PSC-Associated Cholangiocarcinoma: Diagnostic and Therapeutic Considerations

Primary sclerosing cholangitis (PSC) is a chronic inflammatory liver disease, of unknown etiology, targeting cholangiocytes in any portion of the biliary system. Cholangiocarcinoma (CCA) is a feared complication of PSC, with a lifetime risk of 7–14%, no matter the duration of underlying PSC. Since a prominent fibrotic reaction closely abutting the bile ducts is a common trait of both PSC and CCA, PSC may be regarded as paradigmatic of the pathogenetic sequence leading from chronic inflammatory epithelial damage to malignant transformation. The risk of CCA is a major challenge in the management of PSC, raising several questions about disease surveillance, early tumor detection, prevention, and treatment. Herein, we discuss the critical issues of PSC-associated CCA, highlighting the need for a multimodal approach where recent advances in imaging studies, endoscopic procedures, and novel tumoral biomarkers are emphasized.