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Multiple meningiomas arising within the same hemisphere associated with Li-Fraumeni syndrome

BACKGROUND: While meningiomas are some of the most common intracranial tumors, the presence of multiple ones at the time of presentation is rare and can most commonly be observed in patients with well-described syndromes (i.e., neurofibromatosis type 2) or those with prior cranial radiation history....

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Autores principales: Hong, Christopher S., Erson-Omay, E. Zeynep, Moliterno, Jennifer
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Scientific Scholar 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8053471/
https://www.ncbi.nlm.nih.gov/pubmed/33880204
http://dx.doi.org/10.25259/SNI_125_2019
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author Hong, Christopher S.
Erson-Omay, E. Zeynep
Moliterno, Jennifer
author_facet Hong, Christopher S.
Erson-Omay, E. Zeynep
Moliterno, Jennifer
author_sort Hong, Christopher S.
collection PubMed
description BACKGROUND: While meningiomas are some of the most common intracranial tumors, the presence of multiple ones at the time of presentation is rare and can most commonly be observed in patients with well-described syndromes (i.e., neurofibromatosis type 2) or those with prior cranial radiation history. In others, however, the pathophysiology remains unclear. CASE DESCRIPTION: A 49-year-old female with no significant personal or familial oncologic medical history presented with a generalized seizure and was found to have ten meningiomas arising within the right hemisphere. She underwent a two-staged resection of all tumors, with pathology revealing the World Health Organization Grade I meningioma. Whole-exome sequencing revealed somatic NF2 mutations and heterozygous deletion of chromosome 22 overlapping with NF2, and analysis of the germline uncovered mutations of TP53, rendering a diagnosis of Li-Fraumeni Syndrome. CONCLUSIONS: This case represents a novel presentation of multiple meningiomas in a patient with newly diagnosed Li-Fraumeni syndrome, suggesting meningioma may be considered as part of this tumor-predisposed patient population.
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spelling pubmed-80534712021-04-19 Multiple meningiomas arising within the same hemisphere associated with Li-Fraumeni syndrome Hong, Christopher S. Erson-Omay, E. Zeynep Moliterno, Jennifer Surg Neurol Int Case Report BACKGROUND: While meningiomas are some of the most common intracranial tumors, the presence of multiple ones at the time of presentation is rare and can most commonly be observed in patients with well-described syndromes (i.e., neurofibromatosis type 2) or those with prior cranial radiation history. In others, however, the pathophysiology remains unclear. CASE DESCRIPTION: A 49-year-old female with no significant personal or familial oncologic medical history presented with a generalized seizure and was found to have ten meningiomas arising within the right hemisphere. She underwent a two-staged resection of all tumors, with pathology revealing the World Health Organization Grade I meningioma. Whole-exome sequencing revealed somatic NF2 mutations and heterozygous deletion of chromosome 22 overlapping with NF2, and analysis of the germline uncovered mutations of TP53, rendering a diagnosis of Li-Fraumeni Syndrome. CONCLUSIONS: This case represents a novel presentation of multiple meningiomas in a patient with newly diagnosed Li-Fraumeni syndrome, suggesting meningioma may be considered as part of this tumor-predisposed patient population. Scientific Scholar 2021-03-17 /pmc/articles/PMC8053471/ /pubmed/33880204 http://dx.doi.org/10.25259/SNI_125_2019 Text en Copyright: © 2021 Surgical Neurology International https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 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
Hong, Christopher S.
Erson-Omay, E. Zeynep
Moliterno, Jennifer
Multiple meningiomas arising within the same hemisphere associated with Li-Fraumeni syndrome
title Multiple meningiomas arising within the same hemisphere associated with Li-Fraumeni syndrome
title_full Multiple meningiomas arising within the same hemisphere associated with Li-Fraumeni syndrome
title_fullStr Multiple meningiomas arising within the same hemisphere associated with Li-Fraumeni syndrome
title_full_unstemmed Multiple meningiomas arising within the same hemisphere associated with Li-Fraumeni syndrome
title_short Multiple meningiomas arising within the same hemisphere associated with Li-Fraumeni syndrome
title_sort multiple meningiomas arising within the same hemisphere associated with li-fraumeni syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8053471/
https://www.ncbi.nlm.nih.gov/pubmed/33880204
http://dx.doi.org/10.25259/SNI_125_2019
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