IDH1 Mutation

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Molecular profile	Therapy	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
Acute Myeloid Leukemia4
IDH1 Mutation	Ivosidenib	Predictive	A	Supports Sensitivity Response	3	accepted	EID7278 I phase 1 trial for patients with IDH1-mutated AML, patients who received ivosidenib (AG-120). The rate of complete remission was 21.6%, the over all response rate was 30.4%. PMID 29860938 · DiNardo et al., 2018 · Open in CIViC	civic
IDH1 Mutation	Azacitidine + IvosidenibCombination	Predictive	A	Supports Sensitivity Response	5	submitted	EID10313 Phase 3 trial of 146 patients randomized to either IDH1 inhibitor ivosidenib and azacitidine or azacitidine and placebo revealed significantly longer event-free survival in the ivosidenib-group. Patie… (full text at CIViC) PMID 35443108 · Montesinos et al., 2022 · Open in CIViC

Molecular profile

Therapy

Type

Level

Direction · significance

Rating (1–5)

Status

Evidence

Source

Acute Myeloid Leukemia4

IDH1 Mutation

Ivosidenib

Predictive

Supports Sensitivity Response

accepted

EID7278

I phase 1 trial for patients with IDH1-mutated AML, patients who received ivosidenib (AG-120). The rate of complete remission was 21.6%, the over all response rate was 30.4%.

PMID 29860938 · DiNardo et al., 2018 · Open in CIViC

civic

IDH1 Mutation

Azacitidine + IvosidenibCombination

Predictive

Supports Sensitivity Response

submitted

EID10313

Phase 3 trial of 146 patients randomized to either IDH1 inhibitor ivosidenib and azacitidine or azacitidine and placebo revealed significantly longer event-free survival in the ivosidenib-group. Patie… (full text at CIViC)

PMID 35443108 · Montesinos et al., 2022 · Open in CIViC

Cholangiocarcinoma1
IDH1 Mutation	Ivosidenib	Predictive	B	Supports Sensitivity Response	3	accepted	EID7447 In a phase1 trial, 73 patients with cholangiocarcinoma harboring IDH1 mutations were treated with mutant IDH1 inhibitor ivosidenib. The response rate was 5%, median progression free survival was 3.8 m… (full text at CIViC) PMID 31300360 · Lowery et al., 2019 · Open in CIViC	civic

Cholangiocarcinoma1

IDH1 Mutation

Ivosidenib

Predictive

Supports Sensitivity Response

accepted

EID7447

In a phase1 trial, 73 patients with cholangiocarcinoma harboring IDH1 mutations were treated with mutant IDH1 inhibitor ivosidenib. The response rate was 5%, median progression free survival was 3.8 m… (full text at CIViC)

PMID 31300360 · Lowery et al., 2019 · Open in CIViC

civic

Chondrosarcoma1
IDH1 Mutation	(prognostic)	Prognostic	B	Supports Better Outcome	4	accepted	EID7725 In a retrospective study, the authors evaluated the association between IDH1/IDH2 mutation and prognosis in patients with high-grade central chondrosarcoma. Although no association was discovered betw… (full text at CIViC) PMID 31615936 · Zhu et al., 2020 · Open in CIViC	civic

Chondrosarcoma1

IDH1 Mutation

(prognostic)

Prognostic

Supports Better Outcome

accepted

EID7725

In a retrospective study, the authors evaluated the association between IDH1/IDH2 mutation and prognosis in patients with high-grade central chondrosarcoma. Although no association was discovered betw… (full text at CIViC)

PMID 31615936 · Zhu et al., 2020 · Open in CIViC

civic

Glioma1
IDH2 Mutation OR IDH1 Mutation	Vorasidenib	Predictive	A	Supports Sensitivity Response	4	accepted	EID11708 In this study, 331 patients with IDH-mutant grade 2 gliomas were either given vorasidenib (n = 168) or a placebo (n = 163). A median PFS of 27.7 months was reported in the vorasidenib group, compared … (full text at CIViC) PMID 37272516 · Mellinghoff et al., 2023 · Open in CIViC	civic

Glioma1

IDH2 Mutation OR IDH1 Mutation

Vorasidenib

Predictive

Supports Sensitivity Response

accepted

EID11708

In this study, 331 patients with IDH-mutant grade 2 gliomas were either given vorasidenib (n = 168) or a placebo (n = 163). A median PFS of 27.7 months was reported in the vorasidenib group, compared … (full text at CIViC)

PMID 37272516 · Mellinghoff et al., 2023 · Open in CIViC

civic

Brain Glioma2unmapped disease
IDH1 Mutation	(prognostic)	Prognostic	B	Supports Better Outcome	3	accepted	EID1639 Genomewide analysis of 250 low-grade adult gliomas revealed that patients with a wildtype IDH1/2 had significantly shorter overall survival than those with a mutated IDH gene (HR 7.4; 95%CI: 4.0-13.8)… (full text at CIViC) PMID 26061751 · 2015, N. Engl. J. Med. · Open in CIViC	civic
IDH1 Mutation	Olaparib	Predictive	D	Supports Sensitivity Response	3	accepted	EID2933 Mutations in isocitrate dehydrogenase 1 and 2, which result in overproduction of 2-hydroxyglutarate (2HG). 2HG is considered to be an oncometabolite, or a metabolite that promotes carcinogenesis, and … (full text at CIViC) PMID 28148839 · Sulkowski et al., 2017 · Open in CIViC	civic

Brain Glioma2unmapped disease

IDH1 Mutation

(prognostic)

Prognostic

Supports Better Outcome

accepted

EID1639

Genomewide analysis of 250 low-grade adult gliomas revealed that patients with a wildtype IDH1/2 had significantly shorter overall survival than those with a mutated IDH gene (HR 7.4; 95%CI: 4.0-13.8)… (full text at CIViC)

PMID 26061751 · 2015, N. Engl. J. Med. · Open in CIViC

civic

IDH1 Mutation

Olaparib

Predictive

Supports Sensitivity Response

accepted

EID2933

Mutations in isocitrate dehydrogenase 1 and 2, which result in overproduction of 2-hydroxyglutarate (2HG). 2HG is considered to be an oncometabolite, or a metabolite that promotes carcinogenesis, and … (full text at CIViC)

PMID 28148839 · Sulkowski et al., 2017 · Open in CIViC

civic

Evidence by cancer (9 items)

Clinical significance (0)