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NF1 患者中儿童和成人脑胶质瘤新认识的影响:共识会议报告。

Implications of new understandings of gliomas in children and adults with NF1: report of a consensus conference.

机构信息

Center for Neuroscience and Behavioral Medicine, Washington, DC, USA.

Gilbert Family Neurofibromatosis Institute, Brain Tumor Institute, and Children's National Hospital, Washington, DC, USA.

出版信息

Neuro Oncol. 2020 Jun 9;22(6):773-784. doi: 10.1093/neuonc/noaa036.

Abstract

Gliomas are the most common primary central nervous system tumors occurring in children and adults with neurofibromatosis type 1 (NF1). Over the past decade, discoveries of the molecular basis of low-grade gliomas (LGGs) have led to new approaches for diagnosis and treatments. However, these new understandings have not been fully applied to the management of NF1-associated gliomas. A consensus panel consisting of experts in NF1 and gliomas was convened to review the current molecular knowledge of NF1-associated low-grade "transformed" and high-grade gliomas; insights gained from mouse models of NF1-LGGs; challenges in diagnosing and treating older patients with NF1-associated gliomas; and advances in molecularly targeted treatment and potential immunologic treatment of these tumors. Next steps are recommended to advance the management and outcomes for NF1-associated gliomas.

摘要

神经纤维瘤病 1 型(NF1)患者可发生脑和脊髓的胶质瘤,这是最常见的原发性中枢神经系统肿瘤。过去十年中,人们发现了低级别胶质瘤(LGG)的分子基础,这为诊断和治疗开辟了新途径。然而,这些新的认识尚未完全应用于 NF1 相关胶质瘤的治疗。为此,我们成立了一个由 NF1 和胶质瘤专家组成的共识小组,旨在回顾 NF1 相关低级别的“转化”和高级别胶质瘤的现有分子知识;从 NF1-LGG 小鼠模型中获得的新见解;诊断和治疗 NF1 相关胶质瘤老年患者所面临的挑战;以及这些肿瘤的分子靶向治疗和潜在免疫治疗的进展。本共识还提出了一些建议,以进一步改善 NF1 相关胶质瘤的治疗效果。

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