Extrapulmonary small-cell carcinoma. A review of the literature with emphasis on therapy and outcome.

We report 18 patients with extrapulmonary small-cell carcinoma, which we regard as a distinct clinicopathologic entity. The natural history of this disease in some sites appears to differ from that of small-cell lung cancer. One patient with small-cell carcinoma of the esophagus was treated with combination chemotherapy and survived for 1 year. Another patient had ectopic adrenocorticotropic hormone production with small-cell carcinoma of the rectum. Two patients with small-cell bladder carcinoma are also reported in this series. Another patient with small-cell carcinoma in a cervical lymph node metastasis has survived more than 7 years after receiving radiation as the only modality of therapy. This case further substantiates the observations of others that extrapulmonary small-cell carcinoma may pursue an indolent course. Furthermore, in certain sites, local modalities of therapy may result in long-term survival or cure. This is particularly true for small-cell neoplasms of the head and neck region. An association between smoking and extrapulmonary small-cell carcinoma is postulated. The therapy and outcome of this disease is also discussed, with particular emphasis on the effectiveness of combined strategies of surgery, radiation, and chemotherapy.