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Extrapulmonary small cell cancer

Donald C Doll, MD
Section Editor
Rogerio C Lilenbaum, MD, FACP
Deputy Editor
Sadhna R Vora, MD


Small cell carcinoma is a distinct clinicopathologic entity that usually arises in the lung but can also originate in a wide range of extrapulmonary sites. Small cell carcinoma is the preferred terminology, but these tumors have also been referred to as oat cell carcinoma or sarcoma, small cell neuroendocrine carcinoma, small cell tumor, anaplastic cell carcinoma, microcytoma, reserve cell carcinoma, undifferentiated carcinoma, Kulchitsky cell carcinoma, and carcinoma with amine precursor uptake decarboxylation (APUD) cell differentiation. These tumors are thought to originate from totipotent stem cells present in all tissues, although some small cell carcinomas may arise from more well-differentiated tumors.

Extrapulmonary small cell carcinomas (ESCCs) are extremely rare; these tumors have been described most frequently in the urinary bladder, prostate, esophagus, stomach, colon and rectum, gallbladder, larynx, salivary glands, cervix, and skin. In addition, small cell carcinoma will occasionally present with metastatic disease, and a primary site cannot be identified (small cell carcinoma of unknown primary).

The diverse origin of these ESCCs is illustrated by a study of 120 patients, in which the site of origin was the female genital tract (26 percent), gastrointestinal tract (23 percent), genitourinary tract (19 percent), head and neck (16 percent), unknown primary site (13 percent), and other sites (4.3 percent) [1]. Similar findings have been reported by other groups [2,3].

Although extrapulmonary undifferentiated large cell neuroendocrine carcinomas are distinct histologically, the natural history of such lesions is similar to that of ESCC, and the treatment approach is the same. (See "Pathology of lung malignancies", section on 'Large cell neuroendocrine carcinoma'.)

The key features and general management of ESCCs will be reviewed here, followed by a discussion of ESCCs that arise in relatively more common sites. ESCCs arising in the tubular gastrointestinal tract and the pancreas, cervix, urinary bladder, and with an unknown primary are discussed separately, as is Merkel cell carcinoma of the skin, which is included in the differential diagnosis of these tumors.

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Literature review current through: Nov 2017. | This topic last updated: Jul 26, 2017.
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