Molecular Profile Detail

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Profile Name VHL loss
Gene Variant Detail

VHL loss (loss of function)

Relevant Treatment Approaches mTORC1 Inhibitor VEGFR Inhibitor (Pan) VEGFR2 Inhibitor

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Molecular Profile Indication/Tumor Type Response Type Relevant Treatment Approaches Therapy Name Approval Status Evidence Type Efficacy Evidence References
VHL loss renal cell carcinoma sensitive VEGFR Inhibitor (Pan) Bevacizumab Preclinical Actionable In a preclinical study, clear cell renal cell carcinoma xenograft models with VHL loss demonstrated sensitivity to Avastin (bevacizumab) (PMID: 22931246). 22931246
VHL loss renal cell carcinoma sensitive ELR510444 Preclinical - Cell line xenograft Actionable In a preclinical study, renal cell carcinoma cells deficient for VHL were sensitive to ELR510444 both in culture and in cell line xenograft models (PMID: 22295124). 22295124
VHL loss renal cell carcinoma sensitive BPTES Preclinical - Cell culture Actionable In a preclinical study, VHL-deficient renal cell carcinoma cells demonstrated growth inhibition and suppression of DNA synthesis in culture when treated with BPTES (PMID: 28346230). 28346230
VHL loss renal cell carcinoma sensitive Telaglenastat Preclinical - Cell culture Actionable In a preclinical study, VHL-deficient renal cell carcinoma cells demonstrated growth inhibition in culture when treated with CB-839 (PMID: 28346230). 28346230
VHL loss renal cell carcinoma sensitive Olaparib + Telaglenastat Preclinical - Cell line xenograft Actionable In a preclinical study, the combination of CB-839 and Lynparza (olaparib) resulted in a synergistic effect, demonstrating greater reduction in tumor volume compared to either agent alone in renal carcinoma cell xenograft models deficient for VHL (PMID: 28346230). 28346230
VHL loss renal cell carcinoma sensitive BPTES + Olaparib Preclinical - Cell culture Actionable In a preclinical study, the combination of BPTES and Lynparza (olaparib) resulted in a synergistic effect, demonstrating growth inhbition of VHL-deficient renal cell carcinoma cells in culture (PMID: 28346230). 28346230