A Case of Hepatectomized Hepatocellular Adenoma Associated with Glycogen Storage Disease Type Ia

Abstract

A 17-year-old man, who was diagnosed with glycogen storage disease type Ia at the age of two and then had been treated in the Department of Pediatrics in our hospital, was pointed out to have an 8-cm diameter tumor in the liver S6 and multiple nodular lesions in both lobes on CT at the age of 17. A diagnosis of hepatocellular adenoma associated with glycogen storage disease type Ia was most likely. We determined to indicate resection for the S6 lesion in order to avoid risks such as malignant change and rupture. Upon laparotomy, the liver had significantly swollen to show a pattern of fatty liver, and a fist-sized elastic-soft tumor which protruded into inferior surface of the liver was found. Partial hepatic resection was performed. Although he needed two-day postoperative ICU management for strict blood sugar control to treat lactic acidosis or hypoglycemic attack due to glycogen storage disease type Ia, otherwise his postoperative course was uneventful. Histopathological studies revealed no malignant findings that were compatible with hepatocellular adenoma. As of one year and 6 months after the operation, we confirm that the remnant hepatocellular adenoma is enlarging, so that we are proposing him to undergo living donor liver transplant. Treating hepatocellular adenoma associated with glycogen storage disease type Ia, we must select suitable method case by case, by keeping a possibility of liver transplantation in mind and considering a risk of malignant transformation or rupture.