The purpose of this study was to determine the clinical outcomes and patterns of failure of limited-stage extrapulmonary small cell carcinoma (EPSCC) treated with modern chemotherapy and radiation (RT).

We retrospectively identified 18 patients with limited-stage EPSCC treated definitively with three-dimensional conformal RT or intensity modulated radiation therapy and chemotherapy. Patients were treated between November 1987 and May 2006. Primary sites of disease included head and neck (n = 7), genitourinary (n = 7), gynecologic (n = 3), and gastrointestinal (n = 1). Chemotherapy consisted of combined platinum and etoposide in 88% of patients. The median number of chemotherapy cycles was 4 (range 3-6), and the median RT dose was 62 Gy (range 32.4-85 Gy). No patient received prophylactic cranial radiation.

With a median follow-up for all patients of 14 months (range 4-42 months), the median overall survival was 17 months, and median disease-free survival was 6 months. Eleven percent (2 of 18) of patients had a locoregional failure, and 78% (14 of 18) had a distant failure. One of these patients had a brain failure. There were no significant differences between the overall survival for patients with gynecologic, head and neck, and genitourinary disease.

Despite modern chemotherapy and RT, patients with limited-stage EPSCC do poorly. Consistent with previous findings the majority of the first failures are distant. Brain failures in this series were uncommon despite no prophylactic cranial radiation. These findings support the need for further studies in an attempt to improve systemic therapies for this disease.