Treatment of cholangiocarcinoma in patients with primary sclerosing cholangitis: a comprehensive review.

ABSTRACT: Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease, characterised by persistent biliary inflammation resulting in fibrosis and multifocal strictures of the biliary tree. The course of disease is highly variable, ranging from asymptomatic disease to the development of end-stage biliary cirrhosis and an increased risk of biliary tract cancer (BTC), particularly cholangiocarcinoma (CCA). PSC is the most important risk factor for CCA in younger people, with a reported lifetime prevalence ranging from 6% to 13%. Perihilar CCA (pCCA), involving the hepatic duct bifurcation, is the most common CCA amounting to approximately 50% of all cases, whereas intrahepatic CCA (iCCA), located within the hepatic parenchyma, represents less than 10%. CCA is an aggressive tumour, and only

SUBMITTER: Villard C 

PROVIDER: S-EPMC11770459 | biostudies-literature | 2024 Jan

REPOSITORIES: biostudies-literature