Mehu107 - U3 - t1 - Mediastinals Tumors and Cysts

SECTION O
DISORDERS OF THE MEDIASTINUM
83 MEDIASTINAL TUMORS
AND CYSTS
GUANG-SHING CHENG, MD • THOMAS K. VARGHESE JR., MD, MS • DAVID R. PARK, MD
INTRODUCTION Magnetic Resonance Imaging SPECIFIC MEDIASTINAL TUMORS AND

NORMAL ANATOMY OF THE Ultrasonography CYSTS
MEDIASTINUM Nuclear Imaging Lesions of the Anterior Mediastinum
CLINICAL PRESENTATIONS OF TECHNIQUES FOR OBTAINING Lesions of the Middle Mediastinum
MEDIASTINAL DISEASE MEDIASTINAL TISSUE Lesions of the Posterior Mediastinum
Mediastinum in Patients with Malignancy Image-Guided Biopsy Miscellaneous Mediastinal Masses
Asymptomatic Mass Surgical Biopsy GENERAL APPROACH TO A
Compression or Invasion of Adjacent MEDIASTINAL MASS MEDIASTINAL MASS
Tissues Classification Initial Evaluation
Systemic Symptoms and Syndromes Incidence Surgical Management
IMAGING THE MEDIASTINUM
Conventional Radiographic Techniques
Computed Tomography
describes the normal anatomy and contents of the medias-

Additional figures and videos are available online at tinum, the clinical manifestations produced by mediastinal
ExpertConsult. disease, and the means available for diagnostic investiga-
tion. It then describes the features of specific mediastinal
tumors and cysts and outlines an overall clinical approach
to the evaluation of mediastinal disease. The focus of the
discussion of pathology is on lesions that arise primarily in
INTRODUCTION the mediastinum; lung cancer is discussed as it pertains to
findings in the mediastinum.
The mediastinum is the region in the chest between the
pleural cavities that contains the heart and other thoracic
viscera, except the lungs. Interest in the mediastinum as a
separate body region stems from the diversity and impor- NORMAL ANATOMY OF
tance of the structures it contains and the multiplicity of THE MEDIASTINUM
disease processes by which it can be affected. The mass
lesions that arise in the mediastinum represent a heterog- The anatomy of the mediastinum is divided into anterior,
enous group of benign and malignant processes that defy middle, and posterior compartments.1 This three-
easy categorization. The nonspecific clinical manifestations compartment model is consistent with embryonic develop-
of most of these disorders and the relative inaccessibility ment of the region and with the characteristic distribution
for tissue sampling result in considerable challenges to of individual disorders encountered clinically. The ana-
the clinician evaluating mediastinal disease. This chapter tomic relationships of the mediastinal viscera and tissue
1478
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83 • Mediastinal Tumors and Cysts 1479
A B
Figure 83-1 Mediastinal compartments. A, By anatomic convention, the mediastinum is divided into anterior (A), middle (M), and posterior (P) compart-
ments, as outlined on this lateral chest radiograph. B, Another method was developed to facilitate constructing an accurate differential diagnosis for
mediastinal masses detected at chest radiography. Using this radiologic method, the anterior mediastinal compartment (A) is defined as the tissues resid-
ing anterior to a line drawn along the anterior aspect of the trachea and extended along the posterior cardiac margin, from the thoracic inlet to the
diaphragm. The posterior mediastinal compartment (P) is defined as structures residing posterior to a line drawn 1 cm posterior to a line drawn along the
anterior margins of the thoracic vertebrae; the middle mediastinal compartment (M) consists of those tissues residing between these two lines. (From
Whitten CR, Khan S, Munneke GJ, Grubnic S: A diagnostic approach to mediastinal abnormalities. Radiographics 27:657–671, 2007.)
planes are best appreciated on axial images such as are Table 83-1 Normal Mediastinal Contents
shown schematically on a lateral chest radiograph in
ANTERIOR COMPARTMENT
Fig. 83-1.2
The anterior compartment consists of everything ante- Thymus gland
Substernal extensions of thyroid and parathyroid glands
rior and superior to the heart; its boundaries are the Lymphatic vessels and lymph nodes
sternum, the first rib, and an imaginary curved line follow- Connective tissue
ing the anterior heart border and brachiocephalic vessels MIDDLE COMPARTMENT
from the thoracic inlet to the diaphragm. Within the ante-
Heart
rior compartment lie the thymus gland, any substernal Pericardium
extensions of the thyroid and parathyroid glands, and lym- Aortic arch and great vessels
phatic tissue (Table 83-1). Innominate veins and superior vena cava
The middle compartment, dorsal to the anterior medias- Pulmonary arteries
Trachea and main bronchi
tinum, extends from the lower edge of the anterior heart Hila
border along the diaphragm and then cranial along the Lymph nodes
posterior heart border and posterior wall of the trachea. It Phrenic and upper vagus nerves
contains the heart, the pericardium, the aortic arch and its Connective tissue
major branches, the innominate veins and superior vena POSTERIOR COMPARTMENT
cava (SVC), the pulmonary arteries and hila, the trachea, Esophagus
and several groups of lymph nodes. In addition, the phrenic Descending aorta
and upper vagus nerves course through the middle medias- Azygos and hemiazygos veins
tinal compartment. Paravertebral lymph nodes
Thoracic duct
The posterior compartment occupies the space between Vagus nerves (lower portions)
the back of the heart and trachea and the front of the Sympathetic chains
posterior ribs and paravertebral gutters. It extends from Connective tissue
the diaphragm cranial to the first rib. In it are the esopha-
gus, descending aorta, azygos and hemiazygos veins,
1480 PART 3 • Clinical Respiratory Medicine
paravertebral lymph nodes, and thoracic duct. The lower in the supine position. Numerous benign causes of SVC
portions of the vagus nerve and sympathetic chains also lie syndrome are described,10 but bronchogenic carcinoma
within the posterior mediastinum. (Video 83-1 and eFig. 83-1) and lymphoma (Video 83-2
and eFig. 83-2) are now the most common etiologies.11,12
The compression or invasion of nerves may result in
CLINICAL PRESENTATIONS OF hoarseness from involvement of the recurrent laryngeal
nerve, Horner syndrome from involvement of sympathetic
MEDIASTINAL DISEASE ganglia, dyspnea from involvement of the phrenic nerve
causing diaphragmatic paralysis, tachycardia from involve-
MEDIASTINUM IN PATIENTS
ment of the vagus nerve and clinical manifestations of
WITH MALIGNANCY spinal cord compression.
The most common reason that clinicians evaluate the medi-
astinum is in the staging of patients with lung cancer, SYSTEMIC SYMPTOMS AND SYNDROMES
because the extent to which the mediastinum is involved
is crucial to management.3 Staging of lung cancer is Fever, anorexia, weight loss, and other systemic symptoms
discussed in greater detail in Chapters 21 and 53. Careful are nonspecific features of malignancy and inflammation
preoperative evaluation of the mediastinum is critical in that may manifest in patients with mediastinal disease.
determining a patient’s candidacy for surgical resection or In addition, primary mediastinal tumors are associated
other treatment modalities.4,5 with a wide array of distinctive systemic syndromes (Table
Nonthoracic malignancies also may metastasize to the 83-2).6,7,13-15 Some typically have endocrine activity, such
mediastinum. This is particularly common in tumors as intrathoracic goiter, which may present with thyrotoxi-
originating in the head and neck, the esophagus, the cosis. Cushing syndrome is associated with thymomas and
genitourinary tract, the breasts, and the skin (malignant carcinoid tumors. Thymomas are classically associated
melanoma). with myasthenia gravis (Video 83-3 and eFig. 83-4), in
addition to other systemic syndromes. Patients with human
chorionic gonadotropin-secreting germ cell tumors may
ASYMPTOMATIC MASS
manifest with gynecomastia; patients with pheochromocy-
The majority of mediastinal masses are discovered toma may present with hypertension. Hypercalcemia may
incidentally—at least half of all mediastinal masses are be a presenting abnormality observed in patients with para-
asymptomatic and detected by chest radiography performed thyroid adenoma and lymphoma. Hypoglycemia in patients
for unrelated reasons. About 80% of such asymptomatic with certain pleural tumors, teratomas, fibrosarcomas, and
masses are benign, whereas more than half of those that neurosarcomas is also believed to be the result of tumor
produce symptoms are malignant.6-8 products with endocrine activity.
COMPRESSION OR INVASION OF
ADJACENT TISSUES
Table 83-2 Systemic Syndromes Associated with
Symptoms in patients with mediastinal mass lesions are Mediastinal Masses
usually caused by compression or invasion of adjacent
Syndrome Associated Conditions
intrathoracic structures.6,7 Chest pain, from traction on
mediastinal tissues, tissue invasion, or bone erosion, is ENDOCRINE EFFECTS
common. Cough may be due to extrinsic compression of the Hypothyroidism or Mediastinal goiter
trachea or bronchi, erosion into the airway, and sometimes hyperthyroidism
Hypercalcemia Parathyroid adenoma, lymphoma
postobstructive pneumonia. Hemoptysis, hoarseness, or Hypertension Pheochromocytoma,
stridor also may be part of the clinical presentation. Inva- ganglioneuroma, chemodectoma
sion or inflammation of the pleural surface may produce a Cushing syndrome Carcinoid, thymoma
pleural effusion, and cause pain and dyspnea. Compression Hypoglycemia Mesenchymal tumor
or direct invasion of the esophagus may lead to dysphagia. Gynecomastia Germ cell tumor
Diarrhea Ganglioneuroma, neuroblastoma
Rarely, anterior mediastinal tumors can cause pericarditis
or pericardial tamponade, and masses in the middle medi- AUTOIMMUNE EFFECTS
astinum can produce right ventricular outflow obstruction Opsomyoclonus Neuroblastoma
and cor pulmonale. Myasthenia gravis Thymoma
Red cell aplasia Thymoma
The SVC is especially vulnerable to extrinsic compression Myocarditis Thymoma
and obstruction because it is thin-walled and has low intra- Hypogammaglobulinemia Thymoma
vascular pressure. The SVC syndrome results from increased CONGENITAL SYNDROMES
venous pressure in the upper thorax, head, and neck9 and
Neurofibromatosis Neurofibroma
is characterized by dilation of collateral veins in the upper Multiple endocrine Parathyroid adenoma,
portion of the thorax and neck, edema and plethora of the neoplasia pheochromocytoma
face, neck, and upper torso, and suffusion and edema of the UNKNOWN CAUSES
conjunctiva (see Fig. 53-5A and B). Neurologic symptoms
Alcohol-induced pain Hodgkin lymphoma
such as headache, disturbance of consciousness, and visual Fever and night sweats Lymphoma
distortion, may be present. Symptoms are exacerbated
rior and may contain areas of calcium (sometimes teeth or

IMAGING THE MEDIASTINUM bone), fat, and soft tissue. Neural tumors lie posteriorly and
have sharply delineated margins. Bronchogenic cysts tend
The mediastinum is relatively inaccessible for examination to lie against the trachea, carina, or main bronchus. These
or exploration. Accordingly, imaging studies play an impor- findings give clues to the possible origin of a mediastinal
tant role in the initial evaluation of mediastinal disease. mass, but further imaging evaluation is usually required.
These include conventional radiographic studies, computed In the appropriate clinical settings, contrast studies
tomography (CT), magnetic resonance imaging (MRI), trans- remain important diagnostic tools in mediastinal disease.
thoracic and endoscopic ultrasonography, PET, and other Barium esophagrams can demonstrate extrinsic compres-
radionuclide studies. sion, esophageal diverticulum, tumor invasion, or fistula
formation.6 Angiography can identify vascular compres-
sion or invasion, can define the vascular supply of tumors,
CONVENTIONAL RADIOGRAPHIC TECHNIQUES
and can sample blood for hormonal localization of certain
Most mediastinal abnormalities are first detected by stan- tumors. Myelography may help delineate intraspinal exten-
dard posteroanterior and lateral chest radiographs, and sion of posterior mediastinal tumors and differentiate neu-
certain mediastinal mass lesions have characteristic find- rogenic neoplasms from meningoceles. For the most part,
ings (Table 83-3). For example, teratomas are usually ante- these techniques have been supplanted by CT and MRI.
COMPUTED TOMOGRAPHY
Table 83-3 Characteristic Radiographic Findings in
Mediastinal Disease CT imaging is the mainstay of radiographic evaluation of
the mediastinum, because this modality can firmly deter-
Feature Likely Etiology
mine the anatomic location, morphology, and tissue density
Bulky mass on initial Anterior: lymphoma, germ cell tumor, of a mass. The transaxial plane of CT is well suited for
presentation thymoma or thymic carcinoma assessment of mediastinal structures, most of which are
Posterior: neurogenic tumor
oriented perpendicularly to this plane. Administration of
Teardrop-shaped mass Pericardial or bronchogenic cyst intravenous contrast helps delineate vascular structures as
within interlobar fissure
they relate to a mass and other mediastinal structures.
Fat density on CT scan Mediastinal lipomatosis or lipoma
Easily identified CT patterns include the high density of cal-
Calcification in mass In rim of mass: cified tissue and contrast-enhanced blood vessels, and the
Cystic thymoma or thyroid adenoma
Aneurysm characteristic low density of fat (Fig. 83-2).16 Normal ana-
Silicosis (“eggshell” calcification) tomic variations and fluid-filled cysts can be distinguished
In center of mass: confidently from bulky solid masses, which may be irregu-
Thyroid adenoma larly bordered and possess necrotic areas. Additionally, the
Teratoma
site of origin of mediastinal masses can be better identi-
Teeth or bone Teratoma fied.17 Characteristic CT findings in a variety of mediastinal
Phleboliths Hemangioma disorders have been described (see Table 83-3). For example,
Air-fluid level in mass Esophageal disease the specificity of the CT appearance of teratomas (Video
Diaphragmatic hernia 83-4, eFigs. 83-9 and 83-10), thymolipomas (Video 83-5
Developmental cyst
Cystic teratoma and eFig. 83-11), and omental fat herniation (Video 83-6
Abscess and eFig. 83-12) is 100%, but the overall accuracy of CT
Mass with associated Granulomatous inflammation/infection for predicting the diagnosis of all mediastinal masses is less
parenchymal opacity Metastatic bronchogenic carcinoma than 50%.16
Lymphoma with direct extension into Lymph nodes are readily identifiable on CT scan and can
lung be categorized by size and morphology. Mediastinal lymph
Esophageal abnormality with
aspiration pneumonitis nodes greater than 1 cm in diameter in the short axis are
Bronchial compression by primary considered to be abnormally enlarged and are suspicious for
mediastinal mass malignancy in the proper clinical context. Mediastinal
Mass with associated Metastatic malignancy with pleural lymph nodes greater than 2 cm in diameter are virtually
pleural effusion involvement always abnormal. In the most recent systematic review of
Granulomatous inflammation of lymph studies on the use of CT in mediastinal staging of lung
nodes
cancer, the median sensitivity and specificity for identifying
Superior vena cava Recent onset:
obstruction Bronchogenic carcinoma
metastatic lymph nodes using the greater than 1 cm crite-
Lymphoma ria were 55% and 81%, respectively,18 similar to what was
Catheter-associated thrombosis previously reported by Gould and coworkers.19 However,
Long-standing: even in series of patients with proven bronchogenic carci-
Mediastinal fibrosis noma, benign findings were present in 10% to 37% of
Erosion or destruction of Arterial aneurysm lymph nodes that were either larger than 2 cm in diameter
bone Tumors of peripheral nerves or
sympathetic ganglia
or had evidence of central necrosis.20,21
Meningocele Even though CT cannot reliably distinguish between
Spine or rib deformity Enteric cyst benign and malignant disease, it remains the initial imaging
procedure of choice for the evaluation of the mediastinum
A B
Figure 83-2 Mediastinal lipomatosis. Chest radiograph (A) showing diffuse mediastinal widening (arrows) and chest CT scan (B) showing extensive fat
deposition (arrows) in the anterior mediastinum in a patient with mediastinal lipomatosis.
in patients with a primary mediastinal mass13-15 or with

suspected lung cancer.22 CT can precisely define the medi-
astinal anatomy and guide subsequent invasive diagnostic
and staging procedures, or can confirm a clinical suspicion
of extensive mediastinal involvement or visceral organ
invasion that precludes curative resection.
MAGNETIC RESONANCE IMAGING

Although much less frequently used than CT in evaluating
mediastinal lesions, MRI offers several potential advantages
over CT. MRI assesses tissues by measuring radiofrequency-
induced nuclear resonance emissions, and the better con-
trast resolution over CT is advantageous in evaluating soft
tissue structures and tissue boundaries (Fig. 83-3).23-26
Blood vessels are identifiable without the need for contrast
enhancement (Fig. 83-4) (Videos 83-7A and B), thus MRI
can provide an alternative to patients who cannot be given
iodinated contrast material required by CT. Ionizing radia-
tion exposure is also eliminated.27
MRI has utility in evaluating neurogenic tumors, and it
may also be useful in evaluating thymoma and distinguish-
ing it from congenital cyst or thymic carcinoma.28 MRI can
be helpful for defining anatomy before surgical resection of Figure 83-3 Magnetic resonance lymphogram of the thoracic duct.
superior sulcus tumors or those invading the mediastinum, The normal course of the thoracic duct can be seen crossing diagonally
through the lower mediastinum from the patient’s right to left and then
chest wall, or diaphragm.4,22 ascending along the left mediastinum to reach the subclavian vein. In this
Whereas CT is more commonly used for routine staging case the duct is somewhat more dilated and tortuous than normal due to
of lung cancer, MRI may be useful for defining anatomy in the presence of hepatic cirrhosis. (From Takahashi H, Kuboyama S, Abe H,
special circumstances, such as before surgical resection of et al: Clinical feasibility of noncontrast-enhanced magnetic resonance lym-
superior sulcus tumors or tumors invading the mediasti- phography of the thoracic duct. Chest 124:2136–2142, 2003.)
num, chest wall, or diaphragm.5,23 A large multicenter
study comparing CT and MRI in patients with lung cancer of MRI to establish malignancy or benignity requires further
found similar accuracy for the detection of mediastinal study.
node involvement, but MRI was superior for detecting direct
mediastinal tumor invasion (Video 83-8 and eFig. 83-15; ULTRASONOGRAPHY
see also Chapter 18).29 Diffusion-weighted MRI distin-
guished between malignant and benign mediastinal lesions Ultrasonography can confirm the cystic nature of medias-
based on apparent diffusion coefficient levels with a sen tinal masses, but it cannot readily distinguish between
sitivity of 95% and specificity of 87% in a study of 53 benign and malignant cystic lesions. Both transthoracic
mediastinal lesions,24 with evidence of lower apparent dif- and endoscopic ultrasound probes are useful in the evalua-
fusion coefficient values indicating a slower diffusion of tion of mediastinal disease in the context of guiding endo-
water molecules in the malignant lesions. However, the use scopic biopsy procedures.30
NUCLEAR IMAGING
Nuclear imaging studies rely on the localization of markers
based on specific metabolic or immunologic properties of
the target tissue to provide a functional image of a lesion.
The spatial resolution of radionuclide scans is relatively
poor, but the overall diagnostic accuracy may be high if a
sufficiently specific probe is available. Nuclear studies offer
the potential to identify a primary malignancy and identify
distant metastases with a single scan of the entire body.
PET is a widely used nuclear imaging technique that
relies on high-energy photon–emitting probes, such as
18
F-fluorodeoxyglucose (FDG), which are chemically trapped
within metabolically active neoplastic cells. The result is a
high signal-to-background ratio and excellent spatial reso-
lution for a functional image of a tumor (Fig. 83-5). More
recently, use of combined and co-registered PET and CT
images has allowed for more accurate anatomic localization
of the lesions in question but at the cost of lower specificity
and increased false-positive results.18,31
The use of PET in the evaluation of the mediastinum is
largely focused on metastatic disease from thoracic malig-
nancies, because it is useful in staging and preoperative
Figure 83-4 Magnetic resonance imaging of superior vena cava syn-
drome. A 52-year-old woman with multiple myeloma experienced symp- planning for lung cancer. In the evaluation of suspected
toms of superior vena cava (SVC) obstruction following placement of an lung cancer, PET can identify metastatic foci in the medias-
indwelling central venous catheter in preparation for bone marrow trans- tinum and extrathoracic sites and help determine the
plantation. Vertically oriented structures are clearly discernible, and black optimal biopsy approach that will make a histologic diag-
“flow void” is present within the heart and great vessels. The SVC is com-
pletely obstructed by thrombus (arrow).
nosis as well as stage the disease.32 Despite widespread use
of PET scanning, standardized quantitative criteria for
defining an abnormal scan are lacking, and accuracy is far
from perfect.33 False-positive results can be caused by gran-
ulomatous, inflammatory, or infectious conditions. FDG-PET
B E
Figure 83-5 Chest CT and PET scans of a patient with right upper lobe squamous cell carcinoma. The primary tumor and right paratracheal adenopa-
thy (A) and subcarinal adenopathy (B) are evident on the standard CT images (arrows). PET images reveal probe uptake in the primary tumor and right
paratracheal nodes on axial (C) (arrow) and coronal (D) views, but not in the subcarinal node on coronal (D) (arrow) and axial (E) views. The arrowheads
in E point to the main bronchi. At mediastinoscopy, the right paratracheal nodes were found to be malignant, and the subcarinal nodes were enlarged
but benign. (From Vansteenkiste JF, Stroobants SG, De Leyn PR, et al: Mediastinal lymph node staging with FDG-PET scan in patients with potentially operable
non-small cell lung cancer: a prospective analysis of 50 cases. Leuven Lung Cancer Group. Chest 112:1480–1486, 1997.)
A B
Figure 83-6 Mediastinal lymphoma. A, Chest radiograph of a 36-year-old man with gray zone lymphoma presenting as a bulky mediastinal mass.
B, PET-CT scan performed before initiation of treatment shows a hypermetabolic anterior mediastinal mass with invasion of the chest wall and pectoralis
muscles as well as involvement of axillary lymph nodes.
has been shown to be more accurate than CT for mediasti- percutaneous, or transesophageal approaches. Surgical
nal staging of lung cancer with a sensitivity of 80% and a biopsies are obtained by more invasive procedures including
specificity of 88% in an updated meta-analysis18; when mediastinoscopy and thoracoscopy.
lymph nodes are enlarged by CT criteria, sensitivity increases
while specificity decreases.19 Mediastinal lymph node sam-
pling is warranted in the setting of a positive PET scan if IMAGE-GUIDED BIOPSY
the findings of mediastinal involvement would alter the
subsequent surgical approach (see Chapters 21 and 53). Endobronchial Ultrasound-Guided Transbronchial
The utility of PET for the evaluation of primary medias- Needle Aspiration
tinal lesions is not as well established as it is for metastatic For evaluation of mediastinal adenopathy or other lesions
disease. FDG-PET may differentiate between thymoma and in the middle mediastinum, transbronchial needle aspiration
thymic carcinoma,34 but has low sensitivity in differentiat- (TBNA) via the fiberoptic bronchoscope offers a less invasive
ing between nonaggressive and aggressive subtypes of option to surgical mediastinoscopy. Although few signifi-
thymoma.28 It is not used routinely for staging of thymoma. cant complications have been reported, the sensitivity of
However, PET is considered standard of care in the pretreat- blind TBNA is low, ranging from 14% to 50%.36-38
ment workup and follow-up of mediastinal lymphoma (Fig. Endobronchial ultrasound-guided transbronchial needle aspi-
83-6). FDG-PET also plays a role in detecting residual post- ration (EBUS-TBNA) is a recent technology that has signifi-
chemotherapy malignant germ cell tumors, specifically cantly improved the ability of pulmonologists to diagnose
seminomas, of the mediastinum. There is little role for PET and stage non–small cell lung cancer in a minimally inva-
in evaluating neurogenic tumors.35 Hypermetabolic lesions sive manner. With the advent of a curvilinear ultrasound
in the mediastinum may also represent sarcoidosis, myco- probe integrated at the end of the bronchoscope, TBNA
bacterial and fungal infection, or brown fat. with a 22-gauge needle can be performed under real-time
Other nuclear medicine techniques for the evaluation of ultrasonographic guidance. The ability to visualize the area
the mediastinum include radioiodine scanning for the of interest as well as adjacent vascular structures has vastly
detection of ectopic thyroid tissue; a positive result is improved diagnostic yields.39-41 Upper and lower paratra-
pathognomonic for that condition.6 This approach must be cheal, subcarinal, and hilar lymph nodes are readily acces-
planned carefully because iodinated contrast administered sible by EBUS-TBNA, as is any mediastinal or hilar lesion
intravenously for a CT scan may prevent the uptake of that is adjacent to the large airways. EBUS-TBNA has the
radioiodine for several weeks or more. advantage over mediastinoscopy in accessing the posterior
subcarinal lymph nodes as well as hilar nodes or masses, in
addition to being an ambulatory procedure with lower asso-
ciated health care costs.42
TECHNIQUES FOR OBTAINING While the efficacy of EBUS-TBNA is now firmly estab-
MEDIASTINAL TISSUE lished in the evaluation of lung cancer, there is also an
increasing role for the initial evaluation of isolated medias-
Definitive diagnosis of most mediastinal masses requires tinal adenopathy due to other conditions such as sarcoid-
the evaluation of a tissue sample. However, biopsy of medi- osis. In a randomized controlled trial of 50 patients with
astinal tissue should be reserved for instances when diag- clinically suspected sarcoidosis due to the presence of medi-
nostic results will influence subsequent treatment. The astinal and hilar adenopathy, the diagnostic yield of EBUS-
decision to perform a biopsy rather than surgical resection TBNA was superior to blind TBNA, with a sensitivity of 83%
is based on the presumptive diagnosis. If definitive surgical and specificity of 100%.43 In a prospective trial of 77
resection is the treatment choice regardless of the results of patients with isolated mediastinal adenopathy, a specific
a biopsy, then a “diagnostic delay” should be avoided. diagnosis of sarcoidosis, tuberculosis, lymphoma, or other
Available approaches for biopsy of mediastinal lesions malignancy was made in 67 of them, thus obviating the
include needle aspiration and biopsy via transbronchial, need for a more invasive surgical mediastinoscopy.44
EBUS-TBNA can be useful in providing a definitive diagnosis an anterior or parasternal mediastinoscopy) provides access
of primary or recurrent lymphoma; however, its role in the to the lymph nodes in the aortopulmonary window.
initial diagnosis of mediastinal lymphoproliferative disor- Although more invasive than a percutaneous or endobron-
ders is controversial because the amount of tissue provided chial approach, mediastinoscopy has the advantage of pro-
may not be adequate for histologic subtyping.44,45 viding the entire lymph node for histologic examination,
rather than the cellular aspirates or small tissue fragments
Endoscopic Ultrasound-Guided Needle Aspiration produced by needle biopsy techniques. Mediastinoscopy is
and Biopsy most frequently used in the staging of bronchogenic carci-
Endoscopic ultrasound (EUS)–guided sampling relies on the noma,51 but has utility in evaluating mediastinal adenopa-
placement of biopsy needles that are passed through the thy or mass lesions of other etiologies. Either frozen section
working channel of a gastroscope.46 The proximity of the or imprint cytology methods can provide rapid, accurate
esophagus to mediastinal sites relatively inaccessible to results and facilitate immediate decisions about the feasibil-
mediastinoscopy, such as the posterior subcarinal lymph ity of curative resection.52,53
nodes, makes this approach particularly useful in selected Mediastinal anatomy from the perspective of the surgeon
cases. EUS-guided biopsy has similar sensitivity as PET for performing a mediastinoscopy is different from that based
determining inoperability in lung cancer and, importantly, on the lateral chest radiograph as described earlier. For
superior specificity (100% vs. 72%).47 In selected cases, it mediastinoscopy, structures are considered according to
can confirm the presence of mediastinal metastases and whether they lie anterior, posterior, or immediately to the
thereby obviate the need for surgical staging procedures.48 right or left of the trachea.2 Mediastinoscopy is performed
using general anesthesia, but is typically an outpatient pro-
Percutaneous Needle Aspiration and Biopsy cedure when subsequent thoracotomy is not planned to
Percutaneous needle aspiration and biopsy of mediastinal follow immediately.54
masses, usually in the anterior compartment, can be per- Mediastinoscopy is safe and well tolerated. Complications
formed using ultrasound or, more often, CT guidance49 (Fig. of mediastinoscopy include pneumothorax, hemorrhage,
83-7). Percutaneous needle aspiration of the mediastinum recurrent laryngeal nerve or phrenic nerve paralysis, injury
has acceptable morbidity and yields comparable to those to the trachea, esophageal perforation, thoracic duct lacera-
from percutaneous biopsy of pulmonary lesions. As with tion, air embolism, and mediastinitis.
TBNA, serious bleeding is seldom encountered,49 and accu-
rate diagnosis of a wide variety of lesions has been reported. Video-Assisted Thoracoscopic Surgery
Biopsies of mediastinal lymph nodes can also be performed
by video-assisted thoracoscopic surgery (VATS). VATS provides
SURGICAL BIOPSY access to the hilar nodes and inferior pulmonary ligament
lymph nodes on both sides. Additionally, on the right side,
Mediastinoscopy VATS can provide access to the right paratracheal lymph
Mediastinoscopy allows direct inspection and biopsy of nodes and subcarinal nodes. Left-sided VATS can provide
lymph nodes or other masses in the superior portion of the access to the aortopulmonary nodes. VATS can also be a
anterior mediastinum.50 Cervical mediastinoscopy provides tool for the evaluation of pleural and lung abnormalities in
access to the paratracheal and subcarinal lymph nodes, the management of mediastinal diseases. After dissection
whereas an anterior mediastinotomy (otherwise known as through the mediastinal pleura, mediastinal lymph nodes
can be sampled to aid in the staging of malignancies such
as esophageal carcinoma and for the diagnosis and resec-
tion of primary mediastinal tumors and cysts.55-58 VATS
requires a general anesthetic, chest tube placement at the
conclusion of the procedure, and typically a limited stay in
the hospital.
MEDIASTINAL MASS
CLASSIFICATION
Mediastinal masses are considered primary, that is, arising
solely from structures within the mediastinum, or second-
ary, usually as metastatic disease from intrathoracic or
extrathoracic malignancy.
The most practical clinical classification of primary medi-
astinal masses groups together lesions that are characteris-
tically found in the anterior, middle, or posterior mediastinal
Figure 83-7 CT-guided needle aspiration of an anterior mediastinal compartments (Table 83-4), with the recognition that such
mass. The image shows the needle entering the mass by passing lateral
to the sternum and medial to the internal mammary vessels (arrow, left
a simplified scheme overlooks the fact that mediastinal
internal mammary artery). Associated findings include a pretracheal lymph masses will not necessarily respect anatomic borders.
node and bilateral pleural effusions. Masses found within any of the mediastinal compartments
Table 83-4 Disorders Presenting as a Mass in the Mediastinum

Anterior Mediastinum Middle Mediastinum Posterior Mediastinum
Thymic neoplasms Lymphadenopathy Neurogenic tumors
Germ cell tumors Reactive and granulomatous inflammation Meningocele
Teratoma Metastasis Esophageal lesions
Seminoma Angiofollicular lymphoid hyperplasia (Castleman disease) Carcinoma
Nonseminomatous germ cell tumors Lymphoma Diverticula
Embryonal cell carcinoma Developmental cysts Diaphragmatic hernia (Bochdalek)
Choriocarcinoma Pericardial cyst Miscellaneous
Lymphoma Foregut duplication cysts
Hodgkin lymphoma Bronchogenic cyst
Non-Hodgkin lymphoma Enteric cyst
Thyroid neoplasms Others
Parathyroid neoplasms Vascular enlargements
Mesenchymal tumors Diaphragmatic hernia (hiatal)
Lipoma
Fibroma
Lymphangioma
Hemangioma
Mesothelioma
Sarcoma
Diaphragmatic hernia (Morgagni)
NUT midline carcinoma
may be due to lesions more commonly found in another

mediastinal compartment or due to those that have SPECIFIC MEDIASTINAL TUMORS
extended from another area in the mediastinum (Fig. 83-8). AND CYSTS
In a series of 400 consecutive patients with a primary
mediastinal lesion,59 25% had a primary cystic lesion and LESIONS OF THE ANTERIOR MEDIASTINUM
42% had a malignant lesion. The anterior compartment
was the most common compartment for a lesion, which Thymic Neoplasms
was more likely to be malignant, followed by the posterior Thymoma is the most common neoplasm arising in the
and then middle compartments. Although two thirds of anterior mediastinum6,7,17,64 and is increasingly recognized
mediastinal masses are benign, the likelihood of malig- in the course of thorough evaluations of patients with
nancy depends on the location, age of the patient, and pres- myasthenia gravis.6 It remains a rare tumor, with an overall
ence or absence of symptoms.60,61 In a series of 38 patients incidence of 0.13 per 100,000 person-years in the United
with malignant mediastinal tumors, 31 had at least one States.65 The peak incidence of thymomas is between the
sign or symptom.61 ages of 40 and 60 years and is higher in Asians and
African Americans, with equal gender predilection.
Although most thymomas are not biologically aggres-
INCIDENCE
sive, about one third of thymomas found have already
The true incidence of primary mediastinal masses is diffi- invaded their capsules. Advanced disease involves extension
cult to ascertain. In a study of more than 9000 patients into local structures and transdiaphragmatic extension into
in a lung cancer CT screening trial, the prevalence of an the abdomen and pericardial involvement, but lymphoge-
incidentally detected mediastinal mass was 0.77%; on nous and hematogenous metastases are rare.61 The histo-
follow-up annual screening, the incidence was 0.01%.62 logic classification remains a subject of debate and
Historically, thymomas and developmental cysts were the revision.28 The current World Health Organization classifi-
most common masses found in adults, followed by neuro- cation system, which is based on histologic features, does
genic tumors and lymphoma, based on the collection by not accurately predict clinical outcome, thus treatment of
Silverman and Sabiston of nearly 2400 cases from the lit- patients has been based historically on the presence and
erature (Table 83-5).6 More recent series suggest a similar degree of tumor invasion into microscopic and local struc-
pattern,8,63 although Cohen and associates8 have observed tures. Most clinicians use the Masaoka clinical staging
both a rising incidence of mediastinal masses in general system, which is based on the degree of invasion of the
and an increasing proportion of lymphoma and malignant tumor through the capsule into adjacent structures.61
neurogenic tumors over the course of their 45-year survey. Moran and colleagues recently proposed a new staging
Neurogenic tumors, thymomas, and developmental cysts system in which the overall prognosis and recurrence is
account for about 60% of all mediastinal masses. Lympho- based on extent of tumor infiltration.66 Currently the Inter-
mas and germ cell tumors such as teratoma and semi- national Thymic Malignancies Interest Group (ITMIG) and the
noma account for about 25%, and a large number of other International Association for the Study of Lung Cancer (IASLC)
lesions, both benign and malignant, constitute the remain- are collaborating on developing a new TNM-based classifi-
ing 15%.8 cation system, which is expected in 2017.66a
Table 83-5 Relative Frequencies of Mediastinal Masses in

Adults and Children*
Lesion Adults (%) Children (%)
Thymoma 19 —
Developmental cysts 21 18
Bronchogenic 7 8
Pericardial 7 <1
Enteric 3 8
Other cysts 4 2
Neurogenic tumors 21 40
Lymphoma 13 18
Germ cell tumors 11 11
Endocrine (thyroid, parathyroid, 6 —
carcinoid)
Mesenchymal tumors 7 9
Primary carcinoma — —
Other malignancies 3 4
A *Based on Silverman and Sabiston’s review of reported mediastinal masses

in 1950 adults and 437 children.
Data from Silverman NA, Sabiston DC Jr: Mediastinal masses. Surg Clin North
Am 60:757–777, 1980.
thymoma usually distorts the gland’s normal shape and

extends to one side.71 On CT scan, most thymomas present
as a solid 5- to 10-cm anterior mediastinal mass outlined
by fat; up to one third of thymomas contain cystic, necrotic,
or hemorrhagic areas that enhance heterogeneously. Con-
trast CT is necessary for the staging of thymoma, specifi-
cally for discerning vascular involvement. MRI can help
distinguish benign cysts from a cystic thymoma or thymic
carcinoma. At this time, nuclear medicine has little role in
B the evaluation of thymoma. Due to their relatively indolent
nature, most thymomas have low FDG uptake, which limits
Figure 83-8 Thymoma. Typical findings on chest radiograph (A, frontal the utility of PET imaging to discern a benign from a malig-
view) and chest CT (B) showing a mass presenting in the anterior and nant thymic mass.28,61
middle mediastinum. At surgery, this mass proved to be a benign thymoma,
originating in the anterior mediastinum and extending by a slender stalk The mainstay of therapy for thymomas is surgical resec-
into the middle compartment. (Courtesy Dr. Robert Stevens, Wenatchee, WA.) tion, which provides the best chance for an optimal progno-
sis.61 Adjunctive treatment with postoperative radiotherapy
is typically provided,7,64,72 and the addition of preoperative
or adjuvant chemotherapy appears promising for more
Clinically, the majority of patients with thymoma are advanced stages.73-75
asymptomatic, while one-third of patients will present with Patients whose tumors are fully encapsulated with no
nonspecific chest pain, cough, or dyspnea due to local evidence of invasion can expect postoperative survival
tumor effects.67 Forty to 70% have at least laboratory evi- equal to that of the general population. Invasive tumors
dence of one or more of the two dozen systemic “parathy- have a poorer prognosis, with 50% to 77% 5-year and 30%
mic” syndromes that have been recognized.6 Thymomas are to 55% 10-year survival.76 Thymoma recurs after resection
associated with numerous systemic syndromes, most of in nearly a third of patients.76 The largest and most recent
which appear to be autoimmune in origin.6,7,14 The most retrospective survey of thymic tumors from the European
familiar of these is myasthenia gravis, reported in 10% to Society of Thoracic Surgeons database showed that higher
50% of patients with thymoma6 and thought to be due to Masaoka stage (with evidence of invasion), incomplete
autoantibodies to the postsynaptic acetylcholine recep- resection, and nonthymoma histology were factors in
tor.68,69 Other associated syndromes include pure red blood recurrence and worsening survival.76a
cell aplasia, myocarditis, and hypogammaglobulinemia. Thymic carcinoma is an aggressive epithelial malignancy
Patients with thymoma also have an increased incidence of that invades locally and frequently metastasizes.77,78 This
collagen vascular disease, Whipple disease, and malignancy rare cancer develops predominantly in middle-aged men,
elsewhere in the body. who present with symptoms of cough, dyspnea, and chest
On chest radiography, thymomas appear as an ovoid, pain as well as nonspecific systemic symptoms.61 On
smooth or lobulated unilateral mass near the junction imaging, thymic carcinomas are heterogenous masses
of the heart and great vessels (Fig. 83-9).70 Compared with areas of necrosis and calcifications. They are highly
with thymic hyperplasia, which is typically symmetrical, FDG-avid on PET scan.28,34 The prognosis, which depends
A B
Figure 83-9 Thymoma. A, Frontal chest radiograph shows a smoothly marginated mass along the right side of the mediastinum (arrows). B, Axial mag-
netic resonance T1-weighted image through the base of the heart shows that the mass (arrows) is slightly hyperintense compared to the skeletal muscle
and resides within the anterior mediastinum. Note the smooth, well-defined margins of the mass, consistent with an encapsulated thymoma. (Courtesy
Michael Gotway, MD.)
on the histologic grade and the anatomic stage, is generally

poor. Surgical resection is the treatment of choice; chemo-
therapy and radiation therapy are advocated for unresect-
able disease.
Carcinoid tumors occasionally arise in the thymus.79,80
They may cause Cushing syndrome and be associated with
multiple endocrine adenomatosis. Locally invasive carci-
noids may be difficult to resect completely, but characteristi-
cally have a prolonged clinical course.6 Interestingly, the
thymus is also a common site for mediastinal Hodgkin lym-
phoma, and normal thymic tissue may enlarge following
chemotherapy for lymphoma (a process termed thymic
rebound) (eFig. 83-25), mimicking recurrence of the
primary disease.81 Other thymic mass lesions include benign
conditions such as thymic hyperplasia (eFig. 83-26), thymic
cysts, and lipothymomas (see Video 83-5 and eFig. 83-11).82
Germ Cell Tumors Figure 83-10 Teratoma. Axial CT image through the base of the heart
Approximately 10% to 12% of primary mediastinal masses shows a large right-sided anterior mediastinal mass with heterogenous
attenuation. Elements of calcium, soft tissue, and fat (*) are present. The
are derived from multipotent germ cells that migrated presence of fat within an anterior mediastinal mass is most consistent with
abnormally during early embryonic development.6,7,83,84 teratoma. (Courtesy Michael Gotway, MD.)
These neoplasms are classified into three groups: benign
teratoma, seminoma, and nonseminomatous germ cell
tumors.84 tion (occasionally, fully formed teeth and bone) can be iden-
Teratomas, the most common germ cell tumors, are by tified, rendering this one of few mediastinal tumors that
definition made up of tissues foreign to the area in which can be diagnosed confidently before operation16 (Fig.
they arise. Ectodermal derivatives predominate, but struc- 83-10). All teratomas should be resected due to malignant
tures originating in all three primary germ cell layers may potential and effects of impingement on adjacent vital
be found. Dermoid cyst refers to a lesion that contains only structures. In malignant teratoma, adjuvant combination
the epidermis and its derivatives. Teratomas arise most often chemotherapy may result in improved survival.86
in young adults, but have been reported in all age groups; Seminomas and nonseminomatous germ cell tumors are
men and women are affected with equal frequency. Most malignant and nearly always cause symptoms. These
patients with teratomas have symptoms caused by the lesions appear as a large anterior mediastinal mass on chest
tumor; only about a third are asymptomatic.6,85 Usual imaging (Video 83-9 and eFig. 83-29). Seminomas are seen
symptoms are pain, cough, and dyspnea. Teratomas can almost exclusively in men, usually in the third decade of
rupture into the pleural space or into the pericardium. If life.7 Most patients seek medical attention because of chest
the tumor erodes into a bronchus, the patient may have pain, dyspnea, cough, hoarseness, or dysphagia. Semino-
hemoptysis or even expectorate differentiated tissue such as mas are aggressive malignant tumors that extend locally
hair (trichoptysis) or sebaceous material.85 and metastasize distantly, usually to skeletal structures. The
On chest radiographs, teratomas appear smooth, tumor can obstruct the SVC. They may secrete human cho-
rounded, and circumscribed if they are cystic. Solid lesions rionic gonadotropin, but not alpha-fetoprotein. Factors
can appear lobulated and asymmetric. On CT scans (see associated with a poor prognosis include age older than 35
Video 83-4 and eFig. 83-10), soft tissue, fat, and calcifica- years, SVC obstruction, supraclavicular, cervical, or hilar
adenopathy, and fever.87,88 Seminomas are extremely radio- dard of treatment for early stage, bulky mediastinal disease
sensitive and may respond dramatically to chemotherapy, is combined modality therapy, consisting of ABVD (doxoru-
even in cases with dissemination.84,89 With aggressive bicin, bleomycin, vinblastine, and dacarbazine) and radia-
cisplatin-based regimens, long-term survival for all medias- tion. Optimal therapy for nonbulky mediastinal HL is
tinal seminomas is approximately 80%.84,90 controversial; recent clinical trials suggest no difference in
Nonseminomatous mediastinal germ cell tumors include overall survival but an increase in risk for disease progres-
embryonal cell carcinoma and choriocarcinoma.6,7,87 Like sion with chemotherapy alone.101,102 The benefits of radia-
seminoma, these tumors develop mainly in men in the third tion therapy must be balanced with the risk for serious late
and fourth decades and are usually symptomatic. These complications, including pulmonary fibrosis, cardiovascu-
malignancies carry a poorer prognosis relative to cancers lar disease, and secondary malignancies such as breast and
arising from the gonads.84 Embryonal cell carcinoma is also lung cancer.100
called endodermal sinus or yolk sac tumor (Video 83-10 and PMBL is treated with immunochemotherapy, which
eFig. 83-30). These highly aggressive tumors often secrete includes rituximab with CHOP (cyclophosphamide, doxoru-
human chorionic gonadotropin, alpha-fetoprotein, or car- bicin, vincristine, prednisone)-based regimens, followed by
cinoembryonic antigen. Human chorionic gonadotropin radiation. Whereas these standard dose regimens have a
may also produce clinical manifestations, such as gyneco- cure rate of up to 75%, more recent evidence has shown
mastia, in 50% of patients.7 Associations have been noted that dose intensity may improve outcomes in PMBL.
with Klinefelter syndrome91 and with hematologic malig- DA-EPOCH (dose-adjusted etoposide, vincristine, doxorubi-
nancy.92 Most patients present with disseminated disease, cin, cyclophosphamide, prednisone, rituximab) has been
and the prognosis has been less favorable than in semi- shown to confer an overall survival of 97% without the
noma.7 Cisplatin-based treatment regimens have markedly need for radiation treatment in the majority of patients.103
improved the outcome, with more than 50% of patients The prognosis of HL and NHL has improved strikingly in the
achieving long-term survival.84,92 Long-term survival may past 2 decades as staging of the disease has been refined and
also be possible in those who undergo a complete surgical as more effective and less toxic combinations of radiother-
resection following chemotherapy.93 Even disseminated and apy and chemotherapy have evolved. With new chemother-
refractory malignant germ cell tumors may respond to apy regimens, radiotherapy may not be a necessary part of
aggressive chemotherapy94 and salvage regimens involving the treatment plan for PMBL.103a PET scan, which is often
bone marrow transplantation.95,96 used to restage aggressive lymphomas, appears to have a
high negative predictive value and may be helpful for clini-
Lymphoma cians in deciding when to omit radiotherapy after primary
Lymphoma is an important cause of mediastinal mass, chemotherapy; this is being investigated in an ongoing clin-
and is distinguished from other mediastinal lesions in that ical trial.103b,103c Effective salvage regimens including bone
management is primarily medical, not surgical. In most marrow transplantation have been developed to treat
series, lymphoma represents between 10% and 20% of relapsed disease. HL is curable in approximately 75% of
mediastinal masses in both adults and children.6-8,63 patients, although late toxicities of treatment contribute
Lymphomas are the most common anterior and middle significantly to mortality.
mediastinal masses in children; a majority of pediatric
patients with Hodgkin lymphoma (HL) and half of those with Thyroid Lesions
non-Hodgkin lymphoma (NHL) present with a mediastinal In surgical series, ectopic thyroid glands account for fewer
mass.97 HL has a bimodal distribution, arising in adoles- than 10% of mediastinal masses but, in clinical practice,
cents and young adults as well as in those older than 50, these are probably more common. Thyroid tissue within the
whereas NHL arises most commonly in older adults. mediastinum is of two distinct origins. Most commonly, a
Primary mediastinal B-cell lymphoma (PMBL) is a distinct cervical goiter extends substernally into the anterior medi-
subset of NHL that has a similar clinical presentation as astinum.104 Primary intrathoracic goiter, presumably origi-
classic HL of the nodular sclerosing subtype.98 Both present nating from an embryonic nest of heterotopic thyroid
in the third or fourth decade of life and tend to affect tissue, is rare. Most such goiters are in the anterior medias-
females. These tumors present as a bulky anterior mediastinum, but they may arise in the middle or posterior
tinal mass involving the thymus (see Fig. 83-6). SVC syn- mediastinum as well.6,7 Intrathoracic goiter presents pre-
drome is a common presentation of PMBL, less so for HL dominantly in middle aged or older women. Although it is
(see Video 83-2 and eFig. 83-2), which can involve the hilar usually asymptomatic, goiter may cause hoarseness, cough,
nodes and lung parenchyma.99 However, there are distinct or swelling of the face and arms. Intrathoracic thyroid
and nonoverlapping histologic features. HL is characterized tissue is easily recognized by radioactive iodine scanning,105
by the Hodgkin/Reed Sternberg cell in a nodular growth as long as the scan is completed before intravenous iodin-
pattern with a specific immunophenotype of CD30+, ated contrast injection, which may block iodine uptake for
CD45−, and CD15+ in 85% of cases. PMBL is histologically weeks. It may be suspected on the basis of high radiodensity
characterized by an infiltrate of large cells in a diffuse on CT scans, particularly after iodinated contrast injec-
pattern with an immunophenotype of a mature B lympho- tion.106,107 Treatment is surgical resection.
cyte expressing CD20. A B-cell lymphoma that exhibits his-
tologic features of both PMBL and HL has been named Parathyroid Lesions
“gray zone lymphoma.”100 Mediastinal parathyroid tissue accounts for up to 10% of
Distinguishing HL from PMBL is important for guiding cases of hyperparathyroidism, and the mediastinum is the
therapy, thus tissue biopsy is indicated. The current stan- most common site for ectopic parathyroid adenomas in
surgically resistant hyperparathyroidism.108 Half of ectopic

parathyroid adenomas lie in the anterior mediastinum,
usually near the thymus. Parathyroid cysts may enlarge
sufficiently to appear as a mass on the chest radiograph and
to produce symptoms, but ectopic tissue may be difficult to
locate.109 Evaluation of the lesion is typically conducted
using CT angiography, ultrasound, MRI, and the sensitive
technetium-99m sestamibi scanning.110 Selective arteriog-
raphy and venous sampling for parathormone levels have
largely been supplanted by sestamibi radionuclide scan-
ning.110 Parathyroid adenomas are cured by complete resec-
tion, and resection via VATS is increasingly advocated.55
Parathyroid carcinoma may be functional, resulting in
varying degrees of hyperparathyroidism, but is also locally
invasive and may metastasize.111 Cure is possible with
aggressive surgical management112 that may be guided by
imaging and functional localization studies.113
Mesenchymal Tumors
Included in this group of unusual mediastinal masses are
lipomas, fibromas, mesotheliomas, and lymphangiomas
(see Table 83-4).6 They arise from connective tissue, fat, A
smooth muscle, striated muscle, blood vessels, or lymphatic
channels and can be found in any region of the mediasti-
num. Histologically and clinically, they are not substantially
different from their counterparts elsewhere in the body.
Unless the lesion is very large, the presence of symptoms
usually indicates that the lesion is malignant.6,17
Lipoma is the most common mesenchymal tumor of the
mediastinum and is most often located in the anterior medi-
astinum. It may be encapsulated or unencapsulated,
appearing as a smooth and rounded lesion with well-defined
margins. The characteristic low density of lipomas on CT
images (Fig. 83-11) permits a confident diagnosis unless
there is associated heterogeneity, invasion of surrounding
tissues, or poor demarcation of the mass’s perimeter, in
which case malignancy (liposarcoma or lipoblastoma) or
teratoma must be excluded.17 Considerably more common
than lipoma is mediastinal lipomatosis, or generalized over-
abundance of histologically normal unencapsulated fat
(see Fig. 83-2).114 Mediastinal lipomatosis appears on the B
conventional radiograph as a smooth widening or bulging
of normal mediastinal contours, and its low homogeneous Figure 83-11 Lipoma. A, Chest radiograph of a 31-year-old man showing
CT density confirms the diagnosis.17 Mediastinal lipoma- an abnormality at the right cardiophrenic angle noted as an incidental
finding. B, Axial chest CT demonstrates a well-circumscribed homogene-
tosis does not compress or displace other mediastinal ous fat-density mass characteristic of a mediastinal lipoma extending into
structures. the right hemithorax (arrow).
Sarcomas involving the mediastinum are rare but, if
present, are likely to be due to nerve sheath sarcoma, spindle
cell sarcoma, leiomyosarcoma, or liposarcoma.115 Prior
radiation therapy appears to be a predisposing factor in mediastinum as a specific disease has been reported. It is
some cases.116 Primary mediastinal sarcoma is a rare entity possible that these tumors represented a heterogenous
that affects adults; it can present with distant metastases117 group of metastatic carcinoma of unknown origin, or oth-
and tends to have an aggressive course. erwise unclassifiable disease.
In the 1990s, cases of young patients with a highly
Primary Carcinoma aggressive carcinoma presenting in the mediastinum and
Numerous rare neoplasms of the mediastinum have been other midline structures were reported; these tumors
described in larger case series of mediastinal tumors as well were found to have t(15;19) translocations, resulting in
as in isolated case reports.7,118 Case series up through the a BRD4-NUT fusion oncogene,119,120 which was first
1980s report primary carcinoma of the mediastinum as described in 2003. These NUT midline carcinomas are uni-
comprising 1% or fewer of the cases of malignant neo- formly fatal, presenting as large masses with local mass
plasms found in the anterior mediastinum.59,143 Since these effects and distant metastases. The advent of molecular
case series, very little regarding primary carcinoma of the targets in cancer treatment make this discovery a potential
Developmental Cysts
Internal mammary Developmental cysts of various sorts comprise 10% to 20%
of all mediastinal masses.6,127 Most can be identified as
bronchogenic, enteric, or pericardial.6 Bronchogenic and
Right
paratracheal
enteric cysts are often referred to as foregut duplication cysts
Aortopulmonary because of their origin from aberrant portions of the ventral
window and dorsal foregut, respectively.
Bronchogenic cysts are found near large airways, often
Hilar just posterior to the carina (Fig. 83-13), although they may
Subcarinal
be attached to the esophagus or even lie inside the pericar-
Hilar dial space.6 The cyst wall often contains cartilage and respi-
ratory epithelium. Most are discovered incidentally and
Posterior cause no symptoms; however, they may communicate with
mediastinal the tracheobronchial tree and become infected128; enlarge
Paracardiac
sufficiently to cause airway obstruction,129 pulmonary
artery compression,130 or hemodynamic collapse131; or
rupture.132 Enteric or enterogenous cysts are similar in loca-
tion and appearance to bronchogenic cysts, but have diges-
tive tract epithelium. They are relatively uncommon in
adults, but are the most common cysts found in infants and
children, in whom they may be associated with spinal
Figure 83-12 Simplified schematic diagram depicting mediastinal lymph extension and malformations of the vertebral column
node groups. (Redrawn from McLoud TC, Meyer JE: Mediastinal metastases. (called “neurenteric” cysts). Enteric cysts can occasionally
Radiol Clin North Am 20:453–468, 1982.) be multiple and associated with duplications of other por-
tions of the gastrointestinal tract.
therapeutic target in undifferentiated or poorly differenti- Pericardial cysts account for about one third of cystic
ated epithelioid malignancies. masses in adults, but are much less common in children
(see Table 83-5). They lie against the pericardium, dia-
phragm, or anterior chest wall in the right cardiophrenic
LESIONS OF THE MIDDLE MEDIASTINUM angle. Rarely, pericardial cysts may communicate with the
pericardial space. Although typically harmless, pericardial
Enlargement of Lymph Nodes cysts may enlarge enough to cause right ventricular outflow
Numerous classifications exist for the mediastinal lymph obstruction,133 or they may rupture and hemorrhage to
nodes, including the most recently accepted classification cause pericardial tamponade134 or sudden cardiac death.135
by the International Association for the Study of Lung Developmental cysts can usually be identified by CT (see
Cancer.121 Figure 83-12 presents a simplified grouping of Fig. 83-13A) or ultrasonography, and the diagnosis may be
the main mediastinal nodes that corresponds to findings on confirmed by aspiration cytology. MRI is valuable for con-
chest radiography and CT scans.122 Most authors consider firming the cystic nature of these lesions (see Fig. 83-13B-
1-cm diameter at the short axis to be the upper limit of D). Pericardial cysts can be followed with serial imaging
normal.18 and need not be resected unless they cause symptoms.
Evaluating the mediastinum in the context of staging Bronchogenic cysts can also be followed conservatively if
lung cancer is discussed elsewhere in the textbook (see asymptomatic136; however, most authorities favor resection
Chapter 53). Mediastinal lymph node enlargement is most for purposes of diagnosis and to reduce the potential for
often due to lymphoma,123 metastatic cancer,122 granulo- complications.128,132 Alternatives to thoracotomy include
matous inflammation, such as that caused by sarcoidosis, therapeutic aspiration137,138 and thoracoscopic or mediasti-
or infection. Infection should be considered when the ade- noscopic excision.56,139,140
nopathy is associated with a pulmonary opacity. Tubercu-
losis is a notable cause of mediastinal adenopathy that can
mimic sarcoidosis or malignancy and should be suspected LESIONS OF THE POSTERIOR MEDIASTINUM
in a host with known tuberculosis risk factors, such as
recent known exposure or residence in an endemic area.124 Neurogenic Tumors
Fungal infections that cause granulomas, particularly his- The vast majority of tumors in the posterior mediastinum
toplasmosis, may present with mediastinal and hilar ade- represent neoplasms arising from neural tissues,6,7 histori-
nopathy in the absence of a pulmonary opacity.125 cally accounting for about 20% of adult cases and twice
Numerous less common causes of mediastinal adenopa- that proportion in children (see Table 83-5). Neurogenic
thy are described, including Castleman disease or angiofol- tumors appear radiographically as a unilateral paraverte-
licular lymphoid hyperplasia. Mediastinal adenopathy is bral mass.6,141,142 Clinical manifestations include chest pain
common in HIV–infected patients and is usually caused from nerve or bone erosion, dyspnea secondary to tracheal
by infection, although adenopathy may also be caused compression, and neurologic deficits resulting from spinal
by lymphoma, Kaposi sarcoma, and other noninfectious cord compression by intraspinal tumor extension. In addi-
processes.126 tion, many neurogenic tumors are hormonally active. MRI
A C
B D
Figure 83-13 Bronchogenic cyst. Axial unenhanced chest CT (A) shows a mildly hyperattenuating structure (arrow) in the subcarinal space. Axial triple
inversion-recovery (TIR) MRI (B) and axial unenhanced double inversion-recovery (DIR) image (C) show the same lesion (arrows). Note the very high signal
on the fluid-sensitive TIR image (B), consistent with fluid, and the mildly increased signal on the unenhanced DIR image (C), consistent with proteinaceous
or hemorrhagic fluid; the latter mechanism also accounts for the hyperattenuating appearance on CT. Enhanced DIR MRI (D) shows no enhancement of
the lesion (arrow) following intravenous gadolinium administration, typical of a foregut duplication cyst. (Courtesy Michael Gotway, MD.)
Table 83-6 Neurogenic Tumors of the Mediastinum bromas may eventually transform into the malignant neu-
rosarcoma in 10% to 15% of patients.7
NEOPLASMS ARISING FROM PERIPHERAL NERVES
Neurilemoma, or schwannoma (Videos 83-11A and B,
Neurofibroma and eFig. 83-43), is another common neurogenic tumor
Neurilemoma (schwannoma)
Neurosarcoma that arises from the neural sheath. Most often seen in the
third to fifth decade of life, neurilemoma usually causes
NEOPLASMS ARISING FROM SYMPATHETIC GANGLIA
no symptoms and appears as a well-circumscribed, homo-
Ganglioneuroma geneous density on chest radiography (Fig. 83-14A).6,7 It
Ganglioneuroblastoma
Neuroblastoma is always completely encapsulated and does not invade
surrounding tissues, although neurilemomas may extend
NEOPLASMS ARISING FROM PARAGANGLIONIC TISSUE
into intervertebral foramina (Fig. 83-14B and C). Treat-
Pheochromocytoma ment of both neurilemoma and solitary neurofibroma is
Paraganglioma (chemodectoma)
resection.
Malignant tumors of nerve sheath origin are also known
as malignant neurofibroma, malignant schwannoma, or
is usually indicated when there is suspicion for a neurogenic neurogenic fibrosarcoma. These tumors behave aggres-
lesion. Although the majority of neurogenic tumors are sively, with both local invasion and distant metastasis. Half
benign, surgical resection is indicated to alleviate local arise in patients with neurofibromatosis.15 Treatment
symptoms from mass effects of the tumor. requires wide excision and usually adjuvant radiation.15
Neurogenic tumors are classified by site of origin: periph- Tumors arising from nerve cells in sympathetic ganglia
eral nerves, sympathetic ganglia, or paraganglionic tissue generally affect children and display a spectrum of neoplas-
(Table 83-6). Tumors arising from peripheral nerves include tic behavior ranging from ganglioneuroma, a benign tumor,
neurofibromas, neurilemomas, and neurosarcomas. Neu- to the malignant tumors ganglioneuroblastoma and neuro-
rofibromas contain both nerve sheath cells and nerve ele- blastoma. Ganglioneuromas cause symptoms merely by
ments and are the most common tumor of this group. They mass effect, and are usually cured by surgical excision. Gan-
are incompletely encapsulated and may grow quite large, glioneuroblastomas have intermediate behavior and can
producing symptoms by compression of nerves or other invade locally but are less likely to metastasize.7 Neuroblas-
structures. Mediastinal neurofibroma may be one manifes- toma rapidly displaces and invades adjacent structures and
tation of von Recklinghausen disease (neurofibromatosis). may be widely metastatic at the time of presentation.143,144
As with these tumors found elsewhere in the body, neurofi- These tumors often elaborate endocrine peptides and
A B C
Figure 83-14 Series of imaging studies of a 64-year-old asymptomatic man with a surgically proved neurilemoma, or schwannoma. A, Frontal
chest radiograph showing an incidental finding of a discrete mass behind the right clavicular head (arrow). B, Axial chest CT showing homogeneous,
well-circumscribed appearance and posterior location adjacent to the vertebral column. C, Magnetic resonance image showing extension of the tumor
into the intervertebral foramen (arrow).
catecholamines, and are associated with diarrhea, fever, and great vessels, aortic coarctation, innominate vein and
anorexia, and weight loss.6 The propensity to produce cat- SVC aneurysm, persistent left vena cava, azygos (Video
echolamines is used to advantage by promoting tumor 83-14 and eFig. 83-48) and hemiazygos vein enlargement,
uptake of the catecholamine precursor 123I- or 131I-meta- anomalous pulmonary venous return, pulmonary venous
iodobenzylguanidine (MIBG) for purposes of diagnosis, iden- varix, and varices associated with portal hypertension
tification of distant tumor metastases, and treatment.145,146 (eFig. 83-49).152 Idiopathic dilation of the pulmonary trunk
Treatment of neuroblastoma involves using escalating mul- and pulmonary arterial hypertension of any cause may
timodality therapy strategies that are based on the risk for appear as a mediastinal mass, and traumatic aortic transec-
disease progression.147 tion or more subtle vascular injuries may result in medias-
Neoplasms arising from paraganglionic or chemorecep- tinal hematoma.
tor tissue include pheochromocytomas, which are found Angiography has been the traditional means of diagnos-
rarely in the mediastinum and are indistinguishable clini- ing mediastinal masses of vascular origin but, in most cases
cally and histologically from their counterparts in the today, CT imaging with intravenous contrast provides a
abdomen, and paragangliomas (chemodectomas). Para- more convenient diagnosis.153,154 MRI may permit definitive
gangliomas may secrete catecholamines just as do pheo- diagnosis without radiation or contrast exposure.
chromocytomas and thus may also be detected and perhaps
treated with MIBG.148 They appear benign under the micro- Diaphragmatic Hernia
scope and seldom metastasize, but spread locally in an Omental fat (Video 83-15 and eFig. 83-50; see also Video
aggressive fashion and thus have high morbidity and mor- 83-6 and eFig. 83-12) or other abdominal contents may
tality rates.6 protrude through the diaphragm via several potential
routes, producing mediastinal mass lesions in any compart-
ment.17 A hernia through the foramen of Morgagni (eFig.
MISCELLANEOUS MEDIASTINAL MASSES 83-50) produces a cardiophrenic angle mass, usually on
the right side in the anterior mediastinum. Bochdalek
Benign Lesions hernia (eFig. 83-51), in the posterior mediastinum, gener-
Pancreatic pseudocysts may extend into the mediastinum, ally appears on the left side, presumably because the liver
usually through the aortic or esophageal hiatus.7,149,150 prevents herniation on the right. Herniation of fat around
They have been reported in all three mediastinal compart- the esophagus is believed to precede hiatal hernia forma-
ments. Hydatid cysts also arise rarely in the mediastinum.7 tion; either may appear as a mediastinal mass on chest radi-
The posterior mediastinum is occasionally the site of a tho- ography. Fine linear opacities are often demonstrable in
racic duct cyst.6,63 Also, extramedullary hematopoiesis diaphragmatic fat herniations on CT imaging. These are
(Video 83-12 and eFigs. 83-44 and 83-45) in patients with thought to represent omental vessels within the hernia, and
chronic hemolytic anemia may appear as a mediastinal they may help differentiate a hernia from a lipoma.17
mass, often in a posterior location. Meningoceles, out-
growths of the spinal meninges that protrude along the
course of a spinal nerve, can produce posterior mediastinal
masses. GENERAL APPROACH TO A
Vascular Masses and Enlargements MEDIASTINAL MASS
Although not technically masses, a variety of vascular
lesions must be considered in the differential diagnosis of INITIAL EVALUATION
a mediastinal mass seen on the chest radiograph.7,151 With the advent of advanced imaging techniques such as
These include poststenotic aortic dilation, aneurysms CT, MRI, and nuclear imaging, the evaluation of a medias-
(Video 83-13 and eFig. 83-46) or tortuosity of the aorta tinal mass is best made in a multidisciplinary setting
involving the pulmonologist or internist, a radiologist, and ations where there is a high degree of suspicion for lym-
a thoracic surgeon. The differential diagnosis of a medias- phoma. Biopsy is also preferred for locally invasive or frankly
tinal mass depends significantly on patient demographics, unresectable anterior mediastinal lesions. Biopsy options for
the presence of clinical symptoms, as well as the anatomic anterior lesions include CT-guided needle biopsy and ante-
location, size and morphology. For a mediastinal mass that rior mediastinotomy. Cervical mediastinoscopy is less useful
is found incidentally, the primary concern for a clinician is for anterior mediastinal masses because the anterior medi-
to determine whether it is benign or malignant, and, if astinal mass is not near the pretracheal plane. Interventions
benign, whether it has the potential to cause local symp- by VATS are generally reserved for those lesions that are
toms. At a minimum, a chest CT scan with contrast should amenable to resection. Definitive resection of anterior medi-
be obtained to evaluate any mediastinal mass detected by astinal masses can be performed by VATS, median sternot-
conventional radiography. This narrows the differential omy, or a transcervical approach (e.g., Cooper thymectomy).
diagnosis and suggests further imaging or a diagnostic and/ A robotic VATS approach has gained in popularity in recent
or therapeutic procedure. A stable appearance, in compari- years for resection of anterior mediastinal masses.
son to older films, can obviate the need for further investiga- In the middle mediastinum, the majority of masses in
tion in selected patients. A few benign lesions in the adults are malignant, the most common being lymphoma
mediastinum can be diagnosed with confidence based on or metastatic disease. Hence for lesions in the middle
clinical information and CT scan appearance; such benign mediastinum, biopsy is the typical initial approach. Biopsy
lesions include vascular lesions, extramedullary hemato- can be performed by either CT-guided needle techniques,
poiesis, pericardial cysts and developmental cysts, and EBUS or EUS-TBNA, or by mediastinoscopy or VATS. Given
mediastinal lipomatosis. Suspected mediastinal thyroid the proven sensitivity and negative predictive value of
tissue can be confirmed by radioactive iodine scans. EBUS-TBNA, this is the preferred sampling option. When
benign-appearing cystic lesions are found by diagnostic
imaging, the lesion is removed by either VATS or open
SURGICAL MANAGEMENT
thoracotomy.
In most cases, the optimal diagnostic and therapeutic In the posterior mediastinum, most lesions are resected
maneuver is surgical resection of the mass.155 The decision either with a VATS or thoracotomy approach. A thoracot-
to perform a biopsy versus resection is thus based on the omy is preferred if there is any suggestion of malignancy
presumptive diagnosis after imaging. A suggested approach (to ensure free resection margins), size greater than 5 cm,
for evaluation is shown in Figure 83-15. or presence of inflammation or infection that can obscure
In the anterior mediastinum, well-encapsulated lesions dissection planes. Surgical experience plays a large part in
are resected, whereas biopsy will be performed in those situ- determining the technique utilized for intervention.
Suspicion of DIFFERENTIAL
COMPARTMENT RADIOGRAPHIC DIAGNOSIS INTERVENTION
mediastinal disease
APPEARANCE
Thymoma
Germ cell tumor
YES
Thyroid goiter SURGICAL RESECTION
Well-encapsulated Carcinoma
Anterior mass Mesenchymal tumor
CT scan with contrast NO
Lymphoma
NEEDLE BIOPSY OR
Unresectable or
SURGICAL BIOPSY
metastatic disease
Solid or cystic mass Lymphadenopathy
NO YES Middle Metastatic disease
Cystic Lymphoma
EBUS-TBNA
Granulomatous disease
TB/other infection
Bronchogenic cyst
Vascular abnormality
Diaphragmatic hernia Pericardial cyst OBSERVATION
Posterior Solid mass
Extramedullary hematopoiesis
Other benign lesions
Neurogenic tumor
MEDICAL THERAPY
Figure 83-15 A suggested algorithm for the diagnostic approach to mediastinal masses.
Complete reference list available at ExpertConsult.

ACKNOWLEDGMENTS
The authors wish to acknowledge the contributions of Key Readings
David J. Pierson, MD, who wrote this chapter for the first Davis RD Jr, Oldham HNJ, Sabiston DC Jr: Primary cysts and neoplasms
and second editions, and coauthored this chapter with DRP of the mediastinum: recent changes in clinical presentation, methods of
diagnosis, management, and results. Ann Thorac Surg 44:229–237,
for the third edition. These earlier chapters contain more 1987.
extensive and historical references. Duwe BV, Sterman DH, Musani AI: Tumors of the mediastinum (Review).
Chest 128:2893–2909, 2005.
Engels EA: Epidemiology of thymoma and associated malignancies
Key Points (Review). J Thoracic Oncol 5:S260–S265, 2010.
Grant C, Dunleavy K, Eberle FC, et al: Primary mediastinal large B-cell
■ The mediastinum, which contains thoracic viscera in lymphoma, classic Hodgkin lymphoma presenting in the mediastinum,
the center of the chest, can be divided into three com- and mediastinal gray zone lymphoma: what is the oncologist to do?
partments based on anatomic boundaries that can be (Review). Curr Hematol Malig Rep 6:157–163, 2011.
seen on a lateral chest radiograph: anterior, middle, Juanpere S, Canete N, Ortuno P, et al: A diagnostic approach to the medi-
astinal masses. Insights Imaging 4:29–52, 2013.
and posterior. Marom EM: Advances in thymoma imaging. J Thorac Imaging 28:69–80,
■ While the majority of mediastinal masses are benign, 2013.
the likelihood of a mediastinal mass being malignant Silverman NA, Sabiston DC Jr: Mediastinal masses (Review). Surg Clin
depends on the compartment of the mediastinum, North Am 60:757–777, 1980.
patient factors, and the presence of symptoms. Symp- Silvestri GA, Gonzalez AV, Jantz MA, et al: Methods for staging non-small
cell lung cancer: diagnosis and management of lung cancer, ed 3. Amer-
tomatic masses are more likely to be malignant. ican College of Chest Physicians evidence-based clinical practice guide-
■ The most common mediastinal masses in the anterior lines. Chest 143:e211S–e250S, 2013.
compartment include thymomas, germ cell tumors,
and lymphoma. The middle compartment includes
lymphadenopathy and benign developmental cysts.
Isolated mediastinal adenopathy may represent sar-
coidosis, tuberculosis, lymphoma, lung cancer, or
metastatic disease from an extrathoracic malignancy.
The posterior compartment includes mainly neuro-
genic tumors.
■ Most mediastinal tumors and cysts should be surgi-
cally removed due to potential for malignancy and
local compressive and invasive effects on adjacent vital
structures.
83 • Mediastinal Tumors and Cysts 1495.e1
eFIGURE IMAGE GALLERY
A C
B D
eFigure 83-1 Axial contrast-enhanced chest CT in a patient with bronchogenic carcinoma and superior vena cava syndrome shows a confluent mediastinal
mass (arrows) occupying the anterior and middle mediastinum, compressing the superior vena cava (arrowheads), resulting in collateral circulation through
the azygos vein (small arrow). See Video 83-1 for the full study. (Courtesy Michael Gotway, MD.)
A C
B D
eFigure 83-2 Axial contrast-enhanced chest CT in a patient with lymphoma and superior vena cava (SVC) syndrome shows a confluent mediastinal mass
(arrows, B–C) occupying the anterior and middle mediastinum, compressing the SVC (arrowheads, C–D), resulting in collateral circulation through
the azygos vein (small arrows). The caudal SVC is occluded (arrowhead in D). The arrowheads in A and B show the enhanced right brachiocephalic
vein, distended in A, and compressed and laterally displaced adjacent to the mediastinal mass in B. See Video 83-2 for the full study. (Courtesy Michael
Gotway, MD.)
1495.e2 PART 3 • Clinical Respiratory Medicine
eFigure 83-5 Axial enhanced chest CT in a patient with recurrent hyper-

parathyroidism following previous parathyroidectomy shows an intensely
enhancing anterior mediastinal mass (arrows) representing an ectopic para
thyroid adenoma. (Courtesy Michael Gotway, MD.)
B
eFigure 83-3 Axial unenhanced chest CT (A) and fused 18FDG-PET image
(B) in a patient with Cushing syndrome shows an anterior mediastinal
mass (arrows) in a patient with thymic neuroendocrine malignancy (e.g.,
thymic carcinoma). (Courtesy Michael Gotway, MD.)
eFigure 83-4 Axial enhanced chest CT in a patient with myasthenia gravis

shows an anterior mediastinal mass (arrow) representing thymoma. See eFigure 83-6 Detail lateral chest radiograph in a patient with teratoma
Video 83-3 for the full study. (Courtesy Michael Gotway, MD.) shows a tooth (arrow) in the anterior mediastinum. (Courtesy Michael
Gotway, MD.)
A B
eFigure 83-7 Posterior mediastinal mass due to a neurogenic neoplasm. Frontal (A) and lateral (B) chest radiographs show a circumscribed right-sided
mass (arrows). On the frontal projection (A), note the interface between the mass with the mediastinum—the lesion has significant contact with the
mediastinum, evidenced by the lack of visualization of the medial border of the lesion. The appearance is consistent with a posterior mediastinal lesion.
The lateral radiograph (B) shows the lesion (arrows) projected over the spine, also indicative of a posterior mediastinal location. (Courtesy Michael Gotway, MD.)
A B
eFigure 83-8 Middle mediastinal mass due to a bronchogenic cyst. Frontal (A) and lateral (B) chest radiographs show a circumscribed right-sided
mediastinal mass (arrows). The lateral projection (B) shows the mass is related to the subcarinal space—the most common location for mediastinal bron-
chogenic cysts. (Courtesy Michael Gotway, MD.)
eFigure 83-9 Axial enhanced chest CT in a patient with an anterior medi-

astinal teratoma shows the excellent visualization of calcium within the
lesion. (Courtesy Michael Gotway, MD.)
B
eFigure 83-11 Thymolipoma. Axial enhanced chest CT through the mid
(A) and lower (B) thorax shows a fatty mass occupying the right mediasti-
num. See Video 83-5 for the full study. (Courtesy Michael Gotway, MD.)
eFigure 83-10 Axial enhanced chest CT in a patient with an anterior medi-

astinal teratoma shows the excellent visualization of low attenuation fat
and hyperattenuating calcium within the lesion. See Video 83-4 for the full
study. (Courtesy Michael Gotway, MD.)
B
eFigure 83-12 Omental fat hernia due to foramen of Morgagni hernia. Coronal enhanced chest CT through the anterior thorax shows a fatty mass
occupying the right mediastinum, arising from the abdomen and extending through a diaphragmatic defect (arrowheads) into the thorax. A vessel accom-
panying the fat herniation is clearly visible in C. See Video 83-6 full the full study. (Courtesy Michael Gotway, MD.)
eFigure 83-13 Axial enhanced chest CT in a patient with breast cancer shows left internal mammary lymphadenopathy. (Courtesy Michael Gotway, MD.)
A C
B D
eFigure 83-14 Neurogenic tumor on magnetic resonance (MR) imaging. Axial thoracic T1-weighted (A), T2-weighted (B), coronal enhanced
T1-weighted (C), and sagittal unenhanced T1-weighted (D) MR images show a neurogenic tumor (arrows). Note the tumor extension from the neural
foramen (arrowheads). The lesion shows increased signal on T2-weighted images (B) and intense contrast enhancement (C). (Courtesy Michael Gotway, MD.)
A C
B D
eFigure 83-15 Utility of magnetic resonance imaging (MRI) for determining mediastinal involvement in bronchogenic malignancy. A, Axial unen-
hanced chest CT shows a medial right upper lobe bronchogenic malignancy (arrow, A) with extensive contact with the mediastinum, especially the right
brachiocephalic vein (arrowhead, A). B, Axial thoracic steady-state free precession, unenhanced (C) and enhanced (D) T1-weighted MRI shows extension
of the neoplasm (arrows, B–D) into the mediastinal fat with invasion of the right brachiocephalic vein, evidenced by the small intravascular nodule (arrow-
heads, B–D). See Video 83-8 for the full study of part B. (Courtesy Michael Gotway, MD.)
A B
eFigure 83-16 Typical appearance of

thymoma at thoracic imaging. Frontal
(A) and lateral (B) chest radiographs show
a smoothly circumscribed mediastinal
mass (arrows) at the level of the junction
of the heart and great vessels. The
obscuration of the right heart border on
the frontal projection (A) and the pres-
ence of the mass overlying the heart on D F
the lateral projection (B) indicates that
the mass is adjacent to the heart. C, Axial
enhanced chest CT shows the smoothly
circumscribed, enhancing mediastinal
mass. Axial T1-weighted (D) T2-weighted
(E), enhanced, fat saturation T1-weighted
(F), and coronal T1-weighted (G) images
show the mediastinal lesion (arrows) to
advantage. In particular, the indolent
nature of the mass, evidenced by the
maintained fat plane between the mass
and pericardium (arrowheads, G) is well
shown. The lesion shows intermediate-to-
slightly-decreased signal on T2-weighted
imaging (E), consistent with the cellular
nature of the neoplasm. (Courtesy Michael E G
Gotway, MD.)
eFigure 83-17 Cystic degeneration within a thymoma. Axial enhanced chest CT shows an enhancing anterior mediastinal mass containing internal low
attenuation (arrowheads) consistent with cystic change or necrosis. (Courtesy Michael Gotway, MD.)
A C
B D
eFigure 83-18 Utility of chest CT for thymic neoplasm staging. A–D, Axial enhanced chest CT shows a lobulated mass (large arrows, A–D) with foci of
calcification (arrowheads, C and D). The CT scan shows features consistent with an aggressive neoplasm, including lobulation, mediastinal fat infiltration,
regional lymphadenopathy (double arrowhead, B), and marked vascular compression (small arrows, A and C). Compare the appearance of this aggressive
neoplasm with the more indolent lesion shown in e-Fig. 83-16. (Courtesy Michael Gotway, MD.)
A
eFigure 83-19 Utility of chest CT for thymic neoplasm staging. Axial
chest CT shows a circumscribed anterior mediastinal mass (arrow) in
contact with the pericardium. While the circumscribed nature of the lesion
initially suggests a relatively indolent neoplasm, potentially amenable to
surgical resection, chest CT also shows pleural nodules (arrowheads) in the
left lower medial thorax, representing intrathoracic metastatic disease.
(Courtesy Michael Gotway, MD.)
B
eFigure 83-20 Limited utility of 18FDG-PET for thymic neoplasm evalu-
ation. A, Axial unenhanced chest CT shows an anterior mediastinal mass
(arrow) consistent with a thymic neoplasm; note calcification within the
lesion. B, 18FDG-PET image shows modest glucose utilization within the
tumor (arrow), but the degree of tracer uptake does not exceed that of
mediastinal blood pool (arrowheads). In general, tracer activity must
exceed that seen within mediastinal blood pool to confidently suggest a
malignant process. (Courtesy Michael Gotway, MD.)
A B
C D
eFigure 83-21 Thymic neoplasm recurrence. Frontal chest radiograph (A) and enhanced chest CT (B) at presentation show a left-sided mediastinal mass
(arrows) subsequently shown to represent thymic neoplasia; note the lobulation, cystic change (*), vascular compression and displacement, and calcifica-
tion (arrowhead) on CT scan (B), all suggestive of an aggressive neoplasm. C, Frontal chest radiograph performed months following resection of the thymic
neoplasm shows a new lesion abutting the posterior mediastinal surface of the left superior thorax (C, arrowhead), consistent with tumor recurrence.
D, Frontal chest radiograph several months following C shows an enlarging mediastinal mass (double arrowheads) and enlarging pleural metastatic
deposits (arrow, D).
eFigure 83-22 Chest CT scan of thymic carcinoma. Focused axial

enhanced chest CT shows a lobulated anterior mediastinal mass with foci
of stippled calcification and extensive contact between the mass and
vessels, in particular with obliteration of the fat plane between the mass
and main pulmonary artery. (Courtesy Michael Gotway, MD.)
F
eFigure 83-21, cont’d E and F, Enhanced chest CT confirms pleural meta-
static disease (arrows). (Courtesy Michael Gotway, MD.)
E F G
eFigure 83-23 Neuroendocrine thymic malignancy (“thymic carcinoid tumor”). A, Frontal chest radiograph shows a smoothly circumscribed left-sided
anterior mediastinal mass. B, Axial enhanced chest CT scan shows a solid, homogeneously enhancing left-sided anterior mediastinal mass with contralateral
mediastinal shift, but no other overtly aggressive features. Axial double inversion recovery T1-weighted (C) and T2-weighted (D) magnetic resonance
images also show the lesion and its mass effect, but no locally invasive behavior. The T2-weighted (D) image shows intermediate signal within the lesion,
indicative of a relatively cellular neoplasm. The small “black dot” anterior and medial to the mass represents a flow void within an enlarged mediastinal vein.
E and F, Intraoperative view of the lesion during resection shows the large vein on the surface of the neoplasm; this vascular pedicle (arrows, F) was identi-
fied at surgery and ligated. G, Photograph of the resected specimen. (A to D, Courtesy Michael Gotway, MD; E to G, intraoperative photographs courtesy
Andrew H. Goldstein, MD.)
A B C
eFigure 83-24 Hodgkin lymphoma involving the thymus. A, Frontal chest radiograph shows a large left-sided anterior mediastinal mass. Axial (B) and
coronal enhanced (C) chest CT scans show a minimally lobulated slightly inhomogeneous left-sided anterior mediastinal mass. The appearance is similar
to thymic neoplasia. The pericardial effusion (*) and regional necrotic lymph node enlargement (arrowhead) are indicative of an aggressive process.
(Courtesy Michael Gotway, MD.)
B
eFigure 83-25 Thymic “rebound” hyperplasia. A, Enhanced chest CT in a young patient with breast malignancy at time of diagnosis shows minimal soft
tissue mixed with fat (arrows), consistent with normal residual thymus. B, Axial unenhanced chest CT shows marked increase in the soft tissue within the
anterior mediastinum (arrows) several months following chemotherapy, consistent with “thymic rebound.” The appearance can be difficult to distinguish
from neoplasia. C, Enhanced chest CT 1 year after B shows return to a more normal appearance of the thymus (arrows). (Courtesy Michael Gotway, MD.)
A B
eFigure 83-26 Thymic hyperplasia versus thymoma. A, Unenhanced chest CT in a patient with thymic hyperplasia shows a homogeneous low attenu-
ation, smoothly circumscribed anterior mediastinal lesion (arrow) consistent with thymic hyperplasia; note the morphology of this lesion still resembles
normal thymus. This appearance contrasts with B, the lobulated, irregular appearance of thymoma (arrow). (Courtesy Michael Gotway, MD.)
B
eFigure 83-27 Thymic cyst. A, Axial unenhanced chest CT shows a homogeneously (water density) low attenuation anterior mediastinal lesion (arrows).
Note the cleft in the lesion (arrowheads); this morphology is reminiscent of the normal bilobed thymus. Coronal double inversion recovery unenhanced
T1-weighted (B) and fast spin-echo T2-weighted magnetic resonance (C) images show low and high signal, respectively, within the lesion (arrows), con-
sistent with fluid. This lesion has remained stable for a number of years. Both images show the cleft within the lesion (arrowheads). (Courtesy Michael
Gotway, MD.)
eFigure 83-28 Teratoma. Frontal chest radiograph shows a large right-sided mediastinal mass, representing teratoma. (Courtesy Michael Gotway, MD.)
C
eFigure 83-29 Seminoma. A, Frontal chest radiograph shows a large right-sided mediastinal mass. B–D, Axial enhanced chest CT scans show a mildly
inhomogeneous, lobulated mass (arrows) with compression and displacement of the right brachiocephalic vein (arrowhead), resulting in extensive azygos
vein (small arrows, B and C) collateral circulation. See Video 83-9 for the full study. (Courtesy Michael Gotway, MD.)
C
eFigure 83-30 Embryonal cell carcinoma. A, Frontal chest radiograph shows a circumscribed left-sided anterior mediastinal mass (arrows). B-D, Axial
enhanced chest CT shows a heterogeneous anterior mediastinal mass that obliterates fat planes with the adjacent vessels. Internal low attenuation within
the tumor, as seen in the example, is more commonly encountered as non-seminomatous mediastinal germ cell neoplasms than seminomas. See Video
83-10 for the full study. (Courtesy Michael Gotway, MD.)
B D
C E
eFigure 83-31 Anterior mediastinal extension of a thyroid goiter. A, Frontal chest radiograph shows widening of the superior mediastinum with
rightward shift of the trachea resulting from a mediastinal mass (arrows). The superior mediastinal widening extends into the base of the neck. B–E, Axial
unenhanced chest CT shows enlargement of the cervical thyroid gland (double arrowheads, A). The anterior superior mediastinal mass (arrows, C and E)
extends from the base of the neck, originating from, and in contact with, the cervical thyroid gland. The mass contains calcification (single arrowhead, D),
a common finding in thyroid goiters. Continuity of a mediastinal lesion with the cervical thyroid gland is key for confidently establishing a mediastinal
mass as thyroidal in origin. (Courtesy Michael Gotway, MD.)
eFigure 83-33 Middle mediastinal extension of a thyroid goiter. Axial

enhanced chest CT shows a peripherally hyperenhancing mass with
central necrosis posterior to the trachea. This lesion was in contact with
thyroid tissue at the base of the neck, establishing it as mediastinal goiter
extension. (Courtesy Michael Gotway, MD.)
A
C
eFigure 83-32 Ectopic mediastinal thyroid tissue. A, Planar frontal
99m-technetium sestamibi scintigraphy shows faint tracer uptake within
the mediastinum (arrow), separate from the heart (H) and liver (L). Axial
enhanced chest CT shows a heterogeneously enhancing anterior medias-
tinal mass (arrow). C, Axial CT image through the cervicothoracic junction
shows absence of a normal thyroid gland (arrowheads, C). (Courtesy Michael
Gotway, MD.)
A C
B D
E F
eFigure 83-34 Ectopic anterior mediastinal parathyroid adenoma. Axial unenhanced (A) and axial (B), coronal (C), and sagittal (D) enhanced chest
CT shows an intensely enhancing lesion (arrow) in the anterior mediastinum. E, Transverse sonography performed at the level of the suprasternal notch
shows a hypoechoic nodule (arrow) corresponding to the CT abnormality. F, Planar frontal 99m-technetium sestamibi scintigraphy shows avid tracer
accumulation within the anterior mediastinal lesion (arrow), confirming ectopic parathyroid adenoma. (Courtesy Michael Gotway, MD.)
B D
C E
eFigure 83-35 Mediastinal lymphangioma. Frontal chest radiograph in a 13-year-old boy shows a circumscribed left mediastinal mass without medi-
astinal shift. B and C, Axial enhanced chest CT shows an anterior and superior low attenuation mediastinal lesion surrounding, but not compressing, the
left brachiocephalic vein (arrows); this behavior indicates that the lesion is relatively “soft.” Axial fat saturation T1-weighted (D) and T2-weighted (E)
magnetic resonance images show a mass with heterogeneous signal intensity containing multiple fluid-fluid levels (arrowheads). The dependent signal
within the areas of fluid-fluid level formation is bright on both T1- and T2-weighted images, consistent with blood. (Courtesy Michael Gotway, MD.)
eFigure 83-36 Anterior mediastinal lipoma. Axial enhanced chest CT

shows an encapsulated fat-containing lesion (arrow) consistent with a
small lipoma. (Courtesy Michael Gotway, MD.)
B
eFigure 83-37 Mediastinal liposarcoma. A, Axial chest CT shows a predominantly fatty attenuating mass, representing well-differentiated liposarcoma.
B, Axial chest CT shows a mediastinal mass again containing fat, but with considerably more soft tissue than the well-differentiated lesion in A, represent-
ing moderately differentiated mediastinal liposarcoma. C, Axial chest CT shows a large inhomogeneous mass containing fat, but also with soft tissue
components and extensive vascularity, representing poorly differentiated mediastinal liposarcoma. (Courtesy Michael Gotway, MD.)
B D
C E
eFigure 83-38 Mediastinal lipomatosis. A, Frontal chest radiograph shows widening of the superior mediastinum (arrows), but the contours of the
mediastinum are not lobulated, nor is the mediastinum abnormally dense. B–E, Axial enhanced chest CT shows abundant, unencapsulated mediastinal
fat (*) on both sides of midline, without significant nodularity. (Courtesy Michael Gotway, MD.)
A D
B E
C F
eFigure 83-39 Mediastinal lymphadenopathy in a patient with sarcoidosis. A–F, Axial enhanced chest CT shows mildly enlarged lymph nodes in
multiple mediastinal stations: A, highest right paratracheal nodes (station 1R, arrow); B, upper right paratracheal nodes (station 2R, arrow), C–E, lower
right paratracheal nodes (station 4R, arrows); D, lower left paratracheal nodes (station 4L, thin arrow, D) and aortopulmonary window nodes (station 5,
arrowhead, D); F, subcarinal nodes (station 7, double arrowheads, F). (Courtesy Michael Gotway, MD.)
B D
C E
eFigure 83-40 Angiofollicular lymphoid hyperplasia (Castleman disease). A, Frontal chest radiograph shows a right paratracheal mass in a patient
with an anterior chest wall stab wound, with penetrating entry site on the skin marked by a paper clip. B, Axial enhanced chest CT shows an enhancing
right paratracheal mass with small foci of calcification, shown to reflect angiofollicular lymphoid hyperplasia at surgical resection. C, Axial enhanced chest
CT (C), T1-weighted (D) and T2-weighted (E) magnetic resonance images in a different patient show a posterior mediastinal mass (arrows) shown to reflect
angiofollicular lymphoid hyperplasia at surgical resection. (Courtesy Michael Gotway, MD.)
B Presentation C 5 yrs prior to presentation D 7 yrs prior to presentation
eFigure 83-41 Growth of a bronchogenic cyst over time. A, Frontal chest radiograph shows a smoothly circumscribed lesion (arrow) in the left superior
mediastinum. B–D Retrospective review of serial chest radiographs obtained over the previous 7 years shows slow enlargement of the lesion.
E Presentation F 2 yrs prior to presentation G 2 yrs prior to presentation
H 2 yrs prior to presentation I 6 yrs prior to presentation J 7 yrs prior to presentation
eFigure 83-41, cont’d E–J, Axial chest CT shows serial enlargement of the lesion over a 7-year period. (Courtesy Michael Gotway, MD.)
D
eFigure 83-42 Pericardial cyst. A, Frontal chest radiograph shows a smoothly circumscribed right cardiophrenic angle mass (arrow). B, Axial enhanced
chest CT shows a homogeneous water density mass (arrow) abutting the right pericardium. Axial double inversion recovery T1-weighted (C) and axial
triple inversion recovery (D) magnetic resonance images show fluid signal within the mass (arrows). Triple inversion recovery sequences (D) suppress fat
signal, with water signal appearing bright (note the high signal of the cerebrospinal fluid posteriorly). The slight “shading” of the signal intensity posteriorly
within the pericardial cyst may reflect debris or hemorrhage. (Courtesy Michael Gotway, MD.)
eFigure 83-44 Extramedullary hematopoiesis. Enhanced chest CT in a

patient with thalassemia shows enhancing paravertebral soft tissue
(arrows), representing extramedullary hematopoiesis. (Courtesy Michael
Gotway, MD.)
A
C
eFigure 83-43 Schwannoma. A, Frontal chest radiograph shows a
smoothly circumscribed lesion (arrow) at the right thoracic apex. The
extensive contact with the chest wall and lack of visualization of the supe-
rior border of the lesion (often referred to as the “incomplete border” sign)
are consistent with a non–lung parenchymal origin. Axial (B) and coronal
(C) enhanced chest CT shows a homogeneous low-attenuation lesion
(arrows) in contact with the right superior mediastinum and chest wall. The
lesion was shown to reflect schwannoma. See Video 83-11 for the full
studies. (Courtesy Michael Gotway, MD.)
A
eFigure 83-46 Mediastinal mass caused by aortic aneurysm. Frontal
chest radiograph shows a huge left-sided mediastinal mass without visu-
alization of a normal thoracic aorta. See Video 83-13 for an enhanced chest
CT showing a huge aneurysm of the descending thoracic aorta with mural
thrombus. (Courtesy Michael Gotway, MD.)
C
eFigure 83-45 Extramedullary hematopoiesis containing fat. A, Frontal
chest radiograph in a patient with anemia shows an abnormal right-sided
posterior mediastinal contour (arrows); a similar abnormal contour can be
appreciated in the left retrocardiac region. The posterior mediastinal loca-
tion is suggested by the relatively long contour abnormality without
obscuration of the right heart border or lateral displacement of the right
hilum. B and C, Axial enhanced chest CT shows bilateral posterior medias-
tinal masses (arrows) mixed with some areas of low attenuation, the latter
consistent with fat. See Video 83-12 for the full study. (Courtesy Michael
Gotway, MD.)
A B
eFigure 83-47 Mediastinal mass caused by superior vena cava aneurysm. A, Frontal chest radiograph shows a right-sided mediastinal mass (arrow).
Patient had undergone Glenn shunt (SVC-to-PA) for double outlet right ventricle. B, Axial enhanced chest CT shows a 6-cm aneurysm of the superior vena
cava (arrow). (Courtesy Michael Gotway, MD.)
eFigure 83-48 Mediastinal mass

caused by enlargement of the azygos
vein. A, Frontal chest radiograph shows a D
mass (arrow) in the right tracheobronchial
angle; this is the normal position of
the anterior portion of the azygos vein.
B–D, Axial enhanced chest CT shows
dense contrast enhancement of the
enlarged azygos vein (arrows). See Video
83-14 for the full study. (Courtesy Michael C
Gotway, MD.)
A B
eFigure 83-49 Mediastinal mass caused by esophageal varices. A, Frontal chest radiograph shows bilateral paraspinous line displacement (arrows)
consistent with a posterior mediastinal mass. B, Axial enhanced chest CT shows that the posterior mediastinal mass is caused by periesophageal varices
(arrows). (Courtesy Michael Gotway, MD.)
E F
eFigure 83-50 Foramen of Morgagni hernia. A, Frontal chest radiograph shows a smoothly circumscribed right cardiophrenic angle mass. Axial (B-D)
and sagittal (E and F) enhanced chest CT scans show that the right cardiophrenic angle mass (*) contains fat and originates within the abdomen, herniat-
ing into the thorax through an anteromedial diaphragmatic defect (arrowheads, D and F). Note that vessels, seen as thin linear hyperattenuating streaks,
can be seen accompanying the fat herniation (D). See Video 83-15 for the full axial study. (Courtesy Michael Gotway, MD.)
A B
D
eFigure 83-51 Foramen of Bochdalek hernia. Frontal (A) and lateral (B) chest radiographs show a smoothly circumscribed contour abnormality along
the posterior left diaphragm (arrow, B). The finding is difficult to appreciate on the frontal chest radiograph (A), but is readily seen on the lateral projection
(arrow, B). Axial (C and D) and coronal (E) unenhanced chest CT shows that the lesion (*) consists entirely of fat, originating from the abdomen and
extending into the posteromedial thorax through a diaphragmatic defect (arrow, C, and arrowheads, E). (Courtesy Michael Gotway, MD.)
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Mehu107 - U3 - t1 - Mediastinals Tumors and Cysts

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SECTION O

DISORDERS OF THE MEDIASTINUM

INTRODUCTION Magnetic Resonance Imaging SPECIFIC MEDIASTINAL TUMORS AND

describes the normal anatomy and contents of the medias-

rior and may contain areas of calcium (sometimes teeth or

in patients with a primary mediastinal mass13-15 or with

MAGNETIC RESONANCE IMAGING

Table 83-4 Disorders Presenting as a Mass in the Mediastinum

may be due to lesions more commonly found in another

Table 83-5 Relative Frequencies of Mediastinal Masses in

A *Based on Silverman and Sabiston’s review of reported mediastinal masses

thymoma usually distorts the gland’s normal shape and

on the histologic grade and the anatomic stage, is generally

surgically resistant hyperparathyroidism.108 Half of ectopic

Complete reference list available at ExpertConsult.

eFIGURE IMAGE GALLERY

eFigure 83-5 Axial enhanced chest CT in a patient with recurrent hyper-

eFigure 83-4 Axial enhanced chest CT in a patient with myasthenia gravis

eFigure 83-9 Axial enhanced chest CT in a patient with an anterior medi-

eFigure 83-10 Axial enhanced chest CT in a patient with an anterior medi-

eFigure 83-16 Typical appearance of

eFigure 83-22 Chest CT scan of thymic carcinoma. Focused axial

eFigure 83-33 Middle mediastinal extension of a thyroid goiter. Axial

eFigure 83-36 Anterior mediastinal lipoma. Axial enhanced chest CT

B Presentation C 5 yrs prior to presentation D 7 yrs prior to presentation

E Presentation F 2 yrs prior to presentation G 2 yrs prior to presentation

H 2 yrs prior to presentation I 6 yrs prior to presentation J 7 yrs prior to presentation

eFigure 83-44 Extramedullary hematopoiesis. Enhanced chest CT in a

eFigure 83-48 Mediastinal mass

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