Following the discovery of these mutations at a surprisingly high frequency in low grade gliomas, IDH1 and IDH2 mutations were subsequently observed in several other cancers and syndromes, including acute myeloid leukemia [6-8], preleukemic clonal malignancies [9], central and periosteal cartilaginous tumors [10], intrahepatic origin cholangiocarcinomas [11], Ollier disease and Maffucci syndrome [12, 13]. This evidence concerns the gene IDH1 and central nervous system cancer.