Multiple hepatocellular adenomas in a patient with glycogen storage disease type I: various enhancement patterns in MRI with Gd-EOB-DTPA

The patient is a 20-year-old man with glycogen storage disease type I (GSD-type I). In his teens, multiple focal hepatic masses were detected on abdominal ultrasonography (US), which were diagnosed as multiple hepatocellular adenomas from the imaging. During follow-up, these masses had shown intermi...

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Bibliographic Details
Published in:Abdominal imaging 2012-04, Vol.37 (2), p.239-243
Main Authors: Sakamoto, Ayami, Hayashi, Hideyuki, Sakamoto, Ichiro, Isomoto, Ichiro, Eguchi, Susumu, Takatsuki, Mitsuhisa, Ichikawa, Tatsuki, Abe, Kuniko, Hayashi, Tomayoshi, Uetani, Masataka
Format: Article
Language:English
Subjects:
Adenoma
Adenoma, Liver Cell - etiology
Adenoma, Liver Cell - pathology
Adenoma, Liver Cell - surgery
Benign
Biological and medical sciences
Carbohydrates (enzymatic deficiencies). Glycogenosis
Contrast Media
Errors of metabolism
Gadolinium DTPA
Gastroenterology
Gastroenterology. Liver. Pancreas. Abdomen
Glycogen Storage Disease Type I - complications
Glycogenosis
Hepatectomy - methods
Hepatology
Humans
Imaging
Liver Neoplasms - etiology
Liver Neoplasms - pathology
Liver Neoplasms - surgery
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Magnetic resonance imaging
Magnetic Resonance Imaging - methods
Male
Medical sciences
Medicine
Medicine & Public Health
Metabolic diseases
Radiology
Transformation
Tumors
Ultrasonography
Young Adult
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Description
Summary:The patient is a 20-year-old man with glycogen storage disease type I (GSD-type I). In his teens, multiple focal hepatic masses were detected on abdominal ultrasonography (US), which were diagnosed as multiple hepatocellular adenomas from the imaging. During follow-up, these masses had shown intermittent growth in size. In the evaluation of Gd-EOB-DTPA (gadolinium-ethoxybenzyl-diethylenetriamine penta-acetic acid) MR imaging, these masses showed various signal intensities from hypo- to hyperintense during the hepatocyte-specific phase. Intermittent growth and elevation of serum PIVKA-II levels indicate the potential for malignant transformation, so the patient underwent partial hepatectomy. The resected masses were all consistent with benign hepatocellular adenomas histopathologically.
ISSN:0942-8925
2366-004X
1432-0509
2366-0058
DOI:10.1007/s00261-011-9721-1