Biliary Neuroendocrine Neoplasms: A Clinicopathological and Prognostic Analysis of 36 Clinical Cases

Abstract

Abstract Background Biliary neuroendocrine neoplasms are rare diseases characterized by low morbidity, difficult diagnosis, and poor prognosis. In this study, we aimed to provide a better understanding of this disease by analyzing the clinicopathological features, treatment, and factors associated with prognosis of 36 cases of neuroendocrine neoplasms arising from the gallbladder, bile ducts, and ampulla of Vater. Results The early stages of the disease often present with atypical symptoms, leading to a delayed diagnosis in most cases, abdominal discomfort was the primary manifestation at diagnosis. Neuroendocrine carcinoma was the most common pathological stage observed, with small-cell neuroendocrine carcinomas being the predominant subtype. Tumor recurrence occured mostly in the liver. The median follow-up time was 39 months, and the median survival time was 30 months. The 1-, 2-, and 3-year survival rates were 63.9%, 51.0%, and 35.7%, respectively. No significant effects of adjunctive therapy on prognosis were found. Pathological grade and access to R0 margin were found to be significantly associated with overall survival. Conclusions Biliary neuroendocrine neoplasms are challenging to diagnose at an early stage due to the absence of typical symptoms. Liver metastasis is relatively common in desease recurrence and is indicative of a poor prognosis., and poor histological grading and the inability to achieve R0 margin significantly shorten the survival period.