Although, IDH-wild-type and IDH-mutant gliomas are histologically similar, numerous groups have reported that gliomas harboring IDH mutations represent a distinct disease entity that arises from a different cell type and occur in the presence of other genomic abnormalities, such as TP53 mutation or 1p/19q chromosome deletion, and happen mutually exclusively in gliomas with EGFR amplification and chromosome 10 loss [17]. The gene discussed is IDH2; the disease is central nervous system cancer.