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    GENE:

    IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)

    i
    Other names: IDH2, Isocitrate Dehydrogenase (NADP(+)) 2, Isocitrate Dehydrogenase (NADP(+)) 2, Mitochondrial, Isocitrate Dehydrogenase 2 (NADP+), Mitochondrial, Isocitrate Dehydrogenase [NADP], Mitochondrial, Oxalosuccinate Decarboxylase, NADP(+)-Specific ICDH, ICD-M, IDH, IDP, MNADP-IDH, D2HGA2, IDHM, IDPM
    1d
    LS1781: Ascorbic Acid and Chemotherapy for the Treatment of Relapsed or Refractory Lymphoma, CCUS, and Chronic Myelomonocytic Leukemia (clinicaltrials.gov)
    P2, N=80, Recruiting, Mayo Clinic | Trial completion date: Mar 2026 --> Nov 2033 | Trial primary completion date: Dec 2025 --> Feb 2031
    Trial completion date • Trial primary completion date
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • BCL2 (B-cell CLL/lymphoma 2) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • DNMT3A (DNA methyltransferase 1) • SF3B1 (Splicing Factor 3b Subunit 1) • TET2 (Tet Methylcytosine Dioxygenase 2) • SRSF2 (Serine and arginine rich splicing factor 2) • U2AF1 (U2 Small Nuclear RNA Auxiliary Factor 1) • CD4 (CD4 Molecule) • ZRSR2 (Zinc Finger CCCH-Type, RNA Binding Motif And Serine/Arginine Rich 2)
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    IDH2 mutation • TET2 mutation • SF3B1 mutation • EZH2 mutation • SRSF2 mutation
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    cisplatin • carboplatin • gemcitabine • Rituxan (rituximab) • cytarabine • cyclophosphamide • ifosfamide • oxaliplatin • etoposide IV • decitabine • Truxima (rituximab-abbs) • Hemady (dexamethasone tablets) • Mabtas (rituximab biosimilar) • Starasid (cytarabine ocfosfate) • dexamethasone injection
    2d
    All-Trans Retinoic Acid (ATRA) Plus PD-1 Inhibition in Recurrent IDH-Mutant Glioma (clinicaltrials.gov)
    P2, N=55, Recruiting, Stephen Bagley, MD, MSCE | Trial completion date: Jun 2027 --> Jun 2028 | Trial primary completion date: Jun 2026 --> Jun 2027
    Trial completion date • Trial primary completion date • IO biomarker
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • MGMT (6-O-methylguanine-DNA methyltransferase)
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    IDH1 mutation • IDH2 mutation
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    Zynyz (retifanlimab-dlwr)
    3d
    VIGOR: Vorasidenib Maintenance for IDH Mutant Astrocytoma (clinicaltrials.gov)
    P3, N=468, Recruiting, European Organisation for Research and Treatment of Cancer - EORTC | Not yet recruiting --> Recruiting
    Enrollment open
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    IDH2 mutation
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    Voranigo (vorasidenib)
    4d
    18F-DOPA-PET and advanced MRI improve treatment response assessment in IDH1/2-mutant gliomas treated with IDH inhibitors. (PubMed, Clin Cancer Res)
    These results highlight the potential of ¹⁸F-DOPA-PET and advanced MRI sequences as valuable complements to standard RANO 2.0 MRI evaluations for assessing treatment response in glioma patients undergoing IDHi therapy.
    Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    Tibsovo (ivosidenib) • Voranigo (vorasidenib)
    4d
    Exploring CSF microRNA signatures as diagnostic biomarkers in adult-type diffuse gliomas. (PubMed, Noncoding RNA Res)
    Ingenuity Pathway Analysis (IPA) revealed that miR-16-5p and other miRNAs with seed AGCAGCA formed the largest interaction network in GBM, while disease enrichment analysis using Database for Annotation, Visualization, and Integrated Discovery (DAVID) confirmed that the 1000 predicted mRNA targets of DE-miRNAs in GBM were disease relevant. Collectively, these findings identify a robust panel of CSF-derived miRNAs capable of distinguishing IDH-mutant gliomas, GBM, and non-tumor states, supporting the potential of EV-miRNAs as minimally invasive biomarkers for the molecular characterization of diffuse gliomas.
    Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • MIR21 (MicroRNA 21) • MIR142 (MicroRNA 142) • MIR16 (MicroRNA 16) • MIR19B1 (MicroRNA 19b-1) • MIR27B (MicroRNA 27b) • MIR99A (MicroRNA 99a) • MIR150 (MicroRNA 150) • MIR195 (MicroRNA 195) • MIR26A1 (MicroRNA 26a-1) • MIR30A (MicroRNA 30a) • MIR30E (MicroRNA 30e)
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    IDH wild-type
    4d
    Non-coding small RNAs Buffer protein interactions to prevent oncogenic aggregation: structural dampening of aberrant PPIs by RNA. (PubMed, RNA Biol)
    At the network level, physiological PPIs exhibit high shared ncRNA buffering capacity, whereas oncogenic interactions are characterized by reduced or absent RNA overlap. AlphaFold3 modelling of mutant IDH1/2 complexes illustrates how loss of RNA buffering permits excessive stabilization of enzyme-associated interfaces, reflected by directional changes in buried surface area (ΔBSA) and contact heterogeneity.
    Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    IDH1 mutation
    6d
    Bile duct tumor thrombus (intraductal polypoid growth)-positive intrahepatic cholangiocarcinoma: clinicopathologic and genomic analysis. (PubMed, J Pathol)
    These results highlight the importance of evaluating BDTT in SDT, as it may be the main route of hilar extension in aggressive cases.
    Journal
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    KRAS (KRAS proto-oncogene GTPase) • BRAF (B-raf proto-oncogene) • TP53 (Tumor protein P53) • PIK3CA (Phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha) • NRAS (Neuroblastoma RAS viral oncogene homolog) • FGFR2 (Fibroblast growth factor receptor 2) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • ARID1A (AT-rich interaction domain 1A) • NF1 (Neurofibromin 1) • BAP1 (BRCA1 Associated Protein 1) • KMT2D (Lysine Methyltransferase 2D) • MUC1 (Mucin 1) • SMAD4 (SMAD family member 4) • MLL2 (Myeloid/lymphoid or mixed-lineage leukemia 2) • TGFBR2 (Transforming Growth Factor Beta Receptor 2) • MUC4 (Mucin 4, Cell Surface Associated) • CDX2 (Caudal Type Homeobox 2) • MUC2 (Mucin 2) • RSPO3 (R-Spondin 3) • CACNA1A (Calcium Voltage-Gated Channel Subunit Alpha1 A) • MUC17 (Mucin 17) • MUC5AC (Mucin 5AC) • MUC6 (Mucin 6) • PTPRK (Protein Tyrosine Phosphatase Receptor Type K) • TGFBR1 (Transforming Growth Factor Beta Receptor 1)
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    FGFR2 mutation • FGFR2 fusion • FGFR2 rearrangement
    8d
    A Case Report of Promising Response to Combination Therapy With an Immune Checkpoint Inhibitor (Pembrolizumab) and Multi-Targeted Tyrosine Kinase Inhibitor (Pazopanib) in Metastatic De-Differentiated Chondrosarcoma. (PubMed, Cancer Rep (Hoboken))
    This case report is a valuable example of promising emerging systemic therapies for advanced DDCS, where the present standard of care lacks a repertoire of effective therapies.
    Journal • Checkpoint inhibition • PD(L)-1 Biomarker • IO biomarker
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    IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • PTCH1 (Patched 1)
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    Keytruda (pembrolizumab) • pazopanib • Erivedge (vismodegib)
    9d
    Current Pharmacotherapeutic Strategies in Diffuse Gliomas: Focus on Glioblastoma, IDH-Wildtype, and Emerging Targeted Therapies for IDH-Mutant Tumors. (PubMed, Pharmaceuticals (Basel))
    This review focuses on contemporary pharmacotherapeutic approaches used in the management of glioblastoma, IDH-wildtype, including temozolomide-based chemotherapy, corticosteroids for edema control, and antiangiogenic therapy in recurrent disease, with particular emphasis on their clinical efficacy and limitations...Particular attention is given to ivosidenib, a selective inhibitor of mutant IDH1, currently evaluated for the treatment of astrocytoma, IDH-mutant, grade 4...Finally, innovative drug-delivery technologies designed to overcome the blood-brain barrier are briefly discussed as complementary strategies that may enhance the efficacy of both conventional and targeted therapies. Overall, future advances in the treatment of diffuse gliomas will likely depend on the integration of molecularly targeted agents, predictive biomarkers, and advanced delivery platforms aimed at improving patient survival and quality of life.
    Review • Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    IDH1 mutation • IDH wild-type
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    temozolomide • Tibsovo (ivosidenib)
    9d
    Evaluation of the Idylla IDH1-2 Mutation Assay for the Detection of IDH Variants in Solid Tumors and Hematological Malignancies. (PubMed, Int J Mol Sci)
    Based on these data, the Idylla IDH1-2 mutation assay represents a fast and reliable alternative to detect IDH hotspot variants in solid tumors and hematological malignancies using either fixed tissue sections or DNA extracts. Particular attention, however, is needed for the interpretation of cases with cycle of quantification values of the internal controls over 35, for which a variant with low allelic frequency could be missed due to low DNA quantity or quality.
    Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    IDH1 R132 • IDH2 R172
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    Idylla™ IDH1-2 Mutation Assay
    10d
    A Study of Enasidenib in People With Clonal Cytopenia of Undetermined Significance (clinicaltrials.gov)
    P1, N=4, Completed, Memorial Sloan Kettering Cancer Center | Active, not recruiting --> Completed | Trial completion date: Oct 2026 --> Jan 2026 | Trial primary completion date: Oct 2026 --> Jan 2026
    Trial completion • Trial completion date • Trial primary completion date
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    IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    IDH2 mutation • IDH2 R172
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    Idhifa (enasidenib)
    10d
    Age-dependent clinical, molecular, and prognostic differences in patients with AML: a retrospective study. (PubMed, Hematology)
    This study revealed that patients aged ≥50 years displayed more complex genetic aberration profiles and experienced significantly poorer prognoses compared to their younger counterparts. These findings provided novel insights for optimizing treatment strategies for middle-aged and elderly AML patients in the Chinese population.
    Retrospective data • Journal
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    TP53 (Tumor protein P53) • NRAS (Neuroblastoma RAS viral oncogene homolog) • KIT (KIT proto-oncogene, receptor tyrosine kinase) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • DNMT3A (DNA methyltransferase 1) • RUNX1 (RUNX Family Transcription Factor 1) • WT1 (WT1 Transcription Factor) • RUNX1T1 (RUNX1 Partner Transcriptional Co-Repressor 1) • CEBPA (CCAAT Enhancer Binding Protein Alpha)
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    TP53 mutation • KIT mutation • Chr del(5q) • CBFB-MYH11 fusion
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    Venclexta (venetoclax)