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 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.