IDH2 Mutation

CI-VAR-00002680Explore in graph →CIViC 570

Curated evidence

Evidence by cancer (10 items)

Grouped by cancer context first, then therapy. The same variant can be sensitizing in one cancer and irrelevant in another — contexts are never merged. 50 items per page; a cancer group may continue on the next page.

civicCuratedShowing 1–10 of 10 evidence items across 4 cancer contexts on this page · levels, directions and significance as curated at the source; each row links to its CIViC record.

Molecular profile	Therapy	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
Acute Myeloid Leukemia6
IDH2 Mutation	(prognostic)	Prognostic	B	Does Not Support N/A	3	accepted	EID328 In patients with IDH2 R140 and R172 mutation, the presence of mutation does not impact overall survival or disease free survival. PMID 20421455 · Thol et al., 2010 · Open in CIViC	civic
IDH2 Mutation	Enasidenib	Predictive	A	Supports Sensitivity Response	4	accepted	EID5069 A first-in-human phase 1/2 trial assessing the pharmacology, safety and activity of Enasidenib in IDH2-mutant myeloid hematoblastosis patients. In the AML subgroup (n=176, (n=130 R140; n=45 R172; n=1 … (full text at CIViC) PMID 28588020 · Stein et al., 2017 · Open in CIViC	civic
IDH2 Mutation

ClinVar

Clinical significance (0)

ClinVar interpretations are shown as structured records — significance, review status, star rating, conditions — never flattened into one word.

Data not yet available

No ClinVar interpretation attached to this variant.

Chondrosarcoma1
IDH2 Mutation	(prognostic)	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… (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

Brain Glioma2unmapped disease
IDH2 Mutation	(prognostic)	Prognostic	B	Supports Better Outcome	3	accepted	EID1640 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
IDH2 Mutation	Olaparib	Predictive	D	Supports Sensitivity Response	3	rejected	EID2932 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