Hepatocellular carcinoma in association with bile duct hamartomas: report on 2 cases and review of the literature

Hepatocellular carcinoma in association with bile duct hamartomas: report on 2 cases and review of the literature

Author Heinke, Thais Autor UNIFESP Google Scholar
Pellacani, Lucila Bohme Autor UNIFESP Google Scholar
Oliveira Costa, Henrique de Autor UNIFESP Google Scholar
Fuziy, Rogerio Aoki Autor UNIFESP Google Scholar
Franco, Marcello Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
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 hepatocelluar carcinoma associated with multiple bile duct hamartomas (von Meyenburg complex). Bile duct hamartoma is generally and 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. (C) 2008 Elsevier Inc. All rights reserved.
Keywords bile duct hamartoma
liver cell carcinoma
hepatocellular carcinoma
Language English
Date 2008-06-01
Published in Annals of Diagnostic Pathology. New York: Elsevier B.V., v. 12, n. 3, p. 208-211, 2008.
ISSN 1092-9134 (Sherpa/Romeo, impact factor)
Publisher Elsevier B.V.
Extent 208-211
Origin http://dx.doi.org/10.1016/j.anndiagpath.2006.12.003
Access rights Closed access
Type Article
Web of Science ID WOS:000256390200009
URI http://repositorio.unifesp.br/handle/11600/30694

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