A malignant peripheral nerve sheath tumor (MPNST) is defined as a malignant tumor that arises from a peripheral nerve or exhibits nerve sheath differentiation. Tumors arising from the epineurium or the vascularization of peripheral nerves are not included1.
Epidemiology
Malignant peripheral nerve sheath tumors account for approximately 5-10% of all soft-tissue sarcomas and typically occur in patients 20 to 50 years of age. However, cases in pediatric patients have also been described2.
Associated conditions
Approximately 50% of malignant peripheral nerve sheath tumors are associated with neurofibromatosis type 13.
Metastasis
Malignant peripheral nerve sheath tumors spread along peripheral nerves and can metastasize hematogenously in 20% of cases, typically to the lungs4.
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