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Profile Name IDH1 mutant
Gene Variant Detail

IDH1 mutant (unknown)

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 mutant high grade glioma not applicable N/A Guideline Prognostic IDH1 mutations are associated with a favorable prognosis in patients with glioma, and are associated with a survival benefit for patients treated with radiation or alkylator therapy (NCCN.org). detail...
IDH1 mutant high grade glioma not applicable N/A Clinical Study Prognostic In multiple clinical studies, including two meta-analyses, IDH1 mutations were associated with improved overall survival and progression free survival in patients with gliomas (PMID: 23817809, PMID: 26220714, PMID: 23894344). 23894344 23817809 26220714
IDH1 mutant high grade glioma not applicable N/A Guideline Diagnostic IDH1 mutations aid in the diagnosis of gliomas (PMID: 23041832, PMID: 19755387, PMID: 19915484; NCCN.org). detail... 23041832 19755387 19915484
IDH1 mutant acute myeloid leukemia predicted - sensitive LY3410738 Preclinical - Pdx & cell culture Actionable In a preclinical study, LY3410738 reversed mutant IDH1-induced differentiation block in patient-derived acute myeloid leukemia (AML) cells, inhibited 2-HG production, induced differentiation, and eliminated AML cells in patient-derived orthotopic animal models of IDH1-mutant AML (AACR 2019 Annual Meeting, Abstract LB-274). detail...
IDH1 mutant acute myeloid leukemia predicted - sensitive Daunorubicin + Talazoparib Preclinical - Patient cell culture Actionable In a preclinical study, the combination therapy of Talzenna (talazoparib) and Cerubidine (daunoruibicin) resulted in an additive effect in cells from a patient with acute myeloid leukemia harboring an IDH1 mutation, demonstrating decreased colony formation in culture (PMID: 29339439). 29339439
IDH1 mutant acute myeloid leukemia not applicable N/A Clinical Study Prognostic In two meta-analyses, IDH1 mutations were associated with a worse overall survival in patients with acute myeloid leukemia (PMID: 22616558, PMID: 23226625). 22616558 23226625
IDH1 mutant angioimmunoblastic T-cell lymphoma not applicable N/A Guideline Diagnostic IDH1 mutations aid in the diagnosis of angioimmunoblastic T-cell lymphoma (NCCN.org). detail...
IDH1 mutant oligodendroglioma not applicable N/A Guideline Diagnostic IDH1 mutations aid in the diagnosis of oligodendrogliomas (NCCN.org). detail...
IDH1 mutant acute myeloid leukemia predicted - sensitive Talazoparib Preclinical - Patient cell culture Actionable In a preclinical study, cells from a patient with acute myeloid leukemia harboring an IDH1 mutation were sensitive to treatment with Talzenna (talazoparib) in culture, demonstrating decreased colony formation (PMID: 29339439). 29339439
IDH1 mutant acute myeloid leukemia predicted - sensitive Daunorubicin + Olaparib Preclinical - Patient cell culture Actionable In a preclinical study, the combination therapy of Lynparza (olaparib) and Cerubidine (daunoruibicin) resulted in an additive effect in cells from a patient with acute myeloid leukemia harboring an IDH1 mutation, demonstrating decreased colony formation in culture (PMID: 29339439). 29339439
IDH1 mutant chondrosarcoma resistant Dasatinib Preclinical Actionable In a preclinical study, chondrosarcoma cells harboring IDH1 mutations were resistant to Sprycel (dasatinib) in culture (PMID: 27231123). 27231123
IDH1 mutant malignant astrocytoma not applicable N/A Guideline Diagnostic IDH1 mutations aid in the diagnosis of grade II and grade III astrocytomas (NCCN.org). detail...
IDH1 mutant acute myeloid leukemia sensitive Azacitidine + Venetoclax Guideline Actionable Venclexta (venetoclax) in combination with Vidaza (azacitidine) is included in guidelines for adult patients with acute myeloid leukemia harboring an IDH1 mutation (NCCN.org). detail...
IDH1 mutant myeloid neoplasm predicted - sensitive Ivosidenib + Venetoclax Phase Ib/II Actionable In a Phase I/II trial, Tibsovo (ivosidenib) and Venclexta (venetoclax) combination therapy demonstrated safety and preliminary efficacy in patients with advanced myeloid malignancies harboring IDH1 mutations, resulted in an overall response rate of 67% (4/6) and 100% (6/6) at 400 mg and 800 mg doses, respectively (EMJ Hematol. 2020;8(1):50-53; NCT03471260). detail...
IDH1 mutant myelofibrosis not applicable N/A Guideline Diagnostic IDH1 mutations aid in the diagnosis of primary myelofibrosis in the absence of JAK2, CALR, or MPL mutations (NCCN.org). detail...
IDH1 mutant myelofibrosis not applicable N/A Guideline Prognostic IDH1 mutations are associated with inferior leukemia-free survival in patients with myelofibrosis (NCCN.org). detail...
IDH1 mutant acute myeloid leukemia predicted - sensitive Venetoclax Phase II Actionable In a Phase II trial, 33% (4/12) of acute myeloid leukemia patients harboring either IDH1 or IDH2 mutations responded to treatment with Venclexta (venetoclax), demonstrating a complete response or complete response with incomplete blood count recovery (PMID: 27520294). 27520294
IDH1 mutant myeloid neoplasm predicted - sensitive Azacitidine + Ivosidenib + Venetoclax Phase Ib/II Actionable In a Phase I/II trial, Tibsovo (ivosidenib) in combination with Vidaza (azacitidine) and Venclexta (venetoclax) demonstrated safety and preliminary efficacy in patients with advanced myeloid malignancies harboring IDH1 mutations, resulted in an overall response rate of 100% (8/8) (EMJ Hematol. 2020;8(1):50-53; NCT03471260). detail...
IDH1 mutant cholangiocarcinoma sensitive Ivosidenib Guideline Actionable Tibsovo (ivosidenib) is included in guidelines as subsequent therapy for cholangiocarcinoma patients harboring IDH1 mutations (NCCN.org). detail...
IDH1 mutant cholangiocarcinoma sensitive Ivosidenib FDA approved - Has 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/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
IDH1 mutant cholangiocarcinoma sensitive Ivosidenib Phase I Actionable In a Phase I trial, AG-120 treatment resulted in partial response in 6% (4/72) and stable disease in 56% (40/72) of cholangiocarcinoma patients harboring IDH1 mutations (Journal of Clinical Oncology 35, no. 15_suppl (May 2017) 4015-4015; NCT02073994). detail...
IDH1 mutant acute myeloid leukemia sensitive Azacitidine Guideline Actionable Vidaza (azacitidine) is included in guidelines for adult patients with acute myeloid leukemia harboring an IDH1 mutation (NCCN.org). detail...
IDH1 mutant polycythemia vera not applicable N/A Guideline Prognostic IDH1 mutations are associated with inferior overall survival in patients with polycythemia vera (NCCN.org). detail...
IDH1 mutant anaplastic astrocytoma not applicable N/A Guideline Diagnostic IDH1 mutations aid in the diagnosis of grade III astrocytomas (NCCN.org). detail...
IDH1 mutant high grade glioma no benefit Durvalumab + Olaparib Phase II Actionable In a Phase II trial, combination therapy with Lynparza (olaparib) and Imfinzi (durvalumab) was well tolerated, but lacked antitumor activity in glioma patients with IDH1 (8/9) or IDH2 (1/9) mutations, and led to an objective response in one patient with glioblastoma, stable disease as per RECIST but clinical deterioration in two patients, and progressive disease in 6/9 (67%) patients, with a median progression free survival of 2.5 mo (J Clin Oncol 39, no. 15_suppl (May 20, 2021) abstr e14026); NCT03991832). detail...
IDH1 mutant acute myeloid leukemia sensitive Decitabine + Venetoclax Guideline Actionable Venclexta (venetoclax) in combination with Dacogen (decitabine) is included in guidelines for adult patients with acute myeloid leukemia harboring an IDH1 mutation (NCCN.org). detail...
IDH1 mutant acute myeloid leukemia sensitive Cytarabine + Venetoclax Guideline Actionable Venclexta (venetoclax) in combination with Cytosar-U (cytarabine) is included in guidelines for adult patients with acute myeloid leukemia harboring an IDH1 mutation (NCCN.org). detail...
IDH1 mutant acute myeloid leukemia sensitive Cytarabine + Venetoclax Phase Ib/II Actionable In a Phase I/II trial, Venclexta (venetoclax) in combination with low-dose cytarabine resulted in complete remission or complete remission with incomplete count recovery in 72% (13/18) of patients with acute myeloid leukemia harboring IDH1 or IDH2 mutations who were ineligible for intensive chemotherapy (ASH Annual Meeting, Dec 2018, Abstract 284; NCT02287233). detail...
IDH1 mutant glioblastoma not applicable N/A Clinical Study Prognostic In multiple clinical studies, including two meta-analyses, IDH1 mutations were associated with a greater overall survival and progression-free survival in patients with glioblastoma (PMID: 23904262, PMID: 26945349, PMID: 20560678). 20560678 23904262 26945349
IDH1 mutant glioblastoma not applicable N/A Guideline Diagnostic IDH1 mutations aid in the diagnosis of secondary grade IV glioblastomas (NCCN.org). detail...
IDH1 mutant high grade glioma predicted - sensitive AG-881 Phase I Actionable In a Phase I trial, Vorasidenib (AG-881) treatment resulted in an objective response in 13.6% (3/21, 1 partial response, 2 minor response) and stable disease in 77.3% (17/21) of patients with recurrent or progressive non-enhancing glioma harboring mutations in IDH1 (n=20) or IDH2 (n=1), with 60.5% of the patients remained progression-free and alive at 24 months (J Clin Oncol 38: 2020 (suppl; abstr 2504); NCT02481154). detail...
IDH1 mutant glioblastoma predicted - sensitive Bevacizumab + Lomustine Phase II Actionable In a retrospective analysis of a Phase II trial, IDH1 mutation correlated with favorable overall survival in recurrent glioblastoma patients treated with a combination of Avastin (bevacizumab) and Lomustine (PMID: 26762204). 26762204
IDH1 mutant acute myeloid leukemia predicted - sensitive Olaparib Preclinical - Patient cell culture Actionable In a preclinical study, cells from a patient with acute myeloid leukemia harboring an IDH1 mutation were sensitive to treatment with Lynparza (olaparib) in culture, demonstrating decreased colony formation (PMID: 29339439). 29339439
IDH1 mutant acute myeloid leukemia sensitive Ivosidenib Guideline Actionable Tibsovo (ivosidenib) is included in guidelines for patients with relapsed or refractory acute myeloid leukemia harboring an IDH1 mutation (NCCN.org). detail...
IDH1 mutant acute myeloid leukemia sensitive Ivosidenib FDA approved - Has 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... detail... 29860938
IDH1 mutant acute myeloid leukemia sensitive Decitabine Guideline Actionable Dacogen (decitabine) is included in guidelines for adult patients with acute myeloid leukemia harboring an IDH1 mutation (NCCN.org). detail...
IDH1 mutant lung adenocarcinoma resistant Dasatinib Preclinical Actionable In a preclinical study, lung adenocarcinoma cells harboring IDH1 mutations were resistant to Sprycel (dasatinib) in culture (PMID: 27231123). 27231123
Clinical Trial Phase Therapies Title Recruitment Status Covered Countries Other Countries
NCT03991832 Phase II Durvalumab + Olaparib Study of Olaparib and Durvalumab in IDH-Mutated Solid Tumors Recruiting CAN 0
NCT02632708 Phase I Enasidenib + Etoposide + Mitoxantrone Cytarabine + Enasidenib Cytarabine + Idarubicin + Ivosidenib Cytarabine + Daunorubicin + Enasidenib Cytarabine + Enasidenib + Idarubicin Etoposide + Ivosidenib + Mitoxantrone Cytarabine + Ivosidenib Cytarabine + Daunorubicin + Ivosidenib Safety Study of AG-120 or AG-221 in Combination With Induction and Consolidation Therapy in Participants With Newly Diagnosed Acute Myeloid Leukemia (AML) With an IDH1 and/or IDH2 Mutation Active, not recruiting USA 2
NCT04195555 Phase II Ivosidenib Ivosidenib in Treating Patients With Advanced Solid Tumors, Lymphoma, or Histiocytic Disorders With IDH1 Mutations (A Pediatric MATCH Treatment Trial) Recruiting USA 1
NCT04576117 Phase III Selumetinib Selumetinib + Vinblastine A Study to Compare Treatment With the Drug Selumetinib Alone Versus Selumetinib and Vinblastine in Patients With Recurrent or Progressive Low-Grade Glioma Recruiting USA 0
NCT03430791 Phase II Ipilimumab + Nivolumab Nivolumab Trial of Combination TTF(Optune), Nivolumab Plus/Minus Ipilimumab for Recurrent Glioblastoma Terminated USA 0
NCT02576444 Phase II Adavosertib + Olaparib Olaparib Capivasertib + Olaparib Ceralasertib + Olaparib OLAParib COmbinations (OLAPCO) Active, not recruiting USA 0
NCT02481154 Phase I AG-881 Study of Orally Administered AG-881 in Patients With Advanced Solid Tumors, Including Gliomas, With an IDH1 and/or IDH2 Mutation Active, not recruiting USA 0
NCT03127735 Phase I BAY1436032 BAY1436032 in Patients With Mutant IDH1(mIDH1) Advanced Acute Myeloid Leukemia (AML) Completed USA 1
NCT03557359 Phase II Nivolumab Nivolumab for Recurrent or Progressive IDH Mutant Gliomas Unknown status USA 0
NCT02968940 Phase II Avelumab Avelumab With Hypofractionated Radiation Therapy in Adults With Isocitrate Dehydrogenase (IDH) Mutant Glioblastoma Completed USA 0
NCT03047993 Phase Ib/II Azacitidine + Telaglenastat Glutaminase Inhibitor CB-839 and Azacitidine in Treating Patients With Advanced Myelodysplastic Syndrome Recruiting USA 0
NCT02525692 Phase II ONC201 Oral ONC201 in Recurrent GBM, H3 K27M Glioma, and Midline Glioma Recruiting USA 0
NCT02428855 Phase II Dasatinib Phase II Trial of Dasatinib in Patients With Isocitrate Dehydrogenase (IDH)-Mutant Advanced Intrahepatic Cholangiocarcinoma Completed USA 0
NCT03139916 Phase II Bavituximab + Temozolomide Bavituximab With Radiation and Temozolomide for Patients With Newly Diagnosed Glioblastoma Active, not recruiting USA 0
NCT04555577 Phase I MSC2490484A Temozolomide Nedisertib and Radiation Therapy, Followed by Temozolomide for the Treatment of Patients With Newly Diagnosed MGMT Unmethylated Glioblastoma or Gliosarcoma Recruiting USA 0
NCT02492737 Phase I AG-881 Study of Orally Administered AG-881 in Patients With Advanced Hematologic Malignancies With an IDH1 and/or IDH2 Mutation Completed USA 1
NCT02989857 Phase III Ivosidenib Study of AG-120 in Previously Treated Advanced Cholangiocarcinoma With IDH1 Mutations (ClarIDHy) Completed USA 5
NCT02677922 Phase Ib/II Azacitidine + Ivosidenib Azacitidine Azacitidine + Enasidenib A Safety and Efficacy Study of Oral AG-120 Plus Subcutaneous Azacitidine and Oral AG-221 Plus Subcutaneous Azacitidine in Subjects With Newly Diagnosed Acute Myeloid Leukemia (AML) Active, not recruiting USA | CAN 10
NCT05052957 Phase II Carmustine + Filgrastim + O6-benzylguanine + Temozolomide hSTAR GBM (Hematopoetic Stem Cell (HPC) Rescue for GBM) (hSTAR GBM) Not yet recruiting USA 0
NCT04729959 Phase II Atezolizumab + Tocilizumab Testing the Addition of the Immune Therapy Drugs, Tocilizumab and Atezolizumab, to Radiation Therapy for Recurrent Glioblastoma Recruiting USA 0
NCT03749187 Phase I Pamiparib + Temozolomide BGB-290 and Temozolomide in Treating Isocitrate Dehydrogenase (IDH)1/2-Mutant Grade I-IV Gliomas (PNOC017) Recruiting USA | CAN 0
NCT03922555 Phase I Decitabine and Cedazuridine ASTX727 in Recurrent/Progressive Non-enhancing IDH Mutant Gliomas Recruiting USA 0
NCT04774393 Phase Ib/II Decitabine and Cedazuridine + Ivosidenib + Venetoclax Decitabine and Cedazuridine + Enasidenib + Venetoclax Decitabine/Cedazuridine and Venetoclax in Combination With Ivosidenib or Enasidenib for the Treatment of Relapsed or Refractory Acute Myeloid Leukemia Recruiting USA 0
NCT04606316 Phase I Nivolumab Ipilimumab + Nivolumab Surgical Nivolumab And Ipilimumab For Recurrent GBM Recruiting USA 0
NCT03158389 Phase Ib/II Vismodegib Alectinib Idasanutlin Temsirolimus Atezolizumab Palbociclib APG101 NCT Neuro Master Match - N²M² (NOA-20) (N²M²) Recruiting 1
NCT04118036 Phase II Abemaciclib + Pembrolizumab Abemaciclib + Pembrolizumab In Glioblastoma Suspended USA 0
NCT04945148 Phase II Metformin + Temozolomide Oxidative Phosphorylation Targeting In Malignant Glioma Using Metformin Plus Radiotherapy Temozolomide (OPTIMUM) Not yet recruiting 2
NCT02073994 Phase I Ivosidenib Study of Orally Administered AG-120 in Subjects With Advanced Solid Tumors, Including Glioma, With an IDH1 Mutation Active, not recruiting USA 1
NCT03207347 Phase II Niraparib A Trial of Niraparib in BAP1 and Other DNA Damage Response (DDR) Deficient Neoplasms (UF-STO-ETI-001) Suspended USA 0
NCT04015700 Phase I GNOS-PV01 + INO-9012 Neoantigen-based Personalized DNA Vaccine in Patients With Newly Diagnosed, Unmethylated Glioblastoma Recruiting USA 0
NCT02071862 Phase I Everolimus + Telaglenastat Erlotinib + Telaglenastat Docetaxel + Telaglenastat Telaglenastat Paclitaxel + Telaglenastat Cabozantinib + Telaglenastat Study of the Glutaminase Inhibitor CB-839 in Solid Tumors Completed USA 0
NCT03701295 Phase Ib/II Azacitidine + Pinometostat Pinometostat and Azacitidine in Treating Patients With Relapsed, Refractory, or Newly Diagnosed Acute Myeloid Leukemia With 11q23 Rearrangement Completed USA 0
NCT04955938 Phase I Enasidenib + Fedratinib Fedratinib + Ivosidenib A Study of Fedratinib With IDH Inhibition in Advanced-Phase, IDH-Mutated Ph-Negative Myeloproliferative Neoplasms Not yet recruiting USA 0
NCT04396860 Phase II Temozolomide Ipilimumab + Nivolumab Testing the Use of the Immunotherapy Drugs Ipilimumab and Nivolumab Plus Radiation Therapy Compared to the Usual Treatment (Temozolomide and Radiation Therapy) for Newly Diagnosed MGMT Unmethylated Glioblastoma Recruiting USA 0
NCT03503409 Phase II Ivosidenib IDH1 (AG 120) Inhibitor in Patients With IDH1 Mutated Myelodysplastic Syndrome Recruiting 2
NCT04629209 Phase II ONC201 A Phase II, Open Label Study of ONC201 in Adults With EGFR-low Glioblastoma Withdrawn USA 0
NCT04278781 Phase II Ivosidenib AG-120 in People With IDH1 Mutant Chondrosarcoma Recruiting USA 0
NCT03212274 Phase II Olaparib Olaparib in Treating Patients With Advanced Glioma, Cholangiocarcinoma, or Solid Tumors With IDH1 or IDH2 Mutations Recruiting USA 0
NCT04762602 Phase I HMPL-306 A Study of HMPL-306 in Advanced Solid Tumors With IDH Mutations Recruiting USA 0
NCT03220646 Phase II Abemaciclib The Purpose of This Study is to Test Any Good and Bad Effects of a Study Drug Called Abemaciclib (LY2835219) in Patients With Recurrent Brain Tumors. Recruiting USA 0
NCT03718767 Phase II Nivolumab Nivolumab in Patients With IDH-Mutant Gliomas With and Without Hypermutator Phenotype Recruiting USA 0
NCT02858895 Phase II MDNA55 Convection-Enhanced Delivery (CED) of MDNA55 in Adults With Recurrent or Progressive Glioblastoma Completed USA 0
NCT03173248 Phase III Azacitidine Azacitidine + Ivosidenib Study of AG-120 (Ivosidenib) vs. Placebo in Combination With Azacitidine in Patients With Previously Untreated Acute Myeloid Leukemia With an IDH1 Mutation (AGILE) Active, not recruiting CAN 18
NCT04764474 Phase I HMPL-306 A Study of HMPL-306 in Advanced Hematological Malignancies With mIDH Recruiting USA 0
NCT02981940 Phase II Abemaciclib A Study of Abemaciclib in Recurrent Glioblastoma Recruiting USA 0
NCT03878095 Phase II Ceralasertib + Olaparib Testing Olaparib and AZD6738 in IDH1 and IDH2 Mutant Tumors Recruiting USA 0