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Ranganath et al.
Mediastinal Masses in Children

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Residents Section
Pattern of the Month

Residents

inRadiology
Shashi H. Ranganath1
Edward Y. Lee1
Ricardo Restrepo 2
Ronald L. Eisenberg 3
Ranganath SH, Lee EY, Restrepo R, Eisenberg RL

Mediastinal Masses in Children

he mediastinum is the most common location of chest masses in the pediatric

population. Mediastinal masses may be nonvascular or vascular masses and represent congenital anomalies, infections, benign and malignant neoplasms, and
pseudomasses. In both asymptomatic and symptomatic children with mediastinal
masses, imaging plays a crucial role in making the diagnosis and developing a treatment plan.
As in adults, mediastinal masses in pediatric patients are placed in one of three mediastinal
compartments (anterior, middle, posterior) on the basis of the lateral chest radiograph (Fig. 1
and Table 1). Further characterization can be made with ultrasound, CT, and MRI. Combining the characteristic imaging appearances with clinical information (age, physical examination findings, and laboratory analysis) often can provide a precise diagnosis.

Anterior Mediastinal Masses

For practical purposes and to facilitate the diagnosis, anterior mediastinal masses in children can be classified on the basis of their density into the three categories: solid, fatty, and
cystic lesions.

Solid Lesions

Keywords: mediastinum, pediatrics

DOI:10.2214/AJR.11.7027
Received April 4, 2011; accepted without revision
May 23, 2011.
1

Department of Radiology, Childrens Hospital Boston,

Harvard Medical School, Boston, MA.

2
Department of Radiology, Miami Childrens Hospital,
Miami, FL.
3
Department of Radiology, Beth Israel Deaconess
Medical Center, Harvard Medical School, 330 Brookline
Ave, Boston, MA 02215. Address correspondence to
R.L.Eisenberg (rleisenb@bidmc.harvard.edu).

WEB
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AJR 2012; 198:W197W216
0361803X/12/1983W197
American Roentgen Ray Society

Prominent thymus (pseudomass) The thymus is a bilobed encapsulated organ located

anterior to the great vessels and pericardium. Its primary function is the maturation of Tlymphocytes. In infancy and childhood (until about age 5 years), the thymus has a quadrilateral shape with convex margins. After this time, the thymus gradually becomes more triangular and the margins straighten. By age 15 years, the margins of the thymus should be
straight or concave.
On chest radiographs, the normal thymus is not visible in adolescents and adults. In normal
infants and young children, however, the thymus may be very prominent and mistaken for a true
mass (Fig. 2). However, the classic appearance of the sail sign, the morphologic appearance
of a nautical sail, confirms that the thymus is normal (Fig. 3). Indentations produced by anterior
ribs on the soft thymus (wave sign) are another normal pattern. Ultrasound, CT, or MRI can
confirm the presence of a prominent but normal thymus. Even though CT is not typically used
to evaluate the thymus, it is important to realize that this organ may appear prominent and suggest an anterior mediastinal mass to the unwary eye. Indeed, such a pseudomass may be the
source of referral to a pediatric center when the chest CT study of an infant or young child is
interpreted by an adult radiologist (Fig. 4). Cross-sectional imaging shows the thymus as a homogeneous soft-tissue structure without calcification or compression on the airway or vascular
structures. The soft and pliable consistency of this organ can be confirmed at fluoroscopy because the normal thymus changes contour and size with respiration (Fig. 5).
A retrocaval thymus is a normal variant in which there is posterior extension of the thymus
between the superior vena cava (SVC) and great arteries (Fig. 6). On chest radiography, a
retrocaval thymus may produce a confusing appearance that mimics a true mediastinal mass
or right upper lobe collapse. Cross-sectional imaging shows that the retrocaval thymus is contiguous with the anteriorly positioned normal thymic tissue and forms a single structure, with
homogeneous attenuation or signal that is similar to the rest of the thymus. An ectopic cervical
thymus, which results from arrested migration along the thymopharyngeal duct, may be found
from the mandibular angle to the thoracic inlet. In this condition, imaging shows a mass of

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homogeneous attenuation that is TABLE 1: Mediastinal Masses in Children

similar to that of the normally
Anterior Mediastinal Masses
positioned thymic tissue.
Solid Lesions
Thymic hyperplasiaThy 1. Normal thymus
mic hyperplasia, or thymic re a. Prominent thymus (pseudomass)
bound, refers to enlargement of
b. Ectopic thymus (pseudomass)
the gland after atrophy from se 2. Thymic hyperplasia
vere illness or medications (che 3. Thymoma
motherapy, corticosteroid treat a.
Noninvasive thymoma
ment). After chemotherapy, the
b.
Invasive thymoma
thymus atrophies in about 90%
of cases. The thymic gland
4. Thymic carcinoma
grows back over the next sever 5. Lymphoma
al months, and the volume of
a.
Hodgkin lymphoma
the rebounding thymus may ex b.
Non-Hodgkin lymphoma
ceed the baseline value.
6. Teratoma
In addition to CT evaluation of
Cystic Lesions
the size and shape of the thymus
1. Thymic cyst
(Figs. 7A and 7B), MRI also can
2. Lymphatic malformation
show a hyperplastic thymus,
Fatty Lesions
which is typically fatty and can be
1. Lipoma
distinguished from the soft-tissue
2. Thymolipoma
component of more ominous enMiddle Mediastinal Masses
tities, such as lymphoma. ThyVascular Lesions
mic rebound is seen routinely
1. Double aortic arch
on follow-up PET studies of
patients with neoplasms and
2. Right aortic arch with aberrant left subclavian artery
should not be confused with a
3. Left aortic arch with aberrant right subclavian artery
pathologic condition (Fig. 7C).
4. Pulmonary artery sling
A standardized uptake value of
5. Duplicated superior vena cava
more than 4 on PET suggests
Nonvascular Lesions
malignancy, although there can
1. Congenital foregut duplication cysts
be substantial overlap of benign
a. Bronchogenic cyst
and malignant processes.
b. Esophageal duplication cyst
ThymomaThymomas are
c. Neurenteric cyst
epithelial neoplasms containing a
2. Lymphadenopathy
variable amount of lymphocytes.
a. Neoplasm
Although representing about 20%
i. Primary (i.e., lymphoma)
of mediastinal tumors, thymomas
ii.
Metastatic disease
are infrequent in the pediatric
b. Infection
population, accounting for only
12% of mediastinal tumors.
Posterior Mediastinal Masses
Thymomas may be discovered
Sympathetic Ganglion Tumors
incidentally, although about one
1. Neuroblastoma
third of patients have symptoms
2. Ganglioneuroblastoma
related to local compression or
3. Ganglioneuroma
invasion. About 40% of patients
Nerve Sheath Tumors
with thymomas present with a
1. Schwannoma
paraneoplastic syndrome, such
2. Neurofibroma
as hypogammaglobulinemia; red
3. Malignant peripheral nerve sheath tumor
cell aplasia; or, most commonly,
myasthenia gravis.
Thymomas are typically classified as noninvasive or invasive thymoma. Noninvasive thymomas tend to have well-defined margins because they do not extend beyond their fibrous
capsules (Fig. 8). In contrast, an invasive thymoma does extend beyond its fibrous capsule
(Fig. 9), tending to spread locally to invade adjacent mediastinal structures and the chest wall.
An invasive thymoma also may spread contiguously along the pleural surface, usually unilaterally, and often recurs after surgical removal.

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Mediastinal Masses in Children

On chest radiographs, thymomas most often appear as oval anterior mediastinal masses
that usually project on one side of the chest.
They are often seen best on the lateral image,
obliterating the retrosternal clear space. There
may be thin linear peripheral capsular calcification. Invasive thymomas can present with
pleural nodules or masses. CT confirms an
oval or lobulated enhancing anterior mediastinal mass and may show thin capsular calcification. Cystic regions and necrosis are seen in
about 30% of cases, particularly in larger tumors. Signs of invasion include irregular borders; obliteration of mediastinal fat planes surrounding mediastinal vascular structures;
pericardial thickening; and extension to the
chest wall, pleura, or diaphragm. On MRI, a
thymoma typically has high signal intensity
on T2-weighted images, but this modality
usually does not provide more diagnostic inFig. 1Lateral chest radiograph shows three
formation than does CT.
mediastinal compartment locations. Anterior
Encapsulated and invasive thymomas are
mediastinal compartment is area surrounded
histologically identical. Because the diagnoby sternum anteriorly and anterior margin of
pericardium posteriorly. Middle mediastinal
sis of invasive thymoma is therefore based
compartment is bordered by anterior margin of
on visualization of gross or microscopic evipericardium and imaginary line drawn approximately
dence of extension through the capsule, there
1-cm posterior to anterior margin of thoracic
vertebral bodies. Posterior mediastinal compartment
must be surgical excision of the entire mass.
is area surrounded anteriorly by imaginary line
Thymic carcinomaThymic carcinoma is
drawn 1-cm posterior to anterior border of thoracic
an
aggressive epithelial carcinoma that is hisvertebral bodies and posteriorly by paravertebral
gutters.
tologically characterized by malignant features of nuclear atypia, numerous mitotic figures, and necrosis. Rare in the pediatric population, thymic carcinomas usually occur in the fifth
or sixth decades of life. Almost all affected patients are symptomatic at presentation, usually
with chest pain and constitutional symptoms such as weight loss, fatigue, and night sweats.
Thymic carcinomas usually present as large irregular anterior mediastinal masses with
aggressive local spread and mediastinal vascular invasion (Fig. 10). They often contain calcification and enhance heterogeneously with areas of necrosis. Thymic carcinoma has a poor
prognosis because there is aggressive local growth and distant metastatic disease is common.
Treatment options include neoadjuvant chemotherapy to improve the resectability of the tumor and postoperative radiation therapy.
LymphomaLymphoma is the most common anterior mediastinal mass in children. Although Hodgkin lymphoma typically occurs before age 10 years, non-Hodgkin lymphoma is
common in both the first and second decades of life. Hodgkin lymphoma, which is histologically characterized by the presence of Reed-Sternberg cells, has a good prognosis, with an
approximately 90% cure rate. Non-Hodgkin lymphoma is due to clonal proliferation of either
T or B cell origin. Lymphadenopathy from mediastinal lymphoma may cause cough, dyspnea, dysphagia, hemoptysis, or SVC syndrome. Nonspecific B symptoms, which are more
common with aggressive lymphomas, are fever, weight loss, and night sweats.
About 85% of Hodgkin lymphomas show intrathoracic involvement at presentation, most
commonly affecting the anterior superior mediastinal lymph nodes (Fig. 11). The nodes rarely
calcify before treatment, and approximately 5% calcify after therapy. In addition to enlarged
lymph nodes or nodal conglomerations, Hodgkin lymphoma may manifest as multiple pulmonary nodules and multifocal consolidation; about 15% of patients have pleural effusions.
In non-Hodgkin lymphoma, about 50% of cases show intrathoracic involvement (Fig. 12).
The anterior and posterior mediastinal nodal chains are equally involved. Associated thoracic findings include pulmonary nodules that may cavitate, airspace consolidation, and diffuse interstitial thickening. Pleural involvement in non-Hodgkin lymphoma may manifest as
effusions or pleural masses.

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Ranganath et al.

Fig. 2Prominent but normal thymus in 1-week-old boy with cough and possible pneumonia.
A, Frontal chest radiograph shows prominent thymus (arrows). There is no mass effect on trachea and
mainstem bronchi.
B, Lateral chest radiograph shows prominent but normal thymus (T) in anterior mediastinum, with no mass
effect on trachea.
Fig. 3Radiograph shows thymic sail sign in 4-dayold girl with respiratory distress. Note normal thymus
(arrow) with shape similar to nautical sail.

Fig. 4Prominent but normal thymus, which is incidental finding in 1-month-old boy who underwent CT
examination for pulmonary sequestration.
A, Contrast-enhanced axial CT image shows prominent but normal thymus (arrows), characterized by
homogeneous attenuation and without mass effect on adjacent mediastinal structures.
B, Contrast-enhanced coronal CT image again shows prominent but normal thymus (arrows), with no mass
effect on adjacent great vessels or heart.

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Mediastinal Masses in Children

Fig. 5Prominent but normal thymus in 1-year-old boy with suspected pneumonia diagnosed as possible
mediastinal mass by outside hospital radiologist.
A, Fluoroscopy image shows prominent thymus (arrow) at end-expiratory breathing cycle.
B, Size of previously noted prominent thymus (arrow) is markedly decreased at end-inspiratory breathing cycle,
confirming prominent but normal thymus.

Chest radiographs in lymphoma show an

anterior mediastinal mass with obliteration
of the retrosternal clear space or mediastinal
widening (Figs. 11 and 12). Close inspection
of the trachea is valuable in differentiating
an anterior mediastinal mass from normal
thymus in younger children because even a
prominent thymus should not exert mass effect on or displace the trachea.
On CT, lymphomatous involvement of the
thymus often causes homogeneous enlargement of the gland. However, larger nodal
conglomerates often become heterogeneous,
with hypodense and cystic areas suggestive
Fig. 6Azygos extension of normal thymus in 3-yearold boy who underwent MRI for possibly abnormal
of necrosis (Fig. 13). MRI is not typically
mediastinal contours seen on chest radiographs.
used to evaluate mediastinal lymphoma bePosterior extension (arrow) of normal thymus (T)
cause CT is superb at depicting the anatomic
between trachea and superior vena cava is seen on
enhanced T1-weighted image with fat saturation.
extent of disease. PET is often added to CT
Posterior extension of thymus is connected to
both at the time of initial staging and to folnormally positioned normal thymus and has same
low the response to treatment.
signal intensity. There is no mass effect on adjacent
About 80% of patients with Hodgkin lymvessels or airway.
phoma can achieve disease-free survival.
The current preferred treatment involves combining chemotherapy medications, which not
only provides a synergistic effect but also limits the toxicity of individual drugs. Children
with both types of lymphoma are treated with radiation therapy and chemotherapy; bone marrow transplantation is also used in those with non-Hodgkin disease.
TeratomaGerm cell neoplasms arise from collections of primitive germ cells that arrest in the
anterior mediastinum on their migration to the gonads during embryologic development. Because
they are histologically indistinguishable from germ cell tumors arising in the testes and ovaries,
the diagnosis of a primary malignant mediastinal germ cell neoplasm requires exclusion of a primary gonadal tumor as a source of mediastinal metastases. Teratoma accounts for approximately
60% of all germ cell tumors in the mediastinum. Most are asymptomatic; however, when large,
affected patients may present with respiratory distress due to airway compromise. There are two
main types of teratoma: immature malignant teratoma and mature benign teratoma.
On chest radiographs, both immature and mature teratomas usually present as round and
sharply marginated anterior mediastinal masses. Calcification, which occurs in about 25% of
teratomas, may be central, peripheral, or curvilinear. The hallmarks of both immature and
mature teratoma are fat, fluid, and calcified components on CT or MRI. The presence of fat,

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Ranganath et al.

Fig. 7Thymic hyperplasia in 6-year-old girl with

lymphoma.
A, Contrast-enhanced axial CT obtained after
completion of chemotherapy shows atrophic thymus
(arrows).
B, Repeat study 3 months after A shows enlargement
of thymus (arrows).
C, Frontal PET image shows homogeneous normal
level of 18F-FDG uptake in hyperplastic thymus
(arrow). There is no focal increased uptake to
suggest focus of malignancy.

fluid, and calcified components within an

anterior mediastinal mass in a pediatric patient can help differentiate a teratoma from
other types of mediastinal masses (Fig. 14).
Another valuable diagnostic feature is the
presence of solid tissue within the lesion, a
feature more commonly seen in immature
malignant teratomas than in mature benign
tumors. Mature benign teratomas tend to
displace rather than invade adjacent strucC
tures (the latter appearance being more suggestive of malignant immature tumors). Mature benign teratomas have an excellent prognosis
after complete excision. Conversely, immature malignant teratomas must be treated with multiple modalities, including chemotherapy and radiation, in addition to surgery.

Fig. 8Noninvasive thymoma in 17-year-old girl

who presented with cough and abnormal chest
radiographs. Contrast-enhanced axial CT image
shows heterogeneously enhancing anterior
mediastinal mass with well-circumscribed borders
and mass effect on adjacent heart.

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Fig. 9Invasive thymoma in 18-year-old woman

with shortness of breath and weakness. Contrastenhanced CT image shows heterogeneously
enhancing anterior mediastinal mass with focal area
(arrow) extending to subpleural region. Follow-up
study after mass removal showed development of
subpleural tumor nodules.

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Cystic Lesions
Thymic cystA thymic cyst is a rare fluidfilled mass that represents a cystic remnant of
the thymopharyngeal duct. Although typically found in the lateral infrahyoid neck and
intimately associated with the carotid sheath,
a thymic cyst may occur anywhere along the
thymopharyngeal duct from the pyriform sinus to the anterior mediastinum. It may also
be connected to the mediastinal thymus directly or by a fibrous cord. The pathologic
visualization of Hassall corpuscles in the cyst
wall confirms the diagnosis. Often asymptoFig. 10Thymic carcinoma in 6-year-old girl with
matic, when sufficiently large a thymic cyst
shortness of breath, anterior chest wall pain, and
may cause dysphagia, respiratory distress, or
weight loss for past 4 months. Contrast-enhanced
vocal cord paralysis. Most affected patients
axial CT image shows heterogeneously enhancing
anterior mediastinal mass with invasion of anterior
present between 215 years of age, although
chest wall and destruction of sternum (arrow).
thymic cysts rarely may present in adults.
Thymic cysts are usually smooth and thinwalled and may have mural nodularity. There is usually a large dominant cyst, although the
lesion may be multiloculated. Larger thymic cysts may present as dumbbell-shaped cervicothoracic masses, with the mass traversing the thoracic inlet from the lower lateral neck into
the superior mediastinum (Fig. 15).
Contrast-enhanced CT and MRI show a thymic cyst as a nonenhancing low-density cystic mass.
The MRI signal depends on the contents of the cyst fluid, with proteinaceous or hemorrhagic

Fig. 11Hodgkin lymphoma in 15-year-old boy who

presented with cough and weight loss for 6 months.
A, Frontal chest radiograph shows mediastinal
widening (arrows).
B, Contrast-enhanced axial CT image shows
lobulated mediastinal mass (asterisk) with epicenter
in anterior mediastinum. Note mass effect on
adjacent superior vena cava (arrow) and trachea (T).
C, Contrast-enhanced coronal CT image better shows
craniocaudal extension of mass (asterisk). Again
noted is compressed superior vena cava (arrow).

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Ranganath et al.

B
Fig. 12Non-Hodgkin lymphoma in 14-year-old girl
with low-grade fever and weight loss for 3 months.
A, Frontal chest radiograph shows large mediastinal
mass (arrow).
B, Lateral chest radiograph shows location of mass
(asterisk) within anterior mediastinum.
C, Contrast-enhanced axial CT image shows mildly
enhancing anterior mediastinal mass (asterisk).

Fig. 13Hodgkin lymphoma with areas of necrosis or

cystic change in 12-year-old-girl with chronic cough.
Contrast-enhanced axial CT image shows large
anterior mediastinal mass containing areas of low
attenuation (asterisks) that likely represent areas of
necrosis or cystic change.

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Fig. 14Mature teratoma in 3-year-old boy with

respiratory distress. Contrast-enhanced axial
CT image shows large anterior mediastinal mass
containing areas of soft tissue, fat (asterisk), and
calcification (arrow). Mass is causing significant
mass effect on heart and atelectasis of right lower
lobe.

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Mediastinal Masses in Children

Fig. 15Thymic cyst in 7-year-old girl who presented with shortness of breath and abnormal chest radiograph.
A, Contrast-enhanced axial CT image shows large hypodense cystic mass (asterisk) at level of thoracic inlet in
expected location of thymus. Note mass effect on trachea (T).
B, Contrast-enhanced coronal CT image better shows craniocaudal extension of mass (asterisk) in lower neck
and upper mediastinum and mass effect on airway.

Fig. 16Lymphatic malformation in 11-year-old boy

with shortness of breath and chest pain. Contrastenhanced axial CT image shows predominantly cystic
anterior mediastinal mass with multiple internal
septations (arrows) insinuating along mediastinal
space and causing mild mass effect.

Fig. 17Lymphatic malformation in 3-month-old

boy with large right neck mass. Coronal T2-weighted
image shows large predominately cystic mass with
multiple internal septations. Although epicenter of
lesion is in right side of neck, there is clear extension
into superior mediastinum (arrow), finding commonly
seen with these lesions.

material appearing hyperintense on T1-weighted images. Ultrasound may be performed to

characterize the fluid-filled mass, although CT and MRI are superior for showing the extent
of the lesion and its relationship to the mediastinal structures. The prognosis is excellent if the
lesion is completely resected.
Lymphatic malformationA lymphatic malformation is a lymph-containing cystic or
multicystic structure lined by endothelium. In the past, it was referred to as lymphangioma
or cystic hygroma because the suffix oma was used to described neoplasms and malformations. However, because malformations are not neoplasms, words such as lymphangioma or hygroma are misnomers, and the terminology is being abandoned. It is now termed
lymphatic malformation based on the currently accepted classification of vascular malformations by Mulliken and Glowacki. It may occur anywhere in the body and frequently involves the cervical region, with extension into the anterior mediastinum. Most lymphatic
malformations present early in life. Often asymptomatic, they may present with dyspnea
when compressing the airway or other vital mediastinal structures. Depending on the size of

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Ranganath et al.

Fig. 18Mediastinal lipoma in 17-year-old girl with abnormal chest radiograph obtained for evaluation of
possible pneumonia.
A, Contrast-enhanced axial CT image shows homogeneous mass (arrow) with low attenuation value,
compatible with fat and similar to that of adjacent chest wall fat (asterisk).
B, Contrast-enhanced coronal CT image shows mediastinal mass with homogeneous fat attenuation (arrow)
and no mass effect on adjacent aorta (A).

the locules, lymphatic malformations may be classified as macrocystic or microcystic. Imaging studies usually reveal a multicystic mass that is smooth and well defined and contains
septations and a variable number and size of cysts (Figs. 16 and 17). CT and, more frequently,
MRI may show enhancement of internal septa and the cyst wall but no enhancement of central portions (Fig. 16). In the case of previous hemorrhage or infection producing proteinaceous material within the cysts, lymphatic malformations appear as more complex fluid
collections instead of simple fluid-filled cystic masses. The primary treatment of lymphatic
malformations includes surgical resection or percutaneous sclerotherapy, with the latter more
commonly used for the macrocystic type.

Fatty Lesions
LipomaLipomas are encapsulated masses with a composition identical to subcutaneous
fat. They may occur anywhere in the body, including the mediastinum. Because lipomas are
soft and pliable, patients are usually asymptomatic and the lesion is found incidentally on a
chest radiograph or CT image obtained for another reason. Depending on the size and location of the lipoma, chest radiographs may show a mass that is relatively radiolucent compared
with adjacent soft tissues. CT and MRI can confirm the fatty nature of the mass (Fig. 18).
ThymolipomaA thymolipoma is an uncommon benign anterior mediastinal mass that
consists of normal thymic tissue interspersed with fat. Because these are pliable fatty masses,
patients are usually asymptomatic. A thymolipoma usually appears as a large and sharply
marginated mass. Chest radiographs show the low density of the lesion in about one half of
cases. CT and MRI can confirm the diagnosis by showing nonenhancing soft tissue interwoven within fat in a somewhat whorled appearance (Fig. 19). No treatment is necessary unless
this benign tumor becomes massive and exerts mass effect on adjacent structures.

Middle Mediastinal Masses

For practical purposes, middle mediastinal masses in children can be classified into two
groups: vascular and nonvascular lesions.

Vascular Lesions
Double aortic archA double aortic arch is a congenital aortic arch anomaly related to the
embryologic persistence of both the left and right fourth aortic arches. It accounts for about
55% of symptomatic vascular rings. Because the trachea and esophagus are encircled, patients typically present with airway and esophageal compression, causing inspiratory stridor
that worsens with feeding. Patients usually present soon after birth, and there is frequently
associated tracheomalacia.

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Mediastinal Masses in Children

Fig. 19Mediastinal thymolipoma in 7-year-old girl

with abnormal chest radiograph. Contrast-enhanced
coronal CT image shows nonenhancing soft tissue
(curved arrow) interwoven within fat (straight
arrows).

Fig. 20Double aortic arch in 2-week-old boy

with respiratory distress and feeding difficulty.
Frontal fluoroscopic image of upper GI series
shows persistent concentric narrowing (arrows) of
midesophagus.

Chest radiography shows prominent soft tissue on either side of the trachea, which can result in an abnormal midline position or slight tracheal deviation away from the dominant arch.
The right arch is more commonly higher and more prominent than the left. The lateral view
shows anterior and posterior compression of the trachea at the level of the arch. Barium swallow studies show bilateral indentations on the contrast-filled upper esophagus, with focal circumferential narrowing at the expected level of the aortic arch (Fig. 20). On the lateral image,
there is an oblique or nearly horizontal sharp indentation on the posterior esophagus. However, fluoroscopic studies rarely obviate cross-sectional imaging to provide the more defined
anatomic definition required before surgery (Fig. 21). Each arch gives rise to a carotid and a
subclavian artery, resulting in the symmetric four-artery sign on CT and MRI at the thoracic
inlet. The smaller of the two arches may be partially atretic. Treatment of double aortic arch in
symptomatic patients is surgical division performed on the side of the nondominant arch.
Despite surgical treatment, approximately 30% of postoperative patients have persistent airway symptoms because of tracheomalacia or persistent extrinsic airway compression.
Right aortic archRight aortic arch results from the persistence of the right fourth embryologic arch. There are two main types of right aortic arch: right aortic arch with an aberrant left subclavian artery and right aortic arch with mirror image branching. A right aortic
arch with an aberrant left subclavian artery is often an incidental finding, with about 95% of
patients being asymptomatic. However, the presence of a left ligamentum arteriosum can result in a complete vascular ring. Patients with this condition may present with respiratory
distress and dysphagia. Conversely, a right aortic arch with mirror image branching is associated with cyanotic congenital heart disease and usually does not cause respiratory distress.
Chest radiographs show an aortic arch located to the right of the trachea deviating the trachea to the left and a right-sided descending aortic line. On the lateral image, there may be
anterior bowing of the trachea or an indentation on its posterior aspect. Barium swallow studies can show an oblique filling defect coursing from right-inferior to left-superior, with the
lateral image showing a posterior esophageal indentation. Aneurysmal dilatation of the subclavian artery origin may be present in up to 60% of patients with an aberrant left subclavian
artery. Known as a diverticulum of Kommerell, it is well visualized on CT or MR angiogra-

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Ranganath et al.

Fig. 21Double aortic arch in 3-day-old girl with respiratory distress and feeding difficulty.
A, Contrast-enhanced axial CT image shows double aortic arch (arrows) encircling trachea (T) and esophagus (E).
B, Superior view of 3D volume-rendered image of vascular structure and heart shows double aortic arch.

Fig. 22Right aortic arch with aberrant left subclavian artery in 9-year-old boy with respiratory distress.
A, Contrast-enhanced axial CT image shows right aortic arch (RA) with aberrant left subclavian artery (arrow).
Trachea (T) is compressed by right aortic arch with diverticulum of Kommerell at takeoff of aberrant left
subclavian artery.
B, Contrast-enhanced coronal CT image better shows entire course of aberrant left subclavian artery (arrow).
RA = right aortic arch.

phy (Fig. 22). CT angiography is superior to MR angiography because it also provides exquisite evaluation of the airway and lungs; however, it is associated with ionizing radiation. An
associated constricting left ligamentum arteriosum may cause stridor, requiring surgical division of this structure via a left thoracotomy.
Left aortic arch with aberrant right subclavian arteryThe mirror image of a right aortic
arch with aberrant left subclavian artery, a left aortic arch with aberrant right subclavian artery usually does not cause airway compression and is an incidental finding. Rarely, esophageal compression produces dysphagia lusoria (an abnormal condition characterized by difficulty swallowing).
A frontal chest radiograph is usually normal but occasionally may show mediastinal widening. On the lateral image, there may be a posterior mass effect on the trachea. Although a
barium swallow study may show an oblique indentation on the posterior esophagus coursing
from left-inferior to right-superior and suggesting the presence of an aberrant right subclavian
artery, cross-sectional imaging studies such as CT and MRI can confirm the presence and
show the entire course of an aberrant right subclavian artery (Fig. 23). Symptomatic patients
usually require surgery to ligate the right ligamentum arteriosum.

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Mediastinal Masses in Children

Fig. 23Left aortic arch with aberrant right subclavian artery in 2-year-old boy with feeding difficulty and
failure to thrive.
A, Contrast-enhanced axial CT image shows left aortic arch (LA) with aberrant right subclavian artery (arrow).
T = trachea.
B, Posterior view of 3D volume-rendered image shows entire course of aberrant right subclavian artery (arrow).
DA = descending aorta.

Fig. 24Pulmonary artery sling in 3-day-old boy with respiratory distress.

A, Contrast-enhanced axial CT image shows anomalous left main pulmonary artery (arrow) arising from right
pulmonary artery. Anomalous left main pulmonary artery courses between trachea (T) and esophagus (E).
B, Superior view of 3D volume-rendered image shows entire course of anomalous left main pulmonary
artery(arrow).

Pulmonary artery slingPulmonary artery sling, also known as anomalous origin of the
left pulmonary artery, is a condition in which the left pulmonary artery arises from the right
pulmonary artery rather than from the main pulmonary artery. It is the only vascular ring that
courses between the trachea and esophagus and is associated with asymmetric lung aeration.
Affected patients most commonly present with severe stridor and hypoxia.
A lateral chest radiograph may show a round soft-tissue density between the distal trachea
and esophagus. On barium swallow studies, there is usually an anterior indentation on the
esophagus. CT and MRI can confirm the diagnosis and provide preoperative delineation of
the anatomy (Fig. 24). At surgery, the left pulmonary artery is separated from its anomalous
origin and subsequently connected to the main pulmonary artery. If there is concomitant
tracheal stenosis or malacia, the trachea also needs to be surgically repaired.
Duplicated superior vena cavaA left SVC results from persistence of the left common
cardinal vein. It is usually associated with a normal or slightly smaller right SVC, resulting in
a duplicated SVC that is most often an incidental finding but may be associated with congenital heart disease. A left-sided SVC typically drains into the coronary sinus.

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Ranganath et al.

Fig. 25Duplicated superior vena cava in 4-month-old boy who underwent CT for evaluation of possible
central airway anomaly in setting of respiratory distress.
A, Contrast-enhanced axial CT image shows duplicated superior vena cava (arrows).
B, Contrast-enhanced coronal CT image shows duplicated superior vena cava (arrows) with no bridging vein.
Left superior vena cava drains into coronary sinus (CS).

On a frontal chest radiograph, a left-sided SVC often manifests as a straight border forming
a prominent left paratracheal stripe. The presence of a left-sided SVC is often first detected
when a left-sided central catheter or pacer lead is seen entering this vessel. CT and MRI show
a tubular structure that originates from the junction of the left internal jugular and subclavian
veins and courses along the left side of the mediastinum before draining into the coronary
sinus (Fig. 25). No treatment is needed for a left-sided SVC, but note should be made of this
anomaly in case the patient subsequently requires catheter placement or thoracic surgery.

Nonvascular Lesions
Congenital foregut duplication cystsIntrathoracic foregut duplication cysts are uncommon congenital anomalies that result from developmental malformations of the embryonic
foregut. They can be classified into three types: bronchogenic, esophageal duplication, and
neuroenteric cysts.
Bronchogenic cysts are caused by abnormal lung budding and development of the ventral foregut during the first trimester. Although more than one half of pediatric patients with

Fig. 26Bronchogenic cyst in 9-year-old boy who presented with cough and fever.
A, Frontal chest radiograph shows large well-circumscribed mass (asterisk) with internal air-fluid level located
near left hilum.
B, Contrast-enhanced axial CT image confirms mediastinal origin of mass. Well-circumscribed cystic mass
(asterisk) containing air-fluid level is well visualized.

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Mediastinal Masses in Children

Fig. 27Esophageal duplication cyst in 3-yearold boy with dysphagia for solids and wheezing.
Contrast-enhanced axial CT image shows cystic
mass (asterisk) in region of esophagus, causing mass
effect on anteriorly located trachea.

bronchogenic cysts are asymptomatic, some

patients may experience dyspnea if the lesion
is large enough to exert mass effect on the adjacent airways in the mediastinum (Fig. 26).
Bronchogenic cysts can be located anywhere
along the tracheoesophageal tree, but they tend
to occur near the carina or right paratracheal
regions. About 20% are in an intrapulmonary
location. The visualization of respiratory epithelium on histology provides the definitive
diagnosis of bronchogenic cyst.
Esophageal duplication cysts result from
abnormal development of the posterior division of the embryonic foregut (Fig. 27). Dysphagia is the most common presenting
symptom. Although esophageal duplication
cysts are typically located adjacent to the upper third of the esophageal wall, they may be
found within the lung parenchyma if they

Fig. 28Neurenteric cyst in 4-year-old boy with

congenital scoliosis.
A, Frontal radiograph of cervicothoracic junction
shows several segmentation anomalies (arrow) and
levoscoliosis.
B, Coronal T2-weighted image shows round mass
(asterisk) with fluid signal confirming cystic nature
located near vertebral segmentation anomalies
seenin A.
C, Sagittal T2-weighted image shows mass in
posterior mediastinum with proximal connection
(arrow) of cyst (asterisk) into spinal canal.

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become detached from the esophagus during

development and subsequently migrate with a
lung bud. The histologic visualization of a submucosa or muscular layer of the gastrointestinal tract within the lesion provides the definitive diagnosis of esophageal duplication cyst.
Neurenteric cysts result from failure of
separation of the gastrointestinal tract from
the primitive neural crest during early embryonic life. Most neurenteric cysts are
found in the posterior mediastinum (Fig. 28)
where they can extend into or communicate
with the spinal canal and be associated with Fig. 29Non-Hodgkin lymphoma in 7-year-old
congenital osseous defects of the spine. His- boy who presented with night sweats, shortness
tologically, both neural elements and gastro- of breath, and weight loss. Contrast-enhanced
axial CT image shows multiple soft-tissue masses
intestinal epithelium are typically seen. Af- representing adenopathy in both hilar and subcarinal
fected patients often present with pain, regions of middle mediastinum. Note left axillary
which usually leads to early imaging evalua- adenopathy (arrow).
tion and subsequent diagnosis.
On imaging studies, all three types of foregut duplication cysts usually appear as solitary
well-circumscribed round or oval cystic masses (Figs. 2628). About one half of them show
simple fluid attenuation on CT. However, foregut duplication cysts commonly contain proteinaceous material, which may have an attenuation value higher than water. There is no
substantial internal contrast enhancement within a foregut duplication cyst unless there is
superimposed infection. Complete surgical resection is the usual treatment of all foregut duplication cysts, particularly in symptomatic pediatric patients.
LymphadenopathyLymphadenopathy in the middle mediastinal compartment in children is
most commonly due to either neoplastic or infectious processes. Middle mediastinal lymph node
masses are often malignant, reflecting either a primary neoplasm, such as lymphoma (which also
can extend from the anterior mediastinum) or metastases from a distant tumor (Fig. 29). In children,
metastatic lymphadenopathy often results from primary tumors in the abdomen and pelvis, such as
Wilms tumor, testicular neoplasms, and various sarcomas (Fig. 30). Mediastinal metastatic lymphadenopathy usually appears on CT and MRI as homogeneous or heterogeneous soft-tissue masses
that can represent a conglomeration of nodes. Determining the primary tumor responsible for metastatic mediastinal lymphadenopathy is often difficult. The presence of calcification suggests treated
lymphoma or osteosarcoma metastases in pediatric patients. As with other mediastinal masses, patients with metastatic mediastinal lymphadenopathy may be asymptomatic or present
with a variety of symptoms (such as dyspnea
or chest pain) that depend on the extent of the
lymphadenopathy and its mass effect on vital
adjacent mediastinal structures.
The most common infectious causes of
middle mediastinal lymphadenopathy in children are tuberculosis and histoplasmosis (Fig.
31). Affected patients generally also have pulmonary parenchymal abnormalities on imaging studies that aid in reaching a correct diagnosis. However, isolated lymph node enlargement may occur. Treatment consists of approFig. 30Lymphadenopathy from metastatic germ
priate therapy of the underlying infection.

Posterior Mediastinal Masses

Sympathetic Ganglion Tumors
NeuroblastomaAbout 90% of posterior
mediastinal tumors are neurogenic, derived
from the sympathetic chains that are located

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cell tumor in 17-year-old boy with testicular germ

cell tumor confirmed after orchiectomy. Patient
presented with cough and fever and underwent
chest CT for staging after multiple pulmonary nodules
were detected on chest radiography. Contrastenhanced axial CT image shows enlarged subcarinal
lymph node (asterisk) and metastatic pulmonary
nodules(arrows).

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Mediastinal Masses in Children

Fig. 31Tuberculosis in 12-year-old boy with cough and low-grade fever who underwent chest radiography for
positive purified protein derivative test.
A, Frontal chest radiograph shows slightly prominent right paratracheal stripe and dense pulmonary nodule
(arrow) in right upper lobe.
B, Contrast-enhanced sagittal CT image shows lymphadenopathy with coarse calcifications (arrows) in middle
mediastinum.

Fig. 32Neuroblastoma in 1-year-old boy who presented with opsoclonus-myoclonus syndrome. Chest
radiographs showed posterior mediastinal mass.
A, Contrast-enhanced axial CT image shows well-defined oval soft-tissue mass (asterisk) with internal
calcifications located in posterior mediastinum.
B, Contrast-enhanced coronal CT image shows large soft-tissue mass (asterisk) with calcifications extending
from upper thoracic spine to right hilum.

along the thoracic vertebral bodies. In pediatric patients, the vast majority of these neurogenic tumors are neuroblastomas; the rest are ganglioneuroblastoma or ganglioneuroma.
Neuroblastoma is a malignant tumor of primitive neural crest cells. Although most develop
in the adrenal gland, neuroblastomas can arise anywhere along the sympathetic chain, particularly within the posterior mediastinum (about 20% of neuroblastomas in pediatric patients). The typical clinical presentation includes fever, irritability, weight loss, and anemia.
Cord compression by the tumor may cause paraplegia, extremity weakness, and altered bowel or bladder function.
Chest radiographs often show soft-tissue opacity in the paravertebral region, sometimes associated with rib or vertebral erosion or destruction or widening of intercostal spaces. Calcification within the tumor is common, reportedly occurring in up to 30% of cases. Bone metastases
from neuroblastoma are also common and usually appear as lytic and permeative. CT can delineate the margins of the tumor and internal calcification as well as local extension and distant
metastases (Fig. 32). Because it is an invasive tumor, neuroblastoma tends to surround and

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Ranganath et al.

B
Fig. 33Ganglioneuroma in 16-year-old girl with
abnormal chest radiographs obtained for cough and
chest pain.
A, Frontal chest radiograph shows large elliptical
mass (asterisk) in right paraspinal region behind right
hilum. Sharp margins and obtuse angles of mass
suggest posterior mediastinal location.
B, Lateral chest radiograph confirms posterior
mediastinal location of mass (asterisk).
C, Contrast-enhanced axial CT image shows
homogeneous well-defined hypodense mass
(asterisk) abutting spine in posterior mediastinum.

C
encase blood vessels and to spread through the neural foramina into the spinal canal. MRI is the
best imaging modality for showing local spread of tumor and for detecting tumor extension into
the spinal canal, which influences surgical planning and management. On MRI, neuroblastomas
show high signal on T2-weighted images and low signal on T1-weighted images. They tend to
enhance early, reflecting the high vascularity of the tumor. Metaiodobenzylguanidine (MIBG)
scintigraphy is a highly sensitive imaging modality for determining the extent of disease, with
avid uptake of the radionuclide related to catecholamine production by the tumor.
The current treatment of choice for neuroblastoma in pediatric patients is surgical resection, with adjuvant chemotherapy and radiation therapy for advanced disease. The prognosis
varies depending on the location and extent of the tumor. Children with thoracic neuroblastoma have a more favorable prognosis than those with abdominal tumors.
Ganglioneuroblastoma and ganglioneuromaGanglioneuroma and ganglioneuroblastoma are less aggressive tumors than neuroblastomas. These tumors of primitive neural crest
cells also often arise in the posterior mediastinum. Ganglioneuroma is currently considered
to be a benign neoplasm, whereas ganglioneuroblastoma has the potential for distant metastases and thus is considered malignant. Radiologically, they are difficult to differentiate from
neuroblastomas because of similar imaging characteristics on all imaging modalities (Figs.
33 and 34). However, ganglioneuroblastoma and ganglioneuroma may have a more fusiform
configuration than neuroblastoma. Age is also a diagnostic clue: ganglioneuroblastomas and
ganglioneuromas tend to occur in older children, whereas neuroblastomas are seen during the
first few years of life. Histopathologic analysis is required for a definitive diagnosis.

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Mediastinal Masses in Children

Fig. 34Ganglioneuroblastoma in 5-year-old boy with chest pain and limping.

A, Coronal T1-weighted image shows large relatively homogeneously enhancing paraspinal soft-tissue mass
(asterisk).
B, Contrast-enhanced axial T1-weighted image of large paraspinal mass (asterisk) shows extension (arrow) into
spinal canal, causing mass effect on thecal sac (supporting neural origin of mass).

Fig. 35Neurofibroma in 12-year-old boy with known neurofibromatosis type 1.

A, Frontal chest radiograph shows mild widening of right and left paratracheal stripes (arrows).
B, Contrast-enhanced axial CT image shows multiple lobulated soft-tissue masses in paraspinal regions,
following intercostals spaces, and near aortic arch, consistent with neurofibromas (arrows). Note extension
along neural foramen on right.

Nerve Sheath Tumors

Although most posterior mediastinal masses in the pediatric population are sympathetic
ganglion tumors, some reflect benign (schwannoma, neurofibroma) (Fig. 35) or malignant
peripheral nerve sheath tumor. Malignant peripheral nerve sheath tumors are highly cellular
pleomorphic spindle cell sarcomas of nerve sheath origin. In pediatric patients, these tumors
most commonly arise from a preexisting plexiform neurofibroma, as in patients with neurofibromatosis type 1. Less commonly, a malignant peripheral nerve sheath tumor arises de novo
or from a preexisting schwannoma.
A malignant peripheral nerve sheath tumor often appears as a round or oblong posterior mediastinal mass that is associated with a widened neural foramen and follows the course of the involved nerve. On CT, these tumors usually show decreased attenuation because of their lipid
contents or cystic degeneration and have variable enhancement (Fig. 36). Dumbbell-shaped extension into the spinal canal can be seen on both CT and MRI. Features suggestive of a malignant
peripheral nerve sheath tumor, whether arising de novo or from degeneration of a preexisting

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Ranganath et al.
mass, include rapid growth, local invasion, osseous destruction, and pleural effusion. Surgical removal for symptomatic or malignant lesions is the current treatment of choice.
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Conclusion
Mediastinal masses in children may represent congenital anomalies, infections, and
benign and malignant neoplasms. Pseudomasses can pose diagnostic challenges to the
unwary eye. In both asymptomatic and
symptomatic children with mediastinal
masses, imaging evaluation plays a paramount role by providing precise information
regarding location, appearance, size, and relationship to the adjacent mediastinal structures as well as detecting metastases.
Knowledge of the practical diagnostic imaging approach based on the three mediastinal
compartments and the characteristic imaging appearances can lead to a correct diagnosis and subsequent optimal management.
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Fig. 36Malignant peripheral nerve sheath tumor in

20-year-old woman with known neurofibromatosis
type 1. Contrast-enhanced axial CT image shows
multiple round heterogeneously enhancing masses
(asterisks) located in posterior mediastinum and on
pleural surfaces along course of intercostal nerve.

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