Dg. Terry et al., INTRAOSSEOUS MALIGNANT PERIPHERAL-NERVE SHEATH TUMOR IN A PATIENT WITH NEUROFIBROMATOSIS, Skeletal radiology, 27(6), 1998, pp. 346-349
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.