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

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, inflam...

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Autores principales: Baheti, Akshay D., Yeh, Matthew M., O'Malley, Ryan, Lalwani, Neeraj
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4584445/
https://www.ncbi.nlm.nih.gov/pubmed/26430540
http://dx.doi.org/10.4103/2156-7514.163991
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author Baheti, Akshay D.
Yeh, Matthew M.
O'Malley, Ryan
Lalwani, Neeraj
author_facet Baheti, Akshay D.
Yeh, Matthew M.
O'Malley, Ryan
Lalwani, Neeraj
author_sort Baheti, Akshay D.
collection PubMed
description 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.
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spelling pubmed-45844452015-10-01 Malignant Transformation of Hepatic Adenoma in Glycogen Storage Disease Type-1a: Report of an Exceptional Case Diagnosed on Surveillance Imaging Baheti, Akshay D. Yeh, Matthew M. O'Malley, Ryan Lalwani, Neeraj J Clin Imaging Sci Case Report 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. Medknow Publications & Media Pvt Ltd 2015-08-31 /pmc/articles/PMC4584445/ /pubmed/26430540 http://dx.doi.org/10.4103/2156-7514.163991 Text en Copyright: © 2015 Journal of Clinical Imaging Science http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle Case Report
Baheti, Akshay D.
Yeh, Matthew M.
O'Malley, Ryan
Lalwani, Neeraj
Malignant Transformation of Hepatic Adenoma in Glycogen Storage Disease Type-1a: Report of an Exceptional Case Diagnosed on Surveillance Imaging
title Malignant Transformation of Hepatic Adenoma in Glycogen Storage Disease Type-1a: Report of an Exceptional Case Diagnosed on Surveillance Imaging
title_full Malignant Transformation of Hepatic Adenoma in Glycogen Storage Disease Type-1a: Report of an Exceptional Case Diagnosed on Surveillance Imaging
title_fullStr Malignant Transformation of Hepatic Adenoma in Glycogen Storage Disease Type-1a: Report of an Exceptional Case Diagnosed on Surveillance Imaging
title_full_unstemmed Malignant Transformation of Hepatic Adenoma in Glycogen Storage Disease Type-1a: Report of an Exceptional Case Diagnosed on Surveillance Imaging
title_short Malignant Transformation of Hepatic Adenoma in Glycogen Storage Disease Type-1a: Report of an Exceptional Case Diagnosed on Surveillance Imaging
title_sort malignant transformation of hepatic adenoma in glycogen storage disease type-1a: report of an exceptional case diagnosed on surveillance imaging
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4584445/
https://www.ncbi.nlm.nih.gov/pubmed/26430540
http://dx.doi.org/10.4103/2156-7514.163991
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