Center for Neuroscience and Behavioral Medicine, Washington, DC, USA.
Gilbert Family Neurofibromatosis Institute, Brain Tumor Institute, and Children's National Hospital, Washington, DC, USA.
Neuro Oncol. 2020 Jun 9;22(6):773-784. doi: 10.1093/neuonc/noaa036.
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.
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。