Silent Neuroendocrine Tumor of Biliary Confluence

Neuroendocrine tumors (NETs) of the extrahepatic bile ducts are extremely rare. They are heterogeneous entities of varied histopathological features. The pathological types vary greatly with regards to biological behavior and prognosis. We presented such a case of silent tumor of the biliary tract of neuroendocrine origin in a middle-aged female with nonspecific abdominal pain without any clinically obvious signs which on radiological imaging revealed a tumor of hepatic confluence with metastatic deposits in liver for which she underwent left trisectionectomy with segment 7 metastatectomy. As per the literature reviews regarding extrahepatic bile duct NET these tumors are more common in the middle age population with a female preponderance. The tumors were symptomatic in majority of patients and the symptoms are mostly related to tumor mass and its invasion of adjacent structures or metastases rather than hormone and vasoactive peptide secretions. The low incidence and uncommon modes of presentation makes the diagnosis tough and management challenging.