Mohammad N, Wong D, Lum A, et al. - Researchers intended to investigate the predominance of Isocitrate dehydrogenase (IDH) mutations in a single institution cohort of dedifferentiated chondrosarcoma (DDCHS; n = 21) and associate D-2-hydroxyglutarate (2HG) levels to mutation status. A heterozygous IDH1 or IDH2 mutation were harbored by 76% of DDCHS (16/21). With regards to matched normal tissue, in the tumor, 6/14 IDH-mutated DDCHS exhibited raised 2HG. Between IDH-mutated tumors and wild-type tumors, no constant histologic or disease-specific survival changes were seen. Hence, the common presence of a variety of IDH1 and IDH2 mutation variants was validated in this study, designating that if IDH is to be used as a diagnostic marker, a sequencing-based strategy is needed for DDCHS. Moreover, IDH-mutated DDCHS, comparable to other IDH-mutated tumor types, also exhibit raised 2HG level, symbolizing that the oncometabolite activity of 2HG could add to DDCHS oncogenesis and progression.