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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 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 glioblastoma multiforme predicted - sensitive IDH1 Inhibitor DS-1001b Preclinical - Pdx Actionable In a preclinical study, treatment with DS-1001b inhibited subcutaneous and intracranial tumor growth in a patient-derived xenograft (PDX) model of glioblastoma harboring IDH1 R132H, and also demonstrated reduced levels of the oncometabolite 2-hydroxyglutarate (2-HG) within tumors and plasma and increased expression of the astrocyte differentiation marker glial fibrillary acidic protein in tumor cells (PMID: 31727689). 31727689
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 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 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 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 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 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 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
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 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 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 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 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 malignant glioma not applicable N/A Guideline Diagnostic IDH1 R132H is diagnostic and aids in the diagnosis of gliomas (NCCN.org). 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 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 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 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 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 IDH1 Inhibitor DS-1001b Preclinical - Cell culture Actionable In a preclinical study, DS-1001b inhibited IDH1 R132H enzymatic activity in an in vitro assay, and inhibited production of the oncometabolite 2-hydroxyglutarate (2-HG) in transformed human cells expressing IDH1 R132H in culture (PMID: 31727689). 31727689
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 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 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 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 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 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 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 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 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 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
Clinical Trial Phase Therapies Title Recruitment Status
NCT02193347 Phase I Montanide ISA 51 + PEPIDH1M vaccine + Sargramostim + Temozolomide IDH1 Peptide Vaccine for Recurrent Grade II Glioma (RESIST) Active, not recruiting
NCT03343197 Phase I Ivosidenib AG-881 Study of AG-120 and AG-881 in Subjects With Low Grade Glioma Active, not recruiting
NCT04056910 Phase II Ivosidenib + Nivolumab Ivosidenib (AG-120) With Nivolumab in IDH1 Mutant Tumors Not yet 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
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
NCT02977780 Phase II CC-115 Abemaciclib + Temozolomide Temozolomide Neratinib + Temozolomide INdividualized Screening Trial of Innovative Glioblastoma Therapy (INSIGhT) Recruiting
NCT03970447 Phase II Lomustine Regorafenib Temozolomide A Trial to Evaluate Multiple Regimens in Newly Diagnosed and Recurrent Glioblastoma (GBM AGILE) 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
NCT02525692 Phase II ONC201 Oral ONC201 in Recurrent GBM, H3 K27M Glioma, and Midline Glioma Recruiting