Risk of Hepatobiliary-Gastrointestinal Malignancies and Appropriate Cancer Surveillance in Patients With Primary Sclerosing Cholangitis

Patients with primary sclerosing cholangitis (PSC) are at risk of hepatobiliary and gastrointestinal cancers. Increased risk of cancer is a result of the chronic, progressive fibro-inflammatory state which ultimately results in the destruction of biliary ducts. PSC is often associated with inflammatory bowel disease (IBD). Patients with PSC are at significant risk of cholangiocarcinoma (CCA), gall bladder malignancy and those with IBD are at increased risk of colorectal cancer. It is important to implement cancer surveillance protocols in these patients. The aim of these protocols is the prevention or early detection of cancerous or pre-cancerous lesions. Given that PSC is rare, large prospective studies evaluating the risk of malignancy in these patients are not available. A great deal of uncertainty exists regarding how to best implement cancer surveillance in these patients. About 50% of deaths in PSC patients are due to malignancy and many patients eventually progress to end-stage liver disease and succumb to hepatic failure. In this review, we cover cancer surveillance strategies in PSC patients based on existing literature and expert opinions.