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.
Primary malignant neoplasms of nerves (malignant neurilemomas) in patients without manifestations of multiple neurofibromatosis (Von Recklinghausen's disease) Cancer.
Primary malignant neoplasms of nerves (malignant neurilemomas) in patients with manifestations of multiple neurofibromatosis (von Recklinghausen's disease).

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