Adenomas in glycogen storage disease type 1. Two cases with unusual histologic features

Am J Surg Pathol. 1988 Jun;12(6):477-83. doi: 10.1097/00000478-198806000-00008.


Two cases of hepatic adenomas arising in patients with glycogen storage disease type I are presented. In both cases, the adenomas showed unusual histological features including marked fatty change, a polymorphonuclear infiltrate, Mallory hyalin, and lamellar fibrosis. In addition, one case was associated with amyloidosis. Such features have only recently been described in adenomas and have not been reported previously in association with glycogen storage disease type I.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Amyloidosis / pathology
  • Carcinoma, Hepatocellular / complications
  • Carcinoma, Hepatocellular / metabolism
  • Carcinoma, Hepatocellular / pathology*
  • Glycogen Storage Disease Type I / complications*
  • Humans
  • Hyalin / analysis
  • Immunoenzyme Techniques
  • Inclusion Bodies / ultrastructure
  • Liver / pathology*
  • Liver Neoplasms / complications
  • Liver Neoplasms / metabolism
  • Liver Neoplasms / pathology*
  • Male
  • Microscopy, Electron