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Profile Name | IDH1 R132G |
Gene Variant Detail | |
Relevant Treatment Approaches | IDH Inhibitor (Pan) IDH1 Inhibitor |
Molecular Profile | Indication/Tumor Type | Response Type | Relevant Treatment Approaches | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|---|
IDH1 R132G | acute myeloid leukemia | sensitive | IDH1 Inhibitor | Ivosidenib | FDA approved - On Companion Diagnostic | Actionable | In a Phase I trial that supported FDA approval, Tibsovo (ivosidenib) treatment resulted in complete remission (CR) in 21.6% (27/125), CR with partial hematological recovery (CRh) in 8.8% (11/125), and overall response (OR) in 41.6% (52/125) of patients with relapsed or refractory acute myeloid leukemia harboring a susceptible IDH1 mutation (R132C/G/H/L/S) as detected by an FDA-approved test (PMID: 29860938; NCT02074839). | detail... 29860938 detail... |
IDH1 R132G | acute myeloid leukemia | sensitive | IDH1 Inhibitor | Ivosidenib | FDA approved - On Companion Diagnostic | Actionable | In a Phase I trial that supported FDA approval, Tibsovo (ivosidenib) treatment resulted in complete remission (CR) in 28.6% (8/28), CR with partial hematological recovery (CRh) in 14.3% (4/28) of patients age 75 or older with untreated acute myeloid leukemia harboring a susceptible IDH1 mutation (R132C/G/H/L/S) as detected by an FDA-approved test, with a median treatment duration of 4.3 months (PMID: 29860938; NCT02074839). | detail... 29860938 detail... |
IDH1 R132G | Advanced Solid Tumor | predicted - sensitive | IDH1 Inhibitor | DS-1001b | Preclinical - Cell culture | Actionable | In a preclinical study, transformed human cells expressing IDH1 R132G demonstrated sensitivity to DS-1001b in culture, resulting in reduced production of the oncometabolite 2-hydroxyglutarate (2-HG) (PMID: 31727689). | 31727689 |
IDH1 R132G | cholangiocarcinoma | predicted - sensitive | IDH1 Inhibitor | Ivosidenib | FDA approved - On Companion Diagnostic | Actionable | In a Phase III (ClarIDHy) trial that supported FDA approval, Tibsovo (ivosidenib) treatment significantly improved median progression-free survival (2.7 vs 1.4 mo, HR=0.37, p<0.001) and prolonged median overall survival (10.8 vs 9.7 mo, HR=0.69, p=0.06) compared to placebo in patients with advanced cholangiocarcinoma harboring IDH1 mutations including R132C/H/L/G/S, resulted in favorable objective response rate (2%, 3/124 vs 0%, 0/61) and stable disease rate (51% vs 28%) (PMID: 32416072; NCT02989857). | detail... 32416072 detail... |
IDH1 R132G | acute myeloid leukemia | sensitive | IDH1 Inhibitor | Azacitidine + Ivosidenib | FDA approved - On Companion Diagnostic | Actionable | In a Phase III trial (AGILE) that supported FDA approval, Tibsovo (ivosidenib) and Vidaza (azacytidine) combination therapy significantly improved event-free survival (HR 0.33, p=0.002) and median overall survival (24.0 vs 7.9 mo, HR 0.44, p=0.001) compared to Vidaza (azacytidine) plus placebo in patients with newly diagnosed acute myeloid leukemia harboring IDH1 mutations including R132C/H/G/L/S (PMID: 35443108; NCT03173248). | 35443108 detail... detail... |
IDH1 R132G | chondrosarcoma | predicted - sensitive | IDH1 Inhibitor | Ivosidenib | Case Reports/Case Series | Actionable | In a Phase I trial, Tibsovo (ivosidenib) treatment was tolerated, substantially decreased plasma 2-HG levels, and resulted in a median progression-free survival of 5.6 months and stable disease in 52% (11/21) of patients with chondrosarcoma harboring IDH1 mutations, including IDH1 R132G (n=3) (PMID: 32208957; NCT02073994). | 32208957 |
IDH1 R132G | acute myeloid leukemia | sensitive | IDH1 Inhibitor | BAY1436032 | Preclinical - Patient cell culture | Actionable | In a preclinical study, BAY1436032 decreased (R)-2-hydroxyglutarate (R-2HG) levels and increased differentiation of patient-derived acute myeloid leukemia cells harboring IDH1 R132G in culture (PMID: 28232670). | 28232670 |
Clinical Trial | Phase | Therapies | Title | Recruitment Status | Covered Countries | Other Countries |
---|---|---|---|---|---|---|
NCT04056910 | Phase II | Ivosidenib + Nivolumab | Ivosidenib (AG-120) With Nivolumab in IDH1 Mutant Tumors | Recruiting | USA | 0 |
NCT03953898 | Phase II | Olaparib | Using the Anticancer Drug Olaparib to Treat Relapsed/Refractory Acute Myeloid Leukemia or Myelodysplastic Syndrome With an Isocitrate Dehydrogenase (IDH) Mutation | Recruiting | USA | 0 |
NCT04164901 | Phase III | AG-881 | Study of Vorasidenib (AG-881) in Participants With Residual or Recurrent Grade 2 Glioma With an IDH1 or IDH2 Mutation (INDIGO) | Active, not recruiting | USA | ITA | FRA | ESP | DEU | CAN | 4 |
NCT04088188 | Phase I | Cisplatin + Gemcitabine + Pemigatinib Cisplatin + Gemcitabine + Ivosidenib | Gemcitabine and Cisplatin With Ivosidenib or Pemigatinib for the Treatment of Unresectable or Metastatic Cholangiocarcinoma | Recruiting | USA | 0 |