Medical term:
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.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.
neurofibrosarcoma
See NEUROSARCOMA.Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005
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