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成人毛细胞型星形细胞瘤的分子时代:全面综述。

Adult pilocytic astrocytoma in the molecular era: a comprehensive review.

机构信息

Department of Medicine, Neurology, Madigan Army Medical Center, Tacoma, WA 98431, USA.

University of Rochester School of Medicine & Dentistry, University of Rochester Medical Center, Rochester, NY 14642, USA.

出版信息

CNS Oncol. 2021 Mar 1;10(1):CNS68. doi: 10.2217/cns-2020-0027. Epub 2021 Jan 15.

Abstract

Adult pilocytic astrocytoma (PA) is less prevalent than pediatric PA and is associated with a worse prognosis. In a literature review, we found that 88.3% of the molecular alterations in adult PA are associated with MAPK pathway dysregulation. The most common alterations are fusions of . Understanding of the mechanisms underlying this pathway has evolved substantially, heralding advancements in specific targeted therapy. Here, we review clinical and molecular features of adult PA, characteristics predicting aggressive behavior and approaches to standard and investigational therapies. We highlight epigenetic profiling and integrated diagnosis as an essential component of classifying PA.

摘要

成人毛细胞型星形细胞瘤(PA)比儿童 PA 少见,且预后较差。在文献回顾中,我们发现 88.3%的成人 PA 的分子改变与 MAPK 通路失调有关。最常见的改变是. 对该通路相关机制的认识已经有了很大的发展,预示着特定靶向治疗的进步。在这里,我们回顾成人 PA 的临床和分子特征、预测侵袭性行为的特征以及标准和研究性治疗方法。我们强调表观遗传谱分析和综合诊断是 PA 分类的一个重要组成部分。

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