A 33-year-old woman with clinical and radiologic abnormalities interpreted as primary sclerosing cholangitis died of hepatic failure. Unexpectedly, the autopsy revealed focal dilatation of intrahepatic bile ducts (Caroli’s disease) with cholangiocarcinoma. We know of only four previous cases of cholangiocarcinoma complicating focal dilatation of intrahepatic bile ducts. The lumen of the common hepatic duct and that of the common bile duct was narrowed by striking periductal fibrosis. Although tumor was found in the vicinity of these ducts, the periductal fibrotic sleeves were free of neoplastic tissue. These extrahepatic changes still may have been tumor-related, but they also may have been another, hitherto unobserved, manifestation of the fibropolycystic hepatobiliary diseases.
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