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Profile Name | RB1 loss |
Gene Variant Detail | |
Relevant Treatment Approaches | HDAC Inhibitor |
Molecular Profile | Indication/Tumor Type | Response Type | Relevant Treatment Approaches | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|---|
RB1 loss | neuroendocrine tumor | sensitive | Sirolimus | Preclinical | Actionable | In a preclinical study, Sirolimus (rapamycin) slowed pituitary tumors and decreased the occurrence of thyroid tumors in Rb1+/- mice (PMID: 23454836). | 23454836 | |
RB1 loss | retinoblastoma | sensitive | Sirolimus | Preclinical | Actionable | In a preclinical study, Sirolimus (rapamycin) decreased tumor occurrence, tumor hypoxia and tumor vascularization in a retinoblastoma mouse model with functionally inactivated Rb protein (PMID: 21468343, PMID: 1689463). | 21468343 1689463 | |
RB1 loss | Advanced Solid Tumor | no benefit | Palbociclib | Preclinical | Actionable | In preclinical studies, the CDK4/6 inhibitor, Ibrance (palbociclib), was not effective in a variety of solid tumors with Rb1-deficiency (PMID: 26649278). | 26649278 | |
RB1 loss | lung small cell carcinoma | resistant | Trilaciclib | Preclinical - Cell line xenograft | Actionable | In a preclinical study, RB1-deficient small cell lung cancer cell lines were resistant to Trilaciclib (G1T28) in culture and in xenograft models (PMID: 26826116). | 26826116 | |
RB1 loss | lung small cell carcinoma | sensitive | Topotecan + Trilaciclib | Preclinical - Cell line xenograft | Actionable | In a preclinical study, the combination of Trilaciclib (G1T28) and Hycamtin (topotecan) inhibited tumor growth in RB1-deficient small cell lung cancer cell line xenograft models, with greater efficacy than Hycamtin (topotecan) alone (PMID: 26826116). | 26826116 | |
RB1 loss | retinoblastoma | sensitive | HDAC Inhibitor | Trichostatin A | Preclinical - Cell culture | Actionable | In a preclinical study, Trichostatin A (TSA) inhibited growth of retinoblastoma cell lines in culture (PMID: 18483379), which have been demonstrated to be deficient in RB1 (PMID: 23498719). | 23498719 18483379 |
RB1 loss | retinoblastoma | sensitive | HDAC Inhibitor | Vorinostat | Preclinical - Cell culture | Actionable | In a preclinical study, Zolinza (vorinostat) inhibited growth of retinoblastoma cell lines in culture (PMID: 18483379), which have been demonstrated to be deficient in RB1 (PMID: 23498719). | 23498719 18483379 |
RB1 loss | retinoblastoma | sensitive | HDAC Inhibitor | Entinostat | Preclinical - Cell line xenograft | Actionable | In a preclinical study, Entinostat (MS-275) inhibited growth of retinoblastoma cell lines in culture and inhibited tumor growth in a retinoblastoma cell line xenograft model (PMID: 18483379), which have been demonstrated to be deficient in RB1 (PMID: 23498719). | 23498719 18483379 |
RB1 loss | estrogen-receptor positive breast cancer | resistant | Palbociclib | Preclinical - Cell culture | Actionable | In a preclinical study, RB1 loss was associated with acquired resistance to Ibrance (palbociclib) in an estrogen-receptor positive breast cancer cell line in culture (PMID: 27020857). | 27020857 | |
RB1 loss | Merkel cell carcinoma | sensitive | LY3295668 | Preclinical - Pdx & cell culture | Actionable | In a preclinical study, LY3295668 resulted in cell growth inhibition and increased cell cycle arrest and apoptosis in Merkel cell carcinoma patient-derived cell lines with loss of Rb1 expression in culture, and resulted in reduced tumor growth in a patient-derived xenograft (PDX) model (PMID: 34359608). | 34359608 |