[Extrapulmonary small-cell carcinoma - a rarity with important therapeutic consequences].

A report is presented on 12 patients with extrapulmonary small cell carcinoma. In 9 patients the primary tumor could be localized (cervix in 3, esophagus in 3, prostate in 2, pancreas in 1) whereas no primary was found in 3. Seven of 12 patients presented with distant metastases and four developed metastases later. Five of 12 had CNS metastases (brain metastases in 4, spinal cord compression in 1). Six patients were initially treated by surgery or radiotherapy (2 and 4 respectively). All six developed distant metastases during or shortly after local treatment. Five of 6 patients initially treated with chemotherapy responded to the treatment. Three of 12 patients are surviving 18+, 80+ and 81+ months after the initial diagnosis without evidence of disease. The biology and clinical course of extrapulmonary small cell carcinoma are similar to those of its pulmonary counterpart. In planning therapy for extrapulmonary small cell carcinoma, particular importance should be attached to systemic treatment.