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

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Abstract
BACKGROUND Hepatitis C virus (HCV) infection is a major risk factor for the development of hepatocellular carcinoma. However, the risk factors for primary cholangiocellular carcinoma of the liver (PCC‐L) have not been fully investigated. The authors determined the incidence of PCC‐L in patients with HCV‐related cirrhosis. METHODS Between 1980 and 1997, the authors prospectively studied 600 consecutive patients for the appearance of PCC‐L; these patients were positive for 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 carcinoma. 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. However, the other 11 patients received palliative therapy or chemotherapy. The 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 those 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 results indicate that HCV‐related cirrhosis is a major risk factor for PCC‐L in Japanese patients. Cancer 2000;88:2471–7. © 2000 American Cancer Society.