title: Incidence of primary cholangiocellular carcinoma of the liver in japanese patients with hepatitis C virus-related cirrhosis.
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.
authors.affiliation: Department of Gastroenterology, Toranomon Hospital, Tokyo, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Kobayashi
authors.forename: K
authors.initials: K
authors.lastname: Ikeda
authors.forename: S
authors.initials: S
authors.lastname: Saitoh
authors.forename: F
authors.initials: F
authors.lastname: Suzuki
authors.forename: A
authors.initials: A
authors.lastname: Tsubota
authors.forename: Y
authors.initials: Y
authors.lastname: Suzuki
authors.forename: Y
authors.initials: Y
authors.lastname: Arase
authors.forename: N
authors.initials: N
authors.lastname: Murashima
authors.forename: K
authors.initials: K
authors.lastname: Chayama
authors.forename: H
authors.initials: H
authors.lastname: Kumada
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D006528:Carcinoma, Hepatocellular / Q000453:epidemiology / Q000401:mortality / Q000821:virology; D018281:Cholangiocarcinoma / Q000453:epidemiology* / Q000401:mortality / Q000821:virology; D005260:Female; D016174:Hepacivirus; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008103:Liver Cirrhosis / Q000453:epidemiology* / Q000821:virology; D008113:Liver Neoplasms / Q000453:epidemiology* / Q000401:mortality / Q000821:virology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012307:Risk Factors; D015996:Survival Rate