J Korean Med Sci.  2020 Jan;35(1):e5. 10.3346/jkms.2020.35.e5.

Development of Hepatocellular Carcinoma in Patients with Glycogen Storage Disease: a Single Center Retrospective Study

• Jang HJ
• Yang HR
• Ko JS
• Moon JS
• Chang JY
• Seo JK

• KMID: 2466582
• DOI: http://doi.org/10.3346/jkms.2020.35.e5

Abstract

BACKGROUND
Glycogen storage disease (GSD) is an inherited disorder leading to abnormal glucose metabolism and glycogen accumulation, and is associated with various complications including hepatic adenoma and hepatocellular carcinoma. The aim of this study was to analyze the risk factors for hepatic adenoma and its malignant change, and the hepatocellular carcinoma-free survival rate in patients with GSD who developed adenoma.
METHODS
A total of 72 patients with GSD who were enrolled from March 1982 to September 2013 at Seoul National University Children's Hospital were retrospectively analyzed, and the median follow-up period was 19.2 years.
RESULTS
Thirty-two patients (44.4%) developed hepatic adenoma at an age range of 7.9-26.3 years (median, 14.3 years). Among the 32 patients with hepatic adenoma, 4 patients (12.5%) developed hepatocellular carcinoma on an average interval of 6.7 years between the diagnosis of adenoma and the development of hepatocellular carcinoma. GSD type I and portacaval shunt operation were found to be the risk factors for hepatic adenoma development. The hepatocellular carcinoma-free survival rate at 10 years from adenoma development was 82%.
CONCLUSION
The present study found that portacaval shunt operation increases the risk of development of hepatic adenoma in GSD patients, especially in GSD type I. The hepatic adenoma in GSD patients has a potential of malignant transformation, which should be keep in mind in follow-up process of the disease.

Keyword

Glycogen Storage Disease; Adenoma; Hepatocellular Carcinoma

MeSH Terms

Adenoma
Carcinoma, Hepatocellular*
Diagnosis
Follow-Up Studies
Glucose
Glycogen Storage Disease*
Glycogen*
Humans
Metabolism
Portacaval Shunt, Surgical
Retrospective Studies*
Risk Factors
Seoul
Survival Rate
Glucose
Glycogen