恶性周围神经鞘肿瘤的治疗靶点

IF 0.6 Q4 CLINICAL NEUROLOGY

Future Neurology Pub Date : 2019-01-15 DOI:10.2217/FNL-2018-0026

L. M. Wu, Q. Lu

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引用次数: 20

摘要

恶性周围神经鞘肿瘤是一种罕见的侵袭性软组织肉瘤，预后较差。完全切除是唯一已知的最终治疗方法，但并非对所有肿瘤都可行，手术后局部复发是成功治疗的另一个挑战。其他类型肉瘤的治疗尚未证明对MPNST患者有益。在临床试验中，阻断几种已知驱动MPNST发病机制的信号通路的靶向治疗也没有改善患者的预后。这篇综述讨论了现有的治疗方法和目前正在临床测试的靶向化疗方案，以及在临床前研究中确定的潜在治疗途径，包括靶向信号通路，如HIPPO-YAP通路和表观遗传机制以及多药物策略。

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Therapeutic targets for malignant peripheral nerve sheath tumors

Malignant peripheral nerve sheath tumors (MPNSTs) are rare and aggressive soft-tissue sarcomas with dismal prognosis. Complete resection, which is the only known definitive therapy, is not feasible with every tumor, and local recurrence after surgery is another challenge to successful treatment. Treatments used with other sarcoma types have not proven beneficial to MPNST patients. Targeted therapies blocking several signaling pathways known to drive MPNST pathogenesis have also not improved patient outcomes in clinical trials. This review discusses existing therapies and targeted chemotherapeutic options currently being tested clinically, and potential therapeutic avenues identified in preclinical studies that include targeting signaling pathways such as the HIPPO-YAP pathway and epigenetic mechanisms as well as multi-agent strategies.

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来源期刊

Future Neurology CLINICAL NEUROLOGY-

CiteScore

2.10

自引率

0.00%

发文量

期刊介绍： The neurological landscape is changing rapidly. From the technological perspective, advanced molecular approaches and imaging modalities have greatly increased our understanding of neurological disease, with enhanced prospects for effective treatments in common but very serious disorders such as stroke, epilepsy, multiple sclerosis and Parkinson’s disease. Nevertheless, at the same time, the healthcare community is increasingly challenged by the rise in neurodegenerative diseases consequent upon demographic changes in developed countries.