The Core Curriculum: Cardiopulmonary Imaging, 1st Edition (2004)

Chapter 8. Other Thoracic Neoplasms

This chapter covers a variety of benign and malignant thoracic neoplasms, arising with the airway, lung parenchyma, esophagus, and the lymphatic system, as well as metastases to the thorax. Lung cancer was discussed in Chapter 7, pleural neoplasms are discussed in Chapter 17, and the rare tumors of the vascular system are discussed briefly in Chapters 1820, and 21. A full discussion of all these neoplasms could easily be the topic of an entire text unto itself. What you will read here is a discussion of the more common of these entities and, when possible, specific imaging features that can be helpful in distinguishing one from another.

Tracheobronchial Neoplasms

Benign and malignant tracheobronchial neoplasms are rare and vary widely histologically. When they occur in the trachea, they may present with a nonproductive cough due to irritation, hemoptysis if they bleed, or wheezing and/or dyspnea due to airway obstruction. When they occur in the bronchi, they may also cause postobstructive pulmonary infection. Recurrent infection, especially within the same lobe or segment, is suggestive of an endobronchial lesion.

Tracheobronchial neoplasms often present with stridor and wheezing.

On chest radiographs, lobar or segmental airspace disease, obstructive pneumonitis, or atelectasis may be seen. Abscess or bronchiectasis may also develop when the obstruction is longstanding. Endobronchial lesions may create a ball–valve phenomenon, with an open airway at inspiration and a closed airway in early expiration, creating hyperinflation and obstructive emphysema distal to the occlusion. Although sometimes visible on an inspiratory radiograph, this phenomenon is best recognized on expiratory or ipsilateral decubitus radiographs. The tumor itself is uncommonly identified on the chest radiograph; however, the tumor, extent of tumor spread, and associated findings are readily identified on computed tomography (CT). Characteristically, the recognition of malignant lesions is based on the following triad:

1. Abnormal or obstructed airways;

2. A central mass, causing distinct bulging of the proximal contour of the collapsed lobe or segment;

3. Differential enhancement of tumor versus collapsed peripheral lung after administration of intravenous contrast (1).

CT with multiplanar reconstructions is the preferred imaging modality for evaluating airway lesions (Chapter 16). Inspiratory and expiratory CT examinations are usually performed to evaluate the impact of the lesion on the airway during the respiratory cycle. Intravenous contrast is usually administered for better evaluation of the neoplasm with respect to adjacent mediastinal structures, particularly when there is extension outside the airway (2,3,4). Many of the benign and malignant tracheobronchial neoplasms can also arise within the small bronchi of lung parenchyma, and some tumors of the same cell type may arise from pleura, as can be seen from the overlap in Tables 8.1 to 8.4.

Table 8.1: Benign Tracheobronchial Neoplasms

Epithelial tumors
   Papilloma
      Squamous cell papilloma
   Adenoma
Soft tissue
   Vascular tumors
      Hemangiomas
      Hemangioendothelioma
   Fatty tumors
      Lipoma
   Fibrous tumors
      Fibroma
   Smooth muscle tumors
      Leiomyoma
   Skeletal muscle tumors
   Cartilaginous tumors
      Chondroma
   Osteogenic tumors
   Neurogenic tumors
      Neurilemmoma
      Neurofibroma
Miscellaneous tumors
   Granular cell tumor (myoblastoma)
Tumor-like lesions

Adapted from Histological typing of lung tumors, 2nd ed. Vol. 1. International Histological Classification of Tumors No. 1. Geneva: World Health Organization, 1981.

Table 8.2: Benign Pulmonary Neoplasms

Epithelial tumors
   Papilloma
      Squamous cell papilloma
Soft tissue
   Vascular tumors
      Hemangioma
      Fatty tumors
      Lipoma
   Fibrous tumors
      Fibroma
   Smooth muscle tumors
      Leiomyoma
   Skeletal muscle tumors
   Cartilaginous tumors
      Chondroma
   Osteogenic tumors
Neurogenic tumors
      Neurilemmoma
      Neurofibroma
Miscellaneous tumors
   Clear cell tumor
   Benign germ cell tumor
      Teratoma
   Chemodectoma
   Sclerosing hemangioma
Tumor-like lesions
   Hamartoma
   Amyloidoma
   Endometriosis

Adapted from Histological typing of lung tumors, 2nd ed. Vol. 1. International Histological Classification of Tumors No. 1. Geneva: World Health Organization, 1981.

Table 8.3: Malignant Tracheobronchial Neoplasms

Epithelial tumors
   Squamous cell carcinoma
   Adenocarcinoma
   Adenosquamous carcinoma
   Large cell carcinoma
   Small cell carcinoma
   Carcinoid
   Bronchial gland carcinomas
Soft tissue
   Vascular tumors
   Fatty tumors
   Fibrous tumors
   Smooth muscle tumors
   Skeletal muscle tumors
   Cartilaginous tumors
      Chondrosarcoma
   Osteogenic tumors
   Neurogenic tumors
Miscellaneous tumors
   Malignant lymphoma
Tumor-like lesions
Metastases

Adapted from Histological typing of lung tumors, 2nd ed. Vol. 1. International Histological Classification of Tumors No. 1. Geneva: World Health Organization, 1981.

Table 8.4: Malignant Pulmonary Neoplasms

Epithelial tumors
   Bronchial gland carcinomas
      Adenocystic carcinoma
      Mucoepidermoid carcinoma
Soft tissue
   Vascular tumors
      Hemangiopericytoma
      Epithelioid hemangioendothelioma
      Kaposi sarcoma
      Pulmonary angiosarcoma
   Fatty tumors
      Fibrosarcoma
   Fibrous tumors
      Fibrosarcoma
      Malignant fibrous histiocytoma
   Smooth muscle tumors
      Leiomyosarcoma
   Skeletal muscle tumors
      Rhabdomyosarcoma
   Cartilaginous tumors
      Chondrosarcoma
   Osteogenic tumors
      Osteosarcoma
   Neurogenic tumors
      Neurofibrosarcoma
Miscellaneous tumors
   Carcinosarcoma
   Pulmonary blastoma
   Askin tumor
   Malignant lymphoma
   Plasmacytoma
Lymphoma and lymphoproliferative
   disorders
Metastases

Adapted from Histological typing of lung tumors, 2nd ed. Vol. 1. International Histological Classification of Tumors No. 1. Geneva: World Health Organization, 1981.

Benign Tracheobronchial Neoplasms

Papilloma

Squamous papillomas are the most common laryngeal tumor in childhood. In 2% to 17% of patients they involve the tracheobronchial tree (Fig. 16.17). More rarely they involve the lung parenchyma. Lesions may be single or multiple, and the laryngeal disease may be present for up to 10 years before tracheobronchial disease occurs. Malignant transformation has been described and is extremely rare. Papillomas are much rarer in adults than children, usually presenting between the ages of 50 and 70 years. In adults, laryngeal papillomatosis is usually absent. As of 1991 only 59 adult cases were documented in the literature (3,5).

Squamous cell papilloma is the most common laryngeal tumor in children.

The chest radiograph and CT findings of papilloma include pneumonia, bronchiectasis or abscess formation secondary to obstruction. When the lungs are involved, multiple, small, well-defined, scattered pulmonary nodules are found, which may cavitate.

Lipoma

This is a benign tumor of adipose tissue or lipocytes. It has a mean age at onset in the fifth decade. They are more common in smokers than nonsmokers and more common in males than females, with a ratio of 5:1. Approximately 80% of lesions arise in the tracheobronchial tree and 20% within the lung parenchyma; 109 cases have been reported. Lesions are usually solitary, may be pedunculated, and have been reported up to 3 cm in size. Lipomas can also arise from the pleura (Fig. 17.25). Radiologically, findings are related to airway obstruction or the mass itself. On CT they demonstrate characteristic homogeneous fat attenuation. Liposarcoma is the malignant equivalent. It is very rare, with less than 10 cases reported. It usually occurs in the lung parenchyma and occurs equally in men and women. At CT, liposarcoma should be suspected when the fat attenuation mass also contains areas of soft tissue nodularity (5,6,7).

Fibroma

Fibromas occur equally in males and females. They arise from within the bronchial wall, but may also arise within the lung parenchyma. They may grow to be very large. The endobronchial lesions may cause lobar or segmental atelectasis due to obstruction. They may also present as a solitary pulmonary nodule, often incidentally detected in asymptomatic individuals. Fibrosarcoma is the malignant equivalent of fibroma. Similarly, lesions may be endobronchial or lung parenchymal in location. The mean age at onset is 49 years. At CT, both fibroma and fibrosarcoma appear as soft tissue masses; the latter may have features of invasion and spread outside the airway, into or around other mediastinal structures (8,9).

Chondroma

Chondromas usually arise from the cartilage in the walls of large bronchi and are usually 1 to 2 cm in size. When multiple, they should raise suspicion of Carney syndrome, a triad of bronchial chondroma, gastric leiomyoma, and extraadrenal functional paragangliomas. Less commonly, chondromas arise within the lung parenchyma and appear as a solitary pulmonary nodule, usually detected incidentally in asymptomatic individuals. The malignant equivalent is a chondrosarcoma. It is very rare. It occurs equally within the endobronchial tree and lung parenchyma, is slightly more common in women, and has a mean age at onset of 55 years. Like fibromas, the radiologic and clinical features depend on the presence or absence of airway obstruction. In addition to a soft tissue mass, they may also contain areas of calcification (5,7,9).

Carney syndrome: bronchial chondromas, gastric leiomyoma, and extraadrenal paragangliomas.

Granular Cell Tumor (Myoblastoma)

Granular cell tumors, otherwise known as myoblastomas, may arise within the skin, breast, tongue, or esophagus. Approximately 6% are endobronchial in location, of which approximately 100 cases have been reported. They appear as either polypoid or sessile soft tissue masses in the large bronchi or in the trachea near the carina, ranging in size from 5 to 60 mm (Fig. 8.1). Rarely, they arise in the lung parenchyma. The imaging features are similar to fibroma (5,9,10).

Figure 8.1 Endobronchial benign granular cell tumor. Computed tomography of lung at (A) soft tissue and (B) lung window settings demonstrate a right main bronchus soft tissue mass (arrow).

Malignant Tracheobronchial Neoplasms

The most common epithelial neoplasms are bronchogenic carcinomas (Chapter 7). Carcinoid tumors and bronchial gland tumors are discussed here. The most common malignant neoplasms of the trachea are squamous cell carcinoma and adenocarcinoma. CT is the preferred imaging modality for evaluation of malignant tracheal neoplasms.

Carcinoid

Carcinoid tumors represent 5% of pulmonary tumors and are usually seen in either young adults when benign or in adults older than 50 years when more aggressive. Low and intermediate grade tumors occur equally in males and females, whereas the high grade tumors are two to four times more common in men. Carcinoid tumors are also up to 25 times more common in Caucasians than in African Americans. Carcinoid tumors are a spectrum of benign, invasive, and malignant lesions, representing a histopathologic spectrum of neuroendocrine neoplasms that originate from neurosecretory cells (Kulchitsky cells) of the bronchial mucosa. These slow-growing malignant neuroendocrine tumors are part of the amine precursor uptake and decarboxylation system and may secrete a number of hormones, including calcitonin, bradykinin, norepinephrine, antidiuretic hormone, and adrenocorticotrophic hormone. Histologically, they are divided into three groups (Table 8.5).

Carcinoid tumors are neuroendocrine in origin and are on a histopathologic spectrum that ranges from benign carcinoids to small cell carcinoma.

Approximately 80% of carcinoid tumors are endobronchial in location, and the remaining 20% arise in the lung parenchyma as a solitary pulmonary nodule. The latter arise from small bronchi, a feature seen at pathologic examination but usually not recognizable with imaging. Given the histopathologic spectrum, the clinical presentation and radiologic findings are variable, ranging from locally confined and usually low grade tumors to locally invasive tumors. The intermediate and high grade tumors have a tendency to recur and metastasize to extrathoracic sites; the incidence of metastases depends on tumor grade (11,12,13,14).

Classic carcinoid is the least aggressive and has a mean age at onset of 50 years. These tumors are usually centrally located in the airway and are less than 2.5 cm in size. They infrequently metastasize to lymph nodes and have a good prognosis. They occur more frequently in the right middle lobe than other lobes of the lung and have a 5-year survival of 87%. Atypical carcinoid tumors are usually greater than 2.5 cm in size, with a mean age at onset of 57 years. Although they have well-defined margins, lymph node metastases occur in 50% of patients and other organ metastases in 30% of patients. The prognosis is therefore less favorable, with a 5-year survival rate of 56% (12,13,14).

Small cell carcinoma has a mean age at onset of 62 years and is twice as common in males than females. It is the most aggressive form of carcinoid tumor and has the worst prognosis. It is usually an ill-defined lesion (Fig. 8.2), with extensive mediastinal lymph node involvement at the time of diagnosis. Similarly, large cell neuroendocrine carcinoma has a mean age at onset of 62 years and is four times more common in males than females. The 27% 5-year survival of large cell neuroendocrine carcinoma is better than the 9% 5-year survival for small cell carcinoma; however, the 10-year survival rate for both is poor, at just over 5% (12,13,14).

With carcinoid tumors, 90% of the chest radiographs are abnormal. Abnormalities include lobar or segmental atelectasis or hyperinflation, obstructive pneumonitis, bronchiectasis or lung abscess. They can also appear as a solitary nodule (Figs. 8.2 and 8.3) or as a pedunculated mass. Occasionally, they contain areas of apparent calcification (Fig. 8.4), which represents ossification histologically. CT is excellent at demonstrating both the intraluminal and extraluminal component of these tumors. Up to 25% of classic and atypical carcinoid tumors have calcification on CT. Intravenous contrast enhancement of the lesions is classically intense (Fig. 8.5), not surprising given what is often referred to at bronchoscopy as a “cherry red” lesion that commonly has visible vessels on its surface. Carcinoid tumors can also be demonstrated with radiolabeled octreotide scans or with magnetic resonance imaging (MRI) (Fig. 8.6) due to high signal from the tumor and the low background signal from normal lung and flowing blood (2,15).

Table 8.5: Histopathologic Spectrum of Carcinoid Tumors

I

Low grade

Classic carcinoid

II

Intermediate grade

Atypical carcinoid

III

High grade

Small cell carcinoma
Large cell neuroendocrine carcinoma

Figure 8.2 Limited stage small cell carcinoma presenting as a left upper lobe solitary pulmonary nodule on computed tomography.

Figure 8.3 Carcinoid tumor. A. Chest radiograph and (B) computed tomography demonstrate a left lower lobe solitary pulmonary nodule of soft tissue attenuation (arrows).

Figure 8.4 Atypical carcinoid. Computed tomography demonstrates a solitary right lower lobe nodule on (A) lung and (B) soft tissue window settings (arrow). Note calcification seen as central high attenuation (arrowhead), which represents ossification histologically.

Figure 8.5 Carcinoid tumor. Computed tomography with intravenous contrast. Carcinoids are vascular tumors. The tumor (left asterisk) and aorta (right asterisk) have similar enhancement.

Figure 8.6 Carcinoid tumor. T1-weighted coronal magnetic resonance imaging before (A) and after (B) gadolinium. Left main and upper lobe bronchus tumor (arrow), which enhances postcontrast (arrow), and causes upper lobe post-obstruction consolidation (arrowheads). T1-weighted axial magnetic resonance. The endobronchial component is better appreciated (asterisk).

Bronchial Gland Carcinomas

Bronchial gland tumors include adenoid cystic carcinoma, also known as cylindroma, and mucoepidermoid carcinoma. The term “bronchial adenoma” was used in the past to include these tumors as well as carcinoid tumor. Inclusion of the latter group is incorrect, because they have a different cell of origin.

Adenoid cystic carcinoma arises from mixed serous and mucinous glands located in the posterolateral wall of the trachea and major bronchi. They occur in middle age, are more aggressive than carcinoid tumors, and have a greater propensity for both local invasion and metastases. They usually arise in the lower trachea, main or lobar bronchi. Chest radiograph abnormalities are usually due to chronic bronchial obstruction and may be present for a number of years before diagnosis. Adenoid cystic carcinoma has a tendency to grow along the length of the airway in a submucosal manner, so that the length of the airway involved is usually greater than is appreciated with CT examination (Fig. 16.20). The length of the airways excised is usually greater that what is visible with imaging. The tumor can locally recur, often many years after initial resection. In a series from the Mayo Clinic, 55% of patients were alive at 10 years (13,16).

Mucoepidermoid carcinoma is a mixture of mucous-secreting, squamous, and intermediate cells. It usually affects the trachea as a locally invasive sessile or polypoid tumor. It is rare. Half of patients are less than 30 years of age. Low-grade tumors rarely are locally invasive or metastasize; local resection (such as sleeve resection) has an excellent prognosis. High grade lesions are treated with surgery and postsurgical radiotherapy (13,16).

Benign Pulmonary Neoplasms

There is a wide spectrum of benign pulmonary neoplasms that may arise from the many cellular elements present in the lung. In general, they appear on imaging as indeterminate solitary pulmonary nodules. The most common are the pulmonary hamartomas, and they are discussed first. Where possible, specific imaging findings that can lead one to a specific diagnosis are indicated. Others neoplasms that also occur within the tracheobronchial tree have already been discussed.

Hamartoma

Hamartomas are an abnormal quantity, mixture, or arrangement of the normal constituents of the organ in which they are found. They usually occur in patients between 40 and 70 years of age and are two to four times more common in males than females. Most arise within the lung periphery; however, 10% to 20% have an endobronchial origin. Rarely, they may be multiple.

A hamartoma represents an abnormal quantity of cellular elements normally present in the organ in which they are found.

Figure 8.7 Hamartoma. Posteroanterior (A) and lateral (B) chest radiographs demonstrate a right lower lobe lobulated calcified mass.

On chest radiograph or CT lesions are seen as well-defined round or ovoid nodules that can be lobulated or notched (Fig. 8.7). They are usually 2 to 5 cm in diameter, with most less than 4 cm in diameter. Calcification is common, being more common the larger the diameter of the hamartoma (Figs. 8.7 and 8.8). On chest radiography and CT, hamartomas are most commonly seen as homogeneously soft tissue opacity and attenuation, respectively (Fig. 8.9). At chest radiography or CT, the presence of “popcorn calcification” is classic and nearly pathognomonic (Fig. 8.10). Fat attenuation with the nodule on CT is demonstrated in approximately one-third of hamartomas and is pathognomonic (Fig. 8.8).

 

Cavitation is extremely rare. At MRI, hamartomas have intermediate signal on T1-weighted images and high signal on T2-weighted images. Septa may be demonstrated dividing the lesion into lobules within the nodule, and these septa may enhance intensely after intravenous gadolinium. Endobronchial lesions cause radiologic signs of obstruction (5,17).

Figure 8.8 Calcified hamartoma. Computed tomography demonstrates a hamartoma within the lingula (arrow) with central calcifications(arrowhead) and adjacent low attenuation representing fat.

Figure 8.9 Hamartoma. Computed tomography at (A) soft tissue window settings and (B) lung window settings demonstrates a solitary right lower lobe lung nodule of soft tissue attenuation (arrow).

Figure 8.10 Calcified hamartoma on (A) chest radiograph and (B) computed tomography. Soft tissue settings demonstrates a lobulated and calcified right lower lobe mass (arrow). Computed tomography on bone window settings (C) more clearly demonstrates the morphology of the internal calcification (asterisk).

Figure 8.11 Cystic adenomatoid malformation. Computed tomography images (A and B) demonstrate a multicystic right upper lobe mass (arrows) with a small nodular soft tissue component (white arrowhead).

One-fourth to one-third of hamartomas can be specifically diagnosed by the presence of fat or fat and calcium in a nodule on CT.

Infantile hamartoma or congenital cystic adenomatoid malformation is composed of increased structures resembling terminal bronchioles, epithelium, stroma, and smooth muscle. They are slightly more common in males, occur equally on both sides, and usually affect only one lobe. They are prone to recurrent infection and treatment is surgical resection. On chest radiograph and CT, a solid mass with cystic or radiolucent foci is seen, and air–fluid levels may be present (Fig. 8.11) (18).

Cystic adenomatoid malformation is an infantile form of hamartoma.

Hemangioma

Hemangiomas arise from benign proliferations of blood vessels with little surrounding stroma. Histologically, cavernous and capillary types are described. The usual age at diagnosis is 30 to 40 years, and they are more common in females than males. They most commonly arise in the peripheral pulmonary parenchyma in a subpleural location, and rarely may be endobronchial in location. Hemoptysis is a common presenting symptom.

On the chest radiograph and CT, hemangiomas are usually solitary, round, well-marginated nodules or masses, with an average diameter of 3 cm in the lung periphery. Uncommonly, they may be multiple. They are usually soft tissue in attenuation on CT. Discrete round calcifications, representing phleboliths, may be seen on CT, less commonly appreciated on chest radiographs. Hemangiomas may present with obstructive signs when endobronchial (3,7,9).

Leiomyoma

A leiomyoma arises from smooth muscle, either within the lung parenchyma peripherally or walls of the tracheobronchial tree, in 55% and 45% of cases, respectively. They are 1.5 times more common in females than males, and usually present between 30 and 40 years of age. A leiomyoma is usually a solitary pulmonary nodule or mass ranging in size from 2 to 8 cm. Although usually soft tissue in attenuation on CT, they may calcify. They may also appear as multiple small pulmonary nodules, usually 5 to 10 mm in diameter. With central lesions (Fig. 8.12), patients may have wheezing as a presenting symptom, with radiologic findings of obstruction (5,7,9). Leiomyosarcoma is the very rare malignant equivalent.

Figure 8.12 Endobronchial leiomyoma. Computed tomography at (A) soft tissue and (B) lung window settings demonstrates a right main bronchus soft tissue nodule (arrow).

Neurogenic Tumors

Although relatively common in the mediastinum (Figs. 9.15 and 9.17), the neural tissue tumors, such as neurofibroma and neurilemmoma, are exceedingly rare in the lung. They usually occur as a peripheral solitary pulmonary nodule. Even more rarely they may be endobronchial in location and cause signs of obstruction. Neurofibromas are three times more common than neurilemmomas. Neurofibromas are more common in men, whereas neurilemmomas are more common in women. Malignant equivalents of these tumors also occur (5,7).

Benign Clear Cell Tumor

These are very rare tumors of the lung parenchyma that are benign, although histologically they resemble clear cell renal carcinoma. They are slightly more common in women, with a mean age at onset of 51 years (9,10).

Teratoma

Typically thought of as an anterior mediastinal mass (Fig. 9.19), rarely teratomas arise in the lung, with only 33 pulmonary teratomas reported. They are slightly more common in females than males and usually occur in young adults. Most are benign, but a malignant form is described. Expectoration of hair is pathognomonic clinically. On chest radiograph or CT they appear as lobulated masses with calcification that tend to occur in the left upper lobe. Fat has been described on chest radiograph, but not yet on CT; only a few have been reported with CT so far. They may cavitate (10).

Tricholithoptysis may be seen with pulmonary teratomas.

Chemodectoma

Chemodectomas are nonchromaffin paragangliomas with no hormonal activity. They are more frequent in females than males, occurring with a ratio of 4:1.5. The mean age at onset is 58 years. Chemodectomas occur much more frequently in the mediastinum than the lung. Within the lung they appear as a round solitary pulmonary nodule, 1 cm or greater in size, with a 17 cm mass reported (10).

Pulmonary Endometriosis

Intrathoracic endometriosis is very rare. It occurs in two forms: pleurodiaphragmatic and bronchopulmonary. Pleurodiaphragmatic is the most common form and may be a source of recurrent pneumothorax that occurs during menstruation (catamenial) (Fig. 17.19). Less commonly it may involve the lung parenchyma or airway and manifests as catamenial hemoptysis. Radiologically, it appears as single or multiple nodules that may cavitate and may be associated with bronchiectasis (3).

Malignant Pulmonary Neoplasms

Unlike bronchogenic carcinoma, the other malignant pulmonary neoplasms are rare. Like the benign pulmonary neoplasms, malignant pulmonary neoplasms may arise from a variety of cells, giving rise to a list of tumors of varying histopathology and radiologic manifestations (Table 8.4). Lymphoma and lymphoproliferative disease processes are discussed after malignant pulmonary neoplasms in their own section.

Hemangiopericytoma

Hemangiopericytoma usually occurs in patients between 40 and 60 years of age and affects males and females equally. It may be benign or malignant, and this distinction usually depends on lesion size. The larger the lesion, the more likely it is to be malignant. An endobronchial component may occur. Patients may present with hemoptysis, but frequently it is first discovered as an incidental “solitary pulmonary nodule” on a chest radiograph (7,9).

Epithelioid Hemangioendothelioma

Epithelioid hemangioendothelioma is four times more common in females than males. Patients present between 12 and 60 years of age, with a mean onset of 35 years. Half of cases occur in individuals below the age of 40 years. Survival may be up to 15 years in the less aggressive form of the disease; however, there is a more aggressive form of the tumor with a survival of approximately 1 year. At initial presentation, epithelioid hemangioendothelioma often effects multiple organs, including the lung, liver, bones, and soft tissues simultaneously or sequentially. When this occurs it may be difficult to know if the tumor arose initially as a multicentric process, similar to lymphoma, or as a primary lesion with metastases to other tissues, though metastases are uncommon.

The most characteristic feature of epithelioid hemangioendothelioma on chest radiograph or CT is the presence of multiple bilateral lung perivascular nodules with well-defined or ill-defined margins in both lungs (Fig. 8.13). The nodules range in size up to 2 cm, but most are less than 1 cm in size and redundant to calcify. They are usually found in relation to small and medium-sized vessels and bronchi. The lung nodules uncommonly calcify (19,20,21).

Primary Pulmonary Sarcoma

Primary pulmonary sarcomas are usually fibrosarcomas or leiomyosarcomas, although chondrosarcoma (Fig. 8.14), osteosarcoma, malignant fibrous histiocytoma, rhabdomyosarcoma and neurofibrosarcoma also occur. Overall, most pulmonary sarcomas are secondary metastases from extrathoracic primary sites. On imaging they appear as a solitary pulmonary nodule or mass. Rarely, they may be endobronchial and cause atelectasis.

 

Carcinosarcoma and Pulmonary Blastoma

Carcinosarcoma usually occurs in patients over the age of 50 years, with 90% of patients between 50 and 80 years of age. It is much more common in males than females and has an increased incidence in smokers. They usually manifest as a solitary large peripheral mass in the upper lobes. Occasionally, it may be an endobronchial lesion and present with clinical and radiologic signs of obstruction (9,13,22).

Figure 8.13 Epithelial hemangioendothelioma. Chest radiograph (A) and computed tomography (B) demonstrate multiple pulmonary nodules. Abdominal radiograph (C) demonstrates multiple calcified hepatic tumors (arrows).

Figure 8.14 Chondrosarcoma. Computed tomography demonstrates a left lower lobe mass (arrow) with endobronchial component(asterisk) (A) and matrix calcification seen as eccentric high attenuation (arrowhead) (B).

Pulmonary blastoma is now believed to be a form of carcinosarcoma. It occurs in a younger age group than carcinosarcoma. On chest radiograph or CT it appears as a large, solitary, well-defined, peripheral nodule or mass, ranging from 2 cm upward but usually about 10 cm; they may have cystic structures. Occasionally they can be multiple. At least one case of cavitation and calcification is described (9,13,22).

Lymphoma and Lymphoproliferative Diseases

A variety of malignancies may be found in the thorax arising from the lymphatic system, as listed in Table 8.6. Lymphoma, Castleman disease, and plasma cell granuloma are discussed here. The other lymphoproliferative diseases, leukemias, multiple myeloma, and amyloid are discussed in Chapter 13.

Lymphoma

Lymphomas are tumors of the immune system, divided into Hodgkin lymphoma and non-Hodgkin lymphomas. Lymphoma is the seventh leading cause of death from cancer in the United States. The incidence of Hodgkin lymphoma is 2.5 per 100,000. It has a bimodal age distribution, with a large peak in the second and third decade and a smaller peak in the sixth and seventh decade. It occurs more often in males than females, with a ratio of 1.7:1. Non-Hodgkin lymphoma is more common than Hodgkin lymphoma and occurs in an older age group. In the thorax, lymphoma can affect almost any structure, including the intrathoracic and axillary lymph nodes and thymus, as well as extranodal involvement of the pulmonary parenchyma, airway, pleura, pericardium, chest wall and spine (3,11).

Lymphoma is the seventh leading cause of cancer death in the United States.

Thoracic lymph node enlargement is the most common manifestation of both Hodgkin and non-Hodgkin lymphoma (Fig. 9.9). Whereas Hodgkin lymphoma predictably spreads in a contiguous fashion, non-Hodgkin lymphoma is often noncontiguous and asymmetric. Lymph node enlargement is present on the chest radiographs in 67% of Hodgkin lymphoma, versus 43% of non-Hodgkin lymphoma. These figures are much higher for CT in both forms of lymphoma. The incidence of visible lymph node enlargement on chest radiography is lower in younger patients. Most patients with Hodgkin lymphoma have involvement of two or more nodal groups; hilar involvement is rare in the absence of mediastinal involvement. In non-Hodgkin lymphoma, half of cases may have only one nodal group involved. The paracardiac nodes are rarely involved at presentation, but are an important site for relapse.

Lymph node calcification is rare at presentation but may occur after radiation therapy or chemotherapy. Superior vena cava obstruction may occur as a presenting symptom or later in the course of the disease (Fig. 8.15). Thymic enlargement is commonly seen and lymphoma may arise solely within the thymus.

Table 8.6: Lymphoproliferative Disease within the Thorax

Plasma cell granuloma (inflammatory pseudotumor)
Posttransplant lymphoproliferative disorder
Lymphoid interstitial pneumonitis
Lymphomatoid granulomatosis
Lymphoma
Pseudolymphoma
Angioimmunoblastic lymphadenopathy
Castleman disease

Figure 8.15 Lymphoma. Computed tomography demonstrates massive mediastinal lymph node enlargement (asterisk). The superior vena cava is compressed to a thin slit (arrow). Note the collateral vessels within the left anterior chest wall (arrowheads).

Lymph nodes in lymphoma may calcify after treatment.

Pulmonary parenchymal involvement associated with nodal disease occurs in 10% to 15% of patients with lymphoma at presentation and is three times more common in Hodgkin lymphoma than in non-Hodgkin lymphoma (Fig. 8.16). The most common presentation is one or more discrete nodules (Fig. 8.17), which are typically ill defined or contain air bronchograms (Fig. 8.18) and uncommonly cavitate. Lung parenchymal involvement without nodal involvement is rare. Primary pulmonary Hodgkin lymphoma is very rare. Primary pulmonary non-Hodgkin lymphoma is a low grade B-cell lymphoma that arises from mucosa-associated lymphoid tissue and is known specifically as bronchus-associated lymphoid tumor (Fig. 8.19). Because of the widespread pulmonary lymphatic tissue there can be enlarged peribronchovascular lymph nodes and thickening of the interlobular septa. High resolution CT is particularly useful in the evaluation of the interstitium. Lymph node enlargement can extend to involve the adjacent pulmonary parenchyma with resultant chronic airspace disease and air bronchograms. Infectious complications can occur secondary to immunosuppressive therapy due to bacterial, fungal, viral, or mycobacterial infections. CT is particularly useful in early detection of these findings, with high resolution CT being especially useful in the detection of invasive pulmonary aspergillosis. A “CT halo sign,” though not specific, is highly suggestive of invasive aspergillosis in immunocompromised patients (13,23,24,25,26,27).

Hodgkin lymphoma is the most common lymphoma to involve the lungs. Non-Hodgkin lymphoma is the most common primary pulmonary lymphoma.

Nodules with air bronchograms or consolidation are the classic findings of pulmonary lymphoma.

Pleural effusion can occur secondary to lymphatic obstruction or pleural invasion and can be unilateral or bilateral. They are usually small to moderate in size. More commonly pleural effusions in patients with lymphoma are secondary to cardiac failure or infection. Chest wall involvement can also occur. Lymphomatous involvement of the pericardium and myocardium, often due to direct invasion, is seen in a small number of patients (3).

Figure 8.16 Low-grade non-Hodgkin lymphoma. Computed tomography demonstrates a single large right lower lobe mass (asterisk) and innumerable ill-defined bilateral lung nodules.

Figure 8.17 Metastatic lymphoma. Chest radiograph shows multiple bilateral pulmonary nodules of varying size.

Figure 8.18 Pseudolymphoma. Chest radiograph (A), and computed tomography on (B) soft tissue and (C) lung window settings demonstrate a well-defined right lower lobe mass containing air bronchograms (arrow).

Figure 8.19 MALToma. Computed tomography at (A) soft tissue and (B) lung window settings demonstrates a large central mass (asterisk) extending into the lingula and multiple pulmonary nodules.

Pleural effusions in lymphoma patients are more commonly due to heart failure or infection than to lymphoma.

Castleman Disease

Castleman disease is also known as angiofollicular hyperplasia or giant lymph node hyperplasia. It occurs in two forms: a localized form known as unicentric angiofollicular hyperplasia and a generalized form known as multicentric angiofollicular hyperplasia. There are also two histologic forms, the hyaline-vascular type and the plasma cell type. Patients may be asymptomatic. Fever, weight loss, and lassitude are common symptoms with the localized form, especially with the plasma cell type. Elevated erythrocyte sedimentation rate, an IgG, IgA, or IgM hypergammaglobulinemia, and chronic anemia also occur. The generalized form presents with skin lesions, papilledema, or a monoclonal gammopathy. There may also be a symmetric sensorimotor peripheral neuropathy. Current thinking is that this disease is a form of lymphoma (3,11).

Castleman disease is a form of lymphoma with localized and generalized (systemic) forms.

Localized or unicentric angiofollicular hyperplasia is usually of the hyaline-vascular type in up to 96% of cases. Hyaline-vascular localized Castleman disease usually occurs between 30 and 40 years of age and occurs equally in males and females; it may have a slight female predominance. Radiologically, there is usually a solitary, well-circumscribed, encapsulated mass, with or without cervical or mediastinal lymph node enlargement. This form of Castleman disease characteristically enhances intensely with intravenous contrast administration on either CT or MRI (Fig. 9.22). Plasma cell type localized Castleman disease usually occurs in young adults, equally in males and females. Lymph node enlargement usually is more often disseminated. Treatment is with surgical resection, radiotherapy, and steroids; this can achieve a cure rate of up to 100% (3,28).

The hyaline vascular type of Castleman disease enhances intensely with intravenous contrast on CT or MRI. The plasma cell type does not.

Generalized Castleman disease is usually the plasma cell type, occurs in patients over the age of 40 and is twice as common in males than females. It occurs in two forms, hyperplasia without neuropathy and hyperplasia with neuropathy. Both are treated as described above; however, the prognosis is poor, with a mean life expectancy of approximately 30 months. Radiologically, there is disseminated lymph node involvement, with adjacent soft tissue infiltration at multiple sites (3,28).

Plasma Cell Granuloma (Inflammatory Pseudotumor)

Plasma cell granuloma is a benign tumor, which may originate as an organizing pneumonia. Sixty percent of patients are under the age of 40, and it is the most common benign lung tumor in children. It occurs equally in males and females. Most patients are asymptomatic, and it is usually diagnosed as an incidental mass on a chest radiograph. When symptomatic, patients present with fever, cough, chest pain, wheezing or hemoptysis. The chest radiograph and CT demonstrate a solitary well-defined nodule in 70% of cases. In the remainder there may be an ill-defined or spiculated nodule, a large mass or multiple (up to three) lesions. Calcification may be extensive (Fig. 8.20), and cavitation is described. Occasionally, plasma cell granuloma may arise in a bronchus, with consolidation or atelectasis distally (Fig. 8.21). A pleural origin is also described. Plasma cell granuloma may or may not enhance with intravenous contrast on CT or MRI. They are hyperintense to muscle on T1-weighted sequences and high signal on T2-weighted sequences (3,28).

Figure 8.20 Plasma cell granuloma (inflammatory pseudotumor). Chest radiograph (A) and computed tomography (B) demonstrate a 6 cm left upper lobe mass with central calcification.

Plasma cell granuloma (a.k.a. inflammatory pseudotumor) is the most common benign primary lung tumor in children.

Figure 8.21 Plasma cell granuloma (inflammatory pseudotumor). Tomogram (A) and T1-weighted coronal magnetic resonance postgadolinium (B) demonstrate a sessile tracheal nodule (asterisk).

Metastatic Disease

Metastases to the thorax may deposit in many structures, most commonly the lungs, lymph nodes, pleura, ribs, and spine. Metastases to the pericardium are common at autopsy but are uncommonly diagnosed antemortem. Metastases to the airway and esophagus are unusual. The incidence of thoracic metastases varies with the primary tumor and with the stage of the disease.

Lung Metastases

The lung is a very common site for metastases. The tumors most likely to metastasize to the lung are renal cell carcinoma, sarcoma, thyroid cancer, breast cancer, and melanoma (3,11). Tumor may spread by a number of routes, as listed in Table 8.7.

Hematogenous spread is the most common form of dissemination of metastases to the lung. The main radiologic features are multiple bilateral round or ovoid nodules of varying size. They occur in the lower lungs more than the upper lungs due to the greater distribution of blood flow to the lower lobes. Lung metastases are usually in the peripheral one-third of the lung and range in size from 1 to 2 mm to several centimeters (Figs. 8.22 and 8.23). Although these features are seen on both the chest radiograph and CT, CT is more sensitive for the detection of smaller nodules and nodules obscured by the heart and mediastinum. Lung metastases are usually well defined but may have irregular margins if they bleed into the adjacent lung. The latter may be seen with adenocarcinoma, choriocarcinoma, and with metastases after treatment. Uncommonly, metastases appear as innumerable 3- to 5-mm miliary nodules, similar to the miliary pattern seen with miliary tuberculosis. This pattern is most commonly seen with thyroid cancer, renal cell carcinoma, and melanoma. Very rarely metastases present as areas of pulmonary consolidation; this occurs with melanoma and breast cancer (3,13,29).

Hematogenous lung metastases are found in the portion of the lungs with the greatest blood flow, namely the lower lobes.

Table 8.7: Routes of Metastatic Dissemination within the Thorax

Endobronchial
Hematogenous
Lymphatic
Pleural
Direct

Figure 8.22 Pulmonary metastases. Posteroanterior (A) and lateral (B) chest radiographs demonstrate multiple bilateral pulmonary nodules of varying size.

Figure 8.23 Pulmonary metastases from adenoid cystic carcinoma of the head and neck. Posteroanterior (A) and lateral (B) chest radiographs and computed tomography (C) demonstrate one larger mid-left lung lesion (arrow) and several smaller nodules bilaterally.

Miliary hematogenous metastases are most commonly seen with very vascular tumors that shower thousands of tumor cells and then grow slowly, namely thyroid and renal cancers.

Cavitation is not common and occurs most frequently with squamous cell carcinoma, sarcoma, melanoma, transitional cell carcinoma, colon, uterine, and cervix cancers and after chemotherapy. Metastases can cavitate while still small, and usually cavitary and noncavitary nodules coexist. Cavitation with sarcoma metastases causing spontaneous pneumothorax occurs relatively frequently (Fig. 8.24) (Fig. 17.18) (3,13,29).

Calcification is very uncommon but does occur in breast, thyroid (papillary), testicular, and ovarian cancers and in mucinous adenocarcinoma. More frequently, calcification occurs in sarcomas of osteoid or chondroid origin, where calcification occurs as matrix mineralization as in the primary tumor (Fig. 8.25). They may simulate calcified granulomas on chest radiographs and CT. Dystrophic calcification can be seen in treated metastases (3,13,29).

Metastatic osteosarcoma to lung may appear as multiple calcified nodules, mimicking granulomas.

Figure 8.24 Spontaneous bilateral pneumothoraces secondary to metastatic sarcoma on chest radiograph.

Occasionally, metastatic disease can present as a single nodule, occurring in less than 10% of patients. In patients with known extrathoracic malignancy it can be difficult to know if this represents a primary bronchogenic carcinoma or solitary metastasis. If the extrathoracic primary is from carcinoma of the head and neck, bladder, breast, cervix, bile ducts, esophagus, ovary, prostate, or stomach, then this was more likely to be primary bronchogenic carcinoma than lung metastasis. This is also true of squamous cell carcinoma. In patients with carcinomas of the salivary glands, adrenal gland, colon, parotid gland, kidney, thyroid gland, thymus, or uterus, the likelihood of the nodule in the lung representing a solitary metastasis equals the incidence of a secondary primary tumor. This is also true of adenocarcinomas.

Patients with melanoma, sarcoma, or testicular carcinoma are more likely to have a solitary metastasis than a bronchogenic carcinoma (3,13,29,30).

A new solitary pulmonary nodule in a patient with head and neck, bladder, or cervical cancer is more likely to be a new lung cancer than a metastases; all share cigarette smoking as a risk factor.

Figure 8.25 Metastatic osteosarcoma to the lungs. Findings were new compared with a chest radiograph 7 months earlier. Appearance on a chest radiograph simulates multiple calcified granulomas. A. Chest radiograph demonstrates multiple ossified bilateral lung nodules. B. Computed tomography of the head demonstrates the primary osteosarcoma of the left maxilla (asterisk). C and D. Computed tomography images demonstrate bilateral ossified lung nodules.

Lymphatic spread of tumor may occur secondary to direct invasion of lymphatic vessels, by tumor embolization to blood vessels with spread through blood vessel walls and subsequent invasion of lymphatic vessels, and by retrograde spread through the pulmonary lymphatics from involved mediastinal lymph nodes. The most common tumors to spread this way are bronchogenic carcinoma, melanoma, breast, kidney, stomach, pancreas, colon, thyroid, larynx, and cervical cancers. The chest radiograph may be normal; when abnormal there is usually a mixed picture of reticulonodular opacities with thickening of the interlobular septa (Kerley B lines). There may also be pleural effusion. The main differential diagnosis is intersitial pulmonary edema secondary to cardiac failure, and distinguishing the two can be difficult. A normal sized heart or the presence of enlarged mediastinal lymph nodes favors the diagnosis of lymphangitic carcinomatosis, but often progression on repeat radiographs or failure to improve after diuretic or other therapy makes the diagnosis. CT is more sensitive than chest radiography, particularly high resolution CT, which demonstrates irregular or nodular thickening of the interlobular septa (Fig. 8.26). Nodular or patchy airspace shadowing can also occur. Either localized areas of interlobular thickening or diffuse thickening not lower lobe predominant help distinguish from cardiac failure, as does irregular septal thickening. Nodular septal thickening is very specific for lymphangitic carcinomatosis and is termed the “beaded septum sign” (3,13,29).

There are two forms of lymphangitic tumor spread: central hilar mass causing lymphatic obstruction and hematogenous dissemination with lymphatic invasion.

Nodular septal thickening and polygons are the typical features of lymphangitic tumor spread on high resolution CT.

Figure 8.26 Lymphangitic carcinomatosis from bronchoalveolar cell carcinoma. High resolution computed tomography demonstrates nodular thickening of the interlobular septa and fissure.

Airway Metastases

Endobronchial metastases are not common but are found in 18% to 51% of autopsied patients with extrathoracic malignancy if other intrathoracic metastases are present. Metastases to the bronchus alone occur in less than 5% of patients at autopsy. Usually, endobronchial metastases occur late in the clinical course. The most common primary sites are bronchogenic carcinoma, lymphoma, renal cell carcinoma, colorectal carcinoma, breast cancer, melanoma, and thyroid cancer. Metastases may spread from tumor in the local lung parenchyma, via lymph nodes or from an extrathoracic primary. Occasionally, metastases may produce a clinical and radiologic appearance similar to primary bronchogenic tumor, with either focal bronchial narrowing or mass or obstructive signs (Figs. 8.27 and 8.28) (3,11,13,29).

Figure 8.27 Endobronchial metastasis from teratocarcinoma. Computed tomography demonstrates a bronchus intermedius nodule(asterisk) with obstructive consolidation of the middle and right lower lobes.

The most common tumors to manifest clinically with endobronchial metastases are lung, renal, colon and thyroid cancer, melanoma, and lymphoma.

Figure 8.28 Endobronchial metastasis within the right upper lobe bronchus from non–small cell lung cancer. Computed tomography at(A) soft tissue and (B) lung window settings demonstrates a right main bronchus nodule (asterisk) and consolidation of the right upper lobe (arrows).

Figure 8.29 Pleural metastases. Chest radiograph (A) and computed tomography (B and C) demonstrate left pleural thickening(arrows).

Pleural Metastases

Pleural spread of tumor occurs most commonly with lung and breast cancers and lymphoma but also occurs with cancer of the pancreas, stomach, and ovary. The visceral pleura is usually involved, but parietal pleura may also be involved if the visceral pleura is affected. On chest radiography, CT, and ultrasound, pleural nodules may be identified (Fig. 8.29). However, the usual finding is a pleural effusion. This occurs in up to 60% of patients with pleural metastases. The pleural fluid has high protein and lactate dehydrogenase levels and low glucose level and low pH. Pleural effusions do not always occur in the presence of pleural metastases, and even when pleural metastases are present the effusion may be due to lymphatic obstruction (3,11,13,29).

The most common manifestation of ovarian carcinoma in the thorax is a pleural effusion.

Esophageal Carcinoma

There are approximately 11,000 deaths a year in the United States due to esophageal cancer. It is rare under the age of 40, and the incidence increases with each subsequent decade. It is two to four times more common in males than females and twice as common among African Americans than whites. In the last two decades the incidence of adenocarcinoma has increased, and this now represents one-third to one-half of cases. In contrast, up to 20 years ago squamous cell carcinoma accounted for greater than 90% of cases. The reason for this is not fully known, but it is due to an increase in cases of adenocarcinoma rather than a relative reduction in numbers of squamous carcinoma. Adenocarcinoma is found to arise on a background of Barrett’s mucosa, which is due to longstanding gastroesophageal reflux and reflux esophagitis. A variety of other histologic esophageal tumors are listed in Table 8.8; all are very rare (31).

Table 8.8: Rare Esophageal Malignancies

Mucoepidermoid carcinoma
Adenoid cystic carcinoma
Small cell carcinoma
Carcinosarcoma (spindle cell carcinoma)
Sarcoma (leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma)
Kaposi sarcoma
Primary melanoma

Early esophageal cancers can manifest as irregularities, plaque-like lesions, and sessile or polypoid masses with or without ulceration on double-contrast esophograms. The presence of stricturing may suggest Barrett’s esophagitis, a premalignant condition associated with an increased risk of adenocarcinoma (Fig. 8.30). Advanced esophageal cancer may manifest on chest radiography as a dilated or obstructed esophagus, as mediastinal widening, or as thickening of the retrotracheal stripe. CT typically reveals circumferential esophageal thickening; squamous cell carcinoma tends to be located in the upper and middle thirds of the esophagus and adenocarcinoma in the lower third and are therefore more likely to invade the gastric cardia and fundus (Fig. 8.30) (32).

A normal esophagus on CT does not exclude esophageal carcinoma.

Figure 8.30 Esophageal adenocarcinoma. Chest radiograph (A) demonstrates tumor at the gastroesophageal junction indenting the gastric fundus (arrow). Double-contrast esophogram swallow (B) demonstrates tight stricture of the lower esophagus (arrowheads).

P.191

CT is used to stage esophageal carcinoma by the detection of mediastinal, cervical, and abdominal lymph node enlargement, as well as the detection of distant metastases to lung, liver, kidneys, adrenals, and occasionally bone. CT is also important to evaluate for invasion of adjacent mediastinal structures, such as the airway (Figs. 8.31 and 8.32), aorta, and pericardium. CT has moderate to good accuracy at detecting extraluminal spread and tracheobronchial or aortic involvement. Esophageal cancers typically spread to regional lymph nodes, with upper and middle third of the esophagus tumors spreading to mediastinal lymph nodes and lower third tumors spreading to upper abdominal (lymph nodes, gastrohepatic ligament and left gastric nodal chains). CT is only moderately accurate because normal size lymph nodes may contain metastatic disease, lymph node enlargement may be due to benign disease, and it can be difficult to distinguish enlarged nodes from esophageal wall thickening. The prevalence of distant metastases at the time of diagnosis is low; however, CT is very accurate in detecting distant metastases (33,34).

Figure 8.31 Locally invasive esophageal cancer. Computed tomography demonstrates a nasogastric tube within the esophagus (arrow). Left anterolateral esophageal tumor invades the trachea (asterisk).

Figure 8.32 Esophageal carcinoma with tracheoesophageal fistula. This is demonstrated on esophogram (A), with contrast spilling into the tracheobronchial tree and bronchi. The fistula is also demonstrated on computed tomography (arrows) (B).

Figure 8.33 Metastatic esophageal carcinoma at the time of diagnosis. Computed tomography demonstrates the primary esophageal tumor at the gastroesophageal junction with extension into the cardia (arrows) (A), gastrohepatic ligament lymph node metastases(arrowheads) (B), and liver metastases (asterisks) (C).

For a distal esophageal adenocarcinoma, lymph node metastases to paraesophageal and gastrohepatic nodes are considered to be resectable; celiac axis and more caudal nodes in the abdomen and supraclavicular nodes are considered to be unresectable.

Figure 8.34 Upper anastomotic recurrent esophageal carcinoma. A. Initial postoperative radiograph and (B) later radiograph with widened mediastinum due to recurrent tumor.

The accuracy of MRI for staging esophageal cancer is comparable with CT at present. Endoscopic ultrasound can be used to assess the depth of tumor invasion in the esophageal wall and the presence, size, and morphology of lymph nodes. It has an accuracy of 85% to 90% for T staging, is more accurate at staging adenocarcinoma than squamous cell carcinoma, and with local disease is more accurate than CT. The main limitation of endoscopic ultrasound is tumor stenosis, preventing passage of the scope. At some institutions patients are preoperatively staged with CT and endoscopic ultrasound (33,35).

It is also important to assess for distant metastases at the time of diagnosis. Quint et al. (36) showed that 18% of patients had distant metastatis at the time of their newly diagnosed esophageal carcinoma (Fig. 8.33). CT is superb at detecting liver and adrenal metastases. Distant metastases are most commonly diagnosed in abdominal lymph nodes, liver, lung, cervical/supraclavicular lymph nodes, bone, adrenal glands, peritoneum, and brain (33,36).

Postoperatively, CT can be used to assess for disease recurrence. CT accuracy is moderate to good. Recurrent disease is usually at the resection site or anastomosis (Figs. 8.34 and 8.35) or a combination of local-regional disease and distant metastases with abdominal lymph node enlargement (37).

Figure 8.35 Recurrent esophageal tumor. Computed tomography demonstrates recurrent disease with abnormal concentric soft tissue within the distal gastric pull-through (arrows) (A). This results in dilatation proximally with a bezoar (asterisk) present within the dilated gastric pull-through (B). This also resulted in bilateral aspiration pneumonia (C).

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