{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T06:53:57Z","timestamp":1784876037999,"version":"3.55.0"},"reference-count":28,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2023,4,17]],"date-time":"2023-04-17T00:00:00Z","timestamp":1681689600000},"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":[[2023,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Outcome of relapsed disease of localized rhabdomyosarcoma remains poor. An individual treatment approach considering the initial systemic treatment and risk group was included in the Cooperative Weichteilsarkom Studiengruppe (CWS) Guidance.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Second\u2010line chemotherapy (sCHT) ACCTTIVE based on anthracyclines (adriamycin, carboplatin, cyclophosphamide, topotecan, vincristine, etoposide) was recommended for patients with initial low\u2010 (LR), standard\u2010 (SR), and high\u2010risk (HR) group after initial treatment without anthracyclines. TECC (topotecan, etoposide, carboplatin, cyclophosphamide) was recommended after initial anthracycline\u2010based regimen in the very high\u2010risk (VHR) group. Data of patients with relapse (<jats:italic>n<\/jats:italic>\u00a0=\u00a068) registered in the European Soft Tissue Sarcoma Registry SoTiSaR (2009\u20132018) were retrospectively analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients of initial LR (<jats:italic>n<\/jats:italic>\u00a0=\u00a02), SR (<jats:italic>n<\/jats:italic>\u00a0=\u00a016), HR (<jats:italic>n<\/jats:italic>\u00a0=\u00a041), and VHR (<jats:italic>n<\/jats:italic>\u00a0=\u00a09) group relapsed. sCHT consisted of ACCTTIVE (<jats:italic>n<\/jats:italic>\u00a0=\u00a036), TECC (<jats:italic>n<\/jats:italic>\u00a0=\u00a012), or other (<jats:italic>n<\/jats:italic>\u00a0=\u00a015). Resection was performed in 40\/68 (59%) patients and\/or radiotherapy in 47\/68 (69%). Initial risk stratification, pattern\/time to relapse, and achievement of second complete remission were significant prognostic factors. Microscopically incomplete resection with additional radiotherapy was not inferior to microscopically complete resection (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.17). The 5\u2010year event\u2010free survival (EFS) and overall survival (OS) were 26% (\u00b112%) and 31% (\u00b114%). The 5\u2010year OS of patients with relapse of SR, HR, and VHR groups was 80% (\u00b121%), 20% (\u00b116%), and 13% (\u00b123%, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.008), respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Adapted systemic treatment of relapsed disease considering the initial risk group and initial treatment is reasonable. New treatment options are needed for patients of initial HR and VHR groups.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.30363","type":"journal-article","created":{"date-parts":[[2023,4,17]],"date-time":"2023-04-17T07:48:37Z","timestamp":1681717717000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Second\u2010line treatment of pediatric patients with relapsed rhabdomyosarcoma adapted to initial risk stratification: Data of the European Soft Tissue Sarcoma Registry (SoTiSaR)"],"prefix":"10.1002","volume":"70","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3472-9679","authenticated-orcid":false,"given":"Amadeus T.","family":"Heinz","sequence":"first","affiliation":[{"name":"Department of Pediatric Hematology and Oncology University Children\u00b4s Hospital Tuebingen  Tuebingen 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Centre Essen (WPE), University Medical Center Essen, West German Cancer Center (WTZ), German Cancer Consortium (DKTK)  Essen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guido","family":"Seitz","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery University Hospital Giessen\u2010Marburg  Marburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4138-4536","authenticated-orcid":false,"given":"Christian","family":"Vokuhl","sequence":"additional","affiliation":[{"name":"Section of Pediatric Pathology University Hospital Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc W.","family":"M\u00fcnter","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology Stuttgart Cancer Center Klinikum der Landeshauptstadt Stuttgart  Stuttgart Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristian W.","family":"Pajtler","sequence":"additional","affiliation":[{"name":"Hopp Children's Cancer Center Heidelberg (KiTZ) Heidelberg University  Heidelberg Germany"},{"name":"Division of Pediatric Neurooncology German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK), Heidelberg University  Heidelberg Germany"},{"name":"Department of Pediatric Oncology, Hematology, and Immunology Heidelberg University Hospital  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7281-8811","authenticated-orcid":false,"given":"Sabine","family":"Stegmaier","sequence":"additional","affiliation":[{"name":"Stuttgart Cancer Center Zentrum f\u00fcr Kinder\u2010, Jugend\u2010 und Frauenmedizin (Olgahospital), P\u00e4diatrie 5 (P\u00e4diatrische Onkologie, H\u00e4matologie, Immunologie), Klinikum der Landeshauptstadt Stuttgart  Stuttgart Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thekla","family":"von Kalle","sequence":"additional","affiliation":[{"name":"Stuttgart Cancer Center Zentrum f\u00fcr Kinder\u2010, Jugend\u2010 und Frauenmedizin (Olgahospital), Department of Radiology, Klinikum der Landeshauptstadt Stuttgart  Stuttgart Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian P.","family":"Kratz","sequence":"additional","affiliation":[{"name":"Pediatric Hematology and Oncology Hannover Medical School  Hannover Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4022-4917","authenticated-orcid":false,"given":"Jochen","family":"R\u00f6\u00dfler","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology and Oncology, Department of Pediatrics University Hospital Bern (Inselspital)  Bern Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gustaf","family":"Ljungman","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's 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