Management of primary hepatopancreatobiliary small cell carcinoma.

BACKGROUND AND OBJECTIVES: Primary small cell carcinomas (SCC) of the pancreas, liver, gallbladder, and bile ducts have only been described in case reports. We hypothesized that surgical treatment was associated with improved overall survival (OS) for patients with localized hepatopancreatobiliary SCC.

METHODS: The Surveillance, Epidemiology, and End-Results (SEER) database was queried for patients with SCC from 1998 to 2008. Survival was analyzed with Cox proportional hazards models.

RESULTS: Eighty-five patients had nonmetastatic hepatopancreatobiliary SCC and operative treatment data. Hepatic SCC was associated with a 2 month median OS, and no patient underwent surgery. Stage-adjusted median OS for pancreatobiliary SCC patients undergoing resection (19 months, 95% confidence interval [CI]: 10-42 months) was greater than those who were not resected (8 months, 95% CI: 4-12 months, P = 0.0052). Both surgical resection (hazard ratio [HR]: 0.42, 95% CI: 0.29-0.63, P < 0.001) and administration of radiation therapy (HR: 0.50, 95% CI: 0.35-0.71, P < 0.001) independently predicted prolonged OS in adjusted models.

CONCLUSION: Surgical resection was associated with prolonged survival for patients with localized pancreatic, gallbladder, and biliary primaries. While we recognize several biases inherent in a population-based study, these results provide insight into the survival that can be achieved with surgical resection of SCC in these specific locations.