Challenges of cholangiocarcinoma detection in patients with primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic, cholestatic, idiopathic liver disease characterized by fibroobliterative inflammation of the hepatic bile ducts. In a clinically significant proportion of patients, PSC progresses to cirrhosis, end-stage liver disease, and in some cases, cholangiocarcinoma (CCA). The development of CCA in PSC is unpredictable, its surveillance and diagnosis complex, and its treatment options limited unless detected early. Herein we provide a focused review of the current literature regarding CCA surveillance in patients with PSC and discuss the diagnostic and management challenges that exist. Where evidence is limited, we present our perspective and approach as well as directions for future research.