恶性周围神经鞘肿瘤的综合治疗

Q4 Medicine Journal of Oncological Sciences Pub Date : 2020-01-01 DOI:10.37047/jos.2019-73127

Y. Karakaş, O. Dizdar, K. Kösemehmetoğlu, U. B. Bozbulut, Metin Demir, G. Gedıkoğlu, F. Söylemezoğlu, A. Turker, A. Kars

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引用次数: 1

摘要

恶性周围神经鞘瘤(MPNST)是一种罕见的软组织肉瘤，起源于周围神经，假设起源于神经嵴MPNST的发病率为1:10万/年，占所有软组织肉瘤的5-10%大约50%的mpnst是偶发的，其余的继发于先前的辐射暴露和1型神经纤维瘤病(NF1)患者。在NF1患者中，MPNST通常起源于丛状神经纤维瘤和非典型神经纤维瘤。这些患者发生MPNST的终生风险约为8-13%。5-8 mpnst多发生于四肢的大中型神经[j] .中华肿瘤杂志，2020;6(1):23-8

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Multimodality Treatment in Malignant Peripheral Nerve Sheath Tumors

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon soft tissue sarcoma that originates from the peripheral nerves and is hypothesized to be of neural crest origin.1 The incidence of MPNST is 1:100.000/year and accounts for 5-10% of all soft tissue sarcomas.2 Approximately 50% of MPNSTs occur sporadically, the remaining arises secondary to prior radiation exposure and in patients with neurofibromatosis type 1 (NF1).3-5 In the NF1 patients, MPNST usually originates from plexiform neurofibromas and atypical neurofibromas. The lifetime risk of developing MPNST in these patients is around 8-13%.5-8 MPNSTs often arise from large and medium-sized nerves, located in the extremities in 33J Oncol Sci. 2020;6(1):23-8

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