Hepatic adenomata with type 1 glycogen storage disease
R. R. Howell, R. E. Stevenson, Y. Ben-Menachem, R. L. Phyliky and D. H. Berry
In older patients with glucose-6-phosphatase deficiency adenomatous nodules
develop within the liver parenchyma. Investigation of eight such patients,
age 3 to 28 years, using radioisotopic scans, has demonstrated areas of
depressed isotope uptake in the liver in all except the one preteenaged
child. Three patients were further studied with hepatic angiograms and
liver biopsy specimens. A diffuse nodularity, more widespread than apparent
on isotopic scans, was demonstrated on angiograms. Although initial
histologic study in each case showed adenomatous tissue without evidence of
neoplasm, the development of hepatocellular carcinoma in one of our
patients and in others from the literature suggests that the nodules may be
premalignant.
