Somatic mutations in the isocitrate dehydrogenase 1 (IDH1) or IDH2 genes are common in enchondromas and chondrosarcomas, as well as in several neoplasms, including glioma, glioblastoma, acute myeloid leukemia, and intrahepatic cholangiocarcinomas (IHCC) [2–4]. Here, IDH2 is linked to neoplasm.