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| Profile Name | IDH2 R172W |
| Gene Variant Detail | |
| Relevant Treatment Approaches | IDH Inhibitor (Pan) IDH2 Inhibitor |
| Molecular Profile | Indication/Tumor Type | Response Type | Relevant Treatment Approaches | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
|---|---|---|---|---|---|---|---|---|
| IDH2 R172W | acute myeloid leukemia | sensitive | IDH2 Inhibitor | Enasidenib | FDA approved - On Companion Diagnostic | Actionable | In a Phase I/II trial that supported FDA approval, Idhifa (enasidenib) treatment resulted in an overall response rate of 40.3% (71/176) with a median response duration of 5.8 months, complete remission in 19.3% (34/176), and stable disease in 48.3% (85/176) of acute myeloid leukemia patients harboring IDH2 mutations (PMID: 28588020; NCT01915498) and IDH2 R172W is on the companion diagnostic. | 28588020 detail... detail... |
| IDH2 R172W | acute myeloid leukemia | predicted - sensitive | IDH2 Inhibitor | Enasidenib + Venetoclax | Case Reports/Case Series | Actionable | In a Phase Ib/II trial, Idhifa (enasidenib) plus Venclexta (venetoclax) was well tolerated and demonstrated activity in relapsed or refractory acute myeloid leukemia patients harboring IDH2 R140Q (15/27) or IDH2 R172K/W (12/27), with an overall response rate (ORR) of 70% (16/23), with complete remission (CR) in 57% (13/23) of evaluable patients, and an ORR of 83% (10/12) and a CR rate of 67% (8/12) in patients harboring IDH2 R172K or IDH2 R172W (Blood (2023) 142 (Supplement 1): 159; NCT04092179). | detail... |
| IDH2 R172W | oligodendroglioma | sensitive | IDH Inhibitor (Pan) | Vorasidenib | FDA approved - On Companion Diagnostic | Actionable | In a Phase III trial l (INDIGO) that supported FDA approval, Voranigo (vorasidenib) treatment significantly improved progression-free survival (27.7 vs 11.1 months, HR 0.39, p<0.001) and time to next intervention (HR 0.26, p<0.001) compared to placebo in adult and pediatric patients 12 years and older with WHO grade 2 oligodendroglioma or astrocytoma harboring susceptible IDH1 or IDH2 mutations, including IDH2 R172K/M/W/S/G (PMID: 37272516; NCT04164901). | 37272516 detail... detail... |
| IDH2 R172W | astrocytoma, IDH-mutant, grade 2 | sensitive | IDH Inhibitor (Pan) | Vorasidenib | FDA approved - On Companion Diagnostic | Actionable | In a Phase III trial l (INDIGO) that supported FDA approval, Voranigo (vorasidenib) treatment significantly improved progression-free survival (27.7 vs 11.1 months, HR 0.39, p<0.001) and time to next intervention (HR 0.26, p<0.001) compared to placebo in adult and pediatric patients 12 years and older with WHO grade 2 astrocytoma or oligodendroglioma harboring susceptible IDH1 or IDH2 mutations, including IDH2 R172K/M/W/S/G (PMID: 37272516; NCT04164901). | 37272516 detail... detail... |