PurposeThe purpose of this study was to examine trends in the incidence and incidence-based (IB) mortality of functional pancreatic neuroendocrine tumors(F-PNETs), and to identify factors associated with survival times.MethodsData were obtained from the Surveillance, Epidemiology, and End Results database from 2000 to 2017. Trends in the age-adjusted incidence of F-PNETs and IB mortality were examined using the Joinpoint Regression Program. Statistical analyses were run using chi-square tests, Kaplan–Meier curves, and the Cox proportional hazards model. Multiple imputation was used to deal with missing data.ResultsA total of 142 patients with F-PNETs met the study inclusion criteria. It was found that the incidence of F-PNETs decreased over the study period, with an annual percent change (APC) of -2. 5% (95% CI [-4. 3, -0. 5], P<0. 05). This decrease was found to be significant for women, and also when limited to cases with distant disease or rare F-PNETs, with APCs of -4. 2% (95% CI [-7. 4, -0. 9], P<0. 05), -6. 7% (95% CI [-10. 4, -2. 8], P<0. 05), and -9. 1% (95% CI [-13. 5, -4. 4], P<0. 05), respectively. The Cox regression analysis revealed that the tumor size, tumor stage, tumor type, and surgical resection were associated with F-PNETs mortality.ConclusionsThis was the first population-based epidemiological study of F-PNETs and we found a continual decrease in the incidence of F-PNETs from 2000 to 2017. The prognosis and survival times were closely related to the calendar year at diagnosis, tumor stage, and tumor size.