Korean J Gastroenterol.  2001 Sep;38(3):215-219.

A Case of Hepatocellular Carcinoma Developed in Type 1Glycogen Storage Disease

• Kim TH
• Kim KA
• Lim YS
• Kang GH
• Lee HS
• Kim CY

Abstract

Glycogen storage disease (GSD) is one of rare metabolic causes of hepatocellular carcinoma (HCC). We have experienced a case of HCC in a patient with GSD, who was treated by portosystemic shunt with subsequent symptomatic improvement. A 27-year-old man was admitted because of liver tumor on CT scan. Eighteen years ago, he had been admitted to pediatric hospital for the evaluation of short stature, hepatomegaly and frequent epistaxis, and diagnosed as GSD type 1. One year ago, development of multiple hepatic adenomas were confirmed by ultrasound and histologic evaluation. An abdominal CT scan taken before admission revealed that one of hepatic nodules showed augumented enhancing pattern. Thus, he was admitted for the evaluation of this nodule under the impression of malignant transformation. The tumor was identified as well differentiated HCC by liver biopsy. Hepatic angiography showed hypervascular mass, which was treated by transcatheter arterial chemoembolization with compact lipiodol retention.

Keyword

Glycogen storage disease; Hepatic adenoma; Hepatocellular carcinoma

MeSH Terms

Adenoma
Adult
Angiography
Biopsy
Carcinoma, Hepatocellular*
Epistaxis
Ethiodized Oil
Glycogen Storage Disease
Hepatomegaly
Hospitals, Pediatric
Humans
Liver
Portasystemic Shunt, Surgical
Tomography, X-Ray Computed
Ultrasonography
Ethiodized Oil