IDH2 and central nervous system cancer: GBM accounts for 70% of gliomas and can be subdivided in GBM IDH-wild type (the most frequent, >90%), previously known as primary (de novo) GBM and exhibiting a short clinical history, and GBM IDH-mutant, also called secondary GBM, which results from the malignant progression from lower-grade gliomas of diffuse (WHO grade II) or anaplastic (WHO grade III) astrocytomas [6,8] and is related to point mutations in IDH1/2 genes.