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©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Aug 21, 2020; 26(31): 4718-4728
Published online Aug 21, 2020. doi: 10.3748/wjg.v26.i31.4718
Published online Aug 21, 2020. doi: 10.3748/wjg.v26.i31.4718
Risk of malignancy in Caroli disease and syndrome: A systematic review
René Fahrner, Daniel Inderbitzin, Department of Surgery, Bürgerspital Solothurn, Solothurn 4500, Switzerland
Sandra GC Dennler, Department of Surgery, University Hospital Dresden, Dresden 01307, Germany
Author contributions: Fahrner R was involved in the conceptualization, data curation, investigation, project administration, writing-original draft; Dennler SGC was involved in the conceptualization, data curation, investigation, writing review and editing; Inderbitzin D was involved in the conceptualization, data curation, investigation, writing review and editing. All authors have read and approve the final manuscript.
Conflict-of-interest statement: There are no conflicts of interest.
PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 checklist, and the manuscript was prepared and revised accordingly.
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: René Fahrner, MD, Surgeon, Department of Surgery, Bürgerspital Solothurn, Schöngrünstrasse 42, Solothurn 4500, Switzerland. rene.fahrner@spital.so.ch
Received: January 20, 2020
Peer-review started: January 20, 2020
First decision: February 27, 2020
Revised: May 15, 2020
Accepted: August 1, 2020
Article in press: August 1, 2020
Published online: August 21, 2020
Peer-review started: January 20, 2020
First decision: February 27, 2020
Revised: May 15, 2020
Accepted: August 1, 2020
Article in press: August 1, 2020
Published online: August 21, 2020
Core Tip
Core tip: Congenital intrahepatic bile duct dilatation with or without fibrosis is called Caroli syndrome or Caroli disease, respectively. We performed a systematic review of the current literature to investigate the incidence of malignant transformation, risk factors, and surgical treatment options. There were only 12 retrospective cohort studies reporting 561 patients, including 37 patients with malignancy, so an exact preoperative diagnostic work-up, risk factors and preferred surgical treatment were impossible to conclude due to lack of data. Overall survival rates after one year were low (36%), and recurrence rate was high (75%) during the observation period.