1型和2型神经纤维瘤病患者中枢和周围神经系统肿瘤的治疗进展

IF 0.9 4区医学 Q4 CLINICAL NEUROLOGY

Pediatric Neurosurgery Pub Date : 2023-01-01 Epub Date: 2023-02-06 DOI:10.1159/000529507

Chelsea Kotch, Stephanie Nicole Brosius, Thomas De Raedt, Michael Jay Fisher

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引用次数: 2

摘要

背景:1型神经纤维瘤病和2型神经纤维瘤病是两种不相关的、不同的遗传性疾病，以中枢和周围神经系统肿瘤的发展为特征。摘要:1型神经纤维瘤病是最常见的遗传性肿瘤易感性综合征，其终身良性和恶性肿瘤发展的风险增加，如胶质瘤和神经鞘肿瘤。2型神经纤维瘤病典型表现为双侧前庭神经鞘瘤，但也与非前庭神经鞘瘤、脑膜瘤和室管膜瘤相关。历史上，神经纤维瘤病相关肿瘤的有效治疗方法数量有限。关键信息:在过去的十年中，在nf相关肿瘤的精确治疗方面取得了重大进展，除了肿瘤反应外，还越来越强调功能结果。持续的科学发现和NF相关肿瘤靶向治疗的进展对于继续改善NF患者的预后是必要的。

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Updates in the Management of Central and Peripheral Nervous System Tumors among Patients with Neurofibromatosis Type 1 and Neurofibromatosis Type 2.

Background: Neurofibromatosis type 1 and neurofibromatosis type 2 are unrelated, distinct genetic disorders characterized by the development of central and peripheral nervous system tumors.

Summary: Neurofibromatosis type 1 is the most common inherited tumor predisposition syndrome with a lifelong increased risk of benign and malignant tumor development, such as glioma and nerve sheath tumors. Neurofibromatosis type 2 classically presents with bilateral vestibular schwannoma, yet it is also associated with non-vestibular schwannoma, meningioma, and ependymoma. Historically, the number of effective therapies for neurofibromatosis-related neoplasms has been limited.

Key message: In the past decade, there have been significant advances in the development of precision-based therapies for NF-associated tumors with an increased emphasis on functional outcomes in addition to tumor response. Continued scientific discovery and advancement of targeted therapies for NF-associated neoplasms are necessary to continue to improve outcomes for patients with NF.

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来源期刊

Pediatric Neurosurgery 医学-临床神经学

CiteScore

1.30

自引率

0.00%

发文量

审稿时长

>12 weeks

期刊介绍： Articles in ''Pediatric Neurosurgery'' strives to publish new information and observations in pediatric neurosurgery and the allied fields of neurology, neuroradiology and neuropathology as they relate to the etiology of neurologic diseases and the operative care of affected patients. In addition to experimental and clinical studies, the journal presents critical reviews which provide the reader with an update on selected topics as well as case histories and reports on advances in methodology and technique. This thought-provoking focus encourages dissemination of information from neurosurgeons and neuroscientists around the world that will be of interest to clinicians and researchers concerned with pediatric, congenital, and developmental diseases of the nervous system.