Molecular Profile Detail

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Profile Name IDH1 R132G
Gene Variant Detail

IDH1 R132G (gain of function)

Relevant Treatment Approaches IDH Inhibitor (Pan) IDH1 Inhibitor

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Molecular Profile Indication/Tumor Type Response Type Relevant Treatment Approaches Therapy Name Approval Status Evidence Type Efficacy Evidence References
IDH1 R132G cholangiocarcinoma predicted - sensitive IDH1 Inhibitor Ivosidenib Phase III Actionable In a Phase III (ClarIDHy) trial, 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/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). 32416072
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
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 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 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
Clinical Trial Phase Therapies Title Recruitment Status Covered Countries Other Countries
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
NCT04056910 Phase II Ivosidenib + Nivolumab Ivosidenib (AG-120) With Nivolumab in IDH1 Mutant Tumors Not yet recruiting
NCT04164901 Phase III AG-881 Study of AG-881 in Participants With Residual or Recurrent Grade 2 Glioma With an IDH1 or IDH2 Mutation (INDIGO) Recruiting
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