Incidence of primary cholangiocellular carcinoma of the liver in Japanese patients with hepatitis C virus-related cirrhosis

BACKGROUND, Hepatitis C virus (HCV) infection is a major risk factor for th e development of hepatocellular carcinoma. However, the risk factors for pr imary cholangiocellular carcinoma of the liver (PCC-L) have not been fully investigated. The authors determined the incidence of PCC-L in patients wit h HCV-related cirrhosis. METHODS, Between 1980 and 1997, the authors prospectively studied 600 conse cutive patients for the appearance of PCC-L; these patients were positive f or HCV and later developed cirrhosis. The follow-up period ranged from 0 to 18.5 years (median, 7.2 years). RESULTS. During the observation period, PCC-L developed in 14 patients (2.3 %). Among these, 11 (1.8%) had cholangiocellular carcinomas and the other 3 (0.5%) had a combined type of hepatocellular and cholangiocellular carcino ma. Within the same period, hepatocellular carcinoma (HCC) developed in 206 patients (34.3%). The cumulative rates of newly diagnosed PCC-L were 1.6% at 5 years and 3.5% at 10 years, which was about 1000 times higher than the estimated incidence of PCC-L in the general population of Japan. PCC-L was treated by surgical resection in 3 patients who survived for > 3 years. Ho wever, the other 11 patients received palliative therapy or chemotherapy. T he survival rates among PCC-L patients were 39.3%, 23.6%, and 16.5% at the end of 1, 3, and 5 years, respectively, and were significantly lower than t hose of HCC (P = 0.0001). CONCLUSIONS. The results of this study show a relatively high incidence of PCC-L in patients with HCV-related cirrhosis, and also show that this type of liver cancer is associated with a relatively poor prognosis. These resul ts indicate that HCV-related cirrhosis is a major risk factor for PCC-L in Japanese patients. (C) 2000 American Cancer Society.