Well differentiated intrahepatic cholangiocarcinoma in the setting of biliary papillomatosis: a case report and review of the literature.

A 64-year-old man presented with long-standing, vague, epigastric abdominal pain. History, physical examination and laboratory studies were noncontributory. However, serial computed tomography scans revealed a rapidly progressive mass in segment 2 of the liver. Surprisingly, surgical pathology revealed a well-differentiated intrahepatic cholangiocarcinoma associated with biliary papillomatosis (BP). BP is a rare, benign and potentially fatal disease of the intra- and extrahepatic bile ducts. It is typified by numerous multicentric papillary fronds arising from biliary columnar epithelium. Most patients present with symptoms of jaundice and cholangitis. Although a benign disease, a review of the literature demonstrated that BP often recurs after surgical resection, carries a poor prognosis and has a moderately high malignant transformation rate. Treatment options for BP include surgical resection, transplant, ablation, stenting and/or bypass.