IDH1 R132S

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Molecular profile	Therapy	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
Acute Myeloid Leukemia1
IDH1 R132S	Ivosidenib	Predictive	B	Supports Sensitivity Response	—	submitted	EID2340 In a clinical study of 17 relapsed or refractory AML patients harboring IDH1 mutations, patients were associated with response to AG-120 monotherapy; 4/7 patients who achieved a response experienced c… (full text at CIViC) PMID 25583779 · 2015 · Open in CIViC	civic
Glioblastoma1
IDH1 R132S	Cetuximab	Predictive	B	Supports Sensitivity Response	—	submitted	EID4033 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 ·

Molecular profile

Therapy

Type

Level

Direction · significance

Rating (1–5)

Status

Evidence

Source

Acute Myeloid Leukemia1

IDH1 R132S

Ivosidenib

Predictive

Supports Sensitivity Response

—

submitted

EID2340

In a clinical study of 17 relapsed or refractory AML patients harboring IDH1 mutations, patients were associated with response to AG-120 monotherapy; 4/7 patients who achieved a response experienced c… (full text at CIViC)

PMID 25583779 · 2015 · Open in CIViC

civic

Glioblastoma1

IDH1 R132S

Cetuximab

Predictive

Supports Sensitivity Response

—

submitted

EID4033

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 ·

Variation	Clinical significance	Review status	Stars	Conditions	Origin	Submitters	Last evaluated	Source
375893	Pathogenic	criteria provided, single submitter	1	Acute myeloid leukemia; Hepatocellular carcinoma; Medulloblastoma SHH activated; Neoplasm; Cholangiocarcinoma	germline/somatic	4	Jan 01, 2024	clinvar

Variation

Clinical significance

Review status

Stars

Conditions

Origin

Submitters

Last evaluated

Source

375893

Pathogenic

criteria provided, single submitter

Acute myeloid leukemia; Hepatocellular carcinoma; Medulloblastoma SHH activated; Neoplasm; Cholangiocarcinoma

germline/somatic

Jan 01, 2024

clinvar

High-Grade Glioma, NOS1
IDH1 R132S	Bevacizumab	Predictive	B	Supports Sensitivity Response	2	submitted	EID2339 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

High-Grade Glioma, NOS1

IDH1 R132S

Bevacizumab

Predictive

Supports Sensitivity Response

submitted

EID2339

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

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

Brain Glioma3unmapped disease
IDH1 R132S	Temozolomide	Predictive	B	Supports Sensitivity Response	—	rejected	EID2336 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 R132S	Temozolomide	Predictive	B	Supports Sensitivity Response	—	submitted	EID2337 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 compared to wildtype ID… (full text at CIViC) PMID 20975057 · Houillier et al., 2010 · Open in CIViC	civic
IDH1 R132S	Temozolomide	Predictive	B	Supports Sensitivity Response	—	rejected	EID2338 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 R132S

Temozolomide

Predictive

Supports Sensitivity Response

—

rejected

EID2336

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 R132S

Temozolomide

Predictive

Supports Sensitivity Response

—

submitted

EID2337

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 compared to wildtype ID… (full text at CIViC)

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

civic

IDH1 R132S

Temozolomide

Predictive

Supports Sensitivity Response

—

rejected

EID2338

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 (7 items)

Clinical significance (1)