Cholangiocarcinoma arising in Caroli's disease

We studied the clinical, roentgenographic, and pathologic features of what is, to our knowledge, the seventh reported case of cholangiocarcinoma associated with Caroli's disease. Review of the literature reveals that the incidence of carcinoma in patients with this condition is higher than that previously reported for other forms of biliary-tract ectasia. Bile stasis, cholangitis, and the presence of carcinogens and promoters in bile may combine to produce the high incidence of carcinoma in these patients.