IDH2 and central nervous system cancer: One of the epoch-making discoveries in glioma pathogenesis is the identification of hotspot mutations in isocitrate dehydrogenase 1 (IDH1), or less commonly IDH2 genes in more than 70% of diffusely infiltrating World Health Organization (WHO) grade II and grade III astrocytic and oligodendroglial gliomas, as well as in a minor fraction of GBMs that develop from lower grade gliomas (LGGs) [2,30,31].