{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,14]],"date-time":"2026-11-14T10:27:40Z","timestamp":1794652060747,"version":"4.9.1"},"reference-count":29,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2022,6,22]],"date-time":"2022-06-22T00:00:00Z","timestamp":1655856000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,6,22]],"date-time":"2022-06-22T00:00:00Z","timestamp":1655856000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Urol"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Pediatric non-Wilms renal tumors (NWRTs), which comprise a small proportion of renal tumors, are a heterogeneous group of neoplasms with variable malignant potential, mortality, and response to treatment. We performed this study to determine the clinical characteristics, management and prognosis of children with Pediatric NWRTs.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Medical records of all patients (n\u2009=\u2009139) treated for NWRTs over a 12-year period (2008.01\u20132019.10) at a single center were reviewed retrospectively.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>The histopathological groups of NWRTs included malignant rhabdoid tumor of the kidney (MRTK) (n: 30, 21.6%), renal cell cancer (RCC) (n: 26,18.7%), clear cell sarcoma of the kidney (CCSK) (n: 24,17.3%), congenital mesoblastic nephroma (CMN) (n: 21,15.1%), cystic nephroma (CN) (n: 16,11.5%), metanephric tumors (n: 12, 8.6%), renal angiomyoliporma (RAML) (n: 3, 2.2%), renal primitive neuroectodermal tumor (n: 2, 1.4%), renal hemangioma (n: 2, 1.4%), inflammatory myofibroblastic tumor (n: 2, 1.4%), ossifying renal tumor of infancy (ORTI) (n: 1, 0.7%). The distribution of all malignant NWRTs, including MRTK, CCSK, RCC and PNET, according to stage was as follows: stages I (n\u2009=\u200926), II (n\u2009=\u200916), III (n\u2009=\u200929), and IV (n\u2009=\u200911). The summary table shows the treatment offered to children with NWRTs. A total of 123 children were followed up for an average of 42\u00a0months. Sixteen children were lost to follow-up. Tumor-free survival was observed in 94 children. One patient who suffered from RCC is currently receiving targeted therapy and survives with the tumor. Twenty-eight children (22.8%) died.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Pediatric NWRTs comprise 19.1% of all renal tumors in our single center. Most NWRTs can readily be distinguished using a range of immunohistochemical markers. Molecular genetic profiling has allowed much progress in the understanding of this group of tumors, making diagnosis and classification less difficult. The mainstay treatment of malignant NWRTs, including MRTK, CCSK, RCC and PNET, is comprehensive treatment. The mainstay treatment of benign NWRTs, including RAML, CN, ORTI, CMN, metanephric tumors, and renal hemangioma, is surgical resection alone and when the tumor diameter is smaller than 7\u00a0cm and the tumor locates in one pole, NSS can be performed.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12894-022-01042-3","type":"journal-article","created":{"date-parts":[[2022,6,22]],"date-time":"2022-06-22T13:03:10Z","timestamp":1655902990000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center"],"prefix":"10.1186","volume":"22","author":[{"given":"Yi Wei","family":"Fang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hong Cheng","family":"Song","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ning","family":"Sun","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei Ping","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,6,22]]},"reference":[{"issue":"5","key":"1042_CR1","doi-asserted-by":"publisher","first-page":"516","DOI":"10.1111\/j.1365-2559.2008.03110.x","volume":"54","author":"NJ Sebire","year":"2009","unstructured":"Sebire NJ, Vujanic GM. Paediatric renal tumours: recent developments, new entities and pathological features. Histopathology. 2009;54(5):516\u201328.","journal-title":"Histopathology"},{"issue":"2","key":"1042_CR2","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1016\/j.jss.2010.03.061","volume":"163","author":"Y Zhuge","year":"2010","unstructured":"Zhuge Y, Cheung MC, Yang R, Perez EA, Koniaris LG, Sola JE. Pediatric non-Wilms renal tumors: subtypes, survival, and prognostic indicators. J Surg Res. 2010;163(2):257\u201363.","journal-title":"J Surg Res"},{"issue":"3","key":"1042_CR3","doi-asserted-by":"publisher","first-page":"565","DOI":"10.1007\/s00268-011-1410-2","volume":"36","author":"PW Saula","year":"2012","unstructured":"Saula PW, Hadley GP. Pediatric non-Wilms\u2019 renal tumors: a third world experience. World J Surg. 2012;36(3):565\u201372.","journal-title":"World J Surg"},{"issue":"1","key":"1042_CR4","first-page":"18","volume":"50","author":"E Unal","year":"2020","unstructured":"Unal E, Yilmaz E, Ozcan A, Isik B, Karakukcu M, Turan C, Akgun H, Ozturk F, Coskun A, Ozdemir MA, et al. Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia Turkey. Turk J Med Sci. 2020;50(1):18\u201324.","journal-title":"Turk J Med Sci"},{"issue":"3","key":"1042_CR5","doi-asserted-by":"publisher","first-page":"268","DOI":"10.5152\/tud.2018.70120","volume":"44","author":"G Bozlu","year":"2018","unstructured":"Bozlu G, Citak EC. Evaluation of renal tumors in children. Turk J Urol. 2018;44(3):268\u2013327.","journal-title":"Turk J Urol"},{"issue":"5","key":"1042_CR6","doi-asserted-by":"publisher","first-page":"733","DOI":"10.1002\/pbc.22922","volume":"56","author":"MM van den Heuvel-Eibrink","year":"2011","unstructured":"van den Heuvel-Eibrink MM, van Tinteren H, Rehorst H, Coulombe A, Patte C, de Camargo B, de Kraker J, Leuschner I, Lugtenberg R, Pritchard-Jones K, et al. Malignant rhabdoid tumours of the kidney (MRTKs), registered on recent SIOP protocols from 1993 to 2005: a report of the SIOP renal tumour study group. Pediatr Blood Cancer. 2011;56(5):733\u20137.","journal-title":"Pediatr Blood Cancer"},{"issue":"30","key":"1042_CR7","doi-asserted-by":"publisher","first-page":"7641","DOI":"10.1200\/JCO.2004.00.8110","volume":"23","author":"GE Tomlinson","year":"2005","unstructured":"Tomlinson GE, Breslow NE, Dome J, Guthrie KA, Norkool P, Li S, Thomas PR, Perlman E, Beckwith JB, D\u2019Angio GJ, et al. Rhabdoid tumor of the kidney in the National Wilms\u2019 tumor study: age at diagnosis as a prognostic factor. J Clin Oncol. 2005;23(30):7641\u20135.","journal-title":"J Clin Oncol"},{"issue":"8","key":"1042_CR8","doi-asserted-by":"publisher","first-page":"737","DOI":"10.1111\/j.1442-2042.2010.02588.x","volume":"17","author":"M Baek","year":"2010","unstructured":"Baek M, Jung JY, Kim JJ, Park KH, Ryu DS. Characteristics and clinical outcomes of renal cell carcinoma in children: a single center experience. Int J Urol. 2010;17(8):737\u201340.","journal-title":"Int J Urol"},{"issue":"2","key":"1042_CR9","doi-asserted-by":"publisher","first-page":"98","DOI":"10.1097\/00125480-200103000-00006","volume":"8","author":"FS Balarezo","year":"2001","unstructured":"Balarezo FS, Joshi VV. Clear cell sarcoma of the pediatric kidney: detailed description and analysis of variant histologic patterns of a tumor with many faces. Adv Anat Pathol. 2001;8(2):98\u2013108.","journal-title":"Adv Anat Pathol"},{"issue":"7","key":"1042_CR10","doi-asserted-by":"publisher","first-page":"e26437","DOI":"10.1002\/pbc.26437","volume":"64","author":"SL Gooskens","year":"2017","unstructured":"Gooskens SL, Houwing ME, Vujanic GM, Dome JS, Diertens T, Coulomb-l\u2019Hermine A, Godzinski J, Pritchard-Jones K, Graf N, van den Heuvel-Eibrink MM. Congenital mesoblastic nephroma 50 years after its recognition: a narrative review. Pediatr Blood Cancer. 2017;64(7):e26437.","journal-title":"Pediatr Blood Cancer"},{"issue":"8","key":"1042_CR11","doi-asserted-by":"publisher","first-page":"730","DOI":"10.1016\/S1470-2045(07)70241-3","volume":"8","author":"HU Ahmed","year":"2007","unstructured":"Ahmed HU, Arya M, Levitt G, Duffy PG, Mushtaq I, Sebire NJ. Part I: primary malignant non-Wilms\u2019 renal tumours in children. Lancet Oncol. 2007;8(8):730\u20137.","journal-title":"Lancet Oncol"},{"issue":"7","key":"1042_CR12","doi-asserted-by":"publisher","first-page":"1301","DOI":"10.1016\/j.jpedsurg.2008.02.077","volume":"43","author":"D Miniati","year":"2008","unstructured":"Miniati D, Gay AN, Parks KV, Naik-Mathuria BJ, Hicks J, Nuchtern JG, Cass DL, Olutoye OO. Imaging accuracy and incidence of Wilms\u2019 and non-Wilms\u2019 renal tumors in children. J Pediatr Surg. 2008;43(7):1301\u20137.","journal-title":"J Pediatr Surg"},{"issue":"5","key":"1042_CR13","doi-asserted-by":"publisher","first-page":"353","DOI":"10.2350\/10-09-0902-OA.1","volume":"14","author":"J Deisch","year":"2011","unstructured":"Deisch J, Raisanen J, Rakheja D. Immunohistochemical expression of embryonic stem cell markers in malignant rhabdoid tumors. Pediatr Dev Pathol. 2011;14(5):353\u20139.","journal-title":"Pediatr Dev Pathol"},{"issue":"4","key":"1042_CR14","doi-asserted-by":"publisher","first-page":"539","DOI":"10.1016\/j.jpedsurg.2013.10.005","volume":"49","author":"HC Song","year":"2014","unstructured":"Song HC, Sun N, Zhang WP, He L, Fu L, Huang C. Biological characteristics of pediatric renal cell carcinoma associated with Xp11.2 translocations\/TFE3 gene fusions. J Pediatr Surg. 2014;49(4):539\u201342.","journal-title":"J Pediatr Surg"},{"issue":"10","key":"1042_CR15","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1016\/j.earlhumdev.2010.08.018","volume":"86","author":"M Powis","year":"2010","unstructured":"Powis M. Neonatal renal tumours. Early Hum Dev. 2010;86(10):607\u201312.","journal-title":"Early Hum Dev"},{"issue":"4","key":"1042_CR16","doi-asserted-by":"publisher","first-page":"472","DOI":"10.1097\/PAS.0000000000000816","volume":"41","author":"Y Li","year":"2017","unstructured":"Li Y, Pawel BR, Hill DA, Epstein JI, Argani P. Pediatric cystic nephroma is morphologically, immunohistochemically, and genetically distinct from adult cystic nephroma. Am J Surg Pathol. 2017;41(4):472\u201381.","journal-title":"Am J Surg Pathol"},{"issue":"9","key":"1042_CR17","doi-asserted-by":"publisher","first-page":"1267","DOI":"10.1038\/modpathol.2013.242","volume":"27","author":"LA Doros","year":"2014","unstructured":"Doros LA, Rossi CT, Yang J, Field A, Williams GM, Messinger Y, Cajaiba MM, Perlman EJ, Schultz KA, Cathro HP, Legallo RD, LaFortune KA, Chikwava KR, Faria P, Geller JI, Dome JS, Mullen EA, Gratias EJ, Dehner LP, Ashley Hill D. DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma. Mod Pathol. 2014;27(9):1267\u201380. https:\/\/doi.org\/10.1038\/modpathol.2013.242.","journal-title":"Mod Pathol"},{"issue":"9","key":"1042_CR18","doi-asserted-by":"publisher","first-page":"2455","DOI":"10.1093\/annonc\/mdt215","volume":"24","author":"S Zollner","year":"2013","unstructured":"Zollner S, Dirksen U, Jurgens H, Ranft A. Renal ewing tumors. Ann Oncol. 2013;24(9):2455\u201361.","journal-title":"Ann Oncol"},{"issue":"4","key":"1042_CR19","doi-asserted-by":"publisher","first-page":"826","DOI":"10.2214\/AJR.17.17973","volume":"209","author":"BK Park","year":"2017","unstructured":"Park BK. Renal angiomyolipoma: radiologic classification and imaging features according to the amount of fat. AJR Am J Roentgenol. 2017;209(4):826\u201335.","journal-title":"AJR Am J Roentgenol"},{"issue":"11","key":"1042_CR20","doi-asserted-by":"publisher","first-page":"1004","DOI":"10.1016\/j.prp.2016.08.008","volume":"212","author":"W Guan","year":"2016","unstructured":"Guan W, Yan Y, He W, Qiao M, Liu Y, Wang Y, Jiang B, Yu W, Wang R, Wang L. Ossifying renal tumor of infancy (ORIT): the clinicopathological and cytogenetic feature of two cases and literature review. Pathol Res Pract. 2016;212(11):1004\u20139.","journal-title":"Pathol Res Pract"},{"issue":"34","key":"1042_CR21","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000011994","volume":"97","author":"Y Wang","year":"2018","unstructured":"Wang Y, Qi X, Xue P, Zhang C, Cai C, Tu C, Wang K. Inflammatory myofibroblastic tumors in the kidney and abdominal wall mimicking malignancy: a case report. Medicine (Baltimore). 2018;97(34): e11994.","journal-title":"Medicine (Baltimore)"},{"issue":"9","key":"1042_CR22","doi-asserted-by":"publisher","first-page":"842","DOI":"10.1016\/S1470-2045(07)70276-0","volume":"8","author":"HU Ahmed","year":"2007","unstructured":"Ahmed HU, Arya M, Levitt G, Duffy PG, Sebire NJ, Mushtaq I. Part II: treatment of primary malignant non-Wilms\u2019 renal tumours in children. Lancet Oncol. 2007;8(9):842\u20138.","journal-title":"Lancet Oncol"},{"issue":"1","key":"1042_CR23","doi-asserted-by":"publisher","DOI":"10.4081\/rt.2012.e6","volume":"4","author":"M Kourti","year":"2012","unstructured":"Kourti M, Hatzipantelis E, Zaramboukas T, Tragiannidis A, Petrakis G, Athanassiadou-Piperopoulou F. Rare non-Wilms\u2019 tumors in children. Rare Tumors. 2012;4(1): e6.","journal-title":"Rare Tumors"},{"issue":"4","key":"1042_CR24","doi-asserted-by":"publisher","first-page":"1456","DOI":"10.1016\/S0022-5347(05)00671-3","volume":"175","author":"A Cook","year":"2006","unstructured":"Cook A, Lorenzo AJ, Pippi JL, Salle MB, Cartwright LM, Bagi D, Farhat W, Khoury A. Pediatric renal cell carcinoma: single institution 25-year case series and initial experience with partial nephrectomy. J Urol. 2006;175(4):1456\u201360. https:\/\/doi.org\/10.1016\/S0022-5347(05)00671-3.","journal-title":"J Urol"},{"issue":"1","key":"1042_CR25","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.27450","volume":"66","author":"NL Seibel","year":"2019","unstructured":"Seibel NL, Chi YY, Perlman EJ, Tian J, Sun J, Anderson JR, Ritchey ML, Thomas PR, Miser J, Kalapurakal JA, et al. Impact of cyclophosphamide and etoposide on outcome of clear cell sarcoma of the kidney treated on the National Wilms Tumor Study-5 (NWTS-5). Pediatr Blood Cancer. 2019;66(1): e27450.","journal-title":"Pediatr Blood Cancer"},{"issue":"6","key":"1042_CR26","first-page":"743","volume":"8","author":"Q Sun","year":"2018","unstructured":"Sun Q, Miao B, Lao X, Yuan P, Cai J, Zhan H. Primitive neuroectodermal tumor of the kidney at the advanced stage: a case series of eight Chinese patients. Mol Clin Oncol. 2018;8(6):743\u20138.","journal-title":"Mol Clin Oncol"},{"issue":"4","key":"1042_CR27","first-page":"333","volume":"8","author":"D Taheri","year":"2014","unstructured":"Taheri D, Soleimani N, Fesharakizadeh M, Dolatkhah S, Kabiri M, Gholipour A. Inflammatory myofibroblastic tumor: report of a rare case in kidney. Iran J Kidney Dis. 2014;8(4):333\u20135.","journal-title":"Iran J Kidney Dis"},{"key":"1042_CR28","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/315170","volume":"2013","author":"R Horazdovsky","year":"2013","unstructured":"Horazdovsky R, Manivel JC, Cheng EY. Surgery and actinomycin improve survival in malignant rhabdoid tumor. Sarcoma. 2013;2013: 315170.","journal-title":"Sarcoma"},{"issue":"2","key":"1042_CR29","doi-asserted-by":"publisher","first-page":"428","DOI":"10.1016\/j.ijrobp.2012.04.023","volume":"85","author":"JA Kalapurakal","year":"2013","unstructured":"Kalapurakal JA, Perlman EJ, Seibel NL, Ritchey M, Dome JS, Grundy PE. Outcomes of patients with revised stage I clear cell sarcoma of kidney treated in National Wilms Tumor studies 1\u20135. Int J Radiat Oncol Biol Phys. 2013;85(2):428\u201331.","journal-title":"Int J Radiat Oncol Biol Phys"}],"container-title":["BMC Urology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12894-022-01042-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12894-022-01042-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12894-022-01042-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,6,22]],"date-time":"2022-06-22T13:04:22Z","timestamp":1655903062000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcurol.biomedcentral.com\/articles\/10.1186\/s12894-022-01042-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,6,22]]},"references-count":29,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2022,12]]}},"alternative-id":["1042"],"URL":"https:\/\/doi.org\/10.1186\/s12894-022-01042-3","relation":{},"ISSN":["1471-2490"],"issn-type":[{"value":"1471-2490","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,6,22]]},"assertion":[{"value":"22 February 2022","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 June 2022","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 June 2022","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This study was conducted in accordance with the principles of the Declaration of Helsinki,\u00a0and this study was approved by the Institutional Review Board of Beijing Children's Hospital,Capital Medical University with waiver of informed consent.\u00a0Because of the retrospective nature of the study, patient consent for inclusion was waived.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"Not applicable.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"89"}}