Management of Hepatic Adenoma in Glycogen Storage Disease Ia

1995 ◽  
Vol 20 (2) ◽  
pp. 225-228 ◽  
Author(s):  
Joel R. Rosh ◽  
Janna Collins ◽  
Gabriel M. Groisman ◽  
Audrey H. Schwersenz ◽  
Myron Schwartz ◽  
...  
Keyword(s):  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Glycogen Storage ◽  
Hepatic Adenoma

Hepatic ultrasonography in type I glycogen storage disease (von Gierke disease). Detection of hepatic adenoma and carcinoma.

Radiology ◽  
1981 ◽  
Vol 141 (3) ◽  
pp. 753-756 ◽  
Author(s):  
H Grossman ◽  
P C Ram ◽  
R A Coleman ◽  
G Gates ◽  
E R Rosenberg ◽  
...  
Keyword(s):  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Glycogen Storage ◽  
Disease Detection ◽  
Type I ◽  
Hepatic Adenoma

scholarly journals Malignant Transformation of Hepatic Adenoma in Glycogen Storage Disease Type-1a: Report of an Exceptional Case Diagnosed on Surveillance Imaging

2015 ◽  
Vol 5 ◽  
pp. 47 ◽  
Author(s):  
Akshay D. Baheti ◽  
Matthew M. Yeh ◽  
Ryan O'Malley ◽  
Neeraj Lalwani
Keyword(s):  
Malignant Transformation ◽  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Hepatocellular Adenoma ◽  
Glycogen Storage Disease Type ◽  
Glycogen Storage ◽  
Exceptional Case ◽  
Hepatic Adenoma ◽  
Surveillance Imaging ◽  
Magnetic Resonance Imaging Mri

Hepatocellular adenoma is a heterogeneous group of benign neoplasms arising from hepatocellular cells and can be subclassified into four major groups based on genotypic and phenotypic characteristics. These four subtypes are hepatocyte nuclear factor (HNF) 1α-inactivated, β-catenin–activated, inflammatory, and unclassified adenomas. Immunohistochemistry studies have demonstrated that since β-catenin–activated adenomas have a higher risk of malignant transformation, the identification of the subtype of adenoma remains crucial in patient management. However, malignant transformation of hepatic adenoma without β-catenin overexpression can be seen in 30–65% cases. We report a case of malignant transformation of hepatic adenoma without overexpression of β-catenin in a 31-year-old man with a known glycogen storage disease (GSD) Type-1a, which was diagnosed on surveillance magnetic resonance imaging (MRI). The MRI showed a mild interval increase in one lesion with relative stability of the other adenomas. The lesion was presumed to be suspicious for a hepatocellular carcinoma (HCC) and was confirmed on pathology.

Case Report: Hepatic adenoma with bone marrow metaplasia in a patient with glycogen storage disease type 1a

1996 ◽  
Vol 11 (6) ◽  
pp. 556-559 ◽  
Author(s):  
SHIGEAKI MORIURA ◽  
MAKOTO KURODA ◽  
ATSUSHI KIMURA ◽  
YASUSHI IWATSUKA ◽  
SHUHEI IKEDA ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Case Report ◽  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Glycogen Storage Disease Type ◽  
Glycogen Storage ◽  
Disease Type ◽  
Hepatic Adenoma
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