title: Intraductal tubulopapillary neoplasm of the bile duct: potential origin from peribiliary cysts.
abstract: We report two patients with unique biliary tumors histologically similar to pancreatic intraductal tubulopapillary neoplasm (ITPN). One patient underwent right hepatectomy for a partly cystic mass in the hepatic hilum. The other patient had liver transplantation for cryptogenic cirrhosis and multiple hilar cysts detected in the explanted liver, some obliterated by papillary nodules. Histologically both tumors consisted of intracystic non-invasive and well differentiated adenocarcinoma with a papillary and tubular architecture. Associated cysts were peribiliary cysts partly lined by carcinoma cells that were continuous with the intracystic papillotubular masses. Both tumors shared the same immunophenotype: K7(+)/K20(-)/MUC1(+)/MUC2(-)/MUC5AC(-)/MUC6(+). Genetic analysis of KRAS and BRAF revealed wild type genotypes. These pathological and genetic features are similar to those of pancreatic ITPNs. This report suggested that ITPNs may rarely develop in the bile duct seemingly in association with peribiliary cysts.
authors.affiliation: Institute of Liver Studies, King's College Hospital, London SE5 9RS, UK. yoh.zen@kcl.ac.uk
authors.forename: Yoh
authors.initials: Y
authors.lastname: Zen
authors.forename: Anjali D
authors.initials: AD
authors.lastname: Amarapurkar
authors.forename: Bernard C
authors.initials: BC
authors.lastname: Portmann
mesh_terms: D000231:Adenocarcinoma, Papillary / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D001650:Bile Duct Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D001662:Biliary Tract Surgical Procedures; D018281:Cholangiocarcinoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D003560:Cysts / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D004273:DNA, Neoplasm / Q000032:analysis; D006801:Humans; D008297:Male; D008875:Middle Aged
chemical_list: D004273:DNA, Neoplasm