{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T20:51:27Z","timestamp":1789678287672,"version":"4.0.1"},"reference-count":26,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2023,11,7]],"date-time":"2023-11-07T00:00:00Z","timestamp":1699315200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["U10CA098413"],"award-info":[{"award-number":["U10CA098413"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["U10CA098543"],"award-info":[{"award-number":["U10CA098543"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["U10CA180886"],"award-info":[{"award-number":["U10CA180886"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["U10CA180899"],"award-info":[{"award-number":["U10CA180899"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["U10CA180899\u201006"],"award-info":[{"award-number":["U10CA180899\u201006"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["U24CA114766"],"award-info":[{"award-number":["U24CA114766"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["U24CA196173"],"award-info":[{"award-number":["U24CA196173"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acsjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer"],"published-print":{"date-parts":[[2024,3,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Patients with stage IV favorable histology Wilms tumor (FHWT) with extrapulmonary metastases (EPM) constitute a small subset of patients with FHWT. Because of their rarity and heterogeneity, optimal FHWT treatment is not well understood. Children\u2019s Oncology Group protocol AREN0533 assigned patients with FHWT and EPM to intensified chemotherapy, regimen M, after initial DD\u20104A chemotherapy. To improve understanding of prognostic factors and best therapies, experiences of patients with EPM on AREN0533, as well as on protocols AREN03B2 and NWTS\u20105, were reviewed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Combined outcomes for patients with EPM from NWTS\u20105, AREN0533, and AREN03B2 were determined. Those treated on AREN0533 were compared with those treated on NWTS\u20105. Prognostic factors were explored in the pooled cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty\u2010seven patients with FHWT with EPM enrolled on AREN0533, 37 enrolled on NWTS\u20105, and 64 were followed only on AREN03B2. The pooled cohort of all 148 patients demonstrated a 4\u2010year event\u2010free survival (EFS) of 77.3% (95% CI, 70.8\u201384.4) and 4\u2010year overall survival of 88.9% (95% CI, 83.9\u201394.2). Four\u2010year EFS of patients with EPM treated on AREN0533 was 76.0% (95% CI, 64.6\u201389.4) vs 64.9% (95% CI, 51.7\u201382.2) on NWTS\u20105; hazard ratio,\u00a00.64, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.26; no difference in overall survival was observed. Increasing linear age and slow incomplete lung response were associated with worse EFS in a pooled cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Outcomes for patients with EPM are among the lowest for children with FHWT. Further trials with standardized surgical and radiation treatment to metastatic sites, and prospectively collected biologic and treatment details are needed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Clinical trial registration<\/jats:title><jats:p>Clinical Trials.gov identifiers: NCT00379340, NCT00898365, and NCT00002611.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cncr.35099","type":"journal-article","created":{"date-parts":[[2023,11,7]],"date-time":"2023-11-07T08:29:29Z","timestamp":1699345769000},"page":"947-961","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Treatment of children with favorable histology Wilms tumor with extrapulmonary metastases: A report from the COG studies AREN0533 and AREN03B2 and NWTSG study NWTS\u20105"],"prefix":"10.1002","volume":"130","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1524-3518","authenticated-orcid":false,"given":"Daniel J.","family":"Benedetti","sequence":"first","affiliation":[{"name":"Division of Hematology\/Oncology Department of Pediatrics Vanderbilt University Medical Center  Nashville Tennessee USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carly R.","family":"Varela","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology, Pediatric Specialists of Virginia Inova Fairfax Hospital  Fairfax Virginia USA"},{"name":"Janssen Research and Development  Spring House Pennsylvania USA"},{"name":"Division of Oncology Children's National Hospital and the George Washington University School of Medicine and Health Sciences  Washington District of Columbia 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"},{"name":"Children\u2019s Oncology Group  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brett","family":"Tornwall","sequence":"additional","affiliation":[{"name":"Glaukos Corp.  San Clemente California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David B.","family":"Dix","sequence":"additional","affiliation":[{"name":"Division of Oncology British Columbia Children\u2019s Hospital  Vancouver British Columbia Canada"}],"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":"Richard D.","family":"Glick","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery Zucker School of Medicine at Hofstra\/Northwell Cohen Children's Medical Center  New Hyde Park New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Kalapurakal","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology Northwestern University  Chicago Illinois USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Perlman","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Ann and Robert H. 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