Publication Name	World Journal of Gastroenterology
Manuscript ID	54317
Country	Switzerland

Category	Surgery
Manuscript Type	Systematic Reviews
Article Title	Risk of malignancy in Caroli disease and syndrome: A systematic review
Manuscript Source	Invited Manuscript
All Author List	René Fahrner, Sandra GC Dennler and Daniel Inderbitzin
Funding Agency and Grant Number
Corresponding Author	René Fahrner, MD, Surgeon, Department of Surgery, Bürgerspital Solothurn, Schöngrünstrasse 42, Solothurn 4500, Switzerland. rene.fahrner@spital.so.ch
Key Words	Malignancy; Caroli disease; Caroli syndrome; Tumor; Cholangiocarcinoma; Review
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.
Citation	Fahrner R, Dennler SG, Inderbitzin D. Risk of malignancy in Caroli disease and syndrome: A systematic review. World J Gastroenterol 2020; 26(31): 4718-4728

Received	2020-01-20 11:03
Peer-Review Started	2020-01-20 11:07
To Make the First Decision
Return for Revision	2020-02-27 03:33
Revised	2020-05-15 22:10
Second Decision	2020-07-31 09:44
Accepted by Journal Editor-in-Chief
Accepted by Company Editor-in-Chief	2020-08-01 05:51
Articles in Press	2020-08-01 05:51
Publication Fee Transferred
Edit the Manuscript by Language Editor
Typeset the Manuscript	2020-08-17 00:29

ISSN	1007-9327 (print) and 2219-2840 (online)
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/
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