Intra-cranial malignant peripheral nerve sheath tumor of olfactory nerve: A case report and review of literature

Abstract

Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are one of the very rare high grade malignancies usually affecting extremities or trunk. Incidence is 1/Lac. Intra-cranial MPNSTs are even rarer, schwannomatous and commonly affecting cranial nerves VIII &VII). Intra-cranial MPNSTs are usually sporadic, arising de novo. The second most common mode of origin is from malignant transformation from pre-existing schwannomas or neurofibroma. We present an extremely rare and probably the first case of intra-cranial malignant peripheral nerve sheath tumor of the olfactory nerve in a non neurofibrosis patient with no prior history of irradiation.