Incidence, etiologic aspects and clinicopathologic features in intrahepatic cholangiocellular carcinoma--a study of 51 cases from a low-endemicity area.

A total of 51 cases (19 males and 32 females) of intrahepatic cholangiocellular carcinoma (CCC) from a low-endemicity area of primary liver cancer was analyzed during the periods from 1958 to 1979 and from 1984 to 1991. The mean annual age-adjusted incidence rate was 0.44 for males and 0.56 for females per 100,000 inhabitants. CCC was diagnosed before death in only 31%. There was a female predominance in patients over 70 years of age (p < 0.05). At presentation, malaise (85%), weight loss (73%) abdominal pain (50%) and hepatomegaly (80%) were common. The median survival time from diagnosis was 2 months. The mean age at the time of death was 72 years (range 41-92). At autopsy, cholelithiasis was found in 61% (81% in patients older than 70 years) and cirrhosis in 30% of patients. Cholelithiasis was more common in CCC (p < 0.01) than in hepatocellular carcinoma cases with the same mean age. Not one case of inflammatory bowel disease was found. The gross appearance of the tumor was predominantly massive (49%) or multinodular (35%). The most common histological features were tubular pattern of growth (82%) and abundant fibrous stroma. Metastases were particularly associated with the lymph nodes (41%), skeleton (26%) and lungs (16%).