title: Extrapulmonary small-cell carcinoma. A review of the literature with emphasis on therapy and outcome.
abstract: 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.
authors.affiliation: Department of Human Oncology, University of Wisconsin Clinical Cancer Center, Madison 53792.
authors.forename: S C
authors.initials: SC
authors.lastname: Remick
authors.forename: G R
authors.initials: GR
authors.lastname: Hafez
authors.forename: P P
authors.initials: PP
authors.lastname: Carbone
mesh_terms: D000328:Adult; D000368:Aged; D018288:Carcinoma, Small Cell / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D012907:Smoking