Even with the demonstration that an accumulation of D-2HG, in the case of the D-2-hydroxyglutaric aciduria, does not predispose individuals to develop tumors and those patients’ bearing gliomas with IDH1 mutations have better survival expectancies than those bearing the wild-type ones, the overproduction of 2HG in gliomas, driven by the IDH mutations, has been used as an argument to explain the genesis of glioma. This evidence concerns the gene IDH1 and central nervous system cancer.