Molecular Profile Detail

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Profile Name IDH1 act mut
Gene Variant Detail

IDH1 act mut (gain of function)

Relevant Treatment Approaches

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Molecular Profile Indication/Tumor Type Response Type Relevant Treatment Approaches Therapy Name Approval Status Evidence Type Efficacy Evidence References
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 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 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 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...
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 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 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 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 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