IDH1 R132C

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Molecular profile	Therapy	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
Acute Myeloid Leukemia7
IDH1 R132C	(diagnostic)	Diagnostic	B	Supports Positive	3	accepted	EID224 IDH1 R132 mutation is associated with patients of older age, high platelet count during diagnosis, cytogenic normalcy and NPM1 mutation. PMID 20538800 · Abbas et al., 2010 · Open in CIViC	civic
IDH1 R132C	(prognostic)	Prognostic	B	Does Not Support N/A	2	accepted	EID324 IDH1 R132 mutation in patients with AML is not associated with any prognostic value compared to patients with wild-type IDH1. PMID 20538800 · Abbas et al., 2010 · Open in CIViC	civic
IDH1 R132C	BPTES	Predictive	D

Molecular profile

Therapy

Type

Level

Direction · significance

Rating (1–5)

Status

Evidence

Source

Acute Myeloid Leukemia7

IDH1 R132C

(diagnostic)

Diagnostic

Supports Positive

accepted

EID224

IDH1 R132 mutation is associated with patients of older age, high platelet count during diagnosis, cytogenic normalcy and NPM1 mutation.

PMID 20538800 · Abbas et al., 2010 · Open in CIViC

civic

IDH1 R132C

(prognostic)

Prognostic

Does Not Support N/A

accepted

EID324

IDH1 R132 mutation in patients with AML is not associated with any prognostic value compared to patients with wild-type IDH1.

PMID 20538800 · Abbas et al., 2010 · Open in CIViC

civic

IDH1 R132C

BPTES

Predictive

Variation	Clinical significance	Review status	Stars	Conditions	Origin	Submitters	Last evaluated	Source
375891	Pathogenic/Likely pathogenic	criteria provided, multiple submitters, no conflicts	2	Acute myeloid leukemia; Enchondromatosis; Maffucci syndrome; Glioma susceptibility 1; Neoplasm; Juvenile type testicular granulosa cell tumor; Medulloblastoma	germline/somatic	14	May 04, 2026	clinvar

Variation

Clinical significance

Review status

Stars

Conditions

Origin

Submitters

Last evaluated

Source

375891

Pathogenic/Likely pathogenic

criteria provided, multiple submitters, no conflicts

Acute myeloid leukemia; Enchondromatosis; Maffucci syndrome; Glioma susceptibility 1; Neoplasm; Juvenile type testicular granulosa cell tumor; Medulloblastoma

germline/somatic

May 04, 2026

clinvar

High-Grade Glioma, NOS1
IDH1 R132C	AGI-5198	Predictive	D	Supports Sensitivity Response	2	accepted	EID2329 In an experimental in vitro study, glioma cells expressing IDH1 R132C were associated with sensitivity to AGI-5198 (IC50: 0.16M vs. IC50 > 100.00M), as compared to cells expressing wild-type IDH1. Sen… (full text at CIViC) PMID 23558169 · Rohle et al., 2013 · Open in CIViC	civic

High-Grade Glioma, NOS1

IDH1 R132C

AGI-5198

Predictive

Supports Sensitivity Response

accepted

EID2329

In an experimental in vitro study, glioma cells expressing IDH1 R132C were associated with sensitivity to AGI-5198 (IC50: 0.16M vs. IC50 > 100.00M), as compared to cells expressing wild-type IDH1. Sen… (full text at CIViC)

PMID 23558169 · Rohle et al., 2013 · Open in CIViC

civic

Low Grade Glioma1
IDH1 R132C OR IDH1 R132H OR IDH1 R132L OR IDH1 R132G OR IDH1 R132S	Vorasidenib	Predictive	A	Supports Sensitivity Response	5	accepted	EID11508 This phase 3 clinical trial was conducted to assess the progression free survival in patients with IDH-mutant gliomas treated with Vorasidenib, an oral brain-penetrant inhibitor, which showed prelimin… (full text at CIViC) PMID 37272516 · Mellinghoff et al., 2023 · Open in CIViC	civic

Low Grade Glioma1

IDH1 R132C OR IDH1 R132H OR IDH1 R132L OR IDH1 R132G OR IDH1 R132S

Vorasidenib

Predictive

Supports Sensitivity Response

accepted

EID11508

This phase 3 clinical trial was conducted to assess the progression free survival in patients with IDH-mutant gliomas treated with Vorasidenib, an oral brain-penetrant inhibitor, which showed prelimin… (full text at CIViC)

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

civic

Malignant Neoplasm1
IDH1 R132C	Olaparib	Predictive	D	Supports Sensitivity Response	3	accepted	EID10144 IDH1 R132C mutation confers a switch of function mutation where the mutated protein produces 2-hydroxyglutarate (2HG), rather than α-ketoglutarate (αKG). The novel metabolite induces a homologous reco… (full text at CIViC) PMID 28148839 · Sulkowski et al., 2017 · Open in CIViC	civic

Malignant Neoplasm1

IDH1 R132C

Olaparib

Predictive

Supports Sensitivity Response

accepted

EID10144

IDH1 R132C mutation confers a switch of function mutation where the mutated protein produces 2-hydroxyglutarate (2HG), rather than α-ketoglutarate (αKG). The novel metabolite induces a homologous reco… (full text at CIViC)

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

civic

Brain Glioma3unmapped disease
IDH1 R132C	Temozolomide	Predictive	B	Supports Sensitivity Response	—	rejected	EID2326 IDH1 mutations at R132H are the most frequent mutations accounting for 70- 90% of all IDH1 mutations in secondary glioblastoma and 5% of primary glioblastomas. PMID 18772396 · Parsons et al., 2008 · Open in CIViC	civic
IDH1 R132C	Temozolomide	Predictive	B	Supports Sensitivity Response	2	accepted	EID2327 In a study of 189 low-grade gliomas, IDH (IDH1 or IDH2) mutations were found in 132 patients (70%). IDH mutations were associated with improved rate of response to temozolomide as compared to patients… (full text at CIViC) PMID 20975057 · Houillier et al., 2010 · Open in CIViC	civic
IDH1 R132C	Temozolomide	Predictive	B	Supports Sensitivity Response	—	rejected	EID2328 Isocitrate dehydrogenases (IDH) are enzymes of the citric acid cycle catalyzing the oxidative decarboxylation of isocitrate to 2-oxoglutarate. IDH1 mutations at R132H are the most frequent mutations a… (full text at CIViC) PMID 21625441 · Krell et al., 2011 · Open in CIViC	civic

Brain Glioma3unmapped disease

IDH1 R132C

Temozolomide

Predictive

Supports Sensitivity Response

—

rejected

EID2326

IDH1 mutations at R132H are the most frequent mutations accounting for 70- 90% of all IDH1 mutations in secondary glioblastoma and 5% of primary glioblastomas.

PMID 18772396 · Parsons et al., 2008 · Open in CIViC

civic

IDH1 R132C

Temozolomide

Predictive

Supports Sensitivity Response

accepted

EID2327

In a study of 189 low-grade gliomas, IDH (IDH1 or IDH2) mutations were found in 132 patients (70%). IDH mutations were associated with improved rate of response to temozolomide as compared to patients… (full text at CIViC)

PMID 20975057 · Houillier et al., 2010 · Open in CIViC

civic

IDH1 R132C

Temozolomide

Predictive

Supports Sensitivity Response

—

rejected

EID2328

Isocitrate dehydrogenases (IDH) are enzymes of the citric acid cycle catalyzing the oxidative decarboxylation of isocitrate to 2-oxoglutarate. IDH1 mutations at R132H are the most frequent mutations a… (full text at CIViC)

PMID 21625441 · Krell et al., 2011 · Open in CIViC

civic

Evidence by cancer (13 items)

Clinical significance (1)