Cancer surveillance in patients with primary sclerosing cholangitis

Abstract

Primary sclerosing cholangitis (PSC) is a chronic fibroinflammatory syndrome involving the biliary tract, often accompanied by inflammatory bowel disease (IBD). This syndrome is a prototype disease linking chronic inflammation to carcinogenesis. Indeed, PSC is associated with an increased risk of cholangiocarcinoma (CCA), gallbladder cancer, hepatocellular carcinoma (HCC), and colorectal cancer. Herein, we review the risk for these malignancies in PSC and discuss rational cancer surveillance strategies for these patients. Where evidence is limited, we suggest a pragmatic approach. In this regard, we recommend interval screening for CCA with noninvasive imaging modalities and serum carbohydrate antigen 19-9 determinations annually. These imaging studies also serve to screen for gallbladder cancer and HCC. Screening for colorectal cancer is more firmly established in PSC patients with IBD and includes colonoscopy at the time of PSC diagnosis and, thereafter, at 1-2-year intervals. We also highlight areas where more information is required, such as management of biliary tract dysplasia and cancer chemoprevention in PSC.

Fig. 1

Recommendations for decision process for CCA surveillance in PSC. Abbreviations: CCA, cholangiocarcinoma; PSC, primary sclerosing cholangitis; MRI, magnetic resonance imaging; MRCP, magnetic resonance cholangiopancreatography; CA 19-9, carbohydrate antigen 19-9; ERCP, endoscopic retrograde cholangiopancreatography; FISH, fluorescence in situ hybridization.

Fig. 2

Recommendations for decision process for GBC surveillance in PSC. Abbreviations: GBC, gallbladder cancer; PSC, primary sclerosing cholangitis; MRI, magnetic resonance imaging; MRCP, magnetic resonance cholangiopancreatography.

Fig. 3

Recommendations for decision process for CRC surveillance in PSC. Abbreviations: CRC, colorectal cancer; PSC, primary sclerosing cholangitis; IBD, inflammatory bowel disease; HGD, high-grade dysplasia; LGD, low-grade dysplasia.