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Multiple Meningiomas in a Patient with Cowden Syndrome

BACKGROUND:  Cowden syndrome is a rare, multisystem disease manifesting with increased hamartomas and neoplasms. Though meningioma has been documented in patients with Cowden syndrome, the relationship between these two phenomena is still unclear. CASE DESCRIPTION:  We report a case of a 43-year-old...

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Autores principales: Pain, Margaret, Darbinyan, Armine, Fowkes, Mary, Shrivastava, Raj
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Georg Thieme Verlag KG 2016
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4996671/
https://www.ncbi.nlm.nih.gov/pubmed/27563534
http://dx.doi.org/10.1055/s-0036-1584265
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author Pain, Margaret
Darbinyan, Armine
Fowkes, Mary
Shrivastava, Raj
author_facet Pain, Margaret
Darbinyan, Armine
Fowkes, Mary
Shrivastava, Raj
author_sort Pain, Margaret
collection PubMed
description BACKGROUND:  Cowden syndrome is a rare, multisystem disease manifesting with increased hamartomas and neoplasms. Though meningioma has been documented in patients with Cowden syndrome, the relationship between these two phenomena is still unclear. CASE DESCRIPTION:  We report a case of a 43-year-old female patient with a known PTEN mutation and clinical history of Cowden syndrome. A workup of headache demonstrated two skull base meningiomas. At the time of surgery, several additional tiny meningiomas were detected in the same region. CONCLUSIONS:  The development of multiple meningiomas in a patient with predisposition for tumor is more than coincidental. Though PTEN mutations and deletions have not been shown to be critical for meningioma development, this case challenges that conclusion. In light of recent genetic advances in meningioma molecular pathogenesis, the role of the PTEN/AKT/PI3K pathway is discussed.
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spelling pubmed-49966712016-08-25 Multiple Meningiomas in a Patient with Cowden Syndrome Pain, Margaret Darbinyan, Armine Fowkes, Mary Shrivastava, Raj J Neurol Surg Rep BACKGROUND:  Cowden syndrome is a rare, multisystem disease manifesting with increased hamartomas and neoplasms. Though meningioma has been documented in patients with Cowden syndrome, the relationship between these two phenomena is still unclear. CASE DESCRIPTION:  We report a case of a 43-year-old female patient with a known PTEN mutation and clinical history of Cowden syndrome. A workup of headache demonstrated two skull base meningiomas. At the time of surgery, several additional tiny meningiomas were detected in the same region. CONCLUSIONS:  The development of multiple meningiomas in a patient with predisposition for tumor is more than coincidental. Though PTEN mutations and deletions have not been shown to be critical for meningioma development, this case challenges that conclusion. In light of recent genetic advances in meningioma molecular pathogenesis, the role of the PTEN/AKT/PI3K pathway is discussed. Georg Thieme Verlag KG 2016-07 /pmc/articles/PMC4996671/ /pubmed/27563534 http://dx.doi.org/10.1055/s-0036-1584265 Text en © Thieme Medical Publishers
spellingShingle Pain, Margaret
Darbinyan, Armine
Fowkes, Mary
Shrivastava, Raj
Multiple Meningiomas in a Patient with Cowden Syndrome
title Multiple Meningiomas in a Patient with Cowden Syndrome
title_full Multiple Meningiomas in a Patient with Cowden Syndrome
title_fullStr Multiple Meningiomas in a Patient with Cowden Syndrome
title_full_unstemmed Multiple Meningiomas in a Patient with Cowden Syndrome
title_short Multiple Meningiomas in a Patient with Cowden Syndrome
title_sort multiple meningiomas in a patient with cowden syndrome
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4996671/
https://www.ncbi.nlm.nih.gov/pubmed/27563534
http://dx.doi.org/10.1055/s-0036-1584265
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