IDH1 R132

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Molecular profile	Therapy	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
Acute Myeloid Leukemia13
IDH1 R132	(prognostic)	Prognostic	B	Does Not Support N/A	3	accepted	EID160 Mutation status of IDH1 did not change event-free survival of patients with an NPM1 mutation. PMID 20805365 · Schnittger et al., 2010 · Open in CIViC	civic
IDH1 R132	(diagnostic)	Diagnostic	B	Supports Positive	2	accepted	EID223 In leukemia patients, IDH1 R132 mutation is associated with adults and normal karyotype. PMID 20376086 · Ho et al., 2010 · Open in CIViC	civic
IDH1 R132	(prognostic)	Prognostic	B	Does Not Support

Molecular profile

Therapy

Type

Level

Direction · significance

Rating (1–5)

Status

Evidence

Source

Acute Myeloid Leukemia13

IDH1 R132

(prognostic)

Prognostic

Does Not Support N/A

accepted

EID160

Mutation status of IDH1 did not change event-free survival of patients with an NPM1 mutation.

PMID 20805365 · Schnittger et al., 2010 · Open in CIViC

civic

IDH1 R132

(diagnostic)

Diagnostic

Supports Positive

accepted

EID223

In leukemia patients, IDH1 R132 mutation is associated with adults and normal karyotype.

PMID 20376086 · Ho et al., 2010 · Open in CIViC

civic

IDH1 R132

(prognostic)

Prognostic

Does Not Support

Variation	Clinical significance	Review status	Stars	Conditions	Origin	Submitters	Last evaluated	Source
156444	Pathogenic/Likely pathogenic	criteria provided, multiple submitters, no conflicts	2	Glioblastoma multiforme, somatic; Metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria; Glioma susceptibility 1; Enchondromatosis; Metaphyseal chondromatosis; Neoplasm; Oligodendroglioma; Acute myeloid leukemia with NPM1 somatic mutations; Astrocytoma IDH-mutant; IDH1-related disorders	germline/somatic

Variation

Clinical significance

Review status

Stars

Conditions

Origin

Submitters

Last evaluated

Source

156444

Pathogenic/Likely pathogenic

criteria provided, multiple submitters, no conflicts

Glioblastoma multiforme, somatic; Metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria; Glioma susceptibility 1; Enchondromatosis; Metaphyseal chondromatosis; Neoplasm; Oligodendroglioma; Acute myeloid leukemia with NPM1 somatic mutations; Astrocytoma IDH-mutant; IDH1-related disorders

germline/somatic

Anaplastic Astrocytoma2
IDH1 R132	(prognostic)	Prognostic	B	Supports Better Outcome	3	accepted	EID348 In patients with astrocytoma, the presence of IDH1 R132 mutation is prognostic for better survival compared to patients who harbor wild type mutation. PMID 19933982 · Dubbink et al., 2009 · Open in CIViC	civic
IDH1 R132	(prognostic)	Prognostic	B	Supports Better Outcome	5	accepted	EID481 In patients with anaplastic astrocytoma, those with mutations at R132 of IDH1 had better overall survival than wild-type patients. PMID 19228619 · Yan et al., 2009 · Open in CIViC	civic

Anaplastic Astrocytoma2

IDH1 R132

(prognostic)

Prognostic

Supports Better Outcome

accepted

EID348

In patients with astrocytoma, the presence of IDH1 R132 mutation is prognostic for better survival compared to patients who harbor wild type mutation.

PMID 19933982 · Dubbink et al., 2009 · Open in CIViC

civic

IDH1 R132

(prognostic)

Prognostic

Supports Better Outcome

accepted

EID481

In patients with anaplastic astrocytoma, those with mutations at R132 of IDH1 had better overall survival than wild-type patients.

PMID 19228619 · Yan et al., 2009 · Open in CIViC

civic

Cholangiocarcinoma1
IDH1 R132	Ivosidenib	Predictive	A	Supports Sensitivity Response	5	accepted	EID10886 The multicenter randomized double-blind phase 3 study included patients with IDH1-mutant cholangiocarcinoma who had progressed on previous therapy. Patients were randomly assigned to Ivosidenib or pla… (full text at CIViC) PMID 32416072 · Abou-Alfa et al., 2020 · Open in CIViC	civic

Cholangiocarcinoma1

IDH1 R132

Ivosidenib

Predictive

Supports Sensitivity Response

accepted

EID10886

The multicenter randomized double-blind phase 3 study included patients with IDH1-mutant cholangiocarcinoma who had progressed on previous therapy. Patients were randomly assigned to Ivosidenib or pla… (full text at CIViC)

PMID 32416072 · Abou-Alfa et al., 2020 · Open in CIViC

civic

Chondrosarcoma3
IDH1 R132	(diagnostic)	Diagnostic	B	Supports Positive	2	rejected	EID11197 Approximately 1200 mesechymal tumors were screened for IDH1 R132 mutations by Sequenom mass spectrometry, including 220 cartilaginous tumors, 222 osteosarcomas and another 750 bone and soft tissue tum… (full text at CIViC) PMID 21598255 · Amary et al., 2011 · Open in CIViC	civic
IDH1 R132	(diagnostic)	Diagnostic	B	Supports Positive	3	submitted	EID11198 Approximately 1200 mesechymal tumors were screened for IDH1 R132 mutations by Sequenom mass spectrometry, including 220 cartilaginous tumors, 222 osteosarcomas and another 750 bone and soft tissue tum… (full text at CIViC) PMID 21598255 · Amary et al., 2011 · Open in CIViC	civic
IDH1 R132	Ivosidenib	Predictive	A	Supports Sensitivity Response	2	submitted	EID11194 In a phase 1 study (NCT02073994), 21 patients with advanced IDH-mutant chondrosarcoma received the IDH1-mutant specific inhibitor ivosidenib. Plasma levels of the 2-HG oncometabolite decreased to nor… (full text at CIViC) PMID 32208957 · Tap et al., 2020 · Open in CIViC	civic

Chondrosarcoma3

IDH1 R132

(diagnostic)

Diagnostic

Supports Positive

rejected

EID11197

Approximately 1200 mesechymal tumors were screened for IDH1 R132 mutations by Sequenom mass spectrometry, including 220 cartilaginous tumors, 222 osteosarcomas and another 750 bone and soft tissue tum… (full text at CIViC)

PMID 21598255 · Amary et al., 2011 · Open in CIViC

civic

IDH1 R132

(diagnostic)

Diagnostic

Supports Positive

submitted

EID11198

PMID 21598255 · Amary et al., 2011 · Open in CIViC

civic

IDH1 R132

Ivosidenib

Predictive

Supports Sensitivity Response

submitted

EID11194

In a phase 1 study (NCT02073994), 21 patients with advanced IDH-mutant chondrosarcoma received the IDH1-mutant specific inhibitor ivosidenib. Plasma levels of the 2-HG oncometabolite decreased to nor… (full text at CIViC)

PMID 32208957 · Tap et al., 2020 · Open in CIViC

civic

Glioblastoma4
IDH1 R132	(prognostic)	Prognostic	B	Supports Better Outcome	3	accepted	EID349 In patients with glioblastoma, those harboring IDH1 R132 mutation has higher overall survival compared to those who do not have IDH1 mutation. PMID 20127344 · Bleeker et al., 2010 · Open in CIViC	civic
IDH1 R132	(prognostic)	Prognostic	B	Supports Better Outcome	5	accepted	EID480 In patients with a glioblastoma, those with mutations at R132 of IDH1 had better overall survival than wild-type patients. PMID 19228619 · Yan et al., 2009 · Open in CIViC	civic
IDH1 R132	Cetuximab	Predictive	B	Supports Sensitivity Response	—	submitted	EID3718 In a clinical study of 63 recurrent glioma patients, de novo glioblastoma patients treated with cetuximab monotherapy (n=27) and harboring IDH1 codon 132 mutation were associated with improved overal… (full text at CIViC) PMID 22199315 · Lv et al., 2011 · Open in CIViC	civic
IDH1 R132	Cetuximab	Predictive	B	Supports Sensitivity Response	3	submitted	EID4032 In a clinical study of 63 recurrent glioma patients, de novo glioblastoma patients treated with cetuximab monotherapy (n=27) and harboring IDH1 codon 132 mutation were associated with improved overal… (full text at CIViC) PMID 22199315 · Lv et al., 2011 · Open in CIViC	civic

Glioblastoma4

IDH1 R132

(prognostic)

Prognostic

Supports Better Outcome

accepted

EID349

In patients with glioblastoma, those harboring IDH1 R132 mutation has higher overall survival compared to those who do not have IDH1 mutation.

PMID 20127344 · Bleeker et al., 2010 · Open in CIViC

civic

IDH1 R132

(prognostic)

Prognostic

Supports Better Outcome

accepted

EID480

In patients with a glioblastoma, those with mutations at R132 of IDH1 had better overall survival than wild-type patients.

PMID 19228619 · Yan et al., 2009 · Open in CIViC

civic

IDH1 R132

Cetuximab

Predictive

Supports Sensitivity Response

—

submitted

EID3718

In a clinical study of 63 recurrent glioma patients, de novo glioblastoma patients treated with cetuximab monotherapy (n=27) and harboring IDH1 codon 132 mutation were associated with improved overal… (full text at CIViC)

PMID 22199315 · Lv et al., 2011 · Open in CIViC

civic

IDH1 R132

Cetuximab

Predictive

Supports Sensitivity Response

submitted

EID4032

PMID 22199315 · Lv et al., 2011 · Open in CIViC

civic

Glioma2
IDH1 R132	(prognostic)	Prognostic	B	Supports Better Outcome	3	submitted	EID4031 In a clinical study of 63 recurrent glioma patients, patients harboring IDH1 codon 132 mutation were associated with improved overall survival from initial diagnosis (56.5 vs. 21.3 months; P=0.001), a… (full text at CIViC) PMID 22199315 · Lv et al., 2011 · Open in CIViC	civic
IDH1 R132	Safusidenib	Predictive	B	Supports Sensitivity Response	3	submitted	EID7448 In a phase1 study, patients with Glioma harboring IDH1-R132X mutation were treated with selective IDH1-R132X inhibitor DS-1001b. The response rate was 17.1% (6/35) in patients with enhancing lesion a… (full text at CIViC) Open in CIViC	civic

Glioma2

IDH1 R132

(prognostic)

Prognostic

Supports Better Outcome

submitted

EID4031

In a clinical study of 63 recurrent glioma patients, patients harboring IDH1 codon 132 mutation were associated with improved overall survival from initial diagnosis (56.5 vs. 21.3 months; P=0.001), a… (full text at CIViC)

PMID 22199315 · Lv et al., 2011 · Open in CIViC

civic

IDH1 R132

Safusidenib

Predictive

Supports Sensitivity Response

submitted

EID7448

In a phase1 study, patients with Glioma harboring IDH1-R132X mutation were treated with selective IDH1-R132X inhibitor DS-1001b. The response rate was 17.1% (6/35) in patients with enhancing lesion a… (full text at CIViC)

Open in CIViC

civic

High-Grade Glioma, NOS2
IDH1 R132	Bevacizumab	Predictive	B	Supports Sensitivity Response	2	submitted	EID2330 In a clinical study of 63 recurrent glioma patients, patients treated with bevacizumab monotherapy (n=11) and harboring IDH1 codon 132 mutation were associated with improved progression free survival … (full text at CIViC) PMID 22199315 · Lv et al., 2011 · Open in CIViC	civic
IDH1 R132	Bevacizumab + SunitinibSubstitutes	Predictive	B	Supports Sensitivity Response	2	accepted	EID2020 In a clinical study of 63 recurrent glioma patients, the combined cohort of patients treated with either bevacizumab or sunitinib showed that those harboring IDH1 codon 132 mutation were associated wi… (full text at CIViC) PMID 22199315 · Lv et al., 2011 · Open in CIViC	civic

High-Grade Glioma, NOS2

IDH1 R132

Bevacizumab

Predictive

Supports Sensitivity Response

submitted

EID2330

In a clinical study of 63 recurrent glioma patients, patients treated with bevacizumab monotherapy (n=11) and harboring IDH1 codon 132 mutation were associated with improved progression free survival … (full text at CIViC)

PMID 22199315 · Lv et al., 2011 · Open in CIViC

civic

IDH1 R132

Bevacizumab + SunitinibSubstitutes

Predictive

Supports Sensitivity Response

accepted

EID2020

In a clinical study of 63 recurrent glioma patients, the combined cohort of patients treated with either bevacizumab or sunitinib showed that those harboring IDH1 codon 132 mutation were associated wi… (full text at CIViC)

PMID 22199315 · Lv et al., 2011 · Open in CIViC

civic

Intrahepatic Cholangiocarcinoma1
IDH1 R132	Dasatinib	Predictive	D	Supports Sensitivity Response	4	accepted	EID6439 Through a high-throughput drug screen of a large panel of cancer cell lines, including 17 biliary tract cancers, this work found that IDH mutant (IDHm) ICC cells demonstrate a striking response to the… (full text at CIViC) PMID 27231123 · Saha et al., 2016 · Open in CIViC	civic

Intrahepatic Cholangiocarcinoma1

IDH1 R132

Dasatinib

Predictive

Supports Sensitivity Response

accepted

EID6439

Through a high-throughput drug screen of a large panel of cancer cell lines, including 17 biliary tract cancers, this work found that IDH mutant (IDHm) ICC cells demonstrate a striking response to the… (full text at CIViC)

PMID 27231123 · Saha et al., 2016 · Open in CIViC

civic

Myelodysplastic Syndrome2
IDH1 R132	(prognostic)	Prognostic	B	Supports Poor Outcome	4	accepted	EID374 IDH1 R132 mutation in patients suffering from myelodysplastic syndromes is associated with worse overall survival. PMID 20494930 · Thol et al., 2010 · Open in CIViC	civic
IDH1 R132	Ivosidenib	Predictive	B	Supports Sensitivity Response	3	submitted	EID13287 In the relapsed or refractory myelodysplastic syndrome (MDS) substudy of the phase 1, open-label, single-arm, multinational first-in-human trial of ivosidenib (NCT02074839), adults with MDS carrying a… (full text at CIViC) PMID 38640348 · DiNardo et al., 2024 · Open in CIViC	civic

Myelodysplastic Syndrome2

IDH1 R132

(prognostic)

Prognostic

Supports Poor Outcome

accepted

EID374

IDH1 R132 mutation in patients suffering from myelodysplastic syndromes is associated with worse overall survival.

PMID 20494930 · Thol et al., 2010 · Open in CIViC

civic

IDH1 R132

Ivosidenib

Predictive

Supports Sensitivity Response

submitted

EID13287

In the relapsed or refractory myelodysplastic syndrome (MDS) substudy of the phase 1, open-label, single-arm, multinational first-in-human trial of ivosidenib (NCT02074839), adults with MDS carrying a… (full text at CIViC)

PMID 38640348 · DiNardo et al., 2024 · Open in CIViC

civic

Evidence by cancer (30 items)

Clinical significance (1)