These adenomas are rarely identified. Like villous adenomatous polyps elsewhere in the GI tract, they appear as frond-like muco- sal masses and have premalignant potential (Fig. 1-66).
Hamartoma
Cowden’s* disease or multiple hamartoma syndrome, is a rare autosomal dominant-inherited disease that produces widespread hamartomatous disease, particularly of the skin. When it affects the esophagus, multiple small mucosal nodularities can be identi- fied. Similar manifestations can be observed in the colon. These hamartomas are premalignant throughout the body, and frequent patient screening is required.
Leukoplakia
Leukoplakia is due to hyperplastic squamous epithelium that occurs most commonly in the oropharynx because of excessive tobacco use. In the oropharynx it is premalignant, but in the esophagus, where it is less commonly observed, it has debatable malignant potential. When observed, it produces small, whitish, plaque-like mucosal esophageal abnormalities.
*Cowden’s syndrome was first mentioned in 1963; Cowden was the first patient seen with the condition.
Malignant
Carcinoma
Carcinoma of the esophagus continues to have high mortal- ity because the development of these tumors is indolent and therefore the tumors present late. Early detection, however, has a 90% 5-year survival. There are multiple predisposing factors (Box 1-1). Males are four times more likely than women to be affected, mostly because of smoking and alcohol habits. Patients present with dysphagia (difficulty swallowing), particularly of sol- ids, and odynophagia (painful swallowing), pain, weight loss, and anorexia.
Most esophageal carcinomas worldwide are squamous (90%), with adenocarcinomas accounting for approximately 5% to 10% (most of which arise from Barrett esophagus) (see “Submuco- sal Esophageal Masses” earlier in the chapter) (Table 1-6). In Figure 1-65. UGI swallow in an 85-year-old man with multiple small
scattered esophageal plaques (arrow) due to glycogenic acanthosis. Fwoman with an irregular mucosal mass (arrow) due to a villous adenoma.igure 1-66. Barium swallow of the lower esophagus in a 63-year-old
B
ox1-1.
Risk Factors for the Developmentof Esophageal Cancer Alcohol
Tobacco
Chronic reflux esophagitis/Barrett complex HPV infection
Achalasia
Caustic ingestion (lye) Radiation
Head and neck cancer Plummer-Vinson syndrome Celiac disease Tylosis (hyperkeratosis) Pemphigoid Epidermolysis bullosa HPV, Human papillomavirus.
Western Europe and the United States, there has been a marked increase over the last generation in adenocarcinomas so that they now account for nearly 50% of esophageal tumors in some stud- ies. Other tumors are very rare (Table 1-6). Approximately 20% occur in the upper esophagus, 50% in the middle, and 30% in the lower esophagus. The staging is listed in Table 1-7.
The findings at UGI examination depend on the timing of the diagnosis. Early findings may demonstrate small sessile polyps or plaque-like lesions, although with adenocarcinoma, there is usually an associated Barrett esophagus with small plaque-like lesions or more marked irregularity along the Barrett stricture (see Fig. 1-51). Later presentations produce larger lesions, which infiltrate along the length of affected esophagus, with a shelf-like abrupt transition from normal to abnormal esophageal mucosa (not dissimilar to its colonic adenocarcinoma “apple core” coun- terpart), with resultant luminal stricture formation (Fig. 1-67). The mucosa is distorted, blunted, or destroyed, and nodular masses can arise, which can ulcerate (Fig. 1-68). Other lesions can present as flat ulcers surrounded by an edematous rim. A rarer type is varicoid carcinoma, whereby the neoplasm extends sub- mucosally along a length of esophagus, giving the appearance of varicosities or thickened folds (see Fig. 1-33). The mucosal folds may not be as smooth as those in esophageal varices, and this result gives a clue to the diagnosis. The patient may not have a history of cirrhosis. Some gastric adenocarcinomas invade the lower esophagus and extend a few centimeters above the GE junction (Fig. 1-69). At UGI examination, it can be difficult to know for certain whether these tumors are primarily esophageal extending to the GE junction or vice versa, and the diagnosis may only be made at endoscopy and biopsy.
At CT, the primary tumor can be harder to identify unless it is large, particularly if the esophagus is undistended and empty of oral contrast medium. However, CT is far superior to UGI exami- nation for the identification of any local extension, including regional lymphadenopathy (Fig. 1-70). In stages I and II and early
T
able1-7
Staging of Esophageal CarcinomaStage Disease Extension 0 Carcinoma in situ I Lamina propria/submucosa IIA Transmural but not further extension IIB Transmural and regional lymph nodes
III Regional lymph nodes (or other nodes), invasion of adjacent structures
IVB Regional or other nodes
IVB Regional or other nodes and distant metastases
B
A
B
Figure 1-67. UGI swallow (A) and axial contrast-enhanced CT (B) of the lower esophagus in a 68-year-old man with marked mucosal irregularity (A; arrow) typical of esophageal carcinoma. The lesion is less evident on CT, with eccentric wall thickening (B; arrow).
T
able1-6
Esophageal CarcinomaType Frequency
Squamous Worldwide, 90%; Western Europe/ United States, 50%
Adenocarcinoma Worldwide, 5%-10%; Western Europe/United States, 35%-50% Spindle cell carcinoma <1%
Carcinosarcoma <1% Primary malignant melanoma <1% Oat cell carcinoma <1%
e
sophagus29
stage III, tumors will generally not be identified at CT, although simple esophageal thickening may be present (Table 1-7). Once a tumor develops later in stage III, the mass should be detected by CT as the tumor invades the surrounding mediastinal structures and enlarges regional lymph nodes (Fig. 1-71). Stage IV disease should demonstrate distant dissemination of the disease, particu- larly to the lungs, liver, and lymph nodes. The detection of these lymph nodes is more readily identifiable by PET/CT, however, which may also aid in detecting more distant disease (particularly lymph nodes) (Fig. 1-71).
Adenocarcinomas look similar to squamous cell carcinomas, even when arising in a Barrett esophagus. By the time the patient is seen, the Barrett stricture has generally been obliterated by the malignant process (Fig. 1-72).
Other carcinomas are rare, and diagnosis will only be made at histological examination. Spindle cell carcinoma is a rare variant of squamous cell carcinoma due to mesenchymal meta- plastic transformation. These cells are typically large and pol- ypoid. Oat cell carcinoma, primary esophageal melanoma, and carcinosarcoma are even rarer and present similarly to spindle
Figure 1-68. UGI swallow of the midesophagus in a 54-year-old man with nodular distortion of the mucosal folds (arrow) due to esophageal carcinoma.
Figure 1-69. UGI swallow in a 59-year-old woman with irregular and distorted lower esophageal mucosa due to adenocarcinoma of the gastric cardia infiltrating up into the lower esophagus (arrow).
A
B
Figure 1-70. Axial (A) and coronal (B) contrast-enhanced CT in a 71-year-old woman with a lower esophageal carcinoma over several centimeters (large arrow) with mediastinal adenopathy (small arrow).
cell carcinoma. Superficial spreading carcinoma is a variant of squamous cell carcinoma that is characterized by small mucosal nodules rather than plaque-like or polypoid masses. It has a bet- ter prognosis because it is usually confined to the mucosa and submucosa.