Major advances in cancer genetics over the past decade have documented the involvement of IDH mutations in a variety of human malignancies, including gliomas, acute myeloid leukemia, chondrosarcoma, cholangiocarcinoma, and thyroid carcinoma, but also in prostate adenocarcinoma and non-small cell lung cancer [50,51,52]. This evidence concerns the gene IDH2 and cholangiocarcinoma.