Potential driver mutations of central conventional and dedifferentiated chondrosarcoma are gain of function mutations in isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2), which have been identified in 38–70% of the cases.9,10 Its occurrence in the benign precursors lesions (enchondromas), of which 52–87% harbour an IDH1/2 mutation,11,12 further demonstrates that IDH1/2 mutations are an early event in chondrosarcoma genesis. This evidence concerns the gene IDH2 and chondrosarcoma.