Assuming that all IDH-mutated tumors (as well as enchondromas) observed in OD-MS patients derive from one IDH-mutant cell giving rise to different lineages, the observation of different tumors arising in organs deriving from the neuroectoderm (glioma), mesoderm (blood, cartilage) and endoderm (liver) points towards a very early post-zygotic event for the IDH mutation. Here, IDH1 is linked to central nervous system cancer.