Molecular Profile Detail

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Profile Name IDH1 R132C
Gene Variant Detail

IDH1 R132C (gain of function)

Relevant Treatment Approaches IDH Inhibitor (Pan) IDH1 Inhibitor

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Molecular Profile Indication/Tumor Type Response Type Therapy Name Approval Status Evidence Type Efficacy Evidence References
IDH1 R132C IDH1 S280F acute myeloid leukemia predicted - resistant Ivosidenib Case Reports/Case Series Actionable In a clinical study, IDH1 S280F was identified as an acquired mutation in cis with the original IDH1 R132C in a patient with acute myeloid leukemia (AML), who developed resistance to Tibsovo (ivosidenib) after initial response (PMID: 29950729). 29950729
IDH1 R132C IDH1 S280F acute myeloid leukemia predicted - resistant Ivosidenib Case Reports/Case Series Actionable In a clinical case study, a patient with acute myeloid leukemia (AML) harboring IDH1 R132C progressed following an initial response to treatment with Tibsovo (ivosidenib), and was found to have acquired IDH1 S280F (PMID: 33832922). 33832922
IDH1 R132C IDH2 R172K intrahepatic cholangiocarcinoma predicted - resistant Ivosidenib Case Reports/Case Series Actionable In a clinical case study, IDH2 R172K was identified upon progression in a patient with metastatic intrahepatic cholangiocarcinoma harboring IDH1 R132C, along with PIK3CA E545K, who previously responded to Tibsovo (ivosidenib) (PMID: 36056177). 36056177
IDH1 R132C IDH1 D279N intrahepatic cholangiocarcinoma predicted - resistant Ivosidenib Case Reports/Case Series Actionable In a clinical case study, IDH1 D279N was identified upon progression in a patient with metastatic intrahepatic cholangiocarcinoma harboring IDH1 R132C, along with NRAS G13V, who previously responded to Tibsovo (ivosidenib) (PMID: 36056177). 36056177
IDH1 R132C IDH1 S280F acute myeloid leukemia predicted - resistant Ivosidenib Case Reports/Case Series Actionable In a Phase I trial, IDH1 S280F was identified at progression in 3 patients with acute myeloid leukemia harboring IDH1 R132C who previously responded to Tibsovo (ivosidenib) treatment (PMID: 32380538; NCT02074839). 32380538
IDH1 R132C IDH1 D279N IDH2 R140Q acute myeloid leukemia predicted - resistant Ivosidenib Case Reports/Case Series Actionable In a Phase I trial, IDH1 D279N and IDH2 R140Q were identified at progression in a patient with acute myeloid leukemia harboring IDH1 R132C who previously responded to Tibsovo (ivosidenib) treatment (PMID: 32380538; NCT02074839). 32380538
IDH1 G131A IDH1 R132C acute myeloid leukemia predicted - resistant Ivosidenib Case Reports/Case Series Actionable In a Phase I trial, IDH1 G131A was identified at progression in a patient with acute myeloid leukemia harboring IDH1 R132C who previously responded to Tibsovo (ivosidenib) treatment (PMID: 32380538; NCT02074839). 32380538
IDH1 R132C IDH1 D279N IDH1 H315D acute myeloid leukemia predicted - resistant Ivosidenib Case Reports/Case Series Actionable In a Phase I trial, IDH1 H315D and D279N were identified at progression in a patient with acute myeloid leukemia harboring IDH1 R132C who previously responded to Tibsovo (ivosidenib) treatment (PMID: 32380538; NCT02074839). 32380538
IDH1 R132C IDH2 R140Q acute myeloid leukemia predicted - resistant Ivosidenib Case Reports/Case Series Actionable In a Phase I trial, IDH2 R140Q was identified at progression in 5 patients with acute myeloid leukemia harboring IDH1 R132C who previously responded to Tibsovo (ivosidenib) treatment (PMID: 32380538; NCT02074839). 32380538
IDH1 R132C IDH1 T313I acute myeloid leukemia predicted - resistant Ivosidenib Case Reports/Case Series Actionable In a clinical case study, IDH1 T313I was identified as an acquired mutation in a patient with acute myeloid leukemia harboring IDH1 R132C upon progression on Tibsovo (ivosidenib) (PMID: 36222845). 36222845
BRAF V600E IDH1 R132C melanoma predicted - sensitive Dabrafenib + Trametinib Case Reports/Case Series Actionable In a retrospective analysis, treatment with the combination of Tafinlar (dabrafenib) and Mekinist (trametinib) resulted in a partial response with an overall survival of 51.1 months and a duration of treatment of 4.1 months in a melanoma patient harboring BRAF V600E and IDH1 R132C (PMID: 39500140). 39500140
BRAF V600E IDH1 R132C lung squamous cell carcinoma predicted - sensitive Dabrafenib + Trametinib Case Reports/Case Series Actionable In a retrospective analysis, treatment with the combination of Tafinlar (dabrafenib) and Mekinist (trametinib) resulted in a partial response with a progression-free survival of 14.8 months, an overall survival of 34.6 months, and a duration of treatment of 14.9 months in a patient with squamous non-small cell lung cancer harboring BRAF V600E and IDH1 R132C (PMID: 39500140). 39500140