title: Hepatocellular carcinoma in association with bile duct hamartomas: report on 2 cases and review of the literature.
abstract: Biliary hamartomas are hepatic lesions related to defects in the ductal plate and are part of the spectrum of adult polycystic disease. Microscopically, the lesions are small and consist of irregular bile ducts amidst a fibrous stroma. There are reports on the malignant transformation of biliary hamartomas, particularly on cholangiocarcinoma. Case 1: a 19-year-old woman presented with increased abdominal volume, lumbar pain, jaundice, choluria, cachexia, and progression to hepatic insufficiency. At autopsy, the liver weighed 8850 g and showed a 20.5-cm mass in the right lobe and nodules in the left lobe. Case 2: a 39-year-old man presented with an asymptomatic nodule in the right hepatic lobe. A 6.5-cm liver segment containing a 3.0-cm nodule was excised. Histopathology of both cases revealed hepatocellular carcinoma associated with multiple bile duct hamartomas (von Meyenburg complex). Bile duct hamartoma is generally an incidental finding at laparotomy and autopsy because its course is asymptomatic. The literature has documented its possible progression to malignant neoplasia. However, this relationship has only been established with cholangiocarcinomas. We report herein for the first time on the association of the lesion with hepatocellular carcinoma.
authors.affiliation: Department of Pathology, Paulista School of Medicine/Federal University of São Paulo, EPM/UNIFESP 04023-900 São Paulo SP, Brazil.
authors.forename: Thais
authors.initials: T
authors.lastname: Heinke
authors.forename: Lucila Bohme
authors.initials: LB
authors.lastname: Pellacani
authors.forename: Henrique de Oliveira
authors.initials: Hde O
authors.lastname: Costa
authors.forename: Rogério Aoki
authors.initials: RA
authors.lastname: Fuziy
authors.forename: Marcello
authors.initials: M
authors.lastname: Franco
mesh_terms: D000328:Adult; D001649:Bile Duct Diseases / Q000473:pathology*; D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D006528:Carcinoma, Hepatocellular / Q000473:pathology* / Q000601:surgery; D017809:Fatal Outcome; D005260:Female; D006222:Hamartoma / Q000473:pathology*; D006801:Humans; D008113:Liver Neoplasms / Q000473:pathology* / Q000601:surgery; D008297:Male