Background: Information about extrapulmonary small cell carcinoma (EPSCC) is limited and the role of prophylactic cranial irradiation (PCI) is unknown.
Patients and methods: Disease presentation and outcome of all EPSCC at our hospital between 1990 and 2009 were retrospectively analyzed.
Results: Of 30 EPSCC, the male:female ratio was 58%:42%; 83% had a performance status of 0-2. Median age was 71 years (32-80). Seventeen (57%) had limited stage (LS), 13 (43%) extensive stage (ES). The location of the primary tumor was gastrointestinal (n = 8), unknown (6), gynecological (6), urogenital (5), and ear nose throat (5). Four (13%) developed brain metastases (2 ES, 2 LS). In ES, first line chemotherapy (CT) was given in 85%, mostly platinum-etoposide (64%). Response rate was 90%. In LS, CT and radiotherapy (RT) ± resection resulted in persistent remissions in 67% of patients. Median survival was 16 months (1-107 months), 18 months (1-107 months), and 9 months (0.4-25 months) for LS + ES, LS, and ES, respectively. Weight loss ≥5 % and ECOG performance status 3 + 4 were associated with poorer survival (p < 0.001 and p < 0.01, respectively).
Conclusion: The incidence of brain metastases was relatively low (13%). More studies are necessary, before routinely offering PCI to patients with EPSCC. Best survival outcomes in LS were achieved with multimodality treatment including CT and RT. Prognosis was poor in patients with ES.