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@jax.org
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 | 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 | 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 |
Clinical Trial | Phase | Therapies | Title | Recruitment Status | Covered Countries | Other Countries |
---|---|---|---|---|---|---|
NCT04204941 | Phase III | Doxorubicin Doxorubicin + Tazemetostat | Tazemetostat in Combination With Doxorubicin as Frontline Therapy for Advanced Epithelioid Sarcoma | Recruiting | ||
NCT02114229 | Phase II | Cisplatin + Cyclophosphamide + Methotrexate + Vincristine Sulfate Alisertib Alisertib + Carboplatin + Cyclophosphamide + Vincristine Sulfate Cyclophosphamide + Topotecan Alisertib + Carboplatin + Cyclophosphamide + Etoposide Carboplatin + Cyclophosphamide + Methotrexate + Vincristine Sulfate Alisertib + Cisplatin + Cyclophosphamide + Vincristine Sulfate | Phase 2 Study of Alisertib Therapy for Rhabdoid Tumors (SJATRT) | Recruiting | ||
NCT02601950 | Phase II | Tazemetostat | A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma | Recruiting | ||
NCT03274258 | Phase II | Ipilimumab + Nivolumab | Phase II Trial of Nivolumab Plus Ipilimumab in Patients With Renal Medullary Carcinoma | Recruiting | ||
NCT02601937 | Phase I | Tazemetostat | A Phase 1 Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma | Recruiting | ||
NCT04416568 | Phase II | Ipilimumab + Nivolumab | Study of Nivolumab and Ipilimumab in Children and Young Adults With INI1-Negative Cancers | Recruiting |