Primary Intracranial Malignant Nerve Sheath Tumor in the Cerebellopontine Angle in a Woman with Neurofibromatosis Type 2

Primary Intracranial Malignant Nerve Sheath Tumor in the Cerebellopontine Angle in a Woman with Neurofibromatosis Type 2

Neurofibromatosis type 2 is an inherited disease. The mainmanifestation of the disease is the development of symmetric,non-malignant brain tumors in the region of the cranial nerveVIII usually as schwannoma. We report here a 20-year-oldwoman with primary intracranial malignant nerve sheath tumorloca...

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Main Authors: Bita Geramizadeh, Mehrzad Pourjafar, Ramin Mardani, Negar Azarpira, Saeid Farokhi
Format: Article
Language:English
Published: Shiraz University of Medical Sciences 2009-06-01
Series:Iranian Journal of Medical Sciences
Subjects:
Neurofibromatosis 2
malignant nerve sheath tumor
cerebellopontine angle
Online Access:http://ijms.sums.ac.ir/files/PDFfiles/34_2_10-Dr_%20Azarpira.pdf
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spelling doaj-d65f36ff3cfb46619db9a7d06b67dd872020-11-25T02:39:14ZengShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-07161735-36882009-06-01342141144Primary Intracranial Malignant Nerve Sheath Tumor in the Cerebellopontine Angle in a Woman with Neurofibromatosis Type 2Bita GeramizadehMehrzad PourjafarRamin MardaniNegar AzarpiraSaeid FarokhiNeurofibromatosis type 2 is an inherited disease. The mainmanifestation of the disease is the development of symmetric,non-malignant brain tumors in the region of the cranial nerveVIII usually as schwannoma. We report here a 20-year-oldwoman with primary intracranial malignant nerve sheath tumorlocated in the left cerebellopontine angle. Histologically, the tumorshowed malignant spindle cells in fascicular pattern withfocal S100 positivity on immunohistochemistry. A subtotal surgicalresection was performed followed by adjuvant radiotherapy.This is the first case of primary intracranial malignant nervesheath tumor associated with neurofibromatosis type 2 reportedin the literature. The prognosis of this potentially aggressive neoplasmis poor.http://ijms.sums.ac.ir/files/PDFfiles/34_2_10-Dr_%20Azarpira.pdfNeurofibromatosis 2malignant nerve sheath tumorcerebellopontine angle
collection DOAJ
language English
format Article
sources DOAJ
author Bita Geramizadeh
Mehrzad Pourjafar
Ramin Mardani
Negar Azarpira
Saeid Farokhi
spellingShingle Bita Geramizadeh
Mehrzad Pourjafar
Ramin Mardani
Negar Azarpira
Saeid Farokhi
Primary Intracranial Malignant Nerve Sheath Tumor in the Cerebellopontine Angle in a Woman with Neurofibromatosis Type 2
Iranian Journal of Medical Sciences
Neurofibromatosis 2
malignant nerve sheath tumor
cerebellopontine angle
author_facet Bita Geramizadeh
Mehrzad Pourjafar
Ramin Mardani
Negar Azarpira
Saeid Farokhi
author_sort Bita Geramizadeh
title Primary Intracranial Malignant Nerve Sheath Tumor in the Cerebellopontine Angle in a Woman with Neurofibromatosis Type 2
title_short Primary Intracranial Malignant Nerve Sheath Tumor in the Cerebellopontine Angle in a Woman with Neurofibromatosis Type 2
title_full Primary Intracranial Malignant Nerve Sheath Tumor in the Cerebellopontine Angle in a Woman with Neurofibromatosis Type 2
title_fullStr Primary Intracranial Malignant Nerve Sheath Tumor in the Cerebellopontine Angle in a Woman with Neurofibromatosis Type 2
title_full_unstemmed Primary Intracranial Malignant Nerve Sheath Tumor in the Cerebellopontine Angle in a Woman with Neurofibromatosis Type 2
title_sort primary intracranial malignant nerve sheath tumor in the cerebellopontine angle in a woman with neurofibromatosis type 2
publisher Shiraz University of Medical Sciences
series Iranian Journal of Medical Sciences
issn 0253-0716
1735-3688
publishDate 2009-06-01
description Neurofibromatosis type 2 is an inherited disease. The mainmanifestation of the disease is the development of symmetric,non-malignant brain tumors in the region of the cranial nerveVIII usually as schwannoma. We report here a 20-year-oldwoman with primary intracranial malignant nerve sheath tumorlocated in the left cerebellopontine angle. Histologically, the tumorshowed malignant spindle cells in fascicular pattern withfocal S100 positivity on immunohistochemistry. A subtotal surgicalresection was performed followed by adjuvant radiotherapy.This is the first case of primary intracranial malignant nervesheath tumor associated with neurofibromatosis type 2 reportedin the literature. The prognosis of this potentially aggressive neoplasmis poor.
topic Neurofibromatosis 2
malignant nerve sheath tumor
cerebellopontine angle
url http://ijms.sums.ac.ir/files/PDFfiles/34_2_10-Dr_%20Azarpira.pdf
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AT mehrzadpourjafar primaryintracranialmalignantnervesheathtumorinthecerebellopontineangleinawomanwithneurofibromatosistype2
AT raminmardani primaryintracranialmalignantnervesheathtumorinthecerebellopontineangleinawomanwithneurofibromatosistype2
AT negarazarpira primaryintracranialmalignantnervesheathtumorinthecerebellopontineangleinawomanwithneurofibromatosistype2
AT saeidfarokhi primaryintracranialmalignantnervesheathtumorinthecerebellopontineangleinawomanwithneurofibromatosistype2
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