Diffuse Meningiomatosis without Neurofibromatosis: A Rare Diagnosis with Atypical Presentation

Meningiomas are amongst the most common neoplasms of the central nervous system; however, “multiple meningiomas” or “meningiomatosis” account for < 10% of cases. The association with neurofibromatosis 2 is seen in ∼50% of cases. We report a case of 35-year-old female patient who presented with le...

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Autores principales:	Smily Sharma, Pankaj Sharma, Amit Kumar
Formato:	article
Lenguaje:	EN
Publicado:	Thieme Medical and Scientific Publishers Pvt. Ltd. 2021
Materias:	en plaque meningioma meningiomatosis neurofibromatosis 2 Medical physics. Medical radiology. Nuclear medicine R895-920
Acceso en línea:	https://doaj.org/article/672b8da6f0674792a2ec937e7dd72d88
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spelling	oai:doaj.org-article:672b8da6f0674792a2ec937e7dd72d882021-11-13T23:32:31ZDiffuse Meningiomatosis without Neurofibromatosis: A Rare Diagnosis with Atypical Presentation0971-30261998-380810.1055/s-0041-1736394https://doaj.org/article/672b8da6f0674792a2ec937e7dd72d882021-07-01T00:00:00Zhttp://www.thieme-connect.de/DOI/DOI?10.1055/s-0041-1736394https://doaj.org/toc/0971-3026https://doaj.org/toc/1998-3808Meningiomas are amongst the most common neoplasms of the central nervous system; however, “multiple meningiomas” or “meningiomatosis” account for < 10% of cases. The association with neurofibromatosis 2 is seen in ∼50% of cases. We report a case of 35-year-old female patient who presented with left eye proptosis, left forehead swelling, and multiple cranial nerve palsies predominantly on the left side. Imaging evaluation revealed innumerable en plaque meningiomas forming a sheet-like nodular thickening along the dura, causing marked hyperostosis and extending into the orbits, cavernous sinuses, sellar–suprasellar regions, various skull foramina, basal cisterns and into the cervical spinal canal causing mass effect on vital structures of the brain and cervical spinal cord. Similar lesions were found scattered in rest of the spine. We intend to highlight the role of imaging in accurately establishing the diagnosis and evaluating the extent and burden of disease in such rare cases.Smily SharmaPankaj SharmaAmit KumarThieme Medical and Scientific Publishers Pvt. Ltd.articleen plaque meningiomameningiomatosisneurofibromatosis 2Medical physics. Medical radiology. Nuclear medicineR895-920ENIndian Journal of Radiology and Imaging, Vol 31, Iss 03, Pp 768-771 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	en plaque meningioma meningiomatosis neurofibromatosis 2 Medical physics. Medical radiology. Nuclear medicine R895-920
spellingShingle	en plaque meningioma meningiomatosis neurofibromatosis 2 Medical physics. Medical radiology. Nuclear medicine R895-920 Smily Sharma Pankaj Sharma Amit Kumar Diffuse Meningiomatosis without Neurofibromatosis: A Rare Diagnosis with Atypical Presentation
description	Meningiomas are amongst the most common neoplasms of the central nervous system; however, “multiple meningiomas” or “meningiomatosis” account for < 10% of cases. The association with neurofibromatosis 2 is seen in ∼50% of cases. We report a case of 35-year-old female patient who presented with left eye proptosis, left forehead swelling, and multiple cranial nerve palsies predominantly on the left side. Imaging evaluation revealed innumerable en plaque meningiomas forming a sheet-like nodular thickening along the dura, causing marked hyperostosis and extending into the orbits, cavernous sinuses, sellar–suprasellar regions, various skull foramina, basal cisterns and into the cervical spinal canal causing mass effect on vital structures of the brain and cervical spinal cord. Similar lesions were found scattered in rest of the spine. We intend to highlight the role of imaging in accurately establishing the diagnosis and evaluating the extent and burden of disease in such rare cases.
format	article
author	Smily Sharma Pankaj Sharma Amit Kumar
author_facet	Smily Sharma Pankaj Sharma Amit Kumar
author_sort	Smily Sharma
title	Diffuse Meningiomatosis without Neurofibromatosis: A Rare Diagnosis with Atypical Presentation
title_short	Diffuse Meningiomatosis without Neurofibromatosis: A Rare Diagnosis with Atypical Presentation
title_full	Diffuse Meningiomatosis without Neurofibromatosis: A Rare Diagnosis with Atypical Presentation
title_fullStr	Diffuse Meningiomatosis without Neurofibromatosis: A Rare Diagnosis with Atypical Presentation
title_full_unstemmed	Diffuse Meningiomatosis without Neurofibromatosis: A Rare Diagnosis with Atypical Presentation
title_sort	diffuse meningiomatosis without neurofibromatosis: a rare diagnosis with atypical presentation
publisher	Thieme Medical and Scientific Publishers Pvt. Ltd.
publishDate	2021
url	https://doaj.org/article/672b8da6f0674792a2ec937e7dd72d88
work_keys_str_mv	AT smilysharma diffusemeningiomatosiswithoutneurofibromatosisararediagnosiswithatypicalpresentation AT pankajsharma diffusemeningiomatosiswithoutneurofibromatosisararediagnosiswithatypicalpresentation AT amitkumar diffusemeningiomatosiswithoutneurofibromatosisararediagnosiswithatypicalpresentation
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Diffuse Meningiomatosis without Neurofibromatosis: A Rare Diagnosis with Atypical Presentation

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