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Malignant peripheral



        nerve sheath tumour



        (MPNST)










         2-3% of malignant tumours






         Affects adults between 20-50 years of age






         May            arise         de        novo          or       in      pre-existing


            neurofibromas.





         Three groups:



              Neurofibromatosis-related
                 (neurofibrosarcoma)


              Post-irradiation


              Solitary






         Most are associated with major nerves of the

            extremities or trunk







         Aggressive with high metastatic potential





         Gold standard is excision with adequate

            margins as tumour has poor sensitivity to

            radiotherapy and chemotherapy
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