{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T17:15:12Z","timestamp":1774631712621,"version":"3.50.1"},"reference-count":32,"publisher":"Public Library of Science (PLoS)","issue":"7","license":[{"start":{"date-parts":[[2024,7,9]],"date-time":"2024-07-09T00:00:00Z","timestamp":1720483200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"abstract":"<jats:sec id=\"sec001\">\n<jats:title>Background<\/jats:title>\n<jats:p>Although clear cell sarcoma of kidney (CCSK) is rare, it is the second most common renal tumor in children after Wilms\u2019 tumor. NWTS and SIOP are two major groups which had made tremendous efforts on renal tumors, but the strategies are different, for NWTS follows the upfront surgery principle providing definite pathology and the SIOP follows the upfront chemotherapy principle, each has its own advantages. Here we aimed to evaluate the outcomes of CCSK in China following NWTS strategies to analyze the prognostic factors.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"sec002\">\n<jats:title>Methods<\/jats:title>\n<jats:p>For this multicenter retrospective study, a total of 54 patients were enrolled from three children\u2019s hospitals, between April 2003 and December 2021. Treatment comprised upfront radical nephrectomy, followed by radiotherapy and intensive chemotherapy. Clinical records were regularly updated. Prognostic factors and survival rates were evaluated.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"sec003\">\n<jats:title>Results<\/jats:title>\n<jats:p>The 54 enrolled patients had a median age of 37 months (range, 4 months to 11.4 years). The stage distribution was 16% stage I (n = 9), 30% stage II (n = 16), 39% stage III (n = 21), and 15% stage IV (n = 8). Among stage IV, metastasis sites included the lung (n = 6), bone (n = 1), and intra-orbital\/cervical lymph node (n = 1). After a median follow-up of 5.6 years, the 5-year event-free survival (EFS) was 82.4\u00b15.4%, and overall survival was 88.1\u00b14.6%. The EFS was 100% for stage I, 93.8 \u00b16.1% for stage II, 71.1\u00b110.0% for stage III, and 68.6\u00b118.6% for stage IV. Univariate analysis revealed that staging (III\/IV), tumor rupture, and inferior vena cava tumor thrombus were inferior prognostic factors. Multivariate analysis revealed that tumor rupture was independent poor prognostic factor (<jats:italic>P<\/jats:italic> = 0.01, <jats:italic>HR<\/jats:italic> 5.9). Among relapsed patients, relapse occurred a median of 11 months after diagnosis (range, 4\u201341 months), and 50% (4\/8) achieved a second complete remission after multiple treatment. None of the six lung metastasis patients received lung RT, only one patient developed a relapse and was salvaged by RT after relapse.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"sec004\">\n<jats:title>Conclusions<\/jats:title>\n<jats:p>Tumor rupture was independent poor prognostic factor. Upfront surgery of NWTS strategies can make a definite pathology diagnosis, but how to reduce tumor rupture during surgery is important especially in developing countries. The outcomes of patients with stage I\u2013III CCSK in China were comparable to findings in other developed countries. Better outcomes were achieved in stage IV CCSK by using an intensive chemotherapy regimen including carboplatin, which require further confirmation by AREN0321. Lung RT may be safely omitted in selected patients who achieve a compete radiographic response after 6 weeks of systemic treatment (including surgery). Treatment should be encouraged even in CCSK cases with metastasis and relapse.<\/jats:p>\n<\/jats:sec>","DOI":"10.1371\/journal.pone.0306863","type":"journal-article","created":{"date-parts":[[2024,7,9]],"date-time":"2024-07-09T17:29:28Z","timestamp":1720546168000},"page":"e0306863","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":3,"title":["Outcomes of children with clear cell sarcoma of kidney following NWTS strategies in Shanghai China (2003\u20132021)"],"prefix":"10.1371","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8439-2707","authenticated-orcid":true,"given":"Anan","family":"Zhang","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaojun","family":"Yuan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shayi","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dongqing","family":"Xu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Can","family":"Huang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jing yan","family":"Tang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7592-4115","authenticated-orcid":true,"given":"Yijin","family":"Gao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"340","published-online":{"date-parts":[[2024,7,9]]},"reference":[{"issue":"14","key":"pone.0306863.ref001","doi-asserted-by":"crossref","first-page":"2219","DOI":"10.1016\/j.ejca.2012.04.009","article-title":"Clear cell sarcoma of the kidney: A review","volume":"48","author":"SLM Gooskens","year":"2012","journal-title":"Eur J Cancer"},{"issue":"5","key":"pone.0306863.ref002","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1038\/nrurol.2018.14","article-title":"Rationale for the treatment of children with CCSK in the UMBRELLA SIOP\u2013RTSG 2016 protocol","volume":"15","author":"SL Gooskens","year":"2018","journal-title":"Nat Rev Urol"},{"issue":"Suppl 2","key":"pone.0306863.ref003","doi-asserted-by":"crossref","first-page":"e30343","DOI":"10.1002\/pbc.30343","article-title":"Pediatric renal tumor epidemiology: Global perspectives, progress, and challenges","volume":"70","author":"J Libes","year":"2023","journal-title":"Pediatr Blood Cancer"},{"key":"pone.0306863.ref004","first-page":"1916","article-title":"Exclusion of certain renal neoplasms from the category of Wilms\u2019 tumor","volume":"42","author":"JM Kidd","year":"1970","journal-title":"Am J Pathol"},{"issue":"1","key":"pone.0306863.ref005","doi-asserted-by":"crossref","first-page":"119","DOI":"10.5858\/arpa.2018-0353-RS","article-title":"Clear Cell Sarcoma of the Kidney","volume":"144","author":"AP Aldera","year":"2020","journal-title":"Arch Pathol Lab Med"},{"issue":"8","key":"pone.0306863.ref006","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1016\/S1470-2045(07)70241-3","article-title":"Part I: Primary malignant non-Wilms\u2019 renal tumours in children","volume":"8","author":"HU Ahmed","year":"2007","journal-title":"Lancet Oncol"},{"issue":"12","key":"pone.0306863.ref007","doi-asserted-by":"crossref","first-page":"2185","DOI":"10.1002\/pbc.25192","article-title":"Clear cell sarcoma of the kidney: patients\u2019 characteristics and improved outcome in developing countries","volume":"61","author":"W Zekri","year":"2014","journal-title":"Pediatr Blood Cancer"},{"issue":"10","key":"pone.0306863.ref008","doi-asserted-by":"crossref","first-page":"2132","DOI":"10.1200\/JCO.1994.12.10.2132","article-title":"Treatment of Children With Clear-Cell Sarcoma of the Kidney: A Report From the National Wilms\u2019 Tumor Study Group","volume":"12","author":"DM Green","year":"1994","journal-title":"J Clin Oncol"},{"issue":"5","key":"pone.0306863.ref009","doi-asserted-by":"crossref","first-page":"1898","DOI":"10.1002\/1097-0142(19930301)71:5<1898::AID-CNCR2820710529>3.0.CO;2-Q","article-title":"Ifosfamide\/etoposide combination in the treatment of recurrent malignant solid tumors of childhood. 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