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

Author(s): Negar Azarpira | Ramin Mardani | Mehrzad Pourjafar | Bita Geramizadeh | Saeid Farokhi

Journal: Iranian Journal of Medical Sciences
ISSN 0253-0716

Volume: 34;
Issue: 2;
Start page: 141;
Date: 2009;
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Keywords: Neurofibromatosis 2 | malignant nerve sheath tumor | cerebellopontine angle

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.
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