Malignant Peripheral Nerve Sheath Tumors

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) (previously called neurogenic sarcomas, malignant schwannomas, or neurofibrosarcomas) are soft tissue sarcomas, which arise from a peripheral nerve or show nerve sheath differentiation. MPNSTs are associated with a high risk of local recurrence and predominantly hematogenous metastasis. They account for 10 % of all soft tissue sarcomas, and approximately half of these malignancies arise in patients with neurofibromatosis type 1 (NF1). MPNSTs occur in about 2–5 % of patients with NF1 compared with an incidence of 0.001 % in the general population. In contrast, in a large population-based longitudinal study the lifetime risk of developing an MPNST in NF1 was 8–13 %. In patients with NF1, the majority of MPNSTs arise in a previous clinically detectable plexiform neurofibroma, but MPNST may also develop as a primary tumor.KeywordsMalignant peripheral nerve sheath tumorsPeripheral nerve sheath tumorsNervous system tumor pathologyMolecular pathology of nervous system tumorsNeurogenic sarcomasMalignant schwannomasNeurofibrosarcomas