A Rare Association of Multiple Hepatic Lesions: Case Report and Literature Review

Abstract

Faten Limaiem, Faten Gargouri, Inès Haddad, Saâdia Bouraoui, Ahlem Lahmar, Sabeh Mzabi-Regaya

Biliary hamartomas or von Meyenburg complexes are uncommon benign biliary malformations, which are considered as part of the spectrum of fibrocystic diseases of the liver due to ductal plate malformation. Development of intrahepatic cholangiocarcinoma is well described in these complexes. However, only 10 cases of hepatocellular carcinoma arising in association with von Meyenburg complexes have been described in the English-language literature to date. In this paper, the authors report a peculiar case of hepatocellular carcinoma occurring on a background of vonMeyenburg's complexes and solitary unilocular bile duct cyst. It is not clear whether development of hepatocellular carcinomas is an epiphenomenon unrelated to the precursor lesion or biliary hamartomas may progress to liver cancers. Further studies are mandatory so as to elucidate and consolidate this very rare association.