neurofibrosarcoma

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.

neurofibrosarcoma

See NEUROSARCOMA.

单词	neurofibrosarcoma
释义	neurofibrosarcoma neurofibrosarcoma [¦nu̇r·ō‚fī·brō·sär′kō·mə] (medicine) A malignant tumor composed of interlacing bundles of anaplastic spindle-shaped cells which resemble those of nerve sheaths. neurofibrosarcoma 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. neurofibrosarcoma See NEUROSARCOMA.
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