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Presently, most of the information regarding clinical manifestations, treatment, and outcomes of rhabdoid tumors comes from large data registries and case series. Our current understanding of treatments for patients with rhabdoid tumors may inform how we approach patients with RTPS. In this manuscript, we review the genetic and clinical features of RTPS and, using known registry data and clinical reports, review associated tumor types ATRT, RTK, and EERT, closing with potential new approaches to treatment. We propose collaborative international efforts to study the use of SMARC (SWI\/SNF\u2010related, matrix\u2010associated, actin\u2010dependent regulator of chromatin)\u2010targeting agents, high\u2010dose consolidative therapy, and age\u2010based irradiation of disease sites in RTPS.<\/jats:p>","DOI":"10.1002\/pbc.30979","type":"journal-article","created":{"date-parts":[[2024,3,30]],"date-time":"2024-03-30T03:38:35Z","timestamp":1711769915000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Rhabdoid tumor predisposition syndrome: A historical review of treatments and outcomes for associated pediatric malignancies"],"prefix":"10.1002","volume":"71","author":[{"given":"Sarah","family":"Andres","sequence":"first","affiliation":[{"name":"Jacobs School of Medicine and Biomedical Sciences at the University at Buffalo  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen","family":"Huang","sequence":"additional","affiliation":[{"name":"Jacobs School of Medicine and Biomedical Sciences at the University at Buffalo  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret","family":"Shatara","sequence":"additional","affiliation":[{"name":"The Division of Hematology and Oncology St. Louis Children's Hospital, Washington University School of Medicine in St. Louis  St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8262-7469","authenticated-orcid":false,"given":"Mohamed S.","family":"Abdelbaki","sequence":"additional","affiliation":[{"name":"The Division of Hematology and Oncology St. Louis Children's Hospital, Washington University School of Medicine in St. Louis  St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Ranalli","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology\/Bone Marrow Transplant Department of Pediatrics Nationwide Children's Hospital, The Ohio State University  Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8312-6586","authenticated-orcid":false,"given":"Jonathan","family":"Finlay","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Radiation Oncology The Ohio State University College of Medicine  Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6074-9847","authenticated-orcid":false,"given":"Ajay","family":"Gupta","sequence":"additional","affiliation":[{"name":"Division of Pediatric Oncology Department of Pediatrics Roswell Park Comprehensive Cancer Center, University at Buffalo Jacobs School of Medicine and Biomedical Sciences  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,3,30]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1086\/302639"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1055\/s\u20100036\u20101579756"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440\u20101827.2006.01962.x"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2021.586288"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.2147\/cmar.S289544"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078\u20100432.CCR\u201017\u20100595"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1177\/1093526617749671"},{"key":"e_1_2_11_9_1","unstructured":"NemesK BensS BourdeautF et\u00a0al.Rhabdoid tumor predisposition syndrome. 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