These mixed results in glioma models can be explained by the fact that IDH mutations are an early event in gliomagenesis and an oncogenic driver in LGGs; however, during tumour evolution, IDH mutant gliomas acquire a plethora of other subsequent mutations, which might render gliomas, and especially high-grade gliomas, less susceptible IDH mutant inhibitors. This evidence concerns the gene IDH1 and central nervous system cancer.