2008 年，我们发现许多成人神经胶质瘤的代谢基因异柠檬酸脱氢酶 (IDH) 存在迄今未知的突变，这一发现引发了我们对神经胶质瘤生物学和相应分类的理解的革命性进展。 IDH 突变存在于大多数非胶质母细胞瘤成人胶质瘤中，预示着更好的预后。大量的努力已经揭示了这些突变的许多多效性细胞效应，并催生了几条研究方向，以针对治疗效果。本文回顾了 IDH 突变对神经胶质瘤的影响，特别关注最近在这些通常难治性肿瘤中进行的罕见的阳性 3 期试验的研究。

The discovery in 2008 that many adult gliomas harbor a hitherto unknown mutation in the metabolic gene isocitrate dehydrogenase (IDH) initiated revolutionary advances in our understanding of the biology, and correspondingly our classification, of gliomas. IDH mutations are found in most nonglioblastoma adult gliomas and portend a better prognosis. Massive efforts have unraveled many of the pleiotropic cellular effects of these mutations and spawned several lines of investigation to target the effect to therapeutic benefit. In this article are reviewed the implications of the IDH mutation in gliomas, in particular focusing on recent studies that have culminated in a rare positive phase 3 trial in these generally refractory tumors.