Molecular Profile Detail

Request Content

Contact

Missing content? – Request curation!

Request curation for specific Genes, Variants, or PubMed publications.

Have questions, comments, or suggestions? - Let us know!

Email us at : ckbsupport@genomenon.com

Profile Name	IDH1 R132S
Gene Variant Detail	IDH1 R132S (gain of function)
Relevant Treatment Approaches	IDH Inhibitor (Pan) IDH1 Inhibitor

Filtering

Case insensitive filtering will display rows if any text in any cell matches the filter term
Use simple literal full or partial string matches
Separate multiple filter terms with a space. Any order may be used (i. e. a b c and c b a are equivalent )
Filtering will only apply to rows that are already loaded on the page. Filtering has no impact on query parameters.
Use quotes to match on a longer phrase with spaces (i.e. "mtor c1483f")

Sorting

Generally, the default sort order for tables is set to be first column ascending; however, specific tables may set a different default sort order.
Click on any column header arrows to sort by that column
Hold down the Shift key and click multiple columns to sort by more than one column. Be sure to set ascending or descending order for a given column before moving on to the next column.

Filter rows:

Showing 1 to 13 of 13 entries

Molecular Profile	Indication/Tumor Type	Response Type	Relevant Treatment Approaches	Therapy Name	Approval Status	Evidence Type	Efficacy Evidence	References
IDH1 R132S	acute myeloid leukemia	sensitive	IDH1 Inhibitor	Ivosidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase I trial that supported FDA approval, Tibsovo (ivosidenib) treatment resulted in complete remission (CR) in 21.6% (27/125), CR with partial hematological recovery (CRh) in 8.8% (11/125), and overall response (OR) in 41.6% (52/125) of patients with relapsed or refractory acute myeloid leukemia harboring a susceptible IDH1 mutation (R132C/G/H/L/S) as detected by an FDA-approved test (PMID: 29860938; NCT02074839).	detail... 29860938 detail...
IDH1 R132S	acute myeloid leukemia	sensitive	IDH1 Inhibitor	Ivosidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase I trial that supported FDA approval, Tibsovo (ivosidenib) treatment resulted in complete remission (CR) in 28.6% (8/28), CR with partial hematological recovery (CRh) in 14.3% (4/28) of patients age 75 or older with untreated acute myeloid leukemia harboring a susceptible IDH1 mutation (R132C/G/H/L/S) as detected by an FDA-approved test, with a median treatment duration of 4.3 months (PMID: 29860938; NCT02074839).	detail... 29860938 detail...
IDH1 R132S	cholangiocarcinoma	sensitive	IDH1 Inhibitor	Ivosidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase III (ClarIDHy) trial that supported FDA approval, Tibsovo (ivosidenib) treatment significantly improved median progression-free survival (2.7 vs 1.4 mo, HR=0.37, p<0.001) and prolonged median overall survival (10.8 vs 9.7 mo, HR=0.69, p=0.06) compared to placebo in patients with advanced cholangiocarcinoma harboring IDH1 mutations including R132C/H/L/G/S, resulted in favorable objective response rate (2%, 3/124 vs 0%, 0/61) and stable disease rate (51% vs 28%) (PMID: 32416072; NCT02989857).	detail... detail... 32416072
IDH1 R132S	acute myeloid leukemia	sensitive	IDH1 Inhibitor	Azacitidine + Ivosidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase III trial (AGILE) that supported FDA approval, Tibsovo (ivosidenib) and Vidaza (azacitidine) combination therapy significantly improved event-free survival (HR 0.33, p=0.002) and median overall survival (24.0 vs 7.9 mo, HR 0.44, p=0.001) compared to Vidaza (azacitidine) plus placebo in patients with newly diagnosed acute myeloid leukemia harboring IDH1 mutations including R132C/H/G/L/S (PMID: 35443108; NCT03173248).	35443108 detail... detail...
IDH1 R132S	acute myeloid leukemia	sensitive	IDH1 Inhibitor	Olutasidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase II trial (Study 2102-HEM-101) that supported FDA approval, Rezlidhia (olutasidenib) treatment resulted in an objective response rate of 46% (57/123, 37 complete remission (CR), 4 CR with partial hematologic recovery, 14 CR with incomplete recovery, 1 morphologic leukemia-free state, 1 partial response) in patients with relapsed/refractory acute myeloid leukemia harboring a susceptible IDH1 mutation (R132S/C/G/H/L) (J Clin Oncol 39, no. 15_suppl (May 20, 2021) 7006; NCT02719574).	detail... detail... detail...
IDH1 R132S	myelodysplastic syndrome	sensitive	IDH1 Inhibitor	Ivosidenib	FDA approved - On Companion Diagnostic	Actionable	In a Phase I trial that supported FDA approval, Tibsovo (ivosidenib) was tolerated and resulted in a complete response (CR) in 44% (7/16), partial response (PR) in 6% (1/16), and marrow CR in 31% (5/16) of patients with relapsed or refractory myelodysplastic syndrome harboring a susceptible IDH1 mutation (R132C/G/H/L/S) as detected by an FDA-approved test, with a hematologic improvement in >/=1 lineages achieved by 69% (11/16) of patients (J Clin Oncol 40, no. 16_suppl (June 01, 2022) 7053; NCT02074839).	detail... detail... detail...
IDH1 R132S	chondrosarcoma	predicted - sensitive	IDH1 Inhibitor	Ivosidenib	Case Reports/Case Series	Actionable	In a Phase I trial, Tibsovo (ivosidenib) treatment was tolerated, substantially decreased plasma 2-HG levels, and resulted in a median progression-free survival of 5.6 months and stable disease in 52% (11/21) of patients with chondrosarcoma harboring IDH1 mutations, including IDH1 R132S (n=1) (PMID: 32208957; NCT02073994).	32208957
IDH1 R132S	chondrosarcoma	predicted - sensitive		Olaparib	Case Reports/Case Series	Actionable	In a Phase II trial (OLAPCO), Lynparza (olaparib) treatment resulted in stable disease lasting 10 months in a patient with chondrosarcoma harboring IDH1 R132S (PMID: 34994649, NCT02576444).	34994649
IDH1 R132S	acute myeloid leukemia	sensitive	IDH1 Inhibitor	BAY1436032	Preclinical - Patient cell culture	Actionable	In a preclinical study, BAY1436032 decreased (R)-2-hydroxyglutarate (R-2HG) levels, and inhibited growth and increased differentiation of patient-derived acute myeloid leukemia cells harboring IDH1 R132S in culture (PMID: 28232670).	28232670
IDH1 R132S	cholangiocarcinoma	sensitive		Ceralasertib + Olaparib	Preclinical - Cell culture	Actionable	In a preclinical study, the combination of Lynparza (olaparib) and Ceralasertib (AZD6738) resulted in a greater decrease in cell viability compared to Ceralasertib (AZD6738) alone in cholangiocarcinoma cells harboring IDH1 R132S in culture (PMID: 34027408).	34027408
IDH1 R132S	Advanced Solid Tumor	predicted - sensitive	IDH1 Inhibitor	DS-1001b	Preclinical - Biochemical	Actionable	In a preclinical study, transformed human cells expressing IDH1 R132S demonstrated sensitivity to DS-1001b in culture, resulting in reduced production of the oncometabolite 2-hydroxyglutarate (2-HG) (PMID: 31727689).	31727689
IDH1 R132S	intrahepatic cholangiocarcinoma	sensitive		Dasatinib	Preclinical	Actionable	In a preclinical study, intrahepatic cholangiocarcinoma cells harboring IDH1 R132S demonstrated increased sensitivity to Sprycel (dasatinib) induced growth inhibition compared to IDH1 wild-type cells in culture (PMID: 27231123).	27231123
IDH1 R132S	intrahepatic cholangiocarcinoma	sensitive		Saracatinib	Preclinical	Actionable	In a preclinical study, intrahepatic cholangiocarcinoma cells harboring IDH1 R132S demonstrated increased sensitivity to Saracatinib (AZD0530) induced growth inhibition compared to IDH1 wild-type cells in culture (PMID: 27231123).	27231123

Showing 1 to 13 of 13 entries

Filter rows: