{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T05:02:39Z","timestamp":1780376559284,"version":"3.54.1"},"reference-count":18,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,1,12]],"date-time":"2021-01-12T00:00:00Z","timestamp":1610409600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Clear cell sarcoma of the kidney (CCSK) is a rare malignant childhood renal tumour. Recently, the central nervous system (CNS) was found to be the most frequent site of relapse associated with a poor outcome. Optimal treatment strategies are scarce.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and Methods<\/jats:title><jats:p>Retrospective data analysis of all Austrian children with CCSK. They were enrolled in the Austrian\u2010Hungarian Wilms Tumour Study (AHWTS) 1989, the SIOP93\u201001 or the SIOP2001 study between 1990 and 2019. Demographic, diagnostic, treatment\u2010related variables and survival data were analysed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We identified 12 children with CCSK (M\u00a0=\u00a07, F\u00a0=\u00a05; median age 1.6\u00a0years). All had localised disease (stage I: 2; stage II: 2; stage III: 8) at diagnosis, and a first complete remission (CR1) was achieved in 12\/12. Six patients are in an ongoing CR1 (median follow\u2010up 10\u00a0years). Six other patients had a relapse (local 1; brain 5) a median time of 2.4\u00a0years from diagnosis. Two patients died of the disease 4\u00a0months and 2.8\u00a0years after first relapse. Four of five patients with CNS relapse are in CR2 with a median follow\u2010up time of 9.3\u00a0years after relapse diagnosis. Relapse treatment included a combination of chemotherapy, radiation and surgery. Two children received high\u2010dose chemotherapy followed by autologous stem cell rescue, and one child received intrathecal mafosphamide. Long\u2010term side effects after treatment were impaired tubular renal function (n\u00a0=\u00a04), cardiomyopathy (n\u00a0=\u00a01) and growth disorders (n\u00a0=\u00a01).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In this series, the brain was the most common site of relapse. Long\u2010term survival after recurrence was achievable with intensive multimodal therapy.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.28860","type":"journal-article","created":{"date-parts":[[2021,1,15]],"date-time":"2021-01-15T11:49:34Z","timestamp":1610711374000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Clear cell sarcoma of the kidney in Austrian children: Long\u2010term survival after relapse"],"prefix":"10.1002","volume":"68","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9275-5109","authenticated-orcid":false,"given":"Waltraud","family":"Friesenbichler","sequence":"first","affiliation":[{"name":"Department of Paediatric Haematology and Oncology St. Anna Children's Hospital Medical University  Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roswitha","family":"L\u00fcftinger","sequence":"additional","affiliation":[{"name":"Department of Paediatric Haematology and Oncology St. Anna Children's Hospital Medical University  Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriele","family":"Kropshofer","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University Hospital Innsbruck  Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Henkel","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Hospital of Barmherzige Schwestern Linz  Linz Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriele","family":"Amann","sequence":"additional","affiliation":[{"name":"Institute of Pathology Medical University of Vienna  Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1967-8343","authenticated-orcid":false,"given":"Rhoikos","family":"Furtw\u00e4ngler","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University Hospital Homburg\/Saar  Homburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norbert","family":"Graf","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University Hospital Homburg\/Saar  Homburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leo","family":"Kager","sequence":"additional","affiliation":[{"name":"Department of Paediatric Haematology and Oncology St. Anna Children's Hospital Medical University  Vienna Austria"},{"name":"Children's Cancer Research Institute  Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,1,12]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2012.04.009"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200001000-00002"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197805)41:5<1937::AID-CNCR2820410538>3.0.CO;2-U"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197810)42:4<1916::AID-CNCR2820420433>3.0.CO;2-R"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197810)42:4<1922::AID-CNCR2820420434>3.0.CO;2-Z"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrurol.2018.14"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2013.06.036"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/bjc.2014.291"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12254-012-0057-5"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-911X(199602)26:2<145::AID-MPO15>3.0.CO;2-H"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/mpo.1276"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2004.06.058"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.5858\/arpa.2018-0353-RS"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21131"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1701341"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-015-1176-7"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrc3634"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.29685"}],"container-title":["Pediatric Blood &amp; 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