Epidemiologic Trends and Factors Associated With Overall Survival for Patients With Hepatobiliary Neuroendocrine Neoplasms in the United States

Objective: To perform an epidemiological and survival analysis on a cohort of HB-NENs patients using the latest data. Method: In this cohort study, 390 patients diagnosed with HB-NENs between January 1, 1975, and December 31, 2020, were identified from the Surveillance, Epidemiology, and End Results Program. Related data were used for epidemiologic and survival analysis, as well as the development and validation of a nomogram to predict the overall survival probability of individual HB-NENs patients. The study cutoff date was December 31, 2020, and statistical analysis was performed from May 1 to July 31, 2023. Result: The age-adjusted incidence rate of HB-NENs increased 1.76-fold from 1992 to 2020 (annual percentage change [APC], 2.73; 95% CI, 1.86-3.65; P < 0.05). Furthermore, the incidence of HB-NENs in the extrahepatic bile duct increased most significantly (APC, 3.73; 95% CI, 2.03-5.46; P < 0.05), gallbladder also increased most significantly (APC, 3.51; 95% CI, 1.97-5.08; P < 0.05), whereas patients with HB-NENs in the liver had no significant difference. As for tumor type, the incidence increased in HB-NECs (APC, 2.93; 95% CI, 1.34-4.53; P < 0.05) but the incidence was stable in HB-NETs. On multivariable analyses, age at diagnosis, race, tumor size, grade, tumor type, tumor stage, and surgery were significantly associated with overall survival for HB-NENs patients. Furthermore, a nomogram based on associated factors was constructed to predict the 6-month, 1-year and 2-year survival probability, with the concordance indexes of 0.829 (95% CI, 0.800-0.857) for the internal validations and 0.801 (95% CI, 0.745-0.857) for the external validations. Conclusion: In this study, the occurrence and prevalence of HB-NENs have exhibited a persistent upward trend over nearly two decades. Furthermore, this study proposes that a nomogram comprising eight prognostic parameters can effectively quantify the mortality risk in HB-NENs patients.