Hepatic adenoma and hepatocellular carcinoma in 3 brothers with type I glycogenosis

We report 2 cases of type I glycogen storage disease (Von Gierke's disease) discovered in 2 brothers at the age of 7 and 5 years, respectively. Both developed hepatic adenoma at the age of 19 and 17. Hepatocellular carcinoma occurred in the older brother the discovery of adenoma 4 years after....

Full description

Saved in:

Bibliographic Details
Published in:	Gastroentérologie clinique et biologique Vol. 14; no. 1; p. 84
Main Authors:	Kharsa, G, Degott, C, Filoche, B, Hedde, J P, Potet, F, Benhamou, J P
Format:	Journal Article
Language:	French
Published:	France 1990
Subjects:	Adenoma - diagnosis Adenoma - etiology Adenoma - pathology Adolescent Adult Biopsy, Needle Carcinoma, Hepatocellular - diagnosis Carcinoma, Hepatocellular - etiology Carcinoma, Hepatocellular - pathology Glycogen Storage Disease Type I - complications Glycogen Storage Disease Type I - genetics Humans Liver - pathology Liver Neoplasms - diagnosis Liver Neoplasms - etiology Liver Neoplasms - pathology Male Ultrasonography
Online Access:	Get more information
Tags:	Add Tag No Tags, Be the first to tag this record!

Description
Summary:	We report 2 cases of type I glycogen storage disease (Von Gierke's disease) discovered in 2 brothers at the age of 7 and 5 years, respectively. Both developed hepatic adenoma at the age of 19 and 17. Hepatocellular carcinoma occurred in the older brother the discovery of adenoma 4 years after. The frequency of these tumors in patients with type I glycogen storage disease raises problems concerning the treatment and modality of regular surveillance of the liver in these patients. The policy for the detection and treatment of these tumors, and particularly the indications for liver transplantation are discussed.
ISSN:	0399-8320