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Gene Symbol | SMARCB1 | ||||||||||
Synonyms | BAF47 | CSS3 | hSNFS | INI-1 | INI1 | MRD15 | PPP1R144 | RDT | RTPS1 | Sfh1p | SNF5 | SNF5L1 | Snr1 | SWNTS1 | ||||||||||
Gene Description | SMARCB1, SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1, is a member of the SWI/SNF chromatin remodeling complex and regulates transcription of several genes involved in cell proliferation (PMID: 23095836, PMID: 28109176). Smarcb1 inactivation through loss is characteristic of atypical teratoid/rhabdoid tumors (PMID: 23095836), schwannomas (PMID: 28368924, PMID: 28824165), sinonasal carcinomas (PMID: 29797680), and colon carcinoma (PMID: 32083567). | ||||||||||
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Clinical Trial | Phase | Therapies | Title | Recruitment Status | Covered Countries | Other Countries |
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NCT03533582 | Phase II | Cisplatin + Doxorubicin + Gemcitabine + Oxaliplatin + Sorafenib Cisplatin + Doxorubicin + Fluorouracil + Vincristine Sulfate Carboplatin + Cisplatin + Doxorubicin + Etoposide Carboplatin + Cisplatin + Doxorubicin + Irinotecan + Vincristine Sulfate Cisplatin Cisplatin + Doxorubicin + Sorafenib Cisplatin + Doxorubicin Carboplatin + Cisplatin + Doxorubicin | Cisplatin and Combination Chemotherapy in Treating Children and Young Adults With Hepatoblastoma or Liver Cancer After Surgery | Active, not recruiting | USA | NZL | CAN | AUS | 2 |
NCT03213665 | Phase II | Tazemetostat | Tazemetostat in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With EZH2, SMARCB1, or SMARCA4 Gene Mutations (A Pediatric MATCH Treatment Trial) | Active, not recruiting | USA | 1 |
NCT04266912 | Phase Ib/II | Avelumab + Berzosertib | Avelumab and M6620 for the Treatment of DDR Deficient Metastatic or Unresectable Solid Tumors | Active, not recruiting | USA | 0 |
NCT03274258 | Phase II | Ipilimumab + Nivolumab | Phase II Trial of Nivolumab Plus Ipilimumab in Patients With Renal Medullary Carcinoma | Active, not recruiting | USA | 0 |
NCT04965753 | Phase I | FHD-609 | FHD-609 in Subjects With Advanced Synovial Sarcoma or Advanced SMARCB1-Loss Tumors | Terminated | USA | ITA | FRA | ESP | 0 |
NCT05715281 | Phase II | Atezolizumab + Tiragolumab | Testing the Combination of Anti-cancer Drugs Atezolizumab and Tiragolumab in People With Advanced Stage Rare Cancers, RARE3 Trial | Recruiting | USA | 0 |
NCT04416568 | Phase II | Ipilimumab + Nivolumab | Study of Nivolumab and Ipilimumab in Children and Young Adults With INI1-Negative Cancers | Recruiting | USA | 0 |
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) | Active, not recruiting | USA | 0 |
NCT06444880 | Phase II | Cemiplimab + REGN4018 REGN4018 | Phase II Trial of Ubamatamab Alone or in Combination With Cemiplimab in MUC16-Expressing SMARCB1-Deficient Malignancies | Recruiting | USA | 0 |
NCT04204941 | Phase III | Doxorubicin Doxorubicin + Tazemetostat | Tazemetostat in Combination With Doxorubicin as Frontline Therapy for Advanced Epithelioid Sarcoma | Recruiting | USA | GBR | CAN | 1 |
NCT05407441 | Phase Ib/II | Ipilimumab + Nivolumab + Tazemetostat | Tazemetostat+Nivo/Ipi in INI1-Neg/SMARCA4-Def Tumors | Recruiting | USA | 0 |
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 | Completed | USA | NLD | ITA | GBR | FRA | DNK | DEU | CAN | AUS | 0 |
NCT05355753 | Phase Ib/II | CFT8634 | A Study to Assess the Safety and Tolerability of CFT8634 in Locally Advanced or Metastatic SMARCB1-Perturbed Cancers, Including Synovial Sarcoma and SMARCB1-Null Tumors | Terminated | USA | 0 |
NCT02601950 | Phase II | Tazemetostat | A Study of Tazemetostat in Adult Participants With Soft Tissue Sarcoma | Completed | USA | ITA | GBR | FRA | DEU | CAN | BEL | AUS | 1 |
NCT05286801 | Phase Ib/II | Atezolizumab + Tiragolumab | Tiragolumab and Atezolizumab for the Treatment of Relapsed or Refractory SMARCB1 or SMARCA4 Deficient Tumors | Recruiting | USA | CAN | AUS | 0 |
NCT03654716 | Phase I | ALRN-6924 + Cytarabine ALRN-6924 | Phase 1 Study of the Dual MDM2/MDMX Inhibitor ALRN-6924 in Pediatric Cancer | Completed | USA | 0 |
NCT03155620 | Phase II | Tazemetostat Larotrectinib LY3023414 Vemurafenib Palbociclib Olaparib Ulixertinib Erdafitinib Selumetinib Ensartinib | Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders (The Pediatric MATCH Screening Trial) | Recruiting | USA | CAN | AUS | 1 |
NCT03375307 | Phase II | Olaparib | Testing Olaparib in Patients With Advanced or Metastatic (Cancer That Has Spread) Bladder Cancer and Other Genitourinary Tumors With DNA-Repair Genetic Changes | Recruiting | USA | 0 |
NCT03587662 | Phase II | Gemcitabine + Ixazomib Doxorubicin + Gemcitabine + Ixazomib | Ixazomib, Gemcitabine, and Doxorubicin in Treating Patients With Locally Advanced or Metastatic Kidney Cancer | Active, not recruiting | USA | 0 |
NCT05347212 | Phase II | Nivolumab + Relatlimab | Phase II Trial of Immunotherapy in Patients With Carcinomas Arising From the Renal Medulla | Active, not recruiting | USA | 0 |