Thoracic Neoplasms

i n C h i l d re n
Beverley Newman, MB, BCh

KEYWORDS
 Chest  Pediatric  Neoplasm  Imaging

This article provides an overview of neoplasms Decubitus views may be obtained to evaluate
that occur in the thorax in childhood, including a suspected pneumothorax, free or loculated
those of the mediastinum, lung, airway, heart, pleural fluid, or other fluid-containing lesion. Decu-
and chest wall. Different imaging methods and bitus views are usually not helpful when there is
their role in evaluating these lesions are reviewed. complete whiteout of a hemithorax. Even if free
Because of the broad topic, the intent is not to fluid is present, it cannot be appreciated without
cover specific lesions in detail but to emphasize any adjacent air or aerated lung.
helpful distinguishing features. Non-neoplastic Fluoroscopic evaluation of the chest still has
lesions are included only to serve as examples of occasional value, including assessment of an
where they are confused with tumors and should airway foreign body or other airway obstruction,
be included as differential diagnostic consider- laryngotracheobronchomalacia, or a pulsatile
ations. The long-term effects of childhood cancer vascular mass. Since the advent of high-quality
with reference to the thorax are briefly discussed. cross-sectional chest imaging, an esophagram is
rarely part of the imaging assessment of a vascular
ring or mediastinal mass.
IMAGING MODALITIES
Chest Radiographs Ultrasound Imaging
Chest radiographs are still usually the first imaging There are several questions that can be readily
examination obtained in a child with a palpable answered, at least partially, on sonographic
mass or clinical symptoms referable to the chest.1 imaging.2,3 The thymus is the most common cause
Frontal and lateral views are often helpful in evalu- of an unusual upper mediastinal contour (see
ating the location, size, number, and most likely Fig. 3). Ectopic thymic tissue in the neck or behind
differential diagnostic possibilities when abnor- the superior vena cava is a frequent cause of
mality is visible. The radiographs should be care- concern for a mediastinal mass. In addition,
fully scrutinized for extent of involvement, multiple many anterior mediastinal neoplastic lesions, both
lesions, and specific features, such as cavitation benign and malignant, commonly infiltrate or
and calcification as well as pleural, soft tissue, involve the thymus (see Fig. 1). The thymus has
and bone abnormalities. Chest radiographs serve a characteristic appearance on US and normal
as a triage platform for recommending whether or thymic tissue or lesions in or adjacent to the
not and which additional imaging studies are likely thymus are often readily visualized because there
to be most useful (Figs. 1 and 2). When a neoplasm is no intervening air-filled lung between the trans-
is suspected or considered, additional cross- ducer and anterior mediastinum (see Fig. 3).2
sectional imaging, ultrasound (US), CT, or MR Other masses in the anterior mediastinum as well
imaging is frequently required to better charac- as lesions of the chest wall and pleura often are
terize the nature and extent of the mass and its rela- seen on sonography, allowing characterization of
tionship to vital structures (see Figs. 1 and 2; free fluid versus cystic or solid lesions as well as
radiologic.theclinics.com

Fig. 3). information on vascular flow with spectral and

Financial disclosure: the author has nothing to disclose.

Lucile Packard Children’s Hospital at Stanford University School of Medicine, Department of Radiology, 725
Welch Road, Room 1677, Stanford, CA 94305-5913, USA
E-mail address: [email protected]

Radiol Clin N Am 49 (2011) 633–664

doi:10.1016/j.rcl.2011.05.010
0033-8389/11/$ – see front matter Ó 2011 Elsevier Inc. All rights reserved.
634 Newman

Fig. 1. A 12-year-old girl with a benign anterior mediastinal teratoma. (A) Outside chest radiograph contains
a large amount of linear artifact. A prominent lobulated left superior mediastinal mass (arrows) is, however,
appreciated. The lack of silhouetting of the aortic arch and descending aorta suggests an anterior mediastinal
location. (B) Transverse US and (C) axial CT (mediastinal window) of the upper mediastinum shows that the ante-
rior mediastinal mass is probably originating within the thymus. Note the adjacent normal thymic tissue to the
right (arrow). Multiple hypoechoic cystic and echogenic solid components are evident on US (B). On the CT
(C), solid, fluid, and fatty (arrowhead) components are identified.

color Doppler imaging.1–4 Fat and calcification almost all cases, especially in small children who
may also be identified or suspected on US imaging have little natural tissue contrast or fat to separate
(see Fig. 1). adjacent structures. A timed or fluoroscopically
With the advent of routine prenatal US, many triggered CT angiogram may be obtained when
chest masses are first imaged in utero. The specific vascular detail is needed. Multiplanar
majority of these lesions are bronchopulmonary 2-D as well as maximum intensity projection,
malformations but also occasional chest and minimum intensity projection, and 3-D reconstruc-
cardiac neoplasms. tions are readily obtained with CT and MR images,
so that the location, extent, and characteristics of
a lesion can be optimally displayed and measured.
CT
This helps with therapeutic decisions, surgical
CT is an excellent modality for imaging the chest.5 planning, and follow-up.
Evaluation of a potential thoracic neoplasm typi- CT imaging has the advantage of being fast,
cally entails contiguous axial slices between 1 especially with newer multislice or dual-head
and 5 mm depending on the size of the child and scanners, so that sedation or anesthesia is only
anatomic detail required. Dynamic intravenous required in small children or those who cannot
contrast injection (2–3 mL/kg) is desirable in cooperate with keeping still or holding their breath.
 Thoracic Neoplasms in Children 635

Fig. 2. A 4-year-old boy with a cough—bronchogenic cyst. (A) The frontal chest radiograph demonstrates
a smooth bulging mass in the right superior mediastinum (arrow). (B) Coronal T1-weighted image (postcontrast)
and (C) axial T2-weighted image demonstrate a typical well-defined fluid filled cyst, dark on T1 with rim enhance-
ment and bright on T2-weighted images, located close to the airway (arrows).

CT provides excellent spatial resolution with and slice thickness should be chosen to obtain
details of pulmonary parenchyma optimally visual- diagnostic quality imaging with the lowest possible
ized on CT as opposed to MR imaging. Calcifica- dose. The thyroid should be kept out of the
tion is usually better appreciated on CT than MR imaging field whenever possible and breast
imaging whereas fat is typically well characterized shields can be added in female patients to further
on both CT (see Fig. 1) and MR imaging and fluid decrease breast dose. Multiple series, such as
may be more reliably appreciated on MR imaging noncontrast followed by contrast scans or late de-
(T2 bright) (see Fig. 2).6 Proteinaceous fluids, layed scans, should be avoided unless needed.
such as blood or lymph, may have soft tissue Electrocardiographically gated CT is associated
attenuation on CT and bright signal on T1- with a much higher radiation dose and should be
weighted MR images, creating difficulty in reserved for specific indications, such as detailed
separating a cystic from a solid lesion; US may intracardiac and coronary evaluation. Where
be helpful in these circumstances. appropriate, US or MR imaging should be consid-
The disadvantage of CT imaging is exposure to ered as a substitute for CT.
ionizing radiation at levels considerably higher Iodinated contrast should be used with caution
than plain radiographs.6 This is especially impor- in patients with renal dysfunction, especially with
tant in young children who are much more sensi- a creatinine level greater than 2 mg/dL. Prehydra-
tive to ionizing radiation than adults. Careful tion with fluids and use of bicarbonate and N-ace-
attention must be paid to having clear clinical indi- tylcysteine before CT have been advocated to
cations for obtaining CT and tailoring the study to mitigate the nephrotoxic effects of contrast.
the needs of the patient. Parameters, such as milli- Patients on dialysis can have their contrast CT
ampere second, kilovolt (peak), pitch, collimation, scheduled to coordinate with subsequent dialysis.
636 Newman

Fig. 3. Prominent normal thymus—a 3-week-old boy. (A) Chest radiograph with prominent right mediastinal soft
tissue extending to the diaphragm, thought to probably represent normal asymmetric prominence of the thymus.
A normal thymic wave sign (indentation of the margin of the thymus by the adjacent ribs) is seen on the left
(arrow). (B) Transverse US and (C) right sagittal US images demonstrate the normal sonographic appearance of
the anterior thymus (arrows) (hypoechoic with small punctate and linear areas of echogenicity) with asymmetric
prominence of the right lobe (B) extending down to the right hemidiaphragm (C). L, liver.

Although contrast-related allergic reactions are CT for mediastinal lesions and superior when spine
uncommon in children, vigilant monitoring by evaluation is required (eg, neuroblastoma).6 MR
trained staff with quick access to support equip- imaging is more often used than CT for evaluating
ment and drugs is essential. Nonionic contrast cardiac anatomy with the exception of coronary
agents tend to be used because of fewer allergic evaluation. MR imaging is superior to CT for cardiac
and other contrast reactions, such as vomiting. If functional assessment, including ventricular volu-
contrast is needed in children who had a prior mes and mass, ejection fraction, and cardiac
contrast reaction, they can be pretreated with output as well as quantifying volume and direction
oral steroids beginning 24 hours before the of vascular flow, valvular regurgitation, and shunts.
imaging study. If intravenous contrast cannot be The major disadvantage of MR imaging is
used for CT, there should be careful reconsidera- the length of the examination and, therefore,
tion of the value of the study and whether other increased need for sedation or anesthesia as
alternatives, such as US and MR imaging, might compared with CT. The need for well-trained staff,
substitute. who are skilled in monitoring and managing chil-
dren, and constant vigilance regarding the safety
of a child imaged by either CT or MR imaging
MR Imaging
cannot be overemphasized.
MR imaging provides a great deal of anatomic infor- New faster sequences are allowing for more
mation as well as tissue characterization with re- rapid MR imaging scanning with improved spatial
gard to lesions of the chest. MR imaging is resolution. New contrast agents allow for im-
superior to CT for evaluating most lesions of the proved and prolonged vascular opacification.
chest wall but suboptimal for assessing lung Both MR imaging and CT should be used with
parenchyma.4 MR imaging is often equivalent to caution in patients with renal dysfunction. MR
 Thoracic Neoplasms in Children 637

imaging contrast is generally contraindicated with

an estimated glomerular filtration rate of less than
30 mL/min because of the potential risk of nephro-
genic systemic sclerosis, although few cases have
been reported in children. Allergic reactions to MR
imaging contrast agents are rare.
MR imaging safety is paramount: metallic and
personal electronic objects must be removed
and all equipment must be MR imaging compat-
ible. Patients, parents, and personnel need to be
carefully screened before entering the scanner.
Major contraindications to MR imaging include
Fig. 4. A 10 year old with thymic rebound. Prior
presence of a pacemaker, intracranial vascular
history of large cell lymphoma with increasing soft
clips. and orbital metal.
tissue fullness in the anterior mediastinum. Axial cut
Prenatal MR imaging is commonly used to from a PET-CT demonstrates homogeneous diffuse
further evaluate and follow the nature and extent uptake of the agent in the normal prominent thymus
of thoracic masses picked up on antenatal US (arrows).
examinations. MR imaging is contraindicated in
the first trimester of pregnancy and MR imaging
contrast agent use is discouraged throughout The mediastinum is conveniently divided into 3
pregnancy. spaces that span from superior to inferior. The
anterior mediastinum or prevascular space is
Nuclear Imaging located in front of the heart and great vessels;
the middle mediastinum or vascular space encom-
Chest nuclear imaging in children currently has
passes the heart and most of the major great
a limited role. Ventilation and perfusion imaging
vessels; and the posterior mediastinum or post-
may be used to evaluate airway and vascular
vascular space extends from behind the heart to
pathology and assess for pulmonary emboli or
the spine. Special consideration should be given
differential lung flow. Bone scan, gallium scan,
to the inferior mediastinum where masses may
white blood cell scan, or iodine scan or m-iodo-
be due to diaphragmatic lesions especially dia-
benzylguanidine (MIBG) might be used in specific
phragmatic or hiatal hernias.
instances to evaluate for bone lesions, infection,
When necessary, CT or MR imaging can be ob-
thyroid tissue, or neuroblastoma, respectively.
tained to define the nature, origin, and extent of
Fluorodeoxyglucose positron emission tomog-
a mediastinal lesion.6 Frequently, a specific diag-
raphy (FDG PET) detects metabolically active
nosis can be suggested (see Figs. 1 and 5). Malig-
processes, including tumors and inflammation.
nant lymphoma, benign thymic enlargement,
FDG PET and FDG PET-CT, where the PET meta-
teratomas, foregut cysts, and neurogenic tumors
bolic activity and CT anatomic images can be
account for 80% of mediastinal masses in
superimposed, are increasingly used to evaluate
children.5
and follow malignant tumors, especially lymphoma
(Figs. 4 and 5).7 One disadvantage of FDG PET
and PET-CT is the high dose of ionizing radiation. Anterior Mediastinum
The common masses in the anterior mediastinum
MEDIASTINAL MASSES or prevascular compartment are remembered
most easily by the letter, T, and include thymus,
 Mediastinal masses are more common than teratoma, thyroid, and T-cell lymphoma.
intrapulmonary masses in children and Normal variations of the thymus include bilateral
have a greater likelihood of being malignant or unilateral prominence (most often right), thymic
(Box 1). hyperplasia, or rebound (see Figs. 3 and 4) and
 Compartmentalization of the mediastinum ectopic thymic tissue (most often retrocaval or ex-
is useful in generating likely differential tending into the neck). These variations are
diagnoses. a common reason for suspecting a mediastinal
 Several lesions, especially adenopathy, mass on a chest radiograph in a child. The concern
lymphoma, and vascular/lymphatic lesions, can frequently be put to rest and additional studies
can occur in multiple different compartments. avoided when chest radiographs are reviewed by
 Large lesions often span more than one an experienced pediatric radiologist. When con-
compartment. cern persists as to whether the findings represent
638 Newman

Fig. 5. A 7 year old with anterior mediastinal mass—Hodgkin lymphoma. (A) Chest radiograph demonstrates
widening of the mediastinum with irregular contours (arrows). Note the heterogeneous lumpy appearance of
the infiltrated thymus on both CT (B) and corresponding PET-CT (C) images. There are small abnormal nodes in
the hila bilaterally as well as several small pulmonary nodules that take up the FDG, suspicious for intrapulmonary
involvement with lymphoma. (Reprinted from Rudolph C, Rudolph A, Lister G, et al. Imaging the chest. In:
Rudolph’s textbook of pediatrics. 22nd edition. United States: McGraw-Hill; 2011 [Fig. 9]; with permission.)

a variation of normal thymus, directed US imaging Lymphoma is the most common cause of a medi-
by an experienced pediatric imager is often helpful astinal tumor in childhood.9 Both Hodgkin and
in identifying normal hypoechoic thymic tissue non-Hodgkin lymphoma (especially T-cell lympho-
versus thymic infiltration or other mass (see Figs. blastic leukemia/lymphoma type) commonly affect
1 and 3). the mediastinum.7 In Hodgkin lymphoma, a large
Normal thymic tissue is characterized by its anterior heterogeneous lobulated mediastinal
uniform homogeneous appearance as well as mass involving the thymus and adjacent nodes
lack of mass effect on adjacent structures. In with contiguous neck adenopathy is typical (see
young infants it tends to be quadrilateral in shape Fig. 5). There are frequently areas of necrosis within
and more triangular in older adolescents with the mass. Encasement of adjacent mediastinal
progressive fatty infiltration. Ectopic or atypical vessels and displacement and compression of the
thymus is similar in appearance and enhancement airway in children is common in lymphoma.7,9 CT
and usually directly contiguous with the intratho- or MR imaging with sedation or anesthesia must
racic thymus.3 Thymic cysts are common, espe- be undertaken with great care in patients with
cially in ectopic thymus in the neck. The thymus a potentially compromised airway.
itself is frequently the site of origin or directly infil- Non-Hodgkin lymphoma is more common in
trated by anterior mediastinal neoplastic lesions, younger children and is similar in appearance on
including lymphangioma, histiocytosis, teratoma/ imaging to Hodgkin lymphoma, with more frequent
germ cell tumors (see Fig. 1), lymphoma (see involvement of hilar, subcarinal, posterior medias-
Fig. 5), thymolipoma, and thymic carcinoma tinal, and paracardiac nodes as well as noncontig-
(Fig. 6).6,8 uous tumor in the abdomen occurring more
 Thoracic Neoplasms in Children 639

Box 1
fat and calcification (see Fig. 1). Malignant germ
Mediastinal neoplasms in children cell tumors have a male predilection and may be
difficult to definitively distinguish from benign tera-
1. Anterior (prevascular space) T lesions toma. Suggestive features are a larger, less well-
a. Thymic neoplasm (lymphangioma, Lang- defined mass with extensive central necrosis.
erhans cell histiocytosis [LCH], lymphoma, There is a propensity for local invasion and pleural
thymoma, thymolipoma, and thymic and pericardial effusions as well as distant spread.
carcinoma) Nonseminomatous germ cell tumors are associ-
b. T-cell lymphoma/leukemia ated with hematologic malignancies and Klinefel-
c. Teratoma/germ cell tumor ter syndrome.10
d. Thyroid/parathyroid tumor Thymoma and thymic carcinoma are un-
e. Others (hamartoma/lipoma, lipoblasto-
common in children. Thymomas are slow growing,
ma, and so forth)
potentially malignant neoplasms capable of inva-
2. Middle (vascular space) sive behavior and metastases.8,11 They are asso-
a. Lymphoma ciated with myasthenia gravis (30%–50%), red
b. Other adenopathy—Castleman, metastatic cell aplasia, or hypogammaglobulinemia.8,10 A
(Wilms, osteosarcoma, and neuroblastoma) thymoma is usually a unilateral solid/cystic soft
c. Lymphangioma/hemangioma tissue mass in the anterior mediastinum. Diffuse
d. Cardiac/pericardial neoplasms implants on pleural, mediastinal, and pericardial
3. Posterior (postvascular space) surfaces are seen with invasive thymoma;
however, pleural effusion is uncommon.10 Thymo-
a. Neurogenic tumor with sympathetic
mas may occasionally arise in locations other than
ganglia origin: neuroblastoma, ganglio-
the anterior mediastinum, including neck, else-
neuroblastoma, and ganglioneuroma
b. Neurogenic tumor with peripheral nerve where in the mediastinum, heart, or in the
origin: neurofibroma and schwannoma lung.8,12 Thymic carcinoma is a rare aggressive
c. Neurogenic tumor with paraganglia cell thymic neoplasm. This entity is difficult to differen-
origin: paraganglioma tiate by imaging from invasive thymoma, although
d. Lymphoma pleural and pericardial effusions are more
e. Hemangioma/lymphangioma common in association with local spread of thymic
f. Teratoma carcinoma (see Fig. 6). Distant metastases are
g. Esophageal (leiomyoma/leiomyosarcoma) common, including lymph nodes, lung, bone,
h. Bone lesions (LCH, osteosarcoma, and
brain, and liver.8,10
Ewing sarcoma)
Ectopic thyroid and parathyroid tissue may
i. Lipoma/lipoblastoma
rarely occur in the anterior mediastinum in chil-
dren. Thyroid and parathyroid neoplasms, both
benign and malignant, are likewise uncommon.
commonly.7 Lymphoma is optimally staged and An anterior mediastinal neoplasm may also be
followed using FDG PET-CT (see Fig. 5). simulated by paramediastinal upper lobe atelec-
A mature benign mediastinal teratoma is typi- tasis, upper lobe airless bronchial atresia, or lung
cally a well-defined soft tissue mass containing consolidation.

Fig. 6. A 10-year-old—thymic carcinoma. Two axial CT slices (A, B) viewed on mediastinal windows illustrate
a large inhomogeneous lobulated predominantly anterior medistinal mass (arrows). There is contiguous infiiltra-
tion of the pleura with multiple areas of pleural enhancing tumor (arrowheads) and effusion.
640 Newman

Middle Mediastinum (Fig. 7).7 This is a poorly understood rare lympho-

proliferative disorder that occurs in both nodal
The middle mediastinum encompasses the
and extranodal sites. The hyaline vascular type is
vascular space. Adenopathy, bronchopulmonary
more common and less aggressive than the
foregut lesions (bronchogenic cyst predominantly)
plasma cell form. A characteristic feature is aden-
(see Fig. 2), and vascular masses (aneurysm,
opathy that has increased signal on T2-weighted
vascular ring, and vascular/lymphatic malforma-
MR images and enhances markedly with contrast
tion) predominate in this compartment. Cardiac
(see Fig. 7).
and pericardial lesions overlap with middle medi-
Neoplastic processes tend to produce bulkier
astinal masses. They are discussed later.
and more confluent mass-like adenopathy than
Prominent reactive adenopathy often accom-
infectious causes although the underlying etiology
panies bacterial infection in children, is less
may be difficult to predict. Lymphoma, both Hodg-
common in most viral infections (adenovirus is an
kin and non-Hodgkin types, may occur in the
exception), and is particularly prominent with gran-
middle mediastinum. Intrapulmonary malignancies
ulomatous inflammation, including tuberculosis,
may be associated with hilar and mediastinal ad-
histoplasmosis, and sarcoidosis. Granulomatous
enopathy, often ipsilateral. Although intrapulmo-
adenopathy, especially tuberculosis, as well as
nary metastases are more common, metastatic
atypical mycobacteria is characterized by low
mediastinal adenopathy does occur, especially
attenuation or cavitating nodes. Fibrosing media-
with osteosarcoma and other sarcomas as well
stinitis, also known as fibroinflammatory lesion of
as neuroblastoma and Wilms tumor (Fig. 8).
the mediastinum,13 may be related to histoplas-
mosis infection or idiopathic. It is uncommon in
Posterior Mediastinum
children. The ensuing infiltrative fibrous soft tissue
that encases and obliterates vessels and airways The posterior mediastinum is predominantly the
may be difficult to differentiate from infiltrating domain of the neurogenic lesions, including neuro-
mediastinal neoplasms. genic cyst, anterior meningocele, and neurogenic
Another benign entity that can produce promi- tumors of sympathetic ganglion origin (ganglioneur-
nent mediastinal adenopathy is Castleman disease oma, ganglioneuroblastoma, and neuroblastoma)

Fig. 7. A 16-year-old boy—Castleman disease. Axial T1-weighted postcontrast (A) and (B) sagittal T2-weighted
MR images demonstrate confluent bright T2 signal with prominent enhancement of a nodal mass (arrows) in
the middle and anterior mediastinum displacing airway and vessels.
 Thoracic Neoplasms in Children 641

Fig. 8. A 16-year-old boy. (A) Chest radiograph to follow-up osteogenic sarcoma. Note multiple calcified small
and large pulmonary metastases. (B) CT scan demonstrates multiple intrapulmonary metastatic lesions, some of
which are densely calcified as well as large middle mediastinal masses/nodal tumor metastases. (Reprinted
from Newman B, Effmann EL. Lung masses. In: Slovis T, editor. Caffey’s pediatric diagnostic imaging, vol. 1.
11th edition. Philadelphia: Mosby Elsevier; 2008:1294–323; with permission.)

(Fig. 9), peripheral nerve origin (neurofibroma and perhaps because of earlier diagnosis and lower
schwannoma), or paraganglia cells (paragang- stage at the time of presentation. There is typically
lioma).9 Other lesions may include esophageal a paraspinal posterior mediastinal mass that may
masses (eg, esophageal duplication cyst or leio- infiltrate the posterior soft tissues, can spread to
myoma/leomyosarcoma), vascular abnormalities nodes and vessels, or be large enough to compro-
(aneurysm and vascular/lymphatic malformation) mise the airway. There is often a dumbbell lesion
(Fig. 10), and vertebral/paravertebral lesions extending into multiple intervertebral foramina
(extramedullary hematopoiesis (Fig. 11), lipomato- and the extradural space (see Fig. 9). For this
sis, diskitis, osteomyelitis, LCH, osteosarcoma, reason, MR imaging is considered the best imaging
and Ewing sarcoma). Mimickers of a posterior modality to optimally visualize spinal involvement.6
mediastinal mass include the normal right-sided Other tumors may take on a dumbbell appearance,
paraspinal shadow of the confluence of pulmonary including Ewing sarcoma/primitive neurectodermal
veins and rounded left lower lobe atelectasis tumor (PNET), rhabdomyosarcoma, lipoblastoma,
medial to the pulmonary ligament. and vascular masses.7
Among the neurogenic tumors, neuroblastoma Neurofibromas are usually associated with
predominates, especially in young children. Less- neurofibromatosis type 1; the lesions may be
aggressive forms of the ganglioneuroma neuroblas- multiple and discrete, often at multiple foraminal
toma spectrum are more common in older children levels, or may be large and extend into the middle
and may represent maturation of neuroblastoma. mediastinum, especially as a plexiform neurofi-
The lesions commonly contain speckled calcifica- broma. Extensive infiltration may occur along neu-
tion, are typically bright on T2-weighted imaging, rovascular bundles, including into the lung hila as
and enhance moderately (see Fig. 9). Local extent well as along the pleura. They may be confused
and distant metastases are often well shown on with other more aggressive tumors, such as
MIBG scintigraphic scans (Fig. 12).7,9 lymphoma. Neurofibromas, however, tend to be
Neuroblastoma may present with a paraneoplas- hypodense with modest enhancement postcon-
tic syndrome,9 including opsoclonus myoclonus, trast. Other chest stigmata of neurofibromatosis,
profuse watery diarrhea (vasoactive intestinal poly- such as skin lesions, dural ectasia, scoliosis, and
peptide), and pheochromocytoma-like syndrome ribbon rib deformities, may be helpful.
(catecholamines), prompting a search for an under-
lying tumor, sometimes quite occult. The majority
of neuroblastoma tumors occur in the abdomen CARDIAC NEOPLASMS
in the region of the adrenal glands; these can occa-
sionally spread to the chest contiguously through  Cardiac lesions may be intracavitary
the retrocrural nodes. Only approximately 10% to attached to the endocardium, within the
15% are primarily located in the chest and may myocardium, or pericardial (Box 2).
coexist with one or more tumors elsewhere. Neuro-  Cardiac neoplasms are rare in children;
blastoma in the chest may have a better prognosis, most are benign.
642 Newman

Fig. 9. Newborn infant—neuroblastoma. Frontal (A) and lateral (B) chest radiographs. There is a large mass in the
right chest posteriorly (arrows). Thinning and splaying of the right posterior upper ribs (arrowhead) suggest
a posterior mediastinal location, confirmed on the lateral view. Coronal T1-weighted postcontrast MR images
(C, D) demonstrate a large moderately enhancing right posterior mass with vertebral foraminal extension at mu-
tiple levels (arrows). The punctate bright focus within the mass (arrowhead) is probably calcification.
 Thoracic Neoplasms in Children 643

The most common intracardiac neoplasms in

children are rhabdomyoma, myxoma and fibroma
(Figs. 13–16).14 Other benign entities include
hamartoma, lipoma, fibroelastoma, hemangioma,
fibrous histiocytoma, and inflammatory pseudotu-
mor. Asymmetric septal or focal myocardial hyper-
trophy in hypertrophic cardiomyopathy (idiopathic,
familial, or associated with maternal diabetes) may
simulate an intracardiac mass. MR imaging
features that serve to identify hypertrophied
myocardium include similar signal, contractility,
and enhancement characteristics as normal
Fig. 10. Posterior mediastinal lymphangioma. Axial
contrasted CT shows fluid/fluid levels (arrow) in the myocardium on multiple sequences.
mass and no bone or spine involvement. Rhabdomyomas are hamartomas of the heart
that have a strong association with tuberous
sclerosis.15,16 The tumors may be large or small,
typically multiple rather than single.17 The pres-
 Malignant cardiac or pericardial tumors are ence of multiple myocardial masses in a fetus or
more likely to be metastatic than primary. newborn infant is essentially diagnostic of
 Many childhood cardiac tumors are associ- tuberous sclerosis. These are rounded, well-
ated with specific syndromes or systemic defined intramyocardial masses on US. They are
diseases. usually somewhat hyperintense on proton density
 Non-neoplastic entities may simulate and MR images and enhance less than normal myocar-
be difficult to distinguish from a cardiac dium on postcontrast MR images as well as CT
neoplasm. images (see Fig. 13). They may disrupt the
conduction system of the heart or distort or
Cardiac tumors are included in the differential
obstruct the cavity or valves.17 The neonatal rhab-
considerations for a middle mediastinal mass.
domyomas associated with tuberous sclerosis
Cardiac tumors may manifest on chest radio-
tend to decrease in size over time usually
graphs as cardiomegaly, abnormal cardiac con-
completely regressing by age 6 years with no
tour, or pulmonary edema.14 Other entities may
malignant potential.14
simulate a cardiac mass, including normal vari-
The second most common cardiac tumor in
ants, cardiomyopathy, other mediastinal masses,
childhood is a cardiac fibroma. This tumor is
postsurgical changes, myocardial or arterial aneu-
usually solitary, often large, and although benign
rysm, venous varix, abscess, diaphragmatic her-
may cause significant cardiac dysfunction. It is
nia, and pericardial abnormalities.
typified by lower signal than normal myocardium
especially on T2-weighted images with character-
istic delayed contrast enhancement (see Fig. 14).
Calcifications are common within the tumor, better
appreciated on CT than MR imaging.14 Cardiac
fibroma has an association with Gorlin syndrome
(basal cell nevi and multiple other tumors) and
Beckwith-Wiedemann syndrome (better known
for association with macroglossia, ompahlocele,
hemihypertrophy, and abdominal tumors, espe-
cially Wilms tumor).15,16
Cardiac myxoma, usually in the left atrium, is the
most common cardiac tumor in adults but
uncommon in children. Cardiac myxomas in child-
hood have a syndromic association with the
Carney complex (myxomas, endocrine lesions,
and pigmented skin lesions) (see Figs. 15 and
Fig. 11. Extramedullary hematopoiesis. A 22 year old
with thallassemia post–multiple blood transfusions. 16).15,16,18 The cardiac myxomas may be multiple
Axial T2-weighted MR scan. Note the T2 dark signal and widely distributed in both atria and ventricles
in the liver (L) due to iron overload and T2 bright bilat- (see Fig. 16). On MR imaging, atrial myxomas
eral posterior paraspinal soft tissue masses (arrows) may have foci of low signal due to hemorrhage
consistent with extramedullary hematopoiesis. or calcification, most evident on gradient-echo
644 Newman

Fig. 12. Metastatic neuroblastoma in a 5-year-old boy. (A) Coronal CT reformat with diffuse lytic and sclerotic
bony metastases, including spine, skull, scapulae, ribs, and pelvis. (B) MIBG scan—anterior view with diffuse
and patchy bony uptake of radiotracer. There is physiologic uptake in salivary glands, myocardium, liver, and
bladder.

images (see Fig. 16). They are usually attached relative to normal myocardium, as well as post-
to the atrial septum or other endocardium by contrast images, where tumors usually enhance
a stalk and may be mobile and cause inflow or and thrombus (unless chronic and organized)
outflow obstruction of the ventricles or valvular does not (Fig. 18).
dysfunction. Pericardial masses include pericardial cyst, tera-
Primary malignant cardiac tumors are rare toma, hemangioma, and lymphangioma. A range of
and include lymphoma, germ cell tumor, rhabdo- other lesions may simulate a pericardial neoplasm,
myosarcoma, fibrosarcoma, angiosarcoma, and including irregular pericardial thickening/calcifica-
leiomyosarcoma.14 Secondary cardiac involve- tion, pericardial hemorrhagic effusion/hematoma
ment is more common than primary cardiac malig- (see Fig. 18), intrapericardial extension of a dia-
nancy. This is usually associated with venous phragmatic hernia, myocardial diverticulum, and
tumor thrombus extension or embolization, most partial absence of the pericardium.19 CT or MR
likely in Wilms tumor, hepatoblastoma, and neuro- imaging is often helpful in defining the location
blastoma (Fig. 17). Other tumors, such as and likely nature of the mass and may occasionally
osteosarcoma, Ewing sarcoma, and leukemia/ be able to suggest a specific diagnosis (eg, the
lymphoma, can involve the heart by direct contig- presence of both fat and calcification strongly
uous spread. Hematogenous metastases to the suggests a teratoma). Primary malignant tumors
heart are rare. and hematogenous metastases to the pericardium
Intracardiac thrombus may be difficult to differ- are rare.19 More likely is contiguous spread of
entiate from a tumor. Useful sequences include a mediastinal mass such as lymphoma (Fig. 19)
gradient-echo and T2 images, where thrombus is or thymoma to involve the pericardium; these
typically low and most tumors high in signal may result in constrictive pericarditis.
 Thoracic Neoplasms in Children 645

Box 2 also masquerade as pulmonary neoplasms, in-

Cardiac and pericardial neoplasms in children cluding round pneumonia, rounded atelectasis,
lung abscess, fungal or granulomatous nodules,
Cardiac loculated pleural fluid, hematoma, and vasculitic
1. Benign lesions.20,21
Bronchopulmonary malformations are common
a. Rhabdomyoma
congenital non-neoplastic lung masses that need
b. Myxoma
c. Fibroma to be differentiated from other pulmonary tumors.
d. Hemangioma These are frequently found prenatally as large
e. Hamartoma solid or cystic or combined T2 bright lesions in ute-
f. Lipoma ro. Often they are largest in the second trimester
g. Fibroelastoma and progressively decrease in size and conspi-
h. Fibrous histiocytoma cuity later in pregnancy. They may be small or
i. Inflammatory pseudotumor not visible on postnatal chest radiographs but
2. Malignant are usually apparent on CT imaging.22 There is
considerable overlap of the lesions, with features
a. Primary
of multiple entities common rather than excep-
i. Lymphoma
ii. Germ cell tumor tional. The bronchopulomonary malformations
iii. Rhabdomyosarcoma, fibrosarcoma, ang- are thought to represent the spectrum of under-
iosarcoma, and leiomyosarcoma lying airway obstruction in utero with consequent
lung malformation.23 The histologic nature of the
b. Metastatic
lesion may depend on the timing and severity of
i. Contiguous—ostesarcoma, Ewing sar-
coma, rhabdomyosarcoma, and leu- this obstruction malformation sequence.23 In
kemia/lymphoma many cases, careful pathologic evaluation has re-
ii. Venous thrombus/hematogenous— vealed bronchial atresia to be the underlying
Wilms, neuroblastoma, hepatoblasto- obstructive lesion.22,24 Typical findings that may
ma, and rhabdomyosarcoma be identified both prenatally and postnatally
Pericardial include lobar or segmental hyperinflation (congen-
ital lobar/segmental overinflation); central bron-
1. Benign chial mucoid impaction (bronchial atresia); single
a. Teratoma
(bronchogenic cyst) or multiple cysts (congenital
b. Hemangioma/lymphangioma
pulmonary airway malformation [CPAM]), and
2. Malignant systemic arterial supply to the lung (pulmonary
a. Germ cell tumor sequestration).22 Other prenatal pulmonary masses
b. Contiguous/metastatic spread (eg, lym- occur but are uncommon (Fig. 21).
phoma and thymoma)
Benign lung neoplasms encountered in chil-
dren include hamartoma, chondroma, vascular/
lymphatic lesions, LCH, respiratory papillomato-
INTRAPULMONARY NEOPLASMS sis, granular cell tumor, neurofibroma, schwanno-
 Pulmonary masses are less common than ma, teratoma, and benign lymphoproliferative
mediastinal masses in children (Box 3).20 lesions.25
 The majority are non-neoplastic congenital Hamartomas and chondromas are uncommon in
or inflammatory lesions. children. The presence of typical popcorn calcifica-
 Neoplastic intrapulmonary lesions are more tion in a lesion may help in suggesting the diagnosis
likely malignant than benign. of a hamartoma; however, many other benign and
 Malignant lesions are more likely metastatic malignant neoplasms, including metastases, can
than primary. calcify.20 Similarly, non-neoplastic lesions, such as
 It may be difficult to determine the origin of infections or infarcts, may also calcify. Pulmonary
large pleural, mediastinal, or chest wall chondroma is uncommon, tends to be densely
tumors that spread contiguously to involve calcified, and is difficult to differentiate from hamar-
the lung (Fig. 20). toma both on imaging and pathology. The presence
of pulmonary chondroma, especially multiple
Several extrinsic densities may simulate an intra- lesions in a young woman, should raise suspicion
pulmonary mass. These include skin lesions, for Carney triad. This triad describes the synchro-
breast buds, rib and other chest wall abnormali- nous or metachronous occurrence of pulmonary
ties, diaphragmatic hernia or eventration, and chondroma, gastric stromal tumor, and extradrenal
hair braids.21 Rounded intrapulmonary lesions can paraganglioma.25,26 Additional tumors are also
646 Newman

Fig. 13. Cardiac rhabdomyoma. Tuberous sclerosis, newborn infant. (A) Chest radiograph, there is an unusual
cardiac contour with bulging of the left superior heart border (arrow). (B) Axial postcontrast CT. (C) Coronal
T2-weighted MR imaging. There is a large bulging myocardial mass (arrows), moderately T2 bright with slight
enhancement with contrast. (Reprinted from Towbin AJ, Newman B. Cardiac involvement by systemic diseases.
In: Slovis T, editor. Caffey’s pediatric diagnostic imaging, vol. 1. 11th edition. Philadelphia: Mosby Elsevier;
2008. p. 1687–706; with permission.)

associated, including adrenal adenoma and esoph- compared with conventional angiography. There
ageal leiomyoma.27 are frequently soft tissue and visceral AVM’s
Vascular lesions include non-neoplastic entities present elsewhere in the body.
that can mimic tumor, encompassing arterial Diffuse arteriocapillary dilatation and ultimately
aneurysm, venous varix, arteriovenous malforma- diffuse AVMs are also encountered in hepatopul-
tion (AVM), and vasculitic lesions, such as Wege- monary and portopulmonary syndrome and after
ner granulomatosis and Takayasu disease. AVMs superior cavopulmonary anastomosis with exclu-
are the most common intrapulmonary vascular sion of hepatic venous blood from the lungs;
lesion; 70% of these are associated with heredi- the cause of this is poorly understood. Diffuse
tary hemorrhagic telangiectasia, although the pulmonary arteriovenous shunting in these condi-
lesions are usually not clinically manifest until later tions results in hypoxemia and pulmonary
childhood or adulthood.20 There may be 1 or more hypertension.28
large or small AVMs or diffuse tiny AVMs predom- Benign vascular neoplasms include intrapulmo-
inantly in the lower lobes. Location, size, and nary lymphangioma (Fig. 22)29 and hemangioma.
number can often be determined noninvasively Intrapulmonary lymphangioma can be extensive,
with CTA, although diffuse microscopic lesions involving lung, pleura, and mediastinum (see
may be more difficult to appreciate on CTA Fig. 22); as such, it may be difficult to distinguish
 Thoracic Neoplasms in Children 647

Fig. 14. Cardiac fibroma in a 3-year-old girl. Axial MR images. (A) Double inversion recovery (T1), (B) gradient-
echo T2, and (C) delayed T1 postcontrast show a large mildly heterogeneous intracardiac mass (arrows) that is
dark on both T1 and especially T2 sequences with moderate contrast enhancement on delayed (not early) post-
contrast images (C). Despite the large size of the mass with some impingement on both ventricular cavities, there
was no obstruction or cardiac dysfunction.

from a diffusely infiltrating malignant tumor, part of a more generalized diffuse entity that may
such as pleuropulmonary blastoma (PPB).20 involve multiple other organs, including liver, soft
PPB tends, however, to be more heterogeneous tissues, mediastinum, bone, spleen, intestine, and
with some solid nodular components. Pulmonary kidneys.10,20,30 Lymphangioleiomyomatosis with
lymphangiomatosis or hemangiomatosis may be diffuse thin-walled cysts in the lungs is most

Fig. 15. Atrial myxoma, Carney complex in a 12-year-old boy. (A) Cardiac US 4-chamber view with a well defined
hyperechoic mass in the left atrium (arrow). (B) Testicular US with multiple small calcified Sertoli cell tumors.
(Reprinted from Towbin AJ, Newman B. Cardiac involvement by systemic diseases. In: Slovis T, editor. Caffey’s pedi-
atric diagnostic imaging, vol. 1. 11th edition. Philadelphia: Mosby Elsevier; 2008. p. 1687–706; with permission.)
648 Newman

Fig. 16. Multiple atrial myxomas, Carney complex. A 17-year-old boy, status post orchiectomy for Sertoli-Leydig
cell tumor. (A) Double inversion recovery (T1) 4-chamber and (B) Triple inversion recovery (T2) right ventricular
2-chamber images showing multiple T2 bright intracavitary myxomas including left atrium adjacent to the septum
(arrowhead) and a large lobulated mass in the right ventricle (arrows). Note the dark rim around the proximal
portion of the right ventricular mass (broken arrow), likely due to hemorrhage and hemosiderin deposition.

commonly seen in young adult women in associa- The lesions of respiratory papillomatosis are
tion with tuberous sclerosis and occasionally a neoplastic proliferation caused by the papilloma-
encountered in older adolescents. virus acquired in the maternal birth canal during
The lung is affected in approximately 50% of delivery. The disease is usually confined to the
children with systemic LCH; approximately 11% larynx and upper tracheal airway with spread to
have lung involvement at the time of initial dia- the lung in less than 1% of cases (Fig. 24).20,32 In-
gnosis.20,31 There may be focal or diffuse lung trapulmonary spread is thought possibly related to
involvement (upper lobe predominance) with solid surgical/laser spillage during treatment of the
(Fig. 23) and cavitating nodules that form small upper airway lesions. Proliferation in the lung
and large cysts. A cyst that is peripheral in location produces solid and cavitating cystic pulmonary
may break through to the pleural space, presenting masses (see Fig. 24). The ultimate prognosis is
as an acute pneumothorax (approximately 10%). poor, treatment is difficult, recurrence common

Fig. 17. A 4-year-old girl—hepatoblastoma with venous invasion and tumor thrombus. Axial postcontrast CT
images. (A) Upper liver—there is a large heterogeneous right hepatic mass with expanding enhancing tumor
thrombus in the right hepatic vein and inferior vena cava (arrows) extending into the (B) right atrium (arrow).
(Reprinted from Towbin AJ, Newman B. Cardiac Involvement by systemic diseases. In: Slovis T, editor. Caffey’s
pediatric diagnostic imaging, vol. 1. 11th edition. Philadelphia: Mosby Elsevier; 2008. p. 1687–706; with
permission.)
 Thoracic Neoplasms in Children 649

Fig. 18. Pericardial effusion with organized thrombus simulating a pericardial mass. A 1-day-old girl. (A) US sub-
costal view shows large ascites and pericardial effusion with an irregular inhomogeneously echogenic mass that
appears attached to the visceral pericardium (arrows). (B) Right ventricle 3-chamber fast imaging employing
steady state (FIESTA) single shot fast field echo image. (C) Delayed postcontrast image show ascites, large pericar-
dial effusion, and an inhomogeneous pericardial mass (arrows) that appears to have both solid and cystic compo-
nents with moderate patchy delayed enhancement peripherally. There is a dark signal rim and some central dark
signal (B) thought to represent hemorrhage. Pericardial teratoma was thought the most likely diagnosis based on
imaging. Only organized thrombus was found at surgery and pathologic examination.

Fig. 19. A 7 year old with Hodgkin lymphoma. Axial contrasted CT slices. There is bulky tumor in the anterior and
middle mediastinum with marked compression and displacement of the (A) innominate vein (arrow) and airway
(arrowhead) and inferior extension of the tumor to involve the (B) pericardium (arrows). (Reprinted from Towbin
AJ, Newman B. Cardiac involvement by systemic diseases. In: Slovis T, editor. Caffey’s pediatric diagnostic imaging,
vol. 1. 11th edition. Philadelphia: Mosby Elsevier; 2008. p. 1687–706; with permission.)
650 Newman

Box 3 lesion is thought to be benign in behavior but is

Lung neoplasms in children typically large in size and may be difficult to resect
(see Fig. 21).25 Similar to other large chest masses
Benign in utero, there is an increased risk of development
1. Inflammatory myofibroblastic tumor of hydrops fetalis with high fetal mortality,25 so
2. Hamartoma there should be careful follow-up in utero with
3. Lymphangioma/hemangioma US and/or MR imaging.
4. Chondroma Lymphomatoid granulomatosis is an angiocen-
5. Papillomatosis tric destructive lesion with the lung as the primary
6. LCH site of occurrence. There is a basal predominance
7. Neurogenic tumors
of nodular and less well-defined larger confluent
8. Lymphoproliferative lesions; post-transplant
lesions that tend to cavitate and may sometimes
lymphoproliferative disorder (PTLD), smooth
muscle cell proliferation, and lymphomatoid resemble confluent pneumonia or empyema. Lym-
granulomatosis phomatoid granulomatosis and PTLD are similar
9. Congenital peribronchial myofibroblastic entities, both being related to B-cell lymphoprolif-
tumor eration associated with Epstein-Barr virus in
immunocompromised individuals. There is a spec-
Malignant
trum of lymphoproliferation from benign to frank
1. Amine precursor uptake and decarboxyl- B-cell lymphoma.20
ation (APUD) carcinoid tumors PTLD is more common in pediatric than adult
2. Pleuropulmonary blastoma
transplant patients, with the highest frequency in
3. Bronchogenic carcinoma
4. Sarcomas lung or heart-lung transplants.20 PTLD has a predi-
5. Lymphoma lection for the allograft site (with the exception of
6. Metastatic tumor: osteosarcoma, Ewing the heart) in addition to a variety of other locations,
sarcoma, rhabdomyosarcoma, Wilms tumor, including tonsils, lymphoid tissue in the neck, lung,
germ cell tumor, lymphoma, and so forth gastrointestinal tract, and central nervous sys-
tem.20,37 Lesions in the lung consist of single or
multiple small or large nodules or areas of consol-
idation simulating pneumonia (Fig. 26).37 Larger
with high morbidity, and there is approximately lesions tend to cavitate (see Fig. 26); associated
10% long-term risk of conversion of the lesions hilar or mediastinal adenopathy may be present.
to squamous cell carcinoma.20,25,32 Diagnosis of PTLD is made by biopsy of the most
Benign reactive lymphoproliferative lesions in easily accessible site, usually not the lung.
the lung consist of a variety of entities, most Decreasing the dose of immunosuppressive drugs
uncommon. The spectrum includes inflammatory is usually effective treatment for PTLD but it may be
myofibroblastic tumor; mucosal-associated lym- difficult to find a balance between PTLD and organ
phoproliferation, also known as pseudolympho- rejection.37 Occasionally, especially with long-
ma; bronchus-associated lymphoproliferation, standing lesions or delayed diagnosis, PTLD may
subdivided into lymphoid interstitial pneumonitis become a monoclonal lymphoma requiring aggres-
and follicular bronchiolitis; lymphomatoid granulo- sive chemotherapy.
matosis; and PTLD. Spindle/smooth muscle cell tumors are a less
Inflammatory myofibroblastic tumor (also known common but similar neoplastic cell proliferation
as plasma cell granuloma or inflammatory pseudo- to PTLD. Like PTLD, smooth muscle cell tumors
tumor) is the most common benign intrapulmonary are Epstein-Barr virus driven in immunocompro-
neoplastic lesion in children.33–35 It is still contro- mised individuals (HIV and post-transplant), but
versial as to whether this entity represents an atyp- are histologically distinct from PTLD with a less
ical reaction to prior infection versus a low-grade favorable prognosis.20,38 Spindle/smooth muscle
neoplasm.34 There are single or multiple small to cell tumors are usually discrete masses most often
large poorly enhancing rounded masses in the in the chest or abdomen. An endobronchial loca-
lung (Fig. 25) with calcification present in approx- tion of this lesion may also occur.
imately 20% of lesions. Support for the possible Pulmonary lymphoma is most commonly
neoplastic nature of this lesion is occasional associated with mediastinal tumor involvement
aggressive and invasive behavior.33,36 but primary pulmonary lymphoma occurs on occa-
An uncommon solid tumor that may be discov- sion; 12% of children with Hodgkin disease and
ered prenatally or in newborn infants is the 10% of non Hodgkin-lymphoma have lung involve-
congenital peribronchial myofibroblastic tumor ment, most at initial diagnosis.20 The appearance
(also known as congenital fibrosarcoma).25 This of pulmonary lymphoma can be widely varied
 Thoracic Neoplasms in Children 651

Fig. 20. Pleural synovial sarcoma in an 18-year-old man. (A) Chest radiograph with a large left lower chest mass.
The origin of the mass (intrapulmonary versus chest wall) is uncertain. (B) Contrasted chest CT (mediastinal
window) shows a heterogeneous enhancing mass, probably pleural in origin, projecting into the left lower
lung with adjacent atelectasis. There is subtle involvement of the left posterior chest wall (arrow). (C) Postoper-
ative coronal CT reformat (bone window). There has been surgical removal of the mass, left lower lobe and
several ribs with graft placement. There is resultant deformity of the chest wall. No residual or recurrent mass
was seen.

similar to benign lymphoproliferative disorders. lymphoma, and occasionally Wilms tumor).20

The most common appearance is the presence Approximately one-third of lung nodules in chil-
of pulmonary nodules that may cavitate (see dren with a known underlying malignancy are non-
Fig. 5). Less common are alveolar infiltration metastatic. On biopsy, alternative pathologic
(Fig. 27), lymphangitic tumor spread, and endo- diagnoses include intrapulmonary lymph nodes,
bronchial tumor spread.20 infection, scar, drug reaction, myofibroblastic
Pulmonary metastases are considerably more tumor, lipid nodules, vasculitis, and occasionally
common than primary pulmonary malignant le- a second malignancy (see Fig. 25).20,21,40 Lesions
sions.33,39 The most common hematogenous larger than 5 mm are more likely to be meta-
metastases are from osteosarcoma, Wilms tumor, stases.20 Accurate and early recognition of
and other sarcomas (see Fig. 8).20 Pulmonary possible pulmonary metastases is important be-
metastases are most commonly located in the cause long-term survival in osteogenic tumors as
peripheral and basilar lungs. They are typically well as other sarcomas is significantly improved
multiple, occasionally single, well-defined rounded after metastectomy.33,41,42
pulmonary nodules or masses that can become Pulmonary malignancy may also involve the lung
large. Some tend to calcify (osteogenic sarcoma) contiguously from adjacent mediastinum, hilar, or
(see Fig. 8) or cavitate (Fig. 28) (sarcomas, pleural tumor. Spread via lymphatic channels
652 Newman

Fig. 21. Congenital peribronchial myofibroblastic tumor. (A) Prenatal T2-weighted sagittal MR image at 32
weeks’ gestation with a large low signal mass in the left lung (arrows). This has a different appearance from
that of the more common bronchopulmonary malformations, which are bright on T2. The correct diagnosis
was suggested on the basis of the prenatal appearance of the mass. (B) Axial postcontrast newborn CT confirms
the large low attenuation poorly enhancing mass in the left lung (arrows).

(lymphangitic metastases, most common in rhab- for an additional 25% each20; 80% of the APUD
domyosarcoma, neuroblastoma, and lymphoma) tumors in children are low-grade carcinoid
or along the airway is an occasional method of in- tumors20,33 (Fig. 29); less common tumors include
trapulmonary tumoral involvement. mucoepidermoid tumor, cylindroma, and mucous
Primary pulmonary malignant neoplasms are gland adenoma. These are usually endobronchial
rare in children. The most common of these are lesions, most commonly in the lobar bronchi, and
the spectrum of APUD neurendocrine tumors; may spread contiguously to involve adjacent no-
these are responsible for approximately 45% of des or lung.20,43 The lung distal to the lesion shows
primary pediatric lung tumors. Bronchogenic findings of partial (air trapping) or more commonly
carcinomas and mesenchymal tumors account complete airway obstruction (atelectasis/recurrent
infection of the affected lobe or segment). Contrast
CT with particular attention to 2-D and 3-D and
virtual bronchoscopic reconstructions is helpful
in identifying these lesions (see Fig. 29). Carci-
noid tumors infrequently present with Cushing
syndrome or carcinoid syndrome (generally
implies metastases) and are occasionally encoun-
tered as part of a multiple endocrine neoplasia
syndrome.33
Bronchogenic carcinomas are unusual in child-
hood. Approximately half of these are bronchoal-
veoalar carcinoma (BAC) with a smaller number
of squamous cell carcinomas.20 The squamous
cell tumors are usually large at the time of diag-
nosis and aggressive, with a poor prognosis
(Fig. 30). Although the incidence is small (approx-
imately 1%), BAC seems to arise in some cases in
or adjacent to an underlying bronchopulmonary
malformation, principally large cyst CPAM.44,45
Fig. 22. Pulmonary lymphangioma. A 2 year old with
whiteout of the right lung on chest radiograph. CT This relationship can apparently persist even
shows atelectatic right lower lung (arrow) surrounded when the underlying lesion has been previously
by a large low attenuation infiltrating intrapulmo- surgically excised. This has been variably sug-
nary mass extending into the anterior mediastinum gested to be due to incomplete surgical removal
with leftward shift of midline structures. versus an oncogenic potential related to the
 Thoracic Neoplasms in Children 653

Fig. 23. Langerhans cell histiocytosis in a 3-month-old boy. (A) Chest CT lung window - there are multiple well-
defined pulmonary nodules (arrows). (B) Contrasted CT, mediastinal window at the level of the aortic arch. The
thymus is enlarged with heterogeneous low attenuation foci. These subsequently calcified after treatment (not
shown). Note also bilateral axillary adenopathy.

presence of abnormal mucigenic cells in large cyst type 3 is solid (see Fig. 31).25,47,48 Type 1 has an
CPAM.45,46 BAC is usually confined to a segment 82% 5-year survival with only 42% in type 3, with
or lobe, is not aggressive in its spread, and rarely both aggressive local spread and recurrence as
is metastatic. Recognition and differentiation well as distant metastases.20,44,47,48 Lesions may
from infection may be difficult. consist of a small nodule or single cyst or a huge
Mesenchymal tumors in children are mostly cystic or solid mass that can involve lung paren-
sarcomas, now recognized as predominantly chyma, pleura, and mediastinum.20,48
PPB (Fig. 31) with occasional other sarcomas, PPB has a hereditary tumor predisposition with
such as rhabdomyosarcoma, leiomyosarcoma, an association with cystic nephroma in particular
synovial cell sarcoma, or liposarcoma.25 as well as other tumors including rhabdomyosar-
PPB is a dysontogenetic anlage of Wilms tumor, coma, medulloblastoma, thyroid, Hodgkin lymp-
neuroblastoma, and hepatoblastoma.20 PPB homa, leukemia, and germ cell tumors.20,25,47,48
consists of malignant blastema with frequent PPB is unusual prenatal but has been described
mesenchymal sarcomatous components, espe- as early as 19 to 21 weeks, progressively
cially rhabdosarcoma and chondrosarcoma25; increasing in size with advancing gestation,
90% are identified in children less than 6 years different from the typical pattern of bronchopulmo-
old with the cystic form almost invariably in chil- nary malformations (BPMs).44 PPB lesions are
dren less than 3 years of age.47 There are 3 types frequently multiple or bilateral (49%), occurring
representing a progression from cystic to solid both synchronously or metachronously.44 There-
along with increasing age and tumor aggression: fore, surgical removal does not obviate ongoing
type 1 is cystic, type 2 is cystic and solid, and surveillance. A DICER 1 gene mutation on

Fig. 24. Respiratory papillomatosis in a 16-year-old boy. History of asthma since infancy. (A) Initial CT shows
a lobulated intratracheal soft tissue mass (arrow). These were present at multiple levels. No pulmonary abnor-
mality was appreciated. (B) Eight months after surgical extirpation of the tracheal lesions, there are multiple
small solid and cavitating intrapulmonary nodules (arrows) consistent with intrapulmonary spread of the
papillomatosis.
654 Newman

peripheral lesion), known familial tumor predispo-

sition, or family history of childhood cancer.20,48
Malignant intrapulmonary vascular neoplasms,
such as hemangiopericytoma and angiosarcoma,
are uncommon in children.20 Epithelioid heman-
gioendothelioma (also known as intravascular
bronchoalveolar tumor) is a low-grade rare intra-
pulmonary vascular malignancy, more common
in young women with findings of single or multiple
(including bilateral) pulmonary nodules and occa-
sionally hemorrhagic pleural effusion (a poor prog-
nostic feature).51 This tumor is rarely diagnosed
before biopsy and more commonly occurs in liver
Fig. 25. A 5 year old. Inflammatory myofibroblastic rather than lung.
tumors. Several new lung nodules (arrow) were found
on this routine follow-up CT for neuroblastoma. They MASSES AFFECTING THE INTRATHORACIC
were removed surgically because of concern for
AIRWAY
metastases.
 Masses obstructing the airway may be
intrinsic (intraluminal or intramural) or
chromosome 14 in airway epithelial cells has extrinsic.
recently been identified in familial cases of PPB.49  The airway may be affected at any level from
Although not universally accepted, current path- the trachea to smaller intrapulmonary bronchi.
ologic literature suggests that PPB arises de novo  Incomplete intrathoracic airway obstruction
and is not a complication of congenital broncho- produces air trapping of the affected distal
pulmonary malformations and that prior reports lung, bilateral in the case of tracheal obstruc-
linking the two originally misidentified the lesion tion, and unilateral, lobar, or segmental when
and missed the correct diagnosis of PPB.48,50 more distal airways are involved.
Cystic PPB is difficult to separate from other cystic  Complete airway obstruction results in
lung lesions, especially the large cyst CPAM distal atelectasis of the affected lung,
(type 1) on imaging and many cases subsequently frequently with superimposed infection.
identified as PPB are initially diagnosed as CPAM
lesions.20,48,50 Correct pathologic diagnosis also Masses affecting the airway arise in any thoracic
may be subtle.25 Features that suggest PPB rather region, including mediastinal, intrapulmonary,
than CPAM include atypical in utero growth and occasionally chest wall. Ectopic thymic or
pattern (BPMs present and are often largest in thyroid tissue is a rare cause of an intratracheal
second trimester; PPB unlikely to be present early mass in children.52
and progressively enlarges), presence or develop- A foreign body, granulation tissue, or infectious/
ment of solid nodules, multiple lesions, pneumo- inflammatory debris in the airway may sometimes
thorax (uncommon in CPAM; PPB a more be difficult to distinguish from a neoplasm. An

Fig. 26. A 4-year-old boy post–liver transplant with post transplant lymphoproliferative disease (PTLD). (A, B)
Contrasted axial CT scan, mediastinal window (A) and lung window (B) with a large cavitating lesion in the
left upper lobe (arrowheads). There are also multiple smaller ill-defined ground glass nodules in both lungs
(arrows).
 Thoracic Neoplasms in Children 655

Fig. 27. Hodgkin lymphoma in a 15 year old. (A) Chest radiograph. There are large bilateral confluent alveolar
opacities as well as some scattered nodules. (B) Contrasted CT scan (mediastinal window) and (C) (lung window).
Note prominent hilar nodes and contiguous adjacent consolidated lungs as well as several scattered nodules. Air
bronchograms and intact vessels within the mass are a characteristic pulmonary appearance of lymphoma. (A and
C, Reprinted from Newman B, Effmann EL. Lung masses. In: Slovis T, editor. Caffey’s pediatric diagnostic imaging,
vol. 1. 11th edition. Philadelphia: Mosby Elsevier; 2008:1294–323; with permission.)

aggressive intrinsic airway neoplasm is more likely as well as PTLD, leiomyoma, granular cell tumor, ha-
to produce complete airway obstruction with distal martoma, and inflammatory pseudotumor.20,35,53
atelectasis/infection, whereas acute foreign bodies The APUD tumors, especially carcinoid tumor (see
are more likely associated with partial airway Fig. 29), are the most common intrinsic airway
obstruction and air trapping. malignancy; bronchogenic carcinomas and primary
Benign intrinsic airway lesions include heman- bronchial fibrosarcoma are rare in childhood (see
gioma and respiratory papillomatosis (see Fig. 24) Fig. 30).25,43 Extrinsic lesions that commonly cause
airway compression include benign lesions, such as
congenital cysts and cystic hygroma. These may
present with sudden airway symptoms as a result
of acute hemorrhage and enlargement. Extrinsic
malignant neoplasms that tend to compress the
airway include lymphoma/leukemia (see Fig. 19),
other causes of adenopathy (see Fig. 7), germ cell
tumors, and occasionally neurogenic tumors. Any
large aggressive chest neoplasm can displace,
distort, compress, or even infiltrate the airway.
Specific lesions have been discussed earlier.

PLEURAL, DIAPHRAGM, AND CHEST

WALL MASSES

Fig. 28. Cavitating pulmonary metastases in a 14-year-  Pleural masses are often due to direct
old girl with chordoma. The CT (lung window) image contiguous spread from intrapulmonary,
shows multiple well-defined small pulmonary nodules mediastinal or chest wall lesions (Box 4).
both solid (arrows), with central cavitation and cystic  Chest wall masses are relatively uncommon
(white arrow). in children.
656 Newman

Fig. 29. Carcinoid tumor lingula bronchus. A 6-year-old girl with history of recurrent pneumonia. (A) Axial CT slice
(lung window). There is focal bronchiectasis in the inferior lingula (arrow). (B) Curved reformat of the lingula
bronchus shows narrowing of the bronchus with small intraluminal enhancing soft tissue (arrow) as well as ex-
traluminal mass with distal bronchiectasis. Both intraluminal and extraluminal masses were carcinoid tumor.

 Most asymptomatic chest wall lumps are Contiguous involvement of the pleura from adja-
due to benign anatomic variations of the cent intrapulmonary, mediastinal, or chest wall
chest wall. lesions is common.54 Inflammatory pleural thick-
 Many benign lesions can appear infiltrative ening or loculated fluid may be difficult to differen-
whereas malignant lesions may appear tiate from pleural tumor although the latter tends
well circumscribed. to have a more irregular lumpy configuration.
 Metastatic tumor is more common than Pleural effusion is associated with a large number
primary chest wall malignancy. of infectious, inflammatory, and neoplastic causes.
 MR imaging is typically the best method for Thoracic tumors, including pleuropulmonary blas-
detailed imaging evaluation of chest wall toma, teratoma, leukemia/lymphoma, and PNET,
neoplasms. can clinically mimic infectious empyema with

Fig. 30. Squamous cell carcinoma in a 7-year-old girl. (A) Chest radiograph. Large right mass obscuring right lung
and extending across the midline with contralateral mediastinal shift. (B) Coronal MR image, T1 postcontrast.
There is a large infiltrating intrapulmonary mass encasing the right lower bronchi and extending into the medi-
astinum. Moderate right pleural effusion is present. (Reprinted from Newman B, Effmann EL. Lung masses. In:
Slovis T, editor. Caffey’s pediatric diagnostic imaging, vol. 1. 11th edition. Philadelphia: Mosby Elsevier;
2008:1294–323; with permission.)
 Thoracic Neoplasms in Children 657

Fig. 31. A 2 year old—pleuropulmonary blastoma. (A) Chest radiograph. There is almost complete opacification
of the left hemithorax with rightward cardiomedistinal shift. (B) CT axial postcontrast image. There is a heteroge-
neous mass replacing the left lung and extending to the posterior mediastinum with contralateral midline shift.
There are bilateral moderate pleural effusions. (Reprinted from Newman B, Effmann EL. Lung masses. In: Slovis T,
editor. Caffey’s pediatric diagnostic imaging, vol. 1. 11th edition. Philadelphia: Mosby Elsevier; 2008:1294–323;
with permission.)

or without an actual pleural effusion (see is helpful for defining the location, extent, and
Fig. 31).54,55 internal characteristics of these lesions. MR
Benign pleural tumors include hemangioma, imaging is generally preferable for most chest
lymphangioma (Fig. 32), neurofibromas, and wall masses. CT may be preferred when lung detail
lipomas. Malignant tumors may arise in or spread is needed.
to the pleura. These include PPB (see Fig. 31), Several anatomic variations of the chest wall
PNET, sarcomas (see Fig. 20), mesothelioma, can masquerade as a chest wall neoplasm. The
thymic carcinoma (see Fig. 6), metastatic Wilms, most common of these is a congenital abnormality
and neuroblastoma. Large effusions frequently of clavicle, sternum, rib, or costal cartilage, which
accompany and may obscure pleural tumor.56 results in asymmetry of the chest wall or a hard
Non-neoplastic lesions of the diaphragm may focal lump. The abnormalities seen most often
simulate a mediastinal or intrapulmonary mass. are sternal tilt, fusion or pectus, sternoclavicular
These include diaphragmatic hernias, eventration, subluxation, bifid rib/cartilage, and parachondral
and paresis or paralysis of the diaphragm. Neo- node (Fig. 33). When the lesion is asymptomatic,
plasms of the diaphragm are rare in children.54,57 it is most likely due to an incidental chest wall
Rhabdomyosarcoma is the expected primary anomaly, and imaging beyond a chest radiograph
malignant neoplasm; inflammatory pseudotumor is usually unnecessary.58 The benign nature of
has been described as a benign neoplasm arising these lesions can be further elucidated if needed
in the diaphragm.54,57 Intrathoracic masses may by US or low-dose CT.59 Appreciating the nature
displace, evert, and occasionally infiltrate the dia- of the abnormality is often difficult on plain radio-
phragm. Abdominal tumors, such as hepatoblas- graphs and even axial CT, especially in young chil-
toma and neuroblastoma, can extend to and dren. 3-D reconstructions of the CT data are often
occasionally invade the diaphragm. Retrocrural helpful (see Fig. 33).59 Other non-neoplastic chest
extension of tumors, such as lymphoma and neuro- wall masses include trauma (bony fracture or heal-
blastoma, are not uncommon. Juxtadiaphragmatic ing and soft tissue hematoma) and infection,
masses include lipoma, hemangio/lymphangioma, including adenopathy, cellulitis, soft tissue ab-
rhabdomyosarcoma, and esophagogastric tumors. scess, and osteomyelitis. Chest wall extension of
Chest wall tumors are uncommon in children, pulmonary infections, such as tuberculosis, asper-
and although many lesions are benign, the appear- gillus, and actinomycosis, can be difficult to differ-
ance of the lesion can be deceptive. A highly entiate from an aggressive neoplasm. Pathologic
aggressive malignant tumor, such as synovial fractures may occur with both benign or malignant
sarcoma, can appear to be well circumscribed bone lesions, and the nature of the underlying
(see Fig. 20) although a benign vascular malforma- lesion may be difficult to assess acutely.
tion may have a diffusely infiltrative appearance Benign lesions of bone that masquerade as chest
(see Fig. 32).1,54 When needed, CT or MR imaging wall masses may involve the sternum, clavicle,
658 Newman

Box 4 scapula, or ribs; be expansile, lytic, or sclerotic; or

Pleural and chest wall masses in children project from the bone surface. These include exos-
toses, enchondroma, fibrous dysplasia, and aneu-
Pleural rysmal bone cysts.54,60 Some of the lesions (eg,
1. Benign exostoses or fibrous dysplasia) may be clearly char-
a. Hemangioma acterized on plain films and not require additional
b. Lymphangioma imaging. LCH may involve one or more sites in the
c. Neurofibromas chest with an expansile destructive bony lesion
d. Lipomas and soft tissue mass that may be difficult to sepa-
2. Malignant rate from an aggressive lesion, such as Ewing
a. Pleuropulmonary blastoma sarcoma, on imaging. Similarly, osteoblastoma,
b. Contiguous or metastatic tumor: Wilms hemangiomas, or lymphangiomas of bone may
tumor, neuroblastoma, sarcoma, thymo- simulate a more aggressive lesion.54
ma, and thymic carcinoma Chest wall tumors arise from any of the tissue
c. Pleural lipoma/liposarcoma, other sar- components that make up the chest wall including
coma, PNET
blood vessels, nerves, bone, cartilage, muscle,
d. Neurofibrosarcoma
and fat. The most common benign soft tissue
e. Mesothelioma
chest wall neoplasms are vascular lesions. These
Chest wall range from single or mutiple focal small subcuta-
1. Benign neous lesions to large extensive infiltrative
a. Soft tissue origin masses; they can also be intrapulmonary or medi-
i. Hemangioma/vascular malformation astinal in location.6,20 They are divided into high-
ii. Lymphangioma flow or low-flow lesions.
iii. Lipoma/lipoblastoma In infants, hemangiomas are the most common
iv. Hamartoma (including infantile high-flow vascular lesions. They are typified by
mesenchymal hamartoma)
high T2 signal on MR imaging and marked contrast
v. Neurofibroma and ganglion cell
tumor enhancement with an early peripheral to later
vi. Fibroma/fibromatosis/desmoid central enhancement pattern on dynamic imaging.
There may be tubular signal voids from large
b. Bone origin vessels on noncontrast images.4 They tend to
i. Osteochondroma, osteoid osteoma,
grow rapidly in the first year of life and then grad-
osteoblastoma, and aneurysmal bone
cyst ually involute and become infiltrated with fibrofatty
ii. Fibrous dysplasia tissue. Subcutaneous lesions may be associated
iii. LCH with visceral lesions especially in the liver or lung.
In older children, high-flow lesions include atyp-
2. Malignant
ical hemangiomas as well as arterial vascular mal-
a. Ewing sarcoma/PNET
b. Rhabdomyosarcoma, osteosarcoma, syno- formations, and arteriovenous fistulae. Especially
vial sarcoma, chondrosarcoma, liposarcoma, in infants, the rapid-flow lesions may present clini-
leiomyosarcoma, dermatofibrosarcoma, cally with high output cardiac failure due to large
and epithelioid sarcoma shunting or thrombocytopenic bleeding associated
c. Malignant fibrous histiocytoma with platelet sequestration in the mass.1 Multi-
d. Lymphoma/leukemia phasic magnetic resonance angioigraphy (MRA) is
e. Malignant peripheral nerve cell tumor useful in defining the flow pattern and presence of
f. Bone and soft tissue metastases (neuro- arteriovenous connections. A rapid time resolved
blastoma, hepatoblastoma, and leu- MRA sequence (fat-suppressed, T1-weighted,
kemia/lymphoma)
volumetric, spoiled gradient echo) allows for rapidly
Breast repeating sequences affording improved temporal
1. Benign resolution at the expense of spatial resolution.4
a. Hemangioma Differentiating the flow pattern of a vascular lesion
b. Fibroadenoma/giant fibroadenoma often helps with management decisions and may
obviate biopsy, which could be hazardous. High-
2. Malignant
a. Cystosarcoma phylloides flow lesions may be managed (alone or in conjunc-
b. Metastases—lymphoma/leukemia and tion with surgery) with arterial embolization whereas
rhabdomyosarcoma venolymphatic malformations can be injected
c. Secondary malignancy—ductal carcinoma percutaneously with sclerosing agents.
Low-flow lesions include venocapillary and
lymphatic malformations. The nature and extent
 Thoracic Neoplasms in Children 659

Fig. 32. Lymphangioma (A) Thirty weeks’ gestation prenatal coronal T2-weighted image. (B) Coronal T2 image at
10 days of age. Both studies show a large right multicystic chest wall mass extending into the right neck and
mediastinum (arrows).

of these lesions are also well defined by MR (see Fig. 10).4 Lymphatic malformations (also
imaging (see Fig. 32). Venocapillary lesions are known as cystic hygromas) can be large and
characterized by slow flow, dilated veins, and extensive and may be initially recognized on
phleboliths (bright on T2, diffuse venous phase prenatal US or MR imaging (see Fig. 32). Involve-
enhancement with phleboliths appearing as signal ment of the soft tissues of the neck, axilla, and
voids). The macrocystic lymphatic lesion is recog- upper extremity are most common with spread
nized by large or small T2 bright cystic spaces with to the chest wall and mediastinum usually
enhancing walls.1 Microcystic lymphangioma may secondary (see Fig. 32).4,10,30 Lymphangiomas
appear to be a diffusely enhancing soft tissue are particularly associated with Turner syndrome
mass because of the closely packed small cyst and Noonan syndrome.18
walls.4 Protein or blood products within the cysts Careful fetal evaluation and follow-up are
may be bright on T1-weighted MR images and needed, particularly to assess whether a lesion
dense on CT images and produce fluid-fluid levels compromises the airway, so that time, location,

Fig. 33. Rib anomaly simulating a chest wall mass. A 2 year old with a hard palpable mass in the right anterior
chest wall. (A) Axial CT slice (lung window) shows assymmetry of the anterior chest wall with bulging of the right
side with prominent costal cartilage and sternal tilting. Specific rib abnormality was difficult to appreciate on the
axial images. (B) Oblique coronal 3-D reconstruction clearly depicts a bifid right anterior 4th rib as the cause of
the palpable mass (arrow).
660 Newman

and mode of delivery can be planned. If the airway mutation syndromes (Bannayan-Riley-Ruvalcaba
is at risk from this or other lesions, an ex utero in- syndrome [lipoma/lipomatosis, hemangiomas, and
trapartum treatment (EXIT) procedure may be lymphangiomas]) and Cowden syndrome (lipomas,
needed to safely deliver the baby while maintain- fibroangiomas, hamartomas, breast fibroadenoma,
ing the placental circulation, providing extra time oral papillomas) (see Fig. 34). Many of these enti-
to secure the airway. ties are also associated with an increased inci-
Other benign chest wall neoplasms include fatty dence of malignancy.18,62
tumors, such as lipoma (Fig. 34) and lipoblastoma Mesenchmal hamartoma of the chest wall is
(may be locally aggressive); fibrous lesions, such a unique benign entity in infants characterized by
as fibromas, desmoid tumors, and fibromatosis; focal overgrowth of skeletal elements of the chest
and neural origin tumors, such as neurofibromas wall with no malignant propensity. A large usually
and ganglion cell tumors.1,54,60 Although these unilateral mass distorting the chest wall is the
lesions are benign in that they do not metastasize; typical appearance; it is occasionally mutifocal.1,63
they may be extensive and difficult to eradicate Heterogeneous high-signal T1 areas are thought to
locally, with recurrence. The fatty nature of lipomas be due to hemorrhage and high signal regions on
and the fatty components of lipoblastoma follow T2 due to cartilage; there are typically multiple
the readily recognizable imaging characteristics fluid/fluid levels suggestive of secondary aneu-
of normal fat on both MR images (bright on T1, rysmal bone cyst formation.63,64 The CT appear-
dark on short T1 inversion recovery (STIR) and fat ance is that of rib expansion and destruction and
saturation images) and CT images (low attenuation) a large lobulated soft tissue mass with chondroid
(see Fig. 34). Fibrous lesions are less specific but calcification.64
generally are of low signal on T2-weighted MR Malignant chest wall masses may be difficult
images with mild enhancement postcontrast. to differentiate from their benign counterparts.
Neural origin tumors tend to be bright on T2- LCH in particular should be considered in the
weighted MR imaging. Neurofibromas are often differential diagnosis of Ewing sarcoma/PNET
associated with neurofibromatosis type 1; they especially when there is both bone destruction
are well defined and often multiple or lobulated and an adjacent soft tissue mass. Pretreatment
with a typical target-like appearance on T2- biopsy is usually required.54
weighted and postcontrast images. The target Metastases to bone or occasionally soft tissues
appearance consists of lower signal centrally on of the chest wall are more common than primary
T2 with increased central enhancement, thought chest wall malignant tumors.59 Chest wall meta-
to be due to the more densely cellular central static lesions (see Fig. 12) are most often due to
portion of the tumor.60 Soft tissue and other neuroblastoma, hepatoblastoma, and leukemia/
desmoid tumors are associated with Gardner lymphoma.
syndrome (familial adenomatous polyposis) or Ewings sarcoma/PNET family of tumors and
may occur at sites of prior surgery or trauma.61 rhabdomyosarcoma, although uncommon, are
Mutiple or diffuse soft tissue tumors accompany the most frequently encountered malignant chest
several systemic syndromes, such as neuro- wall neoplasms in children, with a high rate of local
fibromatosis type 1 (neurofibromas, nevi, hemangi- recurrence and distant metastases (Fig. 35).54,65
omas, and xanthogranulomas); proteus syndrome Rhabdomyosarcoma of the chest wall is usually
(lipomas/lipomatosis, hemangiomas, lymphangio- of the alveolar (most aggressive form) or embry-
mas, hamartomas, and hyperkeratosis); and PTEN onal type; the tumor may be heterogeneous with

Fig. 34. Chest wall lipomatosis. An 11 year old with PTEN mutation syndrome. (A, B) Axial contrasted CT images
with extensive low attenuation fatty masses and fatty infiltration of the soft tissues of the chest wall.
 Thoracic Neoplasms in Children 661

lymphoma/leukemia (Fig. 36). Degeneration into

chondrosarcoma is of particular concern in multiple
hereditary exostoses, most often occurring in
adulthood. Malignancy is more common in the axial
rather than the appendicular skeleton. Malignant
transformation of an osteochondroma may be diffi-
cult to recognize. Thickening and irregularity of the
cartilaginous cap, lesion enlargement, develop-
ment of a soft tissue mass, and heterogeneous
enhancement are concerning features.4,66
Tumors of the breast are uncommon in children
and are usually benign; these are most often evalu-
ated by US; occasionally, MR imaging may be ob-
tained (Fig. 37). Sonographic evaluation identifies
normal breast tissue and differentiates cystic,
Fig. 35. Ewing sarcoma—8-year-old girl. CT contrasted mixed, or solid lesions and their vascularity.
image at the thoracoabdominal junction shows a large Mammography or CT is usually unnecessary and
slightly heterogeneous chest wall soft tissue mass unhelpful.54 It is important to recognize an enlarged
centered around the anterior rib with bony destruc- breast bud from a mass when imaging an infant or
tive changes (arrows). The posterior component of
child with unilateral or bilateral breast enlargement.
the soft tissue mass exerts external mass effect on
Hemangioma is the most common benign lesion of
the liver (L).
the breast in infants and fibroadenoma in older chil-
dren (see Fig. 37).54 The giant fibroadenoma of
multiple areas of necrosis.66 The chest wall is a site adolescents is typically larger than 10 cm. These
of predilection for Ewing and PNET (also known as are well-defined, uniform rounded large hypoechoic
Askin tumor)1,4; the tumor may originate in bone, masses on US.3 Other benign lesions include papil-
soft tissue, or pleura or rarely may be loma, lipoma, and lymphangioma.3 Primary breast
intrapulmonary.65,67 Imaging helps define the loca- malignancies are unusual in childhood; the most
tion, possible origin, and extent (see Fig. 35). The common tumor is cystosarcoma phylloides.54
chest wall is a difficult region to obtain an Metastases to the breast may occur with lym-
adequate tumor resection with clear margins phoma, leukemia, and rhabdomyosarcoma.
without marked deformity and respiratory compro-
mise (see Fig. 20).65 LONG-TERM EFFECTS OF CHILDHOOD
Other primary chest wall malignant lesions in CANCER
childhood include osteogenic sarcoma, chondro-
sarcoma, synovial sarcoma (see Fig. 20), malignant Late effects of treatment of childhood cancer are
peripheral nerve sheath tumors, liposarcoma, leio- increasingly recognized as common and im-
myosarcoma, malignant fibrous histiocytoma, portant.68,69 Long-term morbidity, often at an early
epithelioid sarcoma, dermatofibrosarcoma, and age, is present in more than half of childhood

Fig. 36. A 15-year-old girl with cutaneous/subcutaneous T cell lymphoma. Axial (A) and coronal (B) postcontrast
CT images with plaque like superficial lesions (arrows) as well as a larger infiltrating lesion of the chest wall soft
tissues on the left (arrowheads) with loss of normal muscle/fat planes.
662 Newman

Fig. 37. An 18-year-old woman. Right breast fibroadenoma. (A) Axial T1-weighted, (B) axial T2-weighted, and (C)
axial postcontrast MR images. There is a 4.5-cm well-defined sharply marginated solid enhancing mass in the
right breast (arrows). This was an incidental finding on this MR, obtained for other reasons. The MR imaging char-
acteristics (T2 bright with enhancement) suggest that this is an adenomatous or myxomatous rather than fibrous
histologic tumor type. This MR imaging appearance does not distinguish fibroadenoma from malignant tumors.

cancer survivors. These include joint disease, malignant bone tumors and soft tissue sarcomas
osteoporsosis, scoliosis, congestive heart failure, as well as leukemia/lymphoma and bronchogenic
restrictive/obstructive lung disease/pulmonary carcinoma, including bronchoalveolar carcinoma,
fibrosis, second cancers, cognitive dysfunc- are concerns for second tumors after radiation/
tion, coronary artery disease, cerebrovascular chemotherapy.40,68,69 Even though treatment
accident, renal failure, cystitis/bladder fibrosis, as- regimens have changed, radiation therapy, in
plenia (infection risk), hepatic cirrhosis, bowel particular, is used less and at lower, more directed
fibrosis/obstruction, hearing and vision loss, doses, close follow-up of childhood cancer survi-
growth failure, and gonadal failure.68 vors is needed. Radiologists as well as clinicians
Resection of thoracic tumors both benign and need to be aware of and vigilant for secondary
malignant may have significant prolonged effects complications of prior malignancies.
in terms of thoracic deformity (see Fig. 20) with
distortion of vessels and airways and lung restric-
tion as well as affecting overall well-being and self-
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