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Molecular Profile Indication/Tumor Type Response Type Relevant Treatment Approaches Therapy Name Approval Status Evidence Type Efficacy Evidence References
IDH1 R132H glioblastoma multiforme resistant Vorinostat Preclinical - Cell culture Actionable In a preclinical study, glioblastoma cells expressing IDH1 R132H were resistant to treatment with Zolinza (vorinostat) in culture, demonstrating increased cell viability (PMID: 31151327). 31151327
IDH1 R132H colon carcinoma sensitive Olaparib Preclinical - Cell culture Actionable In a preclinical study, colon carcinoma cells over expressing IDH1 R132H demonstrated increased sensitivity to Lynparza (olaparib)-induced growth inhibition in culture (PMID: 28148839). 28148839
IDH1 R132H Advanced Solid Tumor sensitive Cisplatin + Talazoparib Preclinical - Cell culture Actionable In a preclinical study, Talazoparib (BMN-673) and Cisplatin synergistically inhibited growth of transformed cells over expressing IDH1 R132H in culture (PMID: 28148839). 28148839
IDH1 R132H 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). 29860938 detail... detail...
IDH1 R132H 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). 29860938 detail... detail...
IDH1 R132H colon carcinoma sensitive Talazoparib Preclinical - Cell culture Actionable In a preclinical study, colon carcinoma cells over expressing IDH1 R132H demonstrated increased sensitivity to Talazoparib (BMN-673)-induced growth inhibition in culture (PMID: 28148839). 28148839
IDH1 R132H malignant glioma sensitive Talazoparib Preclinical - Patient cell culture Actionable In a preclinical study, patient-derived glioma cells harboring IDH1 R132H demonstrated increased sensitivity to Talazoparib (BMN-673)-induced growth inhibition in culture (PMID: 28148839). 28148839
IDH1 R132H Advanced Solid Tumor sensitive Talazoparib Preclinical - Cell culture Actionable In a preclinical study, transformed cells over expressing IDH1 R132H demonstrated increased sensitivity to Talazoparib (BMN-673)-induced growth inhibition in culture (PMID: 28148839). 28148839
IDH1 R132H colorectal cancer resistant IDH1 Inhibitor AGI-5198 + Metformin Preclinical Actionable In a preclinical study, colorectal cancer cells harboring IDH1 R132H demonstrated increased cell proliferation when treated with a combination of Glucophage (metformin) and AGI-5198 (PMID: 26363012). 26363012
IDH1 R132H glioblastoma multiforme sensitive Temozolomide + Vandetanib Phase II Actionable In a Phase II trial, Caprelsa (vandetanib), in combination with Temodar (temozolomide) and radiation therapy, demonstrated a significant increase in PFS and OS in glioblastoma patients harboring IDH1 R132H compared to glioblastoma patients without IDH1 R132H (PMID: 25910950). 25910950
IDH1 R132H Advanced Solid Tumor sensitive Niraparib Preclinical - Cell culture Actionable In a preclinical study, transformed cells over expressing IDH1 R132H demonstrated increased sensitivity to Zejula (niraparib)-induced growth inhibition in culture (PMID: 28148839). 28148839
IDH1 R132H Advanced Solid Tumor decreased response IDH1 Inhibitor AGI-5198 + Talazoparib Preclinical - Cell culture Actionable In a preclinical study, AGI-5198 reverted the sensitivity of transformed cells over expressing IDH1 R132H to Talazoparib (BMN-673)-induced growth inhibition in culture (PMID: 28148839). 28148839
IDH1 R132H colorectal cancer predicted - sensitive Olaparib Preclinical - Cell culture Actionable In a preclinical study, colorectal cancer cells harboring IDH1 R132H were sensitive to treatment with Lynparza (olaparib) in culture, demonstrating decreased colony formation (PMID: 29339439). 29339439
IDH1 R132H colorectal cancer sensitive Metformin Preclinical Actionable In a preclinical study, colorectal carcinoma cells expressing IDH1 R132H were sensitive to Glucophage (metformin), resulting in decreased cell proliferation in culture (PMID: 26363012). 26363012
IDH1 R132H Advanced Solid Tumor sensitive Rucaparib Preclinical - Cell culture Actionable In a preclinical study, transformed cells over expressing IDH1 R132H demonstrated increased sensitivity to Rubraca (rucaparib)-induced growth inhibition in culture (PMID: 28148839). 28148839
IDH1 R132H Advanced Solid Tumor sensitive Olaparib Preclinical - Cell culture Actionable In a preclinical study, transformed cells over expressing IDH1 R132H demonstrated increased sensitivity to Lynparza (olaparib)-induced growth inhibition in culture (PMID: 28148839). 28148839
IDH1 R132H colorectal cancer predicted - sensitive Talazoparib Preclinical - Cell culture Actionable In a preclinical study, colorectal cancer cells harboring IDH1 R132H were sensitive to treatment with Talzenna (talazoparib) in culture, demonstrating decreased colony formation (PMID: 29339439). 29339439
IDH1 R132H 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 inhibited growth and increased differentiation of patient-derived acute myeloid leukemia cells harboring IDH1 R132H in culture (PMID: 28232670). 28232670
IDH1 R132H glioblastoma multiforme predicted - sensitive IDH1 Inhibitor AGI-5198 + Trichostatin A Preclinical - Cell culture Actionable In a preclinical study, the addition of AGI-5198 treatment in glioblastoma cells expressing IDH1 R132H led to decreased resistance to Trichostatin (TSA) treatment in culture, resulting in reduced cell viability (PMID: 31151327). 31151327
IDH1 R132H colorectal cancer resistant IDH1 Inhibitor AGI-5198 + Radiotherapy Preclinical Actionable In a preclinical study, colorectal cancer cells expressing IDH1 R132H were resistant to radiotherapy when treated with AGI-5198 (PMID: 26363012). 26363012
IDH1 R132H glioblastoma multiforme predicted - sensitive IDH1 Inhibitor AGI-5198 + Vorinostat Preclinical - Cell culture Actionable In a preclinical study, the addition of AGI-5198 treatment in glioblastoma cells expressing IDH1 R132H led to decreased resistance to Zolinza (vorinostat) treatment in culture, resulting in reduced cell viability (PMID: 31151327). 31151327
IDH1 R132H malignant glioma not applicable N/A Guideline Diagnostic IDH1 R132H is diagnostic and aids in the diagnosis of gliomas (NCCN.org). detail...
IDH1 R132H malignant glioma predicted - sensitive IDH Inhibitor (Pan) AG-881 Preclinical - Cell line xenograft Actionable In a preclinical study, AG-881 treatment decreased brain tumor 2HG levels in an orthotopic glioma cell line xenograft model harboring IDH1 R132H (Mol Cancer Ther Jan 1 2018 (17) (1 Supp) B126). detail...
IDH1 R132H glioblastoma multiforme resistant Trichostatin A Preclinical - Cell culture Actionable In a preclinical study, glioblastoma cells expressing IDH1 R132H were resistant to treatment with Trichostatin (TSA) in culture, demonstrating decreased apoptotic activity and increased cell viability (PMID: 31151327). 31151327
IDH1 R132H Advanced Solid Tumor sensitive Berzosertib Preclinical - Cell culture Actionable In a preclinical study, transformed cells expressing IDH1 R132H demonstrated increased sensitivity to Berzosertib (VX-970)-induced growth inhibition in culture (PMID: 28148839). 28148839
IDH1 R132H malignant glioma sensitive IDH1 Inhibitor AGI-5198 Preclinical - Cell line xenograft Actionable In a preclinical study, AGI-5198 inhibited growth of a glioma cell line harboring IDH1 R132H in culture and in xenograft models (PMID: 23558169). 23558169
IDH1 R132H malignant glioma sensitive BPTES Preclinical - Cell culture Actionable In a preclinical study, a glioma cell line expressing IDH1 R132H demonstrated increased sensitivity to growth inhibition by BPTES compared to a cell line expressing wild-type IDH1 in culture (PMID: 21045145). 21045145
IDH1 R132H glioblastoma multiforme resistant Valproic acid Preclinical - Cell culture Actionable In a preclinical study, glioblastoma cells expressing IDH1 R132H were resistant to treatment with Valproic Acid in culture, demonstrating increased cell viability (PMID: 31151327). 31151327
IDH1 R132H oligodendroglioma sensitive Decitabine Preclinical - Pdx & cell culture Actionable In a preclinical study, Dacogen (decitabine) decreased colony formation and tumor growth in patient derived xenograft (PDX) models of patient derived oligodendroglioma cells with IDH1 R132H mutations and co-deletion of 1p and 19q (PMID: 24077826). 24077826
IDH1 R132H grade III astrocytoma sensitive Azacitidine Preclinical Actionable In a preclinical study, long-term treatment with Vidaza (azacitidine) resulted in increased cellular differentiation, decreased proliferation, and tumor regression in a patient-derived xenograft (PDX) model of anaplastic astrocytoma harboring IDH1 R132H (PMID: 24077805). 24077805
Clinical Trial Phase Therapies Title Recruitment Status
NCT02977780 Phase II CC-115 Abemaciclib + Temozolomide Temozolomide Neratinib + Temozolomide INdividualized Screening Trial of Innovative Glioblastoma Therapy (INSIGhT) 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
NCT04088188 Phase I Cisplatin + Gemcitabine + Pemigatinib Cisplatin + Gemcitabine + Ivosidenib Gemcitabine and Cisplatin With Ivosidenib or Pemigatinib for the Treatment of Unresectable or Metastatic Cholangiocarcinoma Not yet recruiting
NCT03970447 Phase II Lomustine Regorafenib Temozolomide A Trial to Evaluate Multiple Regimens in Newly Diagnosed and Recurrent Glioblastoma (GBM AGILE) Recruiting
NCT02193347 Phase I Montanide ISA 51 + PEPIDH1M vaccine + Sargramostim + Temozolomide IDH1 Peptide Vaccine for Recurrent Grade II Glioma (RESIST) Active, not recruiting
NCT02525692 Phase II ONC201 Oral ONC201 in Recurrent GBM, H3 K27M Glioma, and Midline Glioma Recruiting
NCT04056910 Phase II Ivosidenib + Nivolumab Ivosidenib (AG-120) With Nivolumab in IDH1 Mutant Tumors Not yet recruiting
NCT03343197 Phase I Ivosidenib AG-881 Study of AG-120 and AG-881 in Subjects With Low Grade Glioma Active, not recruiting