Abstract
BackgroundFor this study, we explored the prognostic profiles of biliary neuroendocrine neoplasms (NENs) patients and identified factors related to prognosis. Further, we developed and validated an effective nomogram to predict the overall survival (OS) of individual patients with biliary NENs.MethodsWe included a total of 446 biliary NENs patients from the SEER database. We used Kaplan-Meier curves to determine survival time. We employed univariate and multivariate Cox analyses to estimate hazard ratios to identify prognostic factors. We constructed a predictive nomogram based on the results of the multivariate analyses. In addition, we included 28 biliary NENs cases from our center as an external validation cohort.ResultsThe median survival time of biliary NENs from the SEER database was 31 months, and the value of gallbladder NENs (23 months) was significantly shorter than that of the bile duct (45 months) and ampulla of Vater (33.5 months, p=0.023). Multivariate Cox analyses indicated that age, tumor size, pathological classification, SEER stage, and surgery were independent variables associated with survival. The constructed prognostic nomogram demonstrated good calibration and discrimination C-index values of 0.783 and 0.795 in the training and validation dataset, respectively.ConclusionAge, tumor size, pathological classification, SEER stage, and surgery were predictors for the survival of biliary NENs. We developed a nomogram that could determine the 3-year and 5-year OS rates. Through validation of our central database, the novel nomogram is a useful tool for clinicians in estimating individual survival among biliary NENs patients.
Highlights
Neuroendocrine neoplasms represent a group of highly heterogeneous diseases and originate from peptidergic neurons and neuroendocrine cells [1]
We identified all patients with a diagnosis of neuroendocrine carcinoma, carcinoid, small cell carcinoma, large cell neuroendocrine carcinoma, and mixed adenoneuroendocrine carcinoma (MANEC) of the gallbladder, bile duct, and ampulla of Vater (AoV) using the SEER codes generated from the International Classification of Diseases for Oncology published by the World Health Organization (WHO)
By reviewing the clinicopathological characteristics of biliary neuroendocrine neoplasms (NENs) patients and exploring the prognosis and related risk factors, we developed a nomogram for the prediction of 3year and 5-year survival rates for these patients, and performed nomogram validation using the data from our center
Summary
Neuroendocrine neoplasms represent a group of highly heterogeneous diseases (depending on the primary site) and originate from peptidergic neurons and neuroendocrine cells [1]. Since the number of cases in each center was too small to conduct a subgroup analysis of biliary NENs, focusing on the primary site of tumors [8], some studies have been performed according to national databases. Cen et al [9] selected 248 gallbladder neuroendocrine neoplasms (GB-NENs) patients from the Surveillance, Epidemiology, and End Results (SEER) database; they suggested that age, marital status, tumor size, and SEER stage are prognostic factors. None of these risk factors can answer the question—asked by both patients and clinicians—about survival rates, especially in regard to individual survival time. We developed and validated an effective nomogram to predict the overall survival (OS) of individual patients with biliary NENs
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