Malignant Peripheral Nerve Sheath Tumors
作者： Brian Weiss , Amy Sheil , Nancy Ratner
DOI: 10.1007/978-1-4939-1830-0_16
关键词: Pathology 、 Population 、 Plexiform neurofibroma 、 Peripheral Nerve Sheath Tumors 、 Neurogenic sarcoma 、 Medicine 、 Soft tissue 、 Neurofibrosarcoma 、 Primary tumor 、 Neurofibromatosis
摘要: Malignant peripheral nerve sheath tumors (MPNSTs) (previously called neurogenic sarcomas, malignant schwannomas, or neurofibrosarcomas) are soft tissue which arise from a show differentiation. MPNSTs associated with high risk of local recurrence and predominantly hematogenous metastasis. They account for 10 % all approximately half these malignancies in patients neurofibromatosis type 1 (NF1). occur about 2–5 NF1 compared an incidence 0.001 the general population. In contrast, large population-based longitudinal study lifetime developing MPNST was 8–13 %. NF1, majority previous clinically detectable plexiform neurofibroma, but may also develop as primary tumor.
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