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Korean J Gastroenterol  <  Volume 72(6); 2018 <  Articles

Korean J Gastroenterol 2018; 72(6): 318-321  https://doi.org/10.4166/kjg.2018.72.6.318
Large Cell Neuroendocrine Carcinoma of the Extrahepatic Bile Duct
Ji Young Park and Tae Joo Jeon
Division of Gastroenterology, Department of Internal Medicine, Sanggye Paik Hospital, Inje University College of Medicine, Seoul, Korea
Correspondence to: Tae Joo Jeon, Division of Gastroenterology, Department of Internal Medicine, Sanggye Paik Hospital, Inje University College of Medicine, 1342 Dongil-ro, Nowon-gu, Seoul 01757, Korea. Tel: +82-2-950-1341, Fax: +82-2-950-1955, E-mail: drjtj@paik.ac.kr, ORCID: https://orcid.org/0000-0002-8137-1633
Received: March 18, 2018; Revised: May 24, 2018; Accepted: June 2, 2018; Published online: December 25, 2018.
© The Korean Journal of Gastroenterology. All rights reserved.

This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Primary neuroendocrine tumors originating from the extrahepatic bile duct are rare. Among these tumors, large cell neuroendocrine carcinomas (NECs) are extremely rare. A 59-year-old man was admitted to Sanggye Paik Hospital with jaundice that started 10 days previously. He had a history of laparoscopic cholecystectomy, which he had undergone 12 years previously due to chronic calculous cholecystitis. Laboratory data showed abnormally elevated levels of total bilirubin 15.3 mg/dL (normal 0.2-1.2 mg/dL), AST 200 IU (normal 0-40 IU), ALT 390 IU (normal 0-40 IU), and gamma-glutamyl transferase 1,288 U/L (normal 0-60 U/L). Serum CEA was normal, but CA 19-9 was elevated 5,863 U/mL (normal 0-37 U/mL). Abdominal CT revealed a 4.5 cm sized mass involving the common bile duct and liver hilum and dilatation of both intrahepatic ducts. Percutaneous transhepatic drainage in the left hepatic duct was performed for preoperative biliary drainage. The patient underwent radical common bile duct and Roux-en-Y hepaticojejunostomy for histopathological diagnosis and surgical excision. On histopathological examination, the tumor exhibited large cell NEC (mitotic index >20/10 high-power field, Ki-67 index >20%, CD56 [+], synaptophysin [+], chromogranin [+]). Adjuvant concurrent chemotherapy and radiotherapy were started because the tumor had invaded the proximal resection margin. No recurrence was detected at 10 months by follow-up CT.
Keywords: Carcinoma, neuroendocrine; Bile duct neoplasms

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