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©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
"Bull’s eye” appearance of hepatocellular adenomas in patients with glycogen storage disease type I — atypical magnetic resonance imaging findings: Two case reports
Federica Vernuccio, Stephanie Austin, Mathias Meyer, Cynthia D Guy, Priya S Kishnani, Daniele Marin
Federica Vernuccio, Department of Biomedicine, Neuroscience and Advanced Diagnostics, University of Palermo, Palermo 90100, Italy
Stephanie Austin, Departments of Pediatrics, Duke University Medical Center, Durham, NC 27710, United States
Mathias Meyer, Department of Radiology, Duke University Medical Hospital, Durham, NC 27708, United States
Cynthia D Guy, Department of Pathology, Duke University, Durham, NC 27710, United States
Priya S Kishnani, Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, United States
Daniele Marin, Department of Radiology, Duke University Medical Center, Durham, NC 27710, United States
Author contributions: Vernuccio F, Marin D and Kishnani PS conceived and designed the study; Vernuccio F wrote the draft of the paper; Austin S, Guy CD and Kishnani PS collected and analyzed clinical and pathological data; Meyer M and Marin D edited the draft of the paper; all authors have read and approved the final version of the manuscript.
Informed consent statement: Clinical research on patients with glycogen storage diseases has been approved by the Institutional Review Board. Informed consent statement was waived.
Conflict-of-interest statement: The authors declare no conflict of interest related to this study.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
http://creativecommons.org/Licenses/by-nc/4.0/ Corresponding author: Federica Vernuccio, MD, Academic Fellow, Department of Biomedicine, Neuroscience and Advanced Diagnostics, University of Palermo, Via del Vespro 129, Palermo 90100, Italy. federica.vernuccio@unipa.it
Received: September 20, 2020
Peer-review started: September 20, 2020
First decision: November 25, 2020
Revised: December 10, 2020
Accepted: December 28, 2020
Article in press: December 28, 2020
Published online: February 6, 2021
Processing time: 120 Days and 1.3 Hours
BACKGROUND
Hepatocellular adenomas are rare tumors that can occur in patients with glycogen storage disease type I.
CASE SUMMARY
We herein report two cases of histologically proven hepatocellular adenomas in patients with glycogen storage disease type I. Magnetic resonance imaging (MRI) was performed after bolus injection of gadoxetate disodium, a liver-specific gadolinium-based MRI contrast agent. In the present cases, some of the hepatocellular adenomas showed unexpectedly a “bull’s eye” appearance on T2-weighted and post-contrast images, which was not previously described as imaging findings of hepatocellular adenomas in glycogen storage disease. A bull’s eye appearance on T2-weighted images can be encountered in both benign (i.e., abscess) or malignant (i.e., epithelioid hemangioendothelioma, cholangio-carcinoma, and metastases) hepatic lesions.
CONCLUSION
We present two cases of hepatocellular adenomas in patients with glycogen storage disease type 1, in which gadoxetate disodium-MRI showed atypical imaging findings for hepatocellular adenomas. At present there is no systematic study evaluating MRI findings of hepatocellular adenomas in patients with glycogen storage disease, further studies are needed to specifically investigate this issue.
Core Tip: Magnetic resonance imaging findings of hepatocellular adenomas in glycogen storage disease may differ from the typical ones observed in the common population.
