasia of femoral diaphysis with

nonspecific marrow replacement (*) on coronal T1-
weighted MR image
Fibrous Dysplasia: Complications [Figure 4-7-18]

• Malignant transformation – 0.5%
• Osteosarcoma most frequently but also
MFH/fibrosarcoma and chondrosarcoma Fibrous dysplasia of femoral diaphysis with
• Both polyostotic and monostotic nonspecific marrow replacement and high signal
• Prior radiation in 30% intensity on coronal T2-weighted MR image (*)
(same patient as previous MR)
Fibrous Dysplasia: Differential Diagnosis
• Bone cyst
• Fibroxanthoma (medullary)
Figure 4-7-18
• Meningioma
• Osteoblastoma (long bone)
• Paget disease
Osteofibrous Dysplasia: Previous Terms

• Cortical fibrous dysplasia
• Intracortical fibrous dysplasia
• Ossifying fibroma – don’t confuse with facial
lesion
• Juvenile adamantinoma
Osteofibrous Dysplasia:
• Unusual lesions – 0.2% of biopsied primary bone Monostotic fibrous dysplasia of the proximal femur with
tumors malignant transformation to MFH on radiograph and gross
• Patients <10 years age; rare after 16 years specimen. Note ground glass appearance distally (*) and more
• Tibia alone (75% - 80%) or also fibula (12%); fibula aggressive bone destruction proximal with extension through
lesser trochanter proximally (arrows)
only (7%), both tibiae (3%), rarely radius/ulna

Osteofibrous Dysplasia: Pathology Figure 4-7-19
• Vascularized fibrous stroma like fibrous dysplasia
• Prominent osteoblastic rimming
• No “alphabet soup” of woven bone
• Can be weakly keratin positive but no epithelial
nests
Osteofibrous Dysplasia: Radiology

[Figures 4-7-19 and 4-7-20]
• Lytic lesion anterior cortex mid tibial diaphysis
• May involve medullary canal
• No soft tissue mass
• Expansile remodeling and sclerotic component
• Causes bowing, fracture, pseudarthrosis and may
progress to involve entire tibia
• Homogeneous intermediate on T1 and high on T2
➢ Homogeneous mild to moderate enhancement Typical osteofibrous dysplasia with elongated/multifocal
intracortical tibial involvement on radiograph and matched
macrosection (*)
Adamantinoma: Clinical Characteristics
• Present with pain/swelling; often history of trauma Figure 4-7-20
• Don’t confuse with mandibular ameloblastoma
• Rare low grade malignancy – 0.1% biopsied
primary bone tumors
• Also previously called angioblastoma
• Male to female ratio 1.3:1, average age 35 years
Adamantinoma: Pathology and Location

• Epithelial nests / prominent keratin staining
• Background of bland fibrous stroma
• May have foci of Ewing-like areas – worse
prognosis
• Tibia (80% - 85%), tibia and fibula (5%), femur Typical osteofibrous dysplasia on MR imaging with elongated
intracortical tibial involvement and homogeneous intermediate
(5%), humerus (4%), ulna (3%), fibula (1%)
signa l intensity on T1-weighting and high signal on T2-
weighting (arrows).
Adamantinoma: Radiology
• Diaphyseal to metadiaphysis – anterior tibial cortex
• Mixed lytic and sclerotic Figure 4-7-21
• May be multifocal with medullary involvement and soft tissue
mass
• Expansile remodeling with cortical thickening
Adamantinoma:
Radiology and Prognosis [Figures 4-7-21 to 4-7-24]
• MRI
➢ Very heterogeneous high intensity T2W
➢ Vascularity with prominent enhancement
• Locally aggressive
• 10 year survival: 10% - 65%
• 15% patients die with metastases
Osteofibrous Dysplasia:
Relationship to Adamantinoma
• Differentiation - patient age
➢ Multiple recurrence
➢ MRI heterogeneous, intense enhancement
• Epithelial nests; both can be keratin positive
• Several cases reported of foci of adamantinoma in osteofibrous
dysplasia and progression to adamantinoma
Adamantinoma of the tibia on radiograph with
mixed lytic and sclerotic lesion centered in the
cortex and an elongated lesion

Adamantinoma of the tibia on multiple MR images (same

Adamantinoma of the tibia on several CT images (same patient
patient as previous radiograph and CT) with elongated lesion
as previous radiograph) with mixed lytic and sclerotic lesion
centered in the cortex (arrows). There is prominent
centered in the cortex (arrows)
heterogeneity on STIR
Desmoplastic Fibroma: Clinical Characteristics Figure 4-7-24

• Rare fibrous lesion of bone
• 0.2% - 0.3% biopsied primary bone neoplasms
• M=F or slight female predilection
• 70% between ages 15 and 40 years
• Desmoid tumor of bone (5–10 times less common than soft
tissue lesion)
Desmoplastic Fibroma: Clinical Characteristics

• Location – femur, tibia, humerus, radius, mandible, pelvis
• Metaphyseal – central
• Pain and swelling (90%) Intraoperative photograph of adamantinoma of
• Pathologic fracture (15%) the tibia with intracortical curretage (same
patient as previous radiograph, MR and CT)
Desmoplastic Fibroma: Pathology
• Histology identical to soft tissue desmoid Figure 4-7-25
• Gross - lobular firm white to gray mass
• Fibroblasts producing well-formed collagen
• Nuclear monotony, variable cellularity, rare
mitosis
• Rarely associated with fibrous dysplasia
Desmoplastic Fibroma: Radiology

[Figure 4-7-25]
• Lytic lesion with expansile remodeling
• May have sclerotic margin
• Internal trabeculae - subperiosteal reinforcement
• May have more aggressive appearance
• May be low intensity T2W MR images
Cortical Desmoid [Figures 4-7-26 and 4-7-27 overleaf] Desmoplastic fibroma of the iliac bone with prominent
• Avulsive cortical injury (chronic) multilocular appearance caused by internal trabeculation
• Posteromedial distal femur metaphysis
• Stress related at attachment
➢ Adductor magnus
➢ Medial head gastrocnemius
• Pathology simulates aggressive lesion
• Children 1st decade (35%)
• More frequent in boys, often bilateral
• Surface irregularity/lucency
• CT – looks like NOF – no soft tissue mass
• MRI – may see surrounding inflammation

Two patients with cortical desmoids (chronic avulsive injury) of

the distal femur (arrows)
Sagittal CT reconstruction showing medial head of
gastrocnemius muscle extending into cortical desmoid (arrow)
Fibromatosis and macrosection of reparative tissue (*) in a different patient
• Deep – grow rapidly, larger, more aggressive
• Superficial – slow growing, small, arise from fascia/aponeurosis
• Can be multifocal 5%-20%
Fibromatosis: Types
• Extra-abdominal desmoid (deep)
Figure 4-7-28
• Aggressive infantile fibromatosis (deep)
• Juvenile aponeurotic fibroma (sup.)
• Infantile dermal/digital fibromatosis (sup.)
• Adult palmar and plantar (sup.)
• Infantile myofibromatosis (both)
Fibromatosis: Pathology
• Gross - glistening white, variable cellularity
• Spindle shaped fibrous cells
• Abundant collagen, can see mitoses
• Infiltrative growth common
• No malignant potential Post-contrast axial T1-weighted MR image showing
extraabdominal desmoid (fibromatosis) with an enhancing
Fibromatosis: Radiology paraspinal mass (*) and ill-defined margins
• Soft tissue mass, unusual to calcify
• Can erode adjacent bone
• CT-soft tissue mass – may show attenuation greater than muscle
• MRI Figure 4-7-29
➢ T1W image – low / intermediate signal
➢ T2W image – variable signal
➢ Fascial tail sign
➢ Low signal bands
• Enhance with contrast
Axial STIR MR image showing extraabdominal desmoid

(fibromatosis) with a soft tissue mass (*) and ill-defined margins
and linear extension (fascial tail sign) laterally (arrow). These
features are also shown on a gross specimen froma different
patient

Fibromatosis: Extraabdominal Desmoid [Figures 4-7-28 and 4-7-29]
• Painless growing deep mass (25–35 years)
• Rarely familial Figure 4-7-30
• Shoulder (20%), chest wall/back (15%), thigh
(12%), mesentery (10%), neck (8%), knee (7%),
buttock (6%)
• Intermuscular along fascia/aponeurosis, infiltrative
Fibromatosis: Aggressive Infantile

[Figures 4-7-30 to 4-7-32]
• Painless soft tissue mass, M>F
• Discovered first two years of life
• Intermuscular – head/neck, shoulder, thigh, foot
• Can erode bone
• Multifocal (10%-15%)
• No metastasis but locally aggressive and recur
Fibromatosis:
Juvenile Aponeurotic Fibroma Aggressive infantile fibromatosis on sagittal T1-weighted MR
image with large mass eroding bone (*) which ultimately led to
• Children/adolescents; M>F amputation following multiple recurrences.Note low intensity
• Hands (77%), feet (13%) – palms and soles bands (arrowheads)
• Painless slowly growing mass
• Calcification and local recurrence (50%) common
• Attached to tendon/aponeurosis Figure 4-7-31
Fibromatosis: Infantile Dermal/Digital

• Birth to age 2 years; F>M
• Fingers > toes; dorsum, spare thumb/great toe
• Bone erosion rare, can have contractures
• Pathology – intracellular inclusion bodies
• Recurrence local 60%
Adult Palmar Fibromatosis

• Palmar (Dupuytren contracture) 1%–2%
population (1/5 people older than 65)
• M>F (4–5:1); ulnar side, thumb and index finger
spared
• Fibrous nodules - cords (40%–60% bilateral)
• Other fibromatosis 5%–20%
• Contractures/recurrence common
Figure 4-7-32 Same patient as previous image showing multicentric

involvement with second site (*) in lower calf demonstrating
high signal intensity on axial T2-weighted MR image. The
patient ultimately required amputation as shown on the gross
specimen with the large recurrent mass (*)
Axial T2-weighted MR image in patient with aggressive

infantile fibromatosis after radiation shows marked low signal
(arrows) resulting from collagenization following successful
nonoperative treatment

Adult Plantar Fibromatosis Figure 4-7-33
• Ledderhose disease
• Less common than palmar
• M>F (2:1); wider age range 55% < age 30
• Starts as single nodule middle to medial sole
• Often leads to early excision, contractures rare
• Associated with palmar disease 5%–20%
Infantile Myofibromatosis
• Discovered at birth or within weeks
• Solitary form (good prognosis)
Multifocal (poor prognosis) – soft tissue, muscle,
viscera
• Bone lesions common but involute
Osseous MFH with radiograph showing solitary geographic
• Lesions grow in perinatal period lytic lesion with wide zone of transition (arrows)
• Myoblastic and fibroblastic lesion
Fibromatosis: Treatment and Prognosis

• Surgical excision Figure 4-7-34
• Recurrence common
• High signal on T2 corresponds to more cellular regions increasing
recurrence
• Re-excision may use radiation therapy
➢ Can follow with MRI
• Can ultimately require amputation
Malignant Fibrous Histiocytoma (MFH) and Fibrosarcoma

• Osseous
• Soft tissue (S.T.)
Malignant Fibrous Histiocytoma: Pathology

• Described 1964 – Stout and coworkers
• Three cell types present CT of osseous MFH shows no matrix
mineralization and cortical penetration
➢ Fibroblastic spindle cells
anteriorly with soft tissue mass (*)
➢ Plump histiocytic cell (from marrow monocytes?) (same patient as previous image)
➢ Giant cells benign and malignant
• Histologic types–storioform (pleomorphic 50%–60%), myxoid (25%), giant cell
(10%), inflammatory (10%), angiomatoid (5%)
• No malignant matrix; diagnosis of exclusion Figure 4-7-35
• WHO 2002 – Undifferentiated high grade
pleomorphic sarcoma (soft tissue lesions only)
Fibrosarcoma: Pathology
• Malignant collagen producing spindle cells
• “Herringbone” pattern – lower grade lesions (I-II)
• Higher grade (III-IV) lesions – more anaplasia
• No matrix or malignant giant cells
Osseous MFH and Fibrosarcoma: Clinical

Features
• Age 40–70 years
• Pain, swelling and pathologic fracture (30%–50%)
• Slight male predilection
• Metaphyseal – around knee (40%–80%), humerus
(10%), pelvis (10%)
• Secondary lesions – Paget disease, osteonecrosis, Coronal T1-weighted MR image and coronal gross specimen
fibrous dysplasia, chronic osteomyelitis shows distal femoral MFH (same patient as previous two
images) not extending to subchondral bone (arrowhead) (as
would be seen with giant cell tumor)

Osseous MFH and Fibrosarcoma: Radiology Figure 4-7-36
[Figures 4-7-33 to 4-7-38]
• Lytic lesions – from geographic IB to
motheaten/permeative – reflects tumor grade
• With geographic IB - IC lesions – GCT like; look for
diaphyseal > epiphyseal extension
• Little periosteal reaction or sclerosis
• May not show increased intensity on T2W MR
images
Soft Tissue MFH: Clinical Features

• Most common adult S.T. sarcoma
• Accounting for 15%–30% all S.T. sarcomas
• Age 50–70 years: M>F 2:1
• Location – deep soft tissues – lower extremity
(50%), upper extremity (20%), retroperitoneum Osseous MFH showing aggressive solitary lytic lesion (arrows)
(15%), head and neck (5%) in supraacetabular region with lateral cortical destruction
difficult to detect
Musculoskeletal Soft Tissue Figure 4-7-37

Sarcoma Incidence Figure 4-7-38
• Malignant fibrous histiocytoma and
Fibrosarcoma 20%–30%
• Liposarcoma 16%–19%
• Rhabdomyosarcoma 10%–19%
• Nonspecific spindle cell sarcoma 5%–15%
• Leiomyosarcoma 5%–10%
• Dermatofibrosarcoma protuberans (DFSP)
5%–10%
• Synovial sarcoma 5%–10%
Soft Tissue Fibrosarcoma: Clinical

Features
Axial T1-weighted MR image of
• Palpable mass – deep soft tissues
Osseous MFH (same patient as osseous MFH (same patient as
• Lower extremity – knee and thigh (45%); upper previous two images) shows
previous image) with
extremity (28%), trunk (17%), head and neck intraosseous lesion and large marrow replacement and
(10%) associated soft tissue mass (*) associated soft tissue mass (*)
• Age– 30 to 55 years, no sex predilection but no matrix mineralization
Soft Tissue MFH and Fibrosarcoma: Radiology

[Figures 4-7-39 to 4-7-42]
• Deep soft tissue mass – MRI > CT for evaluating extent prior to surgery
• MRI
➢ Usually similar to muscle T1W images
➢ High intensity T2W images
➢ May not show increased intensity on T2W images
• Pseudocapsule – low intensity all sequences
• Calcification/ossification: 5%–20% MFH Figure 4-7-39
• May involve underlying bone
• MFH – hemorrhage – high intensity T1W
images
• Can differentiate myxoid lesions look like fluid
with nodular peripheral contrast enhancement
Soft tissue MFH with mass replacing vastus lateralis muscle (*) on
axial T1-weighted MR image [left]; with high signal intensity mass (*)
on axial T2-weighted MR image [right]

T1 T1 GD
Largely hemorrhagic (*) soft tissue MFH in Sagittal T1-weighted MR images before and after contrast
the anterior thigh on CT with the only solid show enhancement of the solid component (arrowheads) of the
component adjacent to the anteromedial MFH and nonenhancing hemorrhagic areas (*)
femur (arrowhead)
Figure 4-7-42
Fibrosarcoma and MFH:
• Treatment - wide local resection/amputation
• Local recurrence common (50%)
follow up imaging
• Metastasis (40%) common
hematogenous - lung, lymph nodes, liver and
bone
Dermatofibrosarcoma Protuberans
(DFSP): Clinical Features
• 6% all soft tissue tissue sarcomas
• Third to fifth decades of life
• Reddish brown to bluish superficial nodule
• May be multiple
• Most common to affect trunk (50%) Soft tissue MFH with soft tissue mass (*) causing extrinsic
➢ Remainder head/neck, upper/lower extremities erosion of adjacent femur (arrowheads)
Dermatofibrosarcoma Protuberans (DFSP): Pathology

• Uniform fibroblasts
• Storiform pattern (may be myxoid)
• May have areas of higher-grade sarcoma
➢ Usually fibrosarcoma (17% - 27%)
Dermatofibrosarcoma Protuberans (DFSP):

Radiologic Characteristics [Figure 4-7-39]
• Subcutaneous mass - no calcifications
• Usually centered on skin and protuberant
• Nonspecific solid intrinsic features CT/MRI
➢ Enhance with contrast, ST attenuation
➢ Intermediate T1; high signal T2
• May have hemorrhage
• Look for linear extension (skin/fascial tail sign)
➢ Satellite nodules

Figure 4-7-43
T1
DFSP on sagital T1, axial STIR and gross

specimen showing protuberant subcutaneous
mass (*) involving the skin with linear extensions
along the skin surface (arrows)
Dermatofibrosarcoma Protuberans (DFSP):

Treatment and Prognosis
• Surgical excision (wide with 3cm margin)
• Local recurrence 20%-55% (within 3 years)
➢ Higher with head/neck lesions (50%-75%)
• Metastases
➢ Lungs (5%-6%)
➢ Lymph nodes up to 25% of metastases
➢ Higher incidence with high-grade component (21%)
Fibrous Lesions References
1. Fitzpatrick KA, Taljanovic MS, Speer DP, Graham AR, Jacobson JA, Barnes GR, Hunter TB. Imaging findings of
fibrous dysplasia with histopathologic and intraoperative correlation. AJR Am J Roentgenol. 2004 Jun;182(6):1389-
98. No abstract available.
2. Jee WH, Choe BY, Kang HS, Suh KJ, Suh JS, Ryu KN, Lee YS, Ok IY, Kim JM, Choi KH, Shinn KS. Nonossifying
fibroma: characteristics at MR imaging with pathologic correlation. Radiology. 1998 Oct;209(1):197-202.
3. Jee WH, Choi KH, Choe BY, Park JM, Shinn KS. Fibrous dysplasia: MR imaging characteristics with radiopathologic
correlation. AJR Am J Roentgenol. 1996 Dec;167(6):1523-7.
4. Murphey MD, Gross TM, Rosenthal HG. Musculoskeletal malignant fibrous histiocytoma: radiologic-pathologic
correlation. RadioGraphics 1994; 14:807-826.
5. Ritschl P, Karnel F, Hajek P. Fibrous metaphyseal defects--determination of their origin and natural history using a
radiomorphological study. Skeletal Radiol. 1988;17(1):8-15.
6. Robbin MR, Murphey MD, Temple HT, Kransdorf MJ, Choi JJ. Imaging of Musculoskeletal Fibromatosis.
7. Torreggiani WC, Al-Ismail K, Munk PL, Nicolaou S, O'Connell JX, Knowling MA. Dermatofibrosarcoma protuberans:
MR imaging features. AJR Am J Roentgenol. 2002 Apr;178(4):989-93.
8. Van der Woude HJ, Hazelbag HM, Bloem JL, Taminiau AH, Hogendoorn PC. MRI of adamantinoma of long bones
in correlation with histopathology. AJR Am J Roentgenol. 2004 Dec;183(6):1737-44.

Alphabet Soup and Cystic Lesions
of The Bone
Mark D. Murphey, MD
Alphabet Soup and Cystic Lesions of the Bone

• Giant cell tumor (GCT)
• Unicameral bone cyst (UBC)
• Aneurysmal bone cyst (ABC)
• Epidermoid inclusion cyst
• Subchondral cyst
• Intraosseous ganglion
• Post-traumatic cyst
Giant Cell Tumor (GCT): Clinical Features

• Approximately 5% of all biopsied primary bone tumors; 18%-23% of Figure 4-8-1
benign bone neoplasms
• Symptoms – pain and swelling often relieved by decreased
activity
• Pathologic fracture 10%–35%
• Adults – 80% between 20–50 Years
• Rare in children 1%–3% (<14 years)
• Sex distribution
➢ F-M ratio 3:2 benign GCT
➢ M-F ratio 3:1 aggressive GCT
Giant Cell Tumor: Location

• Originate metaphyseal side of growth plate and grow to
subchondral bone (84%–99%)
• Long tubular bones 75%–90%
• About the knee 50%–65%; distal femur 23%-30%; proximal
tibia 20%-25%
• Radius (10%-12%); humerus (4%-8%) Giant cell tumor (GCT) of the proximal tibia with
geographic lytic lesion with a mild rim of partial
• Spine – (7%-15%) - vertebral body – sacrum-thoracic-cervical- sclerosis (arrows-unusual in GCT), mild
lumbar expansile remodeling and increased
• Pelvis (4%); hands/feet (5%) radionuclide uptake on bone scan (right image)
• Multifocal (0.5%–1%)– skull and face (Paget disease), Goltz
syndrome Figure 4-8-2
Giant Cell Tumor: Pathology

• Osteoclast like giant cells (90%)
• Mononuclear spindle cell stromal component
• Hemorrhage, necrosis and hemosiderin
• ABC like areas 10%–15%
Osseous Lesions Containing Giant Cells

• GCT/ABC/UBC
• NOF/CMF/OGS
• Brown tumor HPT/chondroblastoma
• Fibrous dysplasia and variants
• Osteoblastoma
• Giant cell reparative granuloma
Giant Cell Tumor: Radiology [Figures 4-8-1 to 4-8-10] CT of giant cell tumor shows no mineralized
• Solitary eccentric geographic lytic lesion extending into matrix (*) (same patient as previous
subchondral bone radiograph)
• Center of lesion-metaepiphysis
• Margin IB (80%-85%), IC (10%-20%), IA (1%-2% but up to 20% by CT)
• No mineralized matrix
Alphabet Soup and Cystic Lesions of Bone 784 Musculoskeletal Radiology

• Expansile remodeling (47%-60%) with apparent cortical permeation Figure 4-8-3
(33% - 50%)
➢ Septations - subperiosteal new bone
➢ Periosteal reaction unusual 10%–30%
• Radiologic characteristic do not reflect clinical
behavior of GCT
• Bone scan - doughnut sign (57%)
• Usually a vascular lesion (75%-90%)
• MRI>CT for evaluation of extent
• Fluid-fluid levels
• Low to intermediate intensity usually
predominates on T2W images (90%–95%)
Figure 4-8-4
Giant cell tumor of distal femur shows well defined geographic (1B
margin-arrow)) lysis extending to subchondral bone. Bone scan
reveals marked increased uptake in femur. Bone scan reveals
marked increased uptake in the femoral GCT but also in the
adjacent tibia and patella. The increased uptake in the tibia and
patella are due to hyperemia and disuse, not tumor involvement
Figure 4-8-5
Axial T1-weighted MR image shows marrow

replacement and small anterior soft tissue mass
(*) resulting from this benign giant cell tumor
(same patient as previous radiograph and bone
scan).
Figure 4-8-6
Sagittally sectioned gross specimen and macrosection
show identical findings as on the previous images of this
benign giant cell tumor including extension to subchondral
bone (arrows) and anterior soft tissue component (*)
Figure 4-8-8
Figure 4-8-7
Giant cell tumor of the fibula

with marked expansile
remodeling of bone Coronal T2-weighted MR image
shows intermediate to low (*) signal
intensity tissue typical of giant cell
tumor Giant cell tumor of the patella
(sesamoids and apophysis are
epiphyseal equivalents for the
differential diagnosis of lytic lesions)
Musculoskeletal Radiology 785 Alphabet Soup and Cystic Lesions of Bone

Sacral Lesions: Differential Diagnosis Figure 4-8-9
• GCT/ABC
• Metastasis
• Myeloma/plasmacytoma
• Chordoma
• Neurogenic tumor
Giant Cell Tumor: Treatment and Prognosis

• Curettage and cryosurgery or en bloc resection and bone graft
• Local recurrence rate 40%–60% historically
• Current recurrence rate 2%–25%
• Recurrence does not correspond to radiologic or microscopic
appearance
• Osseous recurrence - new bone destruction
• Soft tissue recurrence - mass and may calcify/ossify about
Giant cell tumor of the sacrum with
periphery
predominantly low to intermediate signal
• May metastasize - 2%-5% (50% benign histology) intensity (*) on the axial T2-weighted MR image
• Malignant GCT 10%–15% (much <5% in our experience) (more
common with radiation)
Figure 4-8-10
Giant Cell (Reparative) Granuloma
• Rare benign lesion described in 1953 by Jaffe
• Mandible/maxilla and hands/feet
• Phalanges > metacarpal > metatarsal > carpus >
tarsus
• Women > men (jaw), age to 10 - 50 years
• May have history trauma
Giant Cell (Reparative) Granuloma:

Pathology
• Granuloma - like arrangement of fibroblastic
stroma and osteoid on micro Giant cell tumor of the sacrum with predominantly intermediate
signal intensity (*) on the sagittal T1-weighted MR image and
• Metadiaphyseal lytic lesion
large associated soft tissue mass (*) correlating identically with
• Expansile remodeling and trabeculation the sagittally sectioned gross specimen
• Recurrence only if incompletely excised
Giant Cell (Reparative) Granuloma: Radiology [Figure 4-8-11]

• Similar to GCT Figure 4-8-11
• May not extend to subchondral bone (hand)
• Expansile remodeling and trabeculation
• May detect small amount of mineralization
Unicameral Bone Cyst: Simple Bone Cyst

• A fluid - containing lesion lined by mesothelial (epithelial-like)
cells usually arising in metaphysis of long bone adjacent to
physis
Simple Bone Cyst: Clinical Features

• 3% of all biopsied primary osseous neoplasms
• Young patients 85% < 20 years
• M>F; 2:1
• Pathologic fracture 50%
Simple Bone Cyst: Pathology

• Clear, straw - colored fluid filled cyst
• Cyst lining - thin flat epithelial - like cells - mesothelial origin
• Complicated cysts - hemorrhage, fibro-osseous repair tissue
Giant cell (reparative) granuloma in the second

metacarpal

Simple Bone Cyst: Location and Etiology Figure 4-8-12
• Under age 20 - humerus (55%–65%), femur (25%–30%), tibia, fibula, radius
and ulna rare
• Over age 20 - iliac bone and calcaneus
• Cause - lymphatic or venous obstruction vs. synovial origin
Simple Bone Cyst: Radiology [Figures 4-8-12 to 4-8-14]

• Geographic IA lesion - originate in central metaphysis (active)
• Can migrate into the diaphysis (latent)
• Mild expansile remodeling
• Not infrequently multilocular
• Pathologic fracture
➢ ”Fallen fragment” sign (5%)
• CT/MRI- noncomplicated see simple fluid
➢ Thin wall and septal enhancement beware delayed MR imaging with
diffusion
• CT/MRI- complicated case
➢ Solid components, thick walls, fluid level
Simple bone cyst with fracture
Simple Bone Cysts: Treatment and Course (arrow) and “fallen fragment”
• Spontaneous regression or heal after fracture sign (curved arrow)
• Curettage and bone grafting
• Intralesional steroids (70%–95% effective) Figure 4-8-13
• Recurrence 20%–40%
• Extremely rare-malignant transformation
Aneurysmal Bone Cyst (ABC): Definition

• “The so called aneurysmal bone cyst is neither a cyst nor a
neoplasm; rather it is probably a periosteal to intraosseous
arteriovenous malformation not uncommonly seen in association
with other well known benign and even malignant lesions.”
Mirra JM. Bone Tumors. Lea & Febiger 1989
Aneurysmal Bone Cyst: Clinical Features

• 1% of all biopsied primary osseous neoplasms
• 80% between ages 5 and 20 years
• Patients present with pain, swelling, and pathologic fracture
(10%–20%)
• May be associated with trauma Simple bone cyst in the calcaneus (arrow)
• Slightly more common in women
Aneurysmal Bone Cyst: Secondary Lesion Figure 4-8-14

• 1%–32% of cases
• Benign lesions - chondroblastoma, CMF, NOF, GCT, fibrous dysplasia,
UBC, brown tumor, hemangioma, giant cell reparative granuloma
• Malignant lesions - hemangioendothelioma, telangiectatic
osteosarcoma, chondrosarcoma
Osseous Lesions with Prominent Fluid Levels

• Aneurysmal bone cyst (only fluid levels)
• Giant cell tumor (to bone end,metaphyseal center)
• Chondroblastoma (epiphyseal center)
• Osteoblastoma (posterior elements spine)
• Telangiectatic Osteosarcoma (thick walls, osteoid on CT)
• Fibrous dysplasia (diaphysis, “ground glass”)
Aneurysmal Bone Cyst: Pathology

• Gross - “Blood-filled sponge”
• Cavernous blood filled spaces line by fibrous walls
Simple bone cyst in the calcaneus
• May see chondrosseous tissue indicating repair
on CT with septation (arrowheads)

Aneurysmal Bone Cyst: Location
• Long tubular bone 70%–80%
• Spine posterior elements - 15% (thoracic, lumbar, cervical, sacral)
• Pelvis 5%–10%
• Hands 10%–15%
Aneurysmal Bone Cyst: Radiology [Figures 4-8-15 to 4-8-21]

• Only osseous neoplasm named for its radiologic appearance
• Metaphysis (80%–90%), eccentric medullary geographic lytic lesion
• Can appear central with expansion
• Diaphysis (10%–20%), often surface lesions
• Expansile remodeling uneven in distribution creating one aggressive margin
• Spine - expansion can lead to neurologic deficits
• Periosteal membrane intact on CT/MRI
• Bone scan - peripheral activity (65%)
• Fluid-fluid levels (CT/MRI)-nonspecific representing sedimentation of blood
• Angiography-hypovascular lesion with localized areas of increased vascularity
Aneurysmal bone cyst (primary) with more prominent expansile Aneurysmal bone cyst (primary) with “donut” sign (increased
remodeling of bone posteriorly (more aggressive appearance- uptake peripherally and photopenia centrally) on bone
arrow) versus rim of sclerosis in other areas (indolent scintigraphy (same patient as previous radiographs)
appearance-arrowheads)
Figure 4-8-18
Figure 4-8-17
T2
Aneurysmal bone cyst (primary) on axial T2-weighted MR image

shows fluid levels (arrows) from hemorrhage in all parts of the
lesion (same patient as previous bone scan) Aneurysmal bone cyst (primary) with sagittal
gross specimen showing blood filled spaces (*)
lined by thin septae (arrows) (same patient as
previous MRI)

T1 T1 GD T1 T2
Coronal T1-weighted and sagittal T2-weighted MR images of a

Coronal pre and post contrast T1-weighted MR images showing
secondary (chondroblastoma) aneurysmal bone cyst with cystic
thin enhancing periphery and septae (arrows) typical of a primary
areas containing fluid levels (arrows) and anterior solid
aneurysmal bone cyst
component (*)
Figure 4-8-21
Giant cell tumor with ABC component on various MR pulse

sequences with diffuse enhancement and intermediate signal
intensity of the solid component (*) and rim enhancement, high
signal and fluid level in the cystic component (arrows)
Aneurysmal Bone Cyst: Treatment and Prognosis

• Rarely spontaneous regression
• Curettage, cryosurgery and bone grafting
• Recurrence 10%–20%
• Radiotherapy
• Radiofrequency ablation with methylmethacrylate placement
• Embolotherapy*
Cory DA et al. AJR 1989; 153:369
Aneurysmal Bone Cyst: Solid Variant?

• Recently described (1983); controversial
• Radiography - similar to other ABC’s but more often aggressive and axial
location
• Histology-fibrous tissue proliferation, osteoid production, osteoclastic giant
cells, sinusoids

Epidermoid Inclusion Cyst [Figure 4-8-22] Figure 4-8-22
• Two types
• Hand - distal phalanx-traumatic origin
• Radiographs - punched out lesion with surrounding sclerosis,
dorsal cortex often absent
• Skull-intraosseous-frontal and temporal bone-congenital origin
• Radiographs - well defined lytic lesion sclerotic margin and can
cause expansile remodeling of bone
• Pathology - stratified squamous epithelium
Subchondral Cyst
• Other terms - geodes and synovial cyst; no true epithelial or
synovial lining
• Middle to older aged patients
• Around joints and associated with other arthritic changes
• Etiology - synovial fluid intrusion vs. osseous contusion
• Can be large/solitary, articular damage subtle simulating neoplasm
(GCT)
Intraosseous Ganglion [Figures 4-8-23 and 4-8-24] Epidermoid inclusion cyst with well-
• Uncommon lesion; middle aged adults defined terminal phalangeal lytic lesion
• Pain increases with activity (arrows)
• Periarticular, eccentric, geographic IA-B lytic lesion
Figure 4-8-23
• Tibia (medial malleolus), femur, about wrist (>65%
of lesions)
• Pathology - same as soft tissue ganglion
Post-Traumatic Cyst [Figure 4-8-25]

• Occurs as complication of fracture in children
• Usually forearm - radius/ulna
• Caused by hemorrhage then fibrosis
• Radiolucent lesion well defined, may heal or
persist
Figure 4-8-24 Intraosseous ganglion in the medial malleolus with geographic

lysis (arrows) and thin sclerotic margin (1A)
Figure 4-8-25
Intraosseous ganglion in the subchondral region of the medial

malleolus with intermediate signal intensity on T1-weighting
and high signal intensity on T2-weighting (*) and septation
Post traumatic cyst in the radius subsequent to

a fracture (arrows)

References
1. Kransdorf MJ, Sweet DE. Aneurysmal bone cyst: concept, controversy, clinical presentation, and imaging. AJR Am
J Roentgenol. 1995 Mar;164(3):573-80. Review.
2. Martinez V, Sissons HA. Aneurysmal bone cyst. A review of 123 cases including primary lesions and those secondary
to other bone pathology. Cancer. 1988 Jun 1;61(11):2291-304.
3. Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ. From the archives of AFIP.
Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation.
Radiographics. 2001 Sep-Oct; 21(5):1283-309. Review.
4. Parman LM, Murphey MD. Alphabet Soup: Cystic Lesions of Bone. Seminars in Musculoskeletal Radiology 2000;
4(1):89-101.

Juxtaarticular Soft Tissue Masses
Mark D. Murphey, MD
Soft Tissue Masses In and About Joints Figure 4-9-1

• Tumor like-tumoral calcinosis, PVNS, ganglion, synovial cyst, myositis
ossificans
• Benign - synovial lipoma, myxoma, synovial chondromatosis/chondroma,
nodular fasciitis
• Malignant-synovial sarcoma, clear cell sarcoma
Tumoral Calcinosis: Clinical Features

• Usually children/young adults
• Increased incidence in blacks
• Familial tendency (33% of cases)
• Large calcified paraarticular mass, hip, shoulder, elbows, and feet
• Can be associated with CPPD arthropathy, pseudoxanthoma elasticum -
like syndrome
• Also skin ulceration, marrow and dental changes
• Etiology - metabolic (hyperphosphatemia and increased Vitamin D), trauma, Tumoral calcinosis about the
idiopathic shoulder with large calcified
periarticular mass (*) and
radiolucent septations
Tumoral Calcinosis: Pathology (arrowheads)
• Gross-encapsulated multilocular mass, filled with viscous calcium
hydroxyapatite
• Fibrous septations Figure 4-9-2
• May have inflammatory elements
Tumoral Calcinosis: Radiology

[Figures 4-9-1 to 4-9-6]
• Calcified paraarticular mass
• Extensor surface
• Radiolucent septations (“chicken wire”)
• Extraarticular (bursae); no loss ROM
• Average 3 lesions/individual*
• CT/MRI: fluid-fluid levels (liquefied calcium)
➢ More active disease
• Bone scan-best for detection, and localization
• MRI – low signal T1W images
➢ Variable low to high signal T2W images Tumoral calcinosis about the knee
• Pseudoxanthoma elasticum-skin/vascular calcification,
retina angioid streaks
• CPPD arthropathy Figure 4-9-3
• Dental abnormalities-root enlargement, intrapulp calcification
• Marrow involvement - calcific myelitis
*Martinez, Radiology 1990;174:215
Clinical photograph in patient with

tumoral calcinosis about the knee
showing cosmetic deformity but no
decreased range of motion
Juxtaarticular Masses 792 Musculoskeletal Radiology

Tumoral calcinosis about the elbow extensor surface. Tumoral calcinosis about the hip
Contralateral elbow revealed identical findings (not with calcium fluid levels (arrowheads) on CT
shown)
Figure 4-9-6
Tumoral calcinosis about the shoulder with large calcified

periarticular mass (*) showing peripheral and septal
enhancement after contrast (arrowheads)
Periarticular Calcification: Differential Diagnosis

• Scleroderma
• Other collagen vascular diseases
• Chronic renal failure (secondary tumoral calcinosis)
• Milk - Alkali syndrome
Tumoral Calcinosis: Treatment

• Phosphate depletion therapy
• Surgical excision
Pigmented Villonodular Synovitis (PVNS): Clinical Features

• Proliferative disorder of synovium of joint, tendon or bursa
• Young adults 3rd and 4th decades
• Two types diffuse (15%–25%) and localized (75%–85%)
• Symptoms - pain, swelling, ROM loss
P V N S: Pathology
• Etiology unknown - inflammatory/neoplasm/trauma
• Variable degree of villous/nodular synovial proliferation and pigmentation
(hemosiderin) and inflammation components
• Giant cells, fibrous tissue, xanthoma cells
Musculoskeletal Radiology 793 Juxtaarticular Masses

P V N S: Location Figure 4-9-7
• Localized form – usually extraarticular
➢ Giant cell tumor tendon sheath (GCT-TS)
➢ Hand (80%), feet, knee (12%)
• Diffuse form - knee (60%–80%) hip, ankle, shoulder, elbow
GCT-TS: Radiology
• Second most common mass hand/wrist
• Lobulated soft tissue mass < 2 cm
• More common volar surface
• Osseous erosion uncommon 10%–15%
P V N S - Diffuse Form: Radiology

• Erosive bone lesions - 50% : hip (93%), shoulder (75%), knee (26%)
• Geographic IA lytic lesion - extrinsic erosion
• Joint effusion
• Arthrography - brownish or chocolate fluid, multinodular filling defects
P V N S: Radiology [Figures 4-9-7 to 4-9-11]

• Bone scan - mild increase activity (statics) Localized form of PVNS (giant
cell tumor of tendon sheath)
• Angiography - can show impressive vascularity
with volar thumb mass (*) on
• CT - soft tissue mass, increased attenuation sagittal T1-weighted MR image
• MRI – T1W image – low intensity mass T2W image – variable – usually
prominent low intensity regions
Same patient as previous MR image with intermediate signal PVNS of the hip (diffuse type) with radiograph showing
intensity in the giant cell tumor of tendon sheath (*) of the erosions on both sides of the joint (arrows) and maintained
thumb on axial T2-weighted MR joint space
Figure 4-9-10
PVNS hip (same patient as previous radiograph) with marked

low signal intensity tissue on coronal T2-weighted MR image (*)

Synovial Based Lesion: Figure 4-9-11
• PVNS/Synovial chondromatosis
• Arthritis - inflammatory
• Infection - unusual low-grade
• Amyloid
P V N S: Treatment and Results

• Surgical resection/synovectomy
• Recurrence rate
➢ GCT - TS (10%–20%)
➢ Diffuse form (40%–50%)
• Radiation - internal synovectomy - yttrium 90 and
dysprosium 165 PVNS knee (diffuse type) with large amount of low
signal intensity intraarticular hemosiderin laden
Soft Tissue Ganglion: Clinical Features tissue (*)
• Young adults (25–45 years old)
• Most common mass hand/wrist (60% of masses)
• Pain, tenderness or functional impairment (50%), rarely nerve palsy Figure 4-9-12
Soft Tissue Ganglion: Pathology

• Etiology - unknown - neoplasm, inflammation, trauma
• Thick walled unilocular/multilocular cystic spaces
• Gelatinous - mucinous fluid rich in hyaluronic acid and
mucopolysaccharides
Soft Tissue Ganglion: Radiology

[Figures 4-9-12 to 4-9-16]
• Soft tissue mass (1.5–2.5 cm) - dorsum hand/wrist
• Attached to tendon sheaths usually no communication with
joint
• Rarely cause adjacent bone erosion; periosteal reaction, wall
calcification
• CT/Sono/MRI - cystic mass
• May have higher attenuation on CT or signal T1W MR image –
high protein mucin
• Wall/septae may show mild enhancement
Large ganglion in the most frequent location

Figure 4-9-13 dorsal to the proximal carpal row with low to
intermediate signal intensity mass (*) on axial
T1-weighted MR image and homogeneous
high signal intensity on T2-weighting
Figure 4-9-14
Large ganglion in the most frequent location

dorsal to the proximal carpal row with anechoic
appearance on sonography (*)
Ganglion in Guyon canal with intermediate signal

intensity mass (*) on axial T1-weighted MR image
[upper image] and marked high signal intensity on
axial T2-weighted MR image (*) [lower image]
causing ulnar nerve entrapment symptoms

Myxoma: Clinical Figure 4-9-15 Figure 4-9-16
Features
• Location - heart,
subcutaneous,
intramuscular,
juxtaarticular
• Adults 40–70 years of
age
• Slightly more common
in women
• Painless palpable mass
Myxoma: Pathology
• Ovoid/globular whitish
appearance Intraarticular ganglion (*) in the knee on sagittal
• Contain gelatinous T2-weighted MR image with septations and
material Intraarticular ganglion in the knee on CT marked high signal intensity (same patient as
• Unusual to have cystic with low attenuation and multiple previous CT)
spaces septations (*)
• No fibrous capsule, but edema and muscle atrophy surround mass
Myxoma: Radiology [Figures 4-9-17 to 4-9-19] Figure 4-9-17

• Soft tissue mass - location - thigh, shoulder, buttock, upper arm
• Fluid characteristics CT/MRI
• Not simple fluid on sonography
• High protein material may increase CT attenuation or signal on T1W MR
image
• Peripheral rim may enhance with contrast or mild diffuse (57%) pattern
• Septations (43%) thick and mildly nodular regions
• Small rim of fat-like tissue CT (25%), MR (71%)
• Edema surrounding mass MR (79%)
• Rare to recur after removal (partial or complete)
Myxoma: Differential Diagnosis

• Abscess
• Chronic hematoma
• Ganglion/synovial cyst/bursa
• Other myxomatous neoplasms MFH/liposarcoma/neural tumors
Intramuscular myxoma in paraspinal
location on CT with low attenuation
mass (*) simulating a cyst
Figure 4-9-18
Figure 4-9-19
Paraspinal intramuscular myxoma (same

patient as previous CT) with low
echogenicity mass but some internal echoes
(*) are present suggesting that the lesion is
not cystic
Intramuscular myxoma in the forearm on sagittal MR imaging
with low signal intensity on T1-weighting and high signal
intensity on T2-weighting (*) simulating a cyst. However, the
intramuscular location, subtle rim of fat (arrowheads on T1)
and surrounding edema (arrows on T2) exclude a cyst as a
reasonable diagnostic consideration

Synovial Cyst: Definition Figure 4-9-20
• A herniation or continuation of the synovial membrane through the
joint capsule
Synovial Cyst: Location and Etiology

• Most commonly recognized - popliteal
• Shoulder, elbow, hip, hand, foot and ankle
• Types
➢ Primary - unknown cause - children
➢ Secondary - any cause joint distention
➢ Adults - rheumatoid arthritis
Synovial Cyst: Pathology

• Fluid filled may be multilocular
• Dense fibrous wall
• Lined by synovium
Popliteal Cyst - Baker Cyst [Figure 4-9-20]

• Often asymptomatic or pain from other causes
• Uncommon to present as mass
• May dissect in calf simulate DVT
• Imaging shows infiltration of calf (long fusiform lesion) caused by
extension of cyst with dissection and surrounding edema
• Results from communication between knee joint and gastrocnemius
– semimembranosus bursa
• Increase incidence with age – 50% autopsy series
• Incidence varies – arthrography (7% – 42%), sonography (15%),
MRI (5%)
Arthrogram showing the morphology of a
Synovial Cyst: Radiology [Figures 4-9-21 to 4-9-25 continues overleaf] ruptured popliteal cyst with long fusiform
shape and irregular margins caused by
• Fluid filled mass - Sono/CT/MRI
infiltration into surrounding muscle
• May have septations
• Arthrography can show joint communication Figure 4-9-21
• Can have solid components if complicated (rupture) with hemorrhage,
dissection or superimposed infection
• Contrast enhancement of rim/septae
➢ Noncomplicated – thin walls
• Contrast enhancement more complex in complicated/ruptured popliteal
cysts
• Complicated cysts difficult to exclude other causes of mass; must look at
morphology
• Imaging shows infiltration of calf (long fusiform lesion) caused by
extension of cyst with dissection and surrounding edema
Figure 4-9-22
Figure 4-9-23
Synovial cyst (popliteal) on CT with

low attenuation (*) and single
septation (arrowhead) in typical
location with neck between the
semimembranosus and
gastrocnemius tendons
Synovial cyst (popliteal) on axial T1-

weighted MR image with low signal Synovial cyst (popliteal - same patient as
intensity mass (*) and typical location previous MR) on axial T1-weighted post-
(neck between gastrocnemius and contrast MR image with peripheral/septal but
semimembranosis tendons-arrowheads) nonnodular enhancement (arrowheads)
as described in previous CT

Synovial cyst (popliteal - same patient as previous two MR’s)

on axial T2-weighted post-contrast MR image Ruptured popliteal cyst on sagittal T1 and T2-weighted MR
with diffuse high signal intensity (*) image with evidence of hemorrhage (*) and extensive
and neck extending into joint (arrowhead) surrounding edema both superiorly and inferiorly (arrowheads)
Meniscal Cyst: Clinical Features

• Adults (20–40 years); M>F ratio 2:1 Figure 4-9-26
• Cystic masses related to meniscal tears (1%–2% incidence)
• Fluid accumulates from joint through tear
• Pain at night or after exercise
Meniscal Cyst: Radiology

• Radiographs - soft tissue mass
• CT/SONO/MRI - fluid collection adjacent to meniscus
• Lateral > Medial 3–10:1 now more equal
• Medial - small cystic mass within or adjacent to meniscus
• Lateral - larger fluid collection filling potential space between
meniscus and collateral ligament
• MRI - best to evaluate meniscal tear and extension into cyst
• Must repair tear and resect cyst
Synovial Lipoma
• Two types Lipoma arborescens with villonodular fronds of
➢ Localized form fatty tissue (arrows) extending into the knee joint
➢ Diffuse form - lipoma arborescens on sagittal T1-weighted MR image
Synovial Lipoma: Localized Figure 4-9-27

• Rare - knee most frequent
• Solid fatty intraarticular mass
• Filling defect on arthrogram
• CT/MRI - lipomatous tissue
Lipoma Arborescens: Clinical Features

• Diffuse infiltration of synovium by fat
• Monoarticular - knee most common
• Often secondary (but can be primary) to chronic arthritis from
trauma or inflammatory disease
Lipoma Arborescens: Radiology

[Figures 4-9-26 and 4-9-27]
• Radiographs - soft tissue swelling
• Arthrography - multiple filling defects
• CT fatty infiltration Lipoma arborescens (same patient as previous
• MRI best to identify frond-like fatty projections MR ) showing high signal intensity fluid
surrounding fatty nodules (arrows) on sagittal
T2-weighted MR image

Synovial Chondromatosis: Clinical Features Figure 4-9-28
• Formerly synovial osteochondromatosis
• Cartilage metaplasia in synovium
• Knee (50%), hip, elbow, any joint can be involved
• M > F 2:1; 3rd to 6th decade
• Joint pain, decrease range of motion
Synovial Chondromatosis: Pathology

• Hyaline cartilage metaplasia in synovium
• Cartilage nodules (2–3 cm) can break away into joint, grow, reattach
to synovium
• Hypercellularity and nuclear atypia simulate cartilage malignancy
Synovial Chondromatosis: Radiology [Figures 4-9-28 to 4-9-32]

• Radiographs - calcified bodies (70%–75%), may ossify, extrinsic
erosions, joint widened, OA changes
• Bone scan - mild increased activity
• Arthrography - filling defects
• CT thickening about joint, effusion often small if present,
calcification/ossification Synovial chondromatosis wit multiple
• MRI - variable depending on degree of mineralization, some round filling defects on hip
hyperintensity T2W images arthrography. No calcification was
• Can also involve tendons and bursa seen on pre-arthrography radiographs
• Secondary chondromatosis - trauma, OA, RA, AVN, osteochondritis (not shown)
dissecans
Figure 4-9-29
Figure 4-9-30
Synovial chondromatosis of right hip with subtle

calcifications (arrowhead) difficult to detect on
Synovial chondromatosis of the shoulder with radiograph, although joint is widened (arrow)
innumerable calcified intraarticular
osteochondral bodies all similar in size and
shape on radiograph
Figure 4-9-32
Figure 4-9-31
Synovial chondromatosis of right hip (same patient

as previous radiograph and CT) with extensive high
signal intensity intraarticular tissue (*) but
Synovial chondromatosis of right hip (same patient as previous
calcification is not apparent on T2-weighted MR
radiograph) with multiple calcifications (arrowheads) about hip on CT
image

Synovial Chondromatosis: Treatment and Prognosis Figure 4-9-33
• Surgical synovectomy
• External radiation therapy
• Internal RT - nuclear medicine synovectomy?
• Rare degeneration into chondrosarcoma
Soft Tissue Chondroma: Clinical Features

• Less common than synovial chondromatosis
• 3rd and 4th decades, M>F
• Slow growing masses, painless
• Fingers (80%), hands, toes, feet, trunk
Soft Tissue Chondroma: Pathology

• Usually < 3 cm, often attached to tendon
• Mature hyaline cartilage lobular pattern
• Can show ossification
• Fibrous capsule not tenosynovium unlike synovial Soft tissue chondroma of the finger on
radiographs with large calcified mass
chondromatosis (arrowheads)
Soft Tissue Chondroma: Radiology

[Figures 4-9-33 and 4-9-34]
• Nonspecific soft tissue mass related to IP joint
• Also common in infrapatellar fat
• Chondroid matrix, can ossify
• Unusual to erode underlying bone
Heterotopic Bone Formation: Myositis Ossificans

• Young adults, M>F, usually trauma history
• No history trauma 25%–50%; also paraplegics
• Can involve muscles, fascia, tendons, subcutaneous fat
• Initially pain/tenderness and localized mass; pain decreases
with time Same patient: CT with large calcified mass
(arrowheads). Noncalcified portion is low
Heterotopic Bone Formation: Location attenuation consistent with a chondroid lesion
• Extremities - 80%, anterior compartments
• Lower extremity - quadriceps muscle
• Upper extremity - brachialis muscle
• Subcutaneous fat - 30% of cases
Figure 4-9-34
Same patient: MR imaging with high water content soft tissue

mass (arrowheads) consistent with a chondroid lesion
Soft tissue chondroma in Hoffa fat pad

on radiograph with large calcified mass

Heterotopic Bone Formation: Pathology Figure 4-9-35
• Zonal pattern of maturation
➢ Central immature osteoid/fibroblastic tissue
➢ Periphery calcifying osteoid to mature lamellar bone
• Cortical bone with further maturation
Heterotopic Bone Formation: Radiology

[Figure 4-9-35]
• Early soft tissue mass and edema
• Calcification 2–4 weeks then matures (zonal phenomena)
to central trabecular and peripheral cortical bone
• Usually separable from cortex but may be attached
• Bone scan marked increased activity
• Angiography - staining and neovascularity early
• CT – best to see early ossification pattern with peripheral
rim – enhances with contrast
Heterotopic Bone Formation: MR Imaging

[Figure 4-9-35]
• Early to intermediate
Heterotopic bone formation (myositis
➢ Normal with displaced fascial planes (T1W) ossificans) with peripheral rim of
➢ Increased intensity mass with prominent edema (T2W calcification on radiograph (arrow)
image)
• Late - heterogeneous well defined mass marrow fat on
T1W/T2W MR images, no edema, low intensity rim
• Often misinterpreted as malignant tumor
Heterotopic Bone Formation: Treatment and

Prognosis
• May resorb or be asymptomatic
• Resect after maturation (12–18 months)
• Premature resection - recurrence with vengeance
• Rare report malignant transformation
• Malignant myositis (mucinous carcinoma )
Nodular Fasciitis: Clinical Features

• Very common; most frequent tumor-like lesion fibrous Same patient: peripheral rim of
tissue calcification (arrow) separated from
femoral cortex on CT
• Rapidly growing mass 1–2 weeks duration
• Young adults (20–35 years), M<F
• History trauma (10%–15%)
Nodular Fasciitis: Location

• Upper extremity (50%) – volar forearm
• Trunk - chest wall and back
• Head and neck in children
• Rare hand/feet/lower extremity
Nodular Fasciitis: Pathology

• Subcutaneous type (70%) - soft tissue nodule
• Intramuscular type (15%) - not circumscribed multinodular
• Immature fibroblasts in irregular fascicles
Same patient: axial T2-weighted MR
• Reticulin meshwork, collagen minimal, inflammatory and image with heterogeneous mass
mucoid component (arrow) suggesting a more aggressive
neoplastic process as peripheral
calcification is less apparent

Nodular Fasciitis: Radiology and Treatment Figure 4-9-36
Figure 4-9-36]
• Nonspecific soft tissue mass; may show fascial extensions
• CT/MRI-mass with irregular margins and heterogeneous on MRI,
surrounding edema
• Suggests malignancy imaging and pathology
• Surgical resection-recurrence rare (1%–2%) even if incomplete
Synovial Sarcoma: Clinical Features

• Malignant mesenchymal tumor
• Young adults 15–40 years of age
• Fourth to fifth most common soft tissue sarcoma
• Painful deep soft tissue mass
• Often indolent slow growing mass (4 years to diagnosis)
Musculoskeletal Soft Tissue Sarcoma: Incidence

• MFH/Fibrosarcoma 20%–30% Nodular fasciitis of the forearm on
coronal STIR MR image with a
• Liposarcoma 16%–19% high signal intensity subcutaneous
• Rhabdomyosarcoma 10%–19% mass (*) with linear fascial
• Nonspecific spindle cell sarcoma 5%–15% extensions (fascial tail sign
• Leiomyosarcoma 5%-10% arrows) both superiorly and
• Dermatofibrosarcoma protuberans (DFSP) 5%-10% inferiorly
• Synovial sarcoma 5%–10% Figure 4-9-37
Synovial Sarcoma: Location
• Extraarticular adjacent to tendons bursa, ligaments > 90%
• Intraarticular < 10%
• Lower extremity 60% - around knee
• Upper extremity 25% - around wrist
Synovial Sarcoma: Pathology

• Two cell lines
➢ Epithelial (keratin positive)
➢ Spindle cells
• Monophasic / biphasic
• Variable - calcification, hemorrhage
Synovial Sarcoma: Radiology [Figures 4-9-37 to 4-9-40]

• Radiographs - normal (50%) or nonspecific soft tissue mass near joint
• Bone erosion or periosteal reaction (11%–20%), bone invasion (5%)
• Soft tissue calcification up to 30% - best by CT
Synovial sarcoma of the foot with
• Bone scan - increased activity indolent appearing extrinsic
• MRI erosions on radiograph (arrow)
➢ T1W images - similar to muscle
➢ T2W images - usually high intensity Figure 4-9-38
➢ Triple sign on T2W MR (35-57%) (nonspecific)
➢ Very heterogeneous (bowl of grapes)
• Not uncommonly well defined with pseudocapsule - simulates benign
characteristics
• Fluid - fluid level 10%–25% (hemorrhage) worse prognosis in highly
vascular lesions
Synovial Sarcoma: Treatment and Prognosis

• Surgical resection/amputation
• Radiation therapy/chemotherapy
• Local recurrence 30% – 50%
• 5 year survival 36% – 76%; 10 year survival 20% – 63%
• Metastases (16%-25%) – lung (94%), lymph node (10%), marrow
Synovial sarcoma about elbow with

calcification (arrowhead) in mass near
but not in the joint on radiograph

Clear Cell Sarcoma: Clinical Features Figure 4-9-39
• Malignant melanoma of soft parts
• Arise in tendons/aponeurosis
• Deep tissue without skin involvement
• Foot/ankle (43%), knee, thigh, hand
• Adults 20–40 years; F>M
Clear Cell Sarcoma: Pathology

• Cells with clear cytoplasm
• Framework of fibrocollagenous tissue
• Intracellular melanin 60%–75%
• Hemosiderin also present
Clear Cell Sarcoma: Radiology [Figure 4-9-41]

• Soft tissue mass at/in tendon/aponeurosis
• Bone erosion/destruction
• CT/MRI - infiltrative soft tissue mass
• MRI
➢ T1W image - intermediate intensity
Synovial sarcoma about the ankle on coronal T1-
➢ T2W image - may be low intensity
weighted MR image with heterogeneous
hemorrhagic mass (*) invading bone (arrowhead)
Clear Cell Sarcoma: Treatment and Prognosis
• Surgical resection/radiation/chemotherapy Figure 4-9-40
• Poor prognosis
• Local recurrence and metastases
• Mets - lungs, lymph nodes, bone
Figure 4-9-41
Synovial sarcoma about the ankle (same

patient as previous MR) on axial T2 -
weighted MR image with heterogeneous
multicompartment mass
Clear cell sarcoma on MR imaging with

origin in the quadriceps tendon as
evidenced on the axial image with low signal
intensity both anterior and posterior to the
mass (arrowheads). Sagittal T2-weighted
MR shows nonspecific intermediate signal
intensity

Noncalcified Juxta/Intraarticular Soft Tissue Masses
• Synovial/Meniscal cyst
• Ganglion/myxoma
• Gouty tophus
• Hemangioma/PVNS
• Lipoma
Calcified juxta/Intraarticular Soft Tissue Mass

• Aneurysm
• Gouty tophus
• Hyperparathyroidism/hemangioma
• Osteochondromatosis (synovial)
• Tumoral calcinosis
• Soft tissue sarcoma
References
1. Al-Nakshabandi NA, Ryan AG, Choudur H, Torreggiani W, Nicoloau S, Munk PL, Al-Ismail K. Pigmented villonodular
synovitis. Clin Radiol. 2004 May;59(5):414-20. Review.
2. Kransdorf MJ, Meis JM, Jelinek JS. Myositis ossificans: MR appearance with radiologic-pathologic correlation.
AJR Am J Roentgenol. 1991 Dec;157(6):1243-8.
3. Martinez S, Vogler JB 3rd, Harrelson JM, Lyles KW. Imaging of tumoral calcinosis: new observations. Radiology. 1990
Jan;174(1):215-22.
4. Murphey MD, Choi JJ, Kransdorf MJ, Flemming DJ, Gannon FH. Imaging of osteochondroma: variants and
complications with radiologic-pathologic correlation. Radiographics. 2000 Sep-Oct;20(5):1407-34. Review.
5. Murphey MD, McRae GA, Fanburg-Smith JC, Temple HT, Levine AM, Aboulafia AJ. Imaging of Soft Tissue Myxoma
with Emphasis on CT and MRI and Comparison of Radiologic and Pathologic. Radiology 2002; 225:215-224.
6. Ortega R, Fessell DP, Jacobson JA, Lin J, Van Holsbeeck MT, Hayes CW. Sonography of ankle Ganglia with
pathologic correlation in 10 pediatric and adult patients. AJR Am J Roentgenol. 2002 Jun;178(6):1445-9.
7. Robinson P, White LM, Kandel R, Bell RS, Wunder JS. Primary synovial osteochondromatosis of the hip: extracapsular
patterns of spread. Skeletal Radiol. 2004 Apr;33(4):210-5. Epub 2004 Feb 18.
8. Steinbach LS, Johnston JO, Tepper EF, Honda GD, Martel W. Tumoral calcinosis: radiologic-pathologic correlation.
Skeletal Radiol. 1995 Nov;24(8):573-8.
9. Tschirch FT, Schmid MR, Pfirrmann CW, Romero J, Hodler J, Zanetti M. Prevalence and size of meniscal cysts,
ganglionic cysts, synovial cysts of the popliteal space, fluid-filled bursae, and other fluid collections in asymptomatic
knees on MR imaging. AJR Am J Roentgenol. 2003 May;180(5):1431-6.
10. Valenzuela RF, Kim EE, Seo JG, Patel S, Yasko AW. A revisit of MRI analysis for synovial sarcoma. Clin Imaging.
2000 Jul-Aug;24(4):231-5.

Musculoskeletal Angiomatous Lesions
Mark D. Murphey, MD
Angiomatous Lesions
• Hemangioma
• Lymphangioma
• Glomus Tumor
• Angiomatosis and associated syndromes
• Hemangiopericytoma
• Angiosarcoma
Osseous Hemangioma: Clinical Characteristics

• M > F (2:1); 4th – 5th decade
• Majority asymptomatic
• May have soft tissue components
• Common sites : vertebral body (11% of spines), calvarium
Soft Tissue Hemangioma: Clinical Characteristics

• 7% of all benign S.T. neoplasms
• 1.5% of the general population
• Most frequent S.T. neoplasm in children
Figure 4-10-1
• More common in young women – may increase in
size with pregnancy
• Can be subcutaneous, intramuscular or synovial
Hemangioma: Pathology
• Subtype based on predominant vascular
component – but usually mixed tumor
• Capillary – most common – first years of life –
skin, subcutaneous, vertebrae (low flow)
• Cavernous – childhood – larger and deeper
(low flow)
• Arteriovenous – deep or superficial – persistent
fetal capillary bed (high flow)
• Venous – adults – deep involvement –
retroperitoneum, mesentery or extremities Vertebral hemangiomas with thickened vertical trabeculae
(low flow) (arrows-corduroy appearance) on radiograph and coronally
• Epithelioid – dermis/subcutis sectioned gross specimen (different patients)
Osseous Hemangioma: Radiology Figure 4-10-2

[Figures 4-10-1 to 4-10-5]
• Vertebrae – focal or diffuse – vertical striations
(corduroy or polka dot) posterior element
involvement more likely symptomatic
• Calvarium/mandible radiating web-like trabecular
pattern
• Long bone - multifocal lytic honeycomb pattern,
cortical lesions/erosions in diaphysis
• Bone overgrowth
• Arthritis from intraarticular bleeding
Vertebral hemangioma (asymptomatic) of lumbar spine with

"polka dot" appearance and fat between trabeculae on CT
Musculoskeletal Radiology 805 Musculoskeletal Angiomatous Lesions

Vertebral hemangioma (symptomatic patient) with "polka dot"

appearance on CT (arrowheads) and soft tissue extension.
Sagittal T1 and T2-weighted MR images show vertebral
fracture and spinal canal compromise caused by anterior
epidural soft tissue component (arrows) but diagnostic
trabecular thickening is difficult to appreciate
S.T. Hemangioma Radiology: Calvarial hemangioma with spoke wheel pattern of trabecular
S.T. Changes [Figure 4-10-6] thickening (arrows) on radiograph, CT and vascular
• Calcification – curvilinear or amorphous, phlebolith channels/spaces on gross specimen (arrowheads)
(30%-50% of lesions)
• Angiography – irregular enlarged feeding arteries, Figure 4-10-5
contrast pooling, arteriovenous shunting
• Venous lesions seen only with venography
S.T. Hemangioma: Imaging

[Figures 4-10-7 to 4-7-12]
• T1W images – low to intermediate heterogeneous
mass; look for fat overgrowth
• Very high intensity T2W images (low flow)
• Serpentine vessels / cavernous spaces may help
distinguish types; high vs. low flow
• Enhance with contrast Femoral hemangioma on coronal T1-weighted MR image
• Phleboliths – CT > MRI before and after contrast shows multifocal round areas of
• Bone scan – often only limited activity marrow replacement (arrowheads) representing vascular
• Sonography – solid mass Doppler may show low channels with enhancement and serpentine feeding vessels
resistance flow
Figure 4-10-6
Figure 4-10-7
Soft tissue hemangioma of the hand with phleboliths on

radiograph (arrows) and intraoperative photograph
Soft tissue hemangioma of axilla (intramuscular and

cavernous) on CT showing enhancing vascular channels
(large arrows), fat overgrowth (small arrows), and phlebolith
(arrowhead)
Musculoskeletal Angiomatous Lesions 806 Musculoskeletal Radiology

Soft tissue hemangioma of the thigh on sagittal T1-weighted

Soft tissue hemangioma of forearm (intramuscular and cavernous) on (right image) and axial STIR (left image) MR images with
sagittal T1-weighted MR images before and after gadolinium showing associated fat atrophy (arrowheads) and slow flow circular
intermediate signal intensity serpentine vascular channels and spaces vascular spaces (arrow) corresponding to the gross specimen
(arrows) that enhance following contrast and fat overgrowth and histology [4-10-10]
(arrowheads). Axial T2-weighted MR reveals multiple circular high
signal areas corresponding to slow flow cavernous spaces (*) Figure 4-10-11
Figure 4-10-10 Soft tissue hemangioma
(high flow arteriovenous)
about knee with low
signal intensity
serpentine vessels
(arrows) on coronal T2-
weighted MR image
Figure 4-10-12
Soft tissue hemangioma (arrows) with

associated fat atrophy (arrowheads) in
surrounding thigh muscle on gross specimen
(left image). Histology (right image) reveals
Capillary hemangioma
phleboliths (*) with calcification peripherally
on T2-weighted MR
(arrows) and fat atrophy of muscle
image (arrow) with
(arrowheads)
nonspecific high signal
intensity in the face
with typical extensive
strawberry nevus
clinically. No
characteristic features
of fat overgrowth or
serpentine vascular
structures are seen to
suggest hemangioma as the vessels in this type of lesion are to small
(capillary) to discern on imaging as demonstrated on the histology

Hemangioma: Treatment Figure 4-10-13
• Surgical resection / laser therapy
• Embolization
• Radiation in symptomatic unresectable lesions – spine
• Vertebroplasty
• Recurrence (15% – 30%) – large lesions
Lymphangioma:
• Rare lesion in bone, usually S.T.
• Often present at birth (50% – 65%)
• 90% apparent by age 2 years
• Head, neck, axilla – 75% of cases
• Soft fluctuant mass
Lymphangioma of the neck on CT with a
Lymphangioma: Pathology homogeneously low attenuation mass (*)
• Sequestrated noncommunicating lymphoid tissue
• Large multiloculated cystic spaces
• Lined by lymphatic endothelium
• Filled with proteinaceous material Figure 4-10-14
Lymphangioma: Radiology [Figures 4-10-13 and 4-10-14]

• Radiographs – soft tissue mass
• Imaging – large cystic spaces less common serpentine
component may appear complex – solid components (high
signal on T1 25%)
• Cystic hygroma – hydrops fetalis, Turner syndrome
Glomus Tumor [Figure 4-10-15]

• Patients 4th to 5th decade
• Tumor of neuromyoarterial glomus
• Almost all terminal phalanx soft tissue
• Bone erosion/invasion 15%–65%
Angiomatosis
• Multifocal or diffuse infiltration of bone by hemangiomatous or Lymphangioma of the neck in an infant on
lymphangiomatous lesions with or without soft tissue coronal T1-weighted MR image with
involvement heterogeneous mass (arrow) showing both
high and low signal intensity areas extending
Angiomatosis: Clinical Characteristics along the chest wall (arrowhead)
• Young patients – first 3 decades
• M > F (2:1)
• Osseous involvement only – benign course
• Visceral involvement – poor prognosis Figure 4-10-15
• No malignant potential
Angiomatosis: Pathology
• Capillary or cavernous hemangioma’s
• Lymphangioma’s – lymphatic backflow
• Mixed vascular lesion difficult to distinguish
Glomus tumor with erosion of the terminal phalanx of the long

finger on radiograph (arrows) and sagittal macrosection (*)

Angiomatosis: Radiology [Figure 4−10-16]
• MR imaging/CT – some as solitary angiomatous lesions more extensive
• Imaging to evaluate visceral involvement/extent Figure 4-10-16
• Lymphangioma – proven with lymphangiography
and contrast in lesion
• Diffuse round/oval medullary lytic lesions
• May have sclerotic margins
• Location: femur, ribs, spine, pelvis, humerus,
scapula, other long bones, clavicle
Angiomatous Syndromes
• Maffucci syndrome
• Osler – Weber – Rendu
• Klippel – Trenaunay – Weber
• Massive osteolysis of Gorham
• Associated osteomalacia and thrombocytopenia
Maffucci Syndrome
Angiomatosis (lymphangiomatosis) with extensive infiltration of
• Multiple enchondromata the entire lower extremity causing elephantiasis on coronal T1-
• Cavernous soft tissue hemangiomata weighted MR image and clinical photograph
• Often hands/feet, unilateral predominance
• Malignant potential both lesions and viscera
Osler-Weber-Rendu Figure 4-10-17

• Hereditary hemorrhagic
telangiectasia
• Dilated capillaries and veins
• GI, GU, lung, spinal; bone – rare
Klippel-Trenaunay-Weber
[Figure 4-10-17]
• Nonhereditary, lower extremity
• Unilateral cutaneous capillary
hemangioma
• Varicose veins and local gigantism Klippel-Trenaunay-Weber syndrome on clinical photograph and coronal T1-
• Can have arteriovenous component weighted and T2-weighted MR images showing classic triad of
hemihypertrophy,varicose veins and extensive predominantly slow flow
Massive Osteolysis of Gorham: angiomatous lesion (arrowheads). Smaller high flow component is also seen on
“Vanishing Bone Disease” T2-weighted image (arrows)
[Figure 4-10-18]
• Patients < age 40 years
• History trauma 50% Figure 4-10-18
• Upper extremity favored, may extend across joint
• Progressive bone resorption and fragmentation
(simulate neuropathic)
• Pathology - proliferating vascular channels
Osteomalacia and Thrombocytopenia

• Tumor induced osteomalacia: most frequent
vascular lesions
• Hemangioma / hemangiopericytoma
• Kasabach - Merritt syndrome - hemangioma/
hemangiopericytoma associated with
thrombocytopenia and purpura
Gorham vanishing bone disease involving the foot with

radiograph and gross specimen showing extensive sharply
defined bone resorption (arrowheads and arrow)

Intermediate to Malignant Musculoskeletal Angiomatous Figure 4-10-19
Lesions
• Hemangiopericytoma
• Angiosarcoma
Hemangioendothelioma (HE)
• Intermediate – benign or malignant
• Composed of vascular endothelial cells
• Often in young patients
• Bone or soft tissue
• Locally aggressive, unusual to metastasize
Hemangiopericytoma (HPC)
• Intermediate – benign or malignant
• Tumor of cells around vessels – pericytes
• Tumor of middle-aged adults
• Sites – soft tissue of thigh, pelvis and retroperitoneum
• Rare in bone
Angiosarcoma (ASC)
• Malignant; M > F (2:1)
• Composed of hemangiosarcoma or lymphangiosarcoma cellular
elements
• Location: skin, muscular, viscera, bone
• Associated with lymphedema post-mastectomy (Stewart-Treve
syndrome) Malignant hemangioendothelioma of bone with
multifocal lytic lesions (arrowheads) in the tibia
and fibula on radiograph and osseous
Osseous HE, HPC, ASC: Skeletal Location replacement by hemorrhagic tissue (*) on
• Hemangioendothelioma: skull, vertebrae, lower extremity photograph of coronally sectioned gross
• Hemangiopericytoma (rare): pelvis, proximal long bones, specimen
vertebrae, mandible
• Angiosarcoma: long tubular bone lower extremity
Osseous HE, HPC, ASC : Figure 4-10-20

Radiographic Findings T1
[Figure 4-10-19]
• Multifocal lytic lesions – honeycomb appearance
• Aggressive bone destruction with expansion and T2
soft tissue mass
Radiology of HE, HPC, ASC : T1 GD

Advanced Imaging
[Figures 4-10-20 to 4-10-21]
• Angiography – intensely vascular with peripheral
vessels displaced by tumor early, dense blush late
• Sonography – hypo or hyperechoic mass
Doppler – arteriovenous shunting
• MRI
➢ T1W – usually similar to muscle
➢ Can be high intensity hemorrhage
• Look for prominent serpentine vessels
• Fluid - fluid levels, contrast enhancement
• Dominant skin mass in chronic lymphedema (ASC)
Angiosarcoma developing in a patient with chronic leg

lymphedema. Axial T1-weighted, T1-weighted post contrast
and T2-weighted MR images show the enlarged leg with
subcutaneous edema (arrows) and dominant skin mass
(arrowheads) representing the angiosarcoma.
The superficial angiosarcoma is also seen on the clinical
photograph (grey arrows)

Figure 4-10-21
T1 T2
Hemangiopericytoma in of the thigh showing high flow vessels (arrows) in the soft tissue mass (*) and
feeding the lesion on both axial T1-weighted and coronal T2-weighted MR images. Photograph of the
sectioned gross specimen also shows the soft tissue mass (*) and the high flow vessels (arrowheads)
Cannot distinguish HE, HPC or ASC from other soft tissue masses
if prominent serpentine vessels are not recognized
Hemangioendothelioma, Hemangiopericytoma and Angiosarcoma

cannot be differentiated from each other radiologically
Distinction of HE, HPC and ASC from Hemangioma

• Large masses
• Aggressive characteristics with infiltration
• No fat overgrowth
Treatment and Prognosis: HE, HPC and ASC

• Malignant lesions – radiation and chemotherapy
• Local recurrence common
• Metastases common to lung in ASC
Summary:
Musculoskeletal Angiomatous Lesions
• Osseous – Multifocal bone lysis – Honeycomb appearance
• Look for serpentine vascular pattern – MRI
• Overgrowth of fat – MRI
• Multiple associated syndromes and angiomatosis
• Higher grade lesions – HE, HPC, ASC
➢ Larger aggressive lesions
➢ Infiltrative characteristics
References
1. Baudrez V, Galant C, Vande Berg BC. Benign vertebral hemangioma: MR-histological correlation. Skeletal Radiol.
2001 Aug;30(8):442-6.
2. Coldwell DM, Baron RL, Charnsangavej C. Angiosarcoma. Diagnosis and clinical course. Acta Radiol. 1989 Nov-
Dec;30(6):627-31.
3. Fayad L, Hazirolan T, Bluemke D, Mitchell S. Vascular malformations in the extremities: emphasis on MR imaging
features that guide treatment options. Skeletal Radiol 2006; 35:127-137.
4. Laredo JD, Assouline E, Gelbert F, Wybier M, Merland JJ, Tubiana JM. Vertebral hemangiomas: fat content as a sign
of aggressiveness. Radiology. 1990 Nov;177(2):467-72.
5. Lorigan JG, David CL, Evans HL, Wallace S. The clinical and radiologic manifestations of hemangiopericytoma.
AJR Am J Roentgenol. 1989 Aug;153(2):345-9.
6. Murphey MD, Fairbairn KJ, Parman LM, Baxter KG, Parsa MB, Smith WS. From the archives of the AFIP.
Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics. 1995 Jul;15(4):893-917.

Paget Disease
Mark D. Murphey, MD Figure 4-11-1
Paget Disease: Clinical Characteristics

• Described 1877 by Sir James Paget
• Osteitis deformans
• Common disease
➢ 3% over age 40
➢ 10% over age 80
• Slightly more common in men
• Common in Great Britain and descendents (USA,
Australia)
➢ Continental Europe
• Uncommon in Asia
• Many patients asymptomatic (20%)
Paget Disease: Clinical Presentation Marrow replacement by fibrovascular tissue (*) in active Paget
• Pain disease (left image) versus fat (F) in marrow with inactive
disease (right image)
• Osseous bowing and enlargement
• Neurologic symptoms Figure 4-11-2
• High output congestive failure
• Lab
➢ Serum alkaline phosphatase (blastic phase)
➢ Urinary and blood hydroxyproline (lytic phase)
Paget Disease: Etiology

• Unknown
• Possible etiologies
• Infection; viral; intranuclear inclusions; paramyxovirus
(measles)
• Autoimmune
• Connective tissue disease
• Neoplastic
Paget Disease: Pathology [Figure 4-11-1]

• Initially osteoclastic resorption
• Subsequently osteoblastic response (active)
➢ Excessive and disorganized
➢ “Mosaic or jigsaw” pattern
➢ Marrow: fibrovascular reaction
➢ Marrow: fat (inactive)
Paget Disease: Skeletal Distribution [Figure 4-11-2]

• Calvarium, spine (lumbosacral) and pelvis: 25%-75%
• Proximal long bones: 25%–30%
• Humerus (31%), scapula (24%), clavicle (11%)
• Initially monostotic 10%–35%: most polyostotic
Paget Disease: Radiologic Evaluation

• Radiographs – diagnosis
• Bone Scan – assess areas involved
• CT/MRI: to assess complications or unusual cases
Paget Disease 812 Musculoskeletal Radiology

Paget Disease: Radiologic Stages
• Active
➢ Lytic - osteoclastic activity
➢ Mixed - majority of cases
• Inactive
➢ Blastic - osteoblastic activity
• Usually progresses through these phases but not always
➢ Recrudescent lytic phase in patients at rest; simulates tumor
Paget Disease: Radiologic Lytic Phase [Figures 4-11-3 to 4-11-5 ]

• Skull: large well-defined areas; involve both inner and outer tables of
frontal/occipital bones (osteoporosis circumscripta)
• Long Bones: subchondral location with advancing wedge/V shape “blade of
grass/candle flame”
Figure 4-11-3
Figure 4-11-4
Figure 4-11-5
Lytic phase of Paget disease with sharp margins and subchondral extension
Osteoporosis circumscripta with sharply

marginated large area of bone lysis
Musculoskeletal Radiology 813 Paget Disease

Paget Disease: Radiographs – Mixed/Blastic Disease Figure 4-11-6
• Sclerosis and lucency
• Trabecular and cortical thickening
➢ Along the lines of stress
➢ But some disorganization
• Bone enlargement
Paget Disease: Radiographs: Mixed/Blastic Disease

[Figures 4-11-6 to 4-11-9]
• Skull: “cotton wool” appearance obscures inner and outer
tables, often spares facial bones
• Spine: vertebral body (picture frame); ivory vertebral body;
posterior elements may be involved “Cotton wool” appearance on radiograph with
multifocal areas of sclerosis and thickening of
Figure 4-11-7 the diploic space anteriorly
Figure 4-11-8
Picture frame appearance of Paget disease of the spine,

multiple levels, on radiograph and coronal macrosection
Figure 4-11-9
Mixed lytic/blastic Paget disease in skull with

diploic space expansion and hyperemic bone
on CT, gross specimen and histology
Ivory Vertebral Body:
• Blastic metastasis: breast,
prostate, adenocarcinoma GI
tract, carcinoid, transitional cell
carcinoma bladder
• Lymphoma
• Chronic infection Ivory vertebral body in Paget disease on radiograph and
• Chordoma intense uptake on radionuclide bone scan
Paget Disease: Radiographs Mixed/Blastic Phase

[Figures 4-11-10 and 4-11-11]
• Pelvis: asymmetric involvement
➢ Thickened iliopubic and ischial lines
➢ Enlarged pubic rami and ischium
• Long Bones: epiphyseal involvement
➢ Rarely diaphyseal (tibia)
➢ Enlarged bone

Paget disease mixed lytic and blastic in left

pubic rami
Paget disease (noncomplicated) in right

iliac crest with trabecular and cortical
thickening (arrowheads)
Paget disease with coarsened
trabecular pattern and thickening of
the iliopectineal line involving the
entire hemipelvis
Paget Disease: Bone Scintigraphy
• Active disease – marked uptake
Figure 4-11-13
➢ Dynamic and static images T1
• Abnormal before radiographs
• Overview of disease – look at distribution
• Monitor disease and therapy
Paget Disease: CT/MRI Noncomplicated

Diseases
[Figure 4-11-12]
• Not usually needed for diagnosis
• CT- thickened trabeculae
➢ Bone enlargement Paget disease involving the calcaneus (noncomplicated),
mixed lytic/blastic on radiograph, with maintained yellow
➢ Lytic areas
marrow on T1- weighted MRI
Paget Disease: MRI - Noncomplicated Cases

[Figures 4-11-13 to 4-11-16]
➢ Cortically trabecular thickening
➢ Enlarged bone Figure 4-11-14
➢ Low signal (sclerosis)
➢ Yellow marrow/fat (inactive disease)
➢ Heterogeneous signal (active disease)
➢ Marrow replacement non-masslike
➢ Low signal (sclerosis)
➢ Fat signal intensity (inactive disease)
➢ Heterogeneous intermediate/high signal (fibrovascular marrow -
active disease)
➢ No focal mass
Paget disease mixed lytic/blastic

with cortical thickening (arrow)
involving the tibia

T1 T1 GD T2
Coronal T1-weighted (noncontrast) and axial T1-weighted (after contrast) MR Axial T2-weighted MR image shows
images show speckled marrow pattern with enhancement in more active Paget speckled pattern of increased intensity (*)
disease (noncomplicated) (*) and more intense enhancement of the intracortical in more active Paget disease
component that is most active (noncomplicated-same patient as previous
MRI and radiograph)
Paget Disease: Complications Figure 4-11-17
• Osseous deformity
• Fractures
• Neurologic symptoms
• Arthropathy
• Neoplasm
Paget Disease: Osseous Deformity

[Figure 4-11-17]
• Effects of bone softening
• Bowing – common in long bones
• Acetabulae protrusio
• Basilar invagination – 30% of patients with skull involvement
Paget Disease: Fractures [Figure 4-11-18]

• Partial or complete (insufficiency)
• True acute fractures
• Horizontal lucencies (“banana fracture”)
• Convex surface or bone Acetabulae protrusio on radiograph and coxa
• Single or multiple varus deformity of the femur on the coronally
• Often symptomatic sectioned macrosection in patients with Paget
• Sites: femur, humerus, pelvis disease. Note the axial narrowing of the hip joints
• Spine: central compressions
• May heal but high nonunion rate Figure 4-11-18
• At risk for sarcoma (biopsy?)
Paget Disease: Neurologic Symptoms

[Figure 4-11-19]
• Symptoms – impingement of cranial and spinal nerves
• Caused by skull and spine involvement
• Bone enlargement, fractures, bone softening with basilar invagination
and increased osseous vascularity with cord hypoxia
• CT/MRI for evaluation
Paget disease of femur with complete

and incomplete fractures (arrowheads)

Paget Disease: Arthropathy Figure 4-11-19
• Rheumatic diseases with poor association: rheumatoid arthritis,
calcium pyrophosphate deposition (CPPD),
ankylosing spondylitis
• Gout – higher incidence hyperuricemia (40%) from increased
cell turnover
• Osteoarthritis
➢ Hip and knee most common
➢ Abnormal mechanics from deformity
➢ Bone weakening
➢ Hip narrowing can be axial
• Soft tissue calcification
➢ Tendinitis and with vitamin D treatment
Paget Disease: Neoplasm

• Sarcomatous transformation
➢ 1% patients with Paget disease
➢ 5% - 10% patients with extensive disease
• Patients 55 to 80 years old
• Common sites – femur, pelvis, humerus
• Osteosarcoma (50% - 60%)
• MFH/fibrosarcoma (20% - 25%)
• Chondrosarcoma (10%)
• GCT Paget disease at C2 (*) with marked osseous
• Metastasis, myeloma, lymphoma enlargement
Paget Disease: Neoplasm Radiology Figure 4-11-20

[Figures 4-11-20 to 4-11-22]
• Bone destruction predominates
• Cortical involvement and soft tissue mass
• No periosteal reaction
• Persistent nonhealing fracture
• Compare with old radiographs
• Bone scan: photopenic area
• Gallium scan: increased uptake
• MRI
➢ T1W: masslike marrow replacement
➢ T2W: focal mass of high intensity
➢ Soft tissue mass
Figure 4-11-21
Pictoral representation of malignant

transformation in Paget disease
Paget disease with malignant transformation to

osteosarcoma in the proximal tibia where there
is mass-like marrow replacement (*) and soft
tissue extension (arrow) on radiograph and
multiple sagittal T1-weighted MR images

Paget Disease: Neoplasm [Figure 4-11-23] Figure 4-11-22
• GCT – skull or facial bones
➢ More often benign
➢ Rarely multiple (familial)
➢ Lytic expansile lesion
• Metastasis – likely related to
hyperemia
Paget Disease:
• Diffuse sclerosis – chronic renal
failure (CRF), myelofibrosis,
metastasis, lymphoma, sickle cell
anemia
• Trabecular thickening – hemangioma,
chronic infection, osteomalacia,
fluorosis
• Polyostotic lesions – CRF
(hyperparathyroidism), Langerhans
Paget disease with malignant transformation to osteosarcoma in the humeral
cell histiocytosis, unusual infection, midshaft where there is mass-like marrow replacement (*) and soft tissue
metastasis, fibrous dysplasia, extension (arrows) on radiograph and multiple sagittal T1-weighted MR
lymphoma, Gaucher, mastocytosis images, CT and gross specimen
Figure 4-11-23
Paget Disease: Treatment
• Calcitonin – inhibits resorption
• Biphoshonates – inhibits bone
resorption and production
• Mithramycin – cytotoxic antibiotic
• Often relieve pain
Paget Disease:
Radiology Post-Treatment
• Often subtle or no change
• Occasionally improved radiographs
• Watch for fractures: may increase
with diphosphonates
• Bone scans best treatment indicator
Hereditary Hyperphosphatasia:
Juvenile Paget Disease
• Described 1956: Bakwin/Elger Paget disease with benign giant cell tumor of the clavicle associated with
• Autosomal recessive pathologic fracture showing cortical thickening (arrows) and destructive lesion
(*) on CT scans, gross specimen radiograph and gross specimen
• Disorder of infants/children
• Elevated alkaline and acid phosphatase, uric acid
Figure 4-11-24
Juvenile Paget Disease: Radiographic Findings
[Figure 4-11-24]
• Generalized cortical thickening
• All bones including skull involved
• Osteopenia and bowing
• Epiphyses may be spared
• Patients severely deformed
Juvenile Paget disease (hereditary

hyperphosphatasia) on radiographs with
osseous bowing and shortening as well as
trabecular thickening

Paget Disease: Summary
• Common disease: 3% - 4%
• Diagnosis: Radiographs
➢ Lytic: well defined, subchondral (v/wedge shape – candle flame)
➢ Thickened trabeculae and cortex
➢ Osseous enlargement
• Bone scan – overview
• Complications – fractures, osseous deformity, neurologic symptoms, arthritis, neoplasm
• CT/MRI to evaluate complications
• CT/MRI: noncomplicated case
➢ Bone enlargement
➢ Trabecular thickening
➢ T1W: low signal (sclerosis), yellow marrow, heterogeneous non-masslike marrow replacement
➢ T2W: low signal (sclerosis), yellow marrow, heterogeneous high signal, no focal mass
• CT/MRI: complicated case
➢ CT: focal bone destruction / soft tissue mass
➢ T1W: masslike marrow replacement
➢ T2W: focal mass in marrow with high signal and soft tissue mass
References
1. Boutin RD, Spitz DJ, Newman JS, Lenchik L, Steinbach LS.. "Complications in Paget disease at MR imaging."
Radiology. 1998 Dec;209(3):641-51.
2. Cooper C, Dennison E, Schafheutle K, Kellingray S, Guyer P, Barker D.. "Epidemiology of Paget's disease of bone."
Bone. 1999 May;24(5 Suppl):3S-5S.
3. Moore TE, Kathol MH, el-Khoury GY, Walker CW, Gendall PW, Whitten CG.. "Unusual radiological features in
Paget's disease of bone." Skeletal Radiol. 1994 May;23(4):257-60.
4. Smith SE, Murphey MD, Motamedi K, Mulligan ME, Resnik CS, Gannon FH.. "From the archives of the AFIP.
Radiologic spectrum of Paget disease of bone and its complications with pathologic correlation." Radiographics.
2002 Sep-Oct;22(5):1191-216.
5. Vande Berg BC, Malghem J, Lecouvet FE, Maldague B.. "Magnetic resonance appearance of uncomplicated Paget's
disease of bone." Semin Musculoskelet Radiol. 2001;5(1):69-77.
6. Vellenga CJ, Bijvoet OL, Pauwels EK.. "Bone scintigraphy and radiology in Paget's disease of bone: a review." Am
J Physiol Imaging. 1988;3(3):154-68.

Musculoskeletal Infection: Part I
Mark D. Murphey, MD
Musculoskeletal Infection: Routes of Inoculation

• Hematogenous
• Contiguous spread
• Direct implantation/post-surgical
Hematogenous Vascular Supply Figure 4-12-1

Tubular Bones
[Figure 4-12-1]
• Diaphyseal
➢ Enter through cortex
➢ Ascending/descending branches
• Metaphyseal
➢ Sharp turns beneath growth plate
• Epiphyseal
➢ Artery from epiphysis/metaphysis
• Periosteal
➢ From diaphyseal/muscle/soft tissue vessels
• Synovial
➢ From epiphysis/surrounding vessels
Hematogenous Vascular Supply

Tubular Bones
[Figure 4-12-2] Vascular supply to long bone and joint
• Age dependent
• Infant – vessels penetrate growth plate
• Child – vessels don’t extend across plate
• Adult – vessels cross closed growth plate
Figure 4-12-2
INFANT CHILD ADULT

Vascular supply of tubular bone by patient age
Pathophysiology of Acute Osteomyelitis [Figure 4-12-3, opposite]

• Inoculation/colonization/immunologic response
• Marrow and soft tissue edema
• Bone destruction – trabecular/cortical
• Subperiosteal/soft tissue/medullary abscess
• Vascular thrombosis – infarction (sequestrum)
• Periosteal new bone (involucrum)
Musculoskeletal Infection I 820 Musculoskeletal Radiology

Figure 4-12-3
a) Deposition b) Extension c) Lateral Spread
d) Superiosteal Lift e) Stripping
Progression of osteomyelitis through the cortex into the subperiosteal space (*)
Radiographic Abnormality: Acute Osteomyelitis

• Deep soft tissue swelling (within 3 days)
• Osteoporosis to focal bone lysis (7–14 days); magnification views
• Periosteal reaction (10–14 days)
• Increased blood flow: scintigraphy (early)
• Decreased blood flow: scintigraphy (later)
Other Radiologic Studies: MSK Infection

• Nuclear Medicine – BS: 3 phase studies
➢ Gallium: with BS, active infection
➢ WBC: increased specificity
• Sonography – fluid collections/effusions
• CT – bone destruction/sequestra/abscess
• Sinography – sinus tract extent
• MRI: marrow involvement, abscess very sensitive (STIR, GADO)
Osteomyelitis: Organisms
• Staph aureus (80% - 90% of pyogenic cases)
• H. flu, strep (shoulder, trauma from delivery)
• Gram negatives, uncommon except enterics (25% of infections)
• Pseudomonas – penetrating trauma/IVDA
• Salmonella – sickle cell
• Blood cultures positive 50%
Musculoskeletal Radiology 821 Musculoskeletal Infection I

Hematogenous Osteomyelitis: Infant (Up to 1–2 Years) Figure 4-12-4
[Figures 4-12-4 to 4-12-7]
• May be clinically silent (NICU/catheters)
• Group B strep more common than other ages
• Metaphysis/epiphysis location
• Extend into joint
• Most prominent sequestrum/involucrum
• Common soft tissue/subperiosteal abscess
• Fracture/sinus tracts uncommon
• May lead to late sequelae
Figure 4-12-5
Pictorial
representation of
infection
deposition sites
in an infant long
bone
Figure 4-12-6
Osteomyelitis in an infant with soft tissue swelling (circle and

arrow) as the only initial finding. Compare to normal left arm
(right image)
Figure 4-12-7
Subsequent radiographs 1 week (left image) and two

weeks (right image) later reveal development of
periosteal reaction initially (arrows) followed by extensive
involucrum (arrowheads) and sequestrum (*) formation
Figure 4-12-8
Continued follow-up radiographs 1 month (left image), 3

months (middle image) and 1 year (right image) later show
progressive resorption of sequestra representing the majority of
the humerus (*-”bone within bone appearance”) and
subsequent remodeling to nearly normal appearance
Hematogenous Osteomyelitis: Child (1–16 Years)

[Figures 4-12-8 to 4-12-13]
• Tubular bones 75%
• Metaphysis/lower extremity
• Can involve joint (hip /shoulder)
• Sequestrum/involucrum common
• Soft tissue/subperiosteal abscess common
• Fracture uncommon
• Sinus tracts can occur Pictorial representation of infection
deposition sites in a child long
bone

Osteomyelitis in the distal femoral metaphysis of the

Aggressive bone destruction (arrow) and periosteal reaction femur with bone destruction (arrows).
(arrowheads) in the distal ulnar metaphysis on radiograph and
marked uptake of radionuclide on bone scan resulting from
acute bacterial osteomyelitis in a child
There is marrow replacement on the T1-weighted MR image with focal rim enhancing subperiosteal abscess (arrowheads) posteriorly
showing high signal on T2-weighting (*)
Subperiosteal Abscess Figure 4-12-13

[Figure 4-12-14]
• Common in children/infants
➢ Loose periosteum
• Femur/tibia – long extension
• Adults – sinuses/orbit
• Often don’t recognize on Figure 4-12-14
radiographs
• Nuclear Medicine – rim
with photopenia (BS,
WBC)
• CT/MR/SONO – fluid
collection bone surface
Pictorial presentation of Civil war gross specimen and radiograph showing

subperiosteal abscess extensive involucrum (arrows) and sequestrum (*)
resulting from war injury induced osteomyelitis

Hematogenous Osteomyelitis: Adult (Over 16 Years)
[Figures 4-12-15 to 4-12-17]
• Tubular bones less common
• Spine/pelvis/small bones more common: subchondral focus
• Joint involvement/sinus tracts common
• Involucrum/sequestrum/abscess uncommon Figure 4-12-15
• Fracture uncommon except neglected cases
Pictorial representation of infection

Acute bacterial osteomyelitis in an adult. Initial radiograph reveals only soft tissue swelling
deposition sites in an adult long
posterior to olecranon (*). Subsequent radiographs two weeks (right images) later show moth-
bone
eaten to permeative bone destruction resulting from staph aureus osteomyelitis
Subacute Osteomyelitis: Brodie Abscess

[Figures 4-12-18 and 4-12-19]
• Described in 1832 – chronic/subacute
• Walled-off with central fluid, often sterile (staph) Figure 4-12-18
• Children (M>F), metaphysis, tibia
• Intramedullary - channel-like lucencies
• May cross growth plate or be cortical
• Periosteal reaction/sequestra may be seen
Figure 4-12-19
Brodie abscess with sequestra (arrows) in the cortex of

the proximal femur on radiograph, axial CT and coronal
CT reconstruction. Debrided sequestered fragment is
also demonstrated (*)

Chronic Osteomyelitis [Figure 4-12-20] Figure 4-12-20
• Bone formation results in sclerosis
• Sclerosing osteomyelitis of Garre
• No pus, may have mixed radiolucency
• Active infection: new bone destruction, sequestra, aggressive periosteal
reaction on radiographs
• MRI, scintigraphy (bone, gallium, WBC)
Osteomyelitis/Septic Arthritis
Contiguous Spread: Source
• Soft tissue infection
➢ Trauma
➢ Human/animal bites
➢ Puncture wounds
➢ Ulcers
➢ Surgery
Contiguous Spread: Radiographs
• Soft tissue swelling/air
• Periosteal reaction Chronic osteomyelitis of the ulna
• Cortical destruction into marrow with diffuse sclerosis on
• Joint space loss radiograph
Osteomyelitis/Septic Arthritis Figure 4-12-21

Contiguous Spread: Hand
• Tendon sheaths, fascial planes, lymphatics
• Felon-volar tuft destruction
• Paronychia-dorsal tuft destruction
• Bites
➢ Human: MCP joint with fight (Staph/strep)
➢ Animal: dog (90%), 5% infected;
cat (10%), 20%–50% infected
(Pasteurella multocida)
Contiguous Spread: Other Sites [Figure 4-12-21]
• Foot – pathways: medial, intermediate, lateral
➢ Puncture: pseudomonas
➢ Diabetes: multiple organisms
➢ Aerobic/anaerobic
Diabetic patient with ulcer lateral to fifth MTP joint and
• Pelvis – decubitus ulcers (paraplegics)
radiograph shows underlying destruction of distal fifth
➢ Ischial tuberosity chronic osteomyelitis metatarsal head and proximal phalanx resulting from
• Post-operative: ring sequestra osteomyelitis and pyarthrosis. Amputation specimen shows
similar findings
Osteomyelitis: Diabetes Mellitus Figure 4-12-22
• Bone destruction/periosteal reaction
• Bone scan – normal – no osteomyelitis
• WBC scan – good predictive value
• MRI – marrow replacement geographic area T1 and
increased signal STIR, post gado
➢ Normal on T1 or subtle/subcortical only; then
probably marrow reaction not osteomyelitis
(Collins et al AJR 185:2005)
➢ Focal fluid collections
Osteomyelitis vs Neuropathic
Diabetes Mellitus
• Factors favoring infection: Sinus tract (84%/0%); ST
replacement (68%/32%); Fluid collection
(95%/48%); Extensive marrow abnormality
(41%/12%)
• Factors favoring neuropathic: Thin rim enhancement Chronic osteomyelitis with sinus tract and secondary
of effusion (68%/21%); Presence of subchondral epidermoid carcinoma. Aggressive bone destruction is seen
cysts (76%/2%); Intraarticular bodies (53%/12%) distally and medially corresponding to malignancy (arrow
and *) as sinus tract enters bone
Ahmadi et al, Radiology 238; 622-631, 2006

Complications of Osteomyelitis [Figure 4-12-22] Figure 4-12-23
• Sequestra discharged through sinus tracts
• Avascular necrosis
• Fracture/slipped epiphysis
• Growth plate disturbance
• Osteolysis
• Systemic amyloidosis (rare)
• Epidermoid carcinoma
➢ 0.5% long term draining sinus
➢ M>F, 20–40 year latent period
➢ Tibia/femur
➢ Arise deep in sinus tract
Septic Arthritis: Bacterial

• Cause
➢ Hematogenous, contiguous spread, direct implantation, post-
surgical
• Polyarticular – 20%
• Organisms
➢ H. flu – leading cause up to age 2 years
➢ Staph, alpha/beta hemolytic strep.
➢ Pneumococcus, E.coli, Pseudomonas Pictorial representation of septic arthritis
Figure 4-12-24
Septic Arthritis: Pathology [Figure 4-12-23]
• Synovial inflammation /hyperemia/fluid production
• Fibrin deposits inhibit cartilage nutrition
• Attract WBC’s release enzymes (collagenase)
• Pannus formation
• Cartilage destruction/bone erosion
Septic Arthritis: Radiology [Figure 4-12-24]

• Soft tissue swelling / joint effusion (sonography)
• Periarticular osteopenia
• Increased vascularity – scintigraphy
• Pannus – arthrography – gadolinium – enhanced
Septic arthritis and osteomyelitis of the fourth MTP joint with joint
MRI
narrowing and bone destruction (arrowhead)
Septic Arthritis: Figure 4-12-25

Hip Infant/Childhood [Figure 4-12-25]
• Staphylococcus aureus
• Spread from adjacent osteomyelitis
• Radiologic signs
➢ Widened hip joint/effusion (sonography)
➢ Displaced pericapsular fat planes
• Surgical emergency
Septic Arthritis:
Complications/Sequelae [Figure 4-12-26]
• Avascular necrosis Septic arthritis in the right hip of a young child with joint widening
• Slipped epiphysis indicating a joint effusion on radiography
• Growth disturbance
• Osteomyelitis Figure 4-12-26
• Secondary osteoarthritis
• Synovial cyst, tendon/capsule injury
Soft Tissue Infection

• Septic bursitis – usually injury
➢ Staph/prepatellar (child)
• Septic tenosynovitis
• Cellulitis
• Necrotizing fasciitis
• Soft tissue abscess – pyomyositis
➢ Staph (90%)
Complication of septic arthritis with right hip
osseous ankylosis on radiography

Soft Tissue Infection: Radiology [Figure 4-12-27] Figure 4-12-27
• Radiographs
➢ Soft tissue swelling
➢ Air (rare): clostridia, E. Coli (coliform), bacteroides
• CT/MRI/Sonography
➢ Inflammatory changes
➢ To detect focal abscess
➢ Contrast enhancement of rim
Cellulitis
• Acute inflammatory process of deep subcutaneous tissues
• Location
➢ Extremities, thorax, abdomen, neck
• Organisms
➢ Streptococci, staphylococci, H. influenza
Necrotizing Fasciitis: Clinical

• Infection and necrosis of fascia (Staph/Strep)
• Important to distinguish from cellulitis
• Systemic severe toxicity - IVDA
• Extremities, neck, face, perineum
• High mortality (>70% if not treated)
➢ Need prompt aggressive treatment
• Treatment–Fasciotomy, debridement,
antibiotics
Necrotizing Fasciitis: Imaging

• Skin thickening
• Subcutaneous edema and air
• Involves deeper tissue unlike cellulitis
• Focal fluid collections (abscess)
• Gadolinium enhancement
Chronic Granulomatous Disease

of Childhood
• X-linked recessive (males)
• WBC’s cannot kill organisms
• Skin lesions, lymphadenitis,
Soft tissue abscess in the buttock on CT and MR showing focal fluid collection
hepatosplenomegaly (*) with internal foreign material (arrows) after accident
➢ Pneumonias, chronic osteomyelitis
(25%–35%)
• Fatal – 40% cases – low virulent organisms
• Symptoms of osteomyelitis limited
• Bone destruction/limited sclerosis
• Small bones hands/feet, chest wall, spine
Figure 4-12-28
Chronic Recurrent Multifocal Osteomyelitis
(CRMO) [Figure 4-12-28]
• Chronic symmetric plasma cell osteomyelitis
• Indolent/recurrent bone infection
• Age 5–15 years; knee metaphyses, clavicle, face
• Mixed lysis/sclerosis
• May be sterile, plasma cells, lymphs and histiocytes
• SAPHO (Synovitis, Acne, Pustular rash palms/soles,
Hyperostosis, Osteitis)
Musculoskeletal Infectionn Drug Abusers

• Altered WBC function and infected needles
• “S” joints – spine, sacroiliac, and sternal joints
• AC joint, symphysis, ischial tuberosity
• Pseudomonas, Klebsiella, Serratia
• Candida in heroin addicts CRMO with patchy areas of lucency and sclerosis in the distal
tibial metaphysis on radiography. Contralateral ankle, ipsilateral
knee and iliac bone showed similar findings (not shown)

Spine Infections: Routes of Contamination
• Hematogenous
➢ Arterial
➢ Venous: Batson Plexus
• Contiguous source
• Direct implantation/post-operative
➢ Discography (-ectomy), biopsy, laminectomy
Spine Infections: Clinical Characteristics

• Spondylodiskitis Figure 4-12-29
• 2%–4% of all osteomyelitis cases
• M>F (1.5–3:1); age 40–60 years
• Lumbar > thoracic > cervical/sacrum
• Vertebral body
• History: recent primary infection
• Symptoms: back pain, fever, neurologic
Spine Infections: Pathology

• Staph aureus (55%–90%)
• Localizes to anterior subchondral bone
• Rapidly extends into disc (1–3 weeks)
• Can extend into paravertebral soft tissue
Spine Infections: Radiography

• Initially radiographs normal or subtle
subchondral destruction
• Usually seen radiologically after disc involved
• Rapid disc narrowing with irregular endplate Bacterial spondylodiskitis with rapid disk space loss and endplate
destruction destruction over two weeks (right image) due to staph aureus
• Later osteosclerosis (10–12 weeks) infection. Left image initial radiograph
• Treatment: diffuse sclerosis, DDD, disc ankylosis
Spine Infections: CT/MR Imaging [Figure 4-12-29]

• MR imaging optimal
• Bone destruction/marrow replacement (T1W)
• High signal T2W MR images
➢ Disc and adjacent vertebra
• Post gadolinium images helpful
➢ Focal abscess detection
• Paravertebral soft tissue masses (20% pyogenic cases)
➢ Anterior and lateral (psoas)
➢ Epidural
Spine Infections: Differential Diagnosis

• Amyloid in CRF
➢ Low signal T2W MR images
• Tumor-crossing disc
➢ Chordoma, lymphoma, myeloma, GCT
• Other causes disc narrowing (well-defined bone margins)
➢ DDD, CPPD, neuropathic, RA, trauma, sarcoid
Invertebral Discitis:
• Hematogenous – children (1–16 years)
➢ Disc vascularized
• Symptoms – mild after primary infection
• Cultures negative (50%–90%); staph aureus
• Antibiotics given empirically
Intervertebral Discitis: Radiology

• Scintigraphy positive early
• Late disc narrowing and erosion
• MRI findings similar to adults
• Paravertebral soft tissue changes unusual
• Disc often reconstitutes after therapy but may remain deformed with sequelae

Musculoskeletal Infection
Part II: Atypical Organisms
Mark D. Murphey, MD
Unusual Bacterial Musculoskeletal Infection

• Brucella
• Mycobacteria (acid-fast bacilli)
➢ Tuberculosis
➢ Atypical
➢ Leprosy
• Actinomycosis
Brucellosis
• Malta fever – undulant fever
• WHO – 500,000 cases annually
• B. – abortis, melitensis, suis, canis
• Endemic Midwest USA, Saudi Arabia, South America, Spain, and Italy
• Ingested milk/meat – reticuloendothelial system (marrow)
Brucellosis [Figure 4-13-1]

• Musculoskeletal infections 30%–85%
• Septic arthritis – knee, sacroiliac joint, shoulder
• Prepatellar bursitis
• Spine – 53% – lumbar (lower L4)
➢ Focal: subchondral anterior superior endplate (parrot beak phyte)
➢ Diffuse: vertebral body maintained, less disc and paravertebral
involvement, disc gas (25%–30%)
Al-Shahed, Radiographics 94:14:333-348
Figure 4-13-1
Brucellosis spondylodiscitis on radiograph,

CT and sagittal T1- and T1- weighted post-
contrast MR images shows multilevel
involvement of the discs and vertebral
bodies (arrows,open arrows,arrowheads
and *) and paraspinal and anterior epidural
inflammatory changes (curved arrows and
arrows on MR). Note the lack of vertebral
collapse and “parrot beak”
(open arrows on radiographs) osteophytes
Musculoskeletal Tuberculosis: Clinical

Characteristics
• Increasing – immunocompromised patients
• 1%–3% of TB patients – MSK involvement
• Pain, swelling, stiffness – long delay to diagnosis
• Mortality still significant
• Negative skin test – MSK TB unlikely
• Negative CXR (child) – MSK TB unlikely
Musculoskeletal Radiology 829 Musculoskeletal Infection II

Musculoskeletal Tuberculosis: Pathology
• Hematogenous, pulmonary changes 50%
• Tubercles: central giant and epithelioid cells
➢ Central caseating necrosis (may calcify)
➢ Peripheral: lymphocyte mantle
• Synovial tissue/fluid: 80%–90% positive culture
Musculoskeletal Tuberculosis: Sites of Involvement

• Spine: 25%–60% of MSK cases
• Other osseous sites unusual
• Dactylitis
• Arthritis – knee, hip
• Bursitis/tenosynovitis – hand/wrist
• BCG related
Tuberculous Spondylodiscitis
• May result in neurologic symptoms
• Hematogenous – venous (Batson) plexus
• L1 most common: decreases above/below
• More than one level frequent
➢ Usually contiguous
➢ Separate foci: 1%–4%
Tuberculous Spondylodiscitis: Radiology

[Figures 4-13-2 to 4-13-7]
• Subchondral vertebral body (2–5 months)
➢ Anterior 82%, posterior 18%
• Disc extension
• Less common involvement – posterior elements, isolated vertebral body (ivory)
• Subligamentous extension – “gouge” defects
• Kyphosis (gibbus) – thoracic
• Paraspinal extension – psoas (L1-L5)
➢ Calcification: amorphous/teardrop
➢ Abscess (5% psoas)
• MR imaging usually optimal
➢ Assess extent/relationship to canal
➢ Abscess shows rim enhancement
Tuberculous Spondylodiscitis: Differential Diagnosis Pyogenic

• Can be difficult
• Findings favoring tuberculosis
➢ Multilevel involvement
➢ Slow vertebral/disc destruction
➢ Calcified paraspinal mass Figure 4-13-2
➢ Lack of sclerosis
Tuberculous Osteomyelitis
• Not common, usually with arthritis
• Often epiphyseal, any bone, joint
• Children metaphyseal cross plate
• Cystic variety – multifocal defined lytic foci
• Dactylitis – < age 5 (0.5%–14% cases)
➢ Multifocal (25%–35%)
➢ Spina (spike-like) ventosa (puffed full of air)
Pictoral representation of tuberculous

spondylodiscitis
Musculoskeletal Infection II 830 Musculoskeletal Radiology

Figure 4-13-3
a b
Tuberculous spondylodiscitis:
a) Radiographs show myelographic block (arrowhead) with
endplate destruction, collapse, and disc involvement (arrow);
b) CT reveals large paraspinal mass (*);
c) Sagittal T1-weighted MR images show marrow replacement
and disc involvement at two levels (arrowheads) as well as
anterior paraspinal and posterior epidural masses (arrows) ;
d) Sagittal and axial post-contrast T1-weighted MR images
reveal rim enhancement about anterior paraspinal and
posterior epidural abscesses (arrows);
e) High signal intensity is seen on the axial and sagittal T2-
weighted MR images in the involved vertebrae, discs and
paraspinal abscesses (*)
c
e
d
Anterior gouge defects on sagittal T2-weighted MR image and

Sagittal dried bone specimen and gross specimen show
sagittal macrosection resulting from tuberculous
effects of tuberculous spondylodiscitis with gibbus deformities
spondylodiscitis extending under the anterior longitudinal
(arrows) and anterior epidural inflammatory mass (*)
ligament (arrows) and invading other vertebral segments
displacing the spinal cord (c) posteriorly
(arrowheads)

Teardrop paraspinal calcification (*) in CT of the spine in a patient with tuberculous spondylodiscitis and
a tuberculous paraspinal abscess calcified paraspinal abscesses (arrows)
related to spondylodiscitis
Figure 4-13-8
Tuberculous Arthritis [Figure 4-13-8]
• Large joints (knee/hip); monoarticular
• Synovial thickening covered by fibrin
• Granulation tissue erodes cartilage bone
• Slow process/lack proteases
• Areas of cartilage contact spared
Tuberculous Arthritis: Radiology

[Figures 4-13-9 and 4-13-10]
• Phemister triad
➢ Juxtaarticular osteopenia
➢ Slow joint space loss
➢ Peripheral erosions
• Joint effusion
• MRI – nodular synovial thickening
• Less reactive bone, periostitis, osseous ankylosis
Pictorial representation of tuberculous arthritis (right
Figure 4-13-9 image, left image normal joint)
Figure 4-13-10
Pelvis radiographs over a
two month interval show
slow pancompartmental
loss of the right hip joint
resulting from tuberculous
arthritis
Radiograph of tuberculous arthritis with diffuse joint space loss,

marginal erosions (arrows) and osteopenia (Phemister triad) and
sagittal macrosection showing fibrinous exudate (*)
throughout the joint

Atypical Mycobacterium [Figure 4-13-11] Figure 4-13-11
• Early diagnosis/treatment important – skin/pulmonary
• Types – photochromogens (M. Kansasii),
nonchromogens (M. avium); rapid growers
• Bone/joint – multiple lesions, less osteopenia, hand/wrist,
metaphysis/diaphysis lysis/sclerosis, abscess/sinus tracts common
Leprosy: Mycobacterium Leprae

• Africa, South America, Asia
• USA – Texas, Louisiana, Hawaii, Florida
• Long incubation 3–6 years
• Infection through skin/mucous membranes
• M>F; usually detected before age 20
Leprosy: Pathology Types

• Lepromatous – many bacilli more severe/generalized
• Tuberculoid – more reaction/less bacilli/skin & nerve involvement
• Dimorphous – features of both
• Indeterminate
Leprosy Direct: Specific Signs [Figures 4-13-12 and 4-13-13]

• Related to bacilli presence
• Uncommon 1%–2% findings
• Small bones hands/feet (direct spread)
• Punched out or lacelike osseous destruction phalanges, nasal bone
• Periosteal reaction, fragmentation, arthritis
Figure 4-13-12
Sagittal T1-weighted and axial T2-weighted

MR images reveal marked thickening about
the second flexor tendon (arrowheads)
related to atypical mycobacteria
tenosynovitis. Chronic fibrosis causes the
intermediate signal on T2-weighting (arrow)
Figure 4-13-13
Lattice-like lucencies in the phalanges representing a direct

sign of leprosy infection on radiography
Actinomycosis: Clinical Characteristics

• Anaerobic higher bacteria; acid fast-like
• Actinomyces (Israelii), nocardia (asteroides)
• Normal flora in mouth
• Trauma often results in inoculation
Xeroradiography shows medial distal arm calcification that is

located in the ulnar nerve (*) on the resected gross specimen
resulting from leprosy

Actinomycosis: Radiology [Figure 4-13-14] Figure 4-13-14
• Sites mandible, axial skeleton (rib, spine, pelvis),
large joints
• Lysis and sclerosis (bone proliferation)
• Spine (posterior elements) and rib
• Sinus tracts/abscesses (no calcification) common
Spirochetes
• Syphilis
• Yaws
Actinomyces osteomyelitis in the mandible on radiograph
• Lyme disease
showing patchy areas of destruction and sclerosis (arrowheads)
• Tropical ulcer
• Bejel, rat bite, fever, leptospirosis
Congenital Syphilis: Early Changes [Figure 4-13-15]

• Toxic effects degenerating spirochetes/infection
• Osteochondritis – metaphyseal lucent Figure 4-13-15
bands/irregularity
➢ Long bone (tibia: Wimberger sign), rib, spine, sternum
➢ Epiphyseal widening/separation
➢ Heal quickly with treatment
• Osteomyelitis – diaphyseal lysis/sclerosis/periostitis
• Diffuse periostitis
Congenital Syphilis: Late Changes

• Congenital syphilis – 75% diagnosed after age 10
• Hutchinson triad (Hutchinson teeth, interstitial keratitis,
nerve deafness)
• May be similar to early changes
• Usually more like acquired findings
• Dactylitis – fingers more common
• Painless knee effusions (Clutton joints)
Congenital Syphilis: Early Changes

• Bone changes in up to 20% (early as 6 weeks) Congenital syphilis of the lower extremity showing
• Proliferative periostitis periosteal reaction (arrows) and osteochondritis
(arrowheads) of the proximal tibiae (Wimberger sign)
➢ Most common finding
➢ Tibia (saber shin), skull, ribs, sternum
➢ Solid/laminated (rarely perpendicular)
• Osteomyelitis/osteitis/septic arthritis
• Much less common
• Skull: outer table aggressive lysis
• Nasal bone collapse – saddle nose
Congenital Syphilis: Late Changes

• Periostitis/osteomyelitis/osteitis
• Gumma – any bone caseous necrosis related to degenerating spirochete
➢ Bone resorption: Carries sicca
➢ Bone lysis/reactive sclerosis
• Arthritis – uncommon – ankles, MTP, elbows, knees
➢ Swelling, effusion, narrowing, destruction
➢ Neuropathic (5%–10%) – knee, hip, ankle, spine
Yaws
• Treponema pertenue
• Africa, South America, South Pacific
• Very similar to syphilis – less nose changes, more phalanges (spares distally)
• Exostosis maxilla – goundou
Tropical Ulcer
• Central/East Africa
• Lower leg ulcer destroys muscle/tendon
• To bone focal osseous production tibia/fibula
• Multiple organisms including spirochetes
• Epidermoid carcinoma 25% (>10 years latency)

Lyme Disease: Clinical Characteristics Figure 4-13-16
• Recognized 1975; 23,763 new cases (2002)
• Most common vector borne illness in USA
• USA (NE/MidAtlantic), Europe, Far East, Australia
• Tick bite - ixodes dammini (transmissiom)
• Spirochete - Borrelia burgdorferi (deer, mice)
• Skin lesions (erythemia chronicum migrans 20%)
• Joint symptoms (usually 2-6 mths; 2 weeks - 2 yrs)
➢ 80% cases reported May-August
Lyme Disease: Radiology [Figure 4-13-16]

• Knee (80%), shoulder, elbow, temporomandibular, ankle, wrist,
hip, hand/foot
• Monoarticular, oligo, or polyarticular
• Soft tissue swelling/effusion (MRI synovitis)
➢ Adenopathy, myositis, lack subcutaneous edema
• Chronic changes (10%) – osteopenia, joint loss (25%),
erosions, secondary carcinoma
Musculoskeletal Infection: Fungal [Figures 4-13-17 to 4-13-20]

• Aspergillosis
• Blastomycosis
• Candidiasis
• Cryptococcosis
• Histoplasmosis
• Mucormycosis
• Sporotrichosis
• Madura Foot
Fungal Musculoskeletal Infection:

Common Changes
• Frequently in immunocompromised
• Osteomyelitis large punched out lytic lesions
• May have surrounding sclerosis
• Often multifocal/may involve bone protuberances
• Joint involvement – slow progressive destruction
➢ MRI: nodular synovial thickening Lyme arthritis of the knee on lateral radiograph with effusion
(* ) and MR revealing synovitis (arrows), myositis (M) and
Aspergillosis adenitis (A)
• A. Fumigatus – normal URT organism
• Sites related to pulmonary changes (children) or
➢ Hematogenous (adult): rib, sternum, spine
• Arthritis rare
Figure 4-13-17
Blastomycosis
• B. Dermatiditis (N. American)
➢ Ohio, Miss. River Valleys, Mid-Atlantic
• B. Brasiliensis – Mexico, Central/South America
• Skin/pulmonary entry from soil
• Bone involved in up to 60% patients
• Ribs, spine, tibia, carpus, tarsus, skull
• Arthritis <10% patients
Candidiasis (Moniliasis)
• C. Albicans but many other species
• Patients on hyperal, antibiotics, intraarticular steroids
• Bone involvement rare (1%–2%)
• Monostotic/monoarticular – long bone, sternum, spine, knee
• Arthritis more often precedes osteomyelitis
Coccidioidomycosis
• C. Immitis – soil- inhalation
• SW USA, Mexico
• <1% disseminated; (10%–50% MSK changes)
• Metaphyseal, may be symmetric Aspergillus infection with pulmonary
• Joints – ankle, knee, also migratory arthritis and rib (arrowheads) involvement

Cryptococcosis Figure 4-13-18
• Torulosis, C. Neoformans
• Soil – inhalation
• Disseminated disease, 5%–10% MSK changes
• Arthritis unusual
Histoplasmosis
• H. Capsulatum – USA (Miss. River Valley)
• H. Dubosii – Africa – MSK changes 80% cases
• Soil – inhalation; most common fungal in USA
• Pelvis, skull, ribs, small tubular bones
• Arthritis – knee, ankle, wrist, hand
Mucormycosis
• Phymycetes – rhizopus, mucor, absidia
• Diabetes, uremia, burns
• Entry via sinuses
• Skull/face (maxillary/ethmoid sinuses)
Sporotrichosis
• Sporothrix schenckii Blastomycosis of the humerus with
extensive involvement demonstrating
• Saprophyte on vegetation
mixed lysis and sclerosis
• Inhalation/skin wound (rose thorn)
• Disseminated form 80% MSK changes
• Arthritis (monoarticular) common – knee (66%) hand wrist (52%) ankle, elbow
• Osteopenia often not prominent
• Osteomyelitis – due to arthritis
Figure 4-13-19
Figure 4-13-20
Sporotrichosis of
the knee with CT
(post-arthrogram)
and MR showing
enhancing
nonspecific
nodular synovial
thickening
(arrowheads) after
intravenous
contrast
Histoplasmosis with multifocal area of lysis, many of which
involve tuberosities and trochanteric regions ("lumps and
bumps" of bone)

Madura Foot: Mycetoma [Figure 4-13-21] Figure 4-13-21
• Chronic granulomatous infection
• Foot (65%–70%), hand, legs, back/head
• Many organisms can be cause – Eumycetes
(Madurella), Actinomycetes, Monosporium
Apiospermum (USA)
• Tropics – India, Africa, Arabia, Latin America
• Tarsals/metatarsals most involved
Viral/Protozoan Musculoskeletal Infection

• Torch – metaphyseal lucent bands (celery stalking)
• Cat-scratch disease – viral like bacteria AFIPIA felis
(R. Hensalae and Bartanella species)
➢ Look for adenitis (epitrochlear)
Aids: Musculoskeletal Changes [Figure 4-13-22]

• Seronegative arthropathy
• Osteomyelitis, septic joint/bursitis any organism
• Pyomyositis (staph); lower extremity (95%), multiple
(50%)
• Bacillary angiomatosis (rochalimaea hensalae,
quintana) skin lesion/bone destruction (cortex
prominent)
• Neoplasm – Kaposi sarcoma, lymphoma
Helminths/Worms
• Musculoskeletal changes usually soft tissue
calcification
• Loa loa - Africa – subcutaneous calcification (fine
lace-like or thicker bead-like)
• Filariasis – lymphatic obstruction (elephantiasis)
• Guinea worm (dracunculosis) – long calcification
female worm lower extremity hand – can cause
arthritis
• Cysticercosis – linear/oval rice grain calcification
along axis of muscle (up to 20–25 mm length)
Mycetoma (Madura foot) on radiographs, sagittal T1-and T2-
weighted MR images and gross specimen show extensive
Echinococcus: Musculoskeletal Changes multifocal destruction with sclerosis/fibrosis representing
[Figure 4-13-23] chronicity
• Echinococcus (E. multilocularis/granulosis)
• Bone disease: 0.5%–4% (E. granulosis) Figure 4-13-22
• Spine, long bone epiphysis,iIlium, skull, rib
• Multiloculated (bundle of grapes) lysis/expansion
• May be soft tissue loculated, cyst margins may
calcify
Sarcoid: Musculoskeletal
• Usually have chest changes (80%–90%)
• Muscle – myositis (50%–80% patients)
➢ MRI low signal central scar
• Subcutaneous soft tissue nodules (5%)
• Arthritis (10%–35%) – acute/chronic
➢ Hand, wrist, ankle, knee, elbow
Sarcoid - Musculoskeletal:
Osseous Changes [Figures 4-13-24 and 4-13-25]
• 1%–13% of patients; may be asymptomatic
• Lattice like lysis (hands)
• Punched out lytic lesions
• May appear aggressive
• Acroosteolysis, acrosclerosis (30%–50%)
• Areas of sclerosis Coronal STIR MR shows a focal fluid collection (*)
• Marrow replacement creating mottled appearance representing pyomyositis in an HIV patient
(MRI)

Typical lattice like multifocal lucencies of several phalanges

Paraspinal echinococus infection with multiloculated fluid filled resulting from sarcoid
cysts (*) on axial T1- and T2-weighted MR images and gross
specimen
Figure 4-13-25
Sarcoid marrow involvement showing heterogeneous or mottled

marrow signal intensity diffusely on sagittal T1-weighted cervical
spine MR images
References (Musculoskeletal Infection Parts 1 and 2)
1. Erdman WA, Tamburro F, Jayson HT, Weatherall PT, Ferry KB, Peshock RM. Osteomyelitis: characteristics and
pitfalls of diagnosis with MR imaging. Radiology. 1991 Aug;180(2):533-9.
2. Jung NY, Jee WH, Ha KY, Park CK, Byun JY. Discrimination of tuberculous spondylitis from pyogenic spondylitis
on MRI. AJR Am J Roentgenol. 2004 Jun;182(6):1405-10.
3. Lawson JP, Rahn DW. Lyme disease and radiologic findings in Lyme arthritis. AJR Am J Roentgenol. 1992
May;158(5):1065-9. Review.
4. Palestro CJ, Kipper SL, Weiland FL, Love C, Tomas MB. Osteomyelitis: diagnosis with (99m)Tc-labeled
antigranulocyte antibodies compared with diagnosis with (111)In-labeled leukocytes--initial experience. Radiology.
2002 Jun; 223(3):758-64.
5. Sharif HS, Aideyan OA, Clark DC, Madkour MM, Aabed MY, Mattsson TA, al-Deeb SM, Moutaery KR. Brucellar
and tuberculous spondylitis: comparative imaging features. Radiology. 1989 May;171(2):419-25.
6. Sharma P. MR features of tuberculous osteomyelitis. Skeletal Radiol. 2003 May; 32(5):279-85. Epub 2003 Mar 25.
7. Unger E, Moldofsky P, Gatenby R, Hartz W, Broder G. Diagnosis of osteomyelitis by MR imaging. AJR Am J
Roentgenol. 1988 Mar;150(3):605-10.

Imaging of Cervical Spine Trauma
Mark D. Murphey, MD
Cervical Spine Trauma: Demographics

• Most common portion of spine injured
• Responsible for 65% of all spinal injuries
• Mechanism: MVA/Fall/Sports injury
• Spinal cord injury: 40% (10,000 annually)
Cervical Spine Trauma: Patterns

• Areas most commonly involved
➢ C1 – 2 (particularly in children)
➢ C5 – 7
• Other fractures 20%
• Particular association of low cervical fracture with high thoracic and
thoracolumbar injury
Cervical Spine Trauma: Radiographic Signs - Normal

• ABC’S - alignment, bone integrity, cartilage (joint/disc space), soft tissues
• Lateral view - anterior/posterior vertebral body arcs
➢ Spinolaminal arc (except childhood pseudosubluxation C2-3)
• AP view-spinous and lateral mass arcs
Cervical Spine: Normal Measurements

• Lateral atlantoaxial offset
➢ (“open mouth” view) - 2 mm
• Predental space - 3 mm adult; 5 mm child
• Anterior vertebral height vs. posterior
➢ 2 mm (except C5)
• Pretracheal space at C6 - 22 mm adult,
➢ 14 mm child
• Facet width – 2 mm
• Listhesis with flexion/extension - 2 mm
• Retropharyngeal space at C2 - 7-8 mm
➢ Exceptions: ET/NG tubes; inflammatory process/crying child
• Interspinolaminar space - 2 mm
➢ Between 3 continuous levels
Cervical Spine Trauma: Radiographic Evaluation

• Standard 3 view series
➢ AP, lateral, open mouth
➢ Kasabach view modified oblique open mouth
• Oblique views
• Trauma oblique views
• Swimmer (Twining) views
• Upright, lateral, flexion, extension
Trauma Oblique: Radiographs

• Developed by Gehweiler and Abel
• X-ray tube angled 30o - 40o from horizontal
• Add 15o cranial tube tilt
• Better than Swimmer view for cervico-thoracic junction
Flexion and Extension: Radiographs

• Use upright lateral first to evaluate cervical spine straightening/reversal
• To evaluate abnormal alignment/stability
• False negative from muscle spasm
➢ Repeat after delay
• Performed under physician guidance
➢ Passive motion
Musculoskeletal Radiology 839 Imaging of Cervical Spine Trauma

Cervical Spine Trauma: CT
• Indispensable, widely available, rapid study
➢ 1-3 mm sections, coronal/sagittal reconstructions
➢ Spiral/Multichannel/Holography
➢ 3D helpful to depict spatial relationships
Cervical Spine Trauma: MR Imaging/Myelography

• MRI indications
➢ Post traumatic cervical myelopathy/radiculopathy Figure 4-14-1
➢ Clinical symptoms unexplained by other
radiologic studies
➢ Assess ligamentous injury
• Myelography (CT) largely replaced by MRI
➢ CSF obstruction
➢ Nerve root avulsion, dural tear
Cervical Spine Trauma: Stability

• Mechanical – ability to not deform under physiologic
stress
• Neurologic – potential to produce new or increase
previous deficit
• Acute/Chronic
Cervical Spine Trauma:

Radiographics Signs Instability
• Widened interspinous spaces (>2 mm) Pictoral representation of flexion sprain cervical injury with
• Widened apophyseal joints (>2 mm) distraction forces posteriorly causing interspinous widening or
• Anterior listhesis > 3.5 mm fanning (*)
• Narrowed/widened disc space
• Focal angulation of >11o
• Vertebral compression > 25%

Classification by Mechanism
• Hyperflexion
➢ Modified by rotation/lateral flexion Figure 4-14-2
• Hyperextension
➢ Modified by rotation
• Axial loading – burst
• Complex, poorly understood mechanism

Hyperflexion Injuries
• Account for 50% - 80% of injuries
• Flexion forces maximal at C4 – C7 anterior;
distraction posterior
• Sprain; compression fracture
• Facet fracture/subluxation/dislocation
• Flexion teardrop fracture
• Clay (coal) shovelers fracture
• Lateral flexion fractures – unilateral occipital
condyle/lateral mass C1
➢ Uncinate or transverse process Lateral radiograph of flexion sprain cervical injury with
distraction forces posteriorly causing interspinous widening or
Cervical Spine Trauma: Hyperflexion Sprain fanning at C6-7 (arrows)
[Figures 4-14-1 and 4-14-2]
• Disrupted one-level posterior ligaments by distraction
• Acute focal pain/limited ROM
• Delayed instability (30% - 50%) - lack symptoms (delayed flexion/extension
views)
• Radiographic findings
➢ Focal kyphosis, mild anterolisthesis
➢ Widened facet, interspinous/interlaminar spaces
➢ Widened posterior, narrowed anterior disc
➢ Compression fracture often associated
➢ All findings accentuated with flexion; MRI to confirm ligament injury
Imaging of Cervical Spine Trauma 840 Musculoskeletal Radiology

Cervical Spine Trauma: Compression Fracture Figure 4-14-3
[Figure 4-14-3]
• Associated hyperflexion sprain common
• Usually stable unless > 25% compression
• Radiographs
➢ Loss of height superior endplate
➢ Focal cortical angulation
➢ Band of increased density from impaction

Unilateral Facet Injury
• Hyperflexion and rotation
• Common – 13% of cervical injuries
• Radicular symptoms common
• Most frequent C4 – C6
• Often mechanically stable, PLL partially intact
• Unstable with prominent articular mass/laminar
fractures
Unilateral Facet Injury:

Radiologic Characteristics Sagittal MR (proton density and T2) of flexion sprain cervical
[Figures 4-14-4 and 4-14-5] injury with superior endplate fracture (arrow) and distraction
• Anterolisthesis < 50% vertebral width forces posteriorly causing interspinous widening or fanning at
• Dislocated facet anterior (oblique view in foramen) C6-7 (*)
• Abnormal spinolaminar space/facet rotation
➢ “Bow-tie” sign Figure 4-14-4
• Spinous process rotation toward side of dislocation
Unilateral Facet Injury:

• CT
➢ “Naked” facet (may be subtle and partial)
➢ Contralateral facet subluxation common
➢ Articular mass fracture (73%) isolating pillar (17%), posterior
vertebral body fracture (25%)
➢ MRI/MRA – disc herniation and vertebral artery injury not
uncommon
Figure 4-14-5 Pictoral representation of a unilateral facet

injury (circle)
Figure 4-14-6
Radiographs (AP and lateral) of a unilateral facet injury (circle)

with subluxation. Note that on the AP film the spinous
processes above the level of injury are shifted to the left. Also
on the lateral film the facets below the level of injury are
projected as in a lateral projection whereas above the level of
injury they are obliqued consistent with the rotational Sagittal CT of a unilateral facet injury with
component of force locked facets (IF-inferior facet of the level
above; SF-superior facet of the level below)

Cervical Spine Trauma Figure 4-14-7
Facet Injury: Bilateral [Figures 4-14-7 and 4-14-8]
• Hyperflexion may be some rotation
• At least as common as unilateral injury
• Disrupted PLL, disc, and often ALL
• Unstable injury
• High incidence of cord damage
➢ (72% quadriplegia)
• Bilateral facet dislocation may be partial or complete
Bilateral Facet Dislocation:

Radiologic Characteristics [Figures 4-14-9 and 4-14-10]
• Anterolisthesis > 50% vertebral body diameter
• Dislocated inferior facets, anterior to superior facets
• Dislocated facets in foramen – oblique views
• Findings of hyperflexion – fanning, focal kyphosis, disc
narrowing Pictoral representation of a bilateral facet injury with perched
• Spinous processes not rotated facets
• CT – “naked” facets, small fracture fragments often not
seen on radiographs Figure 4-14-8
Figure 4-14-9
Pictoral representation of a bilateral facet injury with locked

facets
Figure 4-14-10
Lateral radiograph of a bilateral facet dislocation
with anterolisthesis (arrow) at C4-5. Also on the
lateral film the inferior facets (IF) of the level
above is anterior to the superior facets (SF) of
the level below
Sagittal CT’s of bilateral facet dislocation in two different

patients with the inferior facets (IF) of the level above anterior to
the superior facets (SF) of the level below

Cervical Spine Trauma: Flexion Teardrop Fracture Figure 4-14-11
[Figure 4-14-11]
• Most severe devastating flexion injury
• Usually lower cervical spine C5-6 (70% of cases)
• Diving accident shallow pool common cause
• Immediate, complete and permanent quadriplegia
(90% of cases)
• Acute anterior cord syndrome – loss pain,
temperature, and touch
➢ Retention position, motion, vibration (posterior
column senses)
Flexion Teardrop Fracture:

• Involved vertebrae and levels above in severe
flexion
• Vertebral body fracture with triangular fragment from Pictoral representation of a flexion teardrop injury
anteroinferior corner
• Central vertebral body not severely involved but
posteriorly displaced Figure 4-14-12
• Bilateral facet subluxation/dislocation
• MRI/MRA – disc herniation and vertebral artery injury
not uncommon
Cervical Spine Fracture:

Clay Shoveler Fracture
• Avulsion C7, C6, T1 spinous process
• Result of abrupt flexion against opposing
interspinous ligament
• Stable injury
• Oblique fracture spinous process
• May see “double’ spinous process sign
(AP radiograph)
• Spinous process fractures can also result from
extension/direct trauma
Radiographs (AP and lateral) of a flexion teardrop injury with
Cervical Spine Trauma: facet widening (circles and solid arrows), interspinous fanning
Hyperextension Injuries (*) and vertebral fracture with teardrop fragment anteriorly
[Figure 4-14-13] (open arrow)
• Usually caused by force to face/forehead
• Compression posteriorly, distraction anterior
• Less common than hyperflexion injuries (19% - 38%)
• Atlas and laminar fractures
• Hyperextension teardrop fracture Figure 4-14-13
• Hangman fracture
• Pillar fracture
Atlas Fractures
• Avulsion of anterior arch C1
➢ Rare stable injury
➢ Results from anterior atlantoaxial ligament
➢ Horizontal cleft in anterior arch (difficult on CT)
• Posterior C1 arch fracture
➢ Bilateral posterior fractures (no anterior
component)
➢ No anterior soft tissue swelling; stable
➢ Distinguish from normal congenital cleft
Laminar Fractures
• Lamina crushed on extension from above/below
• Often in older patients with spondylosis Pictoral representation of an extension injury
• Usually C5 to C7
• Difficult to detect on radiographs
• CT optimal
• Mechanically stable (Intact anterior column/facets)
• Neurologically unstable due to cord impingement by fragments

Hyperextension Dislocation Figure 4-14-14
• Common in older patients with spondylosis
➢ Also bone forming diatheses AS, DISH
• Rupture of ALL, disc and stripping of PLL (unstable)
• Patients usually severe neurologic symptoms
➢ Acute central cord syndrome
• Spinal cord impinged by subluxation and intact posterior
elements
• Often recoils back to relatively normal position
Hyperextension Dislocation: Radiographic

Characteristic [Figure 4-14-14]
• Relatively normal cervical alignment in quadriplegic patient
• Soft tissue swelling (100%)
➢ Only finding 33%
• Avulsed fragment anteroinferior vertebrae (65%)
➢ Longer horizontally (unlike extension teardrop fracture)
➢ In young patients ring apophysis, no neurologic deficit
• Widened disc anteriorly and vacuum (15%)
Hyperextension Fracture/Dislocation:
Pedicolaminar Fracture-Separation
• Combined hyperextension, compression and rotation
• Fractures of pillar, lamina, pedicles and spinous process opposite
side of translation Lateral radiograph of an extension fracture
• Vertebral body often mildly (3 – 6 mm) anteriorly displaced subluxation at C5-6 (arrow) in a patient with
• Spinous process not rotated ankylosing spondylitis (syndesmophytes-
arrowhead)
Hyperextension Fracture/Dislocation:
Pedicolaminar Fracture-Separation
Radiologic Characteristics
• Disc narrowing and vertebral rotation above injury
• Opposite facet may be widened/dislocated
• Commonly involve foramen transversarium
➢ Vertebral artery (MRA)
• Important to distinguish from flexion injury
Pillar Fracture
• Not common, 3% - 11% of cervical injuries (C6 – C7)
• Hyperextension and rotation
• Articular mass compressed on side of rotation
• Stable, radiculopathy common without cord damage
Pillar Fracture: Radiologic Characteristics [Figure 4-14-15]

• Subtle on radiographs Figure 4-14-15
• Disrupted lateral cortical margin (AP view)
• Visualize facets on AP radiographs
• Loss of posterior articular mass overlap
➢ Lateral radiograph (“double outline” sign)
• CT optimal – degree of fragmentation and
additional other fractures
➢ Pedicle, transverse process, lamina
Radiograph, AP tomogram and axial CT of an articular pillar fracture

with offset at the facet (solid arrows) and fracture (open arrow)
seperating the articular pillar from the remaninder of the vertebrae

Hyperextension: Figure 4-14-16
Teardrop Fracture
[Figures 4-14-16 and 4-14-17]
• Often occur in older osteoporotic patients
• Avulsion by ALL of triangular fragment
• Anterioinferior vertebral body (usually C2)
• Fragment vertical height same or larger than length
➢ Unlike avulsion with hyperextension dislocation
• Soft tissue swelling more prominent in younger
patients
• Unstable in extension
Traumatic Spondylolisithes:
“Hangman” Fracture (“Hangee” Fracture)
• Common 5% of all cervical spine injuries
• Hyperextension is probably transient modified by
flexion/compression/distraction Pictoral representation of an extension teardrop fracture
• Unstable injury
• Neurologic symptoms unusual unless distraction
➢ Large canal relative to cord at C2
Figure 4-14-17
➢ “Autodecompression” from bilateral
posterior fractures
Traumatic Spondylosithes:
[Figures 4-14-18 and 4-14-19]
• Effendi classification
➢ I: Minimally displaced fracture
➢ II: More displacement, involves C2 -
3 disc (widening)
➢ III: Bilateral facet dislocation
• Oblique C2 pedicle fracture – lateral view
• Mild anterolisthesis, posteriorly displaced
spinolaminar line
• Associated injuries-anterior corner
fractures C2/C3
➢ C1/high thoracic fractures (10%)
➢ Vertebral artery injuries
Lateral radiograph and tomogram of an extension teardrop fracture with
Figure 4-14-18 avulsed fragment (arrow) from the attached anterior longitudinal ligament (ALL)
Figure 4-14-19
Pictoral representation of the different types of traumatic

spondylolistheses
Lateral radiograph and axial CT of a type 1 traumatic spondylolisthesis

showing the linear nondisplaced fracture (arrows)

Axial Compression Injury: Figure 4-14-20
Burst Fracture
• Not common, 4% of cervical injuries
• Only occurs where cervical spine in neutral
position
• C1 – Jefferson fracture
• Lower cervical burst fracture C3 - 7
Jefferson Fracture [Figure 4-14-20]

• Axial compression drives occipital condyles
toward atlas
• Bilateral fractures anterior/posterior
➢ Lateral displacement
• Unstable, neurologic symptoms unusual
➢ Large neural canal
➢ Outward displacement of fragments
Jefferson Fracture:
[Figure 4-14-21] Pictoral representation of a Jefferson fracture
• Open mouth view best
➢ Laterally displaced lateral masses
• Lateral radiograph may only show soft tissue swelling; look Figure 4-14-21
for posterior fractures
• CT optimal for bilateral fractures
➢ Jefferson variants
• Lateral mass displacement > 7 mm / predental space > 6
mm = ruptured transverse atlantal ligament
• Small nondisplaced fragment medial to articular mass –
intact ligament
Cervical Burst Fracture

• Caused by vertical force driving nucleus pulposis through
endplate with body exploding from within
• Mechanically stable unless posterior ligament injury
• Neurologically unstable – deficit may progress
➢ Fragments change position
➢ Symptoms transient paresthesias to quadraplegia
Axial CT of a Jefferson fracture with four components
Cervical Burst Fracture: (arrows)
• Soft tissue swelling with straightening (but no kyphosis)
• Retropulsed fragments disrupted posterior vertebral body line
• Degree of vertebral body comminution variable
• Vertical fracture – midline/eccentric
• Disrupted joints of Lushka (facets)
Figure 4-14-22
Lateral radiograph, axial CT and sagittal CT of burst type fractures in different patients showing the comminuted fracture (circle),
retropulsed fragments (solid arrows) and fracture at the junction of the lamina and spinous process (open arrow)

Indeterminate Mechanism: Cervical Injuries Figure 4-14-23
• Odontoid fractures
• Occipitoatlantal dissociation
• Torticollis
• Rotary atlantoaxial subluxation/dislocation
Odontoid Fracture
• Most common of C2 fractures (41%)
• 11% - 13% of all cervical spine injuries
• Mechanism – flexion and or extension
• Other fractures (13%) – face, mandible,
posterior arch C1, extension teardrop,
hangman, atlantoaxial dissociation
• Anderson/D’Alonzo classification
Odontoid Fracture: Radiologic

Characteristics [Figures 4-14-23 to 4-14-26]
• Prevertebral soft tissue swelling(may be only
finding) Pictoral representation of different types of odontoid fractures
• Type I
➢ Rare (if occurs) avulsion at tip from alar Figure 4-14-24
ligaments
• Type II
➢ At base (60%-70%)
➢ May miss on CT
➢ High nonunion rate (72%), higher if displacement
> 5 mm
➢ Open mouth view (simulated by Mach effect);
atlantoaxial instability
➢ Os odontodeum distinguished by sclerotic
margins
• Type III (30%-40%)
➢ C2 body
➢ Disruption of Harris ring
➢ “Fat” C2 sign, invariably heal
Figure 4-14-25
Lateral radiograph of a type 2 odontoid fracture (arrows)
Figure 4-14-26
Open mouth radiograph of a type 3 odontoid fracture (arrows)
Coronal and sagittal CT of a type 3 odontoid fracture (arrows)

Cervical Spine Trauma: Role of MR Imaging Figure 4-14-27
• Thecal sac/spinal cord impingement
T1 T2
• Disc herniation/extrusion: 20% - 40% patients
➢ Highest (100%) in patients with anterior cord
syndrome
• Epidural hematoma (1% - 2%); spinal cord
edema/hematoma
• Ligamentous disruption; cervical spondylosis
• Subsequent complications
➢ Syringomyelia, myelomalacia
MR Imaging: Spinal Cord Injury

• Intramedullary swelling
➢ T1W – increased cord caliber
➢ T2W – increased signal
• Intramedullary edema
➢ T2W – increased signal
• Intramedullary hemorrhage Sagittal T1 and T2-weighted MR images show high signal
intensity in the spinal cord (arrows) that subsequently reveals
➢ Variable MR appearance (often
low signal intensity on T2-weighting on a follow-up MR all
heterogeneous) indicative of hemorrhage as seen on the gross specimen
➢ Poor prognostic sign
T2
MR Imaging: Intramedullary
Hemorrhage [Figure 4-14-27]
• Oxyhemoglobin
➢ Hyperacute (minutes – hours)
➢ Intermediate signal T1W
➢ High signal T2W
• Deoxyhemoglobin
➢ Usually first 24 hours
➢ Can be up to 8 days with hypoxia
➢ Intermediate signal T1W
➢ Low signal T2W
• Methemoglobin
➢ Usually after 24 hours
➢ High signal T1W (begins peripherally)
➢ Low signal T2W (early subacute
intracellular)
➢ High signal T2W (late subacute
extracellular)
References
1. Blackmore CC, Mann FA, Wilson AJ.. "Helical CT in the primary trauma evaluation of the cervical spine: an
evidence-based approach." Skeletal Radiol. 2000 Nov;29(11):632-9. Review.
2. Jarolimek AM, Coffey ECC, Sandler CM, West OC. "Imaging of uppercervical spine injuries -- Part III: C2
below the dens." Applied Eadiology. 2004 July; 9-21.
3. Murphey MD, Batnitzky S, Bramble JM. "Diagnostic imaging of spinal trauma." Radiol Clin North Am. 1989
Sep;27(5):855-72.
4. Stabler A, Eck J, Penning R, Milz SP, Bartl R, Resnick D, Reiser M.. "Cervical spine: postmortem assessment of
accident injuries--comparison of radiographic, MR imaging, anatomic, and pathologic findings." Radiology. 2001
Nov;221(2):340-6.

Radiographic Differential Diagnosis of the
Jaws
Christopher G. Fielding, COL, DC, USA
Generalities
• Dentists like plain films
• Act as radiologist
➢ Oral & Maxillofacial Surgery
➢ Oral & Maxillofacial Radiology
Dental Anatomy Review

• Primary dentition (deciduous)
➢ 20 teeth
➢ Eruption starts at 6 months
➢ Completed eruption sequence 3 years
• Permanent dentition
➢ 32 teeth
➢ Eruption starts at 6 years
➢ Completed eruption sequence 12 years
❖ Except 3rd molars (wisdom teeth)
Primary Dentition
Eruption/Exfoliation
Tooth Numbering
Primary Tooth Numbering
Primary tooth “numbering”
Tooth Numbering
Imaging Techniques
• Intraoral
➢ Bitewing
➢ Periapical
➢ Occlusal
• Extraoral
➢ Panoramic
➢ AP, PA, lateral, oblique, Water’s, Townes
➢ CT, MRI, technetium scan
Bitewing
Radiographic Description
• Size : in centimeters
• Border: well circumscribed, poorly circumscribed, ill-
defined
• Shape: unilocular, multilocular, uniform, irregular
• Number: focal, multifocal
• “Color”: radiolucent, radiopaque, mixed;
➢ buzzwords: “ground glass”, “cotton wool”
• Location: exact location within the maxilla or
mandible; location in relation to adjacent structures
(periapical, interradicular, pericoronal, etc)
• Teeth
➢ Impaction, displacement, or resorption
• Periodontal supporting structures
➢ Periodontal ligament space enlargement or loss
Tooth numbering (adult)
of the lamina dura
Musculoskeletal Radiology 849 Radiographic Differential Diagnosis of the Jaws

Features “Unique” to Dental Radiographs Figure 4-15-1
• Dental anatomy
➢ Crown of tooth
➢ Periodontal ligament
➢ Lamina dura
❖ Artifactual thin white line around roots of teeth
➢ Root canal
➢ Apex of root
➢ Inferior alveolar canal
❖ Above - think odontogenic
❖ Below - think fibro-osseous & developmental
Overview
• Radiolucent lesions
➢ Periapical
➢ Pericoronal
➢ Multilocular
• Radiopaque/mixed density lesions
➢ Periapical
➢ Interradicular
➢ Multifocal confluent
➢ Target lesion
• Osteosarcoma of the gnathic skeleton
Periapical cyst/periapical granuloma
Radiolucent Lesions: Periapical
• Periapical granuloma
• Periapical cyst
• Traumatic bone “cyst” Figure 4-15-2
• Nasopalatine duct cyst
• Early focal cemento-osseous dysplasia
Periapical Granuloma and Periapical Cyst

[Figure 4-15-1]
• Inflammatory lesion progressing from nonvital pulp as a
sequelae of caries or trauma
• Pain with or without swelling and drainage
• Radiographic
➢ Circumscribed or diffuse radiolucency which destroys the
periodontal ligament space and lamina dura
➢ Usually limited in size
• Differential diagnosis:
➢ Immature periapical cemental dysplasia (tooth is vital)
➢ Immature cementoblastoma (vital tooth, usually mandibular
molars)
➢ Traumatic bone cyst (vital teeth, intact lamina dura)
• Treatment
➢ Endodontic (root canal therapy)
➢ Extraction
Nasopalatine Duct Cyst [Figure 4-15-2]

• Aka incisive canal cyst Nasopalatine duct cyst (must exceed 6 mm in
• Most common non-odontogenic cyst in the oral cavity diameter, the upper limit for the normal incisive
(developmental cyst) canal)
• Swelling of anterior palate, pain, drainage
• 4th – 6th decades
• Well-circumscribed ULRL midline of palate, apical to central incisors
• Treatment
➢ Surgical enucleation
➢ Recurrence is rare
Radiographic Differential Diagnosis of the Jaws 850 Musculoskeletal Radiology

Fibro-osseous lesions Figure 4-15-3
• Cemento-osseous dysplasia
➢ Periapical
➢ Focal
➢ Florid
• Ossifying fibroma
Cemento-osseous Dysplasia
• Focal
➢ Single site
➢ 90% occur in F
➢ Whites > blacks
➢ Posterior mandible most common site
❖ Many occur in extraction sites
➢ Most lesions smaller than 1.5 cm in diameter
➢ Well defined
• RL ---> mixed density ---> RO
➢ Early lesions RL
➢ Calcification with maturation of lesion
Cemento-osseous Dysplasia [Figures 4-15-3 and 4-15-4]

• Periapical Periapical cemento-osseous dysplasia (early)
➢ Anterior mandible
➢ F predilection (10:1, 14:1) Figure 4-15-4
➢ 70% occur in blacks
➢ Pulps are vital
❖ Teeth are usually unrestored
➢ Asymptomatic
➢ Incidental finding
• RL --->mixed density --->RO
➢ Calcification with maturation of lesion
Cemento-osseous Dysplasia [Figures 4-15-5 and 4-15-6]

• Florid
➢ Multifocal, not limited to anterior mandible
➢ Black F
❖ Usually middle-aged
➢ Pulps are vital
➢ Asymptomatic
❖ Dull pain
❖ Occasional expansion or sinus tract
➢ Incidental finding
• RL --->mixed density --->RO
➢ Calcification with maturation of lesion Periapical cemento-osseous dysplasia
(late/mature)
Florid cemento-osseous dysplasia

Gardner Syndrome
Cemento-osseous Dysplasia Figure 4-15-7

• Treatment/prognosis
➢ No treatment required
➢ Clinical & radiographic diagnosis in most cases
➢ Surgery, extraction, biopsy of sclerotic lesions
❖ Chronic osteomyelitis
Radiolucent Lesions: Pericoronal

• Dentigerous cyst
• Ameloblastoma
• Ameloblastic fibroma
• Odontogenic keratocyst
Dentigerous cyst involving an impacted
Dentigerous Cyst [Figure 4-15-7] mandibular 3rd molar
• The most common developmental odontogenic cyst
• Derived from reduced enamel epithelium of the dental follicle
Figure 4-15-8
• Radiographic
➢ Well demarcated radiolucency that may be extensive
➢ Encompasses the crown of an unerupted or impacted tooth
➢ Third molars and cuspids of young adults most commonly
involved
• Rarely may give rise to:
➢ Ameloblastoma
➢ Mucoepidermoid carcinoma
• Treated by enucleation
Radiolucent Lesions: Multilocular (Macho-Macho)

• Central giant cell granuloma
• Ameloblastoma
• Cherubism
• Odontogenic myxoma
• Hemangioma/AV malformation
• Odontogenic myxoma
• Botryoid odontogenic cyst
Multilocular RL of the Mandible

• Multilocular
• Aneurysmal bone cyst
• Cherubism
• Odontogenic fibroma
Can Cross Midline
• Myxoma (odontogenic)
• Ameloblastoma
• Central giant cell lesion
• Hemangioma/A-V malformation
Odontogenic Keratocyst [Figure 4-15-8]

• The most common multilocular radiolucency
• Usually presents in adults
• Up to 3/4 in posterior mandible and ramus
• Radiographic
➢ Well-defined unilocular, or more commonly multilocular
radiolucency with sclerotic borders
Odontogenic keratocyst involving the posterior
➢ Usually grows in a relatively linear direction mandible and ramus

❖ Obvious expansion may NOT be seen, even with large cysts
➢ Often associated with an impacted tooth
• May grow aggressively with:
➢ Cortical perforation
➢ Tooth displacement and/or resorption
➢ Soft tissue extension
Odontogenic Keratocyst
• Treated by thorough enucleation and curettage
• Occasionally, en bloc resection required
• Up to 1/3 will recur
• Long term radiographic follow-up requiredfollow-up quiredfo
• If multiple, evaluate for nevoid basal cell carcinoma syndrome
(Gorlin syndrome)
Figure 4-15-9
Nevoid Basal Cell Carcinoma Syndrome

(Basal Cell Nevus Bifid Rib Syndrome; Gorlin
Syndrome) [Figure 4-15-9]
• Teenagers, both male and female
• Autosomal dominant disease
• Symptom complex characterized by:
➢ Numerous basal carcinomas of the skin
➢ One or more bifid ribs
➢ Multiple odontogenic keratocysts Multiple odontogenic keratocysts associated with nevoid
❖ The keratocysts are identical to the solitary ones basal cell carcinoma syndrome (Gorlin Syndrome)
❖ Recurrence of OKC’s is the rule in this syndrome
Bifid Rib – Basal Cell Carcinoma Syndrome

(Nevoid Basal Cell Carcinoma Syndrome; Gorlin’s Syndrome)
• Other findings include: Figure 4-15-10
➢ Palmer/planter pitting pitting
➢ Frontal bossing
➢ Calcified falx cerebri
➢ Tendency to develop medulloblastomas
➢ Many others…
• Long-term periodic radiographic follow-up is needed
Central Giant Cell Granuloma [Figure 4-15-10]

• Aggressive reactive process more often in mandible
• Young adults with female predilection
• Radiographic
➢ Unilocular, or more commonly multilocular
radiolucency with well demarcated margins
➢ The most common anteriorly located multilocular
lucency
❖ May cross the midline
Central giant cell granuloma
➢ May cause expansion of the involved bone with
thinning of the cortex
➢ Root divergence and smooth, concave root resorption is common
Central Giant Cell Granuloma

• Histology
➢ Highly cellular fibrovascular stroma with…
❖ Dispersed multinucleated giant cells
❖ Osteoid or osseous trabeculae
❖ Extravasated blood and hemosiderin
➢ Brown tumor of hyperparathyroidism is histologically indistinguishable
❖ Serum calcium determination required
• Surgical enucleation with extraction(s) if necessary
➢ May also try hormonal therapy with calcitonin
• May recur if incompletely removed

Ameloblastoma [Figure 4-15-11] Figure 4-15-11
• Most common odontogenic neoplasm
• Average age is 34 with no sex predilection
• Presents as a painless expansile lesion
• Most common location is posterior mandible and
ramus
• Maxillary lesions may extend to nasal cavity and base
of skull
• Radiographic
➢ Classically a “soap-bubble” multilocular
radiolucency with clearly demarcated borders
➢ Unilocular (“unicystic”) ameloblastomas possible
➢ Most are unassociated with impacted teeth
➢ May displace and/or resorb teeth
➢ Centrifugal growth
❖ May reach gigantic proportions
Ameloblastoma
• Treatment:
➢ Curettage or en bloc resection for mandibular lesions
➢ Resection for maxillary lesions
➢ Recurrence rate is high
➢ Unilocular ameloblastomas have a better prognosis with rare
recurrence and requiring only simple enucleation
➢ Rare malignant transformation has been reported
Cherubism [Figure 4-15-12]

• Rare developmental jaw condition that is generally inherited as an
AD trait
• Usually occurs between ages 2-5 YO
• Bilateral involvement of the posterior mandible developing
characteristic “cherub-like facies”
➢ Maxillary involvement can occur
• Expansile multilocular radiolucency
➢ May cause tooth displacement, eruption failure, Ameloblastoma
impair mastication, speech difficulties
• Treatment:
➢ Prognosis in any given case is unpredictable. Most cases demonstrate
varying degrees of remission and involution after puberty.
➢ Radiotherapy contraindicated
Radiopaque and Mixed Lesions: Periapical

• Periapical cemento-osseous dysplasia
• Cementoblastoma
• Erupting teeth
• Endodontic procedures
• Hypercementosis
• Idiopatic osteosclerosis Figure 4-15-12
• Focal scerosing osteomyelitis
Cherubism (bilateral multilocular radiolucencies)

Cementoblastoma [Figure 4-15-13] Figure 4-15-13
• Benign cemento-osseous tumor, most commonly associated with
a root of a mandibular molar
➢ Usually 1st molar
➢ Some authorities consider this a simple variant of
osteoblastoma
• M=F; < 25y of age
• Slow growth with possible expansion
• Sometimes painful
• Radiographic
➢ Calcified, highly radiodense mass intimately associated with
the root
➢ Root outlines obscured
➢ Usually surrounded by radiolucent rim
• Tx/Prognosis
➢ Excision or root amputation/endodontics Cementoblastoma
➢ May continue growing if incompletely excised, otherwise no recurrence
Idiopathic Osteosclerosis [Figure 4-15-14]

• Focal area of increased radiodensity of unknown cause Figure 4-15-14
➢ Vital pulp
• M=F; arise 1st – 2nd decade
➢ May remain static or slowly increase in size
➢ Once skeletal growth stops, the lesions become static
• 90% in mandible, usually 1st molar region
➢ Usually involving a root apex
• Multifocal in some cases
• XR
➢ Well-defined, rounded or elliptic radiodense mass
➢ No RL rim
➢ 3 mm – 2 cm Idiopathic osteosclerosis
Focal Sclerosing Osteomyelitis [Figure 4-15-15]

• Aka condensing osteitis
• Localized increased radiodensity Figure 4-15-15
• Apex
➢ Widened PDL space or periapical RL
• Molar-premolar areas of mandible commonly
Condensing Osteitis
• Tx/prognosis
➢ Endodontic/ext
➢ 85% regress or resolve
➢ Bone scar
❖ Residual lesion
Focal sclerosing osteomyelitis (condensing
Radiopaque and Mixed Lesions: Interradicular osteitis)
➢ Active ossifying fibroma
• Focal cemento-osseous dysplasia
• Osteoblastoma
• Adenomatoid odontogenic tumor
• Odontoma

Ossifying Fibroma [Figure 4-15-16] Figure 4-15-16
• Only true neoplasm in the “benign fibro-osseous lesion”
category
• Seen more often in teenagers and young adults
• No sex predilection
• Radiographic
➢ Well defined mixed lesion with sclerotic borders
➢ Density will vary with the maturity of the lesion
➢ Tooth bearing and non-tooth bearing areas, especially
posterior mandible
➢ Growth is radial instead of linear (as in fibrous dysplasia)
❖ Expansion buccally and lingually will thin cortex
❖ Bowing of inferior border is characteristic
❖ May expand alveolar crest
• Treatment
➢ Thorough excision of mass
➢ Mass tends to “shell out” easily from the surrounding bone
➢ Up to 1/3 may recur
Radiopaque and Mixed Lesions: Multifocal

Confluent
• Florid cemento-osseous dysplasia
• Paget’s disease of bone
• Chronic sclerosing osteomyelitis
• Gardner syndrome
• Multiple tori and exostoses
Radiopaque and Mixed Lesions: Ground Glass Ossifying fibroma

• Osteopetrosis
Fibrous Dysplasia
• Occurs in children, teenagers and young adults of both sexes Figure 4-15-17
• Four main forms: monostotic, polyostotic, McCune-Albrights,
and craniofacial
➢ Craniofacial FD may involve multiple contiguous bones in
the midface and cranium
➢ McCune-Albright’s disease includes polyostotic FD, focal
skin hyperpigmentation (“café-au-lait spots”) and endocrine
disturbances (usually precocious puberty and/or
hyperthyroidism)
• Painless expansile process of osteoprogenitor tissue
• Slow growth with facial deformity
• More common in the maxilla Fibrous dysplasia (“ground glass”
appearance)
Fibrous Dysplasia [Figure 4-15-17]

• Radiographic
➢ Mature maxillary lesions are homogeneous, “ground glass” or “peau
d'orange”
➢ No margination or borders and blends with adjacent trabecular bone
➢ May obliterate the maxillary sinus
➢ Mandibular lesions more likely mottled or multicystic
➢ Skeletal survey to rule out polyostotic disease
➢ Use plain films or CT as MRI does not demonstrate the traditional
radiographic findings
• Defer surgical treatment (cosmetic recontouring) until skeletal maturity
• Growth may cease, continue, or resume after periods of quiescence
• Quarterly follow-up
• Small chance of sarcomatous transformation, usually osteosarcoma or
fibrosarcoma
➢ Especially in patient’s with a history of radiotherapy

Radiopaque and Mixed Lesions: Target Lesion, Dense Figure 4-15-18
• Odontoma
• Osteoma
• Focal cemento-osseous dysplasia
• Ameloblastic fibro-odontoma
• Osteoblastoma
Odontoma [Figure 4-15-18]

• Equal sex predilection
• More common in teenagers and young adults
• A mixed odontogenic tumor / hamartoma
• Asymptomatic but may prevent tooth eruption
• Types:
➢ Compound
❖ Target lesion with central tooth-like structures
❖ More common in anterior jaws between teeth
➢ Complex
❖ Well demarcated opacity with frayed margins
❖ May have a radiolucent rim
❖ More common in posterior jaws (pericoronal) Compound odontoma
• Treated by enucleation
• Does not recur
Osteosarcoma [Figure 4-15-19]

• Malignancy of mesenchymal cells
• M>F
• Extragnathic Figure 4-15-19
➢ Bimodal (2nd – 3rd decades, 6th decade)
❖ Distal femur/proximal tibia
❖ Axial skeleton/flat bones
• Gnathic
➢ 3rd – 4th decades
➢ Maxilla = mandible
• XR
➢ RL, mixed, RO
➢ Ill-defined periphery
➢ “Sunburst” or “sunray”
❖ 25% of gnathic lesions
Osteosarcoma (widening of the periodontal
➢ “Spiking” root resorption ligament space)
➢ Widening of PDL space
• Treatment/prognosis
➢ Gnathic lesions low-grade??
➢ Radical surgical extension
❖ Best hope for cure
➢ Preoperative chemotherapy
➢ Local uncontrolled dz
❖ Leading cause of death
❖ Usually within 2 years of initial tx
• Risk factors
➢ Paget’s disease
➢ H/O radiation

MRI of the Knee: Part 1
Mark Anderson, MD
Lecture Outline
• Part 1
➢ Technique
➢ Menisci
• Articular cartilage
• Part 2
➢ Bones
➢ Stabilizers
➢ Miscellaneous
Technique
• Surface coil
• High resolution
• T1, T2, fat suppression
• Sagittal, coronal, axial planes
Technique: Pulse Sequences

• T1
➢ Overall anatomy
➢ Menisci
➢ Bones (marrow)
➢ Fat/hemorrhage Figure 4-16-1
➢ Muscle
• T2
➢ Fluid / edema
➢ Tendons, ligaments
➢ Soft tissue injury
• Fast Spin Echo-T2
➢ T2 contrast – faster acquisition
➢ Caution:
❖ Bright fat (marrow pathology)
❖ Blurring effect – proton density (meniscal
tears)
• Gradient Echo (T2*)
➢ Menisci
➢ Articular cartilage (3D)
➢ Susceptibility effects Cadaveric specimen of the menisci
➢ Caution: Marrow Pathology
• STIR (Fat suppressed T2)
➢ Marrow pathology Figure 4-16-2
➢ Soft tissue injury
➢ Articular cartilage
Summary: Pulse Sequences

• Menisci
➢ Short TE (T1, PD, GRE) - caution with FSE
• Bone Marrow
➢ Fat saturation (STIR, Fat Sat FSE T2) - not GRE
➢ T1W in one plane
• Other soft tissues (ligaments, tendons)
➢ T2W with fat saturation (STIR, Fat Sat FSE T2)
• Cartilage
➢ Contrast between fluid and cartilage
Schematic diagram of a meniscus cut in cross-section (upper);
normal sagittal image of posterior horn of the medial mensicus
(lower); normal meniscus at arthroscopy (right)
MRI of the Knee: Part 1 858 Musculoskeletal Radiology

Technique: Imaging Planes Figure 4-16-3
• Sagittal
➢ Menisci
➢ Cruciate ligaments
➢ Extensor tendons
➢ Articular cartilage
➢ Bones
• Coronal
➢ Collateral ligaments Sagittal gradient
echo images
➢ Menisci
(corresponding
➢ Articular cartilage to lines on
➢ Bones diagram) through
• Axial the medial
➢ Patellofemoral joint meniscus
➢ Muscles / tendons Figure 4-16-4
➢ Popliteal fossa
Menisci [Figures 4-16-1 and 4-16-2]

• Fibrocartilage
• Medial/lateral
• Functions:
➢ Joint congruity
➢ Shock absorption Sagittal gradient
➢ Load transmission echo images
(corresponding
to lines on
Medial Meniscus [Figure 4-16-3] diagram) through
• Larger “C” the lateral
• Posterior horn > anterior horn meniscus
• Attached more tightly to the capsule
• Covers 1/2 contact surface of tibial plateau
Lateral Meniscus [Figure 4-16-4]

• Tighter “C”
• Posterior horn = anterior horn
Figure 4-16-5
• Attached more loosely to the capsule
• Covers ¾ contact surface of tibial plateau
• Popliteus tendon (fascicles)
Menisci: Attachments
[Figures 4-16-5 and 4-16-6]
• Tibia
• Capsule Figure 4-16-6
• Ligaments
➢ Transverse
➢ Meniscofemoral Normal transverse intermeniscal ligament
❖ Humphrey
❖ Wrisberg
➢ Oblique menisco-
meniscal
Diagram of the knee (posterior view)

demonstrating the meniscofemoral ligament of
Wrisberg (arrow).
Musculoskeletal Radiology 859 MRI of the Knee: Part 1
Menisci: Variants Figure 4-16-7
[Figures 4-16-7]
• Discoid
➢ Enlarged meniscus
➢ Embryologic, congenital?
➢ Lateral > medial
➢ Prone to tear
➢ Complete / incomplete
➢ Wrisberg variant
• Buckled A. Diagram illustrating a discoid lateral meniscus (L) from above.
➢ “Lax”, “Flounce” B. Normal sized medial meniscus (thin arrow) and enlarged,
➢ Medial meniscus discoid lateral meniscus (large arrow)
➢ Ligament injury/laxity
➢ Positional Figure 4-16-8
• Ossicle
➢ Vestigial, post-traumatic?
➢ May be symptomatic
➢ PHMM most common
➢ Variable MRI signal intensity
Meniscus
• Microstructure
➢ Collagen bundles
❖ Circumferential
❖ Transverse (“tie fibers”)
➢ Resist longitudinal loading
❖ “hoop stresses”
Menisci: Pathology
• Degeneration
• Tear
➢ traumatic vs. degenerative
• 20% asymptomatic pts > 50 y.o. show MR evidence of tear
A. Diagram of a meniscus cut in cross-section
Surgical Considerations revealing an oblique undersurface tear.
• Primary goal B. Similar tear in the posterior horn of the
➢ Preserve as much meniscal tissue as possible medial meniscus on sagittal image
Meniscal Tear: MRI

• Abnormal
• Signal intensity -Morphology
Meniscal Tear: MRI [Figure 4-16-8]

• Signal intensity (Grades)
➢ 1 – Globular
➢ 2 – Linear
➢ 3 – Contact with articular surface ==> tear
Meniscal Tear: “Close”

• Kaplan
➢ 13/20 (65%) no tear
• DeSmet
➢ 1 image only… 30%–55%
➢ > 1 image….... 90%
• Don’t overcall… be descriptive
Meniscal Tear: MRI

• Morphology
➢ Blunted, truncated
➢ Size

Meniscal Tears [Figure 4-16-9] Figure 4-16-9
• Vertical
➢ Radial
➢ Longitudinal
➢ Traumatic
➢ Divides into ant/post or med / lat fragments
• Horizontal
➢ Degenerative
➢ Divides into sup / inf fragments
Meniscal Tears [Figures 4-16-10 and 4-16-11]

• Radial
➢ Perpendicular to axis
➢ Vertical
➢ Traumatic or degenerative
➢ Meniscal subluxation
➢ Irreparable?
Figure 4-16-10
Diagram of radial tear
Figure 4-16-11 A. Radial tear.

B. Longitudinal tear.
C. Horizontal tear.
Figure 4-16-13
Radial tear involving the body of the lateral meniscus (arrow)
Figure 4-16-12
Meniscal Tears [Figure 4-16-12]
• Longitudinal
➢ Vertical
➢ Along axis of meniscus
➢ Bucket handle
❖ displaced fragment
❖ medial meniscus
❖ locking
➢ Peripheral
❖ potentially reparable
❖ outer 1/3 (red zone)
Longitudinal tear
Peripheral Tears [Figure 4-16-13]
• Outer 1/3 of meniscus
• Vascular region
➢ (red/red zone)
• Tend to heal A. Diagram of a peripheral tear.
➢ primary repair B. Peripheral tear involving the posterior horn
of the medial meniscus (arrow).
➢ conservative therapy

Meniscocapsular Separation Figure 4-16-14 Figure 4-16-15
• PHMM
• Fluid at meniscocaps interface
• Poor sensitivity/PPV
Meniscal Tears [Figure 4-16-14]

• “Parrot Beak” Tear
➢ Combination
❖ Radial + longitudinal
❖ Oblique to long axis
➢ Meniscal Flap
Parrott beak
Meniscal Tears [Figure 4-16-15] tearParrott beak tear
• Horizontal
➢ Often degenerative
➢ May be asymptomatic
• Meniscal cysts
Meniscal cyst [Figure 4-16-16]

• Parameniscal/intrameniscal A. Diagram of a horizontal tear.
• Lateral > medial B. Horizontal tear of the body of the meniscus
• Horizontal tear (white arrow) and an associated parameniscal
cyst (black arrow)
Displaced Meniscal Tears [Figures 4-16-17 to 4-16-21] Figure 4-16-16
• Bucket Handle (medial)
• Flipped (lateral)
• Gutter
• Extruded
Figure 4-16-17
Bucket Handle Tear
[Figures 4-16-17 to 4-16-19]
• Coronal
Sensitivity
➢ Displaced fragment (94%)
➢ Blunted body (64%)
• Sagittal
➢ Too few “bowties” (97%)
➢ Double PCL (30%)
Helms AJR 1998 Diagram of a bucket
handle tear of the Horizontal tear with associated parameniscal
meniscus cyst
Coronal image corresponding to the dashed line Sagittal image corresponding to the dashed line shows the
demonstrates the displaced meniscal fragment of this bucket large bucket handle fragment within the notch creating the
handle tear (large arrow) and irregular, truncated body of the “double PCL” sign (P = PCL)
meniscus (thin arrow)

A. Diagram of a “flipped” (longitudinal) meniscal tear.

B. Sagittal image at the level of the dashed line shows the
flipped fragment (short arrow) adjacent to the anterior horn
(long arrow) creating the “double anterior horn” sign. Note also
the small residual posterior horn (circle).
Menisci: Post-surgical
• Truncated, absent, “normal”
• Healed tear can look like new tear
• MR arthography
➢ 0.2 cc Gd + 20 cc saline
➢ T1W with fat-saturation
➢ Gd extending into tear
Menisci: Post-surgical A. Coronal image demonstrating a displaced

• White et al. Radiology, February 2002 meniscal fragment along the medial joint line
➢ 364 patients (arrow).
B. Diagram illustrating the horizontal tear and
➢ Conventional MR flipped fragment
➢ Indirect MR arthrography
➢ Direct MR arthrography
• Direct slightly more accurate, but no significant difference
Menisci: Post-surgical
• Signs of new or recurrent tear?
➢ Fluid in tear
➢ Displaced fragment
➢ Tear in new area
Meniscal Tear: MRI

• Accuracy > 90%
➢ 50% - sagittal only
➢ 3% - coronals only
➢ FSE ~ 80% (?)
• Problem areas:
➢ Free edge
➢ PHLM
Meniscal Tear: Pitfalls

• Anatomy
➢ Transverse ligament
➢ Meniscofemoral ligaments
➢ Oblique meniscomeniscal lig
➢ Lateral inf geniculate artery
➢ Popliteus tendon
➢ Edge artifact
Meniscal Tear: Pitfalls

• Artifacts
➢ Patient motion
➢ Phase artifact (artery)
➢ Magic angle
➢ Gas/hemosiderin
➢ Chondrocalcinosis

Take Home Points
• Meniscal tear?
➢ Signal intensity and morphology
• Small meniscus?
➢ Find the missing fragment
• Healed tear?
➢ Can look just like a new tear
• True pathology or pitfall?
To most easily identify pathology, know the normal anatomy
References
1. Helms CA, Laorr A, Cannon WD, Jr. The absent bow tie sign in bucket-handle tears of the menisci in the knee.

MRI of the Knee: Part 2
Mark Anderson, MD
Lecture Outline Figure 4-17-1

• Part 1
➢ Technique
➢ Menisci
➢ Articular Cartilage
• Part 2
➢ Bones
➢ Stabilizers
➢ Miscellaneous
Bones
• Femur, tibia, patella, fibula Spectrum of acute osseous injuries
• Cortical
➢ compact
➢ subchondral plate
• Cancellous
➢ trabecular Figure 4-17-2
Trabecular Bone
• 10 x load-bearing capacity of cortical bone
• Dissipates forces
• Support for subchondral plate
Bones: Injuries
• Acute
➢ Impaction (contusion, occult fracture)
➢ Avulsion
• Chronic
➢ Fatigue, insufficiency fracture
• Spontaneous osteonecrosis
• Osteochondritis dissecans
Acute Impaction Injuries: Spectrum [Figure 4-17-1]

Focal contusion involving the posterolateral
Acute Trauma: Impaction tibial plateau
• Contusion, bone bruise
• Edema, hemorrhage
• Trabecular fx
• Detection
➢ Explains symptoms
➢ Avoids unnecess arthroscopy
➢ Mechanism of injury
➢ May change Rx plan
Acute Trauma: Contusion [Figure 4-17-2]

• MRI
➢ Reticular
➢ Ill-defined margins
• − T1, + STIR
• 100% heal; 2–4 months

Geographic Contusion [Figure 4-17-3] Figure 4-17-3
• Subchondral
• Cartilage damage
➢ softening, fissuring,
➢ chondral fx
• − proteoglycans*
• Long term sequelae?
• Protect during healing
*Johnson, AJSM, 1998
Focal subchondral contusion along the

posterior weight-bearing portion of the lateral
femoral condyle
Contusion Patterns: ACL Tear [Figure 4-17-4] Figure 4-17-4

• Lat. femoral condyle (sulcus terminalis)
• Post-lateral tibial plateau
• Specific
➢ 95% (adults)
➢ 75% (children)
• “Contrecoup”
➢ Post-medial tibial plateau
➢ Peripheral injury
Patterns: Patellar Dislocation [Figure 4-17-5]

• Lateral dislocation
• Contusions
➢ Lat. femoral condyle
❖ Anterior / non-wgt bearing
➢ Medial patella
• Medial retinacular injury
• Cartilage injury “ACL” contusion pattern (lateral femoral
• Avulsion fracture condyle and posterolateral tibial plateau)
Figure 4-17-5
Acute Trauma: Fracture [Figure 4-17-6]
• Linear
➢ T1
❖ – signal intensity
➢ STIR
❖ + or – signal intensity
Figure 4-17-6
Patellar dislocation contusion pattern (lateral

margin of lateral femoral condyle
and medial patella)
Occult fracture of the lateral tibial plateau on sagittal T1-
weighted and coronal fat-saturated T2-weighted images

Knee Stabilizers Figure 4-17-7
• Central: ACL, PCL
• Medial: MCL
• Lateral:
➢ Iliotibial band
➢ Fibular collateral lig.
➢ Biceps femoris tendon
• Anterior: Patellar Retinacula
Cruciate Ligaments [Figure 4-17-7]

• Intracapsular, extrasynovial
• Intercondylar notch
• Anterior (lateral)
• Posterior (medial)
ACL [Figure 4-17-8]

• LFC --> anterior tibia Diagram of the knee (frontal view)
• Restricts ant displacement of tibia demonstrating the anterior (A) and posterior (P)
• MRI cruciate ligaments
➢ low SI
➢ usually striated
➢ taut Figure 4-17-8
ACL Tear
• 75% of all ligament injuries
➢ Twisting + valgus force
➢ Hyperextension
• Associated injuries
➢ Meniscal tear (40%–70%)
❖ O’Donoghue’s Triad
❖ ACL, MCL, medial meniscus (?)
(Lateral / Medial about equal)
• 70% mid substance; 20% proximal; 10% distal
ACL Tear: MRI [Figure 4-17-9]

• MRI – 90%–95% accurate
• Primary Signs Normal anterior cruciate ligament
➢ Edematous mass (48%)
➢ Non-visualization (“empty notch”) (18%)
➢ Disruption (11%)
➢ Irregular, wavy, horizontal contour
➢ Focal + SI Figure 4-17-9
ACL Tear: MRI

• Secondary Signs
➢ Bone contusions
➢ Deep notch LFC
➢ Segond fracture
❖ 10% ACL tears fx
❖ 75–100% fx - ACL tear
➢ “Anterior drawer”
❖ (uncovered PHLM)
Chronic ACL Tear: MRI

• Non-visualization
• Focal angulation
• Fragments
• “Normal” (scar)
• Without Edema Complete ACL rupture

Post-Op ACL [Figure 4-17-10] Figure 4-17-10
• Graft
➢ Integrity
➢ Signal Intensity
❖ Variable, especially early
➢ Roof Impingement
➢ “Cyclops” lesion
❖ Anterior arthrofibrosis
PCL [Figure 4-17-11]

• MFC -> posterior tibia
• Restricts post tibial displacement
Left: Normal ACL graft (dashed arrow).
• MRI Right: Cyclops lesion (arthrofibrosis) along ventral margin of
➢ Low signal intensity ACL graft (arrow).
➢ Arched
Figure 4-17-11
PCL Tear [Figure 4-17-12]
• Force -> ant tibia – flexed knee
➢ “Dashboard” injury
• Complete Tear (45%)
➢ Midsubstance
• Partial Tear (47%)
• Avulsion (8%)
Medial Collateral Ligament

[Figure 4-17-13]
• Superficial component
• Bursa
• Deep component
➢ Meniscofemoral
➢ Meniscotibial
Normal PCL
MCL Injuries: MRI [Figure 4-17-14]
Grade Clinical MRI
1 Sprain Thickened
Irregular
Adjacent edema
2 Partial Tear Focal SI
3 Complete Tear Discontinuity
Figure 4-17-12
Figure 4-17-13
Full thickness PCL tear (midsubstance)
Normal MCL

Lateral Stabilizers [Figure 4-17-15] Figure 4-17-14
• Biceps femoris tendon
• Fibular (lateral)
➢ collateral ligament
• Iliotibial tract
Posterolateral Corner Injuries

• Hyperextension with varus force
• Isolated injuries rare
➢ often with cruciate injuries
➢ PCL most common
• Urgent exploration indicated
➢ within 3 days – 3 weeks
➢ reconstruct with cruciate(s)
ITB Friction Syndrome

• Lateral pain
• Abnormal contact ITB/LFC
• Bursa develops
➢ Fluid collection/edema
➢ Lateral recess?
Tendons Partial tear of proximal MCL (arrow)

• Medial Figure 4-17-15
➢ Semimembranosus
➢ Sartorius
➢ Gracilis
➢ Semitendinosus
• Lateral
➢ Biceps femoris
➢ Iliotibial tract
• Posterior
➢ Gastrocnemius (med/lat)
• Anterior
➢ Quadriceps tendon
➢ Patellar tendon
➢ Patellar retinacula
Diagram of lateral stabilizers (B = Biceps
Extensor Mechanism [Figure 4-17-16]
femoris tendon; F = Fibular collateral ligament;
• Quadriceps tendon I = Iliotibial tract).
➢ Striated
❖ Vastus lateralis
❖ Vastus medialis Figure 4-17-16
❖ Intermedius
❖ Rectus femoris
• Patellar tendon
➢ Magic angle
Extensor Tendons: Injuries

• Tears
➢ Trauma
➢ Degeneration
❖ Renal disease, steroids
❖ RA, SLE
➢ Partial vs. complete
Extensor mechanism (quadriceps

and patellar tendons)

Patellar Tendinitis [Figure 4-17-17] Figure 4-17-17
• “Jumpers Knee”
• Enlarged (proximal)
• + intrasubstance SI
• Spectrum
➢ partial --> complete tears
Patellofemoral Joint
• Patellar subluxation
➢ Lateral
➢ Hypoplastic intercondylar notch
Patellofemoral Syndrome
• Anterior pain
• Patellar tilt / subluxation
• Impingement of infrapatellar fat
“Cystic” Structures
• Recesses
• Bursae
• Ganglia Patellar tendinits with thickening and
high grade partial tearing in its proximal
Normal Recesses fibers
• Suprapatellar “bursa”
• Infrapatellar cleft Figure 4-17-18
• Popliteus hiatus
• Gastrocnemius/ Semimembranosus
• Posterior recesses
Bursae [Figure 4-17-18]

• Prepatellar
• Infrapatellar
➢ superficial/deep
• Semimembranosus
• Pes anserine
• Tibial collateral
• LCL-Biceps Femoris
Other Cystic Masses [Figure 4-17-19] A. Prepatellar bursitis.

• Meniscal cysts B. Semimembranosus bursitis.
• Ganglia
➢ Intraarticular (cruciates)
➢ Extraarticular (infrapatellar fat)
➢ Intraosseous (cruciate insertions)
• Vascular masses
Figure 4-17-19
Popliteal artery aneurysm (A)

Cystic Adventitial Disease Figure 4-17-20
• Cystic degeneration vessel wall
• Popliteal artery common
• Sudden onset claudication
• MRI findings
➢ intramural cysts
➢ along long axis of vessel
➢ extrinsic compression
➢ MR angiography
Synovial Plica
• Embryologic remnants
• Infrapatellar
• Suprapatellar
• Medial
Loose Bodies
[Figure 4-17-20]
• Intercondylar notch Loose body in posterior joint recess (arrow)
• Baker’s cyst
• Popliteus sheath
• GRE (T2*)
Take Home Points

• Bone contusions? Look for pattern
• ACL torn? Taut...primary/secondary signs
• ACL graft? Taut...roof impingement...cyclops
• MCL? Deep and superficial fibers
• Lateral ligaments? Biceps, LCL, Iliotibial Band
• “Cyst”? Recess, bursa, ganglion, meniscal cyst
References
1. Johnson DL, Urban WP, Jr., Caborn DN, Vanarthos WJ, Carlson CS. Articular cartilage changes seen with
magnetic resonance imaging-detected bone bruises associated with acute anterior cruciate ligament rupture. Am J
Sports Med 1998; 26:409-414.

MRI of the Wrist
Mark Anderson, MD
Figure 4-18-1
Technique: Positioning
• Supine (arm at side)
• Prone (arm extended overhead)
• Surface Coil
➢ Thickness 1–3 mm
➢ Matrix 256–512
➢ FOV 10 cm
Technique: Pulse Sequences

• T1 Anatomic overview
• T2* Ligaments, tendons
• STIR Marrow, fluid
• Gd Cyst/solid, infxn Os styloideum
Synovitis “screen”
Anatomy: Coronal
• Bones
• Intrinsic ligaments.
• TFCC
Anatomy: Axial
• Tendons
• Three levels
➢ Distal radioulnar joint
➢ Pisotriquetral joint
➢ Hamate
• Median nerve (Carpal tunnel)
• Ulnar nerve (Guyon’s canal)
Anatomy: Sagittal
• Carpal alignment
• Pisotriquetral joint
• Triangular fibrocartilage
Anatomy / Pathology
• Bones
• Intrinsic ligaments (SL, LTL)
• TFCC
• Tendons
• Nerves
• Masses
Bones: Normal
• Signal intensity
Alignment
➢ sagittal alignment
➢ ulnar variance
Os Styloideum [Figure 4-18-1]

• Normal variant
• Base of 2nd/3rd metacarpals
• +/- Pain
➢ Bursitis
➢ Ganglion
➢ Trauma
MRI of the Wrist 872 Musculoskeletal Radiology

Occult Osseous Injuries [Figure 4-18-2] Figure 4-18-2
• Contusion
➢ Bone marrow edema
• Fracture
➢ Edema + fx line
Occult Fractures [Figure 4-18-3]

• Trauma Screening Protocol
Scaphoid Fracture [Figure 4-18-4]

• 16% not detected initially
• Complications
➢ AVN Occult fracture of triquetrum
➢ Nonunion
Figure 4-18-3
Scaphoid AVN [Figure 4-18-5]
• Normal T1 = Normal
• − T1 − T2 = Necrotic
• − T1 + T2 = Ischemia vs. traumatic edema
• Contrast enhancement?
AVN Lunate
• Kienbock’s Disease Mid scaphoid fracture
Left: Coronal T1 - Right: Coronal STIR
• Repetitive trauma, fracture, ulna (-) variance
• End arteries Figure 4-18-4
• Central position
AVN Lunate: MRI [Figure 4-18-6]

• More than 50% of lunate
• Abnormal marrow signal
➢ − T1 − T2 = Diagnostic
➢ − T1 + T2 = Earlier stage
Ulnolunate Impaction [Figure 4-18-7

• Ulna plus variance
• Degenerative changes
➢ especially lunate Scaphoid fracture
• TFC tears Figure 4-18-5
Figure 4-18-7 Scaphoid fracture with ischemic changes in proximal pole
Figure 4-18-6
Avascular necrosis of the lunate

Ulnolunate impaction syndrome (Kienbock’s disease).
Musculoskeletal Radiology 873 MRI of the Wrist

Intrinsic Ligaments [Figure 4-18-8] Figure 4-18-8
• Scapholunate
➢ Volar Trapezoidal
➢ Middle Triangular
➢ Dorsal Band-like
• Lunotriquetral
➢ Smaller (2mm)
• Other
➢ Distal carpal row
➢ Incomplete
Intrinsic Ligaments
• Pitfalls
➢ Intermediate signal
➢ Attach to bone or articular cartilage
Ligament Pathology [Figure 4-18-9]

• Absent Normal scapholunate ligament
• Distorted / Elongated
• Widened joint Figure 4-18-9
• Discontinuous
• Fluid signal on T2
Carpal Stability
• Scaphoid <-> Lunate <-> Triquetrum
Scapholunate Dissociation: DISI [Figure 4-18-10]

• Tear or stretching of SLL
➢ dorsal fibers
➢ scaphoid palmar flexes
❖ “signet ring” deformity
➢ lunate dorsiflexes
❖ S-L angle > 600• Scaphoid fracture
Small scapholunate ligament perforation
• DISI deformity
➢ can result from scaphoid fx Figure 4-18-10
Scapholunate Instability: SLAC Wrist [Figure 4-18-11]
• Scapho
• Lunate
• Advanced
• Collapse
• Trauma, RA, CPPD
Figure 4-18-11
DISI deformity
SLAC wrist

Lunotriquetral Instability [Figure 4-18-12] Figure 4-18-12
• LTL tear
• Associated with TFC tears
• VISI deformity
• Difficult diagnosis
Triangular Fibrocartilage Complex

• Radioulnar ligaments
• Meniscus homologue
• UCL and ulnocarpal ligaments
• ECU tendon sheath
TFC: Normal Anatomy [Figure 4-18-13]

• Fibrocartilage
• “Bow tie”
• Ulnar styloid → dist radius
• Attaches to radial cartilage
• Central portion / periphery
VISI deformity
➢ Peripheral 20% vascularized
TFC: Pathology [Figure 4-18-14] Figure 4-18-13

• Tear / Perforation
➢ 95% accuracy
➢ Partial vs. full thickness
➢ Radial / ulnar
➢ Central / peripheral
• Associated injuries
➢ LTL, ECU sheath
Radioulnar Ligaments [Figure 4-18-15]

• Volar / Dorsal margins of TFC
• Flat margins - Attach directly to bone
• Injury → DRUJ instability
Figure 4-18-15 Normal triangular fibrocartilage (TFC)
Figure 4-18-14
Normal volar and dorsal radioulnar ligaments (arrows), and normal TFC
(open arrow).
Extensor Carpi Ulnaris Sheath [Figure 4-18-16]

• Ulnar-sided support
• Injury leads to
➢ subluxation, tenosynovitis, tears
Figure 4-18-16
Small perforation of the TFC
Subluxed extensor carpi ulnaris tendon (arrow)

Tendons Figure 4-18-17
• Axial plane
• Flexors
➢ Carpal tunnel
• Extensors
➢ dorsal compartments
➢ Extensor retinaculum
Extensor Compartments
• 1st Abd. pollicis longus
Ext. pollicis brevis
• 2nd Ext. carpi radialis longus/brevis
• Lister’s Tubercle A. Artifactual intermediate signal in the flexor pollicis longus
tendon on gradient echo image.
• 3rd Ext. pollicis longus
B. Tendon appears normal on fat-saturated T2-weighted image
• 4th Ext. digitorum
Ext. indicis
• 5th Ext. digiti minimi Figure 4-18-18
• 6th Ext. carpi ulnaris
Tendon Pathology
• Tenosynovitis
➢ Surrounding fluid, +/- enlargement
➢ Stenosing (loculated, septations)
• Partial tear
➢ Enlarged / thinned / focal signal
• Complete tear
Magic Angle Phenomenon [Figure 4-18-17]

• Artifactual signal within tendon
• ~ 55º to main magnetic field
• Short TE images
• Disappears on long TE images DeQuervain’s tenosynovitis
Tendon Pathology [Figures 4-18-18 and 4-18-19] Figure 4-18-19

• Extensor Carpi Ulnaris
➢ 6th dorsal compartment
• DeQuervain’s Syndrome
➢ 1st extensor compartment
➢ Tenosynovitis
➢ DDX:
❖ Scaphoid fracture
❖ 1st CMC arthritis
❖ Flexor carpi radialis tenosynovitis
• Flexor tendons
➢ Tenosynovitis
➢ Carpal tunnel syndrome Severe tenosynovitis of the flexor tendons
Carpal Tunnel [Figure 4-18-20]

• Floor – carpal bones Figure 4-18-20
• Roof – flexor retinaculum
• Contents
➢ Flexor tendons
➢ Median nerve
Normal carpal tunnel (long arrow = flexor

retinaculum; short arrow = median nerve)
Median Nerve [Figure 4-18-21] Figure 4-18-21
• Volar / radial position in carpal tunnel
• Stable to decreasing size
• May appear flattened at hamate
Carpal Tunnel Syndrome

• Compressive neuropathy
• Pain, paresthesias
➢ Thumb, index, long, radial 1/2 ring Normal median nerve within the carpal tunnel
• Worse at night
• DX: clinical exam, nerve conduction
Figure 4-18-22
Carpal Tunnel Syndrome: MRI

• Swelling (pisiform)
• Flattening / angulation (hamate)
• Increased signal intensity – T2
• Bowing of flexor retinaculum
Carpal Tunnel: Post-op

• Volar displacement of tendons/nerve
• Free edges of retinaculum
• Retinaculum not seen
CT: Post-op Complications

• Incomplete retinacular release
• Proximal swelling of median nerve
• Scarring around nerve
• Mass lesion in carpal tunnel
Normal ulnar tunnel (Guyon’s canal)
• Median nerve neuroma
Figure 4-18-23
Guyon’s Canal [Figure 4-18-22]
• “Ulnar Tunnel”
• Ulnar nerve, artery, vein
• Boundaries
➢ Floor – flexor retinaculum
➢ Roof – fascia
➢ Lat. to pisiform and hook of hamate
Ulnar Tunnel Syndrome [Figure 4-18-23]

• Ganglion cyst or other mass
• Fracture (hook of hamate)
• Repetitive trauma
Masses: Anomalous Muscles [Figure 4-18-24]

• Accessory palmaris longus
➢ volar Lipoma compressing the structures within the ulnar tunnel
➢ superficial to flexor tendons
• Ext. digitorum manus brevis
➢ dorsal
➢ near extensor indicis tendon
• Isointense to muscle Figure 4-18-24
➢ on all sequences
Accessory palmaris longus muscle (M)

Take Home Points
• High resolution imaging!
• SLL and LTL?...Coronal thin section images
• TFCC?...TFC, radioulnar ligaments, ECU tendon
• Tendons?...Flexor and extensor – axial images
• Masses?...Ganglia 70% dorsal @ SLL
20 % volar @ distal radius
• Nerves?...Median – carpal tunnel
Ulnar – Guyon’s canal

MRI of the Ankle and Foot
Mark Anderson, MD
Technique Figure 4-19-1

• Surface Coil
• One ankle/foot only
• T1, T2, Fat Sat
• Gd?
➢ Cyst vs. Solid
➢ Infection
➢ Synovitis “screening”
Ankle/Foot: Imaging Planes

• Ankle Foot
• Axial Long Axis
• Coronal Short Axis
• Sagittal Sagittal
Bones
• Marrow Edema Fracture of the anterior process of the
calcaneus
➢ Activity related
➢ Contusion/occult fracture Figure 4-19-2
➢ Osteonecrosis
➢ Osteomyelitis
➢ Tumor
Bones: Acute Trauma [Figure 4-19-1]

• Contusion, bone bruise
➢ Marrow edema
➢ Hemorrhage
➢ Trabecular fx
• Fracture
Osteochondral Lesion [Figure 4-19-2]

• Terminology
➢ Osteochondral fracture Osteochondral lesion, medial talar dome
➢ Transchondral fracture
➢ Osteochondritis dissecans (OCD)
• Ankle
➢ Acute trauma
Figure 4-19-3
➢ Talar dome
❖ Mid 1/3 lateral
(inversion, dorsiflexion, LCL)
❖ Posteromedial
(inversion, plantarflexion)
Osteochondral Lesion [Figure 4-19-3]

• Talar Dome
➢ Mid 1/3 lateral (inversion, dorsiflexion, LCL)
➢ Posteromedial (inversion, plantarflexion)
• Staging
➢ 0 Normal cartilage
➢ 1 Abnl SI but intact
➢ 2 Fissuring not to bone
➢ 3 Flap or exposed bone
➢ 4 Loose fragment
➢ 5 Displaced fragment Osteochondral lesion, medial talar dome with
Mintz DN, et al. Arthroscopy 2003;19:353-9 overlying cartilage loss
Musculoskeletal Radiology 879 MRI of the Ankle and Foot

Bones: Os Trigonum [Figure 4-19-4]
• Ununited tubercle
• Os Trigonum Syndrome
➢ Post. Pain
❖ Plantar flexion (ballet) Figure 4-19-4
➢ MRI
❖ Marrow edema
❖ FHL tenosynovitis (stenosing)
Bones: Access. Navicular [Figure 4-19-5]

• Type I – distal PT tendon
• Type II – close proximity to bone
• Cornuate navicular
• Pain syndrome
➢ Type II and Cornuate
➢ MRI: marrow edema
Tarsal Coalition [Figure 4-19-6] Os trigonum syndrome

• 2nd – 3rd Decade Figure 4-19-5
• Vague hindfoot pain
• Calcaneonavicular
• Talocalcaneal
➢ 2° signs: talar beak, “C” sign, etc.
• Cartilaginous, Fibrous, Osseous
Bones: Hallux Sesamoids

• Flexor hallucis brevis tendons
• Stress reaction/fracture (medial)
• Osteonecrosis (lateral)
• DJD (subchondral changes)
Ligaments Accessory navicular

• Syndesmotic
• Lateral
Figure 4-19-6
• Medial
• Spring
• Lisfranc
• Sinus Tarsi
• Plantar Fascia
Ligaments: Syndesmotic [Figure 4-19-7]
• Interosseous ligament
• Anterior tibiofibular
• Posterior tibiofibular
• Talus = rectangular
Calcaneonavicular coalition
Figure 4-19-7
Anterior and posterior tibiofibular ligaments
MRI of the Ankle and Foot 880 Musculoskeletal Radiology

Ligaments: Lateral [Figure 4-19-8] Figure 4-19-8
• “Fibular collateral lig complex”
• Anterior talofibular
• Calcaneofibular
• Posterior talofibular
• Talus = elongated
Ligaments: Medial [Figure 4-19-9]

• Deltoid
➢ “Tibial collateral lig complex”
• Deep (tibiotalar)
• Superficial
➢ Tibionavicular
➢ Tibiocalcaneal (strongest)
➢ Posterior tibiotalar
Ligaments: Injuries [Figure 4-19-10] Normal anterior talofibular ligament

• Interruption
• Laxity Figure 4-19-9
• Thickening/irregularity
• Edema (acute)
• Non-visualization
Figure 4-19-10
Deep fibers of the deltoid ligament
Torn anterior tibiofibular ligament

(syndesmotic injury)
Ligaments: Chronic Injury [Figure 4-19-11] Figure 4-19-11

• Anterolat Impingement Syndrome
➢ ATAF ligament injury
➢ Persistent pain
➢ Scar tissue in lateral gutter
➢ MRI
❖ Intermediate SI tissue
❖ T1 and T2WI
Ligaments: Spring / Lis Franc

• Spring ligament
➢ plantar calcaneonavicular
➢ medial and plantar bands
• Lisfranc ligament
➢ medial cuneiform
➢ base of 2nd metatarsal
Scar tissue in anterolateral gutter (arrow)
secondary to chronic anterior talofibular
ligament injury
Sinus Tarsi [Figure 4-19-12] Figure 4-19-12
• Cone-shaped space
• Wide lateral – tarsal canal medial
• Fat, nerves, vessels, ligaments
➢ Inferior extensor retinaculum
➢ Cervical ligament
➢ Talocalcaneal interosseous lig
Sinus Tarsi Syndrome

• Lateral pain
• Sense of hindfoot instability
• 70% – Prior trauma
• 30% – Inflammatory arthritis
Normal sinus tarsi
• PTT tear/dysfunction
Figure 4-19-13
Sinus Tarsi Syndrome
• MRI Findings
➢ Replacement of normal fat
➢ - SI T1
➢ + or - SI T2
Plantar Fascia [Figure 4-19-13]

• Calcaneus → toes
• Two bands
➢ Medial
➢ Lateral
Plantar Fasciitis
• Inflammation
➢ Mechanical (pes cavus, etc.)
➢ Degenerative (age related) Normal plantar fascia
➢ Systemic disease (RA, seronegative)
• DDx:
Figure 4-19-14
➢ Calcaneal stress fx
➢ Tendinitis
➢ Heel pad trauma/inflammation
Plantar Fasciitis [Figure 4-19-14]

• MRI
➢ Thickened fascia (> 4 mm)
➢ + SI
❖ Fascia and perifascial tissues
❖ Calcaneus
Plantar Fibromatosis [Figure 4-19-15]

• Fibrous proliferation
➢ Fibroblasts and collagen Severe plantar fasciitis with partial tearing
• Solitary or multiple at its origin (arrow).
• MRI
Figure 4-19-15
➢ T1 / - SI
➢ T2 / low to intermediate SI
➢ Variable enhancement
Tendons
• Change orientation
• Pulleys
➢ Osseous or soft tissue
• Magic angle effect
Small, enhancing plantar fibroma (arrow) on

post-contrast, fat-saturated T1-weighted image

Tendon Pathology [Figure 4-19-16] Figure 4-19-16
Achilles Tendon [Figure 4-19-17]

• Gastrocnemius/Soleus
• No tendon sheath (paratenon)
• Bursae
➢ Retrocalcaneal
➢ Tendo Achilles (acquired)
• Flat/concave ventral margin
Achilles Tendon: Pathology

[Figures 4-19-18 and 4-19-19]
• Insertional Tendinitis
➢ Haglund’s Syndrome
➢ Bursitis
➢ Thickened tendon MR images and schematic diagrams of the spectrum of tendon pathology
➢ “Pump bump”
• Non-Insertional Figure 4-19-17
➢ Overuse
❖ 30-50 y.o.-“weekend warrior”
➢ Systemic disease
❖ RA, SLE
➢ Local/systemic steroids
• Peritenonitis
• Chronic Tendinitis
• Partial / Complete Tear
Figure 4-19-18
Normal Achilles tendon
Figure 4-19-19
Haglund’s syndrome (insertional Achilles

tendinopathy and partial tearing;
retrocalcaneal bursitis)
Chronic, non-insertional Achilles tendinopathy

and partial tearing

Plantaris Tendon Figure 4-19-20
• Origin near lateral Gastrocnemius
• Long tendon
➢ Between med head Gastroc/Soleus
➢ Medial margin of Achilles
• Pitfalls
➢ “Partial tear” of Achilles
➢ “Residual fibers” of Achilles
Medial Tendons [Figure 4-19-20]

• Post Tibial “Tom”
• Flex Digitorum “Dick”
• Artery, vein, nerve “and”
• Flex Hallucis “Harry”
Posterior Tibial Tendon

• Oval – 2X size of FDL
• Insertion sites Normal medial flexor tendons (T = posterior
tibial; D = flexor digitorum; H = flexor hallucis;
➢ Medial navicular A = neurovascular structures in tarsal tunnel).
➢ Cuneiforms
➢ Bases of Metatarsals 1–4 Figure 4-19-21
PTT: Pathology
• Tenosynovitis, Tears
• Factors
➢ Degenerative (middle aged
women)
➢ RA
➢ Abnormal stresses
• Loss of arch
PTT Pathology: MRI A B C

[Figure 4-19-21]
Spectrum of posterior tibial tendon pathology
• Tenosynovitis...Fluid
(A = tenosynovitis; B = partial tear; C = complete tear).
• Partial Tear...Thick, thin, split
• Complete Tear...Disruption
PTT Pathology: MRI

• Secondary Signs
➢ Pes planus
➢ Spur/edema post medial malleolus Figure 4-19-22
• Also look for:
➢ Deltoid ligament
➢ Spring ligament
➢ Sinus Tarsi
Lateral Tendons [Figure 4-19-22]

• Peroneus Longus and Brevis
• Posterior to lateral malleolus
➢ Retrofibular groove
• Peroneus Brevis
➢ Anterior or medial
• Common Sheath
Normal peroneus tendons

(L = peroneus longus; B = peroneus brevis).

Peroneus Tendons [Figure 4-19-23] Figure 4-19-23
• Tenosynovitis
• Subluxation/Dislocation
➢ Lateral margin of fibula
➢ Retinacular injury or small avulsion fx
➢ Acute or chronic
• Entrapment (calcaneal fracture)
• Partial/Complete Tear
Peroneus Brevis Split Syndrome [Figure 4-19-24]

• Longitudinal tear (lateral malleolus)
• MRI
➢ C-shaped
➢ Two tendons
➢ Adjacent fluid/edema
Tarsal Tunnel Syndrome [Figure 4-19-25]

• Fibro-osseous tunnel Lateral dislocation of the peroneus tendons
(arrow)
➢ PT, FDL, FHL tendons
➢ Tibial nerve, artery, vein Figure 4-19-24
• Pain, paresthesias – sole of foot
• Etiologies:
➢ Tumor, ganglion cyst, dilated veins, post-traumatic fibrosis
Figure 4-19-25
Ganglion cyst (G) displacing the neurovascular Split peroneus brevis tendon (arrow = intact
bundle (arrow) within the tarsal tunnel peroneus longus tendon)
Morton’s Neuroma [Figure 4-19-26]

• Plantar digital nerve
• Perineural fibrosis Figure 4-19-26
• 3rd (2nd) web space
• MRI
➢ - SI T1 - SI T2
Enhancing Morton’s neuroma (arrow)

Masses: Accessory Muscles [Figure 4-19-27] Figure 4-19-27
• Isointense to Muscle on MRI
• Accessory Soleus
➢ Ventral to Achilles tendon
• Peroneus Quartus
➢ Adjacent to Peroneus Brevis
Take Home Points

• Bone Marrow Edema?... Differential
• Ligaments?... Syndesmotic – rectangular talus
Lateral / Medial Collateral –
elongated talus
• Tendons?... Magic Angle; PTT – 20 signs; P.
brevis split
• Sinus tarsi?... Normal fat on T1W images
• Tarsal tunnel... Space-occupying mass
• Morton’s neuroma,
plantar fibroma?... Low SI on T2W images – Give
Gadolinium!
Accessory soleus muscle
References
1. Mintz DN, Tashjian GS, Connell DA, Deland JT, O'Malley M, Potter HG. Osteochondral lesions of the talus: a new
magnetic resonance grading system with arthroscopic correlation. Arthroscopy 2003; 19:353-359.

Osseous Lesions
Unknown Histogenesis
Unknown Histogenesis Figure 4-20-1

• Ewing sarcoma
➢ Eosinophicic granuloma
➢ Hand-Schüller-Christian disease
➢ Letterer-Siwe disease
Learning Objectives
• Recognize the spectrum of imaging appearances of
these specific osseous lesions
• Identify differentiating features
Ewing Sarcoma
• Highly malignant primary bone sarcoma
• Ewing provided first comprehensive description in
1921, designating it “diffuse endothelioma” of bone Sheets of monotonous malignant "round cells“ with indistinct
• Later (1924) termed “endothelial myeloma” of bone, cytoplasmic margins. Areas of necrosis and hemorrhage are
frequent
and “Ewing Tumor” by Codman
• Origin controversial but likely derived from primitive
mesenchyme
Ewing Sarcoma: Incidence & Distribution

• About 5% of all biopsied tumors
• Usually major long bones, femur most common (25%), then humerus (8%)
• Long bones involved most commonly
• In flat bones, most common pelvis (20%) followed by ribs (11%)
• Rare in hands, sternum, T-spine
Ewing Sarcoma: Clinical Presentation

• Seventy-five percent 10–25 years
• Peak incidence 10 to 15 years
• Range 5 months to 83 years
• Slight male predominence (1.5:1)
• Pain & swelling most common symptoms
• Constitutional signs to include local heat, fever, anemia, leukocytosis, etc.
• Chromosomal trans in 90%; t(11;22) most common, others t(21;22), t(7;22)
• Predilection for Caucasions (95%)
• Usually solitary and nonfamilial; 10% are reported to be multiple at
presentation
• Exceedingly rare familial cases (siblings), case reports in patients with
retinoblastoma
Ewing Sarcoma: Pathologic Features [Figure 4-20-1]

• Characterized by sheets of monotonous malignant “round cells”
• Indistinct cytoplasmic borders
• Frequent areas of necrosis and hemorrhage
• Virtually all PAS positive (glycogen)
• Ewing family includes Ewing sarcoma and primitive neuroectodemal tumor
[PNET]
Musculoskeletal Radiology 887 Osseous Lesions: Unknown Histogenesis

Radiologic Features: Intergroup Ewing Sarcoma Figure 4-20-2
Study
[Figures 4-20-2 to 4-20-10]
• Distribution: diaphysis 35%, metadiaphysis 59%,
metaphysis 5%, epiphysis <1%
• Lesions medullary, symmetric or eccentric
• Soft tissue mass in about 90%
• Reactive bone 40%, but tumor produces no cartilage or
bone
• Cortical thickening 20%
• Periosteal reaction due to irritation or edema or tumor
permeation 85%
• “Onion skin” appearance due to cyclic pattern of
periosteal irritation 55%
Ewings sarcoma. Radiograph. Note metadiaphyseal
• Perpendicular striations due to rapid continuous lifting of
location and absence of identifiable matrix
periosteum 30%
• Pathologic fracture in 10%–15%; soft tissue calcification 10%
MR imaging. Typical features. MR shows large heterogeneous

Note diaphyseal location, complex periosteal reaction and
circumferential soft tissue mass. Soft tissue changes seen to
cortical thickening
better advantage on MR. Coronal T1 (Left) and T2 (Right).
Hair-on-end” periosteal reaction Ewing sarcoma with pathological fracture. Radiograph (left) and
bone scan (right)
Osseous Lesions: Unknown Histogenesis 888 Musculoskeletal Radiology

Ewing sarcoma. Flat bone. MR

T2 (left) and T1 (right).
Ewing sarcoma. Flat bone. Radiograph
Ewing sarcoma. Flat bone (rib). Large soft tissue Ewing sarcoma. Note permeative osteolysis with
mass obscure osseous origin evidence of associated mass
Treatment & Prognosis: Ewing Sarcoma [Figures 4-20-11 and 4-20-12]

• Ablative surgery, chemotherapy and radiation therapy
• About 30% present with metastases
• Mets typically to lungs (85%), bones (69%), pleura (46%), CNS (12%)
• The 5-year survival rate for patients w/o mets at presentation: 55–70%
Ewing sarcoma. Post treatment change Ewing sarcoma. Local recurrence.

Langerhans Cell Histiocytosis (LCH)
• Eosinophilic granuloma
• Hand-Schuller-Christian disease
• Letterer-Siwe disease
LCH: History
• 1940: Jaffe & Lichtenstein eosinophilic granuloma
• 1941: Farber, Green & Farber EG could be solitary or multiple
• 1953: Lichtenstein proposed the name histiocytosis X for the inflammatory
histiocytoses
LCH: Phases
• Solitary or multiple lesions localized to bone: Eosinophilic granuloma (> 5 y)
• Chronic disseminated histiocytosis: Hand-Schuller-Christian disease (1–5 y)
• Acute or subacute disseminated histiocytosis: Letterer-Siwe disease (<1 y)
• Supports the concept that this is a disorder of immune regulation
LCH: Phases
• LCH, localized to bone: limited to a single or a few bones
• LCH, chronic disseminated: multifocal bone lesions and es involvement of
lymph nodes, skin and abdominal viscera
• LCH, acute or subacute disseminated: disseminated multisystem involvement
LCH: More Recently

• Classification challenged as vague with overlapping clinical syndromes
• Classification includes benign and malignant LCH
• More recently classified as localized or multifocal Figure 4-20-13
➢ Single bone lesion or single organ system
➢ Multifocal
Eosinophilic Granuloma: Incidence &

Distribution
• About 1% of all biopsied tumors
• Solitary EG is about twice as common as
multifocal EG
• About 70% involve flat bones, most commonly
skull (25%), pelvis (20%)
• In long bones, femur then humerus
• Hands and feet rare in solitary disease
Eosinophilic Granuloma: Clinical

Presentation
• About 90% are 5–15 years (average 10–12) Histiocytosis. Note histiocytes with reniform shape and clefts.
Scattered eosinophils are seen
• Male:female about 2:1
• More than 95% of patients are white
• Most patients present with pain/tenderness Figure 4-20-14
• Fever may be present and presentation may
suggest osteomyelitis
Eosinophilic Granuloma:
Pathologic Features [Figures 4-20-13 and 4-20-14]
• Characterized by a collection of histiocytes
• Histiocytes are either oval, lobulated or reniform,
w/ clefts or indentations
• Eosinophils may be seen singly, in sheets,
clusters or not at all
• Birbeck bodies on EM
Histiocytosis. EM. Note Birbeck bodies.

Eosinophilic Granuloma: Radiologic Features Figure 4-20-15
• Usually permeative destruction in early phase with periosteal
reaction
• More sharply defined with time, although lesion may still
enlarge
• May have a rind of sclerosis
• There may be an associated soft tissue mass in 5%–10% of
patients
Eosinophilic Granuloma: Radiologic Features

[Figures 4-20-15 to 4-20-25]
• Skull: beveled edge, button sequestrum
• Flat bone: hole within a hole Skull. Beveled edge
• Long bone distribution: diaphysis (58%), metadiaphysis (18%),
metaphysis (28%), epiphysis (2%) Figure 4-20-16
• Spine: vertebra plana
• Mandible/maxilla: floating teeth
Skull. Button sequestrum.

Figure 4-20-17
Flat bone. “Hole within a hole”

Flat bone (a) and long bone (b). “Hole Flat bone (rib). “Hole within a hole”
within a hole”
Figure 4-20-21
Spine. “Almost” vertebral plana

Indolent radiographic appearance. Note
epiphyseal lesion of proximal femoral lesion Spine. Vertebral plana.
Figure 4-20-25
Clavicular lesion. Radiograph (a) and

MRI. Spine. Single lesion. T1 and T2 Floating teeth macrosection (b)

Eosinophilic Granuloma: Prognosis & Treatment
• Benign course
• Simple curettage or intralesional prednisone
• Large lesions and vertebral lesions may be treated with low dose RTX
(300–1000 rad)
• May regress spontaneously
Hand-Schuller-Christian: LCH Chronic Disseminated

• Initially described by Hand (1893), then by Schuller (1916); Christian (1920)
• Classic triad: destructive skeletal lesions, exophthalmos and diabetes insipidus
• Histologically identical to lesions of EG
• About 10% of patients with unifocal EG will develop multifocal and
extraskeletal disease
Hand-Schuller-Christian Disease:
LCH Chronic Disseminated
• Patients are young, usually less than 5 years
• Classic triad in 10%–15%, <50% have DI, exophthalmos about 25%
• Any bone may be involved, 90% have cranial involvement, 7% hand or foot
lesion(s)
• Hepatosplenomegaly and adenopathy
• Anemia, fever, neurologic complaints
• Fatal in about 15%, morbidity may be high
Letterer-Siwe: LCH Acute Disseminated

• Initial reported by Letterer in 1924 (one case) and Siwe in 1933 (7 cases)
• Usually develops within the first year of life
• Disease disseminated and bone lesions small
• Symptoms may be severe
• Fatal in about 95% of those who develop disease before 1 year of age
Summary
• Review the imaging appearances of Ewing sarcoma and the family of lesions
know as Langerhans cell histiocytosis
• Demonstrate how the radiologic images reflect the underlying pathophyiology
and appropriate differentiating features
References
1. Davis et al. Radiographic features of eosinophilic granuloma of bone. AJR 1989;153:1021

2. Shapeero et al. Ewing sarcoma. Radiology 1994;191:825
3. Stull et al. Langerhans cell histiocytosis of bone. RadioGraphics 1992;12:801
4. Wilkins et al. Ewing's sarcoma of bone. Cancer 1986;58:2551

Soft Tissue Lipomatous Tumors
Learning Objectives
• Recognize the spectrum of common lipomatous soft tissue masses
• Identify the radiologic appearance of the common fatty masses
• Identify imaging limitations and pitfalls
Outline:
• Fundamental definitions
• Incidence of soft tissue tumors
• Overview
• Common lipomatous tumors
• Liposarcoma
• Mimics
• Cases
Definitions
• Soft tissue is the nonepithelial extraskeletal tissue, excluding the RES, glia and
supporting tissue of parenchymal organs
• It is derived primarily from mesenchyme, and by convention is comprised of
skeletal muscle, fat, fibrous tissue and the serving vessels and nerves.
Incidence: New Cancers by site 2000

• Breast 184,200
• Lung 164,000
• Colon/rectum 130,200
• CNS 16,500
• Soft Tissue 8,100
• Bone 2,500
CA Cancer J Clin 2000;50:12
Incidence: Variations
• It is estimated that the relative frequency of benign to malignant tumors is
100:1
• US overall annual incidence: 1.4 per 100K
• Age specific incidence ≥ 80 years: 8.0 per 100k
Classification
• World Health Organization subdivides benign lipomatous tumors into 9 groups
• For imaging purposes, it is more useful to use the classification proposed by
Weiss and Goldblum
Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors, 4th ed. St.
Louis; 2001
Classification: Weiss and Goldblum

• Lipoma
➢ Superficial
➢ Deep
➢ Multiple
• Variants of lipoma
➢ Lipoblastoma
➢ Spindle cell lipoma
➢ Pleomorphic lipoma
➢ Angiolipoma
➢ Chondroid lipoma
Musculoskeletal Radiology 893 Soft Tissue Lipomatous Tumors

• Lipomatous tumors Figure 4-21-1
➢ Intramuscular lipoma
➢ Intermuscular lipoma
➢ Lipomatosis nerve
➢ Lipoma tendon sheath
➢ Lipoma joint
• Infiltrating lipomas
➢ Lipomatosis
➢ Symmetric Lipomatosis
➢ Adiposis Dolorosa
• Hibernoma
Lipoma: Clinical CT. Subcutaneous lipoma

• Tumor of mature fat
• Incidence ? Figure 4-21-2
• Presents 40–60 yrs, uncommon <20
• M ~ F, recent reports male predominence
• Superficial and deep (deep: chest wall, retroperitoneum, deep
tissues hands and feet)
• Deep seated tumors rare, < 1% lipomas
• Typically asymptomatic, local pain or tenderness unusual
• More common in obese, tumor fat not available for metabolism
• Usually small, 80% < 5cm; 1% > 10 cm
• Clinical diagnosis 85% accurate
• Usually solitary, 5%–15% multiple
• 50%-80% transloc chromosome 12 q13-15
MR. Subcutaneous lipoma
Lipoma: Radiology [Figures 4-21-1 to 4-21-8]
• Radiographs may demonstrate a fat density mass Figure 4-21-3
• Fatty nature well demonstrated on CT/MR
• CT tissue attenuation -65 to -120HU
• Visual comparison more reliable than #
• Signal intensity equals SQ fat on MR
• No enhancement with contrast (CT/MR)
• May contain other mesenchymal elements
• The most commom is fibrous tissue
• Termed fibrolipoma when significant fibrous tissue present
• May be associated with cortical thickening
• Occasional chondroid and/or osseous metaplasia ; when long
standing-termed “benign mesenchymoma”
CT. Subcutaneous lipoma right shoulder.
Imaging contralateral side may be useful in
identifying subcutaneous lesions
Figure 4-21-4
T1 T2
MR. Fibrolipoma
Soft Tissue Lipomatous Tumors 894 Musculoskeletal Radiology

Deep lipoma. Retroperitoneum

MR: Unencapsulated lipoma
Figure 4-21-7
Lipoma with metaplastic bone formation (benign

mesenchymoma). Radiograph (left) and macrosection (right)
Figure 4-21-8
Lipoma with metaplastic bone formation

(benign mesenchymoma).
Radiograph (left), CT (middle) and MR (right)
Intramuscular Lipoma
• Lipoma arising in skeletal muscle
• Most common member of subgroup of lipomatous tumors (fatty tumors arising
in intimate association with non-adipose tissue)
• Other entities in this category include: intermuscular lipoma, lipoma of tendon
sheath, and lipomatosis of nerve

Figure 4-21-9
Figure 4-21-10
Intermuscular lipoma. Note infiltrating margin.
Intramuscular Lipoma: Clinical

[Figures 4-21-9 to 4-21-11]
• Most common 4th – 7th decades
• Men more commonly affected
• Most frequent large muscles extremities (thigh,
shoulder, upper arm)
• Typically asymptomatic Intramuscular lipoma
• Often incidental finding Figure 4-21-11
“In general, the concept that lipomas give
rise to liposarcomas is not
accepted...Based on consultation material
reviewed at the AFIP over several decades,
we never encountered a clear-cut example
of malignant transformation of lipoma,
although a few possible cases have been
reported in the literature.”
Weiss and Goldblum
Enzinger and Weiss’s Soft Tissue Tumors, 4th ed. 2001
Lipoblastoma [Figure 4-21-12]

• Relatively rare cellular immature lipoma
• Originally described as embryonic lipoma
• Occurs almost exclusively in infants, usually Intramuscular lipoma
presents by 3 yrs of age, occasionally at birth
• Usually in superficial soft tissues or subcutis of the Figure 4-21-12
extremities
• Males affected 2–3X more commonly
• Two-thirds to three-quarters are discrete
• When diffuse, termed lipoblastomatosis
• Radiologically may be indistinguishable from a
liposarcoma
• Liposarcoma is exceedingly rare in children &
most pediatric fatty masses are lipoblastoma
Lipoblastoma in 18 month old. This appearance in an adult

suggests liposarcoma

Lipomatosis [Figures 4-21-13 to 4-21-15] Figure 4-21-13
• Diffuse overgrowth mature adipose tissue
• Rare, but mild cases may go undiagnosed
• Usually present before age 2
• Considered congenital
• Bone hypertrophy frequent association
• Nerve not affected, not confined to extremity
Liposarcoma
• Malignant mesenchymal tumor
• Second most common soft tissue tumor after MFH
• Approximately 16%–18% all sarcomas
• Presents ages 40–60 years
• Exceedingly rare in children
• Usually extremities or retroperitoneum
• Extremity lesions present 5–10 yrs earlier
Lipomatosis. Clinical photo. MR different patient
Classification: World Health Organisation Figure 4-21-14
(WHO) (From low to high)
• Well differentiated
• Myxoid
• Pleomorphic
• Dedifferentiated
Classification:
World Health Organisation (WHO)
• Well differentiated ---> Dedifferentiated
• Myxoid ---> Round cell *
• Pleomorphic
* Round cell liposarcoma was previously a distinct
subtype, now considered the hypervascular variant
of myxoid liposarcoma
Liposarcoma:
Well-Differentiated [Figure 4-21-16] Lipomatosis trunk
• Predominantly fatty mass, usually more than 75% fat
• Irregularly thickened or nodular septa
Figure 4-21-15
• Presence of nodular/globular areas
• A small number of lipomas will have a similar
imaging appearance
Figure 4-21-16
A B
Mild lipomatosis right lower extremity
C
D
Well-differentiated
liposarcoma. Typical features.
Radiograph (a), CT (b),
MR T1 (c) and T2 (d).

Liposarcoma: Atypical Lipoma Figure 4-21-17
[Figure 4-21-17]
• Lesion histologically indistinguishable from well-
differentiated liposarcoma
• Used for lesions in which wide surgical margin is
possible, such as those in subcutaneous tissue
Liposarcoma: Dedifferentiated
[Figures 4-21-18]
• Bimorphic lesion with:
➢ WD liposarcoma
➢ Juxtaposed high grade sarcoma (MFH)
• Most common dedifferentiated sarcoma
• May be a time-related phenomenon
• Imaging typically shows a well differentiated fatty
mass
Well-differentiated liposarcoma. Typical features
• Fatty mass associated with a focal dominant
nonadipose component
Figure 4-21-18
Liposarcoma: Higher Grade Lesions
[Figure 4-21-19]
• Only 50%-80% of the myxoid or pleomorphic types
show fat on imaging studies
• Fat usually minor component (<25%)
• Hypervascular myxoid (round cell) and pleomorphic
types are typically more heterogeneous
Liposarcoma: Myxoid Lesions [Figure 4-21-20]

• Myxoid and round cell lesions are now accepted as
ends of a common spectrum
• About 20% of myxoid lesions will have a “cyst-like”
appearance
Figure 4-21-19 Dedifferentiated liposarcoma, well-differentiated component
Figure 4-21-20
Myxoid liposarcoma, typical imaging features. T1 (upper left),

T2 (upper right), CT (lower left) and gross (lower right)
Myxoid liposarcoma, cyst-like appearance. Radiograph (upper

left), MR T1 (upper right), T2 (lower left), bone scan (lower right)

Liposarcoma: Distribution
• Type % Retro Extrem
• Well-differentiated 54 54 54
• Myxoid 28 10 34
• Pleomorphic 7 5 8
• Dedifferentiated 10 32 4
Liposarcoma: Mimics
• Hemorrhage
• Malignancies engulfing portion fat
• Muscle atrophy with fat replacement
• Myxoid tumors: intramuscular myxoma, ES myxoid chondrosarcoma, myxoid
MFH
• Neural tumors
Summary
• Fatty tumors are common
• There is a wide spectrum of lipomatous tumors
• Imaging of fatty tumors is frequently characteristic
References
1. Christopher et al. WHO Classification of tumors. Lyon, France: IARC Press; 2002
2. Kransdorf et al. Fat-containing masses of the extremities. RadioGraphics 1991;11:81
3. Peterson et al. Malignant fatty tumors. Skeletal Radiol 2003;32:493
4. Weiss & Goldblum. Enzinger and Weiss's Soft Tissue Tumors, 4th ed. St. Louis: CV Mosby; 2001

Metabolic Bone Disease
Figure 4-22-1
Part I
• Rickets and osteomalacia
• Scurvy
Learning Objectives
• Identify the pathophysiologic alterations that occur
in rickets and osteomalacia and scurvy
• Recognize the spectrum of radiological features of
these diseases
Metabolic Bone Disease

• Systemic diseases which effect the skeleton
diffusely and are the result of a metabolic
disorders
Biochemistry of vitamin D
Rickets Figure 4-22-2
• Abnormal mineralization and development of the
growth plate
Osteomalacia
• Inadequate or delayed mineralization of mature
cortical or spongy bone
Pitt MJ. Rad Clin No Amer:1991;29:97
Osteoporosis
• Insufficient quantity of normal bone
Osteopenia
• Increased radiolucency of bone
OsteoPorosis Normal enchondral bone formation

• Paucity of bone Figure 4-22-3
OsteoMalacia
• Malformed bone
Vitamin D: Prohormone
• D2 – Synthetic
• D3 – Natural
Normal bone formation (left); rachitic bone formation (right)
Vitamin D: Biochemistry
[Figure 4-22-1]
Figure 4-22-4
Vitamin D: 1, 25 Dihydroxyvitamin D
[Figures 4-22-2 to 4-22-4]
• Most active form of vitamin D
• Calcium/phosphorus homeostasis
• Maintenance bone mineralization
Rickets: Radiographic Features

• Nonspecific features
• Growth plate abnormalities
• Skeletal deformities
Osteoid seams
Metabolic Bone Disease 900 Musculoskeletal Radiology

Rickets: Nonspecific Features Figure 4-22-5
• Osteopenia
• Growth retardation
Rickets: Growth Plate Abnormalities

[Figures 4-22-5 to 4-22-9]
• Axial widening
• Metaphyseal lucency
• Metaphyseal cupping
Figure 4-22-6
Radiographic changes of rickets
Figure 4-22-7
Healing rickets with metaphyseal lucent bands (a) and

Dietary rickets with treatment; presentation (a), one macrosection (b)
month (b), two months (c), and four months (d)
Rickets: Skeletal Deformities

[Figures 4-22-8 to 4-22-11]
• Craniotabes
• Rachitic rosary
• Bowing of long bones
• Scoliosis
• Basilar invagination
• Triradiate pelvis
Craniotabes Overgrowth wrist cartilage (a) with corresponding clinical photo (b)
Musculoskeletal Radiology 901 Metabolic Bone Disease

Skeletal deformities Basilar invagination (a); triradiate pelvis (b)
Osteomalacia: Classic Radiographic Features

[Figures 4-22-12 to 4-22-14] Figure 4-22-12
• Osteopenia
• Coarse trabecular pattern with unclear margins
• Looser’s zones
• Features can be seen in rickets
Figure 4-22-13
Coarse trabecular pattern with pseudofracture;

radiograph (a) and macrosection (b)
Figure 4-22-14
Looser zone with (a) and without (b) fracture
Vitamin D [Figures 4-22-15 and 4-23 16]

• Vitamin D-deficient
• GI malabsorption
• Neonatal
25-OH Vitamin D
• Liver disease
• Anticonvulsant therapy
Looser zones

Rachitic disease with fracture distal left femur Rachitic disease from nec
Figure 4-22-17
Renal Related
[Figures 4-22-17 to 4-22-19]
• 1, 25 Dihydroxyvit D
➢ Renal osteodystrophy
➢ Vitamin D dep rickets
➢ Tumor related
• Renal Tubular Disorders
➢ X-linked hypophosphatasia
➢ Familial vitamin D res rickets
➢ Fanconi syndromes
➢ Tumor related
➢ Ifosfamide
Renal osteodystrophy (a) and Fanconi syndrome (b)
Figure 4-22-18
Figure 4-22-19
Rickets due to ifosfamide therapy

Oncogenic osteomalacia

No Abnormality [Figure 4-22-20] Figure 4-22-20
• Axial osteomalacia
• Hypophospatasia
• Metaphyseal chondrodysplasia
Scurvy: The Stinking Disease [Figure 4-22-21]

• “…the whole army was infected by a shocking
disorder…those affected had …sore complaint in
the mouth that rotted the gums and caused a most
stinking breath. Very few escaped death…“…the
surest sign of its being fatal was bleeding at the
nose…barbers were forced to cut away very large
pieces of flesh from the gums, to enable their
patients to eat…it was pitiful to hear the cries and
groans on those on whom this operation was
performed.”
Metaphyseal chondrodysplasia
De Joinville , 7th Crusade (1249–1254)
Scurvy Figure 4-22-21

• Sir James Lind, Ships Surgeon, conducted the
first documented controlled study in 1747 and
proved oranges and lemons were effective
treatment for scurvy
Scurvy: Pathophysiology
• Deficiency of dietary vitamin C
• Decrease cellular activity
• Decreased collagen and osteoid production
Scurvy: Radiographic Features

[Figure 4-22-21 and 4-22-22]
• Dense metaphyseal bands
• Ring epiphysis
• Lucent metaphyseal bands
• Metaphyseal beaks Henry VIII was thought to have scurvy due to his ill-
• Periostitis temperament and horrid breath
• Subpepiphyseal infractions
Figure 4-22-22
Figure 4-22-23
Frankels line and Trummerfeld’s zone
Scurvy with treatment; presentation (left), one month (middle),

six months (right)

Summary
• The radiographic features of metabolic bone disease are frequently
characteristic
• These changes accurately reflect the underlying pathophysiology
References
1. Holick. Vitamin D deficiency: what a pain it is. Mayo Clin Proc 2003;78:1457
2. Leggett et al. Scurvy. NEJM 2001;345:1818
3. Narchi et al. Symptomatic rickets in adolescence. Arch Dis Chil 2001;84:501
4. Pitt. Rickets and osteomalacia. In: Resnick. Diagnosis of bone and joint disorders, 4th ed. Philadelphia, W.B.
Saunders Company, 2002:1901
5. Sundaram et al. Oncogenic osteomalacia. Skeletal Radiol 2000; 29:117

Osteonecrosis and Related Conditions
Learning Objectives
• Identify the spectrum of radiological features of osteonecrosis
• Recognize various associated conditions
• Identify differentiating features
Outline
• Definitions
• Pathophysiology of osteonecrosis
• Infarct geometry
• Radiologic-pathologic correlation
• Associations
• Complications
• Related conditions
Definitions
• Osteonecrosis – ischemic death of cellular components of bone and marrow
• Aseptic necrosis – equivalent to ischemic necrosis and avascular necrosis
• Bone infarct – osteonecrosis involving the metaphysis or diaphysis
• Osteochondrosis – variety of conditions in which there is increased bone
density
Figure 4-23-1
Pathophysiology: Osteonecrosis
• Cellular changes from ischemic injury
➢ Interruption of intracellular enzymes
➢ Cessation intracellular metabolic activity
➢ Cell death
• Cellular sensitivity to anoxia
➢ Hematopoietic elements (6 hrs – 12 hrs)
➢ Bone cells (12 hrs – 48 hrs)
➢ Marrow fat cells (48 hrs – 5 days)
Infarct Geometry: Zones [Figure 4-23-1]

• Central zone of cell death
• Ischemic injury
• Active hyperemia
• Normal tissue
Location Infarct geometry

• Osteonecrosis is most common in the epiphysis
• Ischemic necrosis or bone infarct occur almost exclusively in areas Figure 4-23-2
of predominantly fatty marrow
Osteonecrosis: Radiologic-Pathologic
Correlation
[Figures 4-23-2 and 4-23-3]
• Phase I: Cellular death initial response
• Phase II: Cell modulation
• Phase III: Emergence reactive interface
• Phase IV: Remodeling reactive interface
• Phase V: Crescent sign & collapse
Bilateral femoral head osteonecrosis
Osteonecrosis and Related Conditions 906 Musculoskeletal Radiology

Associations
• Trauma • Dysbaric disorders
• Hemoglobinopathy • Gaucher disease
• Steroids • Pregnancy
• Alcoholism • Irradiation Figure 4-23-3
• Collagen vascular disease • Pancreatitis
Osteonecrosis: Causes
• Thrombophilia (increased tendency to develop
thrombosis)
• Hypofibrinolysis (reduced ability to lyse thrombi)
• Found in 76% of patients with osteonecrosis*
*Glueck et al. Osteonecrosis. AAOS 1997
Osteonecrosis: Causes [Figure 4-23-4] Radiograph and specimen radiograph showing osteonecrosis
• Increased size fat cell → compresses sinusoid with collapse and crescent sign
vascular bed → impedes blood flow
Figure 4-23-4
Corresponding gross and macro section showing osteonecrosis

with collapse and crescent sign
Figure 4-23-5
Radiograph showing typical serpentine margin

of infarct
Radiograph showing flattening with collapse and crescent sign

“Aseptic”
• Alcoholism • Trauma
• Sickle cell anemia • Idiopathic Figure 4-23-6
• Exogenous sterosis • Caisson disease (dysbaric)
• Pancreatitis
Imaging Features: Radiographs

[Figures 4-23-5 and 4-23-6]
• Patchy lucent/sclerotic areas
• Serpentine sclerosis
• Arc-like subchondral lucencies
• Articular collapse
• Preservation of joint space
• Surrounding osteopenia
Osteonecrosis of proximal pole of scaphoid
with surrounding osteopenia
Musculoskeletal Radiology 907 Osteonecrosis and Related Conditions

Imaging Features: Scintigraphy Figure 4-23-7
[Figures 4-23-7]
• Decreased or absent uptake initially
• Increased uptake with repair & revascularisation
Imaging Features: CT [Figures 4-23-8 and 4-23-9]

• Variable findings with age of lesion
• Alterations in osseous architecture
• Useful to evaluate the integrity of the articular
surface
Bilateral osteonecrosis with increased tracer accumulation on
Figure 4-23-8 right, left is normal
Figure 4-23-9
Bilateral osteonecrosis with collapse on left
CT showing reactive interface bilaterally
Imaging Features: MRI Figure 4-23-10

[Figures 4-23-10 and 4-23-11]
• Ring or band pattern
• Homogeneous or heterogeneous
• ”Double line sign”
• Joint effusion
• Marrow edema
• Articular collapse
Osteonecrosis and infarcts with “double line “ sign
Figure 4-23-11
Osteonecrosis with edema pattern

Transient Osteoporosis [Figures 4-23-12 to 4-23-14] Figure 4-23-12
• Described originally in 3rd trimester
• Typically young and middle-aged adults
• Progressive hip pain, symptoms regress in 2-6
months
• Edema pattern on MR, osteoporosis on
radiographs
Figure 4-23-13
Transient osteoporosis with edema pattern and no

osteonecrosis
Figure 4-23-14
Note regional osteoporosis of right hip
Radiographic Staging [Figure 4-23-15]

• Stage Findings
• 0 Clinically suspected, imaging normal
• 1 Clinical findings, abnormal scintigram
• 2 Osteopenia, cysts, bone sclerosis
• 3 Crescent sign without collapse
• 4 Flattening with normal joint space Note regional osteoporosis of left hip
• 5 Joint narrowing with abnormal Figure 4-23-15
acetabulum
Complications: Osteonecrosis
[Figures 4-23-16]
• Cartilaginous abnormalities
• Intra-articular loose bodies
• Cyst formation
Figure 4-23-16
Bilateral osteonecrosis
Infarct with malignant transformation Note “screw treads” extending through

infarct with associated high grade
sarcoma

Spontaneous Osteonecrosis Figure 4-23-17
[Figures 4-23-17 and 4-23-18]
• Middle-aged to elderly
• Abrupt onset – pain, swelling, < rom
• Weight bearing surface
• Medial femoral condyle
• ? traumatic, ? vascular, ? meniscal tear
Osteochondritis Dissecans
[Figures 4-23-19 to 4-23-21]
• Fragmentation and possible separation of articular
surface
• Typically childhood to adolescent
• Variable symptoms
• Non-weight bearing surface
• Classic: lateral medial femoral condyle
• Probably traumatic in origin Spontaneous osteonecrosis medial femoral condyle.
Presentation (a) and at 5 months (b)
Figure 4-23-18
Figure 4-23-19
Spontaneous osteonecrosis medial femoral condyle. T1 (left)

and T2 (right)
Osteochondritis dissecans, classic location
MR-Osteochondritis dissecans, classic location Osteochondritis dissecans, classic location, Radiograph (left)
and MR (right)

Osteochondroses
• Varied disorders characterized by:
➢ Predilection for children
➢ Involvement of epiphysis or apophysis
➢ Radiographs showing fragmentation, collapse, sclerosis and reossification
• Osteonecrosis is not a feature in many, and is secondary (to trauma) in others
• Some are normal variations
Osteochondroses: Characterized by Osteonecrosis

• Lunate: Kienböck
• 2nd metatarsal: Frieburg Figure 4-23-22
• Femoral head: Legg-Calvé-Perthes
• Tarsal navicular: Köehler
• Capitulum: Panner
Kienböck’s Disease [Figures 4-23-22]

• Most common in young adults (20–40 yrs)
• Trauma hx +/-, pain, swelling, <rom, 75% have
ulna minus variance
• Fracture & osteonecrosis histologically
• Radiographs normal initially
• ->increased density, altered shape, collapse and
fragmentation
Osteochondroses: Without Osteonecrosis

• Spine: Scheuermann
Kienbocks disease
• Tibial tubercle: Osgood-Schlatter
• Tibial epiphysis: Blount
• Patella: Sinding-Larsen-Johansson Figure 4-23-23
Scheuermann Disease
[Figures 4-23-23]
• Described in adolescent farm workers
• Common, seen in about 4%–8% population
• Presents second decade, M=F
• Radiographs show end plate irregularity, narrowed
disc spaces, and Schmorl’s nodes involving three
or more vertebrae
Osteochondroses: Variations in Normal

Ossification
• Calcaneous: Sever
• Ischiopubic synchondrosis: Van Neck
Scheurmann disease
Summary
• Morphologic changes in osteonecrosis are relatively characteristic, although
they will vary with location.
• There are a variety of predisposing conditions, as well as those patients in
which no cause is found.
• The term osteochondrosis is used for a variety of conditions, many of which
show no evidence of osteonecrosis
References
1. Sweet et al. Osteonecrosis: pathogenesis. In: Resnick D, ed. Diagnosis of bone and joint disorders, 4th ed.
Philadelphia: WB Saunders, 2002
2. Iida et al. Correlation between bone marrow edema and collapse of the femoral head in steroid-induced
osteonecrosis. AJR 2000;174:735
3. Vande Berg et al. MR imaging of avascular necrosis and transient marrow edema of the femoral head.
RadioGraphics 1993;13:501
4. Glueck et al. Thromophilia, hypofibrinolysis, and osteonecrosis. Clin Orthop 1997;334:43

Approach to the Inflammatory
Arthropathies
Donald J. Flemming, MD
Inflammatory Arthropathies Figure 4-24-1

• Rheumatoid Arthritis
• Spondyloarthropathies
➢ Ankylosing spondylitis
➢ Enteropathic arthritis
❖ Crohn, Ulcerative Colitis, Whipple
➢ Psoriatic arthritis
➢ Reiter Disease
• Juvenile Chronic Arthritis
Radiographic Assessment
• Soft Tissue Swelling
• Soft Tissue Calcification
• Mineralization
• Joint Space Change AP radiograph with non specific fusiform soft tissue swelling
• Erosion surrounding the proximal interphalangeal joint of the ring finger.
• Bone Production Clinical photograph in a different patient with rheumatoid
• Subluxation arthritis and synovitis and fusiform soft tissue swelling involving
the proximal interphalangeal of the index and middle fingers
• Distribution
Soft Tissue Swelling [Figure 4-24-1] Figure 4-24-2

• Symmetrical around joint (fusiform)
• Diffuse (“Sausage digit”)
• Lumpy, bumpy
Sausage Digit [Figures 4-24-2 and 4-24-3]
Mineralization
• Normal
• Juxta-articular AP radiograph of patient with psoriatic arthropathy and diffuse
• Diffuse swelling of the second toe producing a sausage appearance of
the digit. Clinical photograph in a different patient with psoriatic
arthropathy and sausage enlargement of the third and fourth
Juxtaarticular Osteoporosis [Figure 4-24-4] toes
Figure 4-24-3
Figure 4-24-4
MR of sausage digit with intermediate signal of tenosynovitis

27 year old man with reactive arthritis and erosive disease in
surrounding the flexor tendon sheath of second digit. Tendon
the right first metatarsal phalangeal joint. Loss of subchondral
sheath of first digit is normal. MIP projections following
bone is seen in the second, third, fourth and fifth metatarsal
intravenous contrast administration show diffuse enhancement
phalangeal joints indicating hyperemia associated with
of second, third and fourth toes in patient with psoriatic arthritis
inflammation
and sausage digits
Approach to the Inflammatory Arthropathies 912 Musculoskeletal Radiology

Joint Space Change [Figures 4-24-5 and 4-24-6] Figure 4-24-5
• Widening
• Normal
• Uniform narrowing
• Asymmetrical narrowing
• Ankylosis
Figure 4-24-6
The interphalangeal joints should all be

similar in dimension as should the
metacarpal phalangeal joints when
compared to the neighboring
Joint space narrowing is important for differential diagnosis. articulations. A “horizontal” scan pattern
Both patients have erosions involving the wrist presenting as is useful to detect subtle joint space
lucencies in the carpal bones. narrowing. Note the loss of joint space
Patient A has rheumatoid arthritis with diffuse narrowing of all of in the distal interphalangeal joint of the
the carpal articulations. ring finger in this patient with post-
Patient B has gout with maintenance of joint space despite traumatic osteoarthritis
extensive erosive disease
Erosions [Figures 4-24-7 and 4-24-8] Figure 4-24-7

• Aggressive
➢ Marginal
➢ Central
• Nonaggressive
• Early erosions
➢ Thin cartilage
➢ Absent cartilage
Figure 4-24-8
Photomicrograph of axially sectioned fifth

metatarsal head showing destruction of
subchondral bone that would present as an
erosion on radiography
Typical diarthrodial joint anatomy. In early disease, the articular

cartilage (light blue) prevents synovial inflammation (red) from
damaging subchondral bone (white). Erosions are seen
earliest where cartilage is thinnest or where cartilage is absent.
Minimal or no cartilage is present at the margins of a typical
synovial joint adjacent to the attachment of fibrous capsule
Musculoskeletal Radiology 913 Approach to the Inflammatory Arthropathies

Bone Production [Figure 4-24-9] Figure 4-24-9
• Reparative Response
➢ “Whiskering”/ “brush” stroke erosions
➢ Overhanging edge of cortex
➢ Subchondral bone
➢ Osteophytes
• Enthesopathy
• Periostitis
• Ankylosis
Rheumatoid Arthritis
• Most common in females – 2–4:1
• Most common in the fourth and fifth decades
• Incidence – 0.2-0.4/1,000 in females
• Prevalence – 0.5% – 1.0%
• Probably heterogeneous disorder
Patient A has psoriatic arthritis with erosions and bone
production in the metatarsal phalangeal and interphalangeal
Rheumatoid Arthritis joints. Bone formation is present amongst the erosive changes
• Genetic influence – HLA-DR4 (DRB1 allele) producing a “whiskering” type appearance and the digits are
• Pregnancy – increased risk postpartum dense secondary to osteitis. Patient B has rheumatoid arthritis
with erosive disease in the metatarsal phalangeal joints but no
• Infectious agents ?
bone production
➢ Chlamydia
RA – Presentations
• Gradual onset, polyarthritis – typical
• Mono or pauciarticular – unusual
Figure 4-24-10
• Abrupt, acute polyarthritis – unusual
• Systemic disease
• Felty Syndrome
➢ RA
➢ Splenomegaly
➢ Leukopenia
RA – Diagnostic Criteria
• Morning stiffness -Three or more joints involved
• Arthritis of hand joints
• Symmetric arthritis
• Rheumatoid factor – 90% patients
➢ Positive CCP increases specificity
• Radiographic changes Classic manifestations of rheumatoid arthritis. Erosions and
• Four criteria to have diagnosis joint space narrowing are present in the proximal
interphalangeal, metacarpal phalangeal and wrist joints in a
bilateral and symmetric distribution
Rheumatoid Arthritis: Radiographic
Manifestations Figure 4-24-11
• Fusiform soft tissue swelling
• Diffuse or juxta-articular osteoporosis
• Uniform joint space narrowing
• Aggressive marginal erosions
• No bone production
• Synovial/subchondral cysts
• Bilateral symmetrical – distribution
PA view (A) of the wrist is shows subtle joint space narrowing in
RA – Hand and Wrist [Figures 4-24-10 and 4-24-11] the wrist in this patient with rheumatoid arthritis. The Norgaard
• “100%” of patients view (B) reveals erosive disease in the pisotriquetral joint the is
• MCP, PIP joint space loss/ erosions impossible to appreciate in the PA projection
• Pancarpal joint space loss/ erosions
➢ ulnar styloid/ pisotriquetral early
• Ulnar drift – carpus and digits
• Swan-Neck, Boutonniere deformities
• Ankylosis rare – limited to carpus

Ball-catcher’s View (Norgaard View) Figure 4-24-12
[Figure 4-24-11]
Rheumatoid Arthritis – MR Findings

[Figure 4-24-12]
• Synovial hypertrophy
➢ fat saturated fast spin echo T2 weighted images
➢ gadolinium
• “Erosions”
➢ low signal T1W
➢ low to high signal on T2W
➢ surrounding edema
• Hyaline cartilage loss
RA- Feet [Figure 4-24-13 ] Coronal T1 weighted MR of the wrist shows numerous erosions
• 80%–90% of patients depicted by intermediate signal replacing fat in subchondral
• May precede hand dz – 10%–20% bone that are difficult to appreciate on the PA radiograph (B)
• Forefoot – MTP disease predominates
• Midfoot – talocalcaneonavicular joint Figure 4-24-13
➢ May see osseous ankylosis
• Hindfoot – retrocalcaneal bursa
RA – Knee and Hip [Figure 4-24-14]

• Knee – 80%
➢ Pancompartmental joint space loss
➢ Minimal erosions
• Hips – 50%
• Axial migration
• Acetabular protrusio
➢ Medial deviation beyond ilioischical line
➢ 3mm in males; 6mm in females
RA – Shoulder and Elbow

• Shoulder – 60% of patients Classic manifestations of rheumatoid arthritis of the feet.
➢ Erosion in humeral head tend to be lateral Erosions and joint space narrowing are seen in the metatarsal
➢ Rotator cuff tear common phalangeal joints. The erosions are more readily seen on the
➢ Bilateral AC joint erosive disease medial aspect of the first through fifth metatarsal heads and the
lateral aspect of the fifth metatarsal head
• Elbow – ~ 34% of patients
RA- Cervical Spine [Figures 4-24-15] Figure 4-24-14

• 60% – 80 % of patients
• Atlantoaxial subluxation
• Odontoid process erosion
➢ MRI best defines extent of pannus
• Apophyseal joint dz
• Erosion of joints of Luschka
• Spinous process erosions
Figure 4-24-15
Rheumatoid arthritis involving the knee. Note tricompartmental

loss of joint space without erosions or osteophyte formation
Rheumatoid arthritis of the cervical spine with instability at C1-

C2. Widening of the atlantoaxial joint is seen only in flexion in
this patient

Spondyloarthropathies
• Family of inflammatory arthritides of synovium and entheses
• Axial and asymmetric peripheral arthritis
• Genetic predisposition – HLA B27
• Infectious etiology
Spondyloarthropathy – Criteria
• Inflammatory spine pain or synovitis
and one or more of following
• Positive family hx
• Psoriasis/ IBD
• Urethritis/cervicitis/diarrhea – within 1 month
• Buttock pain
• Enthesopathy
• Sacroiliitis
HLA B27
• Normal population -USA ~0–8%
• Ankylosing spondylitis – >90%
• Reiter Disease – 63%–75%
• Psoriasis – not increased without arthritis
➢ with peripheral arthritis – 20%
➢ with axial arthritis – 50% Figure 4-24-16
• IBD with axial arthritis – 50%
Psoriatic Arthritis
• Peak ages – 20–40 years
• M:F – 1:1
➢ Spine and DIP – M>F
➢ Symmetric polyarthritis – F>M
• Arthritis in 5%–8% of patients with psoriasis
• Skin dz before arthritis in 75%
• Arthritis before skin dz in 10%
Psoriatic Arthritis: Radiographic

Manifestations
• Maintenance of mineralization
• Dramatic joint space loss
Psoriatic arthropathy of the hands involving the interphalangeal
• Bone proliferation joints of both hands in a bilateral but asymmetric pattern
• Marginal erosions predominate
➢ “Pencil-in-cup” erosions
• Bilateral asymmetric dz
Figure 4-24-17
Psoriatic Arthritis:
Radiographic Manifestations
[Figures 4-24-16 and 4-24-17]
• Hand/Feet
➢ Distribution
❖ IP joints – asymmetric
❖ Ray distribution
❖ RA distribution
➢ Acroosteolysis
➢ Ankylosis – ~ 15%
➢ Calcaneal erosion – plantar bone proliferation
• Wrist – pancarpal
Typical central erosion in patient with erosive osteoarthritis

compared to marginal erosions seen in patient with psoriatic
arthritis

Erosive Osteoarthritis Figure 4-24-18
• Asymmetrical soft tissues around joint
• Normal mineralization
• Nonuniform loss of joint space
• Central “sea gull” erosions
• Osteophytes
• Subchondral sclerosis
• Distribution – symmetrical
Psoriatic Arthritis:
• SI Joints – 30%–50%
➢ Bilateral asymmetrical (symmetrical)
➢ Erosion (iliac > sacral) and repair
• Spine – 17%
➢ Large, bulky, lateral bone outgrowths
➢ Unilateral or bilateral, asymmetrical Typical fluffy inflammatory plantar calcaneal enthesophyte that
parallels the undersurface of the calcaneus in patient with
➢ Infrequent apophyseal involvement in lumbar reactive arthritis. The bone is dense and an erosion is present
spine in the posterior superior aspect of the calcaneus
Reactive Arthritis: (Reiter Disease) Figure 4-24-19

• Young adults
• M:F – 50–1:1
• Annual incidence – 30–40/100,000
• Frequently associated with infection
➢ Urethritis/cervicitis
➢ Diarrhea – Shigella, Salmonella, Campylobacter
Reiter Disease/Reactive Arthritis:

• Diffuse soft tissue swelling
• Early – juxta-articular osteoporosis
• Late – normal mineralization
• Uniform joint space loss
• Aggressive marginal erosions
• Bone production
• Bilateral asymmetrical distribution 25 year old man with reactive arthritis. Erosions and subluxation
• Feet, ankles, knees and SI joints are seen in the metatarsal phalangeal joints in a bilateral but
asymmetric pattern. Subtle bone formation is present along the
medial aspect of the navicular and the medial cuneiform of the
Reiter Disease/Reactive Arthritis: left foot as a manifestation of the asymmetric nature of this
Radiographic Manifestations disease
[Figures 4-24-18 to 4-24-20]
• Feet – 40%–55% Figure 4-24-20
➢ IP’s and MTP’s
➢ Erosions with repair
➢ Periostitis along diaphyses
• Calcaneus – 25%–50%
➢ May be “sole” sight of disease
➢ Plantar and posterior erosions
➢ Enthesophytes
• Ankle – 30%–50%
➢ Joint space loss and periostitis
• Knee
➢ Effusion
➢ Joint space loss and periostitis
• SI joints
➢ Bilateral asymmetric
➢ Erosions and repair
Bone formation at the posteromedial aspect of

the distal tibia in a patient with psoriatic arthritis

Reiter vs. Psoriatic Figure 4-24-21
• Juxtaarticular osteoporosis
• Periostitis without joint findings
• Less ankylosis of IP joints
• Tendency to involve MTP vs. IP joints
• Lower extremity involvement predominates
Ankylosing Spondylitis
• Peak age of onset – 15–35 years
• M:F – 3–5:1
• Incidence ~ 6.6/100,000
• Strong association with HLA B27
Rare in blacks
• Predilection for axial involvement
AS-Radiographic Manifestations Typical presentation of ankylosing spondylitis with erosions and

[Figures 4-24-21 and 4-24-22] sclerosis involving the inferior aspect of the SI joints in a
• Sacroiliac disease bilateral and symmetric pattern
➢ Bilateral symmetric – same as enteropathic
➢ Erosions predominate iliac vs. sacrum Figure 4-24-22
➢ Sclerosis
➢ Ankylosis
• Other pelvic dz
➢ Pubic symphysis – 16%–23 % erosion and
ankylosis
➢ Enthesitis – ilium and ischium
Sacroiliitis: Differential Diagnosis

• Ankylosing Spondylitis
• Enteropathic Arthropathy
• Psoriasis
• Reactive Arthritis
• Osteiitis Condensans
• Infection
Ferguson view of the pelvis with bilateral symmetric sacroillitis
in patient with ankylosing spondylitis
AS-Radiographic Manifestations
[Figures 4-24-23 to 4-24-25]
• Spine Disease – ascends from lumbar to cervical
➢ Discovertebral destruction
❖ Romanus and Andersson lesion
➢ Shiny corner sign
➢ Squaring of vertebral body Figure 4-24-23
➢ Syndesmophyte
➢ Bamboo spine
➢ Trolley track and Dagger signs
➢ Atlantoaxial disease
Density confined to the anterior superior and inferior end plates

of the lumbar spine resulting in the classic “shiny corner”
presentation of ankylosing spondylitis. Note the lack of anterior
concavity of the vertebral bodies that contributes to its
“squared” appearance

Lateral radiograph of the cervical spine in patient with

ankylosing spondylitis shows thin posterior and anterior
The normal lumbar vertebral body is concave anteriorally. The syndesmophytes and fusion of the facet joints. Lateral
41 year old patient with ankylosing spondylitis shows bone radiograph of the thoracic spine also shows thin posterior and
formation at the anterior aspect of the vertebral bodies resulting anterior syndesmophytes
is a squared appearance
DISH: Diffuse Idiopathic Skeletal Figure 4-24-26

Hyperostosis [Figure 4-24-26]
• Common disease – 12% of elderly population
• Flowing bulky paravertebral ossification
➢ Four contiguous vertebral bodies
• Thoracic>lumbar>cervical
• Enthesophytes – particularly pelvis
• Absence of erosions/ joint abnormality
AS-Extraskeletal Manifestations
• Uveitis
• Ascending aortitis/ aortic valve disease
• Cardiac conduction abnormalities
• Interstitial lung dz - upper lobes
Juvenile Chronic Arthritis Bulky paravertebral ossification in patients with DISH is usually
• JRA (seronegative) – 70% easily distinguished from the thin anterior syndesmophytes of
➢ Still Disease, pauci/monarticular, polyarticular ankylosing spondylitis that typically have no horizontal
component
• Juvenile-onset adult type RA – 10%
• Juvenile-onset ankylosing spondylitis
• Psoriatic arthritis
• Enteropathic arthritis
• Reactive/ Reiter arthritis
Still Disease: Systemic Disease (Classic)

• M:F = 1:1
• Age usually less than 5 years
• Acute febrile illness
• Rash
• Generalized adenopathy/hepatosplenomegaly
• Pericarditis
• Mild joint findings – arthralgias/mild arthritis

JRA-Still Disease: Pauci or Monoarticular Figure 4-24-27
• Females more commonly than males
• Large joint disease
➢ Knees, ankles, elbows, and wrists
JRA: Polyarticular
• M:F = 1:1
• Symmetric arthritis
• Hands – MCP, PIP
• Wrists
• Knees/Ankles
• Feet – intertarsal, MTT, MTP, IP’s
• Cervical spine
Juvenile-onset adult type RA

(Seropositive JRA) Classic manifestation of mono or pauciarticular JRA. The left
• F>M knee is enlarged and shows advanced bone age in comparison
to asymptomatic right knee. Note the lack of erosions and joint
• >10 years of age at onset
space narrowing
• Polyarticular
• Subcutaneous nodules
• Vasculitis Figure 4-24-28
JRA: Radiographic Manifestations

[Figures 4-24-27 to 4-24-31]
• Osteoporosis
• Joint space loss
➢ Not prominent in monoarticular
➢ May be rapid in sero(+) JRA
➢ Ankylosis – hands, wrists, cervical spine
• Bone erosion – not prominent finding
• Periostitis
➢ Phalanges, metacarpals, metatarsals
• Ballooned epiphyses
• Accelerated skeletal growth 17 year old man with polyarticular JRA and left hip pain. The
• Premature fusion of physes femoral heads are enlarged resulting in “ballooned”
appearance of the epiphyses
Osteoarthritis
• Most common arthropathy
• ~ 80% of patients over 75 years
• Second only to CHD as cause of work disability for Figure 4-24-29
men > 50 years of age
• Primary – no underlying abnormality
• Secondary -preexisting metabolic, anatomic,
traumatic, or inflammatory condition
Osteoarthritis: Definition
• American College of Rheumatology
“ a heterogeneous group of conditions that lead to
joint symptoms and signs which are associated with
defective integrity of articular cartilage, in addition to
related changes in the underlying bone at the joint
margins.”
Coronal SPGR image of the left hip in patient with JRA showing
marked irregularity in the articular cartilage

Polyarticular JRA of the hands. Generalized osteoporosis and Adult patient with JRA as a child. Note ballooned appearance of
joint space narrowing is present with striking lack of erosions the metacarpal heads right greater than left. Wrist involvement
was also aymmetric in this patient. Minimal erosions are seen
Osteoarthritis: Clinical
• Increasing prevalence with age over 40
• Pain, stiffness, and loss of range of motion
• Symptoms may regress or be cyclic
• Risk Factors
➢ Heredity – AD trait with Heberden nodes
➢ Obesity – risk factor for knee and possibly hand
❖ Not risk factor for hip
➢ Hypermobility – increases risk
➢ Occupation – increased risk in heavy manual labor
❖ No increased risk from recreational sports
➢ Diabetes – increases risk
➢ OA in one joint increases risk for other joints
➢ Osteoporosis – protective effect in hip OA
➢ Cigarette smoking – protective effect
Osteoarthritis and Pain

• Most common and important complaint
• Source
➢ raised intraosseous pressure
➢ synovitis/bursitis/tenosynovitis
➢ periosteal elevation
➢ muscular imbalance
• Less common in very old or young
• Psychosocial factors
• Radiographic predictors
➢ Osteophytes in knee good predictor
➢ Joint space narrowing in hip predictor
➢ Good in first CMC joint
➢ Poor in hand IP joints
Osteoarthritis: Radiographic Manifestations

• Normal mineralization
• Nonuniform joint space loss
• Absence of erosions
• Subchondral new bone formation
• Osteophyte formation
• Subchondral cysts
• Subluxations

Osteoarthritis: Subchondral “Cysts” [Figure 4-24-32] Figure 4-24-32
• Not true cysts
• Intrusion
➢ Defect in cartilage leads herniation of joint fluid into bone
➢ Cyst size based on joint pressure
• Contusion
➢ Repeated insult to subchondral bone leads to resorption
Osteoarthritis: Osteophytes [Figures 4-24-33 to 4-24-35]

• Tend to occur at the margins of joints
• Produce “enlargement” of joint
➢ attempt to stabilize joint
• Can be central – “button osteophyte”
• May not be dramatic in osteoporotic women
Figure 4-24-33
Typical subchondral lucencies in specimen

radiograph of osteoarthritic femoral head
Osteophytes of the interphalangeal joints of the hands are

usually best appreciated on lateral radiographs
Figure 4-24-34
Figure 4-24-35
Osteoarthritis of the hip with superior lateral joint space

narrowing accompanied by subchondral sclerosis, subchondral
cyst formation and osteophyte production
Large osteophytes projecting from the articular

surfaces of the
medial and lateral femoral condyles

Osteoarthritis: Subchondral Sclerosis Figure 4-24-36
• Also known as eburnation
• Stimulated by loss of hyaline cartilage
• Combination of new bone on existing trabeculae
from microfracture and repair
Osteoarthritis: Radiographic Manifestations

[Figures 4-24-36 and 4-24-37]
• Hands
➢ DIP joints – Heberden nodes
➢ PIP joints – Bouchard nodes
• Wrist
➢ First metacarpal-carpal joint
Figure 4-24-37
Patient A shows Heberdon nodes from osteophytes and soft

tissue swelling at the distal interphalangeal joints.
Bouchard nodes are seen at the proximal interphalangeal joints
of patient B
Figure 4-24-38
Hooked osteophytes are seen involving the second and third

metacarpal heads. These may be seen in hemochromatosis,
CPPD arthropathy or osteoarthritis
Osteoarthritis - Knee:
[Figures 4-24-38 and 4-24-39]
• May require weight-bearing views
• Medial compartment – 75%
Non weight bearing AP of the knee (A) shows osteoarthritis in
• Patellofemoral joint – 48% the medial compartment with subchondral sclerosis and
• Lateral compartment – 26% osteophyte formation but the joint space appears maintained.
• Pancompartmental AP weight bearing view of the knee (B) shows the expected
➢ Think deposition dz or prior inflammatory loss of joint space in the medial aspect of the knee
arthropathy
Figure 4-24-39
Osteoarthritis - Hip: Radiographic
Manifestations
• Superolateral migration
➢ 60%
➢ M>F
• Medial migration
➢ 25%
➢ F>M
• Axial migration
➢ Think deposition dz or prior inflammatory dz
56 year old woman with acromegaly. Manifestations of

osteoarthritis are seen but the joint spaces are widened rather
than narrowed

Hip Joint Space Narrowing
Osteoarthritis - Foot: Radiographic Manifestations

• Occurs along lines of weight bearing
• First MTP joint
➢ Hallux rigidus
➢ Hallux valgus
• First MTT joint
• Talonavicular joint
➢ Dorsal talar beak (coalition vs. DJD)
References
1. Brower A: Arthritis in Black and White, 2nd ed. Philadelphia, Pa: WB Saunders; 1997: 252.
2. Resnick D ed. Diagnosis of bone and joint disorders, 4th Ed. Philadelphia: W.B.Saunders, 2002:

MRI of the Rotator Cuff
Donald J. Flemming, MD
MRI of Rotator Cuff Tears

Sensitivity Specificity
Utopia 89%–100% 88%–97%
CSS FT 56% 73%
ESS FT 78% 83%
CCS PT 0% 68%
ESS PT 71% 71%
CCS-Clinical Community; ESS-Experienced Specialist Arthroscopy 1997;
13:710–719
MRI of the Rotator Cuff: Goals

• Review anatomy/positioning
• Review MR appearance of tears
• Discuss problem tears
• Discuss clinical mimics of rotator cuff tear
• Discuss the radiologic report
Shoulder Pain
• Rotator Cuff Disease
• Impingement
• Arthritis
• Adhesive Capsulitis
Figure 4-25-1
• Cervical Spine
• Referred Pain
• Instability
• Fracture
• Osteonecrosis
• Nerve Entrapment Syndromes
• Bursitis
Shoulder Imaging AP radiograph of the shoulder shows superior narrowing of the

• Radiographs humeral acromial space indicative of a large rotator cuff tear.
• Arthrography Coronal oblique T2 weighted in the same patient confirms a
• CT Arthrography large tear in the supraspinatus tendon
• Ultrasound
• MRI
Radiography [Figure 4-25-1]

• Humeroacromial space <7mm
• Massive tear
CT and Ultrasound [Figure 4-25-2]

Figure 4-25-2
MRI of the Shoulder
• Rotator cuff
• Glenoid labrum
• Capsule
• Biceps tendon
• Bone marrow
• Acromial shape
• AC joint Coronally reconstructed CT image following a direct arthrogram
• Sub-deltoid bursa shows a small full thickness rotator cuff tear in the
• Supraspinatus notch supraspinatus tendon. Coronally acquired ultrasound image in
the same patient demonstrates the full thickness tear as a focal
• Coracohumeral lig
hypoechoic defect in the hyperechoic tendon
• Humeral head shape
Musculoskeletal Radiology 925 MRI of the Rotator Cuff

MRI of the Rotator Cuff Figure 4-25-3
• Coil-dedicated shoulder
• Slice thickness – 3–4 mm
• Matrix – 256x192 or 256x256
• FOV – 16 cm
• Patient position
➢ External rotation vs. neutral
➢ ABER
• Contrast – Indirect or Direct
Pulse Sequences
• Spin-echo
• Fast spin-echo (fat-sat)
➢ Sensitive for cuff tear
• STIR Axial gradient echo image (A) with typical planscan for coronal
• Gradient echo oblique images drawn perpendicular to the glenoid. Axial
• 3D volume gradient echo image through the supraspinatus at the superior
aspect of the shoulder in internal rotation (B) shows the plane
Imaging Planes of acquisition will cross the tendon obliquely. Axial gradient
echo image through the supraspinatus at the superior aspect of
• Axial the shoulder in external rotation (C) shows the plane of
➢ Assess subscapularis, biceps tendon acquisition will parallel the tendon
• Coronal obliques
➢ Parallel to supraspinatus tendon Figure 4-25-4
➢ Assess all tendons
• Sagittal oblique (FSE T2)
➢ 900 to coronals
➢ Assess all tendons
Plane of Scan [Figure 4-25-3]

Rotator Cuff
• Dynamic stabilizer
➢ Complex coordination
• Five layers histologically Coronal oblique T1 weighted image through the infraspinatus with
• Components corresponding gross anatomy
➢ SITS muscles
➢ Rotator cuff interval Figure 4-25-5
❖ Coracohumeral ligament
➢ Long head biceps tendon
Coronal Anatomy [Figures 4-25-4 to 4-25-6]
Sagittal Anatomy [Figure 4-25-7
Axial Anatomy [Figure 4-25-8]
Coronal oblique T1 weighted image through the supraspinatus with

Figure 4-25-6 corresponding gross anatomy
Coronal oblique T1 weighted image through the subscapularis with

corresponding gross anatomy
MRI of the Rotator Cuff 926 Musculoskeletal Radiology

Figure 4-25-7
Sagittal oblique T2 weighted image at the level of the glenohumeral joint shows
the normal rotator cuff muscle anatomy
Normal Anatomy
• http://rad.usuhs.mil/rad/anatomy/shoulder/intro.html Figure 4-25-8
Rotator Cuff Tear
• Impingement
• Overuse
• Aging
• Chronic inflammatory disease
• Acute trauma
• Instability
Pathogenesis RCT: Neer

• Stage I (<25 y/o)
➢ Edema/ hemorrhage
Axial gradient echo image shows the normal subscapularis tendon anteriorally.
• Stage II (25-40 y/o)
The long head biceps tendon is normally situated in the bicipital groove
➢ Fibrosis/ thickening
• Stage III (>40 y/o) Figure 4-25-9
➢ Partial/ Complete Tear
Impingement Syndrome
• Clinical - not radiologic diagnosis
➢ Pain with abduction and external rotation
➢ Pain with elevation and internal rotation
(Neer impingement sign)
• Mechanical causes
➢ Acromial shape, position
➢ AC joint osteophyte
➢ Thick coracoacromial ligament
➢ Instability
Impingement [Figure 4-25-9]
Acromial Variation
• Shape
➢ Type I
➢ Type II
➢ Type III
• Lateral Downsloping
• Anterior Downsloping
• Os acromiale
Acromial Variation
• Increase in number increases risk of tear
• Type I - flat
• Type II – curved
• Type III – hooked With abduction, flexion and internal rotation,
the rotator cuff may impinge on the
• Assess on sagittal images
coracoacromial arch

Os Acromiale [Figures 4-25-10] Figure 4-25-10
• Increased risk of Cuff Tear
• Best seen on axial images
• Injury to Syndesmosis
Rotator Cuff Tear Types

• Full thickness
➢ Communication between joint space and SA
bursa
• Partial thickness
➢ Partial undersurface
➢ Partial Bursal surface
➢ Intrasubstance
➢ “Rim rent”
• Myotendinous
Rotator Cuff Tear Coronal oblique T1 weighted image posterior to the

acromioclavicular joint (A) shows the syndesmosis of the
• Primary accessory ossicle. Coronal oblique T1 weighted image at the
➢ Increased signal in tendon acromioclavicular joint (B) shows the AC joint and
➢ Interruption of tendon supraspinatus tendon. The os acromiale is best seen on the
• Secondary axial image (C ) at the level of the AC joint
➢ Retraction of musculotendinous junction
➢ Obliteration of subacromial bursal fat plane Figure 4-25-11
➢ Fluid in subacromial bursa
➢ Atrophy of muscles
➢ Fluid in muscle belly
Increased Signal within Tendon:

Short TE images
• Magic angle
• Connective tissue between fascicles
• Tendon overlap (internal rotation)
• Degeneration
• Tear
• Partial volume
• Injection
Signal within Tendon: Long TE images Axial gradient echo image at the superior aspect of the humeral
• Mild degeneration – Low Magic angle head (A) shows the anterior aspect of infraspinatus tendon
• Severe degeneration – Intermediate Partial Tear overlapping (lateral) the posterior aspect of the supraspinatus
tendon (medial). Coronal T1 weighted image at the level of
• Tear – High
infra and supraspinatus tendon overlap shows normal high
signal within the junction of the two tendons
Tendon Overlap [Figure 4-25-11]
Partial Tears
• Twice as common as full thickness
Figure 4-25-12
• Intrasubstance – most common
• Bursal Surface – least common
➢ Poor response to conservative Rx
• Increased detection
➢ Contrast
➢ ABER
• Significant if >50% of tendon thickness
Coronal T2 weighted image through the supraspinatus tendon
Partial Undersurface [Figure 4-25-12] (left) shows a deep partial undersurface tear. Coronal T2
weighted image through the supraspinatus tendon of a different
patient (right) shows fluid signal interrupting the articular
surface of the supraspinatus tendon but the bursal surface is
intact indicating an undersurface tear. Both patients have SLAP
tears of the superior labrum

Intrasubstance vs Partial US: Figure 4-25-13
Value of ABER [Figure 4-25-13]
Rim Rent Tear [Figure 4-25-14]

• Seen in young patients
• Usually anterior
• Intrasubstance vs. partial undersurface
Subscapularis Tears [Figure 4-25-15]

• Abnormal lift-off test on PE T1 fat saturated image of the shoulder following indirect
• Uncommon – 2% of all tears arthrography in the ABER position (left) shows the
undersurface of the infraspinatus tendon is intact. The
➢ LHBT dislocation-49% conventional coronal oblique T1 weighted image through
• Look for on axial and anterior coronals infraspinatus tendon was suggested an undersurface tear
➢ Sagittals provide more clues
• Devastating to surgeon if missed Figure 4-25-14
• Easy to miss on arthroscopy
Long Head of Biceps Tendon

• Abnormality frequently associated with RCT
• Medial Dislocation
➢ Abnormal bicipital groove
➢ Chronic impingement
➢ Usually extra-articular
➢ Intra-articular with/without subscapularis tendon injury
➢ Associated with degeneration of tendon
Long Head of Biceps Tendon

• Tendonitis Coronal oblique fat sat T2 weighted image
➢ Increased signal in tendon shows horizontally oriented increased signal in
➢ Thickening of tendon the insertional portion of the posterior
• Rupture of tendon supraspinatus tendon consistent with a tear but
➢ Intracapsular it is difficult to determine whether the tear is an
undersurface or intrasubstance defect
➢ Extracapsular
➢ Ovoid/ heart shaped – partial tear
Figure 4-25-15
Subscapularis Insertion [Figure 4-25-16]
Figure 4-25-16
Sagittal T2 weighted image at the level of the lesser tuberosity
shows a focus of high signal in the subscapularis tendon
representing a partial undersurface tear. The tear is also seen
on the axial T1 weighted gradient echo image following indirect
arthrography (right) but is easier to see on the sagittal image
Axial gradient echo image through the subscapularis tendon

(A) that appears to only insert on the lesser tuberosity on this
image. However, photo of a gross specimen and a
photomicrograph through the insertion of the subscapularis
show that the tendon inserts on both the lesser and greater
tuberosity. The transverse ligament covering the bicipital
groove is not actually a ligament but represents the portion of
the subscapularis tendon that inserts on the greater tuberosity
[Courtesy Tim Sanders, M.D.]

Rotator Cuff Atrophy Figure 4-25-17
• Rotator cuff tear
• Acute brachial neuritis
• Nerve entrapment syndromes
➢ Suprascapular nerve entrapment
➢ Quadrilateral space syndrome
Suprascapular Nerve Entrapment

• Suprascapular Notch
➢ Supraspinatus/ Infraspinatus innervation
• Spinoglenoid Notch
➢ Infraspinatus innervation
• Atrophy of SSM and ISM
• Look for mass in region of suprascapular notch
Spinoglenoid Notch Entrapment [Figure 4-25-17]

• Infraspinatus atrophy
Quadrilateral Space Syndrome [Figure 4-25-18]

• Axillary N. Compression
• Fibrous band
• Pain, paresthesia
• Atrophy of deltoid and/or teres minor
• Weightlifters
Figure 4-25-18
Coronal oblique fat saturated T2 weighted

image shows a paralabral cyst extending from a
posterior superior labral tear into the
spinoglenoid notch. A clinical photo (B) in the
same patient shows marked atrophy of the right
infraspinatus muscle belly as indicated by the
loss of soft tissue inferior to the scapular spine.
Sagittal T2 image (C) medial to the
spinoglenoid notch shows atrophy and
denervation edema in the infraspinatus muscle
belly
The quadrilateral space is bounded by teres

minor, teres major, long head triceps and the
humerus
Clinical Mimics of Rotator Cuff Tear

• Calcific tendonitis
• Adhesive capsulitis
• Subacromial bursitis
Calcific Tendonitis
• Rotator cuff most common site
• Primary or secondary disorder?
HADD in tendon
• Concretion – low T1 and T2
• Variable surrounding edema
• May erode cortex/ invade marrow

Adhesive Capsulitis [Figure 4-25-19] Figure 4-25-19
• Clinical mimic of cuff tear
• Capsule thickened
• Abnormal enhancement — IV gad
Pectoralis Major Tear

• Weight-lifters
• Sternal and clavicular heads
➢ Sternal head superior on humerus
➢ Clavicular head inferior
• Sternal head tear most common
• Use torso coil and coronal obliques
• Myotendinous vs. tendon
Myotendinous Tear of Pectoralis [Figure 4-25-20]
Figure 4-25-20
Coronal oblique T1 (left) and fat sat T2 (right) weighted images Axial T1 weighted gradient echo image (A)
through the pectoralis major muscle show hemorrhage shows marked enhancement in the anterior
associated with a myotendinous injury. The distal tendon is inferior capsule following IV contrast
intact administration indicating adhesive capsulitis in
the atraumatic setting
Radiologic Report
• Acromion-os acromiale
• Tendon – normal, tendinosis, tear
• Size and location of tear
➢ Massive>5cm
• Partial thickness tear
➢ > or < 50% thickness of tendon
• Retraction/Muscle atrophy
References
1. Zlatkin MB, Needell SD, Hoffman C. MRI of the Shoulder, 2nd Edition. Lippincott
Williams & Wilkins, Philadelphia, PA. 2003.
2. Steinbach LS, Peterfy CG, Tirman PFJ, Feller JF eds. Shoulder Magnetic Resonance
Imaging. Lippincott Williams & Wilkins, Philadelphia, PA. September 1998

MR Arthrography of Glenohumeral
Instability
Timothy G. Sanders, MD
Glenohumeral Joint
• Intrinsically Unstable joint
➢ Shallow glenoid fossa
➢ Large articular surface of the humeral head Figure 4-26-1
• Static Stabilizers
➢ Joint capsule
➢ Glenohumeral Ligaments
➢ Glenoid labrum
• Dynamic Stabilizers
➢ Rotator cuff
➢ Long Head of the Biceps Tendon
Classification
• TUBS
➢ Traumatic
➢ Unidirectional
➢ Bankart
➢ Surgery Superior glenohumeral ligament
• AMBRI
➢ Atraumatic
➢ Multidirectional Figure 4-26-2
➢ Bilateral
➢ Rehabilitation
➢ Inferior Capsular Shift
Multidirectional Instability
• AMBRI Patient
• Causes of Multidirectional Instability
➢ Hypermobility or Laxity
➢ Stretching or Overuse of Support Structures
• MR Imaging not usually Required
➢ MR Findings Nonspecific
➢ MR Useful if Direction Unknown to Rule Out Conventional
Causes
Anterior Stabilizers Middle glenohumeral ligament

• Labrum
• Glenohumeral Ligaments Figure 4-26-3
• Capsule
• Subscapularis Muscle
• Most Important Anterior Stabilizer: Inferior Glenohumeral
Labroligamentous complex
➢ Anteroinferior labrum
➢ Anterior Band of the Inferior Glenohumeral Ligament
Normal Labrum
• Anterior and Posterior Labrum best seen in the Axial Plane
• LABRUM:
➢ Dark on all Pulse Sequences
➢ May be triangular, rounded, or blunted
Normal Superior Labrum

• Seen Best in the Coronal Plane
• Superior Labrum: Inferior glenohumeral ligament
➢ Dark on all pulse sequences
➢ Triangular
➢ Extends off of Superior Glenoid
MR Arthrography of Glenohumeral Instability 932 Musculoskeletal Radiology

Superior Glenohumeral Ligament [Figure 4-26-1]
• Prevents inferior subluxation with arm in 0º abduction
• Courses from superior glenoid tubercle to lesser tuberosity
• Parallels Coracoid process Figure 4-26-4
Middle Glenohumeral Ligament [Figure 4-26-2]
• Prevents external rotation of humeral head when
arm is between 45º and 60º of abduction
• Originates at superior glenoid tubercle
• Courses obliquely superficial to the anterior labrum
• Blends with the deep fibers of subscapularis
• Most variable of the glenohumeral ligaments
Inferior Glenohumeral Ligament [Figure 4-26-3]

• Most important GHL Left: Scan plane for the ABER view
• Prevents anterior subluxation with arm in full Right: Normal ABER view
abduction and external rotation
• Extends from anterior inferior labrum to humeral neck
• Lax with arm in neutral position Figure 4-26-5
• Redundant when the arm is in neutral position
Scout Position and Scan Plane for ABER [Figure 4-26-4]

• Scan plane along the long axis of the humeral shaft
• Coronal scout with arm in ABER position
• Stretches anterior band of IGHL
Normal Anatomic Variants

• 1. Cartilage Undermining
➢ Articular cartilage hyaline- intermediate signal intensity
➢ Labrum- fibrocartilage- low signal intensity
➢ Smooth, tapering
➢ Does not Extend Completely Beneath Labrum
• 2. Sublabral Foramen (Hole)
➢ Occurs only in the anterosuperior quadrant
➢ Complete detachment of the labrum from the glenoid
• 3. Sublabral Recess [Figure 4-26-5]
➢ Extends toward the glenoid
➢ No signal extends into the black triangle of the superior labrum
➢ Can mimic a SLAP tear
Sublabral recess
• Buford complex [Figure 4-26-6]
➢ 1.5 % of patients Figure 4-26-6
➢ Can mimic anterior labral tear
➢ Thick cord-like MGHL
➢ Absent or diminutive anterior-superior labrum
Anterior Instability
• 95% of all dislocations
• Mechanism
➢ Fall on outstretched arm
➢ Abduction and external rotation
Bufford complex
Musculoskeletal Radiology 933 MR Arthrography of Glenohumeral Instability

Bankart Lesions [Figure 4-26-7] Figure 4-26-7
• The most common injury following anterior
dislocation
• First-time dislocators under 35 y.o.
• Anterior labro-ligamentous avulsion with disruption of
the medial scapular periosteum
Osseous Bankart Lesion [Figure 4-26-8]

• Fracture of inferior glenoid
• Disruption of the cortex of the anteroinferior glenoid
Hill-Sachs Lesion Bankart lesion

• Results from impaction of humeral head against
anterior-inferior glenoid
• Associated with Bankart lesion
Figure 4-26-8
• Normally: Top 3 images round
• Hill-Sachs: flattening or concavity
• Acute: + Edema
Double Axillary Pouch Sign [Figure 4-26-9]

• Double axillary pouch: small collection of contrast in
inferior labrum seen on coronal images
Perthes Lesion
• Bankart variation (non-displaced)
• Labro-ligamentous disruption Osseous Bankart
• Medial scapular periosteum intact
• May resynovialize in place Figure 4-26-9
• Best detected on ABER view
Nondisplaced Tear Anteroinferior Labrum -

Perthes Lesion [Figure 4-26-10]
ALPSA Lesion
• Anterior labroligamentous periosteal sleeve avulsion
• Intact medial periosteal periosteum
• Medialized Bankart lesion
• Surgical repair technique differs from Bankart
Double axillary pouch
Medialized Bankart Lesion [Figure 4-26-11]
• ALPSA- Anterior labroligamentous periosteal sleeve Figure 4-26-10
avulsion
Chronic Medialized Bankart Lesion

• Labrum scars down medially
• Scar tissue mounds up covering medialized labrum
and resynovializes
• Treatment: complete Bankart and reconstruction
Perthes Lesion
Figure 4-26-11
ALPSA lesion

Axillary Nerve Neuropraxy [Figure 4-26-12] Figure 4-26-12
• Axillary nerve can be stretched at time of anterior dislocation
resulting in denervation atrophy: Deltoid and Infraspinatus
muscles
• Denervation atropy:
➢ Acute: edema (high signal on T2)
➢ Chronic: fatty (high signal on T1)
First Time Dislocation Over Age 35

• Clinical presentation can be confusing
• Tear supraspinatus tendon
• Fracture greater tuberosity
• Avulse subscapularis and anterior capsule from the humerus
• MRI can play pivotal role in directing patient therapy
Tear of the Supraspinatus Tendon

• First time dislocation over age 35:
• Bankart lesion uncommon
• Rotator cuff tendon becomes the weak link Denervation atrophy of Deltoid muscle
First Time Dislocation Over Age 35

• Avulsion fracture of the greater tuberosity
Figure 4-26-13
Greater Tuberosity Fracture
• Avulsion of the greater tuberosity is often occult
radiographically
• Can mimic RCT
• Treated conservatively
• MRI can accurately distinguish
Avulsion of Subscapularis
• Subscapularis muscle can avulse off of lesser
tuberosity
• Associated with dislocation of the biceps tendon
• Seen best in axial plane
Humeral Avulsion of the Glenohumeral Ligament (HAGL)
Lesion
Disruption of Subscapularis
• Disruption of subscapularis at musculotendinous junction
• Requires surgical repair
Figure 4-26-14
Hagl Lesion
• Humeral avulsion of the glenohumeral ligament
• Results from dislocation
• No age predilection
• MR findings: contrast extravasation from joint
capsule/ avulsion of subscapularis
Hagl Lesion [Figure 4-26-13]

• Inferior GHL can disrupt anywhere along course
• Humeral attachment/ mid substance Reverse Bankart and Hill Sachs Lesion
• Difficult to detect with scope
• Cause of failed repairs
• Can present on MRI as avulsion of subscapularis muscle
Posterior Instability
• 2% - 4% of all traumatic dislocations
• 20% - 25% of shoulder instability cases in active duty military population
• Adduction with internal rotation
• Seizure, electrocution, weight lifting, swimming, lineman blocking
• “Reverse” Hill Sachs, Bankart
Posterior Instability [Figure 4-26-14]

• Repetitive microtrauma: non-displaced posterior labral tear
• Reverse Bankart
• Reverse Hill Sachs

Glad Lesion [Figure 4-26-15] Figure 4-26-15
• Glenolabral articular disruption
• Forced adduction injury (humeral head impacts the glenoid fossa)
• Clinically a stable lesion
• Partial tear anteroinferior labrum / articular cartilage injury
Glad Lesion [Figure 4-26-15]

• Non displaced tear anteroinferior labrum
• Best seen on ABER
• Articular Cartilage Injury
• Best seen on axial or coronal
Posterior Superior Glenoid Impingement

• Also known as “Internal Impingement”
• Undersurface tearing of posterior rotator cuff (posterior SST or
IST)
• Impingement between posterior labrum and greater
tuberosity
• Throwing athletes- posterior shoulder pain
• Associated with anterior instability

[Figure 4-26-16]
• Undersurface of posterior rotator cuff impinged
between the greater tuberosity and the
posterosuperior labrum
• Seen best on ABER view
Glenolabral Articular Disruption (GLAD) Lesion
• MR Findings: Figure 4-26-16
➢ Undersurface tear of posterior rotator cuff
➢ Degenerative changes of posterosuperior labrum
➢ Cystic change in greater tuberosity
➢ Internal impingement seen on ABER view
Glenohumeral Internal Rotation Deficit (GIRD)

[Figure 4-26-17]
• Scarring and thickening of the posterior capsule and has recently
been described as a source of potential pain in throwing athletes
• MR imaging demonstrates thickening of the posterior capsule
“SLAP” Tears
• The superior labrum, anterior-to-posterior lesion, can include
biceps tendon
• Mechanism
➢ Fall on outstretched arm
➢ Repetitive overhead activity (throwing, swimming)
• Symptoms: pain with overhead activity, catching, popping
sensation Posterior Superior Glenoid Impingement
Important factors to observe Figure 4-26-17

• Abnormal signal in superior labrum
• Extent of lesion
➢ Posterior labrum
➢ Anteroinferior quadrant
• Biceps involvement
• Type of SLAP tear
Glenohumeral Internal Rotation Deficit (GIRD)

SLAP Lesion Figure 4-26-18
• Type I [Figure 4-26-18]
➢ Fraying and degeneration; but labrum firmly
attached
• Type II [Figure 4-26-19]
➢ Fraying of labrum and superior labrum avulsed
from glenoid
➢ Signal extends into the triangle of the superior
labrum
• Type III [Figure 4-26-20]
➢ Bucket-handle tear of the superior labrum; biceps
tendon remains intact SLAP Type I: Fraying of the undersurface of labrum
➢ On MR imaging: fragment seen displaced into
superior joint space
• Type 4 [Figure 4-26-21] Figure 4-26-19
➢ Bucket-handle tear of superior labrum involves
biceps anchor
➢ Biceps involvement best seen on axial or sagittal
images
Figure 4-26-20
SLAP Type II: Avulsion of labrum from glenoid
SLAP Type III: Bucket-handle tear of the superior labrum Figure 4-26-21
Pitfalls: SLAP Tears

• 1. Sublabral recess
➢ No signal in superior labrum
➢ SLAP tear: any signal extending into black
triangle
• 2. Sublabral recess: axial images
➢ Smooth linear collection of contrast
➢ SLAP on axial images: irregular contrast
collection SLAP Type IV: Bucket-handle tear with involvement of biceps
➢ Sublabral recess: coronal images anchor
➢ No displacement of superior labrum
➢ Type 2 SLAP tear
➢ Labrum pulled away from glenoid
Paralabral Cyst
• High association with labral tears and GH joint
instability Figure 4-26-22
• Superior labral cyst: SLAP tears
• Posterior labral cyst: posterior labral tears
• Labral tear may resynovialize
• Can result in shoulder pain and adjacent nerve
entrapment
• DDX: Intramuscular cysts of rotator cuff associated
with PT tear of the cuff tendon
Paralabral Cysts [Figure 4-26-22]

• SLAP tear with superior paralabral cyst
• Suprascapular notch
• Suprascapular nerve entrapment
Left: SLAP tear with paralabral cyst
• Denervation edema: supraspinatus and infraspinatus Right: Denervation edema within supraspinatus and
muscles infraspinatus muscles

Paralabral Cysts Figure 4-26-23
• Posterior paralabral cysts
• Extend into spinoglenoid notch
• Entrapment of suprascapular nerve
• Persistent shoulder pain for 3 years
• Small anterior labral tear with small adjacent
paralabral cyst
Paralabral cysts
• Small inferior labral cyst
• Teres Minor normal
• 3 years later; persistent pain
• Paralabral cyst larger Left: Direct arthroscopic repair of Bankart lesion. Suture
• Axillary nerve entrapment anchors at the 3, 4, and 5 o’clock positions
• Atrophy Teres Minor Right: Sagittal MR image shows location of suture anchors
Labral Repair: Surgical Approach Figure 4-26-24

• Direct repair of labral and capsular lesions
• Indirect repairs
➢ Staple capsulorapphy (Du Toit & Roux)
➢ Subscapularis manipulation to tighten anterior capsule (Putti
Platt/ Magnuson Stack)
➢ Movement of the coracoid process (Bristow procedure)
Direct Repairs
• Arthroscopic/ open (deltopectoral interval)
➢ Suture anchors 3-,4-,5-, o’clock position
• Capsulorapphy (open/ arthroscopic)
➢ Staple redundant capsule
➢ Done in conjunction with direct repair
➢ High failure rate if done as isolated procedure
• Osseous Bankart Recurrent displace Bankart lesion
MR Findings of Bankart Repair

[Figure 4-26-23] Figure 4-26-25
• Suture anchor artifact from repair may
obscure visualization
MR Findings of Failed Bankart

Repair
[Figure 4-26-24]
• Recurrent displaced anterior labrum
Failed Bankart Repair [Figure 4-26-25]

• Missed HAGL lesion
• In one series– up to 30% of failed Left: Missed HAGL lesion. IGHL avulsed from humeral neck
repairs Center: Missed HAGL lesion. IGHL avulsed from humeral neck
Right: HAGL lesion
Recurrent SLAP Following Repair [Figure 4-26-26]
• Displaced fragment anterosuperior labrum Figure 4-26-26
• Osteochondral defect anterosuperior glenoid
5 Months Following SLAP Repair: Recurrent Pain

• Fraying and irregularity of superior labrum; no displaced fragment
• Partial thickness articular surface tear rotator cuff
Recurrent SLAP tear with a displaced fragment

Multidirectional Instability [Figure 4-26-27] Figure 4-26-27
• Treated first with rehabilitation
• Surgery
➢ Inferior capsular shift/plication
➢ Decrease volume of GHJ anteriorly, inferiorly, posteriorly
➢ MR capsular thickening
Hardware Complication [Figure 4-26-28]

• Proud suture anchor
Synovitis - Prior SA Decompression and Rotator Cuff

Debridement: Recurrent Pain
• Synovitis: 4 mm adhesive capsulitis
• Normal postop capsule:
➢ 2-4 mm after surgical procedure
➢ Thickened and nodular capsule
Postop Infection [Figure 4-26-29]

• Infectious versus reactive synovitis difficult to differentiate with
imaging . Normal postoperative MR appearance
• Thickened enhancing capsule; effusion/ joint destruction/ following inferior capsular shift. Thickened
cartilage loss/ cysts, erosions capsule
Acute Chondrolysis of the Glenohumeral Joint [Figure 4-26-30]

• Rapid onset chondrolysis refers to a condition in which widespread
chondrocyte death occurs within a joint over a relatively short period of time
• Devastating complication reported following
arthroscopy and reconstruction of the GHJ in young Figure 4-26-28
individuals
• Rapid onset pain
• Marked loss of motion
• Treatment supportive; eventually total joint
arthroplasty
Chondrolysis Shoulder:
Proposed Etiologies
• Damage from use of thermal probe for capsular
shrinkage
• Marcaine pump
• Unknown infectious agent Left: MR appearance of a proud suture anchor
• Bioabsorbable material Right: CT appearance of a proud suture anchor
• Mechanical trauma at time of arthroscopy
• Chemical trauma to the chondrocytes
• Event during arthroscopy that triggers an immune response and subsequent Figure 4-26-29
migration of inflammatory cells into the GH joint
Figure 4-26-30
Postoperative infection
Acute Chondrolysis of the Glenohumeral Joint following

Shoulder Arthroscopy

References
1. Bankart ASB: Recurrent or habitual dislocation of the shoulder-joint. Br J Surg 26: 23-29, 1938
2. Beltran J, Rosenberg ZS, Chandnani VP, et al: Glenohumeral instability: Evaluation with MR arthrography.
Radiographics 17: 657-673, 1997
3. Chandnani VP, Gagliardi JA, Murnane TG, et al: Glenohumeral ligaments and shoulder capsular mechanism:
Evaluation with MR arthrography. Rad 196: 27-32, 1995
4. Cvitanic O, Tirman PFJ, Feller JF, et al: Using abduction and external rotation of the shoulder to increase the
sensitivity of MR arthrography in revealing tears of the anterior glenoid labrum. AJR 169 837-844, 1997
5. Kaplan PA, Bryans KC, Davick JP, et al: MR imaging of the normal shoulder: Variants and pitfalls. Rad 184: 519-
524, 1992
6. Linker CS, Helms CA, Fritz RC: Quadrilateral space syndrome: Findings at MR imaging. Rad 188: 675-676, 1993
7. Neviaser RJ, Neviaser TJ, Neviaser JS: Concurrent rupture of the rotator cuff and anterior dislocation of the
shoulder in the older patient. JBJS 70-A: 1308-1311, 1988
8. Neviaser TJ: The anterior labroligamentous periosteal sleeve avulsion lesion: A cause of anterior instability of the
shoulder. Arthroscopy 9: 17-21, 1993
9. Neviaser TJ: The GLAD lesion: Another cause of anterior shoulder pain. Arthroscopy 9: 22-23, 1993
10. Palmer WE, Brown JH, Rosenthal DI: Labral-Ligamentous complex of the shoulder: Evaluation with MR
arthrography. Rad 190: 645-651, 1994
11. Petersilge CA, Witte DH, Sewell BO, et al: Normal regional anatomy of the shoulder. MRI Clin North Am 5: 667-
681, 1997
12. Sanders TG, Tirman PFJ, Linares R: The Glenolabral articular disruption lesion: MR arthrography with
arthroscopic correlation. AJR 172: 171-175, 1999
13. Schweitzer ME: MR arthrography of the labral-ligamentous complex of the shoulder. Rad 190: 641-643, 1994
14. Synder SJ, Karzel RP, Pizzo WD, et al: SLAP lesions of the shoulder. Arthroscopy 6: 274-279, 1990
15. Tirman PFJ, Bost FW, Garvin GJ, et al: Posterosuperior glenoid impingement of the shoulder: Findings at MR
arthrography and MR arthrography with arthroscopic correlation. Rad 193: 431-436, 1994
16. Tirman PFJ, Feller JF, Jansen DL, et al: Association of glenoid labral cysts with labral tears and glenohumeral
instability: Radiographic findings and clinical significance. Rad 190: 653-658, 1994
17. Tirman PFJ, Feller JF, Palmer WE, et al: The Buford complex—A variation of normal shoulder anatomy: MR
arthrographic imaging features. AJR 166: 869-873, 1996
18. Tirman PFJ, Steinbach LS, Feller, FJ: Humeral avulsion of the anterior shoulder stabilizing structures after anterior
shoulder dislocation: demonstration by MRI and MR arthrography. Skeletal Radiol 25: 743-748, 1996
19. Wolf EM, Cheng JC, Dickson K: Humeral avulsion of glenohumeral ligaments as a cause of anterior shoulder
instability. Arthroscopy 11: 600-607, 1995

Imaging of Upper Extremity Trauma
Timothy G. Sanders, MD
Imaging of Upper Extremity Trauma

• Anatomic locations Figure 4-27-1
➢ Shoulder girdle, humerus, elbow, forearm, wrist
and hand
• Structures involved
➢ Bones, joints, articular cartilage, tendons,
ligaments
• Mechanism of injury
➢ Acute trauma, sports related, repetitive stress
injury
Sterno-clavicular Joint
• Dislocation most common injury
➢ Anterior more common than posterior
Grade II separation of AC Joint
• Best evaluated with limited CT scan
• Normal distal clavicle extends above manubrium- use symmetry as guide
• Plain film- 40º cephalic angulation
Clavicle Fractures
• Mechanism: indirect trauma- fall on outer prominence of shoulder
• Most common site of injury is middle third
• Healing may result in deformity (extensive callous)
• Distal third fracture must evaluate coracoacromial ligament integrity
Acromio-clavicular Joint Injuries Figure 4-27-2

• Mechanism: fall on outer prominence of shoulder
• AC joint: weak capsule and inherently unstable
• Grade I injury
➢ mild strain of AC joint
➢ Ligaments intact; point tenderness over AC joint
➢ X-rays normal
➢ Treatment conservative- recovery is spontaneous Grade III separation of the left AC joint
• Grade II injury
➢ moderate strain [Figure 4-27-1]
➢ Disrupted AC ligament
➢ CC ligaments intact
➢ X-ray- widening of AC joint; slight uplifting of distal clavicle
➢ Treatment conservative- recovery is spontaneous
• Grade III injury
➢ severe [Figure 4-27-2] Figure 4-27-3
➢ Ruptured AC and CC ligaments
➢ Complete AC separation with increased distance between
coracoid and clavicle
Surgical Repair of AC Joint Separation

• Internal fixation: 8-10 weeks
• Until CC ligaments heal
Osteolysis of Distal Clavicle [Figure 4-27-3]

• Post-traumatic osteolysis
➢ Complication of trauma (occurs within 2 months of injury, self
limiting)
➢ Repetitive stress (wt. lifters)
➢ X-ray: loss of normal cortical line- distal clavicle
• DDX: rheumatoid arthritis, infection, hyperparathyroid
Post-traumatic osteolysis of distal clavicle
Musculoskeletal Radiology 941 Imaging of Upper Extremity Trauma

Scapular Fractures
• Mechanism: direct trauma to scapula (MVA)
• Uncommon fractures
• Frequently missed on X-ray
• Intra-articular fracture important clinically
• Fracture of scapular body- source of considerable pain
• Fracture of the neck of the scapula
• Non-articular fractures: clinically insignificant; musculature holds fragments in
place; conservative treatment
Scapular Fractures
• CT- imaging modality of choice for evaluating scapula
Scapular Fractures
• Acromion process fractures
• Direct trauma
• Restore active range of motion; if severely comminuted- excise fragments
Scapular Fractures: Acromion

• Os Acromiale: unfused acromial ossification center
Scapular Fractures: Coracoid

• Coracoid fracture: may occur in conjunction with type III AC separation
• Treatment conservative
Stress Fracture of Coracoid Process Figure 4-27-4

• Trap shooter’s shoulder
Imaging of Glenohumeral Joint

• Standard AP view is oblique to the GH joint
• Excellent osseous detail (homogeneous distribution of soft
tissues)
• Glenohumeral “True” AP View (Grashey view)
• Beam tilted 45° laterally
• Evaluate GH joint, subluxation, loss of articular cartilage
• Less value for fractures of surrounding structures
• Axillary view
➢ Evaluate for subluxation/ dislocation; fractures of ant/ post
glenoid
• West Point View: variant of the Axillary View
➢ Improves visualization of the anterior glenoid (Bankart lesion) AP view of the shoulder demonstrates anterior
• Scapular “Y” View dislocation of the humeral head
➢ Image along plane of scapula; 60° relative to the AP view
➢ Easily acquired in setting of acute trauma
➢ Evaluate for ant/ post dislocation
➢ Poor evaluation of the osseous structures
Figure 4-27-5
Anterior Dislocation [Figure 4-27-4]
• Mechanism: fall on outstretched arm
• X-ray: humeral head displaced anterior and medial
• Associated lesions
• Under 35 y.o. Hill-Sachs defect; Bankart lesion or variant
• Over 35 y.o. 1. Avulsion fracture greater tuberosity
2. RCT
3. Subscapularis tear
Anterior Dislocation
• Axillary view
• Scapular “Y” view
Lesions Associated with Anterior Dislocation

[Figure 4-27-5]
• Occurs secondary to impaction of humeral head against inferior Hill-Sachs deformity
glenoid rim
Imaging of Upper Extremity Trauma 942 Musculoskeletal Radiology

Lesions Associated with Anterior Dislocation
• Mechanism: results from impaction of humeral head against inferior glenoid
rim
• Osseous Bankart lesion Figure 4-27-6
• Fibrous Bankart lesion best evaluated with MR
Trauma [Figure 4-27-6]

• 24 y.o. female: persistent painful shoulder after skiing accident
• Radiographically occult humeral head fractures best evaluated
with MR imaging
Posterior Instability
• 2% - 4% of all traumatic dislocations
• 20% - 25% of shoulder instability cases in active duty military
population
• Adduction with internal rotation
• Seizure, electrocution, weight lifting, swimming, lineman blocking
• “Reverse” Hill-Sachs, Bankart
Posterior Dislocation [Figure 4-27-7]

• Dislocates straight posterior MR appearance of a radiographically occult
• Sometimes difficult to detect on AP view: 50% missed fracture of the greater tuberosity
• Locked in internal rotation; most reliable sign
• Very obvious on axillary view
“Trough” Sign
• Vertical line of dense cortical bone paralleling the medial cortex of the humerus
• Results from impaction fracture of the posterior Figure 4-27-7
medial aspect of humeral head
• “Reverse” Hills Sachs Fracture
“Positive Rim” Sign

• Widening of the joint is termed the “positive rim sign”
• Normally the space between the anterior glenoid rim
and medial humeral head is <6mm
• If posterior dislocation suspected- get axillary view
Calcific Tendonitis
• HADD: periarticular calcifications: shoulder most Left: AP view of the shoulder demonstrating posterior
common dislocation of the humeral head
• Results from chronic repetitive micro-trauma Right: Axillary view demonstrating posterior dislocation of the
• Easily detected on x-ray; can be subtle on MR humeral head
imaging
Humeral Head Fractures [Figure 4-27-8]

• Neer’s Four-segment Classification
➢ Humeral head
➢ Humeral shaft
➢ Greater tuberosity
➢ Lesser tuberosity Figure 4-27-8
• Fragment: >1 cm displacement or >45° angulation; considered
significantly displaced
• No fragment >1 cm or 45°- considered a non-displaced fracture
• 1 fragment displaced = 2 part fracture; 2 fragments displaced = 3
part fracture; etc
Humeral Shaft Fractures

• Mechanism:
➢ Indirect twisting force: spiral fx
➢ Direct force: transverse fx
➢ Usually involve mid-shaft
• Shaft fractures heal easily
• Rarely require internal fixation
• Ball-and-socket joint tolerates some degree of angular and
rotational malalignment
Neer’s Four-segment Classification

Humeral Shaft Fractures [Figure 4-27-9] Figure 4-27-9
• Common location for pathologic fractures
• Minor trauma
• Children:
➢ Unicameral bone cyst (fallen fragment)
➢ Fibrous dysplasia
• Adults:
➢ Metastatic lesion
Elbow Fractures: Adult

• Radial head: most common fracture of upper limb in young adults
• Mechanism: fall on outstretched arm
• Frequently occult; oblique views; joint effusion
• Treatment: non-displaced- conservative; comminuted- resection
Elbow Fractures: Child [Figure 4-27-10]

• Supracondylar fracture: Most common elbow fracture in childhood
• Risk: brachial nerve injury
• Mechanism: Fall on outstretched arm
• Posterior fat pad sign
• Anterior “sail” sign
• In setting of trauma indicates occult elbow fracture Pathologic fracture through a unicameral bone
• Anterior humeral line cyst
• Intersect the middle 1/3 of capitellum on lateral view
• Subtle supracondylar fracture results in posteriorly displaced capitellum
Figure 4-27-10
Elbow Dislocations
• Mechanism: fall on outstretched arm- common in child and adult
• Posterior or posterolateral
• Fractures (minor): coronoid/ radial head: child- medial epicondyle
Complications of Dislocation
• Brachial artery or nerve damage
• Post-traumatic ossification: stiffness- intra and periarticular
adhesions: forms in subperiosteal and capsular hematoma
Posterior Dislocations in Child

• Associated with medial epicondyle avulsion in 50% of cases
• Up to 30% become entrapped in the joint following reduction
• Treatment: open reduction for >1 cm displacement of medial
epicondyle
Radiocapitellar Line [Figure 4-27-11] Abnormal anterior humeral line

• Line drawn along long axis of radius should intersect capitellum in any
projection
Night Stick Fracture

• Mechanism: direct force to forearm (usually middle 1/3)
• Isolated fracture of the mid-shaft of the ulna: Usually no displacement or
angulation
• Must exclude associated dislocations
Figure 4-27-11
Normal radiocapitellar line

Isolated Fractures Ulna: Monteggia [Figure 4-27-12] Figure 4-27-12
• Ulnar fracture (usually proximal 1/3); radial head dislocation
• Mechanism: fall on outstretched arm (forced pronation of forearm)
• Treatment:
➢ Open adult/closed child
➢ Restrict pronation/ supination
Isolated Fractures Radius: Galeazzi

• Radius fracture/ distal radioulnar joint dislocation
• High incidence non-union, delayed union with closed reduction
• Tx: ORIF- still a tendency to dislocate after ORIF
Monteggia fracture
Essex-Lopresti [Figure 4-27-13]

• Comminuted radial head fracture/ DRUJ dislocation or instability
• Interosseous ligament:
➢ Intact: radial head resection
➢ Disrupted: radial head prosthesis
Fall on Outstretched Hand Figure 4-27-13

• Childhood: distal radial buckle fracture
• Young adults: scaphoid fracture
• Older adults (>40): Colles fracture
Colles Fracture
• Most common fracture of distal radius in patient over 40 y.o.
• FOOSH injury with wrist in dorsiflexion
• Distal fragment displaced dorsally
Colles Fracture Classification

• A- Extra-articular fx radius
• B- (A) + ulnar styloid fx
• C- Intra-articular fx distal radius
• D- (C) + ulnar styloid fx
• E- Comminuted fxs of radiocarpal and radioulnar joints
• F- (E) + ulnar styloid fx
Smith Fracture (Reverse Colles) Comminuted fracture of the radial head

• Volar angulation of distal fragment
• Younger patient
• High energy trauma with wrist in volar flexion
Barton Fracture (Reverse)

• Longitudinal shear injury of the distal radius
• Barton: Dorsal rim - Reverse Barton: Volar rim
• Transverse load with shearing forces
• Often requires internal fixation: unstable fracture
Radial Styloid Hutchinson’s/ Chauffeur’s Fracture

• Intra-articular fracture of the radial styloid
Distal Radial Fracture in Childhood

• Transverse metaphyseal fracture
• Most common forearm fracture ages 4-10 y.o.
• May be complete or incomplete Top: MR imaging demonstrating an
➢ Torus/ Buckle fracture intact interosseous membrane of the
forearm
Evaluation of Suspected Scaphoid Fracture Bottom: MR imaging demonstrating
Snuff Box Tenderness disruption of the interosseous
• 1. Additional radiographic views membrane of the forearm
➢ Oblique view
➢ Scaphoid view
• 2. Cross-sectional imaging
➢ MRI
➢ CT
➢ Bone scan

Scaphoid fracture: Complication rate is high
[Figures 4-27-14 and 4-27-15]
Figure 4-27-14
• AVN
• Delayed/ nonunion
• Osteoarthritis
• AVN complication of scaphoid fracture
• Recurrent blood supply
• Risk factors: delayed diagnosis, displacement,
proximal fracture site
Figure 4-27-15
Left: Avascular necrosis of the proximal pole of the scaphoid

Right: Nonunion of a scaphoid fracture with secondary
osteoarthritis
Figure 4-27-16
Recurrent blood supply to the scaphoid bone
Scapholunate Ligament Disruption [Figure 4-27-16]

• Normal scapholunate distance = 2mm
• Gap accentuated with “clenched” fist view
Increased scapholunate distance indicating
Scapholunate Ligament disruption of the scapholunate ligament
• Wrist MR arthrography can increase detection rate of subtle
interosseous ligament disruption
Carpal Dislocations [Figure 4-27-17]

• Dislocations
➢ Lunate: lunate dislocates in volar direction
➢ Perilunate: capitate dislocated dorsally/ lunate remains normal
Figure 4-27-17
Left: AP view of wrist demonstrates lunate dislocation

Center: Lateral view of wrist demonstrate lunate dislocation
Right: Perilunate dislocation: posterior capitate dislocation, lunate remains in normal
position relative to the radius

Triquetrum Fracture [Figure 4-27-18] Figure 4-27-18
• Shearing forces, dorsal avulsion fracture seen only on the lateral
view
• Point tender over dorsal aspect of wrist
• Mechanism: fall on out-stretched hand
• Immobilize in plaster for 3 weeks
• Associated with lunate, perilunate dislocation
Hamate Fracture [Figure 4-27-19]

• Point tenderness over hook of hamate
• Direct trauma to volar aspect of wrist; surgical resection of hook
Fracture of First MC- Bennett Fracture [Figure 4-27-20]

• Oblique intra-articular fracture/subluxation of base of 1st MC
• The large fragment subluxes; smaller fragment maintains position
• Closed treatment: persistent subluxation/ traumatic arthritis
• Usually treat with ORIF
• Bennett “Bad”
Triquetrum fracture
Bennett Fracture
• Deforming forces
➢ Abductor pollicis longus pulls the distal fragment in a Figure 4-27-19
proximal/dorsal direction
➢ Adductor pollicis muscle stabilizes the volar ulnar lip of the
articular surface in its normal position
➢ Thus results in distraction of the 2 fracture fragments
Fracture of First MC- Rolando Fracture

• Comminuted Bennett fracture
• Dorsal subluxation and a separate dorsal fragment
• Usually treated closed as pinning does not work well on
comminuted fragments
• Worse prognosis than the Bennett fracture
• Rolando “Ruined”
Skier’s Thumb Injury (Gamekeeper’s thumb) Hook of Hamate Fracture

• Disrupt Ulnar Collateral Ligament
• May have small avulsion fracture
• Pin or ORIF
Figure 4-27-20
Stress Views [Figure 4-27-21]
• Stener Lesion: MR imaging
• Interposition of adductor pollicis apponeurosis between torn
UCL and base of proximal phalanx
• Surgical lesion
Figure 4-27-21
Bennett Fracture
Disrupted ulnar collateral ligament

Mallet Finger (Baseball Finger) [Figure 4-27-22] Figure 4-27-22
• Sudden resisted flexion of DIP joint
• Finger jammed or distal tip hit with a ball
• Avulsion of extensor digitorum tendon
• ORIF and splint in full extension 6 weeks
Volar Plate Avulsion

• Hyperextension avulsion injury
• MCP or PIP joint
Avulsion of Flexor Digitorum: Jersey Finger

[Figure 4-27-23]
• Forced hyperextension
• Avulsion of flexor digitorum profundus
• Osseous fragment displaced proximally
Figure 4-27-23
Isolated Tendon and Pulley Injuries [Figure 4-27-
24]
• Rupture of pulley system occurs with forced
extension
• Bowstringing of flexor tendon
• Common injury in rock climbers
Mallet finger: avulsion of
extensor digitorum tendon
Phalangeal Dislocations: Coach’s Finger
[Figure 4-27-25]
• Usually dorsal; often associated with volar plate injury
• Simple: closed reduction;
• Complex: ST entrapment: open reduction
Boxer’s Fracture
• 4th or 5th MC
• Dorsal angulation
• Treatment open >35º angulation
Distal Tuft Fracture Jersey Finger: Avulsion

injury of the flexor digitorum
• Fracture of distal tuft of phalanx
profundus
• Mechanism: crushing injury
• Ignore the fracture and treat soft tissue injury
• Watch for complication of osteomyelitis with open fracture/ nail
bed injury Figure 4-27-24
Figure 4-27-25
Coach’s Finger: Interphalangeal dislocation
References
1. Manaster, BJ. Handbook of Skeletal Radiology, second edition. St Louis,

MO: Mosby, 1997: 171-225
2. Rogers, LF. Radiology of Skeletal Trauma, third edition. Philadelphia, PA,
Churchill Livingstone, 2002, 593-929 Rock Climber’s Finger: Flexor pulley disruption

Crystal Deposition Diseases and
Neuropathic Osteoarthropathy
Crystal Deposition Diseases

• Gouty arthritis
➢ Monosodium urate
• CPPD crystal deposition disease
➢ Calcium pyrophosphate dihydrate
• HA crystal deposition disease
➢ Calcium hydroxyapatite
Related Disorders
• Hemochromatosis
➢ Iron deposition
• Alkaptonuria
➢ Homogentisic acid
Gout
• Monosodium urate crystals
• Intra-articular, periarticular
• Acute inflammatory response
• Chronic granulomatous reaction
Gout: Primary (Idiopathic)

• Male: Female = 20 : 1
• 40–50 years of age
• (Familial history)
Gout: Secondary
• Hereditary diseases
• Myeloproliferative diseases
• Endocrine disorders
• Drug therapy
Gout: Clinical Stages

• Asymptomatic hyperuricemia
• Acute gouty arthritis
• Interval phase
• Recurrent arthritis
• Chronic tophaceous gout
Gouty Arthritis
• Polyarticular
• Asymmetric
• 1st MTP joint (90%)
• Tarsometatarsal
• Carpometacarpal
• Olecranon bursitis
Musculoskeletal Radiology 949 Crystal Deposition Diseases and Neuropathic Osteoarthropathy

Gouty Arthritis [Figures 4-28-1 to 4-28-3] Figure 4-28-1
• Soft tissue swelling
• Tophi (calcification rare)
• Well-defined erosions
• Overhanging edge
• Preserved joint space
• Extra-articular erosions
Figure 4-28-2
Gouty arthritis. Radiograph of the distal

interphalangeal joint shows characteristic well-
Gouty arthritis. A. Pathological specimen shows tophaceous defined erosion of bone with overhanging edge
material (star) and sharp interface with adjacent bone (arrow). of new bone (arrow). Note also preservation of
B. Clinical radiograph shows large well-defined erosions of the joint space
first metatarsal head with overhanging edge of new bone
(arrow). Note multiple other erosions including the base of the
first metatarsal Figure 4-28-3
CPPD Crystal Deposition Disease: Terminology

• Pseudogout
• Chondrocalcinosis
• Pyrophosphate arthropathy
CPPD Crystal Deposition Disease

• Usually idiopathic
• Occasionally hereditary
• Over 50 years of age
• Incidence 5% to 34%
CPPD Crystal Deposition Disease:

Related Disorders
• Primary hyperparathyroidism
• Hemochromatosis
Gouty arthritis. There are multiple well-defined
erosions, particularly at the bases of the
CPPD Crystal Deposition Disease: metacarpals at the common carpometacarpal
Sites of Calcification joint
• Fibrocartilage
• Hyaline cartilage
• Synovial membrane
• Joint capsule
• Ligaments
• Tendons
• Bursae
Crystal Deposition Diseases and Neuropathic Osteoarthropathy 950 Musculoskeletal Radiology

Fibrocartilage Calcification
• Knee menisci
• Symphysis pubis
Figure 4-28-4
Pyrophosphate Arthropathy
• Joint space narrowing
• Bone sclerosis
• Osteophyte formation
Differentiation of Pyrophosphate
Arthropathy from Degenerative Joint
Disease
• Unusual articular distribution
• Unusual intra-articular distribution
• Variable osteophyte formation
• Prominent subchondral cysts CPPD crystal deposition disease. A. Frontal radiograph of the
• Progressive destruction knee shows chondrocalcinosis including meniscal fibrocartilage
• Calcification calcification (white arrows) and hyaline cartilage calcification
(gray arrow).
B. Lateral radiograph shows complete loss of patellofemoral
CPPD Crystal Deposition Disease: Knee joint space
[Figure 4-28-4]
• Meniscal calcification
• Hyaline cartilage calcification
• Patellofemoral arthropathy
• Osseous bodies
CPPD Crystal Deposition Disease: Figure 4-28-5

Wrist and Hand [Figures 4-28-5 and 4-28-6]
• Triangular fibrocartilage calcification
• Radiocarpal arthropathy
• “Stepladder” configuration (SLAC)
• Metacarpophalangeal arthropathy
• Absence of erosions
Figure 4-28-6
CPPD crystal deposition disease. Frontal radiograph of the

wrist shows joint space narrowing between the radius and
scaphoid and between the lunate and capitate (scapholunate
advanced collapse [SLAC]). There is extensive
CPPD crystal deposition disease. Frontal radiograph shows chondrocalcinosis including the triangular fibrocartilage (arrow)
narrowing of the second and third metacarpophalangeal joints as well as a large subchondral cyst in the radius (star)
with sclerosis, osteophytes, and subchondral cysts. Note
hyaline cartilage calcification (green arrow) and probable
synovial and capsular calcification (yellow arrows)

Hemochromatosis
• Primary
➢ Increased iron absorption
• Secondary
➢ Increased iron intake
➢ Multiple blood transfusions
Hemochromatosis: Clinical Findings

• Onset between 40 and 60 years
• Men more often than women Figure 4-28-7
• Bronze pigmentation
• Cirrhosis
• Diabetes mellitus
• Cardiac failure
• Arthropathy
Hemochromatosis:
Pathologic/Radiologic Findings
[Figure 4-28-7]
• Iron in synovioblasts
• CPPD crystal deposition
• Osteoporosis
• Symphysis pubis calcification
• Hyaline cartilage calcification Hemochromatosis. Frontal radiograph shows narrowing of the
• Uniform MCP joint involvement second through fifth metacarpophalangeal joints as well as
multiple large hook-like osteophytes (arrows)
• Hook-like osteophytes
HA Crystal Deposition Disease

• Primary
• Secondary
• Periarticular
• Intra-articular
Primary Periarticular HA Crystal Deposition Disease:

(Calcific Tendinitis)
• Middle-aged
• Monoarticular
• (Asymptomatic)
• Localized pain
• Tenderness to palpation
• Restriction of motion
• (Fever and malaise)
Primary Periarticular HA Crystal Deposition Disease:

(Calcific Tendinitis) [Figure 4-28-8]
• Soft tissue swelling Figure 4-28-8
• Poorly defined calcification
• Sharply defined calcification
• Resorption of calcification
Intra-Articular Hydroxyapatite Crystal

Deposition Disease: Milwaukee Shoulder
• Elderly women
• Shoulder pain
• Decreased mobility
• HA crystal shedding Calcific tendinitis. A. Radiograph of the shoulder in external
• Activated collagenase rotation shows poorly defined calcification corresponding to the
• Neutral protease supraspinatus tendon (arrow).
B. Radiograph of the shoulder of a different patient in internal
• Tissue destruction rotation shows sharply defined calcification corresponding to
the infraspinatus or teres minor tendon (arrow)

Milwaukee Shoulder: Radiologic Findings
• Amorphous calcification
• Glenohumeral joint narrowing
• Subchondral sclerosis
• Rotator cuff disruption
• Acromiohumeral abutment
Alkaptonuria: Clinical Findings

• Homogentisic acid oxidase deficiency
• Onset between 20 and 30 years
• Bluish-black pigmentation (ochronosis)
• Dark colored urine
• Cardiovascular
• Genitourinary
• Upper respiratory
• Articular
Alkaptonuria: Pathologic Findings

• Connective tissue pigmentation
• Fibrocartilage and hyaline cartilage
• Fibrillation, fragmentation
• Granulation tissue
• Osseous proliferation
Alkaptonuria: Radiologic Findings

• Disc calcification
➢ Annulus fibrosus
➢ Diffuse
• Multilevel disc narrowing
• Vacuum phenomena
• Osseous sclerosis
Alkaptonuria: Radiologic Findings [Figure 4-28-9]

• Knees, hips, shoulders
• Symmetric cartilage loss Figure 4-28-9
• Osseous sclerosis
• Collapse and fragmentation
• Intra-articular bodies
Neuropathic Osteoarthropathy
• Charcot joint
• Tabetic arthropathy
• Neurotrophic joint
• Neuropathic arthropathy
• Neuroarthropathy
Neuroarthropathy: Etiologies
• Alcoholism
• Syringomyelia
• Syphilis Alkaptonuria. A. Lateral radiograph of the lumbar spine shows
• Leprosy uniform loss of disc height and associated bone sclerosis.
• Meningomyelocele There is disc calcification bridging all levels anteriorly except
• Congenital insensitivity to pain for L4-L5, which shows large osteophytes and a vacuum
• Steroid administration (intra-articular) phenomenon.
B. Frontal radiograph of the knee shows uniform joint space
loss and bone sclerosis

Neuroarthropathy: Pathogenesis Figure 4-28-10
• French theory
• German theory
• Neurotraumatic
• Neurovascular
Neuroarthropathy: French Theory

• Mitchell (1831)
• Charcot (1868)
• Damage to CNS trophic centers
• Altered bone and joint nutrition
• Osseous and articular atrophy
Neuroarthropathy: German Theory

• Volkmann, Virchow
• Insensitivity to pain
• Recurrent trauma Neuropathic osteoarthropathy. Frontal
radiograph of the shoulder shows almost
Neuroarthropathy: Neurotraumatic Theory complete resorption of the humeral head
• Eloesser (1917) except for some osseous debris medially
(arrow). Note the extremely sharp margin of the
• Posterior sensory nerve section
remaining portion of the humeral shaft
• Continued weightbearing
• Joint destruction
• Chemical analysis: no atrophy
Figure 4-28-12
Neuroarthropathy: Neurovascular Theory
• Neurally initiated vascular reflex
• Increased bone blood flow
• Osteoclastic bone resorption
• Fracture and repair
Neuroarthropathy: Clinical/Pathologic Findings

• Swollen, deformed joint
• Usually painless
• Detritic synovitis
• Rapid progression
Neuroarthropathy: Radiologic Findings

[Figures 4-28-10 to 4-28-12]
• Destruction (atrophy)
• Dislocation
• Disorganization
• Debris
• Detritus
• Density (sclerosis)
• Distension (effusion)
Figure 4-28-11
Neuropathic osteoarthropathy. Frontal

radiograph of the foot shows characteristic
destruction, disorganization, and debris around
the tarsometatarsal joints
Neuropathic osteoarthropathy. A. Lateral radiograph of the foot

shows extensive destruction of the bones of the midfoot with
dislocation and disorganization. Note extensive vascular
calcification from diabetes.
B. Followup lateral radiograph less than one month later shows
further bone destruction with almost complete disappearance
of the talus and the anterior portion of the calcaneus

MRI of the Elbow
Mark E. Schweitzer, MD; William B. Morrison, MD
Figure 4-29-1
Anatomy
• Osseous-radius
ulna
humerus
• Ligamentous-medial collateral
lateral collateral
• Musculotendinous
➢ Posterior: triceps
➢ Anterior: biceps, brachialis
➢ Medial: flexor-pronator
➢ Lateral: common extensor
• Neurovascular
Articular Anatomy
• Capitellum-hemispherical-articulates with radius
• Trochlea-spool 300 degree arch-articulates with ulna
• Coronoid fossa-ant/sup to trochlea, small-articulates with
coronoid
• Olecranon fossa 180 degrees-art. with semilunar notch
• Lesser sigmoid notch- radial side of proximal ulna/PRUJ
Biomechanics
• Three articulations:
➢ Ulnar-tochlear
➢ Radiocapitellar Effusion and synovitis.
➢ Proximal radioulnar Note complex fluid in the joint
➢ 0-140 degrees flex/ext distending the anterior and posterior
➢ 75 pronation fat pads (arrows). The fat pads are
intracapsular but extrasynovial
➢ 80 supination
Effusion [Figure 4-29-1] Figure 4-29-2

• Fat pads are intracapsular and extrasynovial
• DDx:
➢ Fracture
➢ Internal Derangement (e.g., ligament, cartilage)
➢ Arthritis (e.g., RA, OA, septic)
Cartilage Loss
• Difficult to see directly (cartilage thin)
• Subchondral marrow edema best sign
• Phytes-posterior/medial/coronoid
➢ Confirm cart loss / cause impingement
• Associated with effusion and bodies
OCD [Figure 4-29-2]

• Capitellum- 3rd most common site in body (after
ankle, knee) Unstable OCD of the capitellum. Note contrast extending
• Repetitive microtrauma during valgus (assoc with under osteochondral fragment (arrow) on this MR arthrogram
MCL)
• Symptoms = pain, locking
• Sequelae- bodies/OA
• Staging-fluid/cyst under fragment=loose (unstable)
Musculoskeletal Radiology 955 MRI of the Elbow

Pitfalls: NL variation
• Trochlear sulcus
• Posterior capitellar pseudodefect
• DDx: location; no underlying edema
Figure 4-29-3
Intraarticular Bodies [Figure 4-29-3]
• Often adherent to synovium
➢ Intraarticular, not loose
• Usually begin as cartilage defects and grow
➢ Often from OCDs)
• Variable signal characteristics
➢ Use GRE (TE>7), tend to bloom
• In the recesses, usually olecranon / coronoid
• MR imaging: arthrography, or effusion
Synovial Folds
• Embryologic remnant
• Several locations
➢ Posterior/lateral catches
➢ Medial (meniscoid) most common
• May mimic bodies clinically and on MR
Synovial Folds Intraarticular body (arrow) surrounded by

• Lateral plica syndrome: Posterolateral catching/locking contrast in the olecranon recess in a patient
with posterior impingement
Posterior Impingement
• Part of tennis elbow spectrum
• Osteophytes- often related to chronic MCL overload, hyperextension (e.g.,
pitchers)
• Bodies in olecranon fossa
• Non-union of old triceps avulsion
Nerve Impingement [Figure 4-29-4]

• Potential locations of nerve impingement
• Median/radial nerve Figure 4-29-4
➢ Proximal arcade of Struthers (avian spur)
➢ Impinged by fascicle of lacertus fibrosus
➢ Median nerve branch through pronator teres muscle (pronator
syndrome)
➢ Supinator syndrome, radial tunnel syndrome, interosseous
syndrome
➢ Look for muscle edema distally
• Ulnar nerve [Figure 4-29-4]
➢ Cubital tunnel
➢ Focal edema/STS/sublux/mass
➢ Look above and below
➢ Associated with epicondylitis, MCL injury
Epicondylitis
• Medial: golfer’s elbow (common flexor origin)
• Lateral: tennis elbow (common extensor origin) Enlargement of the ulnar nerve (arrow) with
• Spectrum from degeneration to partial to completetear surrounding soft tissue inflammation in a
• Increased T1 signal patient with
• Increased STIR, T2 signal cubital tunnel syndrome
• Linear vs. diffuse
• Histologically- angioblastic changes/ fibrillar collagen degeneration
MRI of the Elbow 956 Musculoskeletal Radiology

Lateral Epicondylitis [Figure 4-29-5] Figure 4-29-5
• Repetitive overload of extensors
• “Tennis elbow”
• 35-70 years old
• Pain/tenderness focally, may radiate proximally
• Usually extensor carpi radialis
Biceps: Anatomy
• Long head: superior glenoid
• Short head: coracoid
• Two heads merge distal to the bicipital groove
• Insertion onto radial tuberosity at elbow
• Intimate with brachialis
• Proximal synovial sheath
• Distally paratenon, bicipitoradialis bursa and lacertus fibrosus
Biceps Pathophysiology
• Degeneration
➢ Primary (overuse injury) Lateral epicondylitis (tennis elbow) with
➢ Or direct frictional effect focal fluid signal (arrow) at the common
extensor tendon origin indicating a partial tear
• Mechanical-pronation leads to impingement between radius and
ulna
• Hypovascular-critical zone
distally like rotator cuff
Figure 4-29-6
Biceps Tendinosis
• Common, but rare to image
• Imaging/ clinically overlap with partial tear, bursitis
• Very distal at insertion
Biceps-Partial Tears
• Attritional
• Pain
• No pop, usually no ecchymosis
• More marrow edema and bursitis
• Surgery not usually needed unless large
Biceps Injury: Distal vs Proximal [Figure 4-29-6]

• Both: muscle belly retraction “popeye arm”
• Fluid dissects around muscle belly
• Both: sudden snap, arm hematoma
• Distal young/ proximal older
• Distal sports/ proximal – chronic impingement, RCT
• Proximal: surgery uncommon (two heads), except for repair of Complete distal biceps tendon tear.
cuff, resection of spur Note end of retracted tendon (arrow) with
• Distal: surgery common muscle bunched up proximally resulting in a
• Associated marrow edema “popeye” arm
• Associated bursitis
Bicipital Radialis Bursitis

• Distal biceps lacks a sheath
• Apparent fluid around is bursal
• Close to insertion
• Ddx; vessel
• Assoc with biceps tears (esp partial), RA, mechanical –maybe 1st

Triceps Injuries
• Fairly uncommon
• Spectrum tendonitis (posterior tennis elbow/ posterior impingement), snapping,
to tear
• Risk factors: steroids, SLE, CRF, RA, gout
• Within 2–3 cm of insertion, usually at Figure 4-29-7
• Associated olecranon bursitis
• Associated soft tissue edema
• Look for avulsion fx
Olecranon Bursitis [Figure 4-29-7]

• Anatomic bursa
• Normally no fluid visible
• Bursitis: Fluid, loss of subQ fat adjacent to olecranon
• Causes
➢ Trauma
➢ RA
➢ Gout
➢ Infection
Muscle Disorders
• Tears
• DOMS
• Neuropathy-Parsonage Turner Syndrome “edema-like” muscle
signal
• Infection
Olecranon bursitis
Osseous Injury [Figure 4-29-8]
• Effusion on Xray: presumed fx Figure 4-29-8
• F/U X-ray vs MR
• Bone marrow edema after trauma: Bone bruise vs. fracture
➢ T1: focal low signal (linear) = fracture
➢ No line, ill-defined edema = bruise
Avulsion
• Chronic avulsive stress
➢ Tendinopathy
➢ Usu subtle edema at enthesis
• Avulsion fx
➢ Ligamentous or tendinous
➢ Thin, longitudinally oriented edema at cortical margin
Osteochondral Impaction
• Analogous bruises from ACL tears
• Transient disloc/sublux
• Often both sides of joint
Fracture Complications
• OA
• Bodies Occult radial neck fracture
• Capsular fibrosis
• Non union / malunion
• Associated ligament injury/instability (e.g., Essex Lopresti)
• AVN
• Pain
• Limited ROM
• Instability

AVN Figure 4-29-9
• Older: typical risk factors for AVN
• Younger = Panner’s disease
➢ Capitellum
➢ Boys; 4-10 years
➢ Decreased vasc to growing epiphysis
➢ Usu. spontaneously resolves
➢ If >10, higher risk of complications
Ligament Disorders
• Medial
• Lateral
Medial Collateral Ligament

• Three segments
➢ Anterior bundle
❖ Most important soft tissue static constraint to valgus
stability
➢ Posterior bundle
➢ Transverse bundle
Medial collateral ligament tear (arrow)
MCL [Figures 4-29-9 and 4-29-10] and osteochondral impaction injury
(arrowhead)
• Anatomy
➢ Anterior/posterior/transverse bands Figure 4-29-10
➢ Strongest is anterior
• Pathophysiology
➢ Overhead throwing /valgus overuse, weakens/incompletely
heals, reinjures
• Partial tears — “T” sign vs. complete tears
• Old tears show thickening +/- bowing
• Association with epicondylitis
Lateral Ligaments
• Components
➢ Lateral collateral ligament proper (LCL) (Radial collateral
ligament)
➢ Annular ligament
➢ Lateral ulnar collateral ligament (LUCL)
Annular Ligament
• Fibro-osseous ring that encircles and stabilizes the radial head
• Attaches on the anterior and posterior edges of the lesser sigmoid
notch Medial collateral ligament tear (arrow) and
• Anterior portion taught in supination and posterior portion taught lateral ulnar collateral ligament tear
in pronation (arrowhead) in a
patient with recent elbow dislocation
Radial Collateral Ligament
• Extends from the lateral epicondyle and attaches to the annular ligament
• Immediately deep to the common extensors
• About half to a third size of MCL
• Maintains humeroradial apposition in the presence of varus stress
Lateral Ulnar Collateral Ligament

• With annular ligament (PRUJ) and radial collateral ligament (radial head)
makes up lateral lig complex
• Sweeps posteriorly past the radial neck and inserts on the ulna
• Stabilizer for rotational and varus stress

Posterolateral Rotatory Instability
• Instability of the elbow manifested by painful clicking of the elbow in extension
• Radial head moves posteriorly in relation to the capitellum
• Essential lesion - tear of the LUCL
• Lateral pivot shift test
➢ Supination with axial and valgus stress
Lateral pivot shift test - supination with axial and valgus stress
LUCL INJURIES
• Caused by a fall on an outstretched hand
• Iatrogenic injury during release or repair of lateral epicondylitis
Epitrochlear Lymph Node

• Mimics a mass
• SubQ
• Inflammatory-like signal
• Cat scratch fever
• Assoc fasciitis
MR Arthrography Indications
• Bodies
• MCL injuries
• OCDs
• Subtle cartilage loss
Elbow MRA
• Ligament tear
➢ Extracapsular leakage of contrast
➢ Medial or lateral collateral ligament tear
• IA bodies
➢ Anterior, posterior recesses
• OCD
➢ Same dx as knee
➢ Esp capitellum
References
1. Jbara M, Patnana M, Kazmi F, Beltran J. MR imaging: arthropathies and infectious conditions of the elbow, wrist,
and hand. Magn Reson Imaging Clin N Am. 2004 May;12(2):361-379.
2. Bordalo-Rodrigues M, Rosenberg ZS. MR imaging of entrapment neuropathies at the elbow. Magn Reson Imaging
Clin N Am. 2004 May;12(2):247-263.
3. Potter HG, Ho ST, Altchek DW. Magnetic resonance imaging of the elbow. Semin Musculoskelet Radiol. 2004
Mar;8(1):5-16
4. Chung CB, Kim HJ. Sports injuries of the elbow. Magn Reson Imaging Clin N Am. 2003 May;11(2):239-53.
5. Steinbach LS, Palmer WE, Schweitzer ME. Special focus session. MR arthrography. Radiographics. 2002 Sep-
Oct;22(5):1223-1246.
6. Zou KH, Carrino JA. Comparison of accuracy and interreader agreement in side-by side versus independent evaluations
of MR imaging of the medial collateral ligament of the elbow. Acad Radiol. 2002 May9(5):520-5.
7. Jbara M, Patnana M, Kazmi F, Beltran J. MR imaging: arthropathies and infectious conditions of the elbow, wrist,
and hand. Magn Reson Imaging Clin N Am. 2004 May;12(2):361-79
8. Bordalo-Rodriguez M, Rosenberg ZS. MR Imaging of entrapment neuropathies at the elbow. Magn Reson Imaging
Clin N. Am. 2004 May; 12(2):247-63.
9. Chung CB, Chew FS, Steinbach L. MR imaging of tendon abnormalities of the elbow. Magn Reson Imaging Clin
N. Am. 2004 May;12(2):233-45.
10. Kaplan LJ, Potter HG. MR imaging of ligament injuries to the elbow. Magn Reson Imaging Clin N. Am. 2004
May;12(2):221-32, v-vi.
11. Fowler KA, Chung CB. Normal MR imaging anatomy of the elbow. Magn Reson Imaging Clin N. Am. 2004
May;12(2):191-206, v.
12. Potter HG, Ho St, Altchek DW. Magnetic resonance imaging of the elbow. Semin Musculoskeletal Radiol. 2004
Mar;8(1):5-16.
13. Savnik A, Jensen B, Norregaard J, Egund N, Danneskiold-Samsoe B, Bliddal H. Magnetic resonance imaging in the
evaluation of treatment response of lateral epicondylitis of the elbow. Eur Radiol. 2004 June;14(6):964-9. Epub
2003 Dec 11.

Skeletal Metastases, Myeloma, Lymphoma
Michael E. Mulligan, MD
Figure 4-30-1
Incidence of Metastases [Figure 4-30-1]
• 30% of all patients with Cancer
• Skeleton – 3rd most common site
• More than 80% due to PTBLK
• #1-Breast, #2-Prostate, #3-Lung
• Spine lesion – Breast 75%
• Femur lesion – Breast 50%
• Skull lesion – MM, B, L
• Hands/Feet – Lung
• P=prostate T=thyroid B=breast L=lung K=kidney
Mechanism of Spread to Bones

Hematogenous/Contiguous
• Marrow vessels unusual, rich sinusoidal system with large
endothelial gaps
• Batson’s plexus has direct connection to IVC/SVC with no valves 48 year old man with renal cell
• Arterial – mechanism for distal mets? carcinoma metastases mimicking
multiple myeloma
Batson OV. Ann Surg 1940;112:138
Figure 4-30-2
Bone Metastases
• Different T1, T2 signal
• Different Gadoliniumenhancement
• The “holy grail” – DWI
Radiology 1998; 207:305-7
Acute Vertebral Collapse - Osteoporosis or Malignancy

[Figure 4-30-2]
• Symptoms and Signs
➢ Pain, most common symptom, but only in 2/3 patients
➢ Pathologic fracture
❖ Common sign, esp bad in spine, femur
Pathologic Fractures [Figure 4-30-3]

• 5–10% of all patients with mets
• 50% or more cortex gone - 2/3 will develop pathologic fracture
61 year old woman with breast
• Less than 50% cortex gone - 1/5 will develop pathologic fracture
cancer, focal depression of L2
• Any lesion in femoral neck
superior endplate is indicative of
• Avulsion of lesser trochanter
metastatic disease
• Any lesion prox femur >2.5cm
• Mirels score - site, size, l/b, pain Figure 4-30-3
• 3.0 cm lesion with persistent pain after XRT
Mirels, H. Clin Ortho Rel Res 1989; 249: 256-64
55 year old man with lung

cancer and cortical “cookie
bite” metastasis
Musculoskeletal Radiology 961 Metastases, Myeloma, Lymphoma

Bone Metastases: Systemic Features [Figure 4-30-4] Figure 4-30-4
• Hypercalcemia
• Hypertropic Osteoarthropathy, triad of
➢ Periosteal reaction
➢ Clubbing
➢ Pain
Hypertrophic Osteoarthropathy
• Classic Triad
• Joint swelling, 30%–40% patients
• 5% patients with lung cancer
• Cause? – may be paraneoplastic, due to a growth hormone releasing factor
Bone Metastases: Radiologic Features

• Pure lysis
• Lysis with blowout (renal, thyroid)
• Mixed lytic/blastic (breast, lung, GI)
• Pure blastic (prostate, carcinoid, medulloblastoma)
Osteoclast – Activating Factor (A Cytokine)

• Stimulates clasts to synthesize collagenase
• Produced by normal activated leukocytes
• Dependent on prostaglandin E
• Prostaglandin inhibitors can reduce/obliterate osteolysis
JBJS 68A:310, FEB 1986 2 year old Irish setter
with lung cancer.
Bone Metastases: Radiologic Features Foreleg bones show
• Ivory vertebra classic periosteal
• Pathologic fx changes of H.O
• Periosteal rx – prostate, lung neuroblastoma, GI tumors
• Soft tissue mass (lung)
• Missing pedicle
• Intracortical – lung cancer
Ivory Vertebra(ae):
Differential Diagnosis [Figure 4-30-5] Figure 4-30-5 Figure 4-30-6
• #1 Paget’s 50%
• #2 Hodgkin’s 30%
• #3 Metastasis 20%
Breast Carcinoma
• The most common source of
bone mets in women
• Spine #1 site
• 65% lytic, 25% mixed,
10% blastic
Prostate [Figure 4-30-6]

• The most common source of
metastases in men
• More than 1/3 of patients
• 75% blastic, 15% mixed, 10%
lytic
• Humoral factor stimulates
blasts
59 year old woman with multiple ivory 76 year old man with florid
vertebrae secondary to breast cancer periosteal reaction around distal
metastases fibula metastasis
Metastases, Myeloma, Lymphoma 962 Musculoskeletal Radiology

Lung Cancer
• 15% of patients have mets to bone
• 80% lytic, 15% mixed, 5% blastic
• Small cell – 20% blastic
Renal Cell Carcinoma [Figure 4-30-7]

• 25%–30% of patients have mets to bone
• 90% Lytic
Figure 4-30-7
71 year old man with blowout type metastasis from

renal cell cancer. Note active hyperemia on
angiogram
Figure 4-30-8
Thyroid Cancer
• 8% of patients have metastases
• Lytic
Neuroblastoma [Figure 4-30-8]

• 1st choice any patient under 10 years old
• Can mimic primary malignancy
• Usually multiple, often symmetric
• Histology confused with Ewing’s
• Periosteal rx – aggressive
Workup of Patient with Metastases

• 1 – History / physical
• 2 – Lab studies
• Direct workup based on 1, 2
• Primary not found in up to 60% patients
3 year old boy with

neuroblastoma. Metastasis in
proximal radius shows
permeative appearance similar
to Ewing’s sarcoma

Solitary Focus Bone Scan [Figure 4-30-9] Figure 4-30-9
• Seen in 2%–15% of cancer patients
• % due to metastatic disease varies by site of involvement
• 10% solitary rib lesions
• 50%–60% in other locations (spine)
• 10% malignant even if there is “DJD” in the area
Rib – J Nucl Med 1985;26:1140–1143
All sites – Radiology 1976;121:663–667
Malignant Round Cell Tumors of Bone

• Myeloma
• Lymphoma
• Neuroblastoma
• Rhabdomyosarcoma
• PNET
Myeloma Types
• MGUS – monoclonal gammopathy of undetermined significance
(1% of all SPEPs)
• Asymptomatic myeloma (no bone lesions)
• Symptomatic multiple myeloma
(classic, generalized, osteosclerotic [POEMS], multiple myeloma
with osteosclerosis, leukemic)
• Non-Secretory myeloma – 3% of all cases
• Solitary plasmacytoma in bone – 3%-5% of cases
• Extramedullary plasmacytoma – 5%
65 year old man with solitary rib
Myeloma: Pathologic Features abnormality on whole body bone
• Plasma cells proliferate in erythropoietic areas scan, proven to be esophageal
• Grossly – dark red, tan; soft cancer metastasis
• Histology – sheets of malignant plasma cells obliterate the
marrow
• Special studies – markers for light chains
• Amyloid – 10%
• Osteoclast stimulating factor
Osteoclast-Stimulating Factor
• A cytokine (lymphotoxin alpha)
• Similar to O-AF
• Produced by myeloma cells, T cells
• Interferon is a cytokine antagonist
• Bisphosphonates used to counter osteoclastic resorption
Cancer 1997; 80:1557-63
Classic Multiple Myeloma: Clinical Features

• Signs and Symptoms: pain, bleeding diathesis, infection, renal insufficiency
• Lab findings: monoclonal spikes (IgG, IgA), B-J proteinuria, anemia,
hypercalcemia, elevated alkaline phosphatase
• Imaging W/U: X-rays, CT/MRI, Nucs, PET, PET/CT
• MM has highly malignant course
B-J=Bence-Jones MM=multiple myeloma

Durie/Salmon PLUS Staging* [Figure 4-30-10]
Radiologist’s role is to help determine the true tumor burden throughout the
skeleton
• Stage IA: normal skeletal survey or single lesion
• Stage IB: < 5 focal lesions or mild diffuse disease
• Stage IIA/B: 5-20 focal lesions or moderately diffuse Figure 4-30-10
• Stage IIIA/B: >20 focal lesions or severe diffuse disease
• Subclasses A&B (A = nl renal function, B = abnl)
• Stage is generally predictive of survival
➢ IA median survival = 60 months
➢ IIIB median survival = 15 months
*Durie et al. Myeloma management guidelines: a
consensus report.
The Hematology Journal 2003; 4: 379-398
Myeloma: Imaging Features [Figure 4-30-11]

• 80% of new MM patients – abnormal skeletal survey
• Multiple myeloma - punched out lesions
➢ endosteal scalloping
• Solitary plasmacytoma: bubbly, any margin +/- soft
tissue mass
• Generalized form- just osteopenia From left to right: mild, moderate, and severe.
• Sclerosing - < 3%, assoc with POEMS syndrome The three types of myelomatous spinal
• Spine MR – 3 patterns; mild, moderate, severe involvement for the Durie/Salmon PLUS staging
system (T1-weighted images)
Sclerotic Myeloma:
Multiple myeloma with sclerosis or POEMS syndrome [Figure 4-30-12]
Figure 4-30-11
• Polyneuropathy
• Organomegaly
• Endocrinopathy
• Monoclonal gammopathy
• Skin changes
NEJM 1992;327:1919–1923
Figure 4-30-12
56 year old man with myeloma.

Humeral radiograph shows typical endosteal
scalloping and macrosection shows plasma cells
filling the marrow space with osteoclasts along the
endosteal surface
Blastic or sclerotic lesions that are

usually painless are typical in the
POEMS syndrome

Myeloma: Differential Diagnosis [Figure 4-30-13] Figure 4-30-13 Figure 4-30-14
• B cell malignancy
➢ ALL, NHL, CLL, Waldenstrom’s
Plasmacytoma
Differential Diagnosis [Figure 4-30-14]
• Metastasis: thyroid, renal
• Primary: Fibrosarcoma, MFH
Primary Lymphocytic Lymphoma of

Bone [Figure 4-30-15]
Figure 4-30-15
56 year old man with myeloma,.
skull radiograph shows typical
“punched out” lytic lesions
71 year old woman

with aggressive
looking solitary
plasmacytoma
mimicking blowout
type metastasis
70 year old man with PLB, skull radiograph

shows multiple lytic lesions, some with central
sequestra (arrow)
Primary Lymphoma: Path Features

• Gross – pinkish-grey, “fish-flesh”
• Histology – similar to nodal lymphoma round cells of various sizes (Ewing’s
monotonous)
• Reticulin stain – meshwork of fibers around each cell
Figure 4-30-16
Primary Lymphoma: Clinical Features
• Non-Hodgkin’s (94%), Hodgkin’s (6%)
• Rare, @ 3% malignant tumors
• Any age, but rare under 10 years
• Stage like soft tissue lymphoma
• Solitary and multifocal (skull, femur, tibia)
• Osteoclast-stimulating factor
PLB – 237 AFIP CASES

• 151 M, 86 F, ratio 1.8:1
• Average age 42 years (range: 2 to 88 years)
• Long bones n=162 (71%)
• Flat bones n=78 (22%)
• Other sites (including spine, small bones) n=17
Mulligan, et al. AJR 1999; 173: 1691-1697
Typical Features – PLB [Figure 4-30-16]

• Location: Metadiaphyseal n=120 (54%)
• Pattern: Permeative n=130 (55%)
• Cortical involvement: n=148 (62%) 57 year old woman with
• Periosteal reaction: interrupted or solid single layer n=57 (65.5%) PLB, tibia lesion shows
• Soft tissue mass: n=113 (48%) all of the most common
Mulligan, et al. AJR 1999; 173: 1691-1697 radiographic features

Variations – PLB [Figure 4-30-17] Figure 4-30-17
• Locations
➢ Epiphysis n=11 (5%)
➢ Diaphysis n=45 (19%)
➢ Intracortical n=16 (7%)
• Patterns
➢ Normal x-ray n=12 (5%)
➢ Geographic n=26 (11%)
➢ “Blow Out” n=2 (< 1%)
➢ Blastic n=4 (2%)
Radionuclide, CT and MRI Findings

• Radionuclides n=56, markedly increased uptake
n=36 (64%)
• CT n=45, MRI n=20
• “Cortical holes” by CT or MR
➢ Large n=20 (31%), small n=45 (69%)
Markedly abnormal bone scan and MR exam in 57
year old man with thigh pain and normal xrays.
➢ Seen by CT n=36 (80%)
This type of extensive marrow replacement pattern
➢ Seen by MR n=20 (100%)
with normal xrays is highly suggestive of “round
blue cell tumors” like PLB and Ewing’s sarcoma
Variations – PLB [Figure 4-30-18]
➢ Multiple layers n=26 (10.2%) Figure 4-30-18
➢ Sunburst n=4 (1.6%)
• Pathologic fracture n=52 (22%)
• Sequestra n=37 (15.6%)
• Crossing joint n=12 (5%)
AJR 1999; 173: 1691-1697
Primary Lymphoma: Differential Diagnosis

• Metastatic lymphoma
• Neuroblastoma / PNET
• Osteomyelitis
• Eosinophilic granuloma
Summary – PLB
• Usually has an aggressive appearance
➢ CT or MRI showing large soft tissue mass without large
cortical holes is typical
• Wide range of appearances
➢ Normal x-rays Large lytic lesions with soft tissue
➢ Geographic lesions mass and sequestra should put PLB
➢ “Blow out” lesions high up in the differential diagnosis
➢ Blastic lesions
References
1. Durie et al. Myeloma management guidelines: a consensus report. The Hematology Journal 2003; 4: 379-398
2. Mirels H. Metastatic disease in long bones. Clin Orthop Relat Res 1989;249:256-264
3. Mulligan M et al. Skeletal Metastatic Disease. In: Pope et al, Imaging of the Musculoskeletal System.
Philadelphia: Elsevier, 2006
4. Mulligan M. Imaging techniques used in the diagnosis, staging, and follow-up of patients with myeloma. Acta
Radiologica 2005;46:716-724
5. Mulligan M, McRae G, Murphey M. Imaging features of primary lymphoma of bone. AJR 1999; 173: 1691-1697
6. Roodman GD. Mechanisms of bone metastasis. N Engl J Med 2004;350:1655-1664
7. Weber K et al. An approach to the management of the patient with metastatic bone disease. Instr Course Lect
2004;53:663-676

Imaging of Hematologic Disease Figure 4-31-1
Thomas Lee Pope, Jr, MD, FACR
Objectives
• To identify some of the common hematologic disorders
• To describe the clinical and epidemiological aspects of these
entities
• To demonstrate the most significant MSK imaging findings
Hematologic Disease
• Hereditary anemias
➢ Sickle cell anemia
➢ Thalassemia
➢ Rare anemias: Fanconi’s, thrombocytopenia with absent
radii syndrome (TAR)
• Coagulation disorders
➢ Hemophilia
➢ Myelofibrosis
Characteristics of the Hereditary Anemias

• Aberrations and/or abnormalities of RBC shape Hand Foot Syndrome: Notice the
• Molecularly distinctive diffuse involvement with regions of
• Autosomal dominant osteolysis and periosteal reaction
• Electrophoresis
• The Five “In’s”
➢ In sufficient ossification
➢ In farction
Figure 4-31-2
➢ In fection
➢ In failure (anemia)
➢ In volution (spleen)
Sickle Cell Disease

• One of the most common inherited blood disorders (> 100,000
born with the disease worldwide per year)
• One of the most prevalent genetic disorders in the US (>
80,000 African Americans)
• Hemoglobin S gene (carrier state)
➢ Autosomal dominant
➢ Carried by 8% of African Americans or ~ 2 million US
Blacks
• Hemoglobin SS disease (Sickle cell anemia)
➢ Autosomal recessive
➢ 1 birth in 400 in African Americans
➢ Occurs in 0.3%-1.3% of NA Blacks or ~ 50, 000 in the US
Economic Impact of SC Disease

• 75,000 hospitalizations yearly
• Average of $6, 300 per hospitalization
• $475 million in health care costs alone
• Does not include lost wages, productivity, etc…
• Likely > $1 billion yearly cost
Acute (upper image) and chronic (lower image)

Salmonella osteomyelitis
Imaging of Hematologic Disease 968 Musculoskeletal Radiology

Characteristics of sickled and normal RBC’s Figure 4-31-3
• NORMAL
➢ Disc-shaped
➢ Soft and compressible
➢ Easily flows through vessels
➢ Life span of > 120 days
• SICKLE
➢ Sickle-shaped
➢ Hard (tough and not malleable)
➢ Sticks in blood vessels
➢ Life span of < 20 days
Major Pathology-Vascular occlusion [Figure

4-31-1]
• Hand-Foot syndrome
➢ Dactylitis
• Infarction (any site)
• Infection
• Marrow hyperplasia
Hand foot syndrome (Dactylitis)

• Up to 50% of “sickle cell anemia” children
• 2 months to 6 years
• Pain, low grade fever, diffuse non-pitting edema of
the extremities MR features of acute Salmonella osteomyelitis
• Vaso-occlusion with osteonecrosis Courtesy of Dr. Hilary Umans New York, NY
• Infection is major DDx
• Distinction: Clinically Figure 4-31-4

• 50X less common than infarction
• Salmonella much more frequent pathogen in SCA
patients
• Infecting organisms:
➢ Salmonella – 70%
➢ Staph aureus – 10%
➢ Shigella sonnei, E coli, Arizona hinshawii and
Serratia
• Proposed mechanisms:
➢ Vascular insufficiency
➢ Decreased phagocytosis-low O Classic features of avascular necrosis with areas
➢ Decreased splenic function
of osteolysis and osteosclerosis with preservation
Multiple hospitalizations
of articular space
Figure 4-31-5
Figure 4-31-6
Radiographic and anatomic gross specimen

correlation of the “rim” sign
MR imaging of intramedullary infarcts
Musculoskeletal Radiology 969 Imaging of Hematologic Disease

Differential Diagnosis: Figure 4-31-7
“Bone within Bone” Appearance
• S – Sickle cell disease
• T – Thoratrast
• O – Osteopetrosis
• P – Paget disease
• Heavy metal
• Hypervitaminosis D
http://chorus.mcw.edu
“H-Shaped” Vertebral Bodies [Figure 4-31-8]

• Another manifestation of ischemia and infarction
• >10 years of age
• Incidence:
➢ 43% of SS
➢ 36% of Sickle/Thalassemia
➢ 25% of SC
“Bone within a bone” apprearance in sickle cell anemia
Thalassemia
• 1925 - Cooley and Lee
• Synonyms: Cooley’s anemia, mediterranean anemia, leptocytosis
Figure 4-31-8
• Impaired alpha or beta chain Hgb production
• Homozygous beta thalassemia (800-1000 US persons - NE
corridor between Boston and NY)
• Heterozygous trait (2.5% of Italian Americans, 7%-10% of Greek
Americans)
Types of Thalassemia
• Alpha
➢ Least severe:
❖ Silent carrier = loss of 1 alpha globulin gene - often
incidental finding
❖ Most severe:
❖ Hydops fetalis = loss of 4 alpha globulin genes - die in
utero
• Beta:
➢ Spectrum
❖ Minor = slight anemia
❖ Major = life-threatening anemia requiring transfusions
❖ Risk of Fe++ overload
“H” shaped vertebral bodies of sickle

cell disease
Figure 4-31-10
Figure 4-31-9
Classic “Hair on end” appearance of Marrow expansion in Thalassemia with widening

Thalassemia of the medullary canal and thinning of the cortices

Imaging Features of Thalassemia Figure 4-31-11
[Figures 4-31-9 to 4-31-12]
• Diffuse marrow expansion
➢ Skull - (“hair on end”) appearance
➢ Face - (“rodent-like facies”)
➢ Long bones – “Erlenmeyer flask” deformity
• Extramedullary hematopoeisis
• Rare minor features: Growth disturbances, fractures, crystal
deposition
Gaucher’s Disease
• Ashkenazic Jews of Eastern European descent
• Defect of beta glucosidase
• Accumulation of glycosyl ceramide in the RE cells of BM,
spleen, and liver
• Hepatosplenomegaly, yellow skin, scleral pigmentation, acid and
alkaline phosphatase elevation
Imaging features
• AVN of the hip and femoral head
• Osteoporosis
• Marrow expansion with cortical thinning
• Erlenmeyer flask deformity
• Lytic lesions and sometimes periostitis Erlenmeyer flask deformity
Differential Diagnosis: “Erlenmeyer Flask” Deformity Figure 4-31-12

• Osteoporosis
• Chronic anemia (Sickle cell disease)
• Gaucher disease
• Niemann- Pick (enzyme deficiency)
• Metaphyseal dysplasia (Pyle’s disease)
Extramedullary Hematopoiesis
[Figures 4-31-13 and 4-31-14]
• Blood production in fetal regions
➢ Liver, spleen, adrenal, thymus, heart, lung, “Erlenmyer flask” deformity in Gaucher’s disease.
nodes, renal pelvis, GI tract, dura mater (almost “Crumpled tissue paper” cytoplasm on histology
anywhere !)
• Major causes:
Figure 4-31-13
➢ Hematologic disease (SS and
thalassemia)
➢ Myelofibrosis
➢ Leukemia
➢ Hodgkin’s
➢ Hyperparathyroidism
➢ Rickets
➢ Carcinomatosis
Extramedullary hematopoesis with hepatosplenomegaly and

Figure 4-31-14 posterior mediastinal masses
Extramedullary hematopoesis with MR correlation

Fanconi’s Anemia [Figure 4-31-15] Figure 4-31-15
• Onset after first decade
• Severe anemia, pancytopenia, brown
pigmentation
• Death 2–3 years after appearance
• Anomalies:
➢ Short stature, microcephaly, delayed
ossification
➢ Hip dysplasia, renal anomalies
➢ Radius absent (50%)
➢ Thumb always absent
Thrombocytopenia with Absent Radii Fanconi’s anemia:

(TAR) [Figure 4-31-16]
• Congenital hypomegakaryocytic thrombocytopenia Figure 4-31-16
• Apparent at birth or shortly thereafter
• Anomalies:
➢ Bilateral radial aplasia always present
➢ Thumb is present (differentiation from Fanconi’s)
• If kids survive for the first two years, the anemia often
spontaneously resolves
Hemophilia
• Oldest known bleeding disorder
• First noted in offspring of Queen Victoria of England
• Mutation in Queen Elizabeth’s X chromosomes
• Group of X-linked recessive disorders
• Gene carried by women and expressed in men
• All races affected TAR with absent radii syndrome: Note that the
• 20, 000 hemophiliacs in US thumb is present
• 400 new cases/year Figure 4-31-17
• Severity – related to lack of clotting factor
• 70% have < 1% of normal clotting factor
Major Types of Hemophilia

• Hemophilia A
➢ 85% of all cases
➢ Factor VIII (antihemophiliac factor-AHF)
deficiency
➢ 70% have < 1% of normal amounts of AHF
• Hemophilia B (Christmas disease)
➢ 15% of all cases Acute hemorrhagic effusions in two patients with
➢ Factor IX (Plasma thromboplastin component- hemophilia
PTC) deficiency
Figure 4-31-18
Joint Disease
• Acute hemarthroses: tense, swollen, red and tender
joints, pain, LOM, fever, increase in WBC
• Stages of joint disease:
➢ Stage I: STS
➢ Stage II: Osteoporosis
➢ Stage III: Osseous lesions
➢ Stage IV: Cartilage destruction
➢ Stage V: Joint disorganization
Knee [Figures 4-31-17 and 4-31-18] Marked articular space narrowing and cartilage
• Dense effusions destruction with massive subchondral cyst
• Juxtaarticular osteoporosis formation
• Subchondral irregularity
• Epiphyseal overgrowth
• Squaring of inferior pole of patella (20%-30%)

Elbow [Figure 4-31-19] Figure 4-31-19
Low SI synovial proliferation [Figure 4-31-20]
Differential Diagnosis of Hemophilia

• Juvenile chronic arthritis
➢ Single joint tough to differentiate
➢ Distribution may be helpful:
❖ JCA – hands/feet/big joints
❖ Hemophilia - knee, ankle, elbow
• PVNS, infection, especially TB (monoarticular)
• NM diseases: CP,
muscular dystrophy,
polio Figure 4-31-20
Hemophiliac involvement of two elbows in two

different patients
Figure 4-31-21
Low signal intensity of synovial proliferation in

Medial talar tilt quite characteristic of hemophilia
hemophilia.
Kerr R: Imaging of MSK complications of hemophilia. Sem in MSK
Figure 4-31-22
Other Less Common Imaging Findings
• Ectopic ST ossification (periarticular – pelvis, thigh,
paraspinal, knee)
• Hemophiliac pseudotumor
• Osteonecrosis (epiphyseal fragmentation)
• Fractures
• Chondrocalcinosis
Pseudotumor [Figures 4-31-22 to 4-31-26]

• 2% of patients
• Femur, pelvis, tibia, hands and feet
• Locations:
➢ Soft tissue, intraosseous, and subperiosteal Soft tissue pseudotumor of hemophilia
Park JS, Ryu KN: AJR 2004;
• ST
➢ Hard palpable subcutaneous masses Figure 4-31-23
• Intraosseous and subperiosteal
➢ Lytic, expansile, destructive, aggressive process
Subperiosteal pseudotumor of hemophilia

Park JS, Ryu KN: AJR 2004; 183:55-61

Intraosseous pseudotumors of hemophilia

Park JS, Ryu KN: AJR 2004; 183:55-61
Differential Diagnosis for Hemophiliac

Pseudotumor
• Malignancy
➢ Osteosarcoma Intraosseous pseudotumor of the ilium in a
➢ Chondrosarcoma hemophiliac
➢ Ewing tumor Kerr R: Imaging of MSK complications of hemophilia in
• Metastases MSK Radiology 7:2, 2003
• Infection
Figure 4-31-26
Myelofibrosis
• Affects progenitor (stem) cells of the bone marrow
• Primary (idiopathic) and secondary forms
• Major manifestations
➢ Fibrotic or sclerotic bone marrow
➢ Extramedullary hematopoiesis
• Other designations:
➢ Idiopathic myelofibrosis
➢ Myeloid metaplasia
➢ Agnogenic myeloid metaplasia
Primary (Idiopathic) Myelofibrosis

• Bone marrow replaced by fibrosis
• Unknown cause
• > 50 year old, incidence - 2/100,000
• Findings:
➢ BM fibrosis with hepatosplenomegaly Intraosseous “blowout” lesions of hemophilia
➢ Anemia
➢ Increased nucleated RBC’s
➢ Leukocytosis or leukopenia Figure 4-31-27
➢ Abnormal WBC’s
Primary (Idiopathic) Myelofibrosis

[Figure 4-31-27]
• Unknown cause
• > 50 year old, incidence - 2/100,000
• Findings:
➢ BM fibrosis with hepatosplenomegaly
➢ Anemia
➢ Increased nucleated RBC’s
➢ Leukocytosis or leukopenia
➢ Abnormal WBC’s
Diffusely dense bones characteristic of
• Diagnosis - BM aspiration
myelofibrosis
• Rx: Transfusions, chemo, Interferon, splenectomy,
radiation
• 50%-80% of patients have elevated serum or urinary uric acid levels
• Secondary gout occurs in 5-20% of patients

Secondary Myelofibrosis
• Malignant disease
➢ Leukemias, Polycythemia vera, MM, Hodgkin’s disease, NHL, cancer
• Chronic infection
➢ Tuberculosis, osteomyelitis
• Toxins
➢ X- or gamma radiation, benzene exposure
Imaging Findings [Figure 4-31-15]

• Generalized osteosclerosis (most common)
• Cortical thickening
• Osteopenia
• Rarely periostitis
Review
• Sickle Cell
➢ Vaso-occlusion
❖ “Hand-Foot” syndrome
❖ AVN and medullary bone infarcts
❖ “H-shaped” (“Lincoln log”) vertebral bodies
❖ “Bone within a bone” appearance
❖ Salmonella infection
• Thalassemia
➢ “Hair on end”
➢ “Pseudohemangiomatous” appearance
➢ “Erlenmeyer flask” deformity (differential-Gaucher’s)
➢ “Rodent” facies
• Hemophilia
➢ Wide intercondylar notch
➢ Erosions
➢ Medial slope of distal tibia at ankle
➢ “Pseudotumor”
• Myelofibrosis
➢ Primary and secondary forms
➢ Diffusely dense bones
➢ Hepatosplenomegaly
➢ BM bx to make dx
References
1. "What is Sickle Cell Disease". Sickle Cell Information Center. December 16, 2003. Copyright © 1997. The
Georgia Comprehensive Sickle Cell Center at Grady Health System, The Sickle Cell Foundation of Georgia, Inc.,
Emory University School of Medicine, Department of Pediatrics, Morehouse School of Medicine.
http://www.scinfo.org/sicklept.htm
2. Funaki B. "Sickle cell anemia: Bone manifestations", "Bone within a bone". Chorus: Collaborative Hypertext of
Radiology. (Kahn CE ed). July 2004. Medical College of Wisconsin. February 1995.
http://chorus.rad.mcw.edu/doc/01060.html
3. Kahn CE. " Erlenmeyer flask deformity". Chorus: Collaborative Hypertext of Radiology. (Kahn CE ed). May
2004. Medical College of Wisconsin.
< http://chorus.rad.mcw.edu/doc/00648.html>
4. Kerr R. Imaging of musculoskeletal complications of hemophilia. Semin Musculoskelet Radiol 2003; 7:127-136.
5. Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. Radiographics 2001; 21:971-994.
6. Park JS, Ryu KN. Hemophilic pseudotumor involving the musculoskeletal system: spectrum of radiologic findings.
7. Wong AL, Sakamoto KM, Johnson EE. Differentiating osteomyelitis from bone infarction in sickle cell disease.
Pediatr Emerg Care 2001; 17:60-63; quiz 64.

Generalized Musculoskeletal Disorders
Figure 4-32-1
Learning Objectives
• To describe a group of entities not well covered in
the rest of the course
• To outline the imaging features of these diseases
• To introduce the listener to these entities so that
he/she can study about them further
Outline of Diseases
• Osteoporosis (and its sequelae)
• Osteogenesis imperfecta
• Collagen vascular-like diseases
➢ SLE
➢ Scleroderma
➢ Polymyositis/dermatomyositis
Disuse osteoporosis in a 76 yo with left sided CVA
Terminology
• Osteopenia – “paucity of bone”
• Osteoporosis
➢ Decreased bone mineral density
➢ Normal in quality
➢ Decreased in quantity
• 30%-50% of cancellous bone must be gone to recognize
Types of Osteopenia
• Localized
• Regional or segmental
• Generalized or diffuse
Localized Osteopenia/Osteoporosis
• Focal areas of bone loss
➢ Infection
➢ Arthritides
Figure 4-32-2
Regional
Osteopenia/Osteoporosis
• Segmental area of
decreased BMD
➢ Disuse (immobilization)
➢ Chronic regional pain
syndrome (CRPS)
(RSD (Reflex
sympathetic dystrophy)
[Figure 4-32-2]
Chronic regional pain
➢ Transient osteoporosis syndrome (Reflex
(bone marrow edema) sympathetic dystrophy)
➢ Regional migratory
osteoporosis Note the increased
radionuclide accumulation
on the early and late views
of the bone scan
Generalized Musculoskeletal Disorders 976 Musculoskeletal Radiology

Disuse/Immobilization Osteoporosis [Figure 4-32-1] Figure 4-32-3
• Major cause
➢ Immobilization for traumatic injury
➢ Motor paralysis
➢ Inflammatory lesions of bones and
joints
• Changes take 7-10 days (maximal at 2-
3 months)
• Patterns: uniform, spotty, bands, cortical
lamination or scalloping
• May appear very aggressive!!!
Chronic Regional Pain Syndrome-

Reflex Sympathetic Dystrophy
[Figure 4-32-1] Transient regional osteoporosis (bone marrow edema) of the hip:
• Elderly
Note the osteopenia of the right hip and the proximal diffuse
• Trivial trauma
increased radionuclide accumulation on the bone scan
• Pain, swelling, temperature changes
Note the marrow replacement in the proximal right femur on T1
and the increased signal intensity (edema) on the T2 weighted
Transient Regional Osteoporosis images
[Figure 4-32-3]
• General term
• Conditions sharing features of:
➢ Rapidly developing, self-limited, reversible osteoporosis
➢ Absence of clear cut inciting events
• Major types:
➢ Transient osteoporosis (bone marrow edema) of the hip
➢ Regional migratory osteoporosis Figure 4-32-4
Regional migratory osteoporosis
• Rapidly developing, self-limiting and reversible
• Knee, ankle, foot and hip
• Joint nearest involved likely to be next involved
Transient Regional Osteoporosis

(bone marrow edema) of the hip
• 1st -women in the third trimester of pregnancy
• LE > UE Pathology of osteoporosis (rib specimens)
• Osteoporosis and BM edema
• Differential diagnosis: AVN, infection
• Generally spontaneously resolves in 9-12 months
Generalized Osteopenia/Osteoporosis
• Diffuse decreased BMD
➢ Senile osteoporosis
➢ Medications (Steroids, heparin)
➢ Systemic diseases (Deficiency states)
❖ Scurvy
❖ Malnutrition
❖ Calcium deficiency
Senile Osteoporosis [Figure 4-32-4]

• Pommer-1985 — “increased porosity”
• Most commonly encountered metabolic disease
• Reduction in bone “quantity,” normal in “quality”
F>M, 4:1, (equal incidence) > 80 yo
• Most pain source: compression fx’s and kyphosis
• PE – kyphosis, shortened stature, and spinal rigidity
Musculoskeletal Radiology 977 Generalized Musculoskeletal Disorders

Epidemiological data Figure 4-32-5
• Surgeon General Report, October, 2004
➢ Half population in US > 50 yo with low bone mass and risk for
fracture
➢ 1.5 million/year osteoporosis-related fx
➢ 34 million with hip osteopenia
• Caucasian females > 50 yo – 40% chance of fx in lifetime (13% for
males)
• Hip fracture:
➢ Risk of mortality within 3 months is 4X greater than normal
➢ 20% of fx victims die or wind up in nursing home within year
after event
• Annual cost of treating osteoporosis: $18 BILLION
Pathology of osteoporosis [Figure 4-32-4]
Osteoporosis “life cycle” - Fracture index [Figure 4-32-5]
Osteoporosis Measurement [Figure 4-32-6]

• Dual energy X-ray absorptiometry (DEXA)
• Conventional X-ray (radiogrammetry)
• Single photon absorptiometry (SPA)
• Dual photon absorptiometry (DPA)
• Neutron activation analysis
• Quantitative CT (QCT) Figure 4-32-6
Lumbar Hip Distal Radius
Dual Energy X-ray Absorptiometry (DEXA)

• Relative tissue attenuation from dual energy X-ray source
• Easy to perform
• Most reproducible technique with the least coefficient of variation (COV)
• Detects changes of 1–3%
• Expressed in gm/cm2
• Primary indication: Estrogen deficiency to determine therapy
BMD Terms
• BMD measured in gm/cm2
• T-score: Patient’s BMD compared to normative data (Normal = 25 yo women)
• Z-score: Patient’s BMD compared to her aged-matched controls
• World health organization uses T scores to classify a patient’s bone mineral
status

WHO Classification of BMD
• STANDARD: Mean BMD of 25 yo women
• NORMAL: BMD from the mean to 1 standard deviation below the mean (mean
to -1SD)
• OSTEOPENIA: T-score from 1 to 2.5 SD below mean (-1SD and -2.5 SD))
• OSTEOPOROSIS: T-score below 2.5 SD below mean (> -2.5 SD)
• Osteoporosis also established by presence of a non-traumatic vertebral
compression fracture
Figure 4-32-7
Important implications
• Fracture risk doubles with each drop of 0.1 below the
mean of the T-score
• Risk of fracture also doubles for each decade the
patient is > 50 yo
• Goal is to eventually be able to calculate an
“absolute fracture risk”- more holistic measurement
method
• Patients respond better to “you have a 70% of Photomicrograph of senile vertebral osteoporosis
developing a fracture” than they do to “your T-score
is whatever”
Senile Osteoporosis: Imaging Features Figure 4-32-8

• Increased radiolucency on X-ray (“osteopenia”)
• Cortical thinning
• Altered trabecular patterns
Senile Osteoporosis: Complications

• Acute fractures
➢ Spine (L>T>C)
➢ Distal radius (Colles)
➢ Proximal femur
➢ Humerus (neck)
➢ Ankle (malleoli)
• Insufficiency fractures Lateral radiographs of three patients with “codfish”
➢ Covered in “osseous stress injury” talk vertebral bodies
Spinal effects of osteoporosis

• Decreased bone density Figure 4-32-9
➢ Accentuation of primary trabeculae
• Cortical thinning
• Changes in vertebral shape
➢ “Biconcave” – CODFISH (“fish”) shape
➢ Endplate deformities (Schmorl’s nodes, cortical
irregularities)
➢ Wedged vertebrae
➢ Vertebrae plana (“pancake”/silver dollar)
Senile vertebral osteoporosis [Figure 4-32-7]
“Codfish” vertebral bodies [Figure 4-32-8]
Femoral neck fracture locations [Figure 4-32-9]
A = subcapital
B = neck
C = basicervical
D = intertrochanteric
E = subtrochanteric

Garden classification [Figure 4-32-10]
• < II = Percutaneous pinning
• > II = THA (AVN risk) Figure 4-32-10
Bohndorf, Imhoff, Pope: Musculoskeletal Imaging: A Multimodality Approach

George Thieme Verlag, 2001
Figure 4-32-11
Garden Type I [Figure 4-32-11]
Intertrochanteric Fractures
• Extracapsular
• Periosteum present
• Low incidence of nonunion or AVN (~1%)
• Distinction from basicervical often difficult (no clinical
concern)
• Most comminuted, 15% severely
• GT/LT may be displaced by gluteus or iliopsoas
• May have other non-suspected injuries in pelvis
Garden I type of femoral neck fracture treated with
Osteogenesis Imperfecta (OI) [Figure 4-32-12] Knowles’ pins
• Skeletal, skin, sclera and dentin abnormality
• 1/30K affected Figure 4-32-12
• 1/50K severely
• 20-50K in US
• 85% AD
• Major types:
➢ Congenita
➢ Tarda
OI vs Child Abuse
• Metaphyseal corner fx’s uncommon in OI
• Sternal, rib, scapular, skull and bucket handle fx’s Congenital type of Osteogenesis Imperfecta
common
• Fx’s continue to occur in protective custody
• Other non-MSK findings not present: Figure 4-32-13
➢ Retinal hemorrhage
➢ Visceral intramural hematomas
➢ Intracranial bleeding
➢ Pancreatitis
➢ Splenic trauma
OI-congenital type [Figure 4-32-12]
OI-tarda [Figures 4-32-13 and 4-32-14]
Tarda form of Osteogenesis Imperfecta: Note

healing fractures with exuberrant callus formation

Figure 4-32-14
Figure 4-32-15
Note multiple fractures, intramedullary rods and

dynamic hip screw and Harrington rods for
Tarda form of Osteogenesis Imperfecta: Note scoliosis
exuberrant callus formation and intramedullary rod
placement for fractures
Neurofibromatosis (NF)
• Described first by Tiresius (1773) and Smith (1849)
• Named for von Recklinghausen - noticed association of neural and cutaneous
elements in 1882!!
• Defects of all three cell layers = phakomatosis
• 1/3000 births
• Estimated ~100,000 in US
• One of humanity’s most common genetic disorders
• Mutation rate is 1/10,000 gametes/generation
• Greater than that for ALL OTHER COMMON GENETIC DISORDERS
• AD with variable gene expression (FH in 60%)
• Equal incidence in male and female and Caucasian and non-Caucasian
Neurofibromatosis (NF)
• Two distinct clinical forms:
➢ NF-1 (vonRecklinghausen’s) - Café-au-lait spots, neurofibromas, skeletal
deformities
➢ NF-2 - Acoustic neuromas
• Can lead to disfigurement, blindness, deafness, dermal/brain/spinal tumors,
loss of limbs, malignancies, learning disabilities
• WAS NOT “Elephant Man’s Disease”…John Merrick had Proteus Syndrome
(cell growth disturbance with hemihypertrophy and macrodactyly)
Imaging features [Figures 4-32-16 to 4-32-20 overleaf]

• Spinal changes:
➢ Dural ectasia
❖ Vertebral scalloping
❖ Foraminal enlargement
❖ Pedicle erosion
➢ Mesodermal dysplastic changes:
❖ Scoliosis
- Typical
- Dysplastic, sharply angulated, < 6 segments of lower T spine
(pathognomonic of NF)
❖ Pencilling and spindling of the transverse processes
• Long bones (due to neurofibromas or mesodermal dysplastic changes)
➢ Pencilling
➢ Bone erosions
➢ Pseudarthrosis (characteristically of the tibia)
➢ Associated with nonossifying fibromas
• Ribs
➢ Scalloped and irregular (“twisted ribbons”)
➢ Erosions of inferior rib surfaces

Neurofibromatosis: Note accentuated scoliosis
Neurofibromatosis: Note kyphosis, vertebral

anomalies and widened neural foramen
Figure 4-32-19
Figure 4-32-18
Neurofibromatosis: Note mesodermal dysplastic

changes in the pelvis of two different individuals
Neurofibromatosis: Note posterior vertebral Figure 4-32-20

scalloping and dural ectasia
Neurofibromatosis with the

characteristic “pseudoarthrosis” of the
tibia

Meningoceles [Figures 4-32-21 and 4-32-22] Figure 4-32-21
• About 2/3 of patients with NF
• 70%-80% of all meningoceles in NF patients
• Most common presentation: Asymptomatic post
mediastinal mass
• Protrusion of dura and arachnoid through IV foramen
and posterior rib cage into the extrapleural thoracic
cavity - nonca++
• Presence of ca++ excludes meningocele
Systemic Lupus Erythematosis (SLE)

• “Lupus” -Latin for wolf – malar erythema looked like
the bite of a wolf Intrathoracic meningocele: Note the posterior
• Generalized connective tissue disorder mediastinal mass on the right
• F>M, second to fourth decade, rare over the age of
Figure 4-32-22
45
• Higher incidence in AA and Hispanics
• Fever, anorexia, weight loss, polyarthralgias, skin
rash
• Chronic disease with acute episodes
• Variable prognosis
Three Categories of SLE

• Discoid-skin rash only, 20% of patients with SLE
• Systemic-chronic, inflammatory, multisystem
disorder of the immune system
• Drug-induced-Chlorpromazine, hydralazine,
isoniazid, methyldopa, procainamide
CT and MR imaging of intrathoracic meningocele
Musculoskeletal Imaging Findings
[Figure 4-32-23] Figure 4-32-23
• Major: deforming nonerosive arthropathy
• Minor:
➢ Osteonecrosis
➢ Insufficiency fractures
➢ ST calcification
➢ Acroosteolysis
➢ Tendon weakening and rupture
➢ Subchondral cysts
➢ Myositis
➢ Polyarthritis
➢ Osteomyelitis and septic arthritis
Symmetric Polyarthritis
• Articular symptoms and signs common-75%-90% of
patients Non-deforming nonerosive arthropathy of SLE
• Frequently bilateral and symmetric (differential diagnosis is post-Streptococcal
• 5%-40% with disease (Jacoud’s) arthropathy
• Hands > knees > wrists > shoulders
• ST swelling, periarticular osteopenia
• Reversible and little functional effect
• !!Cartilage and osseous destruction rare without underlying osteonecrosis!!
Scleroderma
• Unknown cause
• F:M (4:1)
• 3rd to 5th decade
• < 20 cases/million/year
• Variable prognosis
• Up to 65% MSK involvement at presentation
• Death: Lung, heart and renal involvement

CREST Syndrome Figure 4-32-24
• First described by Winterbauer in 1964 as CRST
• Velayos added esophageal involvement to make
CREST in 1979
• 50,000 to 100,000 in US
• 7 times more common in females
• Calcinosis
• Raynaud’s phenomenon
• Esophageal abnormalities
• Sclerodactyly
• Telangiectasia
Calcinosis [Figure 4-32-24]

• Abnormal calcium deposition in ST without calcium Calcinosis of Scleroderma
metabolism abnormality
• Fingers, forearms, and extensor surfaces of elbows and knees
Figure 4-32-25
Raynaud’s phenomenon [Figure 4-32-24]
• Often the first symptom of scleroderma
• Ischemia of fingers, toes and ears
• Numbness, tingling and burning pain
• Attacks precipitated by cold, vibration and emotional
stimuli
Acroosteolysis [Figure 4-32-25]

Acroosteolysis of scleroderma
Gastrointestinal (esophageal) involvement
• Fibrosis and atrophy of the smooth muscle
• Hypermobility, dysphasia, reflux esophagitis and strictures
Sclerodactyly
• Replacement of the normal connective tissue with dense collagen bundles
• Skin = thin, appears smooth and is tightly bound
• Fingers narrow and taper distally
Telangiectasia
• Permanent dilatation of capillaries and venules
• Face, lips, tongue and fingers
Idiopathic inflammatory myopathies

• Dermatomyositis and Polymyositis
• 2:1 female to male ratio
• 5 cases/million/year (incidence increasing)
• Dermatomyositis
➢ Complement-mediated (terminal attack complex) vascular inflammation
• Polymyositis
➢ Direct cytotoxic effect of CD8+ lymphocytes on muscle
Idiopathic inflammatory myopathies

• Dermatomyositis
➢ Men > 40 yo
➢ Skin rash and muscle weakness
➢ Primary malignancies:
❖ Lung, prostate, female pelvic organs, breast or GI tract
➢ Precedes detection of tumor months to years
• Polymyositis:
➢ Primary malignancies: Lung, NHL

Imaging Findings [Figures 4-32-26 to 4-32-29] Figure 4-32-26
• ST abnormalities
➢ ST thickening and edema
➢ Soft and periarticular calcification (IM > SQ)
• Articular abnormalities
➢ Radial subluxation or dislocation of IP of thumb
(“floppy thumb”) = quite characteristic
➢ Erosions of multiple sites in hands
➢ Flexion deformities (MCP)
❖ “Swan neck” deformity Polymyositis with soft tissue calcification
“Floppy thumb” of
dermatomyositis/polymyositis
Dermatomyositis with extensive soft
tissue calcification
“Swan neck” deformity Figure 4-32-29

• MC flexion, PIP hyperextension and flexion at the DIP
• Most common inrheumatoid arthritis
Other imaging Findings

• RBS
➢ Increased accumulation at sites of calcification (Technetium
and gallium)
• MR Imaging
➢ Muscle atrophy
➢ Fatty replacement
➢ Decreased SI correlating with activity of disease
➢ Increased SI on T2WI and STIR
Remember!
• PM, DM, Scleroderma, SLE, mixed CVD and overlap syndromes
may all look alike
➢ ST calcification
➢ Articular and osseous abnormalities
Dermatomyositis with increased signal

intensity in the right gluteal region

Summary
• Osteoporosis
➢ Most common metabolic disease
➢ Insufficiency fractures may mimic mets/myeloma
➢ Diagnosis of exclusion in young patient with osteopenia out of proportion
to age
➢ Easily fractured and exuberant callous formation
➢ Pencilling, “pseudarthrosis,”, posterior scalloping, thoracic meningocele
• Systemic lupus erythematosis
➢ Ulnar deviation without erosions (differential is Jacoud’s (post-
streptococcal arthritis)
• Scleroderma
➢ CREST, acroosteolysis
• Inflammatory muscle disease
➢ Dermatomyositis and polymyositis
➢ Nonspecific findings (look like scleroderma)
➢ Must look for malignancy in these patients
References
1. Bohndorf K, Imhof H, Pope TL (eds). Musculoskeletal Imaging: A Concise Multimodality Approach. New York,
NY, Thieme Medical Publishers, 2001

Osseous Musculoskeletal Stress Injuries
Outline
• Biomechanical considerations
• Historical perspective
• Epidemiology and clinical manifestations
• Anatomic approach with examples
➢ Stress injury “look alikes”
• Avulsive and muscular “tug” injuries
• Unusual activities causing stress injury
• Recommended work-up
“Bone Fatigue”
• 1922 - Muller
➢ Isolated a segment from the radius of a dog
➢ Created a fatigue fracture of the ulna
• 1949/50 - Rutishauser/Majno
➢ First description of the histologic aspects of fatigue fractures
Muller W. Bruns Beitr. klin. Chir. 127:251-290, 1922
R/M. Schweiz. med. Wschr. 79: 281-88, 1949. 6:333-42, 1950.
Historical Perspective
• 1855 - Breithaupt - Prussian military surgeon
➢ Clinical features of painful feet on long marches
• 1887 - Pauzet - army doc
➢ Exostoses on PE from bone proliferation soldiers’ feet
• 1897 - Stechow - Prussian guard
➢ First imaging in 36 cases of MT stress fractures
• 1905 – Blecher
➢ First femoral neck stress fracture
• 1921 – Deutschlander - German physician
➢ Comprehensive study of stress lesions in > 50 yo
• 1936 - Asal – German
➢ First large series of 590 stress fractures in German troops
Types of MSK stress injury

• Soft tissue injury
➢ Bruises
➢ Muscle tears
➢ Musculotendinous injury
• Collagen injury
➢ Tendon and ligament strain/tears
• Cartilage injury
➢ Chondral injury (hyaline and fibrocartilage)
• Osseous abnormalities
➢ Stress reaction (sclerosis/lucency/periosteal reaction)
➢ Stress fracture
Stress injuries
• Most common in lower extremities
• Dissipation of ground reaction forces (GRF) (running, walking, marching,
jumping)
• Bone exposed to stress (ie load) and strain (ie deformation) with weight-
bearing
• Factors influencing bone response:
➢ Bone geometry and bone density
• Jumping and landing – GRF up to 12 X body weight
Musculoskeletal Radiology 987 Osseous Musculoskeletal Stress Injuries

How Do Stress Injuries Develop? Figure 4-33-1
• “Wolff’s law”
➢ Bone remodels in DIRECT reponse to the forces applied to it
• Normally a happy “marriage/relationship” between osteoblasts
and osteoclasts
• Increased stresses cause increased osteoclastic activity with
transient weakening
• Transient weakening predisposes to “microdamage”
• Coalescence of “microdamage” – stress reaction or injury
• Cascade
➢ Fissures
➢ Microfractures
➢ Osteoblastic response (periosteal reaction or cancellous
“clouding”)
➢ Coalescence
➢ Fatigue reaction/injury
Epidemiology
• 20% of all injuries seen in sports medicine clinics
• Between 4.7% and 15.6% of injuries in runners
• 20-25% of stress injuries in tibia, fibula or MT
• Females: 49% with very irregular menstruation, 39% with
irregular menstruation
• Study of 320 athletes with stress injury:
➢ 69% runners
➢ 8% fitness class participants
➢ 5% racket sports
➢ 4% basketball players Grade 4 Stress injury of the tibia,
• Track athletes have highest incidence pathological fracture of the fibula in
patient with RA on steroids
Clinical Features of Stress Injury Condensation of cancellous bone
• Pain associated with activity Perpendicular to the long axis
• Pain resolves without activity
• Pain in characteristic location associated with activity
• PE: ST swelling, point tenderness
Risk Factors for Stress Injury

• Intrinsic
➢ Low BMD
➢ Lower limb misalignment
➢ Muscle fatigue
➢ Weakness/strength imbalance
➢ Pathologic bone
➢ Menstrual/hormonal irregularities
➢ Genetic predisposition
• Extrinsic
➢ Excess volume/intensity of training
➢ Change in training surface (density or topography)
➢ Worn out training shoes
➢ Inadequate nutrition
➢ Cigarette smoking
Osseous Musculoskeletal Stress Injuries 988 Musculoskeletal Radiology

Activities associated with Stress Injury
• Lower extremity
➢ Running
➢ Marching
➢ Soccer (pelvis)
➢ Basketball
➢ Skating (fibula)
➢ Jumping (pelvis)
➢ Swimming (tibia, MT)
➢ Ballet (pelvis, spine)
• Upper extremity
➢ Baseball:
❖ Throwing-Humerus, scapula, olecranon, first rib
❖ Batting-Ribs
❖ Catching-Patella, tibia
➢ Javelin throwing-ulna
➢ Basketball
➢ Volleyball
Activities associated with Stress Injury

• Rowing, kayaking-Second through tenth ribs
• Running with hand held weights-Scapula
• Coughing-ribs
• Trapshooting-Coracoid process
Major categories of Stress Injury

• “Fatigue” fracture
➢ Abnormal muscular stress of torque
➢ Bone of normal elastic resistance
• “Insufficiency” fracture
➢ Normal or physiologic activity
➢ Bone deficient in mineral or elastic resistance
*****Imaging findings are similar
Risk factors - Insufficiency fractures

• Osteoporosis
• Metabolic disease
• Osteomalacia/Rickets
• Cushing’s disease
• Paget disease
MR Grading System Stress injuries

• 0 – Normal study
• 1 – Subtle periosteal edema (IR, FS T2-W images)
• 2 – Periosteal edema and increased marrow SI on FS T2-W images
• 3 – More extensive edema (T1-W and T2-W)
• 4 – Discrete fracture line visible on MR or on radiography
[Figure 4-33-1]
Fredericson M, Bergman AG et al: Am J Sports Med 1995;23:472-481
Anatomic approach
• Lower extremity: Foot (MT, navicular, calcaneus), tibia, femur
• Pelvis (Insufficiency and adolescent)
• Upper extremity
• Unusual causes of stress injury
• Mimics of stress injury
• Recommended workup

Metatarsal stress fractures [Figures 4-33-2 to 4-33-5] Figure 4-33-2
• Second most common stress fracture site behind the
tibia
• First described in military recruits
➢ Flat flexible feet = MT stress fractures
➢ Cavus feet = tibial stress fractures
• Distance runners and ballet dancers
• In order: 2, 3, 1, 4, 5
Navicular stress fracture [Figures 4-33-6 and 4-33-7]

• 0.7%-2.4% of all stress fx’s
• Activities: Track and field (59%), Australian football
(19%), Basketball (10%)
• First description: 1958 in greyhounds
• 1970 - humans
• Most common in runners
• Increasing dorsal midfoot pain radiating down medial
arch
• Rx: Non-weight bearing cast (86% success rate)
Figure 4-33-3
Second MT fatigue fracture
Figure 4-33-4
Right 2nd MT fatigue fracture and 1 month f/u in

42 yo male
Figure 4-33-5
MR imaging of third MT fatigue fracture, soccer

player
Sesamoid necrosis in ballet dancer

Figure 4-33-6
Figure 4-33-7
Classic navicular fatigue fracture

[Case courtesy of Dr. Armando Abreu, Porto Allegro, BR]
Figure 4-33-8
Microangiopathic studies cadaveric Classic fatigue fracture of calcaneus

feet…navicular supplied by both [Case courtesy of Dr. Armando Abreu, Porto Allegro, BR]
ant and post tibial arteries, enter at

small “waist” of cortical bone and
branch out to supply the medial
and lateral 1/3… Central 1/3,
under greatest stress with relative
avascularity
Tibia stress fracture Figure 4-33-9

• Three major types
➢ Medial tibial plateau
➢ Tibial diaphysis
➢ Anterior mid tibia
Medial tibial plateau [Figure 4-33-9]

• Less frequent than tibial diaphysis
• Often misdiagnosed as pes anserinus bursitis or
tendoninitis
• Less critical stress injury Medial tibial fatigue fracture
• Treatment
➢ Rest for 4-6 weeks
➢ Then return to full activity
Figure 4-33-10
Medial tibial stress syndrome
“Shin splints”[Figure 4-33-10]
MR features of medial tibial stress

syndrome (“shin splints”)

“Aggressive” periosteal reaction in the tibia in

fatigue fracture
Diaphyseal fatigue fracture with histologic

correlation from the AFIP fascicles
Figure 4-33-13
Tibial diaphyseal Fatigue Fracture[Figures 4-33-11 and 4-33-12]

• Posteromedial border of tibia
• Tensile forces produced along anterior convex side, compressive
forces along posterior concave margin
• 465 injuries causing exertional leg pain – 75% in posteromedial
tibial diaphysis
• Difficult to tell stress injury from “shin splints” (medial tibial stress
syndrome)
Anterior mid-tibia [Figure 4-33-13]

• Most common in jumping and leaping athletes
• Focal cortical thickening and sclerosis
• “Dreaded black line”
➢ Propensity to nonunion Diaphyseal fatigue fracture with the
➢ Risk of complete displaces fracture “dreaded black line”
• Require more aggressive treatment
Figure 4-33-14
Longitudinal Tibial Stress Fracture
[Figure 4-33-14]
• Devas 1960
• Patients MAY not give h/o increased activity
• Saifuddin (Clin Rad 1994):
➢ Two cases
➢ Stress fx located superomedial to the nutrient
foramen of the tibia
➢ Foramen weakens bone at this site?
➢ ? insufficiency fracture
Longitudinal stress fractures of the tibia Longitudinal fatigue fracture

Craig et al, Henry Ford Hospital, Detroit,
Skeletal Radiology, 2003
• Six cases Figure 4-33-15
• All female (age range - 15-69 yo)
• Diagnosis made by finding cleft on multiple axial
images
• 5/6 patients had:
➢ Edema starting at level of the entrance of the
nutritent vessel into the medullary cavity
➢ Vertical fx identified below this level on the
anteromedial tibial cortex
Compression type of femoral neck fatigue fracture

Femoral neck stress injury [Figures 4-33-15 and 4-33-16] Figure 4-33-16
• Any athlete (jogger/runner) with hip, thigh or groin
pain
• Two types
➢ Tension type:
❖ Superior cortex
❖ Older osteoporotic patients
➢ Compression type:
❖ Younger athletic patients
• Treatment: 2-3 months non-weight bearing with
gradual return
Pelvic Stress Injury [Figures 4-33-17 to 4-33-21]

• Running increases risk of stress lesions in sacrum Fatigue fracture which progressed to complete
and ischial rami femoral neck fracture
• Sacral fractures more common in young women
(sx’s mimic sacroiilitis)
➢ Fatigue:
❖ Anteroinferior sacral wing unilaterally
➢ Insufficiency:
❖ Elderly women, irradiated women
❖ Often bilateral (“Honda” sign)
Figure 4-33-17
Figure 4-33-19
Sacral insufficiency fracture

Figure 4-33-18
Bilateral sacral insufficiency

fractures (the “Honda” sign)
Figure 4-33-20
CT of previous figure on left showing

the classic CT findings of right sacral
insufficiency fracture
Figure 4-33-21
Symphysis pubis stress reaction in soccer player
Bilateral sacral and symphyseal insufficiency

fractures in elderly female S/P external beam
radiation for cervical cancer. Note the bilateral
nature of the sacral fractures

Adolescent Stress Lesions [Figure 4-33-22] Figure 4-33-22
• Muscular “tug” (avulsive) lesions (Classic lesion:
Cortical desmoid)
• Avulsion injury and sequelae
• Tendons, ligaments stronger than bone in
adolescents
• May mimic primary soft tissue neoplasm in acute
setting
• May mimic primary bone neoplasm after healing has
occurred
Recommended Workup - Suspected Stress

Injury
• Correlate clinical situation with imaging
• Think “stress injury” in the correct setting (you may
be the only clinician who does so!!) 16 yo with bilateral healed avulsions whose
• Initial study is the conventional radiograph diagnosis was made at age 42!
• Young patient-CT or MR imaging
• Elderly patient
➢ Typical site-CT or MR
➢ Unusual site-RBS as screen, then CT or MR
References
1. Chamay A. Mechanical and morphological aspects of experimental overload and fatigue in bone. J Biomech 1970;
3:263-270.
2. Craig JG, Widman D, van Holsbeeck M. Longitudinal stress fracture: patterns of edema and the importance of the
nutrient foramen. Skeletal Radiol 2003; 32:22-27.
3. Müller W. Bruns Beitr. klin. Chir. 127:251-290, 1922
4. Rutishauser E, Majno G. [Lesions of normal and pathological bones due to overstrain.]. Bull Schweiz Akad Med
Wiss 1950; 6:333-342.
5. Tschantz P, Rutishauser E. [The mechanical overloading of living bone: initial plastic deformations and adaptation
hypertrophy]. Ann Anat Pathol (Paris) 1967; 12:223-248.

Pelvis and Lower Extremity Trauma:
An introduction
Figure 4-34-1
Outline
• Caveats
➢ Major highlights
➢ Not enough time to cover in depth
➢ You must read more on your own to
supplement this lecture
• Pelvic trauma
• Acetabular trauma
• Lower extremity trauma
➢ Femur
➢ Knee
➢ Ankle
➢ Foot
❖ Talus
❖ Calcaneus
❖ Classic fx’s
Radiographic anatomy [Figure 4-34-1]

Major mechanisms of pelvic injury Pelvic Radiographic Anatomy, ip = iliopubic line, ii =
[Figure 4-34-2] ilioischial line, SIJ = sacroiliac joints
Most Popular Classifications Pelvic Ring Fractures Figure 4-34-2

• Tile
➢ Pelvic stability
• Young-Burgess
➢ Degree of injury
• Major divisions
➢ “Ring sparing”
➢ AP compression
➢ Lateral compression
➢ Vertical shear
➢ Complex
Major mechanisms of pelvic injury (Tony Wilson,

Young-Burgess vs Tile Classifications Seattle Washington)
FRACTURE TYPE YOUNG-BURGESS TILE
Ring sparing Not included Type A
Anterior compression AP compression B1

Types I-III (B1, 1.1-1.3)
Lateral compression Lateral compression B2
Types I-III (B2, B2.1-2.2)
Vertical shear Vertical shear C

(C1-3)
Musculoskeletal Radiology 995 Pelvis-Lower Extremities Trauma

Young-Burgess Classification Pelvic Ring Fractures Figure 4-34-3
• Lateral compression (Most common)
➢ Types I and II
• Anteroposterior (AP) compression
➢ Types I, II and III
• Vertical shear
Lateral compression
• Most common mechanism of pelvic injury
• Lateral blow to the side of the pelvis Lateral compression Type I injury-STABLE
• Three types depending on severity (Note the disruption of the sacral foraminal
• “KEYS TO THIS INJURY:” (arcuate) lines-arrows)
➢ Horizontal fx’s of pubic ramus/rami
➢ Crush (buckling) fx of sacrum
Figure 4-34-4
Lateral compression - Young-Burgess
classification [Figures 4-34-3 to 4-34-5]
• I – Ipsilateral sacral compression fx (stable)
• II- I + associated iliac wing fx
➢ Rotationally unstable
Vertically stable
• III – I + II with contralateral “open book” appearance
(“windswept pelvis”)
“Windswept pelvis” - Lateral compression-

ipsilateral - AP compression- Lateral compression Type I injury
contralateral [Figure 4-34-6]
• Severe anterior force
• Internal rotation of ipsilateral hemipelvis with external rotation of Figure 4-34-5
contralateral hemipelvis
• “Rolled over” look
Figure 4-34-6
“Windswept” pelvis
Lateral compression Type I injury
AP Compression
• Blows to front of pelvis
• MVA
• Three types depending on which ligaments involved
• Increases volume of pelvis
• Major risk = hemorrhage
• Often brain/abdominal injuries
• KEYS TO THIS INJURY:
➢ Vertical fx inf pubic rami (one or both sides)
➢ > 50% post acetabular wall
➢ < 10% sacral fx
Pelvis-Lower Extremities Trauma 996 Musculoskeletal Radiology

AP Compression - Young-Burgess classification
[Figures 4-34-7 to 4-34-9] Figure 4-34-7
• Type I = SP disrupted (all ligs intact)
• Type II
➢ SP diastasis < 2.5 cm
➢ Torn ligaments:
❖ SP, SS, ST and ventral (anterior SI)
• Type III
➢ SP diastasis > 2.5 cm
➢ Torn ligaments:
❖ SP, ST, SS and both ventral (anterior) and
dorsal (posterior) SI
AP compression Type II injury
AP Compression injury with vertical fracture of the

sacrum
AP compression injury (Note marked

widening of the symphysis pubis)
AP Compression, Type II Figure 4-34-10

• Disruption of sacrospinous, sacrotuberous and ventral (anterior) SIJ
ligaments
• Diastasis of SP > 2.5 cm
• Diastasis of both SIJ anteriorly
• “Open book” appearance
• Rotationally unstable
• Vertically and posteriorly stable
AP Compression, Type III

• Type I and II
• Disruption of all SIJ ligaments
• Complete separation of iliac wing from sacrum
• Complete pelvic instability
• Rotationally, vertically and posteriorly unstable
Vertical Shear
• Fall from height or head and back trauma
• Least common
• KEYS TO THIS INJURY:
➢ Disruption of SP or SIJ
➢ Cephalad or caudad displacement of pelvis (best seen on
OUTLET film)
• Rotationally, vertically and posteriorly UNSTABLE
Vertical Shear [Figures 4-34-10 and 4-34-11]

• Disruption of SP, ST, SS, and ant/post SIJ ligaments
• Characteristics
➢ Vertical pubic rami fractures
➢ SIJ disruption +/- adjacent fractures
• Hemipelvis vertically (cranially) displaced
Vertical shear injury – Note vertical
migration of left hemipelvis

Clues Figure 4-34-11
• Horizontally oriented pubic fracture
➢ Think lateral compression, look closely at sacral arcuate lines
• Vertically oriented pubic fracture
➢ With AP displacement, think AP compression
➢ With vertical displacement, think vertical shear
• Posterior wall acetabular fx
➢ Think AP compression
• Central acetabular fx’s
➢ Think lateral compression
Complications Pelvic Ring Disruption [Figure 4-34-12]

• Mortality 5%-50% (reflect severity)
➢ AP compression – 26%
➢ Vertical shear – 25%
➢ Complex – 17%
➢ Lateral compression – 13%
• Head injury and hemorrhage (internal iliac branches or superior
gluteal artery near sciatic notch)
Acetabular Injury Soft tissue findings of vertical shear

• Significant trauma (MVA, falls)
injury
• Associated pelvic ring fractures
• Pattern of acetabular injury depends on:
➢ 1. Position of femoral head at time of traumatic event
❖ FH externally rotated = anterior column
❖ FH adducted = acetabular roof
❖ FH abducted = forces transmitted inferiorly
➢ 2. Direction of force
❖ Anterior force = posterior wall and column
❖ Lateral force = medial acetabular wall (transverse type)
• Therapy depends on proper classification
Acetabular Columns Letournel and Judet

• Anterior Figure 4-34-12
➢ Iliac wing to anterior acetabulum
➢ Incorporates superior pubic ramus
• Posterior
➢ Sciatic notch to posterior acetabulum to ischium
“Inverted Y” column principle

Radiographic Evaluation Acetabulum
[Figure 4-34-13]
• AP pelvis
• Judet views Soft tissue complications of pelvic ring fractures
➢ 45 degree oblique views
➢ Right
❖ RPO = Iliac oblique
❖ RAO = Obturator oblique
➢ Left Figure 4-34-13
❖ LPO = Iliac oblique
❖ LAO = Obturator oblique
Acetabular fractures
• Letournel and Judet classification, 1993
• Ten different patterns
➢ Five elementary (run in single plane)
➢ Five associated (combination of elementary)
• Difficult to remember
• Most common:
➢ Posterior wall
➢ Transverse with posterior wall
➢ Both column (most common type)
• Add T-shaped and transverse = 90% Pelvic CT anatomy (see Harris et al: AJR
2004;182:1363-75)

Letournel and Judet, 1993 Figure 4-34-14
• Elementary (simple) fractures
➢ Posterior wall
➢ Posterior column
➢ Anterior wall
➢ Anterior column
➢ Transverse
• Complex (associated) fractures
➢ T-shaped
➢ Posterior wall posterior column
➢ Transverse posterior wall
➢ Anterior with posterior hemitransverse
➢ Both columns
CT patterns of acetabular fractures

Hunter, RCNA 1997
• Axial CT image through roof of acetabulum
➢ Column Fracture
➢ Transverse Fracture
➢ Wall Fracture
➢ Normal
Wall Fracture [Figures 4-34-14 and 4-34-15]

• Weight-bearing columns of acetabulum not disrupted
• Posterior wall most common
• Major complications:
➢ Hip joint instability
➢ Osteonecrosis
Transverse Fractures [Figures 4-34-16 and 4-34-17]

• Medial and lateral components
• Fx line anterior to posterior
• Separates “walls” from “columns”
• CT = sagittal plane
Figure 4-34-15
Posterior wall acetabular fracture
Figure 4-34-16
CT of posterior wall fracture

(Same patient )
T-type transverse fracture

Column Fracture Figure 4-34-17
• Craniocaudad (coronal) direction
• Front and back halves
• Ant/post only or both
• Associated with other fx’s (post column, post wall)
• Conceptualize: Grasp ASIS could move acetabulum
freely
• Obturator fx = column type or T-shaped fracture
Questions To Ask Yourself…

• Obturator ring fx?
➢ T-shaped or column fx
• Ilioischial line disrupted?
➢ Posterior column or transverse fx patterns
• Iliopectineal line disrupted?
➢ Anterior column or transverse-type fx’s
• Is iliac wing above acetabulum fractured?
➢ Fracture of anterior column
• Is the posterior wall fractured? CT of T-type transverse fracture
➢ Isolated or combo with post column or transverse with Sagittal (lower left)
fx’s and Coronal (lower right) reconstruction
• Is “spur” sign present?
➢ Almost assuredly “both column” fracture
Hip Trauma
• Dislocations
• Hip fx’s
➢ Common injury in multi-trauma
➢ Common in the elderly
❖ Osteoporosis and cerebrovascular disease
❖ Prone to falls
Hip Dislocations
• 5% of all dislocations
• High energy trauma (MVA, MCA, etc.)
• ~90+% posterior
• Commonly associated with femoral shaft, patella and post acetabular fx’s
• Clinically
➢ Limb shortening, internally rotated and adducted
➢ 10-15% transient sciatic nerve palsy (direct impingement)
Hip Fractures
• Intracapsular
➢ Subcapital
➢ Mid cervical
➢ Basicervical
• Extracapsular
➢ Intertrochanteric
➢ Subtrochanteric
• Femoral neck fx’s 3-6X > women
• Intertroch fx = frequency
Subtrochanteric Fractures
• Fracture line extends between LT and point 5 cm distally
• Direct trauma
• Older patient, less force required
• High incidence of malunion or nonunion
➢ ? secondary to greater proportion of cortical bone to trabecular bone in this
region
• Rx: Intramedullary rod

Knee Injury
• Soft tissue signs
➢ ST swelling, lipohemarthrosis
• Fractures
➢ *Supracondylar
➢ *Condylar
➢ *Tibial plateau
➢ Impaction (lateral femoral condylar notch)
➢ Tibial avulsion fractures
➢ Segond fracture
➢ Patellar fractures and dislocation
Tibial Plateau Fractures

• Valgus stress, 85% involve lateral tibial plateau
• Fat-fluid level
• Schatzker classification (6 types)
• Depression = cartilage thickness (3mm)
• Meniscal injury ~ 50%
• Rx: Lateral buttress plate and screw
Schatzker Classification
• I=split fx (younger)
• II=split + depression of LTP (older)
• III=depression - splitting
• IV=MTP +/- depression
• V=split fx through MTP and LTP
• VI=dissociation of TP from underlying diaphysis
Tibial Plafond (pilon) Fracture

• High energy axial loading (talus on tibial plafond)
• Ankle and distal tibial metaphyseal fx, intraarticular
• 20%-25% open
• Associated injuries:
➢ Compartment syndrome, vertebral compression fractures
• Often require ORIF
• Post-traumatic arthritis common
Maisonneuve fracture
• External rotation of ankle
• Fibular fx
• Serious injury
• Requires ORIF with screws
• Removed 8-12 weeks after injury
Ankle Fractures
• Common injuries
• Soft tissue changes (STS, effusion)
• Classification schemes
➢ Lauge-Hansen
❖ Difficult to remember
❖ Not very reproducible
➢ Danis-Weber (AO)
❖ Easy to remember
❖ Reproducible
Danis-Weber Classification
• Type A: Horizontal avulsion fx below mortise, stable, Rx: Closed reduction
and casting (without displaced MM fx)
• Type B: Spiral fibular fx – level of mortise, external rotation, stable or unstable,
Rx: Closed reduction unless fragments displaced
• Type C: Above mortise, disruption of lig attachment of tibia/fibula distal to fx,
unstable, Rx: ORIF

Ankle Fractures Figure 4-34-18
• Key is re-establishing tibiotalar joint congruence
• Mortise view important
• 1-2 mm displacement of talus in mortise dramatically
changes contact area and pressure
• 40% decrease in contact area with 1mm lateral talar
shift
Talar Neck Fractures [Figure 4-34-18]

• 3%-5 % of foot fractures
• Dorsally directed force on braced foot (“aviator’s
astragulus” – WWI pilots), now most commonly MVA
• Main blood supply of talar body enters neck through
sinus tarsi and proceeds retrograde to supply body
• Neck fx’s compromise vascularity
• Hawkins sign (no AVN)
Hawkins II talar neck fracture
Talar Neck Fractures - Hawkins
classification [Figure 4-34-19]
Figure 4-34-20
Figure 4-34-19
Risk of AVN: I = 10%; II = 40%; III = 90%; IV = 100%

Hawkins classification of talar neck fractures
(Bohndorf K, Imhoff H, Pope T: Synopsis of MSK Imaging: A
Multimodality Approach, Thieme)
Lateral Process of Talus fx [Figure 4-34-20]

• Snowboarder’s fx
• Eversion
• Lat process caught between LM and calcaneus
• May be caused by inversion and dorsiflexion
Calcaneal Fractures [Figure 4-34-21]

• Most frequently fractured tarsal bone (60% of all fx’s)
• 2% of all fx’s in adults
• 5% - 9% bilateral
• 10% LS compression fx’s
• Peroneal tendon entrapment or compartment
• Intraarticular 70%, extra-articular 30%
• Most common EA = calcaneal body fx
• Anterior process = 15%
• Difficult to treat if displaced
Figure 4-34-21
Lateral process of talus

fracture (Snowboarder’s
fracture)
Coronal reconstruction
CT of calcaneal fracture of CT of calcaneal
fracture

Classification systems Calcaneal Fractures Figure 4-34-22
• Bohler (1931)
• Essex-Lopresti (1952)
➢ Intraarticular vs extra-articular
➢ Types: Tongue and Joint depression
• Rowe (1963)
• Saunders – CT classification (1992)
• Others: Hanover, Rowe, Palmer, Souer and Remy
Sanders Classification
• I = Non-displaced
➢ Non-operative
• II = 2 parts (split)
➢ ORIF
• III = 3 parts (split and depression
➢ ORIF
• IV = Comminuted
➢ Defies open reduction
• Measures height of PF
• A = most cephalic point of tuberosity to posterior
border of subtalar joint
• B = posterior border of subtalar joint to anterior
process
• Normal: 20 - 40º
Anatomy of the tarsal joints (Bohndorf K, Imhoff H,
Foot Injuries Pope T: Synopsis of MSK Imaging: A
• 5th MT Multimodality Approach, Thieme)
➢ Avulsion (pseudo-Jones or tennis fracture)
➢ Jones
➢ Stress fx (fatigue or insufficiency)
Figure 4-34-23
• LisFranc
Jones Fracture
• Transverse fx
• 2-3 cm distally
• Displaces on weight bearing
• 35%-50% persistent non-union
Google Search: Sponsored links (Jones Fracture lawsuits-Recover medical
expenses-Find attorneys and help nationwide-personal-injury-lawyer.com)
Lisfranc [Figures 4-34-22 and 4-34-23]

• Napoleonic surgeon
• Developed quicker technique of forefoot amputation for gangrene
• Faster wiithout having to cut bone
• Injury in foot never described by him
• Commonly misdiagnosed Divergent (left) and Homolateral
(right) types of LisFranc injury
Summary
• Reviewed major pelvic, acetabular and lower extremity traumatic
lesions
• Meant as an introduction
• Supplement with reading and study
• Xerox major classifications of fractures
• Have readily available in MSK reading area
• Consult classifications frequently
• Supplement clinical experience with personal reading EVERY DAY
References
1. Bohler L: Diagnosis, pathology, and treatment of fractures of the os calcis. J Bone Joint Surg 13:75-89, 1931.
2. Bohndorf K, Imhof H, Pope TL (eds). Musculoskeletal Imaging: A Concise Multimodality Approach. New York,
NY, Thieme Medical Publishers, 2001
3. Borrill J, Funk L, Deakin S. Orthoteers: The guiding light in orthopaedic education. 2006.British Orthopaedic
Association.. <http://www.orthoteers.org/>
4. eMedicine (James WD, Adler J, Lutsep HL, Lorenzo CT, Lin EC, Ho SSW, Roy H, Gellman H, Meyers AD eds)

1996-2005 eMedicine.com, Inc . WedMD. <http://www.emedicine.com/>
5. Essex-Lopresti P. The mechanism, reduction technique, and results in fractures of the os calcis. Br J Surg 1952;
39:395-419.
6. GE: Healthcare reimagined. Copyright General Electric Company 1997-2006. GE Medical Systems. 2006.
<http://www.amershamhealth-us.com/>
7. Harris JH, Jr., Lee JS, Coupe KJ, Trotscher T. Acetabular fractures revisited: part 1, redefinition of the Letournel
anterior column. AJR Am J Roentgenol 2004; 182:1363-1366.
8. Hunter JC, Brandser EA, Tran KA. Pelvic and acetabular trauma. Radiol Clin North Am 1997; 35:559-590.
9. Letournel E, Judet R. Fractures of the acetabulum, 2nd ed. Heidelberg, Germany: Springer-Verlag,1993
10. MacLeod M, Powell JN. Evaluation of pelvic fractures. Clinical and radiologic. Orthop Clin North Am 1997;
28:299-319.
11. Palmer I. The mechanism and treatment of fractures of the calcaneus: open reduction with the use of cancellous
grafts. J Bone Joint surg 1948;30-A(1):2-8
12. Perry DC, DeLong W. Acetabular fractures. Orthop Clin North Am 1997; 28:405-417.
13. Rowe CR, Sakellarides HT, Freeman PA, et al. Fractures of the os calcis: long term follow-up study of 146
patients. JAMA 1963;184:920-923
14. Sanders, R., Hansen, S.T. & McReynolds, I.S.: Fractures of the calcaneus, in Jahss, M. (Ed.): Disorders of the foot
and ankle, Philadelphia, W.B. Saunders, 1991. p. 2326-2354.
15. Souer, R. & Remy, R.: Fractures of the calcaneus with displacement of the thalamic portion. J Bone Joint Surg [Br]
57: 413-421, 1975.
16. Wheeless' Textbook of Orthopaedics. Copyright 1996-2005 Data Trace Publishing Company. Duke University
Medical Center's Division of Orthopaedic Surgery. Data Trace Internet Publishing Company. 2006.

Musculoskeletal Seminar I
Mark D. Murphey, MD
UNKNOWN CASE #1: HISTORY

• 15 year old male with longstanding hindfoot pain
UNKNOWN CASE #1: DIFFERENTIAL DIAGNOSIS

LESIONS WITH SEQUESTRA-LIKE APPEARANCE
• Osteomyelitis
• Metastasis
• Fibrosarcoma/ Malignant Fibrous Histiocytoma (MFH)
• Lymphoma
• Osteoblastoma

CORTICAL LUCENCY/CENTRAL CALCIFICATION
• Osteoid osteoma
• Brodie abscess
UNKNOWN CASE #1: FINDINGS

• Diffuse sclerosis of calcaneus
• CT-solid periosteal reaction causing sclerosis on radiographs
• Subchondral low density lesion with central calcification
T1
• Diffuse edema/focal lesion related to posterior subtalar joint with
joint effusion
UNKNOWN CASE #1: OSTEOID OSTEOMA

• 10–25 years, M>F (3:1)
• Night pain relieved by ASA
• Lytic nidus (<1.5-2.0 cm); central calcification
• Intracortical-extensive periosteal reaction
• Intramedullary-often subtle little sclerosis
• Lymphofollicular synovitis T2
• CT/MRI important for surgical guidance
UNKNOWN CASE #1: OSTEOID OSTEOMA

TREATMENT OPTIONS
• Surgical excision
• Percutaneous removal
• Percutaneous ablation
• Medical
GRE
Musculoskeletal Radiology 1005 Musculoskeletal Seminar I

• 27 year old female with 5 years chronic left hip pain
UNKNOWN CASE #2: OSSEOUS LESIONS

BOTH SIDES OF JOINT
• Arthritis
• Infection
• PVNS
• Synovial chondromatosis
• Amyloidosis
UNKNOWN CASE #2: PVNS

• Young males-3rd to 4th decade
• Synovial proliferation with hemosiderin deposition
• Extrinsic erosions common in hip
• Joint space normal; limited osteopenia
• Can appear like OA in hip
• Joint fluid-nodular thickening at arthrography Proton Density T2
• MRI often characteristic low intensity, fluid foci
• RX -synovectomy-adjuvant radionuclide therapy

• 5 year old male with mild midfoot pain
Symptomatic side
UNKNOWN CASE #3: OSTEOCHONDROSES

• Fragmentation
• Sclerosis
• Collapse
• AVN, trauma, normal
UNKNOWN CASE #3: KOHLER DISEASE

• Described 1908
• M>F; 4–6:1; 3–7 years old
• Often asymptomatic Asymptomatic side
• Unilateral 75–80%
• AVN ?
• Treatment-immobilization
Musculoskeletal Seminar I 1006 Musculoskeletal Radiology

• 13 year old female 1 year of pain now severe and worsening
UNKNOWN CASE #4: BRODIE ABSCESS

• Subacute osteomyelitis
• Medullary or cortical lucency surrounding sclerosis
• Channel-like lesion may extend to or across growth plate
• Staph aureus
• MRI or CT to evaluate soft tissue extension
T2
• 30 year old female with progressive clubbing of fingers
UNKNOWN CASE #5: ACROOSTEOLYSIS

BANDLIKE: DIFFERENTIAL DIAGNOSIS
• Polyvinyl chloride
• Hajdu-Cheney syndrome
• Post-traumatic
UNKNOWN CASE #5:

WORMIAN BONES: DIFFERENTIAL DIAGNOSIS
• Normal
• Cleidocranial dysplasia
• Cretinism
• Hypophosphatasia
• Pyknodysostosis
Musculoskeletal Radiology 1007 Musculoskeletal Seminar I

UNKNOWN CASE #5: HAJDU-CHENEY SYNDROME
• Autosomal dominant; described 1948
• Bathrocephaly, wormian bones, open sutures
• Acroosteolysis
• Poor dentition
• Osteoporosis

• 4 year old male with left hip pain

EPIPHYSEAL LESIONS
• Chondroblastoma
• Giant cell tumor (GCT)
• Subchondral cyst/Intraosseous ganglion
• Infection
• Langerhans cell histiocytosis (LCH)
• Clear cell chondrosarcoma

• Lytic lesion epiphysis and metaphysis
• Small amount surrounding sclerosis
• MRI and CT – no joint fluid or calcification
UNKNOWN CASE #6: EOSINOPHILIC GRANULOMA

(LCH)
• 5–15 years; M:F-2:1
• 95% of patients Caucasian
• Solitary 67%
• Flat bones involved-70%
• Lytic hole within hole appearance
• Diaphysis (58%), metadiaphysis (18%), metaphysis (28%),
epiphysis (2%)
Musculoskeletal Seminar I 1008 Musculoskeletal Radiology

Musculoskeletal Seminar II
Mark D. Murphey, MD

• 62 year old male with polyarticular joint pain

• Asymmetric erosive arthritis hands and feet: MCP, and IP joints
• New bone formation
• Limited osteopenia
UNKNOWN CASE #1: SERONEGATIVE

SPONDYLOARTHROPATHY CHARACTERISTICS
• Asymmetry
• Bone Production
• Less juxtaarticular osteopenia
• Distribution
UNKNOWN CASE #1: PSORIATIC ARTHRITIS

POSSIBLE PRESENTATIONS
• DIP and PIP joints
• Arthritis mutilans
• Oligoarthritis or ray distribution
• Rheumatoid like (rare)
• Sacroiliitis/spondylitis
Musculoskeletal Radiology 1009 Musculoskeletal Seminar II

• 42 year old female with right low back pain

• Unilateral destruction on both sides right sacroiliac joint
• Thickening of iliacus muscle
• Focal sclerotic fragments in joint
UNKNOWN CASE #2: DIFFERENTIAL DIAGNOSIS SACROILIITIS

• Unilateral-Infection, RA, Gout, Psoriatic, Reiter
• Bilateral asymmetric-RA, Gout, Psoriatic, Reiter
• Bilateral symmetric-AS, Enteropathic, Psoriatic, Reiter, RA

• 1 year old female with hand and foot pain

• Soft tissue swelling about several fingers
• Periosteal reaction along several rays: phalanx hand,
metacarpal and metatarsal
UNKNOWN CASE #3:

DIFFERENTIAL DIAGNOSIS – DACTYLITIS
• Infection
➢ Pyogenic
➢ Unusual organism-TB
• Sickle cell anemia
• Thermal injury
UNKNOWN CASE #3: SICKLE CELL ANEMIA

• Musculoskeletal changes
• Osteomyelitis (salmonella)
• H-type vertebrae
• Osteopenia
• Diffuse sclerosis
Musculoskeletal Seminar II 1010 Musculoskeletal Radiology

• 11 year old male with ankle pain after previous fracture
5 months after initial fracture

UNKNOWN CASE #4: SALTER-HARRIS FRACTURE
• 25%–33% growth sequelae
• Only 10% important
• Follow for 2 years-X-rays
• Look for bowing/shortening

• Initial Salter-Harris IV fracture without good reduction
• Subsequent lateral bowing of fibula and tibia
• Epiphyseal plate irregular
• CT/conventional tomography-osseous bar bridging plate

• 38 year old male with calf pain and mass, no history of trauma
T1 T2
Musculoskeletal Radiology 1011 Musculoskeletal Seminar II

• Nonspecific enhancing inflammation and edema in calf
with more focal mass medially
• Biopsy-soft tissue osteosarcoma
• 3 weeks later early calcification on CT predominantly peripheral
• 4 weeks later thick rind of calcification peripherally
UNKNOWN CASE #5: MYOSITIS OSSIFICANS

(HETEROTOPIC BONE FORMATION)
• No history of trauma 25%
• Subsequent calcification
• Zonal phenomenon X-ray and path
• Follow-up for maturation

• 14 year old female with enlarging right foot mass

CALCIFIED SOFT TISSUE MASS
• Gout, collagen vascular disease
• Hyperparathyroidism, tumoral calcinosis
• Hemangioma
• Soft tissue chondro/osteosarcoma

• Soft tissue mass plantar aspect mid to forefoot
• Faint calcification-CT and mag views;
smooth erosion of 2nd and 3rd metatarsals
• Large soft tissue mass on MRI mildly heterogeneous and
hyperintense T2W
UNKNOWN CASE #6: SYNOVIAL SARCOMA

• 20–40 years old
• 68% lower extremity – particularly knee
• Most begin periarticular (< 10% intraarticular)
• Biphasic – epithelioid and spindle cell element on histo (also
monophasic )
• Radiographs – soft tissue mass, joint effusion (10–20%),
calcification (30%), erosion or destruction adjacent bone
• Metastases-lungs and lymph nodes
Musculoskeletal Seminar II 1012 Musculoskeletal Radiology

Musculoskeletal Seminar III
Mark D. Murphey, MD

• 63 year old woman with vague calvarial pain
UNKNOWN CASE #1: DIFFERENTIAL DIAGNOSIS –

LYTIC SKULL LESIONS
• Metastases
• Myeloma
• Paget disease
• Brown tumor

• Focal skull lytic lesions: frontal and parieto-occipital
• Bone scan – multifocal area increased activity
• Radiographs – multiple lesions trabecular
thickening
PAGET DISEASE
• Common – 3% of people over 40 years
• Lytic, blastic or mixed phases
• Most frequent to involve: spine, skull, pelvis
• Trabecular thickening – bone enlargement
PAGET DISEASE: COMPLICATIONS

• Osseous deformity
• Fractures
• Neurologic symptoms
• Arthropaty
• Neoplasm
Musculoskeletal Radiology 1013 Musculoskeletal Seminar III

• 14 year old boy with thigh pain
T1
T2

• Cortical scalloping – femur
• Hair-on-end periosteal reaction
• Broad based soft tissue mass
• No medullary involvement
UNKNOWN CASE #2: PERIOSTEAL OSTEOSARCOMA

• Most chondroblastic
• 85% diaphysis femur/tibia
• Same age group as conventional osteosarcoma

• 55 year old man with hip pain T1
UNKNOWN CASE #3:

FINDINGS
• Osteopenia of left hip
• MRI – decreased intensity left
proximal femur T1W, and
diffuse increased signal T2W
• No focal defects on MRI; effusion
• Enhances with gado; hot on bone scan
• Returns to normal in several months T2
Musculoskeletal Seminar III 1014 Musculoskeletal Radiology

UNKNOWN CASE #3: TRANSIENT OSTEOPOROSIS HIP /
BONE MARROW EDEMA SYNDROME
• Spontaneous pain; worsened by weight-bearing
• Symptoms regress 2–6 months
• Migratory form may recur at nearby joint
• Cause unknown-bone marrow edema
• Relationship to AVN ?

• 82 year old man with slowly enlarging mass in the thigh
T2
T1
UNKNOWN CASE #4: DIFFERENTIAL DIAGNOSIS –

CALCIFIED SOFT TISSUE MASS
• Aneurysm
• Lipoma/liposarcoma
• Soft tissue osteosarcoma/chondrosarcoma

• Large mass thigh with mineralization calcification/ossification
• MRI/CT:
➢ Fat component
➢ Hemorrhagic component
➢ Myxoid component
UNKNOWN CASE #4: MYXOID LIPOSARCOMA

• Myxoid variety most common liposarcoma (40–50%) T1
• Intermediate grade GD
• See lipomatous components with CT/MRI (40–50%)
(We believe 90%-95% by MR)
• Mineralization not rare in liposarcoma

• 40 year old female with arthralgias

• Interphalangeal joint subluxations
• No erosions
• Osteopenia

SUBLUXATIONS/NO EROSIONS
• Systemic Lupus Erythematosis (SLE)
• Mixed Connective Tissue Disease (MCTD)
• Juvenile chronic arthritis
• Ehlers-Danlos
• Jaccoud arthropathy
UNKNOWN CASE #5: SYSTEMIC LUPUS ERYTHEMATOSIS

• Musculoskeletal changes
➢ Deforming nonerosive arthropathy
➢ Tendon rupture
➢ Avascular necrosis (AVN)
➢ Joint and bone infection
➢ Acrosclerosis
Musculoskeletal Seminar III 1016 Musculoskeletal Radiology

• 37 year old female with progressive ankle deformity
➢ What is the underlying disease?
➢ What process involves the ankle subsequently?
➢ How can the processes be correlated?
DIFFERENTIAL DIAGNOSIS – BENIGN POLYOSTOTIC LESIONS

• Hereditary multiple exostoses
• Paget disease
• Neurofibromatosis (Type 1)
UNKNOWN CASE #6: NEUROPATHIC JOINT

• Cause – pain sensation vs. neurovascular
• Destruction, debris, density increase, disorganization
• Diabetes, syphilis, cord-damage-syrinx

• Lytic expansile benign appearing polyostotic lesions
• Fibula, femur, metatarsal
• Subsequently ankle-fragmentation, debris, destruction,
increased density
UNKNOWN CASE #6: FIBROUS DYSPLASIA

• Endocrine Abnormalities
• Sexual precocity
• Cushings
• Acromegaly
• Hyperthyroidism 5 years after initial images
• Diabetes mellitus (hypothalamic dysfunction)
• Diagnosis case #6-fibrous dysplasia (polyostotic) with neuropathic ankle due to diabetes mellitus

Musculoskeletal Seminar IV
Mark D. Murphey, MD

• Several patients with various wrist subluxation patterns: Match with pattern
➢ Lunate
➢ Perilunate
➢ Barton fracture/subluxation
Musculoskeletal Seminar IV 1018 Musculoskeletal Radiology

UNKNOWN CASE #1: WRIST SUBLUXATIONS
• Perilunate – 75%, usually with transscaphoid fracture rest of carpus-dorsal
• Lunate – 25% lunate rotated volar, capitate remains aligned to radius
UNKNOWN CASE #1: WRIST SUBLUXATIONS

• Barton – fracture of dorsal rim of radius with dislocation of carpus
• Reverse Barton – fracture of volar rim of radius – with dislocation of carpus

• 31 year old female with underlying systemic disorder

• Dysplastic changes thoracolumbar junction
• Short segment scoliosis
• Posterior vertebral body scalloping
Musculoskeletal Radiology 1019 Musculoskeletal Seminar IV

POSTERIOR VERTEBRAL SCALLOPING
• Normal variant L4–5
• Neurofibromatosis (Type I)
• Tumor/increased intraspinal pressure
• Achondroplasia
• Acromegaly
• Ehlers-Danlos, Marfan , Osteogenesis Imperfecta
• Mucopolysaccharidosis

DYSPLASTIC THORACOLUMBAR JUNCTION
• Neurofibromatosis (Type 1)
• Cretinism
• Idiopathic
• Achondroplasia
UNKNOWN CASE #2: NEUROFIBROMATOSIS I

MUSCULOSKELETAL MANIFESTATIONS
• Cranium-enlarged empty orbit, left lambdoid suture defect
• Spine – scoliosis, posterior vertebral scalloping, lateral meningocoeles
• Pseudoarthrosis (tibia), bowing, fractures
• Ribbon ribs
• Neurofibroma – 5% malignant degeneration
• Localized gigantism
• Multiple nonossifying fibromas

• 13 year old boy with bilateral hip pain
T1

IRREGULAR EPIPHYSES (MULTIPLE)
• Normal variant
• Hypothyroidism
• Epiphyseal dysplasia
• Trevor disease
• Mucopolysaccharidosis

• Multiple irregular epiphyses
➢ Bilateral femora
➢ Right humerus
• Delayed skeletal maturation
• Changes of slipped capital femoral epiphysis (SCFE)
T2

UNKNOWN CASE #3: CAUSES OF SCFE
• Idiopathic
• Rickets-renal
• Trauma, obesity
• Hypothyroidism, hypoparathyroidism
• Radiation
UNKNOWN CASE #3:

MUSCULOSKELETAL CHANGES IN HYPOTHYROIDISM
• Delayed skeletal maturation, Wormian bones
• Epiphyseal dysgenesis with osteoarthritis
• Thoracolumbar junction gibbus
• SCFE; ligamentous laxity
• Osteoporosis; soft tissue calcification
• Soft tissue edema, carpal tunnel syndrome
UNKNOWN CASE #3: EPIPHYSEAL DYSGENESIS

• Ossification from multiple sites
• Femoral, humeral centers and talus
• Not due to vascular insufficiency
• May disappear with treatment
• May lead to premature osteoarthritis (OA)

• 45 year old man with wrist pain
UNKNOWN CASE #4:

DIFFERENTIAL DIAGNOSIS CHONDROCALCINOSIS
• CPPD deposition/arthropathy
• Hemochromatosis
• All others poor association

• Osteoarthritic changes – unusual locations radiocarpal and MCP
joints (2nd through 5th)
• Chondrocalcinosis – TFFC, no scapholunate separation
• Hook-like osteophytes metacarpal heads
UNKNOWN CASE #4: HEMOCHROMATOSIS -

MUSCULOSKELETAL CHANGES
• Osteoporosis
• Chondrocalcinosis (20–60%)
• Arthropathy (24–50%) looks like osteoarthritis
• Differences from CPPD arthropathy-involvement of 4th and 5th MCP
joints; hook-like osteophytes metacarpal heads; less scapholunate
separation; pericapitate narrowing

• 39 year old woman with low back pain
2 weeks prior to previous radiographs

UNKNOWN CASE #5: DIFFERENTIAL DIAGNOSIS -
NARROW DISK SPACE
• Degenerative disk disease (DDD); herniated disk (trauma)
• Inflammatory arthritis
• Scheuermann disease
• Osteomyelitis
• Neoplasm (very rare)
• Amyloid– chronic renal failure (CRF)
T1 T2

• Rapid disk space narrowing L2–3 over two week interval
• Subtle endplate destruction L2–3 level
• T1W-MR: marrow replacement L2–3 with disk involvement
• T2W-MR: increased marrow intensity L2–3 with disk involvement
UNKNOWN CASE #5: INFECTIOUS SPONDYLODISCITIS

• Usually starts in anterior subchondral bone then spreads rapidly to disk
• Bacterial vs. unusual cause (TB)
• Drug abusers predisposed

• 65 year old man with right pain

CHONDROID LESION
• Benign
➢ Enchondroma, bone infarct, chondroblastoma, chondromyxoid fibroma
(CMF), osteoblastoma
• Malignant
➢ Chondrosarcoma - intramedullary, juxtacortical, clear cell, mesenchymal,
myxoid, dedifferentiated, extraskeletal
T1 T2

• Lytic lesion proximal femur some areas of surrounding sclerosis
• Cortical permeation inferomedial on conventional tomography
• Matrix on CT and tomography-chondroid
• Soft tissue mass best seen on MRI
RADIOLOGIC DIFFERENTIATION OF
CHONDROSARCOMATOUS LESIONS
• Aggressive chondroid lesion with soft tissue mass
➢ High grade conventional chondrosarcoma
➢ Mesenchymal chondrosarcoma
• Large fluid component bone or soft tissue
➢ Myxoid chondrosarcoma
• Change in appearance or foci of more aggressive nature
➢ Diagnosis: Dedifferentiated chondrosarcoma

Musculoskeletal Seminar V
Mark D. Murphey, MD

• 60 year-old man with 6 months of knee pain
➢ Biopsied and diagnosed as myeloma.
➢ Is this a tenable diagnosis?
➢ What is the correct diagnosis and why was the initial pathology incorrect?
T1
T2 Fat Sat
T1 GD
T2 FAT SAT
UNKNOWN CASE # 1: FINDINGS

• Radiographs – Geographic 1A lesion with channel/tract like component
inferiorly (subtle)
• MRI – Marrow replacement T1W
➢ Rim enhancement (fluid filled mass)
➢ Homogeneous very high signal T2W
➢ Surrounding edema
➢ Tract like component inferiorly
• Differential diagnosis – UBC, ABC (no expansion) intraosseous hematoma,
ablated lesion, Brodie abscess
Musculoskeletal Radiology 1025 Musculoskeletal Seminar V

UNKNOWN CASE #1: SUBACUTE OSTEOMYELITIS BRODIE
ABSCESS
• Described in 1832 - chronic/subacute
• Walled-off with central fluid, often sterile (staph- only cultured in 50% of cases)
• Children (M>F), metaphysis, tibia
• Intramedullary; channel-like lucencies
• May cross growth plate or be cortical
• Periosteal reaction/sequestra may be seen
UNKNOWN CASE #1: BRODIE ABSCESS

• Biopsied at margin in reactive tissue
➢ Led to erroneous diagnosis
• Myeloma (untreated) not a tenable diagnosis
➢ asthis is a solid lesion
➢ Importance of radiologic/pathologic correlation

• 59 year old veteran involved in mild MVA (first film) with progressive pain
(second set of films 2 weeks later and MRI)
First Radiograph
Second radiographs 3 weeks prior to

first radiograph
T2
T1
Musculoskeletal Seminar V 1026 Musculoskeletal Radiology

• Relatively rapid destruction of shoulder
• Fragments in joint
• Sharp “surgical” margin
• MRI – replacement of humeral head with high intensity on T2W
• History of drained syrinx 40 years ago and cervical spine MRI shows severe
myelomalacia
UNKNOWN CASE # 2: NEUROPATHIC SHOULDER-SYRINX

• Cause – pain sensation vs. neurovascular
• Radiologic- destruction, debris, density increase, disorganization
• Diabetes, syphilis, cord damage - syrinx
UNKNOWN CASE # 3: HISTORY

• 15 year-old boy with hip pain
T1 STIR

• Marrow replacement right femoral neck T1W
➢ medial transcervical region
• High signal on STIR
➢ surrounding edema periosteum/ST
➢ horizontal low signal linear band medially
• Subsequent near total resolution
STRESS FRACTURE: FEMUR

• Medial femoral neck - fatigue type
➢ heal with symptomatic treatment (3 to 12 months)
➢ crescentic MR abnormality above lesser trochanter
• Lateral femoral neck - insufficiency type
➢ possible cause most subcapital fractures
➢ DO NOT HEAL: COMPLETE/DISPLACED
➢ Garden staging (< Grade 2 percutaneous pins) (> Grade 2 THA due to
development of AVN)
• Usually horizontal/oblique rarely longitudinal
➢ thigh splints (stress reaction)

• 23 year-old man with knee pain, masses and lesions on radiographs
➢ Diagnosis of bone lesions
➢ Underlying condition
T1
T1
T2
T2 WITH FAT SAT

NEUROFIBROMATOSIS 1: SKELETAL MANIFESTATIONS
• Mesodermal dysplasia
• Kyphoscoliosis
• Facial, orbital, lambdoid suture (left) defects
• Multiple nonossifying fibromas
• Meningocele
• Posterior vertebral scalloping
• Rib deformity (ribbon ribs)
• Congenital pseudarthrosis (tibia)
• Focal hypertrophy (gigantism)
• Localized neurofibroma - most common
➢ least characteristic, often deep/multiple
➢ superficial lesions (fibroma molluscum)
• Plexiform neurofibroma - pathognomonic
➢ early childhood
➢ precedes cutaneous neurofibromas

• 53 year-old man with lateral knee mass and pain
T1 STIR
STIR

PROTON DENSITY
STIR

• Tibiofibular/lateral tibiofemoral joint osteoarthritis
• High fluid content multilocular mass laterally
➢ multilocular / surrounding edema
➢ appears to arise from tibiofibular joint
➢ components in bone (tibia and femur) and soft tissue
• D/DX -- Ganglion/Synovial cyst, myxoid tumor

GANGLION/SYNOVIAL CYST
• Etiology unknown- neoplasm, trauma, inflammatory
• Young adults-most common mass hand/wrist
• Pain-may affect adjacent nerves
• Location
➢ ST: Hand, foot, knee, hip, shoulder
➢ Intraosseous: medial malleolus, wrist, knee
➢ BOTH
• Thick walled unilocular/multilocular
➢ high protein content affects CT/T1W MR
➢ walls/septa may enhance
• CT/MRI/Sono - cystic mass
➢ may rupture cause surrounding edema

• 47 year-old woman with mid to low back pain
➢ Most likely diagnosis?
➢ Two other possible diagnoses?
T1 T2 T2
STIR

• Multifocal bone scan areas increased radionuclide activity
➢ spine, SC joints, SI joints
• CT-multifocal sclerosis
➢ anterior/posterior paralleling endplates
➢ erosions/bone production costovertebral joints
➢ cause of hot bone scan
• MRI - multifocal areas marrow abnormality
➢ low T1W, high T2W/STIR
➢ anterior/posterior paralleling endplates
➢ no soft tissue mass
• Radiographs- subtle sclerosis, sacroiliitis
• D/DX - Metastases, myeloma, lymphoma

ANKYLOSING SPONDYLITIS: CLINICAL CHARACTERISTICS
• Peak age of onset 15-35 years
• M:F 3-5:1
• Incidence 6.6/100,000
• HLA-B27 > 90%
• Rare in blacks
• Predilection axial involvement
ANKYLOSING SPONDYLITIS: DISTRIBUTION

• Osseous ankylosis
• Ligament/Tendon ossification
• Spine/SI joints
➢ symmetric
• Pelvis - symphysis, ischium, iliac, hips
• Peripheral changes unusual early (10% - 50%)
➢ asymmetric
ANKYLOSING SPONDYLITIS: SPINE CHANGES

• Osteitis -”shining corners”
• Squared vertebral bodies
• Syndesmophytes
• Bamboo spine
• Calcified disc, fused facets/ligaments
• Pseudarthrosis/fractures
• Atlantoaxial disease/Cauda equina
• Other diagnoses- Reiter/Psoriatic, SAPHO

Radiologic Pathology 2006-2007 - Volume 2 - Index
25-OH Vitamin D 902 Angiomatous lesions 1017

Abscess 796 Angiosarcoma 721, 805, 810
Soft Tissue Infection 827 Ankle 879, 995
Subperiosteal 823 Fractures 1001
Access. Navicular 880 Ankylosing Spondylitis 912, 916, 918, 1033
Accessory Muscles Masses (Ankle and Foot) 886 Ankylosis 914
Accessory Soleus 886 Anterior Dislocation (Glenohumeral) 942
Acetabulae protrusio 816 Anterior Drawer 867
Acetabular Anterior Instability (Glenohumeral) 933
Columns Letournel and Judet 998 Anterior talofibular 881
Injury 998 Anterior tibiofibular ligament 880
Trauma 995 Anterolisthesis 840
Acetabulum 998 Anteroposterior (AP) compression (Pelvic Trauma) 996
Achilles Tendon 883 Arthritis 795, 1006
Achondroplasia 1020 Juvenile Chronic 919
ACL 867 Psoriatic 916
Post-operative 868 Septic 825
Tear 866, 867 Tuberculous 832
Acromegaly 773 Articular cartilage 859
Acromial Variation 927 Aseptic necrosis 906
Acromio-clavicular Joint Injuries 941 Aspergillosis 835
Acromion 942 Aspirin/nonsteroidals (Osteoma) 746
Acroosteolysis 983, 1007, 1008 Atlantoaxial subluxation 915
Actinomyces 833 Atlas Fractures 843
Actinomycosis 829, 833 Atypical Mycobacterium 833
Active infection 825 Autograft Healing 706
Acute Osteomyelitis 821 Avascular Necrosis 719, 959, 1010, 1020
Adamantinoma 721, 771, 776 Aviator’s astragulus 1002
Adductor pollicis 947 Avulsion 865
Adenomatoid odontogenic tumor 855 Avulsive cortical injury 777
Adhesive Capsulitis 931 Axial Compression Injury 846
Adolescent Stress Lesions 994 Axial Osteomalacia 904
Adult Palmar Fibromatosis 779 Axillary Nerve Neuropraxy 935
Adult Plantar Fibromatosis 780 Axillary View 942
Aggressive “Malignant” Osteoblastoma 750 Bacillary angiomatosis 837
Aggressive infantile Fibromatosis 778 Ballooned epiphyses 920
Aggressive Osteoblastoma 750 Bamboo spine 918
Alcoholism 907, 953 Banana fracture 816
Alkaline phosphatase 812 Bankart Lesion 932
Alkaptonuria 949, 953 Bankart Repair 938
Allograft 707 Barton Fracture (Reverse) 945
ALPSA Lesion 934 Baseball Finger 948
Aluminum Toxicity 716 Basilar invagination 816, 901
Ameloblastic fibroma 852 Bathrocephaly 1008
Ameloblastic fibro-odontoma 857 Batson’s plexus 961
Ameloblastoma 852, 854 Benign Bone Tumors: Age Distribution by Decade 723
Amyloid 795 Benign Fibrosis Histiocytoma 773
Amyloidosis 716, 1006 Benign Polyostotic Lesions 1017
B2 – microglobulin 716 Bennett Fracture 947
Carpal tunnel syndrome 716 Biceps Injury 957
Destructive spondyloarthropathy 716 Partial Tears 957
Discovertebral erosions 717 Tendinosis 957
Anatomy of the tarsal joints 1003 Biceps Femoris 869
Aneurysm 804 Biceps Tendon 929
Aneurysmal Bone Cyst 749, 784, 787 Bicipital Radialis Bursitis 957
Angioblastoma 776 Bilateral Facet Dislocation 842
Angiomatoid 780 Birbeck bodies (Eosinophilic Granuloma) 890
Angiomatosis 805, 808 Blade of grass 813
I1
Blastic Disease 814 Calcific
Blastic Lesions 967 Myelitis 792
Blastic Phase 812 Tendinitis 952
Blastomycosis 835 Tendonitis 930
Blount 911 Tendonitis (Glenohumeral) 943
Blow out lesions 967 Calcified falx cerebri 853
BMD 978 Calcified Soft Tissue Mass 1012, 1015
BMD (WHO Classification) 979 Calcinosis 984
Bohler 1003 Calcitonin 818
Bone Autograft 706, 707, 710 Calcium deficiency 977
Bone bruise 865, 879, 958 Calcium hydroxyapatite 718, 792, 949
Bone enlargement 814 Cancellous (Osteoid Osteoma) 746
Bone Graft Complications 708 Candidiasis 835
Fracture 709 Candle flame 813
Joint Instability 709 Cap thickness 758
Nonunion and Pseudarthrosis 708 Capillary hemangioma 809
Resorption 709 Capitate (Dislocation) 946
Bone Graft Substitutes 710 Capitellum 955
Bone Infarct 769, 906, 1024 Capsulorapphy 938
Osteonecrosis 769 Carcinomatosis 971
Bone Island 743 Carpal Dislocations 946
Bone Metastases 961 Carpal Stability 874
Bone Production 914 Carpal Tunnel 872, 876
Bone scan 744 Syndrome 877
Bone Tumors 720 Cartilage metaplasia 799
Cartilage 720 Cartilage nodules 799
Histiocytic 721 Cartilaginous Lesions 757
Marrow 720 Chondroblastoma 757
Notochord 721 Chondromyxoid fibroma 757
Osteoid 720 Chondrosarcoma 757
Unknown Origin 721 Enchondroma 757
Vascular 721 Juxtacortical chondroma 757
Bone Tumors (Incidence) 722 Osteochondroma 757
Important Factors in the Diagnosis of 723 Caseating necrosis 830
Primary Benign 722 Cavernous hemangiomas 761
Primary Malignant 722 Cavernous spaces 806
Bone within Bone 970 Cellulitis 826
Botryoid odontogenic cyst 852 Cementoblastoma 854
Bouchard nodes 923 Central giant cell granuloma 852
Bowing of long bones 901 Cervical Burst Fracture 846
Boxer’s Fracture 948 Cervical Spine Trauma 839
Brachial artery 944 Cervical spondylosis 848
Brachial nerve injury 944 Charcot joint 953
Breast Carcinoma 962 Chondroblastoma 720, 1008, 1024
Bristow procedure 938 Codman Tumor 763
Brodie abscess 748, 824, 1007, 1026 Chondroblasts 764
Brown tumor 711, 853, 1013 Chondrocalcinosis 950, 1021
Brucella 829 Chondroid 738
Brucellosis 829 Chondroid Lesion 1024
Bucket handle 861, 862 Chondroid Matrix (Enchondroma) 761
Bumpy (Soft Tissue Swelling) 912 Chondroid Matrix (Intramedullary Chondrosarcoma) 765
Bursae (Knee) 870 Chondromyxoid Fibroma 720, 1024
Bursitis (Bicipital Radialis) 957 Chondrosarcoma 762, 764, 1024
Bursitis (septic) 826 Dedifferentiated 769
Burst Fracture 846 Extraskeletal 768
Button osteophyte 922 Mesenchymal 768
Cafe-au-lait spots 773, 981 Myxoid 768
Calcaneal Fractures 1002 Chondrosarcomatous Lesions 1024
Calcaneofibular 881 Chordoma 721, 786
Calcaneus 995 Christmas disease 972
Chronic ACL Tear 867
Chronic Granulomatous Disease of Childhood 827
I2
Chronic hematoma 796 Decubitus ulcers 825
Chronic infection 818 Dedifferentiated Chondrosarcoma 764, 769
Chronic Osteomyelitis 825, 852 Deep Endosteal Scalloping 765
Chronic Recurrent Multifocal Osteomyelitis (CRMO) 827 Deforming nonerosive arthropathy 983
Chronic regional pain syndrome 976 Degenerative Joint Disease 951
Chronic renal failure 793 Dental Anatomy 849
Chronic sclerosing osteomyelitis 856 Dentigerous cyst 852
Chronic symmetric plasma cell osteomyelitis 827 Dentition 849
Clavicle Deoxyhemoglobin 848
Clay Shoveler Fracture 843 DeQuervain’s Syndrome 876
Clear Cell Chondrocytes 767 Dermatofibrosarcoma Protuberans (DFSP) 771, 781, 782, 802
Clear Cell Chondrosarcoma 763, 764, 767, 1008 Dermatofibrosis lenticularis disseminata 744
Clear Cell Sarcoma 792, 803 Dermatomyositis 976
Cleidocranial dysplasia 1007 DeSmet 860
Clutton joints 834 Desmoid 746
Coach’s Finger 948 Extraabdominal 779
Coccidioidomycosis 835 Desmoplastic fibroma 771, 777
Codman Tumor 763 DEXA 978
Collagen Vascular Diseases 793, 907, 1012 Diabetes 825
Collagen vascular-like diseases 976 Insipidus 892
Collateral ligament 869 Mellitus 773, 825, 953
Collateral ligaments 859 Diffuse sclerosis 1010
Colles fracture 945 Diphoshonates 818
Column Fracture (Pelvis) 1000 Direct implantation 820
Complete pelvic instability 997 Discitis 828
Compression Fracture 841 Discography 828
Congenital insensitivity to pain 953 Discoid 860
Congenital Syphilis 834 DISI deformity 874
Contiguous spread 820 Distal radial buckle fracture 945
Contusion 865 Distal radioulnar joint 872
Cooley’s anemia 970 Distal Tuft Fracture 948
Coracoid 942 Disuse/Immobilization Osteoporosis 977
Coronoid fossa 955 Double Axillary Pouch Sign 934
Cortical Double line sign 908
Osteoid Osteoma 746 Doughnut sign 785
Desmoid 777 Drug Abusers 827
Fibrous Dysplasia 775 Du Toit & Roux 938
Involvement 738, 739 Dual Energy X-ray Absorptiometry (DEXA) 978
Lucency/Central Calcification 1005 Dupuytren Exostosis 759
Resorption 712 Durie/Salmon PLUS Staging (Myeloma) 965
Thickening (Chondrosarcoma) 765 Dysbaric disorders 907
Cotton wool 814 Dysplasia Epiphysealis Hemimelica: Trevor Disease 759
CPPD 718, 949 Dysplastic Thoracolumbar Junction 1020
Arthropathy 792 Dysprosium 165 795
Deposition/arthropathy 1021 Early focal cemento-osseous dysplasia 850
Cranial Sclerosis 744 Echinococcus 837
Craniotabes 901 ECU tendon sheath 875
CREST Syndrome 984 Ehlers-Danlos 1016
Cretinism 1007, 1020 Elbow 955
Crohn's disease 912 Dislocations and Fractures 944
Cruciate ligaments 859 Embolization 748, 808
Cryptococcosis 836 Enchondroma 720, 760, 769, 1024
Crystal deposition (Thalassemia) 971 Enchondroma vs. Chondrosarcoma 769
Crystal Deposition Disease 718, 949 Enchondromatosis 1017
Cushing syndrome 773 Endocrinopathies 773
Cyst (Paralabral) 938 Endodontic procedures 854
Cystic hygroma 808 Endosteal scalloping 770
Cystic Masses (Knee) 870 Enostosis 720, 743
Cysticercosis 837 Enteropathic arthritis 912
Dactylitis 830, 834, 969, 1010 Enthesopathy 914
Dagger sign 918 Enucleation 852
Danis-Weber Classification 1001
I3
Eosinophilic Granuloma 721, 887, 890, 967 Focal cemento-osseous dysplasia 855, 857
LCH 1008 Focal scerosing osteomyelitis 854
Epicondylitis (Elbow) 956 Foot 879, 995
Epidermoid Carcinoma 826, 834 Injuries 1003
Epidermoid Inclusion Cyst 784, 790 Fracture (First MC) 947
Epidural hematoma 848 Fractures 816
Epiphyseal Lesions 1008 Fractures (Pathologic) 961
Dysgenesis 1021 Frieburg 911
Dysplasia 1020 Full thickness (Rotator Cuff Tear) 928
Epiphysis 767 Galeazzi 945
Epithelial nests 776 Ganglia 870
Epitrochlear Lymph Node 960 Ganglia (Knee) 870
Erlenmeyer flask deformity 971 Ganglion 792
Erosions 913 Ganglion/Synovial Cyst 1031
Erosive Osteoarthritis 917 Ganglion/synovial cyst/bursa 796
Erupting teeth 854 Gardner Syndrome 852, 856
Essex-Lopresti 945, 1003 Gardner Syndrome (Osteoma) 746
Ewing Sarcoma 721, 887, 964, 967 Gastrocnemius 869
Ewing Sarcoma (Intergroup Study) 888 Gastrocnemius/ Semimembranosus 870
Exophthalmos 892 Gaucher disease 818, 907, 971
Exostoses 856 GCT 769
Exostosis (Subungual - Dupuytren Exostosis) 759 Geodes 790
Extensor Carpi Ulnaris Sheath 875 Geographic Contusion (Knee) 866
Extensor tendons 859 Geographic Pattern (Bone Tumors) 725
Extent of Musculoskeletal Neoplasm 738 Geographic 1A: Differential Diagnosis 725
Extra-abdominal Desmoid Fibromatosis 778 Geographic IB: Differential Diagnosis 726
Extra-articular erosions (Gouty Arthritis) 950 Geographic IC: Differential Diagnosis 726
Extramedullary Hematopoiesis 971 Giant Bone Island 743
Extraskeletal Chondrosarcoma 764, 768 Giant Cell (Reparative) Granuloma 780
Facet 840 Giant Cell Tumor 721, 784, 1008
Fallen fragment sign 787 Giant Cell Tumor Tendon Sheath (GCT-TS) 794
Familial vitamin D res rickets 903 Giant Cells 784
Fanconi syndromes 903, 968 Gigantism 1029
Fanconi’s Anemia 972 Glad Lesion 936
Felon 825 Glenohumeral 932
Felty Syndrome 914 Instability 932
Femur 995 Joint 932
Fibrocartilage Calcification 951 Ligaments 932
Fibroma molluscum 1029 Glenohumeral Internal Rotation Deficit (GIRD) 936
Fibromas 746 Glenohumeral Labroligamentous complex 932
Fibromatosis 771, 778 Glenohumeral ligament (avulsion) 935
Fibromatosis: Types 778 Glenoid labrum 932
Fibrosarcoma 771, 780, 781, 802 Glomus 721
Fibrosarcoma/MFH 748 Glomus Tumor 805, 808
Fibrosis Histiocytoma 773 Gnathic Osteosarcoma 754
Fibrous cortical defect 771 Golfer’s elbow 956
Fibrous dysplasia 771, 773, 856, 944, 1017 Goltz syndrome 744, 784
Fibrous Histiocytoma (Malignant) 721 Gorham 809
Fibrous medullary defect 771 Gorlin syndrome 853
Fibroxanthoma (Nonossifying fibroma) 771 Gout 718, 817, 949, 1010, 1012
Fibular (lateral) 869 Gouty arthritis 949, 950
Fibular collateral lig complex 881 Gouty tophus 804
Fibular collateral ligament 867 Gracilis 869
Filariasis 837 Granulomatous Disease of Childhood 827
Finger (Trauma) 948 Grashey view 942
First MC (Fracture) 947 Greater Tuberosity Fracture 935
Flexion Teardrop Fracture 843 Ground glass 774
Flexor Digitorum (Avulsion) 948 Group B strep 822
Flipped (meniscal tear) 862 Guinea worm (dracunculosis) 837
Florid cemento-osseous dysplasia 856 Gumma 834
Fluid - fluid level 764, 785, 802 Guyon’s canal 872, 877
Fluorosis 818 HA Crystal Deposition Disease 952
I4
Hagl Lesion 935 Hypercementosis 854
Hajdu-Cheney Syndrome 1008 Hyperextension 840
Hallux 924 Hyperextension Dislocation 844
rigidus 924 Hyperextension Injuries 843
valgus 924 Hyperextension: Teardrop Fracture 845
Hamartoma 743 Hyperflexion 840
Hamate Fracture 947 Hyperflexion Injuries 840
Hand-Foot syndrome 969 Hyperflexion Sprain 840
Hand-Schüller-Christian disease 887, 892 Hyperparathyroidism 711, 773, 852, 856, 950, 971, 1007,
Hangee Fracture 845 1012, 1021
Hangman Fracture 845 Hyperthyroidism 773
Hanover 1003 Hypertrophic Osteoarthropathy 962
Hawkins classification of talar neck fractures 1002 Hypophosphatasia 904, 1007
Hawkins sign 1002 Hypothyroidism 1020, 1021
Heberden nodes 923 Idiopatic osteosclerosis 854
Hemangioendothelioma (HE) 721, 805, 810 Ifosfamide 903
Hemangioma 721, 804, 805, 1012 Iliotibial band 867
Arteriovenous 805 Iliotibial tract 869
AV malformation 852 Imaging for Staging Musculoskeletal Neoplasm 738
Capillary 805, 809 Immature cementoblastoma 850
Cavernous 805 Immature periapical cemental dysplasia 850
Venous 805 Impingement Syndrome 927
Hemangiopericytoma (HPC) 721, 768, 805, 810 anterolateral 881
Hematogenous Osteomyelitis: Adult 824 Infantile dermal/digital fibromatosis 778
Hematogenous Osteomyelitis: Child 822 Infantile myofibromatosis 778
Hematogenous Osteomyelitis: Infant 822 Infarction 969
Hematogenous Vascular Supply 820 Infection 1006
Hematologic Disease 968 Infectious Spondylodiscitis 1023
Hemochromatosis 949, 950, 952, 1021 Inferior Glenohumeral Labroligamentous complex 932
Hemodialysis elbow 718 Inferior Glenohumeral Ligament 933
Hemoglobinopathy 907 Inflammatory 780
Hemophilia 968, 972 Infrapatellar 870
Hemophiliac pseudotumor 973 Infrapatellar cleft 870
Hemorrhage 781, 790 Insufficiency fractures 979, 983
Hereditary Hyperphosphatasia: Juvenile Paget Disease 818 Interbody Fusion 710
Hereditary multiple exostoses (HME) 757, 760, 1017 Intermedius 869
Heterotopic Bone Formation: Myositis Ossificans 800 Interosseous ligament 880
High - Grade Surface 754 Interosseous syndrome (Elbow) 956
High output congestive failure 812 Interspinous widening 840
Hill-Sachs Lesion 934, 943 Intertrochanteric Fractures 980
Hip Intraarticular Bodies (Elbow) 956
Fractures 1000 Intra-Articular Hydroxyapatite Crystal Deposition Disease
Joint instability 999 952
Trauma 1000 Intracortical fibrous dysplasia 775
Histoplasmosis 836 Intramedullary (Chondrosarcoma) 764
HLA B27 916 Intramedullary Extent 738
Hodgkin 966, 971 Intramedullary Hemorrhage 848
Hook of hamate 877 Intranuclear inclusions 812
Hoop stresses 860 Intraosseous ganglion 784, 790, 1008
H-Shaped Vertebral Bodies 970 Intrasubstance Tear (Rotator Cuff) 929
H-type vertebrae 1010 Invisible Margin (Bone Tumors) 728
Human/animal bites 825 Involucrum 820
Humeral Fractures 943 Irradiation 907
Humphrey 859 Irregular Epiphyses (multiple) 1020
Hutchinson triad 834 Isolated Fractures Radius: Galeazzi 945
Hyaline cartilage cap 757, 758 Isolated Fractures Ulna: Monteggia 945
Hyaluronic acid 795 Isolated Tendon Injuries 948
Hydrops Fetalis 808 ITB Friction Syndrome 869
Hydroxyapatite 715 Ivory vertebra 814, 962
Hydroxyapatite Crystal Deposition 952 Differential Diagnosis 962
Hydroxyproline 812 Jaccoud arthropathy 1016
Hygroma 808 Jaffe-Campanacci syndrome 773
I5
Jaws 849 Laminar Fractures 843
Jefferson Fracture 846 Langerhans Cell Histiocytosis (LCH) 748, 887, 890, 1013,
Jersey Finger 948 1008, 1017
Joint Arthroplasty (Complications of ) 699 Laser therapy 808
Dislocation / Abnormal Alignment 702 Lateral compression (Pelvis Trauma) 996
Fractures and Nonunion 702 Lateral Epicondylitis (Elbow) 957
Heterotopic Bone Formation 699, 703 Lateral inferior geniculate artery 863
Loosening and/or infection 699 Lateral Meniscus 859
Radionuclide Evaluation 700 Lateral Process of Talus Fractures 1002
Small Particle Disease 699, 701, 702 Lateral Stabilizers 869
Joint involvement 738, 739 Lateral Tendons (Ankle and Foot) 884
Abscess 741 Lateral Ulnar Collateral Ligament 959
Bursitis 741 Lauge-Hansen 1001
Diabetic muscle ischemia 741 LCL-Biceps Femoris 870
Fibromatosis 741 Ledderhose disease 780
Gadolinium 742 Legg-Calvé-Perthes 911
Hematoma 741 Leiomyosarcoma 781, 802
intraarticular resection 739 Leprosy 829, 833, 953
Lymphocele 741 Lesion matrix 738
Muscle flap 741 Letournel and Judet classification 998
Myositis ossificans 741 Letterer-Siwe disease 887, 892
Myxoid liposarcoma 741 Leukemia 971
Post-Operative Imaging (Bone Neoplasm) 741 Ligament and Tendon Involvement 739
Radiation necrosis 741 ligament(ous) injury 840
Reactive lymph node 741 Limb Salvage Procedures 737
Seroma 741 Lipoblastoma 896
Soft Tissue Mass - Benign 740 Lipoma 720, 804, 1015
Soft Tissue Mass - Malignant 740 Lipoma Arborescens 798
Subtraction MRI 742 Lipoma Intramuscular 895
Synovial cell sarcoma 741 Lipomatosis 897
Joint Replacement 699 Liposarcoma 720, 781, 802, 893, 897, 1015
Jones Fracture 1003 Atypical 898
JRA 919 Dedifferentiated 898
Polyarticular 920 Higher Grade Lesions 898
Still Disease: Pauci or Monoarticular 920 Myxoid Lesions 898
Jumpers Knee 870 Pleomorphic Liposarcoma 899
Juvenile aponeurotic fibroma 778 Well-Differentiated 897
Juvenile Chronic Arthritis 912, 919, 973, 1016 Loa loa 837
Juvenile Paget Disease 818 Location in Bone: Axial (Bone Tumors) 724
Juvenile-onset adult type RA 920 Locked facets 842
Juvenile-onset ankylosing spondylitis 919 Long Head of Biceps Tendon 929
Juxtaarticular Osteoporosis 912 Long Head of the Biceps Tendon 932
Juxtacortical Chondroma 720, 762, 767 Longitudinal Tibial Stress Fracture 992
Juxtacortical Chondrosarcoma 767 Loose Bodies 871
Juxtacortical Osteosarcoma 752 Looser zones (Osteomalacia) 715
Kaplan 860 Looser’s zones 902
Kaposi sarcoma 837 Lower Extremity Trauma 995
Kasabach - Merritt 809 Low-Grade Chondroid Lesion 769
Keloid formation 744 Low-Grade chondrosarcoma 769
Keratocyst 852 Lumpy 912
Kienböck 911 Lunate 946
Kienböck’s Disease 911 Lung Cancer 963
Klippel-Trenaunay-Weber 809 Lunotriquetral Instability 875
Knee 995 Lyme disease 834, 835
Knee Injury 1001 Lymphangioma 721, 805
Knee Stabilizers 867 Lymphoma 720, 748, 961, 964
Köehler 911 Lymphoma of Bone (Primary Lymphocytic) 966
Kohler Disease 1006 Lytic Patterns (Bone Tumors) 728
Kyphosis 830, 840 lytic phase 812
Labral Repair 938 Lytic Phase 813
Labrum 932 Lytic Skull Lesions 1013
Lamina dura 850 Macho-Macho 852
I6
Madura Foot 837 Multiple enchondromatosis 760, 761
Maffucci syndrome 760, 761, 809 Multiple Myeloma 964
Magic Angle Phenomenon 876 Multiple myeloma with sclerosis or POEMS syndrome 965
Magnuson Stack 938 Multiple tori and exostoses 856
Malignant Bone Tumors: Age Distribution by Decade 723 Musculoskeletal Infection 820
Malignant Fibrous Histiocytoma (MFH) 721, 771, 780, 781 Musculoskeletal Neoplasm - Extent 738
Malignant melanoma of soft parts 803 Musculoskeletal Neoplasm - Staging - Surgical Implications
Malignant myositis 801 737
Malignant transformation (Multiple Enchondromatosis) 761 Musculoskeletal Tumors - Staging 734
Mallet Finger 948 Histologically Benign 735
Malnutrition 977 Histologically Malignant 736
Marrow Edema (Osteoid Osteoma) 747 Mycetoma 837
Marrow hyperplasia 969 Mycobacteria 829
Massive Osteolysis of Gorham 809 Mycobacterium 833
Mastocytosis 818 Mycobacterium Leprae 833
Matrix Formation 723 Myelitis (Calcic) 792
Matrix Formation (Bone Tumors) 729 Myelofibrosis 818, 968, 971, 974
Mazabraud syndrome 773 Myelography 840
McCune Albright syndrome 773 Myeloma 720, 961, 964, 1013
Medial Collateral Ligament (MCL) 867 Myeloma/plasmacytoma 786
Injuries 868 Myelomalacia 848
Medial Collateral Ligament (Elbow) 959 Myeloproliferative diseases 949
Medial Meniscus 859 Myositis 837, 983
Medial migration (Osteoarthritis: Hip) 923 Myositis Ossificans 792, 800, 1012
Medial Tendons (Ankle and Foot) 884 Myotendinous (Rotator Cuff Tear) 928
Median nerve 872, 877 Myotendinous Tear of Pectoralis 931
Impingement 956 Myxoid 780
Melorheostosis 744 Myxoid Chondrosarcoma 764, 768
Meningoceles 983 Myxoma 773, 792
Meningomyelocele 953 Myxomatous neoplasms 796
Meniscal Cyst 798, 870 Narrow Disk Space 1023
Meniscal Flap 862 Nasopalatine duct cyst 850
Meniscal tears 798, 858, 861 Navicular 880
Menisci (Calcification) 951 Neck of the scapula 942
Menisci (Post-surgical) 863 Necrosis - Avascular 719
Meniscofemoral ligaments 863 Neoplasm (Paget Disease) 817
Meniscus homologue 875 Nerve Impingement (Elbow) 956
Mesenchymal cells 768 Nerve root avulsion 840
Mesenchymal Chondrosarcoma 764, 768 Neuroarthropathy 953
Metabolic Bone Disease 900 Neuroblastoma 963, 964, 967
Metachronous Osteosarcoma 750 Neurofibromatosis (NF) 976, 981
Metaphyseal chondrodysplasia 904 Neurofibromatosis (Type 1) 773, 1017, 1020, 1029
Metastases 748, 768, 1013 Neurogenic tumor 786
Skeletal 961 Neuropathic 834
Metastatic lymphoma 967 Joint 1017
Methemoglobin 848 Osteoarthropathy 949, 953
MFH (Malignant Fibrous Histiocytoma) 802 Shoulder-Syrinx 1027
MFH/ fibrosarcoma 769 Neurovascular involvement 738, 739
Middle Glenohumeral Ligament 933 Nevoid basal cell carcinoma syndrome 853
Milk - Alkali syndrome 793 NF (Neurofibromatosis) 981
Milwaukee Shoulder 952 NF-1 (vonRecklinghausen’s) 981
Mithramycin 818 NF-2 - Acoustic neuromas 981
Mixed Connective Tissue Disease (MCTD) 1016 Nidus (Osteoid Osteoma) 746
Mixed/Blastic Disease 814 Night Stick Fracture 944
Monostotic 773, 812 Nocardia 833
Monteggia 945 Nodular Fasciitis 792, 801
Morton’s Neuroma 885 Non-Hodgkin 966
Motheaten (Bone Tumors) - Differential Diagnosis 727 Non-Insertional Achilles Tendon Pathology 883
Mucoepidermoid carcinoma 852 Nonossifying fibroma (NOF) 771, 981
Mucopolysaccharidosis 1020 Nonosteogenic fibroma 771
Mucormycosis 836 Nonspecific spindle cell sarcoma 781, 802
Multidirectional Instability (Glenohumeral) 939 O’Donoghue’s Triad 867
I7
Oblique meniscomeniscal ligament 863 Osteosarcoma 743, 750, 751, 769, 857, 1012
Occult fracture 865 Intramedullary 751
OCD 955 Juxtacortical 752
Odontogenic cyst 852 Parosteal 753
Odontogenic keratocyst 852 Telangiectatic 752
Odontogenic myxoma 852 Osteosarcoma (Sclerosing) 754
Odontoid Fracture 847 Osteosarcoma: Low Grade Intramedullary 754
Odontoma 855, 857 Osteosarcoma: Soft Tissue 755
OI (Osteogenesis Imperfecta) 980 Extraskeletal 755
Olecranon bursitis 949, 958 Osteosarcoma : Intracortical 756
Olecranon fossa 955 Osteosarcomatosis 755
Ollier disease 760 Osteosclerosis 711, 714
Ollier Syndrome 762 Overhanging edge 914
Oncogenic osteomalacia 773 Overhanging edge (Gouty Arthritis) 950
Os Acromiale 928, 942 Oxalosis 718
Os odontodeum 847 Oxyhemoglobin 848
Os Trigonum 880 P V N S 793
Osgood-Schlatter 911 Paget Disease 812, 1013, 1017
Osler-Weber-Rendu 809 Paget’s disease 856
Osseous bowing 812 Pain (Congenital Insensitivity) 953
Osseous deformity 816 Palmar Fibromatosis 779
Osseous Lesions both Sides of Joint 1006 Palmer 1003
Osseous Neoplasm 733 Pancreatitis 907
Ossicle 860 Panne 911
Ossifying fibroma 775, 855, 857 Panner’s disease 959
Osteitis deformans 812 Paralabral Cyst 937
Osteoarthritis 920 paramyxovirus (measles) 812
Osteoarthritis (secondary) 826 Parathormone (PTH) 711
Osteoarthropathy (Hypertrophic) 962 Paravertebral soft tissue 828
Osteoarthropathy (Neuropathic) 949, 953 Paronychia 825
Osteoblastic Metastasis 743 Parosteal Osteosarcoma 753
Osteoblastoma 720, 743, 748, 763, 855, 857, 1008, 1024 Parrot Beak 862
Osteochondral fracture 879 Parsonage Turner Syndrome 958
Osteochondral Lesion 879 Partial thickness (Rotator Cuff Tear) 928
Osteochondritis 834 Patellar Dislocation 866
Osteochondritis Dissecans 799, 865, 879, 910 Patellar Retinacula 867, 869
Osteochondroma 720, 757 Patellar Tendinitis 870
Osteochondroses 911, 1006 Patellar tendon 869
Osteochondrosis 906 Patellofemoral Joint 870
Osteoclast 962 Patellofemoral Syndrome 870
Osteofibrous dysplasia 771, 775, 776 Pathologic Fractures 961
Osteogenesis Imperfecta (OI) 976, 980, 1007 Pattern of Bone Destruction and Lesion Margin 725
Osteoid 738 Geographic 725
Osteoid Osteoma 720, 743, 746, 748, 763, 1005, 1008 Motheaten 725
Osteolysis (Post-Traumatic - Clavicle) 941 Permeative 725
Osteolysis of Gorham 809 Transition Zone 725
Osteoma 720, 743, 745, 857 PCL 867, 868
Osteomalacia 711, 714, 809, 900 PCL Tear 868
Osteomyelitis 718, 748, 821, 824, 825, 827, 830, 856, 967, Pectoralis Major Tear 931
1026 Pedicle erosion 981
Osteomyelitis (salmonella) 1010 Pedicle sclerosis 749
Osteonecrosis 769, 906, 999 Pedicolaminar Fracture-Separation 844
Osteonecrosis (Spontaneous) 910 Pelvic Ring Disruption 998
Osteopathia striata 744 Pelvic Ring Fractures 995
Osteopenia 714, 900, 979, 1010 Pelvic Stress Injury 993
Osteopetrosis 856 Pelvis Trauma 995
Osteophyte (button) 922 Pencilling (Long Bones) 981
Osteophyte formation 921 perched facets 842
Osteopoikilosis 744 Percutaneous ablation (radiofrequency) 748
Osteoporosis 711, 900, 976, 979, 1008 Percutaneous removal (Osteoid Osteoma) 748
Osteoporosis (transient) 909 Embolization 748
Osteoporosis circumscripta 813 Radiofrequency 748
I8
Periapical cemento-osseous dysplasia 854 Posterolateral Rotatory Instability (Elbow) 960
Periapical cyst 850 Post-traumatic cyst 784, 790
Periapical granuloma 850 Pregnancy 907
Periarticular Calcification 715 Prepatellar 870
pericapsular fat planes 826 Primary Lymphocytic Lymphoma of Bone 966
Periodontal ligament 850 Primary Lymphoma 966
Periosteal Osteosarcoma 753, 1014 Primary Periarticular HA Crystal Deposition Disease:
Periosteal reaction 723 (Calcific Tendinitis) 952
Periosteal Reaction (Intramedullary Chondrosarcoma) 765 Prostaglandin (Osteoma) 746
Periosteal reaction (PTH) 711 Prostate Carcinoma 962
Periosteal Reaction: Aggressive 731 Pseudarthrosis (Tibia) 981
Codman triangle 731 Pseudogout 950
Hair-On-End 731 Pseudomonas 821
Laminated 731 Pseudotumor (Hemophiliac) 973
Sunburst 731 Pseudoxanthoma Elasticum 792
Periosteal Reaction: Nonaggressive 730 Psoriasis 916
Buttressing 730 Psoriatic arthritis 912, 916, 1009
Expansion 730 Psoriatic Sacroiliitis 1010
Septation 730 Pubic fracture 998
Solid 730 Pulley Injuries 948
Periosteal/juxtacortical (Chondrosarcoma) 764 Puncture wounds 825
Periostitis 914 Putti Platt 938
Permeative (Bone Tumors) - Differential Diagnosis 727 PVNS 792, 973, 1006
Peroneus Brevis 884 Pyknodysostosis 1007
Peroneus Brevis Split Syndrome 885 Pyomyositis 826
Peroneus Longus and Brevis 884 Pyrophosphate arthropathy 950, 951
Peroneus Quartus 886 Quadriceps tendon 869
Perthes Lesion 934 Quadrilateral Space Syndrome 930
Pes anserine 870 RA 869
Phemister triad 832 Rachitic rosary 901
Phlebolith 806 Radial Collateral Ligament 959
Picture frame 814 Radial Fracture 945
Pigmented Villonodular Synovitis (PVNS) 793 Radial head 944
Pillar Fracture 844 Radial head dislocation 945
Pisotriquetral joint 872 Radial nerve impingement 956
Plantar Fascia 882 Radial Styloid Hutchinson’s/ Chauffer’s Fracture 945
Plantar Fasciitis 882 Radial tunnel syndrome 956
Plantar Fibromatosis 780, 882 Radiation 757
Plantaris Tendon 884 Radiation - internal synovectomy 795
Plasmacytoma 720, 966 Radiation Induced Chondrosarcoma 764
PNET 964, 967 Radiocapitellar Line 944
POEMS syndrome 964, 965 Radiolucent Lesions
Polyarthritis 983 Multilocular (Macho-Macho) 852
Polymyositis 976 Periapical 850
Polyostotic 773, 812 Pericoronal 852
Polyostotic Lesions 732 Radiopaque and Mixed Lesions
Angiomatous lesions 732 Ground Glass 856
Malignant 732 Interradicular 855
Neurofibromatosis (type 1) 732 Multifocal Confluent 856
Paget disease 732 Periapical 854
Popliteus hiatus 870 Target Lesion, Dense 857
Popliteus tendon 859 Radioulnar ligaments 875
Positive Rim Sign 943 Raynaud’s phenomenon 984
Posterior Dislocation (Glenohumeral) 943 Reactive Arthritis 917
Posterior Impingement (Elbow) 956 Rectus femoris 869
Posterior Instability (Glenohumeral) 935, 943 Recurrent Multifocal Osteomyelitis 827
Posterior recesses 870 Reflex sympathetic dystrophy 976
Posterior Superior Glenoid Impingement 936 Reiter Disease 912, 916, 917, 1010
Posterior talofibular 881 Renal Cell Carcinoma 963
Posterior Tibial Tendon 884 Renal disease 869
Posterior tibiofibular ligament 880 Renal Insufficiency - Chronic (MSK Manifestations) 711
Posterior Vertebral Scalloping 1020 Renal Osteodystrophy 711, 903
I9
Renal Tubular Disorders 903 Serpentine sclerosis 907
Rhabdomyosarcoma 769, 781, 802, 964, 967 Serpentine vessels 806
Rheumatoid Arthritis 797, 817, 912, 914, 941 Sever 911
Rheumatoid arthritis, JRA 748 Shepherd’s Crook 774
Ribbon ribs 1029 Shiny corner sign 918
Rickets 714, 900, 971 Sickle cell anemia 818, 968, 1010
Acetabuli Protrusio 714 Simple Bone Cyst 786
Basilar invagination 714 Sinding-Larsen-Johansson 911
Triradiate pelvis 714 Sinus lesions 745
Rim Rent Tear (Rotator Cuff) 929 Sinus Tarsi Syndrome 882
Ring sequestra 825 Sinus tracts 822
Rolando Fracture 947 Skeletal Metastases 961
Romanus and Andersson lesion 918 Skull: beveled edge, button sequestrum 891
Rotator cuff 932 SLAC Wrist 874
Atrophy 930 SLAP Tears 936
Tears 915, 925 SLE 869, 976
Types 928 SLE (Systemic Lupus Erythematosis) 983
Rowe 1003 Slipped Epiphyses 715, 826
Saber shin 834 Small cell carcinoma 964
Sacral Lesions: Differential Diagnosis 786 Smith Fracture (Reverse Colles) 945
Sacroiliac disease 918 Soft Tissue abscess 826
Sacroiliitis 1010 Soft Tissue Chondroma 800
Sacrospinous ligaments 997 Soft tissue chondrosarcoma 1012
Sacrotuberous ligament 997 Soft Tissue Ganglion 795
Saddle nose 834 Soft Tissue Hemangioma 805
Salmonella 821 Soft Tissue infection 825
Salter-Harris Fracture 1011 Soft Tissue Lipomatous Tumors 893
Sanders Classification 1003 Soft Tissue Masses Differential Diagnosis 804
SAPHO 827 Soft Tissue Neoplasm 733
Sarcoid 837 Angiomatous lesions 733
Sarcomatous transformation 817 Elastofibroma and fibromatosis 733
Sartorius 869 Lipomatous lesions 733
Saucerization (Juxtacortical Chondroma) 762 Neurogenic tumors 733
Saunders 1003 PVNS and ganglion 733
Sausage digit 912 Soft Tissue Sarcoma Incidence 781
Scaphoid fracture 945 Solitary Focus Bone Scan 964
Scapholunate Ligament 946 Souer and Remy 1003
Disruption 946 Sphenoid 745
Scapular “Y” View 942 Spina ventosa 830
Scapular Fractures 942 Spinal cord edema/hematoma 848
Schatzker Classification (Tibial Plateau Fractures) 1001 Spine Infections 828
Scheuermann Disease 911 Spinoglenoid Notch Entrapment 930
Sclerodactyly 984 Spirochetes 834
Scleroderma 793, 976, 985 Spondylitis 918
Sclerosing osteomyelitis of Garre 825 Spondyloarthropathies 912, 916
Sclerosing Osteosarcoma 754 Spondylodiscitis 828, 830, 1023
Scoliosis 901, 981 Spondylolisithes 845
Scurvy 900, 904, 977 Spontaneous healing (Osteoid Osteoma) 748
Secondary chondromatosis - trauma 799 Spontaneous osteonecrosis 865, 910
Secondary Chondrosarcoma 764 Sporotrichosis 836
Secondary osteoarthritis 826 Squamous cell carcinoma 852
Segond fracture 807, 1001 Staph aureus 821, 828
Semimembranosus 869, 870 Stener Lesion 947
Semitendinosus 869 Sterno-clavicular Joint 941
Senile osteoporosis 977 Steroid administration 953
Septic Arthritis 718, 748, 825 Steroids 869, 907
Septic bursitis 826 Steroids (Osteopenia/Osteoporosis) 977
Septic tenosynovitis 826 Stewart-Treve syndrome 810
sequestra 824, 967 Still Disease 919
Sequestra-Like Appearance 1005 Storioform 780
Sequestrum 820 Stress fracture 748
Seronegative Spondyloarthropathy 1009 Femur 1027
I 10
Stress Injuries 987 Thrombocytopenia 809
Subacute osteomyelitis 748, 824, 1007, 1026 Thrombocytopenia with Absent Radii (TAR) 968, 972
Subchondral cyst 784, 790, 921, 1008 Thumb Injury 947
Subchondral cyst/intraosseous ganglion 763 Thyroid Cancer 963
Subchondral Resorption 713 Tibial collateral 870
Subchondral Sclerosis 923 Tibial Plateau Fractures 1001
Sublabral Foramen 933 Tile 995
Subligamentous extension 830 Tile Classification 995
Subligamentous/Subtendinous Resorption 713 Tophi 950
Subperiosteal (Osteoid Osteoma) 746 Tori 856
Subperiosteal abscess 822, 823 Torus/ Buckle fracture 945
Subperiosteal Resorption 712 Transchondral fracture 879
Subscapularis (Avulsion) 935 Transient Osteoporosis 909
Subscapularis (Disruption) 935 Transient Osteoporosis Hip 1015
Subscapularis Muscle 932 Transient Regional Osteoporosis 977
Subscapularis Tears 929 Transverse Fractures (Pelvis) 999
Subtrochanteric Fractures 1000 Transverse ligament 863
Subungual Exostosis 759 Trap shooter’s shoulder 942
Sunburst 967 Trauma 839
Super bone scan 718 Trauma (Dysbaric Disorders) 907
Superior Glenohumeral Ligament 933 Trauma (Pelvis and Lower Extremity) 995
Superolateral migration (Osteoarthritis - Hip) 923 Trauma (Upper Extremity) 941
Supinator syndrome (Elbow) 956 Traumatic bone “cyst” 850
Supracondylar fracture 944 Traumatic Spondylolisithes 845
Suprapatellar “bursa” 870 Trevor Disease 759, 1020
Suprascapular Nerve Entrapment 930 Triangular Fibrocartilage (Calcification) 951
Supraspinatus Tendon (Tear) 935 Triangular Fibrocartilage Complex 875
Swan-Neck, Boutonniere deformities 914 Triceps Injuries 958
Symmetric Polyarthritis 983 Triquetrum Fracture 947
Symphysis pubis (Calcification) 951 Triradiate pelvis 901
Synchronous Osteosarcoma 750 Trochlea 955
Syndesmophyte 918 Trochlear sulcus 956
Synovial Chondroma 792 Trolley track sign 918
Synovial Chondromatosis 792, 799, 1006 Tropical ulcer 834
Synovial Trough Sign 943
Cyst 790, 792 T-score 978
Folds (Elbow) 956 Tuberculosis 829, 973
Lipoma 792, 798 Tuberculous
Osteochondromatosis 799 Arthritis 748, 832
Plica 871 Osteomyelitis 830
Sarcoma 781, 792, 793, 795, 802, 1012 Spondylodiscitis 830
Synovitis (Postoperative - Glenohumeral) 939 Tubulation (Osteochondroma) 758
Syphilis 834, 953 Tumoral Calcinosis 792, 1012
Syringomyelia 848, 953 Turner syndrome 808
Systemic Lupus Erythematosis (SLE) 983, 1016 UCL and ulnocarpal ligaments 875
Tabetic arthropathy 953 Ulcer (tropical) 834
Talar Neck Fractures 1002 Ulcerative Colitis 912
Talus 995 Ulcers 825
Tarsal Coalition 880 Ulnar
Tarsal joints 1003 fracture 945
Tarsal Tunnel Syndrome 885 nerve 872
Telangiectasia 984 Tunnel Syndrome 877
Telangiectatic Osteosarcoma 751 Unicameral bone cyst (UBC) 784, 944
Tendinitis (Calcific) 952 Unilateral Facet Injury 841
Tendon Sheath 794 Unusual infection 818
Tennis elbow 956 Upper Extremity Trauma 941
Tenosynovitis 876 Van Neck 911
Tenosynovitis (septic) 826 Vanishing Bone Disease 809
Tetracycline 748 Vastus lateralis 869
TFCC 872 Vastus medialis 869
Thalassemia 968, 970 Ventral (anterior) SIJ ligament 997
Thickened trabeculae 815 Vertebra Plana 891
I 11
Vertebral scalloping 981
Vertebroplasty 808
Vertical shear (Pelvic Trauma) 996
VISI deformity 875
Vitamin D: Prohormone 900
Volar Plate Avulsion 948
Voorhoeve Disease (Osteopathia Striata) 744
Wall Fracture (Pelvis) 999
West Point View 942
Whipple 912
Whiskering 914
Widened hip joint 826
Wimberger sign 834
Wormian Bones 1007
Wrisberg 859
Wrist 872
Wrist Subluxations 1019
Xenograft 709
X-linked hypophosphatasia 903
Yaws 834
Young-Burgess 995
Young-Burgess Classification 996
Young-Burgess vs Tile Classifications 995
Yttrium 90 795
Zonal pattern 801
Zonal phenomena 801
Z-score 978
I 12
Radiologic
Pathology
Fifth Edition
VOLUME 3
Neuroradiology and Pediatric
2006
Editors

2007
Mark D. Murphey, MD


Washington DC, USA
Washington, DC
20306-6000
_____________________________________

_____________________________________
987654321
ISBN 1-933477-00-8
Preface
Acknowledgements


Pathology,
iii
Neuroradiology Pediatric Radiology

Kelly K. Koeller, MD Ellen M. Chung, LTC, MC, USA
Kelly K. Koeller, MD, FACR Chief, Pediatric Radiology
Chief, Neuroradiology Department of Radiologic Pathology
Department of Radiologic Pathology Armed Forces Institute of Pathology
Armed Forces Institute of Pathology Washington, DC
Washington, DC
and Dorothy I. Bulas, MD
Associate Professor of Radiology Professor of Radiology and Pediatrics
Mayo Clinic Children's National Medical Center
Rochester, MN The George Washington University School of Medicine
and Health Sciences
Patricia A. Hudgins, MD Washington, DC
Emory University Medical Center Gael J. Lonergan, MD
Atlanta, GA Chief of Radiology
Children's Hospital of Austin
Mary E. (Lee) Jensen, MD Austin, TX
Director of Interventional Neuroradiology
Professor of Radiology and Neurosurgery William E. Shiels II, D.O.
University of Virginia Health System Chairman, Department of Radiology
Charlottesville, VA Children 's Hospital
Columbus, OH
Erin Simon Schwartz, MD
Assistant Professor of Radiology Marilyn J. Siegel, MD
University of Pennsylvania School of Medicine Professor of Radiology and Pediatrics
Pediatric Neuroradiologist Mallinckrodt Institute of Radiology
The Children's Hospital of Philadelphia Washington University Medical School
Philadelphia, PA St. Louis, MO
and
James G. Smirniotopoulos, MD Former Distinguished Scientist
Professor of Radiology, Neurology, and Biomedical Department of Radiologic Pathology
Informatics Armed Forces Institute of Pathology
Chair, Radiology and Radiological Sciences Washington, DC
Uniformed Services University of the Health Sciences
Bethesda, MD
Wendy R. K. Smoker, MS, MD, FACR
University of Iowa Medical Center
Iowa City, IA
iv
Table of Contents – VOLUME 3
Neuroradiology
Kelly K. Koeller, MD, FACR
Imaging of Demyelinating Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1037
Lymphoma and Uncommon Neuroepithelial Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1045
Cerebral Intraventricular Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1058
Imaging of the Temporal Bone: Anatomy and Congenital Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1068
Imaging of the Temporal Bone: Infectious and Neoplastic Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1076
Imaging of the Orbit: The Globe and Conal Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1088
Imaging of the Orbit: Intraconal and Extraconal Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1097
James G. Smirniotopoulos, MD
Patterns of Location: Infratentorial and Supratentorial . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1106
Patterns of Enhancement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1126
The WHO 2000 Brain Tumor Classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1137
Non-Astrocytic Gliomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1149
Extraaxial Tumors: Other Non-Glial Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1158
Neoplasms of the Meninges . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1164
Pinealomas and, other Pineal Region Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1175
The Phakomatoses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1184
Mary E. Jensen, MD
Subarachnoid Hemorrhage and Intracranial Aneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1210
Intracranial Vascular Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1220
Intracranial Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1231
Paranasal Sinuses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1240
Sella and Parasellar Region . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1250
Congenital Spinal Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1260
Wendy R. K. Smoker, MS, MD, FACR
Imaging of the Suprahyoid Neck: Superficial, Parapharyngeal and Carotid Spaces . . . . . . . . . . . . . . . . .1266
Imaging of the Suprahyoid Neck: Masticator and Parotid Spaces . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1274
Imaging of the Suprahyoid Neck: Pharyngeal Mucosal Space and Oral Cavity . . . . . . . . . . . . . . . . . . . .1282
Spine: Degenerative Disease and Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1290
Spinal Tumors, Cysts, and Mimics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1298
Congenital Abnormalities of the Brain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1307
Neuroradiology Seminar 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1318
Neuroradiology Seminar 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1322
Pediatric Radiology
Childhood Urinary Tract Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1329
Neonatal GI Tract Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1341
Acute GI Disorders of Infants and Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1353
Diseases Affecting The Pediatric Airway . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1363
Vascular Rings and Slings . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1374
Cystic Renal Disease of Childhood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1381
Marylin J. Siegel, MD
Pediatric Renal Tumors: Infancy and Young Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1390
Pediatric Adrenal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1402
Pediatric Pelvic Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1414
Bone Marrow Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1425
Congenital Lung Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1435
Lung Diseases in Neonates . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1444
Pediatric Cardiac Imaging Part I: Vascular Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1453
Pediatric Cardiac Imaging Part II: Congenital Heart Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1463
v
Gael J. Lonergan, MD
Congenital Heart Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1472
Forensic Radiology of Child Abuse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1491
Dorothy I. Bulas, MD
Neonatal Brain: Radiologic Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1501
William E. Shiels II, DO

Pediatric Liver Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1509
Pediatric Hip Sonography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1518
Pediatric Radiology Seminar I: Pulmonary Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1521
Pediatric Radiology Seminar II: Skeletal Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1527
Pediatric Radiology Seminar III: Cystic Fibrosis & Pulmonary Infections of Immunocompromised Child .1535
vi
Neuroradiology
Imaging of Demyelinating Diseases
Imaging Hallmarks
• White matter location
➢ May involve basal ganglia
• Little or no mass effect
• Usually no calcification
• May or may not enhance
Figure 5-1-1
Normal Lesions [Figure 5-1-1]
• Virchow-Robin spaces
➢ Perivascular space of deep penetrating vessels
➢ Follows CSFsignal intensity
• Ependymitis granularis
➢ Frontal horn
Ependymitis granularis [Figure 5-1-2]

• Frontal horn “capping”
• “Swamp” of the brain Perivascular spaces follow CSF signal
➢ Axons with low myelin content on all MR sequences
➢ Interstitial CSF backed up
➢ Loss of ependyma
• Periventricular hyperintensity: increased in ischemic WM states
• No lymphatics in the brain
Demyelinating Disorders
• Multiple sclerosis
• Vascular
• Viral / post-viral demyelination
• Toxic / metabolic encephalopathies
• Iatrogenic white matter degeneration Figure 5-1-2
Multiple Sclerosis
➢ Viral: measles, Epstein-Barr virus (EBV)
➢ Genetic: chromosome 6, human lymphocyte antigens (HLA)
loci
➢ Autoimmune: associations with Graves’, myasthenia gravis,
ulcerative colitis, Crohn's, SLE
• Females (60%): especially with optic neuritis
• 95% cases: 18-50 years old
• Cooler climates: northern Europe, North America; southern South
America
Multiple Sclerosis
• Relapsing-remitting: 70%
➢ Numbness, dysesthesia, burning sensations
➢ 2 clinical attacks from 2 separate lesions
❖ At least 24 hours and at least 30 days apart Ependymitis granularis
➢ Partial or complete remission for months or years
• Progressive: 20%
➢ Primary progressive: slow onset without distinct attacks
➢ Secondary progressive: relapsing-remitting form with progressive disability
• “Monosymptomatic demyelinating”: 10%
McDonald et al, Ann Neurol 2001: 50-121-127
Neuroradiology 1037 Imaging of Demyelinating Disease

Multiple Sclerosis - Clinical Figure 5-1-3
• Uthoff's phenomenon: symptoms worsened with
exposure to heat
• Children: very rare especially before puberty
• Pregnancy
➢ Decreased risk during 2nd and third trimester ?
➢ ...but exacerbation rate of 75% in first 6 months
post-partum
Multiple Sclerosis - Pathology

• Microglial infiltration Myelin disintegration and
• Perivascular cuffing of focal hypercellularity
lymphocytes -->
• Predilection for periventricular zone Active (enhancing) MS plaque compared to

chronic (non-enhancing) MS plaque
Multiple Sclerosis - Imaging
• Periventricular distribution: classic
➢ 3 or more lesions during “event”: sensitive Figure 5-1-4
indicator for MS within 10 years
• Ovoid lesions often perpendicular to walls of
ventricles (Dawson's fingers)
• Little mass effect for size of lesion
• Corpus callosum-septum pellucidum interface
lesions: increase specificity and sensitivity of
diagnosis
Radiology 1991;180:467-474; Radiology 1991;
180:215-221; Neurology 2003; 61:602-611
Multiple Sclerosis - Imaging [Figure 5-1-3]

• Active plaques enhance (ring-like in some)
• Chronic lesions: do not enhance
Atrophy in chronic MS
McDonald Criteria - MR
• Dissemination in space (3 or more)
➢ 1 enhancing lesion or 9 T2 hyperintense Figure 5-1-5
lesions
➢ 1 or more infratentorial lesion
➢ 1 or more juxtacortical lesion
➢ 3 or more periventricular lesions
• Dissemination in time (at least 1)
➢ MRI more than 3 months after clinial event, enhancing
lesion at different site
➢ No enhancing lesion: new T2 lesion or enhancing lesion on
f/u study at least 3 months later
➢ MRI less than 3 months, new enhancing lesion on f/u study
Multiple Sclerosis – MR [Figure 5-1-4]

• Chronic cases: atrophy
• Fluid-attenuated inversion recovery (FLAIR): hyperintense
• MR Spectroscopy
➢ Decreased N-acetyl
aspartate (NAA) in
chronic plaques
➢ Increased choline, Tumefactive MS plaque. Lack of
lipids and lactate mass effect and “non-neoplastic’
(NAA > choline) MR spectrogram are
important clues to correct diagnosis
Imaging of Demyelinating Disease 1038 Neuroradiology

“Tumefactive” Multiple Sclerosis [Figure 5-1-5]
• Tumor-like but…
• Lack of mass effect: most important clue for Figure 5-1-6
demyelination
Multiple Sclerosis – Variant Types [Figure 5-1-6]

• Concentric sclerosis (Balo's)
➢ Alternating bands of myelination and
demyelination, often in concentric fashion
• Acute: (Marburg)
➢ Rapid course
➢ Death in months
➢ Severe axonal loss
• Neuromyelitis optica (Devic syndrome)
➢ Both visual and spinal cord signs
Multiple Sclerosis vs. Transverse Myelitis Balo concentric sclerosis

[Figure 5-1-7]
• Spinal cord MS plaques: 7%
• Multiple sclerosis: peripheral, usually less than two segments, limited to one
side
➢ Clinical cord syndrome: 60% had brain lesions
• Transverse myelitis: usually holocord, commonly Figure 5-1-7
involves gray matter
Tartaglino et al, Radiology. 1995;195:725-32; Tartaglino
et al, Radiology. 1996;201:661-9
Vascular White Matter Disease

• Microangiopathy
• Arteriosclerosis / venous collagenosis
• Hypoxic-ischemic encephalopathy
• Posterior Reversible Encephalopathy Syndrome
(PRES)
• Amyloid angiopathy
• Vasculitis
• Migraine Transverse myelitis
Edwards, ed, Neuroimaging clinics 1993
Senescent White Matter Changes [Figure 5-1-8]

• “Microangiopathy”, “deep white matter ischemia, leukoariosis, etc.”
➢ Demyelination, axonal loss, gliosis, ischemic changes
• 30%-80% "normal" elderly patients
➢ More lesions, more likely to have neuropsychologic and cognitive
problems Figure 5-1-8
➢ ? correlation with dementia
➢ “Binswanger's”: clinical diagnosis, reserved only
for dementia cases
• Do not involve corpus callosum
Arteriosclerosis
• Long penetrating end arteries
➢ Few or no collateral vessels
• Pons, thalami, basal ganglia, deep white matter
• Prevalence increased with age and history of stroke
Venous Collagenosis
• Noninflammatory stenosis
• Occlusion of subependymal veins
• Associated with periventricular white matter changes Senescent white matter changes.
Note subcortical location
Moody et al, Radiology 1995; 194:469-476

Posterior Reversible Encephalopathy Syndrome (PRES) Figure 5-1-9
[Figure 5-1-9]
• Hypertensive events: renal failure, pre/eclampsia,
immunosuppressive drugs
• Loss of normal autoregulation: elevated hydrostatic
pressure mediated by venous vasoconstriction?
• Posterior cerebral circulation: less sympathetic
innervation, less ability to vasoconstrict
➢ Visual field deficits, headache, somnolence
➢ T2 hyperintensity
➢ Diffusion: usually increased (not restricted)
➢ Perfusion (CBV, CBF): decreased, normal, or
increased
Casey et al, AJNR 2000; 21:1199-1206 Brubaker et al,

AJNR 2005; 26:825-830; Schuuring et al AJNR 2003; Senescent white matter changes.
24:2085-2088 Note subcortical location
Viral and Postviral Demyelination

• Encephalitis
• Acute disseminated encephalomyelitis
• Subacute sclerosing panencephalitis
• Human immunodeficiency virus infection and complications
➢ HIV encephalitis
➢ Progressive multifocal leukoencephalopathy
Acute Disseminated Encephalomyelitis (ADEM)

• 1-3 weeks post-infection or vaccination
• Monophasic: rubeola, vaccinia, varicella, mycoplasma, mumps, rubella
➢ No virus or bacteria isolated on autopsy
• Hemorrhagic type (Hurst variant): rapidly progressive onset
• Children > adults
• Good prognosis overall but 10%-20% significant neurological deficit or death
• Diagnosis of exclusion: long-term follow-up needed to rule out MS
Honkaniemi et al, AJRN 2001; 22:1117-1124; Rosman et al, J Child Neurol. 1997;
12:448-54
Figure 5-1-10
ADEM – Pathology and Imaging

• Autoimmune response —-> perivenous
demyelination
• CT: normal or nonspecific hypoattenuation
• No mass effect
ADEM: MR Findings [Figure 5-1-10]

• Asymmetric WM lesions
➢ Varying in size and number
➢ Little or no mass effect
• Variable enhancement
• Optic neuritis, myelitis
Kesselring et al, Brain 1990;113:291-302

Progressive Multifocal Leukoencephalopathy (PML) Figure 5-1-11
• Defective cell-mediated immunity
• Marked decrease in prevalence with highly active anti-retroviral therapy
(HAART)
➢ Pre-HAART: 1-7% of AIDS patients; 55-85% cases related to AIDS
• 5–84 y/o; peak: 6th decade
• JC virus (papovavirus) reactivation
➢ Affects oligodendrocytes: demyelination
• Extremely poor prognosis (death in 6 months) if untreated
Baqi et al, AIDS 1997; 11:1526-7
PML – Path and Imaging [Figure 5-1-11]

• Predominantly parieto-occipital and frontal
➢ Posterior fossa: 1/3 cases
➢ Subcortical white matter
• Typically no mass effect or enhancement
➢ Enhancement indicative of long-term survival?
• Characteristic scalloped lateral margin at gray matter-white
matter junction
• May show hemorrhage
Whiteman et al, Radiology 1993; 187:233-240; Thurnher et al AJNR
2001; 22:977-984
HIV Encephalitis [Figure 5-1-12] PML with relative lack of mass effect
and sparing of cortical gray matter
• Much less common with anti-retroviral therapy
• Deep white matter and gray matter
• Psychomotor slowing, mental status changes, memory problems, apathy Figure 5-1-12
• Direct or indirect infection of oligodendrocytes
• Demyelination and vacuolation
➢ Axonal loss and microglial nodules
Thurnher et al AJNR 2001; 22:977-984
HIV Encephalitis
• Imaging often normal early in course
• Diffuse mild cerebral atrophy
➢ Cortical first, then central
• Ill-defined patchy areas
➢ Central white matter, basal ganglia, thalamus
➢ Bilaterally symmetric
➢ Usually no necrosis or edema
➢ No enhancement
HIV encephalitis with characteristic
Thurnher et al AJNR 2001; 22:977-984; Olson et al, Radiology 1988; cortical atrophy
169:445-448
Toxic Demyelination
• Alcohol
• Ion balance disorders
➢ Osmotic myelinolysis
➢ Extrapontine myelinolysis
• Organic toxins (lipophilic solvents)
• Carbon monoxide poisoning (“interval” form)
• Drug abuse (poisoned heroin)

Alcohol and the Brain [Figure 5-1-13 and 5-1-14] Figure 5-1-13
• Atrophy
➢ Cerebral hemisphere
➢ Superior vermis
• Marchiafava-Bignami disease
➢ Corpus callosum demyelination, necrosis
➢ Rare: cortical laminar necrosis
• Wernicke encephalopathy
➢ Thiamin deficiency
➢ Ophthalmoplegia, ataxia, confusion
➢ Medial thalamic nuclei
➢ Mamillary bodies: atrophy
Arbelaez et al, AJNR 2003; 24:1955-57; Johkura et al, AJNR 2005;
26:670-3; Donnal et al, AJNR 1990; 11:893-894
Marchiafava-Bignami disease with
Osmotic Myelinolysis [Figure 5-1-15] corpus callosal lesions. Note atrophy of
• “Central pontine myelinolysis” cerebral hemisphere and superior
• Variable clinical presentation cerebellar vermis
➢ Spastic quadraparesis, pseudobulbar palsy
• Incidence?: 0.16–3.7 % of autopsy cases
Figure 5-1-14
• Rapid osmotic change
➢ Vascular injury in gray matter - white matter
apposition regions
• Demyelination: spares periphery of pons
• CT: Hypoattenuated
Ruzek et al, AJNR 2004; 25:210-213
Osmotic Myelinolysis [Figures 5-1-15 and 5-1-16]

• MR:
➢ T1WI: hypointense
➢ T2WI: hyperintense
➢ May return to normal in months to year
➢ Extrapontine (10%): basal ganglia, other sites
Wernicke encephalopathy with
Iatrogenic Demyelinating Disorders: pathognomonic hyperintensity of both medial
Chemotherapy thalami
• Mineralizing microangiopathy
➢ Methotrexate
❖ Periventricular, centrum semiovale
❖ Patients < 5 y/o, meningeal leukemia, high-dose
Figure 5-1-16
therapy: greatest risk
Davis et al, AJR 1986; 147:587-592; Cajade-Law et al in
Neuroimag Clin, Edwards, ed. 1993;3:361-377
Figure 5-1-15
Osmotic (central pontine) myelinolysis

on CT and T2 MR images Extrapontine myelinolysis

Radiation Injury [Figure 5-1-17] Figure 5-1-17
• Acute: no imaging findings
• Early delayed: >2 months after therapy
➢ White matter, basal ganglia, cerebral peduncles
• Late:
➢ Focal radiation necrosis: > 1 year
➢ Diffuse radiation injury: > 1 year
❖ Geographic pattern: conforms to radiation port
➢ Necrotizing leukoencephalopathy: as early as 3 months post-
therapy
Rowley and Dillon in Neuroimag Clin, Edwards, ed, 1993;3:379-404
Radiation Necrosis [Figure 5-1-18]

• Tumor-like
• Metabolic imaging
➢ Increased activity: high-grade tumors
➢ Normal or decreased activity: radiation necrosis
➢ Less reliable for low-grade tumors
• MRS: increased lactate and choline in tumors vs. increased
lactate in necrosis
• Diffusion-weighted imaging: Radiation injury to white matter with
➢ Tumors: usually hypointense typical "geographic" pattern of
➢ Necrosis: usually hyperintense involvement
Rowley and Dillon in Neuroimag Clin, Edwards, ed, 1993;3:379-404
Demyelination Imaging Hallmarks Figure 5-1-18

• White matter location
➢ May involve basal ganglia
• Little or no mass effect
• Usually no calcification
• May or may not enhance
Summary
• Normal
➢ Virchow-Robin spaces: follow CSF
➢ Ependymitis granularis: frontal horn
• Multiple Sclerosis
➢ Periventricular
➢ Clinical diagnosis
• Vascular demyelination
➢ Senescent white matter changes Radiation necrosis mimicking appearance of a
❖ Subcortical, do not involve corpus callosum glioblastoma multiforme
➢ Posterior Reversible Encephalopathy Syndrome
(PRES)
• Viral / postviral demyelination
➢ ADEM
➢ PML
➢ HIV encephalitis
• Toxic / metabolic demyelination
➢ Alcohol
➢ Osmotic myelinolysis: centra pons
• Iatrogenic demyelination
➢ Chemotherapy and radiation injury
References
1. Arbelaez A, Pajon A, Castillo M. Acute Marchiafava-Bignami disease: MR findings in two patients. AJNR Am J
Neuroradiol 2003; 24:1955-1957.
2. Baqi M, Kucharczyk W, Walmsley SL. Regression of progressive multifocal encephalopathy with highly active
antiretroviral therapy. Aids 1997; 11:1526-1527.

3. Brubaker LM, Smith JK, Lee YZ, Lin W, Castillo M. Hemodynamic and permeability changes in posterior
reversible encephalopathy syndrome measured by dynamic susceptibility perfusion-weighted MR imaging. AJNR
Am J Neuroradiol 2005; 26:825-830.
4. Casey SO, Sampaio RC, Michel E, Truwit CL. Posterior reversible encephalopathy syndrome: utility of fluid-
attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J
Neuroradiol 2000; 21:1199-1206.
5. Davis PC, Hoffman JC, Jr., Pearl GS, Braun IF. CT evaluation of effects of cranial radiation therapy in children.
6. Donnal JF, Heinz ER, Burger PC. MR of reversible thalamic lesions in Wernicke syndrome. AJNR Am J
Neuroradiol 1990; 11:893-894; discussion 895-896.
7. Frohman EM, Goodin DS, Calabresi PA, et al. The utility of MRI in suspected MS: report of the Therapeutics and
Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 2003; 61:602-611.
8. Gean-Marton AD, Vezina LG, Marton KI, et al. Abnormal corpus callosum: a sensitive and specific indicator of
multiple sclerosis. Radiology 1991; 180:215-221.
9. Honkaniemi J, Dastidar P, Kahara V, Haapasalo H. Delayed MR imaging changes in acute disseminated
encephalomyelitis. AJNR Am J Neuroradiol 2001; 22:1117-1124.
10. Johkura K, Naito M, Naka T. Cortical involvement in Marchiafava-Bignami disease. AJNR Am J Neuroradiol 2005;
26:670-673.
11. Kesselring J, Miller DH, Robb SA, et al. Acute disseminated encephalomyelitis. MRI findings and the distinction
from multiple sclerosis. Brain 1990; 113 ( Pt 2):291-302.
12. McDonald WI, Compston A, Edan G, et al. Recommended diagnostic criteria for multiple sclerosis: guidelines
from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 2001; 50:121-127.
13. Moody DM, Brown WR, Challa VR, Anderson RL. Periventricular venous collagenosis: association with
leukoaraiosis. Radiology 1995; 194:469-476.
14. Nesbit GM, Forbes GS, Scheithauer BW, Okazaki H, Rodriguez M. Multiple sclerosis: histopathologic and MR
and/or CT correlation in 37 cases at biopsy and three cases at autopsy. Radiology 1991; 180:467-474.
15. Olsen WL, Longo FM, Mills CM, Norman D. White matter disease in AIDS: findings at MR imaging. Radiology
1988; 169:445-448.
16. Rosman NP, Gottlieb SM, Bernstein CA. Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic
resonance imaging findings in a child. J Child Neurol 1997; 12:448-454.
17. Rowley HA, Dillon WP: Iatrogenic white matter diseases. Neuroimaging Clin N Am 3:379–404, 1993
18. Ruzek KA, Campeau NG, Miller GM. Early diagnosis of central pontine myelinolysis with diffusion-weighted
imaging. AJNR Am J Neuroradiol 2004; 25:210-213.
19. Schuuring J, Wesseling P, Verrips A. Severe tacrolimus leukoencephalopathy after liver transplantation. AJNR Am J
Neuroradiol 2003; 24:2085-2088.
20. Tartaglino LM, Croul SE, Flanders AE, et al. Idiopathic acute transverse myelitis: MR imaging findings. Radiology
1996; 201:661-669.
21. Tartaglino LM, Friedman DP, Flanders AE, Lublin FD, Knobler RL, Liem M. Multiple sclerosis in the spinal cord:
MR appearance and correlation with clinical parameters. Radiology 1995; 195:725-732.
22. Thurnher MM, Post MJ, Rieger A, Kleibl-Popov C, Loewe C, Schindler E. Initial and follow-up MR imaging
findings in AIDS-related progressive multifocal leukoencephalopathy treated with highly active antiretroviral
therapy. AJNR Am J Neuroradiol 2001; 22:977-984.
23. Whiteman ML, Post MJ, Berger JR, Tate LG, Bell MD, Limonte LP. Progressive multifocal leukoencephalopathy in
47 HIV-seropositive patients: neuroimaging with clinical and pathologic correlation. Radiology 1993; 187:233-240.

Lymphoma and
Uncommon Neuroepithelial Tumors
CNS Lymphoma
• 6.6%-15.4% of all primary brain tumors
➢ Only GBM, meningioma, and low-grade astrocytoma are more common
• Less than 1% of all body lymphomas
• Primary lymphoma much more common than secondary
Miller et al, Cancer 1994; 74:1383-1397; Henry et al, Cancer 1974; 34:1293-1302
Clinical
• Wide age range
➢ Peak: 4th to 5th decades
➢ Smaller peak: 1st decade (AIDS)
• Nonspecific clinical presentation
➢ Expanding mass lesion
➢ Encephalitis
➢ Stroke Figure 5-2-1
➢ Cranial nerve palsies
Koeller et al, Radiographics 1997; 17:1497-1526
Immunocompromised Patients
• “Opportunistic neoplasm”
➢ Incidence much higher than in
immunocompetent patients
• AIDS-defining diagnosis in HIV+ patients
• 2% of AIDS patients develop CNS lymphoma
• CNS mass lesion in AIDS patient
➢ Toxoplasmosis #1, lymphoma #2
➢ Lymphoma: #1 in pediatric AIDS patient
Rosenblum et al, Ann Neurol 1988: 23:S13-S16;
Koeller et al, Radiographics 1997; 17: 1497-1526
Two masses, both lymphoma, with one located
Gross Pathology [Figure 5-2-1] around the ventricle while the other arises from
• Intra-axial nodule the leptomeninges
➢ Grayish-pink, homogeneous, circumscribed
➢ Multifocal: 50%
• Leptomeningeal Figure 5-2-2
• Uveal
• Intradural spinal
Histopathology [Figure 5-2-2]

• “Small blue cell tumor”
• Almost always B-cell type
• Perivascular space
Perivascular distribution of small blue lymphocytes

(arrow, vessel)
Neuroradiology 1045 Lymphoma & Uncommon Neuroepithelial Tumors

CT / MR Findings [Figures 5-2-3 to 5-2-5] Figure 5-2-3
• NCCT: Hyperattenuated mass
➢ Negative CT does not exclude diagnosis
• MR:
➢ T1WI: iso-to-hypointense to gray matter
➢ Little mass effect for size of lesion
• Virtually all enhance
➢ Ring-like: necrosis; common in
immunocompromised hosts
Lee et al, AJR 1986; 147:747-752; Schwaighofer et al,
AJNR 1989; 10:725-729; Dina, Radiology 1991;
179:823-828
Figure 5-2-4
Lymphoma with characteristic
CT hyperdensity and T2 hypointensity
Figure 5-2-5
CNS Lymphoma with true water restricted

diffusion (DWI hyperintensity, left; ADC
hypointensity, right)
General Neuroimaging Features [Figure 5-2-6]

• Supratentorial location
➢ Deep gray matter: “classic”, 33% Lymphoma in AIDS patient with ring-like
➢ Cerebral white matter: 55% enhancement secondary to necrosis
➢ Cerebellar lesions: 10%
• Multiple lesions: 11%-47%
➢ More common in immunocompromised
• Recurrences: 50% at original site
General Neuroimaging Features Figure 5-2-6

• “Hugs “ ependyma or leptomeninges
• “Butterfly” pattern: corpus callosum
• “Ghost” tumor: vanishes with steroid or radiation therapy
➢ Avoid prior to biopsy
• Rare: calcification, hemorrhage
Jiddane et al, J Neurosurg 1986; 65:592-599; Vaquero et al, J Neurosurg 1984;
60:174-176
Lymphoma of right
basal ganglia
Lymphoma & Uncommon Neuroepithelial Tumors 1046 Neuroradiology

PET / SPECT-Thallium [Figure 5-2-7] Figure 5-2-7
• Hypermetabolic
• Non-neoplastic lesions: hypometabolic
• High specificity and sensitivity
• False positives: interpretation errors,
occasional hypermetabolic abscess
Hoffman et al, J Nucl Med 1993; 34:567-
575; Villringer et al, J Comput Assist
Tomogr 1995; 19:532-536
Neuroepithelial Tumors
• Astrocytic
➢ Pleomorphic xanthoastrocytoma Toxoplasmosis or lymphoma ?
• Oligodendroglial PET image shows hypermetabolic activity consistent with
• Mixed glial lymphoma
• Ependymal
• Choroid plexus
• Pineal parenchymal
• Neuroblastic
• Glial of uncertain origin
• Neuronal and mixed neuronal-glial
➢ Ganglioglioma / Gangliocytoma
➢ Desmoplastic Infantile Ganglioglioma
➢ Dysplastic cerebellar gangliocytoma
➢ Dysembryoplastic neuroepithelial tumor
➢ Cerebellar Liponeurocytoma
• Embryonal
➢ Supratentorial PNET
➢ Atypical teratoid / rhabdoid tumor
Ganglioglioma / Gangliocytoma
• About 1% of all brain tumors
• Children and young adults
➢ 80% <30 years old; peak: 10-30 years of age
➢ Males slightly more common
• Most common tumor seen in chronic temporal lobe epilepsy
➢ 15%-25% of medically refractory seizures
• Cerebral hemisphere predilection
➢ Temporal lobe: most common (38%)
➢ Optic nerves, pituitary and pineal glands, spinal cord, ventricles, cranial
nerve (1 report)
Johnson et al, Pediatr Neurosurg 1997; 27:203-207; Athale et al, Neuroradiology
1999; 16:790-792
Ganglioglioma / Gangliocytoma [Figure 5-2-8]

• Good prognosis overall Figure 5-2-8
• Ganglioglioma: WHO I or II
➢ Biphasic tumor: neoplastic ganglion cells and glial cells
➢ Malignant degeneration of glial cells (WHO III): 6%
• Gangliocytoma
➢ Only mature ganglion cells
➢ WHO grade I
➢ AFIP: really cortical dysplasia?
Silver et al, Surg Neurol 1991; 35:261-266; Kalyan-Raman and
Olivero, Neurosurgery 1987; 20:428-433
Ganglioglioma with biphasic

appearance on histology. Arrow
points to a ganglion cell

Ganglioglioma [Figure 5-2-9] Figure 5-2-9
• Typically peripheral mass
➢ Cystic / solid: 52%
➢ Solid: 43%
➢ Purely cystic: 5%
• Little mass effect or surrounding edema
• Calcification common
• Skull remodeling
• Enhancement variable
Zentner et al, AJNR 1994; 57:1497-1502; Dorne et al, AJNR 1986;
7:281-285; Castillo et al, AJNR 1990; 11:109-114
Ganglioglioma [Figures 5-2-10 to 5-2-12]

• Typical MR findings
➢ T1WI: iso-to-hypointense
➢ Occasional T1 hyperintensity
➢ May or may not enhance
• Leptomeningeal spread: rare Ganglioglioma with typical peripheral
Castillo et al, AJNR 1990; 11:109-114; Tampieri et al, AJNR 1991; location and calcification
12:749-755; Tien et al, AJR 1992; 159:391-393
Ganglioglioma
• Positron Emission Tomography (PET): heterogeneous metabolic activity
• MR Spectroscopy: increased choline-creatine ratio
Provenzale et al, AJR 1999; 172:1103-1107; Kumabe et al, Figure 5-2-10
Neurosurgery 1999; 45:183-187
Desmoplastic Infantile
Ganglioglioma / Astrocytoma
• First described in 1987
➢ “Superficial cerebral astrocytoma”
➢ “Desmoplastic cerebral astrocytoma of
infancy”
• Rare: 0.6% of brain tumors
➢ 16% of all infant brain tumors
➢ Vast majority: less than 1 year (range:
up to 17 years) Ganglioglioma in temporal lobe
➢ Males more common (2:1)
• Rapid onset: increasing head circumference
• Usually more than one lobe: typically frontal and parietal
VandenBerg et al, J Neurosurg 1987; 66:58-71; Taratuto et al, Cancer 1984; Figure 5-2-12
54:2505-2512
Figure 5-2-11
Ganglioglioma in medial portion

Large ganglioglioma of right temporal lobe

Desmoplastic Infantile Ganglioglioma / Figure 5-2-13
Astrocytoma [Figure 5-2-13]
• WHO grade I
• Desmoplastic stroma
➢ Neuronal component: ganglioglioma
➢ Neoplastic astrocytes: astrocytoma
• Meningocerebral: involves cortex & leptomeninges
Duffner et al, Neurosurgery 1994; 34:583-589
Desmoplastic Infantile Ganglioglioma /

Astrocytoma
[Figure 5-2-14]
• Large heterogeneous mass
➢ Cyst-like areas
➢ Superficial soft tissue area Desmoplastic infantile ganglioglioma
❖ Slightly hyperattenuated
❖ Frequently attached to dura
❖ Intense enhancement Figure 5-2-14
➢ No calcification
➢ Occasional vasogenic edema
Duffner et al, Neurosurgery 1994; 34:583-589; Martin
et al, AJNR 1991; 12:1195-1197; Tenreiro-Picon et al,
Pediatr Radiol 1995; 25:540-543
Dysplastic Cerebellar Gangliocytoma

(Lhermitte-Duclos Disease)
• Original case, 1920 (Lhermitte and Duclos)
➢ 36-year-old male with progressive neurologic
deficits
➢ Cerebellar mass: “diffuse ganglioneuroma”
• Dysplastic cerebellar gangliocytoma
➢ Numerous other names proposed
• Histogenesis?: hamartoma vs. neoplasm
➢ Hamartoma favored
Lhermitte and Duclos, Bull Assoc Fr Etude Cancer,
1920;9:99-107; Wiestler et al, WHO classification,
2000; 235-237

• 50%: Cowden disease
➢ Autosomal dominant: susceptibility gene
10q23
➢ Colonic polyps, cutaneous tumors, thyroid
tumors, breast tumors, meningioma, glioma DIG with intensely enhancing soft tissue component
➢ MR screening of family members along meningocerebral interface
recommended
➢ Diagnosis established when Lhermitte-Duclos combined with either:
❖ Thyroid cancer
❖ Breast cancer
❖ Macrocephaly
Robinson and Cohen, Neurosurgery 2000; 46:371-383; Kulkantrakorn et al,
Neurology, 1997; 48:725-731; Nelen et al, Nat Genet 1996; 13:114-116

Dysplastic Cerebellar Gangliocytoma Figure 5-2-15
• CT findings
➢ Usually hypoattenuated
➢ Isoattenuated: “normal”
➢ Calcification uncommon
Meltzer et al, Radiology 1995; 194:699-703

(Lhermitte-Duclos Disease) [Figure 5-2-15]
• MR findings
• “Classic” appearance
➢ Cerebellar mass
➢ “Striated”
➢ No edema
➢ No enhancement
• Non-classic cases: non-specific appearance
Classic “striated cerebellum” appearance in
Meltzer et al, Radiology 1995; 194:699-703 Lhermitte-Duclos disease

• T1 hypointensity / T2 hyperintensity: Figure 5-2-16
➢ Inner molecular layer, granular cell layer, and loss of central
white matter within folia
Kulkantrakorn et al, Neurology 1997; 48:725-731
Cerebellar Liponeurocytoma
➢ “Lipomatous medulloblastoma”
➢ 15 cases reported by year 2000
• Similar to central neurocytoma except:
➢ Cerebellar location
➢ Older age group
• Peak age: 5th-6th decades (mean: 51 years old)
Kleihues et al, WHO classification 2000, 110-111
Cerebellar Liponeurocytoma
• No gender predilection
• Cerebellum or cerebellopontine angle
• WHO grade I or II
➢ Well-differentiated neuronal cells
➢ Focal lipomatous differentiation
➢ Low mitotic activity
• Good overall prognosis but few cases
➢ No role for radiation therapy or chemotherapy?
Kleihues et al, WHO classification 2000, 110-111
Cerebellar Liponeurocytoma [Figure 5-2-16]

• Cerebellar mass
• CT: hypoattenuated with focal areas of fat attenuation
• MR: hypointense with scattered focal T1 hyperintensity
• Moderate enhancement
Kleihues et al, WHO classification 2000, 110-111; Cacciola et al, Acta
Neurochir 2002; 144:829-833; Alkadhi et al 2001; J Neurosurg
95:324-331
Inherent T1 hyperintensity and mild
enhancement in cerebellar
liponeurocytoma

Dysembryoplastic Neuroepithelial Tumor Figure 5-2-17
• First described in 1988 (Daumas-Duport et al)
• Benign tumor of cortex or deep gray matter
• Children and young adults with partial seizures
➢ Peak age: 10-30 years
➢ Males more common
• Neurologic deficits not common
• Excellent prognosis even with partial resection
➢ Very rare malignant transformation
Daumas-Duport et al, Neurosurgery 1988;23:545-556;
Daumas-Duport et al, WHO classification, 2000;
Hammond et al, J Neurosurg 2000;92:722-725
Dysembryoplastic Neuroepithelial Tumor

[Figure 5-2-17]
• WHO Grade I DNT with “floating neuron”
• Simple form vs. complex form: controversial
• Multinodular: complex form
• Cortical dysplasia: focal
• “Specific glioneuronal element”: columnar pattern
➢ “Freely floating neurons” Figure 5-2-18
• Temporal lobe: 62%
➢ Frontal lobe: 31%
➢ Other sites: caudate nucleus, cerebellum, pons
Daumas-Duport et al, Neurosurgery 1988;23:545-556;
Daumas-Duport et al, WHO classification, 2000

[Figure 5-2-18]
• CT findings
➢ Hypoattenuated
➢ Calcification: uncommon (~ 5%)
➢ May produce remodel skull
➢ No surrounding edema
• MR findings
➢ Occasional “soap-bubble” appearance
➢ More multinodular than gangliogliomas?
Koeller and Dillon, AJNR 1992;13:1319-1325; Kuroiwa et al,
JCAT, 1994;18:352-356; Ostertun et al, AJNR 1996;17:419-
430
Pleomorphic Xanthoastrocytoma
• Originally described in 1979 (Kepes et al)
➢ 12 cases of supratentorial tumors involving the
leptomeninges
• Believed to arise from subpial astrocytes of the cortex
• Less than 1% of all brain neoplasms
• Importance: characteristic imaging appearance, highly
amenable to surgical resection
Kepes et al, Cancer 1979:44:1839-1852
DNT in classic cortical location and showing

exophytic extension with pressure erosion of
inner table of skull

• Usually adolescents or young adults (average age: 26 years)
➢ Wide age range: 5-82 years
• Long history of seizures
• Supratentorial: 98%
➢ Temporal lobe: 47%
➢ Multi-lobe: 10%
➢ Rare: thalamus, cerebellum, spinal cord, orbital globe
Giannini et al, Cancer 1999; 85:2033-2045; Pahapill et al, Neurosurgery 1996;
38:822-829 Figure 5-2-19
Pleomorphic Xanthoastrocytoma [Figure 5-2-19]
• WHO Grade II:
➢ Pleomorphism
➢ “Lipidized” neoplastic glial cells: xanthoma-like
➢ Overall good prognosis
❖ 81% 5-year survival
❖ 70% 10-year survival
➢ Higher recurrence rate
➢ Malignant transformation: 20%
Kepes et al, WHO classification 2000, 52-54; Giannini et al,
Cancer 1999; 85:2033-2045
Pleomorphic Xanthoastrocytoma PXA with vacuoles secondary to “lipidized”

• “Classic”: large cystic lesion with an enhancing mural neoplastic glial cells
nodule (<50%)
• Meningocerebral location
➢ Meningeal infiltration rare Figure 5-2-20
➢ Cortical involvement common
➢ Perivascular space extension
• Calcification rare
• Usually no surrounding edema
Tien et al, AJR 1992:159:1397-1404; Lipper et al, AJNR
1993; 14:1397-1404; Pahapill et al, Neurosurgery 1996;
38:822-829
Pleomorphic Xanthoastrocytoma [Figure 5-2-20]

• MR findings
➢ Heterogeneous mass
➢ T1WI: hypo-to-isointense
➢ T2WI: hyper-to-isointense
➢ Soft tissue component usually enhances
intensely PXA as large heterogeneous mass
➢ Leptomeningeal enhancement: characteristic and enhancing soft tissue component
Lipper et al, AJNR 1993; 14:1397-1404
Supratentorial Primitive Neuroectodermal Tumor

• “Cerebral medulloblastoma”
• 1% of all pediatric CNS neuroepithelial tumors
➢ 6% of all pediatric PNETs
• Age range: 4 weeks to 10 years (mean age: 5.5 years)
➢ Males more common (2:1)
Rorke et al, WHO classification, 2000, 141-144

• Nonspecific clinical features related to site
➢ Cerebrum: seizures, increased intracranial pressure, motor deficits
➢ Suprasellar region: visual or endocrine problems
➢ Not pineal: pineoblastoma
• Poor prognosis, especially in children < 2 years old
➢ 34% 5-year survival rate (up to 85% for cerebellar medulloblastoma)

• WHO grade IV
• Virtually identical to medulloblastoma
• Undifferentiated or poorly differentiated neuroepithelial cells
• Dense cellularity: “small blue cell tumor”
➢ Pleomorphism
➢ Rosette formation

➢ CT: iso-to-hyperattenuated
➢ Cysts or necrosis common
➢ Calcification: 50%-70%
➢ Vasogenic edema present but not usually
extensive
➢ T1WI: hypointense to gray matter
➢ T2WI: hypointense predominantly
Atypical Teratoid / Rhabdoid Tumor

• First reported in 1978
• Various names
➢ “Rhabdomyosarcomatoid variant of Wilms tumor”
➢ “Embryonal small cell tumor”
➢ “Rhabdoid tumor”
• Biologic behavior and some histologic features
similar to malignant rhabdoid tumor of the kidney
Beckwith and Palmer, Cancer 1978;41:1937-1948; Rorke
and Biegel, WHO classification 2000, 145-148; Rorke et
al, J Neurosurg 1996; 85:56-65; Hanna et al, AJNR
1993; 14:107-115
Atypical Teratoid / Rhabdoid Tumor

• 2.1% of all primary CNS tumors in children
• 94%: < 5 years old
➢ Rarely presents in adults (4 cases)
➢ Males more common (1.4:1)
• Non-specific symptoms (lethargy, failure to thrive)
• Head tilt
• Cranial nerve palsy: usually VI or VII
Rorke and Biegel, WHO classification 2000, 145-148;
Burger et al, Am J Surg Pathol 1998; 22:1083-1092
Supratentorial PNET with CSF dissemination

Atypical Teratoid / Rhabdoid Tumor [Figure 5-2-22] Figure 5-2-22
• WHO grade IV
• Soft lobulated mass
➢ Necrosis and hemorrhage common
• Rhabdoid cells
➢ Mixed with primitive neuroepithelial, epithelial, and
mesenchymal elements
➢ Not a germ-cell tumor
• Poor prognosis: most die within one year
Rorke and Biegel, WHO classification, 2000, 145-148
Atypical Teratoid / Rhabdoid Tumor [Figure 5-2-23]

• Posterior fossa: most common location (52%)
➢ Especially cerebellopontine angle
• Supratentorial: 39%
➢ Including intraventricular
• Pineal: 5% 90% have monosomy or deletion of
• Spinal: 2% chromosome 22
• Multifocal: 2%
Rorke and Biegel, WHO classification, 2000, 145-148 Figure 5-2-23

• Imaging: often mimics medulloblastoma
• CT: hyperattenuated
➢ Cysts and hemorrhage common
➢ Vasogenic edema common
Hanna et al, AJNR 1993:14:109-114; Caldemyer et al, Pediatr
Neurosurg 1994; 21:232-236; Rorke and Biegel, WHO classification,
2000, 145-148

• MR findings
➢ T1WI: hypo-to-isointense
➢ T2WI: variable
❖ Soft tissue: iso-to-hypointense
❖ Cyst-like: hyperintense
➢ Heterogeneous enhancement Posterior fossa ATRT
➢ Subarachnoid seeding: 33% at presentation
Hanna et al, AJNR 1993;14:109-115; Rorke and Biegel, WHO classification, 2000,
145-148
Figure 5-2-24
Posterior fossa ATRT. Hyperattenuation on CT is similar to medulloblastoma but shows foraminal

extension on MR images

Summary - Lymphoma
• Rapid increase in prevalence
• Fourth most common primary cerebral neoplasm
• Second most common intracranial mass in immunocompromised patient
• Periventricular
• CT: hyperattenuation
• T2W: hypointense
Peripheral Mass
• Ganglioglioma
➢ Variable size with common calcification
➢ Most common cause of chronic temporal lobe epilepsy
➢ Gangliocytoma: lacks glial component
• Dysembryoplastic neuroepithelial tumor (DNT)
➢ Almost always involves cortical margin
➢ Uncommon calcification
➢ Temporal lobe: 62%
➢ “Soap bubble” appearance
Cerebellar Mass
• “Striated” cerebellar mass
➢ Dysembryoplastic cerebellar gangliocytoma (Lhermitte-Duclos)
• With fat content
➢ Cerebellar liponeurocytoma
Large heterogeneous mass

• Meningocerebral interface
➢ Desmoplastic infantile ganglioglioma
❖ Large heterogeneous cerebral hemisphere mass
❖ No calcification
➢ Pleomorphic xanthoastrocytoma
❖ “Cyst-like mass with enhancing mural nodule”
❖ Calcification rare
❖ Older patients: adolescents and young adults
Large heterogeneous mass

• Young child
➢ Supratentorial PNET
❖ Large heterogeneous cerebral hemisphere mass
❖ “Cerebral medulloblastoma”
➢ Atypical teratoid / rhabdoid tumor
❖ Predilection for posterior fossa
❖ Mimics medulloblastoma
❖ Subarachnoid seeding common
References
1. Akhaddar A, Zrara I, Gazzaz M, El Moustarchid B, Benomar S, Boucetta M. Cerebellar liponeurocytoma

(lipomatous medulloblastoma). J Neuroradiol 2003; 30:121-126.
2. Alkadhi H, Keller M, Brandner S, Yonekawa Y, Kollias SS. Neuroimaging of cerebellar liponeurocytoma. Case
report. J Neurosurg 2001; 95:324-331.
3. Athale S, Hallet KK, Jinkins JR. Ganglioglioma of the trigeminal nerve: MRI. Neuroradiology 1999; 41:576-578.
4. Beckwith JB, Palmer NF. Histopathology and prognosis of Wilms Tumour: Results from the first National Wilms
Tumor Study. Cancer 1978; 41:1937-1948.
5. Burger PC, Yu IT, Tihan T, et al. Atypical teratoid/rhabdoid tumor of the central nervous system: a highly
malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group
study. Am J Surg Pathol 1998; 22:1083-1092.
6. Cacciola F, Conti R, Taddei GL, Buccoliero AM, Di Lorenzo N (2002) Cerebellar liponeurocytoma. Case report
with considerations on prognosis and management. Acta Neurochir (Wien) 144: 829-833
7. Caldemeyer KS, Smith RR, Azzarelli B, Boaz JC. Primary central nervous system malignant rhabdoid tumor: CT
and MR appearance simulates a primitive neuroectodermal tumor. Pediatr Neurosurg 1994; 21:232-236.

8. Castillo M, Davis PC, Takei Y, Hoffman JC, Jr. Intracranial ganglioglioma: MR, CT, and clinical findings in 18
patients. AJNR Am J Neuroradiol 1990; 11:109-114.
9. Daumas-Duport C, Pietsch T, Lantos PL. Dysembryoplastic neuroepithelial tumour. In: Kleihues P, Cavenee WK,
eds. World Health Organization Classification of Tumours: Pathology and genetics of tumours of the nervous
system. Lyon, France: IARC, 2000; 103-106.
10. Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, Laws ER, Jr., Vedrenne C. Dysembryoplastic neuroepithelial
tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases.
Neurosurgery 1988; 23:545-556.
11. Dina TS. Primary central nervous system lymphoma versus toxoplasmosis in AIDS. Radiology 1991; 179:823-828.
12. Dorne HL, O'Gorman AM, Melanson D. Computed tomography of intracranial gangliogliomas. AJNR Am J
Neuroradiol 1986; 7:281-285.
13. Duffner PK, Burger PC, Cohen ME, et al. Desmoplastic infantile gangliogliomas: an approach to therapy.
Neurosurgery 1994; 34:583-589; discussion 589.
14. Giannini C, Scheithauer BW, Burger PC, et al. Pleomorphic xanthoastrocytoma: what do we really know about it?
Cancer 1999; 85:2033-2045.
15. Hammond RR, Duggal N, Woulfe JM, Girvin JP. Malignant transformation of a dysembryoplastic neuroepithelial
tumor. Case report. J Neurosurg 2000; 92:722-725.
16. Hanna SL, Langston JW, Parham DM, Douglass EC. Primary malignant rhabdoid tumor of the brain: clinical,
imaging, and pathologic findings. AJNR Am J Neuroradiol 1993; 14:107-115.
17. Henry JM, Heffner RR, Jr., Dillard SH, Earle KM, Davis RL. Primary malignant lymphomas of the central nervous
system. Cancer 1974; 34:1293-1302.
18. Hoffman JM, Waskin HA, Schifter T, et al. FDG-PET in differentiating lymphoma from nonmalignant central
nervous system lesions in patients with AIDS. J Nucl Med 1993; 34:567-575.
19. Jiddane M, Nicoli F, Diaz P, et al. Intracranial malignant lymphoma. Report of 30 cases and review of the
literature. J Neurosurg 1986; 65:592-599.
20. Johnson JH, Jr., Hariharan S, Berman J, et al. Clinical outcome of pediatric gangliogliomas: ninety-nine cases over
20 years. Pediatr Neurosurg 1997; 27:203-207.
21. Kalyan-Raman UP, Olivero WC. Ganglioglioma: a correlative clinicopathological and radiological study of ten
surgically treated cases with follow-up. Neurosurgery 1987; 20:428-433.
22. Kepes JJ, Rubinstein LJ, Eng LF. Pleomorphic xanthoastrocytoma: a distinctive meningocerebral glioma of young
subjects with relatively favorable prognosis. A study of 12 cases. Cancer 1979; 44:1839-1852.
23. Kleihues P, Chimelli L, Giangaspero F. Cerebellar liponeurocytoma. In: Kleihues P, Cavenee WK, eds. World
Health Organization Classification of Tumours: Pathology and genetics of tumours of the nervous system. Lyon,
France: IARC, 2000; 110-111.
24. Koeller KK, Dillon WP. Dysembryoplastic neuroepithelial tumors: MR appearance. AJNR Am J Neuroradiol 1992;
13:1319-1325.
25. Koeller KK, Smirniotopoulos JG, Jones RV. Primary central nervous system lymphoma: radiologic-pathologic
correlation. Radiographics 1997; 17:1497-1526.
26. Kulkantrakorn K, Awwad EE, Levy B, et al. MRI in Lhermitte-Duclos disease. Neurology 1997; 48:725-731.
27. Kumabe T, Shimizu H, Sonoda Y, Shirane R. Thallium-201 single-photon emission computed tomographic and
proton magnetic resonance spectroscopic characteristics of intracranial ganglioglioma: three technical case reports.
Neurosurgery 1999; 45:183-187; discussion 187.
28. Kuroiwa T, Kishikawa T, Kato A, Ueno M, Kudo S, Tabuchi K. Dysembryoplastic neuroepithelial tumors: MR
findings. J Comput Assist Tomogr 1994; 18:352-356.
29. Lee YY, Bruner JM, Van Tassel P, Libshitz HI. Primary central nervous system lymphoma: CT and pathologic
correlation. AJR Am J Roentgenol 1986; 147:747-752.
30. Lhermitte J,Duclos P. Sur un ganglioneurome diffus du cortex du cervelet. Bull Assoc Fr Etude Cancer 1920;9:99-
107.
31. Lipper MH, Eberhard DA, Phillips CD, Vezina LG, Cail WS. Pleomorphic xanthoastrocytoma, a distinctive
astroglial tumor: neuroradiologic and pathologic features. AJNR Am J Neuroradiol 1993; 14:1397-1404.
32. Martin DS, Levy B, Awwad EE, Pittman T. Desmoplastic infantile ganglioglioma: CT and MR features. AJNR Am
J Neuroradiol 1991; 12:1195-1197.
33. Meltzer CC, Smirniotopoulos JG, Jones RV. The striated cerebellum: an MR imaging sign in Lhermitte-Duclos
disease (dysplastic gangliocytoma). Radiology 1995; 194:699-703.
34. Miller DC, Hochberg FH, Harris NL, Gruber ML, Louis DN, Cohen H. Pathology with clinical correlations of
primary central nervous system non-Hodgkin's lymphoma. The Massachusetts General Hospital experience 1958-
1989. Cancer 1994; 74:1383-1397.
35. Montagna N, Moreira D, Vaz LC, Reis M. Cerebellar liponeurocytoma: a newly recognized clinico-pathological
entity. Arq Neuropsiquiatr. 2002 Sep; 60(3-B):725-9.
36. Nelen MR, Padberg GW, Peeters EA, et al. Localization of the gene for Cowden disease to chromosome 10q22-23.
Nat Genet 1996;13:114-116

37. Ostertun B, Wolf HK, Campos MG, et al. Dysembryoplastic neuroepithelial tumors: MR and CT evaluation. AJNR
38. Pahapill PA, Ramsay DA, Del Maestro RF. Pleomorphic xanthoastrocytoma: case report and analysis of the
literature concerning the efficacy of resection and the significance of necrosis. Neurosurgery 1996; 38:822-828;
discussion 828-829.
39. Provenzale JM, Arata MA, Turkington TG, McLendon RE, Coleman RE. Gangliogliomas: characterization by
registered positron emission tomography-MR images. AJR Am J Roentgenol 1999; 172:1103-1107.
40. Robinson S, Cohen AR. Cowden disease and Lhermitte-Duclos disease: characterization of a new phakomatosis.
41. Rorke LB, Biegel JA. Atypical teratoid/ rhabdoid tumor. In Kleihues P and Cavenee WK, In: Kleihues P, Cavenee
WK, eds. World Health Organization Classification of Tumours: Pathology and Genetics, Tumours of the Nervous
System. IARC Press Lyon, 2000, page 145-148.
42. Rorke LB, Hart MN, McLendon RE: Supratentorial primitive neuroectodermal tumour (PNET). In: Kleihues P,
Cavenee WK, eds. World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of
the Nervous System. Edited by: Kleihues P, Cavenee WK. Lyon: IARC Press; 2000:141-144.
43. Rorke LB, Packer RJ, Biegel JA. Central nervous system atypical teratoid/rhabdoid tumors of infancy and
childhood: definition of an entity. J Neurosurg 1996; 85:56-65.
44. Rosenblum ML, Levy RM, Bredesen DE, So YT, Wara W, Ziegler JL. Primary central nervous system lymphomas
in patients with AIDS. Ann Neurol 1988; 23 Suppl:S13-16.
45. Schwaighofer BW, Hesselink JR, Press GA, Wolf RL, Healy ME, Berthoty DP. Primary intracranial CNS
lymphoma: MR manifestations. AJNR Am J Neuroradiol 1989; 10:725-729.
46. Silver JM, Rawlings CE, 3rd, Rossitch E, Jr., Zeidman SM, Friedman AH. Ganglioglioma: a clinical study with
long-term follow-up. Surg Neurol 1991; 35:261-266.
47. Tampieri D, Moumdjian R, Melanson D, Ethier R. Intracerebral gangliogliomas in patients with partial complex
seizures: CT and MR imaging findings. AJNR Am J Neuroradiol 1991; 12:749-755.
48. Taratuto AL, Monges J, Lylyk P, Leiguarda R. Superficial cerebral astrocytoma attached to dura. Report of six
cases in infants. Cancer 1984; 54:2505-2512.
49. Tenreiro-Picon OR, Kamath SV, Knorr JR, Ragland RL, Smith TW, Lau KY. Desmoplastic infantile
ganglioglioma: CT and MRI features. Pediatr Radiol 1995; 25:540-543.
50. Tien RD, Cardenas CA, Rajagopalan S.Pleomorphic xanthoastrocytoma of the brain: MR findings in six patients.
AJR Am J Roentgenol. 1992 Dec;159(6):1287-90.
51. Tien RD, Tuori SL, Pulkingham N, Burger PC.Ganglioglioma with leptomeningeal and subarachnoid spread:
results of CT, MR, and PET imaging. AJR Am J Roentgenol. 1992 Aug;159(2):391-3.
52. VandenBerg SR, May EE, Rubinstein LJ, et al. Desmoplastic supratentorial neuroepithelial tumors of infancy with
divergent differentiation potential ("desmoplastic infantile gangliogliomas"). Report on 11 cases of a distinctive
embryonal tumor with favorable prognosis. J Neurosurg 1987; 66:58-71.
53. Vaquero J, Martinez R, Rossi E, Lopez R. Primary cerebral lymphoma: the "ghost tumor". Case report. J
Neurosurg 1984; 60:174-176.
54. Villringer K, Jager H, Dichgans M, et al. Differential diagnosis of CNS lesions in AIDS patients by FDG-PET. J
Comput Assist Tomogr 1995; 19:532-536.
55. Wiestler OD, Padberg GW, Steck PA. Cowden disease and dysplastic gangliocytoma of the cerebellum/Lhermitte-
Duclos disease. In: Kleihues P, Cavenee WK, eds. World Health Organization Classification of Tumours:
Pathology and genetics of tumours of the nervous system. Lyon, France: IARC, 2000; 235-237.
56. Zentner J, Wolf HK, Ostertun B, et al. Gangliogliomas: clinical, radiological, and histopathological findings in 51
patients. J Neurol Neurosurg Psychiatry. 1994;57:1497-1502.

Cerebral Intraventricular Neoplasms
Ten Most Common Intraventricular Tumors*

• Ependymoma (18%) • Choroid Plexus Papilloma (24%)
• Subependymoma (11%) • Choroid Plexus Carcinoma (2%)
• Central Neurocytoma (10%) • Meningioma (6%)
• Subependymal Giant Cell • Metastasis (2%)
Astrocytoma (6%)
• Other Astrocytomas (9%)
• Colloid Cyst (4%)
*Based on 397 cases in AFIP Archives
Ependymoma
• Arise from ependymal cells of ventricular wall
➢ Also central canal of spinal cord
• 3%-9% of all neuroepithelial tumors
➢ 6%-12% of pediatric brain tumors
➢ 30% of all brain tumors in children < 3 years of age
3rd WHO classification, 2000
Ependymoma [Figure 5-3-1]

• Broad age range
➢ Posterior fossa: 6 years old (mean)
➢ Supratentorial: 18-24 years old (mean)
• Fourth ventricle: 58%
➢ Lateral & third ventricle: 42%
➢ Supratentorial: more commonly extraventricular
➢ Rare: ovaries, soft tissues, mediastinum, sacrococcygeal region
JCAT 1995; 19:518-526; Childs Nerv Syst 1991; 7:177-182;
J Neurosurg 1979; 51:383-391 Figure 5-3-1
Ependymoma
• Increased intracranial pressure and hydrocephalus
• Adults: 5-year survival rate = 57%, 10-year = 45%
• Less favorable prognosis
➢ Children: especially those under 2 years of age
➢ Fourth ventricle location
• Gross total resection “curative”
➢ Radiation therapy in partial resection cases
3rd WHO classification, 2000; Childs Nerv Syst 1990; 6:375-378;
Neurosurgery 1995; 37:655-667; Neurosurgery 1993; 32:169-175
Ependymoma
• Well-circumscribed mass
• May extend into brain
• Fourth ventricle: foraminal extension common
Ependymoma extending into

cerebellopontine angle from fourth
ventricle
Cerebral Intraventricular Neoplasms 1058 Neuroradiology

Ependymoma Figure 5-3-2
• WHO grade II
➢ Perivascular pseudorosettes
➢ Rare mitotic figures
• Variants
➢ Cellular
➢ Papillary
➢ Clear cell
➢ Tanycytic
➢ Anaplastic
Ependymoma [Figures 5-3-2 and 5-3-3]

• CT: iso- to hyperattenuated
➢ Calcification: 40%-80%
➢ Enhancing soft tissue component
➢ Non-enhancing cyst-like portion
• MR: heterogeneous
➢ Isointense to gray matter on T1WI
➢ Hyperintense to gray matter on T2WI Ependymoma with calcification on
➢ Post-op residual: decreased survival non-contrast CT
Radiology 1982; 143:97-101; Br J Radiol 1994; 67:223-243; Figure 5-3-3
JCAT 1995; 19:518-526; Neurosurgery
1991; 28:666-672
Subependymoma
• Arise from subependymal glial layer
• Incidence: 0.4 (asymptomatic) - 0.7%
(symptomatic)
• Most smaller than 2 cm diameter
➢ Symptomatic: 3-5 cm
• Hydrocephalus (80%), focal
neurologic deficit (27%), seizures
(9%), subarachnoid hemorrhage
(4.5%)
J Neurosurg 1945; 2:232-240; Acta
Neurochir 1989; 96:15-25;
AJR 1995; 165:1245-1250; Neurosurgery Ependymoma with foraminal extension on MR
1986; 19:594-598
Subependymoma
• Males more common
• Older than 15 years of age: 82%
• Fourth ventricle: > 50%
➢ Lateral ventricle: ~ 45%
• Well-circumscribed avascular mass
➢ Pedicular attachment to ventricular wall
Neurosurgery 1986; 19:594-598; J Neurosurg 1978: 49:689-696
Subependymoma
• Expansive but not infiltrative
• WHO grade I
➢ Dense fibrillary matrix
➢ Cysts and nests
➢ Low mitotic activity
• Low recurrence rate
• 10% mixed with ependymoma or other tumor
Neurosurgery 1986; 19:594-598; 3rd WHO classification, 2000
Neuroradiology 1059 Cerebral Intraventricular Neoplasms

Subependymoma [Figures 5-3-4 and 5-3-5] Figure 5-3-4
• CT: lobulated mass
➢ Iso-to-slight hypoattenuated
➢ Calcification: 32%
➢ Cystic degeneration: 18%
➢ At least some enhancement: 84%
• MR: hypointense compared to white matter on T1WI
➢ Hyperintense on T2WI
Neurosurgery 1986; 19:594-598; AJR 1995; 165:1245-1250;
AJNR 1995; 16:2121-2129; AJNR 1990; 11:83-91; Surg Neurol 1990;
33:329-335
Ependymoma vs. Subependymoma
Iso- to hyperattenuated Iso-to-hypoattenuated
Calcification, cysts more Calcification, cysts

Subependymoma with calcification
common less common
in right frontal horn on CT
ntense enhancement more More variable enhancement
common
Figure 5-3-5
Extraventricular extension Rarely extends beyond
ventricular margin
Neurosurgery 1986; 19:594-598
Central Neurocytoma
➢ Confusion with intraventricular oligodendroglioma
• “Central”: lateral and third ventricles
➢ “Extraventricular central neurocytoma” for those located
elsewhere (brain, cerebellum, spinal cord)
• 0.25%-0.5% of all intracranial tumors
Acta Neuropathol 1982;56:151-156; 3rd WHO classification, 2000;
Brain Pathol 1993; 3:297-306
Central Neurocytoma
• Broad age range: 8 days to 67 years
➢ Mean age: 29 years
➢ 50%: 20-30 years of age
➢ 75%: 20-40 years of age
• Short clinical course (mean: 3 months)
➢ Increased intracranial pressure, mental status changes, visual
deficits
3rd WHO classification, 2000; Brain Pathol 1993; 3:297-306
Central Neurocytoma
• Arise from septum pellucidum or ventricular wall
• Lateral ventricle near foramen of Monro: 50%
➢ Lateral and third ventricles: 15%
➢ Bilateral: 13%
➢ Third ventricle alone: 3% Subependymoma of right lateral
Surg Neurol 1998: 49:197-204; 3rd WHO classification, 2000 ventricle on axial T1-weighted
pre-contrast and post-contrast
images

Central Neurocytoma [Figure 5-3-6] Figure 5-3-6
• WHO grade II
• Striking resemblance to oligodendroglioma
➢ “Fried egg appearance”
➢ Pineocytomatous rosettes
• Neuronal differentiation
➢ ? Glial differentiation
Central Neurocytoma [Figure 5-3-7]

• Well-circumscribed lobulated mass
• Broad attachment to septum pellucidum or ventricular wall Central neurocytoma with
• CT: hyperattenuated compared to brain characteristic "fried egg"
➢ Cyst-like areas: 66% histologic appearance
• MR: hyperintense compared to white matter on T1WI and T2WI
• Hemorrhage rare
Neuroradiology 1991; 33:143-148; JCAT 1989: 13:495-497
Figure 5-3-7
Central neurocytoma with bilateral involvement on CT and MR in a

24-year-old woman with headaches and no neurological deficit
Subependymal Giant Cell Astrocytoma

• Most common brain neoplasm in tuberous sclerosis (6%-16%) Figure 5-3-8
➢ Neurocutaneous phakomatosis
➢ Autosomal dominant in 20%-50% of cases
➢ Tubers and subependymal nodules: 90%-100%
• Can it occur without TS? - controversial
• 1.4% of all pediatric brain tumors
• Most: first or second decades (mean: 11 years)
Neurosurgery 1991;28:864-868; 3rd WHO classification;
Pediatr Radiol 1992;22:485-489; Pediatr Neurosurg 1994: 20:233-239
Subependymal Giant Cell Astrocytoma [Figure 5-3-8]

• Virtually always located near the foramen of Monro
• Hydrocephalus, seizures
• Higher incidence of cardiac rhabdomyomas
• Surgical resection for symptomatic SEGA or with documented growth
• Non-responsive to radiation therapy
3rd WHO classification, 2000; Pediatr Radiol 1992; 22:485-489
Subependymal giant cell
astrocytoma with characteristic
location near foramen of Monro

• WHO grade I
➢ Slow growth with benign biologic behavior and low recurrence rate
➢ Earlier diagnosis associated with longer survival
• Probably arise from subependymal nodules
Figure 5-3-9
• Mixed glioneuronal pattern
Pediatr Neurosurg 1994: 20:233-239
Subependymal Giant Cell Astrocytoma [Figure 5-3-9]

• CT: iso- to slightly hypoattenuated
➢ Calcification common

• MR: hypointense compared to white matter on T1WI,
heterogeneously hyperintense on T2WI
➢ Neonates: hyperintense on T1WI, hypointense on T2WI
• Intense enhancement on CT and MR
➢ Distinguishes SEGA from most subependymal nodules
• Annual surveillance screening post-op and in first-degree
relatives
Pediatr Neurosurg 1994; 20:233-239; AJNR 1999: 20: 907-916;
Pediatr Radiol 1991; 21:432

• Other TS manifestations
➢ Cortical tubers
Subependymal giant cell astrocytoma
➢ Adenoma sebaceum
with calcification on CT
➢ Shagreen patch
➢ Retinal hamartoma
➢ Renal angiomyolipoma
➢ Cardiac rhabdomyoma
Figure 5-3-10
➢ Rectal polyps
Colloid Cyst
• Most common neuroepithelial cyst: probably arises
from endoderm
• Young to middle-aged adults
• Positional headache: acute CSF obstruction
• Antero-superior third ventricle
• Variable composition: mucoid material with old
blood, cholesterol crystals, serous fluid,
paramagnetic ions
Lach et al, J Neurosurg 1993; 78:101-111;
Shaungshotti et al, Arch Pathol Lab Med 1965; 80:214-
224
Colloid Cyst [Figure 5-3-10]

• Variable appearance
➢ Hyperattenuated on CT
➢ Hypo-to-hyperintense on T1WI
➢ Hypo-to-hyperintense on T2WI
• May ring enhance
• Solid enhancement: not colloid cyst
Waggenspack and Guiunto, AJNR 1989;10:105-110;
Lach et al, J Neurosurg 1993; 78:101-111 Colloid cyst on non-contrast axial CT, axial T2-
weighted, sagittal T1-weighted, contrast-enhanced
coronal T1-weighted images

Choroid Plexus
• Neuroepithelial tissue
➢ Cerebrospinal fluid (CSF) production: 450 ml/day (avg.)
➢ Atrium of the lateral ventricle
➢ Foramen of Monro and third ventricle
➢ Fourth ventricle and foramen of Luschka
➢ Absent in cerebral aqueduct
Choroid Plexus Tumors

• Lateral ventricle (50%), fourth ventricle (40%), third ventricle (5%)
➢ Multiple: 5%
• Lateral ventricle: no gender predilection
• Fourth ventricle: males more common
• 0.4%-0.6% of all intracranial tumors
➢ 2%-4% of pediatric brain tumors
➢ 10%-20% of brain tumors in less than 1 year of age
➢ 50% manifest in first decade
• Incidence: 0.3 per million
J Neurosurg 1988: 69: 843-849 Figure 5-3-11

• Hydrocephalus
➢ Increased CSF production
➢ CSF flow obstruction
➢ Hemorrhage: CSF absorption
• Association with Li-Fraumeni and Aicardi syndromes
Neurosurgery 1989; 25:327-335; J Neurosurg 1952; 9:59-67;
J Neurosurg 1998; 88:521-528; Radiology 1989; 173:81-88

• Pedicular attachment common Choroid plexus papilloma with
➢ Lateral ventricle: trigone histology similar to that of normal
➢ Third ventricle: roof choroid plexus tissue
➢ Fourth ventricle: posterior medullary velum
➢ “Bobble-head doll” syndrome: intermittent ventricular obstruction
• CSF Seeding: occurs in both papillomas and carcinomas
Papilloma vs. Carcinoma

• Choroid plexus papilloma • Choroid plexus carcinoma
➢ 80% ➢ 20%
➢ WHO grade I ➢ WHO grade III
➢ 100% 5-year survival ➢ 26-50% 5-year survival
➢ Children and adults ➢ Much more common in children
➢ Lateral ventricle: 1st ➢ Post-op residual disease: very poor
decade prognostic factor
➢ Fourth ventricle: first 5 ➢ Necrosis, parenchymal invasion
decades
Choroid Plexus Papilloma [Figure 5-3-11]

• Histology resembles normal choroid plexus
➢ Prominent fronds of fibrovascular connective tissue
• WHO grade I

Choroid Plexus Carcinoma [Figure 5-3-12] Figure 5-3-12
• Hypercellularity
• Nuclear pleomorphism
• High nucleus-cytoplasm ratio
• Mitotic activity
• Brain parenchyma invasion
• WHO grade III
Choroid Plexus Papilloma

• Well-circumscribed cauliflower-like mass
➢ Prominent lobulated margin
• CT: iso-to-hyperattenuated Choroid plexus carcinoma with
➢ Calcification: 24% markedly heterogeneous histologic
➢ Lateral ventricle: more common on left side? - Probably not appearance
➢ Foraminal extension characteristic
3rd WHO classification, 2000; J Neurosurg 1998; 88:521-528; Radiology 1989;
173:81-88
Choroid Plexus Papilloma [Figure 5-3-13]

• MR: iso-to-hypointense compared to normal brain parenchyma on T1WI
• Flow voids common
➢ Enlarged choroidal artery
➢ Amenable to pre-operative embolization
• US: lobulated echogenic mass
➢ Bi-directional flow throughout diastole
3rd WHO classification, 2000; Radiology 1989: 173:81-88
Figure 5-3-13
Choroid Plexus
Carcinoma [Figure 5-3-14]
• More heterogeneous
• Extraventricular extension
• Vasogenic edema
• Slightly less hydrocephalus?
3rd WHO classification, 2000;
J Neurosurg 1998; 88:521-528;
Radiology 1989; 173:81-88
Choroid plexus papilloma of left lateral ventricle atrium on MR

Figure 5-3-14
Choroid plexus carcinoma with extension into

adjacent brain parenchyma and spread within
ventricles

Intraventricular Meningioma
• Most common atrial mass in adults
➢ Usually older than 30 years of age
➢ Mean: 42 years
• Lateral ventricle >> third or fourth ventricle
• Arise from arachnoidal cap cells within choroid plexus, tela choroidea, or
velum interpositum
• 0.7% of all meningiomas Figure 5-3-15
• Almost all are benign
➢ Predilection for sarcomatous change in children
Neurosurgery 1987; 20:465-468; AJNR 1995; 16:1378-1381;
Radiology 1984; 153:435-442
• CT: well-defined globular mass
➢ Hyperattenuated compared to brain
Neurosurgery 1987: 20:465-468; AJNR 1995; 16:1378-1381;
Surg Neurol 1994: 42:41-45
Intraventricular Meningioma [Figure 5-3-15]

• MR: iso-to-hypointense compared to gray matter on T1WI
➢ Iso-to-hyperintense on T2WI
• Heterogeneous enhancement Intraventricular meningioma on
• MRS: decreased NAA, creatine contrast-enhanced axial T1-
➢ Increased choline weighted MR image
AJNR 1999; 20:882-885; AJNR 1994; 15:435-444
Choroid Plexus Metastasis

• Rare: 0.9-4.6% of all cerebral metastasis
• Renal cell carcinoma and lung carcinoma: most common in adults
➢ Children: neuroblastoma, Wilms’ tumor, retinoblastoma
➢ Others: melanoma, gastric carcinoma, colon carcinoma, lymphoma
• Lateral ventricle: most common Figure 5-3-16
• Renal cell carcinoma metastasis may mimic meningioma
South Med J 1998; 91:1159-1162; Neurosurgery 1983; 13:430-434
Choroid Plexus Metastasis [Figure 5-3-16]

• CT: iso- or hyperattenuated
• MR: hypointense on T1WI, hyperintense on T2WI
• Intense enhancement usually
Br J Radiol 1994; 67:223-243
Summary
• Fourth Ventricle
➢ Ependymoma
➢ Subependymoma
➢ Choroid Plexus Papilloma
• Third ventricle
➢ Colloid Cyst Choroid plexus metastasis
➢ All the others: less common from renal cell carcinoma
• Lateral Ventricle (anterior half)
➢ Subependymoma
➢ Central Neurocytoma
➢ Subependymal Giant Cell Astrocytoma
➢ Ependymoma
➢ Astrocytoma

• Lateral Ventricle (posterior half)
➢ Choroid Plexus Papilloma / Carcinoma
➢ Meningioma
➢ Metastasis
References
1. Baron Y, Barkovich AJ. MR imaging of tuberous sclerosis in neonates and young infants. AJNR Am J Neuroradiol
1999; 20:907-916.
2. Bolen JW, Jr., Lipper MH, Caccamo D. Intraventricular central neurocytoma: CT and MR findings. J Comput
Assist Tomogr 1989; 13:495-497.
3. Chiechi MV, Smirniotopoulos JG, Jones RV. Intracranial subependymomas: CT and MR imaging features in 24
cases. AJR Am J Roentgenol 1995; 165:1245-1250.
4. Coates TL, Hinshaw DB, Jr., Peckman N, et al. Pediatric choroid plexus neoplasms: MR, CT, and pathologic
correlation. Radiology 1989; 173:81-88.
5. Darling CF, Byrd SE, Reyes-Mugica M, et al. MR of pediatric intracranial meningiomas. AJNR Am J Neuroradiol
1994; 15:435-444.
6. Ellenbogen RG, Winston KR, Kupsky WJ. Tumors of the choroid plexus in children. Neurosurgery 1989; 25:327-
335.
7. Furie DM, Provenzale JM. Supratentorial ependymomas and subependymomas: CT and MR appearance. J Comput
Assist Tomogr 1995; 19:518-526.
8. Hassoun J, Gambarelli D, Grisoli F, et al. Central neurocytoma. An electron-microscopic study of two cases. Acta
Neuropathol (Berl) 1982; 56:151-156.
9. Hassoun J, Soylemezoglu F, Gambarelli D, Figarella-Branger D, von Ammon K, Kleihues P. Central neurocytoma:
a synopsis of clinical and histological features. Brain Pathol 1993; 3:297-306.
10. Healey EA, Barnes PD, Kupsky WJ, et al. The prognostic significance of postoperative residual tumor in
ependymoma. Neurosurgery 1991; 28:666-671; discussion 671-672.
11. Hoeffel C, Boukobza M, Polivka M, et al. MR manifestations of subependymomas. AJNR Am J Neuroradiol 1995;
16:2121-2129.
12. Kahn EA, Luros JT. Hydrocephalus from overproduction of cerebrospinal fluid, and experiences with other
parillomas of the choroid plexus. J Neurosurg 1952; 9:59-67.
13. Killebrew K, Krigman M, Mahaley MS, Jr., Scatliff JH. Metastatic renal cell carcinoma mimicking a meningioma.
14. Kleihues P, Cavenee WK eds. World Health organization Classification of Tumours, Pathology & Genetics:
Tumours of the Nervous System. IARC, Lyon, France, 2000.
15. Kudo H, Oi S, Tamaki N, Nishida Y, Matsumoto S. Ependymoma diagnosed in the first year of life in Japan in
collaboration with the International Society for Pediatric Neurosurgery. Childs Nerv Syst 1990; 6:375-378.
16. Lach B, Scheithauer BW, Gregor A, Wick MR. Colloid cyst of the third ventricle. A comparative
immunohistochemical study of neuraxis cysts and choroid plexus epithelium. J Neurosurg 1993; 78:101-111.
17. Lang I, Jackson A, Strang FA. Intraventricular hemorrhage caused by intraventricular meningioma: CT appearance.
AJNR Am J Neuroradiol 1995; 16:1378-1381.
18. Lobato RD, Sarabia M, Castro S, et al. Symptomatic subependymoma: report of four new cases studied with
computed tomography and review of the literature. Neurosurgery 1986; 19:594-598.
19. Majos C, Cucurella G, Aguilera C, Coll S, Pons LC. Intraventricular meningiomas: MR imaging and MR
spectroscopic findings in two cases. AJNR Am J Neuroradiol 1999; 20:882-885.
20. Matsumura A, Ahyai A, Hori A, Schaake T. Intracerebral subependymomas: clinical and neuropathological
analyses with special reference to the possible existence of a less benign variant. Acta Neurochir Wien 1989;
96:15–25.
21. McConachie NS, Worthington BS, Cornford EJ, Balsitis M, Kerslake RW, Jaspan T. Review article: computed
tomography and magnetic resonance in the diagnosis of intraventricular cerebral masses. Br J Radiol 1994;
67:223-243.
22. McGirr SJ, Ebersold MJ, Scheithauer BW, Quast LM, Shaw EG. Choroid plexus papillomas: long-term follow-up
results in a surgically treated series. J Neurosurg 1988; 69:843-849.
23. Menor F, Marti-Bonmati L, Mulas F, Poyatos C, Cortina H. Neuroimaging in tuberous sclerosis: a
clinicoradiological evaluation in pediatric patients. Pediatr Radiol 1992; 22:485-489.
24. Morantz RA, Kepes JJ, Batnitzky S, Masterson BJ. Extraspinal ependymomas. Report of three cases. J Neurosurg
1979; 51:383-391.
25. Morrison G, Sobel DF, Kelley WM, Norman D. Intraventricular mass lesions. Radiology 1984; 153:435-442.
26. Palma L, Celli P, Cantore G. Supratentorial ependymomas of the first two decades of life. Long-term follow-up of
20 cases (including two subependymomas). Neurosurgery 1993; 32:169-175.

27. Pencalet P, Sainte-Rose C, Lellouch-Tubiana A, et al. Papillomas and carcinomas of the choroid plexus in children.
J Neurosurg 1998; 88:521-528.
28. Pollack IF, Gerszten PC, Martinez AJ, et al. Intracranial ependymomas of childhood: long-term outcome and
prognostic factors. Neurosurgery 1995; 37:655-666; discussion 666-657.
29. Raila FA, Bottoms WT, Jr., Fratkin JD. Solitary choroid plexus metastasis from a renal cell carcinoma. South Med
J 1998; 91:1159-1162.
30. Rieger E, Binder B, Starz I, Oberbauer R, Ebner F, Urban C. Tuberous sclerosis complex: oligosymptomatic
variant associated with subependymal giant-cell astrocytoma. Pediatr Radiol 1991; 21:432.
31. Scheinker IM: Subependymoma: A newly recognized tumor of subependymal derivation. J Neurosurg 1945, 2:
232-240
32. Scheithauer BW. Symptomatic subependymoma. Report of 21 cases with review of the literature. J Neurosurg
1978; 49:689-696.
33. Schiffer D, Chio A, Giordana MT, et al. Histologic prognostic factors in ependymoma. Childs Nerv Syst 1991;
7:177-182.
34. Sgouros S, Carey M, Aluwihare N, Barber P, Jackowski A. Central neurocytoma: a correlative clinicopathologic
and radiologic analysis. Surg Neurol 1998; 49:197-204.
35. Sgouros S, Walsh AR, Barber P. Intraventricular malignant meningioma in a 6-year-old child. Surg Neurol 1994;
42:41-45.
36. Shepherd CW, Scheithauer BW, Gomez MR, Altermatt HJ, Katzmann JA. Subependymal giant cell astrocytoma: a
clinical, pathological, and flow cytometric study. Neurosurgery 1991; 28:864-868.
37. Shuangshoti S, Roberts MP, Netsky MG. Neuroepithelial (colloid) cyst: pathogenesis and relation to choroid plexus
and ependyma. Arch Pathol Lab Med 1965; 80:214-224,
38. Sinson G, Sutton LN, Yachnis AT, Duhaime AC, Schut L. Subependymal giant cell astrocytomas in children.
Pediatr Neurosurg 1994; 20:233-239.
39. Spoto GP, Press GA, Hesselink JR, Solomon M. Intracranial ependymoma and subependymoma: MR
manifestations. AJNR Am J Neuroradiol 1990; 11:83-91.
40. Strenger SW, Huang YP, Sachdev VP. Malignant meningioma within the third ventricle: a case report.
41. Swartz JD, Zimmerman RA, Bilaniuk LT. Computed tomography of intracranial ependymomas. Radiology 1982;
143:97-101.
42. Waggenspack GA,Guinto FC Jr. MR and CT of masses of the anterosuperior third ventricle. AJNR, 1989; 10:105-
110
43. Wichmann W, Schubiger O, von Deimling A, Schenker C, Valavanis A. Neuroradiology of central neurocytoma.
Neuroradiology 1991; 33:143-148.
44. Yamasaki T, Kikuchi H, Higashi T, Yamabe H, Moritake K. Two surgically cured cases of subependymoma with
emphasis on magnetic resonance imaging. Surg Neurol 1990; 33:329-335.

Imaging of the Temporal Bone: Anatomy
and Congenital Lesions
Middle Ear
• Epitympanum
➢ Malleus head
➢ Short process of incus
• Mesotympanum
➢ Muscles: tensor tympani (V3), stapedius (VII)
➢ Ossicles: rest of malleus and incus, stapes
➢ Ligaments
➢ Nerves: chorda tympani (VII), Jacobson’s nerve (IX)
Inner Ear: Cochlea

• Anterior to vestibule
• Promontory: bony ridge
• Modiolus: central axis, cochlear nerve
• Apex (cupola)
• Basal turn
• Cochlear aqueduct: perilymphatic duct
Inner Ear: Vestibule

• Posterior to cochlea
• Oval window niche: partition from middle ear
• Lamina cribrosa: partition from internal auditory canal (IAC)
• Vestibular aqueduct: endolymphatic duct
Inner Ear: Semicircular Canals (SCC) [Figures 5-4-1 to 5-4-15]

• Lateral (horizontal)
• Superior: arcuate eminence
• Posterior
• Oriented ≈ 90° to each other
• Rotational acceleration
Figure 5-4-1 Axial CT at superior portion of temporal

bone shows lumen of superior
semicircular canal and upper portion of
mastoid antrum and epitympanum
Figure 5-4-2 Subarcuate artery canal traversing

through “hoop” of superior semicircular
canal
Temporal Bone Anatomy and Congenital Lesions 1068 Neuroradiology

Figure 5-4-3
Superior portion of vestibule with malleus

(anterior) and incus (posterior) within the
epitympanum
Figure 5-4-4
Superior portion of internal auditory canal
with labyrinthine canal containing the
facial nerve on its way to the geniculate
ganglion and tympanic facial segment
along medial wall of middle ear
Figure 5-4-5
Superior portion of cochlea and internal
auditory canal. Note vestibular aqueduct
arising from posterior margin of temporal
bone
Figure 5-4-6 Mid-portion of internal auditory canal and

cochlea. Sinus tympani is located just lateral
to vestibule. Small bony peak lateral to
vestibule is pyramidal eminence. Facial nerve
canal is located posterolateral to pyramidal
eminence. Middle ear shows “parallel lines
sign”: tensor tympani tendon anteriorly and
incudostapedial junction with stapedial struts
posteriorly. Struts mark the site of the oval
window
Figure 5-4-7
Inferior portion of cochlea. Bony plate
separating it from the middle ear is the
cochlear promontory. Note jugular bulb in
posterior temporal bone
Figure 5-4-8
Basilar turn of cochlea. Internal carotid
artery is seen anterior to the cochlea and
is delimited from the middle ear by a
bony plate. Eustachian tube arises just
lateral to the artery and heads along an
anteromedial pathway towards the
nasopharynx
Neuroradiology 1069 Temporal Bone Anatomy and Congenital Lesions

Figure 5-4-9 Coronal view shows internal carotid
artery inferiorly separated from middle
ear by bony plate. Cochlea is located
immediately superior. Geniculate
ganglion is located just superolateral to
cochlea. Middle ear contains ossicles
(malleus anterior to incus) within
epitympanum. Tegmen tympani is bony
plate separating middle ear from brain
Figure 5-4-10
Moving posteriorly, facial nerve segments

are seen above and lateral to cochlea
Figure 5-4-11
Cochlea is separated by cochlear
promontory from middle ear. Anterior
portion of internal auditory canal is just
coming into view. Scutum is well seen
along superior margin of medial external
auditory canal
Figure 5-4-12
Mid-portion of internal auditory canal with
crista falciformis along its lateral margin.
Vestibule now appears with lateral and
superior semicircular canals. Facial
nerve is located immediately inferior to
lateral semicircular canal and above oval
window
Figure 5-4-13
Posterior margin of vestibule with facial

nerve as small soft tissue just prior to
reaching posterior genu
Figure 5-4-14
Facial nerve at posterior genu

Figure 5-4-15
Mastoid segment of facial nerve

extending inferiorly to the stylomastoid
foramen
• Outer ear
• Middle ear
• Inner ear
• Vascular
➢ Internal carotid artery
➢ Jugular vein
• Cholesteatoma
• Encephalocele
Congenital Malformations
• External and middle ear (1st and 2nd branchial arch) develop independent of
inner ear (ectodermal)
• IAC development separate from inner ear development
• Anomalies of all 3 parts are rare
➢ Dysplasias and trisomies 13, 18, 21
Fisher and Curtin, Otolaryngol Clin North Am 1994; 27:511-531
Outer Ear Anomalies

• Congenital aural dysplasia
• Bilateral: 33%
• Genetic disorder association Figure 5-4-16
• External auditory canal (EAC) atresia: failure of recanalization
(26th gestational week)
➢ Fibrous vs. bony plate
➢ CT: middle ear dysplasia or cholesteatoma
Robson et al, Neuroimag Clin North Am 1999; 9:133-135; Mayer et al,
AJNR 1997; 18:53-65
Middle Ear Anomalies

• Temporomandibular joint (TMJ) anomalies
• TMJ higher and more posterior than normal
• Facial nerve displaced
➢ Vertical portion more anterior than normal
➢ Very important pre-operative finding
Robson et al, Neuroimag Clin North Am 1999; 9:133-135; Mayer et al,
AJNR 1997; 18:53-65
Inner Ear Anomalies [Figure 5-4-16]

• Lateral semicircular canal anomaly
➢ Last semicircular canal to form Anomalous shortening and widening
➢ Usually short and wide, less commonly narrow of lateral semicircular canal
Jackler and Luxford, Laryngoscope 1987; 97:2-14

Complete Labrynthine Aplasia Figure 5-4-17
• Michel’s deformity
• 3rd gestational week
• Inner ear absent
• Small cystic cavity: single or multiple
Incomplete Partition / Dilatational Defects [Figure 5-4-17]

• Mondini’s dysplasia (1791): cochlea with 1 and 1/2 turns
• Second most common form of congenital deafness (Schiebe’s
deafness #1)
• 7th gestational week
• Small cochlea with incomplete or absent intrascalar septum
• Basilar turn present
• Common cavity in place of middle and apical turns
Paparella, Ann Otol Rhinol Laryngol Suppl 1980; 89(2 Pt 3):1-10
Other Cochlear Anomalies [Figure 5-4-18]

• Common cavity
➢ 4th gestational week Mondini dysplasia
➢ Cochlea and vestibule fused
➢ 25% of all cochlear anomalies
• Cochlear aplasia
➢ 5th gestational week
➢ Rest of inner ear normal or malformed
• Cochlear Hypoplasia
Figure 5-4-18
➢ 6th gestational week
➢ Small cochlear bud
Large Endolymphatic Duct and Sac (LEDS)

• Large vestibular aqueduct syndrome
• Dilated vestibular aqueduct
➢ Most common radiologic finding in early-onset SNHL
❖ > 1.5 mm diameter (lateral SCC)
❖ MR: look at T2W images
➢ Often associated with incomplete partition cochlear anomalies
➢ Progressive sensorineural hearing loss (SNHL)
➢ Etiology: hyperosmolar protein transmission?
Valvassori and Clemis, Laryngoscope 1978; 88:723-728; Mafee,
AJNR 1992;13:805-819; Jackler and De la Cruz, Laryngoscope 1989;
99:1238-1243; Dahlen et al, AJNR 1997; 18:67-75; Davidson et al,
AJNR 1999; 20:1435-1441
Inner Ear Anomalies: Associations Enlarged vestibular aqueduct. Note

• Otocraniofacial size in comparison to lateral
➢ Crouzon’s, Apert’s, etc. semicircular canal
• Otocervical
➢ Klippel-Feil, Goldenhar’s, etc.
• Otoskeletal
➢ Osteogenesis imperfecta, osteopetrosis, etc.
Romo, Casselman, and Robson in Som and Curtin, Head and Neck Imaging, 4th
ed., Mosby, 2003

Aberrant Internal Carotid Artery [Figure 5-4-19] Figure 5-4-19
• 90% females
• More common on right side
• Pulsatile tinnitus, conductive hearing loss (HL), otalgia
• Enhancing mass in hypotympanum (inferior tympanic canaliculus)
Sinnreich et al, Otolaryngol Head Neck Surg 1984; 92:194-206;
Thiers et al, AJNR 2000; 21:1551-1554
Absent Internal Carotid Artery

• May be incidental discovery
• High association with intracranial aneurysms Aberrant internal carotid artery with
• Nearly 30% present with subarachnoid hemorrhage soft tissue attenuation within middle
Keen, Clin Proc 1946; 4:588-594; Martinez-Granero et al, Rev Neurol ear and absent bony margin (Case
1997; 25:1207-1209 courtesy of Wendy Smoker, MD,
FACR)
Persistent Stapedial Artery
• Rare: most seen at surgery Figure 5-4-20
• Precursor for middle meningeal artery
• Small canal from carotid canal
• Crosses cochlear promontory
• Widened facial canal
• Absence of foramen spinosum
Thiers et al, AJNR 2000; 21:1551-1554
High Jugular Bulb (“Megabulb”) [Figure 5-4-20]

• Various definitions described
• Most common vascular anomaly of petrous temporal bone
➢ 3%–7% incidence
• More common on right side
➢ 75% jugular vein larger on right
• Usually poorly pneumatized mastoids
• No bony dehiscence
• Importance: surgical impact
Overton and Ritter, Laryngoscope 1973; 83: 1986-1991; Caldemyer et
al, RadioGraphics 1997; 17:1123-1139
High jugular bulb with dehiscence
Other Jugular Vein Anomalies along internal auditory canal
• Dehiscent jugular bulb
➢ Direct communication with middle ear
➢ Lateral: pulsatile tinnitus, conductive hearing loss Figure 5-4-21
➢ Medial: Meniere’s disease
• Jugular diverticulum
➢ Above, medial, posterior to petrous pyramid
➢ More common on left-side and in females
Couloigner et al, Eur Arch Otorhinolaryngol 1999; 256:224-229;
Pappas et al, Otolaryngol Head Neck Surg, 1993; 109:847-852
Pial siderosis with thin bands of T2

hypointensity secondary to
subarachnoid hemorrhage

Pulsatile Tinnitus Lesions Figure 5-4-22
[Figures 5-4-21 and 5-4-22]
• Congenital
➢ Aberrant internal carotid artery
➢ Dehiscent jugular bulb
• Tumor
➢ Paraganglioma
➢ Hemangioma
• Vascular
➢ Arteriovenous malformation / fistula
➢ Aneurysm
➢ Pial siderosis: VIII n. especially prone
Congenital Cholesteatoma (Epidermoid)

[Figures 5-4-23 and 5-4-24] Abnormal flow voids secondary to arteriovenous
• Child with conductive HL fistula
• Aberrant epithelial rests
• Epitympanum, incudostapedial joint > petrous
apex Figure 5-4-23 Figure 5-4-24
• Globular mass +/– bone destruction
• Follows fluid signal intensity
• May have peripheral enhancement
Peron and Schuknecht, Arch Otolaryngol 1975:
101:498-505; Gao et al, AJNR 1992; 13:863-872
Congenital Dehiscence of Tegmen

Tympani [Figure 5-4-25]
• Fusion of petrosquamosal suture by 1 year of
age
• Tiny openings: up to 34% of population
➢ Encephalocele, fistula: rare (requires dural
weakening)
• Coronal plane best Ossicular epidermoid with Contrast-enhanced coronal
bone erosion T1-weighted image shows
Gavilan et al, Arch Otolaryngol 1984; 110-206-207;
rim enhancement of
Gottlieb et al, Arch Otolaryngol 1998; 124:1274-1277
epidermoid involving right
temporal bone
Figure 5-4-25
Axial T2-weighted image shows focal hyperintensity in region of epitympanum.

Coronal CT images show soft-tissue density in epitympanum. Surgical exploration
confirmed encephalocele

References
1. Caldemeyer KS, Mathews VP, Azzarelli B, Smith RR. The jugular foramen: a review of anatomy, masses, and
imaging characteristics. Radiographics 1997; 17:1123-1139.
2. Couloigner V, Grayeli AB, Bouccara D, Julien N, Sterkers O. Surgical treatment of the high jugular bulb in patients
with Meniere's disease and pulsatile tinnitus. Eur Arch Otorhinolaryngol 1999; 256:224-229.
3. Dahlen RT, Harnsberger HR, Gray SD, et al. Overlapping thin-section fast spin-echo MR of the large vestibular
aqueduct syndrome. AJNR Am J Neuroradiol 1997; 18:67-75.
4. Davidson HC, Harnsberger HR, Lemmerling MM, et al. MR evaluation of vestibulocochlear anomalies associated
with large endolymphatic duct and sac. AJNR Am J Neuroradiol 1999; 20:1435-1441.
5. Gao PY, Osborn AG, Smirniotopoulos JG, Harris CP. Radiologic-pathologic correlation. Epidermoid tumor of the
cerebellopontine angle. AJNR Am J Neuroradiol 1992; 13:863-872.
6. Gavilan J, Trujillo M, Gavilan C. Spontaneous encephalocele of the middle ear. Arch Otolaryngol 1984; 110:206-
207.
7. Gottlieb MB, Blaugrund JE, Niparko JK. Imaging quiz case 1. Tegmental encephalocele. Arch Otolaryngol Head
Neck Surg. 1998 Nov;124(11):1274, 1276-7.
8. Jackler RK, De La Cruz A. The large vestibular aqueduct syndrome. Laryngoscope 1989; 99:1238-1242;
discussion 1242-1233.
9. Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on
embryogenesis. Laryngoscope 1987; 97:2-14.
10. Keen JA. Absence of both internal carotid arteries. Clin Proc 1945-1946;4:588
11. Mafee MF, Charletta D, Kumar A, Belmont H. Large vestibular aqueduct and congenital sensorineural hearing
loss. AJNR Am J Neuroradiol 1992; 13:805-819.
12. Martinez-Granero MA, Martinez-Bermejo A, Arcas J, et al. [Unilateral agenesis of the internal carotid artery in
childhood: description of a case]. Rev Neurol 1997; 25:1207-1209.
13. Mayer TE, Brueckmann H, Siegert R, Witt A, Weerda H. High-resolution CT of the temporal bone in dysplasia of
the auricle and external auditory canal. AJNR Am J Neuroradiol 1997; 18:53-65.
14. Overton SB, Ritter FN. A high placed jugular bulb in the middle ear: a clinical and temporal bone study.
Laryngoscope 1973; 83:1986-1991.
15. Paparella MM. Mondini's deafness. A review of histopathology. Ann Otol Rhinol Laryngol Suppl 1980; 89:1-10.
16. Pappas DG, Jr., Hoffman RA, Cohen NL, Holliday RA, Pappas DG, Sr. Petrous jugular malposition (diverticulum).
Otolaryngol Head Neck Surg 1993; 109:847-852.
17. Peron DL, Schuknecht HF. Congenital cholesteatomata with other anomalies. Arch Otolaryngol 1975; 101:498-
505.
18. Robson CD, Robertson RL, Barnes PD. Imaging of pediatric temporal bone abnormalities. Neuroimaging Clin N
Am 1999; 9:133-155.
19. Romo LV, Casselman JW, Robson CD. Temporal Bone: Congenital Anomalies. In: Som P.M., Curtin H.D. (eds)
Head and Neck Imaging, 4th edn. Mosby-Year Book Inc., St. Louis, 2003, pp: 1275-1360.
20. Sinnreich AI, Parisier SC, Cohen NL, Berreby M. Arterial malformations of the middle ear. Otolaryngol Head
Neck Surg 1984; 92:194-206.
21. Smith ME, Fisher C, Weiss SW. Pleomorphic hyalinizing angiectatic tumor of soft parts. A low-grade neoplasm
resembling neurilemoma. Am J Surg Pathol 1996; 20:21-29.
22. Thiers FA, Sakai O, Poe DS, Curtin HD. Persistent stapedial artery: CT findings. AJNR Am J Neuroradiol 2000;
21:1551-1554.
23. Valvassori GE, Clemis JD. The large vestibular aqueduct syndrome. Laryngoscope 1978; 88:723-728.

Imaging of the Temporal Bone: Infectious
and Neoplastic Lesions
Figure 5-5-1
Conductive Hearing Loss
• Ossicular motion impeded
➢ Cholesteatoma
➢ Hemangioma
➢ Glomus tympanicum
➢ Trauma: disruption
➢ Congenital
➢ Otosclerosis
Inflammatory Disease
• Mechanism
➢ Eustachian tube dysfunction
➢ Decreased intratympanic pressure
➢ Children: otitis media
➢ Adults: nasopharyngeal carcinoma
• Thin-section CT: soft tissue and fluid look alike
➢ Hounsfield units not helpful
Nemzek and Schwartz in Som and Curtin,
Head and Neck Imaging, 4th ed, 2003, Mosby
Artist rendition of retraction pocket at
superior tympanic annulus caused by
Acquired Cholesteatoma [Figures 5-5-1 to 5-5-5]
negative intratympanic pressure
• Exfoliated keratin within sac of stratified squamous epithelium
• 98% of middle ear cholesteatomas
➢ Probably from retraction pocket in pars flaccida
• Prussak’s space: ossicles displaced medially
• Bone destruction Figure 5-5-2
➢ Scutum and ossicles:coronal plane best
➢ Pars tensa: lateral semicircular canal, axial plane best
Buckingham and Valvassori, Otolaryngol Clin North Am 1973; 6:363
Figure 5-5-3
Retraction pocket fills with epithelial

debris from external auditory canal,
creating a cholesteatoma
Gross photograph of an acquired cholesteatoma,
a sac of keratin lined with squamous epithelium
Temporal Bone Infectious and Neoplastic Lesions 1076 Neuroradiology

Acquired Cholesteatoma Figure 5-5-4
• May not be able to distinguish from simple debris
early in course
• MR: T1 & T2 prolongation
• Does not enhance (granulation tissue does)
• Treatment: excision or exteriorization
➢ Open cavity (canal wall down) mastoidectomy
❖ Radical: stapes left
❖ Modified radical (Bondy): all ossicles left
Phelps and Lloyd, Radiology 1986; 37:359-364;
O’Donoghue et al, Clin Otolaryngol 1987; 12:89; Ishii et
al, JCAT 1991; 15:934-937; Nemzek and Schwartz in
Som and Curtin, Head and Neck Imaging, 4th ed.,
Mosby, 2003
Coronal CT image of acquired cholesteatoma with
Acquired Cholesteatoma: Complications erosion of the scutum and ossicles
[Figure 5-5-6]
• Labyrinthine fistula: lateral SCC most common
➢ Labyrinthitis Figure 5-5-5
• Facial nerve canal
• ∗Tegmen tympani: intracranial
• ∗Sigmoid sinus erosion / thrombosis
• “Automastoidectomy”: into EAC
∗MR recommended
Silver et al, Radiology 1987; 164:47; Schwartz,

Radiology 1984; 153:443-447; Nemzek and Schwartz in
Som and Curtin,
Head and Neck Imaging, 4th ed, Mosby, 2003
Middle ear “soft tissue” without bone erosion (not

cholesteatoma!). Left: granulation tissue. Right:
middle ear fluid
Figure 5-5-6
Cholesteatoma with erosion of

mastoid bone adjacent to sigmoid
dural sinus
Neuroradiology 1077 Temporal Bone Infectious and Neoplastic Lesions

Mastoiditis [Figures 5-5-7 and 5-5-8] Figure 5-5-7
• Osteomyelitis: patchy opacification
➢ Loss of mastoid septations
➢ Demineralization
• Coalescent: single cystic cavity
• Complications
➢ Bezold abscess: zygomatic root, EAC
➢ Gradenigo syndrome: petrous apicitis
❖ VI palsy, V neuralgia, chronic otitis
➢ Sigmoid sinus thrombosis
➢ Meningitis, epidural abscess
Castillo et al, AJR 1998; 17:1491-1495; Mafee et al, Radiology 1985;
155:391
Necrotizing External Otitis

• Osteomyelitis: bone destruction
• Diabetics: Pseudomonas
• AIDS: Aspergillus
• Cartilage portion: fissures of Santorini Coalescent mastoiditis. Only a
➢ Spreads rapidly into adjacent spaces single cavity remains within the
➢ Parotid, facial nerve, intracranial mastoid bone as a result of
• Goal: determine extent of disease by CT and MR osteomyelitis
• In-111 WBC study: post-therapy
Slattery and Brackmann, Otolaryngol Clin North Am 1996; 29:795-806; Ress et al,
Laryngoscope 1997; 107:456-460; Grandis et al, Radiology 1995: 196:499-504
Figure 5-5-8
External Ear Masses [Figure 5-5-9]
• Exostosis: chronic cold water exposure
• Usually broad-based and bilateral
• Bony portion of EAC
• Not an osteoma
➢ Usually unilateral, pedunculated,
and lateral to EAC
DiBartolomeo, Ann Otolaryngol 1979; 88(suppl 61):2-20;
Turetsky et al, AJNR 1990; 11:1217-1218
External Ear Masses

• Keratosis obturans
➢ < 40 years old
➢ Sinusitis, bronchiectasis Mastoiditis with posterior fossa epidural abscess
➢ Hearing loss (Case courtesy of Vanessa Albernaz, MD)
➢ Smooth external auditory canal (EAC) widening
➢ Entire EAC often filled
• EAC cholesteatoma: 0.5% of all cholesteatomas
➢ Otorrhea
➢ Focal erosions Figure 5-5-9
Piepergerdes et al, Laryngoscope 1980; 90:383-391
Bilateral exostoses

External Ear Neoplasms [Figure 5-5-10] Figure 5-5-10
• Skin cancers
❖ Most common malignant ear tumor
➢ Basal cell carcinoma
➢ Melanoma
• “Ceruminoma”
• Parotid tumors
• Metastasis
Schuknecht, Pathology of the Ear, Harvard, 1974; Maya et al in Som
and Curtin, Head Neck Imaging, 4th ed, Mosby, 2003
Cerebellopontine Angle Masses: The “AMEN”

• Acoustic schwannoma (60–91%)
• Meningioma (3%-7%)
• Epidermoid (2%-6%)
• Nonacoustic schwannoma (1%-5%): V, VII
• Others
➢ Ependymoma, medulloblastoma, pilocytic astrocytoma
➢ Paraganglioma External auditory canal erosion
➢ Arachnoid cyst secondary to neoplasm
➢ Lipoma, dermoid, teratoma
Brackmann and Bartels, Otolaryngol Head Neck Surg 1980; 88:555-559;
Valavanis et al, Clinical Imaging of the Cerebellopontine Angle, Springer-Verlag,
1980; Gonzalez-Revilla, Johns Hopkins Hosp Bull 1948(83):187-189 Figure 5-5-11
Acoustic Schwannoma [Figure 5-5-11]

• 8%-10% of intracranial tumors
• 60%-90% of CPA tumors
• Most: 30-70 years old
• Neurofibromatosis type 2 (NF2): children, bilateral
in 96%
➢ Schwann cell tumors, multiple meningiomas,
gliomas
➢ First-degree relative counseling +/- imaging
screening
Kasantikul et al, J Neurosurg 1980; 52:28-35; Martuza Canalicular vestibular schwannoma with smooth
and Eldridge, N Engl J Med 1988; 318:684-688; Kishore remodeling of the canal wall and loss of crista
and O’Reilly, Clin Otolaryngol 2000; 25:561-565 falciformis
Acoustic (Vestibular) Schwannoma [Figure 5-5-12]

• Sensorineural hearing loss, vertigo, tinnitus
➢ Speech discrimination impaired: telephone use
Figure 5-5-12
• Arise from vestibular division CN VIII usually
➢ Direct pressure on cochlear division
• Benign neoplasm
➢ Slow growth (0.2 cm per year)
➢ Well circumscribed globular mass
• Histology: Antoni A and B fibers [Figure 5-12-8]
Komatsuzaki and Tsunoda, J Laryngol Otol 2001;
115:376-379; NIH Consensus Development Conference,
Arch Neurol 1994; 51:201-207 ; Lanser et al, Otolaryngol
Clin North Am 1992; 25:499-520
Antoni A (left) and Antoni B (right) cell populations

of a schwannoma

Vestibular Schwannoma: Imaging Figure 5-5-13
[Figures 5-5-13 to 5-5-16]
• IAC widening with “mushroom expansion
➢ “Giant”: usually no IAC involvement
• CT: usually isodense to cerebellum
➢ Calcification and hemorrhage rare
• T1WI: iso- to hypointense
• T2WI: hyperintense
• Intense enhancement: into porus acousticus and no
dural tail
Maya et al in Som and Curtin, Head and Neck Imaging,
4th ed, Mosby, 2003; Moller et al, Neuroradiology 1978;
17:25-30; Tali et al, AJNR 1993; 14:1241-1247;
Schmalbrock et al, AJNR 1999; 20:1207-1213
Vestibular schwannoma with classic mushroom
morphology on pre-contrast and post-contrast
axial T1-weighted images
Figure 5-5-14
Figure 5-5-15
Coronal T2-weighted FSE image of

right-sided vestibular schwannoma
Figure 5-5-16
Focal enhancement of deep portion of
internal auditory canal secondary to
arteriovenous malformation (not
vestibular schwannoma)
Cystic degeneration of large vestibular

schwannoma

Vestibular Schwannoma: Therapy Figure 5-5-17
➢ Larger masses
❖ Translabyrinthine: protect facial nerve
➢ Smaller masses
❖ Retrosigmoid: suboccipital approach
❖ Middle cranial fossa
• Stereotactic radiosurgery (gamma knife) up to 4 cm
• Poor surgical risk patients: serial MR
Jackler and Pitts, Otolaryngol Clin North Am 1992; 25:361-387;
House and Shelton, Otolaryngol Clin North Am 1992; 25:347-359;
Fucci et al, Am J Otol 1999; 20:497-508; Nakamura et al, AJNR 2000;
21:1540-1546
Meningioma [Figures 5-5-17 and 5-5-18]

• Usually eccentric to porus acousticus
➢ IAC involvement uncommon (16%)
➢ Frequently “trans-spatial”
• Broad dural base: hemispherical
➢ Obtuse bone-tumor angle
➢ Dural tail: 52%-72% Tentorial meningioma with extension
➢ Hyperostosis: highly characteristic into cerebellopontine angle
• NCCT:usually hyperdense (calcification: 25%)
• T1WI: isointense to gray matter
• T2WI: variable Figure 5-5-18
House and O’Conner, Handbook of Neurotological
Diagnosis, Marcel-Dekker, 1987; Valavanis et al,
Neuroradiology 1981; 22:111-121; Moller et al;
Neuroradiology 1978; 17:25-30
Epidermoid [Figure 5-5-19]

• Soft, “pearly tumor”
• Irregular margins: “cauliflower”
• Follows CSF density and signal
• Usually no enhancement
• Diffusion-weighted imaging: hyperintense to CSF
• Differential Diagnosis: arachnoid cyst, cysticercosis,
atypical dermoid, lipoma
Cerebellopontine angle meningioma with
Berger and Wilson. J Neurosurg 1985; 62:214-219;
numerous flow voids and fluid-fluid level. Note
Gao et al, AJNR 1992; 13:863-872;
extension through foramina
Tampieri et al, AJNR 1989; 10:351-356;
Tsuruda et al, AJR 1990; 155:1059-1065
Facial Nerve Palsy Figure 5-5-19

• MR: imaging study of choice
• Bell’s palsy: > 50%, nerve not enlarged
➢ Idiopathic, by definition (probably HSV)
➢ Imaging usually not performed
• Tumors: 6%, enlarged nerve
➢ Geniculate ganglion
❖ Schwannoma
❖ Hemangioma
➢ Epidermoid
➢ Parotid tumor spread
Tien et al, AJNR 1990; 11:735-741; Daniels et al,
Radiology 1989; 17:807-809
Epidermoid of middle cranial fossa with extension

into posterior fossa

Temporal Bone Paragangliomas Figure 5-5-20
• Glomus jugulare: jugular foramen
➢ Jacobson’s (IX) and Arnold’s (X) nerve
• Glomus tympanicum: cochlear promontory
➢ Most common middle ear tumor
➢ Most common etiology of retrotympanic vascular mass
• Vagal paraganglioma: jugular ganglion
• Females 5:1; peak age: 40-60 years old
Rao et al, RadioGraphics 1999; 19:1605-1632
Jugulotympanic Paraganglioma [Figure 5-5-20]

• Neuroendocrine tumor
➢ Paraganglia: chemoreceptor function
➢ Functioning: 1%-3%, catecholamine secretion
• Early symptoms
➢ Conductive HL, pulsatile tinnitus
• Slow growth but locally invasive
➢ Mortality rate: 15%
• Metastasis very rare
• Path: chief cells (“Zellballen”), sustentacular cells
Rao et al, RadioGraphics 1999; 19:1605-1632 “Zellballen” histologic appearance of
paraganglioma
Jugulotympanic Paraganglioma: Imaging Figure 5-5-21

[Figures 5-5-21 to 5-5-23]
• CT: irregular margins, “moth-eaten” erosion
• Glomus jugulare: may extend down carotid sheath
• MR: “Salt and pepper” appearance
➢ “Salt”: hyperintense foci (slow flow, hemorrhage)
➢ “Pepper”: serpentine flow voids
• Intense enhancement
• Angiography: ascending pharyngeal artery
• Radiologist’s goal: define extent
Rao et al, RadioGraphics 1999; 19:1605-1632
Figure 5-5-22
Glomus tympanicum (paraganglioma)

(Case courtesy of William Kelly, MD)
Glomus jugulotympanicum (paraganglioma)

Figure 5-5-23
“Salt-and-pepper” appearance of glomus jugulare on MR images
Jugular Foramen Masses

• Paraganglioma: 90%
• Schwannoma: 9%
• Meningioma: <1% Figure 5-5-24
• Malignant neoplasms: <1%
➢ Carcinoma
➢ Sarcoma
➢ Mets
Papillary Endolymphatic Sac Tumor [Figure 5-5-24] Papillary

• Ipsilateral hearing loss, facial nerve palsy, vestibular endolymphatic sac
dysfunction tumor in different
• Females more common patients
• von Hippel-Lindau association
• Adenomatous tumor
• Bone destruction, intratumoral bone spicules
Heffner, Cancer 1989; 64:2292-2302; Lo, AJNR 1993;14:1322-
1323; Palmer et al, Otolaryngol Head Neck Surg 1989; 100:64-
68; Mukherji et al, Radiology 1997; 202:801-808
Differential Diagnosis Petrous Apex

• Cholesterol granuloma (cyst): most common
• Epidermoid
➢ Follows cerebrospinal fluid (CSF) signal
➢ Solid mass: resection
• Chondrosarcoma
• Mucocele
• Carotid artery aneurysm
• Meningocele
Curtin and Som, Otolaryngol Clin North Am 1995; 28:473-496
Cholesterol Granuloma (Cyst)

• Retention cyst: obstruction in petrous apex
➢ “Chocolate cyst”, “Blue-domed cyst”: within mastoidectomy cavity
• Young, middle-aged adults
• Hearing Loss, tinnitus, cranial nerve palsies
• Hemorrhage and foreign-body reaction: cholesterol crystals and blood
(brownish fluid)
Lo et al, Radiology 1984;153:705-711; Graham et al, Laryngoscope 1985;
95:1401-1406; Latack et al, AJNR 1985; 6:409-413; Griffin et al, AJNR 1988;
8:825-829; Greenberg et al, AJNR 1988; 9:1205-1214

Cholesterol Granuloma (Cyst) [Figure 5-5-25] Figure 5-5-25
• CT
➢ Isodense to brain
➢ Expansile, especially posterior
➢ Sharp smooth margins
• MR: hemorrhage
Lo et al, Radiology 1984;153:705-711;
Latack et al, AJNR 1985; 6:409-413;
Chang et al, Laryngoscope 1998; 108:599-604;
Muckle et al, Am J Otol 1998: 19:219-225;
Palacios and Valvassori, Ear Nose Throat J 1999;
78:234
Cholesterol granuloma
Chondrosarcoma [Figure 5-5-26]
• Most common primary neoplasm of petrous apex
• Off the midline: sutures
➢ Petrosphenoidal
➢ Petro-occipital
• Locally invasive
• Bone destruction: no sclerosis
• T1 and T2 prolongation
• Intense but heterogeneous enhancement
Grossman and Davis, Radiology 1981; 141:403-408; Meyers et al, Radiology
1992; 184:103-108; Bourgouin et al, JCAT 1992; 16:268-273
Temporal Bone Fracture
Longitudinal Transverse
Frequency 80% 20%
Axis Long Short
Blow Temporoparietal Occipital
Middle ear injury Likely Rare
Inner ear injury Rare Common
Facial paralysis 10–20%, usually 40–50%, usually
incomplete & acute & complete
delayed
Tegmen tympani Common Less common
disruption
Schwartz and Curtin in Som and Curtin,
Head and Neck Imaging, 4th ed., Mosby, 2003
Figure 5-5-26
Chondrosarcoma

Ossicular Derangement [Figure 5-5-27]
• Need 1 mm CT images or thinner
• Incus: most vulnerable
➢ Subluxation from malleus
➢ Dislocation
• Incudostapedial disruption
➢ Most common cause of post-traumatic conductive HL
➢ Normal: <1 mm between lenticular process of incus & stapes head
Lourenco et al, Am J Otol 1995; 16:387-392; Swartz et al; Radiology 1989; Figure 5-5-27
171:309-317
Otosclerosis
• Primary endochondral bone within otic capsule replaced by
spongy vascular bone (“otospongiosis”)
• Slowly progressive
• Presents 10–30 years old with tinnitus
➢ Hearing loss later
• Females more common (70%)
• Bilateral 80% (usually asynchronous)
Valvassori, Otolaryngol Clin North Am, 1973; 6:379-389;
Reudi, Arch Otolaryngol 1963; 78:469-477
Otosclerosis
• Fenestral type (80%): CHL
➢ Begins at anterior oval window
• Cochlear type (20%): SNHL
➢ Almost always with fenestral type
➢ “Double Ring” sign
Ossicular derangement
• Demineralized areas: active disease
• Chronic disease: may appear normal
• MR: punctate enhancement
Mafee et al, Radiology 1985; 156:703-708; Swartz et al, Radiology 1985; 155:147-
150; Sakai et al, Am J Otolaryngol 2000; 21:116-118
Summary
• Challenging complex anatomy
• Facial nerve course: critical for pre-operative evaluation
• Bony plate between hypotympanum and ICA canal
• Tegmen tympani
• Cholesteatoma: bone erosion
• Cerebellopontine Angle: “AMEN”
➢ Vestibular schwannoma: most common
➢ Meningioma
➢ Epidermoid
➢ Non-acoustic schwannoma
• Jugular foramen
➢ Paraganglioma
➢ Schwannoma
• Petrous apex
➢ Cholesterol granuloma vs. epidermoid
➢ Chondrosarcoma, Chordoma, Metastasis
• Otosclerosis
• Radiologist’s Goals
➢ Define extent of lesion
References
1. Berger MS, Wilson CB. Epidermoid cysts of the posterior fossa. J Neurosurg 1985;
62:214-219.

2. Bourgouin PM, Tampieri D, Robitaille Y, et al. Low-grade myxoid chondrosarcoma of the base of the skull: CT,
MR, and histopathology. J Comput Assist Tomogr 1992; 16:268-273.
3. Brackmann DE, Bartels LJ. Rare tumors of the cerebellopontine angle. Otolaryngol Head Neck Surg 1980; 88:555-
559.
4. Buckingham RA, Valvassori GE. Tomographic evaluation of cholesteatomas of the middle ear and mastoid.
Otolaryngol Clin North Am 1973; 6:363-378.
5. Griffin C, De La Paz R, Enzmann D MR And CT correlation of cholesterol cysts of petrous bone. AJNR, 1987,
8:825-829.
6. Castillo M, Albernaz VS, Mukherji SK, Smith MM, Weissman JL. Imaging of Bezold's abscess. AJR Am J
Roentgenol 1998; 171:1491-1495.
7. Chang P, Fagan PA, Atlas MD, Roche J. Imaging destructive lesions of the petrous apex. Laryngoscope 1998;
108:599-604.
8. Curtin HD, Som PM. The petrous apex. Otolaryngol Clin North Am 1995; 28:473-496.
9. Daniels DL, Czervionke LF, Millen SJ, et al. MR imaging of facial nerve enhancement in Bell palsy or after
temporal bone surgery. Radiology 1989; 171:807-809.
10. DiBartolomeo JR. Exostoses of the external auditory canal.Ann Otol Rhinol Laryngol Suppl. 1979 Nov-Dec;88(6
Pt 2 Suppl 61):2-20.
11. Fucci MJ, Buchman CA, Brackmann DE, Berliner KI. Acoustic tumor growth: implications for treatment choices.
Am J Otol 1999; 20:495-499.
12. Gao PY, Osborn AG, Smirniotopoulos JG, Harris CP. Radiologic-pathologic correlation. Epidermoid tumor of the
cerebellopontine angle. AJNR Am J Neuroradiol 1992; 13:863-872.
13. Gonzales-Revilla A. Differential diagnosis of tumors at the cerebellopontile recess. Bulletin of the Johns Hopkins
Hospital1948, 83: 187
14. Graham MD, Kemink JL, Latack JT, Kartush JM. The giant cholesterol cyst of the petrous apex: a distinct clinical
entity. Laryngoscope 1985; 95:1401-1406.
15. Grandis JR, Curtin HD, Yu VL. Necrotizing (malignant) external otitis: prospective comparison of CT and MR
imaging in diagnosis and follow-up. Radiology 1995; 196:499-504.
16. Greenberg JJ, Oot RF, Wismer GL, et al. Cholesterol granuloma of the petrous apex: MR and CT evaluation. AJNR
17. Grossman RI, Davis KR. Cranial computed tomographic appearance of chondrosarcoma of the base of the skull.
Radiology 1981; 141:403-408.
18. Heffner DK. Low-grade adenocarcinoma of probable endolymphatic sac origin A clinicopathologic study of 20
cases. Cancer 1989; 64:2292-2302.
19. House JW; O'Connor AF, Marcel Dekker, ed. Handbook of Neurotological Diagnosis, Inc., Science and Practice of
Surgery, Marcel-Dekker,1987.
20. House WF, Shelton C. Middle fossa approach for acoustic tumor removal. Otolaryngol Clin North Am 1992;
25:347-359.
21. Ishii K, Takahashi S, Kobayashi T, Matsumoto K, Ishibashi T. MR imaging of middle ear cholesteatomas. J
Comput Assist Tomogr 1991; 15:934-937.
22. Jackler RK, Pitts LH. Selection of surgical approach to acoustic neuroma. Otolaryngol Clin North Am 1992;
25:361-387.
23. Kasantikul V, Netsky MG, Glasscock ME, 3rd, Hays JW. Acoustic neurilemmoma. Clinicoanatomical study of 103
patients. J Neurosurg 1980; 52:28-35.
24. Kishore A, O'Reilly BF. A clinical study of vestibular schwannomas in type 2 neurofibromatosis. Clin Otolaryngol
Allied Sci 2000; 25:561-565.
25. Komatsuzaki A, Tsunoda A. Nerve origin of the acoustic neuroma. J Laryngol Otol 2001; 115:376-379.
26. Lanser MJ, Sussman SA, Frazer K. Epidemiology, pathogenesis, and genetics of acoustic tumors. Otolaryngol Clin
North Am 1992; 25:499-520.
27. Latack JT, Graham MD, Kemink JL, Knake JE. Giant cholesterol cysts of the petrous apex: radiologic features.
28. Lo WW, Solti-Bohman LG, Brackmann DE, Gruskin P. Cholesterol granuloma of the petrous apex: CT diagnosis.
Radiology 1984; 153:705-711.
29. Lourenco MT, Yeakley JW, Ghorayeb BY. The "Y" sign of lateral dislocation of the incus. Am J Otol 1995;
16:387-392.
30. Mafee MF, Singleton EL, Valvassori GE, Espinosa GA, Kumar A, Aimi K. Acute otomastoiditis and its
complications: role of CT. Radiology 1985; 155:391-397.
31. Mafee MF, Valvassori GE, Deitch RL, et al. Use of CT in the evaluation of cochlear otosclerosis. Radiology 1985;
156:703-708.
32. Martuza RL, Eldridge R. Neurofibromatosis 2 (bilateral acoustic neurofibromatosis). N Engl J Med 1988; 318:684-
688.

33. Maya M.M., Lo W.W.M., Kouvanlikaya I. Tumors and cerebellopontine angle lesions. In: Som P.M., Curtin H.D.
(eds) Head and Neck Imaging, 4th edn. Mosby-Year Book Inc., St. Louis, 2003, pp: 1275-1360.
34. Meyer JR, Gebarski SS, Blaivas M. Cerebellopontine angle invasive papillary cystadenoma of endolymphatic sac
origin with temporal bone involvement. AJNR Am J Neuroradiol 1993; 14:1319-1321; Discussion Lo, 1322-1313.
35. Meyers SP, Hirsch WL, Jr., Curtin HD, Barnes L, Sekhar LN, Sen C. Chondrosarcomas of the skull base: MR
imaging features. Radiology 1992; 184:103-108.
36. Moller A, Hatam A, Olivecrona H. Diagnosis of acoustic neuroma with computed tomography. Neuroradiology
1978; 17:25-30.
37. Muckle RP, De la Cruz A, Lo WM. Petrous apex lesions. Am J Otol 1998; 19:219-225.
38. Mukherji SK, Albernaz VS, Lo WW, et al. Papillary endolymphatic sac tumors: CT, MR imaging, and
angiographic findings in 20 patients. Radiology 1997; 202:801-808.
39. Nakamura H, Jokura H, Takahashi K, Boku N, Akabane A, Yoshimoto T. Serial follow-up MR imaging after
gamma knife radiosurgery for vestibular schwannoma. AJNR Am J Neuroradiol 2000; 21:1540-1546.
40. National Institutes of Health Consensus Development Conference Statement on Acoustic Neuroma, December 11-
13, 1991. The Consensus Development Panel. Arch Neurol 1994; 51:201-207.
41. Nemzek WR, Swartz JD. Temporal Bone: Inflamatory Disease. In: Som P.M., Curtin H.D. (eds) Head and Neck
Imaging, 4th edn. Mosby-Year Book Inc., St. Louis, 2003, pp: 1173.
42. O'Donoghue GM, Bates GJ, Anslow P, Rothera MP. The predictive value of high resolution computerized
tomography in chronic suppurative ear disease. Clin Otolaryngol Allied Sci 1987; 12:89-96.
43. Palacios E, Valvassori G. Petrous apex lesions: cholesterol granuloma. Ear Nose Throat J 1999; 78:234.
44. Palmer JM, Coker NJ, Harper RL. Papillary adenoma of the temporal bone in von Hippel-Lindau disease.
Otolaryngol Head Neck Surg 1989; 100:64-68.
45. Phelps PD, Lloyd GA. Vascular masses in the middle ear. Clin Radiol. 1986 Jul;37(4):359–364.
46. Piepergerdes MC, Kramer BM, Behnke EE. Keratosis obturans and external auditory canal cholesteatoma.
Laryngoscope 1980; 90:383-391.
47. Rao AB, Koeller KK, Adair CF. From the archives of the AFIP. Paragangliomas of the head and neck: radiologic-
pathologic correlation. Armed Forces Institute of Pathology. Radiographics 1999; 19:1605-1632.
48. Ress BD, Luntz M, Telischi FF, Balkany TJ, Whiteman ML. Necrotizing external otitis in patients with AIDS.
Laryngoscope 1997; 107:456-460.
49. Ruedi L. Pathogenesis of Otosclerosis. Arch Otolaryngol 1963; 78:469-477.
50. Sakai O, Curtin HD, Fujita A, Kakoi H, Kitamura K. Otosclerosis: computed tomography and magnetic resonance
findings. Am J Otolaryngol 2000; 21:116-118.
51. Schmalbrock P, Chakeres DW, Monroe JW, Saraswat A, Miles BA, Welling DB. Assessment of internal auditory
canal tumors: a comparison of contrast-enhanced T1-weighted and steady-state T2-weighted gradient-echo MR
imaging. AJNR Am J Neuroradiol 1999; 20:1207-1213.
52. Schuknecht, H. F. Pathology of the ear. Cambridge: Harvard University Press, 1974.
53. Silver AJ, Janecka I, Wazen J, Hilal SK, Rutledge JN. Complicated cholesteatomas: CT findings in inner ear
complications of middle ear cholesteatomas. Radiology 1987; 164:47-51.
54. Slattery WH, 3rd, Brackmann DE. Skull base osteomyelitis. Malignant external otitis. Otolaryngol Clin North Am
1996; 29:795-806.
55. Swartz JD, Curtin HD. Temporal Bone: Trauma. In: Som P.M., Curtin H.D. (eds) Head and Neck Imaging, 4th edn.
Mosby-Year Book Inc., St. Louis, 2003, pp: 1173.
56. Swartz JD, Mandell DW, Berman SE, Wolfson RJ, Marlowe FI, Popky GL. Cochlear otosclerosis (otospongiosis):
CT analysis with audiometric correlation. Radiology 1985; 155:147-150.
57. Swartz JD. Current imaging approach to the temporal bone. Radiology 1989; 171:309-317.
58. Swartz JD. The facial nerve canal: CT analysis of the protruding tympanic segment. Radiology 1984; 153:443-447.
59. Tali ET, Yuh WT, Nguyen HD, et al. Cystic acoustic schwannomas: MR characteristics. AJNR Am J Neuroradiol
1993; 14:1241-1247.
60. Tampieri D, Melanson D, Ethier R. MR imaging of epidermoid cysts. AJNR Am J Neuroradiol 1989; 10:351-356.
61. Tien R, Dillon WP, Jackler RK. Contrast-enhanced MR imaging of the facial nerve in 11 patients with Bell's palsy.
62. Tsuruda JS, Chew WM, Moseley ME, Norman D. Diffusion-weighted MR imaging of the brain: value of
differentiating between extraaxial cysts and epidermoid tumors. AJR Am J Roentgenol 1990; 155:1059-1065;
discussion 1066-1058.
63. Turetsky DB, Vines FS, Clayman DA. Surfer's ear: exostoses of the external auditory canal. AJNR Am J
Neuroradiol 1990; 11:1217-1218.
64. Valavanis A, Schubiger O, Hayek J, Pouliadis G. CT of meningiomas on the Posterior surface of the petrous bone.
Neuroradiology 1981; 22:111-121.
65. Valavanis A, Schubiger O, Naidich TP: Clinical Imaging of the Cerebello-Pontine Angle. Berlin: Springer-Verlag,
1987.
66. Valvassori GE. Otosclerosis. Otolaryngol Clin North Am 1973; 6:379-389.

Imaging of the Orbit:
The Globe and Conal Lesions
Figure 5-6-1
The Bony Orbit [Figure 5-6-1]
• Bones
➢ Frontal
➢ Maxilla
➢ Sphenoid
➢ Zygoma
➢ Ethmoid
➢ Lacrimal
➢ Palatine
The Bony Orbit

• Superior orbital fissure
➢ Middle cranial fossa
➢ Cranial nerves III, IV, VI, V1
➢ Superior and inferior ophthalmic veins
• Inferior orbital fissure The bony orbit
➢ Pterygopalatine (V2) & infratemporal fossae
Figure 5-6-2
Anterior Orbit [Figures 5-6-2 and 5-6-3]
• Orbital septal system
➢ Anterior: well-developped (“preseptal space”)
❖ Arises from periosteum of anterior bony orbit
❖ Attaches to tarsal plates of eyelids
➢ Posterior: incomplete
• Lacrimal gland
• Lacrimal sac and nasolacrimal duct
Globe [Figure 5-6-4]

• Anterior chamber: aqueous humor
Lacrimal glands in superolateral
• Iris and ciliary body
portion of the bony orbit
• Posterior chamber: aqueous humor
• Lens apparatus
Figure 5-6-3
• Vitreous body: gel-like (collagen fibrils)
➢ Most: free water
Figure 5-6-4
Nasolacrimal ducts in inferomedial

portions of bony orbit
Normal globe with anterior and

posterior chambers located anterior
to the lens and the vitreous body
constituting most of the globe
Imaging of the Orbit: The Globe and Conal Lesions 1088 Neuroradiology
Globe [Figure 5-6-5] Figure 5-6-5
• Retina
➢ Neural sensory inner layer (photoreceptors)
➢ Retinal pigmented epithelium (RPE) outer layer
➢ Ora serrata
• Uvea: choroid (vascular); Bruch’s membrane
➢ Iris
➢ Ciliary body
• Sclera: fibrous layer; cornea anteriorly
• Tenon’s capsule (bulbar fascia)
• Normal: only one “layer” seen
Retrobulbar (“Postseptal”) Space

[Figures 5-6-6 to 5-6-8]
• Fat with fibrous septa
• Extraocular muscles (EOM) (“The Cone”)
➢ Rectus: medial, lateral, superior, inferior
❖ Annulus of Zinn: optic canal
❖ Intermuscular septa (incomplete posteriorly)
➢ Oblique: superior (trochlear), inferior
Close-up view of the posterior globe
➢ Levator palpebrae superioris
layers. Retina is innermost, followed
• Optic nerve: glial-lined
by choroid and sclera. The macula
• Vessels
is located lateral to the optic disk
Extraocular muscles. The superior oblique

muscle travels through the trochlea, a bony Coronal view of the six extraocular
strut near the superomedial margin of the muscles and levator palpebrae
bony orbit superioris
Figure 5-6-8
Papilledema with increased fluid

surrounding optic nerves bilaterally
caused by a supratentorial
oligodendroglioma
Neuroradiology 1089 Imaging of the Orbit: The Globe and Conal Lesions
Cranial Nerves III, IV, VI Figure 5-6-9
• Motor control of EOMs
• Cranial Nerve III: all EOMs except
➢ Lateral rectus: Cranial Nerve VI
➢ Superior oblique: Cranial Nerve IV
➢ “LR6SO4”
• Sensory control: V1 primarily, V2 (infraorbital region)
Senile Macular Degeneration

• Most common cause of legal blindness in the elderly
• Hyalinization of macula, thickening of Bruch’s membrane
➢ Pigment epithelium detachment
➢ Serous subretinal space fluid
• Hemorrhage: fibrous scar, macular loss
• Computed tomography (CT): mimics uveal melanoma
• Magnetic resonance (MR): variable
Mafee, in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby,
2003
Posterior Hyaloid Detachment

• Separation of the hyaloid base (posterior hyaloid membrane) Retinal detachment, with
from retinal sensory epithelium characteristic V-shape created by
• Usually adults with myopia anchor points at optic disk and ora
➢ Liquefaction of vitreous serrata
➢ Children: persistent hyperplastic primary vitreous
• Association: macular degeneration
• Intravitreal and curvilinear layer
➢ Not connected to optic disk
Mafee in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003
Retinal Detachment (RD) [Figure 5-6-9]

• Sensory retina separates from RPE
• Rhegmatogenous RD: tear in retina
• RPE: can heal (laser therapy) Figure 5-6-10
• Ultrasonography (US) superior to MR or CT for detection
➢ Causes: mass, fibroproliferative disease, toxocara, choroidal
lesions
• V-shape: apex at optic disk
Mafee and Peyman, Radiol Clin North Am 1987;25:487-507
Choroidal Detachment [Figure 5-6-10]

• Hemorrhagic: contusion
• Serous: ocular hypotony (choroidal inflammation, trauma,
glaucoma therapy)
• U-shaped
➢ Anchor points: short posterior ciliary artery, vortex veins
• No connection with optic disk
Mafee and Peyman, Radiol Clin North Am 1987;25:487-507
Leukocoria
• Retinoblastoma
• Persistent hyperplastic primary vitreous (PHPV)
• Retinopathy of prematurity (ROP)
• Congenital cataract Choroidal detachment, with typical U-
• Coats’ disease shape created by anchor points at
• Toxocariasis ciliary body and vessels away from
• Total retinal detachment the optic disk
Mafee, in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003
Imaging of the Orbit: The Globe and Conal Lesions 1088

1090 Neuroradiology
Retinoblastoma Figure 5-6-11
• Most common intraocular tumor of childhood
➢ Incidence 1:15,000
➢ Virtually all patients < 6 years-old
➢ 80% 3 years old or younger
➢ 13 months: average age at presentation
• No gender or racial predilection
• “Retinoblastoma gene”: chromosome 13q14
➢ “Germinal” (inherited) 85% bilateral
➢ “Somatic” (not inherited) unilateral
• Unilateral: 60%-70%
• Association: osteosarcoma, other sarcomas
Abramson et al, Ophthalmology 1984; 91:1351-1355; Pendergrass
and Davis, Arch Ophthalmol 1980; 98:1204-1210; Ellsworth, Trans
Am Ophthalmol Soc 1969; 67:462-534; Kaufman et al, Radiol Clin
North Am 1998; 36:1101-1117 Gross photograph of retinoblastoma
Retinoblastoma [Figure 5-6-11]

• Ophthalmoscopic diagnosis primarly Figure 5-6-12
➢ Small gray-white intraretinal lesions, calcification, seeding
➢ Ultrasonography: 80% accurate
• Stage 1: confined to the globe
• Stage 2: extraocular extension to orbit or optic nerve
• Stage 3: extra-orbital extension
• 92% 5-year survival for intra-ocular lesions but near 100%
mortality when extends beyond eye
Kodilyne, Am J Ophthalmol 1967; 63:467-481; Abramson et al, Arch
Ophthalmol 1981; 99:1761-1762
Retinoblastoma – Pathology
• Neuroectodermal origin: primitive embryonal retinal cells
(retinoblasts)
• Rosettes: Flexner-Wintersteiner or Homer-Wright type
• Highly malignant: necrosis, mitotic figures
• Calcification
Kyritsis et al, Nature 1984; 307:471-473
Retinoblastoma - Imaging [Figure 5-6-12]
• CT: imaging study of choice Retinoblastoma with characteristic
➢ Calcification: >90% of cases calcification on CT
❖ Child < 3 y/o: highly suggestive for diagnosis
• Tri- / tetralateral retinoblastoma with pineal and/or suprasellar masses
• MR: not as specific as CT
➢ Hyperintense on T1WI and PD
➢ Hypointense on T2WI
➢ May miss lesions as large as 4mm
➢ Better for intracranial extension, extraocular spread
Char, Ophthalmology 1984; 91:1347-1350; Mafee, Radiol Clin North Am 1987;
25:667-681; Mafee et al, Ophthalmology 1989; 96:965-976
Persistent Hyperplastic Primary Vitreous (PHPV) [Figure 5-6-13]

• Failure of embryonic hyaloid system (primary vitreous) to regress normally and
form the secondary vitreous by 5th-6th gestational month
• Isolated or part of more complex abnormality
➢ Bilateral: Norrie’s, Warburg’s
• Usually unilateral leukokoria and microphthalmos
➢ Lens opacity, RD, vitreous hemorrhage
• Persistent hyaloid (Cloquet’s) canal
➢ No calcification
Mafee and Goldberg, Radiol Clin North Am 1987; 25:683-692
Neuroradiology 1089
1091 Imaging of the Orbit: The Globe and Conal Lesions
Retinopathy of prematurity Figure 5-6-13
Coats Disease [Figure 5-6-14]

• Juvenile males most common
• Usually unilateral (85%-90%)
• Peripheral telangiectasias
➢ Leak lipoproteinaceous exudate
➢ Retinal detachment
• May mimic retinoblastoma clinically
Coats, R Lond Ophthalmol Hosp Rep 1908; 17:440-
525; Reese, Am J Ophthalmol 1956; 42:1-8; Edward et
al, Radiol Clin North Am 1988; 36: 1119-1131
Toxocariasis
• Chorioretinitis: Toxocara canis (nematode)
• Granuloma: eosinophilic abscess Persistent hyperplastic primary vitreous (PHPV)
• CT: homogeneous intravitreal density with hyaloid canal
➢ Retinal detachment, organized vitreous,
inflammatory exudate
➢ Irregular thickening of uveoscleral coat
• MR: subretinal exudate, variably hyperintense on all sequences
Margo et al, Pediatr Ophthalmol Strabismus 1983; 20: 180-184; Wilder, Trans Am
Acad Ophthalmol Otolaryngol 1950; 55:99-104 Figure 5-6-14
Uveal Melanoma
• Uvea
➢ Choroid, ciliary body, iris
➢ Derived from mesoderm & neuroectoderm
➢ Most highly vascular part of eyeball
• Melanoma: most common neoplasm of choroid
• Whites (15:1)
➢ Incidence increases with age
• Arises from choroid, elevates and may rupture Bruch’s
membrane (mushroom shape)
Yanoff and Fine, Ocular Pathology, Harper and Row, 1975; Mafee,
Radiol Clin North Am 1998; 36:1083-1099
Gross photograph of Coats Disease
Uveal Melanoma [Figure 5-6-15]
• Metastasis: liver > lung > bone > kidney > brain
• Diagnosis usually made by ophthalmoscopy or US
• CT: elevated, hyperdense, sharply marginated (usually) solid mass
• MR
➢ Hyperintense on T1WI and PDW
• Moderate enhancement
Duffin et al, Arch Ophthalmol 1981; 99:1827-1830; Enochs et al, Radiology 1997; Figure 5-6-15
204:417-423; Mafee in Som
and Curtin, Head and Neck
Imaging, 4th ed., Mosby,
2003
Uveal melanoma

1092 Neuroradiology
Uveal Metastasis [Figure 5-6-16] Figure 5-6-16
• Usually in the plane of the choroid with little increased thickness
• Mottled appearance, diffuse outline
• Breast and lung cancer most common
• Retina or choroid
• Bilateral 1/3 (melanoma rarely bilateral)
• Mucinous adenocarcinoma: mimic melanoma
Mafee, Radiol Clin North Am 1998; 36:1083-1099
Orbital Trauma [Figures 5-6-17 and 5-6-18]

• CT: imaging modality of choice
• Fractures
➢ Isolated: orbital rim (Waters view)
➢ Blowout: inferior wall; medial wall: 1/2
➢ Nasoethmoidal (NOE) complex
❖ canthal injury common
➢ Zygomatic complex (ZC)
➢ Lefort types: I, II, III
➢ Orbital apex-optic canal
• Hemorrhage
➢ Retrobulbar: most common
➢ Extraconal Bilateral uveal metastases
➢ Subperiosteal
➢ Sub-Tenon capsule
➢ Subdural (optic nerve sheath)
• Optic nerve injury
• Eyeball injury: phthisis bulbi
• Foreign Body
Figure 5-6-17
Figure 5-6-18
Blow-out fracture
Orbital fracture extending towards

optic canal
Neuroradiology 1091
Conal Lesions
• Graves
• Pseudotumor
• Others
➢ Lymphoproliferative disease
➢ Metastasis: 7%, breast carcinoma, nodular
➢ Arteriovenous fistula or vascular congestion
➢ Acromegaly
➢ Amyloid
➢ Cysticercosis / Trichinosis
Thyroid Orbitopathy (Graves Disease)

• Autoimmune disorder: orbital soft tissues, thyroid, extremities
➢ Superior cervical lymph channel: drains both thyroid and orbit
• Incidence: 0.5% (U. S.)
• Most common orbital disorder
• Most common cause of exophthalmos in adults Figure 5-6-19
➢ 15%–28% of unilateral exophthalmos
➢ 80% of bilateral exophthalmos
Rubin and Sadun in Yanoff and Duker, Ophthalmology, Mosby, 1999;
Mafee in Som and Curtin, Head and Neck Imaging, 4th ed, Mosby,
2003
Graves Disease
• Most (up to 80%) patients are or will be hyperthyroid
➢ Euthyroid (10%)
• Family history: 30%
• Range: 15–86 years old (peak: 30–50)
• Females much more common (4:1)
• Males, patients > 50y/o: more severe disease
Kendler et al, Arch Ophthalmol 1993; 111:197-201
Graves Disease
• Acute phase
➢ Inflammatory reaction: congestion, hypertrophy, fibrosis of Graves Disease
orbital fat / muscles
➢ Mucopolysaccharides accumulate in EOMs Figure 5-6-20
• Chronic phase: exophthalmos (34%–93%)
➢ Fibrosis
➢ Restrictive myopathy
➢ Diplopia
Rubin and Sadun in Yanoff and Duker, Ophthalmology, Mosby, 1999
Graves Disease: Imaging [Figures 5-6-19 and 5-6-20]

• Muscle Enlargement
➢ Inferior rectus
➢ Medial rectus
➢ Superior muscle complex
➢ Lateral rectus
➢ ? Related to innervation and fiber size
• Tendon spared
• “Dirty” retrobulbar fat: inflammation
• Apex: optic nerve compression
Mafee in Som and Curtin, Head and Neck Imaging, 4th ed, Mosby,
2003
Graves Disease with sparing of the

tendinous insertions

1094 Neuroradiology
Pseudotumor
• Nongranulomatous inflammation
• No known cause
• Second most common (~5%) orbital disease
(after Graves disease)
• Children: 6%–16% of all cases, more frequently bilateral
Blodi and Gass, Br J Ophthalmol 1968; 52:79-93; Flanders et al, J Comput Assist
Tomogr 1989; 13:40-47; Weber et al, Radiol Clin North Am 1999; 37:151-168 Figure 5-6-21
Pseudotumor
• Acute form
➢ Abrupt onset of pain usually
➢ Lid swelling, redness, ptosis, proptosis
• Chronic form
➢ Fixation signs: diplopia, proptosis
• Sites
➢ Lacrimal gland (lacrimal adenitis)
➢ Extraocular muscles (myositic form)
➢ Cavernous sinus (Tolosa-Hunt)
Tolosa, J Neurol Neurosurg Psychiatry 1954; 17:300-302; Hunt,
Neurology 1961; 11:56-62
Pseudotumor: EOM
• Tendons involved (unlike Graves disease)
• Ragged “fluffy” muscle border
• Inward bowing of muscle contour at globe insertion
• “Dirty” retrobulbar fat
• May extend intracranially (apical orbital inflammation) or onto
optic nerve (ON) sheath (perineuritis)
• Bone destruction rare
Pseudotumor with tendinous
Trokel and Hilal, Am J Ophthalmol 1979; 87:503-512; involvement and rapid response to
Flanders et al, J Comput Assist Tomogr 1989; 13:40-47 steroid therapy on follow-up
Pseudotumor: Imaging [Figures 5-6-21 and 5-6-22]

• CT Figure 5-6-22
➢ Nonspecific
➢ Moderate enhancement
• MR
➢ Hypointense on T1WI and T2WI
Pseudotumor with characteristic T1

and T2 hypointensity
Neuroradiology 1093
References
1. Abramson DH, Ellsworth RM, Kitchin FD, Tung G. Second nonocular tumors in retinoblastoma survivors. Are
they radiation-induced? Ophthalmology 1984; 91:1351-1355.
2. Abramson DH, Ellsworth RM, Tretter P, Javitt J, Kitchin FD. Treatment of bilateral groups I through III
retinoblastoma with bilateral radiation. Arch Ophthalmol 1981; 99:1761-1762.
3. Blodi FC, Gas JD. Inflammatory pseudotumour of the orbit. Br J Ophthalmol 1968; 52:79-93.
4. Char DH, Hedges TR, 3rd, Norman D. Retinoblastoma. CT diagnosis. Ophthalmology 1984; 91:1347-1350.
5. Coats G, Lond R. Forms of retinal diseases with massive exudation. Ophthalmol Hosp Rep 1908; 17:440-525.
6. Duffin RM, Straatsma BR, Foos RY, Kerman BM. Small malignant melanoma of the choroid with extraocular
extension. Arch Ophthalmol 1981; 99:1827-1830.
7. Edward DP, Mafee MF, Garcia-Valenzuela E, Weiss RA. Coats' disease and persistent hyperplastic primary
vitreous. Role of MR imaging and CT. Radiol Clin North Am 1998; 36:1119-1131, x.
8. Eller AW, Jabbour NM, Hirose T, Schepens CL. Retinopathy of prematurity. The association of a persistent hyaloid
artery. Ophthalmology 1987; 94:444-448.
9. Ellsworth RM. The practical management of retinoblastoma. Trans Am Ophthalmol Soc 1969; 67:462-534.
10. Enochs WS, Petherick P, Bogdanova A, Mohr U, Weissleder R. Paramagnetic metal scavenging by melanin: MR
imaging. Radiology 1997; 204:417-423.
11. Flanders AE, Mafee MF, Rao VM, Choi KH. CT characteristics of orbital pseudotumors and other orbital
inflammatory processes. J Comput Assist Tomogr 1989; 13:40-47.
12. Hunt WE, Meagher JN, Lefever HE, Zeman W. Painful opthalmoplegia. Its relation to indolent inflammation of the
carvernous sinus. Neurology 1961; 11:56-62.
13. Jakobiec FA, Tso MO, Zimmerman LE, Danis P. Retinoblastoma and intracranial malignancy. Cancer 1977;
39:2048-2058.
14. Kaufman LM, Mafee MF, Song CD. Retinoblastoma and simulating lesions. Role of CT, MR imaging and use of
Gd-DTPA contrast enhancement. Radiol Clin North Am 1998; 36:1101-1117.
15. Kendler DL, Lippa J, Rootman J. The initial clinical characteristics of Graves' orbitopathy vary with age and sex.
Arch Ophthalmol 1993; 111:197-201.
16. Kodilinye HC. Retinoblastoma in Nigeria: problems of treatment. Am J Ophthalmol 1967; 63:469-481.
17. Kyritsis AP, Tsokos M, Triche TJ, Chader GJ. Retinoblastoma--origin from a primitive neuroectodermal cell?
Nature 1984; 307:471-473.
18. Mafee MF, Goldberg MF, Cohen SB, et al. Magnetic resonance imaging versus computed tomography of
leukocoric eyes and use of in vitro proton magnetic resonance spectroscopy of retinoblastoma. Ophthalmology
1989; 96:965-975; discussion 975-966.
19. Mafee MF, Goldberg MF, Greenwald MJ, Schulman J, Malmed A, Flanders AE. Retinoblastoma and simulating
lesions: role of CT and MR imaging. Radiol Clin North Am 1987; 25:667-682.
20. Mafee MF, Goldberg MF. Persistent hyperplastic primary vitreous (PHPV): role of computed tomography and
magnetic resonance. Radiol Clin North Am 1987; 25:683-692.
21. Mafee MF, Peyman GA. Retinal and choroidal detachments: role of magnetic resonance imaging and computed
tomography. Radiol Clin North Am 1987; 25:487-507.
22. Mafee MF. The eye. In: Som PM, Curtin HD, eds. Head and neck imaging. 4th ed. St. Louis: Mosby–Elsevier
Science; 2003
23. Mafee MF. Uveal melanoma, choroidal hemangioma, and simulating lesions. Role of MR imaging. Radiol Clin
North Am 1998; 36:1083-1099
24. Margo CE, Katz NN, Wertz FD, Dorwart RH. Sclerosing endophthalmitis in children: computed tomography with
histopathologic correlation. J Pediatr Ophthalmol Strabismus 1983;20:180-184
25. Pendergrass TW, Davis S. Incidence of retinoblastoma in the United States. Arch Ophthalmol 1980; 98:1204-1210.
26. Reese AB. Telangiectasis of the retina and Coats' disease. Am J Ophthalmol 1956; 42:1-8.
27. Rubin RM, Sadun AA. Ocular myopathies. In: Yanoff M, Duker JS, eds. Ophthalmology. St. Louis: Mosby; 1999.
28. Tolosa E. Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm. J
Neurol Neurosurg Psychiatry 1954; 17:300-302.
29. Trokel SL, Hilal SK. Recognition and differential diagnosis of enlarged extraocular muscles in computed
tomography. Am J Ophthalmol 1979; 87:503-512.
30. Weber AL, Romo LV, Sabates NR. Pseudotumor of the orbit. Clinical, pathologic, and radiologic evaluation.
Radiol Clin North Am 1999; 37:151-168, xi.
31. Wilder HC. Nematode endophthalmitis. Trans Am Acad Ophthalmol Otolaryngol 1950:99-109.
32. Yanoff K, Fine BS, Ocular Pathology . Hagerstown: Harper & Row, 1975.

1096 Neuroradiology
Imaging of the Orbit:
Intraconal and Extraconal Lesions
Figure 5-7-1
Intraconal Lesions
• Optic nerve glioma
• Optic nerve sheath meningioma
• Cavernous hemangioma
• Schwannoma
• Lymphoma
• Fibrous histiocytoma
• Varix, arteriovenous malformation
Optic Nerve Glioma

• 3% of all orbital tumors; 4% of gliomas
• Peak age: 2–8 years (range: birth to 60 years)
• 50% associated with neurofibromatosis type 1 (NF-1) and
frequently bilateral
➢ 10%–15% of all NF-1 cases
• Optic atrophy on ophthalmoscopy
• Arise from glial cells of optic nerve
• Slow growth usually; may grow in spurts
Azar-Kia et al, Radiol Clini North Am 1987; 25:561-581
Optic nerve glioma with characteristic
Optic Nerve Glioma [Figure 5-7-1]
"kinking" of the nerve
• Juvenile: pilocytic astrocytoma
• Adult: glioblastoma multiforme (non-NF-1 cases)
• Fusiform enlargement: kinking, buckling
• CT: iso- to hypodense
➢ Calcification rare
• T1WI: hypointense, T2WI: hyperintense
• Heterogeneous enhancement
Azar-Kia et al, Radiol Clin North Am 1987; 25:561-581; Haik et al, Ophthalmology Figure 5-7-2
1987; 94:709-717
Optic Nerve Sheath Meningioma

• 5% of all orbital tumors
• Less than 1% of all meningiomas
• “Extradural” meningiomas: associated with “blistering” of adjacent
bone
• Slowly progressive loss of vision, proptosis
➢ Optociliary venous shunts, disk pallor, visual loss: highly
suggestive
Sibony et al, Ophthalmology 1984; 11:1313-1326
Optic Nerve Sheath Meningioma [Figure 5-7-2]

• Well-defined tubular thickening of ON
• CT: Calcification common
• T1WI: Isointense to ON
• T2WI: Iso- to hypoattenuated
• “Tram-track” enhancement
➢ Fat suppression essential
• May be eccentric, extend intracranially Optic nerve sheath meningioma with
Daniels et al, AJNR 1982: 3:181-183; Azar-Kia et al, Radiol Clin North tram-track enhancement (upper
Am 1987; 25:561-581 image) and calcification (lower image)
in 2 different patients
Neuroradiology 1097 Imaging of the Orbit: Intraconal and Extraconal Lesions

Cavernous Hemangioma [Figure 5-7-3]
• Most common vascular orbital tumor in adults
• Peak age: 25-40 y/o (range 25-70) Figure 5-7-3
• Well-circumscribed mass
(pseudocapsule)
➢ Intraconal (83%)
• Benign non-infiltrative hamartoma
➢ Large dilated sinusoid-like spaces
➢ Slowly progressive enlargement
➢ Prominent arterial supply usually
absent
• CT
➢ Hyperattenuated
➢ Phleboliths
➢ Bone remodeling
• MR
➢ T1WI: mixed
➢ T2WI: iso-to-hyperintense
• Hemorrhage occasionally
Bilaniuk, Radiol Clin North Am 1999;
37:169-183; Mafee et al, Radiol Clin North
Cavernous hemangioma with pressure erosion of orbital roof
Am 1987; 25:529-559
Schwannoma
• 1% of all orbital tumors: usually intraconal
➢ Arise from cranial nerves, not optic nerve
➢ Isolated or neurofibromatosis association
• Benign with slow growth
➢ Well-encapsulated
• Painless proptosis
• Compresses or engulfs optic nerve
Carroll et al, Radiol Clin North Am 1999; 37:195-202
Schwannoma [Figure 5-7-4]

• Fusiform to oval-shaped mass
• CT: Isoattenuated to extraocular muscles
➢ More hyperdense areas = Antoni A cells
• T1WI: Iso- to hypodense
• T2WI: Hyperintense Figure 5-7-4
• Marked enhancement
Orbital Lymphoma
• Lymphoid tumors: 10%-15% of orbital masses
➢ Lymphoma, pseudolymphoma, lymphoid hyperplasia
• 10% of all lymphomas as primary site
➢ 75% have or will have systemic lymphoma
➢ Lacrimal gland: most common site
➢ EOMs rarely involved
• Non-Hodgkin’s (B-cell): majority
• Proptosis, ptosis, diplopia
• Rubbery firm masses
Valvassori et al, Radiol Clin North Am 1999; 37:135-150; Flanders et
al, Radiol Clin North Am 1997; 25:601-612;
Schwannoma
Imaging of the Orbit: Intraconal and Extraconal Lesions 1096

1098 Neuroradiology
Orbital Lymphoma [Figure 5-7-5] Figure 5-7-5
• Molds itself along margins of normal structures
➢ Bone erosion: late finding
• Usually well-defined, round to oval
• CT: homogeneous, mildly hyperattenuated
• T1WI: hypointense
• T2WI: iso-to-hypointense
• Mild to marked enhancement
Valvassori et al, Radiol Clin North Am 1999; 37:135-150; Flanders et
al, Radiol Clin North Am 1997; 25:601-612;
Fibrous Histiocytoma [Figure 5-7-6]

• Most common primary orbital mesenchymal tumor in adults
• 1% of all primary orbital tumors
• Mean age: 42 y/o
• Usually benign (66%)
➢ Malignant: bone erosion, hemorrhage, post-radiation therapy
for retinoblastoma
• Well-defined intra-or extraconal mass
• Moderate to marked enhancement
Orbital lymphoma with characteristic
Font and Hidayat, Hum Pathol 1982; 13:199; Mafee in Valvassori,
molding of the tumor against the
Mafee, and Carter, Imaging of the Head and Neck, Thieme, 1995
orbital globe
Orbital Varix [Figure 5-7-7]
• Most common cause of spontaneous orbital hemorrhage Figure 5-7-6
• Focal venous dilatation
• Valsalva: stress proptosis
➢ Lobulated mass
• Phleboliths
• Spontaneous thrombosis common
Bilaniuk, Radiol Clin North Am 1999; 37:169-183
Figure 5-7-7
Malignant fibrous histiocytoma
Orbital varix with enlargement upon

Valsalva maneuver on post-contrast
image (bottom)
Neuroradiology 1097
1099 Imaging of the Orbit: Intraconal and Extraconal Lesions
Arteriovenous Fistula [Figure 5-7-8] Figure 5-7-8
• Usually post-trauma
➢ Spontaneous less common (Ehlers-Danlos, osteogenesis
imperfecta, pseudoxanthoma elasticum)
• Orbital bruit, proptosis, chemosis
• CT/MR: dilated superior ophthalmic vein
• Angiography diagnostic
• Endovascular occlusion: treatment of choice
Tan et al, Radiol Clin North Am 1987; 25:849-861
Carotid-cavernous
Extraconal Lesions fistula with enlarged
• Lymphangioma* superior ophthalmic
• Metastasis* vein. Lateral view from
• Rhabdomyosarcoma* cerebral angiogram
• Dermoid/epidermoid shows abnormal flow
• Paranasal sinus disease through cavernous
➢ Infection sinus, petrosal
➢ Neoplasm sinuses, and superior
*commonly “intercompartmental” ophthalmic vein
Lymphangioma [Figure 5-7-9]

• Children and young adults
• Exophthalmos with viral infection
• Lymphoid follicles, dilated spaces
➢ Infiltrative; do not respect fascial planes
➢ Hemorrhage common
• Extraconal space primarily
• CT/MR: Heterogeneous appearance
➢ Hemorrhage or cystic fluid
• Variable enhancement (venous channels)
Bilaniuk, Radiol Clin North Am 1999; 37:169-183; Mafee
et al, Radiol Clin North Am 1987; 25:529-559
Figure 5-7-9
Figure 5-7-10
Lymphangioma with hemorrhage in 2 different patients
Metastatic Lesions [Figure 5-7-10]

• 10% of orbital tumors
➢ 1/3- bony orbit, 1/3- globe, 1/3- scattered
• Increasing incidence (longer survival)
• Primary site
➢ Breast: 42%
➢ Lung: 11%
➢ Unknown primary: 11%
➢ Prostate: 8%
➢ Melanoma: 5%
Orbital metastasis from unknown
➢ Children: neuroblastoma, leukemia, Ewing’s
primary neoplasm

1100 Neuroradiology
Metastatic Lesions Figure 5-7-11
• 9 months average survival (lung carcinoma,
melanoma worst)
• Diplopia, proptosis, pain, vision loss
• Enophthalmos: breast carcinoma
• Isolated lateral rectus enlargement metastasis or
pseudotumor
Rhabdomyosarcoma [Figure 5-7-11]

• Most common primary orbital malignancy tumor in
children
➢ Most: 2-5y/o; 90% younger than 16 y/o
➢ Rapidly progressive but usually painless
• Arise from undifferentiated mesenchyme in orbital Rhabdomyosarcoma with characteristic bone
fat (not from extraocular muscles) destruction
➢ Children: embryonal (70%) and alveolar types
➢ Adults: pleomorphic type
• 90% 5-year survival with complete resection
➢ 35% if significant residual disease
Mafee et al, Radiol Clin North Am 1998; 36:1215-1227;
Rhabdomyosarcoma
• Superior orbit predilection Figure 5-7-12
• Homogeneous mass
• CT: isoattenuated to muscle
➢ Bone destruction common in larger lesions
➢ Necrosis, calcification, hemorrhage uncommon
• T1WI: hypointense
• Moderate to marked enhancement
Mafee et al, Radiol Clin North Am 1998; 36:1215-1227
Dermoid / Epidermoid [Figure 5-7-12]

• Most common congenital orbital lesion
➢ Many manifest in 2nd-3rd decades
• Superolateral: most comon location
• Arise at sutures or diplöe
• Well-defined mass with fat or fluid signal in upper corners of orbit
• Remodel bone without destruction
Kaufman et al, Radiol Clin North Am 1998; 36:1149-1163
Orbital Cellulitis [Figures 5-7-13 and 5-7-14] Dermoid

• Classification
➢ Pre-septal cellulitis: eyelid
➢ Post-septal cellulitis
➢ Subperiosteal phlegmon and abscess
➢ Cavernous sinus thrombosis
• Usually paranasal (ethmoid) sinusitis
• Usually does not extend into intraconal space
Chandler et al, Laryngoscope 1970; 80:1414; Eustis et al, Radiol Clin North Am
1998; 36:1165-1183
Neuroradiology 1099
Peri-orbital cellulitis Orbital cellulitis. Note involvement

along lamina papyracea
Fungal sinusitis [Figure 5-7-15]
➢ Rhino-orbital mucormycosis
➢ Aspergillosis
• Reversal of typical findings in sinus disease
➢ Increasing protein, decreasing water content
➢ CT: hyperattenuated
➢ T2WI: hypointense (can mimic air)
Chandler et al, Laryngoscope 1970; 80:1414; Eustis et al, Radiol Clin North Am
1998; 36:1165-1183
Lacrimal Gland Lesions

• 50% inflammatory/lymphoproliferative
➢ Sarcoid
➢ Sjogren’s
➢ Lymphoma: frequent anterior/posterior extension
➢ Pseudotumor: 15% of all orbital pseudotumor
• 50%: epithelial tumors
➢ 50%: benign (pleomorphic adenoma, benign mixed cell tumor)
➢ 50%: malignant (adenoid cystic, malignant mixed, mucoepidermoid,
adeno, squamous cell, anaplastic) Figure 5-7-15
• Imaging: pre-op planning
Zimmerman et al, Int Ophthalmol Clin 1962;
2:337-367; Mafee et al, Radiol Clin North
Am 1987; 25:767-779
Fungal sinusitis

1102 Neuroradiology
Lacrimal Gland Lesions [Figure 5-7-16 and 5-7-17] Figure 5-7-16
• Inflammatory lesions
➢ Oblong mass
➢ Molded enlargment of lacrimal gland
• Pleomorphic adenoma
➢ Long duration
➢ Rounded mass
➢ Bone remodeling
➢ Bone destruction: malignant epithelial tumors
Jakobiec et al, Am J Ophthalmol Clin 1962; 2:337-367
Lacrimal Sac Lesions [Figure 5-7-18]

• Malignant: 57%
➢ Epithelial: 75%
❖ Squamous cell
❖ Transitional cell
❖ Mucoepidermoid
➢ Mesenchymal: fibrous histiocytoma
➢ Lymphoid: lymphoma
➢ Neural
➢ Metastasis Lacrimal lymphoma
• Benign: 43%
➢ Diverticulum
Figure 5-7-17
➢ Pneumatocele
➢ Mucocele
➢ Papilloma
➢ Polyp
➢ Fibroma
➢ Dermoid
Stefanyszyn et al, Ophthal Plast Reconstr Surg 1994; 10:169-184;
Pe’er et al, Ophthalmology 1996; 103:1601-1605
Figure 5-7-18
Lacrimal pleomorphic adenoma
Lacrimal sac sarcoma with bone destruction
Neuroradiology 1101
Summary
• Retinoblastoma: most common intraocular malignancy of childhood
• Uveal melanoma: most common malignancy of the globe in adults
• Uveal metastasis: frequently bilateral
• Most common diseases of the orbit
➢ 1. Graves: no tendon involvement
➢ 2. Lymphoma
➢ 3. Pseudotumor: involves tendon
• Intraconal lesions
➢ Optic nerve tumors
❖ Glioma
❖ Nerve sheath meningioma
➢ Cavernous hemangioma
➢ Nerve sheath tumors
➢ Lymphoma
➢ Fibrous histiocytoma
➢ Varix
➢ Carotid-cavernous fistula
• Extraconal lesions
➢ Lymphangioma
➢ Metastases: 10% of orbit masses
➢ Dermoid
➢ Sinus disease
• Lacrimal gland lesions
➢ “The 50% gland”
➢ 50% inflammatory/lymphoproliferative
➢ 50% neoplasms
❖ 50% benign, 50% malignant
• Lacrimal sac lesions: most are malignant
References
1. Azar-Kia B, Naheedy MH, Elias DA, Mafee MF, Fine M. Optic nerve tumors: role of magnetic resonance imaging
and computed tomography. Radiol Clin North Am 1987; 25:561-581.
2. Bilaniuk LT. Orbital vascular lesions. Role of imaging. Radiol Clin North Am 1999; 37:169-183, xi.
3. Carroll GS, Haik BG, Fleming JC, Weiss RA, Mafee MF. Peripheral nerve tumors of the orbit. Radiol Clin North
Am 1999; 37:195-202, xi-xii.
4. Chandler JR, Langenbrunner DJ, Stevens ER. The pathogenesis of orbital complications in acute sinusitis.
Laryngoscope 1970; 80:1414-1428.
5. Daniels DL, Williams AL, Syvertsen A, Gager WE, Harris GJ. CT recognition of optic nerve sheath meningioma:
abnormal sheath visualization. AJNR Am J Neuroradiol 1982; 3:181-183.
6. Eustis HS, Mafee MF, Walton C, Mondonca J. MR imaging and CT of orbital infections and complications in acute
rhinosinusitis. Radiol Clin North Am 1998; 36:1165-1183, xi.
7. Flanders AE, Espinosa GA, Markiewicz DA, Howell DD. Orbital lymphoma. Role of CT and MRI. Radiol Clin
North Am 1987; 25:601-613.
8. Font RL, Hidayat AA. Fibrous histiocytoma of the orbit. A clinicopathologic study of 150 cases. Hum Pathol 1982;
13:199-209.
9. Haik BG, Saint Louis L, Bierly J, et al. Magnetic resonance imaging in the evaluation of optic nerve gliomas.
Ophthalmology 1987; 94:709-717.
10. Jakobiec FA, Yeo JH, Trokel SL, et al. Combined clinical and computed tomographic diagnosis of primary lacrimal
fossa lesions. Am J Ophthalmol 1982; 94:785-807.
11. Kaufman LM, Villablanca JP, Mafee MF. Diagnostic imaging of cystic lesions in the child's orbit. Radiol Clin
North Am 1998; 36:1149-1163, xi.
12. Mafee MF, Haik BG. Lacrimal gland and fossa lesions: role of computed tomography. Radiol Clin North Am 1987;
25:767-779.
13. Mafee MF, Pai E, Philip B. Rhabdomyosarcoma of the orbit. Evaluation with MR imaging and CT. Radiol Clin
North Am 1998; 36:1215-1227, xii.
14. Mafee MF, Putterman A, Valvassori GE, Campos M, Capek V. Orbital space-occupying lesions: role of computed
tomography and magnetic resonance imaging. An analysis of 145 cases. Radiol Clin North Am 1987; 25:529-559.

1104 Neuroradiology
15. Mafee MF. Imaging of the orbit. In: Valvassori GE, Mafee MF, Carter BL, eds. Imaging of the head and neck. New
York: Thieme, 1995; 302-328
16. Pe'er J, Hidayat AA, Ilsar M, Landau L, Stefanyszyn MA. Glandular tumors of the lacrimal sac. Their
histopathologic patterns and possible origins. Ophthalmology 1996; 103:1601-1605.
17. Sibony PA, et al: Optic Nerve Sheath Meningiomas. Ophthalmology 1984, 91(11): 1313-1326.
18. Stefanyszyn MA, Hidayat AA, Pe'er JJ, Flanagan JC. Lacrimal sac tumors. Ophthal Plast Reconstr Surg 1994;
10:169-184.
19. Tan WS, Wilbur AC, Mafee MF. The role of the neuroradiologist in vascular disorders involving the orbit. Radiol
Clin North Am 1987; 25:849-861.
20. Valvassori GE, Sabnis SS, Mafee RF, Brown MS, Putterman A. Imaging of orbital lymphoproliferative disorders.
Radiol Clin North Am 1999; 37:135-150, x-xi.
21. Zimmerman LE, Sanders TE, Ackerman LV. Epithelial tumors of the lacrimal gland: prognostic and therapeutic
significance of histologic types. Int Ophthalmol Clin 1962; 2:337-367.
Neuroradiology 1103
Patterns of Location: Infratentorial and
Supratentorial
PATTERN ANALYSIS
• Basic Approach
➢ Where is the lesion ?
❖ Intraaxial
❖ Extraaxial
❖ Intraventricular
➢ Where is the lesion ?
❖ Supratentorial
❖ Infratentorial
➢ How old is the patient ?
❖ Child
❖ Adult
➢ What about Sex ?
INTRA-AXIAL
• Cortex
• Gray-white Junction
• Deep White Matter
• Deep Gray Matter
• Glioma
• Medulloblastoma
• Hemangioblastoma
• Metastases
• Infarct/hematoma
• AVM/congenital
• Abscess/inflammation
EXTRA-AXIAL LESIONS
• Subarachnoid
• Subdural
• Epidural
• Calvarium (Skull Base)
• Subgaleal
• Scalp (Soft-tissues)
• Meningioma
• Pituitary adenoma
• Craniopharyngioma
• Schwannoma
• Chordoma
• Dermoid/epidermoid, cyst, lipoma
• Hematoma, metastasis, infection
BASIC APPROACH
CLASSIC LOCATIONS
• Foramen magnum
• Cerebellopontine angle (CPA)
• Fourth ventricle/Cerebellum
• Sella/parasellar/suprasellar
• Basal ganglia/Third ventricle
• Lateral ventricle/Pineal region
• Deep hemispheric/periventricular
• Cortical and subcortical
• Convexity Extraaxial
Patterns of Location: Infratentorial and Supratentorial 1104
1106 Neuroradiology
Cranial Nerves Figure 5-8-1
• Olfactory (I)
• Optic (II)
• Oculomotor (III)
• Trochlear (IV)
• Trigeminal (V)
• Abducens (V)
When looking into the IAC
• Facial (VII)
(internal auditory canal) notice
• Vestibulocochlear (VIII)
that
7 is UP and Coke (cochlear) is
Internal Auditory Canal
DOWN
• S – Schwannoma (8th >> > 5th)
• A – aneurysm, arachnoid cyst
• M – meningioma, mets
• E – epidermoid, ependymoma, CPP
CPA MASSES Differential

• S – Schwannoma (8th >> > 5th)
• A – aneurysm, arachnoid cyst Figure 5-8-2
• M – meningioma, mets
• E – epidermoid, ependymoma, CPP
CPA MASSES Demographics

• 7/9 (Schwannoma, 8th > > 5th)
• 1/9 Meningioma (tentorial/petrous)
• 1/9 “Other”:
➢ Epidermoid Cyst (1/18)
➢ Mets, aneurysm, etc.
➢ Glioma (ependymoma, CPP)
➢ Arachnoid cyst
➢ Cystadenoma of endolymph
➢ Glomus tumor
Vestibular Schwannoma begins as an
Intracanalicular Schwannoma intracanalicular mass; then it grows out of the
[Figure 5-8-3] canal into the cerebellopontine angle cistern
Vestibular Schwannoma
Figure 5-8-3
[left] T2W image shows CSF, normal nerve, and round mass.
[right] T1WGd image shows enhancement of mass. The
normal 7th and 8th nerves do NOT enhance in this location
Neuroradiology 1105
1107 Patterns of Location: Infratentorial and Supratentorial
Young Schwannoma – Old Schwannoma [Figure 5-8-4] Figure 5-8-4
• Benign Cystic Degeneration
Vestibular Schwannoma
• IAC origin
➢ IAC involved
➢ IAC Enlarged (70%)
• Spherical Mass
➢ encapsulated
• Heterogeneous if large
➢ > 20 mm
• Enhance “always”
Trigeminal Schwannoma [Figure 5-8-5]

The larger – and older – Schwannoma is heterogeneous due to
Figure 5-8-5 benign cystic degeneration
Figure 5-8-6
Trigeminal Schwannoma may present as a “dumbbell” mass,

bilobed, with one lobe in the cavernous sinus and one in the
poster fossa – lateral pontine cistern
Bilateral Vestibular Schwannoma

This mass is hemispheric – but
does not extend into the canal.
Meningioma [Courtesy of Bob Peyster, MD]
Hyperostosis
Meningioma
Figure 5-8-7
Tentorial Meningioma

• Tentorium or Dura
• IAC Normal
• Hemispherical
• Enhance Homogeneous
• Hyperostosis
➢ 15%-40%
• Dural Tail
➢ 70%-90%
[left] T1W image – no enhancement, undulating (wavy) margin.

Note the wispy internal structure.
[right] T2W image – isointense to CSF

1108 Neuroradiology
Epidermoid vs. Arachnoid Cyst
• Epidermoid Inclusion Cyst [Figure 5-8-8] Figure 5-8-8
➢ CPA most common
➢ Extraaxial CPA Lesion
➢ IAC Normal
➢ Undulating Margin
➢ CSF - ‘like’
❖ Not identical
➢ NO Enhancement
➢ Wispy internal structures
• Arachnoid Cyst
➢ Middle fossa common
➢ Extraaxial CPA Lesion
➢ IAC Normal
➢ Rounded Mass
➢ Identical to H2O on CT and all MR
sequences
❖ T1, PD, T2, FLAIR, DWI, ADC
➢ NO Enhancement
➢ NO ‘structure’
CLASSIC LOCATIONS This epidermoid inclusion cyst

• Foramen magnum is only isointense to CSF on the T2W image
• Cerebellopontine angle
• Fourth ventricle/Cerebellum Figure 5-8-9
Central Posterior Fossa Lesion

• Could be Intraaxial
• Could be Intraventricular
• Could be extending from vermis into ventricle
• Could be extending from ventricle into vermis
Fourth Ventricle - Schematic [Figure 5-8-9]
Central Masses [Figure 5-8-10] Schematic of central posterior fossa mass: Did it
begin in the 4th ventricle, in the medullary velum,
CHILD - CEREBELLAR/IVth or in the cerebellum?
• Medulloblastoma (PNET)
• Astrocytoma (usu. Pilocytic) Figure 5-8-10
• Ependymoma
• Post fossa cysts
ADULT - CEREBELLAR/IVth
• Metastasis
• Hemorrhage, infarct
• Glioma
➢ Ependymoma
➢ Astrocytoma
• Abscess
Schematic diagram of medulloblastoma and

ependymoma. Copyright 2005
Neuroradiology 1107
Medulloblastoma [Figures 5-8-11 and 5-8-12] Figure 5-8-11
• ‘Homogeneous’
➢ finely irregular
• Cyst and Hemorrhage are uncommon
<10%
• Hyperdense on NCT
➢ up to 75%
➢ densely cellular
➢ sm. Round blue-cells
• Center is behind 4th vent
• Rounder not angular
Pilocytic Astrocytoma
[Figures 5-8-13 to 5-8-16]
• Cyst and Mural Nodule
➢ balanced morphology
• Wall may not enhance
• Cyst fluid with protein Medulloblastoma, hyperdense on plain CT
• Nodule low density on CT
➢ may calcify up to 25%
• No increase in vascularity Figure 5-8-12
• WHO Grade 1
• Peak at ~10 yrs
Figure 5-8-13
Pilocytic astrocytoma: Classic cyst-with-nodule

morphology
Medulloblastoma
Figure 5-8-14
Figure 5-8-15
Pilocytic astrocytoma: Classic cyst-with-nodule Pilocytic astrocytoma: Classic cyst-with-nodule

morphology morphology

1110 Neuroradiology
Good Rules for Practice [Figure 5-8-17] Figure 5-8-16
• Cerebellum GBM is uncommon
• Pediatric GBM is uncommon
• If I am wrong …
• What’s the worst that it could be?
• What’s the best that it could be?
• The Probability of tandem events occurring is the product of
multiplying the chance of the individual events. E.G. 1/1000 x
1/1000 = 1/million
• Uncommon X Uncommon = RARE
Hemangioblastoma [Figures 5-8-18 and 5-8-19]

• Cyst and Mural Nodule
➢ some solid
➢ some nearly pure cyst
• Wall may not enhance
• Cyst fluid has protein
• Nodule high density
• No Ca++
• Increased vascularity
➢ Flow void Pilocytic astrocytoma: Classic cyst-
➢ Blood products with-nodule morphology.
• WHO Grade 1 Copyright 2005
• Peak at ~ 35 yrs
• Multiple in VHL Figure 5-8-17
Figure 5-8-18
What is this? – Come to the lecture

and find out!
Figure 5-8-19
Hemangioblastoma may be a cyst and nodule in about 1/3 of

cases. Solid forms, and almost completely “cystic” types occur
Hemangioblastoma may be a cyst

and nodule in about 1/3 of cases.
Solid forms, and almost completely
“cystic” types occur.
Copyright 2005
Neuroradiology 1109
Expansile Mass in Brainstem Figure 5-8-20
[Figure 5-8-20]
Intraluminal Mass of IVth

[Figure 5-8-21]
Figure 5-8-21
Expansile lesion of the pons and

medulla, encroaching on the 4th
ventricle, does not enhance
Figure 5-8-22
Ependymoma, arising from the 4th

ventricle floor, and remains within the
lumen of the ventricle
Ependymoma - Schematic [Figure 5-8-22]
Ependymoma [Figures 5-8-23 and 5-8-24]

• Intraventricular
• “Soft” plastic lesion
• Angular extensions
➢ Luschka Schematic of ependymoma
➢ Magendie Copyright 2005
• Heterogeneous
➢ Cystic areas
➢ Chunks of Ca++
• Arise from floor of 4th
Figure 5-8-24
Figure 5-8-23
Ependymoma.
Copyright 2005
Ependymoma

1112 Neuroradiology
Posterior Fossa Masses [Figure 5-8-25] Figure 5-8-25
Lhermitte-Duclos [Figure 5-8-26]
CLASSIC LOCATIONS
• Foramen magnum
SELLA/PARASELLAR REGION Schematic Localization of posterior fossa masses.

• Differential: Copyright 2005
➢ Pituitary adenoma
➢ Craniopharyngioma Figure 5-8-26
➢ Aneurysm (ICA , etc.)
➢ Meningioma
➢ Optic/hypothalamic glioma
➢ Chordoma
➢ Granuloma, e.g., hamartoma,
cyst(arachnoid, dermoid/epi)
➢ Germ Cell (Germinoma)
Pituitary Adenoma [Figure 5-8-27]

• Adult Patient
• Microadenoma
➢ < 10 mm
➢ Entirely within gland
➢ Endocrine Sx
❖ Prolactinoma
❖ Acromegaly
❖ Gigantism Lhermitte-Duclos. T1 and T2 weighted images show a striated
❖ Cushing Disease or “courduroy” appearance, classic for dysplastic gangliocyoma
• Macroadenoma
➢ > 10 mm Figure 5-8-27
➢ balloon sella
➢ Visual Sx
❖ if >6 mm above sella
❖ bitemporal hemianopsia
Elevated Prolactin
• Microadenoma
➢ < 10mm diameter
➢ Entirely intrasellar
• Macroadenoma
➢ > 10 mm
• Stalk Effect
➢ Blocks Prolactin Inhibitory Factor
➢ 40-150 ng PRL vs. 28 for nl.
• Hypothyroidism
➢ “Cross Reaction” from TSH
• Exogenous
• Pharmacologic
Pituitary macroadenoma with hyperintensity from

old hemorrhage
Neuroradiology 1111
Macroadenoma [Figure 5-8-28] Figure 5-8-28
Sella and Suprasellar [Figure 5-8-29]
Craniopharyngioma – 2 Types
• Child
➢ Adam Ant – inomatous
❖ enamel organ of tooth
➢ Commonly Cystic
➢ ‘Machine Oil’
➢ Commonly Calcified
➢ Adherent to brain
❖ pilocytic astrogliosis
• Adult
➢ Squamous and Papillary
➢ Commonly Solid
➢ Calcification less common Pituitary macroadenoma with hyperintensity from old
➢ Easier to resect hemorrhage
Craniopharyngioma [Figure 5-8-30]
Figure 5-8-29
Figure 5-8-30
Craniopharyngioma, you barely see

how the hypothalamus is draped over
the top of the mass
Craniopharyngioma,
expansile remodeling of sella
turcica
Craniopharyngioma “Machine Oil” Figure 5-8-31
Craniopharyngioma [Figure 5-8-31]
Craniopharyngioma

1114 Neuroradiology
Where is the Hypothalamus ? Figure 5-8-32
[Figure 5-8-32]
• Hypothalamic Glioma – Pilocytic astrocytoma
Where is the Clivus?

[Figure 5-8-33]
• Chordoma:
• Notochord rests
• Midline
Figure 5-8-33
Hypothalamic Glioma – Pilocytic astrocytoma
Figure 5-8-34
Chordoma of the clivus

Schematic of location of remnant notochord tissue.
Notochord: Chordoma & Thornwaldt Copyright 2003
[Figure 5-8-34]
SELLA/PARASELLAR
• Differential Features: Figure 5-8-35
➢ ADULT – Pituitary adenoma
➢ CHILD – Craniopharyngioma or
Glioma (hypothalamus or optic ) >
EG, etc
➢ SELLA NORMAL – NOT pituitary
➢ Ca++ – Craniopharyngioma, but...
➢ HYPEROSTOSIS – Meningioma (
exp. “blistering” )
➢ CLIVUS – Chordoma, mets, NP Ca
➢ Remember – rule out vascular
lesions (aneurysms)
Pulsation Artifact: Phase-

encoding direction [Figure 5-8-35]
Giant (> 2.5 cm) cerebral aneurysm
CLASSIC LOCATIONS
• Foramen magnum
Neuroradiology 1113
• Lateral ventricle/Pineal region Figure 5-8-36
Colloid Cyst [Figure 5-8-36]

• Hydrocephalus: Vents > Sulci
THIRD VENTRICLE [Figure 5-8-37]

• Differential:
➢ Colloid cyst
➢ Cysticercosis
➢ Craniopharyngioma Colloid cyst
➢ Hypothalamic and thalamic glioma
➢ CPP, ependymoma
➢ Neurocytoma Figure 5-8-37
➢ Basilar tip aneurysm
Colloid Cyst [Figure 5-8-38 to 5-8-40]

Figure 5-8-38
Colloid cyst
Differential Diagnosis – 3rd ventricle.
Copyright 2005
Figure 5-8-39
Figure 5-8-40
Colloid cyst. T1-weighted image shows slight

hyperintensity before gadolinium and no definite
enhancement Colloid Cyst. Marked hypointensity on T2W
image

1116 Neuroradiology
HYDROCEPHALUS Figure 5-22-41
• Differential Diagnosis:
➢ Over production of CSF (CPP)
➢ Obstruction of CSF flow:
❖ Obstructive/internal hydrocephalus
❖ Communicating/external hydrocephalus
• Under reabsorption of CSF: SAH
• Compensatory:
• Ex vacuo/enlargement
CSF Homeostasis [Figure 5-8-41]
Normal Ventricular System [Figure 5-8-42]
Schematic of CSF Homeostasis

Figure 5-22-42 Copyright 2004
Schematic of Normal Ventricular System

Copyright 2004
Foramen of Monro Obstruction
Aqueduct Obstruction
Non-traumatic hemorrhage
[Figures 5-8-43 and 5-8-44]
Non-traumatic hemorrhage in the right thalamus Non-traumatic hemorrhage in the right thalamus.
Copyright 2006
Neuroradiology 1115
Hypertensive Hemorrhage Figure 5-8-45
Hemorrhage into a mass

• NOTE: Vasogenic Edema
ARTERIOLOSCLEROSIS
What do they have in

Common? [Figure 5-8-45]
• Multiple
• Bilateral
• Symmetric
• Anatomic
• Basal ganglia
• Toxic and/or Metabolic:
➢ Acquired CT – medial lenticular lesion – Globus Pallidus.
➢ Congenital MR – lateral lenticular lesion – Putamen
CO Poisoning [Figure 5-8-46]

Figure 5-8-46
MetOH Intoxication
Tx for MetOH - Fomepazole

• Fomepazole (Antizole, 4-methylperazole) is a
synthetic alcohol dehydrogenase inhibitor for IV
administration
• Clear yellow liquid, mw 82.1, mp 25º C (77º F)
• INDICATIONS: Antidote for ethylene glycol, or
methanol poisoning of suspected EG ingestion
➢ PRECAUTIONS: Dilute in > 100 mL NS, follow
hepatic enzymes & WBC (eos) during Rx,
interaction with ethanol (compete for ADH)
➢ DOSE: 15 mg/kg load, 10 mg/kg Q 12 h x 4
doses, then 15 mg/kg Q 12 h till EG < 20 mg/dL Carbon monoxide toxicity. Notice there are
bilateral medial lenticular (globus pallidus) signal
Deep Lesions abnormalities – hypointense on T1 and
• White Matter: hyperintense on T2
➢ Leukoencephalopathy
➢ “Bad White Matter Disease” Figure 5-8-47
➢ Small vessel disease
➢ Hypertension
➢ Glial Neoplasm
➢ Astrocytoma (incl. GBM)
➢ Oligodendroglioma
• Deep White and Gray Matter
➢ Lymphoma
➢ Toxoplasmosis
Both occur in HIV/AIDS, multiple lesions
Solitary Deep Lesion - Thalamus [Figure 5-8-47]
Glioblastoma – WHO Grade 4

• A solitary, deep, irregular, heterogeneous, ring-enhancing mass with
vasogenic edema
Glioblastoma multiforme of the

thalamus and temporal lobe

1118 Neuroradiology
Glioblastoma Multiforme [Figure 5-8-48] Figure 5-8-48
Toxoplasmosis [Figure 5-8-49]

• 2 patients with typical lesions
BASAL GANGLIA THALAMUS:

• BILATERAL SYMMETRIC
(toxic/metabolic):
➢ PUTAMEN – Methanol
➢ GLOBUS PALLIDUS – CO Poisoning
• BILATERAL ASYMMETRIC
(hematogenous):
➢ INFECTION (TOXO, etc.)
• UNILATERAL (acquired/neoplastic):
➢ THALAMIC GLIOMA (astrocytoma)
➢ HYPERTENSIVE HEMATOMA T2 - T1gad
(exclusion)
Glioblastoma multiforme with extensive vasogenic edema
CLASSIC LOCATIONS
• Foramen magnum Figure 5-8-49
• Lateral ventricle
• Pineal Region
Patterns in Neuroradiology
• Cerebello-Pontine Angle
• Fourth Ventricle/Cerebellum
• Sella/Parasellar
• Basal Ganglia/Third Ventricle
• Lateral Ventricle
• Pineal Region
• Deep Hemispheric/Periventricular
• Cortical/Subcortical
Intraventricular Neoplasms
• Ependymoma (and subependymoma)
• Choroid plexus papilloma
• Subependymal giant cell astro.
Toxoplasmosis
• Meningioma
• Colloid cyst (3rd)
• Medulloblastoma (4th)
• Dermoid/epidermoid
• Central neurocytoma
• Mets, lymphoma, Germ Cell
Subependymal Giant Cell Astro

(From Vince Mathews,
M.D. IU)
Neuroradiology 1117
Lateral Ventricle @ f. Monro Figure 5-8-50
• Lateral Ventricle/caudate
➢ Subependymal Giant Cell Astro.
❖ TUBEROUS SCLEROSIS, Enhances &
Ca++
➢ Subependymoma
❖ Variant of Ependymoma
❖ No Ca++, no enhancement
➢ Central Neurocytoma
❖ Septum pellucidum
➢ Cyst/Cavum septum pellucidum
➢ Huntington Chorea
❖ Atrophy
Lateral ventricular masses [Figure 5-8-50]
Lateral Ventricle - Trigone Schematic of lateral ventricular masses.

• Meningioma Copyright 2005
• Choroid Plexus Papilloma
• Xanthogranuloma
• Metastasis
• Lipoma
• Choroid Cyst
Trigone or Atrium
Trapped Temporal Horn
Choroid Plexus Papilloma

• Attached to normal Choroid Plexus
• Lobulated
➢ Fronds
➢ Papillae
• Trigone of lateral vent
➢ Children
• Fourth ventricle
➢ Adults
• Third ventricle
• CPA cistern
• Hydrocephalus
➢ Obstruction
➢ Production =/= Resorption
CSF Overproduction?
Central Neurocytoma
• Central
➢ Often centered on septum pellucidum
➢ Extension into both lateral ventricles
➢ Hyperdense on CT
➢ Gray matter on MR
➢ Spontaneous Bleed
➢ Calcifications
CLASSIC LOCATIONS
• Foramen magnum

1120 Neuroradiology
• Lateral ventricle/Pineal region Figure 5-8-51
Pineal/Quadrigeminal Cistern Region

• “Pinealomas”
➢ Germ cell tumors
❖ Seminoma
❖ Teratoma
• Pineal cell tumors
➢ Pineoblastoma
➢ Pineocytoma
• Gliomas (regional)
➢ Brainstem, callosum, thalamus Pineal region mass. Germinoma.
• Other Copyright 2005
➢ Dermoid, lipoma, arachnoid cyst
➢ Meningioma
Figure 5-8-52
➢ Vein of Galen malformations
Pineal Region Mass [Figure 5-8-51]
Germinoma [Figure 5-8-52]

• Central
➢ Pineal Region
➢ Suprasellar Cistern
• Homogeneous
• Hyperdense to GM
• Isointense to GM
• Uniform Enhancement
• CSF Seeding ?
• May ENGULF Pineal Ca++
Pineal region Germinoma
Pineal Cyst-Asymptomatic
[Figure 5-8-53]
Figure 5-8-53
• T1W sagittal and T1W-Gd axial
• Cyst is ovoid and hypointense
• Enlargement of pineal
• Cyst wall enhances minimally
• Quadrigeminal plate not compressed
Pineal Cyst
CLASSIC LOCATIONS
• Foramen magnum
• Fourth ventricle/Cerebellum Pineal cyst
DEEP AND PERIVENTRICULAR

• Glioma (astrocytoma, oligodendro.)
• Lymphoma (usually primary in CNS)
• Toxoplasmosis, CMV (ependymitis)
• Leukoencephalopathy (WM)
• Arteriolar sclerosis (HT)
• Infarcts (lacunar, tri-watershed)
• Hemorrhage
Neuroradiology 1119
Glioblastoma Multiforme
[Figures 5-8-54 and 5-8-55]
Figure 5-8-54
Figure 5-8-55
Expansile lesion of the corpus callosum

Lymphoma
Two “butterfly” lesions. One with peripheral dense
PCNSL [Figure 5-8-56] enhancement is a GBM; and, the other with softer more
uniform enhancement is primary CNS lymphoma
Figure 5-8-56
Figure 5-8-57
FLAIR T2W T1W Gd+

Primary CNS Lymphoma – an expansile
enhancing lesion of the corpus callosum
PCNSL: Immunocompetent
Cytomegalovirus – note the thin rim of abnormal
RIM PHOMA enhancement cause by ependymitis
(Courtesy Vince Mathews, M.D.)
CMV [Figure 5-8-57]
Multiple Sclerosis [Figure 5-8-58]
CLASSIC LOCATIONS Figure 5-8-58
Cortical and subcortical

• Foramen magnum
Multiple Sclerosis. Classic Dawson fingers – ovoid
lesions perpendicular to the ventricle from
perivenous inflammation

1122 Neuroradiology
CONVEXITY INTRAAXIAL Figure 5-8-59
• Gray-white junction
➢ Hematogenous neoplasm
➢ Hematogenous infection
➢ Hematogenous thrombi (multiple infarcts)
• Infarction/ischemia
• Vasculitis (infectious,autoimmune)
Hematogenous dissemination [Figure 5-8-59]

• Multiple
• Cortical/subcortical
• Ring Lesions
➢ smooth
➢ round
➢ uniform thickness
CEREBRAL INFARCTION [Figure 5-8-60]

• Abrupt Onset
• Gray Matter Involved Hematogenous dissemination.
• Little Mass Effect Multiple cortical ring-enhancing
• Vascular Territory & Wedge Shape lesions – necrotic metastases from
breast carcinoma on chemotherapy
Figure 5-8-60
Cerebral infarction. MCA territory, with matching

lesions on DWI and ADC map Figure 5-8-61
Time is Brain !
• Therapeutic Windows:
• 3 hours for IV tPA
• 6 hours for IA thrombolysis
• 9 hours for IV ‘Bat Spit’?
➢ an enzyme known as
desmoteplase or DSPA
➢ isolated from the saliva of
Desmodus rotundus
➢ vampire bat, Central and
South America, 1oz
NOTE: Clock starts with last time
patient was observed ‘normal’. If
you wake with a stroke, that PCA Infarct Lights up like a lightbulb
might be bedtime … unless you on MRI DWI
get up at night
PCA Infarct [Figure 5-8-61]
Neuroradiology 1121
CVA : Progression of CT findings Figure 5-8-62
Old Infarct
• Wallerian Degeneration

(DNET) [Figures 5-8-62 and 5-8-63]
• Imaging Features
➢ Cortical, most in temporal lobe
➢ Hypointense T1W, Hyperintense T2W
➢ Nodular Cortical Mass
❖ Multicystic
❖ Megagyric - Assoc. calvarial erosion
➢ No Edema DNET – Usually a cortical lesion, often wedge-
➢ No or Minimal Mass Effect shaped
➢ +/- Calvarial Erosion
➢ Occasional Enhancement
Figure 5-8-63
DDX: TUBEROUS SCLEROSIS
• Cortical Hamartomas (“Tubers):
➢ Appear to Spare Superficial Cortex
➢ Tend to be Multiple
• Accompanying Subependymal Nodules
• + Family History
• Generalized Seizures
CLASSIC LOCATIONS
• Foramen magnum
• Deep hemispheric/periventricular DNET – Usually a cortical lesion, often wedge-
• Cortical and subcortical shaped
CONVEXITY EXTRAAXIAL Differential [Figure 5-8-64] Figure 5-8-64

• EPIDURAL (sub-periosteal)
➢ (Hematoma, empyema, mets)
➢ (biconvex, acute, limited by sutures)
• SUBDURAL (epi-arachnoid)
➢ (Hematoma, empyema, mets)
➢ (Crescentic, subacute, crosses sutures)
• MENINGIOMA
➢ (hyperdense, hemispheric,hyperostosis,
homogeneous enhancement)
Epidural = Subperiosteal [Figure 5-8-65]
Epidural Metastasis
Schematic of epidural (left) and subdural (right)
localization.
Subdural = Epi-arachnoid [Figure 5-8-66] Copyright 2006
Subdural Metastasis
Meningioma – Pre and Post Gd

1124 Neuroradiology
Meningioma - Dural Tail Figure 5-8-65
MENINGIOMA [Figure 5-8-67]

• The 4H+ Tumor
➢ homogeneous
➢ hyperdense
➢ homogeneous enhancement
➢ hemispheric
➢ hyperostosis
➢ hormonally modulated
Figure 5-8-66 Epidural hematoma
Bilateral chronic subdural hematomas

Figure 5-8-67
Meningioma. Hyperdense on plain CT, hemispheric,

homogeneous enhancement, hyperostosis – the 4H+ tumor
Neuroradiology 1123
Patterns of Enhancement
Figure 5-9-1
Why Give Contrast?
Contrast Enhancement
• Vascularity
➢ Blood Volume (rCBV)
Perfusion MTT
➢ Blood Flow (rCBF)
➢ Arteries & veins > capillary
• Permeability Blood
➢ Capillary (leakage) Brain
Barrier
Breakdown
Mechanisms of Enhancement [Figure 5-9-1]

• Vascularity
• Permeability Two mechanisms of Contrast Enhancement:
Increased vascularity (rCBV and rCBF); and,
Contrast Enhancement increased permeability from breakdown of the
• VASCULAR (intravascular) PHASE blood-brain-barrier (BBBB)
➢ Inc. Blood Flow/Hypervascular
➢ AVM, Meningioma, GBM Figure 5-9-2
➢ TRUE "Luxury" Perfusion
➢ Hyperemic Swelling (“malignant brain edema”)
• INTERSTITIAL (extravascular) PHASE
➢ Blood-brain-barrier breakdown)
➢ Acute inflammation (MS)
➢ Neoplasm, Abscess, “granulation” tissue
➢ Ischemia, “luxury” perfusion, contusion
Types of Radiology Contrast

• Barium (BaSO4) for ingestion and enema
➢ Insoluble suspension
• Iodine for ingestion and enema
➢ Gastrograffin
• I+ and Gd+ Intravascular Contrast Agents
➢ Ionic Contrast The bolus creates a high intravascular
❖ High Osmolarity concentration gradient that pushes contrast
- Magnevist® – a chelate of Gd across a permeable membrane into the tissue
- di-N-methylglucamine salt of gadopentetate interstitial space
(Gd-DTPA)
❖ Iso and Low Osmolar Figure 5-9-3
- Iodixanol
➢ Non-Ionic Contrast
❖ ProHance® – Gadoteridol
Variable
❖ Omniscan® - Gadoimide
degrees of
permeability
Time Density Curves [Figure 5-9-2] alteration may
create variable
Variability in BBB permeability time-density
and perfusion [Figure 5-9-3] curves for
interstitial
(extravascular)
enhancement
Patterns of Enhancement 1124

1126 Neuroradiology
Double Dose of Contrast Figure 5-9-4
Steroid Suppression of Enhancement

[Figure 5-9-4]
MR vs. CT [Figures 5-9-5 and 5-9-6]
Figure 5-9-5
Steroids may reduce or completely suppress

visible enhancement
Enhancement on CT and MR are similar – except

for intraarterial and pachymeningeal (dural)
enhancement
What makes the BBB?

Figure 5-9-6
Semi-permeable Capillaries
• Brain
➢ Blood-brain-barrier
• Testicle
➢ Blood-Testicle barrier
• Ovary
➢ Blood-Ovary barrier
Ultrastructure of BBB [Figure 5-9-7]

• Neural capillary
➢ astrocytic feet
➢ continuous BM
➢ tight junctions
➢ no pinocytosis
• Non-neural or ABBB
➢ no astrocytic feet
➢ fenestrated BM
➢ intercellular gaps
➢ pinocytosis
Figure 5-9-7
Enhancement on CT and MR are

similar – except for intraarterial and
dural enhancement
Schematic of Ultrastructure of blood-brain-barrier
Neuroradiology 1125
1127 Patterns of Enhancement
The Berlin Wall and the BBB [Figure 5-9-8] Figure 5-9-8
• Who built the Berlin Wall?
➢ The East Germans
• Why?
➢ To keep out the West Germans
• But … the barrier works in BOTH directions
• Some things are kept out
➢ Drugs, Contrast material
• Some things are kept in:
➢ Hemosiderin
➢ Vasogenic Edema
CNS: Normal Tissues w/o BBB

• DURA (falx and tentorium)
• ARACHNOID ? (it’s avascular)
Hemosiderin cannot be cleared from the brain and
• CHOROID PLEXUS
spinal cord because of the blood-brain-barrier
• PINEAL GLAND (epiphysis)
• PITUITARY GLAND (hypophysis)
• CTZ (area postrema of medulla oblongata)
➢ one of the "Circumventricular Organs"
Physiologic Why?
• Why do we have a BBB?
➢ To protect the brain
➢ To create the ionic environment for nerve conduction
• Why do we have tastebuds?
➢ So that we eat things good for us
❖ Salt
❖ Sweet
❖ Sour
❖ Umami (MSG)
❖ Bitter
Normal Enhancement
• Choroid Plexus
• Pineal
• Pituitary Stalk
• Pituitary Gland (anterior and posterior)
➢ Hypophyseal Portal System
• Cavernous sinus and dural reflections
• Nasal turbinates
• Sinonasal mucosa
• Extracranial muscles and mucosa
Plain vs. Enhanced
Fat -Suppressed T1W - Gd
Nasal Cycle
• Vasocongestion ~/~ vasoconstriction
• 6 – 8 hour cycle alternation
• Humidify and warm the air
• Secrete mucus (1 – 2 liters/day)
• Chronic vasocongestion would cause submucosal edema
• Breathe mostly through the vasoconstricted side (~ 75%-85%)
• Yogi’s can control which nostril
• So can Tom Cruise (Minority Report)

1128 Neuroradiology
Fat -Suppressed T1W – Gd Figure 5-9-9
Musk Ox – Nasal Turbinates
Fat -Suppressed T1W - Gd [Figure 5-9-9]
Cranial Nerve Enhancement

• Optic Nerve – Never normal
• * Seventh Nerve:
➢ Inside facial canal – Yes, asymmetric ~70%
➢ Geniculate ganglion – 98%
➢ Tympanic > labyrinthine > mastoid
➢ May represent perineural vessels Abnormal optic nerve enhancement –
• Eighth Nerve – Never normal optic neuritis
* Neuroradiology 1997 Mar;39(3):207-12.
Figure 5-9-10
Plain vs. Enhanced [Figure 5-9-10]
Contrast Enhancement – Phases

VASCULAR BBB
ANGIO(I–) ++++ –
R-N (Tc+) + (flow) + (static)
C.T. (I–) + +++
MRI (Gd+) +/– +++
Enhancement vs. Edema
Normal pineal enhancement. Visualization

depends on the timing of injection and the
molecular weight of the contrast
• Morphologic Patterns
➢ Homogeneous (solid)
➢ Heterogeneous (non-uniform)
➢ Ring (unilocular/multilocular)
➢ Serpentine ("Gyriform“)
❖ Serpiginous?
Serpiginous
• “A creeping skin eruption.”
• Location
➢ SUPERFICIAL (CORTICAL/GYRAL)
➢ GREY-WHITE JUNCTION
➢ DEEP WHITE MATTER
➢ PERIVENTRICULAR, EPENDYMAL
Neuroradiology 1127
Contrast Enhancement Figure 5-9-11
• Cortical/Gyriform
➢ Cerebral Ischemia / Infarction
➢ CSF or sub-pial spread
➢ Meningo-encephalitis
➢ S.A.H.
➢ Leptomeningeal Malformation (SW)
➢ Meningioangiomatosis (NF2)
Listeria Monocytogenes [Figure 5-9-11]
CNS Bacterial Infections Meningitis - Listeria Monocytogenes

• Birth to Four Weeks
➢ 2-10 cases / 10k births
➢ Group B streptococcus Figure 5-9-12
➢ E. coli
➢ Listeria monocytogenes
• 3 mo. to 3 yrs
➢ Haemophilus influenzae (Type B)
➢ Strep pneumoniae
➢ Meningococcus (Neisseria
meningitidis)
• Over 3 yrs to Adult
➢ Strep pneumoniae
➢ N. meningitidis
Flair shows “dirty CSF” from protein and pus in the SAS
CSF Signal ? [Figure 5-9-12] (Zulmarie Roig, MD, Gil Gonzalez, MD, MGH)
Enhancement? [Figure 5-9-13] Figure 5-9-13

• Leptomeningeal Enhancement -
Pneumococcal Meningitis
Pachymeningeal Enhancement
[Figure 5-9-14]
• Intracranial Hypotension
Figure 5-9-14
Multiple symmetric areas of abnormal leptomeningeal

enhancement from meningitis Notice the abnormal
ehancement of the entire suprasellar cistern
(Zulmarie Roig, MD, Gil Gonzalez, MD, MGH)
Pachymeningeal
enhancement
(Courtesy Laszlo Mechtler,
DNI)

1130 Neuroradiology
Hemorrhagic Infarction [Figure 5-9-15]
Figure 5-9-15
Ischemic Enhancement
• Acute and/or Reperfusion enhancement
➢ True “luxury perfusion” 2° to acidosis
➢ BBBB after 4-6 hours of ischemia
• Subacute to Chronic enhancement
➢ Ingrowth of capillaries from surface
➢ Primarily in GM (cortex and deep)
➢ Peak intensity at 2-3 weeks
➢ Fades away over weeks to months
• Atrophy replaces Enhancement
Blood Brain Barrier [Figure 5-9-16]

• Cirrhosis Hemorrhagic infarction shows early and dense
• Hyperbilirubinemia enhancement due to reperfusion
• Bilirubin bound to Albumin
• Albumin can’t cross the Blood-brain-barrier Figure 5-9-16
• BBB is abnormal in infarct
• Mostly gray-matter
H&P
• Pt is a 25 yo woman, PMHx of BCP, presenting w/
acute mental status changes, afebrile
Actually…
• Pt is a 34 yo marine stationed at Guantanamo Bay
Cuba, presenting w/ acute mental status changes,
febrile
HSV Encephalitis [Figure 5-9-17]
Figure 5-9-17 Gyral enhancement in Sturge-Weber disease
Figure 5-9-18
Herpes encephalitis
Meningioma – Dural Tail [Figure 5-9-18]
Dural Tail
• Curvilinear enhancement Meningioma - Dural Tail
• AKA “dural flair”
• First reported w/meningioma
• First reported to be neoplastic invasion
• What is it REALLY?
➢ Thickening of the dura
➢ Vasocongestion of the dura
➢ Edema of the dura
Neuroradiology 1129
Contrast Enhancement [Figure 5-9-19] Figure 5-9-19
• Ring Lesion
➢ Circumferential or peripheral/marginal
enhancement, surrounding a central non-
enhancing region.
➢ In turn, this is often surrounded by a large area
of “edema”.
➢ May be unilocular or multilocular.
Rules for Ring Enhancing Masses

[Figure 5-9-20]
Figure 5-9-20 Abscess
Differential appearance of ring enhancing lesions
• Contrast leaks into interstitium from vessels without BBB
• Remains localized within millimeters of where it leaks out
• Not “simple diffusion” but rather “BULK FLOW” at a very slow rate
• (Glacier Not River)
• Ring Lesions Differential
➢ M – Metastasis, MS
➢ A – Abscess (Also Cerebritis)
➢ G – Glioblastoma, Granuloma
➢ I – Infarct (Esp. Basal Ganglia)
➢ C – Contusion (Rare)
➢ A – AIDS (Toxo, Etc.)
➢ L – Lymphoma (in Aids)
➢ D – Demyelination (Active)
➢ R – Resolving Hematoma
Radiation Change (Necrosis)
• Ring Lesion Features For Infection
➢ ORGANIZED ABSCESS
➢ thin and uniform wall (3-7mm.)
➢ smooth inner margin does not “fill in” on CT, MR, even after time delay
imaging
➢ CEREBRITIS (infection w/o organization):
➢ variable wall (may be smooth) smooth/variable inner margin
➢ often has “fill-in” on DDD
➢ (w/o fluid level)

1132 Neuroradiology
Contrast Enhancement- Abscess Figure 5-9-21
• 2 – 4 wks. for ORGANIZED WALL
• 2 LAYERS
➢ inner MESENCHYMAL (capillaries,fibroblasts, collagen)
➢ outer ASTROGLIAL (reactive astrocytes)
• WALL facing GM is well formed 3-5 mm
• WALL FACING WM IS THINNER/WEAKER (Daughter Abscess)
• Ventricular Spill (“pyocephalus”)
Abscess [Figures 5-9-21 to 5-9-23]

• Round
• Smooth
• Regular
• Convex all around
• Rim of Edema
• Restricted Diffusion
• MRS shows
➢ AA peaks
➢ Acetate
➢ Succinate
Figure 5-9-22
Cerebral abscess in thalamus
Figure 5-9-23
Abscess. Viscous Pus and Coagulation Necrosis

cause restricted diffusion
Figure 5-9-24
Abscess
Contrast Enhancement [Figure 5-9-24]

• Ring Lesion Features For Neoplasm
• NECROTIC NEOPLASM:
➢ thick and irregular wall
➢ shaggy inner margin (usually)
➢ may “fill in” heterogeneously on DDD
• CYSTIC NEOPLASM:
➢ thin wall +/– MURAL NODULE
➢ PART OF WALL MAY NOT ENHANCE
❖ smooth inner margin
❖ uniform fluid enhancement Ring enhancing lesion: Glioblastoma
❖ or FLUID LEVEL
Neuroradiology 1131
Glioblastoma Multiforme Figure 5-9-25
Pilocytic Astrocytoma [Figure 5-9-25]

• Cyst with mural nodule?
Tumefactive Demyelination
[Figure 5-9-26]
Open (Incomplete) Ring Sign

• Demyelinating Disease
• Fluid-secreting (“Cystic”) Neoplasms
Masdeau JC, Moreira J, Trasi S, Visintainer
P, Cavaliere R, Grundman M: The open ring.
A new imaging sign in demyelinating disease. Figure 5-9-26
J Neuroimaging 1996; 6(2):104-107.
Masdeu JC, Quinto C, Olivera C, Tenner M, Leslie D,
Visintainer P: Open-ring imaging sign: highly specific for
atypical brain demyelination. Neurology 2000;
54(7):1427-1433
Contrast Enhancement: Hematoma

• EARLY: Hyperdense, round/oval homogeneous
mass of RBC’s with proportional mass effect for
volume Edema “Halo”, not spreading
• LATER: Iso-/Hypodense, smaller Reactive capillaries
form outside Uniform rim of enhancement May see
“vasogenic” edema spreading
Tumefactive Demyelination
Hematoma – Halo of serum [Figure 5-9-27]
Figure 5-9-28
Figure 5-9-27
Acute Hematoma - halo of edema

Acute Hematoma - halo of edema Subacute to chronic may have vasogenic edema
Subacute to chronic may have vasogenic edema
Figure 5-9-29
Reactive Ring Enhancement [Figure 5-9-28]
MCA infarct [Figure 5-9-29]
Ring Enhancing Mass

• Benign
➢ Round
➢ Smooth
➢ Thin wall
• Malignant
➢ Undulating
➢ Irregular MCA infarct involving cortex and basal ganglia
➢ Thick wall

1134 Neuroradiology
Post-Operative Enhancement Figure 5-9-30
• RESIDUAL TUMOR
➢ Left behind
• RECURRENT TUMOR
➢ It grew back
• Infection
• Normal Postoperative Change
➢ surgical “trauma”, healing, gliosis
• Radiation Necrosis
Contrast Enhancement – Surgical Change

and/or Residual Neoplasm? [Figure 5-9-30]
• Surgical Enhancement typically after 24-48 hrs
➢ Scan early (24 hours) or scan late (4-6 wks)
Normal enhancement after ventricular shunt
➢ May fade after a few weeks but may last for
catheter insertion
months
➢ Gd+ enhancement may begin in 4-6 hours
• In the Operative Bed
➢ Mixed w/ residual tumor?
➢ Along the Margins of Resection
➢ Thin and uniform in brain (CT/MR)
• LINEAR meningeal/dural enhancement on MR
➢ Not lumpy-bumpy
• Small amts of air, blood are normal
➢ No instruments or sponges, etc.!!
References
1. Ahmadi J, Hinton DR, Segall HD, Couldwell WT. Surgical implications of magnetic resonance-enhanced dura.
Neurosurgery. 1994 Sep;35(3):370-7;discussion 377.
2. Aoki S, Sasaki Y, Machida T, Tanioka H. Contrast-enhanced MR images in patients with meningioma: importance
of enhancement of the dura adjacent to the tumor. AJNR Am J Neuroradiol. 1990 Sep-Oct;11(5):935-8.
3. Asari S, Yabuno N, Ohmoto T. Magnetic resonance characteristics of meningiomas arising from the falcotentorial
junction. Comput Med Imaging Graph. 1994 May-Jun;18(3):181-5.
4. Ekinci G, Akpinar IN, Baltacioglu F, et al. Early-postoperative magnetic resonance imaging in glial tumors:
prediction of tumor regrowth and recurrence. Eur J Radiol 2003; 45:99-107.
5. Goldsher D, Litt AW, Pinto RS, Bannon KR, Kricheff II. Dural "tail" associated with meningiomas on Gd-DTPA-
enhanced MR images: characteristics, differential diagnostic value, and possible implications for treatment.
Radiology. 1990 Aug;176(2):447-50. .
6. Helie O, Soulie D, Sarrazin JL, Derosier C, Cordoliani YS, Cosnard G. [Magnetic resonance imaging and
meningiomas of the posterior cerebral fossa. 31 cases] J Neuroradiol. 1995 Dec;22(4):252-70. French.
7. Henegar MM, Moran CJ, Silbergeld DL. Early postoperative magnetic resonance imaging following nonneoplastic
cortical resection. J Neurosurg 1996; 84:174-179.
8. Hutzelmann A, Palmie S, Buhl R, Freund M, Heller M. Dural invasion of meningiomas adjacent to the tumor
margin on Gd-DTPA-enhanced MR images: histopathologic correlation. Eur Radiol. 1998;8(5):746-8.
9. Hutzelmann A, Palmie S, Freund M, Buhl R, Heller M. [Dura thickening adjacent to intracranial, para-dural space-
occupying lesions in MRI. Histologic correlation] Aktuelle Radiol. 1997 Nov;7(6):305-8. German. PMID:
9467021
10. Hutzelmann A, Palmie S, Zimmer C, Benz T, Leweke F, Freund M. [The meningeal sign: a new appraisal] Rofo.
1996 Apr;164(4):314-7. German.
11. Ildan F, Tuna M, Gocer AP, Boyar B, Bagdatoglu H, Sen O, Haciyakupoglu S, Burgut HR. Correlation of the
relationships of brain-tumor interfaces, magnetic resonance imaging, and angiographic findings to predict cleavage
of meningiomas. J Neurosurg. 1999 Sep;91(3):384-90.
12. Kaufman BA, Moran CJ, Park TS. Computer tomographic scanning within 24 hours of craniotomy for a tumor in
children. Pediatr Neurosurg 1995; 22:74-80.
13. Kawahara Y, Niiro M, Yokoyama S, Kuratsu J. Dural congestion accompanying meningioma invasion into vessels:
the dural tail sign. Neuroradiology. 2001 Jun;43(6):462-5.
14. Lai PH, Ho JT, Chen WL, et al. Brain abscess and necrotic brain tumor: discrimination with proton MR
spectroscopy and diffusion-weighted imaging. AJNR Am J Neuroradiol 2002; 23:1369-1377.
Neuroradiology 1133
15. Martin-Duverneuil N, Sola-Martinez MT, Miaux Y, et al. Contrast enhancement of the facial nerve on MRI:
normal or pathological? Neuroradiology 1997; 39:207-212.
16. Masdeau JC, Moreira J, Trasi S, Visintainer P, Cavaliere R, Grundman M: The open ring. A new imaging sign in
demyelinating disease. J.Neuroimaging 1996; 6(2):104-107.
17. Masdeu JC, Quinto C, Olivera C, Tenner M, Leslie D, Visintainer P: Open-ring imaging sign: highly specific for
atypical brain demyelination. Neurology 2000; 54(7):1427-1433.
18. Nagele T, Petersen D, Klose U, Grodd W, Opitz H, Voigt K. The "dural tail" adjacent to meningiomas studied by
dynamic contrast-enhanced MRI: a comparison with histopathology. Neuroradiology. 1994 May;36(4):303-7.
19. Nakasu S, Nakasu Y, Matsumura K, Matsuda M, Handa J. Interface between the meningioma and the brain on
magnetic resonance imaging. Surg Neurol. 1990 Feb;33(2):105-16.
20. Nakau H, Miyazawa T, Tamai S, Tsuchiya K, Shima K, Shirotani T, Chigasaki H. Pathologic significance of
meningeal enhancement ("flare sign") of meningiomas on MRI. Surg Neurol. 1997 Dec;48(6):584-90; discussion
590-1.
21. Quekel LG, Versteege CW. The "dural tail sign" in MRI of spinal meningiomas. J Comput Assist Tomogr. 1995
Nov-Dec;19(6):890-2.
22. Sakai K, Tada T, Fukasaku K, Kyoshima K, Kobayashi S. Histological examination of the gadolinium-enhanced
dura mater around meningiomas on magnetic resonance imaging. Neurol Med Chir (Tokyo). 1993 Jul;33(7):429-
33.
23. Sato M, Matsumoto M, Kodama N. Meningeal enhancement surrounding meningiomas on Gd-DTPA MRI.
Fukushima J Med Sci. 1998 Jun;44(1):1-11.
24. Sato N, Bronen RA, Sze G, et al. Postoperative changes in the brain: MR imaging findings in patients without
neoplasms. Radiology 1997; 204:839-846.
25. Sekiya T, Manabe H, Iwabuchi T, Narita T. [The dura mater adjacent to the attachment of meningiomas: its
enhanced MR imaging and histological findings] No Shinkei Geka. 1992 Oct;20(10):1063-8. Japanese.
26. Takeguchi T, Miki H, Shimizu T, Kikuchi K, Mochizuki T, Ohue S, Ohnishi T. The dural tail of intracranial
meningiomas on fluid-attenuated inversion-recovery images. Neuroradiology. 2004 Feb;46(2):130-5. Epub 2004
Jan 28.
27. Wilms G, Lammens M, Marchal G, Van Calenbergh F, Plets C, Van Fraeyenhoven L, Baert AL. Thickening of dura
surrounding meningiomas: MR features. J Comput Assist Tomogr. 1989 Sep-Oct;13(5):763-8.
28. Yamaguchi N, Kawase T, Sagoh M, Ohira T, Shiga H, Toya S. Prediction of consistency of meningiomas with
preoperative magnetic resonance imaging. Surg Neurol. 1997 Dec;48(6):579-83.

1136 Neuroradiology
Radiologic Grading of Astrocytoma and
The WHO 2000 Brain Tumor Classification
Brain Neoplasia: Frequency [Figure 5-10-1] Figure 5-10-1
Childhood CNS Tumor Demographics

• 367 Syrian children, collected from 1993-2002
➢ Supratentorial - 47%
➢ Infratentorial - 53%
➢ Male 52%:48% Female
➢ Overall Incidence:
❖ Medulloblastoma 27%
❖ Astrocytoma 26%
❖ Craniopharyngioma 14%
➢ Posterior Fossa Only:
❖ Medullo (PNET) 54%
❖ Astrocytoma 23%
❖ Ependymoma 17%
Kadri H,Mawla AA, Murad L: Incidence of childhood brain The frequency of the various primary central
tumors in Syria (1993-2002) Pediatric Neurosurgery nervous system tumors ranges from 2% for
2005; 41:173-177 meningioma and mixed oligoastrocytoma to 40%
for glioblastoma multiforme (GBM) and 42% for
Pediatric Posterior Fossa infiltrative astrocytoma
• 454 posterior fossa patients
• All under the age of 18
• 402 tumors:
➢ 37.1% Cerebellar astrocytoma (149)
➢ 34.6% Medulloblastoma (PNET) (139)
➢ 11.4% Brain stem astrocytoma (46)
➢ 7% Ependymoma (28)
➢ 9.9% "other" (40)
Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst
1998; 14:426-439.
Traditional Tumor Grading

• PATHOLOGIST
➢ LOW GRADE
➢ HIGH GRADE
• RADIOLOGIST
➢ NON-ENHANCING
➢ ENHANCING
• NEUROSURGEON
➢ “SUCKABLE”
➢ “NON-SUCKABLE”
Kernohan-Sayre (AFIP) Grading System:

• GRADE I – “BENIGN” or “Low-Grade”
• GRADE II – “BENIGN” or “Low-Grade”
• GRADE III – ANAPLASTIC
➢ atypia, pleomorphism, mitoses, etc.
• GRADE IV- MALIGNANT
➢ Mitoses, Vascularity, Endothelial changes
➢ Necrosis
➢ Glioblastoma Multiforme
• NOTE: Numerous modifications exist, most into three grades, e.g..: Low Grade
(Benign), Anaplastic, and GBM (w/ NECROSIS).
Neuroradiology 1135
1137 The WHO 2000 Brain Tumor Classification
Pathologic – Radiologic Correlation
Pathology Radiology
Cellularity T2 SI, DWI & ADC
Endothelial proliferation Enhancement, PWI, and
and Vascularity Permeability Imaging
Necrosis Ring Lesion, MRS, DWI & ADC
Hemorrhage T1 and T2 SI
Labeling Indices MRS, Th 201 and FDG
Infiltration T1 and T2 SI, DTI
ASTROCYTOMA: Five Year Survival
Grading Systems: Sem Rad Onc (1991); 1: 2-9

Kernohan Berger WHO 2000
1 Pilocytic,SEGA
Benign (1) Astrocytoma
2 Astrocytoma
Benign (2)
Anaplastic 3 Anaplastic
Anaplastic (3)
Glioblastoma (4) Glioblastoma 4 Glioblastoma
Define the Problem

• Some Low Grade Enhance
• Some Low Grade Do Not
• Some Low Grade => GBM
WHO Classification
• Defines Histologic Subtypes
• Grades Biologic Potential
• Allows International Cooperation
• Ascending scale of Aggression from 1-4
WHO Correlation
• Low Grade
➢ Long-Term Survival: Possible Cure
➢ Stable History (No Progression): Possible Cure
WHO Grading CNS Tumors

GRADE 1 JPA SGCA GANG MENING
GRADE 2 PXA HPC
GRADE 3 PXA ANAPLASTIC HPC
GRADE 4 GBM
CNS NEOPLASM-GLIAL: Prognostic Factors

• Location
• Age
• Histology
The WHO 2000 Brain Tumor Classification 1136

1138 Neuroradiology
“BENIGN” ASTROCYTOMA Figure 5-10-2
• Two types:
➢ Low grade (“benign”)
❖ Diffuse (Adults)
➢ Low grade “special”
❖ Circumscribed (Children)
Normal
Diffuse Astrocytoma [Figure 5-10-2]
WHO Gr1 - Pilocytic Astrocytoma [Figure 5-10-3]
Circumscribed vs Diffuse
Circumscribed Astrocytoma Diffuse astrocytoma: Individual neoplastic cells

spread out through the white matter. In WHO Gr 2,
Astrocytoma: Circumscribed this is only noted as “increased cellularity”
• “Special” astrocytomas
➢ Astrocytoma of young
➢ Various locations
➢ Well circumscribed (yet, no capsule) Figure 5-10-3
• Do NOT spread along WM
• Do NOT change grade (except PXA)
• Constellation of findings correlates w/ Histology
• Cystic Cerebellar Astrocytoma
Juvenile Pilocytic Astrocytoma
(“PA” or “JPA”)
• Synonyms: Polar Spongioblastoma, Cystic
Cerebellar Astrocytoma
• Cell of Origin: Astrocyte (bi-polar, hairlike)
• Associations: in ON w/ NF-1
• Incidence: 3%–6% of ALL Cranial, 32% of Child
• Age: 5–15 (Zulch 3–7) Sex: Slight F (11/9) Circumscribed astrocytoma, like pilocytic
• Location: Cerebellum, Chiasm/Hypothal, Optic astrocytoma, have “pushing margins” and are
• Treatment: Surgery, patience often fluid-secreting
• Prognosis: 77% at 5 yrs, 75% at 10 yrs, 75% at 15 yrs
Pilocytic Astrocytoma: Radiology

[Figures 5-10-4 to 5-10-6]
• Cerebellum, Diencephalon
➢ rare in BS or Cerebrum Figure 5-10-4
• Majority have significant “cyst”
➢ “Cyst and Mural Nodule”
❖ part of lining does NOT enhance
➢ Nodule may be heterogeneous
➢ Exceptional purely solid
• Nodule NOT hyperdense
• Calcification in 5%–25%
Pilocytic astrocytoma with classic “cyst and

nodule” morphology
Neuroradiology 1137
Gross picture of pilocytic astrocytoma
Pilocytic astrocytoma with classic “cyst and

nodule” morphology Figure 5-10-7
WHO Gr1 - Pilocytic Astrocytoma
Pathology
• Biphasic pattern
➢ dense pilocytic glia
➢ Rosenthal fibers
➢ loose microcystic areas
• No necrosis
• Low grade
• Abnormal capillaries
➢ allow enhancement Pilocytic Astrocytoma
➢ fluid production
Grading Problems in Gliomas Figure 5-10-8

51 Pilocytic (WHO Gr. 1)
KERNOHAN MAYO-ST.ANNE
1 26% 1 2%
2 69% 2 55%
3 6% 3 35%
4 0% 4 8%
• By conventional “feature counting” most pilocytic
astrocytomas were overgraded.
Pilocytic Astrocytoma Pilocytic Astrocytoma

• Variant Appearance (Courtesy of Paul Sherman)
• Variant Location

• A Cyst with mural nodule? Figure 5-10-9
• Not Always !!!
Pilocytic Astrocytoma: Locations [Figure 5-10-9]

• CEREBELLUM
• Chiasm And Optic Nerve
• Hypothalamus/thalamus
• Cerebral Hemisphere
• Spinal Cord (Intramedullary)
Pilocytic astrocytoma of hypothalamus

1140 Neuroradiology
• Pilocytic Astrocytoma Hemangioblastoma
➢ Enhance Enhance
➢ Cyst w/ Nodule Cystic Solid <- cyst w/nodule ->
➢ Hypodense nodule Hyperdense nodule
➢ Calcification Never Ca++
➢ NOT vascular Hypervascular, Flow Voids
➢ Nodule location varies Nodule is “Subpial”
Pilocytic Astrocytoma (Juvenile Pilocytic) Figure 5-10-10

• Childhood, Young Adults
• Benign, no mitosis/necrosis
• Circumscribed – Enhancing
• Cyst Formation, Mural Nodule
• Cerebellum and Diencephalon
➢ (Optic tracts, Hypothalamus)
WHO Grade I
• Circumscribed Astrocytoma
➢ JPA (Pilocytic)
➢ SGCA (Subependymal Giant Cell)
• Ganglioglioma
• Meningioma
Subependymal Giant Cell Astro [Figure 5-10-10] Subependymal Giant Cell Astrocytoma
Astrocytomas
• “SPECIAL” ASTROCYTOMAS
➢ Circumscribed Growth:
❖ Pilocytic
❖ Subependymal Giant Cell
❖ Pleomorphic Xantho-Astrocytoma
Circumscribed Astrocytoma
• Rare Variant of Astrocytoma
• Arises from Subpial Astrocytes
• Affects Superficial Cerebral Cortex and Meninges
• Skull erosion (scalloped excavation)
• Temporal > Frontal > Parietal
• WHO Grade 2,3
• 50% progress over time
• IMAGING:
➢ CT APPEARANCE:
❖ Well-Circumscribed Hypodense or Cystic Mass
❖ Often Isodense Solid Nodule that Intensely Enhances
❖ May Mimic Juvenile Pilocytic Astrocytoma
❖ Calcifications Rare
• MR APPEARANCE:
➢ Well-Circumscribed Mass of Variable Size
➢ Superficial Cortical Location
➢ T1: Low/Mixed Signal,
➢ T2: High/Mixed Signal
➢ Often with Cystic Component
➢ Solid Portion Intensely Enhances
➢ Adjacent Meninges May Enhance (Tail)
➢ Little or No Mass Effect
Neuroradiology 1139
Astrocytomas
• “Ordinary” Astrocytoma
➢ Diffuse Infiltration of WM by:
❖ Fibrillary Astrocytes
❖ Protoplasmic Astrocytes
❖ Gemistocytic Astrocytes
❖ WHO 2,3,4 (NOT 1)
❖ KS & Mayo Grades 1–4
Where do Glioblastomas come from?

• Progressive Transformation from lower grade diffuse astrocytoma
- OR -
• Arise de novo
Diffuse Astrocytoma … too many cells !

• Mild cellular atypia
KERNOHAN 1 2 3 4
(KS)
ANAPLASIA 0 Min >1/2 Marked
CELLULARITY Mild Mild Inc Marked
MITOSIS 0 0 Plus Marked
ENDOTHELIAL 0 Min Min Marked

Proliferation
NECROSIS Marked
TRANSITION <== Broad Sharp ==>

ZONE Figure 5-10-11
Astrocytoma: Diffuse
(Fibrillary, protoplasmic, etc.)
• “Adult type” or “Hemispheric” Astrocytoma
• Diffusely infiltrate brain, along WM tracts
• Continuum, from low-grade to high-grade
• Genetic Alterations 17 => 9 => 10
• Many Progress in Histology over time, changing
from WHO Gr. 2 => Gr. 3 => Gr. 4 (GBM)
• Imaging tends to correlate with histology, especially
at the ends of spectrum
Astrocyte Mutation [Figure 5-10-11]

Successive mutations in Astrocytoma
Diffuse Astrocytoma [Figures 5-10-12 and 5-10-13]
Figure 5-10-12
All three grades of

astrocytoma in one
patient

1142 Neuroradiology
Diffuse Astrocytoma Figure 5-10-13
• ‘Astrocytoma’
• Anaplastic Astrocytoma
• Glioblastoma Multiforme
• The Eastwood Method:
➢ The Good
➢ The Bad
➢ The Ugly
Astrocytoma: Radiologic Grading

• TYPE 1 – WHO 2, KS Grade 1–2, “Benign”
➢ Homogeneous
➢ No Enhancement, No Vasogenic Edema
• TYPE 2 – WHO Grade 3, Anaplastic
➢ Variable Enhancement, Edema
➢ 50% enhance – 50% don’t
• TYPE 3 – WHO Grade 4 Glioblastoma Diffuse astrocytoma is a spectrum of tumors,
➢ Heterogeneous (Necrosis, Blood) pathologically and by imaging. The classification
➢ Ring Enhancement, Edema into three grades (WHO 2,3, or 4) may be an
artificial segmentation along a continuum
“Benign” Astrocytoma: WHO 2, KS 1–2,
Mayo 1
• YOUNGER PATIENT
➢ CHILDHOOD
➢ Young Adults (20’s – 40’s)
• NL VESSELS (NO NEOVASCULARITY) Figure 5-10-14
➢ BBB INTACT
➢ NO EDEMA
➢ NO ENHANCEMENT
➢ NO TUMOR VESSELS
Benign – Diffuse
• HOMOGENEOUS
➢ NO NECROSIS
➢ NO HEMORRHAGE
➢ INCREASED WATER
❖ DARK and Poorly Demarcated on CT
❖ Dark and Sharp on T1W
❖ BRIGHT and Sharp on T2W PD T2
➢ MICROCYST >>> MACROCYST Gr 2 Fibrillary Astrocytoma
(macrocysts occur in JPA, etc.)
Gr 2 Fibrillary Astrocytoma
[Figures 5-10-14 and 5-10-15]
Figure 5-10-15
T1- T1-
non gad
Gr 2 Fibrillary Astrocytoma – no enhancement
after gadolinium
Neuroradiology 1141
Gr 2 Astrocytoma: PWI [Figure 5-10-16] Figure 5-10-16
• Reduced perfusion
Gliomatosis Cerebri [Figure 5-10-17]
Figure 5-10-17
T2 T1-gad
Female, acute stroke (3 days), MCA occlusion
Figure 5-10-18
Gliomatosis Cerebri – a diffuse astrocytoma

infiltrating two or more lobes of the brain
Gliomatosis Cerebri: Diffuse Astrocytoma –

2 lobes
[Figure 5-10-18]
Spread along White Matter Tracts

[Figure 5-10-19]
Figure 5-10-19
Gliomatosis Cerebri – a diffuse astrocytoma
infiltrating two or more lobes of the brain
Figure 5-10-20
Diffuse Grade 2 astrocytoma spreading through

white matter, including corpus callosum Astrocytoma: Microcystic change
Gliomatosis Cerebri
Astrocytoma: Microcystic Change [Figure 5-10-20] Figure 5-10-21
Astrocytoma [Figure 5-10-21]
Pontine astrocytoma – WHO Gr 2

1144 Neuroradiology
Modes of Spread Figure 5-10-22
• 1. Natural passages
• 2. Along surfaces
• 3. Along tracts
• 4. Across the meninges
Spread Along Tracts:

• Corona Radiata
• Peduncles
• Corpus Callosum
• Anterior Commisure
• Arcuate Fibres
Astrocytes Track Along WM Progressive transformation of WHO Grade 2

astrocytoma to Glioblastoma multiforme
Pontine Astrocytoma
Pontine Astrocytoma: WHO 2 Figure 5-10-23
WHO 2 … GBM [Figure 5-10-22]
Expanded Brain
Anaplastic Astrocytoma: Overall

Characteristics
• Grade III malignant glioma
• Less aggressive than GBM, malignant Anaplastic astrocytoma – no enhancement in this example –
with somewhat better prognosis about 50% will enhance
• Frequency: highest in young adults (30 – 40 years)
• Recurrence: often as a higher-grade glioma
• Challenge: difficult to remove completely with surgery
• Median survival: 3 – 4 years
Anaplastic Astrocytoma [Figure 5-10-23]
Anaplastic Astrocytoma ( WHO 3 )

• Increased Cellularity, +/- minimal vascular changes, no necrosis , no
hemorrhage
GBM - Glioblastoma
“Malignant” Astrocytoma:
• Older patient
➢ 40’s and up
➢ exceptions (PNET)
➢ ~ 1/2 arise from previous low grade (2–3)
• Abnormal Vessels (neovascularity)
➢ BBB abnormality
➢ vasogenic edema
➢ contrast enhancement
➢ irregular vessels, shunting, etc.
• HETEROGENEOUS
➢ hemorrhage (old/new)
➢ tumor necrosis
➢ tumor itself
Neuroradiology 1143
Astrocytoma Gr4: Angiogenesis Figure 5-10-24
Glioblastoma Multiforme [Figures 5-10-24 and 5-10-25]
Figure 5-10-25
T1-gad T2
Glioblastoma Multiforme. Photomicrograph at

high power shows both angiogenesis and
pseuopalisading necrosis
Figure 5-10-26
(Gr 4) Glioblastoma: PWI-CBV [Figure 5-10-26]
Glioblastoma – WHO Grade 4
Mechanisms of Enhancement
Ultrastructure of BBB
• Neural capillary ABBB or Non-neural
➢ astrocytic feet no astrocytic feet
➢ continuous BM fenestrated BM
➢ tight junctions intercellular gaps
➢ no pinocytosis pinocytosis
GBM Increased perfusion due to angiogenesis in a

glioblastoma multiforme
• Center of Abnl Density/Intensity
➢ variegated necrosis Figure 5-10-27
• ENHANCING RIM
➢ hypercellular, fleshy neoplasm
➢ greatest neovascularity
• Corona of Abnl Density/Intensity
➢ “edematous” white matter
➢ areas of microscopic neoplastic infiltration
GBM - Glioblastoma
Glioblastoma multiforme with pseudopalisading
Pseudopalisading Necrosis [Figure 5-10-27]
necrosis
Ring Lesion and Infiltration [Figure 5-10-28] Figure 5-10-28
Schematic of Glioblastoma
multiforme – there are neoplastic
cells infiltrating into the surrounding
white matter

1146 Neuroradiology
Glioblastoma Multiforme Figure 5-10-29
(Surrounding Zone of Infiltration)
• GBM arose from a preexisting low grade
➢ surrounding lower grade neoplasm
➢ may also transform over time
• GBM arose de novo
➢ sends cells to invade the brain
Terrorist Cells Infiltrate Brain
GBM - Multifocal [Figure 5-10-29]
DWI of Glioblastoma (Gr 4)

Multifocal glioblastoma multiforme. Microscopic
Ring Enhancing Mass
infiltration does not always produce macrosopic
changes that are visible on CT, MR, or even at
Ring Lesion Differences [Figure 5-10-30] gross pathology
Glioblastoma Multiforme vs. Abscess

(toxoplasmosis). The abscess rim is thinner,
without a shaggy inner margin Glioblastoma Multiforme vs. Abscess
(toxoplasmosis). The viscous pus and white cell
DWI: Necrosis vs. PUS [Figure 5-10-31] infiltrate in the abscess causes restricted diffusion
– bright on DWI
Glioblastomas: Growth/Spread
Figure 5-10-32
Glioblastoma: Ependymal spread
GBM [Figure 5-10-32]
GBM – Thicker on Surface
Two different patients with GBM – the tumor

flourishes when it reaches the rich vascularity of
the cerebral cortex
Neuroradiology 1145
X-Ray Perfusion Imaging [Figure 5-10-33] Figure 5-10-33
MR Perfusion Imaging [Figure 5-10-34]
GBM with increased rCBV
New Tools for Grading and Staging

• Radiology
➢ Perfusion Imaging rCBV and rCBF
➢ Diffusion Imaging, ADC and DTI
➢ Spectroscopy
➢ PET/SPECT
➢ Monoclonal Ab. Glioblastoma multiforme. The early draining veins
• Pathology reflect increased perfusion and a shortened mean
➢ Labeling Index transit time (MTT)
➢ Chromosome Analysis
➢ Histochemical Figure 5-10-34
➢ Electron Microscopy
DTI and Tumor Imaging
Astrocytes Track Along WM
Two Port Radiotherapy

• Bad News
➢ Can’t define full extent of tumor by any current
test
• Good News Glioblastoma multiforme
➢ 90% of tumor recurrence within 2cm of
enhancing rim Figure 5-10-35
Glioblastoma Multiforme [Figure 5-10-35]
Define the Problem

• Some Low Grade Enhance*
• Some Low Grade => GBM
• Some Low Grade Do Not*
• *These are the Circumscribed Astrocytomas
• The others are the Diffuse Astrocytomas
WHO Astrocytoma Summary
Butterfly glioma – Glioblastoma multiforme

References
1. Kadri H,Mawla AA, Murad L: Incidence of childhood brain tumors in Syria (1993-2002) Pediatric Neurosurgery
2005; 41:173-177
2. Levin VA, Leibel SA, Gutin PH. Neoplasms of the central nervous system.
In: DeVita VT Jr, Hellman S, Rosenberg SA, eds. Cancer: Principles & Practice of Oncology. Vol 2. 5th ed.
Philadelphia, Pa: Lippincott-Raven Publishers; 1997:2022-2082.
3. Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst 1998; 14:426-439.
4. Pobereskin LH, Chadduck JB: Incidence of brain tumours in two English counties: a population based study. J
Neurol Neurosurg Psychiatry 2000; 69: 464-471.

1148 Neuroradiology
Non-Astrocytic Gliomas
Figure 5-11-1
PRIMARY NEOPLASMS – Neuroectodermal
• Neuroectoderm –
➢ Embryologic Neural Tube
➢ “Neuroepithelial”
• Broad Categories
➢ Glial Tumors (GLIOMAS)
➢ Embryonal/Immature (P.N.E.T.’s)
➢ Neuronal (Neurocytoma)
➢ Mixed (Ganglioglioma)
Neuroectodermal Tumors
• Astrocytoma
• Circumscribed Ependymoma – Gross Axial section
• Diffuse
• Ependymoma Figure 5-11-2
• Choroid Plexus Tumors
• Oligodendroglioma
• Atypical Rhabdoid Tumor
• Ganglioglioma
• Central Neurocytoma
• Lhermitte-Duclos
• Dysembryoplastic Neuroepithelial Tumor
EPENDYMOMAS – Brain and Cord

• Cell of Origin: Ependyma
➢ Lining of ventricles
➢ Central canal/filum terminale
➢ “rests” in parenchyma
• Subtypes:
➢ Anaplastic/Malignant
➢ Immature (Ependymoblastoma)
➢ Myxopapillary (cauda equina)
EPENDYMOMA
• WHO Classification Ependymoma – heterogeneous
➢ Ependymoma (WHO grade II) central mass
❖ Variants: cellular, papillary, clear cell, tanycytic, mixed
➢ Anaplastic ependymoma (WHO gr III) Figure 5-11-3
➢ Myxopapillary ependymoma
➢ Subependymoma
EPENDYMOMAS – Demographics
• 5%–6% of All Intracranial
• 70% occur in Males
• 70% arise in the Fourth Ventricle
• 70% present in Childhood
• 70% of All Intramedullary
• 5 year survival – 50%
EPENDYMOMAS – Gross Pathology

[Figures 5-11-1 to 5-11-5]
• Soft Intracavitary Mass Ependymoma – Enhancing and
➢ “cast of ventricle” heterogeneous, small “cysts” and calcified
➢ extrude out foramina chunks
➢ invade floor of 4th (pons/medulla)
Neuroradiology 1147
1149 Non-Astrocytic Gliomas
• Heterogeneous Figure 5-11-4
➢ small/large cystic areas
➢ calcification (often chunks)
• Spinal Cord – sharply circumscribed
Figure 5-11-5
Ependymoma – Tumor extends into lateral recess

of 4th ventricle
Figure 5-11-6
Ependymoma – Tumor extends into
vallecula of cisterna magna
Schematic Ependymoma [Figure 5-11-6]
Ependymoma [Figure 5-11-7]
Ependymoma CPA Cistern [Figure 5-11-8]
Figure 5-11-7
Schematic of Ependymoma filling lumen of fourth

ventricle. Copyright 2004
Figure 5-11-8
Ependymoma in the cerebellopontine angle cistern
Ependymoma filling lumen of fourth

ventricle
Non-Astrocytic Gliomas 1148

1150 Neuroradiology
Extra-ventricular Ependymoma Figure 5-11-9
[Figures 5-11-9 and 5-11-10]
• More common in children
• More common in cerebral hemisphere
• Arise from *rests* of Ependymal Cells
➢ Not infiltrating
➢ Not communicating with CSF
• Often partially cystic
CHOROID PLEXUS NEOPLASMS –

Introduction
• Normal Choroid Plexus (CP)
➢ Makes majority of CSF Extra-ventricular Ependymoma – Note fluid-fluid
➢ Forms papillary fronds: level formed by contrast layering within the cyst
❖ Vascular core
❖ Ependyma is modified into Figure 5-11-10
CHOROID EPITHELIUM
• Neoplasms:
➢ CP Papilloma (benign) WHO Grade 1
➢ CP Carcinoma (malignant) WHO Grade 3
CHOROID PLEXUS TUMORS

• Choroid plexus papilloma (WHO grade I)
• Choroid plexus carcinoma (WHO grade III)
CHOROID PLEXUS NEOPLASMS –

Demographics
• Tumor of Childhood:
➢ Uncommon (<1% of CNS)
➢ Intrauterine/congenital
➢ 40% < 1 year old
➢ 86% < 5 years
• Location:
➢ TRIGONE in children
➢ 4th vent. In adults
➢ Less often 3rd and CPA
CHOROID PLEXUS NEOPLASMS Extra-ventricular Ependymoma

Gross Pathology
[Figures 5-11-11 to 5-11-13]
• Lobulated intraventricular mass with papillary fronds
• Secondary Effects:
➢ Ventricular and SAS Enlargement
➢ Spontaneous Hemorrhage
➢ CSF Seeding
➢ Parenchymal Invasion
Choroid plexus papilloma showing Photomicrograph of CPP, showing

innumerable papillae choroid epithelium and vascular core
Neuroradiology 1149
Choroid plexus papilloma showing innumerable

papillae
Choroid Plexus Papilloma Choroid plexus papilloma – Note pattern of

bilateral hydrocephalus
CSF Homeostasis [Figure 5-11-15]
Figure 5-11-15
CSF Homeostasis
Normal Ventricular System: Lateral Ventricles
All Ventricles Enlarged?

• Over Production vs. Under Resorption
Figure 5-11-16
Congenital CPP
Choroid plexus papilloma [Figure 5-11-16]
OLIGODENDROGLIOMA
• Cell of origin
➢ Oligodendrocyte
➢ Makes central myelin
➢ 51% - 90% oligos, remainder astrocytes
• 1%–8% of ALL CNS primary
• Adults > Children (8:1)
• Age peak 35 – 45 yrs)
• Supratentorial – 85%
• Slow growth, Long Hx (10 years)
➢ Prognosis better with 1p and 19q mutations
Choroid plexus papilloma – Note

pattern of bilateral hydrocephalus

1152 Neuroradiology
OLIGODENDROGLIOMA Figure 5-11-17
• Gr 1 - Rare
• Gr 2 - Conventional oligodendroglioma
• Gr 3 - Anaplastic oligo:
➢ Hypercellularity, atypia, mitoses, endothelial
proliferation, necrosis
• Gr 4 - GBM-like (rare)
➢ Not biologically equivalent to Gr 4 fibrillary
astrocytoma
OLIGODENDROGLIOMA – Gross Pathology

• Arises in White Matter
• Grow toward cortex!
• Unencapsulated
• Not as infiltrating as astro.
• Heterogeneous
➢ myxoid areas (“cystic”)
➢ hemorrhage
• C A L C I F I C A T I O. N !
OLIGODENDROGLIOMA – Radiology
• Heterogeneous Hemispheric Mass
➢ Ca++, Cysts – “myxoid change”, Blood products Oligodendroglioma. CT shows very dense
• Extend to Cortex and infiltrate GM calcifications, highly suggestive and characteristic
➢ Gyriform or dot-dash Ca++ of oligodendroglioma
➢ Scalloped erosion inner table
• 2/3 will enhance Figure 5-11-18
➢ +/– anaplasia
• MR +/- special pulse sequences for Ca++ detection
• MR Spectroscopy?
➢ Potential for tumor grade, but not subtype or
genetics
MR - What is it?
Oligodendroglioma [Figure 5-11-17]

• CT Shows DENSE Ca++
Oligo-astrocytoma
• Nothing Specific … Looks like Diffuse Astrocytoma
Oligodendroglioma – Heterogeneous peripheral
Oligodendroglioma [Figure 5-11-18] mass that involves the cortex with thick curvilinear
• CT Shows DENSE Ca++ calcifications
Chickenwire Vascularity [Figure 5-11-19]
Figure 5-11-19
Fried Egg: round dark nucleus

surrounded by a clear halo – this is an
artifact of fixation
Chicken-wire: The capillary vessels
form a "chicken wire“ pattern around
nests of cells
Neuroradiology 1151
Oligodendroglioma Figure 5-11-20
• Combined 1p/19q loss
➢ Associated with prolonged survival
➢ Response to PVC (procarbazine, vincristine
CCNU® [Lomustine]) chemotherapy
➢ 50% volume decrease in 100%
➢ Median survival 10 yrs vs 2 yrs
❖ 95% 5 yr survival
➢ Most powerful predictor on multivariate analysis
Cairncross et al. J NCI 1998;90:1473
Old Elephants Age Gracefully External granular cell layer – normal

Oligo Epend Astro GBM in fetus and infants (up to 12 months
• 90% 50% 25% 15% (Incidence of Ca++) old)
Oligodendroglioma
MEDULLOBLASTOMA [Figure 5-11-20]

• Cell of origin:
➢ “medulloblast” – NOT!
• Bi-potential embryologic cells:
➢ Migrate from 4th to form CRBLL
➢ Glial and neuronal differentiation
➢ External Granular Cells (fetus)
➢ Internal Granular Layer (mature)
• Primitive Neuroectodermal Tumor - PNET
MEDULLOBLASTOMA – Demographics
• 1st or 2nd most common cerebellar neoplasm in children
➢ 1/5 – 1/3 of ALL pediatric CNS
➢ M:F 1.1 – 2:1
• May be congenital (present at birth up to 60 days)
➢ most (1/2) < 15 yrs.
➢ however, 1/3 present from 15–35 yrs.
• 5 year survival >> 50% => approached 75%-85%
• Primitive Neuroectodermal Tumor
Pediatric Posterior Fossa

• 454 posterior fossa patients
• All under the age of 18
• 402 tumors:
➢ 37.1% Cerebellar astrocytoma (149)
➢ 34.6% Medulloblastoma (PNET) (139)
➢ 11.4% Brain stem astrocytoma (46)
➢ 7% Ependymoma (28)
➢ 9.9% "other" (40)
Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst
1998; 14:426-439.
Childhood CNS Tumor Demographics

• 367 Syrian children, collected from 1993-2002
➢ Supratentorial - 47%
➢ Infratentorial - 53%
➢ Male 52%:48% Female
➢ Overall Incidence:
❖ Medulloblastoma 27%
❖ Astrocytoma 26%
❖ Craniopharyngioma 14%
➢ Posterior Fossa Only
❖ Medullo (PNET) 54%

1154 Neuroradiology
❖ Astrocytoma 23%
❖ Ependymoma 17%
Kadri H,Mawla AA, Murad L: Incidence of childhood brain tumors in Syria (1993-
2002) Pediatric Neurosurgery 2005; 41:173-177 Figure 5-11-21
MEDULLOBLASTOMA – Gross Pathology
[Figures 5-11-21 and 5-11-22]
• Arise from:
➢ post. (inf.) Medullary Vellum
➢ Vermis (midline cerebellum)
• Morphology:
➢ expansile, spherical, Unencapsulated
➢ post. 4th ventricle
➢ residual ANT. Crescent of CSF
➢ “HOMOGENEOUS”
❖ (Ca++. Cyst. Heme are UN-common)
Medulloblastoma (PNET) – Rounded
Figure 5-11-22 mass in the central posterior fossa
Figure 5-11-23
Medulloblastoma – a small, round,

blue-cell tumor
RADIATION CHEMOTHERAPY
• Dividing Cells
• Neovascularity
• Pharmaceuticals
➢ Tested against murine leukemia
➢ Small round blue cell tumor
➢ Cis-platinum
❖ Dividing cells
❖ Electrical field
❖ Platinum electrodes
MEDULLOBLASTOMA – Micro Pathology

• Small Round “BLUE CELL” Tumor
➢ Immature, high Nuclear:Cytoplasm
➢ Both Neuronal and Glial features (occasional
astrocytic differentiation)
➢ Form “Rosettes” (Homer-Wright) cells arranged Medulloblastoma (PNET) – Rounded mass in the
in a circle surround core with linear fibrils central posterior fossa
➢ Densely cellular
➢ Necrosis/Hemorrhage are not rare
MEDULLOBLASTOMA – Radiology [Figure 5-11-23]

• Post. Fossa, Behind/in 4th vent.
• “HOMOGENEOUS” (he’s lying!)
➢ grossly uniform,
❖ but, finely heterogeneous
➢ hyperdense on CT (w/o Ca++)
➢ hypo-/isointense to GM on MR (cellularity and high N:C ratio)
➢ (Ca++ and “cysts” < 15%)
• Enhance with contrast
Neuroradiology 1153
Fourth Ventricle - Schematic [Figure 5-11-24] Figure 5-11-24
Medulloblastoma (PNET) [Figures 5-11-25 and 5-11-26]
Figure 5-11-25
Schematic of posterior fossa masses. Can you

Medulloblastoma (PNET) – Rounded really determine where the mass originated when
mass arising in the cerebellum – not it is more than three centimeters in diameter?
from the 4th ventricle roof Copyright 2004
Figure 5-11-26
Medulloblastoma
[Figure 5-11-27]
• ‘Homogeneous’
➢ finely irregular
• Cyst and Hemorrhage are uncommon
<10%
➢ up to 75%
➢ densely cellular
➢ sm. Round blue-cells
• Center is behind 4th vent Medulloblastoma (PNET) – Rounded mass in the central
• Rounder not angular posterior fossa
Ependymoma - Schematic Figure 5-11-27
POSTERIOR FOSSA [Figure 5-11-28]

• INCIDENCE/LOCATION:
➢ Medulloblastoma (PNET) (1/4 - 1/3)
❖ Post. To IVth
➢ Brainstem glioma (1/6)
❖ Ant. To IVth
➢ Ependymoma (1/6)
❖ Inside IVth
➢ Pilocytic Astrocytoma (1/4 - 1/3)
❖ Lat. And/or post. IVth
❖ these are often a cyst w / nodule
Medulloblastoma
Medulloblastoma (PNET) [Figure 5-11-29] Figure 5-11-28
“Zuckerguss” or Sugar Icing (CSF dissemination)
CSF DISSEMINATION
• Neuroectodermal:
➢ PNET (medulloblastoma)
➢ GBM (reaches ventricle or pia)
➢ Ependymoma
➢ Oligodendroglioma (micro curiosity - no Sx)
➢ CPP and CPC
Medulloblastoma (PNET) – Rounded
mass in the central posterior fossa
1156 Neuroradiology
• Non-glial: Figure 5-11-29
➢ Germinoma
➢ Lymphoma (usually secondary)
➢ Leukemia
➢ Carcinomatous Meningitis
CSF Spread - Zuckerguss [Figure 5-11-30]
Lateral Medulloblastoma [Figure 5-11-31]
Medulloblastoma - Desmoplastic
CSF spread of Medulloblastoma (PNET)
• Lateral Hemispheric Location
• Older Patients More peripheral Figure 5-11-30
Cerebral Neuroblastoma [Figure 5-11-32]
Summary
• Ependymoma
➢ Intraventricular, soft, heterogeneous
• Choroid Plexus Papilloma
➢ Very young, Hydrocephalus
➢ Very Small Papillae and Lobulations
• Oligodendroglioma
➢ Superficial, skull remodeling
➢ Dense Calcification: dot-dash and linear
➢ Central posterior fossa
References
1. Kadri H,Mawla AA, Murad L: Incidence of childhood CSF spread of Medulloblastoma (PNET)
brain tumors in Syria (1993-2002. Pediatric Neurosurgery
2005; 41:173-177
Figure 5-11-31
2. Parizek J, et al: Posterior cranial fossa surgery in 454
children. Childs' Nerv Syst 1998; 14:426-439.
3. Jenkinson MD, Smith TS, Joyce K, Fildes D, du Plessis
DG, Warnke PC, Walker C MRS of oligodendroglial
tumors: correlation with histopathology and genetic
subtypes. Neurology. 2005 Jun 28;64(12):2085-9.
4. Cairncross JG, Ueki K, Zlatescu MC, et al. Specific
genetic predictors of chemotherapeutic response and
survival in patients with anaplastic oligodendrogliomas. J
Natl Cancer Inst 1998; 90:1473-1479.
Lateral Medulloblastoma – Desmoplastic variant
Figure 5-11-32
Cerebral Neuroblastoma – PNET
Neuroradiology 1155
Extraaxial Tumors:
Other Non-Glial Lesions
CHORDOMA [Figure 5-12-1]

• Rich Corinthian Leather vs. Physaliphorous Cells “Bubble” or Vacuolated
Figure 5-12-1
CHORDOMA: Normal Notochord
• Nucleus Pulposis of Intervertebral Disk
• Ecchordosis of Clivus
➢ Size/Shape like a grain of rice
➢ Dorsal to clivus
Notochord Embryology
Sclerotomes & Notochord [Figure 5-12-2]
Figure 5-12-2 Chordoma - Physaliphorous Cells

(Courtesy of Joe Parisi, M.D.)
Schematic of sclerotomes surrounding the

notochord. Two adjacent sclerotomes fuse into
one vertebral body. The notochord tissue is
extruded into the intervertebral disc, forming the
nucleus pulposis. Copyright 2003
Chordoma
Notochord: Chordoma & Thornwaldt [Figure 5-12-3]
CHORDOMA
• ORIGIN: Notochordal Rests Figure 5-12-3
• AGE: (30–60)
• LOCATION:
➢ Clivus 35%
➢ Spine 15% (esp. Cx)
➢ Sacrum 50%
CHORDOMA: Imaging
• Location: midline clivus
➢ Extend lateral, dorsal , ventral
• NCT: Bone destruction
➢ Heterogeneous Chordoma (left) and Thornwaldt cyst (right). The
➢ “Cysts”, Ca++ orange line is the embryologic location of the
• ECT: Heterogeneous notochord, the green triangle is the clivus
Extraaxial Tumors: Other Non-Glial Lesions 1156

1158 Neuroradiology
Chordoma - Imaging Figure 5-12-4
• Location
➢ Midline Clivus
❖ May extend lateral, dorsal , ventral
➢ Midline Sacrum
• Lobulated Heterogeneous Bulky Mass
• NO remodeling
• Variable Density/Signal
• Bone sequestra
• Dystrophic calcifications
Clivus is Missing [Figure 5-12-4]
Chordoma
Chordoma – Destruction of the clivus, the basilar
Eccentric Growth [Figure 5-12-5] and vertebral arteries are stretched over a large
bulky, hypovascular mass. Air and contrast
Figure 5-12-5 remain from an earlier myelogram
Figure 5-12-6
Chordoma with eccentric growth, there is a sharp

margin between the tumor and brain, with a thin
hypointense line – the dura
Chordoma [Figures 5-12-6 to 5-12-8]

Chordoma – destruction of the sacrum and bulky
soft-tissue mass. This patient presented with
Figure 5-12-7 symptoms of constipation and rectal fullness
Figure 5-12-8
Chordoma – destruction of the

sacrum and bulky soft-tissue mass.
There are devitalized fragments of
residual bone – sequestra – from the
sacrum
Chordoma – destruction of the
sacrum and bulky soft-tissue mass
Neuroradiology 1157
1159 Extraaxial Tumors: Other Non-Glial Lesions
T1 pre and post, T2 [Figure 5-12-9] Figure 5-12-9
Chordoma
Eccentric Mass [Figure 5-12-10]
Chondrosarcoma [Figure 5-12-11]
Figure 5-12-10
Sacrococcygeal chordoma
Figure 5-12-11
Chondrosarcoma – low grade.

Notice the dense mineralization and
the eccentric location of the mass
DERMOID/EPIDERMOID
• True Cysts
• Inclusion Cysts
• Lined by an Epithelium
Chondrosarcoma
TRUE CYSTS OF THE CNS
• Epidermoid
• Dermoid
• Colloid
• Rathke Cleft
• Ependymal
• Endodermal
TRUE CYSTS
• A fluid filled space, lined by an epithelium.
• Classified by the type of epithelial lining:
➢ Epidermoid
❖ squamous epithelium - ectoderm
➢ Dermoid
❖ squamous and dermal adnexa - ectoderm
➢ Colloid cyst
❖ ciliated cuboidal/columnar epithelium, mucus secreting cells - similar to
endoderm ?
❖ Craniopharyngioma (two types)
❖ Adamantinomatous - children, cystic, calcified
❖ Squamous and papillary - adult, solid
➢ Rathke Cleft cyst
❖ ciliated cuboidal/columnar epithelium - possibly endoderm ?
➢ Teratoma - a neoplasm of multipotential germ cells
NON-GLIAL MASSES
• Dermoid and Epidermoid
➢ MYTH OF THE MESODERM

1160 Neuroradiology
MYTH OF THE MESODERM Figure 5-12-12
• One germ cell layer = epidermoid
➢ Ectoderm
• Two germ cell layers = dermoid
➢ Ectoderm and Mesoderm
• Three germ cell layers = teratoma
➢ Ectoderm, Mesoderm, Endoderm
DERMOID/EPIDERMOID: Histology
• 1. Epidermoid – Squamous Epithelium - ONLY
• 2. Dermoid – Sq. Epi PLUS Dermal Appendages
(hair, sebaceous, sweat glands, etc.)
• 3. Teratoma – Complex tissues, 2 or more germ
layers
Epidermoid Inclusion Cyst. Note: the lesion is
(often mainly ectoderm, “benign cystic”)
nearly, but not exactly, like CSF in signal. The
sagittal image demonstrates internal layers of
EPIDERMOID [Figure 5-12-12] keratin
• AGE: 4 – 6TH Decade
• Location: Midline or lateral (CPA)
• Composition: Sq. epithelium, keratin
Figure 5-12-13
• Thin wall, no Ca++ or Vascularity
• NCT: Lipid to Brain
➢ Ca++/enhance. rare
• MRI: Hetero., CSF to Brain
➢ NOT bright on T1W
➢ **Fluid/Fluid Level RARE
Pearly Tumor
Epidermoid - Dry Keratin [Figures 5-12-13 and 5-12-14]

Epidermoid Inclusion Cyst - Dry Waxy
Keratin
Epidermoid [Figures 5-12-15 and 5-12-16]
Figure 5-12-14
Figure 5-12-15
Squamous epithelium and flaky or dry

keratin
Epidermoid Inclusion Cyst. On T2W images the
lesion is hyperintense – like CSF. However, the Figure 5-12-16
axial image on the right shows some internal
structure
Epidermoid – Gd+ T1W
Midline posterior fossa/fourth ventricle
Neuroradiology 1159
Epidermoid [Figures 5-12-17 and 5-12-18] Figure 5-12-18
Figure 5-12-17
T1W Gd FLAIR
Epidermoid Inclusion Cyst
Figure 5-12-19
Epidermoid Inclusion Cyst. Faint peripheral
enhancement from gliosis. Internal wisps from
layers of keratin, and marked hyperintensity on the
FLAIR image
DERMOID
• AGE: 3rd Decade
• Location: Midline
• Composition: Sq. epi. & appendages
• Thick wall, Ca++ & Vascularity
• NCT: Lipid to Brain, Fluid/Fluid
➢ Ca++/enhance. often
Dermoid with hair and sebaceous
• MRI: Hetero., Lipid to Brain
material
➢ Bright on T1W
➢ **Dysraphism, Sinus tract
Figure 5-12-20
Dermoid Inclusion Cyst
Dermoid [Figures 5-12-19 and 5-12-20]
Ruptured Dermoid
Dermoid - rupture [Figure 5-12-21]
Figure 5-12-21
Epidermal surface, but with
sebaceous glands, hair shafts and
follicles – making this a dermoid
inclusion cyst
Ruptured Dermoid Inclusion Cyst. Notice the

“hairball” at the lipid-CSF interface

1162 Neuroradiology
Body Soil
“As gross as it is, the average person excretes up to 50 grams of body soil per
day! This is because on a normal day we each use 1 liter of sweat, eliminate 1
billion dead skin cells, and run off 10 grams of sebum, otherwise known as body Figure 5-12-22
oil.”
Clorox Commercial, May 2005
(http://www.clorox.com/health_body_soil.html)
Intradiploic Epidermoid [Figure 5-12-22]
COLLOID CYST
• A benign mass, in a Malignant Location
COLLOID CYST [Figure 5-12-23]

• “Paraphyseal” cyst ependymal cyst, choroid cyst
• Congenital lesion Intradiploic Epidermoid Inclusion Cyst
• Cuboidal, low columnar epithelium
• Scant connective tissue Figure 5-12-23
• Foramen of Monro
COLLOID CYST [Figures 5-12-24 to 5-12-26]

• Location: Foramen of Monro
• CT: sharply demarcated
➢ hyperdense to hypodense
➢ < half enhance
• MR: sharply demarcated
➢ T1W – iso. to bright
➢ T2W – bright to dark Colloid cyst
➢ NOTE: Dark Cysts are too thick for
Stereotactic Aspiration Figure 5-12-25
Figure 5-12-24
Colloid Cyst – in the characteristic anterior 3rd Colloid cyst

ventricle location, and causing obstructive
hydrocephalus Figure 5-12-26
Aqueous Protein Solution
Colloid Cyst – Black Hole
Colloid Cyst: two different patients – one cyst is

markedly hyperdense, the other is hypodense.
Why? Variable protein and viscosity
Neuroradiology 1161
Neoplasms of the Meninges
Educational Objectives
• Meningioma is the most common non-glial primary tumor
• Meningioma is the most common extraaxial neoplasm
• Most meningiomas have “typical imaging”
➢ Hemispheric, homogeneous, broad based on the dura, hyperostosis,
hormonally sensitive
• Some meningiomas have “atypical imaging”
• Hemangiopericytoma is NOT a meningioma
Meningioma
• 75% are histologically typical
• 75% are radiologically typical
• Not the same 75%
➢ CT
➢ MR
➢ Angiography
• Atypical Imaging =/= Atypical Histology
• “The atypical appearance of a common lesion may be seen more often than
the classic appearance of an uncommon lesion.”
Primary Meningeal Neoplasms

• MENINGIOMA:
➢ Meningioma (typical and metaplastic
➢ Atypical Meningioma
➢ Anaplastic (Malignant) Meningioma
❖ Papillary Meningioma
• MESENCHYMAL (non-meningothelial)
• Primary MELANOCYTIC Lesions
• UNCERTAIN Origin
➢ Hemangiopericytoma (pericyte)
➢ Hemangioblastoma (mesenchyme)
Meningeal Tumors: WHO Grades

• 91% of Meningioma - Grade 1
➢ Includes most subtypes / metaplastic changes
➢ Transitional, fibroblastic, meningothelial
• 8.3% are ATYPICAL Meningioma - Grade 2
• HEMANGIOPERICYTOMA – Grade 2/3
• PAPILLARY Meningioma - Grade 3
• <1% are ANAPLASTIC Meningioma - Grade 3
Sandhyamani, Rao, Nair, Radhakrishan: Atypical Meningioma:
A Clinicopathological Analysis.Neurology India 2000; 48: 338-342
Meningioma – Benign Subtypes – WHO I

• SYNCYTIAL (Meningothelial)
• FIBROBLASTIC (Fibrous)
• TRANSITIONAL (Features of Both)
• PSAMMOMATOUS
• Microcystic (Humid), Secretory
• METAPLASTIC FEATURES
➢ Lipoblastic, Osteoblastic, Chondroblastic
➢ Myxoid, Xanthomatous, Melanotic
Neoplasms of the Meninges 1162

1164 Neuroradiology
Meningiomas – Cell of Origin
• Dural Fibroblast ? – No
• Arachnoid Cap Cell
➢ “Meningothelial cell”
➢ Arachnoid granulations
➢ Dural sinuses
❖ Sup. Sag.
❖ Sphenoparietal
Meningioma – Etiologic Factors

• Trauma
• Radiation
• Viruses
• Familial (Non-NF2) Meningioma
• Neurofibromatosis – Type - 2
➢ MISME Syndrome
Meningioma – Radiation
• Low Dose (<800 cGray)
➢ Immigrants to Israel (1940’s)
➢ Tinea Capitis (ringworm)
➢ Superficial radiation
➢ High Incidence of Meningioma
• High Dose (>2000 cGray = 2000 RADS)
➢ Used for Skull Base Tumors
➢ Pituitary Adenoma
Meningioma – Molecular Biology

• Postulate Tumor suppressor Gene
• Chromosome 22 – deletion in tumor cells
➢ both sporadic and inherited
➢ w/ or w/o NF-2
• Homozygous for TWO wild-type copies is normal
• Heterozygous for 22 develops neoplasm
➢ Because there is a subsequent loss of the OTHER wild-type gene
• Inherited (germline) deletion of 22
➢ W/Schwannoma = NF2
Meningiomas
• 1/7 to 1/4 of all Intracranial Primary
➢ ~ 6/ 100k / year
➢ small ones in ~ 1.4% of autopsies
• 1/4 – 1/3 of all Intraspinal Tumors
• Middle age (40–60) Your current Age + Ten Years
• Female > Male
➢ Cranial 2–4:1 Figure 5-13-1
➢ Spinal 4–8:1
➢ Progesterone receptors in 2/3
➢ Estrogen receptors less common
Morphology [Figure 5-13-1]

• Globose (spherical, hemispherical)
• En plaque (like a flat bread)
➢ Pancake
➢ Crepe
➢ Wonton wrapper Meningioma - parasagittal
➢ Tortilla
➢ Pita (Greece and Middle East)
➢ Naan (India)
➢ Injera (Ethiopia)
➢ Bolo de milho (Brazil)
Neuroradiology 1163
1165 Neoplasms of the Meninges
Incidental Meningioma [Figure 5-13-2] Figure 5-13-2
Not Incidental
En Plaque Meningioma [Figure 5-13-3]
Meningioma – Location
• Parasagittal 25%
• Convexity 20%
• Ant. Basal 40%
➢ Sphenoid 20% Small incidental meningioma of the
➢ Olfactory 10% tentorium cerebelli
➢ Suprasellar 10%
• Tentorial 10%
• Ventricular 5% Figure 5-13-3
Meningioma – CT Imaging [Figure 5-13-4]
• Non-Contrast
➢ Sharply Circumscribed
➢ Homogeneous
➢ Hyperdense (+/– Ca++)
➢ NOT from psammoma bodies !
➢ Broad Dural Surface
➢ Bone Changes (Hyperostosis)
• Enhanced CT
➢ Homogeneous Enhancement
Figure 5-13-4
En plaque meningioma, on the CT this blends into

the bone
Figure 5-13-5
Meningioma – with classic features of
hyperdensity and hyperostosis
Meningioma – CT Findings (193 pts)

• BENIGN Meningiomas:
➢ Homogeneous Enhancement 72%
➢ Heterogeneous Enhancement 23%
➢ Calcification 27%
➢ Hyperostosis 18%
➢ Midline Shift (large) 72%
➢ “Mushrooming” 0%
J. Neurosurg 71:665–672,1989
Psammomatous Meningioma
Psammomatous Meningioma [Figure 5-13-5]

1166 Neuroradiology
Spreading Vasogenic Edema Figure 5-13-6
Meningioma – Vasogenic Edema

• VASCULAR
➢ Parasitization of MCA, etc.
➢ Compression of cortical aa./vv.
• COMPRESSIVE TRAUMA
• SECRETORY EFFECT “Evil Humors”
• “TRANSCORTICAL FLOW”
➢ Close apposition of tumor to brain
➢ Thinned cortex
➢ +/- infiltration of brain
Meningioma. WHO Grade 1 – benign, yet with extensive
➢ Fluid gradient from meningioma into
vasogenic edema
brain
Meningioma and Edema [Figures 5-13-6 and 5-13-7] Figure 5-13-7
Edema and Prognosis

• Edema =/= Histology
• Edema =/= Size
• Edema =/= Vascularity
• Edema IS Related to Resectability
➢ Smaller “pseudocapsule”
➢ Surgical “cleavage plane”
➢ Tumor “sticks” to underlying brain
• Resectability IS Related to Prognosis
• Edema IS INDIRECTLY Related to Prognosis
Meningiomas – MR Imaging
• “Meningiomas are ISO-intense.” Bar graphs showing high frequency of
➢ Usually on T1W vasogenic edema with meningioma
➢ Vary pulse sequence to see
• EXTRA-axial Features
➢ Gray-matter buckling
➢ Pseudo-capsule of vessels
➢ Meningeal/dural “TAIL”
• GADOLINIUM ENHANCEMENT
Meningioma – Isointense to GM
[Figure 5-13-8]
Figure 5-13-9
Figure 5-13-8
Small peripheral round lesions, nearly isointense

to gray-matter on T1W MR
Meningioma - CT
Meningioma - Small peripheral round lesions, nearly
Meningioma w/Gd+ [Figure 5-13-9] isointense to gray-matter on T1W MR
Neuroradiology 1165
Meningioma [Figure 5-13-10] Figure 5-13-10
• Meningioma
• Tentorium or Dura
• IAC Normal
• Hemispherical
• Homogeneous
• Hyperostosis
Tentorial Meningioma [Figure 5-13-11]
Meningioma - Pseudocapsule
Meningioma – Pre and Post Gd Meningioma. Cerebellopontine angle cistern.

This mass is hemispheric, and the enhancement
MR Signal and Meningioma Types does not involve the IAC (internal auditory canal)
• Most are Isointense to GM (Courtesy of Bob Peyster, MD)
➢ On Both T1W & T2W
• Hyperintense to GM on T1W
➢ “Lipoblastic” (Fatty) Meningioma Figure 5-13-11
➢ Hemorrhage into Meningioma
• Hypointense on T2W
➢ Fibroblastic
➢ Transitional
• Hyperintense on T2W
➢ Meningothelial
➢ “Angioblastic”
➢ Microcystic (“Humid”)
❖ Some Good, Some “bad”
Meningioma - Pseudocapsule [Figure 5-13-12]
Meningioma – Dural Tail [Figure 5-13-13] Tentorial Meningioma
Figure 5-13-13
Figure 5-13-12
Meningioma – Pseudocapsule of CSF and

vessels; and internal serpentine hypointensities
TMeningioma – Pseudocapsule of CSF and

vessels; and internal serpentine hypointensities
dural tail

1168 Neuroradiology
DURAL TAIL
• Curvilinear enhancement
• “dural flair”
• First reported w/meningioma
• First reported to be neoplastic invasion
• What is it REALLY?
➢ Thickening of the dura
➢ Vasocongestion of the dura
➢ Edema of the dura
Dural tail: Histology
Dural Tail: Differential Dx Figure 5-13-14

• Extraaxial Lesions
➢ Meningioma
❖ Most common lesion w/dural tail
❖ Most Common Lesions Overall
➢ Schwannoma
➢ Hemangiopericytoma
➢ Sarcoidosis
➢ Gumma (syphilis)
• Intraaxial Lesions
➢ Pleomorphic Xanthoastrocytoma
➢ Superficial cerebral astrocytoma
➢ GBM (rare)
Cavernous Sinus Meningioma with extensive
Whorls of Spindle Cells dural enhancement along the tentorium
Cavernous Sinus Meningioma [Figure 5-13-14]
Meningioma: Named by region

• Tentorial / Pineal
• Clival
Dural Tail - Schwannoma
MENINGIOMA – *Imaging Features: CT vs. MR

CT MR
• Mass effect 88% 90%
• Extraaxial Location 42% 70%
• Broad Dural Attach. 74% 98%
• Typical. Dense./Intensity 92% 74%
• Hyperostosis 10% 14%
• Homogeneous 76% 76%
• Enhancement
(Homogeneous) 96%(78%) 96%(80%)
• Meningeal. Enhance (tail) 2% 50%
• “Capsule” 14% 68%
*Neuroradiology 1990;32:467–473
Meningioma - Transdural
Other Locations for Meningioma

• Intraventricular
• Orbit
➢ Intraconal
➢ Periorbital
• Nasal Cavity
Neuroradiology 1167
Intraventricular: ~ 5% [Figures 5-13-15 and 5-13-16] Figure 5-13-15
• Usually Adult
• F>M
• Usually Lateral Ventricle
• Usually Trigone/Atrium
• ALWAYS attached to Choroid Plexus
• Vascular pedicle from choroid
Optic Nerve Meningioma
Meningioma – Angiography - Supply

• External Carotid 85%
➢ Some have dual supply
• Internal Carotid 63%
• Tumor Blush 95%
Meningioma – Angiography Figure 5-13-16

Transit Time [Figure 5-13-17]
• Blush or Stain
➢ early arterial
➢ prominent in VENOUS phase
➢ capillaries/sm. arterioles
➢ (too small to see individually)
• Venous Filling
➢ characteristic if delayed
➢ may fill with/ before NI. veins
Normal Choroid Plexus: Nests of
Spoke Wheel Vessels arachnoid cap cells
Meningioma – Angiography – Transit Time

[Figures 5-13-18 and 5-13-19]
• Venous Filling (Stattin, 1996)
Figure 5-13-17
➢ 170 Meningiomas
➢ delayed in 136 (80%)
➢ with Nl. vv. In 10 (6%)
➢ earlier in 24 (14%)
➢ (8 in early arterial phase)
• Leeds & Taveras (1969)
➢ EDV in 6/36 (16%)
Figure 5-13-18
Meningioma – Supplied by branches of the ECA,

showing classic spoke-wheel vascularity
Meningioma – Venous phase,

showing persistent and dense tumor
blush

1170 Neuroradiology
Meningioma – Pre-Op Embolization Figure 5-13-19
• Gd Pre Embo
• Gd Post Embo
AJNR Editorial - September 2003; 24: 1499 - 1500
Meningioma – Angiography – Tumor Blush

• May come early
• Usually very DENSE
• Stays way too long
• (Persistent!)
• Derek Harwood-Nash:The “In-Law Effect”
Meningioma – Effect on Skull

• Hyperostosis (15%-25%)
➢ w or w/o micro invasion
• Pressure Erosion
➢ Periosteal remodeling
• Bone Destruction
➢ microscopic invasion
HYPEROSTOSIS IN MENINGIOMAS MR Perfusion study, showing delayed washout

[Figures 5-13-20 to 5-13-22] from meningioma.
(Courtesy of Dra. Perla Salgado, Mexico City,
Figure 5-13-20 Mexico)
Figure 5-13-21
Variable patterns of hyperostosis from

meningioma
Meningioma Hyperostosis
• Does NOT mean invasion of bone Variable patterns of hyperostosis from
• Implies CHRONICITY meningioma
➢ and benign behavior
• Skull Base
➢ Invasion via HAVERSIAN CANALS
Figure 5-13-22
Hyperostosis from meningioma
Neuroradiology 1169
Meningioma – Atypical Imaging Figure 5-13-23
(Yet, typical Histology) [Figure 5-13-23]
• Focal Lucency Outside (arachnoid cyst)
• Focal Lucency Inside (necrosis, cyst)
• Hypodensity (“humid”, lipoblastic)
• Focal Hyperdensity (metaplasia, hemorrhage)
• Heterogeneous
• Hyperintensity on T1W
• Hyperintensity on T2W
Fatty Metaplasia
Fatty (Lipoblastic) Meningioma
Fatty Metaplasia [Figure 5-13-24] Meningioma with typical histology – yet aggressive
radiologic appearance
Meningioma – Cysts
• Inside of neoplasm Figure 5-13-24
➢ (rim enhancement)
• Between tumor and brain
➢ (”arachnoid cysts”)
• Inside Brain
➢ PIA separates tumor from brain
➢ ?? Results of chronic Edema
➢ Vacuolization of White Matter
Cyst and Mural Nodule
Meningioma [Figure 5-13-25]
Meningioma – Atypical Histology

• Atypical Meningioma ~ 5%–7% WHO Grade 2 Meningioma with Fatty Metaplasia
• Anaplastic Meningioma ~ 1%–3%
➢ ~ 0.2% / 100k per year
Figure 5-13-25
• Higher incidence of Recurrence
• Shorter time to Recurrence
• “Atypical Histology”
➢ necrosis
➢ excessive mitoses
➢ invasion into brain
“Malignant Meningioma”
• < 3% of all Meningioma
• Anaplastic (Malignant) Meningioma
• Papillary Meningioma
• “Benign” Metastasizing Meningioma
• Hemangio-Peri-Cytoma (HPC)
• Malignant Fibrous Histiocytoma (MFH)
Meningioma – One level shows typical
hemispheric shape, the next shows a peritumoral
“cyst”

1172 Neuroradiology
Hemangiopericytoma [Figure 5-13-26] Figure 5-13-26
• Syn: “angioblastic meningioma”
• Cell of Origin – perivascular pericyte of Zahn and/or
Zimmerman
• < 1% of primary CNS
• M 1.4:1 F
• Age – 40’s
• Dural based, bone destruction, lobulated
Hemangiopericytoma – (HPC)
• Narrow dural base (“Mushrooming”)
• No Hyperostosis
• No Calcification in tumor
• Lobulated (not hemispheric)
• Internal Signal Voids (on MRI)
➢ irregular and multiple
• Hypervascular on Angio
➢ irregular patterns
Meningioma – Radiologic Features – CT

Feature Benign / ”Malignant”
• Homogeneous
Enhancement 72% / 36%
• Heterogeneous
Enhancement 23% / 64%
• Hyperostosis 18% / 7%
• Calcification 27% / 0%
• “Mushrooming” 0% / 57%
➢ Narrow attachment and larger “cap”
➢ invaginating into brain
Hemangiopericytoma
Hemangiopericytoma vs. Meningioma
Hemangiopericytoma Meningioma
Narrow Base Broad Base
Lobulated Hemispheric
Heterogeneous Homogeneous
Bone Destruction No Ca++ Hyperostosis Psammomatous Ca++
Irregular Vessels Spoke Wheel Vessels
Meningioma
• The 4H+ Tumor
➢ homogeneous
➢ hyperdense
➢ homogeneous enhancement
➢ hemispheric shape
➢ hyperostosis
➢ hormonally modulated
Meningioma
• 75% are histologically typical
• 75% are radiologically typical
• Not the same 75%
➢ CT
➢ MR
➢ Angiography
• Atypical Imaging =/= Atypical Histology
Neuroradiology 1171
References
1. Ahmadi J, Hinton DR, Segall HD, Couldwell WT. Surgical implications of magnetic resonance-enhanced dura.
Neurosurgery. 1994 Sep;35(3):370-7;discussion 377.
2. Aoki S, Sasaki Y, Machida T, Tanioka H. Contrast-enhanced MR images in patients with meningioma: importance
of enhancement of the dura adjacent to the tumor. AJNR Am J Neuroradiol. 1990 Sep-Oct;11(5):935-8.
3. Asari S, Yabuno N, Ohmoto T. Magnetic resonance characteristics of meningiomas arising from the falcotentorial
junction. Comput Med Imaging Graph. 1994 May-Jun;18(3):181-5.
4. Berger MS. Perfusion MR and the evaluation of meningiomas: is it important surgically? AJNR Am J Neuroradiol
2003; 24:1499-1500. (1)
5. Goldsher D, Litt AW, Pinto RS, Bannon KR, Kricheff II. Dural "tail" associated with meningiomas on Gd-DTPA-
enhanced MR images: characteristics, differential diagnostic value, and possible implications for treatment.
Radiology. 1990 Aug;176(2):447-50.
6. Helie O, Soulie D, Sarrazin JL, Derosier C, Cordoliani YS, Cosnard G. [Magnetic resonance imaging and
meningiomas of the posterior cerebral fossa. 31 cases] J Neuroradiol. 1995 Dec;22(4):252-70. French.
7. Hutzelmann A, Palmie S, Buhl R, Freund M, Heller M. Dural invasion of meningiomas adjacent to the tumor
margin on Gd-DTPA-enhanced MR images: histopathologic correlation. Eur Radiol. 1998;8(5):746-8.
8. Hutzelmann A, Palmie S, Freund M, Buhl R, Heller M. [Dura thickening adjacent to intracranial, para-dural space-
occupying lesions in MRI. Histologic correlation] Aktuelle Radiol. 1997 Nov;7(6):305-8. German.
9. Hutzelmann A, Palmie S, Zimmer C, Benz T, Leweke F, Freund M. [The meningeal sign: a new appraisal] Rofo.
1996 Apr;164(4):314-7. German.
10. Ildan F, Tuna M, Gocer AP, Boyar B, Bagdatoglu H, Sen O, Haciyakupoglu S, Burgut HR. Correlation of the
relationships of brain-tumor interfaces, magnetic resonance imaging, and angiographic findings to predict cleavage
of meningiomas. J Neurosurg. 1999 Sep;91(3):384-90.
11. Kawahara Y, Niiro M, Yokoyama S, Kuratsu J. Dural congestion accompanying meningioma invasion into vessels:
the dural tail sign. Neuroradiology. 2001 Jun;43(6):462-5.
12. Maiuri F et al: Intracranial meningiomas: correlations between MR imaging and histology. Eur J Radiol. 1999; 31:
69-75
13. Nagele T, Petersen D, Klose U, Grodd W, Opitz H, Voigt K. The "dural tail" adjacent to meningiomas studied by
dynamic contrast-enhanced MRI: a comparison with histopathology. Neuroradiology. 1994 May;36(4):303-7.
14. Nakasu S, Nakasu Y, Matsumura K, Matsuda M, Handa J. Interface between the meningioma and the brain on
magnetic resonance imaging. Surg Neurol. 1990 Feb;33(2):105-16.
15. Nakau H, Miyazawa T, Tamai S, Tsuchiya K, Shima K, Shirotani T, Chigasaki H. Pathologic significance of
meningeal enhancement ("flare sign") of meningiomas on MRI. Surg Neurol. 1997 Dec;48(6):584-90; discussion
590-1.
16. Quekel LG, Versteege CW. The "dural tail sign" in MRI of spinal meningiomas. J Comput Assist Tomogr. 1995
Nov-Dec;19(6):890-2.
17. Sakai K, Tada T, Fukasaku K, Kyoshima K, Kobayashi S. Histological examination of the gadolinium-enhanced
dura mater around meningiomas on magnetic resonance imaging. Neurol Med Chir (Tokyo). 1993 Jul;33(7):429-
33.
18. Sandhyamani, Rao, Nair, Radhakrishan: Atypical Meningioma: A Clinicopathological Analysis.Neurology India
2000; 48: 338-342
19. Sato M, Matsumoto M, Kodama N. Meningeal enhancement surrounding meningiomas on Gd-DTPA MRI.
Fukushima J Med Sci. 1998 Jun;44(1):1-11.
20. Sekiya T, Manabe H, Iwabuchi T, Narita T. [The dura mater adjacent to the attachment of meningiomas: its
enhanced MR imaging and histological findings] No Shinkei Geka. 1992 Oct;20(10):1063-8. Japanese.
21. Takeguchi T, Miki H, Shimizu T, Kikuchi K, Mochizuki T, Ohue S, Ohnishi T. The dural tail of intracranial
meningiomas on fluid-attenuated inversion-recovery images. Neuroradiology. 2004 Feb;46(2):130-5. Epub 2004
Jan 28.
22. Wilms G, Lammens M, Marchal G, Van Calenbergh F, Plets C, Van Fraeyenhoven L, Baert AL. Thickening of dura
surrounding meningiomas: MR features. J Comput Assist Tomogr. 1989 Sep-Oct;13(5):763-8.
23. Yamaguchi N, Kawase T, Sagoh M, Ohira T, Shiga H, Toya S. Prediction of consistency of meningiomas with
preoperative magnetic resonance imaging. Surg Neurol. 1997 Dec;48(6):579-83.
Neoplasms of the Meninges 1174 Neuroradiology

“PINEALOMAS” and other
Pineal Region Masses
Pineal Gland Introduction Figure 5-14-1

• “Seat of the Soul”
• Daily (Diurnal) Biorhythms
• Life-Cycles (Puberty, Migration)
• Responds to Light/Dark
➢ Melatonin levels
➢ Accessory Optic Pathway
❖ Retinohypothalamic tract, RAS, Sympathetics
• Third Eye
➢ Phylogenetically The normal pineal gland is ~10-14
➢ Developmentally mm in maximum sagittal diameter
➢ Embryologically
Pineal – Third Eye

• Iguana Figure 5-14-2
➢ Third Eye
➢ Photoreceptor
❖ Transparent scale
❖ Hole in skull
➢ Radiometer for sunlight
• In lower vertebrates it may have a lens and a retina
Biological Clock
• Day-Night Diurnal Rhythms
• Pineal Melatonin Suppresses GnRH
➢ Longer daylight decreases melatonin and leads
to increased Gonadotropin Releasing Hormone
➢ GnRH => LH and FSH
➢ Increased sexual drive and activity in the Spring
… and I thought it was the Pollen … Pineal gland and surrounding region: Third
ventricle, quadrigeminal plate and cistern, bilateral
Normal Pineal [Figures 5-14-1 and 5-14-2] thalami
Normal Pineal Calcification

Figure 5-14-3
• 725 Normal Patients
• “Youngest was 6,5”
• 8%–11% from 8 to 14 yrs.
• 30% for 15 y.o.
• 39%–40% from 17 to 29 yrs.
Radiology 142:659–662, 1982
Pineal Region – Normal Anatomy [Figure 5-14-3]
Pineal Region Mass: Symptoms and Signs

• Parinaud Syndrome
➢ NOT Paranoid
• Precocious Puberty
• Headache, Nausea, Vomiting
➢ Non-specific mass effect
➢ +ICP Pineal Region – Normal Anatomy:
The internal cerebral veins are
important landmarks
Neuroradiology 1173
1175 “PINEALOMAS” and, other Pineal Region Masses
Parinaud Syndrome: Aqueduct/Tectal Syndrome
• Failure of conjugate vertical eye movement
➢ Upward >> downward
• Mydriasis, fixed pupils
• Failed ocular convergence
➢ Lateral midbrain tegmentum
• Blepharospasm
➢ Eyelid spasm
Pineal Region: Differential

• Germ Cell Neoplasms
• Pineal Cell Neoplasms
• Gliomas
• Non-neuroglial Masses
Germ Cell Tumors: WHO Classification

• Germinoma
• Embryonal Carcinoma
• Yolk Sac
➢ Endodermal sinus
• Choriocarcinoma
• Teratoma
➢ Immature, Mature, Malignant
• Mixed Germ Cell
Intracranial Germ Cell Tumors

• Usually primary in the CNS
➢ Arise from “Germ Cell Rests”
➢ Pineal/Quadrigeminal and suprasellar cistern
• Exceptional caese metastatic to CNS
➢ Usual testicular drainage to renal hilus
➢ Para-artic nodes
➢ Rare cases of testicular seminoma CNS mets
➢ Lance Armstrong had mixed Chorio/embryonal
Pineal Region Mass

• 369 pts. Hoffman series
Tumor %
GERM CELL 59
Germinoma 59
Teratoma – Malignant 11
Teratoma – Benign 2
Yolk Sac 2
Choriocarcinoma 2
Embryonal CA 1
Mixed Germ Cell 1
PINEAL REGION MASS

• 369 pts. Hoffman
➢ EPIDERMOID 1+
➢ TRUE PINEAL 14
❖ Pineoblastoma 12
❖ Pineocytoma 2
➢ OTHER NEOPLASMS 27
❖ Gliomas 26
❖ Non-glial (meningioma, etc)
“PINEALOMAS” and, other Pineal Region Masses 1174

1176 Neuroradiology
Dr. Hoffman’s Series Figure 5-14-4
• Dr Harold J Hoffman (1932 – 2004)
• Arguably the most famous pediatric neurosurgeon in
the world.
• Harold joined the Neurosurgical Staff at the Hospital
for Sick Children in 1964
• 1998, the Harold J Hoffman/Shoppers Drug Mart
Chair in Pediatric Neurosurgery was established at
the Hospital for Sick Children
Pineal Neoplasms: Demographics
Germ Cell Tumors-AFIP Series [Figure 5-14-4]

Pineal Region Germ Cell Tumors - AFIP Series
Basic Approach to Pineal Region
Figure 5-14-5
Intracranial Germinoma
• Synonyms: Pinealoma, Seminoma, Dysgerminoma,
Atypical Teratoma
• Cell of Origin: Germ Cell Rests, 2-cells pattern
• Incidence: 1%–2% of ALL Cranial neoplasms
➢ 2%–4% of Child.
➢ 9%–15% in Japan
• Age: 5–35 (remember precocious puberty)
• Sex: 2–7M/F
Pineal Region Germinoma – Two cell pattern, one
• Location: 60–80% Pineal, 22% Suprasellar
cell resembles a lymphocyte
• Treatment: Bx, Radiation, ChemoTx
• Prognosis: >50% at 5yrs
Figure 5-14-6
➢ Radiosensitive and chemosensitive tumor
➢ Median survival ~19 yrs
Intracranial Germinoma
• Central:
➢ pineal (para-pineal)
➢ Suprasellar cistern
• Homogeneous Solid
• Isointense on T1W
• “Hormonally silent”
➢ no AFP/HCG but PLAP+
Germinoma Imaging [Figures 5-14-5 to 5-14-8]

Pineal region seminomas are usually hyperdense
• Sharply circumscribed, midline mass
on plain CT
• Surrounds/Engulfs Pineal Ca++
• Alternate locations
➢ thalamus, 3rd vent., suprasellar cistern
Figure 5-14-7
• NCT – Homogeneous Hyperdense
• ECT – Homogeneous Enhancement
• MR – Isointense to gray matter
• +/– CSF Spread, tumor Ca++
Surgical Planning
• Find the Internal Cerebral Veins and the VOG
• If Tumor is BELOW these veins
➢ Suboccipital Infratentorial Approach
• If Tumor is ABOVE these veins
➢ Interhemispheric Approach
➢ Sub Temporal Approach
➢ Various other techniques Pineal region germinoma
Neuroradiology 1175
Pineal Region Neoplasms Figure 5-14-8
• Germinoma:
➢ Iso on T1W
➢ slightly Hyper on T2W
• Choriocarcinoma:
➢ Hyper on T1W (blood)
• Dermoid, Teratoma:
➢ Hyper on T1W (lipid)
MR AJNR 11:557–565,1990
Pineal Region [Figures 5-14-9 and 5-14-10]

• Teratoma
➢ Sharply circumscribed Pineal region germinoma, extending
➢ Lobulated and Loculated below the tentorium
➢ HETEROGENEOUS
(mixture of lipid, soft-tissue, Ca++) Figure 5-14-10
➢ Enhancement of solid areas
Figure 5-14-9
Ruptured Pineal region teratoma. Note the

lipid/fluid levels in the frontal regions of both lateral
ventricles. The primary tumor is seen in the
midline pineal region
Pineal region teratoma. Note the

peripheral rim of T1-shortening from
lipid
Teratoma vs. Dermoid

• Teratoma is a Neoplasm
➢ From Multipotential Cells/Tissues
➢ “Included Twin” from embryo/fetus
➢ Ectoderm (Skin, Occ. Brain) Common
➢ Lipid (Mesodermal FAT or Sebaceous)
➢ Multiloculated, Lobulated
• Dermoid is an Inclusion Cyst
➢ Only Skin (Ectoderm)
➢ Water and/or Sebaceous Lipid
➢ Unilocular
Famous Quote
Aunt Voula:
“You family now, so I tell you a story. All my life ... I have this lump on the back of
my neck. When I reach the menopause, the lump get bigger. I go to the doctor,
and he performs a...bo-bobopsy. And inside the lump, he finds teeth, and a spinal
column.”

1178 Neuroradiology
Two Theories for Teratoma Figure 5-14-11
• Progressive differentiation from multi-potential (“omnipotential”)
Germ Cells – “primordial germ cells”
• Inclusion of a twin during early gestation - embryogenesis
Dermoid Inclusion Cyst [Figure 5-14-11]
Pineal Region Mass
Endodermal Sinus Tumor [Figure 5-14-12]
Figure 5-14-12
Dermoid Inclusion Cyst in the pineal

region. Histology only revealed
ectodermal elements
Non-seminomatous germ cell tumor – NOT
hyperdense on plain CT, but does engulf a central Figure 5-14-13
calcification
Germinoma
Germinoma - seeding [Figure 5-14-13]
CSF Dissemination
Pineal Neoplasms Laboratory Tests:

Serum and CSF
Neoplasm BHCG AFP PLAP
Germinoma -- -- inc.
Yolk-sac -- inc. --
Chorio Ca. inc. -- --
Germinoma – CSF seeding along the
Embryonal inc. inc. --
edge of the tentorium (arrows)
BHCG = Beta HCG
AFP = Alpha Feto Protein
PLAP = Placental Alkaline Phosphatase
Neuroradiology 1177
Pineal Parenchyma Figure 5-14-14
• Pineoblastoma (PNET)
➢ Young Patients (1st two decades)
➢ Tumor ITSELF Calcifies
➢ ”Exploded” Pineal Ca++
• Pineocytoma (Mature pineal cells)
➢ Young or Old
• Trilateral Retinoblastoma
➢ Inherited Rb (chromosome 13)
➢ 1/3 inherited but 2/3 heritable
➢ Look at ORBITS for signs of Tx
Pineal Parenchyma Mass [Figure 5-14-14]

• Exploded Pineal Calcifications
• Pineoblastoma
➢ Some hyperdense on CT
• Pineocytoma
➢ Isodense on CT Pineal Parenchyma Mass Schematic:
“exploded calcifications” from a mass
Figure 5-14-15 arising inside the pineal gland. This
pattern is seen in both pineal cysts
and neoplasms
Figure 5-14-16
Pineoblastoma – “exploded calcifications” around

the outside rim of the tumor
Pineoblastoma
Figure 5-14-17
Figure 5-14-18
Pineoblastoma with seeding along the edge of the

tentorium. Using this T1W MR alone, this mass is Pineoblastoma. Note how the lesion extends
indistinguishable from a pineal region germinoma below the tentorial hiatus into the posterior fossa

1180 Neuroradiology
Pineoblastoma [Figures 5-14-15 to 5-14-18] Figure 5-14-19
Pineal Cysts
• Autopsy
➢ ~ 5% of Adults
➢ < 2 mm in a Normal Size Gland
• MR Visible
➢ 2%-8% of Adults
➢ 2-7 mm common
➢ 14-25 mm cysts are not rare
➢ May expand the gland
➢ Ring enhancement should be smooth and thin 1-2 mm
• Why do they grow? – Unknown
Pineal Cyst Schematic for Vein of Galen

• Typical cysts: malformations: Straight sinus
➢ Round or Oval obstruction, sinus hypoplasia, AVM or
➢ T1 ~ WM dural fistula draining into the VOG
➢ T2 ~ CSF
➢ T2 Homogeneous Figure 5-14-20
➢ Rim Enhancement< 2mm
➢ No nodularity
• “These findings suggest that typical pineal cysts
may be followed up on a clinical basis alone rather
than on imaging.”
Pineal Region Mass: Other Lesions

• Glial - Astrocytoma
➢ Splenium Of Corpus Callosum
➢ Tectum Of Midbrain
➢ Thalamus
• Congenital
➢ Lipoma
➢ Inclusion Cyst (Epidermoid/Dermoid)
➢ Vein of Galen Malformation Vein of Galen Malformation
• Non-Glial - Meningioma
Vein Of Galen Malformation: Symptoms, Signs Figure 5-14-21

• Childhood – Large shunt
➢ High Output Failure
➢ Persistent Ductus
➢ Hydrocephalus
➢ Cranial Bruit/thrill
• Adult – Small shunt
➢ Asymptomatic
➢ Pineal Symptoms
Vein Of Galen Malformation: Types and

Causes
[Figures 5-14-19 and 5-14-21] Vein of Galen Malformation. Persistence of the
• Parenchymal Avm (Shunt) falcine vein
• Direct Fistulae To Vein
• Dural Fistula (Drains To Vein)
• Sinus Thrombosis (Fetal)
• Hypoplastic Straight Sinus
Hydrocephalus
• Mechanical
➢ Aqueductal Obstruction
• Impaired CSF Resorption
➢ Venous Hypertension
Neuroradiology 1179
Intracranial Lipoma [Figure 5-14-22] Figure 5-14-22
• Congenital, NOT a true neoplasm
• MIDLINE (usually)
• Usually around Corpus Callosum
• Occasional Tectal, Hypothalamic, CPA
• Abnormal Differentiation
➢ Meninx Primativa Into Fat
Figure 5-14-23
Intracranial Lipoma of the quadrigeminal plate.

Notice how the mass presents below the
tentorium because of the herniation caused by
obstructive hydrocephalus
Figure 5-14-24
Meningioma of the quadrigeminal plate
Glioblastoma multiforme [Figure 5-14-25]
Figure 5-14-25
Meningioma of the quadrigeminal

plate – see the dural tail
Figure 5-14-26
Mass in the splenium of the corpus callosum –
Glioblastoma multiforme
Astrocytoma - Splenium [Figure 5-14-25]
Headaches and Parinaud Syndrome [Figure 5-14-26]
Astrocytoma of Tectum
Astrocytoma of the quadrigeminal

plate

1182 Neuroradiology
Pineal Region
• 60% Germ Cell Neoplasm
➢ Seminoma (2/3)
➢ Teratoma
➢ Other – “non-germinoma GCT”
• 15% Pineal Parenchymal
➢ Pineocytoma
➢ Pineoblastoma (PNET)
• OTHER Lesions
➢ Astrocytoma
❖ Splenium, Tectum, Thalamus
➢ Meningioma, Lipoma
➢ VOG Malformations
References
1. Barboriak DP, Lee L, Provenzale JM. Serial MR imaging of pineal cysts: implications for natural history and
follow-up. AJR Am J Roentgenol. 2001 Mar;176(3):737-43.
2. Barbouriak DP, Lee L, Provenzale JM: Serial MR Imaging of Pineal Cysts: Implications for Natural History and
Follow-Up.AJR 2001; 1737-743.
3. Fain JS, Tomlinson FH, Scheithauer BW, Parisi JE, Fletcher GP, Kelly PJ, Miller GM. Symptomatic glial cysts of
the pineal gland. J Neurosurg. 1994 Mar;80(3):454-60.
4. Fleege MA, Miller GM, Fletcher GP, Fain JS, Scheithauer BW. Benign glial cysts of the pineal gland: unusual
imaging characteristics with histologic correlation. AJNR Am J Neuroradiol. 1994 Jan;15(1):161-6.
5. Fujimaki T, Matsutani M, Funada N, Kirino T, Takakura K, Nakamura O, Tamura A, Sano K.J Neurooncol. CT and
MRI features of intracranial germ cell tumors. 1994;19(3):217-26.
6. Hayashida Y, Hirai T, Korogi Y, Kochi M, Maruyama N, Yamura M, Yamashita Y. Pineal cystic germinoma with
syncytiotrophoblastic giant cells mimicking MR imaging findings of a pineal cyst. AJNR Am J Neuroradiol. 2004
Oct;25(9):1538-40.
7. Jinkins JR, Xiong L, Reiter RJ. The midline pineal "eye": MR and CT characteristics of the pineal gland with and
without benign cyst formation. J Pineal Res. 1995 Sep;19(2):64-71.
8. Koenigsberg RA, Faro S, Marino R, Turz A, Goldman W. Imaging of pineal apoplexy. Clin Imaging. 1996 Apr-
Jun;20(2):91-4.
9. Korogi Y, Takahashi M, Ushio Y. MRI of pineal region tumors. J Neurooncol. 2001 Sep;54(3):251-61.
10. Lee DH, Norman D, Newton TH. MR imaging of pineal cysts. J Comput Assist Tomogr. 1987 Jul-Aug;11(4):586-
90.
11. Mamourian A, Towfighi J. MR of pineal cysts. AJNR Am J Neuroradiol. 1994 Oct;15(9):1796-7.
12. Mamourian AC, Towfighi J. Pineal cysts: MR imaging. AJNR Am J Neuroradiol. 1986 Nov-Dec;7(6):1081-6.
13. Mamourian AC, Yarnell T. Enhancement of pineal cysts on MR images. AJNR Am J Neuroradiol. 1991 Jul-
Aug;12(4):773-4. No abstract available.
14. Osborn RE, Deen HG, Kerber CW, Glass RF. A case of hemorrhagic pineal cyst: MR/CT correlation.
Neuroradiology. 1989;31(2):187-9.
15. Reis F, Faria AV, Zanardi VA, Menezes JR, Cendes F, Queiroz LS. Neuroimaging in pineal tumors. J
Neuroimaging. 2006 Jan;16(1):52-8.
16. Sener RN. The pineal gland: a comparative MR imaging study in children and adults with respect to normal
anatomical variations and pineal cysts. Pediatr Radiol. 1995;25(4):245-8.
17. Welton PL, Reicher MA, Kellerhouse LE, Ott KH. MR of benign pineal cyst. AJNR Am J Neuroradiol. 1988 May-
Jun;9(3):612. No abstract available.
Neuroradiology 1181
The Phakomatoses
Phakomatoses or Neurocutaneous Syndromes

• An Introduction
The Phakomatoses
• Neuro-ectodermal
or
• Nerves and Skin
Phakomatoses: Why Study Them?

• They are COMMON diseases
• DIAGNOSED by Imaging
• GENETIC Implications
• SCREEN Relatives
• SURVEILLANCE of Affected
Phakomatoses Mnemonic Tool

• NF-1 (von Reck’s)
➢ TRUE Neurofibromatosis #17
• NF-2 (Bil. VS Syndrome)
➢ M.I.S.M.E. #22
• STURGE-WEBER (Dimitri) Syndrome
➢ Congenital Vascular Lesion
➢ Perhaps NOT inherited
• Tuberous Sclerosis
➢ Pringle’s “HAMARTOMA” Disease
• Von HIPPEL-LINDAU Syndrome
➢ NO cutaneous lesions
➢ Hemangioblastomas and Visceral Lesions
Phakomatoses – Plan
• Demographics
• Diagnostic Criteria
• Ocular/Orbit Lesions
• Skin
• Brain
• Visceral Manifestations
• Complications of Disease
CNS Neoplasms: Chromosomes

• Loss of Heterozygosity (LOH)
➢ Schwannoma - 22q
➢ Meningioma - 22q (Long Arm)
➢ Ependymoma - 22
➢ Medulloblastoma - 17p (Short Arm)
➢ Neurofibrosarcoma - 17p
➢ Retinoblastoma - 13q
➢ Pilocytic Astrocytoma - None !
• TUMOR SUPPRESSOR GENES
Molecular Biology [Figures 5-15-1]

• Genetic “Two Hit Theory”
The Phakomatoses 1182

1184 Neuroradiology
Phakomatoses
• NEUROFIBROMATOSES
➢ Type 1, Chromosome 17q11
Figure 5-15-1
➢ Type 2, Chromosome 22q12
➢ Chromosome 9q, 16p
• STURGE-WEBER (? not inherited)
• von Hippel-Lindau
➢ Chromosome 3p25
Neurofibromatosis
• NF-1, von Recklinghausen (“peripheral” – bad term)
• NF-2, Bilateral Acoustic (“central” – bad term)
• NF-3, Overlap of 1 and 2
• NF-5, Segmental (e.g. a quadrant)
• NF-6, Cafe-au-lait, w/o CNS/PNS
• NF-7, Late Onset
• NF-8, Other
Neurofibromatosis – Types
• Neurofibromatosis Type 1 (NF-1)
➢ von Recklinghausen Disease
➢ “True” Neurofibromatosis
➢ Prominent Cutaneous Signs
➢ Chromosome 17q
• Neurofibromatosis Type 2 (NF -2)
➢ Bilateral Acoustic Schwannoma
➢ “Central Neurofibromatosis”
➢ Minimal Skin Manifestations
➢ Chromosome 22q
Neurofibromatosis Type 1 or von

Recklinghausen Disease Tumor Suppressor Gene: “Two Hit” Hypothesis
• Chromosome 17
Neurofibromatosis
• 1768 MARK AKENSIDE (New York)
• 1793 TILESIUS (Leipzig)
• 1849 R.W. SMITH (England)
• 1822 WISHART (Edinburg) NF-2
• 1882 von RECKLINGHAUSEN (Germany)
Neurofibromatosis - 1
• Clinical
➢ Incidence: 1/2,500 births
➢ Inheritance: Autosomal Dominant
➢ Age at Presentation: Birth to Death
➢ Sx at Presentation: Spots, NFB
➢ Diagnostic Criteria: Cutaneous, PNS
➢ Chromosome Abnl.: 17
➢ Ocular Findings: Myelinated retina
➢ Cutaneous Findings: cafe-au-lait, neurofibroma
➢ CNS Findings: Optic N. Glioma, Hamartoma, Heterotopia, macrocephaly,
mentation problems
NIH Diagnostic Criteria: 2 from list

• Cafe-Au-Lait spots
➢ 6 or more
➢ 5 mm child, 15 mm adult
• Neurofibromas – 2 or more
• Plexiform Neurofibroma – 1
Neuroradiology 1183
1185 The Phakomatoses
• Axillary (Intertriginous) Freckling-1 Figure 5-15-2
• Optic Glioma
• Lisch Nodules (Iris) – 2 or more
• “Distinctive Bone Lesions”
• 1st degree relative with NF-1
Neurofibromatosis – 1
• Clinical
• Chromosome Abnl.: 17
• Ocular: Myelinated retina
• Cutaneous: cafe-au-lait, neurofibroma
• CNS: Optic N. Glioma, Hamartoma, Heterotopia,
Macrocephaly, Mentation
NF-1: Eye Manifestations

• LISCH Nodules (Iris Hamartomas) Lisch Nodules – Named for the 19th century
[Figure 5-15-2]
German physician who first described them, Dr.
➢ Penetrance > 90% Augustus Nodule
➢ Specificity > 90%
Figure 5-15-3
➢ Translucent/pigmented
➢ Small ( < 3mm.), Slit-Lamp Exam
• OPTIC GLIOMA [Figures 5-15-3]
➢ Up to 15% of patients
➢ Pilocytic Astrocytomas
➢ Benign (“Hamartoma -like”), Tx?
➢ True Neoplasms, spread along SAS
➢ up to 1/2 of Childhood ONG w/NF -1
• Cutaneous Manifestations
➢ Cafe-au-Lait spots [Figure 5-15-4]
➢ Intertriginous Freckling
➢ Neurofibromas (Skin and SubQ)
➢ Fibroma Molluscum (TNTC NFB)
➢ Elephantiasis Neuromatosa
❖ diffuse skin thickening/plexiform NFB –
❖ or focal gigantism
• Bone Dysplasia and Remodeling
➢ Macrocephaly Optic Nerve Glioma
➢ Craniofacial dysplasia
❖ especially sphenoid
➢ Vertebrae (scalloping, scoliosis)
➢ Pseudoarthrosis
❖ especially congenital
➢ Genu Valgum/Varum Figure 5-15-4
➢ Twisted “Ribbon Ribs”
Neurofibromatosis – 1 [Figures 5-15-5 to 5-15-8]

• Skull and Spine Dysplasia
➢ Sphenoid Bone (“absent orbit”)
➢ Lambdoid Suture at Temporal Bone
➢ Optic and Auditory Canals (enlarged)
• Scoliosis
➢ Simple or Acute Cx Kyphosis
• Vertebral Scalloping (usu. Lumbar)
• Enlarged Spine Neural Foramina
Café-au-lait spot – a macular (flat)
area of hyperpigmentation

1186 Neuroradiology
Embryo – Differential Development Figure 5-15-5
• Molecular biology
➢ Chemical gradients
➢ Medial <> Lateral
➢ Proximal <> Distal
➢ Anterior <> Posterior
➢ Superior <> Inferior
• Sonic Hedgehog Gene (shh)
➢ Drosophila embryo spiked like a hedgehog
➢ Desert hedgehog (dhh), Indian hedgehog (ihh)
➢ Needed a new name – scientists like videogames … so
Nerve Sheath Tumors

• Schwannoma (Sporadic >> NF-2 > NF-1)
➢ focal mass
➢ usually sensory root, cranial and spinal nerves
• Neurofibroma
➢ usually NF-1, esp. if spinal or paraspinal
➢ spindle or dumb -bell lesion “Empty Orbit” from sphenoid
• Plexiform Neurofibroma (usually NF-1) dysplasia. The left orbit shows the
➢ diffuse or fusiform enlargement outline of normal sphenoid – lesser
• Malignant Peripheral Nerve Sheath Tumor (superior) and greater (inferior)
➢ NF-1 or Sporadic densities
Figure 5-15-6
Figure 5-15-7
Sphenoid Dysplasia
Figure 5-15-8
Progressive Pseudoarthrosis
Focal Gigantism – the overgrowth may affect all

elements, bone, muscle, fat, vessels, etc.
Neuroradiology 1185
Schwannoma – a focal mass, even at the Neurofibroma – a diffuse lesion, even at the
microscopic level microscopic level
Neurofibroma vs. Schwannoma

• Neurofibroma [Figure 5-15-10]
➢ Schwann cells
➢ Fibroblasts
➢ Acellular material
➢ Infiltrating
➢ Resect Parent Nerve
• Schwannoma [Figure 5-15-9]
➢ Schwann Cell Neoplasm
➢ Secondary vascular changes
➢ Mostly cellular
➢ Encapsulated
➢ Nerve Sparing Surgery
Distribution of Nerve Sheath Tumors

• Intra-Cranial – Schwannoma
➢ Sporadic >> NF-2
• Spinal – Both Types (S >> N)
• Dumbbell – Both (N >> S) Figure 5-15-11
• PNS – Both
• Cutaneous – Neurofibroma
➢ Usually N in NF-1)
Intraspinal Neoplasms
• 68 Pts.
• 86 Spinal Nerve Sheath neoplasms
• Sporadic: 42 pts. (65%)
➢ 40 Schwannoma and 2 neurofibroma
• NF-1: 12 Pts. (18%)
➢ All were Neurofibroma
• NF-2: 7 Pts (11%)
➢ 6 Schwannoma/1 “mixed” tumor
• Unknown - 5 Pts. Acute Cervical Kyphoscoliosis – one of the
characteristic lesions of NF-1
Neurofibromatosis : Spine [Figure 5-15-11]
• Scoliosis (NF-1, only?)
➢ Simple ("idiopathic")
➢ Acute Cervical Kyphosis
• Dural Ectasia (NF-1, only?)
➢ Vertebral Scalloping
➢ Arachnoid "cysts"
➢ Lateral Thoracic meningocele

1188 Neuroradiology
Neurofibromatosis : Spine Figure 5-15-12
• Neurofibroma (NF-1)
• Osteoporosis (NF-1, only?)
➢ Idiopathic
➢ Parathyroid Adenoma
• Schwannoma (NF-2)
• Meningioma (NF-2
• Ependymoma (NF-2)
Enlarged Neural Foramen

• DDx:
➢ Nerve Sheath Tumor
❖ Neurofibroma
❖ Schwannoma
➢ Arachnoid Cyst
➢ Bone Dysplasia
Enlarged neural foramina from multiple plexiform
neurofibromata
Neurofibromatosis: Enlarged Neural
Foramen [Figures 5-15-12 to 5-15-14]
• Nerve Sheath Tumor Figure 5-15-13
➢ Neurofibroma
❖ NF-1 >> sporadic
❖ "dumbbell“ shape
➢ Schwannoma
❖ sporadic >> NF-2
• Mesodermal Defect
➢ NF-1 only?
➢ Dural weakness
➢ Bone weakness
Rib Notching [Figure 5-15-15 ]

• Aortic Coarctation
➢ Older than 5-6 years
➢ 3-9 possible
➢ Ribs 5-8 most often Multiple Dumbbell Lesions - neurofibromas
➢ 1-2 arise from subclavian artery
➢ Usually Bilateral
❖ Unilateral on the Right Figure 5-29-14
❖ if Coarctation involves Left Subclavian origin
• A-V Fistulae
• Nerve Sheath tumors
Figure 5-15-15
Rib Notching from extensive plexiform

neurofibromas involving all of the intercostal
nerves
Neuroradiology 1187
Plexiform NF [Figure 5-15-16 ] Figure 5-15-16
Plexiform neurofibromas
Neurofibromatosis - 1: Spine Figure 5-15-17
[Figures 5-15-17 and 5-15-18]
• Scoliosis (Acute Cx Kyphoscoliosis)
• Vertebral Scalloping
• Enlarged Neural Foramina
• Lateral Thoracic Meningocele
Neurofibromatosis - 1 [Figure 5-15-19]

• Posterior Meningocele (sporadic)
➢ dorsal dysraphism, closure of tube
• Anterior Meningocele (sporadic)
➢ neurenteric canal/cyst
➢ anterior vertebral cleft
• Lateral Thoracic Meningocele (NF-1)
➢ “pulsion diverticulum” of SAS
➢ negative intrathoracic pressure
➢ no overlying paravertebral MM. Vertebral Body Scalloping and one
neurofibroma. Both lesions may also
cause enlargement/erosion of the
neural foramna
Figure 5-15-18
Figure 5-15-19
Arachnoid Cyst & Neurofibroma
Lateral Thoracic Meningocele

1190 Neuroradiology
Neurofibromatosis – 1: DBO’s MR Signal Figure 5-15-20
Abnormalities
[Figure 5-15-20]
• T1W Bright Foci
➢ globus pallidus
• T2W Bright Foci
➢ w/o mass, don’t enhance
➢ Cerebellar peduncles, Pons, midbrain
➢ globus pallidus, thalamus, optic radiations
• What in the heck are they??
➢ Ectopic Schwann cells, Melanocytes??
➢ Dysmyelination??
➢ Intracellular proteinaceous fluid?
DBO’s and NF-1

• Incidence: A considerable body of knowledge suggests that
these Unidentified Deep Bright Objects or DBO’s are very
common in children with NF-1. (>90% in some series)
• Age: They are most frequent from 4 –12 years of age. They are
uncommon under the age of 4, and begin to fade away over the
age of 16.
• Location:
➢ Globus Pallidus – 30%
➢ Cerebellum – 23% Deep Bright Objects
➢ Midbrain – 16%
DBO’s of NF-1: Globus pallidus

• Objects
• of Uncertain
• Significance
➢ “NOUS”
Neurofibromatosis
• Malignant Nerve Sheath
➢ Tumor (malignant PNST, neurofibrosarcoma,...)
• Embryonal Malignancies:Wilms,
Rhabdomyosarcoma
• Leukemia (CML)
• Melanoma, Medullary Thyroid Ca.
• Low Incidence of Lung Cancer”
Neurofibromatosis Deep Bright Objects – DBO’s
Malignant Peripheral Nerve Sheath Tumor
Neurofibromatosis Type 2 or Wishart Disease

• Chromosome 22
• Bilateral Acoustic Schwannoma
• "Central Neurofibromatosis"
• Minimal Skin Manifestations
Neurofibromatosis – Type 2
• Incidence: 1/50,000
• Inheritance: Autosomal Dominant
• Age at Presentation: Birth to 40’s (peak in 20’s)
• Sx at Presentation: Hearing loss from VS
• Diagnostic Criteria: VIII masses
• Chromosome Abnl.: 22
• Cutaneous Findings: minimal (skin tags)
• CNS Findings: Schwannoma, Meningioma, Ependymoma (intramedullary
spinal cord)
Neuroradiology 1189
CNS Neoplasms – Chromosome Loss of Figure 5-15-21
Heterozygosity
• NF-2
➢ SCHWANNOMA – 22q
➢ MENINGIOMA – 22q (long arm)
➢ EPENDYMOMA – 22
• NOT Neurofibroma
• NOT Astrocytoma
• NOT Optic Glioma
NF-2 (“Central”), 1 or More

• Bilateral VIIIth Masses Intracanalicular Schwannoma – they all begin
• Relative with NF-2 and either: inside the IAC – because that is where the
➢ Unilateral VIIIth Mass Schwann cells are. The cisternal segment of the
➢ Any Two: nerve has oligodendrocytes
❖ “Neurofibroma”, Meningioma, Glioma, Schwannoma, (Congenital)
Lens Opacity
Figure 5-15-22
Neurofibromatosis – Type 2
• NEJM 319:278-83, 1988 (Gulf of Mexico)
• 23 Pts. (15M/8F), Kindred of 137
• 0.95 Penetrance
• 18 Acoustic Schwannoma (17 bil.)
• 8 Meningioma (3 mult.)
• 4 Ependymoma
• 2 Spinal “Neurofibroma”
Schwannoma [Figures 5-15-21 and 5-15-22]

• 5%-10% of All CNS Tumors
• Benign, Slowly growing
• F > M (Intracranial), M > F (Spinal)
• 30’s – 60’s, w/NF-2 10’s – 30’s
• Sensory Nerves (usually):
➢ CNN VIII (Sup.Vestibular), V, X
➢ Spine: Dorsal Roots
• Majority (>90%) are Sporadic
• Multiple in NF-2, Bilat.VIII Pathognomonic
Neurofibromatosis – 2 [Figure 5-15-23]

Bilateral Vestibular Schwannoma
• Meningiomas:
➢ multiple transitional type meningioma
➢ NOT meningothelial
• Meningioangiomatosis: Figure 5-15-23
➢ cortical (intracortical) vascular tissue
➢ resembles a vascular malformation
➢ meningothelial and fibroblast -like cells
Multiple Schwannomas and Meningiomas and

Ependymomas [Figure 5-15-24]
Neurofibromatosis – 2 [Figure 5-15-24]

• Multiple Meningiomas (up to 45%)
➢ Intraventricular Meningiomas
➢ Childhood Meningiomas
• Multiple Meningiomas
➢ 1%-10% of all patients with meningioma
➢ SPORADIC in 80%-90%
• Intraventricular Meningiomas
➢ SPORADIC in 90%
• Childhood Meningiomas Multiple Schwannomas and
➢ SPORADIC vs. Inherited (NF-2 or Not) Meningiomas
The Phakomatoses 1192 Neuroradiology

Neurofibromatosis Type - 2=> MISME Figure 5-15-24
• M ultiple
• I nherited
• S chwannomas
• M eningiomas
• E pendymomas
Sturge-Weber Disease or Encephalo-

Trigeminal Angiomatosis
• Inheritence ??
• Autosomal Dominant ?
• Autosomal Recessive ?
• Sex-Linked ?
Multiple Schwannomas, Meningiomas, and
STURGE-WEBER: Definition Ependymomas – the MISME syndrome
• A telangiectatic venous angioma of the
leptomeninges, face, and choroid of the eye.
STURGE-WEBER SYNDROME: History Figure 5-15-25

• 1879 STURGE, Clinical description
• 1897 Kalischer, Vascular nature
• 1922 Weber, published radiography
• 1923 Dimitri, “tram-track” Ca++
• 1934 Krabbe, Ca++ in cortex
• 1937 van der Hoeve, Phakomatosis
STURGE-WEBER SYNDROME: Classic Triad

• Facial Neveus Flammeus
➢ Port-Wine Stain
• Seizures
• Mental Deficiency
STURGE-WEBER: Manifestations
• Seizures, Mental Decline Trigeminal Angiomatosis in SWS
➢ Usually begins in first 24 months
• Facial Angioma
➢ At birth Figure 5-15-26
• Angiomatous Overgrowth of soft-tissue and bone
• Leptomeningeal Angioma
• Cortical Atrophy w/Ca++
STURGE-WEBER: Variants
• Facial and Intracranial w/o Eye
• Intracranial and Eye w/o Face
• Intracranial Alone
➢ (Cerebral and Leptomeningeal)
• Klippel-Trenaunay (KT Weber)
➢ Extracranial soft-tissue angiomas)
STURGE-WEBER SYNDROME:
Port Wine Stain (PWS) [Figures 5-15-25 and 5-15-26]
• Facial Neveus Flammeus
• Blanches w/ pressure
• Trigeminal Dermatome
➢ V1 – Ophthalmic
➢ V2 – Maxillary
➢ V3 – Mandibular
Sturge-Weber: Facial overgrowth and

extensive, bilateral, “port-wine stain”.
Neuroradiology 1191
Association of PWS with SWS Figure 5-15-27
• All 3 >> 1+2 >> 1 or 2 alone >> other
• medial aspect of eyelid (V1 or V2)
Medullary Veins [Figures 5-15-27 and 5-15-28]
STURGE-WEBER: Vascular
• Absence of cortical veins
• Poor filling of sagittal sinus
• Persistent Primitive Plexus (SAS)
• Recruitment of Medullary Veins
• Prominent Choroid Plexus
Persistence of Primitive Plexus

• Persistence of primitive vascular plexus Medullary Veins are prominent, bridging veins are
• Absence of cortical veins absent over the occipital and posterior parietal
• Deoxygenated blood lobes
Figure 5-15-28
Cranial Vascular Development
• Begins with primitive plexus
• Progressive Differentiation
➢ Arteries
➢ Capillaries
➢ Veins
• Progressive Lamination
➢ Cerebral (brain) circulation
➢ Dura and Bone circulation
➢ Scalp Circulation
} Connect via bridging
veins
Sequential Induction: Eye Development

• Optic nerve induces lens development
• Lens induces cornea development from surface ectoderm and
adjacent mesenchyme
STURGE-WEBER: Etiology
• Abnormal Development of Capillaries
➢ Poor cortical venous drainage
➢ Absent cortical veins T1W MR with contrast. Prominent
➢ Prominent veins in SAS medullary veins in SWS. Also note
➢ Prominent deep (medullary) veins the widened diploic space of the
➢ Enlarged choroidal vessels frontal bone and gyriform surface
➢ Persistence of Primitive Plexus enhancement posteriorly
STURGE-WEBER: Orbit/Eye [Figure 5-15-29] Figure 5-15-29

• Buphthalmos (‘Ox Eye’)
➢ congenital glaucoma
➢ enlarged globe
• Choroidal Angioma
• Episcleral Telangiectasia
• Angiomatous Overgrowth of EOM’s
STURGE-WEBER: Manifestations
• Seizures, Mental Decline
• Facial Angioma
• Angiomatous Overgrowth
• Leptomeningeal Angioma
• Cortical Atrophy w/Ca++
Buphthalmos or “Ox eye”. Congenital
glaucoma, caused by a choroidal
angioma, has led to enlargement
of the ocular globe

1194 Neuroradiology
Vessels in SAS [Figure 5-15-30] Figure 5-15-30
Normal vs. Venous Outflow Obst. [Figure 5-15-31]
Figure 5-15-31
Multiple small vessels in SAS in SWS
Impaired venous drainage leads to chronic Figure 5-15-32

cerebral ischemia
STURGE-WEBER: Calcification [Figure 5-15-32]

• Abnormal (sluggish) circulation
• Chronic Cerebral Ischemia
• Progressive Cell Loss (Atrophy)
• Progressive Cerebral calcification
➢ early – subcortical WM (?)
➢ Later – middle layers of cortex
DYKE, DAVIDOFF, MASSON

• “Cerebral Hemiatrophy with Homolateral
Hypertrophy of the Skull and Sinuses”
• Surgery, Gynecology, & Obstetrics 1933 pp. 589-600 Sturge-Weber Disease. Cerebral hemiatrophy and
calcification
Sturge-Weber- Hemiatrophy [Figure 5-15-33]
Figure 5-15-33
Noncontrast and postcontrast axial CT (Images 1,

2, 3) sections show prominent subarachnoid
spaces overlying atrophic left frontal lobe.
Cortical calcification and hypodense white
matter of the ipsilateral forceps minor are well
shown also
Neuroradiology 1193
Progression in SWS [Figure 5-15-34] Figure 5-15-34
Dyke, Davidoff, Masson [Figure 5-15-35]

Figure 5-15-35
Dyke, Davidoff, Masson changes from cerebral

hemiatrophy in SWS
STURGE-WEBER [Figures 5-15-36 to 5-15-38]

Progression in SWS
• Gyral Gadolinium Enhancement
same patient two years apart
• Abnormal BBB in Cortex
➢ (Chronic ischemia)
• “Epi-Cortical” enhancement Figure 5-15-36
➢ (slow flow in superficial veins)
STURGE-WEBER: Treatment
• Symptomatic (anticonvulsants)
• Cosmetic Tattooing
• Laser Treatment of Skin Lesions
• Hemispherectomy
• Aspirin (mild antiplatelet)?
Figure 5-15-37 Sturge-Weber Disease: Gyral Enhancement and

Choroid Plexus enlargement
Figure 5-15-38
Sturge-Weber Disease. Gyral hypointensity from

dense calcifications. Angiomatous overgrowth
of the temporalis muscle (arrow)
Sturge-Weber Disease. T2W MR and CT in the
same patient show changes from gyral
calcification

1196 Neuroradiology
Half a Brain
• Uncontrolled Sz
• Under age 2
• Up to age 5-7
• Plasticity
• Uncrossed tracts
➢ 5%–15%
Tuberous Sclerosis or Bourneville Disease

• Chromosomes 9 and 16
Tuberous Sclerosis
• Original “VOGT TRIAD”
➢ FACIAL NEVUS (ADENOMA SEBACEUM)
➢ SEIZURES
➢ MENTAL DEFICIENCY
Tuberous Sclerosis
• AUTOSOMAL DOMINANT
• No Racial/Sexual
• High Spontaneous Mutation
• High Penetrance
➢ “SPORADIC” over-reported
• Multiple Genes
➢ TSC1 – 9q
➢ TSC2 – 16p
Tuberous Sclerosis, NIH Consensus Conference
• Major Features: • Minor Features:

➢ Facial angiofibroma or forehead ➢ Multiple dental enamal pits
plaque ➢ Hamartomatous rectal polyps
➢ Ungual or Periungual fibroma ➢ Bone cysts
➢ >3 Hypomelanotic macules ➢ White matter migration lines
➢ Shagreen patch ➢ Gingival fibromas
➢ Multiple retinal nodular ➢ Non-renal hamartoma
hamartomas ➢ Retinal achromic patch
➢ Cortical Tuber ➢ "Confetti" skin lesions
➢ Subependymal Nodule ➢ Multiple renal cysts
➢ Subependymal Giant Cell
Astrocytoma
➢ Cardiac rhabdomyoma
➢ Lymphangiomyomatosis
➢ Renal angiomyolipoma
Definite TS - (2 Major) or (1 Major + 2 Minor)
Probable TS - 1 Major + 1 Minor
Possible TS - (1 Major) or (2 Minor)
Hyman MH, Whittemore VH:"National Institutes of Health Consensus

Conference:tuberous sclerosis Complex" Arch Neurol 2000; 57: 662-665
Tuberous Sclerosis
• Definitive (need 1)
➢ (1) facial angiofibroma
➢ (2) ungual fibroma
➢ (3) retinal hamartoma
➢ (4) cortical tubers
➢ (5) subependymal nodules
➢ (6) multiple renal AML
Neuroradiology 1195
• Presumptive (need 2)
➢ (1) hypomelanotic nodules
➢ (2) shagreen patch
➢ (3) single renal AML
➢ (4) multicystic kidney
➢ (5) cardiac rhabdomyoma
➢ (6) pulmonary lymphangiomyomatosis
➢ (7) radiographic “honeycomb” lung
➢ (8) first degree relative with TS
Tuberous Sclerosis:
• Seizures 90%
• Adenoma Sebaceum 60%-90%
• Retardation 40%-60%
• Retinal Phakoma 50%
• Xr: Intracranial Ca++ 50%
• Ungual Fibromata 17%
• Giant Cell Astrocytoma 15%
INCIDENCE Of Tuberous Sclerosis:

• CLASSIC TRIAD
➢ VARIABLE Incidence
➢ 1 In 10K- 500K
➢ 1 In 150K In HONG KONG
• MAYO Clinic Criteria
➢ 1 IN 10,000 AT MAYO CLINIC
➢ Local Population Olmsted Cty
• FORME FRUSTE
➢ Five Times More Common Than Classic
Tuberous Sclerosis
• “Hamartomas”
➢ CNS (Cortical Ventricular)
➢ Retina (Phakoma)
➢ Kidney (Angio Myo Lipoma – AML)
• Angiofibromas
➢ Face (“Adenoma Sebaceum”)
➢ Nail Bed (“Fibromas”)
Tuberous Sclerosis:
• Rhabdomyomas – Heart
➢ “Hamartomas”
• Angiomyomatosis – Lung
➢ smooth muscle proliferation
Tuberous Sclerosis: Cutaneous

• “Adenoma Sebaceum”
• Peau D’orange
• Ash-Leaf Macule
• Ungual Angiofibromas
Adenoma Sebaceum
• AKA PRINGLE’S DISEASE
• NOT present at birth
• develop before puberty
• nasolabial fold ->bi-malar
• papules of angiofibroma

1198 Neuroradiology
Pringle’s Disease [Figure 5-15-39] Figure 5-15-39
• Pringle’s Name
➢ Entire Disease
➢ Facial lesion only
• Mild Mental Retardation
• Seizures
• Hard Potatoes
• Tubular Can – ‘Tuberous’
Subungual/Periungual Fibroma [Figure 5-15-40]
Figure 5-15-40
Pringle Disease: A papular (raised)

Subungual (right) and Periungual (left) Fibroma in reddish lesion, often in a bimalar
Tuberous Sclerosis. These are pattern, caused by
angiofibromata, similar to the Pringle facial angiofibromata of the skin in
lesion Tuberous Sclerosis
Depigmentation:
• Ash-Leaf Spots
➢ (Lance- Ovate Shape)
Figure 5-15-41
• Confetti – Like Hypopigmentation
➢ (Inverse Freckle)
Other Cutaneous Manifestations

• Subepidermal Fibrosis:
➢ Dorsal Surfaces
➢ “Shagreen Patch”
➢ “Peau D’orange”
➢ “Pigskin”
➢ “Elephant Hide”
Tuberous Sclerosis: Ocular

[Figure 5-15-41]
• PHAKOMA Tuberous Sclerosis. Astrocytic hamartoma of the
➢ benign astrocytic hamartoma retina – the original “phakoma” of van der
➢ LEUKOKORIA Hoeve
➢ White light reflex
➢ Calcification Common
➢ Especially over Optic Nerve
Tuberous Sclerosis – BRAIN:

• HETEROTOPIAS AND HAMARTOMAS
➢ in white and gray matter
• CORTICAL TUBERS
➢ “HAMARTOMAS”
➢ but with abnormal “N” cells
➢ neither Astrocyte nor Neuron
➢ Decreased Myelination
➢ No laminar architecture
Neuroradiology 1197
• SUBEPENDYMAL NODULES (almost 100%) Figure 5-15-42
➢ “hamartomas” vs. neoplasia
➢ Caudothalamic groove
➢ Polypoid “Candle Gutterings”
• DILATED VENTRICLES
➢ variable
➢ obstructive, atrophic vs. “idiopathic”
• TUMORS 15%
• Sub-ependymal Giant Cell Astrocytoma
➢ True neoplasm, Benign WHO Grade I
Cortical Tubers
[Figure 5-15-42]
Subependymal Nodules
[Figures 5-15-43 and 5-15-44]
Figure 5-15-43
Cortical Tubers in Tuberous Sclerosis
Figure 5-15-44
Neonatal sonogram. Subependymal Nodules in

Tuberous Sclerosis
Tuberous Sclerosis [Figures 5-15-45 to 5-15-47]

• Renal
➢ Angiomyolipoma
➢ Multiple Simple Cysts
➢ Another cause of APCKD
➢ RCC Reported
Figure 5-15-45 Subependymal nodules may enhance without

neoplastic transformation
Tuberous Sclerosis is a
disorder of neuronal
migration and maturation

1200 Neuroradiology
Tuberous Sclerosis. Cortical tubers Subependymal Giant Cell Astrocytoma in

and white matter hyperintensities Tuberous Sclerosis. This is a low grade –
from the abnormal migration and WHO 2 – neoplasm
maturation of the brain
Figure 5-15-48
Angiomyolipoma [Figure 5-15-48]

• 10% w/enough FAT for plain film
• 1/6 OF Solitary AML Pts. Have TS
• 1/3-1/2 of solitary AML Pts. Have other stigmata of TS
• 50-80% of Pts. W/TS will have AML
• 3/4 MULTIPLE
• 1/3 – 1/2 BILATERAL (probably more)
• variable amts. of FAT, Smooth mm., and vessels
Renal Cysts [Figure 5-15-49]
Figure 5-15-49
Multiple renal angiomyolipomas in
Tuberous Sclerosis
Tuberous Sclerosis. In addition to angiomyolipoma, the patients may

also develop multiple and bilateral renal cysts
[different patients]
Neuroradiology 1199
Angiomyomatosis vs. Lymphangiomyomatosis Figure 5-15-50
[Figure 5-15-50]
• “sporadic” cases, all are female
➢ 50% chylothorax
➢ Perilymphatic smooth mm.
➢ May have abdominal LN involvement
• In TS, males can be affected
➢ chylothorax is rare
➢ Periarterial smooth mm around pulmonary aa
Bone Islands [Figure 5-15-51]
Hemangioblastomatosis or Von Hippel-Lindau

Disease Pulmonary
Lymphangioleiomyomatosis in
• Chromosome 3
Tuberous Sclerosis
von Hippel-Lindau
• Incidence of 1/35K – 40K
• 6-7K pts in USA
• AUTOSOMAL DOMINANT
• NO RACIAL/SEXUAL PREDILECTION
• VARIABLE PENETRANCE / EXPRESSIVITY
• Chromosome 3p25-26
von Hippel-Lindau Syndrome: History

• 1864 scattered reports of angiomatous lesions of both retina and cerebellum
• 1894 Collins (England)
➢ two sibs with retinal angioma
• 1904 von Hippel (Germany)
➢ familial retinal hemangioblastoma
• 1926 Lindau (Sweden)
➢ familial retinal and cerebellar hemangioblastomas
• 1964 Melmon and Rosen
von Hippel-Lindau
• 1. CNS and Retinal hemangioblastoma
• 2. Hemangioblastoma and one:
➢ a. renal, pancreatic, hepatic, epididymal cyst
➢ b. pheochromocytoma
➢ c. renal cancer
• 3. Family history and one:
➢ a. hemangioblastoma
➢ b. viscera
➢ c. pheochromocytoma
➢ d. renal cancer
von Hippel-Lindau Figure 5-15-51

SYNDROME: NIH
Classification
• Type I – VHL w/o Pheo
➢ Renal/Pancreatic cysts, RCC
➢ most common type
• Type II – VHL with Pheo
➢ IIA Islet cell tumors (no cysts)
➢ IIB Renal/Pancreatic Disease
❖ least common type
Tuberous Sclerosis – Multiple bone islands. These could also be

considered as “hamartomas”.

1202 Neuroradiology
von Hippel-Lindau Figure 5-15-52
➢ Cerebellum
➢ Retina
➢ Medulla, Cord
• Cysts/Tumor
➢ Kidney
➢ Liver
➢ Pancreas
• Epididymis and Endolymphatic Cystadenoma
• Pheochromocytoma -Adrenal (Certain Families -Type II)
von Hippel-Lindau: Six Classic Lesions [Figure 5-15-52]

• Hemangioblastoma von Hippel-Lindau Syndrome. Retinal
• Retinal Angioma (Hemangioblastoma) angioma demonstrated on
• Pancreatic Cyst flourescein angiogram
• Renal Cysts and Ca
• Pheochromocytoma Figure 5-15-53
• Epididymal Cystadenoma
• Endolymphatic sac tumor
Hemangioblastoma:
• True Neoplasm Endothelial Origin
• Hypervascular
➢ capillary to sinusoidal
➢ dilated feeding artery
➢ dilated draining vein
➢ slow flow
• Stromal Cells
➢ foamy, lipid -laden
Hemangioblastoma – the classic
von Hippel-Lindau: Hemangioblastoma “cyst with nodule” morphology
• Cerebellum 66%
• Retina (“angiomas”) 58%
• Spinal Cord / Roots 28%
Figure 5-15-54
• Medulla 14%
Hemangioblastoma [Figures 5-15-53 to 5-15-60]
Figure 5-15-55
Hemangioblastoma with capillaries and stromal

cells
Hemangioblastoma – Sporadic on left, VHL on

right (multiple lesions).
Neuroradiology 1203 The Phakomatoses

Hemangioblastomas span a spectrum from largely

Hemangioblastoma – densely enhancing nodule
cystic to mostly solid
persists into venous phase
Figure 5-15-58
Figure 5-15-59
Hemangioblastoma – densely enhancing nodule Hemangioblastoma in the medulla

persists into venous phase oblongata
Figure 5-15-60
Hemangioblastoma AND VHL

• 1/6-1/5 of solitary cerebellar hemangioblastomas are
associated w/ VHL
• up to 1/2 of medullary HBL occur in VHL
• “ALL” Multiple HBL are VHL
• there was one family w/o VHL
Erythropoietin
• in cyst fluid
• Elevated ESR
• Elevated Hct
von Hippel-Lindau. Syringohydromyelia with

multiple enhancing nodules of
hemangioblastoma

1204 Neuroradiology
von Hippel-Lindau: Renal Manifestations Figure 5-15-61
CYSTS 25%–63%
ANGIOMAS 7%
ADENOMAS 14%
CLEAR CELL CA 15%–50%
von Hippel-Lindau: Kidney

[Figure 5-15-61]
• Renal Cell Carcinoma
➢ Multiple
➢ Bilateral
➢ Conservative Surgery von Hippel-Lindau. Multicystic renal cell carcinoma
von Hippel-Lindau: Pancreas [Figure 5-15-62]

• Pancreatic cysts 18%-72%
• Pancreatic adenoma 7%
➢ microcystic (“glycogen rich”) Figure 5-15-62
• Pancreatic Ca
➢ reported in single family
• Islet Cell Tumors
Pancreatic Adenoma In VHL

• Microcystic (Not Macrocystic)
• Serous (Not Mucin Producing)
• Not Pre-Malignant
• Glycogen Rich
• Stellate Scar
➢ which may be visible, have Ca++ von Hippel-Lindau. Pancreatic Microcystic Adenoma
– also called glycogen-rich adenoma - Note
VHL – Visceral Manifestations central stellate scar on gross image.
Pheochromocytoma and VHL

• 20% of ALL Pheochromocytoma are VHL
• Typically in Adrenal
• Present YOUNGER w/VHL
• Multiple with VHL
• Mortality (5% of VHL DIE from catecholamines)
• Workup: MR and MIBG (95% sensitive) Figure 5-15-63
➢ 24hr NOREPINEPHRINE
➢ VMA (53% sensitive)
➢ US (40% sensitive)
Endolymphatic Sac Tumor [Figure 5-15-63]

• Petrous Apex Mass
➢ Cholesterol granuloma
➢ Glomus tumor
➢ Vascular variant
➢ Cystadenoma (endolymphatic sac tumor)
Von Hippel-Lindau
➢ Cerebellum
➢ Retina
➢ Medulla, Cord
• Cysts/Neoplasms
➢ Kidney
➢ Liver
➢ Pancreas
➢ Epididymis
➢ Endolymphatic sac
• Pheochromocytoma -Adrenal Endolymphatic Sac Tumor
Neuroradiology 1203
VHL - Multiple hemangioblastomas [Figure 5-15-64] Figure 5-15-64
VHL - Multiple hemangioblastomas
Educational Objectives
• Describe why NF-1 is truly “Neurofibromatosis”
• Describe three neoplasms caused by the chromosome 22 mutation in NF-2
• Describe the vascular abnormalities of Sturge Weber Syndrome
• Explain why Tuberous Sclerosis is a disorder of neuronal migration
• Distinguish von Hippel-Lindau from the ‘neurocutaneous’ phakomatoses
Summary - Phakomatoses Mnemonic Tool

• NF-1 (von Reck’s)
➢ TRUE Neurofibromatosis #17
• NF-2 (Bil. VIII Syndrome)
➢ M.I.S.M.E. #22
• STURGE-WEBER (Dimitri) Syndrome
➢ Congenital Vascular Lesion
➢ perhaps NOT inherited
➢ Pringle’s “HAMARTOMA” Disease
• von Hippel-Lindau Syndrome
➢ NO cutaneous lesions
➢ Hemangioblastomas and Visceral Lesions
References Part 1
1. Aizpuru RN, Quencer RM, Norenberg M, Altman N, Smirniotopoulos JG. Meningioangiomatosis: clinical,
radiologic, and histopathologic correlation. Radiology 1991; 179:819-821.
2. American Journal of Neuroradiology 8[6], 1031-1036. 1987
3. Aoki S, Barkovic AJ, Nishimura K, Kjos B, Brown EW, Riccardi VM et al. Neurofibromatosis Types-1 and Type-
2: Cranial MR Findings. Radiology 1989; 172(2):527-534.
4. Baldwin D, King TT, Chevretton E, Morrison AW. Bilateral cerebellopontine angle tumors in neurofibromatosis
type 2. J Neurosurg 1991; 74:910-915.
5. Barker D, Wright E, Nguyen K, Cannon P. Gene for von Recklinghausen Neurofibromatosis is in the
Pericentromeric Region of Chromosome 17. Science 1987; 236:1100-1102.
6. Brown EW, Riccardi VM, Mawad M, Handel S, Goldman A, Bryan RN. MR Imaging of Optic Pathways in
Patients with Neurofibromatosis.
7. Brzowski AE, Bazan III C, Mumma JV, Ryan SG. "Spontaneous regression of optic glioma in a patient with
neurofibromatosis.". Neurology 1992; 42(3):679-681.
8. Chui MC, Bird BL, Rogers J. Extracranial and Extraspinal Nerve Sheath Tumors: Computed Tomographic
Evaluation. Neuroradiology 1988; 30:47-53.
9. Cohen MM. Invited Historical Comment: Further Diagnostic Thoughts About the Elephant Man. Am J Med
Genetics 1988;777-782.

1206 Neuroradiology
10. DiMario FJ, Ramsby G, Greenstein R, Langshur S, Dunham B. "Neurofibromatosis Type 1: Magnetic Resonance
Imaging Findings.". Journal of Child Neurology 1993; 8:32-39.
11. DiPaolo DP, Zimmerman RA, Rorke LB, Zacki EH, Bilaniuk LT, Yachnis AT. Neurofibromatosis Type 1:
Pathologic Substrate of High-Signal-Intensity Foci in the Brain.
12. Domenicucci M, Santoro A, D'Osvaldo DH, Delfini R, Cantore GP, Guidetti B. Multiple Intracranial
Meningiomas. J Neurosurg 1989; 70:41-44. 1989.
13. Eljamel MSM, Foy PM. Multiple Meningiomas and Their Relation to Neurofibromatosis. Review of the Literature
and Report of Seven Cases. Surg Neurol 1989; 32:131-136.
14. Evans DG, Mason S, Huson SM, Ponder M, Harding AE, Strachan T. Spinal and cutaneous schwannomatosis is a
variant form of type 2 neurofibromatosis: a clinical and molecular study. J Neurol Neurosurg Psychiatry 1997;
62(4):361-366.
15. Evans DGR, Huson SM, Donnai D, Neary W, Blair V, Newton V et al. A Clinical Study of Type 2
Neurofibromatosis. Quarterly Journal of Medicine 1992; 84:603-618.
16. Ferner RE, Chaudhuri R, Bingham J, Cox T, Hughes RAC. "MRI in neurofibromatosis 1. The nature and evolution
of increased intensity T2 weighted lesions and their relationship to intellectual impairment.". J Neurol Neurosurg
Psychiatry 1993; 56:492-495.
17. Halliday AL, Sobel RA, Martuza RL. Benign Spinal Nerve Sheath Tumors: Their Occurrence Sporadically and in
Neurofibromatosis Types 1 and 2. J Neurosurgery 74:248-253, 1991.
18. Harkens K, Dolan KD. "Correlative Imaging of Sphenoid Dysplasia Accompanying Neurofibromatosis.". Ann Otol
Rhinol Laryngol 1990; 99:137-141.
19. Harkin JC, Reed RJ. Tumors of the Peripheral Nervous System. Fascicle 3, Second Series, Atlas of Tumor
Pathology. AFIP Washington D C 1969;-97.
20. Hurst RW, Newman SA, Cail WS. Multifocal intracranial MR abnormalities in neurofibromatosis. AJNR Am J
Neuroradiol 1988; 9(2):293-296.
21. J Pediatr 125, 63-66. 1994
22. Kendall B, Symon L. Investigation of patients representing with cerebellopontine angle syndromes.
23. Listernick R, Charrow J, Greenwald M, Mets M. Natural History of Optic Pathway Tumors in Children with
Neurofibromatosis Type 1: A Longitudinal Study.
24. Martuza RL, Eldridge R, Wertelecki W, Rouleau GA, Superneau DW, Forehand LW. Neurofibromatosis 2
(Bilateral Acoustic Neurofibromatosis) Neurofibromatosis 2: Clinical and DNA Linkage Studies of a Large
Kindred. NEJM 1988; 319:278-283.
25. Mautner VF, Tatagiba M, Lindenau M, Funsterer C, Pulst SM, Baser ME et al. Spinal tumors in patients with
neurofibromatosis type 2: MR imaging study of frequency, multiplicity, and variety [published erratum appears in
AJR Am J Roentgenol 1996 May;166(5):1231]. AJR Am J Roentgenol 1995; 165(4):951-955.
26. Merten D, Gooding C, Newton T, Malamud N. Meningiomas of childhood and adolescence. J Peds 1974; 84:696-
700.
27. Mirowitz SA, Sartor K, Gado M. High-intensity basal ganglia lesions on T1-weighted MR images in
neurofibromatosis. AJNR Am J Neuroradiol 1989; 10(6):1159-1163.
28. Moore BD, Slopis JM, Schomer D, Jackson EF, Levy BM. Neuropsychological significance of areas of high signal
intensity on brain MRIs of children with neurofibromatosis. Neurology 1996; 46(6):1660-1668.
29. Mulvihill JJ, moderator. Neurofibromatosis 1 (Recklinghausen Disease) and Neurofibromatosis 2 (Bilateral
Acoustic Neurofibromatosis): an update. Ann Intern Med 1990; 113:39-52.
30. National Institutes of Health Consensus Development Conference Statement on Acoustic Neuroma, December 11-
13, 1991. The Consensus Development Panel. Arch Neurol 1994; 51(2):201-207.
31. Neurofibromatosis. Conference statement. National Institutes of Health Consensus Development Conference. Arch
Neurol 1988; 45(5):575-578.
32. Neuroradiology 13, 65-84. 1977.
33. Pomeranz SJ, Shelton JJ, Tobias J, Soila K, Altman D, Viamonte M. MR of Visual Pathways in Patients with
Neurofibromatosis. AJNR 1994; 8:831-836.
34. Radiology 195, 721-724. 1995
35. Riccardi V. Neurofibromatosis. Phenotype, Natural History, and Pathogenesis. 2 ed. Baltimore: The Johns Hopkins
University Press, 1992.
36. Russell DS, Rubinstein LJ. Dysgenetic Syndromes (Phacomatoses) Associated with Tumors and Hamartomas of
the Nervous System. Chap 11 (pgs 766-784) in Pathology of Tumors of the Nervous System Williams and Wilkins,
Baltimore, 1989.
37. Seizinger BR, Martuza RL, Gusella JF. Loss of Genes on Chromosome 22 in Tumorigenesis of Human Acoustic
Neuroma. Nature 322:644-647, 1986.
38. Smirniotopoulos JG, Murphy FM. The Phakomatoses. AJNR Am J Neuroradiol 1992; 13:725-746.
39. Stull MA, Moser RP, Kransdorf MJ, Bogumill GP, Nelson MC. Magnetic Resonance Appearance of Peripheral
Nerve Sheath Tumors. Skeletal Radiology 20:9-14, 1991.
Neuroradiology 1205
References Part 2
1. Afra D, Muller W, Slowik F, Firsching R. Supratentorial lobar pilocytic astrocytomas: report of 45 operated cases,
including 9 recurrences. Acta Neurochir (Wien ) 1986; 81(3-4):90-93.
2. Alexander GL. Sturge-Weber Syndrome. Chap 7 in The Phakomatoses Vinken PJ, Bruyn GW Eds., Vol. 14 of
Handbook of Clinical Neurology. Elsevier Publishing Co , New York, 1972.
3. Bachmann K, Markwalder R, Seiler RW. Supratentorial hemangioblastoma: Case Report. Acta Neurochirurgica
1978; 44:173-177.
4. Barath B, Voros E, Bak Z, Bodosi M. Cerebral venous drainage via the ophthalmic veins in the Sturge-Weber
syndrome. Neuroradiology 1994; 36(4):318-320.
5. Bender BL, Yunis EJ. The Pathology of Tuberous Sclerosis. Path Annu 1982; 17:339-382.
6. Choyke PL, Glenn GM, McClellan WM, Patronas NJ, Linehan WM, Zbar B. von Hippel-Lindau Disease:
Genetic, Clinical, and Imaging Features. Radiology 1995; 194:629-642.
7. Choyke PL, Glenn GM, Walther MM, Zbar B, Weiss GH, Alexander RB et al. "The Natural History of Renal
Lesions in von Hippel-Lindau Disease: A Serial CT Study in 28 Patients.". AJR 1992; 159:1229-1234.
8. Dahlen RT, Harnsberger HR, Gray SD, Shelton C, Allen R, Parkin JL et al. Overlapping thin-section fast spin-echo
MR of the large vestibular aqueduct syndrome. AJNR Am J Neuroradiol 1997; 18:67-75.
9. Dyke DG, Davidoff LM, Masson CB. Cerebral Hemiatrophy with Homolateral Hypertrophy of the Skull and
Sinuses. Surgery, Gynecology and Obstetrics 1933; 57:588-600.
10. Feghali JG, Levin RJ, Llena J, Bradley MK, Kantrowitz AB. Aggressive Papillary Tumors of the Endolymphatic
Sac: Clinical and Tissue Culture Characteristics. Am J Otology 16[6], 778-782. 1995.
11. Fetner CD, Barilla DE, Scott T, et al. Bilateral renal cell carcinoma in von Hippel-Lindau syndrome: Treatment
with staged bilateral nephrectomy and hemodialysis. J Urol 17, 534-536. 1977.
12. Filing-Katz MR, Choyke PL, Oldfield E, Charnas L, Patronas NJ, Glenn GM et al. Central nervous system
involvement in Von Hippel-Lindau disease. Neurology 1991; 41:41-46.
13. Filling-Katz MR, Choyke PL, Patronas NJ, et al. Radiologic scrreening for von Hippel-Lindau disease: The role of
Gd-DTPA-enhanced MR imaging of the CNS. Journal of Computer Assisted Tomography 13, 745-755. 1989.
14. Hirose T, Scheithauer BW, Lopes MB, Gerber HA, Altermatt HJ, Hukee MJ et al. Tuber and subependymal giant
cell astrocytoma associated with tuberous sclerosis: an immunohistochemical, ultrastructural, and immunoelectron
and microscopic study. Acta Neuropathol (Berl) 1995; 90:387-399.
15. Ho VB, Smirniotopoulos JG, Murphy FM, Rushing EJ. Radiologic-pathologic correlation: hemangioblastoma.
AJNR 1992; 13:1343-1352.
16. Hoang MP, Amirkhan RH. Inhibin alpha distinguishes hemangioblastoma from clear cell renal cell carcinoma. Am
J Surg Pathol 2003; 27(8):1152-1156.
17. Huson SM, Harper PS, Hourihan MD, Cole G, Weeks RD, Compston DAS. Cerebellar Haemangioblastoma and
von Hippel-Lindau Disease. Brain 1986; 109:1297-1310.
18. Jelinek J, Smirniotopoulos JG, Parisi JE, Kanzer M. Lateral ventricular neoplasms of the brain: differential
diagnosis based on clinical, CT, and MR findings. AJR 1990; 155:365-372.
19. Koeller KK, Sandberg GD. From the archives of the AFIP. Cerebral intraventricular neoplasms: radiologic-
pathologic correlation. Radiographics 2002; 22(6):1473-1505.
20. Levine E, Weigel JW, Collins DL. Diagnosis and management of asympptomatic renal cell carcinomas in von
Hippel-Lindau syndrome. Urology 21, 146-150. 1983.
21. Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM et al. von Hippel-Lindau disease. Lancet
2003; 361(9374):2059-2067.
22. Megerian CA, McKenna MJ, Nuss RC, Maniglia AJ, Ojemann RG, Pilch BZ et al. Endolymphatic Sac Tumors:
Histopathologic Confirmation, Clinical Characterization, and Implication in von Hippel-Lindau Disease.
Laryngoscope 105, 801-808. 1995.
23. Mukherji SK, Albernaz VS, Lo WW, Gaffey MJ, Mergerian CA, Feghali JG et al. Papillary endolymphatic sac
tumours: CT, MR imaging, and angiographic findings in 20 patients. Radiology 202[3], 801-808. 1997.
24. Murphy FM, Smirniotopoulos JG. Hemangioblastoma: Radiologic-Pathologic Correlation. Paper Presented at the
87th Annual Meeting of the Roentgen Ray Society . 1987.
25. Neumann H. Basic criteria for clinical diagnosis and genetic couselling in von Hippel-Lindau Syndrome. VASA
1987; 16:220-226.
26. Nixon JR, Houser OW, Gomez MR, Okazaki H. Cerebral Tuberous Sclerosis: MR Imaging. Radiology 170:869-
873, 1989.
27. Nixon JR, Houser OW, Gomez MR, Okazaki H. Cerebral Tuberous Sclerosis: MR Imaging. Radiology 1994;
170:869-873.
28. Ouallet JC, Marsot-Dupuch K, Van Effenterre R, Kujas M, Tubiana JM. Papillary adenoma of endolymphatic sac
origin: a temporal bone tumor in von Hippel-Lindau disease. Case report. J Neurosurg 1997; 87:445-449.
29. Ozek MM, Ozek E, Pamir MN, Ozer AF, Erzen C. Subependymal giant cell astrocytomas in tuberous sclerosis.
Turk J Pediatr 1993; 35:145-150.

1208 Neuroradiology
30. Shepherd CW, Scheithauer BW, Gomez MR, Altermatt HJ, Katzmann JA. Subependymal giant cell astrocytoma.
Neurosurg 1991; 28(6):864-868.
31. Sperner J, Schmauser I, Bittner R, Henkes H, Bassir C, Sprung C et al. MR-Imaging Findings in Children with
Sturge-Weber Syndrome. Neuropediatrics 1990; 21:146-152.
32. Stimac GK, Solomon MA, Newton TH. CT and MR of Angiomatous Malformations of the Choroid Plexus in
Patients with Sturge-Weber Disease. AJNR 1986; 7:623-627.
33. Sullivan TJ, Clarke MP, Morin JD. The ocular manifestations of the Sturge-Weber syndrome. J Pediatr Ophthalmol
Strabismus 1992; 29(6):349-356.
34. Tibbs Jr. RE, Bowles Jr. AP, Raila FA, Fratkin JD, Hutchins JB. Should Endolymphatic Sac Tumors Be Considered
Part of the Von Hippel-Lindau Complex? Pathology Case Report. Neurosurgery 40[4], 848-855. 1997.
35. Tishler PV. A Family with Coexistent von Recklinghausen's Neurofibromatosis and von Hippel-Lindau's Disease.
Neurology 25:840-844, 1975.
36. van der Hoeve T. Eye Diseases in Tuberose Sclerosis of the Brain and in Recklinghausen's Disease. Transactions of
the Ophthalmological Society of the United Kingdom 43; 1923;534-541.
37. Wippold FJ, Baber WW, Gado M, Tobben PJ, Bartnicke BJ. Pre- and Postcontrast MR Studies in Tuberous
Sclerosis. J Comp Assist Tomogr 1992; 16:69-72.
38. Wippold II FJ, Baber WW, Gado M, Tobben PJ, Bartnicke BJ. Pre- and Postcontrast MR Studies in Tuberous
Sclerosis. J Comp Assist Tomogr 1992; 16:69-72.
39. Wylie IG, Jeffreys R, MaClaine GN. Cerebral hemangioblastoma. Br J Radiol 1973; 46:472-476.
Neuroradiology 1207
Subarachnoid Hemorrhage and
Intracranial Aneurysms
Mary E. Jensen, MD
Epidemiology
• Most common cause of non-traumatic SAH is aneurysmal rupture
• Annual incidence of aneurysmal SAH: 1:10,000
➢ Higher than primary brain tumors, MS
➢ Remained stable over last 30 yrs
➢ Females 1.6 x over males
➢ Blacks 2.1 x over whites
➢ Higher in Japan and Finland
• Accounts for 2-5% of all new strokes each yr
➢ 21,000 – 33,000 Americans
➢ Average age much lower than other strokes
❖ Peaks in 6th decade
Epidemiology - Prevalence of Unruptured Aneurysms

• Common incidental finding
➢ 3.6% on prospective autopsy series
➢ 6% on prospective angiography series
➢ Highest in patients with AD polycystic kidney disease, familial
predisposition, atherosclerosis
• Multiplicity
➢ 20%-30% of patients have multiple aneurysms Figure 5-16-1
❖ Usually 2 or 3
Types of Aneurysms
[Figures 5-16-1 and 5-16-2]
• Saccular (“berry”)
➢ 90% of all aneurysms
• Fusiform
➢ Serpentine: Partially thrombosed
aneurysm containing tortuous
vascular channels
➢ Cirsoid: Dilated, elongated and
tortuous Saccular, paraophthalmic (left) Fusiform, vertebral (center)
• Dissecting Serpentine, MCA (right)
• Blister/bleb
Figure 5-16-2
Non-Modifiable Risk Factors
• Personal history of SAH
➢ Risk of developing a new aneurysm: 2%
❖ Annual incidence of SAH: 6:10,000
• Family history of SAH
➢ 5%-20% of patients with SAH have a positive
family history
❖ First-degree relatives of pts with SAH have 3-
7X increased risk
➢ Second-degree relatives have same risk as
general population
➢ Probably an autosomal transmission that does
not follow a specific mendelian model
• Female gender Dissecting, PICA (left) Blister-bleb, AComA (right)
➢ Before the 5th decade, the risk is higher in men
➢ Risk is greater in post-menopausal women than men
❖ Supplemental low-estrogen hormones may impart some protection
Subarachnoid Hemorrhage-Intracranial Aneurysms 1208

1210 Neuroradiology
Non-Modifiable Risk Factors Figure 5-16-3
• Age
➢ Very rare in children
• Heritable disorders of connective tissue
➢ AD polycystic kidney disease
➢ Ehlers-Danlos disease IV
➢ Neurofibromatosis type 1
➢ ? Fibromuscular dysplasia, Marfan’s
syndrome
• Associated with anatomic variants
➢ Persistent trigeminal artery
➢ Fenestrations
➢ Azygous ACA
Mid-basilar aneurysm with severe distal stricture (arrows)
Aneurysms in Children
• Incidental aneurysms rarely found at autopsy Figure 5-16-4
• Etiology: trauma, infection, congenital
• Location, size, age and presentation are
different from adults
• Age of presentation bimodal
➢ 0-6 yrs, then 8-adolescence
• Posterior circulation aneurysms 3x more
prevalent
• Large/giant aneurysms more common
➢ greater proportion of giant
aneurysms, pts < 2 yrs
47 y.o. female with Marfan’s and rapidly growing mid-basilar
Fusiform Basilar Aneurysm in a aneurysm
Child [Figure 5-16-3]
Aneurysm Associated with Connective Tissue Disorder

[Figure 5-16-4]
Aneurysm Associated with Anatomical Variants [Figure 5-16-5]
Modifiable Risk Factors

• Smoking
➢ Only risk factor that has been consistently identified in all populations
studied
➢ Risk is 3-10X higher than non-smokers
➢ Risk is proportional to number of cigs
➢ Increased risk of new aneurysm formation in pts with SAH who continue to
smoke
• Hypertension
➢ Probably a risk factor for both SAH and aneurysm formation
• Alcohol consumption
➢ Heavy, binge drinking Figure 5-16-5
• Cocaine use
• ? Hypercholesterolemia, DM, obesity
Bilobed vertebrobasilar junction aneurysm

associated with fenestration (left).
Large aneurysm associated with an azygous ACA
(right)
Neuroradiology 1209
1211 Subarachnoid Hemorrhage-Intracranial Aneurysms
Pathogenesis: Saccular Aneurysms
• Congenital defect in tunica media no longer considered the cause of aneurysm
formation
➢ Gaps in muscles layers are also seen in unaffected individuals
➢ In aneurysms, the gap is not at the neck but in the sac
• Acquired changes due to environmental factors (HTN, smoking, EtOH abuse)
are more likely
➢ Formation of intimal thickening proximal and distal to branch points
❖ May cause increased strain in the more elastic portion of the wall
➢ Structural abnormalities in structure proteins of the extracellular matrix
Figure 5-16-6
Pathogenesis: Saccular Aneurysms
Hemodynamic factors
• Wall shear stress
➢ Frictional force of viscous blood
❖ High WSS fragments the internal elastic lamina
- Initiation of aneurysm formation
❖ Low WSS degenerates endothelial cells via apoptosis
- Responsible for aneurysm growth/rupture
• Increased flow
➢ 10%-20% of patients with brain AVMs have aneurysms
Pathogenesis: Fusiform Aneurysms

• Fusiform
➢ Four major histological findings
❖ Fragmentation of internal elastic lamina Double channel appearance of
❖ Neoangiogenesis within the thickened intima dissecting aneurysm aneurysm
❖ Intramural hemorrhage and thrombus formation
❖ Repetitive intramural hemorrhage from neovascularity within the
thrombus
Figure 5-16-7
➢ Similar histologic features seen with ASVD
ASVD starts with lipid deposition, not fragmentation of
the IEL
Pathogenesis: Dissecting Aneurysms

• Cystic medial necrosis
➢ Widespread disruption of the arterial wall
❖ Medial disruption with subadventitial
dissecting hemorrhage causing true lumen
stenosis AP and lateral views show “pearl and string”
❖ Formation of a dilated pseudoaneurysm appearance of dissecting aneurysm
covered only by thin adventitia
CTA and MRA of Dissecting Vertebral Aneurysm [Figure 5-16-6]
DSA of Dissecting Vertebral Artery Aneurysm [Figure 5-16-7]
• Subarachnoid hemorrhage
➢ Unique headache
➢ Nausea/vomiting
➢ Meningeal irritation
➢ Photophobia
➢ Focal or global neurological deficits
➢ Subhyaloid hemorrhages

1212 Neuroradiology
Subarachnoid Hemorrhage Patterns [Figure 5-16-8] Figure 5-16-8
• Mass effect
➢ Headache
➢ Focal neurological findings
➢ Seizures
➢ Risk of rupture of unruptured aneurysm causing
mass effect is 6%/yr
• Cerebral ischemia
➢ Distal embolization from intra-aneurysmal
thrombus
Giant ICA Terminus Aneurysm [Figure 5-16-9]
Outcomes of SAH
• 12% die before reaching hospital
• 40% of hospitalized patients die within first month
• >1/3 of survivors have major neurological deficits
➢ Unable to live independently
• Many “good outcomes” have persistent cognitive
AComA pattern (left images) - Basilar tip pattern
deficits
(right images)
Outcomes of SAH - Major factors associated with poor outcome
• Patient’s level of consciousness on
admission
Figure 5-16-9
➢ Based on the sum score of the
Glasgow Coma Scale
❖ Eye opening (4 pts), best motor
response (6 pts), best verbal
response (5 pts)
➢ Age
➢ Amount of blood on CT
➢ Predicts risk of delayed cerebral
ischemia using two parameters
❖ Amount of subarachnoid clot 59 y.o. female evaluated for headache and cognitive
❖ Ventricular hemorrhage dysfunction
➢ Risk is additive
Clinical Grading Scale [Figure 5-16-10]
Radiologic Grading Scale [Figure 5-16-11]
Neuroradiology 1211
SAH-induced Vasospasm Figure 5-16-12
• Occurs angiographically in 30%-70% of patients
• Clinical symptoms seen in 20%-45% of patients
• Adds 10%-20% significant morbidity/mortality
• Smooth muscle constriction and vessel wall edema,
infiltration and fibrosis leads to luminal narrowing
and decreased compliance
• Time course
➢ Peak: 7-10 days
• Treatment
➢ Fluid status, blood pressure, calcium channel Severe vasospasm of the supraclinoid ICA and M1
blockers segment leading to poor perfusion of the right MCA
➢ Induced hypertension territory
➢ Angioplasty, intra-arterial infusion of vasodilators
SAH-induced Vasospasm [Figure 5-16-12] Figure 5-16-13
Risk of Unruptured Aneurysm [Figure 5-16-13]
Diagnostic Testing: Non-contrast Head CT

• First imaging study
➢ Detect 98%-100% of cases in first 12 hours
➢ Sensitivity goes down to 30% at 2 wks
• Reportable findings
➢ Presence, amount, location of blood
❖ Increased density in subarachnoid space
❖ Predominately in basilar cisterns
➢ Intraparenchymal hematoma
➢ Hydrocephalus
➢ Cerebral edema, herniation
➢ Negative image of aneurysm in Figure 5-16-14
SAH
CT of Subarachnoid Hemorrhage
[Figure 5-16-14]
CT Findings of Ruptured
Aneurysm [Figure 5-16-15]
Diagnostic Testing:
Lumbar puncture
Usually done when CT is negative or Acute SAH (left image)-SAH 5 days post bleed with CTA
equivocal showing PComA aneurysm (right two images)
• Should be done 6-12 hours after event
➢ Time it takes for xanthochromia to Figure 5-16-15
occur
➢ Xanthochromia is diagnostic
❖ Detectable in all patients
between 12 hrs and 2 wks
• Findings
➢ Elevated opening pressure
➢ Elevated RBCs that do not clear
❖ Unreliable way to r/o traumatic
tap
➢ Xanthochromia
NCCT shows extensive SAH, intraparenchymal hematoma with
fluid-fluid level, uncal and subfalcine herniation, small amount
of intraventricular blood, and an MCA aneurysm (arrow)

1214 Neuroradiology
Diagnostic Testing: MRI Figure 5-16-16
• FLAIR detects early SAH as well as CT
➢ Impractical
❖ Equipment availability
❖ Patient motion
➢ Better than CT for detecting older SAH
❖ Great for patients with negative CT, positive
LP who are not referred immediately
SAH on CT and MRI FLAIR [Figure 5-16-16]
Diagnostic Testing: CTA

• Advantages
➢ High sensitivity (97%-100%) CT three days and MR four days post-SAH
➢ Equal to IA DSA (arrows) with interval development of
➢ Good even if SAH present intraventricular hemorrhage seen on MR
➢ Source images, reconstructions
• Disadvantages Figure 5-16-17
➢ Sensitivity varies with size
➢ Use of iodinated contrast and
radiation
➢ Negative study requires IA DSA
➢ Technical issues
❖ Post-processing time is
substantial
❖ Venous contamination with poor
cardiac output
CTA with Active Extravasation CTA shows the location, size and relationship of the aneurysm
[Figure 5-16-17] to the surrounding branches, and active extravasation into the
hematoma
CTA of Small Aneurysms
[Figure 5-16-18] Figure 5-16-18
Diagnostic Testing: MRA

• Advantages
➢ Non-invasive
➢ High sensitivity
❖ 86%-100% in aneurysms 3-5 mm
❖ 81%-100% in cases of SAH
➢ Multiplanar viewing
• Disadvantages
➢ Complex/disturbed flow degrades image
➢ Availability of equipment
CTA of Small Aneurysms
➢ Patient movement
❖ Anesthesia needed for uncooperative patients
MRA of Multiple Aneurysms [Figure 5-16-19] Figure 5-16-19
Diagnostic Testing: DSA

• Gold standard
• Carries risk of complication
➢ 1.8% transient or permanent
➢ SAH patients hypercoagulable
• Detects smallest aneurysms
• Gives important information for treatment decisions
Following coiling of the three aneurysms larger
than 2 mm, the MRA shows no change in the two
untreated aneurysms
Neuroradiology 1213
DSA: Evaluation of Aneurysm Figure 5-16-20
• Location and number
• Size and configuration
• Characteristics of aneurysm neck
➢ neck to dome ratio; absolute size of neck
➢ slit, oval, round, wide
➢ relationship to adjacent vessels
• Suspected rupture site (“tits”)
• Collateral circulation distal to aneurysm
• Hemodynamics
• Calcification
• Intraluminal thrombus
Location
• Anterior circulation (80%-85%)
➢ ICA/PComA junction
➢ AComA complex
➢ MCA trifurcation
• Posterior circulation (15%-20%)
➢ Basilar terminus (5%)
➢ PICA
➢ SCA
Common locations for aneurysms on the anterior
Aneurysm Locations: Anterior Circulation circulation
[Figure 5-16-20]
Figure 5-16-21
Aneurysm Locations: Posterior Circulation
[Figure 5-16-21]
Aneurysm Measurements
• Aneurysm
➢ Small: less than 10 mm
➢ Large: 10-25 mm
➢ Giant: > 25 mm
• Aneurysm neck
➢ Absolute size
❖ Small: 4mm or less
➢ Dome to neck ratio Common locations for aneurysms on the posterior circulation
❖ Small: 2:1 or greater
Aneurysm Sizes [Figure 5-16-22]

Figure 5-16-22
Small superior hypophyseal (left)

Large paraophthalmic (center)
Giant ICA terminus (right)

1216 Neuroradiology
3D Angiography [Figure 5-16-23] Figure 5-16-23
Rupture Sites [Figure 5-16-24]
Imaging Algorithm for Suspected

SAH
Non-contrast head CT
• SAH
➢ CTA or DSA
If negative, repeat CTA in 1-3 weeks
If negative, image the brain and spinal cord
• No SAH
➢ LP
Abnormal or equivocal, go to SAH algorithm
3D angiography better defines the aneurysm neck than biplane
DSA
Imaging Algorithm for Screening
• For high risk patients Figure 5-16-24
➢ Family history of intracranial
aneurysms
❖ Asymptomatic individuals with
two or more affected members
❖ Non-invasive imaging every >1
year, < 5 years
➢ Asymptomatic adults with
autosomal dominant polycystic
kidney disease
❖ 5%-10% asymptomatic patients
have aneurysms
❖ Clustering in some families with
20%-25% with aneurysms
Paraophthalmic (left)
AComA (center)
Other Causes of SAH Basilar tip (right)
• Non-aneurysmal perimesencephalic
SAH
• Infectious intracranial aneurysms
• Brain AVMs
➢ Flow related aneurysms
➢ Intra/perinidal aneurysms
• Dural AVMs
• Intracranial dissections
➢ Spontaneous
➢ Iatrogenic
• Spinal vascular malformations
Non-aneurysmal Perimesencephalic Hemorrhage

• Responsible for 10% of non-traumatic SAH
• Defined only by the characteristic location of blood and lack of aneurysm
➢ Blood confined to perimesencephalic cistern
➢ Centered anteriorly
➢ May have small amount of sedimentation in posterior horns
➢ 2.5%-10% of posterior fossa aneurysm hemorrhages mimic this pattern
➢ Gradual headache
➢ Focal findings, LOC uncommon and transient
➢ Seizure at presentation essentially excludes the diagnosis
➢ 1/3 with transient amnesia
• Outcomes
➢ Short convalescence
➢ No rebleeding
➢ No symptomatic vasospasm
Neuroradiology 1215
Non-aneurysmal Perimesencephalic Hemorrhage [Figure 5-16-25] Figure 5-16-25
Infectious Intracranial Aneurysms

• Rare- 2%-6% of all brain aneurysms
• Presentation: stroke,seizure, SAH
• Pathology
➢ Septic embolization of lumen or vasovasorum
❖ Focal arteritis, necrosis, and aneurysm
formation
• Location
➢ Distal MCA>PCA and ACA
• Treatment
➢ Resection, parent artery occlusion Non-aneurysmal perimesencephalic hemorrhage
➢ Antiobiotic therapy
Infectious Intracranial Aneurysms [Figure 5-16-26]

Figure 5-16-26
Other Causes of SAH
• Saccular aneurysm of spinal artery
• Atrial myxoma
• Pituitary apoplexy
• Coagulation disorders
• Superficial siderosis of the CNS
Aneurysms: Treatment Options

• Nothing
• Follow
➢ Very small unruptured aneurysms Separate patients with septic aneurysms
➢ Unruptured aneurysms in cavernous (Courtesy A. Brooks, M.D.)
carotid/carotid cave
• Surgical clipping Figure 5-16-27
• Endovascular treatment
➢ Reconstructive: coiling +/- stent
placement/balloon remodeling
❖ Saccular aneurysms
➢ Deconstructive: parent artery
occlusion
❖ Giant, dissecting, septic
Treatment Outcomes
• ISAT trial Permanent occlusion of the left ICA with detachable balloons
➢ Comparative study of coiling vs. results in flow perpendicular to the neck of the aneurysm and
clipping clotting of the dome
❖ 23.9% relative/7.4% absolute
risk reduction for coiling vs. clipping
➢ Controversial study Figure 5-16-28
• Should patients be offered the option of
coiling vs. clipping in the acute setting?
Deconstructive Therapy
[Figures 5-16-27 and 5-16-28]
A paraophthalmic aneurysm treated by endosaccular occlusion

with coils
Subarachnoid Hemorrhage-Intracranial Aneurysms 1218 Neuroradiology

References
1. Aryan H, Giannotta SL, Fukushima T, et al. Aneurysms in children: review of 15 years experience. J Clin
Neurosci 2006;13(2):188-92.
2. Claassen J, Bernardini GL, Kreiter K, et al. Effect of cisternal and ventricular blood on risk of delayed cerebral
ischemia after subarachnoid hemorrhage: the Fischer scale revisited. Stroke 2001; 32:2012-20.
3. Hamada Y, Mannoji H, Kaneko Y. A ruptured dissecting aneurysm of the vertebral artery: comparison of the
angiographic and histological findings. Neuroradiology 2001; 375-8.
4. Hoh BL, Cheung AC, Rabinov JD, et al. Results of a prospective protocol of computed tomographic angiography
in place of catheter angiography as the only diagnostic and pretreatment planning study for cerebral aneurysms by
a combined neurovascular team. Neurosurgery 2004; 54(6):1329-40.
5. Molyneux A, Kerr RS, Yu LM, et al. International subarachnoid aneurysm trial (ISAT) of neurosurgical clipping
versus endovascular coiling in 2143 patients with ruptured intracranial aneurysms: a randomised comparison of
effects on survival, dependency, seizures, rebleeding, subgroups, and aneurysm occlusion. Lancet 2005;
366(9488):809-17.
6. Nakatomi H, Segawa H, Kurata A, et al. Clinicopathological study of intracranial fusiform and dolichoectatic
aneurysms: insight on the mechanism of growth. Stroke 200; 31:896-900.
7. Schievink W. Intracranial aneurysms. [Review] NEJM 1997; 336(1):28-40.
8. Shojima M, Oshima M, Takagi K, et al. Magnitude and role of wall shear stress on cerebral aneurysm:
computation fluid dynamic study of 20 middle cerebral artery aneurysms. Stroke 2004; 35:2500-05.
9. Suarez JI, Tarr RW, Selman WI. Aneurysmal subarachnoid hemorrhage. Review NEJM 2006; 354:387-96.
10. Van Gijn J, Rinkel GJ. Subarachnoid hemorrhage: diagnosis, cause and management. [Review Article] Brain
2001; 124:249-78.
11. Velthuis BK, Rinkel GJ, Ramos LM, et al. Subarachnoid hemorrhage: aneurysm detection and preoperative
evaluation with CT angiography. Radiology 1998; 208: 423-430.
12. Wani AA, Behari S, Sahu RN, et al. Paediatric intracranial aneurysms. J Pediatr Neurosci 2006; 1:11-15.
13. Wiebers DO, Whisnant JP, Huston J 3d, et al. Unruptured intracranial aneurysms: natural history, clinical
outcome, and risks of surgical and endovascular treatment. Lancet 2003; 362(9378):103-110.
Neuroradiology 1217
Intracranial Vascular Malformations
Mary E. Jensen, MD
Classification of IVMs
• Traditional (path-based)
➢ Arteriovenous malformations
❖ Pial
❖ Dural
➢ Venous vascular malformations
❖ Venous angioma
➢ Capillary telangectasias
➢ Cavernous angiomas
• Functional (flow-based)
➢ AV Shunting
❖ Pial AVM
❖ Cerebral AVF
❖ Dural AVF
➢ Non-shunting
❖ Capillary malformations
❖ Venous malformations
❖ Cavernous malformations
Epidemiology
• Detection rate of AVM
➢ 1.1:100,000 (excluding autopsy cases)
➢ 2.1:100,000 (including autopsy cases)
➢ 1.2:100,000 person-years (symptomatic)
• Prevalence
➢ 0.1% of US population (300,000 people)
➢ Point prevalence 18:100,000 adults
• Account for
➢ 1%-2% of all strokes
➢ 3% of strokes in young adults
➢ 9% of subarachnoid hemorrhage
➢ 4% of all intraparenchymal hemorrhages
❖ 1/3 of young adult hemorrhages
Etiology
• Congenital
➢ Sporadic
• Genetic
➢ Hereditary hemorrhagic telangectasia
❖ 4%-13% with cerebral AVMs
❖ ?ENG, ACVR1 gene mutations
❖ Encode proteins in TGFB1 receptor complexes
➢ Neurocutaneous disorders
Pathogenesis
• Wedge-shaped abnormal tangle of arteries and veins
➢ Arteries and veins linked by fistulae
➢ No normal capillary bed
➢ Deficient muscularis in small arteries
• Hemodynamic effects
➢ Fistulous effects
❖ High flow, rapid shunting
❖ Induced hypotension in feeders and adjacent areas (“arterial steal”)
➢ Opening of collateral pathways, “angiomatous change”
➢ Dysplastic appearance of feeding pedicles, “flow-induced angiopathy”
❖ Luminal dilatation or stenosis
❖ Aneurysms
Intracranial Vascular Malformations 1218
1220 Neuroradiology
• Usually present before 40 years of age
➢ Men and women equally affected
• Hemorrhage is #1 complication (>50%)
➢ 2%-4% yearly rupture rate
❖ 6%-18% annual rate after first bleed
- Increased rate for the first year reported but not consistently noted
➢ 10%-18% mortality from hemorrhage
❖ 1.0%-1.5% annual rate
• Seizures: 20%-25%
➢ Generalized more common than focal
• Headache: 15%
• Focal neurologic deficit: <5%
➢ Steal phenomenon is uncommon
• Pulsatile tinnitus
• Children (< 2 yr)
➢ Large head from hydrocephalus
➢ seizures
Location
• Supratentorial (85%), infratentorial (15%)
➢ Cortical AVMs (72%)
❖ Gyral, sulcal, mixed
➢ Subcortical AVMs (1%) Figure 5-17-1
➢ Deep AVMs (27%)
❖ Subarachnoid, parenchymal,
plexal, mixed
Hemorrhage Predictors
• Many are not consistent
➢ Radiologic findings
➢ Prior hemorrhage
➢ Small AVM in diameter or volume
➢ Increased feeding artery pressures
➢ Periventricular/intraventricular
location
An enlarged lenticulostriate running through the ventricle is the
Radiologic Predictors presumed source of hemorrhage
• Features that may result in a higher risk
of bleeding
➢ Arterial aneurysms (10%) Figure 5-17-2
➢ Intranidal aneurysms (20%-58%)
➢ Arterial supply from perforators
➢ Location
❖ Intraventricular/periventricular
❖ Basal ganglia, thalamus
➢ Deep venous drainage
➢ Single venous drainage outlet
➢ Venous stenosis
Intraventricular Hemorrhage
[Figure 5-17-1] CTA, MRA and DSA all show intranidal aneurysm within a
small AVM
Intraparenchymal Hemorrhage
[Figure 5-17-2]
Neuroradiology 1219
1221 Intracranial Vascular Malformations
Hemorrhage Risk: Summary
• Lowest-risk group (1%)
➢ No history of prior hemorrhage
➢ >1 draining vein
• Highest-risk group (8.9%)
➢ History of prior hemorrhage
➢ Single draining vein
➢ Diffuse nidus
AVM Grading
• There are many grading systems
• The most commonly used is the Spetzler-Martin system
➢ Size (<3 cm = 1, 3-6 cm = 2, >6 cm = 3)
➢ Eloquence of surrounding brain
(non-eloquent = 0, eloquent = 1)
➢ Pattern of venous drainage (superficial = 0, deep = 1)
Spetzler-Martin Grading System

• This system is used to stratify surgical outcome
• Grades I through II
➢ Extremely low rates of surgically related morbidity and mortality
• Grade IV and V
➢ High risk
Spetzler-Martin Grading Scale

• Grade III
➢ Heterogeneous group
❖ S1V1E1 same risk as I/ II (III-)
- Microsurgery
❖ S2V0E1 same risk as IV/V (III+)
- Manage conservatively
❖ S2V1E0
- Intermediate risk Figure 5-17-3
- Judicious selection for
surgery
Radiographic Evaluation
• CT
➢ Usual first study
➢ Hemorrhage, calcifications,
parenchymal changes, iso- or
hyperdense serpentine structures
➢ Contrast study outlines boundaries
• CTA
➢ Vascular elements NCCT shows enlarged MCA trunk and sylvian arteries,
❖ Location of feeding intraventricular hemorrhage, focal atrophy and hyperdense
arteries/draining veins vessels
❖ Associated aneurysms
➢ Volumetric determination Figure 5-17-4
CT of Brain AVM [Figure 5-17-3]
CTA of Brain AVM [Figure 5-17-4]
CTA shows AVM components and their relationship to the

parenchymal hemorrhage

1222 Neuroradiology
Radiographic Evaluation Figure 5-17-5
• MR
➢ Location and topography
➢ Presence or absence of acute, subacute or
chronic hemorrhage
➢ Associated parenchymal changes such as
edema, ischemia, gliosis, atrophy, mass effect,
radiation effects
• MRA
➢ Same as CTA
➢ Useful in stereotactic radiosurgery planning
MRI of Brain AVM [Figure 5-17-5]
MRA of Brain AVMs [Figure 5-17-6]
• Angiography
➢ Arterial supply, regional and individual
➢ High flow arteriopathy
❖ stenosis
❖ dolichoectasia
❖ flow related aneurysms (10%)
➢ Arterial supply to the brain
T1 images without and with Gd (left images)
❖ Pial
T2 and FLAIR images (right images)
❖ Dural
➢ Assessment of nidus
❖ plexiform vs. fistulous Figure 5-17-6
❖ intra-nidal aneurysms and ectasias
❖ true nidus vs. angiomatous change
DSA of Cerebral AVMs [Figure 5-17-7]
Nidal Components [Figure 5-17-8]
En Passage Vascular Supply [Figure 5-17-9]
AVM Associated Aneurysms [Figure 5-17-10]

MRA of shows extent of parietal AVM, and the
Angiomatous Change [Figure 5-17-11] enlarged feeding arteries and draining veins
Figure 5-17-8
Figure 5-17-7
Superselective
injections of a
temporal lobe AVM
(A) show different
nidal components
including a
macrofistula (B) and a
racemose nidus (C)
Typical appearance of gyral (left) and sulcal (right)

AVM
Neuroradiology 1221
Radiographic Evaluation Figure 5-17-9
• Angiography
➢ Venous drainage, regional and individual
➢ High-flow venopathy
❖ dural sinus high-flow
❖ venous thrombosis
❖ venous enlargement, stenoses, varix
➢ Normal drainage of the brain
Venous Abnormalities [Figure 5-17-12]
Treatment
• Controversial
➢ Observation
➢ Microsurgery Superselective study (right) of temporal AVM
➢ Embolization shows multiple “en passage” feeders with distal
➢ Stereotactic radiosurgery arterial supply to normal brain
➢ Combination therapy
Figure 5-17-10
Arterial feeder (left) Perinidal (center) Intranidal (right)
Figure 5-17-11
Figure 5-17-12
Difference between angiomatous change (circle)

and nidus (box)
Two patients with high-flow venopathy—a venous
aneurysm in a deep AVM (left) and a venous varix
with a stricture (right, arrow)

1224 Neuroradiology
Pre-Radiosurgery Embolization [Figure 5-17-13] Figure 5-17-13
Intracranial AVFs
• Dural
➢ Most common type of cerebral AV fistula
➢ Shunt occurs primarily from dural arteries to
dural sinuses or cortical veins
• Cerebral
➢ Rare
❖ Vein of Galen malformation
❖ Pial
Pre- and post-embolization of distal pericallosal
artery with coils and NBCA
Etiology/Pathogenesis
• Adults (Dural AVF)
➢ Acquired lesions
❖ Present in later life (40s-60s) Figure 5-17-14
❖ Shunting within dural/venous wall
❖ Sinus thrombosis
- Environment conducive to the
development of DAVF
- Triggered by factors which stimulate
angiogenesis
• Children (Pial AVF)
➢ Congenital lesions
❖ ?vascular remodeling of the venous
endothelial cells in the capillary bed caused
by a trigger such as hypoxia
Newborn with congestive heart failure and cranial
Pial A-V Fistula [Figure 5-17-14] bruit
Location of DAVFs
• Transverse - sigmoid sinus: 62.6 %
• Cavernous sinus: 11.9 %
• Tentorial - incisural: 8.4 %
• Convexity - sagittal sinus: 7.4 %
• Orbital - anterior falx area: 5.8 %
• Sylvian - middle fossa area: 3.7 %
• Others: marginal sinus, etc
Classification of DAVFs
• Borden, Djindjian and Merland, Cognard
➢ Venous drainage
❖ Sinus vs. cortical veins
➢ Direction of flow
❖ Antegrade, retrograde, both
➢ Involvement of cortical venous drainage
❖ Benign vs. aggressive
❖ Determines risk
Borden Classification
Neuroradiology 1223
Benign: All Classifications [Figure 5-17-15] Figure 5-17-15
Aggressive: All Classifications [Figure 5-17-16]
• Symptoms and signs vary with location
• Tinnitus, bruit
➢ Orbital congestion
➢ Focal neurological deficits
➢ Global neurological deficits
➢ Hemorrhage Benign transverse sinus DAVF with fistulous flow
❖ ICH: 35%-42% overall; only to sinus
75%-95% in tentorial, ACF
Figure 5-17-16
Hemorrhage Associated with DAVF
[Figure 5-17-17]
Clinical Outcomes
• Without cortical venous involvement
➢ Symptom improvement or resolution
❖ No treatment: 81%
❖ Treatment: 86%
• With cortical venous involvement
➢ non-hemorrhagic neurological deficit: 6.9%/yr
➢ Hemorrhage: 8.1%/yr Aggressive transverse sinus DAVF with all fistulous
➢ Mortality: 10.4%/yr flow into the cortical veins
• Malignant transformation of benign lesion: <1%
Figure 5-17-17
• Acute symptoms
➢ CT to rule out hemorrhage
➢ MRI/A, CTA to show prominent vessels
❖ Not adequate for demonstration of shunt
➢ DSA
❖ Confirm diagnosis
❖ Identify angioarchitecture
❖ Evaluate hemodynamics
• Chronic symptoms
➢ Contrast CT/MR
➢ DSA
Tentorial DAVF with SAH (left) and transverse
Aggressive DAVF [Figure 5-17-18] sinus DAVF with ICH (right)
Figure 5-17-18
Treatment
• Benign
➢ Observation
• Aggressive
➢ Endovascular therapy
❖ Transvenous, transarterial,
combined
➢ Surgery
❖ Failed endovascular therapy
❖ Isolated sinus that required
direct puncture/exposure
Diffusion study shows temporal lobe edema and old
Developmental Venous Anomaly hemorrhage; Gd MRI shows marked venous congestion and a
• Most common type of vascular multi-channel left transverse sinus; DSA confirmed
malformation development of a DAVF

1226 Neuroradiology
➢ Accounts for 60% of CVM
➢ 2.5%-9% prevalence on enhanced MR
• Congenital
➢ Develop during 30th to 45th day as embryo
➢ Focal underdevelopment of superficial or deep “adult” veins
➢ Anomalous medullary veins converge into a centrally located collector vein
Caput medusa
Pathology
• Veins are closely aggregated and dilated
➢ lack muscular and elastic fibers
➢ abnormally hyalinized
• Normal adjacent arteries
• Adjacent parenchyma
➢ Normal
➢ Gliosis, neuronal degeneration, demyelination
➢ May be associated with focal neuronal migrational abnormalities
• Incidental finding in most cases
➢ Associated with H/N vascular malformations
• Rarely cause neurologic symptoms
➢ Posterior fossa
❖ Dizziness, ataxia, diplopia
➢ Cerebrum
❖ Seizures, headaches, focal deficits
• Hemorrhage
➢ Not the DVA that hemorrhages, but the associated cavernoma
• Treatment
➢ Surgery may be required to remove hemorrhage/cavernoma
➢ Leave DVA
Imaging Findings
• NCCT
➢ Normal
➢ Ill-defined hyperdense area without edema or mass effect
• CECT
➢ Diffuse enhancement of linear vessels adjacent to the ventricle
❖ Stellate pattern
❖ Converge on collector vein
❖ Well-visualized on CTA
• MR
➢ Variable degrees of T2 and T1 prolongation in the adjacent parenchyma
❖ Due to increased blood pool
➢ Signal intensity void in draining vein on T2
➢ Occasionally gliosis
➢ Associated with cavernomas in 8%-33% of DVAs
➢ CE MR
❖ Dilated deep veins converge on a collector vein
❖ Follows a transhemispheric course to a normal vein
• Angiography
➢ Pathognomonic
➢ Normal arterial phase
➢ Radially oriented dilated medullary veins converge on an enlarged
transcortical draining vein
❖ Caput medusa opacifies at the same time as normal veins
❖ Collector vein seen on late venous phase
➢ Location
❖ In the deep white matter near the margin of an adjacent ventricle
❖ Frontal>parietal>brachium pontis/dentate
❖ Frontal lobes may opacify earlier and show a capillary blush
Neuroradiology 1225
Developmental Venous Anomaly [Figure 5-17-19]
DSA of Developmental Venous Anomaly [Figure 5-17-20]
Cavernous Angioma
• Prevalence: 0.5%-0.7% on MR/autopsy
• Account for 5%-13% of cerebral VMs
• Sporadic
• Familial form
➢ Autosomal dominant, Hispanic
➢ Multiple lesions
➢ Increased number of lesions with aging
➢ Correlation of symptoms with the presence of high signal intensity lesions
on MR
Figure 5-17-19
Pathology
• Gross findings
➢ Small, reddish-purple (“mulberry”)
lesions
➢ Few mms to several cms
➢ Multiple or single
➢ Often encapsulated and multilobar
➢ Occasionally calcified
➢ Often associated with DVA
➢ Found throughout the CNS
Pathology
• Histologic findings
➢ Thin-walled vascular sinusoids
➢ Endothelium lacks smooth muscle,
elastin, and intervening parenchyma
➢ Lacks blood brain barrier
➢ Surrounded by hemosiderin
deposits and gliosis
➢ May or may not be thrombosed
NCCT and T1 and T2 weighted MRI shows dystrophic
Clinical Presentation microcalcifications and/or hemosiderin staining in the right
• Usually present between age 30-50 basal gangliar region
years
• Symptoms
➢ Seizures
❖ Lesions usually in frontal or temporal lobe
➢ Focal neurologic deficits Figure 5-17-20
➢ Acute hemorrhage
❖ Risk is 0.25%-6% per year
❖ Increased risk with previous hemorrhage,
pregnancy
➢ Headache
Imaging Findings
• CT
➢ Variable density
❖ Hemorrhage
❖ Calcification
- Rim, coarse, stippled, granular DSA shows “caput medusa” in early venous phase
• DSA followed by filling of the collector vein
➢ Cavernous angioma not visualized
➢ Associated DVA

1228 Neuroradiology
CT of Cavernous Angioma [Figure 5-17-21] Figure 5-17-21
Imaging Findings - MRI

• Type 1
➢ Hyperintense core on T1
➢ Hyper or hypointense core on T2
➢ Corresponds to subacute
hemorrhage
• Type 2 “popcorn”
➢ Reticulated mixed signal on T1
➢ Reticulated mixed signal on T2 with
hypointense rim
CT findings include a hypodense center with rim calcification
➢ Corresponds to lesions with multiple
(left) and fine granular calcifications throughout the lesion
hemorrhages of various age
(right)
• Type 3
➢ Iso or hypointense on T1
➢ Hypointense lesion with hypointense rim on T2
➢ Corresponds to chronic hemorrhage with hemosiderin staining
• Type 4
➢ Not visible on T1 or T2
➢ Punctate hypointense lesion on GRE
➢ Corresponds to tiny lesion or telangiectasia
MRI of Cavernous Malformations Figure 5-17-22

[Figure 5-17-22]
Cavernous Malformation with DVA

[Figure 5-17-23]
Treatment
• Stereotactic microsurgery
➢ Reasons for surgery
❖ Repeat hemorrhage
T1, T2 and GRE images show a Type 2 lesion in the right
❖ Mass effect from enlarging
frontal lobe and a Type 4 lesion in the left occipital lobe
lesion
❖ Seizure focus
• Radiosurgery
➢ Controversial
➢ Associated with hemorrhage and
radiation-induced mass effect
• Follow
➢ Deep lesions
➢ Familial lesions
Figure 5-17-23
Capillary Telangiectasia
• Represents 16%-20% of all CVMs
➢ Nearly always asymptomatic
➢ Sx: headache, vertigo, ataxia, hearing loss
• Congenital vs. acquired lesions
➢ Obstructed venous drainage; radiation
• May be associated with VA, CM, AVM
➢ “Transitional malformations”
Gd MRA shows DVA associated with a cavernous

malformation
Neuroradiology 1227
Pathology
• Location
➢ Pons, cerebral/cerebellar hemispheres, spinal cord
• Histology
➢ Thin-walled, “capillary-type,” ectatic blood vessels
➢ Interspersed with normal brain
• Size
➢ Few millimeters to 2 cm
• Hemorrhage
➢ Rare
➢ Usually from an associated vascular malformation
Figure 5-17-24
Imaging [Figure 5-17-23]
• CT
➢ Usually normal
• MR
➢ T1: hypo- or isointense
➢ T2: iso- or slightly hyperintense
➢ GRE: hypointense
❖ Most consistent finding
❖ Due to intravascular
deoxyhemoglobin
➢ CE-MR
❖ Faintly enhance in a “stippled”
or “brushlike” pattern
❖ 2/3d with enlarged vessel
Typical MR findings in a capillary telangiectasia
References
1. Al-Shani R, Warlow C. A systematic review of the frequency and prognosis of arteriovenous malformations of the
brain in adults. Brain 2001; 124:1900-26.
2. Augustyn GT, Scott JA, Olson E, et al. Cerebral venous angiomas: MR imaging. Radiology 1985; 156:391–395
3. Camacho DL, Smith JK, Grimme JD, et al. Atypical MR imaging perfusion in developmental venous anomalies.
AJNR Am J Neuroradiol 2004; 25(9):549-52.
4. Gault J, Sarin H, Awadallah N, et al. Pathobiology of human cerebrovascular malformations: basic mechanisms
and clinical relevance. Neurosurgery 2004; 55(1):1-17.
5. Lai CW, Agid R, van den Berg R, ter Brugge K. Cerebral arteriovenous fistulas induced by dural arteriovenous
shunts. AJNR J Neuroradiol 2005; 26:1259-62.
6. Lawton MT, UCSF Brain Arteriovenous Malformation Study Project. Spetzler-Martin Grade II arteriovenous
malformations: surgical results and a modification of the grading scale. Neurosurgery 2003; 52(4):740-8.
7. Mast H, Young WL, Koennecke HC, et al. Risk of spontaneous haemorrhage after diagnosis of cerebral
arteriovenous malformation. Lancet 1997; 350:1065-8.
8. Ogilvy C, Steig P, Awad I, et al. Recommendations for the management of intracranial arteriovenous
malformations. A statement for health care professionals from a special writing group of the Stroke Council,
American Stroke Association. Circulation 2001; 103:2644-57.
9. Peebles TR, Vieco PT. Intracranial developmental venous anomalies: diagnosis using CT angiography. J Comput
Assist Tomogr 1997; 21(4): 582-6.
10. Sakata N, Takebayashi S, Kojima M, et al. Different roles of arteriosclerosis in the rupture of intracranial
dissecting aneurysms. Histopathology 2001; 38(4):325-37.
11. Stapf C, Mohn JP, Choi JH et al. Invasive treatment of unruptured brain arteriovenous malformations is
experimental therapy. Current Opinion in Neurology 2006; 19(1):63-8.
12. The Arteriovenous Malformation Study Group. Arteriovenous malformations of the brain in adults. NEJM 1999;
340(23):1812-18.
13. The Scottish Intracranial Vascular Malformation Group. Prospective, population-based detection of intracranial
vascular malformations in adults. Stroke 2003; 34:1163-69.

1230 Neuroradiology
Imaging of Intracranial Infections
Patricia A. Hudgins, MD
Summary
• Meningeal Disease
➢ Epidural abscess
➢ Subdural empyema
➢ Meningitis
• Parenchymal Infection
➢ Cerebritis to abscess
➢ Encephalitis – HSV, CJD, ADEM
➢ Parasitic – Cysticercosis, Lyme disease
➢ Tuberculosis
➢ Mycotic
➢ AIDS related infections
Intracranial Infection
• Location
➢ Intra-axial – Parenchymal Figure 5-18-1
➢ Extra-axial – Epidural, subdural, leptomeningeal
• Response to infection
➢ Edema and swelling
➢ Mass and mass effect
➢ Abnormal enhancement
➢ Chronic – Atrophy
Blood-brain barrier
• Tight junctions (no gaps) exist between normal endothelial cells
• Little communication between capillary & extracellular space or
neurons
• Infection results in loss of tight junctions, with increased
permeability of endothelial membranes = LOSS OF BBB
• Loss of BBB + increased local blood volume = ABNORMAL
ENHANCEMENT
Subdural empyema
Intracranial infection – Location Meninges (courtesy of Amirsys, Inc.)
• Dura mater –
➢ 2 layers
➢ Outer layer is calvarial periosteum
➢ Inner layer is separation between dura mater and arachnoid
• Collection between inner table of skull & dura is EPIDURAL
Figure 5-18-2
Intracranial infection – Location Meninges [Figure 5-18-1]
• Arachnoid Mater
➢ Thin connective tissue
➢ Parallels dura mater
• Collection between dura & arachnoid is SUBDURAL
• Subdural space is potential space w/ bridging veins
Intracranial infection – Location Meninges[Figure 5-18-2]

• Pia mater –
➢ Blood vessels
➢ Covers surface of brain
• Space between arachnoid & pia is SUBARACHNOID space
• LEPTOMENINGES Arachnoid + Pia
Leptomeningeal pattern of infection

(courtesy of Amirsys, Inc)
Neuroradiology 1229
1231 Imaging of Intracranial Infections
Extra-axial Infection Epidural [Figures 5-18-3 and 5-18-4] Figure 5-18-3
• Usually starts in paranasal sinuses or mastoids
• Low density on CT
• Enhancing periphery
• ? SI on T1
• ? SI on T2, FLAIR
• Enhancing dura
• Check for osteomyelitis of calvarium
Acquired CNS infections

• Meningitis and complications
• Cerebritis and abscess
• Encephalitis
• Parasitic, rickettsial, spirochetal infections
• TB, other granulomatous infections
• Immunocompromised host, especially AIDS
Epidural abscess with typical
Acute Bacterial Meningitis enhancing dural surface
• Organisms Figure 5-18-4
➢ Neonates: group B streptococcus
➢ Children: H. influenza
➢ Adults: Streptococcus pneumoniae
• Pathogenesis
➢ Hematogenous seeding, choroid, leptomeninges
➢ Contiguous spread from sinusitis, mastoiditis
➢ Neonatal meningitis - maternal GU infxn, PROM
• Clinical - H/A, neck stiffness, photophobia, cranial
nerve dysfunction, lethargy
Meningitis – role of imaging Epidural collection crosses midline. Subdural

Complications abscess does not cross midline, and can extend
• Hydrocephalus (especially communicating) along interhemispheric fissure
• Subdural effusions, empyema
• Venous sinus thrombosis/infarction Figure 5-18-5
• Arterial infarction
• Vasculitis
• Cerebritis or abscess
• Ventriculitis/ependymitis
ACUTE MENINGITIS: Patterns [Figure 5-18-5]

• Dura –Arachnoid
➢ Pachymeningitis
• Pia-Subarachnoid Space
➢ Leptomeningitis
Two patterns of meningitis
(courtesy of Amirsys, Inc).
Meningitis: CT Findings
• Early on, CT usually normal Figure 5-18-6
• Sulcal or cisternal effacement
• Pia/subarachnoid enhancement
• Hydrocephalus
• ? Source – Paranasal sinus, mastoid
Acute Meningitis: MR Findings [Figure 5-18-6]

• T1 and T2 images may be normal
• FLAIR – ? SI in SAS
• Gd - Leptomeningeal enhancement
• ? Hydrocephalus
• ? Infection source
Meningeal infection best
detected on FLAIR image, with
increased SI in subarachnoid
Imaging of Intracranial Infections 1230

1232 Neuroradiology
Acute Meningitis: MR Findings [Figure 5-18-7] Figure 5-18-7
• T1 and T2 images may be normal
• FLAIR – ? SI in SAS
• Gd - Leptomeningeal enhancement
• ? Hydrocephalus
• ? Infection source
Meningitis, complications
• Hydrocephalus
• Ependymitis
➢ Subependymal enhancement
➢ In HIV disease, diff dx is lymphoma
Meningitis, complications [Figure 5-18-8]

• Subdural effusions
• Often sterile
➢ If infected, restricted diffusion on DWI Meningitis with subarachnoid pattern
• H. flu of enhancement on T1 gad. Image
• More common in children
• Bilateral, resolve spontaneously
• CT/MR similar to CSF Figure 5-18-8
• Membranes may enhance
Meningitis, complications
• Infarction
• May be arterial or venous
• Well-demarcated – best way to differentiate from cerebritis
• Typical CT/MR features with restricted diffusion on DWI
Diffusion weighted imaging (DWI)

• Powerful tool, physiologic information
• Differentiates unrestricted from restricted free water diffusion
• “Restricted” diffusion
➢ Acute and subacute infarction
➢ Intracranial abscess
➢ Rare MS plaque
➢ Epidermoid (vs. arachnoid cyst) Bilateral sterile effusions after H. flu
meningitis
Meningitis, complications [Figure 5-18-9]
Summary Figure 5-18-9

➢ Epidural empyema
➢ Meningitis
➢ Tuberculosis
➢ Mycotic
➢ AIDS related infections Thalamic and basal ganglia infarctions due to
acute meningitis
Cerebritis to brain abscess
• Early and late cerebritis
• Early and late capsule/abscess
• Etiology
➢ Direct spread (sinus, mastoid, odontogenic)
➢ Hematogenous
➢ Surgery or trauma
➢ 25% - no source found
Neuroradiology 1231
Cerebritis to brain abscess Figure 5-18-10
• Early cerebritis
➢ 3-5 days after infection
➢ Unencapsulated – white cells, edema, necrosis,
petechial hemorrhage
• Late cerebritis
➢ 4-14 days after infection
➢ Poorly delineated rim, necrotic core, white
cells/inflammatory cells, granulation tissue
Cerebritis to brain abscess

• Early capsule
Abscess is typically associated with vasogenic
➢ 2-4 weeks
edema and rim enhancement
➢ Collagenous capsule +/- daughter abscesses
➢ Necrotic core
➢ Mild mass effect Figure 5-18-11
• Late capsule
➢ Weeks/months
➢ Thick capsule
➢ Edema, mass effect resolve
Abscess - pyogenic [Figure 5-18-10]

• Supratentorial is most common
• Usually solitary (may have small surrounding cysts)
• Streptococcus, Staphylococcus
• Infants – Citrobacter, Proteus, Pseudomonas,
Serratia
Abscess with restricted diffusion, confirmed on
ADC map
Abscess [Figures 5-18-11 and 5-18-12]
• Mass effect, edema, enhancement
• T2 WI – ? SI rim Figure 5-18-12
• DWI/ADC
➢ Restricted diffusion
• MR spectroscopy
➢ Lactate (1.3 ppm)
• Complications
➢ Herniation
➢ Intraventricular rupture
➢ Choroid plexitis
➢ Leptomeningitis
Encephalitis “Inflammation of the brain”

• Diffuse infection
• Post-infectious/immunization
➢ Acute disseminated encephalomyelitis
• Viral
➢ Herpes simplex virus
➢ Others: measles, mumps, etc.
Herpes encephalitis
• Adults - HSV-1 (oral) Frontal sinusitis resulting in frontal abscess, with
• Neonates – HSV-2 (genital) low signal rim on T2, and enhancement
• Confusion, progressing to coma
• 50%-70% mortality, especially when delay to diagnosis (most common cause
of fatal encephalitis)
• Necrotizing hemorrhagic encephalitis, often resulting in diffuse atrophy
• Axonal spread from reactivation in trigeminal ganglion

1234 Neuroradiology
Herpes encephalitis [Figure 5-18-13] Figure 5-18-13
• Predilection for limbic system
➢ Temporal lobes
➢ Insula
➢ Cingulate gyrus
➢ Subfrontal region
Herpes encephalitis
• Imaging may be normal early in disease course
• MR > CT, especially early
• MR
➢ Inc. SI on T2 & FLAIR
➢ Mass effect
➢ +/- enhancement Herpes encephalitis of left temporal lobe
➢ 60% bilateral
➢ May have small punctate hemorrhage Figure 5-18-14
Herpes encephalitis [Figure 5-18-14]
• Subtle, gyral enhancement
• Unilateral early, then bilateral
• Temporal lobes, insular cortex, inferior frontal lobes,
cingulate gyri
Creutzfeldt-Jakob Disease
• Transmissible spongiform encephalopathy (TSE)
• Prion: proteinaceous infectious particle
• Sporadic – most common (85%-90%)
• Genetic (10%-15%)
• Infectious (rare)
Creutzfeldt-Jakob Disease
CLASSIC CJD VARIANT CJD
• Older (mean 68 yrs) Younger (mean 28 yrs)
• Sporadic BSE contaminated food
• Shorter duration Longer duration Herpes encephalitis, with high SI in temporal
• Dementia Behavior changes lobes and patchy enhancement
• Rare “pulvinar sign” “Pulvinar sign” on MR >
75%
• Variable amounts of PrPres Lots of PrPres
Creutzfeldt-Jakob Disease: Imaging

• Symmetric high signal on T2 & FLAIR
• Caudate head, basal ganglia, thalamus (“pulvinar sign”)
• Diff. restriction > 2 weeks (c/w infarct)
• Cortical, limbic system involvement 1/3
• Occipital lesions: 20%
• Rapidly progressive atrophy
CNS Tuberculosis
• Increasing incidence – homeless, prisoners (2%-5% of pts. w/ TB)
• AIDS population
• Hematogenous dissemination from lungs
• Most have pulmonary TB
• Drug resistance increasing & ominous
• Either meningitic or tuberculoma, rarely both forms found together
CNS Tuberculosis Leptomeningeal

• Cisterns fill with gelatinous exudate
• Hydrocephalus, infarctions, CN palsies
• Leptomeningeal enhancement, hydrocephalus
• Disease at COW – arteritis & infarctions, esp. in children
Neuroradiology 1233
CNS Tuberculosis Cerebritis [Figure 5-18-15] Figure 5-18-15
CNS TuberculosisTuberculoma
• Most common parenchymal form(may
be extra-axial)
• Supratentorial > infra
• Dense fibrous capsule with caseous
necrotic core
CNS Tuberculosis Tuberculoma TB cerebritis with typical pattern of right temporal edema and
[Figure 5-18-16] enhancement
• Small or coalesced larger nodules
• Edema, mass effect
• Wall may have increased SI on T1, decreased SI on T2 Figure 5-18-16
• Old/treated lesions may Ca+
Neurocysticercosis
• Taenia solium (Pork tapeworm)
• Worldwide, most common CNS infection
• Worldwide, most common cause of epilepsy
• Central/South America; East/SE Asia; India; Africa
• CNS disease: 60%-90%
• Seizures, intracranial hypertension, focal deficit
• Four variants
➢ 1. Parenchymal
➢ 2. Intraventricular
➢ 3. Cisternal Multiple tuberculomas, with low signal intensity rim
➢ 4. Spinal on T2 and enhancement
Parenchymal Cysticercosis
• Vesicular stage
➢ Eccentric nodule (scolex), no edema or enhancement
• Colloidal stage
➢ Dying scolex, capsule thickens, extensive edema & enhancement
• Granular nodular stage
➢ Cyst ? in size, small enhancing nodules, no edema
• Nodular calcified stage
➢ Cyst involutes, calcifies, no edema or enhancement Figure 5-18-17
Vesicular stage
• Larva is fully grown,with thin intact capsule
surrounding distended bladder
• Fluid in bladder is clear, no surrounding inflammatory
reaction.
• Well-defined cyst, scolex enhances (mural nodule)
• Wall does not enhance, no edema
Colloidal stage [Figure 5-18-17] Colloidal stage of CNS cysticercosis, with thick
• Larva degenerates, cyst fluid turbid capsule, edema, and mass effect
• Cyst wall thickens
• Vasogenic edema
• Cyst fluid ? SI on T1WI
• Cyst wall ? SI on T2WI
• Vasogenic edema & enhancement

1236 Neuroradiology
Parenchymal Cysticercosis Figure 5-18-18
Granular Nodule Stage
• Cyst retracts, capsule thickens, and scolex calcifies
• +/- perifocal edema
• CT: Isodense cyst, calcified scolex
• MR: T1WI – isointense to brain
T2WI – hypointense to brain
Intraventricular Cysticercosis [Figure 5-18-18]

• 10-20% of neurocysticercosis
• Fourth ventricle - most common site
• +/- hydrocephalus
• Cyst can parallel CSF density and SI
• Does not calcify
Cisternal Cysticercosis
• Basilar cisterns or sylvian fissures Intraventricular and subarachnoid
• MRI more sensitive than CT cysticercosis, with hydrocephalus
• Can incite leptomeningeal inflammatory response
• Hydrocephalus, infarctions
CNS Lyme Disease

• Borrelia burgdorferi
• Common tick borne disease in NE US
• Presents as meningitis, neuritis (incl. CN), vasculitis
• Multifocal wm lesions, +/- enh
Figure 5-18-19
Fungal Disease in CNS
• Cryptococcosis
• Mucormycosis
• Aspergillosis
• Others
AIDS Related Conditions

• HIV Encephalitis
• Toxoplasmosis (vs lymphoma)
• Cryptococcal meningitis
• Progressive multifocal leukoencephalopathy (PML)
Human Retroviruses
• HIV (HIV-1 and HIV-2) and HTLV-1
• HIV-1 found in CNS in AIDS (neurotrophic) Patchy symmetric abnormal signal
• Encephalopathy, myelopathy, peripheral neuropathy, myopathy intensity in patient with HIV
• HIV replicates in multinucleated giant cells & macrophages in encephalitis
CNS
• Oligodendrocytes, astrocytes, neurons less frequently directly infected
Subacute HIV encephalitis AIDS Dementia Complex (ADC)

[Figure 5-18-19]
• Most common CNS complication (30%)
• Dementia, behavior changes, headache
• Virus in MNGC’s
• CT – normal
• MR – atrophy
➢ PVWM lesions
➢ Grey matter (late)
Neuroradiology 1235
HIV Leukoencephalopathy Figure 5-18-20
• Disease on MR parallels clinical progression
• NAA/Cho & NAA/Cr ratios reduced due to neuronal
loss
PML [Figure 5-18-20]

• Papovavirus (JC virus)
• Patchy non-enhancing white matter lesions
• No mass effect
• Hypointense on T1
• Asymmetric PML has low SI on T1 weighted images, with no
enhancement
HIV Encephalitis vs PML
HIV PML
Diffuse Asymmetric Figure 5-18-21
Central Peripheral
T1 images nl Hypointense T1

• Protozoan Toxoplasma gondii
• Soil organism
• Endemic in US
• Reactivates in CNS in immunocompromised
• Most common opportunistic infection
• CD4 < 100 cell/mm3
• Fever, HA, neurologic deficits, seizures
• Basal ganglia, cerebral hemispheres Single large toxoplasmosis abscess
Toxoplasmosis
• Necrotic debris, inflammatory cells, organisms Figure 5-18-22
• Vasogenic edema
• Robust enhancement
• 15% are solitary
• Pyrimethamine + sulfa or clindamycin
• Life-long maintenance abs

• With therapy, lesions may calcify
• Resolution of vasogenic edema
• New lesions or new edema develop if medication is
stopped Old, treated toxoplasmosis abscesses may calcify
Cryptococcus neoformans
• Ubiquitous fungi within contaminated soil
• Inhaled, then hematogenous spread immunocompromised hosts
• Most common fungus in AIDS (6%-7%)
• Subacute meningitis, HA, AMS, fever
• DX – India ink preparation, antigen in CSF, fungal culture of CSF Figure 5-18-23
• Perivascular spaces distended with mucoid material and fungus
Cryptococcus meningitis
• Basal ganglia,often bilateral
• CT may be normal
• Non-enhancing low density lesions in Virchow-Robin
spaces “gelatinous pseudocysts”
Cryptococcus meningitis [Figure 5-18-23]

• On MR, pseudocysts are decreased SI on
T1,increased SI on T2 Dilated peri-vascular spaces in cryptococcosis
• No significant enhancement

1238 Neuroradiology
Highly Active Antiretroviral TX (HAART) Immune reconstitution syndrome
• AIDS treatment regimen that includes protease inhibitor & reverse transcriptase inhibitor
• Suppresses viral replication
• Increase in CD4 counts, decrease in viral load
• Increased survival
• May result in “unexpected” imaging findings
HAART
• Ability to mount immune response may change imaging findings, especially enhancement patterns
• Enhancement in crypto. meningitis
Summary
➢ Epidural empyema
➢ Meningitis
➢ Tuberculosis
➢ Mycotic
➢ AIDS related infections
References
1. Chang L, Ernst T. MR spectroscopy and diffusion-weighted MR imaging in focal brain lesions in AIDS.
Neuroimaging Clin N Am. 1997 Aug;7:409-26.
2. Collie DA, Summers DM, Sellar RJ, et al. Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign:
MR imaging findings in 86 neuropathologically confirmed cases. AJNR Am J Neuroradiol. 2003l 24:1560-9.
3. Enzman DR, Britt RH, Obana WG, et al. Experimental staphylococcus aureus brain abscess. AJNR 7:395-402,
1986
4. Fernandez RE, Rothbert M, Ferencz G, et al. Lyme disease of the CNS: MR imaging findings in 14 cases. AJNR
1990;11:479-481
5. Filippi CG, Sze G, Farber SJ, et al. Regression of HIV encephalopathy and basal ganglia signal intensity
abnormality at MR imaging in patients with AIDS after the initiation of protease inhibitor therapy. Radiology
1998;206:491-498
6. Galassi W, Phuttharak W, Hesselink JR, et al. Intracranial meningeal disease: comparison of contrast-enhanced
MR imaging with fluid-attenuated inversion recovery and fat-suppressed T1 weighted sequences. AJNR 2005; 26:
553-9
7. Gaviani P, Schwartz RB, Hedley-Whyte ET, et al. Diffusion-weighted imaging of fungal cerebral infection. AJNR
Am J Neuroradiol. 2005 May;26(5):1115-21.
8. Han XY, Weinberg JS, Prabhu SS, et al. Fusobacterial brain abscess: a review of five cases and an analysis of
possible pathogenesis. J Neurosurg 2003; 99: 693-700
9. Kramer LD, Locke GE, Byrd SE, et al. Cerebral cysticercosis: documentation of natural history with CT.
Radiology 1989;171:459-462
10. Lai PH, Li KT, Hsu SS, et al. Pyogenic brain abscess: findings from in vivo 1.5 T and 11.7 T in vitro proton MR
spectroscopy. AJNR 2005; 26:279-88
11. Murata T, Shiga Y, Higano S, et al. Conspicuity and evolution of lesions in Creutzfeldt-Jakob disease at diffusion-
weighted imaging. AJNR Am J Neuroradiol. 2002; 23:1164-72
12. Nadal Desbarats L, Herlidou S, de Marco G, et al. Differential MRI diagnosis between brain abscess and necrotic
or cystic brain tumors using the apparent diffusion coefficient and normalized diffusion-weighted images. Magn
Reson Imaging 2003; 21: 645-650
13. Post MJD, Tate LG, Quencer RM, et al. CT, MR, and pathology in HIV encephalitis and meningitis. AJNR 1988;
9:469-476
14. Prusiner SB. Prions and neurodegenerative diseases. N Engl J Med. 1987; 317:1571-81.
15. Sze G, Zimmerman RD. The magnetic resonance imaging of infections and inflammatory diseases. Radiol Clin
North Am. 1988: 26:839-59.
16. Tien RD, Felsberg GJ, Osumi AK. Herpesvirus infections of the CNS: MR findings. AJR Am J Roentgenol. 1993
Jul;161:167-76.
17. Wehm SM, Heinz ER, Burger PC, et al. Dilated Virchow-Robin spaces in cryptococcal meningitis associated with
AIDS: CT and MR findings. J Comput Assist Tomogr 1989;13:756-762
Neuroradiology 1237
The Paranasal Sinuses
Summary Figure 5-19-1

• Development
• Anatomy, especially for endoscopic sinus surgery (ESS)
• Infection
➢ Acute
➢ Chronic
➢ Complications
• Neoplasms
➢ Benign, including tumor-like lesions
➢ Malignant
Sinus – Anatomy & Development

• Nose and nasal cavities - Functions
➢ Respiration - humidifies and warms inspired air
➢ Defense – mucous blanket
➢ Olfaction – fibers pass through cribriform plate to CN I
Normal ethmoid anatomy in coronal
• Nose – external nose, pyriform aperture
plane
• Nasal septum – cartilage, ethmoid bone, septum
• Nasal cavities – internal nasal airways
Figure 5-19-2
➢ Inferior turbinate – nasolacrimal duct
➢ Medial turb. – max, frontal, ant ethmoid sinus
➢ Superior turb – post. ethmoid, sphenoid sinus
Sinus – Anatomy & Development

• Lined by mucosa w/ serous and mucinous glands
• “Mucoperiosteum” – mucosa attached to bone
• Sinus functions – protect CNS (collapsible) is only definite
function
Ethmoid sinus
• Bilateral
• Groups of cells formed by septa and lamella
• Anterior ethmoids
➢ Multiple small cells
➢ Middle turbinate
• Posterior ethmoids Pneumatized agger nasi cell on left
➢ Fewer but larger cells on coronal CT
• Basal lamella
➢ Lateral insertion of middle turbinate Figure 5-19-3
➢ Separates ant from post ethmoids
Ethmoid sinus - anatomy [Figure 5-19-1]

• Lamina papyracea
• Roof of the ethmoids
• Drainage
➢ Infundibulum, lateral to uncinate process
• Ethmoid bulla
Ethmoid sinus - variants [Figures 5-19-2 and 5-19-3]

• Agger nasi cell
➢ Most ant. ethmoid
• Supraorbital air cell
➢ Needs to be differentiated from frontal sinus Bilateral Haller cells, inferomedial
• Haller cell ethmoid cells, between uncinate
➢ May obstruct max. sinus outflow process and orbital wall
Paranasal Sinuses 1238

1240 Neuroradiology
• Onodi cell Figure 5-19-4
➢ Close to optic nerve
Maxillary sinus [Figure 5-19-4]

• First to form in utero
• Rudimentary at birth, final form during 2nd decade
• Recesses
➢ Zygomatic
➢ Palatine
➢ Alveolar
Maxillary sinus
• Drainage
➢ Ostium is at superomedial portion
➢ Drains into infundibulum Maxillary sinus cilia beat secretions
• Uncinate process up and medial, drain via maxillary
➢ Medial wall of ostium sinus ostium into infundibulum
➢ Located at insertion of inferior turb on lateral nasal wall Figure 5-19-5
➢ Contiguous with lacrimal bone anteriorly
Maxillary sinus - variants [Figure 5-19-5]

• Hypoplasia
• Sclerotic walls – chronic inflammation
• Atelectatic
• Lateralized medial wall
Maxillary sinus - variants [Figure 5-19-6]

• Hypoplasia
• Sclerotic walls – chronic inflammation
• Atelectatic
• “Silent sinus syndrome”
• Lateralized medial wall CT finding of small sinus with thick
sclerotic walls found in chronic sinus
Frontal sinus inflammation on right
• Absent at birth, finish pneumatizing in 2nd decade Figure 5-19-6
• Essentially anterior ethmoid cell
• Drainage is variable
➢ Ethmoid infundibulum
➢ Frontal recess
• Usually asymmetric, may be septated
Ostiomeatal complex [Figure 5-19-7]

• Lateral nasal wall
• 3 projections –
turbinates Figure 5-19-7
• Divide nasal cavity
into 3 separate
passages - meatus
Right maxillary sinus is severely
atelectatic, with depressed walls
consistent with silent sinus syndrome
Ostiomeatal complex
(courtesy of Amirsys, Inc.)
Neuroradiology 1239
1241 Paranasal Sinuses
Normal OMU [Figure 5-19-8] Figure 5-19-8
• 3 turbinates/meati
• Frontal recess
• Infundibulum and middle meatus
Normal OMU – Frontal Recess

• Ant. coronal images
• Drains frontal sinus to ant. middle meatus
• Contents
➢ Frontal sinus
➢ Agger nasi cell
➢ Frontal cells
➢ Frontal recess
➢ Middle meatus
Normal OMU on coronal CT shows
maxillary sinus, ostium, ethmoid
Normal OMU – Infundibulum [Figure 5-19-9] infundibulum, uncinate process,
• Uncinate process middle turbinate and middle meatus.
• Infundibulum – connects max sinus ostium to middle meatus
• Ethmoid bulla – largest ethmoid cell Figure 5-19-9
• Hiatus semilunaris – space between uncinate process and
ethmoid bulla
Normal OMU - Checklist

• Anterior
➢ Frontal sinus, recess
➢ Agger nasi cell, frontal cells
• Posterior
➢ Uncinate process
➢ Maxillary sinus, ostium, infundibulum
➢ Ethmoid bulla (retrobullar recess)
➢ Middle turbinate/meatus
➢ Hiatus semilunaris
Uncinate process marks lateral
aspect of infundibulum
Sphenoid sinus
• Extremely variable pneumatization
• Dev. complete by 2nd decade
• Planum sphenoidale, posterior wall, anterior wall, inferolateral & pterygoid
recesses
• Ant. wall is roof of nasopharynx
• Rarely non-pneumatized, consider anemia or ciliary dysmotility syndrome
• Sphenoethmoidal recess
Endoscopic sinus surgery

• Theory: mucosa is secondarily involved by inflammatory disease, usually due
to sinus ostial obstruction
Figure 5-19-10
• Surgically relieve obstructing lesion (polyp, anatomic
variant, etc) will allow sinus to drain normally and
mucosal edema & inflammation will improve
• Recurrent or chronic sinusitis should improve
Ostiomeatal Complex Pattern [Figure 5-19-10]

• Middle meatus
• Maxillary sinus ostia
• Ethmoid infundibulum
• Anterior ethmoid cells
• Hiatus semilunaris
Ostiomeatal complex
(Courtsey of Amirsys, Inc)

1242 Neuroradiology
Infection – Acute Sinusitis Figure 5-19-11
• No indications for imaging common cold
• Secondary bacterial sinusitis
➢ Strep. pneum, H. flu, beta-hemolytic strep
• Facial pain, fever, discharge
Acute Sinusitis – CT/MR

• Ethmoids often primary source
• Asymmetric
• Mucosal thickening
➢ Moderate or severe
➢ Non-specific
➢ Thickened enhancing mucosa with submucosal
edema
• Air/fluid levels
➢ Frontal = sinusitis
➢ Maxillary = sinusitis in correct setting
➢ Ethmoid – rare
➢ Sphenoid - nonspecific
Orbital sub-periosteal infection. Graphic and axial
Acute sinusitis - complications CECT show ethmoid opacification, and
• Local extension peripherally enhancing mass in medial extra-conal
➢ Orbital – sub-periosteal abscess location. (courtesy of Amirsys, Inc.)
➢ Intra-cranial – epidural empyema
• Venous occlusion – cavernous sinus, transverse sinus (mastoid), superior
sagittal sinus
• Chronic sinusitis
Figure 5-19-12
➢ Recurrent acute
➢ Chronic – patient has no periods without disease
Sinusitis – subperiosteal infection

[Figure 5-19-11]
• Most common local complication
• Pre-septal swelling clinical dx
• Post-septal infection best detected with imaging
• CECT is test of choice, to exclude post-septal, sub-
periosteal abscess Axial CECT shows small interhemispheric
subdural abscess. Note posterior frontal wall is
Sinusitis – Complications - Local extension intact
[Figure 5-19-12]
• Noncontrast sinus CT not enough
• Enhanced CT and MR are indicated for complicated Figure 5-19-13
sinusitis
• Intracranial infection can occur without
bone defect
Frontal Sinusitis – Intracranial

abscess [Figure 5-19-13]
Sinusitis Mimics “Incidental”

lesions found on screening sinus T2, sagittal and axial post-Gd T1 MR images show right frontal
CT abscess, with surrounding edema. Note thin dural
• Subarachnoid hem. enhancement, opacified frontal sinuses and soft tissue
• GBM inflammation
• Colloid cyst
• Hydrocephalus
• Subdural hematoma
• Esthesioneuroblastoma
Neuroradiology 1241
• Lymphoma of maxillary antrum Figure 5-19-14
• SCCa maxillary sinus
• Adenoid cystic ca
Sinusitis - Fungal
• Two distinct forms
• Acute, fulminant invasive fungal sinusitis
• Allergic fungal sinusitis with sino-nasal polyps
• Imaging appearance variable for both
Allergic fungal sinusitis with polyps (AFS-SNP)

[Figure 5-19-14]
• Immune-competent
• Chronic nasal obstruction, “recurrent sinusitis”
• Cycle of sinusitis, mucosal edema, polyp formation, ostial
stenosis, sinusitis… Coronal non-contrast CT shows
• Polyps massive expansion of ethmoid and
frontal sinuses, with contents both
Allergic fungal sinusitis with polyps (AFS-SNP) low and high density
[Figure 5-19-15]
• CT
➢ Pansinus/nasal cavity opacification
Figure 5-19-15
➢ Expanded airless sinuses
➢ Thin deossified sinus walls
➢ Sinus contents variable in density
➢ Mixed low and high density on noncontrast CT
Allergic fungal sinusitis with polyps (AFS-SNP)

[Figure 5-19-16]
• MRI
➢ Extremely complex SI
➢ May be increased or decreased on T1
➢ Variable on T2, regions of frank signal void
➢ Marked expansion may encroach on surrounding structures,
including orbit & skull base
Axial non-contrast CT shows marked
Fungal sinusitis - invasive [Figure 5-19-17] thinning of posterior frontal sinus
• Immune deficient walls, without destruction
• Early – non-spec. presentation
• CT – early
➢ Mucosal disease Figure 5-19-16
➢ Nasal cavity soft tissue due to mucosal or turbinate necrosis
Figure 5-19-17
Complex, mixed signal intensity

Early invasive fungal within expanded airless sinuses on
sinusitis may have only a T2 WI’s.
benign appearing nasal (courtesy of Amirsys, Inc.)
cavity mass

1244 Neuroradiology
Fungal sinusitis - invasive [Figure 5-19-18] Figure 5-19-18
• CT/MR late
➢ Local invasion
➢ Dirty retro-antral space
➢ Intracranial/orbitalspread
➢ Bone destruction
Fungal sinusitis - invasive [Figure 5-19-19]

• Variable SI
• May have dramatic decrease of SI on T1 & T2
• Heterogeneous enhancement pattern
Figure 5-19-19
CECT shows complete left nasal

cavity obstruction, moderate left
maxillary sinus mucosal thickening,
and severe facial swelling
Complex sinus contents on T2 WI’s

in invasive fungal sinusitis
Fungal sinusitis
• Acute invasive Figure 5-19-20
➢ Immunesuppressed
➢ Acute
➢ Pain, fever, local invasion
➢ May be fulminant, rapidly progress
➢ Often treated surgically local resection, orbital
exenteration
➢ High mortality
• Allergic fungal
➢ Clinically well
➢ Aspirin intolerance
➢ Chronic
➢ Presents with nasal obstruction
➢ Txed with endoscopic polyp resection
➢ High rate of recurrence
Benign Sinus Lesions

• Antro-choanal polyp
• Mucocele
• Osteoma Coronal graphic of left antrochoanal polyp.
• Juvenile nasopharyngeal angiofibroma (JNA) (courtesy of Amirsys, Inc.)
• Inverted papilloma
Antrochoanal polyp [Figure 5-19-20]

• Benign maxillary polyp
• Extends from maxillary antrum through ostium into nasal cavity
• When large may extend to nasopharynx
• Entire lesion must be removed to avoid recurrence
Neuroradiology 1243
Mucocele [Figure 5-19-21] Figure 5-19-21
• Airless, expanded sinus
• Sinus walls thinned, may appear dehiscent
• Frontal, ethmoid, maxillary, sphenoid in order of
frequency
• CT/MR signal intensity variable, depending on age of
secretions
• Check sinus ostium for obstructing lesion
Fibrous Dysplasia [Figure 5-19-22]

• Medullary bone replaced by fibroosseous tissue
T1 sagittal (top) and T2 axial (bottom) images
• Presents < 30 yrs
show expanded, airless right frontal sinus
• Facial asymmetry, esp. cheek
mucocele
• Most common – maxilla & mandible
• Obstruction of sinus ostium results in mucocele, especially
ethmoid Figure 5-19-22
Fibroosseous Lesions - Fibrous Dysplasia

• Fibroosseous
• Therefore, often heterogeneous
• CT – ground glass
• MR – complex appearance
➢ Mixed increased & decreased SI
➢ Enhances robustly – often leads to misinterpretation as tumor
Benign Sinus Tumors - Fibroosseous - Osteoma

[Figure 5-19-23]
• Benign proliferation of mature bone
• Frontal & ethmoid sinus – most common location
• Usually small & incidental
• May obstruct sinus ostium with sinusitis or mucocele formation
• CT – may be very dense, or more fibrous
• MR – decreased SI if primarily osseous
Figure 5-19-23
T1 sagittal (top) and T2 axial

(bottom) images show expanded,
airless right frontal sinus mucocele
Left ethmoid infundibulum osteoma
Juvenile Nasopharyngeal Angiofibroma (JNA)

• Benign but aggressive vascular mass
• Exclusively in males, adolescents
• Presenting sxs depend on location
➢ Nasal obstruction
➢ Minor or major epistaxis
➢ Facial asymmetry/deformity
➢ Proptosis
➢ Serous otitis media
➢ Headache

1246 Neuroradiology
JNA Figure 5-19-24
• Arises in posterior nasal cavity (choana)
• Involves nasal cavity, nasopharynx, masticator space, sphenoid
sinus, cavernous sinus
• Blood supply
➢ ECA - int. max & asc. pharyngeal arteries
➢ ICA supply usually implies intracranial extension
➢ Both ipsi and contralateral ECA/ICA supply
JNA - Imaging [Figure 5-19-24]

• Nasal cavity & nasopharyngeal mass
• Expansion of pterygopalatine fossa
• Anterior bowing of posterior max. sinus wall
• CECT & MR with gado – robust enhancement
JNA - Imaging [Figure 5-19-25] Left JNA expands PPF, displaces

• Heterogeneous on T1 & T2 sequences with flow voids posterior maxillary sinus wall
• Axial & cor best
• Check for sphenoid sinus, cavernous sinus invasion
• T2 images best map entire lesion
Benign sinus lesions - Inverted Papilloma

• Epithelial tumor of mucosa
• Endophytic growth pattern
Figure 5-19-25
• Benign appearing mass in nasal cavity/middle meatus
• Associated with SCCa 10%-20% of time
• Bone remodeling without destruction
• On MR
➢ Enhancement
➢ Convoluted cerebriform pattern
Malignant Sinus Lesions

• “Sinusitis” mimic
• Adults, M > F
• Usually advanced when detected
➢ Early small lesions clinically attributed to inflammatory sinus
disease
• Maxillary, ethmoid most common
• SCCa – 80%-90%
JNA shows robust enhancement and
Malignant Sinus Lesions intracranial extension
• Squamous cell ca (most common)
• Glandular origin
• Olfactory neuroblastoma
• Sinonasal undifferentiated ca
• Melanoma
• Lymphoma
Figure 5-19-26
COMMON IMAGING FINDINGS
• Local extension/invasion
• Intracranial extension
Most important imaging goal extension of

disease [Figure 5-19-26]
Graphics show patterns of spread of sinus

malignancy
Neuroradiology 1245
SCCa Staging – critical findings Figure 5-19-27
• Primary site
➢ Size
➢ Bone - maxillary or orbit walls, skull base
➢ Local - cheek, nasal cavity, nasopharynx, orbit
SCCa Staging (maxillary)

• T1 – Antral mucosa w/o bone involvement or erosion
• T2 - Hard palate or sinus walls
• T3 – Cheek, orbital walls, pterygoid plates, ethmoids
• T4 - Skull base (cribriform plateor sphenoid sinus), frontal sinus,
nasopharynx, orbital apex
Squamous cell carcinoma [Figure 5-19-27]

• Most arise in max sinus or nasal cavity Low SI left nasal cavity SCCa, with
• M > F, adults > 50 obstructed secretions
• Usually advanced at detection
• Obstructed sinus secretions may make imaging
appearance complex Figure 5-19-28
Malignant sinus - SCCa [Figure 5-19-28]

• CT – bone thinning or destruction
• MR – T2 best differentiates tumor from benign
secretions
➢ Tumor - decreased SI
➢ Secretions - increased SI
Malignancies of Glandular Origin

• 10% of all sinus malignancies Subtle erosion of planum sphenoidale (top) from
• Typical local extension & bony erosion nasal cavity & sphenoid sinus SCCA (bottom)
• May be higher SI on T2
➢ Adenocarcinoma
Figure 5-19-29
➢ Adenoid cystic ca
❖ Propensity for perineural spread
➢ Mucoepidermoid ca
Adenoid cystic ca [Figure 5-19-29]

• Sinus mass with osseous erosion
• Perineural spread
➢ Widened foramen or canal
➢ Enlarged enhancing nerve
➢ Obliteration of fat at skull base foramen
T1 MR shows maxillary mass with hard palate low
Esthesioneuroblastoma AKA Olfactory SI on left
Neuroblastoma Perineural spread along vidian nerve
• Tumor of neural crest origin
• Accounts for 2% of sinonasal malignancies Figure 5-19-30
• Arises in nasal cavity near cribriform plate
• Age range 3 to elderly
• Nasal cavity mass, erosion of cribriform plate, often
with intracranial extension
• Peripheral intracranial tumoral cysts
Esthesioneuroblastoma [Figure 5-19-30]
Intracranial extension from

esthesioneuroblastoma

1248 Neuroradiology
Summary
• Development
• Anatomy, especially for endoscopic sinus surgery (ESS)
• Infection
➢ Acute
➢ Chronic
➢ Complications
• Neoplasms
➢ Benign, including tumor-like lesions
➢ Malignant
Neuroradiology 1247
The Sella and Parasellar Region
Recommended Imaging Techniques [Figures 5-20-1 and 5-20-2]

• MR Imaging Figure 5-20-1
➢ Multiplanar:
❖ Sagittal & Coronal
➢ Small FOV 16-18
➢ 3mm
➢ T1WI, T2WI
➢ Post T1WI +/- FS
➢ Dynamic enhanced
• Dynamic Imaging
➢ Microadenomas
➢ 3 – 4 slices
➢ T1 FSE, Turbo SE Normal sella, sphenoid sinus, pituitary gland,
➢ Image continuously after contrast (10s) infundibulum, and suprasellar region on graphic
➢ Increases sensitivity and post-gado. T1-WI
Pituitary: Normal Anatomy Figure 5-20-2
• Anterior Lobe
➢ Lateral
❖ PRL (10%-30%)
❖ GH (50%)
➢ Midline
❖ ACTH (10%-30%)
❖ TSH (5%)
❖ FSH/LH (10%)
➢ Location of adenomas parallels the distribution
• Posterior Lobe
➢ Infundibulum
Normal adult pituitary gland on sag T1-WI and cor
➢ Pituicytes (glial)
T2-WI
➢ Axons
➢ Vasopressin (ADH)
➢ Oxytocin Figure 5-20-3
Pituitary: Normal Anatomy

• Posterior Lobe
➢ Posterior Pituitary Bright Spot (PPBS)
➢ High SI on T1
➢ Doesn’t suppress on fat sat
Parasellar Region: Normal Anatomy [Figure 5-20-3]

• Parasellar Structures
➢ Cavernous Sinus
➢ Cranial Nerves
❖ III, IV, V1, V2, VI
➢ Cavernous ICA
➢ Optic Chiasm
➢ Hypothalamus
Normal coronal graphic through the
➢ Sphenoid Sinus
sella, including the pituitary gland,
suprasellar cistern, infundibulum, and
cavernous sinuses
Sella and Parasellar Region 1248

1250 Neuroradiology
Parasellar Region: Normal Anatomy Figure 5-20-4
• Parasellar Structures
➢ Optic Chiasm
➢ Hypothalamus
❖ Tuber cinereum
❖ Mamillary bodies
➢ Sphenoid Sinus
Parasellar Region: Normal Anatomy

[Figure 5-20-4]
• Bony Structures
➢ Planum sphenoidale
➢ Tuberculum sellae
➢ Sella turcica
➢ Dorsum sellae
Parasellar Region: Normal Anatomy
Sella and Parasellar Pathology

• Differential Diagnoses
➢ Intrasellar Skull base bony structures around the sella,
➢ Suprasellar including the planum sphenoidale, tuberculum
➢ Infundibular sellae, sella turcica, and dorsum sellae
Intrasellar Pathology
• Nonneoplastic Lesions
Figure 5-20-5
➢ Hyperplasia (physiologic, end organ failure)
➢ Cysts (RCC, pars intermedia cyst)
➢ Lymphocytic hypophysitis
• Primary Neoplasms
➢ Pituitary adenoma (Most common)
➢ Craniopharyngioma (Only 5% purely intrasellar)
➢ Meningioma (Purely intrasellar rare)
➢ Pituitary carcinoma (Extremely rare)
• Metastasis (1%)
Pituitary Neoplasms Graphic and post-gado T1-WI of small right

microadenoma
• Adenoma
➢ Prolactinoma 30%
➢ Null cell 25%
➢ GH 20%
➢ ACTH 10%
➢ FSH/LH 10%
➢ PRL-GH 5%
➢ Mixed, TSH 1%-5%
➢ Incidental pituitary lesions are common (17%)
Sella: Pathology [Figure 5-20-5]

• Pituitary Microadenoma
➢ 10 mm or less
➢ 10%-20% of autopsies
➢ Micro >>> Macro
➢ Convex margin
➢ Stalk deviation
➢ Sella floor thin
Neuroradiology 1249
1251 Sella and Parasellar Region
Pituitary Microadenoma Figure 5-20-6
Sella: Pathology
• Pituitary Microadenoma
• Dynamic Imaging
➢ Increases sensitivity (10% seen only on dynamic
MR)
➢ Enhances slower than normal gland
Physiologic Hypertrophy
• Maximum normal height
➢ 6 mm infants and children Pituitary hypertrophy in a patient with
➢ 8 mm males, postmenopausal females hypothyroidism
➢ 10 mm young women (convex superiorly)
➢ 12 mm late pregnancy, postpartum females (convex superiorly)
• Abnormal hypertrophy
➢ End-organ failure (esp. hypothyroid) Figure 5-20-7
➢ Neuroendocrine tumor (rare)
Pituitary Gland Hypertrophy [Figure 5-20-6]
Sella: Rathke Cleft Cyst

• Clinical
➢ Intrasellar 40%
➢ Suprasellar extent 60%
➢ 3mm – 3cm
➢ Most incidental
➢ Symptomatic Intrasellar Rathke cleft cyst, hyperintense on T1
➢ Pituitary dysfunction non-contrast MR, with small intra-cyst nodule seen
➢ Visual change, HA on T2-WI
Rathke Cleft Cyst: CT

• 75% hypodense
• 25% iso/hyperdense
• Ca++ rare
• May be difficult to differentiate from other benign cysts or craniopharyngiomas
Rathke Cleft Cyst: MR

Imaging Features
• Signal varies - cyst content
➢ 50%-60% T1 hyperintense
➢ 30%-40% follow CSF
• 75% intracystic nodule
• +/- rim enhancement
Rathke Cleft Cyst [Figure 5-20-7]
Lymphocytic hypophysitis
• During pregnancy or shortly after delivery
• F >>> M
• Pituitary insufficiency
• H/A & visual changes
• Amenorrhea or inability to lactate
• Diffuse enlargement of adenohypophysis
• May mimic hyperplasia or adenoma

1252 Neuroradiology
Lymphocytic hypophysitis - Pathology
• Diffuse infiltration of the adenohypophysis by lymphocytes & plasma cells
• ? Autoimmune
• Infundibuloneurohypophysitis
➢ Affects infundibulum & neurohypophysis
➢ Thickened pituitary stalk
➢ Diabetes insipidus
Lymphocytic hypophysitis
Other Intrasellar Masses Uncommon

• Craniopharyngioma (5% intrasellar)
• Metastasis (1% of sellar masses)
• Aneurysm (medially-projecting from cavernous ICA)
• Meningioma (rare purely intrasellar)
Suprasellar Masses: Five Most Common

• 75% of all sellar/parasellar masses
➢ Pituitary macroadenoma (35%-50%) Figure 5-20-8
➢ Approximately 10% each
❖ Meningioma
❖ Aneurysm
❖ Craniopharyngioma
❖ Astrocytoma (hypothalamic-chiasmatic)
Suprasellar Differential Diagnosis

• Adult Lesions
➢ Pituitary Macroadenoma
➢ Meningioma
➢ Aneurysm
• Pediatric Lesions
➢ Craniopharyngioma
➢ Chiasmatic / hypothalamic Glioma
➢ Hypothalamic Hamartoma
Sella and suprasellar macroadenoma
with narrowing where lesion extends
Pituitary Macroadenoma [Figure 5-20-8] through the diaphragma sellae
• Clinical / Pathologic (courtesy of Amirsys, Inc.)
➢ Most common suprasellar mass (50%)
➢ 10% of intracranial tumors
➢ Snowman shape
➢ Compressive symptoms
➢ Rare in prepubescent children, adolescent males
Suprasellar: Pathology
• Macroadenoma
➢ > 10mm
➢ Enlarged sella turcica
➢ Sellar/suprasellar
➢ MR test of choice
➢ Robust enhancement
➢ ? Cav. sinus invasion, mass effect on chiasm
Pituitary Adenoma
• Prolactinoma
➢ 30% of adenomas
➢ Female >> Males
➢ Galactorrhea
➢ Amenorrhea
➢ Serum PRL > 150ng/mL
➢ If > 1000ng/mL, cavernous sinus invasion
Neuroradiology 1251
Pituitary Macroadenoma: CT
• CECT
➢ Enlarged sella turcica
➢ Moderate to strong enhancement
➢ May be heterogeneous (cysts, hemorrhage)
Pituitary Macroadenoma: MR [Figure 5-20-9] Figure 5-20-9

Imaging Features
• Isointense GM: T1, T2WI
• May have hemorrhage, cystic components
• Figure-eight, snowman
• Robust but heterogeneous enhancement
• Determining cavernous sinus invasion difficult
Pituitary Macroadenoma: MR
Cavernous Sinus Invasion

• More aggressive
• Can’t be resected
• > 2/3 surrounds ICA
• ICA venous sulcus compartment filled
Pituitary macroadenoma with right
Invasive Pituitary Macroadenoma [Figure 5-20-10] cavernous sinus invasion
• May extend inferiorly into sphenoid sinus & skull base
• Must differentiate from skull base primary tumor
• Will involve bony sella turcica & pituitary gland
Figure 5-20-10
Invasive macroadenoma
Pituitary Apoplexy
1. Hemorrhage into tumor
2. Pituitary gland infarction
• Acute onset Figure 5-20-11
• Headache, visual changes, vomiting
• Usually hemorrhagic, may be non-hem
• May be life threatening
Pituitary Apoplexy [Figure 5-20-11]
Suprasellar: Pathology
• Meningioma
➢ 2nd most common (adults)
➢ 15% of meningiomas Pituitary apoplexy can be hemorrhagic or not
❖ Tuberculum sellae
❖ Clinoid processes
❖ Cavernous sinus
➢ Look for pituitary gland distinct from mass
➢ Sag images helpful

1254 Neuroradiology
Suprasellar: Meningioma [Figures 5-20-12 and 5-20-13] Figure 5-20-12
Suprasellar: Aneurysm
• Third most common lesion in adults
• Noncalcified suprasellar mass
• Must differentiate from other suprasellar masses
➢ Macroadenoma
➢ Meningioma
Suprasellar: Aneurysm - CT
• Noncalcified central suprasellar mass
• Can be difficult to distinguish from adenoma, Suprasellar meningioma on T2 and post-contrast
meningioma T1 coronal images
Parasellar: Aneurysm - MRI [Figure 5-20-14] Figure 5-20-13

• Flow void or complex mass separate from pituitary
• Phase artifact
Figure 5-20-14
Suprasellar meningioma with typical extension

along planum sphenoidale
Figure 5-20-15
Left parasellar ICA aneurysm, with

typical flow void
Suprasellar: Aneurysm
Suprasellar Mass: Adult [Figure 5-20-15] Invasive macroadenoma vs. meningioma

• Macroadenoma
➢ Pituitary is mass Figure 5-20-16
➢ Enhancement
• Meningioma
➢ Pit separate
➢ Marked C+
➢ Dural tail
• Aneurysm
➢ Pit separate
➢ Flow void
➢ Complex SI
Suprasellar: Craniopharyngioma [Figure 5-20-16]

• Clinical
➢ Most common suprasellar mass in children
❖ 5-15 yrs
❖ 50-60 yrs
Graphic of craniopharyngioma,
➢ Visual changes
depicting complex sellar and
➢ Endocrine dysfunction
suprasellar mass
➢ Mass effect
➢ H/A, N, V, papilledema
Neuroradiology 1253
Suprasellar: Craniopharyngioma Figure 5-20-17
• Pathology
➢ Adamantinomatous
❖ Classic
❖ “Crank-case oil” in cysts
➢ Papillary (Adults)
➢ 70% suprasellar with small sellar component
➢ 5% purely intrasellar
Craniopharyngioma: CT
• NECT scan
➢ Adamantinomatous
❖ 90% Ca++ (rim)
❖ 90% Cystic
➢ May enlarge sella
➢ Papillary type
❖ 50% Ca++
❖ Majority solid
• CECT scan
➢ 90% enhance
➢ Solid Complex multiloculated sellar and suprasellar
➢ Nodular craniopharyngioma
➢ Rim
Craniopharyngioma: MR
• Variable signal
• Often heterogeneous
• Ca++ difficult to detect
• Nodular & rim enhancement
• Occasionally optic tract hyperintensity on T2WI – mass effect
Craniopharyngioma: MR [Figure 5-20-17]
Chiasmatic-hypothalamic glioma
• Clinical
➢ Second most common suprasellar mass in children
➢ Often large at presentation
➢ H/A, visual, endocrine abnormalities common
➢ M=F
➢ 15%-30% have NF-I
Chiasmatic-hypothalamic glioma
• Pathology
➢ 30% of all pilocytic astrocytomas occur in chiasm or hypothalamus
➢ 75% Pilocytic astrocytoma
➢ 25% Low-grade fibrillary
➢ Long-term survival (90% at 5 yrs, 75% at 10 yrs)
Figure 5-20-18
Chiasmatic-hypothalamic glioma - MR
• Variable signal
• Iso-, hypointense on T1WI
• Hyperintense on T2WI
• Variable enhancement
• Spread along optic tracts common
Chiasmatic-hypothalamic glioma [Figure 5-20-18]
T2 and post contrast T1 axial images show a non-

enhancing hypothalamic/chiasmic glioma

1256 Neuroradiology
Hypothalamic Hamartoma [Figure 5-20-19] Figure 5-20-19
• Clinical
➢ Precocious puberty
❖ Usually < 2yrs
➢ Gelastic seizures
➢ M>F
➢ Pallister-Hall
❖ Facial anomalies
❖ Polydactyly
❖ Imperforate anus
Hypothalamic Hamartoma Graphic and post contrast T1 sagittal image

• Pathology through hypothalamic hamartoma
➢ Hamartoma of tuber cinereum (courtesy of Amirsys, Inc.)
➢ Congenital nonneoplastic heterotopia
➢ Between infundibular stalk, mamillary bodies
Figure 5-20-20
Hypothalamic Hamartoma : MR
• Signal follows GM
• Isointense on T1WI
• May be slightly T2 hyperintense
• Pedunculated or sessile
• May project into 3rd ventricle
• Do not enhance
Hypothalamic Hamartoma
Comparison of suprasellar pediatric lesions
Suprasellar Mass: Child [Figure 5-20-20]
• Cranio
➢ Complex mass
➢ 90% cystic
➢ 90% calcified
• Astrocytoma
➢ Chiasm/Hypoth
➢ T2 hyperintense
➢ Variable C+
• Hamartoma
➢ Hypothalamus
➢ GM signal
➢ No C+
Infundibulum Differential Diagnosis

• Lesions Figure 5-20-21
➢ Germinoma
➢ Langerhans cell histiocytosis (LCH)
➢ Sarcoid
➢ Lymphoma, Metastasis
• Rare Lesions
➢ Hypophysitis
➢ Pituicytoma
Infundibular: Germinoma [Figure 5-20-21]

• Clinical
➢ Suprasellar region is 2nd most common site
➢ M = F suprasellar
➢ 90% present < 20 yrs
➢ Endocrine dysfunction
❖ Diabetes insipidus Graphic of infundibular lesion
❖ Panhypopituitarism (courtesy of Amirsys, Inc.)
➢ Very radiosensitive
➢ Up to 90% 10 survival
Neuroradiology 1255
Germinoma
• Pathology
➢ Pineal most common
➢ Pineal + suprasellar 10%
➢ Similar to seminoma
➢ 2/3 of germ cell tumors are germinoma
Germinoma: Imaging
• CT & MR
➢ Combined lesion typical but may affect only infundibular stalk
➢ May be hyperdense (CT)
➢ Isointense T1WI
➢ Hyper- to isointense T2WI
➢ Enhances homogeneously
➢ CSF dissemination common
Germinoma: MR
Langerhans Cell Histiocytosis Figure 5-20-22

• Clinical
➢ First decade
➢ M>F
➢ Diabetes insipidus
➢ High signal of neurohypophysis is commonly
absent
➢ Thickening of stalk
➢ Formerly Histiocytosis X
Langerhans Cell Histiocytosis [Figure 5-20-22]
Sarcoid
• Clinical
➢ Chronic, multisystem, inflammatory disease
➢ Noncaseating granulomas
➢ Neurologic findings 5%
➢ Diabetes insipidus or hormone deficiency
➢ Steroid responsive
Sarcoid Coronal and sagittal images show typical stalk

thickening and enhancement of LCH
Lymphoma [Figure 5-20-23]
• Clinical
➢ NHL (B-cell) Figure 5-20-23
➢ 90% supratentorial
➢ Pituitary gland, hypothalamus, stalk
➢ 6th - 7th decade
➢ AIDS: 4th decade
Lymphoma
• Imaging
➢ Pituitary gland, hypothalamus, stalk
➢ T1 Iso- to hypointense
➢ T2 hypointense Lymphoma
➢ Homogeneous enhancement

1258 Neuroradiology
Metastasis
• 1% of sellar/parasellar masses
• Usually occurs with known primary
• Can involve third ventricle, hypothalamus, infundibular stalk
• May be both supra-, intrasellar Figure 5-20-24
Metastasis: Pituitary Gland
Metastasis:Infundibulum
Infundibular Mass: Adult

• Sarcoid
➢ Systemic dz
➢ Thickened stalk Differential diagnosis of infundibular mass in a child.
➢ Enhancement
• Lymphoma
➢ +/- Systemic dz
➢ Stalk or gland
➢ Enhancement
Infundibular Mass: Child [Figure 5-20-24]

• LCH
➢ Thickened stalk
➢ “Bright spot” gone
➢ Enhancement
• Germinoma
➢ Stalk +/- pineal
➢ T2 hyperintense
➢ CSF spread
• Meningitis
➢ Meningeal
➢ Diffuse
➢ Enhancement
Presentation Summary
• Intrasellar Mass
➢ Microadenoma, Rathke cleft cyst
• Suprasellar Mass
➢ Craniopharyngioma, Macroadenoma, Meningioma, Aneurysm
• Infundibular Lesion
➢ Germinoma, LCH
➢ Granulomatous disease, LH
References
1. Bonneville JF, Cattin A, Racle A, et al: Dynamic CT of the laterosellar extradural venous spaces. AJNR 1989; 10:
535-542
2. Cottier J-P, Destrieux C, Brunereau L, Bertrand P, Moreau L, Jan M, Herbreteau D. Cavernous sinus invasion by
pituitary adenoma: MR imaging. Radiology 2000; 215:463-469
3. Elster AD, Chen MYM, Williams DW III, et al: Pituitary gland: MR imaging of physiologic hypertrophy in
adolescence. Radiology 1990;174: 681-685
4. Elster AD, Sanders TG, Vines FS, et al: Size and shape of the pituitary gland during pregnancy and post partum:
measurement with MR imaging. Radiology 1991; 181 :531-535
5. Elster AD. Modern imaging of the pituitary. Radiology 1993; 187: 1-14
6. FitzPatrick M, Tartaglino LM, Hollander MD, Zimmerman RA, Flanders AE. Imaging of sellar and parasellar
pathology. Radiol Clin North Am 1999;37:101-121
7. Sato N, Tanaka S, Tateno M, Ohya N, Takata K, Endo K. Origin of posterior pituitary high intensity on T1-
weighted magnetic resonance imaging: immunohistochemical, electron microscopic, and magnetic resonance
studies of posterior pituitary lobe of dehydrated rabbits. Invest Radiol 1995; 30:567-571
Neuroradiology 1257
Congenital Spinal Anomalies
Figure 5-21-1
Spinal Dysraphism [Figure 5-21-1]
• Defect of closure of neural tube
• For defects of primary neurulation
➢ Involving tubulation of neural plate, separation
from ectoderm, disjunction of superficial from
neural ectoderm
Myelomeningocele [Figures 5-21-2 and 5-21-3]

• Failure of neurulation & placode elevation from
expansion of SAS
➢ Placode protrudes through osseous & cutaneous
defect
• Most LS, also more proximal, normal appearing
distal cord
➢ > = 4 sites initiating neurulation, site that fails Neural tube development. Neural plate and neural
determines defect location crest derive from midline ectoderm, notochord and
somites arise from midline mesenchyme
Figure 5-21-2
Figure 5-21-3
Axial schematic of
lumbar MMC with
placode forming dorsal
wall of expanded CSF
space
Sagittal MR (22 weeks

gestation) showing posterior
osseous defect and neural
tissue traversing expanded
CSF space
Congenital Spinal Anomalies 1258

1260 Neuroradiology
Myelocele (Myeloschisis) [Figure 5-21-4] Figure 5-21-4
• Placode of OSD in plane with back
• Less common, embryologically similar
• Clinical signs & function similar
HemiMMC/Hemimyelocele
• Canal split, only one hemicord affected
• Rare, crucial to recognize as septum can tether cord --> decline
in function after repair
• Present with markedly asymmetrical neurological abnormalities
Chiari II Malformation [Figure 5-21-5]

• Absence raises suspicion that “MMC” really terminal
myelocystocele or lipoMMC Axial lumbar myelocele with placode
• From poor distention rhombencephalic vesicle, herniation CSF at level of skin defect
leakage
Figure 5-21-5
• Not assoc with CSD, may develop in TM
CSD with subcutaneous mass

• Lipoma with dorsal defect (lipoMMC/lipomyelocele(schisis))
• Terminal myelocystocele
• Meningocele
➢ Cervical myelocystoceles & meningoceles extremely rare
Lipoma with Dorsal Defect

• Premature disjunction of cutaneous ectoderm from neuroectoderm
allows mesenchyme to contact inner portion of neural tube
• As tube tries to close, mesenchyme --> fat, interferes with neurulation
• Lipomas contain ectodermal, mesodermal, endodermal elements =
teratomas
➢ Grows in proportion to overall adipose, rarely with AVM, ▲ retether
vs MMC
Lipomyelomeningocele [Figure 5-21-6] Sagittal MR (22 weeks

• Lipoma outside canal, expanded SAS gestation) with small posterior
➢ Placode deformed, with rotation toward lipoma & protrusion of fossa, hindbrain herniation,
meninges contralaterally loss of supratentoral CSF
➢ Nerve roots deformed, short on side of lipoma (tether cord), spaces
elongated on side of meninges
Lipomyelocele (lipomyeloschesis) [Figure 5-21-7]

• Lipoma traverses defect to attach to placode within or along edge of canal
Axial lumbar lipomyelomeningocele Axial lumbar lipomyelocele with

with asymmetrical placode/lipoma placode/lipoma interface along edge
interface outside canal of canal
Neuroradiology 1259
1261 Congenital Spinal Anomalies
Meningocele [Figure 5-21-8] Figure 5-21-8
• Meningeal-lined CSF sac protruding through defect
• Cord does not enter sac, may be assoc with hypertrophy of filum
or cord tethering
• ? From CSF pulsations
• Lateral assoc with NF 1, also post-trauma, connective tissue
disorders
Terminal Myelocystocele [Figure 5-21-9]

• Large, skin covered lumbosacral mass
• Dilatation of terminal ventricle
• Herniates through SB
• High assoc with caudal cell mass anomalies (GU, lower GI, abd
wall)
• Cb herniation late
• Incontinent, extremely poor LE function
Figure 5-21-9
Thoracic meningocele
Figure 5-21-10
Schematic of terminal
myelocystocele. The expanded CSF
spaces are separate from the
markedly dilated terminal ventricle.
CSD w/o Subcutaneous Mass

• Simple Dysraphic States
• Posterior spina bifida
• Intradural, intramedullary, filum terminale lipoma
• Persistent terminal ventricle
• Cutaneous stigmata: dimple, hemangioma, hair
• 4.8% nl neonates (74% simple dimple, No SB)
• Atypical dimples = high risk
➢ Larger than 5mm
➢ > 2.5cm cephalad to anus
➢ Hemangiomas, hairy patches, tails
Posterior Spina Bifida

• Most basic, commonly encountered
• L5 or S1, in isolation or with CSD when cord tethered
• Posterior arch of L5 can remain unfused until 5-6 yrs
Intraspinal Lipoma [Figure 5-21-10]

• Mesodermal cells contact primitive ependyma
• LS, any level
Intradural, extramedullary lipoma
• Usually intradural, rarely entirely intramedullary

1262 Neuroradiology
Fibrolipomatous Infiltration of Filum [Figure 5-21-11] Figure 5-21-11
• Anomaly of secondary neurulation, from totipotential caudal cell
mass
• Axial T1 most sens
➢ ?Small amount of fat anatomical variant vs any fat or
thickening (>2mm) = tether
Tight Filum Terminale [Figure 5-21-12]

• Impaired retrogressive diff --> short, hypertrophic filum
• Conus low, assoc with SB, scoliosis, ± dermal sinus
• Axial T2 for detection
Figure 5-21-12
Fibrous thickening of filum terminale seen as

hypointense on axial T2
Fat deposition within filum terminale
Persistent Terminal Ventricle [Figures 5-21-13 and Figure 5-21-13

5-21-14]
• Incomplete regression of TV of secondary
neurulation, continuity with central canal ? small
cavity
• PTV vs terminal myelocystocele (--> severe
manifestation from inability of CSF to escape)
• Common, transient finding until 5 yrs
• Identical to CSF, in conus or conus-filum transition
• Anatomic variant, no clinical sig, but > 4-5mm can --
> pain & neuro signs
Complex Dysraphic States

Disruption during gastrulation --> notochordal
derangement
• Dorsal enteric fistula Development of distal spine from caudal cell mass
• Neurenteric cysts via retrogressive differentiation
• Split cord malformation
• Dorsal dermal sinus
• Caudal regression syndrome
• Segmental spinal dysgenesis Figure 5-21-14
Gastrulation [Figure 5-21-15]

• Cells migrate towards primitive streak, through primitive groove --> endoderm
& mesoderm
• Prospective notochordal cells in cranial margin of Hensen node become
notochordal process
Dorsal Enteric Fistula

• Most multifaceted, failure of notochordal integration
• Persistence of neurenteric canal --> cleft from bowel to dorsal skin surface,
Terminal ventricle on
through vertebral column & spinal canal
axial ultrasound through
• Usually lumbar
conus medullaris
• High assoc with other CNS & non-CNS anomalies (renal, GI, CDH, pulmonary)
Neuroradiology 1261
Neurenteric Cyst [Figure 5-21-16] Figure 5-21-15
• From partial regression of neurenteric canal
• Lined by secretory epith, contents iso to CSF or proteinaceous
• Intraspinal, ventral to T cord, anywhere
• Assoc with dysplastic vert > GI, resp anom
• Present late teens, compressive signs & sx
Split Cord Malformation [Figure 5-21-17]

• Diastematomyelia (splitting) & diplomyelia (duplication), not
radiologically distinguishable
• Type I: less common, osseous septum dividing two dural tubes
& hemicords, assoc vertebral anomalies
• Type II: more common, hemicords in single dural tube ±
fibrous septum
• Each hemicord contains a central canal, dorsal horn & ventral
horn, each --> 1 nerve root
• Can be asymmetrical, smaller easily missed
• Septum --> tethering, ?SCM at CTJ under-recognized
• Despite lack of s/sx, ~ 75% abnl voiding Migration of prospective notochordal
• Cutaneous stigmata (Type I), F > M cells during gastrulation
• Present: scoliosis, ULE weak, wasting, tether
• 8%-45% of OSD, separate from site of non-neurulation --> rarity Figure 5-21-16
of hemiMMC
Dorsal Dermal Sinus [Figures 5-21-18 and 5-21-19]

• Epith-lined tube from dorsal skin to cord/coverings
• Focal nondisjunction neuroectoderm & cutaneous ectoderm
• Risk for bacterial infxn
• Epithelium secretes squamous debris, can exude cheesy material
• P/w mass or recurrent infxn
• ◗ 10% intraspinal dermoid
Figure 5-21-18
Thoracic osseous anomalies seen

with neurenteric cyst
Axial lumbar myelocele with placode

at level of skin defect
Axial lumbar myelocele with placode Scoliosis and hairy patches indicating
at level of skin defect underlying split cord malformation

1264 Neuroradiology
Caudal Regression Syndrome [Figure 5-21-20] Figure 5-21-20
• Spectrum from coccygeal/LS hypogenesis --> sirenomelia
• Infants of diabetic mothers, 1 in 7500 live births
• Assoc with:
➢ OEIS (omphalocele, exstrophy, imperforate anus, spinal
anomalies)
➢ VACTERL (vertebral, renal, cardiac, limb anomalies with
anorectal atresia & TEF)
➢ Currarino triad (sacral hypogenesis, anorectal malformations,
presacral teratoma or meningocele)
• Level determines type & severity
➢ Type I: < =S1, even mid-T, cord terminates high, blunted tip &
deformation of cauda common, ant & post separation of roots
➢ Type II: > = S2, less severe, distal-most conus absent,
tethered by tight filum, lipoma, or CSD with subQ mass
➢ Mild CRS: only tip of conus absent, cord not tethered, may be
missed
Segmental Spinal Dysgenesis

• Focal segment of lumbar or thoracic spine agenetic-markedly
hypogenetic, cord segmentally disrupted, distal canal unaffected
• Distal cord large, focal kyphosis --> early presentation
• Anomalous lower extremities, incontinence
? Within CRS, morphology depends on level of notochordal
disruption Blunted conus and absent distal
Distal --> CRS, Proximal --> SSD sacrum/coccyx of caudal regression
➢ Frequency of CRS (11:1), indicates higher degree of syndrome
susceptibility of the caudal cell mass to derangement
References
1. Barkovich AJ. Pediatric Neuroimaging. 4th Ed. Lippincott, Williams & Wilkins, Philadelphia 2000.
2. Dias MS, Partington M. Embryology of myelomeningocele and anencephaly. Neurosurg Focus 2004; 16:E1.
3. Ellison D, Love S, Chimelli L, Harding BN, Lowe J, Vinters HV. Neuropathology: A Reference Text of CNS
Pathology. 1st ed. Mosby International Ltd, London 1998.
4. Tortori-Donati P, Rossi A, Cama A. Spinal dysraphism: a review of neuroradiological features with embryological
correlations and proposal for a new classification. Neuroradiology 2000; 42:471-491.
Neuroradiology 1263
Imaging of the Suprahyoid Neck:
Superficial, Parapaharyngeal and Carotid Spaces
Wendy R. K. Smoker MS, MD, FACR
Figure 5-22-1
Cervical Fascia [Figure 5-6-1]
• Superficial Cervical Fascia
➢ Fat-filled layer of connective tissue that completely surrounds
the neck and permits the skin to glide easily over deeper
structures
• Deep Cervical Fascia
➢ Superficial Layer (Investing Fascia)
➢ Middle Layer (Visceral or Pre-tracheal Fascia)
➢ Deep Layer (Perivertebral Fascia)
Fascial Spaces of the Suprahyoid Neck

• Superficial Space
• Parapharyngeal Space
• Carotid Space
• Masticator Space
• Parotid Space
• Pharyngeal Mucosal Space
• Retropharyngeal/Danger Space
• Perivertebral Space
• Posterior Cervical Space
Superficial Space-Contents
• Sternocleidomastoid muscle
• Trapezius muscle
• Platysma muscle
• Lymph nodes
• Blood vessels/EJV Hemangioma
• Hair follicles Figure 5-22-2
• Fat
Superficial Space-Pathology
• Hair follicles
➢ Sebacceous cyst
• Blood vessels:
➢ EJV thrombosis
➢ Hemangiomas/vascular malformations
• Lymph nodes
➢ Reactive/suppurative adenopathy;
➢ Nodal metastases
• Fat
➢ Lipoma/liposarcoma
• Pseudomass
➢ Fibromatosis coli
Hemangiomas [Figures 5-22-1 and 5-22-2]

• Most common head and neck TUMOR of infancy and
childhood
• Rarely present at birth but manifest in early infancy,
grow slowly, and involute by adolescence
• 80% are isolated lesions
• Females > males Large facial hemangioma: Isointense on T1WI,
• Isointense on T1WI, hyperintense on T2WI hyperintense on T2WI, and intense enhancement
• +Enhancement
Suprahyoid Neck: Superficial, Parapharyngeal, Carotid Spaces 1266 Neuroradiology

Lymphatic Malformations [Figures 5-22-3 and 5-22-4] Figure 5-22-3
• Arise from sequestrations of the primitive embryonic
yolk sac
• Classified according to lymphatic vessel size
(capillary, cavernous, cystic) Largest is the cystic
variety, previously termed cystic hygroma.
• Typically multiseptated
• Fluid-fluid levels within the lesions are almost
pathognomonic
Madelung’s Disease
• Almost exclusively a disease of middle aged
alcoholic males
• 50 years of age
• Appearance of lesions is preceded by 10 years of
heavy drinking A large lymphatic malformation with multiple
• Non encapsulated fatty masses septations
• SIGHT DIAGNOSIS
Liposarcoma
• Slowly enlarging, painless, non-ulcerated mass
• Middle-aged onset
• Most arise de novo; frequently arise from the stroma rather than the
submucosal or subcutaneous fat Figure 5-22-4
• WHO classification recognizes 5 categories of
liposarcomas:
➢ Well differentiated (adipocytic, sclerosing, and
inflammatory subtypes)
➢ Dedifferentiated
➢ Myxoid
➢ Round cell
➢ Pleomorphic
Fibromatosis Coli [Figure 5-22-5]

(Stenocleidomastoid Tumor of Infancy)
• Diffuse SCM enlargement
2 year-old child with a large, mixed, veno-
• Most common type of congenital torticollis
lymphatic malformation
• Fibrocollagenous infiltration-Cause?????
• Typical course:
➢ Not detected at birth
➢ Palpable neck mass at 2-4 weeks of age
➢ Increases in size for a few weeks (growth phase)
➢ Most recede spontaneously at 4-8 months of age
• This is a LEAVE ALONE lesion
Figure 5-22-5
Parapharyngeal Space (PPS) [Figure 5-22-6]
• Is an “in-between” space lying between other
fascially-defined spaces. Not fascially-defined itself.
• Few lesions actually arise within this space but
typically originate from surrounding spaces and
produce characteristic encroachment on the PPS fat.
Can thereby define the space of origin.
• The “parapharyngeal space” can be considered as
consisting of two compartments:
➢ Prestyloid = Parapharyngeal Space (PPS)
➢ Retrostyloid = Carotid Space (CS)
Fibromatosis coli
Neuroradiology 1267 Suprahyoid Neck: Superficial, Parapharyngeal, Carotid Spaces

Parapharyngeal Space - Contents Figure 5-22-6
• Fat
• Branches of the mandibular nerve (V3)
• Internal maxillary artery branches
• Ascending pharyngeal artery
• Pharyngeal venous plexus
• Ectopic salivary gland tissue
Parapharyngeal Space - Pathology

[Figures 5-22-7 to 5-22-11]
• Pseudomass
➢ Asymmetric pterygoid plexus of veins
• Congenital/Vascular
➢ Atypical second BCC, hemangioma,
lymphangioma, aneurysm The normal “in-between” location of the PPS,
• Inflammatory colored in the diagram. T1W MR images
➢ Cellulitis/abscess optimally demonstrate the symmetric, “fat-
• Benign Tumor filled” PPS spaces.
➢ Pleomorphic adenoma from ectopic salivary
gland rests, neurogenic tumor, lipoma
• Malignant Tumor
➢ MECa and ACCa from ectopic salivary gland rests, direct spread from
malignancies in surrounding spaces, liposarcoma, distant mets (rare)
Figure 5-22-7
Figure 5-22-9
Small PPS hemangioma (arrows) demonstrates calcification on

the CT image. The lesion is iso-intense on T1WI,
hyperintense on T2WI, and exhibits intense enhancement
Figure 5-22-8
Small PPS
lymphangioma
(arrows) is iso-
intense on T1WIs
and demonstrates a
fluid-fluid level on
the T2WI
PPS odontogenic abscess (arrows) in

a 17 year-old male, status-post
left third molar removal 5 days
ago, now with facial pain and
swelling

Predominantly PPS lipoma. Since the PPS is not

fascially-defined, intrinsic pathology is free to
extend along fascial planes, as can be seen
with this lipoma
Pleomorphic adenoma, well-centered within the PPS, is
clearly separated from the deep lobe of the parotid
gland (arrows)
Carotid Space - Contents

• Internal carotid artery
• Cranial nerves (IX-XII)
• Sympathetic Chain
• Internal jugular vein
• Deep cervical (internal jugular) lymph node chain
Carotid Space - Pathology

• Pseudomass
➢ Ectatic CCA or ICA, asymmetric IJV (can mimic a vascular tumor)
• Congenital
➢ Second branchial cleft cysts
• Inflammatory
➢ Cellulitis/abscess, adenopathy
• Vascular Lesions
➢ IJV thrombosis or thrombophlebitis, ICA thrombosis, aneurysm, or
dissection
• Benign Tumor
➢ Paragangliomas (jugular, vagal, carotid body), nerve sheath tumors,
meningioma (from jugular foramen) Figure 5-22-12
• Malignant Tumor
➢ SCCa nodal metastases, direct invasion by SCCa, NHL,
other nodal mets
Second Branchial Cleft Cyst [Figure 5-22-12]

• The most common of the branchial cleft cysts
➢ 66%-75% in children
• “Classic location” at, or just caudal to, the angle of mandible (but
may present in a variety of locations):
➢ Posterior to the submandibular gland
➢ Anterior to the SCM
➢ Lateral to the carotid space
➢ Occasionally see a “beak” with the cyst pointing medially
between the ECA and ICA (track)
• Often enlarge with URIs and become painful if infected Classic displacements produced by
second branchial cleft cysts:
ICA Dissection with Pseudoaneurysm [Figure 5-22-13] Anterior displacement of the
submandibular gland (SMG),
medial displacement of the
carotid space (CS) structures,
and posterior displacement of the
sternocleidomastoid muscle
(SCM)

Paragangliomas Figure 5-22-13
• Highly vascular tumors arising from non-chromaffin
cells of neural crest origin
• Usually asymptomatic from endocrine standpoint but
rare catecholamine-secreting lesions do occur
• No resemblance to true glomus tumors found in skin
and superficial soft tissues so term “glomus tumor”
should be avoided
• Familial and non-familial patterns exist with
synchronous lesions seen in 5-8% of non-familial
cases and up to 25% of familial cases
• Highly vascular “Salt and Pepper” appearance of
larger lesions on MR suggestive in correct locations
• Both regional and distant (lung, liver) metastatic
disease are reported in 10-15% of cases.
• Etiology is unclear-probably due to hypoxic stimuli
• Four common locations:
➢ Middle ear cavity-tympanicum paragangliomas
➢ Jugular foramen-jugular paragangliomas Hemorrhage in the wall of the vessel is best-
➢ High carotid space-vagal paraganliomas demonstrated on non-contrast T1WI (arrow).
➢ Carotid bifurcation-carotid body paragangliomas The angiogram demonstrates the dissection
to be maximal just below the base of the skull
Jugular Paragangliomas [Figure 5-22-14] and shows a pseudoaneurysm at the level of
• Arise in adventitia of IJV, from Arnold’s nerve (IX) and approximately C1 (arrow)
Jacobson’s nerve (X)
• Represent the most common tumor found in the jugular foramen
• Permeative erosive changes with amputation of the jugular spine Figure 5-22-15
demonstrated on CT
• Multicentric in 5% of sporadic cases and up to 25%
in familial cases
• Sx: Pulsatile tinnitus; IX-XI cranial neuropathy +/- XII
• Malignant with mets in approximately 3%

Figure 5-22-14
High carotid space meningioma (arrows) is iso-

intense on T1WI, mixed intensity on T2WI,
and markedly enhances. Calcification
demonstrated on CT mitigates against
consideration of a paraganglioma
A right jugular paraganglioma extends inferiorly into the

high carotid space (arrows). Prominent flow-voids
are seen on the T1WIs. The lesion erodes the
jugular tubercle and fills the hypoglossal canal, the
normal counterparts labeled on the left (arrows).
Note late chronic denervation atrophy of the right
hemitongue

Vagale Paragangliomas [Figure 5-22-16] Figure 5-22-16
• Arise from paraganglia located around nodose
ganglion, the more caudal of the two vagal ganglia
• Situated just below skull base, lower than typical
jugulare lesions and higher than typical carotid body
tumors
• Usually lie entirely within carotid space (post-styloid
parapharyngeal space)
• As vagus nerve lies dorsal to ICA, these tumors
usually displace ICA anteriorly
• Approximately 10% incidence of malignancy
Figure 5-22-17
Large vagal paraganglioma
displaces the ICA
anteriorly with the ECA
(arrows). Large flow
voids are seen. The
MRA optimally
demonstrates the
anterior vascular
displacements (arrows)
Enhancing lesion in the high carotid space, which

displaces the ICA anteriorly (arrows), suggests
a vagal paraganglioma. However,
identification of associated calcification and
sclerosis (arrows) of the jugular tubercle, and
lack of destruction, makes the diagnosis of
meningioma

Figure 5-22-18
Carotid space vagal
schwannoma
displaces the
ICA anteriorly
(arrows). The
lesion is very
homogeneous
and exhibits no
flow voids
Figure 5-22-19
Carotid Body Paragangliomas

[Figures 5-22-19 and 5-22-20]
• Arise from paraganglia located in the “crotch” of the
carotid bifucation-most common location
• Pathognomonic finding is “splaying” of the ECA and
ICA and “filling” the bifucation
• Multiple in 5%-14% of sporadic cases and up to 33%
in familial cases
• Sx: Only 8% of these lesions are large enough to
present as carotid space mass; may have X and/or Classic displacements associated with carotid
XII neuropathy body paraganglioma with splaying of the ECA
• Malignant in 10%-15% of cases and ICA (arrows)
Figure 5-22-20
Patient with large ipsilateral

vagal and jugular
paragangliomas

References
1. Ablin DS, et al. Ultrasound and MR imaging of fibromatosis colli (sternomastoid tumor of infancy). Pediatr
Radiol. 28(4):230-233, 1998.
2. Ahuja A, et al. Madelung disease: Distribution of cervical fat and preoperative findings at sonography. MR, and
CT. AJNR Am J Neuroradiol. 19(4):707-710, 1998.
3. Alkadhi H, et al. Evaluation of topography and vascularization of cervical paragangliomas by magnetic resonance
imaging and color duplex sonography. Neuroradiology. 44(1):83-90, 2002.
4. Bancroft LW, et al. Imaging characteristics of spindle cell lipoma. AJR Am J Roentgenol. 181(5):1251-1254,
2003.
5. Bousson V, et al. Dissections of the internal carotid artery: Three-dimensional time-of-flight MR angiography and
MR imaging features. AJR Am J Roentgenol. 173(1):139-143, 1999.
6. Eldevik OP, et al. Imaging findings in schwannomas of the jugular foramen. AJNR Am J Neuroradiol.
21(6):1139-1144, 2000.
7. Fruin ME, et al. The carotid space in the suprahyoid neck. Seminars Ultrasound CT MR 11:504-510, 1990.
8. Gilbert MR, et al. Meningioma of the jugular foramen: Glomus jugulare mimic and surgical challenge.
Laryngoscope. 114(1):25-32, 2004.
9. Gilmer-Hill HS, et al. Neurogenic tumors of the cervical vagus nerve: Report of four cases and review of the
literature. Neurosurgery. 46(6):1498-1503, 2000.
10. Harnsberger et al. Diagnostic Imaging: Head and Neck. Amirsys Publishers, 2005.
11. Heis HA, et al. Carotid body tumors. Int Surg. 88(4):226-230, 2003.
12. Koeller KK, et al. Congenital cystic masses of the neck: Radiologic-pathologic correlation. RadioGraphics.
19(1):121-146, 1999.
13. Macdonald AJ, et al. Primary jugular foramen meningiomas: Imaging appearance and differentiating features.
AJR Am J Roentgenol. 182(2):373-377, 2004.
14. Mafee MF, et al. Glomus faciale, glomus jugulare, glomus tympanicum, glomus vagale, carotid body tumors, and
simulating lesions: Role of MR Imaging. Radiol Clin North Am. 38(5):1059-1076, 2000.
15. Nadgir RN, et al. Simultaneous bilateral internal carotid and vertebral artery dissection following chiropractic
manipulation: Case report and review of the literature. Neuroradiology. 45(5):311-314, 2003.
16. Noujaim SE, et al. Paraganglioma of the temporal bone: Role of magnetic resonance imaging versus computed
tomography. Top Magn Reson Imaging. 11(2):108-122, 2000.
17. Rao AB, et al. From the archives of the AFIP. Paragangliomas of the head and neck: Radiologic-pathologic
correlation. Armed Forces Institute of Pathology. RadioGraphics. 19(6):1605-1632, 1999.
18. Sharma S, et al. Fibromatosis colli in infants: A cytologic study of eight cases. Acta Cytol. 47(3):359-362, 2003.
19. Snitzer EL, et al. Magnetic resonance imaging appearance of fibromatosis colli. Magn Reson Imaging.
15(7):869-871, 1997.

Masticator and Parotid Spaces
Wendy R. K. Smoker MS, MD, FACR Figure 5-22-21
Masticator Space - Contents

[Figure 5-22-21]
• Muscles of Mastication
➢ Lateral and medial pterygoid muscles
➢ Masseter muscle
➢ Temporalis muscle
• Inferior alveolar nerve (branch of V3)
• Inferior alveolar artery and vein
• Ramus and posterior body of the mandible
Masticator Space
Normal Anatomy Coronal Plane
[Figure 5-22-22]
Figure 5-22-22
Normal anatomy of the masticator space. Axial images

best demonstrate muscles within this space. The coronal
and parasagittal images demonstrate the mandibular
division of the trigeminal nerve (V3) (arrow) and are best to
assess for perineural tumor
Figure 5-22-23
The fascia attaches to the skull base

MEDIAL to the foramen ovale putting
the foramen within this fascial
compartment. The cortical margins of
the foramen ovale are arrowed. V3 is
well-visualized traversing the foramen
There is volume loss and

complete fatty replacement
of the muscles innervated by
V3, including those
innervated by the mylohyoid
branch (mylohyoid and
anterior digastric muscles).
The responsible
meningioma is indicated by
the arrow
Suprahyoid Neck: Masticator and Parotid Spaces 1274 Musculoskeletal Radiology

Masticator Space - Pathology Figure 5-22-24
[Figures 5-22-23 to 5-22-32]
• Pseudotumors
➢ Denervation atrophy patterns
➢ “Benign” masseteric hypertrophy
➢ Accessory parotid tissue
• Congenital Lesions
➢ Hemangiomas/Lymphangiomas
• Vascular
➢ Aneurysm
• Inflammatory/Infection
➢ Odontogenic abscess, with or without, osteo is most common
Benign masseteric hypertrophy
• Benign Neoplasms
Note benign-appearing enlargement
➢ Lipomas
of the right masseter muscle. Not
➢ Neurogenic tumors
infrequently, this is perceived as a
➢ Aggressive fibromatosis (Desmoid)
parotid mass
➢ Hemangiopericytomas
• Malignant Neoplasms
➢ Malignant schwannomas Figure 5-22-26
➢ Non-Hodgkin Lymphoma
➢ Chondrosarcoma
➢ Osteogenic sarcoma
➢ SCCa spread from contiguous spaces
➢ Osseous mets to mandible
(Long Parotid Tails and) Accessory Parotid

Tissue
[Figure 5-22-25]
• Encountered in approximately 20% of the population
• May be unilateral or bilateral
• Has separate ductal system draining into Stensen’s
duct
• Neoplastic involvement is uncommon, but when it
does occur, 50% of lesions are malignant (as
oppossed to main parotid gland in which majority of
tumors are benign)
Masseter hemangioma (arrows): Isointense on
Figure 5-22-25 T1WI, hyperintense on T2WI and exhibits
enhancement
Figure 5-22-27
The right parotid tail is covering the entirety of the

masseter muscle (arrows). Patient complained of a
right cheek mass
Severe masticator space infection spreading up

along the temporalis muscle (arrows on coronal
image) with medial pyerygoid muscle abscess
(arrows on axial images). The elderly male had
suffered a fall, complained of very severe
headaches, and was suspected of having a SDH.
PMH indicated he had a dental procedure 2
months prior to his fall!
Neuroradiology 1275 Suprahyoid Neck: Masticator and Parotid Spaces
Masticator Space Infections Figure 5-22-28
[Figure 5-22-27]
• Most common cause is seeding from infected tooth following dental
manipulation
• In patients s/p zygomatic arch wiring for treatment of facial fractures, evaluate
the suprazygomatic MS
• Check bone windows for mandibular osteomyelitis
• Check all spaces for pus pockets
• Generally, one drain is necessary for each fascial space involved
Benign Neoplasms
[Figure 5-22-28]
• Neurogenic Tumors
• Aggressive Fibromatosis (Extraabdominal Desmoid)
• Lipomas
Aggressive Fibromatosis [Figures 5-22-29]

• Extra-abdominal desmoid tumor of fibrous origin
• More aggressive than their abdominal counterparts-infiltrate muscles and
encase vessels and nerves
• High recurrence rate after incomplete resection
• Isointense to muscle on T1WI
• Variable T2 signal depending on amount of fibrous tissue and collagen
• Enhance after contrast
Figure 5-22-29
Massive enlargement of
the right foramen ovale
(arrows) produced by a
large V3 schwannoma
traversing the foramen
Aggressive fibromatosis. CT demonstrates loss of the fat

planes within the left masticator space and bowing of the
posterolateral wall of the maxillary sinus (arrow). The lesion
(predominantly replacing the lateral pyerygoid muscle) is
isointense on T1WI, very hypointense on T2WI (reflecting
the very fibrous content) and demonstrates mild
enhancement (arrows)
Suprahyoid Neck: Masticator and Parotid Spaces 1276 Neuroradiology

Malignant Neoplasms
[Figures 5-22-30 to 5-22-32]
• Malignant schwannoma
• Non-Hodgkin lymphoma
• Chondrosarcoma
• Osteogenic sarcoma
• SCCa spread from contiguous spaces
• Metastases (usually mandibular)
Figure 5-22-30
Non-Hodgkin lymphoma. There is loss of the fat planes within the masticator space, including
enlargement and infiltration of the temporalis muscle in the suprazygomatic masticator space
(arrow). The right pterygopalatine fossa is enlarged indicating V2 perineural extension.
Involvement of the extraconal orbit and extra-axial middle cranial fossa are evident. Involvement
of the lateral pterygoid and temporalis muscles are evident (arrows) and extension along V2 and
V3 is noted (open arrows)
T1 WI
Chondrosarcoma - This lesion replaces fat at the

entrance to the inferior alveolar canal, widens the
foramen, and replaces the normal fatty marrow
(colored arrows). The normal fat at the entrance
to the inferior alveolar canal on the left is indicated
by the white arrows
Mandibular breast metastasis -
There is almost complete destruction
of the right mandible in this 42 year-
old woman presenting with trismus.
This was the INITIAL manifestation
of her breast carcinoma

Parotid Space - Contents Figure 5-22-33
[Figures 5-22-33 and 5-22-34]
• Parotid gland and duct (Stenson’s duct) coursing through buccal
space
• Facial nerve (VII): Creates a surgical plane that divides parotid into
superficial and deep lobes; Not usually identifiable on either CT or
MR; Courses immediately lateral to retromandibular vein
• Retromandibular vein (lateral to ECA)
• External carotid artery
• Intraparotid lymph nodes: 20-30 nodes; first order drainage for adj
scalp, EAC, and deep face
Parotid Space - Pathology [Figure 5-22-36]

• Pseudomass
➢ Long parotid tails, accessory parotid glands, parotid agenesis Stylomandibular tunnel - Superficial
• Congenital parotid space pathology may push
➢ First branchial cleft cyst; hemangiomas; lymphangiomas through and enlarge the
• Inflammatory / Infection stylomandibular tunnel (double
➢ Abscess or cellulitis; benign lymphoepithelial lesions; reactive headed arrow) and compress the
adenopathy; sialoliths; sialectasis (including autoimmune) PPS fat from a posterolateral
• Benign Neoplasm direction (shaded arrow)
➢ Pleomorphic adenoma; Warthin’s tumor; oncocytoma; lipoma;
VII neurogenic tumor Figure 5-22-34
• Malignant Neoplasm
➢ Mucoepidermoid CA; adenoid cystic CA; NHL; acinic cell CA;
malignant myxoid tumor; metastases (SCCa, melanoma, NHL)
1st Branchial Cleft Cysts [Figure 5-22-35]

• Account for 8% of BCCs
• Typical History: Middle aged female with h/o multiple parotid
abscesses unresponsive to drainage and antibiotics (Otorrhea if
cyst connects to bony-cartilaginous junction of EAC)
• If there is an external sinus, it is typically found in the skin at angle
of mandible
• Will image as cyst superficial to, within, or deep to parotid gland.
Wall thickness varies with degree of inflammation Normal anatomy: Dots=parotid
• CT is preferred to MR as it better defines cystic nature of lesion in glands; Parotid ducts (short arrows)
some cases traverse the buccal space fat and
pierce the buccinator muscles (long
Benign Lymphoepithelial Lesions [Figure 5-22-37] arrows) to enter the vestibule of the
• Old term of benign lymphoepithelial cysts replaced as both solid oral cavity opposite the second
and cystic lesions occur maxillary molar
• Typical history: Bilateral parotid swelling associated with cervical adenopathy,
usually in a patient seropositive for HIV virus (parotid lesions may occur prior
to seroconversion and may be the initial presentation)
• Imaging features: Bilateral parotid enlargement associated with intragladular
cystic and solid lesions. May see associated cervical adenopathy
Figure 5-22-35
A well-circumscribed, first
branchial cleft cyst involves the
parotid tail (arrows). Other cystic
lesions cannot be differentiated

Figure 5-22-36
Figure 5-22-37
A large hemangioma (arrows) replaces much of

the left parotid gland, isointense on T1WI, Lymphoepithelial lesions - There are multiple
hyperintense on T2WI, and exhibiting marked cystic and solid lesions in the parotid glands
enhancement bilaterally in this 27 year-old HIV positive
Sjogren’s Syndrome [Figure 5-22-38]

• Clinical triad:
Figure 5-22-38
➢ Enlarged salivary glands with xerostomia
➢ Enlarged lacrimal glands with keratoconjunctivitis
sicca
➢ Connective tissue disease (RA most common)
• Increased risk of developing a lymphoma, often
aggressive biologically
• Sialography: Punctate, globular, cavitary, and
destructive lesions can all be seen
• CT/MR: Enlarged glands with honeycomb
appearance; Some cysts may be quite large and
indistinguishable from LEL of AIDS by imaging alone
Pleomorphic Adenoma [Figures 5-22-39 and 5-22-40]

• Most common benign parotid tumor (80%); if left
untreated, est. 25% will undergo malignant Sjogren’s syndrome - The non-contrast CT scan
degeneration demonstrates cystic enlargement of the parotid
• Path: Mixture of epithelial and myoepithelial cells glands with specks of calcification identified,
• Typical patient: >50 years of age with slow-growing suggestive of an infectious process. The MR
lump in the cheek images reveal multiple, various-sized cysts in the
• Sharply marginated; round, oval, or lobulated superficial and deep lobes of both glands
• CT: Variable enhancement, infrequent dystrophic
calcification, and internal pockets of low density when large due to “mucoid
matrix”
Figure 5-22-39
• MR: Very hyperintense on T2WI; may
have internal pockets of greater
hyperintensity if “mucoid matrix”
Classic superficial lobe pleomorphic adenoma

Large deep lobe
pleomorphic adenoma
herniates through and
widens the right
stylomandibular tunnel
(arrows)
Warthin Tumor:
Warthin’s Tumor Large cystic/solid right parotid superficial mass in a
(Papillary Cystadenoma Lymphomatosum) 65 year-old man. The heterogeneity would suggest
[Figure 5-22-41] a lesion other than a pleomorphic adenoma
• Second most common benign tumor of the parotid space Figure 5-22-42
• 80% male, greater than 50 years of age
• Usually slow-growing mass in region of parotid tail
• Arise from ectopic salivary gland rests within
intraparotid lymph nodes
• Limited to PAROTID GLAND ONLY
• Bilateral in 10%-15% of patients
• Imaging: Well-circumscribed, usually 3-4 cm
➢ Complex mixture of solid and cystic components
➢ Appears more complex than typical PA
Oncocytoma
• Occurs exclusively in adults over 50 years of age; No
sex predilection; about 1% of parotid tumors
• Oncocytes are large cells with granular eosinophilic
cytoplasm that may be found in groups, normally
within the parotid gland. Multiple facial nerve neurofibromas (arrows) in a
• An ONCOCYTOMA describes a solid tumor patient with NF2. Also note the ipsilateral V2
composed entirely of oncocytes neurofibroma in the right retromaxillary fat, bowing
• Imaging features are essentially identical to those of the posterolateral maxillary wall
a pleomorphic adenoma Figure 5-22-43
• May be multiple
Malignant Tumors
• The smaller the salivary gland, the higher
the chance a mass is malignant
• Sublingual mass-70% malignancy
• Submandibular mass-60% malignancy
• Parotid mass-20% malignancy
• Mucoepidermoid Carcinoma-more than 80%
occur in parotid glands; most common
malignant salivary gland tumor in most
series
• Adenoid Cystic Carcinoma-2%-6% of parotid
tumors
• Acinic Cell Carcinoma-10%-30% of parotid
tumors
• Adenocarcinoma-rare in major salivary Low-grade superficial parotid lobe mucoepidermoid
glands carcinoma. The lesion is somewhat complex and exhibits
indistinct margins laterally (arrows) leading away from the
diagnosis of a benign neoplasm

Mucoepidermoid Carcinoma [Figure 5-22-43] Figure 5-22-44
• Most common malignant lesion in parotid gland
• Most common between 35 and 60 years of age but
may be found at any age and is most common
malignant tumor in persons under 20 years of age.
• Slight predilection for women
• Clinical: Rock hard mass with associated pain and
itching over the course of the facial nerve.
• Imaging features depend on grade:
➢ Low grade: Benign appearance; cannot
distinguish from pleomophic adenoma
➢ Higher grade: Infiltrating, indistinct margins; look
for PNT along VII!!!
➢ Low to intermediate signal on T1 and T2WI
References VII Perineural Tumor - This patient has recurrent

adenoid cystic carcinoma of the parotid gland.
1. Curtin HD. Detection of perineural spread: Fat Note perineural extension of tumor along the facial
suppression versus no fat suppression. AJNR Am J nerve (VII) up through the stylomastoid foramen
Neuroradiol. 25(1):1-3, 2004. (arrow on coronal) to extend to the geniculate
2. Go JL, et al. The trigeminal nerve. Semin Ultrasound CT ganglion (arrow on axial)
MR. 22(6):502-520, 2001.
3. Harnsberger et al. Diagnostic Imaging: Head and Neck.
Amirsys Publishers, 2005.
4. Holliday RA, et al. Benign lymphoepithelial parotid cysts and hyperplastic cervical adenopathy in AIDS risk
patients: A new CT appearance. Radiology. 168:439-441, 1998.
5. Izumi M, et al. MR imaging of the parotid gland in Sjogren’s syndrome: A proposal for new diagnostic criteria.
AJR. 166:1483-1487, 1996.
6. Marsot-Dupuch K, et al. Mandibular nerve: MR versus CT about 10 proved unusual tumors. Neuroradiology.
32:492-496, 1990.
7. Palacios E, et al. Benign asymmetric hypertrophy of the masticator muscles. Near Nose Throat J. 79(12):915,
2000.
8. Russo CP, et al. MR appearance of trigeminal and hypoglossal motor denervation. AJNR Am J Neuroradiol.
18(7):1375-1383, 1997.
9. Shah GV. MR imaging of salivary glands. Magn Reson Imaging Clin N Am. 19(4):631-662, 2002.
10. Triglia JM, et al. First branchial cleft anomalies: A study of 39 cases and a review of the literature. Arch
Otolaryngol Head Neck Surg. 124(3):291-295, 1998.
11. Tryhus MR, et al. The normal and diseased masticator space. Semin Ultrasound CT MR. 11:476-485, 1990.
12. Williams LS, et al. MR imaging of the trigeminal ganglion, nerve, and the perineural vascular plexus: Normal
appearance and variants with correlation to cadaver specimens. AJNR Am J Neuroradiol. 24(7):1317-1323, 2003.
13. Yonetsu K, et al. Deep facial infections of odontogenic origin: CT assessment of pathways of space involvement.
AJNR. 19:123-128, 1998.

Pharyngeal Mucosal Space and Oral Cavity
Pharyngeal Mucosal Space Figure 5-22-45

[Figures 5-22-45 and 5-22-46]
• Extends from skull base to hyoid bone and
includes the nasopharynx, oropharynx, and
hypopharynx
• The mucosa lining the upper aerodigestive tract
Pharyngeal Mucosal Space - Contents

• Mucosa
• Waldeyer’s ring of lymphatic tissue
• Minor salivary glands (esp. in soft palate)
• Superior and middle pharyngeal constrictor
muscles
• Cartilaginous (distal) end of eustachian tube
(torus tubarius) (nasopharynx)
• Levator palatini muscle (nasopharynx)
Pharyngeal Mucosal Space - Pathology

• Pseudomass
➢ Lymphoid hyperplasia
• Congenital
➢ Tornwaldt cyst
• Infection / Inflammatory
➢ Post-inflammatory cysts (retention cysts);
cellulitis/abscess
• Benign Neoplasms Extension of the nasopharynx
➢ NPSCCa; non-Hodgkin lymphoma; minor salivary gland neoplasms
(mucoepidermoid Ca, adenoid cystic Ca…)
Figure 5-22-46
A) Arrows indicate cartilaginous ends of eustachian tubes;

B) short arrows=medial pterygoid muscles; long arrows=PPS fat;
C) long arrows on PPS fat; short arrows indicate air in lateral pharyngeal recesses
Suprahyoid Neck: Pharyngeal Mucosal Space and Oral Cavity 1282 Neuroradiology
Nasopharyngeal Carcinoma (NPSCCa) [Figures 5-22-47 and 5-22-48] Figure 5-22-47
• Centered in lateral pharyngeal recess
• Invasion of levator palatini results in eustachian tube
dysfunction and serous otitis media with CHL (check
mastoids!)
• Perivascular spread (via foramen lacerum) and
perineural extension (mainly V3) are common so
skull base must be carefully assessed
• Nodal metastases present in 90% at presentation
(retropharyngeal, level II, and level V-first)
• Distant metastases-uncommon at presentation
(<10%) (bone, lung, and liver)
• Strong relationship with Epstein-Barr virus (EBV)
PMS Non-Hodgkin Lymphoma (NHL)

[Figure 5-22-49]
• Tends to remain localized and grow slowly
• NHL in H&N from nasopharyngeal lymphoid tissue in
35% of cases (versus palatine tonsil lymphoma in CT shows NPSCCa of left lateral recess (arrows)
50% and lingual tonsil lymphoma in 15%) with ipsilateral foramen ovale enlargement (arrow).
• 50% have associated adenopathy at presentation MR also demonstrates the mass (arrow) with
• 20% may have GI tract NHL involvement enhancing perineural tumor extending through the
• AIDS and Sjogren’s Syndrome predispose foramen (arrow)
• Usually >50 years; M:F = 1.5:1
• Bulky mass filling nasopharynx Figure 5-22-48
• T1WI-isointense, T2WI-hyperintense, enhance
48 year-old male
with bilateral IX-XII
Figure 5-22-49
palsies
Very large
NPSCCA replaces
the entire
basiocciput (distal
clivus), occipital
condyles, jugular
tubercles, and
portions of the C1
lateral masses
(seen on the
coronal images).
Coronal images
also demonstrate
extensive bilateral
This homogeneous, bulky, NHL mass fills the adenopathy
nasopharynx but exhibits no infiltrtion of adjacent
spaces and no skull base involvement
Neuroradiology 1283 Suprahyoid Neck: Pharyngeal Mucosal Space and Oral Cavity
Oropharynx Figure 5-22-50
[Figure 5-22-50]
Oral PMS Anatomy
Oro - PMS Pathology

[Figure 5-22-51]
• Congenital
➢ Thyroglossal duct cyst; lingual thyroid
• Infection / Inflammatory
➢ Post-inflammatory cysts (retention
cysts); cellulitis/abscess (tonsillar)
➢ SCCa (base of tongue and faucial
pillars); non-Hodgkin lymphoma; minor
salivary gland malignancies (especially
soft palate)
• Pseudomass
➢ Lymphoid hyperplasia (lingual tonsil)
Extension of the oropharynx
Thyroglossal Duct Cysts
[Figure 5-22-52]
• Most common non-odontogenic extracranial head and neck cyst Figure 5-22-51
• 20% are suprahyoid in location
• 2-4 cm
• Occasionally septated
• Capsular enhancement
• Smooth margins
• DDx: submental node
Figure 5-22-52
Oropharyngeal Mucosal Space Anatomy
A) Arrows indicate tonsillar pillars; B) Long arrows

indicate tonsillar pillars; short arrows indicate fat in
PPS; C) Long arrows indicate tonsillar pillars;
short arrow indicates soft palate; D) Arrows
indicate regions of glossotonsillar sulci
Foramen cecum thyroglossal duct cyst (arrows).
Lingual Thyroid Gland Figure 5-22-53
[Figure 5-22-53]
• Failure of thyroid descent from foramen cecum
• Accounts for 90% of ectopic thyroid
• High female predominance (7:1)
• Only functioning thyroid tissue in 70-80%
• CT: Hyperdense with avid enhancement
• MR: Iso-to hyperintense to muscle on both T1- and
T2WI with strong enhancement
• Look in lower neck to confirm lack of gland in normal
location
• Subject to typical “thyroid pathology”
Figure 5-22-54
Lingual thyroid gland. Note absence of thyroid in

normal location in lower neck
Figure 5-22-55
Bilateral tonsillar abscesses

Figure 5-22-56
Right tonsillar SCCa with an

ipsilateral level 2A node
Large base of tongue SCCa
Oral Cavity Normal Anatomy Figure 5-22-57
[Figures 5-22-57 to 5-22-59]
Figure 5-22-58
The location of the oral cavity.

The oral cavity is separated from the
oropharynx by the circumvallate papillae,
the soft palate complex, and the anterior
tonsillar pillars
Figure 5-22-59
T1 WI
A: Sagittal MR.
Black arrows=intrinsic muscles of the
tongue;
white arrows=genioglossus muscles;
dotted arrows=geniohyoid muscles
T1 WI
B. Coronal MR.
Vertical white arrows=sublingual
spaces;
horizontal black/white
arrows=submandibular spaces;
vertical black/white arrows=platysma
muscles
Oral Cavity Pathology Figure 5-22-60
[Figure 5-22-60]
• Congenital Lesions
➢ Hemangiomas/vascular
malformations; dermoids/epidermoids
• Inflammatory Lesions
➢ Cellulitis/abscesses; sialoliths;
ranulas
➢ Pleomorphic adenomas; aggressive
fibromatosis
➢ SCCa (floor of mouth, oral tongue…);
minor salivary gland neoplasms
(SMG, SLG)
Dermoid Cysts
[Figure 5-22-61]
• Refers to dermoid. epidermoid, and
teratoid lesions
• Most uncommon of the congenital lesions Multiple oral cavity venous malformations in a child with Blue
• Sublingual and submandibular locations Rubber Bleb Nevus Syndrome
• Low density/intensity, unilocular, well-circumscribed Figure 5-22-61
• Cyst wall enhances with contrast
• Individual fat globules=dermoid
• In absence of fat, cannot DDx epidermoid from
dermoid
Ranulas
[Figures 5-22-62 and 5-22-63]
• Mucoceles/mucous retention cysts of the floor of the
mouth
• Secondary to trauma or obstruction of sublingual
gland/ducts
• Thin-walled; unilocular; non-enhancing
• Two varieties:
• Simple ranula-in SLS (true)
• Plunging ranula-in SMS (pseudocyst)
Dermoid cysts in 2 patients with classic “bag of

Figure 5-22-62 marbles” appearance
Figure 5-22-63
Simple ranula in a 30 year-old

female with obstructive sleep apnea Plunging ranulas in 3 different patients
Pleomorphic Adenomas Figure 5-22-64
[Figure 5-22-64]
• Most common benign glandular tumor--majority in
parotid gland
• 8% in submandibular gland; 0.5% in sublingual gland
• Well demarcated; homogeneous when small
• Heterogeneous (cystic changes necrosis,
hemorrhage)
• Hypo-isointense on T1WI
• Hyperintense on T2WI
• Homo/heterogeneous enhancement
Exostoses
[Figure 5-22-65]
• Dense cortical bone w/ or w/o cancellous bone
• Incidental unless they preclude proper denture fitting
• Occasionally very large and interfere with swallowing
• Torus palatinus
• Torus mandibularis Sublingual gland pleomorphic adenoma
➢ interna / externa
• Torus maxillaris
➢ interna / externa
Figure 5-22-65
Figure 5-22-66
This T3N2b oral tongue SCCa (double arrows)

invades the ipsilateral mylohyoid muscle (dots) to
extend to the floor of the mouth. An ipsilateral
node is indicated by the single arrow
Arrows indicate torus palatinus,

bilateral mandibular tori interna, and
bilateral maxillary externa tori
Figure 5-22-67
Left floor of mouth SCCa (arrows)

extends to block the submandibular
gland ducts bilaterally (arrows)
References
1. Fischbein NJ, et al. Clinical utility of positron emission tomography with 18F-fluorodeoxyglucose in detecting
residual/recurrent squamous cell carcinoma of the head and neck. AJNR Am J Neuroradiol. 19(7):1189-1196,
1998.
2. Harnsberger et al. Diagnostic Imaging: Head and Neck. Amirsys Publishers, 2005.
3. King AD, et al. In vivo proton MR spectroscopy of primary and nodal nasopharyngeal carcinoma. AJNR AM J
Neuroradiol. 25(3):484-490, 2004.
4. Mukherji SK, et al. Squamous cell carcinoma of the oropharynx and oral cavity: How imaging makes a
difference. Semin Ultrasound CT. 19:463-475, 1998.
5. Roh JL, et al. Nasopharyngeal carcinoma with skull base invasion: A necessity of staging subdivision. Am J
Otolaryngol. 25(1):26-32, 2004.
6. Sigal R, et al. CT and MR imaging of squamous cell carcinoma of the tongue and floor of the mouth.
RadioGraphics. 16:787-810, 1996.
7. Smoker WRK, et al. Computed tomography of the nasopharynx and related spaces. Seminars Ultrasound CT MR.
7:107-130, 1986.
7. Smoker WRK. The Oral Cavity in Head and Neck Imaging (4th ed) Som and Curtin, eds. Mosby Year Book
Publishers. pp 1377-1464, 2002.
8. Weber AL, et al. Malignant tumors of the oral cavity and oropharynx: Clinical, pathologic, and radiologic
evaluation. Neuroimaging Clin N Am. 13(3):443-464, 2003.
Spine: Degenerative Disease and
Infections
Figure 5-23-1
Low Back Pain Annual Costs
• 250,000 operations/year
• 18-56 billion dollars/year
• 85% of costs are due to recurrent or chronic disability
Degenerative Disc Disease

Lumbar Nomenclature [Figures 5-23-1 and 5-23-2]
• Normal
• Bulge (symmetric, asymmetric)
• Annular tear/fissure
• Herniation (focal or broad-based)
➢ Protrusion
➢ Extrusion
➢ Extrusion with free fragment
Annular Tear/Fissure [Figure 5-23-3]

• Typically seen with degenerated discs but even seen in 25% of
patients < 20 years of age
• Disc protrusions/extrusions often associated
• Primary failure of the annulus – all layers involved
• Present with back/radicular pain
• MR: 1-2 mm band of increased T2 signal (High intensity zone-HIZ) Disc bulges
➢ linear area of contrast enhancement (96%) Upper: Symmetric
➢ not as sensitive as discography Lower: Asymmetric (>50% of
Figure 5-23-3 circumference)
Figure 5-23-2
Multilevel disc bulges
Note high intensity zone on

these T2WIs (arrows)
Spine: Degenerative Disease and Infections 1288

1290 Neuroradiology
Disc herniations and types [Figures 5-23-4 and 5-23-5]

Focal: <25% of Broad-based: Protrusion Extrusion
circumference > 25% but < 50% of
circumference
Protrusion Extrusion
Extrusion with migration plus sequestered fragment
L4-5 Disc Extrusion with Migration [Figure 5-23-6]
Figure 5-23-6
Degenerative changes typically

induce secondary changes in the
adjacent vertebral bodies This extruded disc retains a connection to the parent L4-5 disc
(degenerative discovertebral as it migrates down behind the L5 vertebral body (arrows)
changes) (Modic Changes)
Low T1WI
Type I Vascularized Marrow (edema) High T2WI
High T1WI
Type II Proliferation of endplate fatty marrow High T2WI
Low T1WI
Type III Dense bone devoid of marrow (sclerosis) Low T2WI
Neuroradiology 1289
1291 Spine: Degenerative Disease and Infections
Lumbar Spinal Canal and Foraminal Stenosis Figure 5-23-7
Lumbar Facet Arthropathy [Figures 5-23-7 and 5-23-8]
Figure 5-23-8
Multilevel degenerative disease with severe right L1-2 foraminal

stenosis from a combination of disc bulge, facet arthropathy,
and ligamentum flavum buckling
Facet Joint Synovial Cysts [Figure 5-23-9] Note the nerve root exits
• Intraspinal juxta-articular synovial cysts are uncommon; associated with facet superiorly within the
arthopathy neural foramen, just
• Most in lumbar spine, especially L4-5 under the pedicle
• Slight female preponderance; mean age 58 years (arrow). Note the normal
• Origin: DJD? Trauma?? “keyhole” configuration
• No specific symptom history; waxing and waning symptoms as these increase of the neural foramen
and decrease in size
• CT: Cystic lesion w/ or w/o calcified rim adjacent to degenerated facet
• MR: Iso- or slightly hyperintense on SE sequences
Figure 5-23-9
• DDx: Ganglion cyst (does not communicate with joint)
Failed Back Surgery Syndrome

(FBSS) [Figure 5-23-10]
• 10%-40% of patients
• Intractable pain with variable incapacitation
• Differential considerations include:
➢ Recurrent/residual disc herniation
➢ Post-op infection
➢ Second level disease
➢ Facet disease
➢ Arachnoiditis
➢ Neuritis Synovial cyst produces
➢ Epidural fibrosis (“Scar”) compression on the
➢ Miscellaneous thecal sac from a
posterolateral location
(arrows). Compression
of the nerve roots is best
appreciated on the MR
myelogram (right image)

1292 Neuroradiology
FBSS: Type I Arachnoiditis [Figure 5-23-11] Figure 5-23-10
Figure 5-23-11
FBSS: Recurrent Disc. A large recurrent disc

(arrows) demonstrates mild enhancement
but the surrounding epidural fibrosis
enhances to a much greater degree
Type I arachnoiditis. Nerve roots are

clumped centrally within the thecal sac
(arrows)
FBSS: Type II Arachnoiditis [Figure 5-23-12]
Figure 5-23-12
Type II arachnoiditis. Nerve roots are

“plastered” to the margins of the thecal sac
producing an “empty” appearance (large
arrows). The more proximal nerve roots are
seen as distinct entities (small arrows)
Cervical Spinal Canal Stenosis
Cervical Spinal Canal Stenosis [Figures 5-23-13 and 5-23-14]
Figure 5-23-13
Congenital cervical spinal canal stenosis

may be produced by either pedicle or lamina
hypoplasia. The underlying dimensions of
the canal should be assessed on every study
as even mild degenerative disease can be
significant in the face of underlying
congenital stenosis
Neuroradiology 1291
Ossification of the Posterior Figure 5-23-14
Longitudinal Ligament (OPLL) C2
[Figures 5-23-15 and 5-23-16]
• Begins with calcification followed by
frank ossification of the posterior
longitudinal ligament in upper C-spine
and progresses into upper T-spine C4-5
• Can see on plain films in 0.12% of
asymptomatic North Americans and
20%-30% of symptomatic patients
• Most easily seen on CT
• On MR, thick band of decreased signal C5-6
on T1 and T2WI with mass effect on
thecal sac and cord
• Associated with DISH
Acquired cervical spinal canal stenosis. Disc disease, coupled

with ligamentum flavum buckling, produces severe canal
stenosis at multiple levels (dark arrows) with associated
pathologic signal in the spinal cord (white arrows), most severe
Figure 5-23-15 at C5-6
T1WI
OPLL and DISH

Thick hypointense signal is identified
between the spinal cord and posterior
vertebral bodies on MR (arrows). CT
confirms the diagnosis of OPLL severely
compromising the spinal canal diameter.
DISH is present at lower levels
Figure 5-23-16
Ossification of Ligamentum Flavum

Marked thecal sac and cord compression
produced by OLF

1294 Neuroradiology
Infections Figure 5-23-17
Pyogenic Osteomyelitis
[Figures 5-23-17]
• Ill-defined T1-hypointense vertebral
marrow with loss of adjacent endplate
definition on both sides of infected disc;
hyperintense on T2WI; enhances
• Lumbar-48%; Thoracic-35%; Cervical-
6.5%
• Paraspinal and/or epidural involvement
in 75%
• S aureus most common; E coli if gram
negative; salmonella in sickle cell
• Source: GU, GI, lung, cardiac,
cutaneous/mucous-seeds vascularized
subchondral bone
• Disc first site of involvement in children
(vascularity)
Pyogenic osteomyelitis. Note paraspinal and epidural
• Bimodal: Pediatric patients and 6th-70th
involvement (arrows).
decade; also IV drug abusers and those
with HIV
• Pain, tenderness, and fever Figure 5-23-18
Post Gd
Tuberculous Osteomyelitis
[Figure 5-23-18]
• AKA: Pott’s disease
• Typical: Gibbus vertebrae with relatively
intact disc and paraspinal abscess
• Abscesses dissect over considerable
length T2 WI
• Mid-thoracic or thoracolumbar most
common
• Inoculum in anterior vertebral body;
spreads under anterior longitudinal
ligament; spares discs due to absence
of proteolytic enzymes
• M=F; presents in 50s; fever infrequent Tuberculous osteomyelitis. Note gibbus deformity and
• Concomitant pulmonary TB in 10% extension under the anterior longitudinal ligament (arrows)
Epidural Phlegmon / Abscess

[Figures 5-23-19 and 5-23-20]
• S aureus most common (57-73%), then Figure 5-23-19
TB (25%)
• Predisposing conditions: IV drug users,
immunocompromised states, DM, CRF,
alcoholism…
• Anterior from adjacent discitis or
osteomyelitis
• Posterior from GU, GI, lung, cardiac…
• Direct inoculation from penetrating
trauma, surgery….
• Peaks in 50s and 70s
• Lumbar EA may mimic herniated disc
Epidural Phlegmon. A definite abscess cavity is not defined

but abnormal enhancing soft tissue fills the posterior epidural
space (arrows)
Neuroradiology 1293
Miscellaneous Figure 5-23-20
Brachial Plexus Traction Injury

[Figure 5-23-21]
• AKA: Traumatic meningocele
• Avulsion of root(s) of brachial plexus
invariably from traumatic injuries:
(Adults-ATVs, motorcycles…; infants:
complicated deliveries)
• Location critical to planning and
prognosis:
➢ Pre-gang (central to dorsal root
ganglion)-worse prognosis
➢ Post-gang (peripheral to dorsal
root ganglion)-better prognosis
• Demonstration of “rootless”
meningoceles are diagnostic Epidural and paraspinal abscesses.
The paraspinal abscess is confirmed on the DWIs.
Idiopathic Transdural Cord The etiology was a septic right facet joint
Herniation
[Figure 5-23-22]
• Typical presentation is Brown-Sequard
Syndrome
• Clinical deficits tend to be progressive
unless treated
• Surgical reduction of the herniated cord
Figure 5-23-21
can lead to improvement in the motor
deficit
• Typical patient is an adult, affected
location is the upper and mid-thoracic
level (T2-T7)
• ??Cause??
➢ Cong weakness of ventral dura?
➢ Damage by disc hernitation?
➢ Abnormal adhesions of cord to
dura?
➢ Large root sleeve diverticulum?
• Interruption of the usually smooth Brachial Plexus Traction Injury
ventral margin of the cord over a short (8 year-old boy s/p ATV accident with “dead” right arm).
segment (pulled anteriorly) Multiple traumatic meningoceles are demonstrated (arrows)
• Primary differential: Is cord is being
displaced by a posterior mass
(arachnoid cyst?) or is cord tethered Figure 5-23-22
ventrally?
Idiopathic transdural spinal cord

herniation (arrows)

1296 Neuroradiology
References
1. Akman S, et al. “Magnetic resonance imaging of tuberculous spondylitis”. Orthopedics. 26(1):69-73, 2003.
2. Ben Hamouda K, et al. “Thoracic myelopathy caused by ossification of the ligamentum flavum: A report of 18
cases”. J Neurosurg. 99(2 Suppl):157-161, 2003.
3. Boos N, et al. “Classification of age-related changes in lumbar intervertebral discs: 2002 Volvo Award in basic
science”. Spine. 27(23):2631-2644, 2002.
4. Carragee EJ. “The clinical use of magnetic resonance imaging in pyogenic vertebral osteomyelitis”. Spine.
22(7):780-785, 1997.
5. Cinotti G, et al. “Stenosis of lumbar intervertebral foramen: Anatomic study on predisposing factors”. Spine.
27(3):223-229, 2002.
6. Consensus statement on nomenclature and classification of lumbar disc pathology by NASS, ASSR, and ASNR.
2001.
7. Dix JE, et al. “Spontaneous thoracic spinal cord herniation through an anterior dural defect”. AJNR Am J
Neuroradiol. 19(7):1345-1348, 1998.
8. Doi K, et al. “Cervical nerve root avulsion in brachial plexus injuries: Magnetic resonance imaging classification
and comparison with myelography and computerized tomography myelography”. J Neurosurg. 96(3 Suppl):277-
284, 2002.
9. Eastwood JD, et al. “Diffusion-weighted imaging in a patient with vertebral and epidural abscesses”. AJNR Am J
Neuroradiol. 23(3):496-498, 2002.
10. Fassett DR, et al. “Spinal epidural lipomatosis: A review of its causes and recommendations for treatment”.
Neurosurg Focus. 16(4):Article 11, 2004.
11. Geers C, et al. “Polygonal deformation of the dural sac in lumbar epidural lipomatosis: Anatomic explanation by
the presence of meningovertebral ligaments”. AJNR Am J Neuroradiol. 24(7):1276-1282, 2003.
12. Modic MT, et al. “Degenerative disk disease: Assessment of changes in vertebral body marrow with MR
imaging”. Radiology. 166(1 Pt 1):193-199, 1988.
13. Munter FM, et al. “Serial MR imaging of annular tears in lumbar intervertebral disks”. AJNR Am J Neuroradiol.
23(7):1105-1109, 2002.
14. Ross JS, et al. “Assessment of extradural degenerative disease with Gd-DTPA-enhanced MR imaging:
Correlation with surgical and pathologic findings”. AJNR Am J Neuroradiol. 10(6):1243-1249, 1989.
15. Ross JS, et al. “Association between peridural scar and recurrent radicular pain after lumbar discectomy:
Magnetic resonance evaluation”. Neurosurgery. 38:855-861, 1996.
16. Ross JS, et al. “MR imaging of lumbar arachnoiditis”. AJR. 1987;149:1025-1032.
17. Sasaoka R, et al. “Idiopathic spinal cord herniation in the thoracic spine as a cause of intractable leg pain: Case
report and review of the literature”. J Spinal Disord Tech. 16(3):288-294, 2003.
18. Van Goethem JW, et al. “Review article: MRI of the postoperative lumbar spine”. Neuroradiology. 44(9):723-
239, 2002.
19. Wang MY, et al. “Intradural spinal arachnoid cysts in adults”. Surg Neurol. 60(1):49-55; discussion 55-56, 2003.
20. Watters MR, et al. “Transdural spinal cord herniation: Imaging and clinical spectra”. AJNR Am J Neuroradiol.
19(7):1337-1344, 1998.
Neuroradiology 1295
Spinal Tumors, Cysts, and Mimics
First Things First!! - Localize the Lesion!! [Figure 5-24-1]

Figure 5-24-1
Extradural Extramedullary Intramedullary

Intradual
Localizing a spinal lesion to the appropriate “compartment” (extradural, extramedullary-intradural, or
intramedullary) allows a tailored differential diagnosis
Common Intramedullary Lesions
• Non-neoplastic:
➢ Acute trauma (contusion, edema…)
➢ Syringohydromyelia
❖ Syrinx-cavity in cord NOT lined by ependyma
❖ Hydromyelia-dilatation of the central canal lined by ependyma
➢ Demyelinating disease (MS, ADEM…)
• Neoplastic
➢ Ependymoma
➢ Astrocytoma
THERE IS NO SPECIFIC IMAGING PATTERN THAT RELIABLY

PERMITS DIFFERENTIATION BETWEEN EPENDYMOMAS AND
ASTROCYTOMAS
Uncommon Rare
• Non-neoplastic Lesions • Non-neoplastic Lesions
➢ Acute cord ischemia/stroke ➢ Vascular lesions (cavernomas, AVM, etc)
➢ Myelitis (Post viral ADEM, etc) ➢ Infections (sarcoid, TB, Lyme disease…)
• Neoplastic Lesions • Neoplastic Lesions
➢ Hemangioblastoma ➢ Metastases
➢ Astrocytoma (anaplastic and GBMs)) ➢ Lipoma
➢ Subependymoma
➢ Oligodendroglioma
➢ Ganglioma
➢ Paraganglioma
Spinal Tumors, Cysts, and Mimics 1296

1298 Neuroradiology
Ependymomas [Figure 5-24-2] Figure 5-24-2
• Most common intramedullary tumor in
adults- 60%
• Mean age 43 years; females
predominate (slightly)
• Arise from ependymal cells lining
central canal
• Slow-growing; canal expansion is
typical
• 56% cervical; 28% thoracic; 16%
lumbar
• 75% are isointense on T1WI; 100%
hyperintense on T2WI
• Cystic degeneration and hemorrhage
are common
• Hemosiderin deposition-common at Ependymoma - Note lesion isointensity on T1WIs and
periphery relatively homogeneous enhancement on the Post-Gd images.
• Heterogeneous enhancement in 65% This lesion exhibits unusual hypointensity on the T2WI, most
likely due to the prominent amount of hemosiderin noted on
Astrocytomas [Figure 5-24-3] histology. Also note the associated areas of cystic
• Second most common IM tumor overall; degeneration, indicated by the arrows. Significant cord edema
most common tumor in children in best appreciated as marked increased signal intensity on the
• Cervical=thoracic; M=F; mean age 21 T2WI
years Figure 5-24-3
• Typically pilocytic and diffuse fibrillary
types (low grade); anaplastic
astrocytomas and GBMs are rare
• May extend to involve the entire cord
• Cyst formation is common; Syrinx-
above or below tumor
• Iso- to hypointense on T1WI;
Hyperintense on T2WI
• Hemorrhage is LESS COMMON than
for ependymomas
• Virtually 100% enhance
Hemangioblastoma [Figure 5-24-4]

• Uncommon; 1%-5% of cord tumors
• Peak age between 30 and 40 years
• One third associated with VHL
syndrome
• 85% intramedullary or combined
intramedullary/ extramedullary-
intradural
• 50% thoracic; 40% cervical; 80% are
solitary
• Isointense on T1WI; hyperintense on
T2WI; strong enhancement; may see
flow voids, etc. Ependymoma - Lesion demonstrates isointensity on T1WIs,
• On angio, highly vascular mass; dense hyperintensity on T2WIs, and manifests only very faint
prolonged tumor stain, prominent enhancement on post-Gd images (somewhat unusual). A large
draining veins signal void is present on the T2WIs, possibly representing an
area of hemosiderin/calcification. Significant edema is also
best appreciated on the T2WIs
Neuroradiology 1297
1299 Spinal Tumors, Cysts, and Mimics
Intramedullary Metastases [Figure 5-24-5] Figure 5-24-4
• Intramedullary metastases are rare
• 1%-3% of all intramedullary tumors
• No specific imaging characteristics to
clearly distinguish from other
intramedullary lesions Breast and lung
most common
• Also lymphoma, leukemia, and
malignant melanoma
Non-Neoplastic Intramedullary
Pathology
Cavernoma [Figure 5-24-6]
Acute Disseminated
Encephalomyelitis [Figure 5-24-7]
Figure 5-24-5 Classic appearance of an intramedullary hemangioblastoma

with T1 isointensity, T2 hyperintensity, and marked
enhancement with numerous flow-voids, best seen on the T2
and post-Gd images (arrows)
Figure 5-24-6
Classic cavernoma appearance with central slight

T1 hyperintensity and heterogeneous T2
hyperintensity (popcorn appearance). Note the rim
of hemosiderin which “blooms” on the T2WI. The
finding of multiple areas of intracranial
hemosiderin on the gradient echo (GRE) image
further solidifies the diagnosis.
(Courtesy M Modic)
Figure 5-24-7
Intramedullary Metastasis (79 year-old female with

lung carcinoma and brain metastases) - Note subtle Acute Disseminated Encephalomyelitis (15 year-
enlargement of the spinal cord without significant old male with headache, lethargy, and nuchal
signal change on the T1WI, hypointensity of the rigidity) - Note significant cord expansion with
lesions on the T2WI, and lesion enhancement. The slight T1 hypointensity, marked T2 hyperintensity,
smaller lesion (arrow) would be almost impossible to and very patchy enhancement. This boy had a
detect without contrast URI two weeks prior to onset of symptoms

1300 Neuroradiology
Multiple Sclerosis [Figure 5-24-8]
Extramedullary-Intradural Lesions
Common Uncommon
• Neurogenic Neoplasms • Lipomas

➢ Neurofibromas • Arachnoid Cysts
➢ Schwannomas • Epidermoids/Dermoids
• Meningiomas • Drop metastases
• Myxopapillary • AVM
Ependymomas • Infection
• Arachnoiditis • Paragangliomas
Figure 5-24-8
Nerve Sheath Tumors
• Most common extramedullary-intradural tumors
(70%-75%). 15% extradural; 15% dumbell
• Schwannomas slightly more common than
neurofibromas
• Neurofibromas: M=F; 20-30 years. No true capsule
➢ Localized, diffuse, or plexiform
• Schwannomas: M=F; 30-60 years. Encapsulated
➢ 40% cystic changes; 10% hemorrhage; target
sign
• Neural foraminal enlargement; pedicle thinning
• Isointense on T1WI; Hyperintense on T2WI; Enhance
• Multiple in neurofibromatosis
Multiple Sclerosis - Note absence of significant
spinal cord expansion. The MS plaques are not
appreciated on the T1WI, only faintly suspected on
[Figures 5-24-9 to 5-24-11]
the T2WI, but very well seen on the STIR
Neurofibroma Schwannoma sequence. Faint enhancement of one, probably
more active, plaque can be seen on the post
• Schwann cells • Schwann cell neoplasm contrast sequence (arrow)
• Fibroblasts • Secondary vascular
• Acellular material changes Figure 5-24-9
• Infiltrating • Mostly cellular
• Resect parent nerve • Encapsulated
• Nerve sparing surgery
Figure 5-24-10
T1WI T2WI Post Gd
Schwannoma at the level of the conus. Sagittal

images nicely demonstrate the classic Classic dumbbell neurofibroma at C2-3
subarachnoid cap at the inferior margin of the demonstrates isointensity on T1WIs,
lesion with widening of the subarachnoid space. hyperintensity on the T2WI and manifests
Note hypointensity on the T1WI and hyperintensity very significant enhancement
on the T2WI. The post-contrast images on the coronal post-Gd image
demonstrate a nice “target sign.” The lesion
occupies most of the spinal canal and significantly
compresses the spinal cord, best seen on the axial
post contrast image (arrow)
Neuroradiology 1299
Meningiomas [Figures 5-24-12 and 5-24-13] Figure 5-24-11
• Second most common spinal tumor (25%)
• 90% intradural; 10% extradural or dumbell
• Females > males at 4:1; Primarily 5th-6th decade
• Thoracic (80%) > Cervical (16%) > Lumbar (4%)
• More often anterior in cervical region
• Below C7, more common posterior to cord
• 85% intradural; usually single unless NF 2
• Iso on T1WI; iso or hyperintense on T2WI;
hypointense if Ca++
• Marked homogeneous enhancement
Figure 5-24-12
A large schwannoma is seen arising from the S1

nerve root (arrows), isointense on T1WI,
heterogeneously hypointense on T2WI, and
primarily peripheral enhancement following
contrast
Figure 5-24-13
Meningioma (C Spine-Ventral) - The sagittal

images clearly localize this lesion as
extramedullary-intradural by demonstrating a nice
subarachnoid cap (widening of the subarachnoid
space-arrows). The lesion severely compresses
the spinal cord, best appreciated on the axial T2WI
image (outlined). The lesion is essentially
isointense to cord on T1 and T2WIs
Myxopapillary Ependymoma [Figure 5-24-14]

• 27%-30% of all ependymomas; 90% of filum tumors Meningioma (T Spine-Dorsal) - Note lesion
• Genetically different from intracranial ependymomas isointensity on T1 and T2WIs and homogeneous
• Occur exclusively in conus and filum terminale-from enhancement. The subarachnoid cap is best
ependymal cells in filum appreciated at the superior margin of the tumor on
• Males:females = 2:1; peak between 30 and 40 years the T2WI
• Slow growing and may fill entire lumbar spinal canal Figure 5-24-14
• Vertebral scalloping; canal enlargement
• Highly vascular—hemorrhage is common
• Iso on T1WI; hyper on T2WI; hypointense margin if
hemosiderin
• Intense enhancement in 100%
“Other” Extramedullary-Intradural
Neoplastic Lesions
A large myxopapillary ependymoma fills and

expands the lumbar spinal canal, scalloping the
lower vertebrae. Note isointensity on the T1WI,
hyperintensity on theT2WI and marked
enhancement. The small superior component
(arrow) was suspected on the T2WI but only
confirmed on the post-contrast image

1302 Neuroradiology
Hemangioblastoma [Figure 5-24-15] Figure 5-24-15
Lipomas [Figure 5-24-16]

• Originate from fat cells in subpial region
• Vertebral and dermal anomalies are NOT associated
with these lipomas
• 25% are diagnosed within the first 5 years of life
• No sex predilection All Post-Gd
• Excessive weight gain and pregnancy may
predispose
Figure 5-24-16
Small hemangioblastoma in a patient with von

Hipple Lindau syndrome. The enlarged feeding
vessels (arrows) are well-demonstrated and may
initially suggest a diagnosis of AVF
Figure 5-24-17
Large cervical lipoma has caused marked

widening of the spinal canal and severe spinal
cord compression. The lesion manifests fat signal
intensity on the T1 and T2WIs. The intraoperative
photograph shows fat bulging through the dura
Type III Meningeal Cysts (Arachnoid Cysts)

[Figure 5-24-17]
• Intradural arachnoid cysts are rare
• Unclear etiology: ?congenital; ?hemorrhage;
?inflammation
• Thoracic spine is most common location
• 80% arise near septum posticum and located
posterior to cord
• Most communicate with subarachnoid space
• CTM: compressed cord displaced anteriorly
• MR: signal intensity of CSF so may not be able to
identify unless cord is displaced/deformed
Dermoid Cysts [Figure 5-24-18]

• Congenital (100%)
• Symptomatic before age 20; M=F
• 80% in lumbosacral or cauda
• Hypointense areas-? Water content from sweat gland
secretions
• Fat hyperintensity on T1WI
• May cause chemical meningitis if rupture with A large dorsal arachnoid cyst has produced
cholesteol crystals discharged into CSF marked expansion of the spinal canal with severe
pedicle thinning and cord compression. The
compressed spinal cord is outlined on the soft-
tissue windows.
The lesion exhibits fluid signal intensity on the T1
and T2WIs. Note severe cord compression
(arrows)
Neuroradiology 1301
T1WI
Implantation Epidermoid Cyst (7 year-old female-had LP as an

infant) - The lesion is slightly hyperintense on the T1WI, very
hyperintense on the T2WI (such that the margins cannot be
separated from the surrounding CSF), and does not enhance.
A tract from the prior LP is not identified
Lumbar dermoid cyst. The
complex nature of the lesion with
identification of fat signal (high) Figure 5-24-20
on the T1WI should suggest the
diagnosis.
(Courtesy M Modic)
Epidermoid Cysts [Figure 5-24-19]
• Less than 1% of spinal tumors
• Congenital-60%; Aquired-40%
• Upper thoracic-17%; lower thoracic-
26%; lumbosacral-22%; cauda equina-
35%
• Strong association with lumbar puncture
in neonatal period (implantation
epidermoid)
• Iso or slightly hyperintense on T1WI;
hyper on T2WI
• Mild rim enhancement
• DWI can DDx from arachnoid cysts Drop Metastases (25 year-old woman with breast CA and
and other lesions radicular symptoms) - Numerous enhancing lesions on the
cauda equina are identified
Drop Metastases
[Figures 5-24-20 and 5-24-21]
• CNS Primary Tumors Figure 5-24-21
➢ Astrocytomas
➢ Medulloblastomas
➢ Pineal cell tumors
➢ Ependymomas
➢ Germ cell tumors
• Non-CNS Primary Tumors
➢ Breast
➢ Lung
➢ Lymphoma
➢ Melanoma
➢ Pituitary
Diffuse Mantle Cell Lymphoma.
Note marrow signal in the thoracic spine is
diffusely abnormal (darker that that of the
intervertebral discs). There is infiltration of
virtually all of the nerve roots of the cauda
equina such that they are not visualized as separate entities on the sagittal image. Enlargement of
individual roots is best appreciated on the axial images
1304 Neuroradiology
Extradural Lesions
Common Uncommon
• Epidural Metastases • Epidural Abscess

• Herniated discs, etc • Arachnoid cysts
• Degenerative lesions (osteophytes, • Lipomas
ligament infolding…) • Primary vertebral tumors-benign
• Lymphoma • Primary vertebral tumors-malignant
• Infection (discitis, etc…) • Paget’s disease
• Epidural hematoma
• Epidural lipomatosis
• Extramedullary hematopoesis
• Angiolipomas
Epidural Hematoma [Figure 5-24-22]
Epidural Lipomatosis
[Figures 5-24-23 and 5-24-24]
• Most often associated with chronic steroid use (exogenous or endogenous
Cushing’s syndrome)
• Also associated with rapid weight gain and obesity
• Thoracic spine most common
• Myelopathic symptoms predominate Figure 5-24-22
Figure 5-24-23
Three year-old girl with 2 day h/o neck pain and

UE/LE weakness - A large epidural hematoma
(from C2 to T5) is producing severe spinal cord
compression. A fluid-fluid level is indicated by the
arrows. (Courtesy A Gean)
Figure 5-24-24
Epidural Lipomatosis - Severe

accumulation of dorsal epidural fat in
the thoracic region is producing
severe cord compression
Epidural lipomatosis is producing severe

compression of the cauda equina in the lumbar
spine
Neuroradiology 1305 Spinal Tumors, Cysts, and Mimics

References
1. Arslanoglu A, et al. “MR imaging characteristics of pilomyxoid astrocytomas”. AJNR Am J Neuroradiol.

24(4):1906-1908, 2003.
2. Chu BC, et al. “MR findings in spinal hemangioblastoma: Correlation with symptoms and with angiographic and
surgical findings”. AJNR Am J Neuroradiol. 22(1):206-217, 2001.
3. Cohen-Gadol AA, et al. “Spinal meningiomas in patients younger than 50 years of age: A 21-year experience”. J
Neurosurg. 98(3 Suppl):258-263, 2003.
4. Conti P, et al. “Spinal neurinomas: Retrospective analysis and long-term outcome of 179 consecutively operated
cases and review of the literature”. Surg Neurol. 61(1):34-43; discussion 44, 2004.
5. Fujiwara F, et al. “Intradural spinal lipomas not associated with spinal dysraphysm: A report of four cases”.
Neurosurgery. 37(6):1212-1215, 1995.
6. Garg RK. “Acute disseminated encephalomyelitis”. Postgrad Med J. 79(927):11-17, 2003.
7. Hickman SJ, et al. “Imaging of the spine in multiple sclerosis”. Neuroimaging Clin N Am. 10(4):689-704, viii,
2000.
8. Khong PL, et al. “Childhood acute disseminated encephalomyelitis: The role of brain and spinal cord MRI”.
Pediatr Radiol. 32(1):59-66, 2002.
9. Kikuchi K, et al. “The utility of diffusion-weighted imaging with navigator-echo technique for the diagnosis of
spinal epidermoid cysts”. AJNR Am J Neuroradiol. 21(6):1164-1166, 2000.
10. Koeller KK, et al. “Neoplasms of the spinal cord and filum terminale: Radiologic-pathologic correlation”.
RadioGraphics. 20:1721-1749, 2000.
11. Murphey MD, et al. “Imaging of musculoskeletal neurogenic tumors: Radiologic-pathologic correlation”.
RadioGraphics. 19:1253-1280, 1999.
12. Potgieter S, et al. “Epidermoid tumours associated with lumbar punctures performed in early neonatal life”. Dev
Med Child Neurol. 40(4):266-269, 1998.
13. Simon JH. “Brain and spinal cord atrophy in multiple sclerosis”. Neuroimaging Clin N Am. 10(4):753-770,
2000.
14. Sun B, et al. “MRI features of intramedullary spinal cord ependymomas”. J Neuroimaging. 13(4):346-351, 2003.
15. Thakkar SD, et al. “Spinal tumours in neurofibromatosis type I: An MRI study of frequency, multiplicity and
variety”. Neuroradiology. 41(9):625-629, 1999.
16. Wanebo JE, et al. “The natural history of hemangioblastomas of the central nervous system in patients with von
Hippel-Lindau disease”. J Neurosurg. 98(1):82-94, 2003.
17. Wang MY, et al. “Intradural spinal arachnoid cysts in adults”. Surg Neurol. 60(1):49-55; discussion 55-56, 2003.
18. Yamada CY, et al. “Myxopapillary ependymoma of the filum terminale”. AJR Am J Roentgenol. 168(2):366,
1997.

1306 Neuroradiology
Congenital Abnormalities of the Brain
Figure 5-25-1
Timing of Events
• Disorders of Dorsal Induction
➢ (3-4 weeks)
• Disorders of Ventral Induction
➢ (5-10 weeks)
• Disorders of Neuronal Proliferation
➢ (2-5 months)
Disorders of Dorsal Induction

(Insult at 3-4 weeks)
• Cranioschesis
➢ Anencephaly
➢ Cephaloceles
• Chiari Malformations
➢ Chiari I
➢ Chiari II (Arnold Chiari Malformation)
➢ Chiari III
➢ Chiari IV (Cerebellar hypoplasia)
Anencephaly
• Symmetric absence of calvaria, cerebral hemispheres,
diencephalon
• Replaced by flat amorphous vascular- neural mass
• Facial structures and orbits present
• Most common CNS malf (1:1000)
• Invariably fatal (aborted/stillborn)
• Implications for future pregnancies:
➢ 1 in 50 (2%) risk in next child
➢ if 2 prior pg with CNS mal, risk is 1 in 10)
Cephaloceles
• Skull base (enchondral bone)
➢ Failure of neural tube closure or failure of ossification centers
to unite Large cephalocele containing brain,
• Calvarium (membraneous bone) ventricles, and midline fat. Note
➢ Defective bone induction, pressure erosion, or failure of neural absence of the corpus callosum and
tube closure an open-lip schizencephalic cleft
• Occipital (Arrow)
➢ 80%-90%; F>M; assoc with neural tube defects; most
common in Caucasian NAs and Europeans
• Frontal
• Sincipital
➢ Nasofrontal
➢ Nasoethmoidal-Most common in SE Asia; M>F
➢ Nasoorbital
• Basal
➢ Transethmoidal, transsphenoidal, sphenomaxillary, sphenoorbital
Frontal Lipoencephalocystocele [Figure 5-25-1]
Neuroradiology 1305
1307 Congenital Abnormalities of the Brain
Chiari I Malformation Figure 5-25-2
• Probably due to occipital bone dysplasia and small posterior
fossa
• Caudal displacement of “pegged” cerebellar tonsils into upper
cervical spinal canal
• Associated with:
➢ Syringohydromyelia (25%-50%)
➢ CVJ anomalies in up to 50%
➢ Associated with many brainstem/lower cranial nerve sxs
(hearing loss, vertigo, abnormal gag/swallowing, etc.)
Chiari I Malformation [Figure 5-25-2]

• Tonsillar ectopia
➢ number of millimeters the tonsillar tips extend below the
foramen magnum (basion to opisthion) Chiari I Malformation. Note
❖ < 3mm – Normal protrusion of cerebellar tonsils
❖ 3-5mm – “Low-lying” (arrow) below the plane of the
❖ >5 mm is quoted to be 100% specific and 92% sensitive foramen magnum (dotted line) and
for Chiari I cervical syringohydromyelic cavity.
• Difference with age
➢ tonsils regress with age The fourth ventricle (dot) is normal in
➢ between 5-15 years, even 6mm may be OK if asymptomatic location and configuration
• Up to 50% have osseous CVJ anomalies
➢ Basiocciput hypoplasia (short clivus)
➢ Platybasia
➢ Atlanto-occipital non-segmentation
➢ Non-segmentation of C2-C3
➢ Klippel-Feil deformity
Chiari II Malformation - (Arnold Chiari Malformation)

• Downward displacement of the cerebellar tonsils, inferior cerebellar vermis,
fourth ventricle, and medulla into the upper cervical spinal canal
• Very commonly associated with a lumbar myelomeningocele
Chiari II Malformation - Imaging findings

Figure 5-25-3
[Figures 5-25-3 to 5-25-5]
• Luckenschadel skull-universal at birth
➢ Typically associated with Chiari II
➢ An ossification disturbance of the membraneous skull
➢ Is NOT the result of hydrocephalus
➢ Disappears by approximately 6 months of age
• Macrocephaly with colpocephaly
• Large massa intermedia
• Falx and tentorium hypoplasias
• Beaking of the tectum
• “Triple peak” appearance of pons/medulla
• Low torcula (shallow posterior fossa)
• Syringohydromyelia (50%)
Luckenschadel Skull-plain and CT
Congenital Abnormalities of the Brain 1306

1308 Neuroradiology
Chiari II Malformation-sagittal.
Note tectal beaking,
caudal displacement of cerebellar tonsils to C4, slit-like,
caudally-elongated fourth ventricle,
vertical straight sinus (small posterior fossa),
and concavity of the clivus
Chiari III Malformation [Figure 5-25-6]

• Herniation of cerebellum +/- brainstem, fourth ventricle, and upper
cord into a low occipital or high cervical encephalocele
Figure 5-25-6 Chiari III Malformation
Chiari IV Malformation [Figure 5-25-7]

• A severe cerebellar hypoplasia with essentially a CSF-filled
posterior fossa
• Very small brainstem—pons most severely affected
Figure 5-25-7 Chiari IV Malformation
Chiari II Malformation-axial. Note

tectal beaking in A (arrow); “triple-
peak” appearance in B (arrows); and
tentorial hypoplasia in C (arrows)
Neuroradiology 1307
Disorders of Ventral Induction Figure 5-25-8
(Insult at 5-10 weeks)
• Holoprosencephaly
➢ Alobar, semilobar, lobar
➢ Septo-optic dysplasia (DeMorsier’s syndrome)
• Cerebral hemiatrophy (Dyke-Davidoff-Masson syndrome)
• Posterior fossa malformations
➢ Dandy Walker malformation and variants
➢ Joubert’s syndrome
➢ Rhombencephalosynapsis
Alobar Holoprosencephaly [Figure 5-25-8] Alobar holoprosencephaly

• Most severe form-no diverticulation
• Midline facial deformities (cycloplegia, cebocephaly, hypertelorism, Figure 5-25-9
hypotelorism, cleft anomalies…)
• Microcephaly with monoventricle (absent septum pellucidum)
DDx: Hydranencephaly
• Horseshoe-shaped forebrain
• Fusion of basal ganglia and thalami
• Absence of: Corpus callosum, falx/interhemispheric fissure,
olfactory tracts and bulbs
• Azygos anterior cerebral artery
Alobar Holoprosencephaly - DDx: Hydranencephaly

[Figure 5-25-9]
• CSF replaces cerebrum supplied by the internal carotid arteries
bilaterally (ICA occlusions occurring between 3 and 6 months in
utero)
• Cerebellum, brainstem, and thalami are not involved as they are
supplied by the posterior circulation
• Associated with: TORCH infections, maternal syphillis,
abdominal trauma, radiation
Hydranencephaly.
Semilobar Holoprosencephaly [Figure 5-25-10] Note normal, non-fused thalami and
• Small brain with monoventricle (absent septum pellucidum) presence of falx anteriorly which
• Falx may be present distinguish this from
• Fusion of basal ganglia and thalami alobar holoproencephaly
• Corpus callosum may be absent or incomplete
• Some rudimentary sulcation may be present
• Olfactory bulbs and tracts are absent
• Azygos anterior cerebral artery
Lobar Holoprosencephaly [Figure 5-25-11]

• LEAST severe form
• Normal size brain
• Monoventricle (absence of the septum pellucidum)
• Usually no facial deformities
• Lack of cleavage is usually subtle and frontal
• CORONAL IMAGING IS BEST TO DETECT
Septo-Optic Dysplasia (DeMorsier’s Syndrome) [Figure 5-25-12]

• Partial or complete absence of the septum pellucidum
• Hypothalamic / pituitary axis abnormalities in 66%
• Absent fornix and corpus callosum dysgenesis
• Schizencephaly in approximately 50%
• Diminutive optic nerves and chiasm

1310 Neuroradiology
Lobar holoprosencephaly
Figure 5-25-12
Semilobar holoprosencephaly
Septo-optic dysplasia
Neuroradiology 1309
Cerebral Hemiatrophy Figure 5-25-13
(Dyke-Davidoff-Masson Syndrome) [Figure 5-25-13]
• Clinical: Hemiparesis, mental retardation, seizures
• Small cerebral hemisphere from in utero ischemia or infarction
after infection or trauma
• Compensatory ipsilateral:
➢ Lateral ventricular enlargement
➢ Calvarial thickening
➢ Enlarged paranasal sinuses
➢ Enlarged mastoid air cells
Posterior Fossa Malformations

• Dandy-Walker Complex
➢ Dandy-Walker malformation
➢ Dandy-Walker variant
➢ Mega cisterna magna
• Joubert’s syndrome
• Rhombencephalosynapsis
Dandy-Walker Malformation [Figure 5-25-14]

• Hypoplasia of the cerebellar hemispheres, hypoplasia or aplasia
of the inferior cerebellar vermis, and marked enlargement of the
fourth ventricle
• Etiology unclear but probably NOT failure of development of
fourth ventricular foranima
• Prominent imaging features include:
➢ Enlarged posterior fossa—macrocephaly
➢ Torcular-lambdoid inversion
➢ Absence of the falx cerebelli
➢ Hydrocephalus (91% at diagnosis)
➢ Other brain anomalies are common
• 66% have associated anomalies including corpus callosal
hypogenesis(30%)
• Polymicrogyria/ heterotopia(5%-10%),
• Occipital cephaloceles (16%), syringohydromyelia
• Association with various syndromes – Fetal Alcohol, TORCH,
Aicardi , Klippel Feil…..
Cerebral hemiatrophy.
Small left cerebral hemisphere. Note
left mastoid air cells and frontal sinus
are larger than right counterparts.
The left middle cranial fossa is also
diminutive
Figure 5-25-14
Note hydrocephalus,
torcular-lambdoid inversion
with large posterior fossa
cyst, and severe vermian
hypoplasia

1312 Neuroradiology
Joubert’s Syndrome [Figure 5-25-15] Figure 5-25-15
• Rare syndrome of vermian aplasia with
Joubert’s Syndrome.
brainstem hypoplasia
Note vermian
• Autosomal recessive inheritance
hypoplasia and the
• Variable symptoms: Ataxia, mental
classic “molar tooth”
retardation, pendular nystagmus,….
appearance of the
• “Molar-tooth sign” from prominent superior
brainstem, best
cerebellar peduncles
appreciated on the
• “Bat-wing” shape to fourth ventricle
axial images
(Courtesy M Castillo)
Rhombencephalosynapsis [Figure 5-25-16]

• Congenital fusion of cerebellar hemispheres,
dentate nuclei, and superior cerebellar
peduncles with vermian agenesis
• “Keyhole” configuration of fourth ventricle
• Absent septum pellucidum +/- SOD or
holoprosencephaly
• Often hydrocephalus (aqueduct stenosis)
• Clinical: Variable depending upon other
anomalies
Figure 5-25-16
Disorders of Neuronal Proliferation
(Insult at 2-4 months)
• Microcephaly (usually intrauterine ischemia)
• Generalized Megalencephaly
• Unilateral Megalencephaly (hemimegalencephaly)
• Hydranencephaly
• Neurocutaneous Disorders
• Aqueduct Anomalies (stenosis, etc.)
Unilateral Megalencephaly
(Hemimegalencephaly) [Figure 5-25-17]
• Clinical: Intractable siezures, mental retardation, hemiplegia,
developmental delay
• Cerebral hemisphere may appear so anomalous as to appear
unrecognizable
• Association with Linear Sebaceous Nevus syndrome
• Multiple migration and sulcation anomalies
➢ Polymicrogyria/Agyria
➢ Grey matter heterotopias
Figure 5-25-17
Rhombencephalosynapsis
2 year-old male with ataxia and
developmental delay
Hemimegalencephaly. Note enlargement of the right hemisphere with

diffuse cortical thickening in a pachygyric appearance with abnormal
sulcation. The ipsilateral lateral ventricle is markedly enlarged
Neuroradiology 1311
Disorders of Neuronal Proliferation Figure 5-25-18
(Insult at 2-5 months)
• Lissencephaly (agyria, pachygyria)
• Non-lissencephalic cortical dysplasias (microgyria and
polymicrogyria)
• Schizencephaly
➢ Type I (closed-lip); Type II (open-lip)
• Grey matter heterotopias
• Callosal abnormalities
➢ Complete/partial agenesis
➢ Pericallosal lipomas
Lissencephaly [Figure 5-25-18]

• “Smooth Brain”
• Interruption during last phase of migration (11-26 weeks)
• Usually severe disabilities, developmental delay, seizures…
• Lissencephaly Type I (Classic)
• Lissencephaly Type II (Cobblestone)
Lissencephaly Type I
• Arrested neuronal migration
• Also termed agyria-pachygyria complex
• Thick gray and thin white matter
➢ Agyria: Parietal-occipital
➢ Pachygyria (“incomplete lissencephaly”): frontal and
temporal
• “Figure 8” or hourglass configuration
• Assoc with Miller-Dieker syndrome
➢ Large deletion gene on chromosome 17p13.3
Lissencephaly Type II
• Also termed “Cobblestone” lissencephaly Classic lissencephaly
• Due to neuronal overmigration
• Associations include: Figure 5-25-19
➢ Walker-Warburg Syndrome
➢ Fukuyama congenital muscular dystrophy
Non-Lissencephalic Cortical Dysplasias

Microgyria/Polymicrogyria
• Innumerable, small cerebral convolutions with thickened cortex
and abnormal cortical histology
• Cortex around Sylvian fissure commonly involved
• DDx: Stenogyria (packed gyri) associated with the Chiari II
malformation
• Typically associated with other migrational disorders
Schizencephaly (Cleft Brain) Type I

(Closed-Lip (Fused)) [Figure 5-25-19]
• Cleft with fusion of opposing grey matter layers (no intervening
CSF) extending from cortex to ventricle
• Fusion of ventricular ependyma and pia covering the brain (pial-
ependymal seam)
• Ventricular diverticulum (“tit”) at base of cleft is very useful for
identification of closed cleft
• May have near-normal mentation +/- seizures and spasticity;
bilateral=worse prognosis
• 35%-50% have septo-optic dysplasia
Closed-lip schizencephaly. Note gray matter lining the cleft and a
ventricular “tit” at base of the seam (arrow). Patient also has septo-
optic dysplasia—note absence of the septum pellucidum
1314 Neuroradiology
Schizencephaly Type II Figure 5-25-20
(Open-Lip (Separated)) [Figure 5-25-20]
• CSF interposed between grey matter lining the cleft
• IF NO GREY MATTER INTERPOSED, CONSIDER
PORENCEPHALY
• Usually severe mental retardation, seizures, hypotonia, spasticity,
blindness, inability to walk or speak
• Absent septum pellucidum (80%-90%)
• Genetic counseling is required as there is a 5%-20% incidence of
brain abnormalities in siblings
Grey Matter Heterotopias [Figures 5-25-21 to 5-25-23]

• Arrest of migrating neurons between ventricle and pial surface of
the brain (looks like GM on all imaging sequences)
• Seizures and mental retardation are common
• Associated with schizencephaly, callosal agenesis, agyria,
hemimegalencephaly…
• Three varieties
➢ Nodular/periventricular-subependymal (nodules of GM indent
lateral ventricles; irregular ventricular walls)
➢ Focal or diffuse subcortical (clumps of GM within the WM)
➢ Band (circumferential and symmetric)
❖ Most severe form with worst prognosis
❖ Neurons fail to reach their destination. Open-lip schizencephaly in two
❖ Confluent band of gray matter between lateral ventricle different patients. Note absence of
and cortex separated from both by layer of white matter septum pellucidum in the patient with
bilateral schizencephaly

Nodular/periven
tricular
heterotopia—
note signal
intensity of the
periventicular
nodules is
identical to that
of cortical grey
matter Band heterotopia with the
classic “double cortex” sign
Figure 5-25-22 Bilateral subcortical nodular heterotopia (A and C-arrows) with complete callosal
dysgenesis noted in B. Also note closed-lip schizencephaly in A (open arrow)
A B C
Neuroradiology 1313
Callosal Dysgenesis [Figures 5-25-24 and 5-25-25] Figure 5-25-24
• Corpus callosum forms anterior to posterior:
➢ Genu ---> Body ---> Splenium --->Rostrum
• Complete Agenesis
➢ Clinical: Siezures, developmental delay, microcephaly
➢ Imaging:
❖ Absent corpus callosum
❖ Elevated third ventricle
❖ Separated lateral ventricles
❖ Colpocephaly
• Partial Dysgenesis
➢ Acquired: Anterior CC is affected
➢ Developmental: Posterior CC is affected A
• Lipomas
➢ Associated with ACC in 40%
➢ Located in interhemispheric fissure
➢ Often encase the pericallosal arteries
Figure 5-25-25
B
Partial callosal dysgenesis: (A) The
splenium is absent. (B) Note gyri
separating the occipital horns in a
position normally occupied by the
splenium on the axial image
Callosal dysgenesis (complete). Note radially arranged gyri

converging toward the third ventricle in A; parallel, separated lateral
venticles in B; Probst bundles (arrows); vertical orientation to
hippocampi in C; and colpocephaly in D D

1316 Neuroradiology
References
1. Altman NR, Naidich TP, Braffman BH. Posteri or fossa malformations. AJNR Am J Neuroradiol 13:691-724,
1992
2. Barkovich AJ, Chung SH, Norman D. MR of neuronal migration anomalies. AJNR Am J Neuroradiol 8:1009-
1017, 1987
3. Barkovich AJ, Chung SH. Unilateral megalencephaly: Correlation of MR imaging and pathologic characteristics.
AJNR Am J Neuroradiol 11:523-531, 1990
4. Barkovich AJ, Jackson DE, Jr., Boyer RS. Band heterotopias: A newly recognized neuronal migration anomaly.
5. Barkovich AJ, Kjos BO, Norman D, Edwards MS. Revised classification of posterior fossa cysts and cystlike
malformations based on the results of multiplanar MR imaging. AJNR Am J Neuroradiol 10:997-988, 1989
6. Barkovich AJ, Kjos BO. Grey matter heterotopias: MR characteristics and correlation with developmental and
neurological manifestations. Radiology 182:493-499, 1992
7. Barkovich AJ, Kjos BO. Non-lissencephalic cortical dysplasia: Correlation of imaging findings with clinical
deficits. AJNR Am J Neuroradiol 13:95-103, 1992
8. Barkovich AJ, Kjos BO. Schizencephaly: Correlation of clinical findings with MR characteristics. AJNR Am J
Neuroradiol 13:85-94, 1992
9. Barkovich AJ, Norman D. MR imaging of schizencephaly. AJNR Am J Neuroradiol 9:297-302, 1988
10. Barkovich AJ, Norman D. Anomalies of the corpus callosum. AJNR Am J Neuroradiol 9:493-501, 1988
11. Barkovich AJ. Subcortical heterotopia: A distinct clinicoradiologic entity. AJNR Am J Neuroradiol 17:1315-1322,
1996
12. Byrd S, Osborn R, Bohan T, Naidich T. The CT and MR evaluation of migration disorders of the brain, II:
Scizencephaly, heterotopia, and polymicrogyria. Pediatr Radiol 19:219-222, 1989
13. Catilo M. Bouldin TW, Scatliff JH, Suzuki K. Radiologic-pathologic correlation: Alobar holoprosencephaly.
14. Fitz CR. Holoprosencephaly and related entities. Neuroradiol 25:225-238, 1983
15. Naidich TP, Altman NR, Braffman BH, McLone DG, Zimmerman RA. Cephaloceles and related malformations.
16. Osenbach RK, Menezes AH. Diagnosis and management of the Dandy-Walker malformation: 30 years of
experience. Pediatr Neurosurg 18:179-189, 1992
17. Truit CL, Barkovich AJ, Shanahan R, Marlado TV. MR imaging of rhombencephalosynapsis: Report of three cases
and review of the literature. AJNR Am J Neuroradiol 12:957-965, 1991
Neuroradiology 1315
Neuroradiology Seminar 1:
Discussion of Unknown Cases
History
• 6-year-old girl with 10 days of vomiting
Pediatric Posterior Fossa Tumors

• Medulloblastoma (PNET):1/3
• Juvenile pilocytic astrocytoma (JPA): 1/3
• Brain stem glioma: 1/6
• Ependymoma: 1/6
Medulloblastoma or JPA?
• Use non-contrast CT
• Medulloblastoma: hyperdense
• JPA: iso- or hypodense
• Most reliable imaging feature to distinguish between these tumors
Medulloblastoma
• Most common (?) childhood brain tumor
• Childhood: 75% < 15 y/o, 50% < 10 y/o
➢ Peak 4-8 y/o; second peak 15-35 y/o
• Vermis into fourth ventricle
• Cysts, calcification, hemorrhage rare
• CT: 90-95% homogeneous, slightly hyperdense on NCCT, uniform
enhancement
Medulloblastoma
• MR
• Cerebellopontine angle involvement rare
• CSF spread: 20-25% at time of diagnosis
➢ Check spine post-gad after brain MRI
Neuroradiology Seminar 1 1318 Neuroradiology

History
• 46-year-old male with marked short-term memory loss and bizarre behavior
over 9 month period
Sellar Masses: “SATCHMO”

• Sellar tumor, Sarcoid
• Aneurysm, Arachnoid cyst
• Teratoma
• Hypothalamic glioma, Histiocytosis, Hamartoma of tuber cinereum
• Meningioma
• Optic glioma
Craniopharyngioma
• Arises from squamous epithelial remnants
• 50% <20 y/o; second peak: middle age
• Location
➢ 70%: both intrasellar and suprasellar
➢ 20%: intrasellar only
➢ 10%: purely suprasellar
Craniopharyngioma
• CT: “typical” = cystic with enhancing rim
➢ Partially calcified enhancing mural nodule
• MR: hyper or hypointense on T1WI; hyperintense on T2WI
➢ Appearance does not correlate well with chemical composition of contents
• Enhancement of rim: more common than in Rathke’s cleft cyst
History
• 30-year-old woman (12 weeks pregnant) with headaches, nausea, and
vomiting. Her obstetrician saw papilledema on fundoscopic exam and asked
for an imaging study.
Neuroradiology 1319 Neuroradiology Seminar 1

Hemangioblastoma
• Young, middle-aged adults
• 10-20% in von Hippel-Lindau (often multiple)
• Cerebellar hemispheres: most common
➢ Cervical spinal cord: #2 location
• Cystic with mural nodule: 60%
➢ Solid 40%
• Calcification very rare
• Nodule enhances intensely
History
• 62-year-old female with increasingly severe headaches
Pineal Region Masses

• Germ Cell Tumors (60%)
• Pineal parenchymal tumors (14%)
➢ Pineocytoma
➢ Pineoblastoma
• Others
➢ Pineal cyst, arachnoid cyst, lipoma
➢ Vein of Galen AV fistula
➢ Glioma
➢ Meningioma
Tentorial Meningioma
• Most common primary non-glial intracranial neoplasm (16% of all brain
tumors)
• Females > males (2:1)
• Multiple 6-9%
• Rare in children unless neurofibromatosis
• Arise from meningothelial arachnoid villi and possibly dural fibroblasts or pial
cells
History
• 35 year-old African-American male with ataxia and headaches

• Germinoma
• Lymphoma
• Optic nerve glioma
• Metastasis
• Tuberculosis
• Sarcoidosis
Sarcoidosis
• Etiology: unknown
• Worldwide prevalence
• United States: more common in African-Americans and women
• Peak age: 20-40 years old
• Multi-organ disease
➢ CNS: 5% of cases
Sarcoidosis – Clinical
• Adenopathy
• Skin rash
• Ocular abnormalities
• Elevated angiotensin converting enzyme
• Diagnosis: biopsy of skin or nodes
Sarcoidosis – Imaging
• 4 forms
➢ Parenchymal mass
➢ Periventricular
➢ Leptomeningeal
➢ Mixed
• Enhances intensely
➢ Combination of parenchymal and leptomeningeal enhancement: clue to
diagnosis
Sarcoidosis – Imaging
• Hyperintense on T2-weighted images
• Parenchymal: may mimic glioma
• Dural: may mimic meningioma
• Hydrocephalus
• Lesions diminish with steroid therapy

Neuroradiology Seminar 2:
Discussion of Unknown Cases
History
• 11-year-old male with sudden onset of headache and vomiting at school,
followed 5 days later by onset of left hemiplegia and weakness
Ring-enhancing Masses
“MAGICAL DR”
• Metastasis
• Abscess
• Glioblastoma Multiforme
• Infarct (subacute)
• Contusion
• AIDS:
➢ Toxoplasmosis
➢ Lymphoma (usually immunocompromised)
• Demyelinating disease
• Resolving hematoma, Radiation necrosis
• Most common primary CNS neoplasm overall
➢ 12-15% of all primary tumors
❖ 50% of all astrocytomas
➢ Most common supratentorial neoplasm in adults
• Peak age: 45-70 years old; children: 10%
• Shorter clinical duration (usually less than 3 months)
• Heterogeneous hemispheric mass with abundant vasogenic edema
• Most develop from pre-existing astrocytomas
• Subcortical white matter: frontal-temporal predilection

• WHO Grade IV
➢ Mitotic activity
➢ Pleomorphism
➢ Hemorrhage: common
➢ Endothelial proliferation and/or necrosis
• Subarachnoid seeding: 2-5%
• Heterogeneous mass
➢ Necrosis and hemorrhage common
➢ Calcification: rare
• Enhancement: >90%
➢ Ring-enhancement: central necrosis
• “Butterfly” pattern: corpus callosum extension
History
• 70-year-old male with recurrent basal cell carcinoma and squamous cell
carcinoma. Had radiation therapy 18 months prior to this study.
Radiation Necrosis vs. Tumor

• Conventional MR: difficult
• Advanced imaging
➢ MR spectroscopy: lactate, acetate, and succinate peaks without choline
peak
➢ DWI: restricted water diffusion
➢ PET/SPECT: hypometabolic
History
• 42-year-old male with 2-day history of left upper extremity and shoulder
weakness.

Lymphoma
• Increasing prevalence in immunocompromised and immunocompetent
populations
• Dismal prognosis: some survivors at 4 years from time of diagnosis
• B-cell lymphoma: perivascular spaces
• Periventricular or leptomeningeal
➢ Primary: brain parenchyma
➢ Secondary: dura
Lymphoma
• Non-contrast CT: hyperdense mass
• Long TR images: hypointense
• Enhancement virtually always but may be heterogeneous
Lymphoma in AIDS
• Necrosis more common than in immunocompetent hosts
• CT: hypodense
• Ring enhancement
Lymphoma vs. Toxoplasmosis

• Anti-toxoplasma therapy for 3 weeks
➢ Smaller: toxo
➢ No change: probable lymphoma
• PET / SPECT-Thallium
➢ Lymphoma: hypermetabolic
History
• 41-year-old male.
• History withheld.
Contusion
• Rough inner table of skull
➢ Anterior cranial fossa (frontal)
➢ Middle cranial fossa (temporal)
• “Bowl of Jell-O” model
• Spectrum: contusion —-> hematoma
➢ Perivascular space —-> parenchyma
• Variable clinical disability

History
• 7-year-old female with optic neuritis
Multiple Sclerosis
• Etiology remains unknown
• Cooler climate predilection
• Children: very rare especially before puberty
• Optic neuritis
➢ Retrobulbar pain, central loss of vision, Marcus-Gunn pupil
➢ Strong affinity for females and MS
Multiple Sclerosis: MR
• T1WI: Hypointense
• T2WI: Hyperintense
➢ Frequently shows lesions that are clinically unsuspected
• Active plaques enhance
• Chronic plaques: no enhancement
• MR often shows more disease than predicted clinically

Pediatric Radiology
Childhood Urinary Tract Infection
Ellen Chung, LTC, MC
Urinary Tract Infection

• Most common disorder of the urinary tract in children
• Second most common infection in children
• More common in girls after first 3-6 mo – short urethra
• Diagnosis – bag vs. clean-catch vs. catheter
➢ Bag specimen only valuable if negative
UTI
• E. coli responsible for vast majority of cases
• High recurrence rate
• Infants and young children are less likely to have specific symptoms
• Lower versus upper tract infection – fever, systemic illness
Imaging Studies
• Ultrasound
➢ Infant and young child kidneys versus adult
❖ Growth
❖ Fetal lobulation
❖ Increased cortical echogenicity <1 yo
❖ Hyopechoic pyramids
➢ Hydronephrosis vs. splaying of renal sinus fat
➢ Renal size
➢ Scarring, cortical thinning
➢ Anomalies
➢ Bladder – filling-defects, diverticula, wall thickening, PVR
➢ Lateral position of ureteral orifice
Ultrasound: Limitations
• US is NOT a screening exam
• Less sensitive than VCUG/RNC for diagnosis of VUR
• Less sensitive than CECT and DMSA for acute pyelonephritis
• Less sensitive than DMSA for renal scar
• Renal agenesis
Figure 6-1-1
• Renal ectopia
➢ Simple
➢ Crossed
• Renal fusion
➢ Horseshoe
➢ Lump or cake
Renal Agenesis [Figure 6-1-1]

• 1 in 1000 live births
• Ureter and ipsilateral hemitrigone are absent
• Medial positioning of colonic flexure
• Look in pelvis for ectopic kidney
• If observed kidney is large, it is the only functioning kidney
Medial positioning of the hepatic

flexure, which almost touches the
spine, indicating absence of right
kidney from right renal fossa.
Differential includes renal agenesis,
renal ectopia, or nephrectomy
Pediatric Radiology 1327

1329 Childhood Urinary Tract Infection
Renal Agenesis [Figures 6-1-2 and 6-1-3] Figure 6-1-2
• Ipsilateral adrenal is elongated and may
mimic kidney on US in newborn
• 25% have associated anomalies
➢ Mullerian duct duplication –
ipsilateral obstruction common
➢ Seminal vesicle cyst – ipsilateral
Renal Ectopia and Fusion

• Failure to separate into two blastemas or
In the absence of the kidney, the adrenal gland (arrow) is
to migrate from pelvis in utero elongated, and may be mistaken for a small kidney. The
• Most common ectopia is pelvic finding is bilateral in this patient, who has associated
• Most common type of fusion is pulmonary hypoplasia with bilateral pneumothorax. Bilateral
horseshoe renal agenesis is incompatible with life
• Anomalous kidney is often small,
dysmorphic and malrotated Figure 6-1-4
• Increased risk of trauma, stones, infection, renovascular
hypertension and possibly tumors
Horseshoe Kidney [Figure 6-1-4]

• 1 in 400-600 live births
• Higher incidence in Turner syndrome
• Low position with abnormal axis
• Usually malrotated
• Midline parenchymal isthmus well seen on US in young children
• Multiple renal arteries and veins
• Ureters cross isthmus
• Associated with UPJO
Figure 6-1-3
Two cervices (arrows) and two widely separated Ultrasound of the kidneys shows ill-
uterine horns (arrow heads) associated with right defined lower poles on the
renal agenesis longitudinal images and a midline
parenchymal connection anterior to
the aorta on transverse imaging.
The midline parenchymal isthmus is
Renal Ectopia [Figures 6-1-5 and 6-1-6] well seen in babies and toddlers with
• Most are small and dysmorphic horseshoe kidney. The kidneys are
• Collecting system is superficial and renal sinus echo complex is abnormally echogenic in this patient
absent or eccentric with renal dysfunction
• May be mistaken for a mass
• Blood supply from iliac arteries
Childhood Urinary Tract Infection 1328

1330 Pediatric Radiology
Imaging Studies Figure 6-1-5
• VCUG
➢ Gold standard exam for
reflux and urethral
abnormalities
➢ Versus
cystosonography
➢ Patient and parental
preparation
➢ Male voiding image
➢ Early-filling and oblique Empty left renal fossa with pelvic kidney behind the bladder and directly in
views front of the spine. Note the abnormally small size, somewhat dysmorphic
appearance, and the eccentric renal sinus echo complex
➢ Cyclic study in young
infants - more sensitive
Figure 6-1-6
Indications for VCUG
• Febrile UTI
• Abnormal ultrasound
• Patient < 6 yo and
➢ First UTI male or recurrent UTI female
➢ First degree relative with VUR (RNC)
➢ Solitary functioning kidney
• Any patient with neurogenic bladder
• Follow-up of patient with VUR (RNC)
• Post-op to confirm success
Imaging Studies
• Radionuclide cystogram
➢ Lower radiation dose
❖ Female gonads
➢ Continuous imaging
➢ Lack of spatial resolution
❖ Ureteral insertion
❖ Male urethra
❖ Grade I VUR Crossed fused renal ectopia on
excretory urogram. Note orthotopic
Imaging Studies insertions of left and right ureters
• Renal cortical scintigraphy
➢ DMSA or glucoheptonate
➢ More sensitive than ultrasound for renal scarring and pyelonephritis
➢ Indications
❖ Recurrent breakthrough infections
❖ Suspected acute pyelonephritis but equivocal laboratory or imaging
findings
Imaging Studies
• CT
➢ Disadvantage of ionizing radiation and need for intravenous contrast
compared with US
➢ More sensitive than US for acute pyelonephritis
Imaging Studies
• MR
➢ Gd-enhanced MR may be more sensitive than renal scintigraphy in acute
pyelo
➢ Sensitive for renal scarring
➢ Less available
➢ Costly
➢ Need for sedation

Imaging Studies Figure 6-1-7
• Diuretic Renography
➢ Obstructive versus nonobstructive
dilatation when VCUG shows no
reflux
➢ Differential function
VUR
Vesicoureteric Reflux
• Primary abnormality related to length
and angle of submucosal course of
distal ureter
• Versus secondary – bladder outlet Internationally standardized VCUG reflux grading system.
obstruction, neurogenic bladder, Illustration by Heike Blum, MFA
bladder diverticulum
• Up to 50% of children with UTI
Figure 6-1-8
Vesicoureteric Reflux
• Normal US in 75%
• Hydronephrosis – especially if changing
• Mild pelviectasis does not predict VUR
• Renal scar, cortical thinning, or lack of growth
• Urothelial thickening
VCUG Reflux Grading System [Figure 6-1-7]
What to Look for in Addition to VUR

• Ureteral insertion
• Bladder filling defect
• Axis of collecting system
• Coexisting obstruction
VCUG showing ureter inserting into a Hutch
• Intrarenal reflux diverticulum. This finding is an indication for
surgery
Abnormal Ureteral Insertion
• Into or near diverticulum – secondary rather than primary VUR
• Ectopic insertion – too close to bladder neck
Bladder Diverticula [Figure 6-1-8]

• Usually secondary to urethral obstruction and neurogenic bladder Figure 6-1-9
• Congenital diverticula also occur – usually solitary
• Herniation of urothelium through defect in muscular
wall of bladder
• Hutch diverticulum – at UVJ, associated with VUR
Ectopic Ureter [Figure 6-1-9]

• Lower than normal
• 3-4 x more frequent in females – usually upper pole
of duplex system
• Girls may present with lifelong, day and night
incontinence Left image from a VCUG showing an ectopic right
• In males, the ectopic ureter inserts above the ureter inserting into the urethra below the external
external sphincter sphincter in this girl with lifelong day and night
• The associated kidney may be dysplastic or atrophic, incontinence. Right image shows the ectopic
especially upper pole of a duplex ureter is an upper pole ureter of a duplex system,
which is usually the case in girls with ectopic
ureter; however, this is a very unusual image,
because ectopic upper pole ureters do not reflux.
This image was obtained because the catheter
preferentially selected the upper pole ureter rather
than the bladder when it was placed in the urethra

Ureterocele [Figure 6-1-10] Figure 6-1-10
• Dilation of the distal ureter, usually caused by
stenosis of the UVJ, with invagination into the bladder
• Ectopic vs simple
• In females, usually ectopic
• Can be so large as to cause obstruction of bladder
outlet or contralateral UVJ
• Cobra-head or spring onion appearance
• With bladder filling, can evert into distal ureter and
mimic a diverticulum
Abnormal Axis of Intrarenal Collecting

System [Figure 6-1-11]
• Normally a line drawn through the upper- and lower-
most calcyces points to the opposite shoulder.
• Causes of abnormal axis
➢ Duplicated collecting system
Everting ureterocele. On upper left early filling
➢ Malrotation with or without ectopia/fusion view, the ureterocele is easily identified as a
anomaly round filling defect. It becomes progressively
➢ Mass, especially neuroblastoma smaller the increased bladder filling until, in the
lower right image, it everts into the distal ureter,
Ureteropelvic duplication [Figures 6-1-12 and 6-1-13] mimicking a Hutch diverticulum
• Common 1 in 160
• Spectrum from bifid pelvis to complete duplication
• 50% bilateral
• Complete duplication associated with increased Figure 6-1-11
incidence of UTI, VUR, scarring and obstruction
• Upper pole ureter is ectopic and may be obstructed
• Lower pole moiety may have VUR or UPJ obstruction
Figure 6-1-12
The kidney on the left demonstrates a normal

axis, directed toward the opposite shoulder. The
kidney on the right shows the axis directed toward
the ipsilateral shoulder. This is the drooping lily
sign of a duplex kidney
Figure 6-1-13
There is grade II VUR on the right

and a straight renal axis due to
duplication. There is grade V VUR on
the left and a drooping lily sign. Note
the filling defect in the left side of the
bladder. This is due to the urine-filled
ureterocele of the obstructed upper
pole. The course of the lower pole
ureter is unusual because it is
intertwined with the dilated upper pole
ureter. The upper pole ureter is not
seen on the VCUG, because it is
obstructed and does not reflux The dilated upper pole ureter and its ureterocele
are seen on ultrasound

Coexisting Obstruction [Figure 6-1-14] Figure 6-1-14
• Not associated with each other, but both common
• The presence of contrast in upper tract due to reflux
allows one to diagnose the obstruction
• 3 signs
➢ Hesitation
➢ Dilution
➢ Delayed drainage
Intrarenal Reflux
• Important factor in the pathogenesis of renal scarring
• More commonly occurs at the poles
• Indication for surgery
Vesicoureteric Reflux [Figures 6-1-15 to 6-1-17]

• Relationship between VUR and renal scarring -
controversy
➢ Can reflux of sterile urine cause scarring?
➢ Does asymptomatic bacteriuria require tx?
• 3 Conditions are necessary for renal scarring to
occur
➢ UTI
➢ VUR
➢ Intrarenal reflux
Figure 6-1-15 VCUG showing the 3 signs of VUR with coexisting

obstruction. First, the obstruction works in both
directions, so the contrast hesitates to go past the
obstruction. Second, behind the obstruction is a
lot of trapped, unopacified urine. When the
contrast gets past the obstruction, it is diluted by
the unopacified urine. Third, there is delay in the
drainage of the contrast that got past the
obstruction
Figure 6-1-16
Illustration and gross specimen of the simple

papilla. In the simple, unfused papilla with its
pyramidal shape, the collecting ducts empty onto
the papillary surface at an oblique angle. As the
surrounding calyx becomes distended with urine,
these slit-like openings tend to close off
Figure 6-1-17 In the compound papilla, there is distortion of the

surface, which is greatest where there is the most
fusion. As shown in this diagram and gross
specimen, at these flatter, distorted surfaces, the
openings of the collecting ducts are more rounded.
When the calyx gets full, these cannot close, and
urine in the calyx can flow retrograde, which is
intrarenal reflux
The blush of renal parenchyma seen in the VCUG

of this patient with VUR shows the typical brush
or fan like configuration of the papillary ducts and
collecting tubules. This is macroscopic intrarenal
reflux

Acute Pyeloneprhitis Figure 6-1-18
• May be hematogenous, but usually an ascending
infection
• Barriers to infection
➢ Perineal resistance – which is overcome by
❖ Vaginal reflux, labial adhesions
❖ Uncircumcised male
➢ Frequent voiding
➢ Functioning UVJ
➢ Papillary/calyceal structure – resistance to
intrarenal reflux
Acute Pyeloneprhitis - Clinical

• Infants usually present with fever, but may present
with nonspecific symptoms
• Older children present with fever, flank pain but may
call it abdominal pain Pathologic appearance of acute pyelonephritis.
• If straight forward clinical picture, no imaging needed The gross image on the left shows that the surface
in acute setting of the involved parenchyma is pale compared to
the surrounding normal parenchyma. Note the
sharp demarcation between normal and abnormal.
Acute Pyelonephritis - Pathology [Figure 6-1-18] There is a patchy or lobar distribution. On the cut
• Gross Path – full thickness, loss of C-M specimen on the right, the medial upper pole is
differentiation, enlargement, sharp demarcation, normal. In the lateral upper pole and mid portion,
urothelial thickening the infection involves a full-thickness wedge from
the papilla to the surface of the kidney. The tissue
• Histo – tubulointerstitial nephritis is expanded. There is disruption of the
corticomedullary differentiation and a striated
Acute Pyelonephritis - Imaging [Figure 6-1-19]] appearance
• If fails to respond to therapy, US vs. CT to evaluate
for complication
• CT/US/Nuc - triangular, peripheral focus of decreased flow, decreased cortico-
medullary differentiation
• Diffuse or focal enlargement – can appear mass-like
• VCUG indicated – may perform while hospitalized
Figure 6-1-19
US demonstrating focal enlargement with decrease flow mimicking a mass. The more
sensitive enhanced CT shows more diffuse triangular and striated areas of decreased
enhancement typical of acute pyelonephritis
Complicated Upper Tract Infection

• Renal or perinephric abscess
• Pyonephrosis – must be treated urgently
Reflux Nephropathy
• Renal scarring – chronic pyelonephritis – post-infectious nephropathy
• Related to bacterial infection, VUR, and intrarenal reflux
• Usually at poles – especially upper
• Scarring can be prevented or limited if early diagnosis of upper tract infection

Reflux Nephropathy [Figure 6-1-20] Figure 6-1-20
• Destruction centered in medulla
• Full thickness - indentation overlying dilated calyx (versus fetal
lobulation)
• Echogenic focal scar
• Small kidney, no growth
• Compensatory hypertrophy between scars – can appear mass-
like
• Almost all cases of severe scarring have VUR
Vesicoureteric Reflux - Treatment

• Medical with annual follow-up US, RNC and urine culture
• Surgical reimplantation
• Endoscopic subureteric injection
• Surgery is considered for
➢ Failure to resolve or worsening
➢ Scarring or growth failure
➢ High grade VUR
➢ Intrarenal reflux
➢ Frequent breakthrough infections
Secondary VUR Upper pole cortical thinning due to

reflux nephropathy. Note that the
upper pole calyx extends almost to
Neurogenic Bladder the interface between the kidney and
• Failure of detrusor muscle and internal and external sphincters to the liver
function in concert to hold and release urine
• Emptying or filling phase dysfunction
• Causes – spinal dysraphysm, caudal regression, paraplegia, presacral
teratoma, anterior sacral meningocele
• Suspect in patients with recurrent UTI and constipation
• Lower motor neuron lesion – large, smooth-walled bladder
• Upper motor neuron lesion – small, trabeculated bladder
Neurogenic Bladder - Imaging

• Trabeculated, thick-walled or large and atonic
• Taller than wide
• Funnel-shaped bladder neck
• Large post-void residual
• Look for spinal dysraphism
Bladder Augmentation
• Used to treat small, noncompliant bladders
• If small bowel is used, gut signature and peristalsis are seen on US
• Complication of bladder rupture
• Alternative - autologous bladder cells grown in tissue culture
Antenatal Pelvicaliectasis
• Prevalence of prenatal sonography has changed the natural history of some
causes of neonatal hydronephrosis
• Common causes
➢ VUR
➢ UPJO
➢ Obstructed upper pole of duplex system
➢ PUV
Antenatal Pelvicaliectasis - Work-Up

• Perform postnatal ultrasound after DOL 4 or 5
• VCUG if mod-severe hydro
• In male with bilateral severe hydro, perform VCUG before discharge
• Repeat at 6 weeks if normal or mild
• If VCUG negative, perform diuretic renography or excretory urogram

Posterior Urethral Valves Figure 6-1-21
• Most common cause of urethral obstruction in the male infant
• Usually present in infancy (first UTI male) or prenatally diagnosed
• Less commonly delayed presentation with failure to thrive,
incontinence, or hypertension
Posterior Urethral Valves - Imaging [Figures 6-1-21 and 6-1-22]

• 3 types
➢ Type 1 - folds attach below the veru montanum
➢ Type 2 – folds attach above the veru
➢ Type 3 – diaphragm with central opening
• Trabeculated or thick-walled bladder
• Dilated posterior urethra – perineal window US
• Bilateral hydronephrosis – not a constant finding but bilateral
hydro in a male infant is PUV until proven otherwise
Posterior Urethral Valves [Figure 6-1-23]

• In utero obstruction causes renal dysplasia (dysgenesis)
• Prognosis related to degree of renal dysplasia
• Factors that protect one or both kidneys Trabeculated bladder in 1 month-old
➢ Large bladder male with bilateral prenatal
➢ Bladder or calyceal diverticula hydronephrosis. Note the irregularity
➢ Unilateral VUR or no VUR (50%) of the wall despite the bladder being
➢ Urinary ascites full
• No VUR ◗ “valve bladder”
Same patient showing posterior

urethral valve and dilated posterior Ultrasound of a patient with PUV showing
urethra irregular, thickened bladder wall, patulous UVJ,
and hydronephrosis. Note also the cysts and poor
corticomedullary differentiation due to associated
Prune Belly Syndrome renal dysplasia
• AKA Eagle-Barrett or Triad syndrome
• Triad
➢ Hypoplastic or absent abdominal wall musculature
➢ Cryptorchidism
➢ Urinary tract anomalies
• Almost exclusively males
Prune Belly Syndrome – 2 Types

• Severe –
➢ Complete urethral obstruction (bladder like PUV)
➢ Renal dysplasia and pulmonary hypoplasia
➢ Associated anomalies of GI tract, genital tract, CHD
➢ Death in first year of life

• Mild-Moderate Figure 6-1-24
➢ Functional abnormality of bladder emptying –
floppy, dilated bladder
➢ Urachal remnant
➢ Mild to markedly dilated renal pelvis and ureters
➢ Ureters mimic small intestine
➢ +/- pulmonary hypoplasia
➢ Long-term survival
Prune Belly Syndrome [Figures 6-1-24 and 6-1-25]

• Dilated ureter, bladder and urethra with patchy foci of Young infant with Eagle-Barrett
deficient musculature Syndrome. Note dilated flaccid
• Other findings – dilated posterior urethra, bladder, reflux into tortuous ureters
megalourethra, urethral diverticula, dilated prostatic with the appearance of small bowel
utricle loops, urachal remnant at dome of
bladder and dilated posterior urethra
without valve
Other Causes of Hydronephrosis
Ureteropelvic Junction Obstruction

• Most common cause of upper tract obstruction
Figure 6-1-25
• Previously presented with flank pain, mass, UTI or hematuria with
mild trauma
• Now commonly prenatally diagnosed
• Dilated pelvis and calices – no dilated ureter
• Ddx: extrarenal pelvis
UPJO
• Associations
➢ Increased risk of abnormality of contralateral kidney – most
common is UPJO
➢ Renal dysplasia
➢ VUR
➢ UVJ obstruction
➢ Lower pole moiety of duplex kidney
➢ Horseshoe kidney
Radiograph of same patient showing
UPJO - Treatment flaccid abdominal wall musculature.
• Mild – mod obstruction is followed and treated if it worsens Also the infant is intubated with small
lungs, bell-shaped thorax and medial
• Severe obstruction in young children is treated with right pneumothorax due to associated
dysmembered pyeloplasty pulmonary hypoplasia
• In adults and adolescents alternative treatment is endopyelotomy
• Often remain dilated after repair
UPJO [Figures 6-1-26 and 6-1-27] Figure 6-1-26

• Intrinsic versus extrinsic
➢ Intrinsic – fibrosis, stricture, valve/fold, etc
➢ Extrinsic – crossing vessel
❖ Intermittent symptoms and findings
❖ Dietl’s crisis
➢ Prenatally diagnosed – 10-15% extrinsic
➢ Diagnosed due to symptoms - 50% extrinsic
CT of patient with UPJO obtained when the patient
presented with severe abdominal pain and
peritoneal signs following a MVC. Note the dilated
pelvis and calyces, and the urine in the
retroperitoneum due to UPJ rupture. Note also the
large crossing vein that was found to be the cause
of the UPJO at surgery. Prior to this accident the
patient previously complained of chronic
abdominal pain and was treated for lactose
intolerance and had an appendectomy for a
normal appendix

Primary Megaureter [Figure 6-1-28] Figure 6-1-27
• Functional obstruction analogous to Hirschprung
disease of the GI tract
• Short aperistaltic segment of distal ureter
• Proximal ureter dilates – aperistaltic segment fixed
and narrowed
• More common in boys and on the left
• Predisposed to UTI and stone formation
• Surgically repaired only if severe or symptomatic
Congenital Megacalyces
• Nonobstructive enlargement of calyces and
hypoplasia of the medullary pyramids
• Benign nonprogressive condition
• Can coexist with megaureter
• Occasional stone formation, hematuria
Complications of endopyelotomy performed in a
Congenital Megacystis-Megaureter patient with UPJO due to crossing vessel. The
• Large bladder without obstruction upper left image shows the origin of the left main
• Large ureteral orifices with free reflux and voiding into renal artery. The lower right image shows the
markedly dilated ureters origin of an accessory renal artery to the lower
pole, which on the lower left image is seen to
cross the stent in the ureter (arrow). This artery
Summary was cut in the procedure causing the perirenal
• VUR may be primary or secondary to bladder outlet hematoma seen well in the lower two images.
obstruction/ neurogenic bladder or abnormal ureteral Note also the infarction of the anterior lower pole
insertion in the upper right image, due to spasm of the cut
artery
• Primary VUR is familial and resolves by age 6, but
secondary VUR requires surgical intervention
• In primary VUR the VCUG appears normal except for reflux Figure 6-1-28
• US is insensitive and is not a screening exam
• On US, look for scarring and lack of
growth
• RNC best for sibling screen, girls, and
follow-up
• VCUG best for symptomatic patients
and boys
• Surgical options are surgical
reimplantation or subureteric injection
• Bilateral hydro in an infant male is due
to PUV until proven otherwise
• Not all boys with valves have reflux and
hydronephrosis
• In utero obstruction causes renal
dysplasia
• UPJO is associated with abnormality of
Primary megaureter. Excretory urogram shows dilation of the
other kidney left ureter proximal to a short, fixed, relatively narrow segment
• UPJO diagnosed in utero or on of ureter near the UVJ
screening US is caused by intrinsic
abnormality in 85% of patients
• UPJO diagnosed due to symptoms is caused by extrinsic compression
(crossing vessel) in 50% of patients
• Extrinsic compression causes transient sx and findings
• Endopyelotomy is contraindicated in patients with crossing vessels

References
Texts
1. Kirks DR, ed. Practical Pediatric Imaging, 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 1998.
2. Siegel MJ, ed. Pediatric Sonography, 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 2002.
3. Swischuk LE. Imaging of the Newborn, Infant, and Young Child, 5th ed. Philadelphia: Lippincott-Williams & Wilkins,
2004.
Journal Articles
1. American Academy of Pediatrics Committee on Quality Improvement Subcomittee on Urinary Tract Infection. Practice
parameter: the diagnosis, treatment and evaluation of the initial urinary tract infection in febrile infant and young
children. Pediatrics 1999;103:842-852.
2. Berrocal T, Gaya F, Arjonilla A. Vesicoureteral reflux: diagnosis and grading with echo-enhanced cystosonography
versus voiding cystourethrography. Radiology 2001;221:359-365.
3. Blane CE, DiPietro MA, Strouse PJ, et al. Pediatric renal pelvic fullness: an ultrasonographic dilemma. J Urol
2003;170:201-203.
4. Blane CE, DiPietro MA, Zerin JM, et al. Renal sonography is not a reliable screening examination for vesicoureteral
reflux. J Urol 1993;150:752-755.
5. Brown T, Mandell J, Lebowitz RL. Neonatal hydronephrosis in the era of sonography. AJR Am J Roentgenol
1987;148:959-963
6. Daneman A, Alton DJ. Radiographic manifestations of renal anomalies. Radiol Clin North Am 1991;29:351-363.
7. Davey MS, Zerin JM, Reilly C, et al. Mild renal pelvic dilation is not predictive of vesicoureteral reflux in children.
Pediatr Radiol 1997;27:908-911.
8. Donnelly LF, Gylys-Morin VM, Wacksman J. Unilateral vesicoureteral reflux: association with protected renal function
in patients with posterior urethral valves. AJR Am J Roentgenol 1997;168:823-836.
9. Eggli KD, Eggli D. Color Doppler sonography in pyelonephritis. Pediatr Radiol 1992;22:422-425.
10. Elder JS, Peters CA, Arant BS Jr, et al. Pediatric vesicoureteral reflux guidelines panel summary report of primary
vesicoureteral reflux in children. J Urol 1997;157:1846-1851
11. Fernbach SK, Feinstein KA, Schmidt MB. Pediatric voiding cystourethrography: a pictoral guide. RadioGraphics
2000;20:155-168.
12. Gross GW, Lebowitz RL. Infection does not cause reflux. AJR Am J Roentgenol 1981;137:929.
13. Hoffer FA, Lebowitz RL. Intermittent hydronephrosis: a unique feature of ureteropelvic junction obstruction caused
by a crossing renal vessel. Radiology 1985;156:655-658.
14. Lavocat MP, Granjon D, Allard D, et al. Imaging of pyelonephritis. Pediatr Radiol 1997;27:159-165.
15. Lebowitz RL, Blickman JG. The coexistence of ureteropelvic junction obstruction and reflux AJR Am J Roentgenol
1983;140:231-238.
16. Lebowitz RL, Olbing H, Parkkulainen KV, et al. International system of radiographic grading of vesicoureteral reflux.
International Reflux Study in Children. Pediatr Radiol 1985;15:105-109.
17. Lonergan GJ, Pennington DJ, Morrison JC, et al. Childhood pyelonephritis: comparison of Gadolinium- enhanced
MR imaging and renal cortical scintigraphy for diagnosis. Radiology 1998; 207:377-384.
18. Mentzel JJ, Vogt S, Patzer L, et al. Contrast enhanced sonography of vesicoureterorenal reflux in children: preliminary
results. AJR Am J Roentgenol 1999;173:737-740.
19. Orellana P, Baquedano P, Rangarajan V. Relationship between acute pyelonephritis, renal scarring and vesicoureteral
reflux. Results of a coordinated research project. Pediatr Nephrol 2004;19:1122-1126.
20. Paltiel HJ, Mulkern RV, Perez-Atayde A. Effect of chronic low-pressure sterile vesicoureteric reflux on renal growth
and function in a porcine model: a radiologic and pathologic study. Radiology 2000;217:507-515.
21. Paltiel HJ, Rupich RC, Kiruluta HG. Enhanced detection of vesicoureteral reflux in infants and children with use of
cyclic voiding cystourethrography. Radiology 1992;184:753-755.
22. Rooks VJ, Lebowitz RL. Extrinsic ureteropelvic junction obstruction from a crossing renal vessel: demography and
imaging. Pediatr Radiol 2001;31:120-124.
23. Sargent MA. What is the normal prevalence of VUR? Pediatr Radiol 2000; 30:87-593.
24. Van den Abbeele AD, Treves ST, Lebowitz RL, et al. Vesicoureteral reflux in asymptomatic siblings of patients with
known reflux: radionuclide cystography. Pediatrics 1997;79:147-153.
25. Walsh G, Dubbins PA. Antenatal renal pelvis dilatation: a predictor of vesicoureteral reflux? AJR Am J Roentgenol
1996;167:887-890.

Neonatal GI Tract Obstruction
Figure 6-2-1
Objectives
• To become familiar with the diagnosis of the major
causes of proximal and distal GI obstruction in the
neonatal period (first 24 to 48 hours)
• To develop an approach to the evaluation of the
obstructed neonate including a method of performing
diagnostic and therapeutic enema
Neonatal Intestinal Obstruction - General

Principles
• Any obstruction distal to the ampulla of Vater causes
biliary emesis
• Perforation may be present without free air
• Loop immediately proximal to atresia often
disproportionately dilated
• Start evaluation with plain radiographs
• Small and large bowel cannot be differentiated on
plain film
Neonatal Intestinal Obstruction

• High
➢ Proximal to mid ileum
➢ Few dilated loops
➢ UGI or no further imaging
• Low
➢ Distal ileum, colon
➢ Many dilated loops
➢ Contrast enema 5 types of esophageal atresia/distal fistula
ESOPHAGUS Figure 6-2-2

Esophageal Atresia
• Error in differentiation of the foregut into trachea and esophagus
• Spectrum from esophagotrachea to H-type fistula without
esophageal atresia
• Presentation – feeding intolerance, regurgitation, choking,
aspiration, increased oral secretions, symptoms in first 24h of life
in 85 - 95%
• Half have other anomalies - VACTERL
Esophageal Atresia [Figures 6-2-1 and 6-2-2]

• Junction of upper and middle 1/3’s
• Fistula 0.5 to 1.0 cm above carina in 89%
• 5 types
• Length of the gap - longest without fistula
• Atresia with distal fistula is most common
• H-type – present later due to chronic aspiration, actually N-
shaped
Most common type is esophageal

atresia with distal fistula

1341 Neonatal GI Tract Obstruction
Esophageal Atresia - Imaging [Figures 6-2-3 and 6-2-4] Figure 6-2-3
• Prenatal
➢ Polyhydramnios
➢ +/- no stomach bubble
➢ +/- dilated fluid-filled proximal pouch
• Neonatal
➢ Anterior tracheal displacement on lateral CXR
➢ Distal bowel gas (85%)
➢ Gasless (8%)
➢ Dilated air-filled pouch
➢ OG tube coiled in pouch
➢ UGI usually not indicated – use air
Esophageal Atresia - Imaging

• Cross-sectional imaging: 3D CT, virtual EA/distal TEF. AP radiograph shows dilated, air-
filled proximal pouch. Lateral view shows NG
bronchoscopy coiling in pouch and anterior displacement of the
• Document side of arch for surgical planning trachea
• Ddx: pharyngeal perforation – pneumomediastinum,
pleural effusion
Esophageal Atresia – Post-op

• Complications
➢ Anastomotic leak
➢ Anastomotic stricture
➢ Recurrent fistula
➢ Reflux esophagitis/stricture
• Expected findings
Figure 6-2-4
➢ Disordered motility below anastomosis
➢ GER
➢ Tracheomalacia
VACTERL
• Non-random association of anomalies
• No one patient has all
• High perinatal mortality > 60%
• 46% of patients with TEF
• Recurrence risk (offspring) 2-3%
• Recurrence risk (siblings)
➢ TEF or EA < 1%
➢ Other VACTERL lesions 1.2%
Ddx: Pharyngeal Perforation

• Traumatic delivery
• Traumatic intubation
• Nasogastric tube placement
STOMACH
Gastric Atresia/Antral Web

• Very rare
• Atresia presents near birth
• Web is usually perforated so present in childhood with recurrent
nonbilious emesis Esophagram showing H-type fistula
with filling of tracheobronchial tree
Microgastria
• Rare
• Isolated vs. associated anomalies, especially asplenia
• Small tubular midline stomach and dilated distal esophagus
• Duodenal bulb may also dilate
Neonatal GI Tract Obstruction 1340

DUODENUM Figure 6-2-5
Normal Rotation of Midgut Loop [Figure 6-2-5]

• GI tract straight short tube
• Midgut divided by SMA
• 270 degrees counterclockwise rotation 6th week
• Normal mesentery is broad based
Malrotation/Malfixation - Pathogenesis
• All types referred to as malrotation
• Malfixation - short root of mesentery predisposes to midgut volvulus
• Ladd bands –
➢ Attempts to secure the bowel
➢ Most common site is from high medial cecum across 2nd-3rd portion of
duodenum to porta hepatis
➢ May cause obstruction Normal intestinal rotation
and fixation showing
long root of the
Figure 6-2-6 mesentery (line)
Figure 6-2-7
Illustrations showing malrotation and midgut volvulus (center)
Midgut Volvulus - Presentation

• Prenatal Most common location of
Ladd band
➢ Necrosis of bowel and multiple atresias
• First month of life – most patients
➢ Bilious emesis, occasional bloody stool Figure 6-2-8
• Older child
➢ Chronic recurrent abdominal pain, failure to thrive, diarrhea,
malabsorption
➢ Volvulus can occur at any age
Midgut Volvulus - Pathology [Figure 6-2-8]

• Volvulus impedes venous and lymphatic return leading to bowel
wall edema
• If prolonged, arterial obstruction and bowel infarction
Malrotation – Plain Radiograph [Figure 6-2-9]

• Evaluation of emesis/obstruction begins with plain film
• Classic - partial obstruction of duodenum 2nd -3rd portion -->
malro until proved otherwise
• May mimic gastric outlet obstruction
• Ileus or distal SBO
• Gasless abdomen
• Normal
Chronic midgut volvulus

Malrotation – UGI Figure 6-2-9
• Duodenal jejunal junction – diagnostic
• Normal position on AP is on or to the left of the left pedicle of L1
• Normal position on lateral is posterior/retroperitoneal
• AP must be perfectly positioned
Malrotation – UGI [Figures 6-2-10 and 6-2-11]

• Jejunum in RUQ
• DJJ may be displaced by distended bowel, masses and enlarged
organs
• If DJJ is equivocal, empty stomach with NG tube or complete
small bowel follow-through
Figure 6-2-10
Plain radiograph of a newborn with

bilious emesis. High small bowel
obstruction is noted, with all air-filled
loops on the right
Normal position of the DJJ on frontal view (left
image) on or to the left of the left pedicle of L1.
Lateral view from an UGI (right image) showing
the normal retroperitoneal location of the Figure 6-2-11
duodenum
Midgut Volvulus – UGI [Figure 6-2-12]

• Duodenal obstruction
• Beak
• Corkscrew appearance of duodenum and jejunum
Malrotation – Contrast Enema

• No longer part of work-up
• Cecum and DJJ rotate independently
• Cecum normal in 20% of patients with malrotation so enema is no
longer used in the work-up
• High mobile cecum is a common normal variant – 15%
• Entire colon may be to left of midline in malrotation
• More often cecum is high and medial
Malrotation. Duodenal jejunal junction

Figure 6-2-12 low and to the right of midline
Lateral view showing obstruction of

the 2nd – 3rd portion of the
duodenum and corkscrew
appearance of the jejunum diagnostic
of malrotation

Malrotation – Ultrasound/CT [Figures 6-2-13 and 6-2-14] Figure 6-2-13
• Dilated proximal bowel, wall thickening, ascites
• Inversion of SMA/SMV relationship1
• Normally the SMV is to the right of the SMA
• 33% surgically proven malrotation have normal relationship
• 8 of 9 with SMA/SMV inversion had malrotation
• Below the portal confluence, look for the swirl sign
1Zerin JM, DiPietro MA. Superior mesenteric vascular anatomy as
ultrasound in patients with surgically proved malrotation of the midgut.
Radiology 1992;
183:693-4 Ultrasound images showing inversion
of the SMV/SMA relationship. The
Figure 6-2-14 SMV is usually larger and the SMA is
usually surrounded by echogenic fat,
but he identity of each vessel must
be confirmed with Doppler or by
connecting SMV to the portal vein
Midgut volvulus in 4 yo with acute

onset of emesis. Inversion of
SMA/SMV relationship (lower image)
and swirl sign (upper image) of
volvulus. SMV (arrow) is to the left of
the SMA
Malrotation - Treatment
• Ladd procedure
➢ Reduce midgut volvulus
➢ Lyse bands
➢ Place in orientation of nonrotation – all small bowel on the right and all
colon on the left
➢ Inversion appendectomy
➢ 95% have no recurrence
Malrotation – Associated Congenital Anomalies

• Omphalocele
• Gastroschisis
• Diaphragmatic hernia
• Bowel atresia/stenosis
• Heterotaxy – not surgically repaired
Duodenal Atresia/Stenosis/Web
• Atresia much more common than stenosis
• 3-6 weeks gestation – failure of canalization of solid tube of foregut
• Annular pancreas in 20%
• May have preduodenal portal vein

Duodenal Atresia - Clinical Figure 6-2-15
• 30% have Down’s syndrome
• Associated with other atresias, biliary anomalies, CHD,
VACTERL
• Almost always just distal to ampulla of Vater --> bilious emesis
Duodenal Atresia - Imaging [Figures 6-2-15 to 6-2-18]

• Polyhydramnios
• Double bubble
• Windsock deformity
• How do we know it is not midgut volvulus? Dilation of duodenal
bulb indicates it is a chronic condition
• Double bubble is diagnostic, but if obstruction is not complete,
an UGI is indicated
Figure 6-2-16
Double bubble is diagnostic of duodenal

atresia. vein
Figure 6-2-17
Prenatal ultrasound for

polyhydramnios shows fluid-filled
double bubble. This finding should
prompt chromosomal analysis for Annular pancreas. Upper GI shows
possible Trisomy 21 circumferential narrowing of second
Figure 6-2-18 portion of duodenum
Caution: Incomplete obstruction mandates an

UGI. Double bubble appearance with distal gas
caused by midgut volvulus with beak appearance
on upper GI

JEJUNUM Figure 6-2-19
Jejunal Atresia and Stenosis

• Atresia more common than stenosis
• Ischemic injury to developing gut
➢ Primary vascular accident – more common
➢ Secondary to mechanical obstruction, e.g. in utero volvulus
Jejunal Atresia - Presentation

• Bilious emesis first hours of life
• Distended epigastrium with scaphoid lower abdomen
• +/- failure to pass meconium Intraoperative photograph
demonstrating jejunal atresia
Atresia/Stenosis - Pathology [Figure 6-2-19]
• Most common sites are proximal jejunum and distal ileum
• 20% multiple
• 5 types
• Two types inherited
➢ Apple-peel (Christmas tree)
➢ Syndrome of multiple intestinal atresias and intraluminal calcification Figure 6-2-20
Jejunal Atresia - Imaging [Figure 6-2-20]

• Triple bubble
• Dilated loop of bowel proximal to atresia is disproportionately
dilated with bulbous end
• Proximal bowel may be fluid-filled and mimic a mass
• UGI is rarely indicated
• Surgeon may request BE to evaluate for additional distal atresia
Proximal Neonatal Intestinal Obstruction - Ddx

• Esophageal atresia
• Gastric atresia/web
• Malrotation/midgut volvulus
• Duodenal atresia
• Jejunal atresia
• All surgical – UGI only to determine who needs to go emergently
Low Intestinal Obstruction

• Plain film
➢ Multiple dilated loops of bowel Triple bubble sign of jejunal atresia.
Upper GI is not necessary
• Contrast enema
➢ Dilute ionic water-soluble contrast (cystography)
➢ Low osmolar nonionic contrast
• Microcolon
➢ Unused colon of small caliber, <1 cm
ILEUM Figure 6-2-21

Ileal Atresia [Figure 6-2-21]
• Primary vascular accident or secondary to
mechanical obstruction (in utero volvulus)
• Plain film - low obstruction
• Contrast enema –
➢ Microcolon
➢ Abrupt cut off of contrast column at atresia
➢ No filling of dilated ileum
Plain radiograph (left) shows a large number of

dilated tubular loops of bowel. Contrast enema
(right) reveals a microcolon

Meconium Peritonitis [Figures 6-2-22 and 6-2-23] Figure 6-2-23
• In utero bowel perforation with leakage of meconium
• Linear, clumps or at periphery of pseudocyst, or generalized
• Scrotal
• Perforation may have sealed
• Intraluminal – severe stasis, anorectal or cloacal
malformation
Figure 6-2-22
Newborn with scrotal swelling due to

calcifications in both scrotal sacs from
meconium peritonitis
Figure 6-2-24
Generalized meconium peritonitis.

Note calcifications around liver and
spleen. Perforation was due to ileal
atresia
Meconium Ileus [Figure 6-2-24]

• Inspissated abnormal meconium in the distal ileum
and colon
• Almost all have cystic fibrosis - presenting feature of
Meconium ileus. Illustration of pellets of
CF in 5-20% inspissated meconium causing distal small bowel
• 50% complicated - volvulus, perforation, atresia or obstruction. Intraoperative photograph shows
peritonitis thick, viscous meconium in dilated small bowel
with microcolon distal to the obstruction
Meconium Ileus – Plain Film [Figure 6-2-25]
• Classic
➢ Distal obstruction Figure 6-2-25
➢ Bubbly appearance in RLQ,
➢ Variation in caliber of loops
➢ Paucity of air-fluid levels
• Complicated
➢ Peritoneal calcifications
➢ Bowel wall edema
➢ +/- free air
Meconium Ileus - Contrast Enema [Figure 6-2-26]

• Microcolon (smallest)
• Distal 10 - 30 cm of ileum small in caliber but still larger than
colon
• Multiple round filling defects
• Contrast eventually fills dilated ileum proximal to obstruction
Meconium ileus. Supine radiograph

shows many dilated, unfolded loops
of bowel and classic soap-bubble
lucencies in the right lower quadrant
(arrow)

Meconium Ileus - Treatment Figure 6-2-26
• 1968 Noblett – nonoperative therapy - Gastrograffin enema
• Treatment enema - half-strength Gastrograffin or full urographic
contrast
➢ 1-2 enemas per day - several attempts
➢ 50-80% success rate
➢ <2% perforation rate
• Hydrostatic reduction failure =>complicated meconium ileus =>
surgical intervention
Berdon Syndrome
• Megacystis-microcolon-intestinal hypoperistalsis syndrome
• Functional small bowel obstruction
• Malrotation common
• Transient microcolon and dilated SB loops
• Large unobstructed bladder, also dilated ureters and pelvicalyceal
system
• 4:1 F:M
• Associated GU and cardiac anomalies
• Poor prognosis for long term survival
Meconium ileus. Contrast enema
shows a microcolon and pellet-like
COLON/RECTUM filling defects in the distal small bowel
Colonic Atresia Figure 6-2-27

• Rare (stenosis even rarer)
• Diaphragm, web, fibrous cord, gap
• Intrauterine vascular accident
• Plain film - disproportionately dilated, bulbous loop of proximal
colon
• Contrast enema - microcolon distal to atresia
Functional Immaturity of the Colon

• 1956 Clatworthy - “meconium plug syndrome”
• 1974 Davis -“small left colon syndrome”
• 1975 Lequesne and Reilly – small left colon could occur with or
without meconium plug
• 1977 Berdon - "functional immaturity” – above are overlapping
entities in a spectrum of functional neonatal intestinal obstruction
Functional Immaturity of the Colon Functional immaturity of the colon.

Contrast enema showing abrupt
• Abnormal intestinal motility in the left colon transition to small caliber left colon at
• May have a meconium plug – effect rather than cause of the splenic flexure
obstruction
• Risk factors
➢ Infant of diabetic mother Figure 6-2-28
➢ Mother treated with magnesium sulfate
Functional Immaturity - Contrast Enema

[Figures 6-2-27 and 6-2-28]
• Narrow descending and rectosigmoid colon with abrupt transition
to distended colon at splenic flexure
• Passage of plug classic but not common
• Passage of lots of meconium - nonspecific
• Clinical improvement after enema in hours or days
Meconium plug

Differentiation from Hirschprung Disease
Functional Hirschprung
Immaturity Disease
Location of Always at splenic Uncommon at
transition zone flexure splenic flexure
Quality of transition Abrupt Gradual
zone
Caliber of left colon Small Normal
Distensibility of Distensible Non distensible

rectum
Figure 6-2-29
Hirschprung Disease
• Functional obstruction of colon due to absence of
intramural ganglion cells of myenteric plexus
• Failure of distal intestine to relax
• Etiology - ? arrest of craniocaudal migration of
neuroblasts in the distal colon in 12th week
• 1 in 5000 live births
Hirschsprung Disease - Presentation

• 80% present in 1st 6 weeks of life
• Term newborns
• Boys:Girls 3-4:1 for short segment
• Equal sex distribution for total colonic
• 1/3 develop NEC-like enterocolitis in first month of Hirschprung disease. Gross specimen in center
shows transition zone with narrow distal sigmoid
life and rectum. Histology specimen from aganglionic
• May present with neonatal appendicitis segment (left) shows hypertrophied neural
bundles. Histology specimen from proximal colon
Hirschprung - Location shows normal ganglion cells
• Ultrashort segment – we don’t see
• Short segment – rectosigmoid (73%) Figure 6-2-30
• Intermediate - long segment
➢ Descending colon (14%)
➢ Proximal colon (10%)
• Total colonic – familial (1-3%)
Hirschprung - Pathology [Figure 6-2-29]

• Superficial suction biopsy
➢ Absent ganglion cells
➢ Hypertrophied submucosal nerve bundles
Hirschsprung – Plain Films [Figure 6-2-30]

• Distal obstruction
• Paucity of rectal gas
• Prone cross table lateral may show transition zone
• Pneumatosis of bowel proximal to aganglionic segment possible
• 5% present with pneumoperitoneum – usually total colonic
Hirschsprung – Contrast Enema [Figure 6-2-31] Hirschprung disease. Plain

• No balloon catheters in the rectum radiograph shows distal bowel
• Lateral view best obstruction and paucity of gas in
• Only enough contrast to diagnose rectum
• Low rectosigmoid index (normally > 1)
• Irregular contractions of aganglionic segment
• Early evacuation film may help
• 24 hour delayed film – retention and lack of movement to the left, but not specific
• Initial enema may be normal in neonates

Total Colonic Aganglionosis [Figure 6-2-32] Figure 6-2-31
• Microcolon
• Normal caliber colon
• Dilated colon
• Small bowel transition zone
• Family history
Associated Anomalies
• 3%-5% Down syndrome
• Esophageal dysmotility syndromes
• Malrotation
• Ileal and colonic atresia Hirschprung disease. Lateral image
• Neurocristopathies from contrast enema showing
➢ Neuroblastoma abnormally low recto sigmoid ratio
➢ Ondine curse – central hypoventilation and congenital and saw tooth contractions in
neuroblastoma aganglionic segment
Imperforate Anus
• Anorectal malformation – abnormal separation of GU tract from hindgut Figure 6-2-32
• High vs. low - levator sling development
determines surgical approach and
prognosis
• Low – fistula to perineum
• High – fistula to
➢ Boys – post urethra, calcified
meconium, air in bladder
➢ Girls – vagina or vestibule
Imperforate Anus [Figure 6-2-33]

• Associated anomalies
➢ VACTERL
➢ GU
➢ L-S spine
➢ Dysraphism Twin boys with total aganglionosis, twin (A) manifesting a
➢ Tethered cord microcolon; twin (B) with normal colon caliber & small bowel
➢ Currarino triad – imperforate anus, transition zone (arrow)
sacral defect, presacral mass
• Post-op incontinence => MRI
Figure 6-2-33
Neonatal Low Intestinal Obstruction Ddx
• Ileal atresia
• Meconium ileus
• Colonic atresia
• Functional immaturity of the colon
• Hirschprung disease
• Imperforate anus
Neonatal Bowel Obstruction Always remember, and

please don’t ever forget . . .
• Start with plain film
• All complete high obstructions are surgical. Usually no further
imaging required.
• UGI for incomplete high obstruction or normal film
• Contrast enema for low obstruction
• Differentiates surgical from medical causes
• Therapeutic in medical cases
Sacral defect associated with history

of imperforate anus

References
1. Buonomo C. Neonatal Gastrointestinal Emergencies. Radiologic Clinics of North America 1997;35: 845-864
2. Cohen MD. Choosing Contrast Media for the Evaluation of the Gastrointestinal Tract of Neonates and Infants.
Radiology 1987;162:447-56
3. Kao SC, et al. Nonoperative treatment of simple meconium ileus: a survey of the Society for Pediatric Radiology.
Pediatr Radiol 1995;25: 97-100
4. Kirks DR, et al. Practical Pediatric Imaging. 1998
5. Kirks DR. Emergency Pediatric Radiology. American Roentgen Ray Society. 95th Annual Meeting April, 1995
6. Long FR, Kramer SS, Markowitz RI, Taylor GE. Radiographic patterns of intestinal malrotation in children.
RadioGraphics 1996;16:547-556.

Acute GI Disorders of Infants and Children
Necrotizing Enterocolitis – Pathogenesis

• Hypoxemia ◗ ischemic bowel ◗ bacterial invasion
• Feeding plays a role – hyperosmolar, early
• Risk factors
➢ Prematurity
➢ Term with CHD Figure 6-3-1
➢ Hirschprung disease
➢ UAC
NEC – Clinical Features [Figure 6-3-1]

• Onset 3-6 DOL
• Abdominal distension
• Vomiting
• Metabolic acidosis
• Temperature lability
• Hypotension
• Apnea/bradycardia
Necrotizing Enterocolitis – Pathology [Figure 6-3-2]

• Ileum and colon most common
• Coagulative and hemorrhagic necrosis
• Dilated, friable bowel
• Submucosal and subserosal gas bubbles
• Complications – perforation, sepsis, stricture Distended and discolored abdomen
in a neonate with severe NEC
NEC – Radiographic Findings [Figure 6-3-3]
• Normal intestinal gas pattern of a neonate – uniform polygonal lucencies
throughout the abdomen Figure 6-3-2
• NEC – nonspecific plain film findings
➢ Distended loops
➢ “Sentinel loop” sign
➢ Wall edema
➢ Ascites
Figure 6-3-3
Gross specimen of ileum showing

submucosal and subserosal
pneumatosis in NEC
Normal neonatal bowel gas pattern
Pediatric Radiology 1353 Acute GI Disorders of Infants and Children

NEC – Radiographic Findings [Figures 6-3-4 to 6-3-6] Figure 6-3-4
• NEC – Specific plain film findings
➢ Pneumatosis intestinalis
❖ Subserosal – curvilinear
❖ Submucosal – bubbly, looks like stool
➢ Portal venous gas
• Indication for surgical intervention
➢ Free air
Figure 6-3-5
NEC with diffuse pneumatosis and

pneumoperitoneum below the liver tip on left
lateral decubitus view (right image).
Figure 6-3-6
Branching portal venous air
NEC – Other Imaging [Figure 6-3-7]

• US
➢ Thickened bowel wall
➢ Mural gas
➢ Portal venous gas - mobile
• Contrast enema
➢ Contraindicated acutely
➢ Late strictures - 20%
Free air around the falciform ligament
STOMACH (arrow). The American football sign
Hypertrophic Pyloric Stenosis Figure 6-3-7

• Acquired hypertrophy of antropyloric circular muscle
• Etiology unknown
• Common – 1 in 500 live births in the US
• Males more often affected – 5:1
• First born
• Family history – 5%
HPS – Clinical Features

• Present at 2-9 weeks of age
• Rare after 3 mo
• Uncommon in preemies
• Progressive nonbilious projectile vomiting
• Dehydration
• Failure to thrive
HPS – Pathology [Figures 6-3-8 and 6-3-9]

• Neurons supplying the circular muscle layer lack nitric oxide
synthetase activity
Colonic stricture due to prior NEC
• Circular muscle layer undergoes hypertrophy and elongation
HPS – Imaging [Figures 6-3-10 and 6-3-11]

• Plain film
➢ +/- dilated stomach with little distal gas
➢ Gastric hyperperistalsis
• Ultrasound – diagnostic
Acute GI Disorders of Infants and Children 1352

➢ Long pyloric channel (>17 mm) with thick muscular wall (> 3 mm) Figure 6-3-8
➢ Measure sonolucent part of one wall
➢ May have fluid/debris filled stomach
➢ No passage of fluid through pylorus
Figure 6-3-9
Normal pylorus on left and hypertrophic pyloric

stenosis on right
Figure 6-3-11
Gross specimen of hypertrophic

pyloric stenosis Figure 6-3-10
Upper image shows hypertrophic

pyloric stenosis with thickened
sonolucent muscle and marked
AP radiograph shows markedly elongation compared to normal (lower
dilated stomach with deep image)
indentations due to hyperperistalsis
and little distal bowel gas
Figure 6-3-12
HPS – Imaging
• Ultrasound
➢ Borderline measurements ◗ follow-up in 1 or 2 days
➢ Pyloric US is only good for HPS - if US negative, look for
other causes of vomiting
❖ SMA/SMV inversion
❖ Hydronephrosis
➢ Pitfalls: overdistended stomach, coapted antrum
HPS – Imaging [Figure 6-3-12]

• UGI
➢ No longer used unless post-op with persistent symptoms
➢ Multiple signs – string, double string, beak, tit, shoulder and
mushroom
• Ddx: pylorospasm, antral gastritis
UGI in HPS showing

string sign (A), beak sign
(B), shoulder sign (C),
and tit sign (D)

1355 Acute GI Disorders of Infants and Children
HPS – Treatment Figure 6-3-13
• Hydration and electrolyte replacement
• Pyloromyotomy – U.S.
• Nonoperative treatment – U.K. and Scandinavia
Gastrostomy Tubes [Figure 6-3-13]

• Usually in neurologically impaired child
• Use water-soluble contrast, not too hyperosmolar
• Trouble shooting
➢ Tube not in track or not in stomach
➢ Obstruction due to balloon migration into gastric
outlet
➢ GJ tubes in babies may cause
How to do a G-tube check. Frontal view suggests
intussusception/curtain-rodding the tube is properly positioned, but lateral view
tangential to the tube track, shows the tube is in
Bezoar [Figure 6-3-14] the track and not in the stomach
• Lactobezoar – too concentrated formula
• Trichobezoar – young and emotionally disturbed children
Figure 6-3-14
• Can embolize
• Can be treated endoscopically unless embolic
SMALL BOWEL
Duodenal Hematoma
• Etiology – blunt abdominal trauma
➢ Handlebar
➢ Seat belt
➢ Inflicted trauma
• Associated injury to other organs, especially
pancreas Embolic trichobezoar mimicking malrotation as
• Usually present with vomiting, pain, less commonly emboli connected to gastric bezoar by hair that
mass, jaundice straightened the bowel
Duodenal Hematoma – Imaging

• US - mixed echogenicity mass which becomes more hypoechoic as it liquefies
• UGI
➢ Intramural mass causes curved impression on duodenum
➢ Widened, separated folds ◗ stack of coins
NAT-Visceral Injury
• Seen at all ages
• Usually blunt
• Delay in seeking treatment
• 20%-50% mortality
• Proximal SB hematoma, distal SB perforation
Differential of Intramural Hemorrhage

• Blunt abdominal trauma
• Coagulation disorder
➢ Hemophilia
➢ Blood dyscrasia with pancytopenia
➢ Henoch-Schonlein purpura
• Ischemia
Henoch-Schönlein Purpura
• Idiopathic anaphylactoid reaction with diffuse vasculitis
• In the small bowel it causes intramural hemorrhage
• Jejunum most frequently involved
• Enteroenteric intussusception common

HSP – Clinical Features Figure 6-3-15
• Characteristic purpuric rash of LE’s and buttocks
• Abdominal pain – may precede rash by days
• Arthritis
• Nephritis
• Vasculitis
• Acute scrotum
HSP - Pathology [Figure 6-3-16]

• Gross – focal mucosal hemorrhage, edema, and Ultrasound and CT of HSP showing markedly
erosions thickened wall and echogenic/enhancing
• Histo - acute leukoclastic vasculitis of small vessels thickened mucosa of the dilated small bowel
in the submucosa or deep lamina propria
• Ddx: SLE, enterohemorrhagic strains of E. Coli (0157:H7 which causes
hemolytic uremic syndrome)
HSP – Imaging Findings [Figures 6-3-15 and 6-3-16]

• Thickening of SB wall
Figure 6-3-16
• Focal areas of dilatation alternating with stenosis
• Separation of bowel loops
• Submucosal masses
• Enteroenteric intussusception
Inguinal Hernia
• Most common cause of intestinal obstruction in
young infants
• Usually a clinical diagnosis
• Incarceration or strangulation can cause bowel Gross images showing the dilated markedly
obstruction thickened small bowel and classic purpuric rash
• Most male - 90%
• Bowel, fat, fluid, ovaries can herniate
Inguinal Hernia - Imaging [Figure 6-3-17]

• Plain film – look for air in scrotum or thickened inguinal fold
• Ultrasound
➢ Bowel or fat in the inguinal canal or scrotum
➢ Color Doppler to evaluate for flow to incarcerated bowel Figure 6-3-17
Intussusception - Clinical [Figure 6-3-18]
• 90% ileocolic or ileoileocolic
• 90% due to lymphoid hyperplasia – seasonal (winter and spring)
• 6 mo to 3.5 years (peak 5-9 mo)
• Outside that age range consider lead points
• Colicky pain, vomiting, bloody stools, lethargy, palpable mass
RLQ
• 10% recurrence rate
Figure 6-3-18
Young infant with distal bowel

obstruction with air over the right
inguinal canal
Ileocolic (left) and ileoileocolic (right)

intussusception

Intussusception - Pathology Figure 6-3-19
• Invagination of one segment of bowel into another
• Edema, congestion, coagulative and hemorrhagic necrosis
• Prominent Peyer patches
Intussusception - Pathology
• 8-10% pathologic lead points
➢ Meckel diverticulum if younger
➢ Lymphoma if older
➢ Polyp
➢ Appendix
➢ Henoch-Schonlein purpura
Intussusception - Imaging [Figures 6-3-19 to 6-3-21]

• Plain film
➢ Left lateral decubitus films
➢ No air in cecum or filling defect
➢ Air crescent sign
➢ SBO possible – especially in infant Intussusception. Crescent of air
• US – graded compression surrounds intussusceptum in the
transverse colon
➢ Pseudokidney/donut/target sign
➢ Doppler flow to wall ◗ viable
➢ Ascites – nonspecific Figure 6-3-20
Intussusception Reduction
• Contraindications
➢ Free air/peritoneal signs
➢ Septic shock
➢ Hx >24 hours
• Preparation
➢ Surgical consult – capable surgeon present
➢ IV – antibiotics
➢ Someone to monitor patient US in 10 mo with intussusception showing donut
➢ 16-G Angiocath to treat tension (left) and pseudokidney signs. Echogenic material
pneumoperitoneum within the intussuscepiens is mesenteric fat
Intussusception Reduction
• Air up to 120 mm Hg1 or Figure 6-3-21
• Water soluble contrast
• 3 attempts, 3 minutes each
• Largest tip possible
• Squeeze buttocks when at IC valve
• If losing air, apply forward pressure to tip
• Endpoint is reduction of soft tissue mass AND rush
of air into the SB
1Shiels WE, Maves CK, Hedlund G, Kirks DR. Air enema
for diagnosis and reduction of intussusception: clinical CT of colonic intussusception due to polyp. Note
experience and pressure correlates. Radiology fat and vessels inside the intussuscepiens
1991;181:169-172
Intussusception Reduction [Figure 6-3-22]

• Air slightly more effective than water soluble contrast
• Perforations with air are smaller1
• Perforations usually occur near IC valve and are associated with necrotic
bowel
• “Perforation” is probably the uncovering of a perforation that was already there
• If concerned for recurrence, go straight to enema
1Shiels WE, Kirks DR, Keller GL, et al. Colonic perforation by air and liquid
enemas: comparison study in young pigs. AJR 1993;160:931-935.

Meckel Diverticulum Figure 6-3-22
• Omphalomesenteric/vitelline duct remnant
• Most common congenital anomaly of the GI tract
• 2% of population
• 45% present under age 2y
• Within 2 ft of IC valve
• 2% complications – intussusception, obstruction,
hemorrhage, diverticulitis, perforation
Meckel Diverticulum - Presentation

• Most commonly become symptomatic in first years of
life
• Bleeding due to ulceration caused by secretion of
acid by ectopic gastric mucosa
• Obstruction due to inverted Meckel diverticulum
serving as a lead point for intussusception
• Vitelline band can serve as a fulcrum for volvulus
Meckel Diverticulum - Pathology [Figure 6-3-23]

• 1-5 cm long
• Antimesenteric border Therapeutic air contrast enema in older child with
• Most discovered incidentally are lined by small bowel intussusception due to lymphoma. Watch as
mass moves from hepatic flexure to cecum. The
epithelium procedure is not successful until the mass is
• Those that present with symptoms are more likely to completely reduced and air rushes into the ileum
contain ectopic gastric mucosa - 15-25% as shown in the lower right image of another
• Can be giant patient
Meckel Diverticulum - Imaging [Figures 6-3-24 and 6-3-25] Figure 6-3-23

• Plain film –
➢ May be visible if giant – mottled air collection
➢ May be filled with air or entherolith
• Ultrasound
➢ Gut signature – resembles normal bowel
➢ When inflamed, mimics appendicitis
• Tc99mPertechnetate scan positive if contains gastric mucosa
Figure 6-3-24
Meckel diverticulum
Figure 6-3-25
Lethargic 4 mo with small bowel obstruction.

Ultrasound shows fluid-filled lead point due to
Meckel diverticulum
Meckel diverticulum on small bowel

follow-through

Longitudinal (left) and transverse images showing Intraoperative image showing ileocolic
intussusception with large fluid-filled lead point, a intussusception and sectioned resected specimen
duplication cyst showing duplication cyst lead point
Enteric Duplication Cyst
• Developmental tubular or cystic structures adjacent to the GI tract
• Share wall and blood supply with adjacent bowel
• Usually round and do not communicate with bowel lumen
• Occasionally tubular, communicating with GI tract at one end and blind-ending at the other
Duplication – Clinical Features

• Thoracic – respiratory symptoms, incidental finding
• Abdominal – obstruction, mass, pain, GI bleeding, incidental on prenatal ultrasound
Duplication Cyst - Location

• Ileum - 40%
• Thorax (posterior mediastinum) - 20
• Jejunum - 10
• Stomach - 10
• Colon - 10
• Multiple - 5
Duplication Cyst - Pathology [Figure 6-3-27]

• Often share muscularis layer with adjacent bowel (intramural)
• Mesenteric border
• Filled with mucoid material
• Histologically, recapitulate normal GI tract
• May contain ectopic gastric mucosa (20%) or pancreatic tissue
Duplication Cyst - Imaging [Figures 6-3-26 and 6-3-28]

• Plain film – as soft tissue mass if large
• Ultrasound
➢ Preferred study
➢ “Rim” sign – gut signature in the wall
➢ Peristalsis
➢ May contain debris
Figure 6-3-28
Crohn Disease
• ¼ present in childhood
• Abdominal pain, diarrhea, hematochezia, weight loss
• CT for abscess, UGI/SBFT for diagnosis
• Ddx: TB, yersinia, pseudomembranous colitis,
lymphoma
Appendicitis
• Children more often have atypical presentation
• Children have a higher rate of negative laparotomy Duplication cyst (longitudinal and transverse)
and of perforation than adults showing round fluid-filled cavity surrounded by a
• Rare in infants wall with gut signature. Note echogenic rim of
mucosa

Appendicitis
• Appendicolith 10-15%
• US – blind-ending, uncompressible, > 6mm
• CT – ? IV, oral, rectal contrast – young children have no fat
• Pitfalls
➢ Perforated appendix may be decompressed
➢ Tip appendicitis
COLON/RECTUM
Neutropenic Enterocolitis
• AKA typhlitis
• Necrotizing enterocolitis often affecting the right colon in patients with neutropenia
• Pathologically similar to NEC in premature infants and pseudomembranous colitis
• Transmural – risk of perforation
Neutropenic Enterocolitis - Clinical

• Usually leukemic children on chemotherapy, but also those with aplastic Figure 6-3-29
anemia or immunosuppression for organ transplantation, and
AIDS
• Fever, nausea, vomiting, diarrhea, abdominal tenderness
• Mortality has decreased from 80% to less than 20% due to early
recognition and treatment
Neutropenic Enterocolitis - Imaging

• All imaging findings are nonspecific – clinical setting most helpful
• Plain films
➢ No gas in RLQ
➢ Dilated ascending colon
➢ Pneumatosis
• Ultrasound
➢ Thickened bowel wall and thickened mucosa
➢ Dilated, fluid-filled loops
➢ No ascites
Neutropenic Enterocolitis - Imaging [Figure 6-3-29]

• Contrast enema – contraindicated
• CT
Neutropenic enterocolitis. KUB shows
➢ Transmural wall thickening lack of bowel gas on the right and
➢ Infiltration of the surrounding fat markedly thickened haustra in the
➢ Can exclude other causes of RLQ pain right transverse colon. Note also the
• Ddx: pseudomembranous colitis, leukemic infiltration, intramural dual lumen catheter overlying the
hemorrhage, ischemic colitis abdomen, a clue to the underlying
diagnosis of leukemia
References
Texts
1. Donnelly LF. Fundementals of Pediatric Radiology. Philadelphia: W.B. Saunders Company, 2001.
2. Kuhn JP, Slovis TL, Haller JO, eds. Caffey’s Pediatric Diagnostic Imaging. Philadelphia: Mosby, 2004
3. Stringer DA, Babyn PS, eds. Pediatric Gastrointestinal Imaging and Intervention. Hamilton: B.C. Decker Inc.,
2000
4. Stocker JT, Dehner LP, eds. Pediatric Pathology. Philadelphia: Lippincott Williams & Wilkins, 2002
5. Swischuk LE. Imaging of the Newborn, Infant, and Young Child. Philadelphia: Lippincott, 2004
Journal Articles
1. Berrocal T, Lamas M, Gutieerez J, et al. Congenital anomalies of the small intestine, colon and rectum.
RadioGraphics 1999:19:1219-1236
2. Blumhagen JD, Maclin L, Krauter D, et al. Sonographic diagnosis of hypertrophic pyloric stenosis. AJR
1988;150:1367-1370
3. Buonomo C. Neonatal Gastrointestinal Emergencies. Radiology Clinics of North America 1997; 35: 845-864

4. Kleinman PK, Brill PW, Winchester P. Resolving duodenal-jejunal hematoma in abused children. Radiology
1986;160:747-750.
5. O’Keeffe FN, Stansberry SD, Swischuk LE, Hayden CK, Jr. Antropyloric muscle thickness at US in infants: what
is normal? Radiology 1991;178:827-830
6. Merritt CR, Goldsmith JP, Sharp MJ. Sonographic detection of portal venous gas in infants with necrotizing
enterocolitis. AJR 1984;143:1059-1062.
7. Segal SR, Sherman NH, Rosenberg HK, et al. Ultrasonographic features of gastrointestinal duplications. J
ultrasound Med 1994; 13:863-870.
8. Sivit CJ, Taylor GA, Newman KD, et al. Safety-belt injuries in children with lap-belt ecchymosis: CT findings in
61 patients. AJR 1991;157:111-114.
9. Sloas MM, Flynn PM, Kaste SC, Patrick CC. Typhlitis in children with cancer: a 30 year experience. Clin Infect
Dis 1993;17:484-90

Diseases Affecting the Pediatric Airway
Normal AP views of the airway. The left image

Normal sagittal MR of an adult and lateral soft shows the vocal cords open and the right image
tissue neck radiograph of a child showing normal shows the vocal cords coapted (arrows). Note
upper airway structures. also the false cords (block arrows). Between the
1.Adenoids (none in adult) true and false cords is the laryngeal ventricle. The
2. Vallecula true cords create shoulders on the subglottic
3. Epiglottis trachea resembling those of a wine bottle
4. Aryepiglottic folds
5. Subglottic trachea Figure 6-4-3
6. Retropharyngeal soft tissues
ACUTE UPPER AIRWAY OBSTRUCTION
Viral Croup
• Laryngotracheobronchitis
• Characteristic barking, brassy cough w/
inspiratory stridor Figure 6-4-4
• Most frequent cause of
stridor 6 mo - 3 yo
• Self-limited disease-7
days
• Parainfluenza
• Subglottic edema 5-10
mm below cords
• Less than 1% need
intubation
Viral croup. Note the
hypopharyngeal overdistension and
subglottic tracheal narrowing and
indistinctness (block arrow). The
most important finding on this film is
the normal pinkie-like epiglottis
(arrow)
Viral croup, AP view, showing

subglottic narrowing and loss of the
normal “shoulders” of the subglottic
trachea

1363 Diseases Affecting The Pediatric Airway
Figure 6-4-5
Epiglottitis [Figure 6-4-5]
• Life-threatening supraglottic inflammatory disease
• Clinically appear toxic
• Typically 3-6 yo
• Haemophilus influenzae, Group A strep
• Do not make uncomfortable
• Edema of epiglottis and aryepiglottic folds
Epiglottitis DDX
• Caustic ingestion or thermal injury
• Angioneurotic edema
• Radiation
• Sarcoidosis
• Hemorrhage Epiglottitis with epiglottis shaped like
• Abscess a thumb, thickened aryepiglottic
• Epithelial cyst folds, and loss of the normal
concavity of the aryepiglottic folds
• Omega epiglottis (arrow)
Retropharyngeal Cellulitis [Figures 6-4-6 to 6-4-8]

• Most common in children between 6 to 12 months
• Fever, stiff or wry neck, dysphagia
• Straightening or reversal of normal cervical lordosis
• Thickening of the prevertebral soft tissues
Figure 6-4-7
• May cause airway compression, vasospasm or venous thrombosis
(Lemierre syndrome)
• May extend into mediastinum or rupture into airway
Figure 6-4-6
AP and lateral films demonstrate a normal

epiglottis and aeryepiglottic folds with thickened
prevertebral soft tissues and reversal of the normal
lordosis of the cervical spine
Figure 6-4-8
Adapted from Rivera and Young,

unpublished material, 1992. A (solid Retropharyngeal cellulitis. Contrast enhanced
line) represents the thickness of the axial CT shows marked prevertebral soft tissue
prevertebral soft tissues anterior to swelling and ill defined hypodensity without ring
the intervertebral disc space C2-3. B enhancement
(dashed line) represents the AP
diameter of the base of C2
Diseases Affecting The Pediatric Airway 1362

Figure 6-4-9
Retropharyngeal Cellulitis - DDX

• Adenopathy
➢ Leukemia/lymphoma
➢ Kawasaki
➢ Langerhans cell histocytosis
• Edema
➢ SVC syndrome
➢ Angioneurotic edema
Parapharyngeal Abscess [Figure 6-4-9]

• Plain film cannot distinguish between cellulitis and abscess
• CT skull base to aortic arch
• Low density center surrounded by an enhancing rim characteristic
of an abscess
• Drainable pus vs. focal cellulitis – delayed imaging helps
• Ddx: necrotic nodes Axial CT of the neck demonstrates a
low density ring enhancing lesion in
the left parapharyngeal space
Membranous Tracheitis consistent with an abscess
• AKA Membranous croup, bacterial tracheitis
• Uncommon, life-threatening
• Exudative plaques form along tracheal walls like those seen in diphtheria
• Irregular tracheal wall, asymmetric subglottic narrowing, loss of definition of
the wall
ACUTE LOWER AIRWAY OBSTRUCTION
Reactive Airways Disease

• Very common with increasing incidence
• Obstruction due to bronchospasm
• May have other associated allergic disorders
• Precipitating factors
➢ Infections, especially RSV
➢ Weather changes
➢ GER
➢ Aspirin or NSAIDs
Reactive Airways Disease

• Chest radiograph – normal to hyperinflated with mild peribronchial cuffing and
parahilar increased markings
• Complications
➢ Atelectasis
➢ Air trapping
➢ Pneumonia
➢ Air block phenomena
Aspirated Foreign Body – Clinical

• Choking
• Loss of phonation
• Cough
• Wheezing
• Asymmetric or decreased breath sounds
• Hemoptysis
• Recurrent or persistent pneumonia

Aspirated Foreign Body – Radiology [Figure 6-4-10] Figure 6-4-10
• Maintain a high index of suspicion in crawling infants
and toddlers
• If unwitnessed, may present with chronic symptoms
suggesting pneumonia
• The vast majority are not radiopaque
• Most commonly lodge in the bronchus
• If initial radiographs are normal (up to 1/3 of
patients), do additional views to look for air trapping
– lack of change of volume of the affected lung
• Atelectasis and air trapping 18 month old with normal PA and lateral chest
radiographs but high clinical suspicion of aspirated
• Suspect in children <3 yo with pneumonia that fails foreign body. Right lung fails to collapse with
to clear after 2 weeks or with air block phenomena dependent positioning due to tortilla chip in the
right mainstem bronchus
Aspirated Foreign Body
• Treatment – bronchoscopic removal
• Complications
➢ Pneumothorax Figure 6-4-11
➢ Pneumomediastinum
➢ Chronic/recurrent pneumonia
➢ Bronchiectasis
Hydrocarbon aspiration
• Hydrocarbons – furniture polish, gasoline, kerosene, lighter fluid
• Aspirated due to low viscosity and surface tension
• Severe chemical pneumonitis with destruction of surfactant
• Radiographic abnormalities develop within 24h
• Pneumatoceles may develop
Upper esophageal foreign body

• Can be a cause of stridor
• Accompanied by dysphagia, but this symptom may go unnoticed
• Coins in esophagus are seen en face on PA chest, whereas coins
Axial CT in bone window showing
in trachea are seen in tangent on PA chest due to posterior gap in right bony choanal atresia (arrow).
the cartilage rings of the trachea Note the thickening of the posterior
• UGI helpful for radiolucent esophageal foreign bodies. Start with midline vomer and medial deviation
water soluble contrast. of the lateral wall of the nasal cavity
CHRONIC CONDITIONS AFFECTING THE AIRWAY
Nasal Cavity: Paranasal Sinuses

Figure 6-4-12
Choanal Atresia [Figure 6-4-11]
• Bony or membranous septum at posterior nasal septum
• If bilateral, will present early as neonates are obligate nasal
breathers
• CT with very thin cuts following nasal suction +/- topical
decongestants
• 90% are bony
• Thickening of the vomer and medial bowing of the lateral wall
of the nasal cavity
• Air-fluid level
Axial CT showing piriform aperture
CHARGE Association stenosis (arrows). Note the hour-glass
• Coloboma configuration of the nasal cavity
• Heart defect
• Atresia of the choana
• Retardation of growth and developmental delay
• Genital hypoplasia
• Ear deformities and deafness

Pyriform Aperture stenosis [Figure 6-4-12] Figure 6-4-13
• AKA “inlet” stenosis
• Cyanosis with feeds
• Width less than 11 mm considered abnormal
• Also well evaluated with CT
• Associated with abnormalities of pituitary gland and
dentition and craniofacial syndromes
Congenital Midline Nasal Mass

• Frontoethmoidal cephalocele
• Nasal glial heterotopia
• Nasal dermoid Nasal dermoid (arrow).Note midline bony efect,
seen best on CT
Congenital Midline Nasal Mass
• CT shows bony defect – 1- 1.5 mm w/ sagittal reformats
• MR shows intracranial extension and associated brain abnormalities Figure 6-4-14
➢ Cephalocele – dysgenesis of CC,interhem cyst/lipoma, cortical
dysplasia, craniofacial dysraphism
➢ Dermoid – intracranial dermoid/epidermoid
➢ Nasal glial heterotopia – no intracranial extension
Nasal Dermoid
• Midline, usually round and cystic
• May have hypertelorism or nasal pit
• May occur anywhere along dermal sinus tract
• Tract may communicate with the cranial contents
Dermoid bony defect [Figure 6-4-13]

Cephalocele [Figure 6-4-14]
• Meninges or meninges and brain herniate into the nasal cavity
through a defect in the cribiform plate or an open suture Sagittal T2-weighted MRI showing
• Anterior cephaloceles – sincipital (roof of nose) or basal (skull sphenoethmoidal encephalocele
base) with herniation of meninges and
optic chiasm
• Sincipital – nasofrontal, nasoethmoidal, or naso-orbital
• Nasal obstruction, rhinorrhea, epistaxis
Nasal Glial Heterotopia

• AKA nasoglioma
• Related to cephalocele
• No communication with intracranial contents
Juvenile Angiofibroma
• Highly vascular, locally invasive, histologically benign
• Adolescent boys w/ nasal obstruction, sinusitis and epistaxis
• Originates in sphenopalatine foramen, nasopharynx or posterior nasal cavity Figure 6-4-15
• Spreads early into pterygopalatine fossa,
infratemporal fossa, middle cranial fossa, orbit or
sphenoid sinus
Juvenile Angiofibroma [Figure 6-4-15]

• CT - sharply marginated mass with homogeneous
enhancement; anterior bowing of the posterior wall of
the maxillary sinus
• MR - iso- to hypointense to muscle on T1 and iso- to
hyperintense on T2 Juvenile angiofibroma. CT on left shows anterior
• MR differentiates tumor from secretions in obstructed bowing of posterior wall of maxillary sinus due to
sinus mass in pterygopalatine fissure. T1-weighted
• Prominent tumor vessels may be seen as flow voids contrast enhanced MR shows enhancing mass in
– internal maxillary artery left nasal cavity and ethmoid air cells with
nonenhancing trapped secretions in the maxillary
sinus

Sinus Mass Differential Figure 6-4-16
• Fungal sinus infection
• Mucocele
• Esthesioneuroblastoma
• Lymphoma
• Melanoma
Esthesioneuroblastoma [Figure 6-4-16]

• AKA olfactory neuroblastoma
• Very rare tumor originating from neural crest cells in
the olfactory groove
• Olfactory neuroblastoma
• Extensive destruction
• Extends intracranially
Rhabdomyosarcoma
• 3rd most common childhood primary malignancy of
the head and neck
• 40% arise in the head and neck Esthesioneuroblastoma on CT and coronal Gd-
enhanced T1W MRI showing mass in nasal cavity
• Orbit and nasopharynx most common with invasion of the right frontal lobe with
• Also paranasal sinuses and middle ear surrounding edema
• Usually embryonal cell subtype
• May spread intracranially via skull base foramina
• CT-iso to brain and enhances uniformly
• MR- T1 iso to muscle, T2 hyperintense and heterogeneous
• MR shows intracranial extension through fissures and foramina well
• CT shows bony erosion well
• Enhancement may make extracranial portions of the tumor less conspicuous
on MR
• Fat saturate post-gadolinium sequences of orbits and face
Nasal polyps
• Uncommon in children except those with cystic fibrosis
• Can also be associated with recurrent infection and allergies
• In CF can be so large and chronic as to widen the nasal passages
Antrochoanal Polyp [Figure 6-4-17]

• Polyp originating in the maxillary sinus
• Protrudes through an opening into the nasopharynx
• Unilateral nasal congestion is the most common symptom
• May present as large mass in oropharynx
• Ipsilateral maxillary sinus is opacified
• Incomplete resection associated with high recurrence rate
Palatine Tonsil and Adenoid Enlargement

• Adenoids are lymphoid tissue in the posterior nasopharynx
• Absent at birth
• Absence after 6 months suggests immune deficiency Figure 6-4-17
• May cause snoring, hypoxemia, respiratory acidosis,
pulmonary hypertension
• May obstruct Eustachian tubes
• Adenoidal pillow normally smooth
• Physiologic enlargement maximum age 3-5 years
• Significant enlargement obliterates nasopharyngeal
airway
• Mononucleosis (EBV,CMV) rare <3, mean 14 yo
Antrochoanal polyp extending from right maxillary

sinus into nasopharynx

Oral Cavity Hypopharynx Figure 6-4-18
Macroglossia
• Down’s syndrome
• Hypothyroidism
• Beckwith-Wiedemann syndrome
• Storage diseases
Mandibular Hypoplasia
• Hemifacial microsomia – unilateral
➢ Ipsilateral TMJ hypoplasia
➢ Congenital ear defects
• “First arch” syndromes
➢ Pierre Robin sequence
➢ Goldenhar syndrome Left image is an IV contrast-enhanced CT
➢ Weyer mandibulofacial dysostosis showing left parotitis. Compared to the normal
➢ Treacher Collins mandibulofacial dysostosis right parotid gland, the left is enlarged, enhancing
and has small hypodense foci consistent with
➢ Trisomies intragland abscesses. The image on the right
➢ Airway obstruction by normal sized tongue shows a large calcification in the duct of the right
submandibular gland
Inflammatory disease of the salivary glands
[Figure 6-4-18]
• Acute infection is viral or bacterial
• Bacterial sialadenitis is most common in parotid gland Figure 6-4-19
• Bacterial infection usually due to decrease in flow of
secretions
• Predisposing conditions include sialolithiasis
• Can occur in dehydrated newborns
• On CT the affected gland is enlarged and
hyperdense
• Intragland abscess may be seen
• Most calcified duct stones are visible on noncontrast
CT
• US can be used to evaluate salivary glands
• Obstruction of a sublingual duct or accessory duct is
a ranula
Inflammatory disease of the salivary glands

• Chronic disease
➢ Recurrent bacterial infection
➢ Granulomatous disease
➢ Autoimmune disease – Sjogren syndrome
➢ Lymphoepithelial cysts in HIV disease
Hypopharyngeal cyst
• Epiglottic or aryepiglottic folds
• Retention cysts or lymphatic malformations
• Inspiratory stridor or choking during feeding
• Present in infancy or early childhood Vallecular cyst (arrow). Bright on T2WI (top) and
• Treated with marsupialization nonenhancing on post-Gd T1WI (bottom)
Vallecular Cyst [Figure 6-4-19]

• Caused by obstruction of submucosal mucous and serous glands between the
epiglottis and base of tongue
• If large can cause SOB, hoarseness, dysphagia, failure to thrive, acute airway
obstruction
Tongue Base Mass

• Hemangioma
• Enlarged lingual tonsils
• Thyroglossal duct cyst
• Lingual thyroid

Lingual Thyroid [Figure 6-4-20] Figure 6-4-20
• Document orthotopic thyroid whenever evaluating a
near midline tongue-base or neck mass
• Ectopic thyroid most commonly near foramen cecum
Subglottic Trachea Neck
Childhood Neck Neoplasms

• Neuroblastoma
• Neurofibroma/schwannoma
• Lymphoma
• Hemangioma
• Lymphatic malformation/lymphangioma
• Teratoma
Subglottic Hemangioma
• Rare cause of stridor in infants, but most common Lingual thyroid. Left CT image shows
subglottic soft tissue mass causing upper airway homogeneous intensely enhancing mass at base
obstruction of tongue. Lower right CT image shows no thyroid
gland in the base of the neck. Upper right image
• Noisy breathing at 6-12 months is a pertechnetate scan showing no uptake above
• 50% have cutaneous hemangiomas the sternal notch and most uptake at the base of
• Asymmetric narrowing of subglottic trachea tongue
• Ddx: subglottic cyst, papilloma, cervical ectopic
thymic tissue, tracheal granuloma
Hemangioma
• Benign vascular tumor of infancy – high flow lesion Figure 6-4-21
• Most common tumor of infancy
• Usually absent at birth and appears in first few
months
• Early proliferative phase, later involutional phase
• May involve skin and have characteristic appearance
Hemangioma [Figure 6-4-21]

• May occur in the liver and cause high output heart
failure
• Very well marginated, intensely enhancing with large
feeding vessels Hemangioma on contrast enhanced T1-weighted
• If it does not look like a hemangioma, it is not a MR image in the posterior neck. The mass very
hemangioma well defined and homogeneous except for the
multiple vascular flow voids. Compare to well
• Only require treatment if affect airway or vision or marginated, lobulated surface of the resected
cause intractable heart failure tumor
Hemangioma
• PHACE syndrome
➢ Posterior fossa abnormalities
Figure 6-4-22
➢ Hemangioma of the face
➢ Arterial abnormalities
➢ Coarc/cardiac defects
➢ Eye abnormalities
• Kassabach-Merritt syndrome
➢ Thrombocytopenia and consumptive
coagulopathy associated with vascular tumor
Congenital teratoma of anterior neck. MR

and CT show complex appearance
characteristic of a mature teratoma with
cystic and solid components. Note
deviation of the trachea

Teratoma [Figure 6-4-22]
• Arise from pleuripotential cells
• 20% malignant
• Neck – large anterior mass in neonate, respiratory distress and dysphagia
• Heterogeneous – cyst, calcification, fat
Lymphoma
• Common malignant tumor in the neck
• Both Hodgkin's and NHL
• NHL more frequently involves extranodal sites, including tonsils and adenoids
• Immunodeficiency predisposes
• Enlarged lymph nodes and conglomerate masses of nodes
• On ultrasound the nodes may be sonolucent
Lymphoma
• On CT, isointense to muscle
• On MR, isointense to muscle on T1WI and hyperintense on T2WI
• Burkitt lymphoma – mass originating in jaw
Neuroblastoma
• Primary or metastatic in neck
• Neck or thoracic primary has better prognosis than intra-abdominal primary
• Calcification common
• US may show increased blood flow
• May involve skull or extend intracranially
Figure 6-4-23
Fibromatosis Colli [Figure 6-4-23]
• Focal thickening or mass of sternocleidomastoid muscle
associated with torticollis
• Noted at or shortly after birth
• Histologically – atrophy and partial replacement of muscle with
fibrous tissue
• US preferred – shows continuity of mass with SCM
• Resolves with physiotherapy
Larynx and Trachea - Intrathoracic Airways
Laryngomalacia
• Most common laryngeal abnormality of the neonate
• Early inspiratory stridor US of sternocleidomastoid muscle
• Worsens at rest - unusual showing fusiform enlargement
• Laryngeal collapse during inspiration with hypopharyngeal characteristic of fibromatosis colli
overdistension seen on airway fluoroscopy
• Usually resolves by age 1 year
Laryngotracheal cleft
• AKA persistent esophagotrachea
• Extreme form of failure of separation of trachea from foregut
• Spectrum from posterior laryngeal cleft to common tube
• May have abnormal cry or mutism
• Symptoms mimic esophageal atresia
• Esophagram shows massive aspiration
Tracheomalacia
• Collapse of the trachea with expiration
• Delayed development of cartilage
• Focal or generalized
• Recurrent infections and stridor
• Compressed in AP diameter
• Associated with esophageal atresia and vascular ring
• Also common in Down’s syndrome

Tracheal Stenosis [Figure 6-4-24] Figure 6-4-24
• Acquired – traumatic intubation
• Congenital
➢ Subglottic smooth, circumferential narrowing –
looks like croup
➢ Intact cartilage rings
➢ Associated with pulmonary sling, TE fistula,
pulmonary atresia or hypoplasia
• Biphasic stridor
Tracheobronchomegaly
• AKA Mounier-Kuhn
• Dilation of airways in inspiration
• Due to congenital deficiency of elastic tissue
• 3rd-5th decade
• Dilated trachea and central bronchi with diverticulosis
of trachea Bronchogram showing long segment tracheal
• Perihilar bullae stenosis in a patient status post surgical
correction of pulmonary sling. Note that the
trachea is of smaller caliber than either mainstem
Tracheal bronchus [Figure 6-4-25] bronchus
• RUL bronchus arises directly from trachea
Figure 6-4-25
• 1% of the population
• May supply whole RUL or a supernumerary segment
• Persistent RUL pneumonia, atelectasis or air trapping
• Associated with TEF, tracheal stenosis, pulmonary
sling, Down syndrome
Bronchial Atresia [Figure 6-4-26]

• Lobar or segmental luminal fibrosis of bronchus
• Recurrent infections, dyspnea or asx
• UL and RML most frequent
• Involved lung is fluid then air-filled with air trapping
• Tubular soft tissue density – trapped mucus just distal
to atresia
Laryngeal- Tracheopapillomatosis
• Most common laryngeal tumors in infancy Tracheal bronchus. Postmortem bronchogram
• Human papilloma virus implicated and specimen showing small bronchus to
supernumerary right upper lobe arising directly
• 2/3 pts less than 4 yo from the trachea
• Dx made on endoscopy with nodules on vocal cords
• Transbronchial spread < 5%, related to surgical procedures Figure 6-4-26
• Pulmonary solid lesions with cavitation
• Poor prognosis with pulmonary involvement
Bronchial atresia with abrupt cut off

of bronchus dilated and filled with a
mucous plug (arrow). Note also the
adjacent air trapping
References
Texts
1. Ball WS Jr. Pediatric Neuroradiology. Philadelphia: Lippincott-Raven, 1997.
2. Barkovich AJ. Pediatric Neuroimaging. 3rd ed. Philadelphia: Lippincott-Raven, 2000.
3. Donnelly LF. Fundamentals of Pediatric Radiology. Philadelphia: W.B. Saunders Company, 2001.
4. Kirks DR, ed. Practical Pediatric Imaging. 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 1998.
Journal Articles
1. Capitanio MA and Kirkpatrick JA. Upper respiratory tract obstruction in infants and children. Radiol Clin North
Am 1968;6:265
2. Chinwuba C, Wallman J and Strand R. nasal obstruction: CT assessment. Radiology 1986;159:503
3. Dunbar JS. Upper respiratory tract obstruction in infants and children. AJR Am J Roentgenol 1970;109:227-246.
4. John SD, Swischuk LE. Stridor and upper airway obstruction in infants and children. RadioGraphics 1992;12:625-
643.
5. Panicek DM, et al. The continuum of pulmonary developmental abnormalities. RadioGraphics 1987;7:747.

Vascular Rings and Slings
Vascular Rings Figure 6-5-1

• Double aortic arch
• Right arch with aberrant LSCA
• Pulmonary sling
• Left arch with aberrant RSCA
• Anomalous innominate artery
• Descending aorta-carina compression
• Respiratory symptoms – tight complete rings
➢ Stridor
➢ Recurrent respiratory difficulties
➢ Apnea
• Feeding difficulties
Early in fetal development the
➢ Choking with feeds primitive aorta develops as a ventral
➢ Failure to thrive tube which separates into two ventral
➢ Solid food dysphagia aortae. Two dorsal aortae fuse to form
a single vessel supplying the lower
Evaluation of Stridor/Dysphagia body. As the pharyngeal pouches
develop at the rostrum of the embryo,
• PA and lateral chest and high kV airway films – determine side of so too do paired pharyngeal arterial
arch arches, numbering 6 (but no 5th arch
• Esophagram with airway fluoroscopy in humans) between the ventral and
➢ MRI/MRA or CTA if vascular ring suspected dorsal aortae. These arches go on to
➢ Otherwise, CT fuse, partially regress and fully
regress to form the pulmonary
arteries and the mature left-sided
Embryology of the Normal Left Aortic Arch aortic arch and its branches
[Figures 6-5-1 to 6-5-4]
• Arches 1 and 2 regress
• Arch 3 --> common and proximal internal carotid arteries
• Arch 4
➢ Right --> regresses (portion of right subclavian)
➢ Left --> persists as LEFT AORTIC ARCH
• Arch 5 – rudimentary in humans
• Arch 6 – pulmonary arteries and ducti arteriosi Figure 6-5-2
➢ Right regresses
➢ Left becomes ligamentum arteriosum
• Dorsal aortae
➢ Right regresses – part of RSCA
➢ Left becomes descending aorta
In the development of the normal left

aortic arch, the gap or complete
regression occurs in the 8th segment
of the right dorsal aorta. The normal
arch has 3 branches – the
brachiocephalic (innominate), the left
common carotid, and the left
subclavian arteries
Vascular Rings and Slings 1372

Figure 6-5-3
Edwards postulated the existence of a double arch with
bilateral ducti arteriosi in the embryo. This double arch can be
represented by this ring. The T in the center represents the
pulmonary arteries. The black lines connecting the main
pulmonary arteries to the aorta represent the bilateral ducti
arteriosi. The anterior center of the ring (dotted circle)
represents the ventral or ascending aorta branching into
bilateral arches which join to form the descending aorta in the
posterior center of the ring (dotted circle). Each arch gives rise
to its own carotid and subclavian artery. The anterior solid
circles represent the bilateral carotid arteries, and the posterior
solid circles are the bilateral subclavian arteries. The portion of
the ring between the carotid and subclavian arteries is formed
from the 4th pharyngeal arch. The portion of the ring dorsal to
the subclavian artery represents the contribution of the 8th
segment of the dorsal aorta. These are the most common
sites of gaps or complete regression of a portion of the ring
that give rise to the different types of aortic arches
Figure 6-5-4
Figure 6-5-5
Diagram representing the normal regression In the development of the aberrant right
of the 8th segment of the right dorsal aorta subclavian artery, there is early obliteration of the
and right ductus arteriosus in development of right 4th arch, and the 8th segment of the right
the normal left arch. The right subclavian dorsal aorta persists, so the right subclavian artery
artery is separated from the descending maintains its connection to the descending aorta,
aorta and arises in common with the right becoming the last branch from the aortic arch
common carotid artery from the ascending (arrow). Thus, there are four, rather than the
aorta normal three, branches of the aortic arch and
there is no brachiocephalic (innominate) artery.
The descending aorta is on the left, so the
Aberrant RSCA [Figures 6-5-5 to 6-5-8] aberrant RSCA then crosses behind the
• Asymptomatic in children – normal variant esophagus to get to the right side
• Due to early complete obliteration of right 4th arch
and persistence of 8th segment of the right dorsal aorta
• Left arch
• RSCA originates distal to LSCA Figure 6-5-6
• Posterior impression on the esophagus
The gap or obliteration

occurs in the right 4th arch.
The aortic arch is on the
left

1375 Vascular Rings and Slings
When the RSCA crosses from left to right a

posterior impression is created on the esophagus, The oblique course of the aberrant
as seen on the lateral view (left image). In the AP right subclavian artery creates a
view (right image), a normal left aortic arch is sloped contour of the aortic knob
seen as well as an oblique impression on the (arrow) that may be visible in older
contrast-filled esophagus extending from the left children and adults
arch to the right shoulder
Determining Side of Arch [Figures 6-5-9 and 6-5-10] Figure 6-5-10

• Tracheal deviation, buckling or impression – the most reliable sign
indicating the side of the aortic arch
• Asymmetric density of pedicles
• Descending aorta can be on right or left with right arch
Figure 6-5-9
PA chest radiograph showing right-

sided impression on the trachea due
to right aortic arch
Normal tracheal buckling away from

the left arch on an expiratory chest
radiograph (arrow)
Right Arch
• Branching patterns
➢ Aberrant LSCA
➢ Mirror image
➢ Isolated LSCA – congenital subclavian steal
• High association with congenital heart disease
Mirror Image Right Arch [Figures 6-5-11 and 6-5-12]

• Association with CHD 98%
• 25% of patients with tetralogy of Fallot have mirror image right arch
• 35% of patients with truncus arteriosus have mirror image right arch
• 90% of patients with mirror image right arch have tet

Patient with right-sided arch and

descending aorta and enlarged heart
with upturned apex, a classic plain
film diagnosis of Tetralogy of Fallot
Figure 6-5-13
Diagram showing the mirror-image

branching right arch from the front.
The ductus is on the left (arrow) so
this is not a ring. A rare variant with
ductus arteriosus from proximal
descending aorta coursing behind
esophagus to left pulmonary artery,
the so-called retro esophageal
ductus, is a true ring
Aberrant LSCA [Figure 6-5-13]

• Mirror image of aberrant RSCA
• Left ductus arteriosus completes the ring
• Symptomatic patients typically have a a tight ductus
or large diverticulum of Kommerell (dilation of origin
Diagram showing development of right arch
of aberrant artery) aberrant left subclavian artery which is the mirror
• This type needs to be distinguished from double arch image of left arch aberrant right subclavian artery
with MRI or angiography (shown on the left). The right image shows early
• Association with congenital heart disease 5-12% obliteration of the left 4th pharyngeal arch,
• Dysphagia lusoria separating the left subclavian artery from the left
common carotid artery. Persistence of the 8th
• Posterior impression on the esophagus segment of the left dorsal aorta maintains
• Right-sided impression on the trachea continuity of the left subclavian artery with the
• Ddx: double arch descending aorta. The left ductus completes the
ring
Right arch with aberrant LSCA and left
ductus [Figure 6-5-14] Figure 6-5-14
This diagram shows the right arch with aberrant

left subclavian artery viewed from anterior. Note
the four branches of the aortic arch, the last of
which is the left subclavian artery. The left
ductus (arrow) completes the ring, so that the
trachea and esophagus are completely
surrounded by aorta and pulmonary arteries

Right arch aberrant LSCA with posterior impression Figure 6-5-16
on esophagus [Figures 6-5-15 to 6-5-18]
Figure 6-5-15
CT of same patient showing right

arch and posterior course of the
aberrant left subclavian artery (arrow)
Esophagram shows posterior impression on the esophagus at Figure 6-5-17

the level of the aortic arch. When the patient is in the AP
projection the right arch is identified. If there were a left arch,
this patient would have an asymptomatic normal variant. With
the right arch, the diagnosis is a complete vascular ring
Cervical aortic arch Figure 6-5-18

• Arch above the clavicle – not a specific
arch anomaly
• May have pulsatile mass in
supraclavicular fossa
• 80% are right arches
• Half are symptomatic rings
• Most common variant - right arch that
descends on the right then crosses to
left behind esophagus and gives off left
subclavian artery and left ductus
This sagittal MR image in a 4 month
Double Aortic Arch old with recurrent respiratory
[Figures 6-5-19 to 6-5-21] infections, shows the aberrant
• Persistence of both left and right fourth subclavian artery in cross section
arches posterior to the trachea (arrow).
Arteriogram showing a Normally there is no large artery
• Most common symptomatic vascular behind the trachea
ring right arch with aberrant
LSCA and a
• Right is usually larger, higher and diverticulum of
posterior Kommerell (arrow), or Figure 6-5-19
• Anterior and bilateral lateral enlargement of the
impressions on the trachea origin of the aberrant
• Posterior and bilateral lateral subclavian artery
impressions on esophagus
• Treatment is to ligate the nondominant arch
Figure 6-5-20
Esophagram in same
patient. The double arch
causes bilateral lateral
impressions on the
esophagus as seen on PA chest radiograph in a patient with
the AP view (left image). double aortic arch, showing a higher
On the lateral view (right larger right sided aortic impression
image), there is a on the trachea and a smaller, lower
prominent posterior left sided impression (arrow). The
impression caused by left impression is often difficult to
the joining arches. Thus, discern, so double aortic arch is in
there are 3 impressions the differential of right aortic arch
on the esophagus seen on plain film

Pulmonary Sling [Figures 6-5-22 and 6-5-23] Figure 6-5-21
• AKA anomalous pulmonary artery
• Left PA originates from right
• Anterior impression on esophagus at level of carina
• Posterior impression on trachea
• Ductus passes from origin of RPA to aorta forming a complete
ring around the trachea only
• Compression of bronchus intermedius by anomalous artery
• Associated tracheal abnormalities
➢ Tracheomalacia
➢ Complete tracheal rings
➢ T-shaped trachea
• Other associated anomalies – abnormal pulmonary lobation,
bronchus suis, CHD
Figure 6-5-22
3-D MRA of a double aortic arch,
viewed from posterior
Figure 6-5-23
Left image is a lateral view from an esophagram

showing posterior impression on the trachea and
anterior impression on the esophagus at the level
of the hila. Black blood MRI image shows the left
Illustration of pulmonary sling viewed pulmonary artery originating from the right and
from anterior with ascending aorta cut coursing behind the trachea to get to the left lung
away to show the left pulmonary
artery (arrow) originating from the
right, then passing between the
trachea and esophagus to get to the
left side
Innominate Artery Compression Syndrome

• Normally the innominate artery passes in front of the trachea just below the
thoracic inlet
• In infants it arises more to the left than in adults and there is also thymus in
this region, so it may cause symptomatic compression of the trachea
• Increased incidence of symptomatic compression in patients with dilated
esophagus
• Compression decreases with advancing age
Midline Aorta Carina Compression Syndrome

• Midline course of descending aorta or abnormal position of the carina allows
aorta to compress carina or mainstem bronchus
Other CV Abnormalities That Can Compress the Airway
Congenital Heart Disease

• Tetralogy of Fallot with absent pulmonary valve
• Large left to right shunts
• Massive cardiomegaly

Absent Pulmonary Valve Syndrome [Figure 6-5-24] Figure 6-5-24
• Variant of Tetralogy of Fallot
• Severe pulmonic regurgitation
• Aneurysmal dilatation of proximal left and right
pulmonary arteries
• Compression of adjacent bronchi
PA and lateral chest radiographs in patient

Tetralogy of Fallot with absent pulmonary valves.
Note the massive enlargement of the pulmonary
arteries, hyperinflation of the lungs due to bilateral
bronchial compression and associated right aortic
arch
References
Texts
1. Donnelly LF. Fundamentals of Pediatric Radiology. Philadelphia: W.B. Saunders Company, 2001.
2. Kirks DR, ed. Practical Pediatric Imaging. 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 1998.
3. Swischuk LE. Imaging of the Newborn, Infant, and Young Child, 5th ed. Philadelphia: Lippincott-Williams &
Wilkins, 2004.
Journal Articles
1. Berdon WE and Baker DH. Vascular anomalies and the infant lung: rings, slings and other things. Semin
Roentgenol 1972;7:39-63.
2. Berdon WE. Rings, slings and other things: vascular compression of the infant trachea updated from the
midcentury to the millennium—the legacy of Robert E. Gross, MD, and Edward B. D. Neuhauser, MD. Radiology
2000;216:624-632.
3. Bisset GS III et. Al. Vascular rings: MR imaging. AJR Am J Roentgenol 1987;149:251
4. Donnelly LF, Bisset GS 3rd , McDermott B. Anomalous midline location of the descending aorta: a cause of
compression of the carina and left mainstem bronchus in infants. AJR AM J Roentgenology 1995; 164:705-707.
5. Donnelly LF, Strife JL, Bisset GS III. The spectrum of extrinsic lower airway compression in children: MR
imaging. AJR Am J Roentgenol 1997;168:59-62
6. Kussman BD, Geva T, McGowan FX. Cardiovascular causes of airway compression. Paediatr Anaesth 2004;14:60-
72.
7. Newman R, Meza MP, Tobin RB, et al. Left pulmonary artery sling: diagnosis and delineation of associated
tracheobronchial anomalies with MR. Pediatr Radiol 1996;26:661-668
8. Pickhardt PJ, Siegel MJ, Gutierrez FR. Vascular rings in symptomatic children: frequency of chest radiographic
findings. Radiology 1997;205:581-582
9. Shuford WH, Sybers RG, Edwards FK. The three types of right aortic arch. AJR 1970;109:67-74

Cystic Renal Disease of Childhood
Terminology
• Cyst
• Polycystic kidney disease – ARPKD and ADPKD only
• Multicystic kidney
Old Classification1
• Type I, infantile polycystic kidney disease
• Type II, multicystic dysplastic kidney
• Type III, adult polycystic kidney
• Type IV, cortical cysts associated with massive hydronephrosis
1 Osthanondh V, Potter EL. Pathogenesis of polycystic kidneys: historical survey.
Arch Pathol 1964;77:459
Genetically-based Classification2
• Nongenetic
➢ Multicystic dysplasia
➢ Multilocular cyst (tumor)
➢ Simple cyst/calyceal diverticulum
➢ Medullary sponge kidney
➢ Acquired cystic disease
• Genetic
➢ Autosomal recessive polycystic kidneys
➢ Autosomal dominant polycystic kidneys
➢ Juvenile nephronophthisis (AR)-medullary cystic disease (AD) complex
➢ Cysts associated with multiple malformation syndromes
2Glassberg KI, Stephens FC, Lebowitz RL, et al. Renal dysgenesis and cystic
disease of the kidney: a report of the Committee on Terminology, Nomenclature
and Classification, Section on Urology, American Academy of Pediatrics. J Urol
1987, Oct; 138:1085
Simple Renal Cyst

• Uncommon in children
• Usually solitary
• Found with increasing frequency due to US screening in patients with UTI
• Arise in renal cortex
• Do not communicate with collecting system
Simple Renal Cyst

• Observed on PNUS ◗ screen for malformation syndrome
• US criteria for simple cyst ◗ no further imaging unless recurrent symptoms of
infection
• Otherwise ◗ CT to exclude tumor
• No treatment unless symptomatic or obstructing collecting system
Calyceal Diverticulum
• Cyst that communicates with collecting system
• Need contrast study to distinguish from cyst
• Urine stasis leads to infection and stone formation
• Look for stone in tic on US, KUB, or non-con CT
• Delayed images show contrast-filling of the cyst and a neck
• Treatment – surgical ablation if symptomatic
Pediatric Radiology 1381 Cystic Renal Disease of Childhood

Medullary Sponge Kidney Figure 6-6-1
• Congenital focal dilation of collecting tubules usually presenting in
adulthood
• Associated with stones or infection but may be found incidentally
• Medullary nephrocalcinosis – radially aligned
• Streaky linear densities in involved pyramids
• US may show calcifications before plain film
Acquired Renal Cysts

• AIDS
• Hemo- and peritoneal dialysis
➢ Increase in number and size with length time on dialysis
• Complications – intracyst or subcapsular or perinephric
hemorrhage
Multicystic Dysplastic Kidney

• Most common form of cystic renal disease in infants and children
• One of the most common causes of renal mass in first week of
life
• Rarely bilateral Classic MCKD gross specimen
• Due to early severe in utero obstruction showing nonreniform shape, cysts of
• Extreme end of spectrum of UPJO multiple sizes and no identifiable
renal parenchyma
• Risk of abnormality of contralateral kidney 20-50% - UPJO, VUR
• Negligible renal function
• Nodular blastemal elements in 3-5% - risk of Wilms tumor
MCDK - Presentation
• PNUS Figure 6-6-2
• Neonate with abdominal mass
• Incidentally in older child – mimics agenesis
MCDK – Gross Pathology [Figures 6-6-1 to 6-6-3]

• Macrocysts of variable size
• Randomly distributed
• Cysts do not communicate
• Hydronephrotic variant – large central cyst
• Rarely segmental – upper pole of duplex or lower crossed fused
ectopic
• No identifiable normal parenchyma Hydronephrotic variant of MCDK with
• Associated atresia of ureter or infundibulopelvis reniform shape and patent central
pelvis
Figure 6-6-3
Stillborn baby with bilateral MCDK.

Plain radiograph shows small, bell-
shaped thorax with airless lungs and
bulging flanks. Autopsy specimen
(viewed from posterior) reveals bilateral
enlarged kidneys which are much
larger than the hypoplastic lung
Cystic Renal Disease of Childhood 1380

MCDK - Histopathology [Figure 6-6-4] Figure 6-6-4
• Histologic hallmark – presence of immature dysplastic-appearing tubules
surrounded by collarettes of PAS-staining condensed mesenchyme
• Cysts of varying size formed by dilated, dysplastic tubules
• Cysts can occur in any part of the nephron
• High nuclear to cytoplasmic ratio ◗ dysplasia
MCDK - Imaging [Figures 6-6-5 and 6-6-6]

• Large mass with cysts of varying sizes scattered throughout
• Cysts do not communicate
• Hydronephrotic type – large central cyst but no identifiable parenchyma
• No identifiable cortex or medulla
• Nonreniform shape
• Nuclear renogram – no significant excretion
• VCUG – 25% VUR
Figure 6-6-5
Photomicrograph of
dysplastic kidney showing
primitive ducts surrounded
by mesenchymal collarettes
Figure 6-6-6
Prenatal ultrasound showing right

MCDK
MCDK - DDx
• Multilocular cystic kidney
➢ A tumor
➢ Cysts within the intervening septa
• Severe hydronephrosis
➢ UPJO
➢ Nuclear renogram
➢ Contralateral kidney also affected Renal ultrasound showing normal right kidney and
left MCDK. Note noncommunicating cysts and
MCDK – Course and Prognosis lack of visible normal renal parenchyma
• Natural history of a true MCDK is to resolve
• Formerly these were all removed due to rare reports of nephroblastoma1 Figure 6-6-7
• Now followed to resolution
• If they do not resolve, surgical removal is indicated to prevent
complications of infection and neoplasm
1Strife JL, Souza AS, Kirks DR, Strife CF, Gelfand MJ, Wacksman J.
Multicystic dysplastic kidney in children: US follow-up. Radiology.
1993 Mar;186(3):785-8.
Autosomal Recessive Polycystic Kidney Disease

(ARPKD)
• Kidneys and liver – ectasia and fibrosis
• Kidneys – ectasia of the collecting tubules Delayed CT diagnosis of left MCDK
• Liver – biliary duct ectasia and periportal fibrosis which has regressed to a partially
• Latter develops in early childhood calcified nubbin
• Degrees of renal and liver involvement are inversely proportional and
determine age of presentation and prognosis

1383 Cystic Renal Disease of Childhood
ARPKD – Spectrum of Presentation Figure 6-6-8
Blyth and Ockenden Clinical Classification1
[Figures 6-6-9 and 6-6-10]
• “Perinatal” – 90% of tubules involved, bilateral nephromegaly,
Potter syndrome, death in first week
• “Neonatal” – 60%, present in first month, death by 1 year
• “Infantile” – 25%, present at 3-6 mo
• “Juvenile” – 10%, presentation in first decade
➢ Incidental finding on US
• Presentation in second decade with renal failure
CT showing Wilms tumor which arose
1Blyth H, Ockenden BG. Polycystic disease of kidney and liver in a left MCDK
presenting in childhood. J Med Genet. 1971 Sep;8(3):257-84.
Figure 6-6-9
Figure 6-6-10
ARPKD Autopsy
ARPKD – Pathology - Kidneys [Figure 6-6-11]

• Large kidneys
• “Cysts” are dilated collecting tubules predominantly in the medulla
• Dilated (1-2mm) tubules arranged in a fan-shape
• Cortex relatively spared
• No dysplasia
• On cut section, cortex and medulla unrecognizable
• Few coalescent macrocysts
ARPKD – Pathology- Liver [Figures 6-6-12 and 6-6-13]

• All associated with congenital hepatic fibrosis = ductal plate
malformation Gross image showing Potter facies
• Dilation of interlobular bile ducts associated with a variable
amount of portal fibrosis (Caroli syndrome)
• All portal areas are expanded and contain dilated ducts at the periphery with
blood vessels in the middle
• Sinusoidal portal hypertension
ARPKD – Plain Film

• Bilateral flank masses in newborns
• Small, bell-shaped thorax
• Pneumothorax Figure 6-6-11
• Older children may have slightly enlarged kidneys,
hepatosplenomegaly, and/or ascites
ARPKD - Ultrasound [Figure 6-6-14]

• Large kidneys with increased echogenicity in the
medulla due to multiple accoustic interfaces of
dilated, ectatic ducts
• May mimic nephrocalcinosis
• Compressed, spared cortex may form relatively dark
rim ARPKD. From left to right cut gross specimen,
• Poor delineation of cortex, medulla, sinus photomicrograph and diagram show dilated
collecting tubules in the medulla

Figure 6-6-12
Formation of the intrahepatic biliary radicals
begins with a single layer of primitive ductal plate
surrounding the portal vein. Some insult can
cause congenital hepatic fibrosis by promoting
fibroblast proliferation.
Figure 6-6-13
Figure 6-6-14
Gross specimen showing ectatic

biliary ducts of Caroli disease
ARPKD - Ultrasound [Figure 6-6-15]

• High frequency linear transducer may resolve tubular structures
in fan-like array or tiny cysts
• Occasional macrocysts
• Those who present as children have milder renal findings –
normal or mild nephromegaly, +/- increased echogenicity of ARPKD. Coronal ultrasound shows
both kidneys to be markedly enlarged
medulla, loss of corticomedullary differentiation and echogenic centrally with a
relatively sonolucent rim of
Ultrasound - Liver [Figure 6-6-16] compressed cortex
• Increased echogenicity +/- ductal ectasia in older children
• Portal radicals surrounded by bile ducts
• Splenomegaly, varices, and ascites may also be seen in older children
ARPKD. High frequency linear transducer 12 yo diagnosed in infancy with ARPKD, now
ultrasound images show echogenic, markedly preop for liver transplant. Ultrasound on left shows
enlarged kidneys in which tiny cysts can be markedly dilated biliary ducts with hepatic artery
resolved and portal vein branches in the center, creating a
target appearance. CT shows same target
appearance of biliary ducts as well as cysts in the
renal medulla and splenomegaly due to portal
hypertension

ARPKD - Other Imaging [Figures 6-6-17 to 6-6-19]
• Imaging beyond US is rarely necessary
• Spoke-wheel or striated appearance of nephrogram
• Prolonged nephrographic phase
• Calyces compressed, separated and distorted
• In older children, mild tubular ectasia similar to medullary sponge kidney
• MR - few cortical cysts in about half of patients
Figure 6-6-18
Figure 6-6-17
ARPKD. CT of infant showing

markedly enlarged kidneys with
striated delayed nephrograms due to
compression of parenchyma by
radially oriented dilated fluid-filled
collecting ducts. The kidneys are of
density similar to water due to the
unopacified urine in the dilated ducts.
Note rim of enhancing cortex (arrow).
Also note left kidney hypodense
macrocyst (block arrow)
Excretory urogram of ARPKD

showing bilateral massively enlarged
kidneys with striated nephrograms
and distorted collecting systems
ARPKD - Prognosis
• Infantile – poor prognosis due to renal insufficiency and
pulmonary hypoplasia Figure 6-6-19
• Outcome in childhood is better than previously thought
• Early recognition and management are important
Large Echogenic Kidneys in Neonate
• Glomerulocystic disease
• ADPKD
Glomerulocystic Disease
• Rare, sporadic or heritable (AD)
• May be found in some patients with malformative syndromes
• Occasionally found in children with family history of ADPKD
Glomerulocystic Disease Clinical Features

• Present early with renal failure and palpable abdominal masses
• Renal function normal but deteriorates with age
ARPKD with biliary duct ectasia. CT

of the kidneys shows delay of transit
of contrast into dilated, urine-filled
collecting tubule

Glomerulocystic Disease Pathology [Figure 6-6-20] Figure 6-6-20
• Cystic dilation of Bowman capsule and proximal
convoluted tubule
• Periportal fibrosis, bile duct hyperplasia and hepatic
cysts may also be found
Glomerulocystic Disease Ultrasound

• Echogenic normal-sized to enlarged kidneys
• Poor corticomedullary differentiation
• Tiny cysts may be seen in cortex (vs. ARPKD)
Autosomal Dominant Polycystic Kidney

Disease (ADPKD)
• Much more common than AR
• Three genetic loci – PKD1-3
• Family screening routine
• Spontaneous mutations frequent Glomerulocystic disease in a patient with
• Cysts become larger and more numerous with age Zellweger or cerebrohepatorenal syndrome. Gross
and low magnification images show small cortical
Autosomal Dominant Polycystic Kidney cysts
Disease (ADPKD)
• Cysts not usually seen in 1st and 2nd decades but can be seen in neonates
screened for positive family history
• Affects multiple organs – kidneys, liver, pancreas, spleen, seminal vesicles,
ovaries
• Association with occult intracranial aneurysms – screen in adulthood
ADPKD - Presentation
• Present in 4th-5th decade with hypertension and renal failure
• Does not present in childhood but may be found incidentally or secondary to
flank pain due to bleeding into cyst
• Rarely presents in infancy – minimal cysts in enlarged, echogenic kidneys
ADPKD - Pathology
• Enlarged but reniform kidneys
• Cysts of varying size scattered throughout the kidney (cortex and medulla)
• Usually bilateral but may be asymmetric or even unilateral
• Abnormality of the ampullary and interstitial portions of the collecting tubules
and nephrons
• Hepatic fibrosis is rare
Figure 6-6-21
ADPKD - Ultrasound [Figure 6-6-21]
• Infantile presentation – small, spherical cysts on high resolution
US (vs. fan-like, tubular appearance in AR)
• Older children - cysts of varying size in cortex and medulla
• Can be unilateral at presentation
• Normal size or slightly enlarged
• Look for cysts in liver and pancreas
ADPKD - Prognosis
• Most develop renal failure in 4th-5th decade
• Presentation in infancy – more severe renal cystic disease, more
hypertension, more rapid progression to renal failure than adult
relatives
Medullary Cystic Disease Complex

• Juvenile nephronophthisis – AR, presents in first decade
• Medullary cystic disease – AD, presents in 3rd decade Neonatal ADPKD. Kidneys are
• Polydypsia, polyuria, salt wasting, severe anemia echogenic but high resolution
• Progressive renal failure ultrasound helps to distinguish small
• Growth retardation cysts (arrows) (vs. radially aligned,
tubular cysts in ARPKD)

Medullary Cystic Disease Complex Pathology Figure 6-6-22
[Figure 6-6-22]
• <1.5 cm cysts that increase in size and number with age
• Cysts in medulla or subcortical region -70% of patients
• Secondary glomerulosclerosis – small, fibrotic kidneys
• Associated retinitis pigmentosa, hepatic fibrosis, skeletal defects
and CNS abnormalities in familial juvenile form
Medullary Cystic Disease Complex Imaging

[Figure 6-6-23]
• Small echogenic kidneys
• Multiple small medullary or corticomedullary cysts – may not be
present when very young
Cysts Associated with Syndromes

• Zellweger - cerebrohepatorenal syndrome – AR, lethal
peroxisomal deficiency
• Meckel-Gruber – AR, posterior encephalocele, polydactyly, cystic
kidneys, congenital hepatic fibrosis
• Beckwith-Wiedemann syndrome – macroglossia, visceromegaly
and omphalocele, Wilms tumor, also medullary cysts in 13-19%
Cysts Associated with Syndromes

Medullary cystic disease. Sectioned
• Turner gross specimen shows cysts in the
• Down’s medulla and corticomedullary
• Orofaciodigital junction
• Jeune, short rib polydactyly
• TS – cortical cysts 33-50%
• VHL – multifocal cystic adenocarcinomas in 45% after 3rd decade
Figure 6-6-23
Summary
• ARPKD and MCKD are most common in perinatal period
• Classic MCDK is managed conservatively
• Renal dysplasia is caused by in utero obstruction
• VCUG indicated in MCDK to exclude contralateral VUR (solitary
functioning kidney)
• ARPKD is a spectrum of renal and hepatic disease
• ARPKD and ADPKD can both be diagnosed in infants as well as
adolescents/adults
References 12 yo girl with medullary cystic

disease
Texts
1. Kuhn JP, Slovis TL, Haller JO, eds. Caffey's Pediatric Diagnostic Imaging, 10th Ed. Philadelphia: Mosby, 2004.
2. Hartman DS. Renal cystic disease. AFIP Atlas of Radiologic-Pathologic Correlation. Fascicle I. Philadelphia: WB
Saunders; 1989:1-5.
3. Siegel MJ, ed. Pediatric Sonography, 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 2002.
4. Stocker T, Dehner L, ed. Pediatric Pathology, 2nd ed. Philadelphia: Lippincott-Williams & Wilkins, 2002.
5. Swischuk LE. Imaging of the Newborn, Infant, and Young Child, 5th ed. Philadelphia: Lippincott-Williams &
Wilkins, 2004.
Journals
1. Blane CE, Barr M, DiPietro MA, Sedman AB, Bloom DA.
2. Blickman JG, Bramson RT, Herrin JT. Autosomal recessive polycystic kidney disease: long-term sonographic
findings in patients surviving the neonatal period. AJR Am J Roentgenol. 1995 May;164(5):1247-50.
3. Corrales JG, Elder JS. Segmental multicystic kidney and ipsilateral duplication anomalies. J Urol. 1996
Apr;155(4):1398-401.
4. Diard F, Le Dosseur P, Cadier L, Calabet A, Bondonny JM. Multicystic dysplasia in the upper component of the
complete duplex kidney. Pediatr Radiol. 1984;14(5):310-3.

5. Evans WP, Sumner TE, Lorentz WB Jr, Resnick MI. Association of crossed fused renal ectopia and multicystic
kidney. J Urol. 1979 Dec;122(6):821-2.
6. Narchi H. Risk of Wilms' tumour with multicystic kidney disease: a systematic review. Arch Dis Child. 2005
Feb;90(2):147-9.
7. Oddone M, Marino C, Sergi C, Occhi M, Negri F, Kotitza Z, et al. Wilms' tumor arising in a multicystic kidney.
Pediatr Radiol. 1994;24(4):236-8.
8. Renal obstructive dysplasia: ultrasound diagnosis and therapeutic implications. Pediatr Radiol. 1991;21(4):274-7.
9. Traubici J, Daneman A. High-resolution renal sonography in children with autosomal recessive polycystic kidney
disease. AJR Am J Roentgenol. 2005 May;184(5):1630-3.

Pediatric Renal Tumors:
Infancy & Young Children
Marilyn J. Siegel, MD
Objectives
• Describe a variety of renal masses in infants and children
• Recognize the imaging features of these masses
• Understand unique clinical and pathologic features of these tumors
Clues to Assessing Renal Tumors

• Imaging:
➢ Dominant tissue composition
❖ Soft tissue
❖ Fluid (cystic)
❖ Fat
• Other clues:
➢ Patient age
➢ Pattern of metastases
Soft Tissue Masses

• < 5 years of age
➢ Nephroblastomatosis
➢ Rhabdoid tumor
➢ Clear cell sarcoma
➢ Ossifying renal tumor of infancy
➢ Mesoblastic nephroma
• > 5 years of age
➢ Renal cell cancer
➢ Lymphoma
Wilms Tumor: Epidemiology

• 85% of renal masses
• 6%-7% of all childhood cancers
• Approximately 500 cases/year
➢ 6/1,000,000 children
• Mean age at diagnosis = 3 yrs
➢ 90% < 7 yrs
• Sex (M:F) equal
Risk Factors
• Race: Afro-Americans > Caucasians > Asians
• Familial predisposition (1%)
➢ Autosomal dominant
• Aniridia
• Deletion of tumor suppressor genes on short arm of chromosome 11
➢ 11p13 locus (WT1 gene)
➢ 11p15 locus (WT2 gene)
Risk Factors: Congenital Syndromes

• WAGR syndrome (Wilms tumor, aniridia, genital abnormalities, retardation)
(WT1 gene)
• Beckwith-Wiedemann syndrome & hemihypertrophy (WT2 gene)
• Drash syndrome (nephritis & male pseudohermaphrodism) WT 1 gene
• Trisomy 18
Renal Tumors 1388

Wilms Tumor: Gross Features [Figure 6-7-1] Figure 6-7-1
• Large cortical mass
➢ (400 to 500 gm)
• Hemorrhage & necrosis (90%)
• Pseudocapsule
• Spares collecting system
• Calcification & fat <10%
Favorable Histology (85%) [Figure 6-7-2]

• Triphasic composition
➢ Metanephric blastema
➢ Immature stroma
➢ Tubular elements
• Good prognosis if triphasic
“Unfavorable” Histology (15%) [Figure 6-7-3] Wilms tumor, gross pathology

• Anaplastic changes
➢ Nuclear enlargement
❖ > 3x size
Figure 6-7-2
➢ Hyperchromatic nuclei
➢ Atypical mitotic figures
• Implies poor prognosis & resistance to conventional therapy
Figure 6-7-3
Wilms tumor. Classic

triphasic histology
showing immature
blastema,
tubules/glomeruli, and
stroma
Wilms tumor. Atypical histology showing anaplastic nuclear
changes
Wilms’ Tumor: Clinical Features

• Mean age: 3-36 mos (range, 6 mos-4 yr)
• Symptoms
➢ Mass: 90%
➢ Pain, fever, hematuria: 30%
➢ Hypertension: 75% - 90%
➢ Aniridia, hemihypertrophy
➢ Budd-Chiari syndrome
Wilms’ Tumor: US Findings

• Well-defined margins
• Solid, intrarenal mass
• Tumor matrix
➢ Homogeneous: 50%
➢ Heterogeneous : 50%
• IVC thrombus (5%-10%)
• Relatively avascular

1391 Renal Tumors
Wilms’ Tumor Figure 6-7-4
Wilms’ Tumor [Figure 6-7-4]
Wilms tumor, ultrasound. Two gray-scale sonograms showing

well-defined, echogenic mass (M) in lower pole of right
Wilms’ Tumor: Caval Thrombus US kidney. Doppler sonogram (right image) showing flow in
surrounding parenchyma. Tumor is avascular
Wilms’ Tumor
Sensitivity (%) Accuracy (%)
US 100 25
CT 100 > 95
• US performed to confirm presence of a mass and its location

• CT to determine tumor extent Figure 6-7-5
Wilms’ Tumor
• Contrast enhanced CT
➢ low density, intrarenal mass
➢ rim of compressed parenchyma
• Little enhancement
• Central necrosis 75%
• May contain fat or calcifications
Wilms Tumor
• Exophytic mass, pseudocapsule Wilms tumor, CT. Transverse and coronal CT
reformation showing large mass extending
Wilms’ Tumor exophytically from lower pole of right kidney
Wilms Tumor [Figure 6-7-5]
Calcified Wilms Tumor (< 5%) [Figure 6-7-6] Figure 6-7-6

Role of CT
• To confirm presence of tumor and assess tumor extent
• Important diagnostic questions:
➢ Tumor thrombus (5%-10%)
➢ Contralateral tumor (5%-10%)
➢ Hepatic or lung metastases
Wilms Tumor: Venous Extension

• Renal or caval extension: 5-10% of cases
• Often clinically silent
• May prolapse through tricuspid valve Calcified Wilms tumor
• Tumor thrombus in heart or hepatic segment of IVC alters surgical approach
➢ Cardiopulmonary bypass
Renal Tumors 1390

Wilms Tumor with IVC Extension [Figure 6-7-7] Figure 6-7-7
Tumor Thrombus
Tumor Thrombus - Bilateral Wilms’ Tumors

[Figure 6-7-8]
Bilateral Wilms’ Tumor

• 5%-10% of patients at diagnosis (synchronous
disease)
• Younger mean age: 27 mos
• Dominant renal mass & small contralateral tumor or
bilateral large masses
Wilms tumor with inferior vena caval extension of
Wilms’ Tumor: Bilateral Tumors tumor
Wilms’ Tumor
Figure 6-7-8
Wilms’ Tumor: Metastases
• About 10% have metastases at
diagnosis
➢ Lung: 85%-90% of all mets
➢ Liver: 10%-15%
• Plain radiographs have FN rate of 7-
29% when CT positive
• CT is study of choice for distant
staging
Bilateral Wilms tumor with caval invasion (arrow)
Wilms’ Tumor [Figure 6-7-9]
Wilms’ Tumor: Magnetic Resonance Imaging

• Dark on T1-weighted images Figure 6-7-9
• Bright on fat-suppressed images
• Contrast enhances
• Tumor thrombus seen as a luminal
defect
Wilms Tumor: MRI
Wilms Tumor Staging: NWTS-5

• I. Limited to kidney, completely excised
• II. Extracapsular extension, but
completely removed
• III. Residual tumor confined to
abdomen
• IV. Hematogenous mets
• V. Synchronous bilateral tumors
Wilms Tumor: Treatment

• En bloc resection of affected kidney
• Excisional biopsy of nodes and lung Wilms tumor, lung metastases
nodules
• Postop chemotherapy
• Preoperative chemotherapy for invasive disease or bilateral Wilms, then
surgery

1393 Renal Tumors
4-Year Relapse Free Survival
• Stage I: 91%
• Stage II: 88%
• Stage III: 79%
• Stage IV: 78%
• Stage V: 70%
Screening High Risk Patients (BWS, WAGR syndromes)

• US every 3 months until age 6 or 7
• Hypothesis is that tumor will be detected at a lower stage
• Risk of developing Wilms tumor increases if nephromegaly at birth
Figure 6-7-10
Other Soft Tissue Tumors - Nephroblastomatosis
• Defined as "the presence of nephrogenic rests or nephrogenic
blastema beyond 36 weeks gestation”
• Important because it is a precursor to Wilms tumor
Nephrogenic Rests: Location

• 2 types by location
➢ Perilobar
➢ Peripheral cortex or columns of Bertin
• Intralobar
➢ Deep cortex
➢ Greater risk of Wilms tumor
Nephrogenic Rests [Figure 6-7-10]
Nephroblastomatosis: Imaging
• Findings vary with “burden”
• Small lesions may be inapparent
• Larger lesions
➢ Multifocal cortical nodules & masses
➢ Nephromegaly with confluent solid peripheral rind Nephrogenic rests. Pathology. Small
cortical rests in peripheral cortex
Nephroblastomatosis: Imaging (arrowheads) and larger Wilms
• US: Hypo-, iso-, or hyperechoic masses or diffuse renal tumor in deep cortex
enlargement
• CT: Poorly enhancing low attenuation confluent subcortical rind or peripheral
nodules
• MRI: Low signal on T1, iso- or slightly increased signal on T2
Diffuse Nephroblastomatosis [Figure 6-7-11] Figure 6-7-11
Diffuse Nephroblastomatosis
• Clue is peripheral tissue rind
Diffuse Nephroblastomatosis
Diffuse nephroblastomatosis. Confluent peripheral soft tissue

tumor rind, which may involve both cortex and medulla or
the subcapsular space
Renal Tumors 1392

Nephroblastomatosis:Cortical Nodules [Figure 6-7-12] Figure 6-7-12
Focal nephroblastomatosis. Longitudinal sonogram and contrast-enhanced CT and MR

showing small peripheral nodules. The peripheral distribution is the clue to the diagnosis
Nephroblastomatosis: Cortical Nodule

Figure 6-7-13
Nephroblastomatosis: Treatment
• Controversial
• Chemotherapy in some centers
• Close imaging surveillance for enlarging masses in others
• Renal sparing surgery
Rhabdoid Tumor of Kidney

• 2% of childhood renal neoplasms
• Arises from renal medulla
• Mean age 16 months (90% cases < 3yrs)
• Synchronous CNS lesions (10%)
➢ Metastases
➢ Primary neuroectodermal tumor, typically posterior fossa
Rhabdoid Tumor: Pathology [Figure 6-7-13]

• Pathology
➢ Large mass (<300 gm)
➢ Infiltrating
• Histology
➢ Mononuclear cells
➢ Eccentric nuclei and eosinophilic cytoplasm
Rhabdoid Tumor of Kidney: Radiologic features Rhabdoid tumor. Gross pathology

• Heterogeneous soft tissue mass showing large intrarenal mass
• Calcification: 66% arising in medulla and infiltrating
• Peripheral low density crescent: 70% parenchyma. Histology,
➢ Characteristic but non-diagnostic mononuclear cells with eccentric
❖ Mesoblastic nephroma nuclei
Rhabdoid Tumor
• Clue: peripheral low density collection
Figure 6-7-14
Bilateral Rhabdoid Tumors
[Figure 6-7-14]
Rhabdoid tumor. Two CT scans showing a

large necrotic mass in the left kidney
with a peripheral low density crescent
(arrow). Also noted is a solid mass in
the right kidney and multiple hepatic
metastases

1395 Renal Tumors
Rhabdoid Tumor with PNET
Figure 6-7-15
Rhabdoid Tumor: Outcome
• Poor prognosis
• Highly aggressive tumor
➢ mets 80% at diagnosis
➢ lung, liver, brain, nodes
• 20% survival @ 18 months
Clear Cell Sarcoma (Bone metastasizing renal tumor of

childhood)
• 4% of pediatric renal neoplasms
• Peak incidence 2nd year of life
• Arises from medulla
• Aggressive tumor
➢ Mets to bone (40%-75%), lung, nodes
Clear cell sarcoma.
Clear Cell Sarcoma: Pathology [Figure 6-7-15] Solid mass with
• Pathology infiltrating margins
➢ Solid, white surface
➢ Infiltrating margins
• Histology Figure 6-7-16
➢ Clear cytoplasm
➢ Vascular stroma
Clear Cell Sarcoma: Imaging Features [Figure 6-7-16]

• Heterogeneous intrarenal mass
• Cystic changes (70%)
➢ dilated tubules
➢ mucoid substance
• Indistinct margins
• Bone metastases
Clear Cell Sarcoma
Clear Cell Sarcoma Clear cell sarcoma. Large infiltrating

• Cystic Change mass in the left kidney with cystic
areas
Clear Cell Sarcoma: Outcome
• Treatment is nephrectomy & chemotherapy
• Survival rates 60%-70% Figure 6-7-17
Mesoblastic Nephroma
• 5% of all renal tumors
• Diagnosis in neonatal period
➢ mean age, 2 months
• Incidental mass
• May be diagnosed in utero
• Other findings
➢ hypertension (renin)
➢ hypercalcemia (parathormone)
Mesoblastic Nephroma: Path [Figure 6-7-17]

• Firm, rubbery, yellow-gray
• Unencapsulated Mesoblastic nephroma, gross
• Hemorrhage & necrosis uncommon pathology. Solid rubbery mass
with minimal hemorrhage. The
tumor replaces most of the kidney
Renal Tumors 1396 Pediatric Radiology

Mesoblastic Nephroma: Histology Figure 6-7-18
• Classic pattern: mature spindle cells
• Cellular pattern: increased mitoses,
potentially more aggressive
• May entrap glomeruli & tubules
Mesoblastic Nephroma: Imaging

• Homogeneous mass
• Cystic changes rare
Mesoblastic Nephroma
• Mimics Wilms tumor
• Clue: patient age
Mesoblastic Nephroma [Figure 6-7-18]
Mesoblastic Nephroma: Therapy

and Prognosis
Mesoblastic nephroma. Longitudinal sonogram and CT
• Most behave in benign fashion
showing soft tissue mass replacing renal parenchyma.
• Cured by nephrectomy
Histologic specimen showing mature spindle cells. Gross
• Metastases & recurrence very rare
specimen showing classic solid tumor without necrosis or
➢ Associated with atypical cellular
hemorrhage
histology
• Overall prognosis excellent
Figure 6-7-19
Ossifying Renal Tumor of Infancy
• Very rare!! < 20 cases reported
• Age range 6 days-14 months
• Most patients < 4 months
• Small, 2-3 cm diameter
• Typically present with hematuria
Cancer 1980; 45: 609-612
Ossifying Renal Tumor [Figure 6-7-19]

• Arises in medulla
• Ill-defined margins
• Extends into collecting system
• Obstructs collecting system
Ossifying renal tumor, gross
pathology. Medullary mass
Ossifying Renal Tumor of Infancy involving collecting system with
• Plump, ovoid spindle cells with associated osteoid & bone associated hydronephrosis
production
Ossifying Renal Tumor of Infancy: Imaging Features

• Central solid mass causing hydronephrosis
• Calcification (ossification) 80%
➢ May mimic a staghorn calculus
Figure 6-7-20
Ossifying Renal Tumor of Infancy

[Figure 6-7-20]
Ossifying renal tumor of infancy. Non-

contrast scan (left panel) showing
soft tissue mass with calcification.
Enhanced CT scan (right panel)
showing a central tumor (T) with
associated hydronephrosis

1397 Renal Tumors
Ossifying Renal Tumor of Infancy:Treatment & Prognosis
• Managed surgically
• Apparently benign
• No reported cases of malignant spread or recurrence to date
Cystic Masses
➢ Multicystic dysplastic kidney Figure 6-7-21
• > 5 years of age
➢ Simple renal cysts (rare)
Multilocular Cystic Renal Tumor: Clinical Findings

• Biphasic age distribution
➢ Boys 3 months to 2 years
➢ Women > 40 years
• Usually asymptomatic mass
• Pain & hematuria from ureteral prolapse of cyst
Multilocular Cystic Renal Tumor [Figure 6-7-21] Multilocular cystic renal tumor. Gross
• Composed of cysts & septa pathology showing multiple fluid-
• Encapsulated filled locules with surrounding
• Mean diameter 7 to 10 cm septations
Multilocular Cystic Renal Tumor Histologic Features

• Two grossly identical but histologically distinct lesions Figure 6-7-22
➢ Cystic nephroma (CN)
➢ Cystic partially differentiated nephroblastoma (CPDN)
Cystic nephroma (CN) [Figure 6-7-22]

• Peak age, 18 months
• Cysts 2 to 5mm
• Mature renal elements in septa
➢ No blastema
Cystic Partially Differentiated Nephroblastoma

(CPDN) [Figure 6-7-23] Cystic nephroma, histology. Fibrous
• Mean age, 12 months septa with differentiated tubules
• Cysts, 2 to 3 mm in septa
• Immature elements in septa
➢ Usually blastema
Figure 6-7-23
Multilocular Cystic Renal Tumors:Imaging
• Cystic, fluid-filled mass
• Water density, signal intensity
• Variable thickness septations
• Septations enhance, but not fluid contents
Multilocular Cystic Renal Tumor (CN)

• Septa enhance
Multilocular Cystic Renal Tumor
Cystic partiallly differentiated

nephroblastoma, histology.
Immature blastemal cells in septa
Renal Tumors 1396

Multilocular Cystic Renal Tumor [Figure 6-7-24] Figure 6-7-24
Multilocular Cystic Renal Tumor:

Therapy & Prognosis
• Both cystic and partially differentiated
forms usually cured by complete
resection
• Partially differentiated tumor has
potential for aggressive behavior &
recurrence
➢ warrants follow-up imaging
Multilocular cystic renal tumor. CT and gross path (left panel)
Multilocular Cystic Renal Tumor: showing multicystic mass with septations. T1-weighted
Differential Diagnosis (middle panel) and gadolinium contrast enhanced image
• Multicystic dysplastic kidney (right panel) also showing a multicystic mass
➢ Non-functioning
➢ Entire kidney involved
➢ Conservative treatment, not surgery
Figure 6-7-25
• Usually found in neonates

[Figure 6-7-25]
Multicystic Dysplastic Kidney -CT
Fat-containing
Masses:Angiomyolipoma
• Any age, but more often after 5 years
• Associated with tuberous sclerosis
• 80% of tuberous sclerosis patients have
angiomyolipomas Multicystic dysplastic kidney. Gross path and longitudinal
• Imaging: sonogram showing multiple cysts of variable size with no
➢ Bilateral fatty, renal masses normal renal tissue
➢ Solitary lesions, very rare
Figure 6-7-26
Angiomyolipoma: Imaging [Figure 6-7-26]
Tumors of Older Children - Renal Cell Cancer

• < 2% of all renal cell neoplasms
• Mean age, 9 years
➢ flank pain, hematuria, mass
• Imaging findings:
➢ solid renal mass
➢ average diameter, 4 cm
Renal Cell Cancer: Pathology - Classic Clear Cell

Cancer
• Non-specific mass with hemorrhage and necrosis
• Tumor cells with clear cytoplasm, arranged in nests
Angiomyolipomas, tuberous sclerosis.

Transverse sonogram showing highly
echogenic kidney. CT, multiple fatty
tissue masses. The presence of fat is
the clue to the diagnosis

1399 Renal Tumors
Renal Cell Cancer Figure 6-7-27
• Clue: small lesion, older pt age
Renal Cell Cancer [Figure 6-7-27]
Renal Cell Cancer: Outcome

• Metastases at diagnosis: 30%
➢ 75% lung
➢ other sites- lymph nodes, bone,
liver
• Prognosis Renal cell cancer, 10 year old boy. Two contrast-enhanced CT
➢ regional spread: 75% survival scans showing a solid intrarenal mass with associated
➢ vascular invasion or metastases: 0 perinephric hemorrhage
to 10% survival
Lymphoma
• Secondary involvement from direct extension or hematogenous spread
• Non-Hodgkin >> Hodgkin disease
• Occurs late in course of disease Figure 6-7-28
Lymphoma: Imaging Patterns

• Multiple or solitary nodules (80%)
• Diffuse infiltration
• Direct invasion
• Perinephric involvement
Renal Lymphoma: Nodules

[Figure 6-7-28]
Summary: Lesions you need to

know
➢ Nephroblastomatosis
➢ Rhabdoid tumor
➢ Clear cell sarcoma
➢ Ossifying renal tumor of infancy
➢ Mesoblastic nephroma Lymphoma, multiple appearances. Multiple nodules, top left
• > 5 years of age image. Solitary nodule, lower left image. Perinephric
➢ Renal cell cancer tumor, top right image. Diffuse infiltration, bottom right
➢ Lymphoma image.
References
1. Siegel MJ. Urinary Tract. In: Siegel MJ, ed. Pediatric Sonography, 3rd ed. Lippincott Williams & Wilkins.
Philadelphia. 2002; 385-473.
2. Siegel MJ. The Kidney. In: Siegel MJ, ed. Pediatric Body CT. Philadelphia, Lippincott Williams & Wilkins,
1999; 226-252.
3. Siegel MJ. MRI of the pediatric abdomen. MRI Clin North Am 1995; 3:161-182.
4. Geller E, Smergel E, Lowry P. Renal neoplasms of childhood. Rad Clin North Am 1997; 35:1391-1413.
5. Green DM, Coppes MJ, Breslow NE, et al. Wilms tumor. In: Pizzo PA, Poplack DG, (eds). Principles and Practice
of Pediatric Oncology, 3rd ed. New York. Lippincott-Raven. 1997; 733-759.
6. Lowe LH, Isuani BH, Heller RM, et al. Pediatric renal masses: Wilms tumor and beyond. RadioGraphics 2000;
20:1585-1603.
7. Navoy JE, Royal SA, Vaid YN, Mroczek-Musulman EC. Wilms’ tumor: unusual manifestations. Pediatr Radiol
1995; 25:S76-S
8. DeBaun MR, Siegel MJ, Choyke PL. Nephromegaly in infancy and early childhood: a risk factor for Wilms
tumor in Beckwith-Wiedemann syndrome. J Pediatr 1998; 132:401-404.
Renal Tumors 1398

9. Lonergan GJ, Martinez-Leon MI, Agrons GA, Montemarano H, Suarez ES. Nephrogenic rest,
nephroblastomatosis, and associated lesions of the kidney. Radiographics 1998; 18:947-968.
10. Rohrschneider WK, Weirich A, Rieden K, Darge K, Troger J, Graf N. US, CT, and MR imaging characteristics of
nephroblastomatosis. Pediatr Radiol 1998; 28:435-443
11. Agrons GA, Kingsman KD, Wagner BJ, Sotelo-Avila C. Rhabdoid tumor of the kidney in children: a comparative
study of 21 cases. AJR 1997; 168:447-45
12. Chung CJ, Lorenzo R, Rayder S, Schemankewitz E, Guy CD, Cutting J, Munden M. Rhabdoid tumors of the
kidney in children: CT Findings. AJR 1995; 164:697-700.
13. Kabala JE, Shield J, Duncan A. Renal cell carcinoma in childhood. Pediatr Radiol 1992; 22:203-205.
14. Davidson AJ, Choyke PL, Hartman DS, Davis CJ, Jr. Renal medullary carcinoma associated with sickle cell trait:
radiology findings. Radiology 1995; 195:83-85
15. Chepuri NB, Strouse PJ, Yanik GA. CT of renal lymphoma in children. AJR 2003; 180:419-431.
16. Hugosson C, Mahr MA, Sabbah R. Primary unilateral renal lymphoblastic lymphoma. Pediatr Radiol 1997;
27:23-25
17. Wooten SL, Rowen SJ, Griscom NT. Congenital mesoblastic nephroma. RadioGraphics 1991; 11:719-721
18. Agrons GA, Wagner BJ, Davidson AJ, Suarez ES. Multilocular cystic renal tumor in children: radiologic-
pathologic correlation. RadioGraphics 1995; 16:653-669.
19. Hopkins JK, Giles HW, Wyatt-Ashmead J, Bigler SA. Cystic nephroma. Radiographics 2004; 24:589-593

1401 Renal Tumors
Pediatric Adrenal Masses
Objectives
• Discuss differential diagnoses of adrenal masses in neonates and children
• Describe imaging features of common adrenal masses in a pediatric
population
• Describe pitfalls in diagnosis
Adrenal Masses of Childhood: Differential Considerations

• Neoplastic
➢ Medullary tumors
➢ Adrenocortical neoplasms
➢ Metastases
• Non-neoplastic
➢ Hemorrhage
Figure 6-8-1
➢ Congenital hyperplasia
➢ Storage disorders
Adrenal Medullary Tumors

• Neoplastic tumors
➢ Neuroblastic tumors
❖ Neuroblastoma
❖ Ganglioneuroblastoma
❖ Ganglioneuroma
➢ Pheochromocytoma Spectrum of neuroblastic tumors, histology.
Neuroblastoma (NB) (left panel), characterized
Neuroblastic Tumors: Histogenesis by immature small blue cells.
• Arise from neural crest tissue Ganglioneuroblastoma (GNB) (middle panel)
• Involve adrenal medulla or anywhere in sympathetic containing neuroblasts and mature cells
chain (gangliocytes). Ganglioneuroma (GN) (right
• Spectrum of differentiation and biologic behavior panel) composed of mature gangliocytes and
mature stroma
Neuroblastic Tumors: Spectrum of
Histology
• Neuroblastoma (NB): immature cells
➢ Small round blue cells
• Ganglioneuroblastoma (GNB): both neuroblasts & mature cells (gangliocytes)
• Ganglioneuroma (GN): mature gangliocytes & mature stroma
Neuroblastic Tumors: Histology [Figure 6-8-1]
Neuroblastic Tumors: Spectrum of Catecholamine Production

• Less mature tumors more active than mature tumors
➢ 90% of all tumors produce catecholamines
• Vanillylmandelic acid (VMA) - metabolite of epinephrine & norepinephrine
• Homovanillic acid (HVA) - metabolite of dopamine
• Vasoactive intestinal peptide (VIP) - elaborated by ganglion cells
Neuroblastoma: Epidemiology
• Most common extracranial solid neoplasm of childhood
• 2nd most common abdominal malignancy (after Wilms tumor)
• 10% of pediatric cancers
• 500-525 new cases/yr in the US
• Mean age ~ 2 yrs.
• 75% < 5 yrs.
Adrenal Masses 1400

Neuroblastoma: Symptoms Figure 6-8-2
• Symptoms are age dependent
• Infants < 6 mos:
➢ Skin nodules
❖ blueberry muffin syndrome
➢ Hepatomegaly (liver mets)
❖ Pepper syndrome
➢ Respiratory compromise
❖ from massive liver
➢ Abdominal mass is small & may not be palpable
Blueberry Muffin Syndrome
Neuroblastoma: Symptoms
• Infant > 6 months
• Palpable abdominal mass
• 75% have systemic symptoms
➢ bone pain
➢ hepatomegaly
➢ paraplegia
➢ opsoclonus, ataxia
➢ diarrhea (VIP production)
Distribution of neuroblastoma
Neuroblastoma: Location [Figure 6-8-2]
• Abdomen: 75%
• Thorax: 20% Figure 6-8-3
• Neck: 1%-5%
• Pelvis: 2%-3%
• Unknown primary: 1%
➢ Present with mets
Neuroblastoma: Pathology [Figure 6-8-3]

• Mean size 6-8 cm
• Hemorrhage and necrosis common
• Path-deep red to gray-white to tan appearance
Neuroblastoma: Histology
• Small blue cell tumors with occasional cluster of cells arranged in
rosettes
Neuroblastoma: Genetic Associations

• N-myc oncogene
➢ Located on distal end of chromosome 2p
Neuroblastoma, gross pathology.
➢ Multiple copies (n-myc amplification) associated with
Large solid extrarenal mass, not
aggressive tumor behavior
encapsulated
• Deletion of short arm of chromosome p1
➢ More aggressive tumor behavior
Role of Imaging
• Identification of primary tumor
• Determination of extent of local disease
• Detection of distant metastases
Abdominal Neuroblastoma: Spectrum of Imaging Features
Neuroblastoma: Approach to Diagnosis

• Screening abdominal US (if palpable mass)
• Chest X-ray
• CT &/or MRI following abnormal US or X-ray

1403 Adrenal Masses
• MIBG Figure 6-8-4
• Bone marrow aspirate/biopsy
Neuroblastoma: Imaging Spectrum

• Typical findings
➢ Infants (> 6 mos) & children
➢ Predominantly solid mass
• Atypical findings
➢ Neonates
➢ Predominantly cystic
Neuroblastoma: Typical US Findings

• Extrarenal mass
• Irregular margins
• Heterogeneous 90%
• Homogeneous 10%
• Mixed pattern reflects high cellularity, dystrophic calcification and
necrosis
Neuroblastoma: US [Figure 6-8-4]

• Typical appearance infants & children
• Predominantly solid mass
Neuroblastoma: Typical CT Findings Neuroblastoma. Typical appearance

• Extrarenal mass in infants and children. Solid
• Smooth or irregular margins extrarenal mass with small cystic
• Density less than adjacent tissues areas and/or calcification
• No definable capsule
• Midline extension
Figure 6-8-5
• Calcifications 85%

• 25% of cases ipsilateral
• No midline extension

• 75% of cases-midline extension
Neuroblastoma: Atypical Findings

• Neonates
• Predominantly cystic tumor Neuroblastoma. Two CT images showing a well-defined, right
➢ degenerative change or microcysts adrenal mass containing calcification and displacing the
• Appearance non-specific, mimics kidney inferiorly. The mass is localized to the right
hematoma abdomen and does not cross the midline
• Diagnosis requires evidence of
metastatic disease, + VMA analysis or
serial US
Figure 6-8-6
Neuroblastoma. Three CT scans showing a large left adrenal

mass with irregular margins and areas of necrosis. The
tumor crosses the midline, encases vessels, and displaces
the left kidney laterally
Adrenal Masses 1402

Neonatal Neuroblastoma [Figure 6-8-7] Figure 6-8-7
Neuroblastoma: MRI
• T1-weighted images:
➢ low signal intensity (black)
• Fat-suppressed images:
➢ high signal intensity (bright)
• Contrast enhances
Neuroblastoma: MRI [Figure 6-8-8]
Local Extension: Diagnostic

Questions
• Midline extension (> 30%) (see Fig. 6)
• Vascular encasement (>30%)
• Liver metastases (5-10%)
• Intraspinal extension (15%)
Neonatal neuroblastoma. Predominantly cystic mass arising in
Midline Extension: Vascular the right adrenal gland, reflecting necrosis, hemorrhage or
Encasement [Figure 6-8-9] intrinsic cystic changes
Spinal Invasion [Figure 6-8-10]
Cervicothoracic Neuroblastoma: Imaging Features

Figure 6-8-10
Figure 6-8-8
Neuroblastoma. T1-weighted axial image (left panel) showing a low

signal intensity mass with high signal intensity areas representing
hemorrhage. T2-weighted image (middle panel) showing a high
signal intensity mass. Coronal gadolinium-enhanced image (left
panel) showing an enhancing suprarenal mass
Figure 6-8-9
Neuroblastoma. Transverse CT
scan (top panel) and sagittal
STIR (bottom panel) images
showing intraspinal tumor
extension (arrows)
Neuroblastoma. T2-weighted axial (left panel) and coronal

STIR images (right panel) showing large left adrenal mass,
which crosses the midline and encases the aorta, renal
vessels and superior mesenteric artery

1405 Adrenal Masses
Thoracic Neuroblastoma [Figure 6-8-11] Figure 6-8-11
Thoracic Neuroblastoma
• Posterior mediastinum
• Fusiform shape
• Soft tissue density
• Calcifications (50%)
• Extends over several interspaces
• Osseous erosions (rib/vertebra)
Thoracic Neuroblastoma [Figure 6-8-12]
Thoracic neuroblastoma. Posterior mediastinal

Figure 6-8-12
mass
Figure 6-8-13
Pelvic neuroblastoma. Transverse and sagittal CT

scans show a large presacral pelvic mass (M)
which invades the spinal canal (arrows)
Thoracic neuroblastoma. Transverse, coronal and

sagittal CT images showing a right paraspinal
mass with a fusiform shape extending over
several vertebral body levels
Thoracic Neuroblastoma: MRI - Intraspinal Extension
Pelvic Neuroblastoma [Figure 6-8-13]
Neuroblastoma Metastases: Age Dependent Pattern

• < 6 months
➢ Liver (usually diffuse)
➢ Skin Figure 6-8-14
➢ Bone marrow
• > 6 months
➢ Cortical bone & bone marrow
➢ Liver (solitary or diffuse)
➢ Lymph nodes
Hepatic Metastases (5%-10%)

[Figure 6-8-14]
• Infant with diffuse liver mets
• Pepper syndrome
Neuroblastoma. Transverse hepatic sonogram showing diffuse

parenchymal heterogeneity. Transverse CT showing
diffuse hepatic metastases and a small right primary tumor
(M).
Adrenal Masses 1404

Hepatic Metastases
Distant Metastases: Skeleton Imaging Studies (sensitivity)

• Bone scintigraphy: 90%
• Metaiodobenzylguanidine (MIBG)
➢ I-123 MIBG: 95%
• In-111 pentetreotide
• Total body MRI: > 95% Figure 6-8-15
• FDG PET: 100%
• X-ray: 35%-70%
Sites for Skeletal Metastases

• Metaphyses of long bones
• Calvarium-dura
• Ribs & vertebral bodies
• Flat bones
• I.e., the sites of red marrow
Neuroblastoma, bone metastases. Plain
radiographs (left panel) showing lytic
Neuroblastoma: Radionuclide Imaging metaphyseal lesions. Bone scintigraphy (right
Sensitivity panel) showing increased radionuclide activity
• Primary tumor 35-90% in metaphyseal ends of the long bones
• Skeletal metastases
➢ Radionuclide 90%
➢ X-ray 35-70%
❖ lytic or permeative
• Metastases are usually asymmetric and metaphyseal in location
Skeletal Metastases: Bone Scintigraphy [Figure 6-8-15]
Split Suture Sign - Dural Metastases
MIBG Scintigraphy [Figure 6-8-16]

• Norepinephrine analogue Figure 6-8-16
• I-123 MIBG
• Sens: 80% to 95%
• Sens >> bone scan
Skeletal Metastases
• MRI
• FDG-PET
Staging Neuroblastoma. MIBG scintigraphy. Increased activity in right

• Stage 1: adrenal mass and in skeleton
➢ Localized tumor confined to area of
origin; complete excision
• Stage 2A:
➢ Unilateral tumor with incomplete excision; ipsilateral nodes negative
microscopically.
• Stage 2B:
➢ Unilateral tumor complete or incomplete excision; positive ipsilateral nodes
• Stage 3:
➢ Tumor across midline; or unilateral tumor with contralateral node
involvement; or midline tumor with bilateral lymph node involvement
• Stage 4:
➢ Spread to distant lymph nodes, bone, bone marrow, liver, or other organs
• Stage 4S:
➢ Unilateral primary tumor with spread limited to liver, skin and/or bone
marrow.
➢ Limited to infants < 1 year of age.

1407 Adrenal Masses
Neuroblastoma: Stage Distribution [Figure 6-8-17] Figure 6-8-17
Neuroblastoma: Treatment
• Resectable disease: surgery
• Unresectable disease:
➢ chemotherapy
• Radiation therapy for tumor that does not regress with
chemotherapy
Neuroblastoma: Overall Survival

• Stage 1: 94%
• Stage 2: 90%
• Stage 3: 64%
• Stage 4: 24%
• Stage 4S: 75% Distribution of stages of neuroblastoma.
Majority of patients have advanced
disease at time of diagnosis
Favorable Outcome: Associated Factors
• Low stage
• Young patient age (< 1 yr)
• No N-myc amplification
• No chromosome 1p
• Stroma-rich
Neuroblastoma: Outcome- 3-year survival

• Stage & age of patient at diagnosis most important predictors of outcome
• Stages 1, 2, & 4S: 75%-90%
• Children < 1 year Figure 6-8-18
➢ Stage 3 disease: 80%-90%
➢ Stage 4 disease: 60%-75%
• Children > 1 year
➢ Stage 3: 50%
➢ Stage 4: 15%
• Other neuroblastic tumors
➢ Ganglioneuroma Other neuroblastic tumors. Ganglioneuroblastoma (left panel).
• Pheochromocytoma Ganglioneuroma (right panel). The appearance overlaps
• Adrenocortical tumors that of neuroblastoma
• Metastases
Other Neuroblastic Tumors [Figure 6-8-18]

• Ganglioneuroblastoma
➢ Neuroblasts & ganglion cells
• Ganglioneuroma
➢ Mature ganglion cells
Neurofibroma
Pheochromocytoma
• Mean age: 11 yr, (range 6-18 yr)
• 10% malignant
• Bilateral 20%: associated with:
➢ MEN-IIA & IIB
➢ von Hippel-Lindau syndrome
➢ von Recklinghausen syndrome (NF1)
• Clinical
➢ Paroxysmal hypertension, headaches, diaphoresis
Adrenal Masses 1406

Pheochromocytoma: Locations Figure 6-8-19
• 97% abdominopelvic
➢ Adrenal (90%)
➢ Paraganglioma (10%)
❖ Organ of Zuckerkandl
❖ Retroperitoneum
❖ Pelvis
• Thorax (rare site)
Pheochromocytoma: Gross Path [Figure 6-8-19]

• Rounded solid mass
• 3-5 cm
• Hemorrhage &necrosis common
• Rarely purely cystic
Pheochromocytoma: Imaging
• Cystic changes, hemorrhage common
• US: Solid circumscribed mass with variable heterogeneity
• CT: Heterogenous mass (when large), enhances
• MR imaging: high signal T2-WT image
• MIBG avid
Pheochromocytoma [Figure 6-8-20] Pheochromoctyoma. Gross

pathology showing a round
Adrenocortical Tumors cystic mass with hemorrhagic
• Rare in children contents
• Adrenal carcinoma most common
• Mean patient age: 9 years Figure 6-8-20
• Hormonally active producing virilization,
less often Cushing syndrome
• Adenomas rare
Adrenocortical Cancer: Path &

Imaging [Figure 6-8-21]
• Large solid mass (mean, 6 cm)
• Lobulated surface
• Cystic areas of hemorrhage and
necrosis common
• Calcification 20%
Pheochromocytoma. Longitudinal sonogram showing an
• Mets to liver, lung, bone
echogenic suprarenal mass (M). Coronal T2-weighted
image showing a high signal intensity mass (M) with
Adrenocortical Cancer [Figure 6-8-22] central necrosis
Adrenal cancer. Longitudinal sonogram (left panel) showing a

solid adrenal mass. Coronal T1-weighted image (middle
Adrenocrotical cancer. Cut section panel) showing a large, left suprarenal mass with necrosis.
shows a solid mass with areas of T2-weighted image (right panel) showing a high-signal
hemorrhage and necrosis intensity mass with areas of necrosis

1409 Adrenal Masses
Adrenal Metastases Figure 6-8-23
• Rare in children
• Lymphomatous involvement may occur
• Nonspecific uniform solid adrenal mass
Adrenal Masses of Childhood: Differential

Considerations
• Neoplastic
➢ Adrenocortical neoplasms
➢ Metastases
• Non-neoplastic—neonatal diseases
➢ Hemorrhage
➢ Storage disorders
First you need to know------US of Normal Adrenal

[Figure 6-8-23]
• Echogenic medulla
• Hypoechoic cortex
• Inverted V or Y shape
• Mean length, 15 mm
• Mean width, 3 mm
Adrenal Hemorrhage Normal adrenal gland. Upper panel:

• Most common neonatal adrenal mass V shaped adrenal gland. Lower
• Result of passive venous engorgement during delivery panel Y-shaped adrenal gland.
• Predisposing conditions: Note: hypoechoic cortex,
➢ birth trauma, hypoxia, IDM, coagulopathy, renal vein echogenic medulla
thrombosis, sepsis
• Does not cause adrenal insufficiency
• R > L (3-4:1), may be bilateral Figure 6-8-24
Adrenal Hematoma
➢ Abdominal mass
➢ Anemia
➢ Jaundice
Adrenal Hematoma: US
• Appearance varies with age of
hematoma
➢ Day1-2: echogenic or complex
mass (fibrin,debris)
➢ Day 3 to 2 wks: complex
(liquefaction)
➢ Later: cystic (hypo- or anechoic)
• Shrinkage within 1 to 2 weeks
• May calcify as early as 1 week
• Associated caval thrombus
Adrenal hematoma. Longitudinal sonograms on day 1 (left
Adrenal Hematoma upper panel), on day 12 (left lower panel), at 6 weeks
[Figures 6-8-24 and 6-8-25] (right upper panel) and at 2 months (right lower panel)
showing changes in echogenicity and near complete
resolution of a right adrenal hematoma
Adrenal Masses 1408

Differential Diagnosis: Neuroblastoma Figure 6-8-25
• Neuroblastoma does not liquefy
➢ (but may spontaneously regress)
• Neuroblastoma makes catecholamine
byproducts
• US surveillance to document resolution
of hemorrhage (may take 6 to 8 weeks)
Adrenal Hematoma
• Calcified hematomas
Adrenal hematoma. Left panel. Day 3, complex suprarenal
Congenital Adrenal Hyperplasia mass. Right panel, day 10, nearly complete involution
• Autosomal recessive enzymatic
deficiency Figure 6-8-26
• 21-hydroxylase deficiency most common (>90%)
• Androgen overproduction
➢ virilism in girls
➢ premature masculization in boys
➢ advanced somatic development in both sexes
• Aldosterone underproduction
➢ salt wasting crisis
Congenital Adrenal Hyperplasia: Pathology [Figure 6-8-26]

• Bilateral adrenal enlargement with cerebriform appearance
• Mean length > 20 mm
• Mean width > 4 mm
Congenital Adrenal Hyperplasia: Imaging

• Adrenal enlargement Congenital adrenal hyperplasia.
• Wavy contour Gross section showing large
• Cerebriform appearance bilateral adrenal glands with
cerebriform pattern
Woman Disease
• Rare inborn lysosomal acid lipase deficiency Figure 6-8-27
• Cholesterol esters accumulate in all
organs
• Presents in infancy, death in 1 year
Woman Disease: Imaging

• Marked adrenomegaly
• Preserved adreniform contour
• Punctate or coarse calcifications
• Hepatosplenomegaly
Wolman Disease [Figure 6-8-27]
Adrenal Tumor-Neonate:
Hemorrhage or Neuroblastoma?
• Enlarged adrenal gland?
➢ Both
• Cystic and/or solid?
➢ Both
• Complete involution?
➢ Hemorrhage
• Liquefies?
➢ Hemorrhage Wolman disease. Plain radiographs (upper panels) showing
enlarged, calcified adrenal glands. Longitudinal sonogram
(left lower panel) showing a suprarenal mass with marked
shadowing. CT (right lower panel) showing bilateral
calcified adrenal glands.

1411 Adrenal Masses
Adrenal Tumor-Neonate: Hyperplasia or Wolman Disease Figure 6-8-28
• Enlarged adrenal gland?
➢ Both
• Solid?
➢ Both
• Cerebriform pattern?
➢ Hyperplasia
• Calcifies?
➢ Wolman
• Hormonally active?
➢ Hyperplasia
The Spectrum: Neonate

[Figure 6-8-28]
Adrenal Tumor-Infant & Older Child:

Neuroblastoma, Cancer, Pheo
• Solid mass?
➢ All
• Cystic changes?
➢ All Neonatal adrenal lesions. Neuroblastoma (upper left panel).
• Bone mets? Wolman (lower left panel). Hemorrhage (upper right
➢ Neuroblastoma panel). Congenital adrenal hyperplasia (lower right panel)
• Virilization?
➢ Adrenal cancer
• Paroxysmal hypertension
➢ Pheochromocytoma
Spectrum of Adrenal Lesions: US - Infant & Older Child

[Figure 6-8-29]
Spectrum of Adrenal Lesions: CT - Infant & Older Child

The Need to Know Adrenal

Masses
• Neoplastic
❖ Neuroblastic
❖ Pheochromocytoma
➢ Adrenocortical cancer Adrenal lesions infant and older children, ultrasound.
• Non-neoplastic Neuroblastoma (left panel). Pheochromoctyoma (middle
➢ Hemorrhage panel). Cancer (right panel)
➢ Wolman disease
Figure 6-8-30
Adrenal lesions infant and older children, CT. Neuroblastoma

(left panel). Pheochromoctyoma (middle panel). Cancer
(right panel)
Adrenal Masses 1410

References
1. Siegel MJ. Adrenal glands, pancreas and other retroperitoneal structures. In: Siegel MJ (ed), Pediatric Sonography,
3rd. Philadelphia. Lippincott Williams & Wilkins. 2002; 475-527.
2. Siegel MJ. Adrenal glands, pancreas and other retroperitoneal structures. In: Siegel MJ (ed), Pediatric Body CT.
Philadelphia. Lippincott-Williams & Wilkins. 1999; 253-286.
3. Westra SJ, Zaninovic AC, Hall TR, Kangarloo H, Boechat MI. Imaging of the adrenal gland in children.
RadioGraphics 1994; 14:1323-1340.
4. Abramson SJ. Adrenal neoplasms in children. Radiol Clin North Am 1997; 35:1415-1453.
5. Brodeur GM, Maris JM. Neuroblastoma. In: Devita VT, Hellman S, Rosenberg SA, eds. Cancer Principles and
Practice of Oncology. Lippincott Williams & Wilkins. Philadelphia. 2001; 895-933.
6. Lonnergan GJ, Schwab CM, Suarez ES, Carlson CL. Neuroblastoma, ganglioneuroblastoma and ganglioneuroma:
radiologic-pathologic correlation. Radiographics 2002; 22:911-934.
7. Berdon W, Ruzal-Shapiro C, Abramson S. The diagnosis of abdominal neuroblastoma: Relative roles of
ultrasonography, CT and MR. Urol Radiol 1992; 14:252-262
8. Cassady C, Winter WD. Bilateral cystic neuroblastoma: imaging features and differential diagnoses. Pediatr
Radiol 1997; 27:758-759.
9. Teoh SK, Whitman GJ, Chew FS. Neonatal neuroblastoma. AJR 1997; 168:54.
10. Meyer JA, Harty MP, Khademian Z. Imaging of neuroblastoma and Wilms’ tumor. Magn Reson Imaging Clin
2002; 10:275-302.
11. Siegel MJ. MR imaging of pediatric abdominal neoplasms. MRI Clin North Am 2000; 8:837-851
12. Gelfand M J. Meta-iodobenzylguanidine in children. Semin Nucl Med 1993; 23: 231-242
13. Shulkin BL, Wieland DM, Baro ME, et al. PET hydroxyephedrine imaging of neuroblastoma. J Nuc Med 1996;
37:16-21.
14. Argons GA, Lonergan GJ, Dickey GD, Perez-Monte JE. Adrenocortical neoplasms in children: radiologic-
pathologic correlation. Radiographics1999; 19:989-1008.
15. Riberio J, Ribeiro RC, Fletcher BD. Imaging findings in pediatric adrenocortical carcinoma. Pediatr Radiol 2000;
30:45-51.
16. Sivit CJ, Hung W, Taylor GA, Catena LM, Brown-Jones C, Kushner DC. Sonography in neonatal congenital
adrenal hyperplasia. AJR 1991; 156:141–143.
17. Özmen MN, Aygün N, Kiliç I, Kuran L, Yalçin B, Besim A. Wolman’s disease: ultrasonographic and computed
tomographic findings. Pediatr Radiol 1992; 22:541–542.

1413 Adrenal Masses
Pediatric Pelvic Masses
Objectives Figure 6-9-1

• Review normal anatomy
• Discuss causes of pelvic masses
• Describe imaging features of common and some uncommon
pelvic lesions
“To recognize the abnormal, one must first know the
normal”
J. Caffey
Normal Maturation: Growth

• Ovarian volume
• Uterine volume
Age vs. Ovarian Volume

• Neonate (1day-3 mos) 1.1 cm3
• Infant (4-12 mos) 0.7 cm3
• Early childhood (2-8 yrs) 0.8-1.1 cm3
• Late childhood (9-14 yrs) 2.2-4.2 cm3
• Pubertal 9.8 cm3
Ovarian Maturation: Morphology

• Prepubertal ovary
➢ Usually homogeneous Normal prepubertal ovary. Two 2-
➢ But may be heterogeneous due to presence of primordial year-old girls. Left panel: The ovary
follicles (< 1cm) is relatively homogeneous. Right
• Pubertal ovary panel: Longitudinal sonogram
➢ Usually heterogeneous showing multiple small follicles,
➢ Reflects presence of primordial & functional follicles (1- 3cm) measuring less than 9 mm in
diameter. B = bladder
Ovarian Morphology: Need to Know
• Presence of cysts is normal in infants & children
• Do not confuse for pathology
Figure 6-9-2
Normal Maturation
• 10 follicles stimulated each cycle
• 1 becomes dominant
• Grows to 15 to 30 mm
Normal Prepubertal Ovary: US

[Figure 6-9-1]
Normal Pubertal Ovary: US

[Figure 6-9-2]
Normal pubertal ovaries. Left panel: Longitudinal sonogram on

day 10 of the menstrual cycle shows multiple follicles less than
10 mm in diameter (calibers=right ovary). Right panel:
Longitudinal sonogram on day 20 of the menstrual cycle shows
a dominant follicle (arrow), measuring 18 mm in length. This
likely represents a corpus luteum cyst
Pediatric Pelvis Masses 1412

Normal Ovary: CT and MR [Figure 6-9-3] Figure 6-9-3
Uterine Morphology: Maturation

• Prepubertal uterus
➢ tubular shape
➢ fundus equal to cervix
➢ endometrial canal seen in neonates,
not in infants and children
• Pubertal uterus
➢ fundus elongates and thickens Normal ovaries. Left panel: CT scan of a 6 year old girl. The
➢ fundus larger than cervix ovaries are not well seen. Middle panel: CT scan of a 13-year-
➢ endometrial canal again present old girl shows homogeneous right ovary (arrow) and a small
developing follicle, measuring 2.5 cm diameter, in the left ovary.
Age vs. Uterine AP Diameter Right panel. Fat-saturated T2 weighted MR of an adolescent
• Neonate (1day-3 mos) 2.1 cm girl showing multiple high signal intensity follicles in both
• Infant (4-12 mos) 0.8 cm ovaries (arrowheads)
• Early childhood (2-8 yrs) 0.7 cm
• Late childhood (9-14 yrs) 1.4 cm Figure 6-9-4
• Pubertal 1.6-3 cm
Normal Uterus: US [Figure 6-9-4]
Normal Uterus: CT and MR

[Figure 6-9-5]
Pelvic Mass Lesions Normal uterine morphology. Left panel: Neonatal uterus.
• Anterior Pelvis
Longitudinal sonogram shows a tubular uterus (arrowheads)
➢ Ovarian
with prominence of the uterine fundus and a thin, hyperechoic
➢ Bladder and lower genital tract
endometrial stripe. Middle panel: A 2-year-old girl. The uterus
• Posterior Pelvis (Presacral)
is small and tubular with no differentiation between fundus and
➢ Neurogenic tumors
cervix and no recognizable endometrial stripe. Right panel.
➢ Teratomas
Sonogram showing a pear-shaped uterus with a fundus that is
larger than the cervix. The endometrial stripe (calipers) is again
Pelvic Masses: Imaging Approach visualized and varies in thickness with the phase of the
• US is screening examination of choice
menstrual cycle
for most clinically suspected masses
• CT is study of choice for evaluation of gynecologic, bladder and prostate
lesions shown on sonography
• MRI usually reserved for presacral masses
Ovarian Masses Figure 6-9-5

• Cystic
➢ Follicular cysts
➢ Paraovarian cysts
➢ Cystic neoplasms
❖ Teratoma, cystadenoma
• Solid
➢ Malignant germ cell tumors
➢ Sex cord-stromal tumors
Ovarian Tumors: First Key Point Normal uterus. Left panel: CT scan of a 5-year-old girl. The
• Epithelial tumors are extremely rare in uterus (arrow) is seen as a small oval soft tissue structure.
the 1st two decades Middle panel: Normal pubertal uterus. CT scan of a 15-year-
➢ Put them low on the list old girl shows an oval uterine fundus that demonstrates zonal
• THINK SIMPLE CYSTS, GERM CELL differentiation-- higher attenuation myometrium and
TUMORS, OR STROMAL TUMORS endometrium and the lower attenuation endometrial canal.
Right panel: T2-weighted image from a 12-year-old girl shows
normal zonal anatomy--endometrial complex (e), junctional
zone (arrow), and outer myometrium (m)

1415 Pediatric Pelvis Masses
Follicular Cysts Figure 6-9-6
• Most common ovarian mass
• Over stimulated follicle
• Imaging findings
➢ fluid-filled lesion
➢ unilocular
➢ thin-walled
➢ avascular
➢ 3 to 8 cm
Functional Ovarian Cyst: US

[Figure 6-9-6]
Functional ovarian cysts, US. Left panel: Sonogram showing a
6-cm anechoic cyst (C) with imperceptible walls and sound
Functional Ovarian Cyst CT/MR transmission. Right panel: Color Doppler sonogram of another
[Figure 6-9-7]
patient with an ovarian cyst (C). The cystic contents show no
color signal. Minimal flow is noted in the adjacent parenchyma
Hemorrhagic Ovarian Cyst: US
• US Findings
➢ Complex mass (>85%) Figure 6-9-7
❖ Septations, fluid-debris level
➢ Hyperechoic mass (<15%) (early)
➢ Acoustic transmission
• Doppler: avascular
• Painful masses
Hemorrhagic Ovarian Cysts:

Spectrum of US Features
[Figure 6-9-8]
Hemorrhagic Cysts: CT/ MRI

[Figure 6-9-9]
Functional ovarian cysts, CT and MR. CT (left panel) showing
• CT a 6 cm low attenuation cyst (C) arising from the right ovary
➢ High density displacing the bladder (B) to the left. Fat-saturated T2-
➢ “fluid-fluid” level weighted image (right panel) showing a high signal intensity,
• MR right ovarian cyst, measuring 5 cm in diameter. The normal
➢ Mixed signal zonal anatomy of the uterus--endometrial complex, inner
➢ “fluid-fluid” level junctional zone, and outer myometrium-- is noted
Figure 6-9-8
Figure 6-9-9
Hemorrhagic ovarian cysts. Different adolescent patients. Left

panel: Early hemorrhage. A hyperechoic cyst (arrow) with
acoustic enhancement. Ut = uterus. Middle panel: Late
hemorrhage. A complex cyst (C), measuring 5 cm in diameter,
with internal echoes and septations. Right panel: A complex
mass (arrows) with fluid-debris level
Hemorrhagic ovarian cyst, CT/ MRI. Upper image: CT

showing high attenuation right ovarian cyst (arrow). Lower
image: T2-weight fat-saturated MR showing mixed signal
intensity cyst (arrows) with a blood-fluid level (arrowhead). U =
uterus

Follicular & Hemorrhagic Cysts: More Points
• Most cysts regress within 2 cycles
• If the cyst remains for > 3 cycles, likely not functional
➢ Suspect paraovarian cyst or neoplasm
• Cysts > 6 cm should be followed
Hemorrhagic Ovarian Cyst: Serial US to document resolution
DDX: Follicular Cysts

• Paraovarian cysts
• Teratoma
Figure 6-9-10
• Cystadenoma
Paraovarian Cysts
• Arise in broad ligament or fallopian tube
• Imaging:
➢ round or oval
➢ fluid-filled
• No cyclic changes
• Do not regress
• Can make diagnosis if cyst is separate
from ovary
Paraovarian cyst. Left panel: Longitudinal sonogram showing
Paraovarian Cysts (Fallopian a large cyst adjacent to a normal right ovary (arrow). BL =
Tube) bladder. Right panel: Fat-saturated T2-weighted axial MR
image showing a high signal intensity cyst separate from both
Paraovarian Cyst [Figure 6-9-10] ovaries
Cystic Neoplasms: Ovarian Teratoma

• Most common ovarian tumor
➢ 95% of ovarian neoplasms
• 90% benign
• 75% asymptomatic Figure 6-9-11
➢ 25% pain due to torsion
• Bilateral 8% to 15%
• Large, mean diameter 15 cm
• Usually adolescent girls
Mature Teratoma: Pathology

[Figure 6-9-11]
• Gross
➢ Cystic mass
➢ Foci of fat, Ca++, bone
• Histology Ovarian teratoma, pathology. Cut section showing cystic
➢ Respiratory, GI elements teratoma containing small nidus of calcification posteriorly
➢ Sebaceous glands, hair skin (arrow). Histologic section showing sebacous glands and skin
➢ Muscle, cartilage
➢ No malignant elements
Cyst Ovarian Teratoma: Imaging

• 90% predominantly cystic
➢ >>50% fluid contents
➢ Minimal soft tissue
❖ mural nodule
❖ septations
➢ Foci of fat & Ca++
• 10% contain only fat & hair contents
• Avascular

Cystic Ovarian Teratoma: US [Figure 6-9-12] Figure 6-9-12
Multiple Benign Teratomas: CT

[Figure 6-9-13]
Teratoma
15 yo girl with abdominal
distention
Ovarian Teratoma: MRI
Ovarian Cystadenoma
• Epithelial tumor Mature teratomas, US. Different patients. Left panel:
• < 5% neoplasms, benign Longitudinal sonogram showing a predominantly cystic mass
• Mucinous >> serous with a an echogenic mural nodule (dermoid plug) (arrow).
• 4 to 20 cm diameter B=bladder; UT=uterus. Right panel: Sonogram showing a
• Epithelial linings complex cystic mass containing large echogenic mural nodule
➢ Simple (serous type) anteriorly (arrow) with acoustic shadowing
➢ Columnar, mucinous (mucinous
type) Figure 6-9-13
Cystadenoma: Imaging [Figure 6-9-14]
• Usually large
• Cystic
• Multilocular with septations
• Sometimes unilocular
➢ Particularly serous type
Serous Cystadenoma
Ovarian Malignancies
• Germ cell tumors (85%)
• Stromal tumors (15%)
• Epithelial tumors
Malignant Germ Cell Tumors

• 10% of germ cell tumors
• Immature: neural (brain) tissue
• Malignant: frank malignant elements
• Predominantly soft tissue (>50%) plus fat and Mature ovarian teratomas, CT. Spectrum of
calcium appearances
Malignant Ovarian Tumors: Path [Figure 6-9-15]
Mucinous cystadenoma. Longitudinal sonogram

showing a multilocular mass. CT showing a cystic Malignant ovarian tumors, pathology. Left panel,
mass with enhancing septations dysgerminoma. Right panel, malignant teratoma.
Malignant tumors typically have lobulated surfaces
and contain predominantly solid elements with
areas of necrosis and hemorrhage

Malignant Germ Cell Neoplasms: Clinical & Imaging Figure 6-9-16
• Pelvic or abdominal masses
• Imaging findings suggestive of
malignancy:
➢ solid or complex mass
❖ > 50% soft tissue elements
➢ thick, irregular walls
• Nonspecific findings: > 10 cm size,
Ca++
• Metastasize to lymph nodes and liver Dysgerminomas. Left panel: Longitudinal sonogram showing a
➢ (rarely to omentum or mesentery) large predominantly solid mass with hypoechoic areas
representing necrosis. Middle and right panels. Different
Dysgerminoma [Figure 6-9-16] patients. Contrast-enhanced CT scans showing a large mostly
solid mass with cystic components
Malignant Teratoma Clue:
Predominantly Solid Mass Figure 6-9-17
Endodermal Sinus Tumor (Yolk Sac Cancer)
Sex Cord-Stromal Tumors (15%)

• Prepubertal girls
• Granulosa theca cell & Sertoli-Leydig
• Hormonally active
➢ Estrogens (Granulosa-Theca)
➢ Androgens (Sertoli-Leydig)
• Can be malignant or benign Sex cord stromal tumors, pathology. Left panel:
➢ Spread to peritoneum and liver granulosa theca cell tumor. Right panel: Sertoli-
Leydig tumor. Both tumors appear as large,
Sex Cord-Stromal Tumors: Pathology predominantly solid mass with varying size areas
[Figure 6-9-17] of necrosis
Sex Cord-Stromal Tumors: Imaging Figure 6-9-18

• Granulosa cell tumors
➢ mixed solid-cystic mass with thick,
irregular septations
• Sertoli-Leydig tumors
➢ homogeneous solid mass or mixed
solid-cystic mass
• Metastases, although rare, are to
peritoneal surfaces and liver
Granulosa-theca Cell Tumor

[Figure 6-9-18]
Granulosa-theca cell tumor. 5 year-old girl with breast
Sertoli-Leydig cell tumor development and vaginal bleeding. Transverse sonogram
[Figure 6-9-19]
showing a heterogenous solid right ovarian mass with cystic
areas. CT showing a low attenuation mass with irregular
septations
Figure 6-9-19
Setoli-Leydig cell tumor. Young girl with

virilization. Two transverse CT scans
showing a heterogeneous mass with
solid and cystic (necrotic) areas. The
appearance mimics malignant germ cell
tumors, but the diagnostic clue is
hormonal activity

Ovarian Cancer
• Rare lesion in pediatric population
• Epithelial origin
• Imaging
➢ Solid mass
➢ Often necrotic
➢ Smaller in size than germ cell tumors
➢ Spreads to mesentery and omentum
Ovarian Cancer (<5% of tumors)
Which Ovarian Tumor?

• Cystic with mural nodules?
➢ Teratoma
• Cystic with septations?
➢ Cystadenoma
• Solid with cystic elements?
➢ Malignant germ cell tumors, stromal tumors
• Older age (pubertal)?
➢ Germ cell tumors, Cystadenoma
• Younger age? Figure 6-9-20
➢ Stromal tumors
Uterine Masses
• Cystic
➢ hydrocolpos
• Solid
➢ rhabdomyosarcoma
Hydrocolpos
• Vaginal obstruction
➢ due to stenosis or membrane
• Result is pelvic/abdominal mass Hydrocolpos-hematocolpos, US. Left panel. Neonate.
• Imaging findings Longitudinal sonogram showing dilated fluid-filled vagina (V)
➢ Fluid-filled midline mass outlining the cervix (C). B = bladder. Right panel.
➢ Well defined walls Hematocolpos. Longitudinal sonogram showing a dilated
➢ Internal debris or blood vagina (V) with low level central echoes, representing blood.
❖ (hemato- or Uterus (U) is normal
hematometrocolpos)
Hydrocolpos—Hematocolpos [Figure 6-9-20]
Hydrocolpos: CT & MRI [Figure 6-9-21]
Rhabdomyosarcoma
• Most common pelvic malignancy
• Bimodal age distribution: Figure 6-9-21
➢ 2 to 6 and 14 to 16 years
• Embryonal cell type
• Sites:
➢ Head/Neck 38%
➢ GU 21%
➢ Extremity 18%
Hydrocolpos, CT and MR. CT showing a dilated fluid-filled

vagina (V). Axial T2-weighted MRI showing high-signal
intensity fluid in the vaginal canal (V). B = bladder

Rhabdomyosarcoma: Female Pelvis Figure 6-9-22
• Arises in vagina
• Vaginal bleeding
➢ Soft tissue mass
➢ Enlarged pelvic nodes
• Mets to liver, lung, node and bone
Vaginal Rhabdomyosarcoma [Figure 6-9-22]
Rhabdomyosarcoma: Male Bladder &

Prostate
• Bladder
➢ Trigone or bladder base
➢ Polypoid mass
• Prostate gland
➢ Solid mass Vaginal rhabdomyosarcoma. Sagittal T1-weighted
➢ Deforms base of bladder and fat-suppressed T2-weighted images showing
➢ Elongates prostatic urethra a soft tissue mass filling the vaginal canal (arrow).
(Case courtesy of Mary E. McCarville, MD,
Prostatic Rhabdomyosarcoma [Figure 6-9-23] Memphis, TN)
Rhabdomyosarcoma: Prostate
Bladder Rhabdomyosarcoma
Bladder Masses: Differential Diagnosis Figure 6-9-23

• Pheochromocytoma
• Neurofibroma
• Transitional cell
• Leiomyosarcoma
Which Lower Genital

Tract Tumor?
• YOU DON’T NEED CLUES
• Solid, infiltrative? Prostatic rhabdomyosarcoma. CT scans showing an
➢ Rhabdomyosarcoma enlarged prostate gland (P) The planes between the
mass and right obturator internus muscles are
Presacral Masses obliterated. Invasion of pelvic sidewalls confirmed at
• Cystic surgery
➢ benign teratoma
➢ meningocele
• Solid
➢ malignant teratoma
➢ neuroblastoma
Sacrococcygeal Teratoma
• CA++: 60%
• Malignancy: 10% newborn
90 > 2 months
• Location: 45 % external
45% external & internal
10% presacral
• Bony defect: very low frequency
• Arise from coccyx
• Do not invade spinal canal

Sacrococcygeal Teratoma: Imaging Figure 6-9-24

• Benign teratomas
➢ Contain predominantly fluid or fat
➢ Sometimes Ca++
• Malignant teratomas
➢ Predominantly solid
• Fed by sacrococcygeal and iliac arteries
Benign Sacrococcygeal Teratoma
Benign SC Teratoma [Figure 6-9-24]
Anterior Meningocele
• Herniation of spinal contents through a
congenital defect in a vertebral body
• Scimitar shaped sacrum Benign sacrococcygeal teratoma. T1-weighted MR (left panel)
• CT/MR showing a predominantly fat-containing mass with small fluid-
➢ absent sacral elements filled nodules. Fat-saturated T2-weighted MR (right panel).
➢ tethered cord The fatty tissue has decreased in signal intensity. Fluid
components are bright. The tumor arises from the coccyx
Anterior Meningocele (arrow)
Presacral Masses
• Cystic Figure 6-9-25
➢ benign teratoma
➢ meningocele
• Solid
➢ malignant teratoma
➢ neuroblastoma
Malignant SC Teratoma [Figure 6-9-25]
Which Presacral Tumor?

• Cystic Mass, normal spine?
➢ Teratoma
• Cystic mass, abnormal spine? Malignant sacrococcygeal teratoma. Transverse CT scan (left
➢ Anterior meningocele panel), T1-weighted MR (middle panel) and T2-weighted MR
• Solid with normal spine (right panel) showing a large presacral mass with a
➢ Malignant teratoma predominance of soft-tissue components
• Solid with spinal canal invasion
➢ Neuroblastoma
Figure 6-9-26
Presacral neuroblastoma. CT showing a

soft-tissue mass (M) anterior to the sacrum
and posterior to the bladder (BL). The
tumor has invaded the right obturator fossa
(arrow). Sagittal STIR image showing a
presacral soft-tissue mass (M) extending
into the spinal canal (arrowheads) and
displacing the bladder superiorly. F = foley
catheter

Lateral Pelvic Masses
• Adenopathy
➢ Lymphoma
• Neurofibroma
Neurofibromas
• Nerve sheath tumors
• Often plexiform in the pelvis
➢ multiple or interlacing masses
• Affect about 5%-15% of people with NF1
• Imaging
➢ Multiple soft tissue masses
➢ Target sign T2 MR
➢ Enlarged sacral foramen
Plexiform Neurofibromatosis
• Clue: enlarged sacral foramen
Neurofibromatosis: T2 MR
Lymphadenopathy
Top 10 Pelvic Lesions: What you need to know

• Functional ovarian cyst
• Ovarian teratoma
• Malignant ovarian tumors
• Sex cord stromal tumors
• Hydrocolpos
• Sacrococcygeal teratoma
• Anterior meninogocele
• Presacral neuroblastoma
• Neurofibromatosis (NF1)
References
1. Garel L, Dubois J, Grignon A, Filiatrault D, Van Vliet G. US of the pediatric female pelvis: a clinical perspective.
Radiographics 2001; 21:1393-1407.
2. Siegel MJ. Female pelvis. In: Siegel MJ, ed. Pediatric Sonography, 3rd ed. Philadelphia. Lippincott Wiliams &
Wilkins.
3. States LJ, Bellah RD. Imaging of the pediatric female pelvis. Semin Roentgenol 1996; 31:312-329.
4. Boechat IN. MR imaging of the pediatric pelvis. MRI Clin North Am. 1996; 4:679-697.
5. Rigsby CK, Siegel MJ. CT appearance of pediatric ovaries and uterus. J Comput Assist Tomogr 1994; 18:72-76.
6. Siegel MJ. Pelvic organs and soft tissues. In: Siegel MJ, ed. Pediatric Body CT. Philadelphia, Lippincott
Williams and Wilkins, 1999, pp 287-311.
7. Siegel MJ. Magnetic resonance imaging of the adolescent female pelvis. Mag Reson Imaging Clin North Am
2002; 10:303-324.
8. Baltarowich OH. Female pelvic organ measurements. In: Goldberg BB, Kurtz AB, eds. Atlas of Ultrasound
Measurements. Chicago. Year Book Medical Publishers. 1990; 190-242.
9. Cohen HL, Eisenberg P, Mandel F, Haller JO. Ovarian cysts are common in premenarchal girls: a sonographic
study of 101 children 2-12 years old. AJR 1992; 159:89-91.
10. Siegel MJ. Pelvic tumors. Radiol Clin North Am 1997; 35:1455-1475.
11. Fried AN, Kenney CM III, Stigers KB, Kacki MH, Buckley SL . Benign pelvic masses: sonographic spectrum.
RadioGraphics 1996; 16:321-334
12. Kim JS, Woo SK, Suh SJ, Morettin LB. Sonographic diagnosis of paraovarian cysts: value of detecting a separate
ipsilateral ovary. AJR 1995; 164:1441-1444
13. Jabra AA, Fishman EK, Taylor GA. Primary ovarian tumors in the pediatric patient: CT evaluation. Clin Imaging
1993;17:199-203.

14. Quillin SP, Siegel MJ. CT features of benign and malignant teratomas in children. J Comput Assist Tomogr 1992;
16:722-726.
15. Castleberry RP, Cushing B, Perlman E, Hawkins EP. Germ cell tumors. In: Pizzo PA, Poplack DG, et al.
Principles and Practice of Pediatric Oncology. Philadelphia: Lippincott Raven 1997; 921-945.
16. Outwater EK, Wagner BJ, Mannion C, McLarney JK, Kim B. Sex-cord-stroma and steroid cell tumors of the
ovary. Radiographics 1998; 18:1523-1546.
17. Blask ARN, Sanders RC, Gearhart JP. Obstructed uterovaginal anomalies: demonstration with sonography. Part I
neonates and infants. Radiology 1991; 179:79-83.
18. Blask ARN, Sanders RC, Rock JA. Obstructed uterovaginal anomalies: demonstration with sonography. Part II
teenagers. Radiology 1991; 179:84-88
19. Argons GA, Wagner BJ, Lonergan GJ, Dickey GE, Kaufman MS. Genitourinary rhabdomyosarcoma in children:
20. Fletcher BD, Kaste SC. Magnetic resonance imaging for diagnosis and follow-up of genitourinary, pelvic, and
perineal rhabdomyosarcoma. Urol Radiol 1992; 14:262-272.
21. Argons GA, Wagner BJ, Lonergan GJ, Dickey GE, Kaufman MS. Genitourinary rhabdomyosarcoma in children:

Bone Marrow Imaging
Lecture Objectives
• Review normal bone marrow anatomy
• Discuss MRI marrow techniques
• Describe features of normal marrow on MRI
• Recognize causes of pathologic marrow on MRI
Why study bone marrow?

• We all have it
• It shows up on every MRI
• It is an important site of pathology
• We need to know the normal before we can recognize the abnormal
M. Siegel, “Stating the Obvious”
Why Study Bone Marrow? It Has Clinical Applications

• MRI has become the imaging modality of choice for evaluating marrow
changes.
• MRI can help characterize the composition of marrow or marrow process in
question
• MRI provides excellent anatomic detail of surrounding structures, boundaries
of disease
PART I: Bone Marrow Constituents
Bone Marrow: 3 Components

• Osseous Matrix
• Red Marrow Figure 6-10-1
• Yellow Marrow
Marrow Components
• Osseous matrix
➢ (aka cancellous or spongy bone)
➢ Provides support for cellular components
Marrow Components
• Red Marrow
➢ Cellular, active or myeloid marrow
➢ Composed of red & white blood cells & platelets
Normal marrow constituents.
• Yellow Marrow
Histologic section showing
➢ Inactive or fatty marrow
hematopoietic and yellow marrow
➢ Composed of fat cells
The Major Marrow Constituents: Histology [Figure 6-10-1]
Red and Yellow Marrow: Different Chemical Composition
Red Yellow
Water 40 15
Fat 40 80
Protein 20 5
• MRI appearance of marrow reflects the relative
fractions of red & yellow marrow

1425 Bone Marrow Imaging
Bone Marrow Vascularity
• Red
➢ Rich sinusoidal vascular supply
➢ Favors metastases, infection
• Yellow
➢ Sparse vascular supply
➢ Favors infarction
PART II: MRI Techniques
PART II: Technique

• Depends on clinical indication
• Dedicated MRI of limited body part is used in the evaluation of localized pain
or to follow a focal lesion
• Whole body MRI is used for staging, restaging and surveillance
Basic Pulse Sequences

• For imaging pathology, these are your main sequences:
➢ Spin echo T1
➢ STIR or T2 with fat suppression
Whole-body MRI
• Technique
➢ Vertex to toes
➢ Coronal plane
❖ Sagittal plane
• Table moves 4-6 times
➢ Total imaging time
❖ ~ 7-20 minutes
Lauenstein, Radiology 2004;233:139
Whole-body MRI: Results

• 51 patients. 43 with metastases
• Reference standards: CT and bone scan
• All brain, lung, liver metastases > 6 mm seen
• Small lung metastases missed
➢ did not change therapeutic strategies
• Whole-body MR imaging on per-patient basis -- 100% sensitivity & specificity
values
Lauenstein, Radiology 2004;233:139
Part III: MR Features of Normal Marrow
Normal Marrow: T1
• This is your “Fat Finder” sequence
• Yellow marrow will be bright due to abundant fat composition
• Red marrow contains much less fat, and will be intermediate in signal. (But
enough fat not to be dark!!!!)
Normal Marrow: Fat Suppression

• STIR & T2 with fat saturation
• Both sequences exhibit T2 weighting, while suppressing fat signal
• Red Marrow remains intermediate
• Yellow marrow is dark, as fat is suppressed
Normal Marrow Imaging: T2

• T2 weighted imaging causes both red and yellow marrow to be intermediate in
intensity, making them look similar
• Not a widely used sequence
Bone Marrow Imaging 1424

Part III: MRI Features of Normal Marrow Figure 6-10-2
• Red Marrow
➢ T1-weighted: dark (> muscle < fat)
➢ STIR/FS: mildly bright (>muscle <
fat)
• Yellow Marrow
➢ T1-weighted: bright (= fat)
➢ STIR/FS : black (< muscle)
What About Gadolinium?

• Used if lesion is identified on T1 or fat
suppressed images
• Increases lesion conspicuity Normal red marrow. T1-weighted (left panel) and fat-saturated
• Worthwhile to remember that normal T2-weighted (right panel) images. Hematopoietic marrow in
adult red & yellow marrow does not the pelvis and femora exhibits a signal intensity equal to
enhance that of muscle
• Red marrow in neonate enhances
• Pathologic lesions enhance Figure 6-10-3
Normal Red Marrow Signal: Neonate
[Figure 6-10-2]
• In neonate, marrow has minimal lipid content
• Red marrow = muscle
Red Marrow Signal: Older Child [Figure 6-10-3]

• Red > muscle
Normal Yellow Marrow Signal

[Figure 6-10-4]
Review: Red/Yellow Signal Intensity (T1)

• Yellow > red > muscle
• Unossified cartilage is equal to muscle
Red marrow signal intensity. Red marrow has
Review: Red/Yellow Signal Intensity (STIR) signal intensity slightly higher than that of
• Red > muscle > yellow muscle on T1-WT and STIR images, reflecting
• Unossified cartilage is bright on T2, due to its greater fraction of lipids. Yellow epiphyseal
“watery” content marrow has signal intensity identical to that of
subcutaneous fat on both sequences
MR Normal Marrow: Variations in
Distribution
• Conversion of red to yellow marrow occurs during growth and development
and has a predictable and orderly pattern Figure 6-10-4
• You need to know this to avoid mistakes in diagnosis
Patterns of Marrow Distribution

• Neonate: virtually all red marrow
• Shortly after birth red-to-yellow marrow conversion
begins
➢ Overall: extremities to axial
➢ In a given bone: epiphysis/apophysis ? diaphysis
--> metaphysis
Marrow Conversion
Appendicular to Axial Conversion
Custer. J Lab Clin Med 1932
Long Bone Conversion Normal yellow marrow. T1-weighted image (left

• Distal to proximal in individual bones panel) and fat-saturated T2-weighted image
• Epiphysis to diaphysis to metaphysis (Right panel). Yellow marrow has a signal
intensity similar to subcutaneous fat on both
sequences

Marrow Distribution [Figure 6-10-5]
• Adult distribution by age 25 years
Figure 6-10-5
• From nearly 100% red marrow at birth to 40% red marrow in adulthood
Vogler JB III. Radiology 1998; 168:679-693.
Variations in Marrow Distribution

• A common EXCEPTION to the rule that red marrow converts to
yellow marrow is that the epiphyses & apophyses completely
convert to yellow marrow once they ossify
• Controversial whether they ever contain red marrow
Variations in Marrow Distribution

• Another exception is that red marrow can persist in the proximal
humeral and femoral epiphyses of adults
Variations in Red Marrow Distribution

• Epiphysis: subchondral, curvilinear focus
• Other patterns are likely abnormal
Bone Marrow Variations: Vertebral Marrow [Figure 6-10-6]

• Yellow marrow around basivertebral vein common in children
• Other patterns common in adults
• Islands of fat can mimic metastases
Ricci C. Radiology 1990;177:83-88.
Normal Vertebral Marrow: MRI
Summary Key Points: Criteria for Normal Marrow

• Shows expected signal intensities on all image sequences
• Shows expected distribution in the skeleton for patient age
• Is symmetric
• % of fatty marrow increases with age
PART IV: Marrow Pathology

• Reconversion
• Replacement Adult marrow distribution. Yellow
• Depletion marrow predominates in the
appendicular skeleton. Red
• Vascular – mediated lesions
marrow is found mainly in the
➢ Edema
skull, flat bones, spine, proximal
➢ Ischemia
metaphyses of both the humeri
and femora
Marrow Reconversion
• Opposite of conversion
• Results when increased demand for hematopoiesis Figure 6-10-6
• Generally symmetric
• Causes:
➢ Chronic anemias (sickle cell, thalassemia)
➢ Increased O2 needs (altitude, athletes, smokers)
➢ Granulocyte colony stimulating factor
➢ Cyanotic heart disease
Reconversion
• Axial skeleton responds first, followed by extremities
Marrow Reconversion
• Reverse order from normal conversion process! Vertebral marrow, red-yellow marrow
• Proximal to Distal!! distribution. Yellow marrow is
located near basivertebral vein in
neonates and young children
(upper right diagram). Other
patterns predominate in
adolescents and adults

Marrow Reconversion: Histology [Figure 6-10-7] Figure 6-10-7
• Increased red cells
• No fat cells to generate signal
Marrow Reconversion: Sickle cell anemia

• T1-WT images: dark signal intensity
• Red = or slightly > muscle
• STIR/Fat sat T2 images: slightly bright
Reconversion: Sickle Cell Anemia [Figure 6-10-8]
Reconversion: Thalassemia
Granulocyte Colony Stimulating Factor

• Stimulates marrow hyperplasia
• Focal abnormality (mimics tumor)
• Clues to diagnosis
➢ onset 2 to 8 weeks after treatment
➢ increased white blood cell count
➢ affects metaphysis and diaphysis
G-CSF Marrow reconversion. Histologic

section showing predominance of
Marrow Replacement or Infiltration [Figure 6-10-9] red cell elements
• Implantation of cells in marrow that do not normally exist there
• Follows red marrow distribution Figure 6-10-8
• Causes
➢ Neoplastic (lymphoma, leukemia,
mets)
➢ Inflammatory (osteomyelitis)
➢ Myeloproliferative (fibrosis)
➢ Lipidoses
Marrow Replacement
• T1 WT: dark
• STIR/Fat sat: very bright
• Can be diffuse or focal
• Predominates in red marrow (axial
skeleton) Marrow reconversion. Two different patients. Coronal T1-
weighted image of the knees shows diffuse low signal
Diffuse Replacement Disorders: intensity red marrow in the distal femoral and proximal tibial
Leukemia [Figure 6-10-10] metaphyses. Scattered high signal intensity foci represent
islands of yellow marrow and/or infarcts
Figure 6-10-10
Figure 6-10-9
Marrow replacement. The

normal hematopoietic Marrow replacement by leukemia. Coronal T1-weighted image
elements have been (left panel) shows diffusely low signal intensity marrow
replaced by neoplastic cells, throughout the pelvis and femora. Fat-saturated T2-
characteristic of chronic weighted image (right panel). Replaced marrow has a
lymphocytic leukemia signal intensity much brighter than that of muscle

Diffuse Replacement Disorders: Metastatic Disease
Focal Replacement: Metastases

• Metastases prefer red marrow
• Preferred sites:
➢ Vertebrae - 69%
➢ Pelvis - 41%
➢ Femur - 25%
➢ Skull - 14%
Vertebral Metastases: Breast Cancer
Review: Hyperplasia or Tumor?

• Distinguishing criteria:
➢ Red marrow: orderly distribution
❖ Tumor: random distribution
➢ Red marrow: usually symmetric bilaterally
❖ Tumor: usually asymmetric
➢ Red marrow: minimally bright on STIR/FS
❖ Tumor: extremely bright
Hyperplasia or Tumor? Fat Suppressed Images

• Normal red marrow
➢ SI: = or slightly > muscle
• Tumor infiltration
➢ SI: >>>>> muscle
Figure 6-10-11
Pitfall: Compression Fractures
• Acute compression due to osteopenia?
• Malignant compression fracture?
Malignant Compression Fracture

• Abnormal signal intensity
• May involve pedicle, posterior element or entire vertebral body
• Convex posterior cortex
• Epidural mass
• Paraspinal soft tissue mass
• Marrow enhancement post gadolinium
Pathologic Fracture
Osteoporotic Fracture
• Partial body involvement
➢ Usually involves end plate
• Signal of spared marrow is normal
• Thoracolumbar junction Vertebral fractures. Pathologic
• Clustering of abnormalities fracture (left panel). Gadolinium-
• Thin paraspinal soft tissue mass enhanced T1-weighted MR
• Improves in 6 to 8 weeks shows abnormal signal intensity
in entire vertebral body, convex
Compression fractures posterior cortex, and small
epidural mass. Osteoporotic
Pathologic and Osteoporotic Fractures [Figure 6-10-11] fracture (right panel). T1-
weighted MR shows partial body
Variations in Appearance of Metastases involvement (upper end plates),
• Blastic and fibrotic lesions normal marrow signal, and
• Low signal on T1 and fat -suppressed images clustering of abnormalities at
• Often heterogeneous thoracolumbar junction
Other Infiltrative Processes with Low T1 and High T2 Signal

• Osteomyelitis
• Myeloproliferative disorders
• Lipidoses

Osteomyelitis [Figure 6-10-12] Figure 6-10-12
• Infection produces increased cellularity and water content in
marrow (edema)
• T1: dark signal
• FS: bright signal
• Adjacent ST changes
➢ edema
Vogler JB III. Radiology 1998; 168:679-693
Osteomyelitis [Figure 6-10-13]

• T1: DARKER >> muscle. Water, cells replace marrow
• FST2: BRIGHTER >>muscle
Figure 6-10-13
Osteomyelitis. Inflammatory cells (I)

have replaced normal marrow
elements. T=supporting
trabeculae
Figure 6-10-14
Osteomyelitis. Coronal T1-weighted image (left

panel) showing low signal intensity area in
distal femoral metaphysis. Fat-saturated T2-
weighted image (right panel) showing
increased signal intensity
Osteomyelitis: MR
Myelofibrosis [Figure 6-10-14] Myelofibrosis. Marrow space contains

• Marrow replacement by fibrosis
abundant fibrotic tissue and
• Sequela of treated malignancy
sparse red cells
➢ Rarer as primary disorder
• Results in lowering of signal intensity
Figure 6-10-15
Myelofibrosis [Figure 6-10-15]
Mimics of Fibrosis
• Gaucher disease
➢ Marrow replacement by glucocerebroside-laden
cells
• Hemosiderin deposition
➢ Marrow replacement by iron
➢ Usually from transfusion therapy
• Decreased T1 and T2-weighted signal
• Slightly increased signal on STIR
Gaucher Disease
• Due to deficiency of the enzyme acid beta- Primary myelofibrosis. Coronal T1-weighted (left
glucocerebrosidase, which helps to break down panel) and T2-weighted (right panel) MR
glucosyl ceramide images show low signal intensity marrow in
• Follows distribution of red marrow the ilia, femora and vertebral bodies
• Proximal to distal

Gaucher Disease [Figure 6-10-16] Figure 6-10-16
Hemosiderin [Figure 6-10-17]

• Low SI on all sequences (black)
Marrow Depletion
Fatty Replacement [Figure 6-10-18]
• Absence of red marrow
• Causes
➢ chemotherapy
➢ radiation therapy
➢ aplastic anemia
• Marrow has signal of fat
Radiation & Chemotherapy Gaucher disease. Decreased signal intensity on T1-weighted

• Typical changes
images (left panel) and on STIR (right panel) images
➢ 1-2 days: edema
➢ 7-14 days: fatty replacement
➢ 3-4 wks: regenerating red marrow
• May see irreversible changes (fat or fibrosis) with high doses
Figure 6-10-17
Myeloid Depletion: MRI
• Signal follows fat
➢ high T1
➢ dark with fat sat
• Usually see sharp cut off lines at
radiation port
Andrews. Radiographics 2000
Myeloid Depletion [Figure 6-10-19]

• Follows signal intensity of fat
Figure 6-10-19
Hemosiderin deposition. Patient with sickle cell anemia who

received transfusional therapy. T1-weighted (left panel)
and T2-weighted (right panel) MR images showing diffusely
low marrow signal intensity. Scattered high foci area
represent residual fat and/or edema due to infarcts
Figure 6-10-18
Marrow depletion. Marrow elements

have been replaced by fatty
Myeloid depletion following radiation treatment. Sagittal tissue
T1-weighted image shows diffuse high signal
intensity fatty marrow in multiple vertebral bodies,
which were included in the radiation port

Myeloid Depletion Figure 6-10-20
• Pre-treatment
• Post-treatment
Vascular Mediated Disorders:

Edema
• Results in increased extracellular water
• Signal changes are those of water
• T1: low signal
• STIR/FS: bright signal
• Regionally limited
Marrow edema. Multiple causes. Bone bruise, left panel.
Vascular Mediated Disorders: Fracture, middle panel. Transient osteoporosis, right panel
Ischemia (infarct)
• Result of death of yellow & red marrow elements
• Signal changes represent balance of cell death and repair
• Regionally limited
➢ Subarticular
➢ Likes yellow marrow
Ischemia & Edema: Causes

Not all individually detailed in this lecture
Ischemia Edema
Steroids Trauma
Sickle cell Stress facture
SLE Transient osteoporosis
Gaucher Regional migratory osteoporosis
Pancreatitis Reflex sympathetic dystrophy
Radiation
• CLUE: These favor yellow marrow
Marrow Edema (STIR): Common Causes [Figure 6-10-20]
Marrow Ischemia: Avascular Necrosis [Figure 6-10-21]

Figure 6-10-21
Bone Marrow Disorders
T1 STIR/FS
Hyperplasia Dark Intermediate
Replacement Dark Bright
Fibrosis Dark Dark
Depletion Bright Dark
Vascular Dark Bright

Mediated
Avascular necrosis.T1-weighted (left panel) and

fat-saturated T2-weighted (right panel) MR
images showing low signal intensity
epiphyseal lesion. Ischemic injuries favor
yellow marrow

References
1. Siegel MJ. MR imaging of paediatric haematologic bone marrow disease. J Hong Kong Coll Radiol 2000; 3:38-
50.
2. Steiner RM, Mitchell DG, Rao VM, Schweitzer ME. Magnetic resonance imaging of diffuse bone marrow disease.
Radiol Clin North Am 1993; 31:383-409.
3. Vande Berg BC, Malghem J, Lecouvet FE, Maldague B. Magnetic resonance imaging of the normal bone marrow.
Skeletal Radiol 1998;27:471-483
4. Vanel D, Dromain C, Tordivon A. MRI of bone marrow disorders. Eur Radiol 2000; 10:224-229.
5. Vogler JB III, Murphy WA. Bone marrow imaging. Radiology 1998; 168:679-693.
6. Eustace S, Tello R, DeCarvalho V, et al. A comparison of whole body turboSTIR MR imaging and planar 99mTc-
methylene diphosphonate scintigraphy in the examination of patients with suspected skeletal metastases. AJR
1997; 169:1655-1661.
7. Lauenstein TC, Goedhe SC, Herborn CU, et al. Three-dimensional volumetric interpolated breath-hold MR
imaging for whole-body tumor staging in less than 15 minutes: a feasibility study. AJR 2002 Aug;179(2):445-9.
8. Lauenstein TC, Goedhe SC, Herborn CU, et al. Whole-body MR imaging: evaluation of patients for metastases.
Radiology 2004; 233:139-148.
9. Mazumdar A, Siegel M, Narra V, Luchtman-Jones L. Whole-body fast inversion recovery MR imaging of small
cell neoplasms in pediatric patients: a pilot study. AJR 2002; 179:1261-1266.
10. O’Connell MJ, Hargaden G, Powell T, Eustace SJ. Whole-body short tau inversion recover MR imaging with a
moving table top. AJR 2002; 179: 866-868.
11. Padhani A, Husband J. Bone. In: Husband JES, Reznek RH, eds. Imaging in Oncology. Isis Medical Media.
Oxford 1998; pgs 765-786.
12. Kricun ME. Red-yellow marrow conversion: its effect on the location of some solitary bone lesions. Skeletal
Radiol 1985; 14:10-19.
13. Custer RP, Ahlfeldt FE. Studies of the structure and function of bone marrow: variations in cellularity in various
bones with advancing years of life and their relative response to stimuli. J Lab Clin Med 1932; 17:960-962.
14. Moore DG, Dawson KL. Red and yellow marrow in the femur: age-related changes in the appearance at MR
imaging. Radiology 1990; 175:219-223.
15. Ricci C, Cova M, Kang YS et al. Normal age-related patterns of cellular and fatty bone marrow distribution in the
axial skeleton: MR imaging study. Radiology 1990; 177:83-88.
16. Taccone A, Oddone M, Dell’ Acqua A, Occhi M, Ciccone MA. MRI “road-map” of normal age-related bone
marrow. Pediatr Radiol 1995; 25:596-606.
17. Mirowitz SA. Hematopoietic bone marrow within the proximal humeral epiphysis in normal adults: investigation
with MR imaging. Radiology 1993; 188:689-93.
18. Fletcher BD, Wall JE, Hanna SL. Effect of hematopoietic growth factors on MR images of bone marrow in
children undergoing chemotherapy. Radiology 1993; 189:745-751.
19. Ryan SP, Weinberger E, White KS, et al. MR imaging of bone marrow in children with osteosarcoma: effect of
granulocyte colony-stimulating factor. AJR 1995; 165:915-920.
20. Shellock FG, Morris E, Deutsch AL, et al. Hematopoietic bone marrow hyperplasia: high prevalence on MR
images of the knee in asymptomatic marathon runners. AJR 1992; 335-338.
21. Baker LL, et al. Benign versus pathologic compression fractures of vertebral bodies: assessment with
conventional spin-echo, chemical shift, and STIR MR imaging. Radiology 1990; 174:495
22. Blomlie V, Rofstad EK, Skjonsberg A, Tverå, Lien HH. Female pelvic bone marrow: serial MR imaging before,
during, and after radiation therapy. Radiology 1995; 194:537-543.
23. Otake S, Mayr N, Ueda T et al. Radiation-induced changes in the MR signal and contrast enhancement of
lumbosacral vertebrae: do changes occur only inside the radiation therapy field? Radiology 2002; 222:179-183.
24. Stevens SK, Moore SG, Kaplan I. Early and late radiation changes in the spine: magnetic resonance imaging. AJR
1990; 154:745-750.
25. Hermann G, Shapiro RS, Abdelwahab IF, Grabowksi G. MR imaging in adults with Gaucher disease type I:
evaluation of marrow involvement and disease activity. Skeletal Radiol 1993; 22:247-251.
26. Levin TL, Sheth SS, Hurlet A, Comerci SC, Ruzal-Shapiro, Piomelli S, Berdon WE. MR marrow signs of iron
overload in transfusion-dependent patients with sickle cell disease. Pediatr Radiol 1995; 25:614-619.

Congenital Lung Malformations
Learning Objectives
• Review clinical & pathologic features of common lung anomalies
• Describe imaging appearances of the common congenital anomalies
• Emphasize imaging differential diagnosis
Congenital Lung Anomalies

• Normal vascularity
➢ Lobar emphysema Figure 6-11-1
➢ Cystic adenomatoid malformation
➢ Bronchogenic cyst
➢ Parenchymal agenesis, hypoplasia
• Abnormal vascularity
➢ Scimitar syndrome
➢ Sequestration
➢ Arteriovenous malformation
Congenital Lobar Emphysema

• Misnomer; true emphysema not present
• Infantile lobar “emphysema” = Congenital lobar emphysema. Gross section showing an
“overinflation” overinflated lobe that fails to deflate post resection. Histology
• Progressive lobar air-trapping from showing alveolar overdistention
bronchial obstruction;
➢ deficiency of bronchial cartilage
➢ intraluminal web
• Onset: 50% 1st week, 90% < 6 months
• Neonates--dyspnea, cyanosis, cough
• Later-asymptomatic, wheezing
Congenital Lobar Emphysema: Pathologic Features [Figure 6-11-1]

• Sponge-like mass, fails to deflate on resection
• Compressed normal lung deflates & reexpands
• Histo- Alveolar distention 5-10X normal
Figure 6-11-2
Lobar Emphysema: Imaging
• Lobar hyperinflation
• Atelectatic adjacent lung
• Initially opaque if retained lung fluid
• Mass effect: mediastinal shift,
attenuated vessels
• Lobar predilection:
➢ LUL (50%), RML (24%), RUL (18%)
Lobar Emphysema [Figure 6-11-2]

• Neonate with respiratory distress
Congenital lobar emphysema. Chest radiograph showing large
Companion Case hyperlucent left upper lobe with atelectasis of adjacent lung.
• 2-week-old boy with mild dyspnea CT showing hyperinflated left upper lobe with attenuated
vascularity
Congenital Lobar Emphysema
• Usually diagnosed in neonates & infants
• 20% diagnosed in adolescents & adults

1435 Congenital Lung Malformations
Lobar Emphysema Figure 6-11-3
DDX: Swyer-James Syndrome

➢ viral infection in childhood **
➢ unilateral hyperlucent (low
attenuation) lung
➢ small or normal size **
➢ bronchiectasis **
➢ air-trapping on expiration
**Helps to differentiate from CLE Swyer-James syndrome. Inspiration CT (left panel) and
expiration CT (right panel) showing a low attenuation right lung
Swyer-James Syndrome with air trapping on expiration. Also noted is bronchiectasis
• 10-year-old boy, cough
Swyer-James Syndrome [Figure 6-11-3]
Cystic Adenomatoid Malformation

• 25% of congenital lung lesions
• Result of abnormal proliferation of bronchioles Figure 6-11-4
• Normal arterial supply & venous drainage
• Communicates with bronchial tree
CCAM: Clinical
• 70%-90% symptomatic as neonates
➢ Cyanosis, grunting, tachypnea
• Rare in older children & adults
➢ Presents as pneumonia or recurrent infection
• May be antenatal diagnosis
CCAM: Histologic Types

• Stocker classification
➢ Type I: (50%) Large cyst(s) (> 2 cm)
➢ Type II: (40%) Multiple cysts (< 2 cm)
➢ Type III: (10%) Microcysts on cut-section
Stocker JT. Hum Pathol 1977; 8:155-171
CCAM: Imaging
• Air-filled mass, mediastinal shift
➢ Except may be opaque initially if fluid-filled
• Type I
➢ Multicystic lesion, with dominant cyst(s)
➢ Air fluid levels
• Type II
➢ Heterogeneous, uniform small cysts
• Type III
➢ Large, homogeneous, solid
➢ Resembles consolidation
Type I CCAM: Large Cysts [Figure 6-11-4]

• Neonate with dyspnea Cystic adenomatoid malformation,
Type I. Chest radiograph and CT
showing large cystic lesion in the right
middle lobe. Gross pathologic
section also shows a large cystic
mass
Congenital Lung Malformations 1434

Type II CCAM: Small Cysts [Figure 6-11-5] Figure 6-11-5
CCAM [Figure 6-11-6]
Figure 6-11-6
Cystic adenomatoid malformation, Type II. Chest

radiograph showing a lucent area in the left lower
lobe. Two CT scans and gross pathologic section
Cystic adenomatoid malformation. Chest showing a cystic mass with multiple small cysts
radiograph on day 1 showing an opaque right
upper lobe mass. Follow-up radiographs on days
3 and 7 show a more cystic appearing mass. CT
showing a multicystic mass in the right upper lobe
CCAM Antenatal US Diagnosis

• Polyhydramnios
• Fetal hydrops
➢ Ascites
➢ Anasarca
➢ Placental edema
• Solid or cystic lung mass
• High perinatal mortality
Spectrum of CCAM
• 10% diagnosed in adolescents & adults
• Infection common
• May mimic infiltrate or mass on imaging studies
Infected CCAM
• Adolescent with cough
Figure 6-11-7
Infected CCAM [Figure 6-11-7]
Bronchogenic Cyst
• Failure of lung bud to incorporate into
primitive lung
• Lung (30%), mediastinum (70%)
• Asymptomatic & incidental finding
• Symptomatic from mass effect
Infected cystic adenomatoid malformation. Chest radiograph

showing an air-space opacity with a cystic component
superiorly. Transverse CT showing multiple cysts with thick
walls and some air-fluid levels

Bronchogenic Cyst: Path [Figure 6-11-8] Figure 6-11-8
• Gross
➢ Separate from lung
➢ Round or ovoid
➢ Clear or turbid contents
• Histo
➢ Lined by respiratory epithelium
➢ Bronchial glands, cartilage, smooth muscle in wall
Pulmonary Bronchogenic Cyst: Imaging

• Smooth, rounded, unilocular mass
• Fluid-filled, usually serous fluid
➢ Sometimes protein or mucin
• Air or air-fluid levels if infected
• No mediastinal shift
• Non-enhancing
Bronchogenic Cyst
• 7-day old girl, wheezing
Bronchogenic cyst, pathology. Gross
Bronchogenic Cyst [Figure 6-11-9] specimen showing a fluid-filled lung
• 7- year-old girl with cough mass. Histologic section showing
respiratory epithelium lining the cyst
Figure 6-11-9 wall
Figure 6-11-10
Bronchogenic cyst. Transverse and coronal CT

scans showing a well defined, smoothly
marginated mass containing only air in the right
upper lobe
Bronchogenic cyst. Chest radiograph showing a
DDX: Cystic Adenomatoid Malformation right paratracheal mass. Transverse CT scans
• Septations and multiple cysts favor CAM showing a water attenuation mass with
imperceptible margins in the right paratracheal
Review area
• Lobar emphysema
• CCAM
• Bronchogenic cyst
Figure 6-11-11
Mediastinal Bronchogenic Cysts
• Same characteristics as pulmonary cysts
➢ Round, solitary, air or fluid filled
Mediastinal Bronchogenic Cyst

[Figure 6-11-10]
Bronchogenic Cyst: MRI
Differential Diagnosis: Enteric Cysts Enteric cyst. Transverse CT (left panel) showing a
[Figure 6-11-11]
water attenuation mass in the lower mediastinum.
Gross section (middle image) showing a cystic
mass. Histologic section (right panel) showing
gastrointestinal lining

Enteric Foregut Cyst Figure 6-11-12
Pulmonary Underdevelopment
• Agenesis: Complete absence of lung tissue, artery, & small or
absent bronchus
• Hypoplasia: Small lung & bronchus (artery may or may not
develop)
Lung Agenesis [Figure 6-11-12]

• Small bronchus
• No lung or PA
Companion Case: Lung Agenesis
Arrested Pulmonary Development

• Pulmonary hypoplasia
➢ Small lung (hypoplasia)
➢ Small bronchus
➢ Absent or small pulmonary artery
➢ Mediastinal shift to side of hypoplasia
Pulmonary Hypoplasia [Figure 6-11-13]

• 2-month-old boy, mild dyspnea
Figure 6-11-13
Lung agenesis. Chest radiograph in

a neonate showing an opacified
hemithorax. CT scan showing
absence of the right lung and
pulmonary artery and a small
rudimentary bronchus (arrow)
Pulmonary hypoplasia. Frontal chest radiograph
in a 2 month old boy showing a small right lung
and mediastinal shift to the right. Lateral
radiograph showing a posterior sternal stripe, Figure 6-11-14
representing fibrofatty tissue (arrow)
Pulmonary Hypoplasia [Figure 6-11-14]
Pulmonary Hypoplasia
• Small lung & bronchus No PA
Congenital Anomalies with Abnormal

Vasculature
• Hypoplasia with anomalous venous return
➢ Scimitar syndrome
• Pulmonary sequestration
• Arteriovenous malformation
Pulmonary hypoplasia. Transverse chest CT
Hypogenetic Lung Syndrome scans showing a small right lung, main right
• Lung hypoplasia with PAPVR bronchus, absent right pulmonary artery and
• PAPVR into IVC, portal vein/ hepatic vein, or right mediastinal shift to the right
atrium
• Often asymptomatic and discovered
during evaluation of another anomaly

Hypogenetic Lung Syndrome: Imaging Features Figure 6-11-15
• Usually right lung
• Anomalous pulmonary venous return
• Lung hypoplasia
• Hypoplasia of Rt pulmonary artery
• Dextrocardia
• May have abnormal systemic arterial supply to lung
Hypogenetic Lung Syndrome [Figure 6-11-15]
Pulmonary Sequestration
• No normal connection with bronchial tree or
pulmonary arteries
• Systemic blood supply
• 2 types
➢ Intralobar (acquired)
➢ Extralobar (congenital)
Hypogenetic lung syndrome. Transverse CT scan
Pulmonary Sequestration showing a small right lung and mediastinal shift
• ELS ILS towards the right. Also noted a left aortic arch with
➢ Own pleura Shared pleura anomalous right subclavian artery. Coronal
➢ Neonate Children & adults multiplanar (middle panel) and 3D volume
➢ 90% left 90% left rendered image (viewed posteriorly) showing an
➢ Solid Cystic or solid anomalous vein (arrow) draining the right lower
➢ Syst. arterial supply Syst. arterial supply lobe
➢ Syst.venous drainage Pulm. venous drainage
➢ Assoc. anomalies +/- assoc. anomalies Figure 6-11-16
Sequestration: Vascular Findings

• ILS
➢ Arterial supply
❖ Thoracic aorta (70%)
❖ Other-abdominal aorta, intercostal
➢ Venous drainage
Pulmonary (95%), systemic (5%)
• ELS
➢ Arterial supply
❖ Abdominal aorta (80%)
❖ Other—thoracic aorta, pulmonary artery
➢ Venous drainage
❖ Systemic (80%), pulmonary (20%) Intralobar sequestration,
pathology. Cut section shows
Pulmonary Sequestration abnormal inflamed parenchyma
• Intralobar
• Extralobar Figure 6-11-17
Intralobar Sequestration: Path [Figure 6-11-16]

• Gross
➢ Edge may abruptly abut normal lung or blend
diffusely
• Microscopic
➢ Chronic inflammation
➢ Bronchopneumonia
Extralobar Sequestration: Path [Figure 6-11-17]

• Separate from normal lung
➢ Pyramidal, rounded, ovoid
➢ Resembles normal lung Extralobar sequestration. Gross specimen
• Microscopic resembles normal lung Histologic section shows
➢ Dilated bronchioles, alveoli & subpleural dilated bronchioles, alveolar ducts, and alveoli.
lymphatics Also noted is a well-formed bronchus near one
➢ Well-formed bronchus near edge of lesion (50%) edge of the lesion

Sequestration: Clinical Figure 6-11-18
• ELS
➢ Often asymptomatic
➢ Dyspnea, cyanosis occasionally (10%)
➢ Associated anomalies
❖ CHD, diaphragmatic hernia, CCAM
• ILS
➢ Symptomatic
➢ Cough, recurrent pneumonia
Sequestration: US Features
• Gray-scale
Intralobar sequestration. Chest radiograph
➢ Echogenic mass
showing a left lobe air space opacity. Sonogram
❖ Supradiaphragmatic-likely ILS
showing a supradiaphragmatic echogenic mass
❖ Subdiaphragmatic (suprarenal)-likely ELS
with a feeding artery in the left lower lobe
• Doppler US
➢ Feeding artery off aorta Figure 6-11-20
➢ Draining vein usually not identified
Sequestration: CT Features
• Anomalous feeding artery
• Draining vein to pulmonary or systemic veins
• Parenchymal findings
➢ ILS:
❖ Infiltrate
❖ Abscess
❖ Focal emphysema
➢ ELS
❖ Triangular/round solid mass
Intralobar Sequestration: Infiltrate [Figure 6-11-18]
Intralobar Sequestration: Infiltrate & Emphysema

[Figure 6-11-19]
Figure 6-11-19
Intralobar sequestration. Transverse

CT showing a cystic mass with an
enhancing wall in the left lower lobe,
Intralobar sequestration. Transverse CT at lung representing an abscess. 3D
windows (left panel) showing a left lower lobe reconstruction (viewed posteriorly)
infiltrate with surrounding emphysema. showing an anomalous artery (arrow)
Transverse contrast enhanced CT (right panel) from the distal thoracic aorta
showing an opacified anomalous artery (arrow) supplying the sequestered lung
from the distal thoracic aorta supplying the
sequestered lung
Intralobar Sequestration: Drainage via PV to Left Atrium
Intralobar Sequestration Abscess Formation

• 6-year-old girl, fever and cough
Intralobar sequestration: Abscess Formation [Figure 6-11-20]

Extralobar Sequestration: Triangular Mass Figure 6-11-21
[Figure 6-11-21]
Extralobar Sequestration:
Round Lower Lobe Mass [Figure 6-11-22]
Extralobar Sequestration:
Systemic Drainage
Extralobar Sequestration
• Solid, well-defined LLL mass
Extralobar sequestration. Chest radiograph
showing a triangular mass (arrow) at the left lung
Sequestration: MRI
base. Transverse sonogram showing an
heterogeneous mass (arrows) which was below
ELS with CCAM (40%)
the left hemidiaphragm
Summary
• ILS Figure 6-11-22
• ELS
Arteriovenous Malformation
• 80% Hereditary telangiectasia (OWR)
➢ 15% sporadic
➢ 5% cardiac surgeries (Glenn or Fontan)
• Symptomatic in older patients (cyanosis,
polycythemia, dyspnea)
• 80%-90% are simple AVMs
➢ single feeding and draining vessel
➢ commonly lower lobes
Arteriovenous Malformation Extralobar sequestration. Coronal CT reformation

(left panel) and 3D reconstruction (right panel)
Pulmonary AVM showing two anomalous feeding arteries entering
• Simple architecture a solid left lower lobe sequestration
Simple Pulmonary AVM
Multiple AVMs [Figure 6-11-23] Figure 6-11-23
Congenital Lung Anomalies

• ABNORMAL LUNG - NORMAL VASCULATURE
• NORMAL LUNG - ABNORMAL VASCULATURE
Multiple pulmonary arteriovenous malformations.

Two transverse CT scans and 3D reconstructions
showing multiple pulmonary arteriovenous
malformations (arrows)

References
1. Siegel MJ. Lung, pleural and chest wall. In: Pediatric Body CT. Philadelphia, Lippincott Williams & Wilkins.
1999;101-.140
2. Kim WS, Lee KS, Kim IO, et al. Congenital cystic adenomatoid malformation of the lung: CT-pathologic
correlation. AJR 1997; 168:47-
3. Rosado-de-Christenson JL, Stocker JT. Congenital cystic adenomatoid malformation. J Comput Assist Tomogr
1989; 13:612-616
4. Shackelford GD, Siegel MJ. CT appearance of cystic adenomatoid malformation. J Comput Assist Tomogr 1989;
13:612-616.
5. Felker RE, Tonkin IL. Imaging of pulmonary sequestration. AJR 1990; 154:241-249
6. Frazier AA, Rosado de Christenson ML, Stocker JT et al. Intralobar sequestration: radiologic-pathologic
correlation. RadioGraphics 1997; 17:725-745.
7. Ko SF, Ng SH, Lee TY, et al. Noninvasive imaging of bronchopulmonary sequestration. AJR 2000; 175:1005-
1012
8. Lee E, Siegel MJ, Sierra LM, Foglia RP. Evaluation of angioarchitecture of pulmonary sequestration in pediatric
patients using multidetector CT angiography. AJR 2004; 183:183-188.
9. Rosado-de-Christenson ML, Frazier AA, Stocker JT, Templeton PA. Extralobar sequestration: radiologic-
pathologic correlation. From the archives of the AFIP. RadioGraphics 1993; 13:425-441.
10. Konen E, Raviv-Zilka L, Cohen RA, et al. Congenital pulmonary venolobar syndrome: Spectrum of helical CT
findings with emphasis on computerized reformatting. RadioGraphics 2003; 23:1175-1184
11. Woodring JH, Howard TA, Kanga JF. Congenital pulmonary venolobar syndrome revisited. RadioGraphics 1994;
14:349-369.
12. Remy J, Remy-Jardin M, Giraud F, et al. Angioarchitecture of pulmonary arteriovenous malformations: clinical
utility of three-dimensional helical CT. Radiology 1994; 191:657-664.
13. Hoffman LV, Kuszyk BS, Mitchell SE, et al. Angioarchitecture of pulmonary arteriovenous malformation:
characterization using volume-rendered 3D CT angiography. Cardiovasc Intervent Radiol 2000; 23: 165-170.
14. Rotondo A, Scialpi M, Scapati C. Pulmonary arteriovenous malformation: evaluation by MR angiography. AJR
1997; 168: 847-849.

Lung Diseases in Neonates
Neonatal Respiratory Distress: Spectrum of Disorders

[Figure 6-12-1]
• Surgical Disease
• Congenital Heart Disease Figure 6-12-1
• Medical Disease
Objectives
• Discuss lung diseases in preterm and term neonates
• Review treatment complications
• Understand important differential findings
Neonatal Medical Lung Diseases

• WHAT YOU WILL SEE:
➢ Respiratory distress syndrome
➢ Retained lung fluid
➢ Meconium aspiration
➢ Neonatal pneumonia
• WHAT YOU MAY SEE:
➢ Chylothorax
➢ Congenital surfactant protein B deficiency
Medical Lung Diseases:

Premature Neonate
Neonatal respiratory distress, spectrum of causes
Premature Births
• < 37 weeks
• ~500,000 preterm deliveries/year
• 12% of all live births
• Lung disease most common problem
CDC National Vital Statistics Reports, Vol. 52, No. 10, Dec 17, 2003
Respiratory Distress Syndrome (RDS)

• Same as hyaline membrane disease (HMD) & surfactant deficiency disease
• 1% of pregnancies, typically prematures
➢ 26-34 weeks
• Post-term infants of diabetic mothers
• More frequent and severe in males
• More common in whites than blacks
Figure 6-12-2
RDS/HMD: Pathogenesis
• Surfactant deficiency
• Leads to alveolar collapse
• Increased capillary permeability
• Hyaline membrane formation
What are Hyaline Membranes? [Figure 6-12-2]

• Line terminal & respiratory bronchioles
• Contain
➢ Necrotic alveolar cells
➢ Plasma transudate Hyaline membranes. Eosinophilic
➢ Aspirated squames hyaline membranes surrounded by
➢ Fibrin collapsed air spaces
Lung Diseases in Neonates 1442

RDS/HMD: Pathogenesis
• Mature airspace
• Surfactant deficiency
• Bronchial collapse & atelectasis
RDS/HMD: Gross Pathology

• Firm atelectatic lungs (“hepatization”)
• Alveolar edema
• +/- focal hemorrhage
RDS/HMD: Imaging Findings

• Small lung volumes
Figure 6-12-3
• Diffuse bilateral granular opacities
• Air bronchograms
• Loss of vascular definition
• No pleural effusion
RDS/HMD [Figure 6-12-3]
RDS/HMD: Classic Clinical Approach

• Confirm suspected diagnosis with radiographs
• Positive pressure ventilator therapy
➢ Increases risk of air leak
➢ Increases risk of BPD
RDS/HMS: Other Treatment Options Respiratory distress syndrome, untreated. Low

• Maternal (in utero) steroid administration lung volumes with diffuse bilateral granular
• Exogenous surfactant opacities
Exogenous Surfactant Therapy: Clinical Impact

• Slow liquid bolus into airway
• Desired effect is decreased:
➢ Oxygen requirement
➢ Mortality from RDS
➢ Air leak
Surfactant Therapy:Imaging Findings

• Uniform improvement in granularity at 24-48 hrs (38%) Figure 6-12-4
• Asymmetric improvement (35%)
• Small cystic lucencies (17%)
• No improvement (10%)
• Focal hemorrhage or hemorrhagic pulmonary edema
(1%-2%)
Pediatr Radiol 1997; 27:26-31
Surfactant Therapy [Figure 6-12-4]
Complications of Treatment
• Related to respirator Chest radiograph (left image) showing RDS,
➢ Air leaks pretreatment. Chest radiograph (right image)
➢ Bronchopulmonary dysplasia showing RDS, 24 hours after surfactant therapy
Air Leak: Pathogenesis
• Results from positive pressure ventilation of noncompliant lungs
• Rupture at bronchiolar-alveolar junction
• Gas dissects into interlobular septa and septal lymphatics (interstitial air)
• May enter pleural space, mediastinum, pericardium, peritoneum, venous
system

1445 Lung Diseases in Neonates
Pulmonary Interstitial Emphysema [Figure 6-12-5] Figure 6-12-5
Acute Pulmonary Interstitial Emphysema:

Imaging Findings
• Tubular & cystic lucencies
• Focal or diffuse
• Unilateral or bilateral
• Pulmonary overexpansion
• Mediastinal shift (unilateral PIE)
• Small cardiac silhouette (diffuse bilateral PIE)
Pulmonary Interstitial Emphysema [Figure 6-12-6]
RDS/Pulmonary Interstitial Emphysema
Persistent Interstitial Pulmonary Emphysema [Figure 6-12-7]

• PIE lasting > 1 week
• Associated with pseudocysts
➢ Aggregate thick-walled cysts
• Right parahilar location
Air leak, pulmonary interstitial
• May resolve completely or may need resection
emphysema. Gas in
• Mimic solid mass when fluid-filled
interlobular septa
Williams, et al. AJR 1988; 150:885-887
Figure 6-12-6
Persistent PIE: CT Findings
• 17 preterm neonates
• Hyperexpanded complex cystic
masses developed from typical
PIE (mean, 13 days)
• 53% underwent resection
Donnelly LF, et al. AJR 2003;
180:1129
Persistent PIE with Air Leak

“Pseudocysts” [Figure 6-12-8]
Figure 6-12-8
Acute pulmonary interstitial

Figure 6-12-7 emphysema. Chest radiograph
showing hyperinflated lungs with
cystic lucencies bilaterally.
Pathologic section showing interstitial
air
Persistent pulmonary interstitial

emphysema. Chest radiograph
Persistent interstitial pulmonary showing cysts in right parahilar
emphysema. Gross specimen. region. CT showing complex
Mass-like aggregate of thick- multicystic mass in the right
walled cysts lower lobe

Air Leak “Pseudocysts” Figure 6-12-9
Air Leak: Pneumothorax [Figure 6-12-9]

• May be subtle in infants
• Collects anteriorly in supine patients
• Look for “deep sulcus” sign and asymmetric lucent
lung
• Mediastinal shift with tension pneumothorax
Air Leak: Pneumomediastinum

• Most are clinically insignificant
• Usually does not dissect into neck because of Left panel. Two different patients. Chest
persistence of sternopericardial ligament radiographs in both patients show a tension
• Thymus elevation (“spinnaker sail sign”) pneumothorax with mediastinal shift and
• Gas beneath heart hyperexpansion of the affected hemithorax. Left
➢ “Continuous diaphragm sign” image, also note underlying changes of hyaline
membrane disease. Right image, also note
Pneumomediastinum [Figure 6-12-10] underlying pulmonary interstitial emphysema
Air Leak: Pneumopericardium [Figure 6-12-11]

• Gas delimited superiorly by pericardial reflection about great vessels
• No thymic displacement
• May require needle compression of tamponade Figure 6-12-10
Pneumopericardium
Air Leak: Pneumopericardium & PTX
Air Leak: Systemic Gas Embolism

• Pathogenesis uncertain
➢ Alveolar-venous fistula?
➢ Lymphatic gas entering right heart?
• Virtually 100% fatal Pneumomediastinum. Elevation of thymic lobes
on frontal radiograph (spinnaker sail sign).
Bronchopulmonary Dysplasia: Clinical Substernal air seen on lateral radiograph
Diagnosis
• > 3 days positive pressure ventilation + 02 during 1st 2 weeks of life
• Respiratory distress lasting > 28 days Figure 6-12-11
• Require supplemental 02 > 28 days to maintain Pa02 > 50 mm Hg
• Characteristic radiologic findings
Bronchopulmonary Dysplasia: Risk Factors

• Stress factors
➢ Barotrauma
➢ Oxygen cytotoxicity (free radicles)
➢ Infection
• Pulmonary edema (related to PDA)
• Host Factors
➢ Genetics (family history of atopy & asthma)
➢ Endogenous low steroids
Bronchopulmonary Dysplasia: Pneumopericardium. Air extends to

Basic Pathologic Features level of great vessels. Also noted is
• Tracheal & bronchial injury underlying pulmonary interstitial
➢ Mucosal ulceration & necrosis emphysema
• Pulmonary arterial injury
➢ Intimal & adventitial thickening
➢ Hypertensive vascular disease
❖ Bronchiolar & alveolar injury
➢ Cell necrosis & septal edema

• Reparative phase Figure 6-12-12
➢ Alveolar septal fibrosis
Stocker JT. Hum Pathol 1986; 17:943
Bronchopulmonary Dysplasia [Figure 6-12-12]
Classic Stages of BPD:Imaging Findings

• I: 0-4 days granular opacities of RDS
• II: 4-10 days “increased opacities”
• III: 10-30 days “Bubbly Lungs”
• IV: > 30 days disordered aeration
• All 4 stages rarely observed
Variant in Stages of BPD“Precocious BPD”

• “Bubbly” lungs develop at end of 2nd postnatal week
• < 1000 gram newborns
• Lower respiratory tract infection
Crouse DT et al. Clin Infect Dis 1993; 17: S 122-130
Classic BPD: “Bubbly Lungs” [Figure 6-12-13]

Figure 6-12-13
Bronchopulmonary Dysplasia
• HRCT Bronchopulmonary
➢ Emphysema dysplasia. Gross path
➢ Cystic or bullous changes specimen (left ipanel)
➢ Septal lines showing fibrotic lungs.
• No zonal predominance Histologic specimen
(right panel) showing
Bronchopulmonary Dysplasia dilated air spaces and
[Figure 6-12-14] septa thickened by a
combination of edema,
BPD: Classic Temporal inflammatory cells and
Course fibroblasts
Bronchopulmonary dysplasia.
BPD: Chronic Course: Hyperinflation and bubbly cystic
Disorganized Aeration lungs
BPD: Prognosis Figure 6-12-14

• Pulmonary function slowly improves
• Radiographs normalize in most by 3 years
• PFTs remain abnormal for years
• Refractory pulmonary hypertension = poor prognosis
& lung transplant
Terminology: Need to recognize

• BPD is a chronic insult Bronchopulmonary dysplasia. CT showing
• Any insult that increases the need for mechanical emphysematous changes, septal lines extending
ventilation can lead to chronic injury to pleural surface and bullous formation
• Mechanical ventilation increases risk of lung
damage!
• Will have the same appearance as BPD
Medical Lung Diseases: Term Neonate
Other Neonatal Lung Diseases

• Retained fetal lung fluid
• Aspiration syndromes
• Pneumonia (term & premature neonates)
• Surfactant B protein deficiency
• Chylothorax

Retained Fetal Lung Fluid or Transient Tachypnea of Newborn Figure 6-12-15
• Associated with C-section, maternal sedation,
maternal diabetes
• Lung fluid normally removed by capillary and
lymphatic resorption & retrograde tracheal flow
• Lack of compression by birth canal (C-section) &
decreased maternal & neonatal beta-adrenergic
responsiveness may alter clearance
Retained Fetal Lung
Retained Fetal Lung Fluid: Radiographic Retained fetal lung fluid. Frontal chest radiograph
Findings showing reticular opacities and small right pleural
• Increased lung volumes effusion. Lateral radiograph showing fissural fluid
• Reticular opacities
• Fissural fluid
• Small pleural effusions Figure 6-12-16
• Clearing in 24 to 48 hours
Retained Fetal Lung Fluid

[Figures 6-12-15 and 6-12-16]
Meconium Aspiration Syndrome

• In utero defecation due to fetal distress
• Usually > 34 weeks gestation
Retained fetal lung fluid. Frontal chest radiograph
• 10%-15% of pregnancies have meconium stained
day 1 (left panel) showing reticular opacities.
amniotic fluid
Chest radiograph day 2 (right panel) showing
• 5% of meconium stained neonates develop
interval clearing of interstitial fluid
meconium aspiration syndrome
• 30%-50% require mechanical ventilation
Figure 6-12-17
Meconium Aspiration Syndrome:
Pathologic Features
• Acellular debris
➢ Squamous epithelial cells
• Tenacious green-yellow meconium plugs in airways
• Pneumonitis
• Hemorrhagic edema
medic.med.uth.tmc.edu/
Meconium Aspiration: Pathophysiology

• Primary Meconium aspiration. Hyperinflated
➢ Mechanical Obstruction lungs and coarse interstitial opacities
➢ Chemical inflammation
➢ Surfactant inactivation
• Secondary Figure 6-12-18
➢ Air trapping
➢ Air leak
➢ Atelectasis
Meconium Aspiration: Imaging [Figure 6-12-17]

• Hyperinflation
• Coarse patchy opacities
• Air leak (25%-40%)
➢ Pneumomediastinum
➢ Pneumothorax Meconium aspiration. Hyperinflated lungs with
➢ Pleural effusion (rare) large pneumothoraces and pneumomediastinum
Meconium Aspiration [Figure 6-12-18]

Meconium Aspiration: Treatment
• Antibiotics
• Surfactant
• Mechanical ventilation Figure 6-12-19
• Inhaled nitric oxide (iNO)
• May require ECMO
• 94% overall survival
Neonatal Pneumonia
• 0.5% of term infants
• Frequently associated with neonatal sepsis
• Clinical and radiographic challenging diagnosis
Neonatal Pneumonia: Etiology

• In utero (hematogenous) infection Neonatal pneumonia. Coarse interstitial opacities,
➢ CMV, syphilis, listeriosis increased lung volumes
• Ascending infection (PPROM)
➢ Group B beta hemolytic strep
➢ E. coli Figure 6-12-20
• During delivery
➢ Strept
➢ Chlamydia trachomatis
• Postnatal infection
➢ Bacterial
Neonatal Pneumonia: Path

• Air spaces contain neutrophils & squamous epithelial cells without
fibrin
• Air spaces surrounded by thin cellular septa
Neonatal Pneumonia: Radiologic Findings

• Nonspecific, need to correlate clinically
• Diffuse granularity (may mimic RDS)
• Patchy and streaky opacities (may mimic TTN or meconium)
• Pleural effusion (65%) Neonatal pneumonia. Patchy
• Lung volumes usually normal, but may be increased confluent opacities. Normal lung
volumes
Neonatal Pneumonia [Figures 6-12-19 and 6-12-20]
Review & Quiz Time Figure 6-12-21

TTN, Pneumonia or Meconium?
Concede that lung findings are nonspecific:

Look for the clues!! [Figure 6-12-21]
Meconium, pneumonia, TTN?

• Hyperinflation?
➢ Meconium, TTN, pneumonia
• Air leak?
➢ Meconium aspiration
• Normal aeration?
➢ Pneumonia
• Patchy confluent opacities?
➢ Pneumonia
• Fissural fluid?
➢ TTN
Clues to diagnosing neonatal lung disease

OR Stated Another Way
• Meconium aspiration
➢ Reticular opacities, increased lung volumes, effusion rare (<10%)
• Neonatal pneumonia
➢ Reticular or confluent opacities, normal or increased aeration, effusions
common (65%)
• TTN
➢ Reticular opacities, increased lung volumes, pleural or fissural fluid (90%)
For Comparison
• Respiratory distress syndrome
➢ Decreased long volumes
➢ Granular lung disease
➢ NO effusions in uncomplicated disease!!
Medical Lung Diseases: Presenting in 1st week
Chylothorax
• Rupture of thoracic duct
• Usually due to birth trauma
➢ Rarely congenital anomaly
• Full term infants
➢ 70% symptomatic in 1st week
• Right > left pleural effusions
➢ Rarely bilateral
Chylothorax
• Thoracentesis usually yields cloudy fluid because of the high lipid content of
chyle
• Treatment includes thoracentesis, chest tube drainage, and feedings of
medium-chain triglycerides
• Most lymphatic ruptures seal with combined chest tube and dietary treatment
Chylothorax
• Differential diagnosis includes causes of nonchylous pleural effusions
➢ Wet-lung disease
➢ Hydrops fetalis
➢ Turner’s syndrome
➢ Pulmonary vein obstruction
➢ Esophageal rupture
Chylothorax [Figure 6-12-22]
Alveolar Proteinosis
• Due to congenital surfactant protein B deficiency
• Autosomal recessive
• PAS-positive abnormal surfactant lipids & proteins accumulate in alveoli &
macrophages
• Diagnosis confirmed by alveolar lavage & peripheral blood DNA analysis Figure 6-12-22
• Treatment - lung transplantation
Pediatr Radiol 2001; 31: 327-331
Chylothorax. Day 1 (left image), large right pleural effusion.

Day 7 (middle image), decreased fluid following thoracenteses.
One month (right image), resolution, following thoracentesis
and feedings of medium chain triglycerides

Surfactant B Deficiency: Imaging Features Figure 6-12-23
• Mimics RDS, but in term infant
• Chest
➢ Low lung volumes
➢ Hazy lungs
• CT
➢ Ground-glass opacities & septal lines
➢ “Crazy paving”
Surfactant B Deficiency [Figure 6-12-23]

• Mimics HMD Surfactant B deficiency. Chest radiograph
• Crazy paving showing low volume, hazy lungs. CT showing
ground-glass opacities and thickened septal lines
Neonatal Medical Diseases: The Big Four
[Figure 6-12-24]
Figure 6-12-24
The top 4 neonatal medical diseases
References
1. Center for Disease Control and Prevention: National Center for Disease Statistics. National Vital Statistics
Reports, Vol. 52, No. 10, Dec 17, 2003. Web: www.cdc.gov/nchs
2. Dinger J, Schwarze R, Rupprecht E. Radiological changes after therapeutic use of surfactant in infants with
respiratory distress syndrome. Pediatr Radiol 1997; 27:26-31.
3. Donnelly LF, Lucaya J, Ozelame V, et al. CT findings and temporal course of persistent pulmonary interstitial
emphysema in neonates: a multiinstitutional study. AJR Am J Roentgenol 2003; 180:1129-1133.
4. Medic: medical education information. University of Texas - Houston, Department of Pathology and Laboratory
Medicine. http://medic.med.uth.tmc.edu/
5. Newman B, Kuhn JP, Kramer SS, Carcillo JA. Congenital surfactant protein B deficiency--emphasis on imaging.
Pediatr Radiol 2001; 31:327-331.
6. Stocker JT. Pathologic features of long-standing "healed" bronchopulmonary dysplasia: a study of 28 3- to 40-
month-old infants. Hum Pathol 1986; 17:943-961.
7. Williams DW, Merten DF, Effmann EL, Scatliff JH. Ventilator-induced pulmonary pseudocysts in preterm
neonates. AJR Am J Roentgenol 1988; 150:885-887.

Pediatric Cardiac Imaging Part I:
Vascular Anomalies
Lecture Outline
• Review MRA and CTA techniques
• Discuss CT and MR appearances of common thoracic vascular anomalies
MR Angiography: Basic Technique

• Black blood sequence: Single shot FSE with half fourier reconstruction (RARE,
HASTE)
➢ Evaluation of airway, vessel lumen
• Bright blood sequence: Steady-state free precession (FIESTA, FISP, true
FISP)
➢ Evaluation of shunts/jets due to dephasing associated with turbulent flow
• Contrast-enhanced MR angiography
➢ Evaluation of stenoses, bronchial collaterals, anomalous pulmonary veins
CEMRA depicts blood flow from PA to systemic arteries to

systemic veins
CT Angiography: Basic Protocol

• PE protocol
• Thin collimation (< 1mm)
• Fast table speed
• Reconstruct 1 to 2 mm intervals (3D)
• Low mAs and kVp
• Bolus tracking for scan initiation
• Trigger @ 100-120 HU
Position of the ROI: Over the Area of Interest

• Aorta and surgical shunts
➢ Ascending aorta
• Pulmonary artery
➢ Main PA or branches
• Pulmonary veins: LA
Reconstructions Increase diagnostic accuracy

• Multiplanar
• MIPs
• Volume Rendering
Which One is Best? MRA or CTA

• NO BEST ANSWER
➢ It depends on what you need to know
• Both accurate for anatomic detail
• MR >CT for functional information
• CT>MR for showing stents and calcifications
Applications for Thoracic MRA & CTA

• Congenital vascular anomalies
➢ Aorta
➢ Pulmonary vessels
➢ Vena cava
• Systemic diseases
➢ Marfan disease
➢ Kawasaki disease
Pediatric Radiology 1453 Vascular Anomalies

Aortic Arch Anomalies Figure 6-13-1
• Symptomatic lesions
➢ Vascular rings
❖ Right arch with aberrant Lt SCA
❖ Double arch
Anomalous innominate artery
• Asymptomatic lesions
➢ Left arch with aberrant Rt SCA
➢ Cervical arch
Vascular RingsDouble Aortic Arch

• Right arch is dominant
• Left arch may be patent or atretic
• Complete ring
• Clue: 2 arches Double aortic arch. Transverse CT scans showing two arches.
➢ 4 artery sign (no brachiocephalic The right arch is larger and more superior than the left. The two
artery) arches unite posterior to the esophagus and a single aorta
descends on the left
Double Arch
• 6 month old boy with cough
Double Arch: Patent Left Limb [Figure 6-13-1] Figure 6-13-2

CTA: Double Aortic Arch
Double Arch
Infant with wheezing
MRA: Double Arch
Double Arch Atretic Segment [Figure 6-13-2]
Double Arch Atretic Segment

• Look at the Airway
Double aortic arch with hypoplastic left segment
Right Aortic Arch: (arrow)
Aberrant left subclavian artery
• True ring-completed by ligamentum d. arteriosum
• Left SCA is last vessel off aorta
• Encircles trachea & esophagus Figure 6-13-3
Right aortic arch
Right Arch Aberrant Left SCA
Right Arch Aberrant Left SCA [Figure 6-13-3]

• 6 wk old boy with CHD
MRA: Right arch/aberrant LT SCA

• GRE MR
• CE MRA
Mirror Image Right Arch

• Mirror image branching
Right aortic arch with aberrant left subclavian
➢ Asymptomatic
artery, CT The aberrant subclavian artery courses
➢ Not a true vascular ring
behind the esophagus and trachea. LCCA=left
➢ Congenital heart disease (98%)
common carotid artery; RCCA=right common
❖ Tetralogy of Fallot
carotid artery; RSA=Right subclavian artery;
❖ Truncus arteriosus
LSA=left subclavian artery
Vascular Anomalies 1454 Pediatric Radiology

Mirror Image Right Arch [Figure 6-13-4] Figure 6-13-4
• Tetralogy of Fallot
Innominate Artery Compression

• Anterior tracheal compression by the
right innominate artery
• Symptoms--respiratory obstruction,
repeated infection, stridor and on
occasion respiratory arrest Mirror image right aortic arch. Transverse CT scans showing a
right sided aortic arch without a posterior crossing vessel
Innominate Artery Compression
[Figure 6-13-5]
Pulmonary Arterial Anomalies

• Agenesis (interruption) of main PA
• Pulmonary sling
• Ductus arteriosus
Interrupted Pulmonary Artery

• Rt or Lt PA is congenitally absent
• Interrupted 1 cm beyond origin
• Leads to increased systemic blood flow (collateral circulation) to affected
hemithorax Figure 6-13-5
• Affected lung absent or hypoplastic
➢ Growth dependent on collateral supply

Hypoplastic Lung [Figure 6-13-6]
• Bronchial collaterals
Absent Pulmonary Artery

Absence of lung
Pulmonary Sling
• Created by anomalous course of left PA
➢ Arises from right pulmonary artery & crosses between trachea
& esophagus
• Usually symptomatic in children, dyspnea
Figure 6-13-6
Innominate artery compression.

Transverse and sagittal reconstruction
CT showing anterior compression of
the trachea by the right innominate
artery (arrow)
Interrupted pulmonary artery. Transverse CT

showing the right pulmonary artery (arrow)
abruptly interrupted about 1 cm beyond its origin.
Right lung is hypoplastic. Coronal reformation CT
showing fibrofatty tissue filling a small right
hemithorax

Pulmonary Sling Figure 6-13-7
3 month old girl [Figure 6-13-7]
Pulmonary Sling
MRA: Pulmonary Sling
Patent Ductus Arteriosus

• Tubular connection between proximal descending aorta and left
pulmonary artery
• Isolated lesion or associated with other anomalies
Patent Ductus Arteriosus [Figure 6-13-8]
Ductus
• Calcified ductus
Figure 6-13-8
Pulmonary sling. Chest radiograph
(upper panel) showing tracheal
compression. CTA (upper right panel)
showing the left pulmonary artery
(LPA) arising from the right pulmonary
artery (RPA) and crossing behind the
trachea to reach the left hilum. 3D
rendering of the airway (lower panel)
confirming right-sided tracheal
compression
Figure 6-13-9
Patent ductus arteriosus, CTA. Multiple

transverse CT scans showing the patent ductus
(arrow) connecting the proximal descending aorta
and pulmonary artery
Patent Ductus: MRI [Figure 6-13-9]
Pulmonary Venous Anomalies

• Anomalous return
➢ Anomalous drainage to systemic veins
➢ Anomalous drainage to left atrium
Patent ductus arteriosus, MRA. Transverse MR
Partial Anomalous Return images and 3D sagittal reconstruction showing the
patent ductus (arrow) connecting the proximal
Partial Anomalous Venous Return descending aorta and pulmonary artery
• All PAPVR are LT to RT shunts but the shunt is
usually clinically insignificant
• Some present with pulmonary hypertension

RUL Anomalous Return to SVC Figure 6-13-10
MRA: Anomalous RUL pulmonary vein

[Figure 6-13-10]
RUL PAPVR
• 90% have venosum ASD
RLL Anomalous Return

Transverse MR (left image) showing anomalous
RLL Anomalous Return [Figure 6-13-11] right upper lobe pulmonary vein (arrow) draining
• MIP CTA into the superior vena cava. T1-weighted image
• GRE MR (right image) showing associated sinus venosus
atrial septal defect (arrow)
Scimitar Syndrome
• Hypogenetic lung syndrome, pulmonary venolobar syndrome
• Vein draining RLL enters IVC, portal vein/ hepatic vein, or right atrium
• Hypoplastic lung
• Small pulmonary artery
Figure 6-13-11
Scimitar syndrome
• 10 year old girl
• Pneumonia suspected
Scimitar syndrome
Scimitar Syndrome [Figure 6-13-12]
Figure 6-13-12
Right lower lobe anomalous
pulmonary venous return. Coronal
CT showing right lower lobe vein
draining into the inferior vena cava
Scimitar syndrome. Anomalous return of the right lower lobe is

associated with a hypoplastic right lung
LUL Anomalous Return [Figure 6-13-13]

Figure 6-13-13
Anomalous left upper lobe pulmonary

venous return. Transverse CT images
showing the left upper lobe pulmonary vein
(arrow) coursing next to the aorta and
passing adjacent to the left pulmonary
artery. There is no vessel noted at the level
of the coronary sinus which helps to
differentiate this condition from left superior
vena cava

LUL Anomalous Return
Figure 6-13-14
Anomalous LUL Vein [Figure 6-13-14]
Systemic Venous Anomalies

• Systemic
➢ Persistent left superior vena cava
➢ Interrupted inferior vena cava
Left (Double) Superior Vena Cava

• 0.5% of general population
• 5% CHD patients
• Drains subclavian vein
• Empties into coronary sinus
• Small Rt SVC, 90% of cases
Anomalous left upper lobe venous return. Axial
Left Superior Vena Cava MR images showing the left upper lobe pulmonary
[Figures 6-13-15 and 6-13-16] vein (arrow) coursing adjacent to the aorta and
pulmonary artery. Coronal contrast enhanced
Figure 6-13-15 MRA (right panel) showing the anomalous left
upper lobe vein (arrow) draining into the
brachiocephalic vein. Also noted is anomalous
drainage of the right upper lobe vein (arrowhead)
into the superior vena cava
Figure 6-13-16
Left superior vena cava. Transverse images
showing an enhancing left sided cava (arrow) and
a smaller right superior vena cava. The left
superior vena cava courses past the aortic arch
and left pulmonary hilum to enter into a dilated
coronary sinus
Left paramediastinal structures
• Left superior intercostal vein
• Anomalous LUL venous return
Another Lt-sided Vessel

Superior Lt Intercostal Vein
• Superior intercostal vein
➢ Drains left 2nd-4th intercostal spaces
Left superior vena cava. Coronal and sagittal
➢ Opens into brachinocephalic vein
multiplanar CT reconstructions showing left
➢ Joins accessory hemiazygous vein
superior vena cava (arrows) draining into the
➢ Aortic nipple shadow on CXR
coronary sinus
Left Superior Intercostal Vein [Figures 6-13-17 and 6-13-18]

Left superior intercostal vein creating aortic nipple

shadow (arrow)
Summary: Left Paramediastinal Structures Left superior intercostal vein. The vein (arrow)
• Left superior vena cava courses adjacent to the aortic arch and connects
➢ Subclavian vein to coronary sinus with the accessory hemiazygous vein inferiorly
• Anomalous LUL pulmonary vein
➢ Left pulmonary hilum to BCV
• Left superior intercostal vein Figure 6-13-19
➢ BCV to accessory hemiazygos vein
Review: Paramediastinal Veins [Figure 6-13-19]
Right paramediastinal structures
• Azygous vein
➢ joins hemiazygos
➢ ascends on right
➢ drains into SVC Summary: Left image, left superior vena cava.
• Superior intercostal vein Middle image, anomalous left upper lobe
➢ joins azygos vein pulmonary venous return. Right image, superior
➢ drains 2nd-4th intercostal veins left intercostal vein
Right Azygous System Figure 6-13-20
Azygous Vein [Figure 6-13-20]
Right Superior Intercostal Vein [Figure 6-13-21]
Figure 6-13-21
Normal azygous vein (arrow) draining into superior

vena cava (SVC).
Right superior intercostal vein. CT scans

showing the right superior intercostal vein
(arrow) draining into the azygous vein

Azygos Continuation IVC Figure 6-13-22
• Absence of infrahepatic segment of IVC
• Blood from lower half of body returns to heart via
dilated azygous and hemiazygous veins
• Isolated condition or associated with CHD
Azygos Continuation IVC [Figure 6-13-22]
Dilated Azygous Vein: Azygous continuation of the inferior vena cava.

Differential Diagnoses CT scans showing dilated azygous and
• Azygous continuation IVC hemiazygous veins and absence of the
• Congestive heart failure infrahepatic inferior vena cava
• Constrictve pericarditis
• Acquired obstruction SVC or IVC
• Pericardial effusion
• Tricuspid insufficiency
Figure 6-13-23
Acquired Vascular Lesions
• Aortic aneurysm & dissection
➢ Marfan syndrome
• Coronary artery aneurysms
➢ Kawasaki disease
Kawasaki Disease
• Aka Mucocutaneous lymph node syndrome
• Clinical features Coronary artery aneurysm, Kawasaki disease. CT
➢ fever of a neonate (left panel) showing dilated left
➢ rash anterior descending and circumflex arteries
➢ conjunctivitis (arrows) . CT scan of an adolescent girl (right
➢ erythema of lips & buccal mucosa panel) showing calcified aneurysm (arrow) of the
➢ myocarditis left coronary artery
➢ coronary artery aneurysms
Coronary Artery Aneurysms

• Arise in proximal part of arteries
• Fusiform, saccular, or cylindrical
Coronary Artery Aneurysms-Kawasaki [Figure 6-13-23]
Marfan Disease
• Inherited connective tissue disorder transmitted as an autosomal dominant
trait
• Localized to a mutation in chromosome 15
• Cardinal features
➢ tall stature
➢ slender limbs and fingers
➢ ectopia lentis
➢ pectus excavatum
➢ scoliosis
Common Cardiovascular Findings

• Aortic-root dilatation
➢ involves sinuses of Valsalva
➢ prevalence 70%-80%
• Aortic dissection
➢ Ascending >> descending aorta
• Mitral valve prolapse (55%-69%)

Marfan Disease Aortic Aneurysm & Dissection Figure 6-13-24
• 15 year old boy with Marfan’s syndrome
• Dilated aortic root
Marfan Disease: Dissection
Marfan Disease [Figure 6-13-24]
Review: Top 10 Vascular Lesions

• Double aortic arch
• Right arch with anomalous SCA
• Absent pulmonary artery
• Pulmonary sling
• Patent ductus arteriosus
• Anomalous pulmonary venous return
• Double SVC
• Interrupted IVC
• Aortic aneurysm & dissection--Marfan
• Coronary artery aneurysm-- Kawasaki Marfan disease, aortic aneurysm and dissection.
CT showing a dilated ascending aorta (A, top left
image) and a dissection of the descending aorta
(arrows)
References
1. Katz M, Konen E, Rozenman, et al. Spiral CT and 3D image reconstruction of vascular rings and associated
tracheobronchial anomalies J Comput Assist Tomogr 1995; 19:564-568.
2. Lee Ed, Siegel MJ, Hildebolt CF, Gutierrez FR, Bhalla S, Fallah JH. Multidetector CT Evaluation of Pediatric
Thoracic Aortic Anomalies: Comparison of Axial, Multiplanar, and Three-Dimensional Images. AJR 2004;
182:777-784
3. Lawler LP, Fishman EK. Multi-detector row CT of thoracic disease with emphasis on 3D volume rendering and
CT angiography. RadioGraphics 2001; 21: 1257-1273
4. Remy-Jardin M, Remy J, Mayo JR, Muller NL. Thoracic aorta. In: CT Angiography of the Chest. Lippincott
Williams & Wilkins. Philadelphia. 2001; 29-50.
5. Czum JM, Corse WR, Ho VB. MR angiography of the thoracic aorta. Magn Reson Clin N Am 2005; 13:41-64.
6. Choe YH, Kim YM, Han BK, Park KG, Lee HJ. MR Imaging In the morphologic diagnosis of congenital heart
disease. RadioGraphics 1997; 17:403-422.
7. Ho VS, Corse WR, Hood MN, Rowedder AM. MRA of the thoracic vessels. Semin Ultrasound CT MR 204:192-
216.
8. Gilkeson RC, Ciancibello L, Zahka K. Multidetector CT evaluation of congenital heart disease in pediatric and
adult patients. AJR 2003; 180:973-980.
9. Gup HW, Park I-S, Ko JK, et al. CT of congenital heart disease: normal anatomy and typical pathologic
conditions. Radiographics 2003; 23: S147-165.
10. Hopkins KL, Patrick LE, Simoneaux SF, et al. Pediatric great vessel anomalies: initial clinical experience with
spiral CT angiography. Radiology 1996; 200:811-815.
11. Stella VB, Toutouzas P. Patent arterial duct and aortopulmonary window. In: Gatzoulis MA, Wevbb GD,
Daubeney PEF. Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh 2003; 247-252.
12. Morgan-Hughes GJ, Marshall AJ, Roobottom C. Morphologic assessment of patent ductus arteriosus in adults
using retrospectively ECG-gated multidetector CT. AJR 2003; 181:749-754.
13. Mahnken AH, Wildberger JE, Spuntrup E, et al. Unilateral absence of the left pulmonary artery associated with
coronary-to-bronchial artery anastomosis. J Thorac Imaging 2000; 15:187-190;
14. Gupta H, Mayo-Smith WW, Mainiero MB, Dupuy DE, Abbott GF. Helical CT of pulmonary vascular
abnormalities. AJR 2002; 178: 487-492.
15. Park HS, Im JG, Jung JW, et al. Anomalous left pulmonary artery with complete cartilaginous ring. J Comput
Assist Tomogr 1997; 21:478-480.
16. Zwetsch B, Wicky S, Meuli R et al. Three-dimensional image reconstruction of partial anomalous pulmonary
venous return to the superior vena cava. Chest 1995; 108:1743-1735,
17. Dillon EH, Camputaro C. Partial anomalous pulmonary venous drainage of the left upper lobe vs duplication of
the superior vena cava: distinction based on CT findings. AJR 1993;160: 375-379.
18. Van Praagh S, Carrera ME, Sanders S, Mayer JE, Van Praagh R. Partial or total direct pulmonary venous drainage
to the right atrium due to malposition of septum primum. Chest 1995; 107:1488-1498.

19. Woodring JH, Howard TS, Kanga JF. Congenital pulmonary venolobar syndrome revisited. Radiographics 1994;
14:349-369.
20. Remy-Jardin M, Remy J, Mayo JR, Muller NL. Superior vena cava syndromes. In: CT Angiography of the Chest.
Lippincott Williams & Wilkins. Philadelphia. 2001; 130-139
21. White CS, Blaffa JM, Haney PH, Pace ME, Campbell AB. MR imaging of congenital anomalies of the thoracic
veins. RadioGaphics 1997; 17:595-608.
22. Bass JE, Redqine MD, Kramer LA, Huynh PT, Harris JH. Spectrum of congenital anomalies of the inferior vena
cava: cross-sectional imaging findings. Radiographics 2000; 20:639-652.
23. Yamada I, Nakagawa T, Himeno Y, Numano F, Shibuya H. Takayasu arteritis.: evaluation of the thoracic aorta
with CT angiography. Radiology 1998; 209:103-109

Pediatric Cardiac Imaging Part II:
Lecture Outline
• Review CT & MRI techniques
• Describe CT and MR appearances of common congenital heart diseases
➢ Neonates and infants
➢ Older children & adolescents
• Discuss treatment options
Techniques: Cardiac CTA/MRA

• MRA: Same as mediastinal vascular lesions (see part I), but add cine
sequence
➢ Black blood: delineation of anatomic structures and vessel lumens
➢ White blood: evaluation of shunts/jets as turbulent flow causes dephasing
➢ Gd for vessels: vessel stenoses
➢ Cine: function & flow dynamics
CT Angiography: Basic Protocol

• Use a PE protocol
• Thin collimation (< 1mm)
• Fast table speed
• Low mAs and kVp
• Bolus tracking for scan initiation
➢ Trigger @ 100-120 HU
Pediatric Cardiac Imaging: What you need to know

• Neonates & Infants:
➢ Top Ten Diagnoses
➢ CT & MRI Features
Top 10 Congenital Heart Diseases Neonates & Infants

SHUNTS OBSTRUCTIVE CYANOTIC HEART LESIONS
VSD Coarctation Tet of Fallot
ASD HLHS TGV
PDA TAPVR*
AV Canal Tricuspid atresia*
Truncus
* Adds to 11 because TA &TAPVR have similar frequency
I. The Shunt Lesions

• Atrial septal defect
• Ventricular septal defect
• Patent ductus arteriosus
• Atrioventricular canal
• (chest x-ray: cardiomegaly & increased vascularity
Atrial Septal Defects

• Sinus venosus (10%)
➢ Level of SVC
➢ associated with PAPVR
• Secundum (60%)
➢ Level of fossa ovalis
• Primum (30%)
• Lower atrial septum
➢ Part of AV canal defect
Pediatric Radiology 1463 Congenital Heart Disease

Sinus Venosus ASD [Figure 6-14-1] Figure 6-14-1
Sinus Venosus ASD: MRI
Secundum ASD
• Large PAs
• Mid septal defect
Secundum ASD [Figure 6-14-2]
Ostium Primum ASD

• Low septal defect Sinus venosus ASD. CT scan at level of superior
➢ Just above AV valves vena cava (left panel) shows anomalous right
• Two types upper lobe vein (arrow) draining into superior vena
➢ Partial: involves atrial septum & mitral valve cava. CT scan more caudal (right panel) shows
➢ Complete: involves atrial & ventricular septums & sinus venosus ASD (arrow)
both AV valves
❖ Aka AV canal Figure 6-14-2
Ostium Primum ASD: CT/MR
• Partial primum ASD
➢ Low ASD + mitral insufficiency
• AV canal
➢ Low ASD, high VSD + common AV valve
Complete Atrioventricular Septal Defect [Figure 6-14-3]
ASD: Overview [Figure 6-14-4]

ASD Repair
• Occluder devices Secundum ASD (arrow). Atrial septal
➢ Small secundum lesions defect (arrow) is at the level of fossa
➢ Amplatzer or CardioSeal ovalis and aortic valve. The right
• Surgical repair atrium is enlarged
➢ Large Secundum ASD
➢ Sinus venosus
➢ Primum ASD
Figure 6-14-3
Repaired ASD
• Occluder device for small secundum
ASD
Complete atrioventricular septal defect (aka AV canal and

endocardial cushion defect). CT scan and MRI show a low
atrial septal defect (ASD) and high ventricular septal defect
(VSD). In this lesion, there is a common atrioventricular valve
Figure 6-14-4
Summary. Sinus venosus ASD (left panel). Secundum ASD

(middle panel). Primum ASD (right panel)
Congenital Heart Disease 1464 Pediatric Radiology

Occluder Devices [Figure 6-14-5] Figure 6-14-5
Repaired ASD
• Septal patch for large secundum ASD & venosus &
primum ASD
Ventricular Septal Defects

• Most common CHD
• Locations
➢ Sub-aortic (80%)
❖ (perimembranous)
➢ Intramuscular)
➢ Sub-pulmonic
➢ RV inlet (associated with AV canal) Amplatzer occluder for smaller ASD. CT scans
showing two disks connected by a short neck in
Approximate Location of VSDs the location of the atrial septum. RA=right atrium,
LA=left atrium, PA=dilated pulmonary artery
Subaortic VSD [Figure 6-14-6]
Figure 6-14-6
Muscular VSD [Figure 6-14-7]
Subpulmonic ( supracristal) VSD

[Figure 6-14-8]
Overview VSD [Figure 6-14-9]

• VSD
• Subaortic Most Common
• Muscular Multiple
• RV Inlet With canal
• Subpulmonic TOF Subaortic (perimembranous) ASD (arrow). CT and MR showing
the ventricular septal defect at the level of the aortic valve (A)
VSD-Treatment
• Small lesions may close spontaneously 30% to 40% Figure 6-14-7
• Small lesions that fail to close are occluded with septal occluder device
• Large lesions closed with patch graft

• Tubular connection between proximal descending aorta and left PA
• Isolated lesion or associated with other anomalies
Figure 6-14-8
Intramuscular VSD (arrow).
The defect is within the
interventricular septum
Figure 6-14-9
Subpulmonic VSD. MR
showing the septal defect Summary. Subaortic VSD (far left panel).
(arrow) at the level of the right Muscular VSD (middle left panel). Ostium
ventricular outlet in the primum (inlet) VSD (middle right
supracristal area panel).Subpulmonic VSD (far right panel)

PDA [Figure 6-14-10] Figure 6-14-10
II. More Top 10 Obstructive Lesions

• Coarctation of the aorta
• Hypoplastic left heart
• (X-ray: cardiomegaly & edema)
Obstructive Lesions Aortic Coarctation

• Post-ductal
➢ Distal to left SCA
➢ Normal diameter arch
➢ Collaterals common
• Pre-ductal
➢ Above left SCA
➢ Hypoplastic arch
Post-Ductal Coarctation-CT Patent ductus arteriosus.

• Clues: dilated ascending aorta, post-stenotic dilatation & collaterals Sagittal MRA showing
patent ductus between
Post-ductal Coarctation-CTA [Figure 6-14-11] aorta and left pulmonary
artery
MR: Postductal Coarctation
Figure 6-14-11
Preductal Coarctation-CT [Figure 6-14-12]
• Pre-ductal
➢ Above left SCA
➢ Arch hypoplasia
➢ Collaterals uncommon
Coarctation Repair
• Resection & end-to-end anastomosis
• Stents, angioplasty, patch aortoplasty
After balloon dilatation
Complications Coarctation Repair (5-30%)

• Complications
➢ Re-stenosis
➢ Stent fracture
➢ Pseudo-aneurysm
❖ 5% to 12% angioplasty
❖ 33% patch aortoplasty Figure 6-14-12
Stent Restenosis
Post-op Complication: Pseudoaneurysm
Hypoplastic left heart syndrome

• Presents as CHF in neonate
• Classic findings
➢ Small or absent LV
➢ Hypoplastic ascending aorta Postductal coarctation,
➢ Hypoplasia aortic & mitral valves neonate. Transverse CT
➢ ASD & PDA (upper left image) shows
a small caliber
descending aorta
Preductal coarctation, (arrow). Sagittal CT
neonate with heart (middle and right
failure. Sagittal CT images) show the level
showing coarctation of obstruction (arrow)
(arrow) above origin of just below origin of left
left subclavian artery subclavian artery

Hypoplastic Left Heart [Figure 6-14-13]
Part III.
Common Cyanotic Diseases
SHUNTS OBSTRUCTIVE CYANOTIC HEART LESIONS
VSD Coarctation
ASD HLHS Tet of Fallot
PDA Tricuspid Atresia
AV Canal TGV
Truncus
TAPVR Figure 6-14-13
Cyanotic CHD
• Indicates that unoxygenated venous
blood is reaching aorta
• Causes:
➢ Right heart obstruction with Rt to Lt
shunting via a septation defect
❖ TOF, tricuspid atresia
➢ Mixing of pulmonary & systemic
blood due to incomplete separation Hypoplastic left heart syndrome. Transverse CT (left image)
of chambers showing small left ventricle (arrow). CT (right image) at a more
❖ TGV, Truncus, TAPVR proximal level shows small ascending aorta (arrow)
Tetralogy of Fallot: Clues: 4 findings

• The Tetrad: Figure 6-14-14
➢ Subaortic VSD
➢ Infundibular pulmonic stenosis
➢ Overriding aorta
➢ Right ventricular hypertrophy
Tetralogy of Fallot: CTA

• Membranous VSD, RVH, infundibular
PS, overriding aorta
Tetralogy of Fallot-MR [Figure 6-14-14] Tetralogy of Fallot, MRI. Transverse MR (left panel) shows
perimembranous VSD (arrow) and right ventricular
Surgical Repair TOF [Figure 6-14-15] hypertrophy (RVH). Sagittal MR (middle panel) shows
• Initial surgery is palliative narrowed pulmonary outflow tract (arrow). (PA=normal size
➢ Blalock-taussig shunt main pulmonary artery). Sagittal MR (right panel) shows aorta
➢ Subclavian artery to PA (Ao) overriding right and left ventricles, VSD (arrow), and right
ventricular hypertrophy
Figure 6-14-15
Blalock-Taussig shunt for palliation of

tetralogy of Fallot. 3D volume
rendered CT showing subclavian
artery (SCA) to pulmonary artery (PA)
anastomosis (arrow)

Surgical Repair TOF [Figure 6-14-16] Figure 6-14-16
• Definitive repair
➢ enlargement of PA via patch graft of pulmonary
valve annulus or outflow tract
➢ closure of VSD
Tricuspid Atresia [Figure 6-14-17]

• Fatty bar between RA & RV
• Hypoplastic RV
• Large RA
• VSD
Definitive repair of tetralogy of Fallot. Transverse
Tricuspid Atresia CT (left image) at level of pulmonary artery shows
• RAE, fatty tricuspid valve, small RV a normal caliber main pulmonary artery with
adjacent graft material. CT at level of ventricles
Tricuspid Atresia: Surgical Repair [Figure 6-14-18] (right image) shows closure of VSD
• Glen shunt
➢ SVC to PA
Figure 6-14-17
• Fontan
➢ RA to PA
Total Cavopulmonary Fontan [Figure 6-14-19]

• Cavopulmonary Fontan
➢ Conduit between SVC & IVC which is joined to main PA
Lesions with Increased Flow

D-Transposition of Great Vessels
• Ventriculoarterial discordance
• Aorta arises from RV
• Pulmonary artery from LV
• Circuits are in parallel
• ASD, VSD, PDA common
Tricuspid atresia classic features. CT
showing atretic valve replaced by
fatty tissue (arrow), right atrial (RA)
enlargement, and right ventricle
hypoplasia
Figure 6-14-18
Figure 6-14-19
Surgical repairs for tricuspid atresia. Glenn shunt

(left panel) is a superior vena cava (S) to
pulmonary artery anastamosis. Fontan proceudre
(right panel) is a right atrium (RA) to main
pulmonary artery anastomosis. Note the markedly
dilated right atrium.
(Left panel, Glenn shunt, reprinted from Core
Curriculum, Siegel M, Coley B 2005).
Total cavopulmonary shunt. Superior
vena cava (SVC) and inferior vena
cava (IVC) joined via conduit (C),
which is anastomosed to pulmonary
artery (PA)

D-Loop Transposition [Figure 6-14-20] Figure 6-14-20
Surgical Repair: Atrial Switch

• Intra-atrial baffle
• Blood from SVC and IVC enters superior chamber &
directed to LV and PA
• Blood from pulmonary veins enters inferior chamber
& directed to RV & Aorta
Atrial Switch [Figure 6-14-21]
Mustard Procedure
• Systemic venous baffle
• Pulmonary venous baffle
D-Transposition of great vessels. Sagittal MRA
Jantene Procedure (left panel) showing aorta arising from right
• Current procedure of choice ventricle (RV) and pulmonary artery from left
• Great artery switch ventricle (LV). Transverse MRI and CT showing
• Aorta and PA sectioned above valves & reconnected aorta (A) anterior and to right of pulmonary artery
to proper ventricles (PA)
• Coronary arteries reimplanted
Figure 6-14-21
Arterial Switch: Jatene Procedure [Figure 6-14-22]
Total Anomalous PV Return

• Pulmonary veins drain to RA (not LA)
• 4 types
➢ I. supracardiac (55%)
➢ II. cardiac (coronary sinus) (30%)
➢ III. infracardiac (to portal vein or IVC) (12%)
➢ IV. two of the above (3%)
• ASD essential for survival
• Repair: reimplant
Total Anomalous Return: Surgery

• Veins reanastomosed to LA
Truncus Arteriosus
• I. Single PA arises from truncus (80%)
• II. R and L PA arise from posterior truncus
• III. R and L PA arise from sides of truncus. Atrial switch, mustard
• IV. R and L PA arise from descending aorta procedure. Coronal CT
(upper image) shows
systemic baffle
directing blood from
right heart to left heart
Figure 6-14-22 where it exits into
pulmonary arteries.
Transverse CT (lower
image) shows systemic
baffle and also
pulmonary venous (PV)
baffle which directs
blood from pulmonary
veins into right ventricle
where it exits into aorta
Jatene procedure, arterial switch. Pulmonary artery (PA) lies

anterior and to right of aorta (A)

Truncus Arteriosus Type I [Figure 6-14-23] Figure 6-14-23
Type IV Truncus (pseudotruncus)

[Figure 6-14-24]
Truncus: Surgical Repair

• Pulmonary arteries detached from common artery
(truncus arteriosus) and connected to RV using a
conduit.
• VSD is closed with a patch
Additional Lesions: Older Children & Truncus arteriosus. Type I A single pulmonary
Adolescents artery arises from the truncus (T)
• Aortic stenosis
• Pulmonic stenosis
Figure 6-14-24
Aortic Stenosis
• Valvular >> sub- or supravalvular
• Usually due to bicuspid valve
• CT/MR: dilated ascending aorta
➢ Bicuspid valve
Aortic Stenosis-CT [Figure 6-14-25]
Bicuspid Aortic Valve

• Ca++ uncommon before 4th decade
Truncus arteriosus type IV. Oblique MRA showing
AS and Bicuspid Valve-MR both pulmonary arteries arise from the descending
• Thickened, domed leaflets during systole aorta. Transverse MR showing an associated
• Thick wall left ventricle VSD (arrow)
Aortic Stenosis: Treatment Figure 6-14-25

• Balloon dilatation
• Valvotomy
• Valve replacement
• Ross procedure
➢ aortic valve replaced with patient’s pulmonary
valve
➢ pulmonary valve replaced with cadaveric valve
Valvular Pulmonic Stenosis

• Valvular stenosis most common (95%)
➢ 90% due to commissural fusion
➢ 10% due to a dysplastic valve
❖ thickened, but non-fused commissures
Aortic stenosis. Transverse and coronal CT
Pulmonary Artery Stenosis [Figure 6-14-26] showing dilated ascending aorta
• CT/MR
➢ Dilated main PA Figure 6-14-26
➢ Dilated Lt PA
➢ Rt ventricular hypertrophy
Pulmonic valve stenosis. CT

showing dilated main (M) & left
(L) pulmonary arteries

Pulmonic Stenosis: Repair
• Surgical valvotomy
• Percutaneous balloon valvuloplasty
• Less successful in patients with valvular dysplasia
Summary
• Diagnosis of CHD depends on knowledge of the anatomic abnormality, the clinical findings &
understanding of imaging findings
References
1. Amplatz K, Moller JH Radiology of Congenital Heart Disease Mosby –Year Book Inc 1993
2. Boxt LM. MR imaging of congenital heart disease. Magn Reson Imaging Clin North America;1996:4:327-359
3. Choe YH, Kim Ym, Han BK, Park KG, Lee HJ. MR imaging in the morphologic diagnosis of congenital heart
disease. RadioGraphics 1997; 17:403-422.
4. Poustchi-Amin M, Gutierrez FR, Brown JJ, et al. How to plan and perform a cardiac MR imaging examination.
Radiol Clin North Am 2004; 42:497-514
5. Gilkeson RC, Ciancibello L, Zahka K. Multidetector CT evaluation of congenital heart disease in pediatric and
adult patients. AJR 2003; 180:973-980.
6. Goo HW, Park I-S, Ko J-K, et al. CT of congenital heart disease: normal anatomy and typical pathologic
conditions. Radiographics 2003; 23: S147-165
7. Gutierrez FR, Canter CE, Mirowitz SA: MR appearance of congenital heart defects. In: Gutierrez FR, Brown JJ,
Mirowitz SA (eds.): Cardiovascular magnetic resonance Imaging. St. Louis: Mosby Year Book, 1992;72-83.
8. Higgins CB: Congenital heart disease. In: Higgins CB, Hricak H, Helms CA (eds.): Magnetic Resonance Imaging
of the body. 3rd ed. Philadelphia: Lippincott-Raven, 1997; 461-518.
9. Lee E, Siegel MJ, Guttierez F, Hildebolt CF, Bhalla S, Fallah JH. Multidetector CT evaluation of thoracic aortic
anomalies in pediatric patients and young adults: comparison of thoracic axial, multiplanar, and 3D images. AJR
2004; 182:777-78410.
10. Kaemmerer H. Aortic coarctation and interrupted aortic arch. In: Gatzoulis MA, Wevbb GD, Daubeney PEF.
Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh 2003; 253-264.
11. Becker C, Soppa C, Fink U et al. Spiral CT angiography and 3D reconstruction in patients with aortic coarctation.
Eur Radiol 1997; 7:1473-1477.
12. Roest AA, Helbing WA, van der Wall EE. Postoperative evaluation of congenital heart disease by magnetic
resonance imaging. J Magn Res Imag 1999; 10:656-666.
13. Donnelly LF, et al. MR imaging of cono-truncal abnormalities. AJR 1996; 166:925-928.
14. Connelly M. Common arterial trunk. In: Gatzoulis MA, Webb GD, Daubeney PEF. Adult Congenital Heart
Disease. Churchill Livingstone. Edinburgh 2003; 265-271.
15. Jacobs ML. Congenital heart surgery nomenclature and database project: truncus arteriosus. Ann Thorac Surg
2000; 69:S50-S55.
16. Kim TH, et al. Helical CT angiography and three-dimensional reconstruction of total anomalous pulmonary
venous connections in neonates and infants. AJR 2000; 175: 1381-1386.
17. Bardo DM, et al. Hypoplastic left heart syndrome. Radiographic 2001; 21:705-717.
18. Mavroudis C, Backer CL, Deal BJ. Venous shunts and the Fontan circulation in adult congenital heart disease. In:
Gatzoulis MA, Wevbb GD, Daubeney PEF. Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh
2003; 79-83.
19. Feeedom RM, Li J, Yoo S-J. The complications following the Fontan operation. In: Gatzoulis MA, Wevbb GD,
Daubeney PEF. Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh 2003; 85-91.

Topics
• Assessing the chest film
• Acyanotic CHD with increased PBF
• Cyanotic CHD with increased PBF
• Cyanotic CHD with decreased PBF
Increased PBF Increased PBF Decreased PBF

ACYANOTIC CYANOTIC CYANOTIC
VSD TGV Tet of Fallot
ASD TAPVR Ebstein
PDA Truncus Tric Atresia
ECD Tric Atresia Pulm Atresia
AP Window Single Chamber
HLHS
Assessing the Chest Film

• Pulmonary blood flow (PBF) – KEY!!!
• Cardiomediastinal silhouette
➢ Configuration
➢ Size
• Aortic arch side
• Situs
Pulmonary Blood Flow

• Normal
• Increased (arterial overcirculation and venous congestion)
➢ Requires 2 : 1 (pulmonary : systemic) shunt to see increased PBF @ CXR
• Decreased
• Systemic collaterals (AKA bronchial or major aorticopulmonary collaterals)
Pulmonary Blood Flow Indicators

• Gestalt – too big, seen too far in periphery (look at hilar vessels – most
reliable and most technique - independent)
• Main pulmonary artery Figure 6-15-1
• Interlobar artery diameter = supraaortic tracheal diameter (+/– 2
mm)
• Bronchovascular couplet: diameter artery = diameter bronchus in
upper zones in “normal” patients (less than 2:1 shunt)
• Hepatic window – if vessels too large here or seen on end, there
is increased PBF
Chamber Assessment [Figure 6-15-1]

• RA: not reliably assessed by plain film & infrequently a diagnostic
discriminator
• RV: retrosternal space >1/3 filled
• LA: upper 1/2 of posterior cardiac border
• LV: lower 1/2 of posterior cardiac border
Does either or both:
• Touch spine?
• Extend beyond posterior tracheal line?
RV touches lower 1/3 of sternum; LA

is upper 1/2 and LV is lower 1/2 of
posterior cardiac border
Congenital Heart Disease 1470

Cardiomediastinal Silhouette Size
• Cardiothoracic ratio:
➢ Infant < .65
➢ Adult < .50
• Infant cardiomegaly:
➢ Apex touches lateral chest wall on AP
➢ Posterior border touches spine on lateral
Cardiomediastinal Silhouette Configuration

• Acyanotic CHD: usually normal shape
➢ Cardiomegaly common
• Cyanotic CHD: more often bizarre
➢ Absence of thymus - think cyanotic disease (stressed infant, agenesis)
Acyanotic CHD with Increased PBF

• Increased blood volume in lungs
• Can only occur in 1 of 2 ways: Figure 6-15-2
➢ Passive congestion: impeded pulmonary venous return
❖ Example: congestive heart failure
➢ overcirculation: too much blood delivered to lungs
❖ Example: ventricular septal defect
Acyanotic CHD with Increased PBF – shunts

[Figures 6-15-2 and 6-15-3]
• Unifying theme:
➢ Pressure difference R v. L
• Blood will preferentially flow thru an opening from
high pressure L to lower pressure R
• Blood is recirculated thru pulmonary bed (result: increased PBF)

( L to R shunts – INTRACARDIAC)
• Ventricular septal defect (VSD)
• Atrial septal defect (ASD)
• Patent ductus arteriosus (PDA)
• Endocardial cushion defect (ECD)
• Aorticopulmonary window (APW)
Pressures in infant cardiac chambers
(L to R shunts – EXTRACARDIAC)
• Vein of Galen aneurysm
• Hepatic hemangioendothelioma
Figure 6-15-3
• Peripheral AV fistula
Acyanotic CHD with Increased PBF(FAILURE)

• Hypoxic cardiac injury (birth asphyxia, drowning)
• Anomalous origin left coronary artery
• Sepsis
• Endocardial fibroelastosis
• Glycogen storage disease (Pompe)
• Viral myocarditis
• Adriamycin toxicity
Caveat
• In infants, all forms of increased PBF may lead to pulmonary
edema
• Cannot reliably distinguishing arterial overcirculation from passive
congestion
• Consider the 3 causes of increased PBF:
➢ Intracardiac shunts
➢ Extracardiac shunts
➢ Cardiac failure
VSD: blood flows from LV to RV

1473 Congenital Heart Disease
Ventricular Septal Defect (VSD) [Figure 6-15-3]
• Most common CHD (25%)
VSD: Hemodynamics
• Blood flows from LV to RV
• Volume overload in RV
• RV enlarges and hypertrophies
• Blood in RV goes to lungs, LA, LV.
• When it arrives in LV, it “decompresses” into RV
➢ LV remains normal
• 4 basic types
➢ Membranous 80%
➢ Muscular (often multiple) 10%
➢ Supracristal 5%
➢ Inflow / AV canal type 5%
➢ Size matters, not location!
Radiology [Figures 6-15-4 and 6-15-5]

• Large heart
• RV and LA large
VSD: Presentation
• Usually in 2nd month of life
➢ Pulmonary vascular resistance has dropped
sufficiently to allow increased shunting Figure 6-15-4
• Presentation
➢ Frequent pneumonias
➢ Failure to thrive
➢ CHF
VSD: Natural History

• Small: close spontaneously by 3 years
➢ Muscular ingrowth
➢ By 2 months of age: follow v. repair
• Large: repair via trans-atrial approach
➢ Goretex patch
➢ Pericardial patch VSD: cardiomegaly, large RV, and large LA
VSD
• All need to be closed
Figure 6-15-5
• Large:
➢ Significant L to R shunting
➢ Less turbulence
➢ Pulmonary HTN, Eisenmenger syndrome
• Small:
➢ Less L to R shunting
➢ More turbulence
➢ Higher risk of bacterial endocarditis
Atrial Septal Defect (ASD) [Figures 6-15-6 a and b]

• Incidence 10%
• 3 : 1 female : male MRI of VSD
• Most common shunt to present after the age of 3
• Size determines shunt
• Locations
➢ Ostium secundum (fossa ovalis) 60%
❖ Patent foramen ovale
➢ Ostium primum (part of cushion) 30%
➢ Sinus venosus (just below SVC, usually with partially anomalous pulm.
venous return from RUL)

ASD: Hemodynamics Figure 6-15-6a
• Blood shunts LA to RA to RV to lungs to LA
• RV & RA enlarge in response to increased volume
• L heart: no increased volume (blood from lungs returning to LA
decompresses into RA)
➢ LA, LV, and aorta are normal

• RV large; no L-sided chamber enlargement
Figure 6-15-6b
ASD locations: uppermost is
sinus venosus, middle is
ostium secundum, lowest is
ostium primum
ASD
Figure 6-15-7
ASD: Complications
• Pulmonary HTN & Eisenmenger syndrome
➢ 30% with mod-large ASD develop HTN
• Atrial arrhythmias (volume overload)
• Paradoxical emboli
ASD
ASD: R to L Shunting [Figure 6-15-9]
• “Paradoxical” emboli
• R to L shunting may occur: Figure 6-15-8
➢ Pregnancy (decreased SVR)
➢ Diving,valsalva, cough
➢ COPD, pulmonary embolus
Paradoxical Embolus
• Ischemic stroke: no cause in
35-40%
Figure 6-15-9
➢ “Cryptogenic stroke”
➢ Paradoxical embolus from
ASD?
• Pts with cryptogenic ischemic
stroke and PFO, on
anticoagulant therapy: ASD at MR
➢ 1.2% risk/year of TIA
➢ 3.4% risk/year of TIA or
stroke
R to L shunting
across septal defect

ASD Figure 6-15-10
• Repair all between 2-5 years
• Repair methods:
➢ Amplatzer occluder (secundum only)
❖ Repair mortality <1%
➢ Suture (small, any location)
➢ Patch (any location)
❖ Goretex
❖ Pericardium
Patent Ductus Arteriosus (PDA) [Figure 6-15-10]

• Incidence 8%
• In utero, allows fetal blood returning from placenta to bypass
underdeveloped lungs
• 2:1 F:M
• Birth:
➢ Lungs aerate, fluid leaves
➢ Pulmonary pressure drops
➢ Ductus closes by 15 hours
➢ All RV blood to lungs
➢ Normal PBF
• Ductus may remain open
Patent ductus arteriosus
➢ High pulmonary pressures
❖ Prematurity
❖ Meconium aspiration
❖ Pulm hypoplasia (diaph hernia,oligo, ARPKD)
➢ Idiopathic
PDA: shunt
• Shunt direction determined by:
➢ Pulmonary vs systemic pressure
• In otherwise normal infants, it will be L to R
Hemodynamics
• Shunt is from Ao to PA
• LA, LV, Ao: increased volume
• L heart enlarges
• RV: increased pressure from afterload; hypertrophy
Radiology [Figure 6-15-11]

• LA, LV, Ao arch large
• Some RVH
PDA
• Anatomically closed by 3 weeks
• If open @6 months, certainly will remain
• Flow depends on:
➢ Width
➢ Length Figure 6-15-11
➢ Shape

• L to R shunt: pulmonary HTN
• Bacterial endocarditis
➢ Turbulent flow
➢ Continuous flow
➢ 0.5%/year cumulative risk
➢ SBE prophylaxis until repair
PDA: increased PBF, enlargement of RV, LA, LV

Patent Ductus Arteriosus Figure 6-15-12
• Treatment:
➢ Small: coils
➢ Medium to large:
❖ Mesh occluder
➢ Neonatal
❖ Clip (any size ductus)
❖ Prostaglandin inhibitors: only for stable
neonates
❖ Arterial access limited by small size 1 2 3
• Tiny, incidental PDA: Rx controversial Spectrum of cushion defects:
• SBE prophylaxis if residual shunt 1-Normal
2-Moderate ECD: ASD & cleft AV valve leaflets
Endocardial Cushion Defect (ECD) / 3-Severe ECD: ASD, VSD, common AV valve
Atrioventricular Canal (AV Canal) [Figure 6-15-12]
• Incidence 4%
• Spectrum of lesions & severity: Figure 6-15-13
➢ Ostium primum ASD
➢ + / - common anterior and posterior mitral and
tricuspid valve leaflets
➢ + / - inlet VSD (most severe)
• Presentation/radiographic appearance depends
on severity
➢ Variable cardiomegaly and increased PBF
• 40% have Down syndrome (look for 11 or 13 rib
pairs, 6 sternal ossification centers)
➢ Most common cardiac lesion in Down
syndrome
ECD: increased PBF, RV and LA enlargement
• Chamber enlargement varies with nature of ECD Figure 6-15-14
➢ Cardiomegaly & increased PBF
• “Gooseneck” deformity at angio from LV outflow obstruction
Aorticopulmonary Window (APW) [Figures 6-15-15 and 6-15-16]

• Rare
• Communication between ascending aorta and pulmonary trunk or
RPA
• Large L to R shunt
• Predominantly L chamber enlargement
➢ Similar to PDA
Figure 6-15-15
ECD: gooseneck deformity of LV

Figure 6-15-16 outflow tract
Aorticopulmonary window AP window: increased PBF, RV, LA, & LV

enlargement

Eisenmenger Physiology [Figures 6-15-17 and 6-15-18] Figure 6-15-17
• L to R shunting creates increased volume in pulmonary arteries;
results in increased pressure
• Pulmonary arteries respond by undergoing hypertrophy &
endothelial thickening, creating pulmonary hypertension (a
vicious cycle) if not corrected (may begin in utero)
• Pulmonary pressure may become higher than systemic pressure
• Shunt switches from L to R to R to L
• Deoxygenated blood from R heart shunts to L heart and
systemic circulation Normal (left) and hypertensive (right)
• Cyanosis results (“cyanosis tardive”) pulmonary artery, showing
endoluminal narrowing
• “Pruned” appearance of PBF on CXR:
➢ Dilated central arteries Figure 6-15-18
➢ Diminutive peripheral arteries 1 2
• Irreversible course of events
• Goal of surgery is to prevent this dreaded complication
Figure 6-15-19
Eisenmenger syndrome in VSD:

1-VSD initially shunts L to R
2-With onset of pulmonary HTN,
blood begins to shunt R to L
Figure 6-15-20
ASD with Eisenmenger syndrome:

large hilar arteries, diminutive
peripheral vessels
Cyanotic CHD [Figures 6-15-21 and 6-15-22]

• Cyanosis: Hb saturation < 85%
• Deoxygenated Hb (blood from R heart)
has entered systemic (L heart) Figure 6-15-21
circulation
• This can only happen if there is . . .
➢ R to L shunt (from R-sided outlet
obstruction, such as Tet of Fallot,
Eisenmenger syndrome)
➢ Mixing of R & L sided blood in
Pulmonary artery banding
systemic circulation (eg: truncus reduces PBF, reducing risk of
arteriosus)-”admixture” lesion Eisenmenger syndrome
Deoxygenated blood from R

side enters L side, leading to
cyanosis

Admixture Lesions [Figure 6-15-23] Figure 6-15-22
• Mixing of R & L circulations, across a large VSD,
ASD, PDA, or a “single chamber” where there
usually are 2
• Admixed blood flows to pulmonary circulation
• These are the “cyanotic with increased PBF” lesions
Admixture Lesions
“5T’s” and H
• Transposition of the great vessels
• Total anom. pulmonary venous return
• Truncus arteriosus
• Tricuspid atresia
• Single chamber
➢ single ventricle R to L shunt lesions shunt PBF to L side of heart;
➢ double outlet right ventricle there is decreased PBF
➢ common atrium)
• Hypoplastic left heart syndrome Admixture lesions allow intracardiac mixing of R
and L circulations; blood preferentially flows to
lower pressure pulmonary vascular bed,
Complete Transposition of Great Vessels increased PBF results and patient is cyanotic
(d-TGV, D loop TGV) [Figure 6-15-24]
• Incidence 8%
• Most common cyanotic CHD presenting in neonate Figure 6-15-23
➢ TOF most common overall
• 2 : 1 male : female
• Classic TGV:
➢ Aorta arises from RV
➢ Pulm art from LV
• 2 closed circuits
➢ Incompatible with life w/o L-R communication
• All have ASD
• Half with VSD

• PA not border-forming on left
➢ Mediastinum appears narrow
➢ “Egg on a string” appearance
• Vast majority with L aortic arch
• Small to absent thymus (makes perception of narrow vascular
pedicle possible)
DORV: admixture lesion

Figure 6-15-24
Figure 6-15-25
D-TGV: cardiomegaly, narrow

mediastinum, increased PBF
D (classic) transposition

Surgical Repair [Figures 6-15-26 and 6-15-27] Figure 6-15-26 Figure 6-15-27
• Arterial switch (Jatene) – switching aorta &
PA to normal location
• Atrial septostomy (palliative) - opens ASD to
improve admixture
➢ Balloon septostomy = Rashkind
• Atrial switch/baffle (Mustard, Senning) -
systemic blood returns to LA, pulmonary
returns to RA
“D” and “L” Designations

[Figures 6-15-28 and 6-15-29]
• Refers to aortic or ventricular position
• “D” may refer to:
➢ Aortic position (D is ABNORMAL)
➢ Ventricular looping & position (D is
NORMAL) Jatene switch repair of Atrial baffle (Mustard
• d-TGV = D loop TGV = complete TGV TGV procedure) palliation of
TGV
• “L” designation may refer to
➢ Aorta to left and anterior to pulmonary
artery (not normal, but close) Figure 6-15-28 Figure 6-15-29
➢ Ventricular looping & position (L is
ABNORMAL)
Congenitally Corrected TGV (L-TGV

or L loop TGV)
• Ventricular inversion key abnormality
➢ There is great vessel transposition, too,
but...
• Blood circulatory pattern normal
• Radiograph: NORMAL
• High incidence associated anomalies
(responsible for morbidity)
➢ Ebstein-like changes in tricuspid valve
➢ VSD
➢ Pulmonary stenosis/atresia
• AV valves (tricuspid & mitral) go with the
D (classic) transposition of L-TGV (transposition of
inverted ventricles Ao & PA ventricles)
• Coronary arteries are anatomically inverted
as well
➢ RV (inverted LV) supplied by two coronary arteries
➢ LV (inverted RV) supplied by one Figure 6-15-30
• Increased mortality:
➢ Coronary artery disease (single vessel)
➢ Associated anomalies
Total Anomalous Pulmonary Venous Return (TAPVR) [Figure

6-15-30]
• Incidence 2 %
• PBF returns to RA
• Admixes with systemic return in RA
• MUST have communication with L heart
➢ All have ASD
➢ Admixed blood: R to L flow
TAPVR type 2 with return of

PBF to RA

Darling’s Classification of TAPVR : Type 1 (supracardiac) Figure 6-15-31
[Figure 6-15-31]
• 55%
• Pulmonary veins drain cephalad into L SVC (aka left vertical vein), into
left BCV, then into SVC (occasionally azygos)
• “Snowman” heart
➢ Almost half are obstructive (from bronchial compression, vein
stenosis)
Type 2 (cardiac) [Figure 6-15-32]

• 30%
• Pulmonary veins drain into coronary sinus or directly into RA
• Non-specific appearance (like any L to R shunt). Rarely obstructive
Type 3 (infracardiac)
[Figure 6-15-33]
• 12%
• Common pulmonary vein descends thru esophageal hiatus
• Drains into portal vein/ductus venosus, hepatic vein, or IVC
• Pulmonary venous return is always obstructed because: Type 1 (supracardiac) TAPVR;
➢ Long course of vein PBF returns to SVC
➢ Passage through diaphragm
➢ Return through hepatic parenchyma when draining into Figure 6-15-32
PV/ductus venosus (most common)
• Pulmonary edema
• Heart size is NORMAL
➢ Lungs act as capacitor for obstructed pulmonary venous
return
• Distinctive appearance: normal size heart with pulmonary edema
• Type 4 = combination lesion of some the above (5%)
• Partial APVR (clinically insignificant) [Figure 6-15-34]
➢ Most commonly is return of RUL pulmonary vein directly into
SVC
➢ Scimitar syndrome is PAPVR of hypoplastic RML, RLL; drains
to IVC
TAPVR: Variable Physiology

• Increased PBF
• Obstruction occurs in all type 3 and in 25 – 40% of type 1 (from
bronchial compression or intrahepatic drainage), which creates
pulmonary edema Type 2 TAPVR; PBF returns to RA
Figure 6-15-33
Figure 6-15-34
Type 3 (infracardiac) TAPVR;

PBF returns to portal vein, Partial APVR; PBF from 1-2
hepatic vein, or IVC lobes returns to RA

TAPVR 1: Radiology [Figures 6-15-35 and 6-15-36] Figure 6-15-35
• Cardiomegaly and increased PBF
• “Snowman” heart from prominent left vertical vein
and distended SVC
• Superior mediastinum looks wide and round
TAPVR 2: Radiology [Figure 6-15-37]

• Non-specific
• Looks like many L to R shunts (large heart with
increased PBF)
Figure 6-15-37
TAPVR 1: “Snowman” heart & increased PBF
Figure 6-15-36
TAPVR 2: increased PBF, non-specific

appearance
TAPVR 1: MRI of L SVC

TAPVR 3: Radiology [Figures 6-15-38 and 6-15-39] and large R SVC
• Always obstructive
➢ Pulmonary edema Figure 6-13-39
• Heart size normal (it is not seeing increased volume)
• Normal size heart + pulmonary edema = TAPVR III
Figure 6-15-38
TAPVR 3: normal size heart +

pulmonary edema + increased PBF
Type 3 (infracardiac) TAPVR:
Surgical Repair pulmonary venous return to portal
• Depends on type vein
• Aim is to patch-graft pulmonary vein to LA or create pulmonary
venous conduit to LA

Truncus Arteriosus [Figures 6-15-40 ]
• 1%
• Failure of septation of truncal artery into Ao & PA
• Single great artery arises from heart, via single valve
• Gives rise to Ao, PA, and coronary arteries
• Truncal artery overrides a VSD (may have ASD and/or PDA too)
• R aortic arch in 35%
• Collett & Edwards classification (anatomic)
➢ Type 1: PA arises as single main PA
➢ Type 2: PAs arise separately but close
➢ Type 3: PAs arise independently, with widely spaced origins
➢ Type 4: “PA” arise from descending Aorta (aka pseudotruncus)
❖ Really bronchial arteries
Figure 6-15-40
Type 1 truncus arteriosus: Type 2 truncus Type 3 truncus Pseudotruncus

single artery arises from (bronchial collaterals
RV & LV arise from descending
Ao)
• R aortic arch + increased PBF + cyanosis: truncus arteriosus likely Figure 6-15-41
• Always have large heart
• Resembles TGV
• Type 2 & 3 give “hilar comma” or “hilar waterfall” sign from
vertical course of pulmonary arteries descending to pulmonary
hilum
Repair [Figure 6-15-43]

• Rastelli: RV to PA Conduit
Figure 6-15-43
Truncus with R Ao arch

Figure 6-15-42
Truncus arteriosus: cardiomegaly +

Rastelli repair increased PBF

Tricuspid Atresia [Figure 6-15-44] Figure 6-15-44
• 1.5%
• Very variable
➢ Appearance
➢ Physiology
➢ Associated defects
• Blood flows RA to LA (across ASD) to LV to RV (across VSD) and
out PA; admixture occurs in LA
RV rudimentary
• Classified into 2 types: [Figure 6-15-45]
➢ With TGV (25%) (increased PBF)
➢ Without TGV (75%) (decreased PBF)
• Therefore, the great vessel served by RV is usually
underperfused. This would be the:
➢ Pulmonary artery in normally related great vessels (therefore
PBF is decreased)
➢ Aorta in TGV (therefore PBF is increased)
Figure 6-15-45
• Non-specific
Tricuspid atresia with normally related
• Increased or decreased PBF Ao & PA (decreased PBF)
depending on transposition
• Difficult dx to exclude!
Figure 6-15-46
Surgical Repair
[Figure 6-15-47]
• Correction with Fontan
(conduit between RA and
main PA and closure of VSD)
Single Ventricle/DORV/
Common Atrium
[Figure 6-15-48]
• All have single dominant
chamber (where normally
there are 2 separate L and R Tricuspid atresia with TGV:
chambers) into which all cardiomegaly + increased PBF
blood flows
• Admixture occurs in this Tricuspid atresia with transposition
single chamber of Ao & PA (increased PBF)
Figure 6-15-47
Figure 6-15-48
Single chamber lesions: single ventricle, double outlet right ventricle,

& common atrium Fontan repair of tricuspid atresia

Single Ventricle [Figures 6-15-49] Figure 6-15-49
• All blood returns to the atria and into
single ventricle (admixture occurs here),
and is pumped from this into the great
vessels
• May have increased or decreased PBF
depending on outflow tract obstructions
• Radiographic appearance hence very
variable
Double Outlet Right Ventricle

[Figure 6-15-50a]
• Origin of both great vessels from RV
• Usually with VSD Single ventricle Single ventricle: cardiomegaly +
• Admixture occurs in RV and admixed increased PBF
blood is pumped to systemic and pulmonary circulations
Single Ventricle vs DORV

[Figure 6-15-50b] Figure 6-15-50a Figure 6-15-50b
• To the patient, not much physiologic
difference
• Both have:
➢ Admixture of circulations in dominant
ventricle
➢ A functional single ventricle that
pumps admixed blood to both
pulmonary and systemic circulations
Common Atrium [Figures 6-15-51]

• Essentially huge ASD permitting
significant admixture between RA & LA
➢ No gradient L-R
Double outlet right
• Very uncommon lesion ventricle
• Large heart with increased PBF (non-
DORV: cardiomegaly + increased
specific) PBF
Figure 6-15-51
Figure 6-15-52
Common atrium (similar appearance to large ASD)
Hypoplastic Left Heart Syndrome: HLHS [Figure 6-13-52]

• 8%
• Most common cause cardiac death 1st week of life
• Hypoplastic LV, AoV, proximal Ao, LA, MV (degrees variable)
• L-sided outflow tract obstruction
• Variable severity
• Systemic perfusion of aorta entirely thru PDA @ pulmonary pressures
• RV failure rapidly ensues, especially as ductus closes in 24 – 48 hours
• Infants are dusky (poorly perfused with admixed blood)
➢ They are too poorly perfused to appear cyanotic
Hypoplastic left heart
• Dusky infant in failure in first 48 hrs of life = HLHS syndrome

Radiology [Figure 6-15-53] Figure 6-15-53
• Typically very large heart
• Failure
• One of very few conditions that cause failure in first 24 hours of
life
Surgical Repair
• Cardiac transplant
• Norwood (3 stage repair)
➢ Stage 1: (birth)
❖ Conduit RV to Ao root
❖ Divide PA from RV and ductus
❖ Perfuse PA via BT shunt
➢ Stage 2: (6 mos)
❖ Construct Glenn shunt (SVC to PA) to reduce RV volume HLHS: cardiomegaly + pulmonary
edema
load
➢ Stage 3: (18 mos)
❖ Extracardiac Fontan. Direct blood from IVC & SVC thru RA to PA Figure 6-15-54
Conduit RV to Aorta: newborn [Figure 6-15-54]
Blalock-Taussig Shunt: newborn [Figure 6-15-55]
Bidirectional Glenn Shunt: 4-6 mos [Figure 6-15-56]
Extracardiac Fontan: 2 years [Figure 6-15-57]

Figure 6-15-56
Fontan: 2+ years [Figure 6-15-58]
Figure 6-15-55
Rastelli repair for HLHS:

conduit from RV to Aorta
Figure 6-15-57
Glenn shunt: SVC to R PA

(bidirectional = perfuses R
and L PA)
Figure 6-15-58
BT shunt: subclavian
artery to ipsilateral PA
Extracardiac Fontan: SVC

and IVC grafted to PA
(bypasses RA & RV)
Fontan: RA to PA graft
(adds atrial kick to perfusion
pressure)

Cyanotic CHD with Decreased PBF – R to L Shunt Lesions
[Figure 6-15-59] Figure 6-13-59
• Patients with decreased PBF are ALWAYS cyanotic
• Blood shunts right to left (bypasses lungs so is not oxygenated);
there is decreased PBF
R to L Shunts
• 2 common features:
➢ Opening between R and L sides of heart (allows R to L
shunting)
➢ R-sided outflow tract obstruction (pulmonary stenosis/ atresia,
tricuspid stenosis/atresia)
R to L Shunt Lesions “TET P”

• Tetralogy of Fallot
• Ebstein malformation
• Tricuspid atresia
• Pulmonary atresia
Tetralogy of Fallot [Figure 6-15-59]

• Incidence 9%
• Most common cyanotic CHD
• Associated with Down Syndrome Tetralogy of Fallot
➢ ECD most common cardiac lesion in Down syndrome
• Tetrad:
➢ VSD (usually large)
➢ Infundibular pulmonary stenosis Figure 6-15-60
➢ Overriding aorta
➢ R ventricular hypertrophy
Associated Anomalies
• R aortic arch in 25% (usually mirror image branching)
➢ R arch also seen in 35–40% Truncus (most highly associated)
➢ But TOF much more common
• Pulmonary valvular stenosis (90%)
• Peripheral pulmonary stenoses (75%)
• ASD (10%) (w/ VSD=pentalogy of Fallot)
• Enlarged systemic collateral arteries
Physiology Tetralogy: coeur en sabot heart

• Large VSD means pressure LV = RV
• Severity of pulmonary stenosis dictates amount of R to L shunt
➢ Mild stenosis = little/ no shunting = “pink tet”) Figure 6-15-61
• Tet spells – paroxysmal dyspnea progressing to cyanosis and
unconsciousness (unknown etiology)
• Squatting: patient squats to increase systemic resistance,
decrease R to L shunting, and improve PBF. Especially important
when exercising / playing (SVR decreases with exercise, so R to
L shunting worsens)

• Boot-shaped heart (“coeur en sabot” or heart in a boot)
• Concave PA segment – gives unusually straight (horizontal) upper
L cardiac border
• The more severe the pulmonary outflow obstruction, the more
“classic” the X-ray appearance
Tetralogy: angiogram of large VSD,
overriding Ao, pulmonary stenosis

Surgical Repair [Figure 6-15-62] Figure 6-15-62
• Corrective:
➢ Patch VSD
➢ Ao isolated to LV
➢ Widen infundibulum,
➢ Repair other anomalies
Waterston [Figure 6-15-63]
Potts [Figure 6-15-64]
Tetralogy corrective repair
Figure 6-15-65
Waterston palliative shunt Potts palliative shunt
Surgical Complications [Figure 6-15-65]

• RV outflow tract aneurysms
• Obstruction of a shunt
➢ Look for asymmetry of PBF
➢ Change from baseline
Ebstein Malformation [Figure 6-15-66] RV outflow tract aneurysm, S/P Tet

• 2 tricuspid valve leaflets displaced into RV
repair
➢ RV functionally small
➢ “Atrialization” of RV
➢ RV outflow tract obstructive
• Tricuspid valve insufficiency
• ASD shunts R to L
• RA is large
➢ Cardiomegaly Figure 6-15-66 Figure 6-15-67
• Cardiomegaly (RA dilation)
• Decreased PBF
Ebstein malformation: large heart,

Ebstein malformation decreased PBF

Tricuspid Atresia [Figure 6-15-68] Figure 6-15-68
• Incidence 1.5%
• 75% of TA have decreased PBF (they do NOT have 1 2
associated TGV)
• ASD shunts R to L
• Small VSD
• PA arises from diminutive RV
• PBF decreased

• Mild cardiomegaly
• Increased or decreased PBF
➢ Depends on whether PA or Ao arises from RV
• Very variable
Tricuspid atresia:
Surgical Repair [Figure 6-15-70] 1-With transposition (increased PBF)
• Palliative L to R conduits 2-With normal great vessels (decreased PBF)
• Fontan:
➢ Conduit from RA to PA
➢ RV usually closed off Figure 6-15-69 Figure 6-15-70
Single Ventricle Physiology

• Common disorders:
➢ Tricuspid atresia
➢ Hypoplastic left heart
➢ Pulmonary atresia
➢ DORV
• Increased or decreased pulmonary
vascularity
➢ Determined by R outflow
obstruction (eg.: pulmonary
stenosis) Tricuspid atresia with normally related
vessels: decreased PBF
Single Ventricle Protocol Fontan palliation of
All: single ventricle serves Ao tricuspid atresia
• Step 1 (newborn): adjust PBF
➢ BT shunt for decreased PBF
➢ Banding, other Rx for increased PBF Figure 6-15-71
• Step 2 (4-6 mos): take volume load off heart
➢ Bidirectional Glenn shunt
• Step 3 (2 yrs):
➢ IVC and SVC serve PA
❖ Via RA = Fontan
❖ Directly = extracardiac Fontan
Pulmonary Atresia with Intact Ventricular Septum [Figure 6-15-71]

• Incidence 1%
• Pulmonary atresia with VSD = tet physiology
• Similar to tricuspid atresia physiologically
• Lungs perfused via PDA
• “Ductal dependent” lesion

• Appearance variable
• Usually large heart Pulmonary atresia with
intact ventricular septum

Top 10 CHD
1. VSD 25%
2. ASD 10
3. Tetralogy of Fallot 9
4. PDA 8
5. TGV 8
6. Hypopl. L Heart 8
7. Coarctation 5
8. AV Canal Defects 4
9. TAPVR 2
9. Tricuspid Atresia 1.5
10.Truncus 1
Syndromes & Cardiac Lesions

Down ECD, tetralogy, VSD
DiGeorge Interrup. Ao arch, truncus
Ellis-van Creveld ASD, common atrium
Holt-Oram ASD
Noonan Pulmonary stenosis
Rubella Peripheral PS, PDA
Turner Coarctation Ao
Williams Supravalv. Ao stenosis
References
Textbooks
1. Gedgaudas E. Cardiovascular radiology. Philadelphia, Pa: WB Saunders, 1985.
2. Tonkin, ILD. Pediatric cardiovascular imaging. Philadelphia, Pa: WB Saunders, 1992.
3. Amplatz K, Moller JH. Radiology of congenital heart disease. St Louis, Mo: Mosby, 1993.
Journal Articles
1. Alexiou C, Mahmoud H, Al-Khaddour A, et al. Outcome after repair of tetralogy of Fallot in the first year of life.
Ann Thorac Surg. 2001;71:494-500
2. Bichell DP, Geva T, Bacha EA, Mayer JE, Jonas RA, del Nido PJ. Minimal access approach for the repair of atrial
septal defect: the initial 135 patients. Ann Thorac Surg. 2000;70:115-8.
3. Coussement AM, Gooding CA. Objective radiographic assessment of pulmonary vascularity in children.
Radiology 1973;109:649-654.
4. El-Najdawi EK, Driscoll DJ, Puga FJ, et al. Operation for partial atrioventricular septal defect: a forty-year review.
J Thorac Cardiovasc Surg. 2000;119:880-889.
5. Fisher RG, Moodie DS, Sterba R, et al. Patent ductus arteriosus in adults - long term follow-up: nonsurgical versus
surgical treatment. J Am Coll Card. 1986;8:280-284.
6. Harvey JR, Teague SM, Anderson JL, et al. Clinically silent atrial septal defects with evidence for cerebral
embolization. Ann Intern Med. 1986;105:695-687.
7. Jacobs ML, Pourmoghadam KK. The hemi-Fontan operation. Semin Thorac Cardiovasc Surg, 2003;6:90-97.
8. Kreutzer C, De Vive J, Oppido G, et al. Twenty-five-year experience with Rastelli repair for transposition of the
great arteries. J Thorac Cardiovasc Surg. 2000 Aug;120:211-223.
9. Murphy JG, Gersh BJ, McGoon MD, et al. Long term outcome after surgical repair of isolated atrial septal defect.
N Engl J Med 1990;323:1645-1650.
10. Ohye RG, Bove EL. Advances in congenital heart surgery. Curr Opin Pediatr. 2001;13(5):473-481.
11. Thibeault DW, Emmanouilides GX, Nelson RJ, et al. Patent ductus arteriosus complicating the respiratory distress
syndrome in preterm infants. J Pediatr 1975; 86:120-126.
12. Williams DL, Gelijns AC, Moskowitz AJ, et al. Hypoplastic left heart syndrome: valuing the survival. J Thorac
Cardiovasc Surg. 2000;119(4 Pt 1):720-731.

Forensic Radiology of Child Abuse
Figure 6-16-1
Scope of the problem1: U.S. Data for the year 2000
• 3 million cases reported
• 879,000 substantiated cases
• ~1200 fatalities (U.S.) in the year 2000 from abuse
• 1.22% of all children in US are abused
1Child Maltreatment 2000: Reports from the States to the National Child Abuse
and Neglect Data System. In: US Department of Health and Human Services
Children’s Bureau (online). Available at:
http://www.calib.com/nccanch/prevmnth/scope/ncands/cfm
Common radiographic findings in abuse

• Long bone fx (shaft & metaphyseal)
• Rib fx
• Skull fx
• Subdural & subarachnoid hemorrhage
• Cerebral edema
• Visceral injury
Long bone shaft fracture [Figure 6-16-1]

• MOST common fracture in abuse (?4x more common than metaph. fx?) when Inflicted femoral shaft
all ages considered fracture
➢ Infants: metaphyseal, rib, & skull fx more common1,2
• Not specific for abuse . . .
Figure 6-16-2
• Except in the very young
➢ Shaft fx (esp spiral) in a non-walking infant is suggestive of abuse w/o
convincing & verifiable history
• Most common sites: femur, humerus
1Kleinman PK, Marks SC, Jr., Richmond JM, Blackbourne BD. Inflicted skeletal injury: a
postmortem radiologic-histopathologic study in 31 infants. AJR 1995; 165:647-650.
2Worlock P, Stower M, Barbor P. Patterns of fractures in accidental and non-
accidental injury in children: a comparative study. Br Med J 1986; 293:100-102.
Spiral Fracture [Figure 6-16-2] Figure 6-16-3
Developmental Milestones
4 mos raises head 90º
5–6 mos rolls over
8–9 mos sits alone
15 mos walks alone
18 mos climbs stairs
24 mos runs well
36 mos alternates feet up stairs
Metaphyseal Fracture1 [Figure 6-16-3]

• High specificity for abuse
• Also known as “corner” and “bucket-
handle” fractures
• Most common in lower extremity Spiral humeral fracture
➢ Knee
➢ Ankle
• Fracture through most immature bone
➢ Primary spongiosa (metaphysis)
1Kleinman PK, Marks, SC, et al. AJR 1986;
146:895-905 Metaphyseal fracture

1491 Forensic Radiology of Child Abuse
Shaking mechanism [Figure 6-16-4] Figure 6-16-4
• From rapid, forceful acceleration and deceleration:
➢ Shearing forces oriented perpendicular to long axis of bone
• Child may be held around chest (or by limb) & shaken violently
• Microfracture thru primary spongiosa
• Thicker collar of metaphyseal bone at periphery
• Periosteum usually normal except with extensive injury

• Subtlest radiographic finding: metaphyseal lucency (non-specific,
seen in leukemia, stress)
• “Corner” fracture and “bucket-handle” fracture are different
projections of the same fracture
• Appearance depends on shape of metaphysis itself & projection
of fracture
Healing of metaphyseal fractures

• Callus unusual – difficult to date
• Ηeal quickly (10 days to several wks)
➢ prompt radiography is ESSENTIAL
• The younger the infant, the quicker the healing Shaking mechanism
• Usually no deformity or sequela except in the unusual case of a
hip metaphyseal fracture (may cause coxa vara)
Rib fracture [Figures 6-16-4, 6-16-6 and 6-16-7

• Fracture anywhere on rib
• Posterior fracture: high specificity for abuse
➢ All locations: very suspicious Figure 6-16-5
• Occur with chest compression, typically during
violent shaking
• Seen in 35 – 60% of abused infants (<18 mos)1,2
• Lateral rib fracture: AP compression “folds” rib
laterally, causing fx
• Posterior rib fracture: posterior compression levers
rib end over transverse process
➢ Fx most pronounced at ventral cortex
➢ Posterior compression/impact necessary for
fracture to occur
1 Kleinman PK, Marks SC, Jr., Richmond JM, Blackbourne BD. Lateral and frontal views of metaphyseal fracture
Inflicted skeletal injury: a postmortem radiologic-histopathologic
study in 31 infants. AJR 1995; 165:647-650.
2Worlock P, Stower M, Barbor P. Patterns of fractures in accidental and non-accidental
injury in children: a comparative study. Br Med J 1986; 293:100-102.
Figure 6-16-6
Figure 6-16-7
Cross sectional diagram of chest squeeze by

adult hands
Multiple bilateral rib fractures
Forensic Radiology of Child Abuse 1490

Visualization of posterior rib fractures1 Figure 6-16-8
[Figures 6-16-8 and 6-16-10]
• High miss rate acutely unless oblique views or bone scan
performed
• Production of callus (best at 7-14 days) aids visualization
• To increase detection:
➢ Skeletal technique chest film (NOT chest radiograph
technique)
➢ Bone scintigraphy
➢ Chest CT
➢ Oblique chest films (bone technique)
✧ Consider as part of original skel survey Posterior rib fractures without
➢ Follow-up films in > 7 days evidence of healing
1Kleinman PK, Marks SC, Adams VI, Blackbourne BD. Factors affecting
Figure 6-16-9
visualization of posterior rib fractures in abused infants. AJR 1988; 150:635-638.
Rib fracture & CPR

• Posterior compression NOT a
feature of CPR
• Experimental studies fail to
reproduce posterior fractures
with CPR or report their
occurrence1,2,3
• Rib fractures (anterior and
lateral) rarely seen after CPR in
normally mineralized bones of Occult posterior rib fractures seen at scintigraphy
infants & young children
1Kleinman PK, Schlesinger AE. Mechanical factors associated with posterior rib fractures: Figure 6-16-10
laboratory and case studies. Pediatr Radiol 1997; 27:87-91.
2Feldman KW, Brewer DK. Child abuse, cardiopulmonary resuscitation, and
rib fractures. Pediatrics 1984; 73:339-342.
3Spevak MR, Kleinman PK, Belanger PL, Primack C, Richmond JM.
Cardiopulmonary resuscitation and rib fractures in infants. A postmortem
radiologic-pathologic study. JAMA 1994; 272:617-618.
Healing of fractures1
• No callus fx < 14 days old
• Callus fx > 7 days old
• Caveat: these are general estimates and should not be thought of
as fixed time frames, as healing & callus formation are a
continuum
CT of healing left posterior rib
• The younger the child, the faster the healing fracture
1O’Connor JF, Cohen J., in: Kleinman PK Diagnostic Imaging of Child Abuse, 2nd
Figure 6-16-11
ed. Mosby, 1998 168–177.
Spine injury [Figure 6-16-11]

• Abuse (typically shaking) results in:
➢ Compression & superior endplate fxs at
thoracolumbar junction from hyperflexion1
➢ Avulsion injury to interspinous ligament and
spinous process cartilage (hyperflexion
mechanism)2
• Spinous process fractures are high specificity,
though unusual
1Kleinman PK, Marks SC. Vertebral body fractures in child
abuse. Radiologic-histopathologic correlates. Invest Radiol
1992; 27:715-722.
2Swischuk LE. Spine and spinal cord trauma in the battered
Vertebral compression fractures
child syndrome. Radiology 1969; 92:733-738.

Scintigraphy: Advantages
• More sensitive than plain films for rib fractures
• Probably slightly more sensitive for detection of some other fractures
Scintigraphy: Limitations [Figure 6-16-12]

• Higher radiation dose
• Insensitive for skull fxs (always need supplementary plain films of skull)
• Less sensitive than plain films for the detection of metaphyseal & vertebral fx
• Can’t determine age or type of fracture
• More technically & professionally demanding & more costly Figure 6-16-12
Evaluation of skeletal injury

• Skeletal survey with high-detail film (mammography
film or high-detail extremity film ideal)
• At least 2 views of any abnormality
• Do all of above even if post-mortem!
• consider chest CT, scintigraphy, or repeat CXR to
visualize missed posterior rib fxs
• Specimen radiography of abnormalities in post-
mortem child with subsequent dissection & dating
Skull fracture missed at scintigraphy
The skeletal survey (all: bone technique)
• AP thorax
• AP humeri
• AP forearms
• Oblique hands
• AP feet
• AP femora
• AP & LAT tibiae
• AP & LAT skull
• AP pelvis
• LAT C-spine
• LAT thorax
• LAT L-spine
High specificity
• Metaphyseal fracture
• Posterior rib fracture
• Spinous process fracture
• Sternal fracture
• Scapular fracture
Moderate specificity
• Multiple fractures, especially bilateral
• Fractures of different ages
• Epiphyseal separation
• Vertebral body fracture & subluxation
• Digital fractures
• Complex skull fractures
Common, but low specificity

• Clavicular fracture
• Long bone shaft fracture
• Linear skull fracture
Cranial injury
• Leading cause morbidity & mortality
➢ Mortality peaks at 6 months
• Mechanisms:
➢ Shaking
➢ Direct blow
➢ Strangulation / suffocation
• Shaking alone is sufficient to cause fatal CNS injury (shearing forces)

• In the first year of life1: Figure 6-16-13
➢ 64% of all head injuries needing admission or with positive CT
findings are inflicted (excluding simple skull fracture)
➢ 95% of all serious head injuries are inflicted
1Billmire ME, Myers PA. Serious head injury in infants: accident or abuse?
Pediatrics 1985; 75:340-342.
Specific injuries [Figures 6-16-13 and 6-16-14]

• Edema
➢ Most common, but non-specific as to mechanism (blow,
strangulation, post-traumatic apnea, etc)
• Shear injury at grey-white junction and in large WM tracks (c.
callosum)
• Cortical contusion, laceration
• SAH & SDH (when interhemispheric, very worrisome for abuse)1
1Zimmerman RA, Bilaniuk LT, Bruce D, Schut L, Uzzell B, Goldberg HI.
Left cerebral edema and SDH
Computed tomography of craniocerebral injury in the abused child. Radiology
1979; 130:687-690.
Figure 6-16-14
Interhemispheric extra-axial hemorrhage
[Figures 6-16-15 to 6-16-17]
• Blood adjacent to falx, usually asymmetric & posterior
• Difficult to distinguish SAH from SDH here
• From violent shaking: hemispheres impact falx, on rebound
bridging veins to sagittal sinuses are torn
• May be associated with posterior convexity and tentorial
hematomas
• Interhemispheric extraaxial hemorrhage may be difficult to
distinguish from normal falx (caveat: normal falx may appear
strikingly bright in presence of global cerebral edema)
➢ Thicker than expected
➢ Irregular thickness
➢ Asymmetric thickness
➢ Extension into a posterior convexity SDH
Figure 6-16-15 Left frontal white matter tear
Figure 6-16-16
Diagram of SDH
Figure 6-16-17
Interhemispheric hemorrhage
Interhemispheric and left tentorial

extraaxial hemorrhage

The reversal sign1,2 [Figure 6-16-18] Figure 6-16-18
AKA the bright cerebellum sign
• Diffuse cortical and subcortical WM edema
• Basal ganglia, thalami, brainstem, & cerebellum retain normal
attenuation so appear bright
• Dismal prognosis: hemispheric injury may go on to multicystic
encephalomalacia, atrophy
1Han BK, Towbin RB, De Courten-Myers G, McLaurin RL, Ball WS, Jr.
Reversal sign on CT: effect of anoxic/ischemic cerebral injury in
children. 1990; 154:361-368.
2Harwood-Nash DC. Abuse to the pediatric central nervous system.
AJNR 1992; 13:569-575.
The reversal sign

• Basal ganglia, thalami, brainstem, & cerebellum increased
relative attenuation may be due to
➢ Neovascularity Cerebral edema, parenchymal
➢ Neuronal preservation hemorrhage, and extraaxial
hemorrhage
➢ Vascular engorgement
➢ Mineralized neurons
➢ Petechial hemorrhages
Cerebral edema
• Not specific for abuse
• May be seen in:
➢ Drowning
➢ Non-abusive head trauma
• When abuse-related, may be from:
➢ Direct brain injury
➢ Strangulation
➢ Venous pressure from chest compression
➢ Post-traumatic apnea
Sequellae
CT MRI
• Acute • Delayed presentation
• Polytrauma • Differing ages of injuries/blood collections
• Normal or equivocal CT with high suspicion (better than CT for
shear and SDH)
• Sequela
Dating of intracranial blood1

1Bradley WG, Radiology 1993 189: 15
Stage Form T1 MR T2 MR
hyperacute (<12-24 hrs) oxy Hb iso-low high (slightly)
acute (1-3 days) deoxy Hb iso-low low

early subacute (3-7 days) intracell met Hb high low
late subacute (1-2 wks) extracell met Hb high high
chronic (>2 wks) ferritin, iso >>>> low high in center

hemosiderin, low in rim
approaches
CSF with time

Hemorrhage dating by MR [Figure 6-16-19] Figure 6-16-19
• An inexact science
• Affected by
➢ Technical aspects MR scanner, sequences
➢ Concentration of Hb
➢ Relative concentrations of degradation products
(a continuum)
➢ Arachnoid tear with leak of CSF into SDH
Rebleeding in a Chronic SDH

• Outer membrane itself shown to bleed frequently1
• Elevated tissue plasminogen activator (TPA) found in
Right SDH
chronic SDH fluid1
• Little (if anything) in the literature to support a single, significant
rebleed as cause of rapidly enlarging SDH2 Figure 6-16-20
• No documented cases of significant rebleed occurring in safe
environment
1Chen JC, Levy ML. Causes, epidemiology, and risk factors of chronic
subdural hematoma. Neurosurg Clin N Am 2000; 11:399-406.
2Block RW. Child abuse--controversies and imposters. Curr Probl Pediatr
1999; 29:249-272.
Sinus Thrombosis & Infarct [Figure 6-16-20]
Cord injury
• In 9 of 11 abuse fatality autopsies in infants1,2
➢ Subarachnoid
➢ Subdural
➢ Epidural
➢ 4 of 6 with ventral cord contusion
• Probably overestimates real incidence
1Hadley MN, Sonntag VK, Rekate HL, Murphy A. The infant whiplash-shake
injury syndrome: a clinical and pathological study. Neurosurgery 1989;
24:536-540. Right transverse sinus thrombosis
2Feldman KW, Weinberger E, Milstein JM, Fligner CL. Cervical spine MRI in
abused infants. Child Abuse Negl 1997; 21:199-205.
Figure 6-16-21
Skull fracture [Figure 6-16-21]
• Overall, poorly correlated with CNS injury
(linear fx most common in NAT)
➢ 10% of all abuse cases have skull fx
• Non-specific (altho common) unless:
➢ Stellate / eggshell
➢ Multiple
➢ Diastatic (>3 mm)
➢ Occipital
➢ Inconsistent history (“rolled off changing table”)
Skull fracture from falls & stairs

• Falls:
➢ In 529 falls from heights up to 150 cm, 4 skull, 4 clavicle, 1 Diastatic eggshell skull fractures
humerus (1.7% incidence) fractures and no significant
neurologic injuries occurred
➢ Conclusion: “household” falls rarely associated with fx,
almost never with any intracranial injury1,2,3
➢ Stairs: more injurious, though significant injuries usually single (not multiple
body parts)4,5
1Helfer RE, Slovis TL, Black M. Injuries resulting when small children fall out of bed.
Pediatrics 1977; 60:533-535.

2Nimityongskul P, Anderson LD. The likelihood of injuries when children fall out of bed. J
Pediatr Orthop 1987; 7:184-186.
3Lyons TJ, Oates RK. Falling out of bed: a relatively benign occurrence. Pediatrics 1993;
92:125-127.
4Joffe M, Ludwig S. Stairway injuries in children. Pediatrics 1988; 82:457-461.
5Chiaviello CT, Christoph RA, Bond GR. Stairway-related injuries in children. Pediatrics
1994; 94:679-681.
Visceral Injury
• Seen in all ages of abused children
• Usually blunt trauma (punch or kick to abdomen, rapid deceleration after being
thrown)
• 50% mortality rate of clinically apparent visceral injury
➢ Delay in seeking treatment pivotal!
• Estimated to account for 12% of all abuse fatalities
➢ 2-4% of all abusive injuries
Small Intestine [Figure 6-16-22] Figure 6-16-22

• Hematoma (prox SB), perforation (distal SB)
• Intramural hematoma duod & proximal jejunum most common
abdominal injury
➢ 90% in duod & prox jejunum, most near ligament of Treitz
➢ ? is bowel most tethered most vulnerable?
• Pt presents with pain, vomiting
• Mural, asymmetric mass on UGI
• Coiled spring appearance of heaped-up proximal mucosa
• +/- free air if perforation
➢ Sepsis
Bowel Injury
Duodenal hematoma
Pancreas
• Probably compressed against spine with blunt trauma
• Abuse is a common cause of pancreatitis (trauma is most Figure 6-16-23
common cause in children)
• May develop pseudocysts
Pancreatic Injury Radiography [Figures 6-16-23 and 6-16-24]

• Ascites
• Decreased pancreatic echogenicity at US, ductal dilation
• Peripancreatic inflammation
Other abdominal injuries [Figure 6-1-25]

• Liver: contusion, laceration
• Kidney: contusion, laceration, fx
• Bladder: rupture Pancreatic laceration
• Adrenal: hemorrhage
Pancreatitis Liver contusion

Hypoperfusion complex Figure 6-16-26
• Severe abdominal injury resulting in hemodynamic instability
• Imaging:
➢ Small caliber Ao and IVC (intravascular volume depletion)
➢ Fluid-filled bowel with enhancing wall (concentrated contrast)
➢ Ascites
➢ Intensely enhancing kidneys
Shock Bowel (Hypoperfusion Complex) [Figure 6-16-26]
Differential diagnosis of abuse injuries

• Birth trauma: clavicle & humerus fxs
• Normal variant: periosteal new bone in infants 2–8 mos of life
(single layer, smooth, bilaterally symmetric) Hypoperfusion complex
• Osteogenesis imperfecta: fxs, periosteal rxn
(should also see osteopenia, +/– wormian bones, blue sclera)
• Rickets: widened, irregular physes with metaphyseal irregularity
(look for osteopenia)
• Scurvy: white metaphyseal line of Frankel with sub-metaphyseal lucency,
spurs, periostitis (look for osteopenia, Wimberger ring around epiphyses)
• Vit A intoxication: periosteal rxn
(pronounced periostitis in ulnae & tubular bones of hands & feet)
• Caffey’s (presumed viral illness of infancy rarely seen today):
periostitis mandible, clavicle, ulna, irritable, febrile child < 6 mos old
• Syphilis: metaphyseal lucencies, periostitis
(destructive metaphyseal osteomyelitis lesions), + serology
• Leukemia: metaphyseal lucency
(see lucent bands symmetrically, osteopenia)
• Menke’s kinky hair: metaphyseal spurring, flared anterior rib ends
(no true metaphyseal fx, findings bilateral & symmetric, characteristic hair)
• Remember to consider:
➢ congenital insensitivity to pain
➢ spinal dysraphism
Child abuse
• We as radiologists are uniquely able to diagnose abuse
• We may be the 1st to recognize abuse
• Radiographic findings in abuse are among the most specific & diagnostic in medicine
• Our findings may be PIVOTAL to investigation & prosecution
References
1. Billmire ME, Myers PA. Serious head injury in infants: accident or abuse? Pediatrics 1985; 75:340-342.
2. Block RW. Child abuse--controversies and imposters. Curr Probl Pediatr 1999; 29:249-272.
3. Bradley WG. MR appearance of hemorrhage in the brain. Radiology 1993 189: 15-26
4. Chen JC, Levy ML. Causes, epidemiology, and risk factors of chronic subdural hematoma. Neurosurg Clin N Am
2000; 11:399-406.
5. Chiaviello CT, Christoph RA, Bond GR. Stairway-related injuries in children. Pediatrics 1994; 94:679-681.
6. Child Maltreatment 2000: Reports from the States to the National Child Abuse and Neglect Data System. In: US
Department of Health and Human Services Children's Bureau (online). Available at:
http://www.calib.com/nccanch/prevmnth/scope/ncands/cfm
7. Feldman KW, Brewer DK. Child abuse, cardiopulmonary resuscitation, and rib fractures. Pediatrics 1984; 73:339-
342.
8. Feldman KW, Weinberger E, Milstein JM, Fligner CL. Cervical spine MRI in abused infants. Child Abuse Negl
1997; 21:199-205.
9. Hadley MN, Sonntag VK, Rekate HL, Murphy A. The infant whiplash-shake injury syndrome: a clinical and
pathological study. Neurosurgery 1989; 24:536-540.
10. Han BK, Towbin RB, De Courten-Myers G, McLaurin RL, Ball WS, Jr. Reversal sign on CT: effect of
anoxic/ischemic cerebral injury in children. 1990; 154:361-368.
11. Harwood-Nash DC. Abuse to the pediatric central nervous system. AJNR 1992; 13:569-575.
12. Helfer RE, Slovis TL, Black M. Injuries resulting when small children fall out of bed. Pediatrics 1977; 60:533-535.

13. Joffe M, Ludwig S. Stairway injuries in children. Pediatrics 1988; 82:457-461.
14. Kleinman PK, Marks SC, Adams VI, Blackbourne BD. Factors affecting visualization of posterior rib fractures in
abused infants. AJR 1988; 150:635-638.
15. Kleinman PK, Marks SC, Blackbourne B. The metaphyseal lesion in abused infants: a radiologic-histopathologic
study. AJR 1986; 146:895-905.
16. Kleinman PK, Marks SC, Jr., Richmond JM, Blackbourne BD. Inflicted skeletal injury: a postmortem radiologic-
histopathologic study in 31 infants. AJR 1995; 165:647-650.
17. Kleinman PK, Marks SC. Vertebral body fractures in child abuse. Radiologic-histopathologic correlates. Invest
Radiol 1992; 27:715-722.
18. Kleinman PK, Schlesinger AE. Mechanical factors associated with posterior rib fractures: laboratory and case
studies. Pediatr Radiol 1997; 27:87-91.
19. Lyons TJ, Oates RK. Falling out of bed: a relatively benign occurrence. Pediatrics 1993; 92:125-127.
20. Nimityongskul P, Anderson LD. The likelihood of injuries when children fall out of bed. J Pediatr Orthop 1987;
7:184-186.
21. O’Connor JF, Cohen J., in: Kleinman PK Diagnostic Imaging of Child Abuse, 2nd ed. Mosby, 1998 168-177.
22. Spevak MR, Kleinman PK, Belanger PL, Primack C, Richmond JM. Cardiopulmonary resuscitation and rib
fractures in infants. A postmortem radiologic-pathologic study. JAMA 1994; 272:617-618.
23. Swischuk LE. Spine and spinal cord trauma in the battered child syndrome. Radiology 1969; 92:733-738.
24. Worlock P, Stower M, Barbor P. Patterns of fractures in accidental and non-accidental injury in children: a
comparative study. Br Med J 1986; 293:100-102.
25. Zimmerman RA, Bilaniuk LT, Bruce D, Schut L, Uzzell B, Goldberg HI. Computed tomography of craniocerebral
injury in the abused child. Radiology 1979; 130:687-690.
Forensic Radiology of Child Abuse 1500 Pediatric Radiology

Neonatal Brain: Radiologic Evaluation
Dorothy I Bulas, MD
Learning Objectives
• Review the differences in cranial anatomy of the preterm and term infant
• Review hemorrhagic (IVH) & nonhemorrhagic (PVL) injuries due to partial
asphyxia.
• Understand radiologic work up of hypoxic ischemic injury of the neonate
including neurosonographic techniques (transmastoid view, Doppler)
Figure 6-17-1
Sonographic Technique
• Anterior fontanelle
➢ Coronal and sagittal planes
• Axial views
➢ Posterior and mastoid fontanelle
• Doppler
➢ MCA/ACA resistive index
➢ Color Doppler of fluid collections
Posterior Fontanelle [Figure 6-17-1]
Mastoid Fontanelle Scanning

• 24/200 patients with PF abnormality Ultrasound image via the posterior
fontanelle demonstrates the occipital
• Mastoid view: horn filled with choroid
➢ Improved visualization (96%)
➢ Increased diagnostic confidence (75%)
➢ Only technique to show abnormality (46%) Figure 6-17-2
Luna & Goldstein, AJR 2000; 174
Transmastoid view -Trapped fourth ventricle

[Figure 6-17-2]
Sagittal Sinus Thrombosis [Figure 6-17-3]
Resistive Index – MCA or ACA

Peak Systole – End Diastole
• Peak Systole
➢ Minimize affect of angulation Transmastoid view demonstrates
❖ Age dependent values lateral and fourth ventriculomegaly
❖ Term infants 0.7 + 7%
❖ By age 2 0.5+ 15%
• An increase in diastolic flow results in decrease RI
• A decrease in diastolic flow results in increase RI
• As ICP increases above mean arterial pressure, diastolic flow Figure 6-17-3
reverses w/ RI > 1.0.
Normal Anatomy: Premature Brain

• Preterm cerebral vessels penetrate from meninges to
periventricular walls
➢ single vessel walls
• Term cerebral vessels-watershed at cortex
➢ smooth muscle AA, collagen VV
Nelson et al AJNR 1991:215
Longitudinal ultrasound via the
anterior fontanelle demonstrates
irregular flow of the sagittal sinus
consistent with sagittal sinus
thrombosis

1501 Neonatal Brain
Neonatal Hypoxic-Ischemic Injury Figure 6-17-4
• Injuries are the result of partial or global asphyxia due to various
causes: intrauterine, sepsis, apneic episodes
• Partial asphyxia: IVH, PVL
• Global asphyxia: multifocal ischemic necrosis
Injury patterns: Term infant [Figure 6-17-4]

• Prolonged Partial Asphyxia
➢ Watershed infarction (ACA-MCA-PCA; “parasagittal”) - cortex
/ white matter
• Profound asphyxia:
➢ Basal ganglia,
➢ Rolandic cortex
Germinal Matrix
• Origin of neuronal/glial development.
• Rich arterial supply perforators of ACA, MCA, PCA.
• Drain to deep venous system.
Axial CT demonstrates diffuse cortical
• Regression posterior to anterior b/w 24-28 wks infarction in a term infant with severe
• Involutes by 32 wks hypoxic ischemic injury
Germinal Matrix Hemorrhage

• Most common origin of preterm hemorrhages
• 20% of preterms
• <1500 gms
• Venous origin

• Capillaries and sinusoids in caudothalamic groove with poor stromal support
• Vessels converge to form draining veins
• Theory- increased flow vv rupture at convergence points.
Nakamura et al Mod Path 1991;475
Germinal Matrix Hemorrhage: FACTORS

• Asphyxia-abolishes autoregulation-
CBF varies with SBP > injury to endothelium of germinal matrix vessels.
• Hypoxia- decrease myocardial energy reserve > circulatory failure >
hypotension > cerebral ischemia > venous hypertension
Funato M, Munksgard 1994:456; Milligan D Lancet 1980:26:896

• O2 delivery reduced w/ hemorrhage.
➢ Switch from aerobic to anaerobic metabolism Figure 6-17-5
➢ Increase lactate formation.
• Blood ruptures into ventricles
➢ Dilatation,
➢ Disrupt ependymal lining
Modified Papile Classification

• Grade I Subependymal hemorrhage
• Grade II Intraventricular blood w/ no/min ventricular
dilatation
• Grade III Intraventricular blood w/ prominent
ventricular dilatation
• Grade IV/PHI Parenchymal hemorrhage associated with Sagittal US demonstrates a Grade 1
IVH subependymal hemorrhage
Grade I IVH [Figure 6-17-5]
Neonatal Brain 1500

Grade II IVH w/out dilatation [Figure 6-17-6] Figure 6-17-6
Grade II IVH w/ distended

ventricle [Figure 6-17-7]
Grade III - IVH w/ distended

ventricle [Figure 6-17-8]
Periventricular Hemorrhagic
Infarction [Figure 6-17-9]
Figure 6-17-9
Coronal and sagittal US images demonstrate Grade II
intraventricular hemorrhage with blood extending into the
ventricle
Figure 6-17-7
Coronal sonogram demonstrates a

heterogeneous region in the right
parietal periventricular white matter
consistent with a periventricular
hemorrhagic infarction and
ventriculomegaly Coronal US and axial CT images of Grade III
intraventricular hemorrhage with blood filling and
dilated the ventricles
Periventricular Hemorrhagic
Infarction (PHI/ Grade IV IVH) Figure 6-17-8
• 15% with IVH dev PHI
• Same side as IVH, after IVH has occurred
• Usually unilateral or asymmetric
Periventricular Hemorrhagic Infarction (PHI/ Grade IV

IVH)
• Hemorrhagic necrosis in PVWM dorsal/lat of lateral ventricle
where medullary veins confluent.
Periventricular Hemorrhagic Infarction

• PET scans demonstrate parenchymal ischemia beyond
boundaries of hemorrhage
• Further injury may be due to accumulation of metabolic toxins due
to impaired blood flow Coronal sonogram demonstrate
dilated ventricles filled with
Volpe JJ. Neurology of the Newborn 1987 hemorrhage consistent with an
evolving grade III intraventricular
Periventricular Hemorrhagic Infarction Sonography hemorrhage
Time Pathology US findings

Early hemorrhagic infarcts echogenic areas
PVWM mass effect
surrounding ischemia
Subacute cystic cavities retraction of clot,
diminished myelin porencephalic cyst
ventricular debri/dilat
Chronic porencephalic cyst porencephalic cyst
ventricular dilatation ventricular dilatation
gliosis atrophy

1503 Neonatal Brain
IVH: Sonography Figure 6-17-10
• US very useful -lesions well visualized deep in the brain
• Difficult to make diagnosis clinically
• Screening recommendations
➢ Preterms <32 wks
➢ Birth wt <1.5 kg
• ?When- majority of bleeds occur 3-4 days after birth.
➢ First screening US 4-7 days after birth
IVH ? CT/MRI
• Difficult transporting unstable preterms
• CT may miss bleed after several days Pulsed Doppler demonstrates no flow
during diastole with resistive index of 1
• MRI - Hemosiderin staining can be seen up to 1 yr
➢ May demonstrate additional abnormalies
➢ Focal WM loss
➢ Diffuse hypoxic-ischemic changes
IVH/PVH: Follow up [Figure 6-17-10]

• Doppler useful to differentiate HC /atrophy
➢ RI >.8 sign of increased ICP.
IVH/Periventricular Hemorrhagic Infarction: Outcome

• Normal US 90% nl neurologic outcome
• Grade 1 90% nl neurologic outcome
• Grade 2 85% nl neurologic outcome
• Grade 3 50-70% nl outcome, 8% mortality
• PHI 90-100% major motor deficit,
64% dec cognitive function,
60% mortality
IVH: Outcome
• 3 mechanisms for poor outcome
➢ 1. Damage from ventricular dilatation-may be reversible if shunted
➢ 2. Shunt related complications: infection, sepsis, obstruction, seizures.
➢ 3. Hypoxic ischemic injury-white matter edema, gliosis, axonal swelling
• Spastic hemipareses, intellectual deficits.
• Prognosis parallels size of parenchymal echodensity
Periventricular Leukomalacia: Etiology

• ?Arterial ischemia?
➢ Size of zones decrease with gestational age
❖ Older infants w/PVL - strong hx of hypotension
❖ Very premature infant-no hx of hypotension
• ?Cytokine response?
➢ Maternal chorioamniotitis assoc. w/ PVL
❖ ?fluctuation in cerebral blood flow
❖ ?Inflammatory cytokine response to infection
• Prevalence in preterms before 25%
➢ Now reported as low as 7%.
• Yet reports of increasing survival of very low weight preterms with increase
rate of CP
• ?are we accurately identifying PVL sonographically?
• Size of zones decrease w/ gestational age
➢ Older infants w/PVL - strong hx of hypotension
➢ Very premature infant-no hx of hypotension
• Resultant injury of PVL > than border zones
➢ ?Intrinsic vulnerability of periventricular glial cells and intrinsic metabolic
properties.
➢ O2 reduced >lactic acid accumulation
Neonatal Brain 1502

• Glial cells are differentiating to astrocytes & oligodendroglia with active Figure 6-17-11
myelination. Intense metabolic activity w/ high O2 demands-
vulnerable to hypoxia [Figure 6-17-11]
Periventricular Leukomalacia:Sonography
• Infarction occurs in deep cerebral WM
• Bilateral symmetric
• US not sensitive in detecting PVL
➢ Difficult to distinguish from anisotropic effect of periventricular
halo
➢ Echodensities resolve and can be missed
Periventricular Leukomalacia:Sonography
Coronal sonogram demonstrates
multiple cysts in the periventricular
Time Pathology US Findings white matter consistent with
periventricular leukomalacia
Acute focal necrosis patchy areas of inc
1-3 days petechial hemor echogenicity PVWM
Subacute diminished myelin decreasing echogenicity

1-2 wks may be normal
2-3 wks cystic cavities small cysts “Swiss cheese”
Chronic cysts disappear cysts disappear
gliosis vent dilatation
Periventricular Leukomalacia
• Less severe PVL-diminished myelin result in dilated ventricles.
• Severe PVL will cavitate
• Cysts eventually resolve -gliotic scarring Figure 6-17-12
Periventricular Leukomalacia:CT
[Figures 6-17-12 and 6-17-13]
• Difficult transporting premature infants
• Due to high water content, difficult differentiating acute PVL from nl
preterm brain
• Chronic: CT useful extent of lesions, atrophy
➢ Irregular lateral ventricles
➢ Prominent sulci
➢ Subcortical gray matter abuts ventricles
➢ Small cysts missed
Figure 6-17-13
Axial CT image demonstrates

diffuse periventricular and cortical
edema with intraventricular
hemorrhage
Axial image demonstrates

ventricular dilatation with irregular
walls and marked loss of
periventricular white matter

1505 Neonatal Brain
Periventricular Leukomalacia: MRI Figure 6-17-14
[Figure 6-17-14]
• Acute/subacute: dec. sig T1, inc. sig T2 in PVWM
• High water content makes edema difficult to detect on T1, due to
delayed myelination, high signal on T2 apparent. Periatrial gliosis;
involves the subependymal periatrial white matter (tapedum)
• Distal corpus callosum (pre-splenium) thinned
Periventricular Leukomalacia:Outcome
• Outcome correlates with cystic cavitation
• PVWM traversed by fibers of motor cortex results in spastic
diplegia
➢ Lower> Upper extremities
• Infants with larger lesions often delayed
Nonhemorrhagic Infarction
• Rare, term infants
• 90% major handicaps
• Perinatal asphyxia
➢ Emboli Axial T2w image demonstrates high
➢ Congenital Heart disease signal in the periventricular white
matter
➢ Meningitis
➢ Polycythemis
Profound Asphyxia
• Energy requirements related to state of myelination.
• Most metabolically active and mature regions with most advanced
➢ myelination, Figure 6-17-15
➢ perfusion
➢ glucose uptake
are regions that suffer the most damage
• Complete arrest - injury determined by
➢ metabolic maturity of brain
➢ myelination
➢ autoregulation
➢ watershed pattern
➢ excitatory neurotransmitter release
➢ severity and duration of event Coronal US demonstrates diffuse increased
echogenicity of the cerebral cortex with slit
ventricles
Profound Asphyxia: Sonography Anterior cerebral artery Resistive Index < .5
• Diffuse hypoxic ischemic encephalopathy often consistent with arterial vasodilatation due to loss
superimposed on IVH/PVL of sutoregulation
Profound Asphyxia in Preterms

• Preterm have low O2 demand and immature cardiopulmonary control
• Neuronal injury can occur in ventral pons, inferior olivary nuclei, subiculum of
hippocampus
• Unlike term infant, less involvement of basal ganglia, thalami, brainstem
• PVWM injury dominating
Profound Asphyxia in Term Infants

• Involvement of basal ganglia, brainstem
➢ hippocampus
➢ posterior and medial lentiform nuclei
➢ lateral thalami
➢ cortical gyri
Profound asphyxia Term: Sonography [Figure 6-17-15]

• Acute - increased echogenicity
➢ small vents/sulci
➢ Doppler - Loss of autoregulation RI < .6
Neonatal Brain 1504

Profound asphyxia: CT [Figure 6-17-16] Figure 6-17-16
• Acute-low attenuation cortex, basal ganglia, periventricular white
matter, laminar necrosis
• Chronic-diffuse atrophy prominent extraaxial fluid spaces and
sulci
Profound asphyxia:
MRI findings in Preterm Infants
• PVWM changes dominate
➢ T1,T2 shortening in peritrigonal WM
➢ T1,T2 prolongation in cerebral WM
• Chronic - atrophy
➢ Relative sparing of cerebral cortex
➢ Hippocampal and brainstem atrophy
➢ Thin corpus collosum
Axial CT images demonstrate low
Profound asphyxia: MRI findings in term infants attenuation of the cortex, basal
ganglia as well as periventricular
• Short T, at times short T2 relaxation times white matter.in this term infant
➢ Basal ganglia following profound asphyxia
➢ Hippocampus
➢ Posterior and medial lentiform nuclei
➢ Lateral thalami Figure 6-17-17
➢ Cortical gyri
Cerebellar Infarction [Figure 6-17-17]

• Cerebellum felt to be less vulnerable to anoxic damage - sparing
during hypoxic ischemic episodes
• May not be rare in preterms
• Due to echogenicity of CBL, infarction and hemorrhage easily
missed US
• Most common watershed distribution - between the superior
cerebellar artery and the PICA
• Can be lobar /holohemispheric;
• +/- hemorrhagic
• Most in early premature (28-32) with hx of hypotension
Mercuri Ped Rad 1997;27:139
Tsuru Acta Neuropath 1995:90;400 Coronal MRI demonstrates right
cerebellar infarction in a child with a
history of prematurity
US- Posterior Fossa Hemorrhages [Figures 6-17-18 and 6-17-19]
• Easy to miss
• Close evaluation of sagittal view
• Additional views
Figure 6-17-19
➢ transmastoid view
➢ posterior fontanelle
Figure 6-17-18
Lateral image of the

skull demonstrates
where the
transducer is placed
for imaging via the
mastoid .B. Axial
Sagittal midline image of a normal brain clearly sonogram
demonstrates the vermis (arrow). B. Sagittal demonstrates a
midline image demonstrates a heterogeneous heterogenous lesion
region in the posterior fossa with no visualization in the posterior fossa
of the vermis in an infant with a posterior fossa consistent with a
hemorrhage posterior fossa
hemorrhage

1507 Neonatal Brain
Conclusions
• Neurosonology is an integral part of care in the neonate.
• Type of hypoxic injuries vary with gestational age
• US sensitive for IVH screening, less sensitive for identifying PVL,
infarcts and posterior fossa hemorrhages
• Flexibility in technique – Doppler, transmastoid view- important
in identifying subtle anomalies
References
1. Barkovich AJ, Sargent SK: Profound asphyxia in the premature infant: imaging findings AJNR 1995;16;1837.
2. Benson JE et al. Intracranial Neonatal Neurosonography: An Update. Ultrasound Quarterly 2002: 18;89
3. Boo NY et al. Early cranial US changes as predictors of outcome during first yr of life. J Ped Child Healthy 2000;36:363
4. Bulas DI, Taylor GA, Fitz CR, Revenis ME, Glass P, Ingram JD. Posterior fossa intracranial hemorrhage in infants
treated with extracorporeal membrane oxygenation: Sonographic findings. AJR 1991; 156:571.
5. Bulas DI. Vezina G: Anoxic injury in the Preterm infant Radiologic evaluation Radiologic Clinics of North America,
Vol 37, Nov 1999:1147.
6. Bulas DI. TCD: Practical Applications in Pediatrics. Applied Radiology 1999, April 7-15.
7. Chadduck WM, Duong DH Kast JM et al: Pediatric cerebellar hemorrhage. Child Nerv Syst 1995;110:579.
8. Perlman JM, Rollins N: Surveillance protocol for the detection of intracranial abnormalities in premature neonates. Arch
Pediatr Adol Med 2000;154:822.
9. Rumack CM et al. Timing and course of neonatal intracranial hemorrhage using US. Radiology 1985:154:101
10. Rumack C, Drose J. Neonatal and Infant Brain Imaging ed Rumack et al. Diagnostic Ultrasound. Elsevier Mosby
2005
11. Taylor GA. Recent advances in neonatal cranial ultrasound and Doppler techniques. Clin Perinataol 1997;24:677
12. Seibert JJ et al. Use of power Doppler in pediatric neurosonography: a pictorial essay. Radiographics 1998;18:879
13. Volpe JJ: Neurobiology of periventricular leukomalacia in the premature infant Pediatr Res 2001;50:553-562
14. Vohr B, Allan WC, Scott DT et al: Early onset IVH in preterm neonates: Incidence of neurodevelopmental handicap.
Semin Perinatol 1999;23:212.
Neonatal Brain 1506

Pediatric Liver Tumors
Pediatric Liver Tumors: GOALS

• Types and presentations
• Imaging
• Therapeutic implications/correlation
Pediatric Liver Tumors: ABC’s of Liver Tumors

• Age of patient
• Biologic imaging features
• Chemistry-blood
➢ Alpha-fetoprotein
➢ Endothelial growth factor
Categorization
• Benign Epithelial Tumors
• Benign Mesenchymal Tumors
• Malignant Epithelial Tumors
• Malignant Mesenchymal Tumors
• Metastases
Benign Epithelial Tumors

• Focal Nodular Hyperplasia
• Hepatocellular Adenoma
• Hepatic cysts
Benign Mesenchymal Tumors

• Mesenchymal Hamartoma
• Hemangioma
Malignant Epithelial Tumors

• Hepatoblastoma
• Hepatocellular Carcinoma
• Fibrolamellar Carcinoma
Malignant Mesenchymal Tumors

• Undifferentiated embryonal sarcoma
• Embyronal Rhabdomyosarcoma
• Angiosarcoma
Metastases
• Neuroblastoma
• Burkitt’s Lymphoma
• Sarcomas
• Wilms’ tumor
• Other
Mesenchymal Hamartoma
• Mesenchymal tissue, disorganized bile ducts, hepatocytes, fluid filled spaces
• Developmental disturbance:
➢ Portal/biliary obstruction, lymphangiomatous tissue
Pediatric Radiology 1509 Pediatric Liver Tumors

Mesenchymal Hamartoma: Pathology [Figures 6-16-1 and 6-16-2] Figure 6-18-1
• Multicystic, variable size: 2–25cm
• 15–30% pedunculated
• 80% right lobe, slow growing- fluid accumulation
• Sharply demarcated, lobulated
• Mesenchymal stroma, hepatocytes, cysts:
➢ Biliary/lymphangiomatous origin (Portal tract)
Figure 6-18-2
Hepatic mesenchymal hamartoma

with smoothly marginated non-
aggressive cysts within a fibrous
stroma
Figure 6-18-3
Photomicrograph with fibrous stroma
and cysts predominantly lined by flat
vascular endothelium from the
lympatic component of the
hamartoma
Mesenchymal Hamartoma: Clinical

• Enlarging abdominal mass
• Child < 2 yrs old
➢ Can Dx in utero
• Otherwise asymptomatic
• Negative alpha-fetoprotein Sonography with smooth walled cysts
with thin septations, similar to those
seen in lymphatic malformations
Mesenchymal Hamartoma: Imaging
[Figures 6-18-3 to 6-18-6]
• Hepatomegaly, abdominal mass Figure 6-18-5
• US: Multilocular cysts, hypoechoic
• Less frequent-solid mass with few cysts
• Rare to find hemorrhage, Ca +2
• MR: Cysts-low T1, high T2 signal
Figure 6-18-4
CT with few macrocysts in the

mesenchymal hamartoma
Sonography demonstrating Figure 6-18-6

microcystic component in the
hamartoma
Cystic mesenchymal hamartoma with single

macrocyst with normal adjacent liver
Pediatric Liver Tumors 1508

Mesenchymal Hamartoma: Treatment Figure 6-18-7
• SURGICAL
• Enucleation, partial hepatectomy
• Incision/drainage of cysts
• Marsupialize large cyst(s)
Mesenchymal Hamartoma
• Age: Young (less than 3y/o)
• Biologic imaging: Cystic
• Chemistry: Normal alpha-fetoprotein
Infantile Hemangioendothelioma
• Most common hepatic tumor- first year of life Large vascular spaces in
• Usually Dx in first few months of life hemangioendothelioma with normal
• Female predominance 1.5:1 adjacent liver
• Growing mass, clinically symptomatic
• Vascular, high flow, mass-stroma
• History of involution in first year, if survivor
Figure 6-18-8
Infantile Hemangioendothelioma: Pathology

[Figures 6-18-7 and 6-18-8]
• Solitary, may be multicentric
• 0.2–15 cm diameter
• May be well demarcated, no capsule
• Dilated vascular spaces- anastomosing,+ stroma
• Central necrosis/fibrosis, hemorrhage, Ca2+
➢ Evidence of regression
Infantile Hemangioendothelioma: Clinical

• Infant, usually less than 2 months
• Abdominal mass, CHF, Kasabach/Merritt, DIC
• Occasionally asymptomatic hepatomegaly
• Massive hemoperitoneum
• Multiple cutaneous angiomas-40%
• Normal alpha-fetoprotein (positive EGF)
Infantile Hemangioendothelioma: Imaging Top micrograph with small “tight”

[Figures 6-18-9 to 6-18-15] vascular spaces in a
• Hepatomegaly, abdominal mass hemamgioendothelioma from a child
• US: Heterogeneous, speckled Ca2+ with no heart failure. Bottom
➢ High flow, venous/arterial: A-V shunts specimen from a child with high
output failure from left-to-right
➢ Aortic caliber decrease-after celiac artery shunting in the
• CT: Hypodense, 40% Ca2+, periph. enhancement hemangioendothelioma; note the
• MR: Low T1, High T2 signal, +/– hemorrhage large vascular spaces associated
• Angio: Vascular, A-V shunting, large celiac A. with arteriovenous anastomoses
Figure 6-18-9
Figure 6-18-10
Large vascular spaces in

Flow-failure chest radiographic image hemangioendothelioma prior to color
from left-to-right shunt at the level of flow and duplex Doppler interrogation
the liver in patient with hepatic
hemangioendothelioma

1511 Pediatric Liver Tumors
Figure 6-18-11
Figure 6-18-12
Decrease in abdominal aortic caliber

from left-to-right shunting of blood at
the level of the celiac artery in patient
with hemanioendothelioma
Figure 6-18-13
Diffuse hepatic involvement of
hemangioendothelioma
Figure 6-18-14
Multiple discrete liver hemangiomas

in patient who is clinically
asymptomatic
Figure 6-18-15
Draping peripheral feeding vessels in

liver hemangioma
Dynamic CT with centripetal contrast

enhancement of liver multiple
hemangiomas

Infantile Hemangioendothelioma: Treatment Figure 6-18-16
• Spontaneous involution in 12–18 months
• CHF- embolotherapy with interferon
➢ Interferon-alpha 1, 2a, 2b response in weeks
• Embolize hepatic artery- coils, balloons PRN
➢ A-V shunts
➢ Particles (large) where possible
• May couple with surgery PRN
Hemangioendothelioma
• Age: Young (Infants)
• Biologic imaging: Photomicrograph of hepatoblastoma
➢ Vascular-High flow state demonstrating cords and nests of
➢ Solid stroma malignant cells
• Chemistry: Normal alpha-fetoprotein
➢ Positive endothelial growth factor
Figure 6-18-17
Hepatoblastoma
• Most common primary liver tumor in childhood
• Third most common abdominal malignancy
➢ After neuroblastoma, Wilms’ tumor
• May be familial
• Associations: Trisomy 18, Beckwith-Wiedemann,
hemihypertropy, familial polyposis, exposure- metals,
petroleum products, paints, oral contraceptives, Fetal
Alcohol Syndrome
Hepatoblastoma: Pathology- Gross

• Solitary 80%, right lobe predominance
• May be lobulated Slide left with right hepatic lobe nodular solid
• 5–20 cm hepatoblastoma in an otherwise normal liver.
• Nodular with fibrous bands throughout Slide right with same tumor demonstrating the
• Fleshy; +/– hemorrhage, necrosis, Ca2+ typical nodular appearance in the gross specimen
• Rare diffuse infiltration, adjacent liver-normal
Hepatoblastoma: Pathology-Histologic [Figure 6-18-16] Figure 6-18-18

• Epithelial
➢ Fetal 30%, Pink-cytoplasm, sparse mitoses
➢ Embryonal 20%- blastemic appearance (blue-H&E)
➢ Macrotrabecular- cords of tumor cells- 3%
➢ Small cell- 3%
• Mixed epithelial-mesenchymal- 45%; Teratoid
➢ osteoid differentiation, also muscle, cartilage
• AFP stain positive
Hepatoblastoma: Clinical
• Abdominal mass, hepatomegaly
• Anemia
• Child 90% < 5 yrs old, 65–70% under 2 yrs
• Males> females 2:1
• Positive alpha-fetoprotein
• No prior history of liver disease
Hepatoblastoma: Imaging
[Figures 6-18-17 to 6-18-19]
• Hepatomegaly, abdominal mass, 10–15% Ca2+
• US: Heterogeneous, predominantly solid
• CT: Heterogeneous, lobulated Unenhanced CT with focus of
• MR: Low T1, High T2 calcification in a teratoid variety of
• Angio: Tumor neovascularity hepatoblastoma. Heterogeneous
enhancement of same tumor involving
left and right hepatic lobes

Hepatoblastoma: Treatment [Figure 6-18-20] Figure 6-18-19
• Pre-op chemotherapy ( 50%…90%)
• Surgical: Resectable 90%; 35% mortality
➢ Fetal best prognosis
• Vascular definition- multisegmentectomy
• Mets: Pulmonary, periaortic nodes, brain
• Chemoembolization/RF after surgery PRN
Hepatoblastoma
Heptoblastoma with solid nature in CT and gross
• Age: Young (infants, young children) specimen. Cystic change is irregular due to
• Biologic imaging features: Solid central necrosis of the solid tumor
• Chemistry: Positive alpha-fetoprotein
Figure 6-18-20
• #2 primary liver malignancy in childhood
• Often underlying liver disease/cirrhosis
➢ Tyrosinemia, alpha-1-antitrypsin deficiency, glycogen storage
dz, biliary atresia, chronic hepatitis (HBsAg +), Fanconi
anemia, methotrexate induced hepatic fibrosis
• May be primary
Hepatocellular Carcinoma: Pathology [Figure 6-18-21]

• Multinodular, diffuse; less common solitary
• 2–25 cm
• Hemorrhage, cysts scattered with nodules
• Fibrous/cirrhotic background
• Adjacent liver abnormal
• Cords and nest- malignant hepatocytes
• Fibrolamellar variant: favorable prognosis Chemoembolization of residual tumor
focus following surgery in patient who
Hepatocellular Carcinoma: Clinical is not a liver transplantation candidate
• Abdominal mass, hepatomegaly
• Abdominal pain Figure 6-18-21
• Child > 4 yrs old, 12–14 yrs mean age
• Male predominance > 2:1
• Positive alpha-fetoprotein
• + prior history of liver disease
Hepatocellular Carcinoma: Imaging

[Figures 6-18-22 and 6-18-23]
• Hepatomegaly, abdominal mass
• US: Heterogeneous, mostly solid, hypoechoic
• CT: Hypodense/isodense
➢ Heterogeneous enhancement Nodular nature(cords and nests of
• MR: Low T1, High T2 (esp Fat Sat FSE), tumor cells) of solid hepatocellular
➢ PV invasion carcinoma
• Similar appearance to aggressive hepatoblastoma
Figure 6-18-23
Figure 6-18-22
Nodular
appearance of
solid epithelial
tumor
(hepatocellular
carcinoma) in
14 year old
patient, with
positive AFP
Nests of inhomogeneously enhancing
HCC

Hepatocellular Carcinoma: Treatment [Figures 6-18-24 to 6-18-26] Figure 6-18-24
• Pre-op chemotherapy
• Surgical: Resectable 20%, >75% mortality
➢ Fibrolamellar- best prognosis
• Vascular definition- multisegmentectomy
• Chemoembolization/RF after surgery PRN
• Age of patient: Teens
• Biologic imaging: Solid, multifocal
• Chemistry-blood
➢ Positive alpha-fetoprotein
Undifferentiated Embryonal Sarcoma

• #4 liver malignancy in childhood RF ablation of focal hepatoma (left image) with
• AKA malignant mesenchymoma needle seen during US guided RF ablation (right
image).
• Highly aggressive neoplasm
• Most children 6–10 yrs old, M=F
• Abdominal mass, fever, wt loss Figure 6-18-25
• Normal alpha-fetoprotein
Undifferentiated Embryonal Sarcoma:

Pathology
• Solid, globular, areas of necrosis/hemorrhage
• 2–30 cm
• Right lobe dominance (75%)
• Fibrous pseudocapsule, occasional-pedunculated
• Spindle/stellate shaped sarcomatous cells
• Myxoid background
• Local recurrence and metastasis
Left slide with small echogenic focus of
Undifferentiated Embryonal Sarcoma: microbubbles form at the tip of needle (arrow) in
Imaging [Figures 6-18-27 to 6-18-30] the earliest phase of coagulation necrosis. The
• Hepatomegaly, abdominal mass small focus of microbubbles grows to become a
• US: Complex mass large area of echogenic necrotic tumor (right)
• CT: Hypodense-heterogeneous enhancement
• MR: Low T1, High T2 Figure 6-18-26
• Angiography: Hypovascular
Figure 6-18-27
Focal scar 1 year following RF tumor

Figure 6-18-28 ablation
Complex US appearance of
embryonal sarcoma in 11 year female
Demonstrable pseudocapsule
anteriorly surrounding mesenchymal
sarcoma

MR with heterogeneous cystic and solid foci in

embryonal sarcoma
Multiple areas of cystic change in
embryonal sarcoma in 10 y/o male
Undifferentiated Embryonal Sarcoma: Treatment

• Surgical: Resection
• Chemotherapy, XRT
• Poor prognosis
Figure 6-18-31
• Mean survival = 12 months
• Recurrence Dx 12–16 months after surgery
Undifferentiated Embryonal Sarcoma

• Age of patient: Adolescents/teens
• Biologic imaging: Mixed solid/cystic
• Chemistry-blood
➢ Negative alpha-fetoprotein
Liver Metastases [Figures 6-18-31 and 6-18-32]

• Neuroblastoma- Stage 4 and 4s
• Burkitt’s lymphoma
• Wilms’ tumor
• Leukemia (AML) Studded appearance of stage 4S
• Sarcomas neuroblastoma diffusely involving the
• Other malignancies liver
ABC’s of Pediatric Liver Tumors Figure 6-18-32

• Age of patient
• Biologic imaging features
• Chemistry-blood
➢ Alpha fetoprotein
➢ Endothelial growth factor
Pediatric Liver Tumors: Summary

Summary chart of classic features of common pediatric liver tumors
Tumor Age Characteristics

Inf. Hemangio < 1 yr Solid, Ca+2, vasc, AFP (-), involutes
with interferon
Mes Hamartoma < 2 yr Cystic > solid, AFP (-)
Typical appearance of Burkitt
Hepatoblastoma < 3 yr Ca+2, solid, vasc, AFP(+) solitary Lymphoma involving both the liver
HCC > 4 yr Solid, vasc, AFP (+), multifocal and the posterior gastric wall in
Emb Rhabdo < 5 yr Solid > cystic, mild vasc, AFP(-) patient with fatty liver
UES > 6 yr Cystic > solid, AFP (-)
Metastases any Solid or cystic

References
1. Von Schweinitz, D. Management of liver tumors in childhood. Semin Ped Surg 2006; 15(1):17-24
2. Stocker JT. Hepatic tumors in children. Clin Liver Dis. 2001 Feb;5(1):259-81, viii-ix.
3. von Schweinitz D. Neonatal liver tumours. Semin Neonatol. 2003 Oct;8(5):403-10.
4. Tiao GM, Bobey N, Allen S, Nieves N, Alonso M, Bucuvalas J, Wells R, Ryckman F. The current management of
hepatoblastoma: a combination of chemotherapy, conventional resection, and liver transplantation. J Pediatr. 2005
Feb;146(2):204-11.
5. Burrows PE, Dubois J, Kassarjian A. Pediatric hepatic vascular anomalies. Pediatr Radiol. 2001 Aug;31(8):533-45.
6. Dubois J, Hershon L, Carmant L, Belanger S, Leclerc JM, David M. Toxicity profile of interferon alfa-2b in children:
A prospective evaluation. J Pediatr. 1999 Dec;135(6):782-5.
7. Dachman AH, Pakter RL, Ros PR, Fishman EK, Goodman ZD, Lichtenstein JE. Hepatoblastoma: radiologic-pathologic
correlation in 50 cases. Radiology. 1987 Jul;164(1):15-9.
8. Gerber DA, Arcement C, Carr B, Towbin R, Mazariegos G, Reyes J. Use of intrahepatic chemotherapy to treat advanced
pediatric hepatic malignancies. J Pediatr Gastroenterol Nutr. 2000 Feb;30(2):137-44.
9. Sun XY, Wu ZD, Liao XF, Yuan JY. Tumor angiogenesis and its clinical significance in pediatric malignant liver tumor.
World J Gastroenterol. 2005 Feb 7;11(5):741-3.
10. Rhim H, Dodd GD 3rd, Chintapalli KN, Wood BJ, Dupuy DE, Hvizda JL, Sewell PE, Goldberg SN. Radiofrequency
thermal ablation of abdominal tumors: lessons learned from complications. Radiographics. 2004 Jan-Feb;24(1):41-
52.
11. Iannitti DA, Dupuy DE, Mayo-Smith WW, Murphy B. Hepatic radiofrequency ablation. Arch Surg. 2002
Apr;137(4):422-6.

Pediatric Hip Sonography:
Practical Radiologic Pathology
Figure 6-19-1
Ultrasound of the Hip
• Developmental dysplasia
• The irritable hip
➢ Septic arthritis, toxic synovitis,
➢ Arthritis, LCP, hemophilia
• US guided intervention
• Practical points
DDH - Risk Factors

• Family History: 12-36%
• Breech: up to 23% (esp female)
• Torticollis, metatarsus adductus, oligohydramnios
Coronal left hip sonography:
Transducer in right hand, left hand
DDH - Ultrasound Technique
• High frequency linear pistons the left leg/hip
• Static views (Graf)
➢ Coronal Figure 6-19-2
• Dynamic views (Harcke)
➢ Transverse and coronal
➢ Barlow maneuver
• Standard minimum exam
Dynamic Standard Minimum Exam [Figures 6-19-1 and 6-19-2]

• Static coronal image
• Static transverse image
• Transverse image with stress
Coronal Image [Figures 6-19-3 and 6-19-4]

Transverse right hip sonography:
Transducer in left hand, right hand
pistons the right leg/hip
Figure 6-19-3
Figure 6-19-4
Coronal hip anatomy illustrates hyaline cartilage of

the acetabular roof, femoral head, and triradiate
cartilage; fibrocartilaginous acetabular labral tip
Essential static coronal hip sonogram
with Graf alpha angle. Note the
critical horizontal orientation of the
iliac body for accurate depiction of
normal and pathologic anatomy
Pediatric Hip Sonography 1516

Static Transverse Image [Figure 6-19-5] Figure 6-19-5
DDH - Coronal
• Rigidly standardized protocol
➢ Exam technique, interpretation
➢ Alpha, beta angles
• Reproducible
• Large European experience
DDH - Practical Grading

• Normal - Graf I
• Physiologically Immature - Graf IIa
• Dysplasia - Graf IIb and above
The Irritable Hip

• Toxic synovitis
• Septic arthritis
• Arthritis - JRA, post-infectious
• Legg-Calve-Perthes
• Hemophilia Note femoral metaphysis in view in
the transverse image obtained with
The Irritable Hip the hip flexed. Femoral head deeply
• Pain seated in the hip socket
• Limitation of motion
• Fever, WBC, ESR, CRP
• 50% with effusion
Hip Effusion - Ultrasound

• Direct visualization
• More sensitive than plain film
• Guided aspiration

1519 Pediatric Hip Sonography
Hip Effusion - Ultrasound Figure 6-19-6
• High frequency linear
• Supine, hip neutral
• Anterior, parallel femoral neck
• Comparison views
Hip Effusion - Ultrasound

• Fluid distends joint capsule
• >2mm difference abnormal
• Fluid echogenicity unreliable
• Doppler flow unreliable
Septic Arthritis [Figures 6-19-6 and 6-19-7]

• Fever
• Elevated WBC, ESR, CRP
• Symptoms more severe
• Staph, strep, H. flu Cursors delineate the effusion in the
• Must obtain fluid for Dx anterior hip joint space
Essential Principles of US Guidance [Figure 6-19-8] Figure 6-19-7
Figure 6-19-8
Small hip effusion in left image;

Critical principles of sonographic guidance; needle right image with needle aspiration of the effusion
aligned in the center of the sound beam, straight
longitudinal alignment of needle along the
transducer face/sound beam short axis
References
1. Harcke HT, Grissom LE. Pediatric hip sonography. Diagnosis and differential diagnosis. Radiol Clin North Am.
1999 Jul;37(4):787-96.
2. Grissom LE, Harke HT. Developmental Dysplasia of the Pediatric Hip with Emphasis on Sonographic Evaluation.
Semin Musculoskelet Radiol. 1999;3(4):359-370
3. ACR Practice Guideline for the Performance of the Ultrasound
Examination for Detection of Developmental Dysplasia of the Hip. American College of Radiology Practice
Standards/Guidelines 2004. American College of Radiology, Reston, VA.Headquarters Office: 1891 Preston White
Dr, Reston, VA 20191, (703) 648-8900© 2004 American College of2004 American College of Radiology
4. Eich GF, Superti-Furga A, Umbricht FS, Willi UV. The painful hip: evaluation of criteria for clinical decision-
making. Eur J Pediatr. 1999 Nov;158(11):923-8.
5. Buchmann RF, Jaramillo D. Imaging of articular disorders in children. Radiol Clin North Am. 2004 Jan;42(1):151-
68, vii.
6. Givon U, Liberman B, Schindler A, Blankstein A, Ganel A. Treatment of septic arthritis of the hip joint by repeated
ultrasound-guided aspirations. J Pediatr Orthop. 2004 May-Jun;24(3):266-70.
Pediatric Hip Sonography 1518

Pediatric Seminar 1: Pulmonary Infections
CASE 1: 5 month with fever and cough
Normal thymus mimicking RUL pneumonia
Follow-up
Follow-up radiograph in one month shows expected decrease

in size of the thymus and the thymic sail sign
Normal Thymus
• Sail sign, wave sign, notch or sulcus
• No mass effect on trachea or vessels
• Homogeneous in density
• Commonly seen on CXR up to age 5 years then regresses in latter half of first
decade
• Thymic rebound
• Masses can arise in thymus – leukemia/lymphoma, teratoma, thymolipoma,
lymphatic malformation

1521 Pediatric Seminar 1: Pulmonary Infections
CASE 2: 12 yo with fever and cough
Round Pneumonia
• Unique to children – usually < 8 yo
• Usually posterior lower lobes
• Almost always bacterial –
pneumococcus
• Exclude bone erosion – ddx: neoplasm
CT reveals a complex mass in the

right posterior mediastinum with
calcifications and extension through
the chest wall and into the spinal
canal. This is neuroblastoma
Tumors that look like pneumonia

• Neuroblastoma In the differential of round pneumonia is mediastinal or chest
• Chest wall masses wall mass. PA and lateral chest radiographs show a round
mass in the region of the medial right lower lobe. Note the
• Parenchymal mass splaying of the ribs on the right adjacent to the mass. This is
➢ Pleuropulmonary blastoma therefore a mass and not a pneumonia
➢ Plasma cell granuloma
➢ Cyst
Pulmonary Blastoma
• Arises from primitive mesenchymal blastema
• Histologically reminiscent of Wilms tumor
• Unlike other embryonal tumors, it is more commonly found in adults
• May present as solitary nodule or huge mass
• Heterogeneous, cystic areas
Plasma Cell Granuloma

• AKA inflammatory pseudotumor
• Localized proliferation of a variety of cells, mostly plasma cells
• Reactive, organizing pneumonia
• Patient often asx
• Chunky calcifications
• May be cystic
• May have spiculated margins or air bronchograms
Pediatric Seminar 1: Pulmonary Infections 1520

CASE 3: 17 mo with fever and cough without improvement after
1 week of oral abx
PA and lateral chest radiographs show dense opacification of the right Ultrasound of the right chest
lower lobe with silhouetting of the right hemidiaphram. Right lateral shows pleural fluid with
pleural thickening is also seen septations and consolidated,
airless lung that transmits sound.
Complicated Pneumonia The hyperechoic foci with ring-
• Suspect in cases of near white out down artifact represent round air
• H. flu less than 2 y.o. collections
• Pneumococcus
• S. aureus
• CT vs. US
➢ Ultrasound may be
better determining
which fluid collections
need drainage
Multiple axial CT images show the

pleural fluid adjacent to the right
lower lobe consolidation. Note that
the lung enhances and is not
necrotic. The fluid-filled cavities with
non-nondependent air collections are
therefore most consistent with small
abscesses rather than necrotic
cavities
CASE 4: 17 yo with fever and cough, seizure disorder and DM
Round cavity in superior segment of

right upper lobe. Clinical history
suggests mild immunocompromise
and possibility of aspiration

• Pulmonary abscess
• Cavitary pulmonary necrosis
• Pneumatocele
Pulmonary Abscess
• CT – fluid or air-filled cyst with enhancing, thick, irregular wall
• Both necrosis and small abscesses in children have good outcomes with
antibiotics only
Pneumatoceles
• Thin walled cavity seen in the recovery phase usually
of infection
• Staphylococcus, pneumococcus, tuberculosis
• Blunt chest trauma, hydrocarbon pneumonitis,
Langerhans cell histiocytosis
• Bronchial obstruction leading to air trapping and
alveolar rupture Pulmonary necrosis. Contrast this appearance to
• Pneumothorax or mediastinum case #3 with empyema and abscesses. Note
decreased enhancement of surrounding
parenchyma compared to more anterior
parenchyma with preservation of enhancement of
the visceral pleura (arrow)
CASE 5: Adult with history of pulmonary infection as a child
Left PA chest radiograph shows asymmetric density of lungs and

smaller left PA compared to right. Right expiratory PA chest
radiograph shows air trapping in left lung
Swyer-James Syndrome
➢ Idiopathic, viral, toxic inhalation,drug reaction, collagen vascular dz,
transplant, chronic aspiration.
➢ Adenovirus as child – unilateral hyperlucent lung
• Small hyperlucent lung, hypoplastic ipsilateral artery
• Reticular nodular pattern with hyperinflation, central bronchiectasis

CASE 6: Adult with right chest pain
A round mass is seen in the right phrenicovertebral angle posterior to

the right atrium
• Bronchopulmonary foregut
malformation
• CPAM
• Neurogenic tumor
• Sequestration
Sequestration
• Area of pulmonary tissue that
does not have a normal
connection to the bronchial tree
• Systemic arterial supply
• Can present as an infant or
young child or as a young adult
• Often a history of recurrent
infections in the same lobe
• May contain air
Sequestration
• LLL most common
• Multiloculated cystic or solid
mass
• Intralobar vs. extralobar
➢ Pleural investment CT reveals a predominantly cystic mass with focal nodular thickening
➢ Extalobar – own pleural along the posterior wall in the right posterior mediastinum. The lower
investment right image reveals an additional simple cyst in the left superior
mediastinum
➢ Extralobar – systemic venous
drainage
➢ Extralobar – infants, associated anomalies
➢ Extralobar-congenital/intralobar-acquired
• CT vs. MRI vs. US – define vascular supply and drainage
Arteriogram shows the blood supply to the right lower

lobe sequestration is coming from the celiac axis below
the diaphragm. Abdominal blood supply frequent, and
alters the surgical approach. The superior mediastinal
lesion was a foregut duplication cyst

CASE 7: 9 yo with SOB
PA and lateral chest radiographs show bilateral lower lobe

consolidation with silhouetting of both hemidiaphragms. Also note the
large cardiac silhouette, absence of the splenic shadow, and
cholecystectomy clips (unusual in children)
Acute Chest Syndrome

• Fever, chest pain, shortness of breath
• Rib infarction with splinting and atelectasis versus infection
• Treated with oxygen, antibiotics and pain medication +/- plasma exchange
• Look for associated findings of SS
➢ CM
➢ Absent splenic shadow
➢ Evidence of gallbladder disease
➢ Skeletal findings
CASE 8: 5 yo with fever and SOB

Pediatric TB – Clinical Findings
• Asymptomatic
• Cough
• Fever
• Malaise / FTT
• Respiratory distress
• Lethargy
Pediatric TB – Radiologic Findings

• Normal
• Focal infiltrate – Ghon lesion Left image shows unilateral hilar and right paratracheal
• Unilateral hilar adenopathy – Ranke adenopathy.
complex Right image also shows secondary RML atelectasis
• Paratracheal adenopathy
• Subcarinal adenopathy
• Calcified granulomatous nodes

Pediatric Seminar 2: Skeletal Dysplasia
Radiologic Approach
• Assess Proportion
➢ Rhizo-, meso-, or acromelia
➢ +/- platyspondyly
• Assess Components of Bone
➢ Epiphyses small or irregular ◗ epiphyseal dysplasia
➢ Metaphyses widened, flared, or irregular ◗ metaphyseal dysplasia
➢ Diaphyses widened or thickened ◗ diaphyseal dysplasia
Achondroplasia Group
• All have abnormalities of the same chromosomal locus and gene product,
fibroblast growth factor receptor 3 (FGFR3)
➢ Thanatophoric dysplasia
➢ Achondroplasia
➢ Hypochondroplasia
Thanatophoric Dysplasia
• AD
• Probably the most common lethal bone dysplasia
• Skull - kleeblatschadel in type II
• Thorax - very short ribs and handlebar clavicles
• Spine – small flat vertebral bodies with round
anterior ends, U or H-shaped on AP
Thanatophoric Dysplasia
• Pelvis
➢ Small, flared iliac bones
➢ Very narrow sacrosciatic notches, flat dysplastic Thanatophoric dysplasia. Note small flat vertebral
bodies, very short ribs, and “telephone receiver”
acetabula femora
• Extremities – telephone receiver
• Femora
Achondroplasia
• Most common nonlethal skeletal dysplasia
• AD, spontaneous mutation rate 8-%
• Skull
➢ Large with midface hypoplasia
➢ Small skull base and foramen magnum
Achondroplasia
• Spine
➢ Very short pedicles – risk of spinal canal stenosis
➢ Decrease in interpediculate distance – lumbar spine
• Pelvis
➢ Elephant-ear iliac wings
➢ Flat acetabular roofs
➢ Narrow sacrosciatic notches
Achondroplasia
• Extremities
➢ Rhizo- > meso- and acromelia
➢ Hands – brachydactyly with metaphyseal cupping of MC’s
➢ Knees – chevron and inverted chevron deformities
➢ Hips proximal femoral fade out and hemispheric capital femoral epiphyses

1527 Pediatric Seminar 2: Skeletal Dysplasia
Case 1 – 5 yo with short stature
Achondroplasia. AP view of the pelvis

shows flared “elephant ear” iliac
wings, shallow acetabular roof,
Achondroplasia. AP view of the extremities show short widened narrowed sacrosciatic notches
bones with flared metaphyses and chevron deformities of the conferring a champagne-glass
distal femora and proximal tibiae configuration to the pelvis, and
narrowing of the interpediculate
distance in the lower lumbar spine
Short Rib-Polydactyly Group
• Includes
➢ SRP I-IV - some with, some without polydactyly
➢ Asphyxiating thoracic dysplasia
➢ Chondroectodermal dysplasia
• Shortest ribs of all dysplasias
Short Rib-Polydactyly
• Thorax – shortest ribs, horizontal ribs
• Pelvis – small ilia, notched acetabula
• Extremities
➢ Micromelia
➢ Rolling pin-shaped or round-ended or spiked
femora
➢ Ovoid tibiae
➢ Polydactyly in some types
Asphyxiating Thoracic Dysplasia (Jeune

Syndrome) Achondroplasia. AP views of the hands show
• Mixed prognosis brachydactyly with cupping of the metacarpal
➢ Some succumb early from respiratory metaphyses
compromise
➢ Others die later from progressive nephropathy
• Thorax
➢ Long and barrel-shaped
➢ Handlebar clavicles
➢ Short horizontal ribs with flared ant ends
Asphyxiating Thoracic Dysplasia (Jeune Syndrome)

• Spine – normal
• Pelvis
➢ Trident acetabular roof
➢ Flared iliac wings
➢ Narrowed SS notches
• Extremities – cone-shaped epiphyses in hands
Pediatric Seminar 2: Skeletal Dysplasia 1526

Case 2 – Newborn with severe respiratory distress
Asphyxiating thoracic dystrophy.

Gross photo shows bilateral
Asphyxiating thoracic dystrophy polydactyly of the hands and very
small chest
Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome)

• Nonskeletal findings important in diagnosis
➢ Hair, nail and teeth abnormalities
➢ Congenital heart disease
• Thorax – small with short ribs
• Pelvis
➢ Trident acetabula
➢ Small, flared iliac wings
➢ Narrowed SS notches
Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome)

• Extremities
➢ Generalized shortening
➢ Exostosis of proximal medial tibia
➢ Post-axial polydactyly
➢ Capitate-hamate fusion
➢ Extra carpal bone
➢ Cone-shaped epiphyses
Case 3 – 6 year old with history of congenital heart disease
Chondroectodermal dysplasia. AP
views of the hands show bilateral
postaxial polydactyly and cone
shaped epiphyses (arrows)

Chondrodysplasia Punctata Group
• All have epiphyseal stippling
• Rhizomelic
➢ AR, death in first year
➢ Spine – coronal clefts
➢ Symmetric bilateral shortening of femora
• Conradi-Hunermann
➢ X-linked dominant
➢ Asymmetric shortening of limbs
➢ Diffuse stippling of the spine
Case 4 – newborn boy with respiratory distress
Chondroectodermal dysplasia. AP
view of pelvis and LE’s shows short
long bones and short, flared iliac
wings, with trident-shaped acetabular
roofs
Chondrodysplasia punctata. Note diffuse stippled

epiphyses and coronal clefts in the thoracic spine
Metaphyseal Chondrodysplasia Group

• All have normal spine and wide irregular metaphyses
• Jansen-type
➢ Most severe
➢ Infantile presentation
➢ AD
➢ Extremities – extensive irregular, expanded metaphyses
➢ Hyperparathryroidism
Metaphyseal Chondrodysplasias
• Schmid-type – mildest, metaphyseal flaring, especially around knees
• Shwachman-Diamond – AR
➢ Pancreatic insufficiency – malabsorption and lipomatosis of pancreas
➢ Cyclic neutropenia – recurrent infections
Metaphyseal Chondrodysplasias
• McKusick-type
➢ Cartilage-hair hypoplasia
➢ High frequency in the Amish and Finnish populations
➢ Hirschprung disease
➢ Immune deficiency and increased risk of malignancy, especially leukemia
and lymphoma

Case 5 – 12 yo boy with short stature and unusual hair
McKusick-Type
• Spine – square vertebral bodies
• Extremities – flaring, cupping and fragmentation of
metaphyses, especially at the knees
• Hands – shortening with metacarpal and phalangeal
cupping and coning
Dysplasias with Prominent Membranous

Bone Involvement
➢ AD, marked variability in expression
➢ Drooping narrow chest, hypermobile shoulders, Metaphyseal chondrodysplasia, McKusick type.
Note widening and irregularity of metaphyses
and dental anomalies about the knees
➢ Mild short stature
➢ Skull – wormian bones and wide, open anterior fontanelle
Dysplasias with Prominent Membranous Bone Involvement

➢ Thorax – hypoplasia or absence of clavicles, downward sloping ribs
➢ Spine – posterior wedging of vertebral bodies
➢ Pelvis - high, narrow iliac wings, absence or hypoplasia of pubic bones
➢ Extremities – tapered distal phalanges
Case 6 – Fretful 8 mo whose pediatrician thinks he has bilateral

clavicular fractures
Cleidocranial dysplasia with wormian bones
Cleidocranial dysplasia. With

hypoplastic clavicles with
pseudarthroses
Cleidocranial dysplasia. Note

absence of ossified pubic bones, Cleidocranial dysplasia.
narrowed sacrosciatic notches and Tapered distal phalanges
narrow ilia

Dysostosis Multiplex Group
• Mucopolysaccharidoses and mucolipidoses
• All AR
• All produce similar radiographic complex of findings
Hurler Syndrome
• Present in infancy or early childhood
• Skull – J-shaped sella
• Thorax
➢ Short thick clavicles
➢ Oar-shaped ribs
• Spine
➢ Gibbus deformity
➢ Inferior beaked T-L vertebral bodies
Hurler Syndrome
• Pelvis – small flared iliac wings with inferior tapering and steep acetabular
roofs
• Extremities
➢ Wide diaphyses of long bones and metacarpals
➢ Pointed proximal metacarpal poles
Case 7 – short 3 yo with unusual facial appearance
Dysostosis multiplex due to Hurler syndrome.

AP chest shows thick clavicles and paddle-
shaped ribs. Lateral spine shows gibbus
deformity at thoracolumbar junction and
inferior beaking of vertebral bodies
Hurler syndrome. AP pelvis shows
flared iliac wings with inferior tapering
and steep acetabular roofs
Hurler syndrome. Another patient

with a J-shaped sella. Dental
abnormalities are related to
enlargement of the tongue
Another patient with Hurler syndrome showing the

pointed proximal poles of the metacarpals

Morquio Syndrome
• No J-shaped sella
• Vertebral beak is in the middle
• Ribs are widened but not oar-shaped
• Proximal metacarpal poles are rounded
Dysplasias with Decreased Density

• Very large group of conditions that share an abnormality of type I
collagen
• Osteogenesis Imperfecta Type II
➢ Invariably lethal Hurler syndrome. Second patient with
➢ Skull – poor or absent ossification coarsening of the soft tissues of the
➢ Thorax – small chest with beaded ribs face including the tongue
Osteogenesis Imperfecta
• Type II
➢ Spine – very poor ossification with collapse of vertebral bodies
➢ Extremities – accordion femora
• Other types
➢ Skull – more than 8-10 wormian bones, variable ossification
➢ Extremities – variable osteoporosis and fractures
Case 8 – 31-week fetus
Osteogenesis imperfecta. Prenatal ultrasound shows no

shadowing by the skull. The near side of the brain is much too
well visualized. Also the transducer is indenting the skull
Increased Bone Density

• Osteopetrosis (Albers-Schonberg Disease)
➢ Failure to resorb primary spongiosa
➢ Severe precocious type – AR
➢ Delayed type – AD
➢ Reduced bone marrow space ◗ anemia and extramedullary
hematopoesis
Osteopetrosis – Radiographic Findings

• Generalized increased bone density
• Skull – thick and dense especially at the base with foraminal
narrowing
• Spine – “sandwich” or “picture-frame” vertebral bodies
• Extremities
➢ Widened metaphyses with dense bands OI. No ossification of the
membranous portions of the skull.
➢ Bone-within-bone appearance Small chest with beaded ribs and
accordioned long bones due to
multiple in utero fractures

Osteopetrosis with diffusely dense
bones, bone-within-bone
appearance, and deformity due to
pathologic femoral neck fractures
Osteopetrosis. Acute
Osteopetrosis with sandwich femoral neck fracture on
vertebrae earlier film
Pyknodysostosis
• AR, presents in infancy
• Micrognathia, short fingertips, fractures
• Generalized osteosclerosis
• Skull
➢ Wormian bones
➢ Marked delay in closure of sutures and fontanelles
➢ Obtuse mandibular angle
• Thorax – resorption of acromial ends of clavicles
• Extremities – resorption of phalangeal tufts
Case 9 – 17 year old with short stature
Pyknodysostosis. Skull shows

persistent unfused sutures and
very obtuse mandibular angle
Pyknodysostosis. Diffuse increased

bone density and resorption of
phalangeal tufts

Pediatric Seminar 3: Cystic Fibrosis and
Pulmonary Infections of the
Immunocompromised Child
Case 1 – 15 yo with fever and respiratory distress
Cystic Fibrosis Epidemiology1

• Most common lethal autosomal
recessive disease in white populations
(1:2500 live births)
• Rare in blacks (4% of cases)
• Very rare in Asians (0.2% of cases)
• Exceedingly rare in Native Americans
(0.02% of cases)
1Cystic Fibrosis Foundation Registry 1990
Annual Data Report.
PA chest radiograph shows
bilateral hyperinflation and
Cystic Fibrosis Epidemiology1 right much greater than left
• Mean age at diagnosis 6 mo linear and nodular opacity
• 80% diagnosed by age 3 years predominantly in the upper
• 10% of newly diagnosed are 18 years or lobes.
older Waters view of the paranasal
sinuses demonstrates
• 52.8% male complete opacification of the
• 11% of new diagnoses due to family paranasal sinuses
history of CF
1Cystic Fibrosis Foundation Registry. 2004 Annual Data Report.
Cystic Fibrosis CF Gene

• Long arm of chromosome 7
• Encodes a large single chain protein, CF transmembrane conductance
regulator (CFTR)
• CFTR forms cell membrane chloride channel
• 3 base pair deletion (delta F508 mutation) accounts for 70% CF cases
• Remaining cases due to over 1100 different mutations
Cystic Fibrosis Cellular Physiology

• Normal CFTR: Epithelial chloride channel supplies luminal water by osmosis
• Abnormal CFTR: Decreased water flow produces viscous inspissated luminal
secretions
• Exocrine duct obstruction
• Enhanced bacterial colonization
Cystic Fibrosis Exocrine Sites

• Bronchioles and small bronchi
• Pancreas
• Intestinal crypts
• Biliary ducts
• Vas deferens
• Cervix
• Sweat and salivary glands

1535 Pediatric Seminar 3: Cystic Fibrosis
Cystic Fibrosis Clinical Presentation
• Neonatal intestinal obstruction in 16-20%
• Respiratory manifestations & pansinusitis
• Failure to thrive
• Malabsorption
• Unexplained hypochloremic acidosis
• Positive family history
Cystic Fibrosis Protean Expressions

• Pulmonary Manifestations
➢ Recurrent infection
➢ Pulmonary insufficiency
• Gastrointestinal Manifestations
➢ Pancreatic abnormalities
➢ Intestinal obstruction
➢ Nonobstructive bowel manifestations
➢ Hepatobiliary disease
➢ Gastrointestinal malignancy
Cystic Fibrosis Lung Disease

• Principal cause of morbidity and mortality
• Abnormal mucus obstructs terminal airways
• Decreased mucociliary transport
• Air trapping & increased dead space
• Colonization by S. Aureus, H. flu, Pseudomonas sp., atypical mycobacteria,
Burkholderia cepacia complex
• Bronchiectasis
• Alveoli usually spared
Cystic Fibrosis Pathogenesis of Lung Disease

• Airway macrophages promote neutrophil (PMN) influx
• Elastase from autolyzed PMNs digests elastin, causing bronchiectasis and
fibrosis
• Elastase is powerful mucus secretagogue
• Neutrophil death release high molecular weight DNA (Pulmozyme)
• Abnormal CFTR protein may bind pathogens (mucoid strain of Pseudomonas)
Cystic Fibrosis Pulmonary Complications

• Pneumothorax
• Allergic bronchopulmonary aspergillosis
• Acute and chronic respiratory failure
• Hemoptysis from dilated bronchial arteries
• Pulmonary hypertension
• Cor pulmonale
Lung Disease in CF Pathologic Features

• Bronchi filled with mucoid exudate laden with degenerating neutrophils
• Bronchial mucosa features increased goblet cells & focal metaplastic
squamous epithelium
• Ciliary changes
• Bronchiectasis
Pediatric Seminar 3: Cystic Fibrosis 1534

Case 2 – 18 yo with respiratory distress
PA chest radiograph shows tunneled

central venous catheter, bilateral
linear and confluent opacities, and
bilateral hilar enlargement
Lung Disease in CF Radiographic Features

• Bronchial impaction (“finger in glove” appearance)
• Saccular bronchiectasis with upper lobe preponderance
• Thin-walled subpleural cysts
Case 3 - 14 yo with fever and cough

Lung Disease in CF Cysts
• Cyst seen on CXR - saccular bronchiectasis vs abscess
• Less likely pneumatocele
• AFL may be seen in bronchiectasis or abscess
• Abscess rare in older children except with CF
• Subpleural blebs may be seen on HRCT
Case 4 – 13 yo with recurrent respiratory tract infections
PA radiograph of the chest shows a

round mass with air fluid level in the
right middle lobe

High resolution chest CT image on the left is in expiratory
phase of respiration and shows mosaic pattern typical of small
airways disease. Image on right shows dilated bronchi with
thickened walls due to bronchiectasis
Lung Disease in CF - HRCT

• Currently CT is not part of the routine follow-up of CF patients. CXR and PFT
are.
• HRCT is much more sensitive than CXR for bronchiectasis – 90%
• HRCT is much more sensitive for early and reversible changes of CF than
CXR or PFT
• HRCT is becoming an outcome surrogate for CF
• Objective evaluation of HRCT is prerequisite
Case 5 – 14 yo with malabsorption
Axial IV contrast enhanced CT image

shows complete replacement of the
pancreas with fat
Exocrine Pancreatic Insufficiency

• > 80% have clinical pancreatic insufficiency
• Insufficient lipolytic & proteolytic enzymes for normal digestion & absorption of
nutrients
• Steatorrhea correlates with enzyme output < 10% of normal
• Delta 508 mutation – higher incidence of pancreatic insufficiency and earlier
onset of lung disease and colonization with pseudomonas
“Pancreatic Sufficiency”
• 10-15% of CF patients
• Do not require enzyme supplements
• Better nutritional status
• Older at diagnosis – later onset of lung disease
• Lower Pseudomonas colonization rates
• May convert to pancreatic insufficiency with age (genetically determined)
Cystic Fibrosis Endocrine Dysfunction

• Glucose intolerance in 30-50%
• Diabetes mellitus develops in 1% of children & 13% of adults
• Screened annual starting at age 14
• DM due to pancreatic fibrosis & other unknown factors

Cystic Fibrosis of the Pancreas Pathologic Features
• Proximal duct obstruction from inspissated pancreatic juice
• Acinar atrophy & inflammation
• Progressive interstitial fibrosis
• Fatty replacement
• Duct ectasia
• Micro- & macrocysts
• Calcification – punctate and diffuse or chunky
Cystic Fibrosis of the Pancreas Imaging Findings

• Radiographs: Punctate pancreatic calcifications
• US: Small echogenic pancreas
• CT: Fat attenuation, +/- calcifications, small cysts, complete pancreatic
replacement by macrocysts (rare)
• MRI: Variable signal intensity depending on amount of fat & fibrosis
Case 6 - 12 yo with failure to thrive
Sonogram on the left shows simple cyst anterior to the SMV

and posterior to the left lobe of the liver. CT shows two simple
cysts in the pancreas
Case 6 – Newborn with emesis and abdominal distension
Contrast enema in the same patient

KUB of infant shows many loops of showing a microcolon. The ileum is of
dilated, unfolded bowel with soap larger caliber than the colon and
bubble lucencies in the right lower shows multiple filling defects (arrow)
quadrant

GI Manifestations of CF Intestinal Obstruction
• Meconium ileus
• Meconium plug syndrome
• Distal intestinal obstruction syndrome (“meconium ileus equivalent”)
• Intussusception
• Fibrosing colonopathy
GI Manifestations of CF Meconium Ileus

• Earliest clinical manifestation of CF
• 10-15% of CF patients present with meconium ileus
• All pts. with meconium ileus have CF
• Dysfunction of secretory intestinal epithelium plus panc enzyme insufficiency
• Distal small bowel obstruction from dessicated meconium pellets
Meconium Plug Syndrome

• Colonic obstruction in neonates
• Not a syndrome but a symptom
• 25% have CF
• The rest have functional immaturity of the colon or Hirshprung disease
• Contrast enema may relieve obstruction
Distal Intestinal Obstruction Syndrome (DIOS)

• Formerly termed “meconium ileus equivalent”
• Reserve dx for patients with obstruction
• 10-15 % of CF pts (usually adolescents & adults)
• Results from fluid loss and poor compliance with pancreatic
enzyme replacement
• May mimic appendicitis (appy uncommon in CF)
• Distal obstruction pattern on plain films
• Fecal mass in RLQ
• Enema may be therapeutic but usually treated medically
GI Manifestations of CF Rectal Prolapse

• Occurs in 20% of CF patients
• Presents in first years of life
• Resolves spontaneously by approx. 5 years
• Associated with bulky stools, diarrhea, or constipation
• Improved by pancreatic enzyme supplementation
GI Manifestations of CF Intussusception Contrast enema shows shortening

• Occurs in approx. 1% of pts. with CF and loss of haustration in ascending
• Mean age of presentation = 10 years colon with short focal narrowing. Also
• Usually ileocolic there is a large round filling defect
• Lead points: adherent fecal residue, enlarged lymphoid follicles, proximal to the narrowing.
Colonscopy revealed the narrowing
chronically distended appendix, or DIOS was a stricture and the filling defect
was an inflammatory pseudopolyp
GI Manifestations of CF Fibrosing Colonopathy
• Usually right colon
• High-strength pancreatic enzyme supplementation compounded by high
protease intake strongly implicated
• Submucosal fibrosis, fatty infiltration, mural thickening, haustral loss,
shortening, stricture formation

Case 7 – 10 yo with abdominal pain
Air lucency is seen in the wall of the

colon in the left upper quadrant on
this CT image representing
pneumatosis
Cystic Fibrosis Nonobstructive Bowel Manifestations

• Thickened nodular mucosal folds in duodenum & small bowel
• “Jejunization” of colon
• Duodenal ulcer seen at autopsy in 10%
• Gastroesophageal reflux
• Barrett esophagus
• Pneumatosis intestinalis
Cystic Fibrosis Hepatobiliary Disease

• Cholelithiasis (cholesterol stones) in 12-24%
• Microgallbladder at autopsy in 25%
• Atrophy or obstruction of cystic duct
• Distal CBD stricture
• Fatty liver
• Focal biliary cirrhosis and portal hypertension
Case 8 – 16 year old with hematemesis
Left images shows longitudinal

linear filling defects in the
esophagus indicating varices.
Also note bronchial artery
embolization coils. Axial image
from a contrast enhanced CT
shows fat density in the liver,
cholecystectomy clips, and a
large coronary vein in the left
upper quadrant. (Patient
underwent splenectomy as an
infant.)
Focal Biliary Cirrhosis

• Pathognomonic for CF
• Up to 40% of CF pt
• Attributed to thickened intrahepatic bile duct secretions
• Periductal inflammation, focal biliary fibrosis, & ductular proliferation
• Multinodular cirrhosis in 5 - 12%
• Portal hypertension and end-stage liver disease 1%

Radiologic Pathology 2006-2007 - Volume III - INDEX
1st Branchial Cleft Cysts 1278 Saccular 1212

Aberrant Internal Carotid Artery 1073 Angiomyolipoma 1201, 1399
Aberrant LSCA 1377, 1454 ANGIOMYOMATOSIS vs. LYMPHANGIOMYOMATOSIS
Aberrant RSCA (Right Subclavian Artery) 1375 1202
Abnormal Ureteral Insertion 1332 Annular Tear/Fissure 1290
Abnormal Axis of Intrarenal Collecting System 1333 Annulus of Zinn 1089
Abscess 1133 Anomalous innominate artery 1454
Abscess (Intracranial) 1234 Antenatal Pelvicaliectasis 1336
Absent Internal Carotid Artery 1073 Anterior chamber: aqueous humor 1088
Absent Pulmonary Artery 1455 Antoni A and B fibers 1079
Absent Pulmonary Valve Syndrome 1380 Antral Web 1342
Abuse injuries (Child) - Differential Diagnosis 1499 Antrochoanal polyp 1245, 1368
Accessory Parotid Tissue 1275 Anus (Imperforate) 1351
Achondroplasia 1527 Aortic Arch 1378
Acinic Cell Carcinoma 1280 Double 1378
Acoustic Schwannoma 1079 Embryology 1374
Acquired Cholesteatoma 1076 Anomalies 1454
Acquired Renal Cysts 1382 Aortic Coarctation 1466
Active plaques (MS) 1038 Aortic Stenosis 1470
Acute Chest Syndrome 1526 Aorticopulmonary Window 1477
Acute disseminated encephalomyelitis 1040 Appendicitis 1360
Acute Disseminated Encephalomyelitis (ADEM) 1040 Aqueous humor (Globe) 1088
Acute GI Disorders (Infants and Children) 1353 Aqueous Protein Solution 1163
Acute Meningitis 1232 Arachnoid Cyst 1109
Acute Pulmonary Interstitial Emphysema 1446 Arnold Chiari Malformation 1308
Acute Pyeloneprhitis 1335 Arrested Pulmonary Development 1439
Acyanotic CHD 1472 Arteriosclerosis 1039
Acyanotic CHD with Increased PBF 1473 Arteriosclerosis / venous collagenosis 1039
ADC (Aids Dementia Complex) 1237 Arteriovenous Fistula (Orbit) 1100
Adenoid Cystic Carcinoma 1280 Arteriovenous Malformation (Lung) 1442
Adenoid Enlargement 1368 Ash-Leaf Spots 1199
Adenoma 1251 Asphyxia 1506
Adenoma (Pleomorphic) 1279 Asphyxiating Thoracic Dysplasia (Jeune Syndrome) 1528
Adenoma Sebaceum 1198 Aspirated Foreign Body 1365
Admixture Lesions 1479 Aspiration syndromes 1448
Adrenal Astrocyte Mutation 1142
Hematoma 1410 Astrocytoma 1058, 1141, 1109, 1145
Hemorrhage 1410 Circumscribed 1139
Masses (Pediatric) 1402 Diffuse 1142
Medullary Tumors 1402 Pilocytic 1139
Atresia
Metastases 1410
Adrenocortical Cancer 1409 Colonic 1349
Adrenocortical Tumors 1409 Duodenal 1345
Aggressive Fibromatosis 1276 Esophageal 1341
AIDS Dementia Complex (ADC) 1237 Ileal 1347
AIDS related infections (Intracranial) 1231 Jejunal 1347
Air Leak 1445 Tricuspid 1468
“Pseudocysts” 1447 Atrial Septal Defect 1463, 1474
Pneumomediastinum 1447 Atrial Switch 1469
Pneumothorax 1447 Atrioventricular Canal 1477
Airway (Pediatric) 1363 Atrioventricular Septal Defect 1464
Albers-Schonberg Disease 1533 Atypical Teratoid / Rhabdoid Tumor 1053
Alcohol 1041 Autosomal Dominant Polycystic Kidney Disease (ADPKD)
Allergic fungal sinusitis 1244 1387
with polyps 1244 Autosomal Recessive Polycystic Kidney Disease (ARPKD)
Alobar Holoprosencephaly 1310 1383
Alveolar Proteinosis 1451 AVM (Brain) 1222
AMEN Differential Diagnosis 1079 AVM Grading (Intracranial) 1222
Amyloid angiopathy 1039 Azygos Continuation 1460
Anencephaly 1307 Azygous Vein 1459
Aneurysm (suprasellar) 1255 Basal Ganglia Thalamus 1119
Aneurysms (Intracranial) 1210 Beckwith-Wiedemann syndrome 1388
Deconstructive Therapy 1218 Benign Astrocytoma 1139, 1143
Dissecting 1212 Benign Lymphoepithelial Lesions 1278
I1
Benign Sacrococcygeal Teratoma 1422 Child Abuse 1491
Benign sinus lesions 1247 Differential Diagnosis 1499
Berdon Syndrome 1349 Childhood Neck Neoplasms 1370
Bezoar 1356 Choanal Atresia 1366
Bezold abscess 1078 Cholesteatoma 1074, 1076
Biliary Cirrhosis (Cystic Fibrosis) 1541 Cholesterol Granuloma (Cyst) 1083
Binswanger's 1039 Chondrodysplasia Punctata 1530
Bladder Diverticula 1332 Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome)
Blood Brain Barrier 1131 1529
Blowout Orbital Trauma 1093 Chondrosarcoma 1084
Blunt trauma 1498 Chordoma 1158
Blyth and Ockenden Clinical Classification (ARPKD) 1384 Choriocarcinoma 1178
Bone Marrow Components 1425 Chorioretinitis 1092
Bone Marrow Imaging (Pediatric) 1425 Choroid Plexus
Bony Orbit 1088 Carcinoma 1063, 1064
Borden Classification (Dural AVF) 1225 Metastasis 1065
Borrelia burgdorferi 1237 Neoplasms 1151
Bourneville Disease 1197 Papilloma 1063
Brachial Plexus Traction Injury 1296
Tumors 1063
Brain (Congenital Abnormalities) 1307 Choroidal Detachment 1090
Brain Tumor 1137 Chylothorax 1448, 1451
Branchial Cleft Cyst 1269, 1278 Clear Cell Sarcoma (Kidney) 1396
Bright cerebellum sign 1496 Cleft Brain 1314
Bronchial Atresia 1372 Clivus 1115
Bronchogenic Cyst 1437 Cloquet’s canal 1091
Bronchopulmonary Dysplasia 1447 Closed-Lip (Fused) 1314
Butterfly pattern 1046 CNS Bacterial Infections 1130
Callosal Dysgenesis 1316 CNS infections (acquired) 1232
Calyceal Diverticulum 1381 CNS Lyme Disease 1237
Capillary Telangiectasia (Brain) 1229 CNS Lymphoma 1045
Carbon monoxide poisoning 1041 CNS Neoplasms – Chromosome Loss of Heterozygosity 1192
Carcinoma (Choroid Plexus) 1151 CNS Neoplasms: Chromosomes 1184
Cardiac Imaging (Pediatric) 1453 CNS Tuberculosis 1235
Cardiomediastinal Silhouette Size 1473 Coats’ Disease 1092
Carotid Artery 1073 Cochlea 1068
Carotid Body Paragangliomas 1272 Colloid Cyst 1058, 1062, 1116, 1163
Carotid Space 1269 Colonic Aganglionosis (Total) 1351
Catecholamine Production 1402 Colonic Atresia 1349
Caudal Regression Syndrome 1265 Common Atrium 1485
Cavernous Angioma (Brain) 1228 Complete Atrioventricular Septal Defect 1464
Cavernous Hemangioma 1098 Complete Labrynthine Aplasia 1072
Cavernous Malformation (Brain) 1229 Complete Transposition of Great Vessels 1479
Cavernous Sinus Invasion (Pituitary Macroadenoma) 1254 Complicated Pneumonia 1523
Cellulitis (Orbit) 1101 Compression Fractures 1430
Central Neurocytoma 1058, 1060, 1061 Conal Lesions 1094
Central Posterior Fossa Lesion 1109 Congenital
Cephaloceles 1307 Abnormalities of the Brain 1307
Cerebellar Infarction 1507 Adrenal Hyperplasia 1411
Cerebellar Liponeurocytoma 1050
Anomalies (UTI) 1329
Cerebellopontine Angle Masses 1079
Cerebral edema 1496 Cholesteatoma (Epidermoid) 1074
Cerebral Hemiatrophy (Dyke-Davidoff-Masson Syndrome) Dehiscence of Tegmen Tympani 1074
1312 Heart Disease 1379, 1463, 1472
Cerebral Infarction 1123 hyperplasia (Adrenal) 1410
Cerebral Intraventricular Neoplasms 1058 Lobar Emphysema 1435
Cerebral Neuroblastoma 1157 Lung Malformations 1435
Cerebritis 1231 Megacalyces 1339
Ceruminoma 1079 Megacystis-Megaureter 1339
Cervical aortic arch 1378
Midline Nasal Mass 1367
Cervical Fascia 1266
Chamber Assessment 1472 Spinal Anomalies 1260
Congenitally Corrected TGV 1480
CHARGE Syndrome 1366 Contrast Enema (Malrotation) 1344
Chemotherapy 1042, 1155 Contrast Enhancement 1126
Chiari I Malformation 1308 Contrast Enhancement- Abscess 1133
Chiari II Malformation - (Arnold Chiari Malformation) 1308 Contrast Enhancement: Hematoma 1134
Chiari III Malformation 1309 Contusion (CNS) 1324
Chiari IV Malformation 1309 Convexity Extraaxial Differential 1124
Chiasmatic-hypothalamic glioma 1256 Convexity Intraaxial 1123
I2
Cord Herniation (Idiopathic Transdural) 1296 Distal Intestinal Obstruction Syndrome (DIOS) 1540
Cord injury 1497 Dorsal Induction (Disorders of) 1307
Coronary Artery Aneurysms-Kawasaki 1460 Double Aortic Arch 1378
Cortical Tubers 1200 Double Arch 1454
Cranial injury 1494 Double Outlet Right Ventricle 1485
Cranial Double Ring sign 1085
Nerve Enhancement 1129 Drug abuse 1041
Nerves 1107 Ductus Arteriosus 1456
Nerves III, IV, VI 1090 Duodenal
Vascular Development 1194 Atresia/Stenosis/Web 1345
Craniopharyngioma 1114, 1253, 1255, 1319 Hematoma 1356
Creutzfeldt-Jakob Disease 1235 Duplication Cyst (GI tract location) 1360
Crohn Disease 1360 Dural Tail 1131, 1169
Crohn's 1037 Dyke-Davidoff-Masson Syndrome 1196, 1312
Cryptococcus meningitis 1238 Dysembryoplastic Neuroepithelial Tumor 1051
Cryptococcus neoformans 1238 Dysgenesis (Callosal) 1316
CSF Dysgerminoma 1419
Dissemination 1156 Dysostosis Multiplex 1532
Dysphagia 1374
Homeostasis 1152
Dysplasias with Prominent Membranous Bone Involvement
Spread - Zuckerguss (Sugar Icing) 1157
CT Angiography: Basic Protocol 1453 1531
Cyanotic CHD 1467, 1472, 1478 Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos
Cyanotic CHD with Decreased PBF 1487 Disease) 1049
Cyst (Neurenteric) 1264 EAC cholesteatoma 1078
Cystadenoma (Ovary) 1418 Ear 1068
Cystic Ebstein Malformation 1488
Echogenic Kidneys in Neonate 1386
Adenomatoid Malformation 1436
Ectopic Ureter 1332
Fibrosis 1535
Eisenmenger Physiology 1478
CF Gene 1535 Elevated Prolactin 1113
of the Pancreas 1539 Ellis-van Creveld Syndrome 1529
Masses (Pediatric Renal Tumors) 1398 Embryonal
neoplasms (Ovary) 1415 Sarcoma 1515
Neoplasms: Ovarian Teratoma 1417 small cell tumor 1053
nephroma 1398 Emphysema (Pulmonary Interstitial) 1446
Partially Differentiated Nephroblastoma 1398 Encephalitis 1040, 1231
Encephalocele 1074
Renal Disease of Childhood 1381
Endocardial Cushion Defect 1477
Renal Tumor (Multilocular) 1398
Cysticercosis (Intracranial) 1236 Endolymphatic Sac Tumor 1205
Cysts of the CNS 1160 Enteric Duplication Cyst 1360
Dandy-Walker Malformation 1312 Enteric Fistula (Dorsal) 1263
Darling’s Classification 1481 Enterocolitis (Necrotizing) 1353
Dating of intracranial blood 1496 Enterocolitis (Neutropenic) 1361
DAVF (Dural AVF) 1225 Ependymitis granularis 1037
Dawson's fingers 1038 Ependymoma 1058, 1109, 1112, 1149
DBO’s MR Signal Abnormalities 1191 Epidermoid 1081, 1161
Deep and Periventricular 1121 Epidermoid Inclusion Cyst 1109
Degenerative Disc Disease 1290 Epidermoid vs. Arachnoid Cyst 1109
Degenerative Disease (Spine) 1290 Epidural abscess 1231
Dehiscence of Tegmen Tympani 1074 Epidural Phlegmon / Abscess 1295
Dehiscent jugular bulb 1073 Epiglottitis 1364
DeMorsier’s Syndrome (Septo-Optic Dysplasia) 1310 Epstein-Barr virus (EBV) 1037
Demyelinating Diseases 1037 Esophageal Atresia 1341
Demyelination 1039, 1040, 1041 Esthesioneuroblastoma 1248, 1368
Ethmoid sinus 1240
Imaging 1043
Dermal Sinus (Dorsal) 1264 Exocrine Pancreatic Insufficiency 1538
DERMOID 1162 Exostoses 1288
Dermoid / Epidermoid (Orbit) 1101 Exostosis (External Ear) 1078
Dermoid Cysts 1287 External auditory canal (EAC) atresia 1071
Desmoplastic Infantile Ganglioglioma / Astrocytoma 1048 External Ear Masses 1078
Differential diagnosis of abuse injuries 1499 External Ear Neoplasms 1079
Dilated Azygous Vein 1460 External Otitis 1078
Dirty retrobulbar fat (Grave's Disease) 1094 Extraaxial Lesions 1106
Disc Extraaxial Tumors 1158
Disease 1290 Extraconal Lesions 1100
Extrusion with Migration 1291 Extralobar Sequestration 1440
Extraocular muscles (EOM) 1089
herniations and types 1291
Disorders of Neuronal Proliferation 1313, 1314 Extrapontine myelinolysis 1041
I3
Facet Joint Synovial Cysts 1292 Hemangioendothelioma 1511
Facial Nerve Palsy 1081 Hemangioma 1370
Failed Back Surgery Syndrome (see also FBBS) 1292 Hemangiomas (suprahyoid Neck) 1266
Fallot 1487 Hemangiopericytoma 1173
FBSS (Failed Back Surgery Syndrome) 1293 Hemangiopericytoma vs. Meningioma 1173
Cervical Spinal Canal Stenosis 1293 Hematoma (Duodenal) 1356
Ossification (Posterior Longitudinal Ligament) 1294 Hemimegalencephaly 1313
Posterior Longitudinal Ligament (Ossification) 1294 Hemorrhage 1502
Type I Arachnoiditis 1293 Adrenal 1410
Type II Arachnoiditis 1293 Child Abuse 1495
Fibromatosis (Aggressive - Masticator Space) 1276 Intracranial 1221
Fibromatosis Colli 1267, 1371 Hemorrhagic Cysts (Ovary) 1416
Fibrosing Colonopathy (Cystic Fibrosis) 1540 Hemorrhagic Infarction 1131
Fibrous Dysplasia (Paranasal Sinuses) 1246 Hemorrhagic Ovarian Cysts 1416
Fibrous Histiocytoma (Orbit) 1099 Henoch-Schönlein Purpura 1356
Filum Terminale (Congenital Anomalies) 1263 Hepatobiliary Disease (Cystic Fibrosis) 1541
Fissures of Santorini 1078 Hepatoblastoma 1513
Focal Biliary Cirrhosis 1541 Hepatocellular Carcinoma 1514
Follicular cysts 1415 Hernia (Inguinal) 1357
Foreign Body 1365, 1366 heroin 1041
Forensic Radiology of Child Abuse 1491 Herpes encephalitis 1234
Fourth Ventricle 1109 Heterotopias (Gray Matter) 1315
Fractures (Healing) 1493 High Jugular Bulb (“Megabulb”) 1073
Fried egg appearance 1061 Highly Active Antiretroviral TX (HAART) 1239
Frontal horn “capping” 1037 Hip Effusion 1519, 1520
Frontal Lipoencephalocystocele 1307 Hip Sonography 1518
Frontal sinus 1241 Hirschprung Disease 1350
Functional Ovarian Cyst 1416 HIV encephalitis 1040, 1041
Fungal sinusitis 1102, 1245 HIV Leukoencephalopathy 1238
Ganglioglioma / Gangliocytoma 1047 Holoprosencephaly (Alobar) 1310
Ganglioneuroblastoma 1402, 1408 Holoprosencephaly (Semilobar) 1310
Ganglioneuroma 1402 Horseshoe kidney 1330
Gastric Atresia 1342 HSV Encephalitis 1131
Gastrostomy Tubes 1356 Hurler Syndrome 1532
Gastrulation 1263 Hyaline Membranes 1444
Gaucher Disease 1431 Hydranencephaly 1310
Germ Cell Tumors 1176, 1418 Hydrocarbon aspiration 1366
Germ Cell Tumors-AFIP Series 1177 Hydrocephalus 1181
Germinal Matrix 1502 Hydrocolpos 1420
Germinoma 1121, 1177 Hydronephrosis 1338
Germinoma (Infundibular) 1257 Hyperostosis in Meningiomas 1171
Ghost tumor 1046 Hypertrophic Pyloric Stenosis 1354
GI Disorders (Acute - Infants and Chidren) 1353 Hypogenetic Lung Syndrome 1439
GI Tract Obstruction 1341 Hypoperfusion complex (Child Abuse) 1499
Glioblastoma Multiforme 1119, 1147, 1322 Hypopharyngeal cyst 1369
Glioma 1109 Hypoplastic left heart syndrome 1466, 1485
Glioma (Chiasmatic-hypothalamic) 1256 Hypothalamic Hamartoma 1257
Globe 1088 Hypothalamus 1115
Globus pallidus 1191 Hypoxic-ischemic encephalopathy 1039
Glomerulocystic Disease 1386 Iatrogenic Demyelinating Disorders: Chemotherapy 1042
Glomus jugulare 1082 Iatrogenic white matter degeneration 1037
Glomus tympanicum 1082 Idiopathic Transdural Cord Herniation 1296
Gradenigo’s syndrome 1078 Ileal Atresia 1347
Grading Problems in Gliomas 1140 Ileus (Meconium) 1348
Graf System (Pediatric Hip) 1519 Imaging Studies (UTI) 1331
Granulocyte Colony Stimulating Factor 1429 Immaturity of the Colon (Functional) 1349
Granulosa-theca Cell Tumor 1419 Immune reconstitution syndrome 1239
Graves’ 1037 Immunocompromised Child 1535
Graves’ Disease 1094 Immunocompromised Patient 1045
Great Vessels (Transposition) 1468 Imperforate Anus 1351
Grey Matter Heterotopias 1315 Incudostapedial disruption 1085
HAART 1239 Infantile Hemangioendothelioma 1511
Hamartoma 1510 Infarction 1506, 1507
Hamartoma (hypothalamic) 1257 Infections (Intracranial) 1231
Healing of fractures 1493 Infections (Spine) 1290, 1295
Healing of metaphyseal fractures 1492 Inferior orbital fissure 1088
Hemangioblastoma 1109, 1111, 1203, 1320 Inflammatory disease of the salivary glands 1369
Hemangioblastomatosis 1202 Infratentorial 1106
I4
Inguinal Hernia 1357 Lipoma (Intraspinal) 1262
Inner Ear Anomalies 1071 Liposarcoma (Suprahyoid Neck) 1267
Innominate Artery Compression 1455 Lissencephaly 1314
Innominate Artery Compression Syndrome 1379 Listeria Monocytogenes 1130
Interhemispheric extra-axial hemorrhage 1495 Liver Metastases 1516
Internal Auditory Canal 1107 Liver Tumors (Pediatric) 1509
Internal Carotid Artery 1073 Lobar emphysema 1435
Interrupted Pulmonary Artery 1455 Long bone shaft fracture 1491
Intestinal Obstruction (Neonatal) 1341 Long Parotid Tails 1275
Intraaxial Lesions 1106 Low Back Pain 1290
Intraconal Lesions 1097 Low Intestinal Obstruction 1347
Intracranial Lumbar 1292
Aneurysms 1210 Facet Arthropathy 1292
blood (Dating) 1496 Spinal Canal and Foraminal Stenosis 1292
Germ Cell Tumors 1176 Lung Agenesis 1439
Lung Disease(Cystic Fibrosis) 1537
Germinoma 1177
Infections 1231
Lyme disease 1231
Lipoma 1182 Lymphangioma (Orbit) 1100
Vascular Malformations 1220 Lymphatic Malformations (SupraHyoid Neck) 1267
Intralobar Sequestration 1440
Lymphocytic hypophysitis 1252
Intramural Hemorrhage (GI - Differential) 1356
Lymphoepithelial Lesions (Benign - Suprahyoid Neck) 1278
Intrarenal Collecting System (Abnormal Axis) 1333
Lymphoma 1324, 1371
Intrarenal Reflux 1334
CNS 1045
Intrasellar Pathology 1251
Intraventricular Lesions 1106 Orbit) 1098
Intraventricular Meningioma 1065 Pediatric Renal Tumors) 1400
Intussusception 1357 Sella) 1258
Cystic Fibrosis 1540 in AIDS 1324
Reduction 1358 vs. Toxoplasmosis 1324
Inverted Papilloma (Paranasal Sinuses) 1247 Macroglossia 1369
Irritable Hip 1519 Madelung’s Disease 1267
Ischemic Enhancement 1131 Malfixation (Duodenum) 1343
Jantene Procedure 1469 Malignant
Jejunal Atresia 1347 Astrocytoma 1145
Jeune Syndrome 1528 Compression Fracture 1430
JNA (Juvenile Nasopharyngeal Angiofibroma) 1246 Germ Cell Tumors 1418
Joubert’s Syndrome 1313 Meningioma 1172
Jugular bulb 1073
Sinus Lesions 1247
Jugular Malrotation – UGI 1344
Diverticulum 1073 Malrotation (Duodenum) 1343
Foramen Masses 1083 Mandibular Hypoplasia 1369
Paragangliomas 1270 Marburg 1039
Jugulotympanic Paraganglioma 1082 Marchiafava-Bignami disease 1042
Juvenile Angiofibroma 1367 Marfan syndrome 1460
Juvenile Nasopharyngeal Angiofibroma (JNA) 1246 Marrow
Kawasaki disease 1460 Components 1425
Keratosis obturans 1078 Conversion 1427
Kernohan-Sayre (AFIP) Grading System 1137
Depletion Fatty Replacement 1432
Kidney (Medullary Sponge) 1382
Distribution 1427
Kidney (Multicystic Dysplastic) 1399
Kidney (Rhabdoid Tumor of) 1395 Reconversion 1428
Labrynthine Aplasia 1072 Replacement or Infiltration 1429
Masses - Ring Enhancing (CNS) 1322
Lacrimal Gland Lesions 1102
Masticator Space 1274
Lacrimal Sac Lesions 1103
Mastoiditis 1078
Langerhans Cell Histiocytosis (Sella) 1258
Mature Teratoma (Ovary) 1417
Large Endolymphatic Duct and Sac (LEDS) 1072
Maxillary sinus 1241
Laryngeal- Tracheopapillomatosis 1372
McKusick 1531
Laryngomalacia 1371
Measles 1037
Laryngotracheal cleft 1371
Meckel Diverticulum 1359
Left (Double) Superior Vena Cava 1458
Meckel-Gruber Syndrome 1388
Left paramediastinal structures (Differential Diagnosis) 1458
Meconium Aspiration Syndrome 1449
Left Superior Vena Cava 1458
Meconium Ileus 1348
Leukocoria 1090
Leukoencephalopathy (HIV) 1238 Cystic Fibrosis 1540
Meconium Peritonitis 1348
Lhermitte-Duclos Disease 1049 Meconium Plug Syndrome 1540
Lingual Thyroid 1370 Mediastinal Bronchogenic Cysts 1438
Lingual Thyroid Gland 1285 Medullary Cystic Disease Complex 1387
Lipoencephalocystocele (Frontal) 1307
I5
Medullary Sponge Kidney 1382 polyps 1368
Medullary Tumors (Adrenal) 1402 Nasopharyngeal Carcinoma (NPSCCa) 1283
Medulloblastoma 1109, 1154, 1318 Neck Neoplasms (Child) 1370
Medulloblastoma - Desmoplastic 1157 Necrotizing Enterocolitis 1353
Megabulb 1073 Necrotizing External Otitis 1078
Megacalyces 1339 Neonatal
Megalencephaly (Unilateral) 1313 Brain 1501
Megaureter 1339 GI Tract Obstruction 1341
Melanoma (Uveal) 1092 Hypoxic-Ischemic Injury 1502
Membranous Tracheitis 1365 Low Intestinal Obstruction (Differential Diagnosis) 1351
Meninges (Neoplasms) 1164 Pneumonia 1450
Meningioma 1065, 1081, 1108, 1125, 1164
Respiratory Distress 1444
MENINGIOMA
Lung Diseases 1444
*Imaging Features: CT vs. MR 1169 Neoplasms (Benign - Masticator Space) 1276
Angiography Transit Time 1170 Neoplasms (Malignant - Masticator Space) 1277
Atypical Imaging 1172 Neoplasms of the Meninges 1164
Dural Tail 1131 Nephroblastoma (Cystic Partially Differentiated) 1398
Hyperostosis 1171 Nephroblastomatosis 1394
Vasogenic Edema 1167 Cortical Nodule 1395
Suprahyoid Neck 1270 Diffuse 1394
Nephrogenic Rests: Location 1394
Suprasellar 1254 Nephroma (mesoblastic) 1396
Tentorial 1320 Nerve Sheath Tumors 1187
MR Imaging 1167 Neuroblastic Tumors 1402
Meningitis 1231 Neuroblastoma 1371, 1402
Meningocele 1262
Stage Distribution 1408
Mesenchymal Hamartoma 1509 Neuroectodermal Tumor 1053
Mesoblastic Nephroma 1396 Neuroepithelial Tumors 1047
Metabolic imaging 1043 Neurofibroma vs. Schwannoma 1188
Metaphyseal Chondrodysplasia 1530 Neurofibromas (Pelvis) 1423
Metaphyseal fracture 1491-1492 Neurofibromatosis 1185
Metastasis (Pituitary and Infundibulum) 1259 Type 2 1191
Metastatic Lesions (Orbit) 1100
Type 1 or von Recklinghausen Disease 1185
Methotrexate 1042 Neuromyelitis optica (Devic syndrome) 1039
Michel’s deformity 1072 Neuronal Proliferation (Disorders of) 1313, 1314
Microangiopathy 1039 Neutropenic Enterocolitis 1361
Microgastria 1342 Non-Astrocytic Gliomas 1149
Microgyria 1314 Non-Glial Lesions 1158
Middle Ear 1068 Nonhemorrhagic Infarction 1506
Midgut Loop (Normal Rotation) 1343 Non-Hodgkin Lymphoma (NHL) -Pharyngeal Mucosal Space
Midgut Volvulus 1343 1283
Migraine 1039 Non-Lissencephalic Cortical Dysplasias
Mirror Image Right Arch 1376 Microgyria/Polymicrogyria 1314
Modic Changes 1291 Normal
Modified Papile Classification 1502 Cranial Nerve Enhancement 1129
Brain (Neonatal) 1501 Enhancement 1128
Mondini’s dysplasia 1072
Monosymptomatic demyelinating 1037 Marrow (MR Features) 1426
Morquio Syndrome 1533 Pineal Calcification 1175
MR Angiography: Basic Technique 1453 Thymus 1521
Mucocele 1246 Vertebral Marrow: MRI 1428
Mucoepidermoid Carcinoma 1281 Norrie’s 1091
Mucopolysaccharides 1094 Obstruction (GI Tract - Neonatal) 1341
Multicystic Dysplastic Kidney 1382, 1399 Olfactory Neuroblastoma 1248
Multilocular Cystic Renal Tumor 1398 Olidodendroglioma 1152
Multiple sclerosis 1037, 1039, 1325 Oncocytoma (Suprahyoid Neck) 1280
MR 1038 Ophthalmic veins 1088
Mustard Procedure 1469 Opportunistic neoplasm 1045
myasthenia gravis, 1037 Optic Nerve
myelinolysis 1041 Glioma 1097
myelitis 1040 Sheath Meningioma 1097
Myelocystocele (Terminal) 1262 Optic neuritis 1040
Myelofibrosis 1431 Oral Cavity Normal Anatomy 1286
Myeloid Depletion: MRI 1432 Orbit 1088
Myelomeningocele 1260 Orbital
Nasal Cellulitis 1101
Cycle 1128 fissures 1088
Dermoid 1367 Lymphoma 1098
I6
septal system 1088 Persistent Interstitial Pulmonary Emphysema 1446
Trauma 1093 Persistent Stapedial Artery 1073
Varix 1099 Petrous Apex (Differential Diagnosis) 1083
Organic toxins 1041 Phakomatoses 1184
Oropharynx 1284 Pharyngeal Mucosal Space 1282
Osmotic myelinolysis 1041, 1042 Pharyngeal Perforation 1342
Ossicular Derangement 1085 Pheochromocytoma 1408
Ossifying Renal Tumor of Infancy 1397 Phlegmon / Abscess (Epidural) 1295
Osteogenesis Imperfecta 1533 Pial A-V Fistula 1225
Osteoma (Paranasal Sinuses) 1246 Pilocytic Astrocytoma 1110, 1134, 1139
Osteomyelitis 1431 Pilocytic Astrocytoma (Juvenile Pilocytic) 1141
Osteomyelitis (Spine) 1295 Pineal Calcification 1175
Pyogenic 1295 Pineal Cyst 1121, 1181
Tuberculous 1295 Pineal
Osteopetrosis (Albers-Schonberg Disease) 1533 Neoplasms Laboratory Tests 1179
Osteoporotic Fracture 1430 Parenchyma 1180
Ostiomeatal complex (Paranasal Sinuses) 1241 Region Masses 1175, 1320
Ostium Primum ASD 1464
Region Neoplasms 1178
Otitis 1078 Pineal/Quadrigeminal Cistern Region 1121
Otosclerosis 1085 Pinealomas 1121, 1175
Otospongiosis 1085 Pineoblastoma 1121, 1180
Outer Ear Anomalies 1071 Pineocytoma 1121, 1180
Ovarian Pituitary 1250
Cancer 1420 Pituitary
Cyst 1416 Adenoma 1113, 1253
Cystadenoma 1418 Apoplexy 1254
Maturation 1414 Macroadenoma 1253
Tumors 1415 Neoplasms 1251
Ovary (Prepubertal vs Postpubertal) 1414 Plasma Cell Granuloma 1522
Palatine Tonsil Enlargement 1368 Pleomorphic Adenoma 1279, 1288
Pancreas (Child Abuse) 1498 Pleomorphic Xanthoastrocytoma 1051, 1141
Pancreatic injury 1498 Plexiform Neurofibromatosis 1423
Papillary Cystadenoma Lymphomatosum 1280 Pneumatoceles 1524
Papillary Endolymphatic Sac Tumor 1083 Pneumonia 1523
Papilloma 1151 Pneumonia (term & premature neonates) 1448
Papovavirus 1238 Polycystic Kidney Disease (autosomal Recessive) 1383
Paradoxical Embolus 1475 Polymicrogyria 1314
Paragangliomas 1082, 1270 Port Wine Stain 1193
Paranasal Sinuses 1240 Post fossa cysts 1109
Paraovarian cysts 1415, 1417 Posterior chamber 1088
Parapharyngeal Abscess 1365 Posterior Fossa
Parapharyngeal Space 1267, 1268 Malformations 1312
Parasellar Region 1250 Tumors (Pediatric) 1318
Parinaud Syndrome 1175 Posterior Hyaloid Detachment 1090
Parotid Space 1278 Posterior Reversible Encephalopathy Syndrome (PRES)
Parotid Tail 1275 1039, 1040
Partial Anomalous Venous Return (Pulmonary) 1456 Posterior rib fractures (visualization) 1493
Patent Ductus Arteriosus 1456, 1476 Posterior Urethral Valves 1337
Patterns of Enhancement 1126 Precocious Puberty 1175
Patterns of Location 1106 Premature Births 1444
Pediatric Premature Brain 1501
Adrenal Masses 1402 Prepubertal ovary 1414
Airway 1363 Primary Megaureter 1339
Hip Sonography 1518 Pringle’s Disease 1199
Liver Tumors 1509 Profound Asphyxia 1506
Progressive multifocal leukoencephalopathy 1040
Pelvic Masses 1414
Progressive Multifocal Leukoencephalopathy (PML) 1041
Posterior Fossa Tumors 1318
Prolactin 1113
Renal Tumors 1390 Prolactinoma 1251, 1253
Tuberculosis 1526 Proteinosis (Alveolar) 1451
Pelvic Masses (Pediatric) 1414
Proximal Neonatal Intestinal Obstruction 1347
Pelvicaliectasis (Antenatal) 1336
Pseudotumor (Orbit) 1095
Peritonitis (Meconium) 1348
Pubertal ovary 1414
Periventricular Hemorrhagic Infarction 1503
Pulmonary
Sonography 1503
Periventricular Leukomalacia 1504 Abscess 1524
periventricular white matter 1039 Arterial Anomalies 1455
Persistent hyaloid (Cloquet’s) canal 1091 Artery Stenosis 1470
Persistent Hyperplastic Primary Vitreous (PHPV) 1091
I7
Atresia with Intact Ventricular Septum 1489 Rotation of Midgut Loop 1343
AVM 1442 Round Pneumonia 1522
Blastoma 1522 Rules for Ring Enhancing Mass 1132
Blood Flow 1472 Sacrococcygeal Teratoma 1421
SAH (Subarachnoid Hemorrhage) 1210
Bronchogenic Cyst 1438
Aneurysms (Intracranial) - Infectious 1218
Hypoplasia 1439
Aneurysms (Intracranial) - Treatment Options 1218
Infections 1521
Clinical Grading Scale 1213
Infections (Immunocompromised Child) 1535
CT 1214
Interstitial Emphysema 1446
CTA 1215
Sequestration 1440
DSA 1215
Sling 1379, 1455
induced Vasospasm 1214
Underdevelopment 1439
Infectious Intracranial Aneurysms 1218
Venous Anomalies 1456
Pulsatile Tinnitus Lesions 1074 Lumbar puncture 1214
Pyeloneprhitis 1335 MRA 1215
Pyknodysostosis 1534 MRI 1215
Pyloric Stenosis 1354 Outcomes 1213
Pyogenic Abscess (Intracranial) 1234 Patterns 1213
Pyogenic Osteomyelitis 1295
Radiologic Grading Scale 1213
Pyriform Aperture stenosis 1367
Risk Factors 1210
Radiation 1155
Screening 1217
Injury (Brain) 1043 Salivary glands (Inflammatory disease) 1369
Necrosis vs. Tumor (CNS) 1323 Salt and pepper appearance 1082
Ranulas 1287
Sarcoidosis (CNS) 1321
Rathke Cleft Cyst 1252
Sarcoidosis (Sella) 1258
Reactive Airways Disease 1365
SATCHMO 1319
Rebleeding 1497
SCCa (Squamous Cell Carcinoma) 1248
Rectal Prolapse (Cystic Fibrosis) 1540
Schizencephaly 1314
Rectus: medial, lateral, superior, inferior 1089
Schwannoma 1107, 1188, 1192
Red Marrow Signal 1427
Acoustic - Vestibular 1079
Reflux Nephropathy 1335
Renal Agenesis 1329 Orbit 1098
Scimitar Syndrome 1457
Renal Cell Cancer 1399
Sclera 1089
Renal Cyst 1381
Scutum 1076
Renal Ectopia 1330
Second Branchial Cleft Cyst 1269
Renal Ectopia and Fusion 1330
Secundum ASD 1464
Renal Tumors (Infancy and Young Children) 1390
Segmental Spinal Dysgenesis 1265
Respiratory Distress (Neonatal) 1444
Sella 1250
Respiratory Distress Syndrome (RDS) 1444
Sella/Parasellar Region - Differential 1113
Retained fetal lung fluid 1448, 1449
Sellar Masses: “SATCHMO” 1319
Retina 1089
Semicircular Canals (SCC) 1068
Retinal Detachment (RD) 1090
Semilobar Holoprosencephaly 1310
Retinoblastoma 1091
Senescent White Matter Changes 1039
Gene 1091 Senile Macular Degeneration 1090
Retinopathy of prematurity 1092
Septic Arthritis (Hip) 1520
Retrobulbar (“Postseptal”) Space 1089
Septo-Optic Dysplasia (DeMorsier’s Syndrome) 1310
Retropharyngeal Cellulitis 1364
Sequestration 1525
Reversal sign 1496
Sertoli-Leydig cell tumor 1419
Rhabdoid Tumor 1054
Sex Cord-Stromal Tumors 1419
Rhabdoid Tumor of Kidney 1395
Shaking mechanism 1492
Rhabdomyosarcoma 1368, 1420
Short Rib-Polydactyly 1528
Orbit 1101
Shunt Lesions 1463, 1487
Male Bladder & Prostate 1421 Sickle Cell Anemia 1429
Rhabdomyosarcomatoid variant of Wilms tumor 1053
Rhombencephalosynapsis 1313 Simple Renal Cyst 1381
Rib fracture 1492 Single Ventricle 1484
Rib Notching 1189 Physiology 1489
Sinus Mass Differential 1368
Right aortic arch 1454
Sinus Venosus ASD 1464
Right Arch 1376
Sinuses (Paranasal) 1240
Right paramediastinal structures (Differential Diagnosis)
Sinusitis 1243
1459
Sinusitis (Fungal) 1102
Right to Left Shunts 1487
Sjogren’s Syndrome 1279
Ring Enhancing Mass 1132
Skeletal Dysplasia 1527
Ring Lesion Features For Infection 1132
Skeletal injury (evaluation) 1494
Ring Lesions Differential 1132
Skull fracture 1497
Ring-enhancing Masses (CNS) 1322
SLE 1037
Risk Factors (subarachnoid Hemorrhage) 1211
Soap-bubble appearance 1051
I8
Spetzler-Martin Grading System (Intracranial Vascular Temporomandibular joint (TMJ) anomalies 1071
Malformations) 1222 Tenon’s capsule 1089
Sphenoid sinus 1242 Tentorial Meningioma 1320
Spina Bifida 1262 Teratoid Tumor 1053
Spinal Anomalies (Congenital) 1260 Teratoma 1178, 1371
Caudal Regression Syndrome 1265 Ovary 1417
Chiari II Malformation 1261 Sacrococcygeal 1421
Complex Dysraphic States 1263 vs. Dermoid (Pineal Region) 1178
Dorsal Dermal Sinus 1264 Tetralogy of Fallot 1455, 1467, 1487
Thanatophoric Dysplasia 1527
Dorsal Enteric Fistula 1263
Thiamin deficiency 1042
Fibrolipomatous Infiltration of Filum 1263
THIRD VENTRICLE 1116
Gastrulation 1263 Thoracic MRA & CTA 1453
HemiMMC/Hemimyelocele 1261 Thymus (Pediatric - Normal) 1521
Intraspinal Lipoma 1262 Thyroglossal Duct Cysts 1284
Lipoma with Dorsal Defect 1261 Thyroid Gland (Lingual) 1285
Lipomyelomeningocele 1261 Thyroid Orbitopathy (Graves’ Disease) 1094
Meningocele 1262 Time Density Curves 1126
Myelocele (Myeloschisis) 1261 Tinnitus 1074
Tongue Base Mass 1369
Myelomeningocele 1260
Top 10 Pelvic Lesions 1423
Neurenteric Cyst 1264
Total Anomalous Pulmonary Venous Return 1480
Persistent Terminal Ventricle 1263 Total Anomalous PV Return 1469
Posterior Spina Bifida 1262 Total Colonic Aganglionosis 1351
Segmental Spinal Dysgenesis 1265 Toxic Demyelination 1041
Spinal Dysraphism 1260 Toxocara canis 1092
Split Cord Malformation 1264 Toxocariasis 1092
Terminal Myelocystocele 1262 Toxoplasmosis 1119
Tight Filum Terminale 1263 Intracranial 1238
Spinal Dysraphism 1260 Tracheal bronchus 1372
Spine 1290 Tracheal Stenosis 1372
Spine injury 1493 Tracheomalacia 1371
Split Cord Malformation 1264 Transient Tachypnea of Newborn 1449
Squamous cell carcinoma 1248 Transposition of Great Vessels 1468
Stapedial Artery 1073 Transverse Myelitis 1039
Stridor 1363, 1374 Tricuspid Atresia 1468, 1484, 1489
Sturge-Weber Syndrome 1193 Trilateral Retinoblastoma 1180
Subacute sclerosing panencephalitis 1040 Truncus Arteriosus 1469, 1483
Subarachnoid Hemorrhage 1210 Tuberculosis (Intracranial) 1231
Subdural empyema 1231 Tuberculosis Pediatric 1526
Subependymal Giant Cell Astrocytoma 1058, 1061 Tuberous Sclerosis or Bourneville Disease 1197
Subependymal Nodules 1200 Tumefactive Demyelination 1134
subependymal veins 1039 Tumor Blush 1171
Subependymoma 1058, 1059 UGI (Upper GI Tract - Malrotation) 1344
Subepidermal Fibrosis 1199 ulcerative colitis 1037
Subglottic edema 1363 Uncommon Neuroepithelial Tumors 1045
Subglottic Hemangioma 1370 Undifferentiated Embryonal Sarcoma 1515
Sugar Icing 1156 Unilateral Megalencephaly (Hemimegalencephaly) 1313
Superior and inferior ophthalmic veins 1088 Upper esophageal foreign body 1366
Superior Left Intercostal Vein 1458 Ureterocele 1333
Superior orbital fissure 1088 Ureteropelvic duplication 1333
Suprahyoid Neck 1266 Ureteropelvic Junction Obstruction 1338
Suprasellar Masses 1253 Urethral Valves (Posterior) 1337
Supratentorial 1106 Urinary Tract Infection (Child) 1329
Supratentorial Primitive Neuroectodermal Tumor 1052 US Guidance 1520
Surfactant 1445 Uterine Morphology: Maturation 1415
Surfactant B protein deficiency 1448 Uvea: choroid 1089
Swyer-James Syndrome 1436, 1524 Uveal Melanoma 1092
Systemic Gas Embolism 1447 Uveal Metastasis 1093
Systemic Venous Anomalies 1458 VACTERL 1342
Taenia solium 1236 Vagal paraganglioma 1082
Tegmen Tympani 1074 Vagale Paragangliomas 1271
Temporal Bone Fracture 1084 Vaginal Rhabdomyosarcoma 1421
Temporal Bone: Vallecular Cyst 1369
Anatomy 1068 Valvular Pulmonic Stenosis 1470
Varix (Orbit) 1099
Congenital Lesions 1068
Vascular Anomalies (Pediatric Cardiac Imaging) 1453
Infectious Lesions 1076
Vascular Malformations (Intracranial) 1220
Neoplastic Lesions 1076
I9
Vascular Mediated Disorders (Bone Marrow) 1433
Edema 1433
Ischemia 1433
Ischemia & Edema: Causes (Bone Marrow) 1433
Vascular White Matter Disease 1039
Vasculitis 1039
Vein Of Galen Malformation 1181
Venous Anomaly (Intracranial - Developmental) 1226
Venous Collagenosis 1039
Ventral Induction (Disorders of) 1310
Ventricular Septal Defect 1474
Ventricular Septal Defects 1465
Vesicoureteric Reflux 1332
Vestibular Schwannoma 1079, 1108
Vestibule 1068
Viral and Postviral Demyelination 1040
Viral Croup 1363
Virchow-Robin spaces 1037
Visceral injury 1498
Vitreous body 1088
Volvulus (Midgut) 1343
von Hippel-Linmdau Syndrome: NIH Classification 1202
von Recklinghausen Disease 1185
Warburg’s 1091
Warthin’s Tumor 1280
Wernicke encephalopathy 1042
White Matter Changes (Senescent) 1039
WHO 2000 Brain Tumor Classification 1137
Whole-body MRI 1426
Wilms Tumor 1390
Wishart Disease 1191
Wolman Disease 1411
Xanthoastrocytoma 1052
Xanthoastrocytoma (Pleomorphic) 1141
Yellow Marrow Signal 1427
Zellweger Syndrome 1388
I 10

Radiology - AFIP - 2006-2007 Lecture Notes

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Radiology - AFIP - 2006-2007 Lecture Notes

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Radiologic

Angela D. Levy, COL, MC, USA

Paula J. Woodward, MD Illustrators

Department of Radiologic Pathology

© Copyright 2006 by the American Registry of Pathology.

Made in the United States of America

Library of Congress Cataloging-in-publication Data [in process]

The annual production of the Radiologic Pathologic Correlation course and

The Department of Radiologic Pathology of the Armed Forces Institute of

Describing Diffuse Lung Disease

An Approach to Diffuse Lung Disease Figure 1-1-2

Radiology and pathology form

Fibrosis results in reduced lung volumes

Airways disease results in increased

Chest Radiology 3 An Approach to Diffuse Lung Disease

Plain Film and CT Opacities

Plain Film and CT Opacities

Anatomy - Secondary Lobule

An Approach to Diffuse Lung Disease 4 Chest Radiology

The secondary lobule with lymphatics in the

Septal pattern on HRCT and gross specimen

Chest Radiology 5 An Approach to Diffuse Lung Disease

Sarcoidosis: Clinical Features

An Approach to Diffuse Lung Disease 6 Chest Radiology

Sarcoidosis: Respiratory System

Non-Caseating Granuloma and Fibrosis

Sarcoidosis and the Secondary Lobule [Figure 1-1-14]

Chest Radiology 7 An Approach to Diffuse Lung Disease

Distribution of nodules in sarcoidosis Masses in Sarcoidosis

Ground glass in Sarcoidosis Conglomerate masses and fibrosis in sarcoidosis

Sarcoidosis: Computed Tomography [Figure 1-1-15]

An Approach to Diffuse Lung Disease 8 Chest Radiology

Sarcoidosis: Adenopathy [Figures 1-1-16 and 1-1-17]

20% develop fibrosis or Stage 4 disease

Sarcoidosis Stage III

Sarcoidosis and the Parenchyma: Computed Tomography

Chest Radiology 9 An Approach to Diffuse Lung Disease

Peribronchovascular and Pleural Nodules

Ground Glass Opacities

Consolidation and Large Nodules

Bronchovascular Bundle Distortion [Figure 1-1-14]

Fibrosis and Honeycombing

Sarcoidosis: Differential Diagnosis

An Approach to Diffuse Lung Disease 10 Chest Radiology

Transbronchial Spread of Tuberculosis

Extrinsic Allergic Alveolitis

Sarcoidosis: Cardiac Involvement

Cardiac Sarcoidosis [Figure 1-1-19]

Sarcoid infiltration on MRI is represented as focal zones of

Chest Radiology 11 An Approach to Diffuse Lung Disease

Mycetoma [Figure 1-1-20]

An Approach to Diffuse Lung Disease 12 Chest Radiology

Chest Radiology 13 An Approach to Diffuse Lung Disease

The Idiopathic Interstitial Pneumonias

The lung volumes are low and

The Idiopathic Interstitial Pneumonias

The Idiopathic Interstitial Pneumonias

The Idiopathic Interstitial Pneumonias 14 Chest Radiology

Usual Interstitial Pneumonia: Histology

Idiopathic Pulmonary Fibrosis Imaging [Figure 1-2-3]

IPF-Progressive Volume Loss

Idiopathic Pulmonary Fibrosis [Figure 1-2-4]