title: Surgery alone is sufficient therapy for children and adolescents with low-risk synovial sarcoma: A joint analysis from the European paediatric soft tissue sarcoma Study Group and the Children's Oncology Group.
abstract: Multimodal risk-adapted treatment is used in paediatric protocols for synovial sarcoma (SS). Retrospective analyses suggest that low-risk SS patients can be safely treated with surgery alone, but no prospective studies have confirmed the safety of this approach. This analysis pooled data from the two prospective clinical trials to assess outcomes in SS patients treated with a surgery-only approach and to identify predictors of treatment failure.
  
  
  
  Patients with localised SS enrolled on the European paediatric Soft tissue sarcoma Study Group (EpSSG) NRSTS2005 and on the Children Oncology Group (COG) ARST0332 trials, treated with surgery alone were eligible for this analysis. Patients must have undergone initial complete resection with histologically free margins, with a grade 2 tumour of any size or a grade 3 tumour ≤5 cm.
  
  
  
  Sixty patients under 21 years of age were eligible for the analysis; 36 enrolled in the COG (from 2007 to 2012) and 24 in the EpSSG study (from 2005 to 2012). The 3-year event-free survival was 90% (median follow-up 5.2 years, range 1.9-9.1). All eight events were local tumour recurrence, whereas no metastatic recurrences were seen. All patients with recurrence were effectively salvaged, resulting in 100% overall survival.
  
  
  
  This joint prospective analysis showed that patients with adequately resected ≤5 cm SS, regardless of grade, can be safely treated with a surgery-only approach. Avoiding the use of adjuvant chemotherapy and radiotherapy in this low-risk patient population may decrease both short- and long-term morbidity and mortality.
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy. Electronic address: andrea.ferrari@istitutotumori.mi.it.
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.affiliation: Department of Biostatistics, University of Florida, Gainesville, FL, USA.
authors.forename: Yueh-Yun
authors.initials: YY
authors.lastname: Chi
authors.affiliation: Clinical Trials and Biostatistics Unit, IRCCS Istituto Oncologico Veneto, Padova, Italy.
authors.forename: Gian Luca
authors.initials: GL
authors.lastname: De Salvo
authors.affiliation: Department of Pediatric, Adolescent and Young Adult Oncology, Institut Curie, Paris, France.
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
authors.affiliation: Department of Pediatric Oncology, Royal Manchester Children's Hospital, Manchester, United Kingdom.
authors.forename: Bernadette
authors.initials: B
authors.lastname: Brennan
authors.affiliation: Pediatric Orthopaedics, Primary Children's Hospital, Salt Lake City, UT, USA.
authors.forename: R Lor
authors.initials: RL
authors.lastname: Randall
authors.affiliation: Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, TN, USA.
authors.forename: M Beth
authors.initials: MB
authors.lastname: McCarville
authors.affiliation: Pediatric Pathology, Children's Hospital Colorado, Aurora, CO, USA.
authors.forename: Jennifer O
authors.initials: JO
authors.lastname: Black
authors.affiliation: Pathology Department, Padova University, Padova, Italy.
authors.forename: Rita
authors.initials: R
authors.lastname: Alaggio
authors.affiliation: Hematology/Oncology, Seattle Children's Hospital, University of Washington, Fred Hutchinson Cancer Research Center, Seattle, WA, USA.
authors.forename: Douglas S
authors.initials: DS
authors.lastname: Hawkins
authors.affiliation: Pediatric Hematology and Oncology Division, Padova University, Padova, Italy.
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.affiliation: Department of Pediatrics, Stanford University School of Medicine, Palo Alto, CA, USA.
authors.forename: Sheri L
authors.initials: SL
authors.lastname: Spunt
mesh_terms: D000293:Adolescent; D059186:Chemoradiotherapy, Adjuvant / Q000379:methods / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D005121:Extremities; D006801:Humans; D053208:Kaplan-Meier Estimate; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy / Q000209:etiology; D011446:Prospective Studies; D012307:Risk Factors; D016879:Salvage Therapy; D013584:Sarcoma, Synovial / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*
keywords: Adjuvant treatment; Low risk; Pediatric sarcoma; Surgery; Synovial sarcoma