INTRAOSSEOUS MALIGNANT PERIPHERAL-NERVE SHEATH TUMOR IN A PATIENT WITH NEUROFIBROMATOSIS

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcoma s that almost always arise in soft tissue. They can develop in pre-exi sting neurofibromas or schwannomas, de novo from peripheral nerves, or following radiation therapy. Primary intraosseous MPNST is rare and h as been reported most frequently in the mandible. Of the reported case s involving the long bones, none has been associated with neurofibroma tosis type 1 (NF-1). We report a case of MPNST arising in the femur in a patient with NF-1.