malignant peripheral nerve sheath tumour

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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 ?
Up until now, the preferred treatment for MPNST has been surgical removal, but that oftentimes is difficult or impossible due to the tumor's location around nerves.
The term MPNST replaces a number of previously used names including malignant schwannoma, neurofibrosarcoma, and neurogenic sarcoma [2, 3].
La cirugia radical es el tratamiento de eleccion, sin embargo, la resecabilidad depende de la localizacion tumoral, siendo de un 20% en los MPNST paraespinales y de un 95% en las extremidades (1,4).
5] MPNST show poor prognosis, with reported rates of 5-year disease specific survival of 16% to 60%.
In addition, individuals with an improperly-functioning NF1 gene have an increased risk of developing cancerous tumors such as MPNSTs, he said.
Although only three cases of MPNST were observed, it is interesting to note that all occurred in male patients.
Moreover, patients and physicians fundamentally agree on which manifestations of NF1 are most important to treat, with MPNST, plexiform neurofibromas, and optic gliomas rated as top priorities for both groups.
Utility of Sox-10 to distinguish MPNST from synovial sarcoma with a focus on intraneural synovial sarcoma [USCAP Abstract 56].
No randomized study strongly suggested chemotherapy to MPNST patient.
This rare case of MPNST with intracranial involvement illustrates the dismal prognosis for patients with such a lesion.
Between 25% and 75% of patients who develop MPNST have NF1.