The mediastinum includes the organs located between the
two lungs. Because the mediastinum is invested by the medial parietal
pleura, a mass isolated to the mediastinum will generally have a smooth
contour (created by the pleural surface). Lung parenchymal masses, on
the other hand, are not surrounded by pleura and may therefore have an
irregular contour. Mediastinal masses can be recognized on chest
radiographs when there is an abnormal contour to the normal mediastinal
structures. On the right, these normal structures include, from
superior to inferior, the brachiocephalic vessels, superior vena cava,
azygos arch, ascending aorta, and right atrium. On the left, the
mediastinal contour, from superior to inferior, is made up of the
brachiocephalic vessels, aortic arch, main pulmonary artery, and left
ventricle. On the lateral chest radiograph, the mediastinum extends
from the inner margin of the sternum to the inner margins of the
posterior ribs.
The mediastinum can be divided into compartments, and
many classification systems have been devised to simplify the
differential diagnosis when an abnormality is seen in one or more
compartments. A system based on anatomic subdivisions (the superior,
anterior, middle, and posterior mediastinal compartments) will be used
in this chapter (Fig. 6-1). Approximately 60%
of all mediastinal masses arise in the anterior mediastinum, 25% appear
in the posterior mediastinum, and 15% occur in the middle mediastinum (1).
When an abnormality is not isolated to one mediastinal compartment, as
is often the case with large mediastinal masses, the list of diagnostic
possibilities can be determined by localizing the abnormality to the
mediastinal compartment serving as the "epicenter" of the abnormality
or by considering all abnormalities that occur in the compartments
involved. Associated radiologic findings can also help to narrow the
list of diagnostic possibilities; these include deviation of the
trachea (commonly seen with thyroid masses); presence of axillary,
abdominal, and retroperitoneal adenopathy (suggesting the diagnosis of
lymphoma); or posterior rib erosion or destruction (consistent with a
posterior mediastinal mass, such as a neurogenic tumor).
Anterior Mediastinum
Also referred to as the prevascular space,
the anterior mediastinum is bounded above by the superior mediastinum,
laterally by pleura, anteriorly by the sternum, and posteriorly by
pericardium and great vessels. This compartment contains areolar
tissue, lymph nodes, lymphatic vessels, thymus gland, thyroid gland,
parathyroid glands, and internal mammary arteries and veins.
Masses occurring in the anterior mediastinum are listed in Table 6-1. In addition to the "4 Ts" (thymoma, thyroid mass, teratoma, and terrible
lymphoma), which account for the great majority of lesions, there are
many other less common causes of an anterior mediastinal mass. These
include vascular tortuosity or aneurysm, cardiac tumors or prominent
pericardiac fat (Fig. 6-2), cystic hygroma, bronchogenic cyst, pericardial cyst, hemangioma, lymphangioma, parathyroid adenoma (Fig. 6-3),
various other mesenchymal tumors (e.g., fibroma or lipoma), sternal
tumor, primary lung tumor invading the anterior mediastinum, Morgagni
hernia (Fig. 6-4), abscess (Fig. 6-5), and mediastinal lipomatosis (Fig. 6-6).
An intrathoracic
thyroid
mass is usually a benign multi-nodular goiter that originates in the
neck and extends downward into the mediastinum through the thoracic
inlet. This continuity is an important diagnostic feature on chest
radiography. Many thyroid masses displace or narrow the trachea (
Fig. 6-7).
Another useful sign of a thyroid mass is the relative high attenuation
value of the thyroid tissue, at least 20 Hounsfield units above that
seen in adjacent muscles on both precontrast and postcontrast computed
tomographic (CT) images (
2). CT scans can show
cystic components. Calcification is common and usually caused by benign
disease when it is dense, amorphous, and well defined with a nodular,
curvilinear, or circular configuration. Distinguishing between benign
and malignant thyroid masses on chest radiography or CT scanning is not
possible unless the tumor has clearly spread beyond the
thyroid
gland (indicating malignancy). Malignant thyroid masses can also have
calcifications, generally with a configuration of fine dots grouped in
a cloudlike formation (
3,
4,
5).
However, the patterns of benign and malignant calcifications serve as
general guidelines, and malignant medullary thyroid carcinoma can
contain well-defined, occasionally ring-shaped, dense calcifications
that are similar in appearance to the calcifications seen with benign
thyroid goiter. Radionuclide imaging is a very sensitive and specific
method of determining the thyroid nature of an intrathoracic mass (
Fig. 6-8), but CT provides more information about the mass.
Thymomas are tumors consisting of thymic epithelial cells and reactive lymphocytes, with noninvasive or invasive patterns of growth (
6).
The presence or absence of tumor spread beyond the capsule (usually
determined surgically), rather than the histologic appearance within
the thymus, determines whether a tumor is benign or malignant. Thymomas
occur at an average age of 50 years and with equal frequency in men and
women (
6). They are associated with a variety
of autoimmune diseases, most notably myasthenia gravis. Approximately
40% of patients with thymoma have myasthenia gravis, and the incidence
of thymoma in patients with this disease is approximately 15% (
7). Most thymomas arise in the upper anterior mediastinum, anterior to the ascending aorta above
the right ventricular outflow tract and main pulmonary artery (
Fig. 6-9).
They can extend into the adjacent middle or posterior mediastinum, and
they can occur or extend into the lower third of the mediastinum, as
low as the cardiophrenic angles. Punctate, curvilinear, or ringlike
calcification is common in both benign and invasive thymomas (
8).
On a CT scan, thymomas are usually of homogeneous attenuation and show
uniform enhancement, but rarely they can appear cystic with discrete
nodular components (
Figs. 6-10 and
6-11).
In patients under age 40, diagnosing a small thymoma can be difficult
because the normal gland is variable in size. A normal thymus, in
contrast to a thymic mass, conforms to the shape of the adjacent great
vessels on CT and magnetic resonance imaging (MRI). A mass gives rise
to focal thymic enlargement, usually with its center away from the
midline, whereas a normal gland is approximately symmetric and
maintains a somewhat triangular shape on axial imaging. Invasive
thymomas inhabit the mediastinal fat, spreading to the pericardium and
pleura. Unless mediastinal invasion has occurred, distinguishing benign
from invasive thymoma is not possible with CT scanning. Transpleural
spread may manifest as so-called "drop metastases" at a site distant
from the primary lesion (
Fig. 6-12), and
imaging of the entire pleural space and upper abdomen is therefore
important. Extensive pleural involvement may mimic malignant
mesothelioma. Other less common thymic masses include cyst (
Fig. 6-13),
abscess, thymolipoma, malignant lymphoma (most notably Hodgkin
lymphoma), thymic carcinoid, germ cell tumors, and thymic carcinoma (
Fig. 6-14).
A teratoma is a neoplasm
derived from more than one embryonic germ layer. Other germ cell tumors
include benign dermoid cyst, malignant seminoma (the most common germ
cell tumor), teratocarcinoma, embryonal carcinoma, endodermal sinus
tumor (yolk sac tumor), choriocarcinoma, and mixtures of these types.
Germ cell tumors in the mediastinum arise from primitive rest cells and
generally are not metastatic from gonadal tumors. Some malignant germ
cell tumors secrete beta-human chorionic gonadotropin and
alpha-fetoprotein, which can be used to diagnose and monitor
progression of disease.
Benign teratomas are found in patients of all ages but are most common in adolescents and young adults (
Fig. 6-15).
They usually produce a well-defined, rounded, or lobulated mass in the
anterior mediastinum. They grow slowly, although rapid increase in size
may occur as a result of hemorrhage, producing imaging features
suggestive of a malignant mass.
Calcification,
ossification, teeth, or fat may be visible on a chest radiograph and on
CT scans. CT scans may show cystic components and/or a fat–fluid level.
A cyst wall with curvilinear calcification is often present.
Unequivocal fat within the mass confirms the diagnosis of teratoma, but
the absence of fat or calcium does not exclude a teratoma (
Fig. 6-16).
Imaging features of malignant germ cell tumors are
similar to those of benign teratoma except that fat density is not
noted and calcification is rare. The malignant tumors grow rapidly, and
metastases may be seen in the lungs, bones, or pleura. The adjacent
mediastinal fat planes may be obliterated. The tumors may be of
homogeneous attenuation or show areas of contrast enhancement
interspersed with rounded areas of decreased attenuation from necrosis
and hemorrhage. Rarely, coarse tumor calcification may be seen (9).
Lymphoma often extends
beyond the anterior mediastinal compartment, involving a variety of
lymph node chains, and is discussed along with middle mediastinal
masses (see following). When lymphoma is isolated to the anterior
mediastinum, the CT appearance can be similar to that of thymoma and
germ cell neoplasms.
Middle Mediastinal Masses
Also referred to as the vascular space,
the middle mediastinum is bounded in front by the anterior mediastinum
and posteriorly by the posterior mediastinum. The middle mediastinum
contains the heart and pericardium, ascending and transverse aorta,
superior vena cava and azygos vein that empties into it, phrenic
nerves, upper vagus nerves, trachea and its bifurcation, main bronchi,
pulmonary artery and its two branches, pulmonary veins, and adjacent
lymph nodes. The esophagus is variably described as a middle or
posterior mediastinal structure. The main categories of abnormalities
occurring in the middle mediastinum include adenopathy,
aneurysm/vascular abnormalities, and abnormalities of development (Table 6-2). Less common middle mediastinal abnormalities include giant lymph node hyperplasia (Castleman disease; Fig. 6-17); neural tumor (involving the vagus or phrenic nerve); abscess; fibrosing mediastinitis; hiatal hernia (Fig. 6-18); primary tumors of the trachea or esophagus (namely, leiomyoma, leiomyosarcoma, or carcinoma; Figs. 6-19 and 6-20); and hematoma.
Adenopathy
There are many causes of mediastinal and hilar lymph
node enlargement (adenopathy). Three main categories to consider are
neoplasm, infection, and noninfectious granulomatous disease (i.e.,
sarcoidosis). Neoplastic causes include malignant lymphoma,
lymphoproliferative disorders, leukemia, and metastatic carcinoma (most
notably from the lung, esophagus, breast, kidney, testis, and head and
neck).
Lymphoma is classified as either Hodgkin or non-Hodgkin lymphoma (Figs. 6-21 and 6-22).
The onset of Hodgkin lymphoma most commonly occurs in the second or
third decade, with a secondary peak in the fifth or sixth decades of
life. Non-Hodgkin lymphoma occurs in all age groups. The main feature
of malignant lymphomas on chest radiography or CT scanning is
mediastinal and hilar lymphadenopathy, in some cases with accompanying
pulmonary, pleural, or chest wall involvement. The enlarged nodes can
calcify, especially after therapy, in irregular, eggshell, or diffuse
patterns. The appearances of intrathoracic adenopathy on chest
radiographs and CT scans are similar in Hodgkin and non-Hodgkin
lymphoma, but the frequencies and distributions of the abnormalities
differ. Any intrathoracic nodal group may be enlarged in patients with
lymphoma. In general, the anterior mediastinal and paratracheal nodes
are the most frequently involved, with tracheobronchial and subcarinal
nodes also commonly enlarged. The majority of cases of Hodgkin lymphoma
show enlargement of two or more nodal groups, whereas only one nodal
group is involved in about half the cases of non-Hodgkin lymphoma.
Hilar adenopathy is rare without accompanying mediastinal adenopathy.
The enlarged nodes may be discrete or matted together, and the margins
can be well defined or ill defined. Low-density areas can be seen,
resulting from cystic degeneration. Hodgkin lymphoma can arise
primarily in the thymus. Parenchymal involvement of the lung at initial
presentation is unusual. Parenchymal involvement in Hodgkin disease is
almost always accompanied by intrathoracic adenopathy (except after
irradiation), whereas in non-Hodgkin lymphoma, isolated pulmonary
involvement occurs more than 50% of the time (10).
The most common pattern of parenchymal involvement is one or more
discrete nodules, which can cavitate. Another common pattern is round
or segmental, focal or patchy areas of dense airspace opacity often
with air bronchograms, mimicking pneumonia.
Pleural effusions are seen on CT scans in 50% of patients with lymphoma (11).
Most pleural effusions are unilateral exudates, occasionally chylous in
nature, and they can be large. Both pleural and pericardial effusions
can occur and have nodular solid components. Chest wall invasion and
rib destruction are seen on occasion.
The most frequent
infections
that give rise to intrathoracic adenopathy are caused by mycobacterial
disease (most notably tuberculosis) and fungal disease (particularly
histoplasmosis;
Fig. 6-23), each of which can occur without evident pneumonia. Some patients develop a chronic progressive immune
response to dead
Histoplasma
capsular antigens, resulting in a condition known as fibrosing
mediastinitis. In this condition, nonmalignant fibrous tissue encases
and obliterates vasculature (arteries, veins, lymphatics) and airways
in the mediastinum (
Fig. 6-24). Subcarinal and
right paratracheal nodes are most commonly involved. Calcification of
nodes and simultaneous encasement of airways and vasculature are
characteristic CT findings of this disease. Intrathoracic nodal
enlargement can also be seen in tularemia, whooping cough, anthrax,
plague, and mycoplasmal, viral, and other more common bacterial
infections.
Sarcoidosis is a frequent
cause of intrathoracic adenopathy in young adults. When multiple node
groups are involved and adenopathy is symmetrically distributed in the
hila and mediastinum in young asymptomatic adults, sarcoidosis is the
likely cause. The hilar lymph nodes are frequently potato shaped and
clear of the cardiac borders, a feature that is often useful in
distinguishing sarcoidosis from lymphoma (
Fig. 6-25).
Enlargement of paratracheal and bilateral hilar lymph nodes (the
Garland triad, or the "1-2-3 sign") is a nonspecific pattern of
adenopathy that is common in patients with sarcoidosis (
Fig. 6-26).
Adenopathy is more easily identified and quantified via
CT scanning than chest radiography. The CT signs of adenopathy are (a)
an increase in size of individual nodes, (b) invasion of surrounding
mediastinal fat, (c) nodal masses, and (d) diffuse soft tissue density
throughout the mediastinum obliterating the mediastinal fat. There are
numerous studies regarding normal lymph node size on CT scans (12,13,14).
In general, nodes greater than 10 mm in short-axis diameter are
considered abnormal although nonspecific and not necessarily malignant.
Likewise, nodes less than 10 mm in short-axis diameter can be
pathologic.
Aneurysm/Vascular Abnormalities
As a structure in the middle mediastinum, the aorta can
also give rise to abnormalities in this compartment. It is very
important to distinguish an aortic aneurysm from other mediastinal
masses, particularly if biopsy is being considered. The aorta commonly
becomes atherosclerotic and ectatic with advancing
age, and it can become aneurysmal. Rupture is the feared complication of aortic aneurysm (
Fig. 6-27).
Most atherosclerotic aneurysms are fusiform in shape, although some are
saccular. Fusiform aneurysms usually arise in the aortic arch or
descending thoracic aorta. Saccular aneurysms usually arise from the
descending aorta, or occasionally from the aortic arch, but they are
unusual in the ascending aorta. Aortic aneurysms can be distinguished
from other mediastinal masses by recognizing their conformity to the
aorta and by the presence of curvilinear calcification in the wall of
the aneurysm. Traumatic aortic pseudoaneurysm is discussed in
Chapter 8.
Mycotic aneurysms of the aorta occur in patients with predisposing
causes: intravenous drug abusers, patients with valvular disease or
congenital disorders of the heart or aorta, patients who have undergone
previous cardiac or aortic surgery, patients with adjacent pyogenic
infection, and those who are immunocompromised. Mycotic aneurysms are
usually saccular in shape, enlarge rapidly, and lack calcification of
the wall.
Aortic dissections are collections of blood within the
media of the aortic wall that communicate with the true aortic lumen
through one or more tears in the intima. Most dissections begin within
an intimal tear, and bleeding splits the aortic media. Two
classification systems are used to describe aortic dissection. The
DeBakey classification divides aortic dissection into three types (15).
Type I refers to dissections that start in the ascending aorta and
extend into the descending aorta; type II, to dissections confined to
the ascending aorta; and type III, to dissections that start just
beyond the left subclavian artery and are confined to the descending
aorta. The Stanford classification refers to type A (involving the
ascending aorta) and type B (confined to the descending aorta) (16). The diagnostic feature of aortic dissection on contrast-enhanced CT scanning is two lumina separated by an intimal flap (Fig. 6-28).
The intimal flap is seen as a curvilinear low-attenuation area within
the opacified aorta. A false lumen usually fills and empties in a
delayed fashion compared with a true lumen. The false lumen may be
partially or totally filled by thrombus and therefore may not opacify.
The true lumen is usually compressed by the false lumen. Displacement
of calcified atheromatous plaques by the dissection can be demonstrated
on precontrast CT scans when contrast enhancement of the two lumina
cannot be achieved (as with a thrombosed false lumen). Aortic
dissection is further discussed along with other aortic pathology in Chapter 19.
Abnormalities of Development
Developmental mediastinal cysts include bronchogenic
cysts, esophageal duplication cysts, neurenteric cysts, and pericardial
cysts. The first three are also referred to as bronchopulmonary foregut
malformations, signifying their origin from the embryologic foregut as
the result of abnormal ventral budding of the tracheobronchial tree (17).
Mediastinal cysts containing cartilage are classified as bronchogenic,
and those with gastric epithelium as enteric. Neurenteric and some
esophageal duplication cysts arise within the posterior mediastinum,
but they will be discussed here with middle mediastinal masses.
Bronchogenic cysts have a
fibrous capsule, often contain cartilage, are lined with respiratory
epithelium, and contain mucoid material. Most arise in the mediastinum
or hilar areas, but they can also arise within the lung parenchyma.
These cysts can rapidly increase in size as a result of hemorrhage,
infection, or distension with air, indicating communication with the
airways. They are seen on chest radiographs as well-defined
solitary masses in the mediastinum or hilum (
Figs. 6-29 and
6-30)
and are usually found in close proximity to the major airways. The
single most frequent site is between the carina and the esophagus.
Calcification, either rim calcification or milk of calcium within the
cyst, has been described (
18). CT scans usually
show a simple cystic mass with an imperceptible or thin smooth wall.
The CT attenuation is generally that of water (-10 to +10 Hounsfield
units) but can be higher (as high as 120 Hounsfield units) when filled
with milk of calcium or proteinaceous material that accumulates after
infection or hemorrhage (
Fig. 6-31). The cysts can be unilocular or multilocular. Curvilinear calcification of the wall can be seen.
The imaging features of esophageal duplication cysts
may be identical to those seen with bronchogenic cysts, except that an
esophageal duplication cyst will always have a peri-esophageal location
(Fig. 6-32). Neurenteric cysts are posterior
mediastinal cystic lesions connected to the meninges through a midline
defect in one or more vertebral bodies. Associated vertebral anomalies
suggest the diagnosis. Pericardial cysts
arise most frequently in the right cardiophrenic angle as a result of
anomalous outpouching of the parietal pericardium. The cysts typically
contact the heart, diaphragm, and anterior chest wall (Fig. 6-33). The majority are sharply marginated, somewhat triangular, and of near-water attenuation on CT scans (Fig. 6-34).
Posterior Mediastinal Masses
Also referred to as the postvascular space,
the posterior mediastinum lies behind the heart and pericardium and
contains the thoracic descending aorta, esophagus, thoracic duct,
azygos and hemiazygos veins, lymph nodes, sympathetic chains, and
inferior vagus nerves. Neural tumors are the most common tumors to
develop in the posterior mediastinum, but a variety of uncommon
abnormalities can occur in this compartment, as listed in Table 6-3 (Figs. 6-35, 6-36, 6-37, 6-38).
Neural tumors can be differentiated into nerve sheath
tumors and ganglion cell tumors. Nerve sheath tumors comprise
schwannomas, neurofibromas, and their malignant counterparts. The
schwannoma is the most common intrathoracic
nerve sheath tumor (
Fig. 6-39).
Both schwannomas and neurofibromas are derived from Schwann cells and
occur most commonly in patients in their 30s and 40s. Almost all
intrathoracic nerve sheath tumors arise from either the intercostal or
sympathetic nerves.
The ganglion cell tumors include neuroblastoma
(malignant), ganglioneuroma (benign), and ganglioneuroblastoma
(intermediate between benign and malignant). The adrenal gland is the
most common primary site for these tumors, with the mediastinum being
the second most common site. Ganglioneuroma can occur between the ages
of 1 and 50, while neuroblastoma and ganglioneuroblastoma occur during
childhood, generally under the age of 20 (19).
Nerve sheath and ganglion cell tumors are seen as
well-defined masses with a smooth or lobulated outline on imaging
studies. Some are very large and can occupy most of a hemithorax.
Calcification can be seen in all types. In neuroblastoma, the
calcification is usually finely stippled, whereas in
ganglioneuroblastoma and ganglioneuroma it is denser and coarser. The
bone adjacent to the tumor shows a scalloped edge, with preservation
but thickening of the bony cortex. The ribs can be thinned and splayed
apart, and the intervertebral foramina can appear widened. On CT scans,
many tumors have mixed attenuation, including low-attenuation regions,
that enhance on images taken after administration of intravascular
contrast material.
Paragangliomas are tumors of the paraganglionic cells
and can be benign or malignant chemodectomas or pheochromocytomas.
Mediastinal paragangliomas are rare, comprising only 2% of the large
series of neural tumors of the thorax (19).
Paragangliomas occur in the area of the aortic arch and are classified
as aortic body tumors. They form rounded soft tissue masses that are
extremely vascular and enhance brightly on CT scans after
administration of intravenous contrast material.