Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas on peripheral nerves or nerve sheath. These MPNSTs can occur on any cell type and differs from one to other. A sarcoma is regarded as MPNST only when it is raised on the peripheral nerve or it exists on neurofibroma. MPNSTs usually present as an enlarging palpable mass. Pain is a variable complaint. Rapid enlargement occurs more often in the setting of NF1 and should raise concern for malignant degeneration of a neurofibroma.
In Belgium The median follow-up time was 4.4 y. For 16 tumours in 12 patients with available follow-up data, we observed an actuarial tumour control of 87.5% at 2 y and 80.2% at 5 y, based on the Kaplan-Meier method. No patient developed facial weakness. Serviceable hearing was preserved in 78% of cases. Patients treated for bilateral and unilateral tumours had similar outcomes.