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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Bibliographic Details
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
Description
Summary: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.
ISSN:0253-0716
1735-3688

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