Abstract
In primary biliary cirrhosis (PBC), the development of hepatocellular carcinoma (HCC) was thought to represent a rare complication. In contrast, extrahepatic malignancies have been reported to be significantly associated with PBC. The aim of this study was to determine the incidence of HCC and of extrahepatic malignancies in a large cohort of patients with PBC. A total of 212 patients with documented PBC (19 men and 193 women) were followed up for a median of 6 (range, 1-23) years. In total, 23 (10.8%) cases of malignancy were diagnosed; eight (3.8%) patients with HCC and 15 (7.0%) with extrahepatic malignancies. PBC patients were found to have a 10-year risk of 4% for developing HCC and of 13% for developing extrahepatic malignancies. The risk for HCC was significantly higher in the PBC patients with cirrhosis (15% at 10 years of follow-up). In contrast, the histologic stage of PBC does not influence the risk for extrahepatic malignancy. Our study confirms that there is a risk of HCC in Greek patients with PBC, particularly in patients with stage IV PBC. The risk of extrahepatic malignancies is higher than that of HCC, but it is not influenced by the histologic stage of the liver disease.
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