Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Nov 27, 2019; 11(11): 743-751
Published online Nov 27, 2019. doi: 10.4254/wjh.v11.i11.743
Cholangiocarcinoma after flow diversion surgery for congenital biliary dilatation: A case report and review of literature
Ryo Ataka, Takashi Ito, Toshihiko Masui, Satoru Seo, Takamichi Ishii, Satoshi Ogiso, Shintaro Yagi, Kojiro Taura, Shinji Uemoto
Ryo Ataka, Takashi Ito, Toshihiko Masui, Satoru Seo, Takamichi Ishii, Satoshi Ogiso, Shintaro Yagi, Kojiro Taura, Shinji Uemoto, Department of Hepato-Biliary-Pancreatic Surgery and Transplantation, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan
Author contributions: Ataka R designed and wrote the manuscript; Ito T performed operation and edited the manuscript; Masui T, Seo S, Ishii T, and Ogiso S performed operation; All authors discussed the results and contributed to the final manuscript.
Informed consent statement: The patient agreed to allow his case to be published including any relevant laboratory data and images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE checklist, and the manuscript was prepared and reviewed according to the CARE checklist.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Takashi Ito, MD, PhD, Doctor, Surgeon, Department of Hepato-Biliary-Pancreatic Surgery and Transplantation, Graduate School of Medicine, Kyoto University, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto 606-8507, Japan. itotaka@kuhp.kyoto-u.ac.jp
Telephone: +81-75-7513242 Fax: +81-75-7514263
Received: July 18, 2019
Peer-review started: July 21, 2019
First decision: August 7, 2019
Revised: August 19, 2019
Accepted: October 2, 2019
Article in press: October 2, 2019
Published online: November 27, 2019
Processing time: 112 Days and 23.8 Hours
Abstract
BACKGROUND

Pancreaticobiliary maljunction (PBM) can be classified into two categories, PBM with congenital biliary dilatation (CBD) or PBM without biliary dilatation, and the management of PBM is often controversial. The treatment for PBM with CBD is prophylactic flow diversion surgery, and some authors have reported that the incidence of cancer after extrahepatic bile duct excision is less than 1%. A very rare case of intrahepatic cholangiocarcinoma 6 years after flow diversion surgery for PBM with CBD is reported.

CASE SUMMARY

A 30-year-old man was diagnosed as having PBM with CBD, Todani classification type IVA, because of abnormal liver enzyme profiles. He underwent flow diversion surgery and cholecystectomy, and the specimen showed adenocarcinoma foci, pT1, pStage IA. Five and a half years passed without any recurrence of bile duct cancer. However, 6 years after his operation, computed tomography showed a gradually growing nodule in the bile duct. Fluorodeoxyglucose positron emission tomography showed high uptake, and magnetic resonance imaging showed restricted diffusion signals. On double balloon enteroscopy, the nodule at the posterior bile duct-jejunum anastomosis was directly visualized, and its biopsy specimen showed adenocarcinoma. The patient underwent right lobectomy and biliary reconstruction. The pathological diagnosis was intraductal papillary neoplasm with high-grade intraepithelial neoplasia, pTis, pN0, pStage 0. The patient’s postoperative course was uneventful, and he has had no recurrence up to the present time.

CONCLUSION

This case suggests the necessity of careful observation after flow diversion surgery, especially when PBM with CBD is detected in adulthood.

Keywords: Congenital biliary dilatation; Pancreaticobiliary maljunction; Cholangiocarcinoma; Recurrence; Metachronous neoplasm; Case report

Core tip: Pancreaticobiliary maljunction (PBM) is one of the risk factors for biliary tract cancer. A rare case of intrahepatic cholangiocarcinoma 6 years after flow diversion surgery for PBM with congenital biliary dilatation (CBD) is presented. Careful follow-up after flow diversion surgery is important to detect cholangiocarcinoma in its early stage, especially when PBM with CBD is detected in adulthood, and when cancer has already developed in the bile duct.