Cervical Dumbbell: Ganglioneuromas Causing Myelopathy in a Case of Neurofibromatosis Type-I: Case Report and Literature Review

Background Data: Ganglioneuromas are rare benign tumors arising from the sympathetic nervous system. They occasionally grow in a dumbbell fashion extending into the spinal canal extradurally. However, ganglioneuromas of the cervical spine with intradural extension or multiple locations or in associa...

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Autores principales:	AbdAllah Labib, Walid Attia
Formato:	article
Lenguaje:	EN
Publicado:	Egyptian Spine Association 2017
Materias:	Ganglioneuroma Cervical myelopathy Extra medullary tumors Neurofibromatosis Neurology. Diseases of the nervous system RC346-429
Acceso en línea:	https://doaj.org/article/3972a76af7804bd18daa83a2f61266bc
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spelling	oai:doaj.org-article:3972a76af7804bd18daa83a2f61266bc2021-12-02T04:46:59ZCervical Dumbbell: Ganglioneuromas Causing Myelopathy in a Case of Neurofibromatosis Type-I: Case Report and Literature ReviewDOI:10.21608/ESJ.2017.67582314-89502314-8969https://doaj.org/article/3972a76af7804bd18daa83a2f61266bc2017-07-01T00:00:00Zhttp://www.esj.journals.ekb.eg/article_6758.htmlhttps://doaj.org/toc/2314-8950https://doaj.org/toc/2314-8969Background Data: Ganglioneuromas are rare benign tumors arising from the sympathetic nervous system. They occasionally grow in a dumbbell fashion extending into the spinal canal extradurally. However, ganglioneuromas of the cervical spine with intradural extension or multiple locations or in association with neurofibromatosis type-I are rare. Purpose: Our aim is to describe a man with cervical compressive myelopathy at C2 level caused by dumbbell shaped intradural extramedullary ganglioneuroma and multilevel spinal lesions (ganglioneuromas) associated with neurofibromatosis type-I with genetic predisposition. Study Design: Case report and review of the literature. Patient and Method: Twenty eight year-old man with NF-I presented with acute history of progressive quadriparesis. Magnetic resonance imaging showed bilateral dumbbell masses at the C2 level compressing the spinal cord. A systemic imaging survey of the patient showed numerous asymptomatic foraminal and extraforaminal tumors at all neuroforamina of the spine. Results: Surgical decompression of the spinal cord by resections of bilateral tumors at the C2 level alleviated patient symptoms. Histopathological diagnosis was ganglioneuroma. The patient was heterozygous in the NF-I gene for a sequence variant defined as c.970T>C, and predicted to result in the amino acid substitution p.Cys324Arg. Conclusion: The case reported here is very unusual because of its type, its location as dumbbell spinal ganglioneuroma at level of 2nd cervical spine, being associated with other ganglioneuromas at other spinal levels and being associated with NF-I molecular mutation. (2017ESJ143)AbdAllah Labib Walid AttiaEgyptian Spine AssociationarticleGanglioneuromaCervical myelopathyExtra medullary tumorsNeurofibromatosisNeurology. Diseases of the nervous systemRC346-429ENEgyptian Spine Journal, Vol 23, Iss 1, Pp 43-48 (2017)
institution	DOAJ
collection	DOAJ
language	EN
topic	Ganglioneuroma Cervical myelopathy Extra medullary tumors Neurofibromatosis Neurology. Diseases of the nervous system RC346-429
spellingShingle	Ganglioneuroma Cervical myelopathy Extra medullary tumors Neurofibromatosis Neurology. Diseases of the nervous system RC346-429 AbdAllah Labib Walid Attia Cervical Dumbbell: Ganglioneuromas Causing Myelopathy in a Case of Neurofibromatosis Type-I: Case Report and Literature Review
description	Background Data: Ganglioneuromas are rare benign tumors arising from the sympathetic nervous system. They occasionally grow in a dumbbell fashion extending into the spinal canal extradurally. However, ganglioneuromas of the cervical spine with intradural extension or multiple locations or in association with neurofibromatosis type-I are rare. Purpose: Our aim is to describe a man with cervical compressive myelopathy at C2 level caused by dumbbell shaped intradural extramedullary ganglioneuroma and multilevel spinal lesions (ganglioneuromas) associated with neurofibromatosis type-I with genetic predisposition. Study Design: Case report and review of the literature. Patient and Method: Twenty eight year-old man with NF-I presented with acute history of progressive quadriparesis. Magnetic resonance imaging showed bilateral dumbbell masses at the C2 level compressing the spinal cord. A systemic imaging survey of the patient showed numerous asymptomatic foraminal and extraforaminal tumors at all neuroforamina of the spine. Results: Surgical decompression of the spinal cord by resections of bilateral tumors at the C2 level alleviated patient symptoms. Histopathological diagnosis was ganglioneuroma. The patient was heterozygous in the NF-I gene for a sequence variant defined as c.970T>C, and predicted to result in the amino acid substitution p.Cys324Arg. Conclusion: The case reported here is very unusual because of its type, its location as dumbbell spinal ganglioneuroma at level of 2nd cervical spine, being associated with other ganglioneuromas at other spinal levels and being associated with NF-I molecular mutation. (2017ESJ143)
format	article
author	AbdAllah Labib Walid Attia
author_facet	AbdAllah Labib Walid Attia
author_sort	AbdAllah Labib
title	Cervical Dumbbell: Ganglioneuromas Causing Myelopathy in a Case of Neurofibromatosis Type-I: Case Report and Literature Review
title_short	Cervical Dumbbell: Ganglioneuromas Causing Myelopathy in a Case of Neurofibromatosis Type-I: Case Report and Literature Review
title_full	Cervical Dumbbell: Ganglioneuromas Causing Myelopathy in a Case of Neurofibromatosis Type-I: Case Report and Literature Review
title_fullStr	Cervical Dumbbell: Ganglioneuromas Causing Myelopathy in a Case of Neurofibromatosis Type-I: Case Report and Literature Review
title_full_unstemmed	Cervical Dumbbell: Ganglioneuromas Causing Myelopathy in a Case of Neurofibromatosis Type-I: Case Report and Literature Review
title_sort	cervical dumbbell: ganglioneuromas causing myelopathy in a case of neurofibromatosis type-i: case report and literature review
publisher	Egyptian Spine Association
publishDate	2017
url	https://doaj.org/article/3972a76af7804bd18daa83a2f61266bc
work_keys_str_mv	AT abdallahlabib cervicaldumbbellganglioneuromascausingmyelopathyinacaseofneurofibromatosistypeicasereportandliteraturereview AT walidattia cervicaldumbbellganglioneuromascausingmyelopathyinacaseofneurofibromatosistypeicasereportandliteraturereview
_version_	1718400999280869376

Cervical Dumbbell: Ganglioneuromas Causing Myelopathy in a Case of Neurofibromatosis Type-I: Case Report and Literature Review

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