Bile duct carcinoma in patients with ulcerative colitis. Relationship to sclerosing cholangitis: report of six cases and review of the literature.

Six cases of bile duct carcinoma were encountered among 1207 patients with ulcerative colitis, a prevalence rate of 0.5%. The relative risk of bile duct carcinoma in patients with ulcerative colitis was 31.3. Colitis was extensive in all six patients with a mean duration of 23.2 years before the diagnosis of carcinoma. The mean age at the diagnosis of carcinoma was 38.5 years. Three patients had undergone colectomy 5-16 years earlier, and in four patients pericholangitis and sclerosing cholangitis preexisted. The tumors, histologically adenocarcinomas, were located in the common bile duct in five patients and in the hepatic duct in one. The mean survival was 11.8 months (one patient is still alive with recurrent carcinoma). Pericholangitis and sclerosing cholangitis is a frequent preexisting lesion in patients with bile duct carcinoma complicating ulcerative colitis and may be considered a premalignant lesion in these patients. Both sclerosing cholangitis and bile duct carcinoma are rare in Crohn's disease.