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Gene | IDH1 |
Variant | act mut |
Impact List | unknown |
Protein Effect | gain of function |
Gene Variant Descriptions | IDH1 act mut indicates that this variant results in a gain of function in the Idh1 protein. However, the specific amino acid change has not been identified. |
Associated Drug Resistance | |
Category Variants Paths |
IDH1 mutant IDH1 act mut |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
IDH1 act mut | malignant astrocytoma | sensitive | Ivosidenib | Guideline | Actionable | Tibsovo (ivosidenib) is included in guidelines for patients with astrocytoma harboring an IDH1 activating mutation (NCCN.org). | detail... |
IDH1 act mut | high grade glioma | predicted - sensitive | Ivosidenib | Clinical Study | Actionable | In a retrospective analysis, Tibsovo (ivosidenib) treatment was well tolerated in patients with glioma harboring an IDH1 mutation, and resulted in a partial response in 13.6% (3/22), minor response in 22.7% (5/22), and stable disease in 54.5% (12/22) of patients with nonenhancing disease, and stable disease in 62.5% (5/8) of patients with enhancing disease (PMID: 38496920). | 38496920 |
IDH1 act mut | oligodendroglioma | sensitive | Ivosidenib | Guideline | Actionable | Tibsovo (ivosidenib) is included in guidelines for patients with oligodendroglioma harboring an IDH1 activating mutation (NCCN.org). | detail... |
IDH1 act mut | chondrosarcoma | sensitive | Ivosidenib | Guideline | Actionable | Tibsovo (ivosidenib) is included in guidelines as systemic therapy for patients with conventional or dedifferentiated chondrosarcoma harboring an IDH1 activating mutation (NCCN.org). | detail... |
IDH1 act mut | low grade glioma | predicted - sensitive | Ivosidenib | Clinical Study | Actionable | In a retrospective analysis, Tibsovo (ivosidenib) treatment was well tolerated in patients with glioma harboring an IDH1 mutation, and resulted in a partial response in 13.6% (3/22), minor response in 22.7% (5/22), and stable disease in 54.5% (12/22) of patients with nonenhancing disease, and stable disease in 62.5% (5/8) of patients with enhancing disease (PMID: 38496920). | 38496920 |
IDH1 act mut | oligodendroglioma | sensitive | Vorasidenib | Guideline | Actionable | Voranigo (vorasidenib) is included in guidelines as adjuvant therapy for patients with WHO grade 2 1p19q codeleted oligodendroglioma harboring an IDH1 activating mutation (NCCN.org). | detail... |
IDH1 act mut | astrocytoma, IDH-mutant, grade 2 | sensitive | Vorasidenib | Guideline | Actionable | Voranigo (vorasidenib) is included in guidelines as adjuvant therapy for patients with WHO grade 2 astrocytoma harboring an IDH1 activating mutation (NCCN.org). | detail... |
IDH1 act mut | chondrosarcoma | predicted - sensitive | Olutasidenib | Phase Ib/II | Actionable | In a Phase I/II trial, Rezlidhia (olutasidenib) treatment demonstrated acceptable safety and preliminary clinical activity in patients with IDH1-mutant intrahepatic cholangiocarcinoma, and led to stable disease in 31% (4/13) of evaluable patients (J Clin Oncol 38, no. 5_suppl (May 28, 2020); NCT03684811). | detail... |
IDH1 act mut | intrahepatic cholangiocarcinoma | predicted - sensitive | Olutasidenib | Phase Ib/II | Actionable | In a Phase I/II trial, Rezlidhia (olutasidenib) treatment demonstrated acceptable safety and preliminary clinical activity in patients with IDH1-mutant intrahepatic cholangiocarcinoma, and led to stable disease in 23% (6/23) of the evaluable patients (J Clin Oncol 38, no. 5_suppl (May 28, 2020); NCT03684811). | detail... |
IDH1 act mut | cholangiocarcinoma | predicted - sensitive | LY3410738 | Phase I | Actionable | In a Phase I trial, LY3410738 treatment demonstrated safety and resulted in D-2-HG inhibition in patients with IDH-mutant advanced solid tumors, and led to partial response in 1 and stable disease in 22 of 42 patients with relapsed or refractory cholangiocarcinoma and partial response in 3 and stable disease in 9 of 22 patients with glioma (Cancer Res (2023) 83 (8_Supplement): CT098; NCT04521686). | detail... |
IDH1 act mut | brain glioma | predicted - sensitive | LY3410738 | Phase I | Actionable | In a Phase I trial, LY3410738 treatment demonstrated safety and resulted in D-2-HG inhibition in patients with IDH-mutant advanced solid tumors, and led to partial response in 1 and stable disease in 22 of 42 patients with relapsed or refractory cholangiocarcinoma and partial response in 3 and stable disease in 9 of 22 patients with glioma (Cancer Res (2023) 83 (8_Supplement): CT098; NCT04521686). | detail... |