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Fluorescence in situ hybridization analysis to detect COL1A1\u2010PDGFB fusion genes was positive in 86% (12\/14) of evaluated patients. Primary resection was performed in all patients. Patients had IRS group I (<jats:italic>n<\/jats:italic>\u2009=\u200928), II (<jats:italic>n<\/jats:italic>\u2009=\u20099), and III (<jats:italic>n<\/jats:italic>\u2009=\u20092); not available (<jats:italic>n<\/jats:italic>\u2009=\u20091). To achieve complete remission (CR), a secondary resection was performed in 18 patients resulting in microscopically complete (R0, <jats:italic>n<\/jats:italic>\u2009=\u200934\/40) and microscopically incomplete (R1, <jats:italic>n<\/jats:italic>\u2009=\u20095\/40) resection. All patients achieved CR. The 5\u2010year event\u2010free survival (EFS) and overall survival was 86% (\u00b112; CI, 95%) and 100% (\u00b10; CI, 95%), respectively. R0 resection\/IRS I was significantly favorable for the 5\u2010year EFS. Local relapse occurred after a median time of 1.1 years (range, 0.04\u20105.1) in 15% (6\/40) after CR. All patients with RD underwent resection and achieved CR. Three patients had fibrosarcomatous DFSP, two were alive after R0 resection.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Complete surgical resection is mandatory to prevent relapse of DFSP.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jso.25943","type":"journal-article","created":{"date-parts":[[2020,5,20]],"date-time":"2020-05-20T05:50:19Z","timestamp":1589953819000},"page":"263-272","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Dermatofibrosarcoma protuberans in children and adolescents: Primary and Relapsed disease\u2014Experience of the Cooperative Weichteilsarkomstudiengruppe (CWS)"],"prefix":"10.1002","volume":"122","author":[{"given":"Julia","family":"Krewer","sequence":"first","affiliation":[{"name":"Department of Pediatric Surgery and Urology University of Frankfurt  Frankfurt 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f\u00fcr Kinder\u2010, Jugend\u2010 und Frauenmedizin, Olgahospital, Institute of Radiology  Stuttgart Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0665-6268","authenticated-orcid":false,"given":"Monika","family":"Scheer","sequence":"additional","affiliation":[{"name":"Klinikum Stuttgart\u2010Olgahospital, Stuttgart Cancer Center, Zentrum f\u00fcr Kinder\u2010, Jugend\u2010 und Frauenmedizin, Pediatrics 5 (Oncology, Hematology, Immunology)  Stuttgart Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"M\u00fcnter","sequence":"additional","affiliation":[{"name":"Klinikum Stuttgart, Institute of Radiotherapy  Stuttgart Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan S.","family":"Bielack","sequence":"additional","affiliation":[{"name":"Klinikum Stuttgart\u2010Olgahospital, Stuttgart Cancer Center, Zentrum f\u00fcr Kinder\u2010, Jugend\u2010 und Frauenmedizin, Pediatrics 5 (Oncology, Hematology, Immunology)  Stuttgart Germany"},{"name":"Department of Pediatric Hematology and Oncology University of Muenster  Muenster Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joerg","family":"Fuchs","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery and Urology University Children's Hospital  Tuebingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix","family":"Niggli","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology University of Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simone","family":"Hettmer","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, Faculty of Medicine University of Freiburg  Freiburg 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