In recent years, recurrent heterozygous hotspot mutations in isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) genes, specifically at residues p.R132 and p.R140/p.R172, respectively, have been frequently identified in cartilage tumors, such as enchondromas, central conventional chondrosarcomas, and dedifferentiated chondrosarcomas. This evidence concerns the gene IDH2 and chondrogenic neoplasm.