Hepatic Adenomatosis in Glycogen Storage Disease Type Ia: Report of a Case With Unusual Histology

摘要: Hepatic adenomatosis is a well-known complication of glycogen storage disease type Ia (von Gierke disease). Although most these tumors have an appearance similar to sporadic hepatocellular adenomas, unusual histologic features been reported, including Mallory hyaline, varying degrees fibrosis, and aggregates neutrophils. We report the fourth case hyaline in adenomas 28-year-old woman undergoing segmental hepatectomy for enlarging liver nodules. Other prominent findings included steatohepatitis nonspecific granulomatous inflammation--2 that are commonly seen but not, our knowledge, previously reported Ia.