Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 16, 2021; 9(29): 8773-8781
Published online Oct 16, 2021. doi: 10.12998/wjcc.v9.i29.8773
Isolated mass-forming IgG4-related sclerosing cholangitis masquerading as extrahepatic cholangiocarcinoma: A case report
Sanghyun Song, Sungho Jo
Sanghyun Song, Sungho Jo, Department of Surgery, Dankook University Hospital, Cheonan 31116, Chungnam Province, South Korea
Author contributions: Song S participated in the operation, reviewed the literature, and drafted the manuscript; Jo S performed the operation, designed the report, and was responsible for the revision of the manuscript for important intellectual content; all authors were involved in managing the case and editing the manuscript, and issued final approval for the version to be submitted.
Informed consent statement: Written informed consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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: Sungho Jo, MD, PhD, Professor, Department of Surgery, Dankook University Hospital, 201 Manghyang-ro, Dongnam-gu, Cheonan 31116, Chungnam Province, South Korea. agapejsh@dankook.ac.kr
Received: June 26, 2021
Peer-review started: June 26, 2021
First decision: July 16, 2021
Revised: July 20, 2021
Accepted: July 29, 2021
Article in press: July 29, 2021
Published online: October 16, 2021
Processing time: 110 Days and 16.5 Hours
Core Tip

Core Tip: IgG4-related sclerosing cholangitis (IgG4-RSC) is an emerging disease and shows frequent other-organ involvement, including of the pancreas. However, when isolation without pancreatic involvement and biliary mass formation are combined, IgG4-RSC becomes extremely rare and brings about a striking confusion in differentiation from cholangiocarcinoma. Avoiding unnecessary surgery and providing proper treatment in patients with isolated and mass-forming IgG4-RSC could be achieved through high index of suspicion according to the amount of information on the five cardinal features (histology, imaging, serology, other-organ involvement, and response to steroid therapy).