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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 France The prevalence of malignant peripheral nerve sheath tumor (MPNST) among the general population is only 0.001%.