DR. KAUSTUBH MULAY

DR. SANTOSH G. HONAVAR

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are unusual malignant soft tissue tumors that arise from the Schwann cells or within neurofibromas. Majority are associated with neurofibromatosis type-1. Orbital involvement is rare. Even more rare is its origin from a schwannoma. A variety of histological differentiation has been described in MPNSTs. However, occurence of more than one differentiation in the same tumor is unusual. We describe one such case of MPNST with divergent differentiation arising within a schwannoma.