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Review
. 2019 Feb 14;25(6):659-671.
doi: 10.3748/wjg.v25.i6.659.

Cancer risk in primary sclerosing cholangitis: Epidemiology, prevention, and surveillance strategies

Brian M Fung  1 , Keith D Lindor  2 , James H Tabibian  3
Affiliations
Review

Cancer risk in primary sclerosing cholangitis: Epidemiology, prevention, and surveillance strategies

Brian M Fung et al. World J Gastroenterol. .

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.

Keywords: Bile duct diseases; Chemoprotection; Cholangiocarcinoma; Colorectal cancer; Gallbladder carcinoma; Hepatocellular carcinoma; Inflammatory bowel disease.

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Conflict of interest statement

Conflict-of-interest statement: The authors have no financial disclosures or conflicts of interest.

Figures

Figure 1
Figure 1
Lifetime incidence of various cancers associated with primary sclerosing cholangitis and their respective risks compared with the general population. Estimations of lifetime incidence are made from limited available data (predominantly based on 10- to 30-year longitudinal studies), and thus may often underestimate true lifetime risk. 1The risk in patients with PSC-IBD (not PSC alone) is 4× the risk in patients with UC alone and 10× the risk in the general population. PSC: Primary sclerosing cholangitis; PSC-IBD: Inflammatory bowel disease co-existing with primary sclerosing cholangitis; UC: Ulcerative colitis.
Figure 2
Figure 2
Overview of cancer surveillance in patients with primary sclerosing cholangitis, beginning at time of primary sclerosing cholangitis diagnosis. This overview is based on recommendations from the American Association for the Study of Liver Disease practice guidelines[44]. CCA: Cholangiocarcinoma; GBC: Gallbladder carcinoma; HCC: Hepatocellular carcinoma; IBD: Inflammatory bowel disease; US: Ultrasound; PSC: Primary sclerosing cholangitis.
Figure 3
Figure 3
Suggested cholangiocarcinoma surveillance in patients with primary sclerosing cholangitis. Adapted from Tabibian et al[103], with permission. Red arrows represent assumes stable findings; CA 19-9: Carbohydrate antigen 19-9; CCA: Cholangiocarcinoma; ERC: Endoscopic retrograde cholangiography; GB: Gallbladder; GBC: Gallbladder cancer; FISH: Fluorescence in situ hybridization; HCC: Hepatocellular carcinoma; MRCP: Magnetic resonance cholangiopancreatography; US: Ultrasound; ERCP: Endoscopic retrograde cholangiopancreatography; MRI: Magnetic resonance imaging.
See this image and copyright information in PMC

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