The importance of IDH1 mutations and the associated D-2HG production3 to the clinical manifestation of the gliomas is emphasized by the fact that the World Health Organization (WHO) released a novel classification of glioma in 2016 to include IDH1 mutations as molecular markers that dictate the classification4 and more recently distinguished grade 4 IDH1mut tumors from the IDH1WT glioblastoma5,6. Here, IDH1 is linked to central nervous system cancer.