{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T20:49:56Z","timestamp":1790110196022,"version":"4.0.1"},"reference-count":19,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2025,1,13]],"date-time":"2025-01-13T00:00:00Z","timestamp":1736726400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["acsjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer"],"published-print":{"date-parts":[[2025,1,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>In the fifth National Wilms Tumor Study, patients received vincristine and dactinomycin (VA) without radiation for stage I focal anaplastic Wilms tumor (FAWT) and VA plus doxorubicin (DD4A) and radiation for stage II\u2013IV FAWT. Four\u2010year event\u2010free survival (EFS) and overall survival (OS) for stage I FAWT were 67.5% and 88.9% and for stage IV FAWT were 61.4% and 71.6%, respectively. Therapy intensification for stage I and IV FAWT was evaluated as secondary objectives in AREN0321.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Central review in the AREN03B2 Renal Tumors Classification, Biology, and Banking Study confirmed patient stage and tumor histology. Patients were then enrolled in AREN0321 and received DD4A with radiation for stage I\u2013III FAWT and vincristine, doxorubicin, cyclophosphamide, carboplatin, and etoposide (UH\u20101\/revised UH\u20101) with radiation for stage IV FAWT. Outcomes of patients with FAWT who were treated in AREN0321 (<jats:italic>n<\/jats:italic>\u00a0=\u00a025) and in AREN03B2 (<jats:italic>n<\/jats:italic>\u00a0=\u00a020) treated as per AREN0321 were analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In the pooled data analysis from AREN0321 and AREN03B2, 4\u2010year EFS and OS were both 100% for stage I\u2013II FAWT (<jats:italic>n<\/jats:italic>\u00a0=\u00a021), 82.4% (95% CI, 66.1%\u2013100%) and 87.8% (95% CI, 73.4%\u2013100%) for stage III FAWT (<jats:italic>n<\/jats:italic>\u00a0=\u00a017), respectively, and both 85.7% (95% CI, 63.3%\u2013100%) for stage IV FAWT (<jats:italic>n<\/jats:italic>\u00a0=\u00a07). Four patients enrolled in AREN0321 had events: treatment failure occurred in three patients with stage III FAWT, and one treatment\u2010related death was observed in a patient with stage IV FAWT following revised UH\u20101. No EFS or OS events occurred in patients with FAWT enrolled in AREN03B2 only.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Patients with stage I and II FAWT have outstanding survival when treated with DD4A and radiation. Intensification of therapy may have improved survival for stage IV FAWT, albeit with an increased toxicity risk.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cncr.35713","type":"journal-article","created":{"date-parts":[[2025,1,13]],"date-time":"2025-01-13T07:43:47Z","timestamp":1736754227000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Treatment of focal anaplastic Wilms tumor: A report from the Children's Oncology Group AREN0321 and AREN03B2 studies"],"prefix":"10.1002","volume":"131","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7784-0476","authenticated-orcid":false,"given":"Amy E.","family":"Armstrong","sequence":"first","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology Washington University School of Medicine  St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Najat C.","family":"Daw","sequence":"additional","affiliation":[{"name":"Division of Pediatrics The University of Texas MD Anderson Cancer Center  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lindsay A.","family":"Renfro","sequence":"additional","affiliation":[{"name":"Division of Biostatistics University of Southern California  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5181-116X","authenticated-orcid":false,"given":"James I.","family":"Geller","sequence":"additional","affiliation":[{"name":"Division of Oncology Cincinnati Children\u2019s Hospital Medical Center University of Cincinnati  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John A.","family":"Kalapurakal","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology Northwestern University  Chicago Illinois USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geetika","family":"Khanna","sequence":"additional","affiliation":[{"name":"Department of Radiology and Imaging Sciences Emory University and Children\u2019s Healthcare of Atlanta  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0269-3045","authenticated-orcid":false,"given":"Arnold C.","family":"Paulino","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology The University of Texas MD Anderson Cancer Center  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6853-6221","authenticated-orcid":false,"given":"Elizabeth J.","family":"Perlman","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Ann and Robert H. Lurie Children\u2019s Hospital of Chicago Northwestern University Feinberg School of Medicine  Chicago Illinois USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6161-1815","authenticated-orcid":false,"given":"Peter F.","family":"Ehrlich","sequence":"additional","affiliation":[{"name":"Section of Pediatric Surgery University of Michigan  Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth W.","family":"Gow","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery Stony Brook University  Stony Brook New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne B.","family":"Warwick","sequence":"additional","affiliation":[{"name":"Department of Pediatrics F. Edward H\u00e9bert School of Medicine Uniformed Services University  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2645-8266","authenticated-orcid":false,"given":"Paul E.","family":"Grundy","sequence":"additional","affiliation":[{"name":"Division of Immunology, Hematology, Oncology, Palliative Care and Environmental Interactions University of Alberta  Edmonton Alberta Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Conrad V.","family":"Fernandez","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology IWK Health Centre Dalhousie University  Halifax Nova Scotia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth A.","family":"Mullen","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology\/Oncology Dana\u2010Farber Cancer Institute and Boston Children\u2019s Hospital  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey S.","family":"Dome","sequence":"additional","affiliation":[{"name":"Division of Oncology Children's National Hospital and George Washington University School of Medicine and Health Sciences  Washington District of Columbia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the Children's Oncology Group AREN0321 and AREN03B2 Study Committees","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,1,13]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478\u2010199608000\u201000001"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2005.04.7852"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097\u20100142(197805)41:5<1937::aid\u2010cncr2820410538>3.0.co;2\u2010u"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0046\u20108177(88)80152\u20107"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.1994.12.10.2126"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2019.05.033"},{"key":"e_1_2_9_8_1","first-page":"753","volume-title":"Principles and Practice of Pediatric Oncology","author":"Fernandez CV","year":"2016"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2004.09.044"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.19.01265"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2017.77.1931"},{"key":"e_1_2_9_12_1","volume-title":"R: A Language and Environment for Statistical Computing","year":"2019"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2022.01.036"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/(sici)1096\u2010911x(199905)32:5<317::aid\u2010mpo1>3.0.co;2\u2010f"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.1993.11.6.1014"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2001.19.2.488"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/j.2040-4603.2019.tb00082.x"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.3390\/cancers13050976"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.34107"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.3390\/cancers13133142"}],"container-title":["Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/acsjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cncr.35713","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T10:25:44Z","timestamp":1760264744000},"score":1,"resource":{"primary":{"URL":"https:\/\/acsjournals.onlinelibrary.wiley.com\/doi\/10.1002\/cncr.35713"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1,13]]},"references-count":19,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2025,1,15]]}},"alternative-id":["10.1002\/cncr.35713"],"URL":"https:\/\/doi.org\/10.1002\/cncr.35713","archive":["Portico"],"relation":{},"ISSN":["0008-543X","1097-0142"],"issn-type":[{"value":"0008-543X","type":"print"},{"value":"1097-0142","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1,13]]},"assertion":[{"value":"2024-09-16","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-12-03","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-01-13","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e35713"}}