HEPATOCELLULAR ADENOMA IN GLYCOGEN-STORAGE-DISEASE TYPE-IV

The development of,hepatocellular adenoma has been recognized in assoc iation with glycogen storage disease type I and, less often, with glyc ogen storage disease type III, but, to our knowledge, it has not been reported in glycogen storage disease type IV. We had the opportunity t o study an 11-month-old male infant who underwent orthotopic liver tra nsplantation for cirrhosis that developed in the setting of glycogen s torage disease type IV. A clinically unsuspected hepatocellular adenom a was present in the explanted liver. Glycogen storage disease type IV should be considered as a potential precursor to the development of h epatocellular adenoma. Recognition of this association is important, b oth in terms of the differential diagnosis of tumors that occur in thi s setting and also to anticipate potential complications of this benig n neoplasm.