Thus, while mutations in IDH1 or -2 are not essential for chondrosarcoma proliferation, the results of the present study show an effect of D-2-HG on differentiation of MSCs during bone development, promoting chondrogenic and inhibiting osteogenic differentiation, indicating a crucial role for IDH1/2 mutations in the development of benign enchondromas. This evidence concerns the gene IDH1 and chondrosarcoma.