{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T19:49:48Z","timestamp":1790624988040,"version":"4.1.0"},"reference-count":25,"publisher":"Wiley","issue":"13","license":[{"start":{"date-parts":[[2024,2,23]],"date-time":"2024-02-23T00:00:00Z","timestamp":1708646400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["acsjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer"],"published-print":{"date-parts":[[2024,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>On the fifth National Wilms Tumor Study, treatment for clear cell sarcoma of the kidney (CCSK) included combined vincristine, doxorubicin, cyclophosphamide, and etoposide (regimen I) plus radiation therapy (RT), yielding 5\u2010year event\u2010free survival (EFS) rates of 100%, 88%, 73%, and 29% for patients who had with stage I, II, III, and IV disease, respectively. In the Children\u2019s Oncology Group study AREN0321 of risk\u2010adapted therapy, RT was omitted for stage I disease if lymph nodes were sampled, and carboplatin was added for stage IV disease (regimen UH\u20101). Patients who had stage II\/III disease received regimen I with RT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Four\u2010year EFS was analyzed for patients enrolled on AREN0321 and on\u00a0those enrolled on AREN03B2 who received AREN0321 stage\u2010appropriate chemotherapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eighty\u2010two patients with CCSK enrolled on AREN0321, 50 enrolled on AREN03B2 only. The 4\u2010year EFS rate was 82.7% (95% confidence interval [CI], 74.8%\u201391.4%) for AREN0321 and 89.6% (95% CI, 81.3%\u201398.7%) for AREN03B2 only (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.28). When combining studies, the 4\u2010year EFS rates for patients who had stage I (<jats:italic>n<\/jats:italic>\u00a0=\u00a010), II (<jats:italic>n<\/jats:italic>\u00a0=\u00a047), III (<jats:italic>n<\/jats:italic>\u00a0=\u00a065), and IV (<jats:italic>n<\/jats:italic>\u00a0=\u00a010) disease were 90% (95% CI, 73.2%\u2013100.0%), 93.4% (95% CI, 86.4%\u2013100.0%), 82.8% (95% CI, 74.1%\u201392.6%), and 58.3% (95% CI, 34%\u2013100.0%), respectively. There were no local recurrences among seven patients with stage I disease who were treated without RT. One stage I recurrence occurred in the brain, which was the most common site of relapse overall. Among patients with local stage III tumors, neither initial procedure type, margin status, nor lymph node involvement were prognostic.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Patients with stage I CCSK had excellent outcomes without local recurrences when treated without RT. Patients with stage IV disease appeared to benefit from a carboplatin\u2010containing regimen, although their outcomes remained unsatisfactory. Further research is needed to improve outcomes for patients with\u00a0advanced\u2010stage disease (ClinicalTrials.gov identifiers NCT00335556 and NCT00898365).<\/jats:p><\/jats:sec>","DOI":"10.1002\/cncr.35266","type":"journal-article","created":{"date-parts":[[2024,2,23]],"date-time":"2024-02-23T22:24:55Z","timestamp":1708727095000},"page":"2361-2371","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Treatment and outcomes of clear cell sarcoma of the kidney: A report from the Children's Oncology Group studies AREN0321 and AREN03B2"],"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":"Lindsay A.","family":"Renfro","sequence":"additional","affiliation":[{"name":"Division of Biostatistics University of Southern California  Los Angeles California USA"},{"name":"Children\u2019s Oncology Group  Monrovia California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian","family":"Tfirn","sequence":"additional","affiliation":[{"name":"Children\u2019s Oncology Group  Monrovia California 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":"John A.","family":"Kalapurakal","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology Northwestern University  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":"Geetika","family":"Khanna","sequence":"additional","affiliation":[{"name":"Department of Radiology and Imaging Sciences Emory University Children\u2019s Healthcare of Atlanta  Atlanta Georgia 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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