Intraosseous malignant peripheral nerve sheath tumor in a patient with neurofibromatosis.
Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcomas that almost always arise in soft tissue. They can develop in pre-existing neurofibromas or schwannomas, de novo from peripheral nerves, or following radiation therapy. Primary intraosseous MPNST is rare and has been reported most frequently in the mandible. Of the reported cases involving the long bones, none has been associated with neurofibromatosis type 1 (NF-1). We report a case of MPNST arising in the femur in a patient with NF-1.
Terry
D G
DG
VA Medical Center, Imaging Service, Portland, OR 97201, USA.
Sauser
D D
DD
Gordon
M D
MD
D000328:Adult; D005266:Femoral Neoplasms / Q000175:diagnosis* / Q000473:pathology; D005269:Femur / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis* / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000473:pathology; D012680:Sensitivity and Specificity; D012983:Soft Tissue Neoplasms / Q000175:diagnosis* / Q000473:pathology; D014057:Tomography, X-Ray Computed