Juvenile

Nasopharyngeal
Angiofibroma

Garrett Hauptman MD
Seckin Ulualp MD
January 3, 2007
 JNA
 Overview
 Anatomy
 Diagnosis
 Radiology
 Staging
 Treatmen
t
Overview
 JNA
 Benign highly vascular tumor

 Locally invasive, submucosal spread

 Vascular supply most commonly

from internal maxillary artery
 Also: internal carotid, external carotid,
common carotid, ascending pharyngeal
 JNA Facts and Statistics
 Up to 0.5% of head and neck tumors

 Occurring almost exclusively in males

 Average age of onset = 15 years old

 Intracranial Extension between 10-20%

 Recurrence Rates as high as 50%

Anatomy
 Origin

 Considered to be posterolateral
nasal wall at sphenopalatine
foramen

 Blood supply
 Primarily internal maxillary artery off of
external carotid
 Origin
 Posterolateral nasal wall near
sphenopalatine foramen
 Routes of Spread
 Medial growth
 Nasal cavity
 Nasopharynx

 Lateral growth
 Pterygopalatine fossa
 Vertical expansion through inferior orbital fissure to orbit
possible
 Infratemporal fossa
 Superior expansion through pterygoid process may involve
middle cranial fossa
 Lateral and posterior walls of sphenoid sinus can be eroded
 Cavernous sinus may be involved
 Pituitary may be involved
Sphenopalatine Foramen
 Sphenopalatine vessels

 Nerves
 Nasopalatine
 Posterior superior nasal
 Histology
 Myofibroblast is cell of origin
 Fibrous connective tissue with abundant
endothelium-lined vascular spaces
 Pseudocapsule of fibrous tissue
 Blood vessels lack a complete muscular
layer
Diagnosis
Midface and Anterior Skull
Base Tumors
 Juvenile Nasopharyngeal Angiofibroma
 Osteoma
 Craniopharyngioma
 Olfactory Neuroblastoma
 Chordoma
 Chondrosarcoma
 Rhabdomyosarcoma
 Nasopharyngeal Carcinoma
 Diagnosis
 History
 Physical Exam
 Radiological study
 CT Scan
 MRI

 Angiogram
 Characteristic
Presentation

 Teenage or young adult male

 Recurrent epistaxis

 Nasal obstruction
 Additional Findings at
Presentation
 Conductive hearing loss
 Rhinolalia
 Hyposmia/Anosmia
 Swelling of cheek
 Dacrocystits
 Deformity of hard and/or soft palate
 Orbital proptosis
 Appearance

 Smooth lobulated mass in the

nasopharynx or lateral nasal wall

 Pale, purplish, red-gray, or beefy red

 Compressible
Radiology
 Radiological Studies
 CT Scan
 Excellent for bone detail
 Lesion enhances with contrast on CT

 MRI
 Differentiate tumor from other soft tissue structures

 Angiogram
 Evaluation of feeding blood vessels

Holman-Miller Sign
Characteristic anterior bowing of posterior maxillary
wall
 Coronal CT: Bone
Window
 Widening of left
sphenopalatine
foramen

 Lesion fills left

choanae

 Extends into
sphenoid sinus
 Axial CT: Soft Tissue
Window with Contrast
 Homogenous
enhancement

 Widening of left
sphenopalatine
foramen

 Extension into
 Nasopharynx
 Pterygopalatine fossa
 Axial CT: Soft Tissue
Window with Contrast
 Homogenous
enhancement

 Widening of right
sphenopalatine
foramen

 Extension into
 Nasopharynx
 Pterygopalatine fossa
 Axial MRI: T1
 Heterogeneous
intermediate signal

 Flow voids
represent enlarged
vessels

 Extension into
 Nasopharynx
 Masticator space
 Coronal MRI: T1 with
Contrast
 Diffuse intense
enhancement

 Multiple flow voids

within
hypervascular mass

 Extension into
 Nasopharynx
 Pterygopalatine
fossa
 Axial MRI: T2
 Heterogeneous
intermediate to high
signal enhancement

 Multiple flow voids

within hypervascular
mass

 Extension into
 Nasopharynx
 Pterygopalatine fossa
 External Carotid
Arteriogram

Feeding vessel = Internal Maxillary

Artery
Staging
Radkowski Nasopharyngeal
Angiofibroma Staging
System

Radkowski et al. Arch.

Treatment
 Treatment Options
 Surgery
 Gold standard
 Radiation therapy
 Reserved for unresectable, life-threatening tumors
 Chemotherapy
 Recurrent tumors with previous surgery and
radiation
 Hormone therapy
 Estrogens and antiandrogens used to decrease
tumor size and vascularity
 Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or
without craniotomy
 Preoperative
Embolization
 24 to 72 hours preoperatively
 Gelfoam or polyvinyl alcohol foam
 Gelfoam: resorbed in approximately 2 weeks
 Polyvinyl alcohol: more permanent
 Efficacy
 Stage I patients reduced from 840cc to 275cc
blood loss
 Complications
 Brain and ophthalmic artery embolization
 Facial nerve palsy
 Skin and soft tissue necrosis
Liu L et al. Analysis of intra-operative bleeding and recurrence of juvenile nasopharyngeal
angiofibromas. Clin Otolaryngol. 2002
Embolization
Embolization
 Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or
without craniotomy
 Endoscopic Transnasal

 Middle turbinectomy may be performed for

improved exposure
 Endoscopic Transnasal

 Middle meatus antrostomy

 Resection of posterior maxillary wall
 Endoscopic Transnasal

 Sphenopalatine artery ligation

 Tumor resection from pterygopalatine fossa
 Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or
without craniotomy
 Transpalatal

 Soft palate is split and retracted

 Transpalatal

 Hard palate resection for enhanced exposure

 Transpalatal

 Palatine bone and inferior aspect of pterygoid plate

resected
 Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or
without craniotomy
 Denker Approach

 Wide anterior antrostomy

 Removal of ascending process of maxilla
 Removal of inferior half of lateral nasal
wall
 Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or
without craniotomy
 Midface Degloving with
Maxillary Osteotomies

 Gingivobuccal incision
 Nasal intercartilaginous incisions with
transfixion incision
 Midface Degloving with
Maxillary Osteotomies

 Soft tissue elevation

 Midface Degloving with
Maxillary Osteotomies

 Le Fort I osteotemies
 Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or
without craniotomy
 Maxillectomy

 Maxillary osteotomies
 Sagittal osteotomy
Maxillectomy
 Alternative Approaches to
Nasal Cavities and
Paranasal Sinuses
 Lateral Rhinotomy
 Weber-Ferguson incision
 Weber-Ferguson with Lynch
extension
 Weber-Ferguson with lateral
subciliary extension
 Weber-Ferguson with subciliary
extension and supraciliary extension
 Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or
without craniotomy
Infratemporal Fossa with or
without Craniotomy
 Choosing the Surgical
Approach
 Retrospective chart review of
surgical intervention- 37 patients
 Staged using CT scan and/or MRI
 Follow-up CT scan or MRI: 3 months,
6 months x 3 years, yearly
 Recurrence rate = 27%

Hosseini et al. Eur Arch

Otorhinolaryngol. 2005.
 Surgical Planning
 Smaller tumors (IA, IB, IIA, IIB, IIC)
 Trans-nasal endoscopic
 Transpalatal
 Transantral: lesions extending laterally up
to pterygopalatine fossa
 Larger tumors (IIIA, IIIB)
 Lateral rhinotomy
 Midfacial degloving

 Extensive resection with higher morbidity

 Limited resection with higher recurrence

Hosseini et al. Eur Arch Otorhinolaryng
2005.
 Changing Technique
 Retrospective chart review of surgical
intervention- 30 patients
 Marked shift towards endonasal
procedures while tumor stages
remained the same
 Endonasal approach contraindicated
in Stage IV and some Stage III cases
 May be used in conjunction with other
approach in these cases
Mann et al. Laryngoscope.
2004.
Surgical Approach

Mann et al. Laryngoscope. 200

 Surgical Technique
Approach (65 Endoscop Open
pts) ic
EBL 225 ml 1250 ml

Complications 1 30

Length of Stay 2 days 5 days

Recurrence 0% 24 %
Rate

Pryor et al. Laryngoscope. 2

 Surgical Technique
 Retrospective study of 24 patients using
Radkowski staging scale
 10 patients IA through IIA had transpalatal
approach
 Before 1999
 9 patients IA through IIIA had transnasal
endoscopic approach
 After 1999
 5 patients IIA through IIIA had lateral
rhinotomy or degloving approach
 Recurrence in 1 case with 12-56 month follow-
up range
 Transpalatal approach

Tosun et al. J Craniofac

Surg. 2006.
2006
 Surgical Technique
 Transnasal endoscopic approach can
replace transpalatal approach
 Less morbidity
 Patients with IIA through IIIA previously
treated with lateral rhinotomy may be
treated with transnasal endoscopic
approach
 Tumors extending to infratemporal fossa
require lateral rhinotomy and degloving
for optimal exposure
 Greater morbidity

Tosun et al. J Craniofac Surg. 20

 Surgical Technique
 Surgical limitations of endoscopic
resection evaluated in literature review
 Extremely limited IIIA and IIIB may be
approached endoscopically
 Preoperative embolization
recommended
 Unlikely that limits on endoscopic
resection of JNA have been reached

Douglas et al. Curr Opin Otolaryngol Head

 Gamma Knife Surgery

 2 case reports used as booster

treatment for residual tumor after
surgery
 No change in tumor size of one patient,
regression in other patient Dare et al.
Neurosurgery. 2003.

 1 case report used as primary

treatment modality successfully
Park et al. J Korean Med
Sci. 2006.
External Beam Radiation
 Retrospective review of efficacy of
radiation as primary treatment
modality for JNA
 15 patients received 3000-3500 cGy
 Recurrence rate of 15%
 External beam radiation is effective
mode of treatment of advanced JNA

Reddy et al. Am J
Otolaryngol. 2001.
External Beam Radiation
 Retrospective review of efficacy of
radiation as primary treatment
modality for JNA
 27 patients received 3000-5500 cGy
 Recurrence rate of 15% 2-5 years
post-treatment
 External beam radiation is effective
mode of treatment of advanced JNA
Lee JT et al. Laryngoscope.
2002.
External Beam Radiation
 Long-term sequelae of concern
 Growth retardation, panhypopituitarism, temporal
lobe necrosis, cataracts, radiation keratopathy

 Retrospective review reported 2 cases out of

55 patients developing secondary
malignancies
 Thyroid carcinoma 13 years after receiving
3500cGy
 Basal cell carcinoma of skin 14 years after
receiving 3500cGy initially, then 3000cGy for
recurrence
Cummings et al. Laryngoscope
1984.
 Chemotherapy
 Chemotherapy alternative therapy
 1 unresectable tumor had
chemotherapy for palliation
 Adriamycin and decarbazine
 Extensive regression of tumor

 Possible alternative to radiation?

Shick et al. HNO.

1996.
 Hormonal Therapy
 Estrogen, progesterone, and androgen
receptors have been identified with
varying frequencies in JNAs
 Some JNAs lack these receptors

 Limited utility
 Delays surgery
 Feminizing side effects
 Cardiovascular complications
 Hormonal Therapy
 Efficacy of treatment with flutamide
evaluated in 7 patients
 Before and after measurement
comparison made using CT scan
 No statistically significant difference in
size
 No difference in blood loss

 No advantage with treatment

Labra A et al. Otolaryngol Head Neck

Surg. 2004.
 Surveillance

 Frequent physical examinations

 CT Scan / MRI
 Recurrence Rates
 Post-operative
 Stage I and II = 7%
 Stage III = 39.5%

Herman F et al.
Laryngoscope 1999.

 Tumor stage – extracranial vs.

intracranial tumor
 Extracranial = 5%
 Intracranial = 50%
Bremer JW et al.
Laryngoscope 1986.
 Conclusions

 Rare, benign, vascular tumor found

almost exclusively in young males

 Surgery is the gold standard with a

trend towards endoscopic approaches

 Frequent follow-up after treatment is

necessary
Questions
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