Fung BM, Lindor KD, Tabibian JH. Cancer risk in primary sclerosing cholangitis: Epidemiology, prevention, and surveillance strategies. World J Gastroenterol 2019; 25(6): 659-671 [PMID: 30783370 DOI: 10.3748/wjg.v25.i6.659]
Corresponding Author of This Article
James H Tabibian, MD, PhD, Health Sciences Clinical Associate Professor, David Geffen School of Medicine at UCLA, Director of Endoscopy, Department of Medicine, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B-182, Sylmar, CA 91342, United States. jtabibian@dhs.lacounty.gov
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Minireviews
Open-Access Policy of This Article
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/
World J Gastroenterol. Feb 14, 2019; 25(6): 659-671 Published online Feb 14, 2019. doi: 10.3748/wjg.v25.i6.659
Cancer risk in primary sclerosing cholangitis: Epidemiology, prevention, and surveillance strategies
Brian M Fung, Keith D Lindor, James H Tabibian
Brian M Fung, UCLA-Olive View Internal Medicine Residency Program, Olive View-UCLA Medical Center, Sylmar, CA 91342, United States
Keith D Lindor, Office of the University Provost, Arizona State University, Phoenix, AZ 85004, United States
James H Tabibian, Division of Gastroenterology, Department of Medicine, Olive View-UCLA Medical Center, Sylmar, CA 91342, United States
Author contributions: Tabibian JH and Fung BM reviewed the literature for relevant original studies and other content; Fung BM designed and/or formatted the figures; Tabibian JH and Lindor KD reviewed the figures; Fung BM drafted the manuscript; Tabibian JH and Lindor KD provided supervision; all authors provided critical input and approved of the manuscript.
Conflict-of-interest statement: The authors have no financial disclosures or conflicts of interest.
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: James H Tabibian, MD, PhD, Health Sciences Clinical Associate Professor, David Geffen School of Medicine at UCLA, Director of Endoscopy, Department of Medicine, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B-182, Sylmar, CA 91342, United States. jtabibian@dhs.lacounty.gov
Telephone: +1-747-2103205 Fax: +1-747-2104573
Received: October 30, 2018 Peer-review started: November 1, 2018 First decision: November 29, 2018 Revised: January 10, 2019 Accepted: January 14, 2019 Article in press: January 15, 2019 Published online: February 14, 2019
Abstract
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by progressive fibroinflammatory destruction of the intra- and/or extrahepatic biliary ducts. While its features and disease course can be variable, most patients with PSC have concurrent inflammatory bowel disease and will eventually develop liver cirrhosis and end-stage liver disease, with liver transplantation representing the only potentially curative option. Importantly, PSC is associated with a significantly increased risk of malignancy compared to the general population, mainly cholangiocarcinoma, gallbladder carcinoma, hepatocellular carcinoma, and colorectal cancer, with nearly 50% of deaths in patients with PSC being due to cancer. Therefore, robust surveillance strategies are needed, though uncertainty remains regarding how to best do so. In this review, we discuss the epidemiology, prevention, and surveillance of cancers in patients with PSC. Where evidence is limited, we present pragmatic approaches based on currently available data and expert opinion.
Core tip: Primary sclerosing cholangitis is a rare cholestatic liver disease characterized by progressive fibroinflammatory destruction of the bile ducts. It is associated with a significantly increased risk of malignancy over the general population, with nearly 50% of deaths in patients with primary sclerosing cholangitis caused by cancer, thus necessitating robust surveillance strategies. In this article, we provide a synopsis of the epidemiology, prevention, and surveillance of cancers in patients with primary sclerosing cholangitis.
