{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T19:49:50Z","timestamp":1790624990607,"version":"4.1.0"},"reference-count":20,"publisher":"Wiley","issue":"23","license":[{"start":{"date-parts":[[2020,9,14]],"date-time":"2020-09-14T00:00:00Z","timestamp":1600041600000},"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":["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"}]}],"content-domain":{"domain":["acsjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>To the authors' knowledge, AREN0321 is the first prospective clinical study of pediatric and adolescent renal cell carcinoma (RCC). Goals of the study included establishing epidemiological, treatment, and outcome data and confirming that patients with completely resected pediatric RCC, including lymph node\u2013positive disease (N1), have a favorable prognosis without adjuvant therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>From 2006 to 2012, patients aged &lt;30 years with centrally reviewed pathology of RCC were enrolled prospectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 68 patients were enrolled (39 of whom were male; median age of 13 years [range, 0.17\u201022.1 years]). Stage was classified according to the American Joint Committee on Cancer TNM stage seventh edition as stage I in 26 patients, stage II in 7 patients, stage III in 26 patients, and stage IV in 8 patients, and was not available in 1 patient. Sixty patients underwent resection of all known sites of disease, including 2 patients with stage IV disease. Surgery included radical nephrectomy (53 patients [81.5%]), partial nephrectomy (12 patients [18.5%]), and unknown (3 patients [4.4%]). Histology was TFE\u2010associated RCC (translocation\u2010type RCC; tRCC) in 40 patients, RCC not otherwise specified and\/or other in 13 patients, papillary RCC in 9 patients, and renal medullary carcinoma (RMC) in 6 patients. Lymph node status was N0 in 21 patients, N1 in 21 patients (tRCC in 15 patients, RMC in 3 patients, papillary RCC in 2 patients, and not otherwise specified and\/or other in 1 patient), and Nx in 26 patients. The 4\u2010year event\u2010free survival and overall survival rates were 80.2% (95% CI, 69.6%\u201090.9%) and 84.8% (95% CI, 75.2%\u201094.5%), respectively, overall and 87.5% (95% CI, 68.3%\u2010100%) and 87.1% (95% CI, 67.6%\u2010100%), respectively, for the 16 patients with N1M0 disease. Among patients presenting with metastases, 2 of 8 patients (2 of 5 patients with RMC) were alive (1 with disease) at the time of last follow\u2010up, including 1 patient who was lost to follow\u2010up (succinate dehydrogenase deficiency). The predominant RCC subtypes associated with mortality were tRCC and RMC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Favorable short\u2010term outcomes can be achieved without adjuvant therapy in children and adolescents with completely resected RCC, independent of lymph node status. A prospective study of patients with tRCC and RMC with M1 or recurrent disease is needed to optimize treatment.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cncr.33173","type":"journal-article","created":{"date-parts":[[2020,9,17]],"date-time":"2020-09-17T03:16:32Z","timestamp":1600312592000},"page":"5156-5164","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["A prospective study of pediatric and adolescent renal cell carcinoma: A report from the Children's Oncology Group AREN0321 study"],"prefix":"10.1002","volume":"126","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5181-116X","authenticated-orcid":false,"given":"James I.","family":"Geller","sequence":"first","affiliation":[{"name":"Division of Pediatric Oncology Cincinnati Children's Hospital Medical Center, University of Cincinnati Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5785-3704","authenticated-orcid":false,"given":"Nicholas G.","family":"Cost","sequence":"additional","affiliation":[{"name":"Division of Urology Department of Surgery University of Colorado School of Medicine the Children's Hospital Colorado Aurora Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yueh\u2010Yun","family":"Chi","sequence":"additional","affiliation":[{"name":"Department of Biostatistics, Children's Oncology Group Statistics and Data Center University of Florida Gainesville Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brett","family":"Tornwall","sequence":"additional","affiliation":[{"name":"Department of Biostatistics, Children's Oncology Group Statistics and Data Center University of Florida Gainesville Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariana","family":"Cajaiba","sequence":"additional","affiliation":[{"name":"Department of Pathology Anne and Robert H. Lurie Children's Hospital University of Michigan School of Medicine Ann Arbor Michigan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth J.","family":"Perlman","sequence":"additional","affiliation":[{"name":"Department of Pathology Ann and Robert H. Lurie Children's Hospital of Chicago Northwestern University Feinberg School of Medicine Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yeonil","family":"Kim","sequence":"additional","affiliation":[{"name":"Biostatistics and Research Decision Sciences Merck Research Laboratories Merck &amp; Company Inc Rahway New Jersey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth A.","family":"Mullen","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology Dana\u2010Farber Cancer Institute\/Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard D.","family":"Glick","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery Steven and Alexandra Cohen Medical Center of New York New York New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geetika","family":"Khanna","sequence":"additional","affiliation":[{"name":"Mallinckrodt Institute of Radiology Washington University School of Medicine in St. Louis St. Louis Missouri"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Najat C.","family":"Daw","sequence":"additional","affiliation":[{"name":"Department of Pediatrics The University of Texas MD Anderson Cancer Center Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6161-1815","authenticated-orcid":false,"given":"Peter","family":"Ehrlich","sequence":"additional","affiliation":[{"name":"Section of Pediatric Surgery Department of Surgery C.S. Mott Children's Hospital University of Michigan School of Medicine Ann Arbor Michigan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Conrad V.","family":"Fernandez","sequence":"additional","affiliation":[{"name":"Division of Pediatric Oncology IWK Health Centre Dalhousie University Halifax Nova Scotia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey S.","family":"Dome","sequence":"additional","affiliation":[{"name":"Division of Pediatric Oncology Children's National Hospital George Washington University School of Medicine and Health Sciences Washington DC"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the Children's Oncology Group (COG) Renal Tumor Committee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,9,14]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpurol.2009.04.007"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1586\/era.10.188"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.20548"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.23331"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.11422"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.31578"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21652"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.29368"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/annonc\/mdq029"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.22346"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.27766"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.26586"},{"key":"e_1_2_8_14_1","first-page":"3953","article-title":"Bevacizumab in pediatric patients: how safe is it?","volume":"31","author":"Pasquale MD","year":"2011","journal-title":"Anticancer Res"},{"key":"e_1_2_8_15_1","doi-asserted-by":"crossref","first-page":"e53","DOI":"10.4081\/rt.2009.e53","article-title":"Temsirolimus in the treatment of renal cell carcinoma associated with Xp11.2 translocation\/TFE gene fusion proteins: a case report and review of literature","volume":"1","author":"Parikh J","year":"2009","journal-title":"Rare Tumors"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0b013e318267fb12"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0b013e318266bf34"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000000774"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.urology.2007.03.037"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.urology.2007.06.1124"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.22611"}],"container-title":["Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fcncr.33173","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cncr.33173","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/cncr.33173","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acsjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cncr.33173","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T20:46:26Z","timestamp":1760301986000},"score":1,"resource":{"primary":{"URL":"https:\/\/acsjournals.onlinelibrary.wiley.com\/doi\/10.1002\/cncr.33173"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,9,14]]},"references-count":20,"journal-issue":{"issue":"23","published-print":{"date-parts":[[2020,12]]}},"alternative-id":["10.1002\/cncr.33173"],"URL":"https:\/\/doi.org\/10.1002\/cncr.33173","archive":["Portico"],"relation":{},"ISSN":["0008-543X","1097-0142"],"issn-type":[{"value":"0008-543X","type":"print"},{"value":"1097-0142","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,9,14]]},"assertion":[{"value":"2020-05-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-07-17","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-09-14","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}