A case of mixed adenoneuroendocrine carcinoma of the extrahepatic bile duct

Abstract

A 64-year-old man was admitted to our hospital for investigation of jaundice and liver function abnormalities. Abdominal computed tomography (CT) revealed intrahepatic duct dilatation due to tumor at the middle of bile duct of 2cm from the confluence of hepatic ducts. The bile cytology showed adenocarcinoma. Subtotal stomach-preserving pancreatoduodenectomy and extended left hepatectomy with regional lymph node dissection was performed. Pathological examination showed neuroendocrine tumor (NEC) with concurrent adenocarcinoma of the bile duct, finally diagnosed as mixed adenoneuroendocrine carcinoma (MANEC) in accordance with the 2010 WHO Classification of Tumors of the Digestive System. The patient had liver metastases, 6 months after the operation. We started chemotherapy with Gemcitabine and CDDP, but liver metastases progressed, dying 15 months postoperatively. MANEC of the bile duct is poor prognosis. Therefore, multidisciplinary treatment including surgery and chemotherapy must be developed.