Abstract
Patients with primary sclerosing cholangitis (PSC) confer a high risk of cholangiocarcinoma (CCA). The molecular mechanisms of CCA development in PSC are incompletely understood, but pro-oncogenic processes resulting from chronic biliary inflammation are presumably of central importance. Distinguishing benign from malignant biliary strictures in PSC patients is challenging and accurately diagnosing CCA in PSC often requires a multifaceted approach involving imaging, serological testing, biliary brush cytology and fluorescence in situ hybridization (FISH). Lack of early detection tools leads to a late diagnosis in the majority of cases. Surgical resection or liver transplantation represent the only curative intent treatments in PSC-CCA, but is only an option for the small subset of patients where CCA is detected at an early stage. Current palliative treatment modalities result in only a modest increase in survival. Overall, PSC-CCA carries a dismal prognosis with a 5-year survival less than 20%. Advances aiming at improving strategies for early detection, treatment and surveillance of CCA will be essential to provide better future patient care for PSC patients. Herein, we review the pathogenetic mechanisms for PSC-CCA as well as strategies for diagnosing and managing premalignant and malignant stages of CCA in PSC.
Highlights
Primary sclerosing cholangitis (PSC) is a chronic immune‐mediated liver disease characterized by multifocal inflammatory and fibrotic strictures of the large intra‐ and extrahepatic bile ducts.[1]
primary sclerosing cholangitis (PSC)‐CCA carries a dismal prognosis with a 5‐year survival rate of less than 20%
CCA development represents a major determinant for poor progno‐ sis in PSC, and lack of accurate diagnostic methods for early detec‐ tion of CCA and the limited therapeutic options for advanced stages of CCA are among the main challenges in current handling of PSC patients
Summary
Primary sclerosing cholangitis (PSC) is a chronic immune‐mediated liver disease characterized by multifocal inflammatory and fibrotic strictures of the large intra‐ and extrahepatic bile ducts.[1] The esti‐ mated prevalence of PSC is in the range of 1‐16 per 100 000, with significant regional differences in prevalence across Europe.[1] The disease primarily affects young individuals (median age of onset 30‐40 years) with concurrent inflammatory bowel disease (IBD) (approximately 2/3 of patients).[1] In absence of any effective medi‐ cal treatment, progressive bile duct disruption leads to cholestasis and hepatic injury with liver transplantation representing the only curative intervention.[1] Outcome after liver transplantation is good with a 5‐year survival rate above 70%, but biliary tract cancers
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