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- W94225896 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" @default.
- W94225896 created "2016-06-24" @default.
- W94225896 creator A5040815350 @default.
- W94225896 creator A5052612386 @default.
- W94225896 creator A5057851992 @default.
- W94225896 date "2014-11-04" @default.
- W94225896 modified "2023-09-23" @default.
- W94225896 title "Malignant Peripheral Nerve Sheath Tumors" @default.
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