title: Well differentiated intrahepatic cholangiocarcinoma in the setting of biliary papillomatosis: a case report and review of the literature.
abstract: 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.
authors.affiliation: Department of Surgery, University of Calgary, Calgary, Alberta.
authors.forename: Heather
authors.initials: H
authors.lastname: Cox
authors.forename: Michael
authors.initials: M
authors.lastname: Ma
authors.forename: Ronald
authors.initials: R
authors.lastname: Bridges
authors.forename: Estifanos
authors.initials: E
authors.lastname: Debru
authors.forename: Oliver
authors.initials: O
authors.lastname: Bathe
authors.forename: Francis
authors.initials: F
authors.lastname: Sutherland
authors.forename: Elijah
authors.initials: E
authors.lastname: Dixon
mesh_terms: D001650:Bile Duct Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D001652:Bile Ducts / Q000473:pathology; D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D001707:Biopsy, Needle; D018281:Cholangiocarcinoma / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D002760:Cholangiopancreatography, Endoscopic Retrograde / Q000379:methods; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D007813:Laparotomy / Q000379:methods; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D010212:Papilloma / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D018570:Risk Assessment; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome