Extrapulmonary small cell carcinoma: A single-institution experience and review of the literature

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Abstract
Small cell carcinoma (SCC) is a distinct pathologic entity that may also occur in extrapulmonary sites. In this report the retrospective results of multimodal therapy of primitive extrapulmonary (E) SCC, in a single institution series, are presented. Twenty-four patients (pts) with ESCC were referred to the Centro di Riferimento Oncologico, Aviano, Italy, from 1986 to 1992. Clinico-therapeutic findings were evaluated in 20 pts. Their ages ranged from 20 to 87, with a median of 60.5 years. Primary tumor sites were urinary bladder (5 pts), prostate (4 pts), larynx (3 pts), kidney (2 pts), ovary, skin, oropharynx, trachea, uterine cervix, ethmoid, and stomach (1 pt each); lymph node metastases of unknown origin were observed in 3 pts. More than 50% of pts presented extensive disease. Histologically, 16 cases were pure ESCCs and 8 cases were combined, 4 of them with adenocarcinoma, 2 with transitional cell carcinoma, and 2 with squamous cell carcinoma. Immunohistochemical studies, performed in 7 cases, demonstrated the epithelial nature of these tumors. The cis-platin-VP16 (PE) regimen was used in 13 pts, and 9 of them (69%) obtained objective responses after chemotherapy (CT) alone, with 3 complete remissions (CR) and 6 partial remissions (PR). Median CR and PR duration was 13+ and 24 months, respectively. Radiotherapy was performed in 7/13 pts after induction CT and before consolidation CT. The objective response rate was 100%, with 6 CR and 1 PR. No severe toxic side effects and no toxic deaths were reported. A patient treated with surgery alone for a urinary bladder tumor showed continuous long-term survival, while 1 of 2 pts treated with radiotherapy alone obtained PR. The PE regimen has an activity similar to the one observed in pulmonary SCC.