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

F. Limaiem, F. Gargouri, I. Haddad, S. Bouraoui, A. Lahmar, S. Mzabi-Regaya
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Abstract

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 von-Meyenburg'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.
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罕见的多发性肝脏病变:病例报告及文献复习
胆道错构瘤或von Meyenburg复合体是罕见的良性胆道畸形,被认为是由导管板畸形引起的肝脏纤维囊性疾病的一部分。肝内胆管癌的发展在这些复合物中得到了很好的描述。然而,迄今为止,英语文献中仅报道了10例与von Meyenburg复合物相关的肝细胞癌。本文报告一例以von-Meyenburg氏复合体和单眼胆管囊肿为背景的肝细胞癌。目前尚不清楚肝细胞癌的发展是一种与前体病变无关的附带现象,还是胆道错构瘤可能发展为肝癌。进一步的研究是必要的,以阐明和巩固这种非常罕见的联系。
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