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The purpose of this prospective study was to achieve excellent event\u2010free survival (EFS) and overall survival (OS) while preserving renal tissue through preoperative chemotherapy, completing definitive surgery by 12\u00a0weeks from diagnosis, and modifying postoperative chemotherapy based on histologic response.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The treating institution identified whether a predisposition syndrome existed. Patients underwent a central review of imaging studies through the biology and classification study AREN03B2 and then were eligible to enroll on AREN0534. Patients were treated with induction chemotherapy determined by localized or metastatic disease on imaging (and histology if a biopsy had been undertaken). Surgery was based on radiographic response at 6 or 12\u00a0weeks. Further chemotherapy was determined by histology. Patients who had stage III or IV disease with favorable histology received radiotherapy as well as those who had stage I through IV anaplasia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total, 34 patients were evaluable, including 13 males and 21 females with a mean age at diagnosis of 2.79\u00a0years (range, 0.49\u20108.78\u00a0years). The median follow\u2010up was 4.49\u00a0years (range, 1.67\u20108.01\u00a0years). The underlying diagnosis included Beckwith\u2010Wiedemann syndrome in 9 patients, hemihypertrophy in 9 patients, multicentric tumors in 10 patients, WAGR syndrome in 2 patients, a solitary kidney in 2 patients, Denys\u2010Drash syndrome in 1 patient, and Simpson\u2010Golabi\u2010Behmel syndrome in 1 patient. The 4\u2010year EFS and OS rates were 94% (95% CI, 85.2%\u2010100%) and 100%, respectively. Two patients relapsed (1 tumor bed, 1 abdomen), and none had disease progression during induction. According to Response Evaluation Criteria in Solid Tumor 1.1 criteria, radiographic responses included a complete response in 2 patients, a partial response in 21 patients, stable disease in 11 patients, and progressive disease in 0 patients. Posttherapy histologic classification was low\u2010risk in 13 patients (including the 2 complete responders), intermediate\u2010risk in 15 patients, and high\u2010risk in 6 patients (1 focal anaplasia and 5 blastemal subtype). Prenephrectomy chemotherapy facilitated renal preservation in 22 of 34 patients (65%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>A standardized approach of preoperative chemotherapy, surgical resection within 12\u00a0weeks, and histology\u2010based postoperative chemotherapy results in excellent EFS, OS, and preservation of renal parenchyma.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cncr.32958","type":"journal-article","created":{"date-parts":[[2020,5,27]],"date-time":"2020-05-27T09:19:15Z","timestamp":1590571155000},"page":"3516-3525","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":69,"title":["Results of Treatment for Patients With Multicentric or Bilaterally Predisposed Unilateral Wilms Tumor (AREN0534): A report from the Children's Oncology Group"],"prefix":"10.1002","volume":"126","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6161-1815","authenticated-orcid":false,"given":"Peter F.","family":"Ehrlich","sequence":"first","affiliation":[{"name":"Section of Pediatric Surgery CS Mott Children's Hospital University of Michigan Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yueh\u2010Yun","family":"Chi","sequence":"additional","affiliation":[{"name":"Department of Biostatistics University of Florida Gainesville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Murali M.","family":"Chintagumpala","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Oncology Texas Children's Hospital Cancer Center at Baylor College of Medicine Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fredric A.","family":"Hoffer","sequence":"additional","affiliation":[{"name":"Department of Radiology, Fred Hutchison Cancer Center University of Washington Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth J.","family":"Perlman","sequence":"additional","affiliation":[{"name":"Division of Pediatrics and Oncology Ann &amp; Robert H. 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