A Case of Hepatoma with Glycogen Storage Disease Type I

Abstract

A case of hepatoma with glycogen storage disease type I is described. A 40-year-old man was admitted to the hospital because of liver dysfunction. Imaging modalities using ultrasonography, computed tomography, (CT) and angiography revealed a liver tumor in the left lateral segment. Since examination of peripheral blood revealed a decrease in fasting blood sugar, and an increase in α-fetoprotein, and tissue biopsy of the liver showed a decrease in glucose-6-phosphatase, we diagnosed the tumor as hepatoma with glycogen storage disease type I. The patient underwent left lateral segmentectomy of the liver. Seven months later, we detected local recurrence by CT in the abdominal wall and the remnant liver in the right posterior segment. Eleven months after the initial hepatectomy, the patient underwent a repeated hepatectomy, and resection of the abdominal wall tumor, greater omentum, transverse colon and right adrenal gland. Histological findings of all resected specimens were hepatocellular carcinoma. The postoperative couse was uneventful and the patient was discharged one month after the second operation.