Biliary neuroendocrine tumors: a narrative review of current diagnostics and management strategies

Biliary neuroendocrine tumors (NETs) are exceedingly rare malignancies that differ substantially from more common biliary tract cancers such as cholangiocarcinomas. Recent advances in imaging, pathology, and systemic therapies have prompted a re-evaluation of their clinical management. As research on these tumors grows, a clear and standardized synthesis of emerging evidence is necessary to guide clinicians and inform future investigations. In this article we summarized and critically discuss current knowledge on biliary tract NETs, with the aim of providing a structured narrative review that enhances understanding of their epidemiology, therapeutic approaches, and clinical outcomes.

This narrative review of the literature integrates findings from searches of electronic medical databases, manual reference checks, and authoritative clinical and pathological sources to gather contemporary data on the incidence, diagnostic modalities, treatment strategies, and prognostic factors for biliary NETs.

Current evidence on biliary NETs is examined, including global epidemiologic trends, diagnostic challenges, advances in imaging and histopathological classification, and evolving therapeutic options such as surgery, systemic therapy, and loco-regional treatments. Practical implications for multidisciplinary management are highlighted, with emphasis on how recent data may influence clinical decision-making and patient outcomes.

Biliary NETs are a biologically heterogeneous and rare group of neoplasms. The present management guidelines are largely based on the larger gastrointestinal endocrine tumor (GEP-NET) literature, due to the lack of prospective biliary-specific clinical trials. Localized, well-differentiated lesions can only be cured by surgical resection, but high-grade neuroendocrine carcinomas require platinum-based systemic therapy. Future research must focus on holistic molecular characterization, the development of multicenter registries and the conduct of site-specific therapeutic trials to augment the accuracy of evidence-based clinical practice.