Advances in the diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis

✍ Scribed by Laura E. Moreno Luna; Gregory J. Gores

• Publisher: John Wiley and Sons
• Year: 2006
• Tongue: English
• Weight: 170 KB
• Volume: 12
• Category: Article
• ISSN: 1527-6465
• DOI: 10.1002/lt.20938

✦ Synopsis

Primary sclerosing cholangitis (PSC) is an idiopathic chronic cholestatic liver disease characterized by inflammatory destruction of the biliary tree. It slowly progresses, resulting in biliary fibrosis and obliteration leading to end-stage liver disease. The most feared complication of PSC is the development of cholangiocarcinoma (CCA). 1 The prevalence of CCA in patients with PSC ranges 4.8-36.4%. 2-7 PSC is the most common known predisposing factor for CCA in the United Kingdom (lifetime risk 5-15%). 8 It has been reported that the incidence of developing CCA in patients with PSC is 0.6-1.5% per year. 9,10 The highest incidence is the first 2 years after the diagnosis of PSC. Indeed, in 37-50% of patients with PSC who develop CCA, the diagnosis of malignancy was made within the first year after the diagnosis of PSC. 10,11 Either only a certain subset of patients with PSC are at risk for the development of CCA and do so early in the course of their disease, or many patients have symptomatic, difficult-to-diagnose CCA at presentation. CCA in patients with PSC has an onset about 3 decades earlier than CCA in patients without PSC. 12 The cholangiographic findings and clinical symptoms of CCA are nonspecific and are difficult to differentiate from benign dominant bile duct strictures, which are common in PSC. 13 Therefore, CCA in the setting of PSC remains difficult to diagnose. It is often found at autopsy as an advanced stage of disease, or unexpectedly at the time of liver transplantation. 10,12,14