HEPATOCELLULAR ADENOMAS IN GLYCOGEN-STORAGE-DISEASE TYPE-I AND TYPE-III - A SERIES OF 43 PATIENTS AND REVIEW OF THE LITERATURE

Background: Hepatocellular adenomas may develop in patients with glyco gen storage disease types I and III, and the malignant degeneration of adenomas in hepatocellular carcinoma has been reported in ten cases, The aim of this work was to study the characteristics of hepatic adeno mas in a lar ge series of 43 patients with glycogen storage disease ty pes I and III and to determine the optimal means of follow-up, Methods : The charts of 43 patients with glycogen storage disease type I and I II were studied, In all these patients, abdominal ultrasonography and the determination of serum alpha-fetoprotein had been performed yearly and serum concentrations of several proteins were determined once. Re sults: 51.8% of patients with type I and 25% of patients with type III glycogen storage disease had hepatic adenomas at the time of the stud y, The male to Female ratio was 2 to 1 in type I, and no female had ad enomas in type III. No evidence of malignant transformation was observ ed during file follow-up period, Serum concentrations of several prote ins were significantly higher in patients with hepatic adenomas than i n patients without such lesions, Conclusions: In patients with glycoge n storage disease type I and III, the determination of alpha-fetoprote in serum concentration has to be combined with yearly hepatic ultrasou nd examinations. other investigations such as CT scan should be consid ered when the size of any adenoma increases. The malignant transformat ion of hepatocellular adenoma into hepatocellular carcinoma remains a rare event. (C) 1991 Lippincott-Raven Publishers.