IDH mutations are considered gain-of-function and lead to the disruption of the normal catalytic activity of IDH1/2, ultimately resulting in increased conversion of α-KG to D-2-hydroxyglutarate (D-2HG), which acts as an oncometabolite, promoting tumor proliferation and metastasis development through several pathways, such as DNA methylation and activation of VEGFR [19,22,23] (Figure 2). Here, IDH1 is linked to neoplasm.