Gene

IDH2

isocitrate dehydrogenase (NADP(+)) 2

Explore in graph →CI-GENE-00005341HGNC:5383 ENSG00000182054 NCBI 3418 CIViC Cancer geneApproved

Curated evidence

Clinical evidence (27)

CIViC items involving this gene, grouped by molecular profile and therapy, with native levels and directions. 50 items per page.

civicCuratedShowing 1–27 of 27 evidence items · levels, directions and significance as curated at the source; each row links to its CIViC record.

Therapy	Cancer	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
IDH2 Mutation8
(prognostic)	Chondrosarcoma	Prognostic	B	Supports Better Outcome	4	accepted	EID7726 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…

Cohorts

Alteration frequency by cohort (66)

Frequency = cases affected / cases profiled within one cohort. Cohorts are never pooled.

cbioportalObserved66 cohort rows (showing 16, sorted by frequency) · frequency = affected / profiled, as published by each cohort; cohorts are not pooled. Hover a row badge for its release.

Cohort	Mapped cancer	Alteration	Affected (n)	Profiled (n)	Frequency (%)	Rank in cohort	Source
Uterine Sarcoma/Mesenchymal (MSK, Clin Cancer Res 2020) usarc_msk_2020	Uterine Corpus Sarcoma	Ssm

Literature

Linked publications (15)

25 per page, newest first.

pubmedPublishedShowing 1–15 of 15 publications · bibliographic data as recorded by PubMed; abstracts are not reproduced.

Vorasidenib in IDH1- or IDH2-Mutant Low-Grade Glioma.
· N Engl J Med · 2023 · PMID 37272516 · validated civic_curation pubmed
Enasidenib in mutant IDH2 relapsed or refractory acute myeloid leukemia.
· Blood · 2017 · PMID 28588020 · validated civic_curation pubmed
IDH1 and IDH2 mutations in gliomas.
· N Engl J Med · 2009 · PMID 19228619 · validated civic_curation pubmed
Genomic Classification and Prognosis in Acute Myeloid Leukemia.
· N Engl J Med · 2016 · PMID 27276561 · validated civic_curation pubmed
IDH2 mutations are frequent in angioimmunoblastic T-cell lymphoma.

IDH1 Mutation + IDH2 MutationIDH1 IDH21
Vorasidenib	Glioma	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

IDH1 Mutation + IDH2 MutationIDH1 IDH21

Vorasidenib

Glioma

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

IDH2 R1406
(prognostic)	Myelodysplastic SyndromeCURATED_BROADER	Prognostic	B	Does Not Support N/A	3	accepted	EID330 R140Q mutation in IDH2 does not have prognostic value in patients with MDS. PMID 22033490 · Patnaik et al., 2012 · Open in CIViC	civic
〃	Acute Myeloid LeukemiaCURATED_BROADER	Prognostic	B	Supports Better Outcome	3	accepted	EID350 In AML, patients with IDH2 R140 mutation have improved overall survival compared to those with wild-type IDH2. PMID 21596855 · Green et al., 2011 · Open in CIViC	civic
〃	Acute Myeloid LeukemiaCURATED_BROADER	Prognostic	B	Does Not Support N/A	2	accepted	EID327 In this meta-analysis of adult non-promyelocytic AML (11 studies), patients with IDH2 R140 or R172 mutations had event free survival and overall survival similar to those with wild-type IDH2. PMID 22616558 · Zhou et al., 2012 · Open in CIViC	civic
〃	Myelodysplastic SyndromeCURATED_BROADER	Prognostic	B	Does Not Support N/A	2	accepted	EID329 In patients with myelodysplastic syndrome, the presence of IDH2 R140Q (N=3) or IDH1 R132 (N=2) mutations did not confer prognostic value (overall survival) compared to wild-type IDH1/2 patients (N=61;… (full text at CIViC) PMID 21997850 · Lin et al., 2012 · Open in CIViC	civic
〃	Acute Myeloid LeukemiaCURATED_BROADER	Prognostic	B	Does Not Support N/A	2	accepted	EID1542 In patients with acute myeloid leukemia, the presence of IDH2 R140Q (n = 7), IDH2 R172K (n = 3) or IDH1 R132 (N=4) mutations did not confer prognostic value (overall survival) compared to wild-type ID… (full text at CIViC) PMID 21997850 · Lin et al., 2012 · Open in CIViC	civic
Enasidenib	Acute Myeloid LeukemiaCURATED_BROADER	Predictive	D	Supports Sensitivity Response	3	submitted	EID10292 AG-221 induces differentiation in four IDH2R140Q-mutant human AML xenograft mouse models in vivo and conferred a dose-dependent, statistically significant survival advantage in an in vivo aggressive h… (full text at CIViC) PMID 28193778 · Yen et al., 2017 · Open in CIViC	civic

IDH2 R1726
(prognostic)	GlioblastomaCURATED_BROADER	Prognostic	B	Supports Better Outcome	5	accepted	EID482 In patients with a glioblastoma, those with mutations at R172 of IDH2 had better overall survival than wild-type patients. PMID 19228619 · Yan et al., 2009 · Open in CIViC	civic
〃	Anaplastic AstrocytomaALIAS	Prognostic	B	Supports Better Outcome	5	accepted	EID483 In patients with anaplastic astrocytoma, those with mutations at R172 of IDH2 had better overall survival than wild-type patients. PMID 19228619 · Yan et al., 2009 · Open in CIViC	civic
(diagnostic)	Peripheral T-Cell Lymphoma, Not Otherwise Specified	Diagnostic	B	Supports Positive	4	accepted	EID628 IDH2 R172 mutations were frequently observed in patients with the angioimmunoblastic T-cell lymphoma subtype of peripheral T-cell lymphomas but not observed in other subtypes. PMID 22215888 · Cairns et al., 2012 · Open in CIViC	civic
(prognostic)	Acute Myeloid LeukemiaCURATED_BROADER	Prognostic	B	Supports Better Outcome	4	accepted	EID1511 In a study of 1,540 patients with Acute Myeloid Leukemia, 18 patients had a mutation in IDH2(R172). IDH2(R172) mutations rarely showed co-mutation with NPM1 (odds ratio= 0.06; P=4x10-5), whereas IDH2… (full text at CIViC) PMID 27276561 · Papaemmanuil et al., 2016 · Open in CIViC	civic
〃	Peripheral T-Cell Lymphoma, Not Otherwise Specified	Prognostic	B	Does Not Support Better Outcome	3	accepted	EID627 In patients with angioimmunoblastic T-cell lymphoma (a subtype of peripheral T-cell lymphoma), IDH2 R172 mutations (N=16) had no impact on overall survival when compared to patients with wildtype IDH2… (full text at CIViC) PMID 26268241 · Wang et al., 2015 · Open in CIViC	civic
(functional)	—UNRESOLVED	Functional	D	Supports Neomorphic	4	submitted	EID13229 In vivo study demonstrated that for 21 chondrosarcoma patient samples that had significant IDH variation, CpG methylation sequencing of these biopsies was able to linked IDH variant levels to hypermet… (full text at CIViC) PMID 24065766 · Lu et al., 2013 · Open in CIViC	civic