title: Incidence, etiologic aspects and clinicopathologic features in intrahepatic cholangiocellular carcinoma--a study of 51 cases from a low-endemicity area.
abstract: 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%).
authors.affiliation: Department of Medicine, Göteborg University, Sahlgren's University Hospital, Ostra, Sweden.
authors.forename: J
authors.initials: J
authors.lastname: Kaczynski
authors.forename: G
authors.initials: G
authors.lastname: Hansson
authors.forename: S
authors.initials: S
authors.lastname: Wallerstedt
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001650:Bile Duct Neoplasms / Q000453:epidemiology* / Q000209:etiology / Q000473:pathology; D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D001659:Biliary Tract; D006528:Carcinoma, Hepatocellular / Q000453:epidemiology; D018281:Cholangiocarcinoma / Q000453:epidemiology* / Q000209:etiology / Q000556:secondary; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D011379:Prognosis; D014680:Veins