title: Synovial sarcoma in children and adolescents: the European Pediatric Soft Tissue Sarcoma Study Group prospective trial (EpSSG NRSTS 2005).
abstract: BACKGROUND
  To report the results of the first European prospective nonrandomized trial dedicated to pediatric synovial sarcoma.
  
  
  METHODS
  From August 2005 to August 2012, 138 patients <21 years old with nonmetastatic synovial sarcoma were registered in 9 different countries (and 60 centers). Patients were treated with a multimodal therapy including ifosfamide-doxorubicin chemotherapy and radiotherapy, according to a risk stratification based on surgical stage, tumor size and site, and nodal involvement.
  
  
  RESULTS
  With a median follow-up of 52.1 months (range 13.8-104.4 months), event-free survival (EFS) was 81.9% and 80.7%, and overall survival (OS) was 97.2% and 90.7%, at 3 and 5 years, respectively. The only significant prognostic variable at univariate analysis was the risk group: 3-year EFS was 91.7% for low-risk, 91.2% for intermediate-risk, and 74.4% for high-risk cases. In 24 low-risk patients (completely resected tumor ≤5 cm in size) treated with surgery alone, there were two local relapses and no metastatic recurrences. Among 67 high-risk patients (unresected, or axial tumor or nodal involvement), 66 underwent surgery after neoadjuvant chemotherapy. Response to chemotherapy was 55.2%, including 22.4% cases with complete or major partial remissions, and 32.8% with minor partial remissions.
  
  
  CONCLUSIONS
  This study demonstrates that collaborative prospective studies on rare pediatric sarcomas are feasible even on a European scale, with excellent treatment compliance. The overall results of treatment were satisfactory, with higher survival rates than those previously published by pediatric groups. Nonetheless, larger, international projects are needed, based on a cooperative effort of pediatric and adult oncologists.
  
  
  BACKGROUND
  European Union Drug Regulating Authorities Clinical Trials No. 2005-001139-31.
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan andrea.ferrari@istitutotumori.mi.it.
authors.forename: A
authors.initials: A
authors.lastname: Ferrari
authors.affiliation: Clinical Trials and Biostatistics Unit, IRCCS Istituto Oncologico Veneto, Padova, Italy.
authors.forename: G L
authors.initials: GL
authors.lastname: De Salvo
authors.affiliation: Department of Pediatric Oncology, Royal Manchester Children's Hospital, Manchester, UK.
authors.forename: B
authors.initials: B
authors.lastname: Brennan
authors.affiliation: Department of Pediatric Oncology-Hematology, Erasmus MC/Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: M M
authors.initials: MM
authors.lastname: van Noesel
authors.affiliation: Clinical Trials and Biostatistics Unit, IRCCS Istituto Oncologico Veneto, Padova, Italy.
authors.forename: A
authors.initials: A
authors.lastname: De Paoli
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan.
authors.forename: M
authors.initials: M
authors.lastname: Casanova
authors.affiliation: Department of Pediatrics, CHC-Clin Espérance, Montegnée, Belgium.
authors.forename: N
authors.initials: N
authors.lastname: Francotte
authors.affiliation: Department of Diagnostic Paediatric Histopathology, Royal Manchester Children's Hospital, Manchester, UK.
authors.forename: A
authors.initials: A
authors.lastname: Kelsey
authors.affiliation: Department of Pathology, Padova University, Padova, Italy.
authors.forename: R
authors.initials: R
authors.lastname: Alaggio
authors.affiliation: Department of Pediatrics, Institut Gustave Roussy, Villejuif, France.
authors.forename: O
authors.initials: O
authors.lastname: Oberlin
authors.affiliation: Division of Pediatric Hematology and Oncology, Padova University, Padova, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Carli
authors.affiliation: Department of Pediatric Hematology Oncology, Meyer Children's Hospital, Rambam Health Care Campus, Haifa, Israel.
authors.forename: M
authors.initials: M
authors.lastname: Ben-Arush
authors.affiliation: Department of Pediatric Oncology, Institut D'Hematologie et D'Oncologie Pédiatrique, Centre Léon Bérard, Lyon, France.
authors.forename: C
authors.initials: C
authors.lastname: Bergeron
authors.affiliation: Department of Pediatric Oncology, Emma Children's Hospital-Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: J H M
authors.initials: JH
authors.lastname: Merks
authors.affiliation: Department of Pediatric Oncology, Children's Hospital for Wales, Heath Park, Cardiff.
authors.forename: M
authors.initials: M
authors.lastname: Jenney
authors.affiliation: Department of Pediatric Oncology, Royal Hospital for Children, University of Bristol, Bristol, UK.
authors.forename: M C
authors.initials: MC
authors.lastname: Stevens
authors.affiliation: Division of Pediatric Hematology and Oncology, Padova University, Padova, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Bisogno
authors.affiliation: Department of Pediatric, Adolescent and Young Adult Oncology, Institut Curie, Paris, France.
authors.forename: D
authors.initials: D
authors.lastname: Orbach
mesh_terms: D000293:Adolescent; D002648:Child; D005060:Europe / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D011446:Prospective Studies; D013584:Sarcoma, Synovial / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D012983:Soft Tissue Neoplasms / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy
keywords: adolescents; children; clinical trial; cooperative groups; pediatric sarcoma; synovial sarcoma