从工作台到床边:推进前庭分裂瘤的治疗。

IF 3.7 Q1 CLINICAL NEUROLOGY Neuro-oncology advances Pub Date : 2024-06-24 eCollection Date: 2024-01-01 DOI:10.1093/noajnl/vdae107
Shaolei Guo, Xuan Zheng, Wenli Chen, Umar Raza, Ailiang Zeng, Farhana Akter, Quan Huang, Shun Yao
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引用次数: 0

摘要

前庭裂神经瘤是一种罕见的颅内肿瘤,起源于前庭神经的许旺细胞。尽管这些肿瘤是良性的,但会产生明显的肿块效应和使人衰弱的症状,包括听力逐渐减退、眩晕、面神经功能障碍和头痛。目前的临床治疗方案包括等待和扫描、手术、放射治疗和标签外药物治疗。然而,每种方法都有其自身的挑战和局限性,这凸显了对治疗方法的迫切需求。在过去的二十年中,对前庭裂隙瘤分子基础的广泛研究揭示了基因异常、信号通路失调、受体酪氨酸激酶下游、细胞外基质破坏、炎症性肿瘤微环境和脑脊液成分改变等驱动疾病发生和发展的不可或缺的因素。有了这些知识,人们正在积极寻求针对这些疾病独特分子特征的新型治疗干预措施。这篇综述强调了解决美国食品和药物管理局批准的前庭裂隙瘤药物匮乏问题的紧迫性,重点介绍了关键的分子发现及其转化为疗法的潜力。报告深入探讨了不断发展的治疗开发前景,目前正在从实验室向临床转化。这些正在进行的努力有望在未来极大地改变前庭分裂瘤患者的生活。
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From bench to bedside: Advancing towards therapeutic treatment of vestibular schwannomas.

Vestibular schwannomas are rare intracranial tumors originating from Schwann cells of the vestibular nerve. Despite their benign nature, these tumors can exert significant mass effects and debilitating symptoms, including gradual hearing loss, vertigo, facial nerve dysfunction, and headaches. Current clinical management options encompass wait-and-scan, surgery, radiation therapy, and off-label medication. However, each approach exhibits its own challenges and harbors limitations that underscore the urgent need for therapeutic treatments. Over the past 2 decades, extensive elucidation of the molecular underpinnings of vestibular schwannomas has unraveled genetic anomalies, dysregulated signaling pathways, downstream of receptor tyrosine kinases, disrupted extracellular matrix, inflammatory tumor microenvironment, and altered cerebrospinal fluid composition as integral factors in driving the development and progression of the disease. Armed with this knowledge, novel therapeutic interventions tailored to the unique molecular characteristics of those conditions are actively being pursued. This review underscores the urgency of addressing the dearth of Food and Drug Administration-approved drugs for vestibular schwannoma, highlighting the key molecular discoveries and their potential translation into therapeutics. It provides an in-depth exploration of the evolving landscape of therapeutic development, which is currently advancing from bench to bedside. These ongoing efforts hold the promise of significantly transforming the lives of vestibular schwannoma patients in the future.

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CiteScore
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