malignant peripheral nerve sheath tumour


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Related to malignant peripheral nerve sheath tumour: 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.
References in periodicals archive ?
DISCUSSION: Malignant peripheral nerve sheath tumour arises from a peripheral nerve or intrinsic nerve sheath differentiation.
Multiple brain metastases from malignant peripheral nerve sheath tumour (MPNST).
Immunohistochemical and molecular analysis of p53, MDM2, proliferating cell nuclear antigen and Ki67 in benign and malignant peripheral nerve sheath tumours.
Malignant peripheral nerve sheath tumours (MPNSTs) are the most frequent malignant neoplasms, occurring in up to 10% of affected individuals.

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