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Hepatocellular carcinoma and focal nodular hyperplasia of the liver in a glycogen storage disease patient

World Journal of Hepatology 2012年第6期4卷 191-195页

作者： yoshihiro mikuriya Akihiko Oshita Hirotaka Tashiro Hironobu Amano Tsuyoshi Kobayashi Kouji Arihiro Hideki Ohdan Division of Frontier Medical Science Department of Surgery Graduate School of Biomedical Sciences Hiroshima University 1-2-3 Kasumi Minami-ku Hiroshima 734-8551 Japan Department of Pathology Hiroshima University Hospital1-2-3 Kasumi Minami-ku Hiroshima 734-8551 Japan

Glycogen storage disease type Ia (GSD-Ia; also called von Gierke disease) is an autosomal recessive disorder of carbohydrate metabolism caused by glucose-6-phosphatase deficiency. There have been many reports describing hepatic tumors in GSD patients; however, most of these reports were of hepatocellular adenomas, whereas there are only few reports describing focal nodular hyperplasia (FNH) or hepatocellular carcinoma (HCC). We report a case with GSD-Ia who had undergone a partial resection of the liver for FNH at 18 years of age and in whom moderately differentiated HCC had developed. Preoperative imaging studies, including ultrasonography, dynamic computer tomography (CT) and magnetic resonance imaging, revealed benign and malignant features. In particular, fluorodeoxyglucose-positron emission tomography (FDG-PET)/CT revealed the atypical findings that FDG accumulated at high levels in the non-tumorous hepatic parenchyma and low levels in the tumor. Right hemihepatectomy was performed. During the perioperative period, high-dose glucose and sodium bicarbonate were administered to control metabolic acidosis. He had multiple recurrences of HCC at 10 mo after surgery and was followed-up with transcatheter arterial chemoembolization. The tumor was already highly advanced when it was found by chance; therefore, a careful follow-up should be mandatory for GSD-I patients as they are at a high risk for HCC, similar to hepatitis patients.

关键词： Glycogen storage disease type Ia Hepato-cellular carcinoma Focal nodular hyperplasia Hepatectomy Metachronous

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