{"doi":"10.1002/pbc.31490","title":"Rhabdoid Tumor of the Kidney and Soft Tissues: Results from National Wilms Tumor Study‐5 and Children's Oncology Group Study AREN0321","abstract":"PURPOSE: National Wilms Tumor Study-5 (NWTS-5) and AREN0321 evaluated the outcomes of children with rhabdoid tumor of the kidney (RTK) and malignant rhabdoid tumor of soft tissues (MRT). PATIENTS AND METHODS: Eligible patients with RTK were enrolled prospectively on NWTS-5 (1995-2002) and treated with carboplatin and etoposide alternating with cyclophosphamide (Regimen RTK). Patients with RTK or MRT were enrolled on AREN0321 (2005-2012) and received vincristine, doxorubicin, and cyclophosphamide alternating with carboplatin, cyclophosphamide, and etoposide (Regimens UH-1 or dose-reduced Revised UH-1). We report event-free survival (EFS) and overall survival (OS) from each study. RESULTS: Thirty patients received Regimen RTK on NWTS-5; on AREN0321, 20 received UH-1 and 19 received Revised UH-1. Patient and disease characteristics were statistically similar between studies. Patients on AREN0321 had significantly improved EFS and OS compared to those on NWTS-5 (4-year EFS = 23.1% vs. 16.7%; p = 0.020; 4-year OS = 30.6% vs. 20.0%; p = 0.014), mostly driven by patients with Stage I/II disease (p = 0.05). Median time to an event was 3.6 months on NWTS-5 compared to 7.2 months on AREN0321. There were no differences in EFS or OS by revised versus original Regimen UH-1 on AREN0321, or by renal versus extra-renal primary disease when the studies were pooled. CONCLUSIONS: The more intensive treatment regimen used on AREN0321 improved EFS and OS overall, a result driven by patients with Stage I/II disease. Despite this improvement, outcomes for patients with rhabdoid tumor remain unsatisfactory and there is a need for novel therapeutic strategies.","journal":"Pediatric Blood & Cancer","year":2024,"id":429476,"datarank":0.41960852373763613,"base_score":2.302585092994046,"endowment":2.302585092994046,"self_citation_contribution":0.3453877639491069,"citation_network_contribution":0.0742207597885292,"self_endowment_contribution":0.3453877639491069,"citer_contribution":0.0742207597885292,"corpus_percentile":55.782470797555504,"corpus_rank":5717,"citation_count":9,"citer_count":7,"citers_with_citation_signal":2,"citers_with_endowment":2,"datacite_reuse_total":0,"is_dataset":true,"is_dataset_confidence":0.553,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2024-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":381789,"name":"Lindsay A. 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Mullen","orcid":"0000-0002-6704-0002","position":10,"is_corresponding":false},{"id":473840,"name":"Najat C. Daw","orcid":"0000-0002-6941-8005","position":11,"is_corresponding":false},{"id":343607,"name":"Fredric A. Hoffer","orcid":null,"position":12,"is_corresponding":false},{"id":1232158,"name":"Zelig Tochner","orcid":null,"position":13,"is_corresponding":false},{"id":342738,"name":"Kenneth W. Gow","orcid":"0000-0003-0509-298X","position":14,"is_corresponding":false},{"id":343610,"name":"Eric J. Gratias","orcid":null,"position":15,"is_corresponding":false},{"id":796063,"name":"Deborah Ward","orcid":"0000-0002-3603-964X","position":16,"is_corresponding":false},{"id":1231588,"name":"James R. Anderson","orcid":"0000-0002-3107-0672","position":17,"is_corresponding":false},{"id":342741,"name":"Conrad V. Fernandez","orcid":"0000-0002-0746-8306","position":18,"is_corresponding":false},{"id":342742,"name":"Jeffrey S. Dome","orcid":"0000-0002-9037-369X","position":19,"is_corresponding":false},{"id":342739,"name":"James I. Geller","orcid":"0000-0001-5181-116X","position":0,"is_corresponding":true}],"reference_count":39,"raw_metadata":null,"created_at":"2026-07-19T01:59:06.668462Z","pmid":"39702900","pmcid":"PMC11801664","fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}