Neuroendocrine tumor of common hepatic duct

Extrahepatic bile ducts (EHBD) remain one of the rarest primary sites for neuroendocrine tumors (NETs) accounting for 0.1 % to 0.2 % of all NETs of the gastrointestinal tract. We present one such case, a 28-year-old female with vague abdominal symptoms and periportal lymph node enlargement on radiological examination. Endoscopic retrograde cholangiopancreaticography revealed 1.5 cms long common hepatic duct stricture and brush cytology showed no malignant cells. Endoscopic ultrasound guided fine needle aspiration cytology from the lymph nodes reported malignant cells. Roux-en-Y hepaticojejunostomy with excision of the extrahepatic biliary tree and radical lymphadenectomy was done. The diagnosis of well-differentiated neuroendocrine carcinoma was confirmed on histological and immunohistochemical studies. NETs of biliary tract are difficult to diagnose preoperatively. The aggressive use of curative and cytoreductive surgery is the frontline of treatment. But the rare alternative diagnosis of NETs should be kept in mind by the surgeon for proper timely management.