Intra-cranial malignant peripheral nerve sheath tumor of olfactory nerve

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.

Keywords

• Intra-cranial malignant peripheral nerve sheath tumor
• Olfactory Nerve Tumor
• MPNST