Primary intraosseous malignant peripheral nerve sheath tumor
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Malignant peripheral nerve sheath tumor (MPNST) usually arises in peripheral nerve sheath cells. Up to 50% of MPNSTs arise in patients with neurofibromatosis-1 (NF-1). They usually arise in the soft-tissue. The intraosseous location of MPNST is rare. Mandible is the most common site of bony involvement. Involvement of bones of the hand is quite unusual. There are isolated case reports of MPNST involving maxilla, spine, sternum, and long bones of extremities. Origin from small bones of hand and foot is exceptionally rare. This article reports a case of MPNST involving metacarpal bones of the left hand treated with surgery followed by adjuvant radiation and chemotherapy and review the pertinent literature...

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