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Pancreatic endocrine carcinomas (PECAs) are uncommon, with an incidence of 1 per 100,000. Past studies of chemotherapy and hepatic arterial embolization have described median survival durations of approximately 2 to 3 years. Overall survival from time of diagnosis of metastases has never been reported in a large cohort of patients. Our objective was to evaluate the stage-specific prognosis of patients with metastatic PECAs and to assess the impact of clinical and pathologic prognostic factors.We evaluated all cases of differentiated, metastatic PECAs seen at the H. Lee Moffitt Cancer Center between the years 1999 and 2003, measuring survival from time of diagnosis of metastases.Ninety cases of metastatic PECAs were identified. Median overall survival was 70 months, and the 5-year survival rate was 56%. Age, sex, and tumor type (functional vs nonfunctional) did not impact prognosis. Tumor grade, however, was highly prognostic for survival.Median overall survival is 70 months (5.8 years) among patients with metastatic PECAs. This prolonged survival duration may reflect the impact of multimodality treatments. Tumor grade (low vs intermediate grade) represents a highly significant prognostic factor.