malignant peripheral nerve sheath tumour

(redirected from malignant neurilemoma)

malignant peripheral nerve sheath tumour

A poorly understood malignancy, presumably of Schwann cell origin, which is thought to arise from the neural crest. It is the most common malignancy of peripheral nerves, and represents 5 to 10% of all soft-tissue tumours. It occurs in the lower extremities in relatively young patients (age 20 to 50). Half of cases occur in patients with neurofibromatosis type 1, in which the male:female ratio is 4:1; 2/3 of MPNSTs arise from neurofibromas, 1/3 arise de novo. Origin from pre-existing schwannomas, ganglioneuromas or phaeochromocytomas are rare; 10% arise in a background of therapeutic radiation.
 
Management
Wide local excision; radiation and chemotherapy have been used with varying degrees of failure.
References in periodicals archive ?
Malignant peripheral nerve sheath tumor--also known as neurogenic sarcoma, malignant neurilemoma, malignant schwannoma, and neurofibrosarcoma--is an uncommon neoplasm that originates in Schwann's cells of the nerve sheath.

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