Article ID Journal Published Year Pages File Type
4130663 Annals of Diagnostic Pathology 2008 4 Pages PDF
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.

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