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 | SMARCB1 loss |
Gene Variant Detail | |
Relevant Treatment Approaches | CDK Inhibitor (Pan) CDK4/6 Inhibitor EZH2 inhibitor Fenretinide MRX34 |
Molecular Profile | Indication/Tumor Type | Response Type | Relevant Treatment Approaches | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|---|
SMARCB1 loss | rhabdoid cancer | sensitive | EZH2 inhibitor | Tazemetostat | Preclinical - Cell line xenograft | Actionable | In a preclinical study, Tazemetostat (EPZ-6438) inhibited growth of SMARCB1-deficient malignant rhabdoid tumor cell lines in culture, and inhibited H3K27 trimethylation and induced tumor regression in a SMARCB1-deleted human malignant rhabdoid tumor cell line xenograft model (PMID: 23620515). | 23620515 |
SMARCB1 loss | atypical teratoid rhabdoid tumor | sensitive | CFI-400945 | Preclinical - Cell culture | Actionable | In a preclinical study, CFI-400945 inhibited proliferation and decreased survival and migration of SMARCB1-deficient atypical teratoid rhabdoid tumor cells in culture (PMID: 28398638). | 28398638 | |
SMARCB1 loss | nasal cavity carcinoma | predicted - sensitive | EZH2 inhibitor | Tazemetostat | Case Reports/Case Series | Actionable | In a clinical study, Tazverik (tazemetostat) resulted in a clinical benefit in two patients with loss of SMARCB1, including one patient with sinonasal carcinoma and the other patient with sinonasal undifferentiated carcinoma, with stable disease lasting 13 and 8 months, respectively (PMID: 35820243). | 35820243 |
SMARCB1 loss | epithelioid sarcoma | sensitive | Barasertib | Preclinical - Cell culture | Actionable | In a preclinical study, Barasertib (AZD1152) inhibited viability of SMARCB1-deficient epithelioid sarcoma cell lines in culture (PMID: 38315003). | 38315003 | |
SMARCB1 loss | rhabdoid cancer | sensitive | Barasertib | Preclinical - Cell culture | Actionable | In a preclinical study, Barasertib (AZD1152) inhibited viability of SMARCB1-deficient rhabdoid cancer cell lines in culture (PMID: 38315003). | 38315003 |