title: Synovial sarcoma of children and adolescents: the prognostic role of axial sites.
abstract: BACKGROUND
  The outcome of patients with non-extremity synovial sarcoma (SS) is generally worse than that of patients with limb tumours.
  
  
  METHODS
  The present study analysed a series of 115 consecutive SS patients treated in Italian paediatric protocols (period 1979-2005), mainly focusing on the 30 cases arising from 'axial' sites (16 head-neck, 8 trunk, 4 lung-pleura and 2 retroperitoneum).
  
  
  RESULTS
  Initial gross resection was achieved in 40% of axial cases and in 80% of limb SS (p<0.0001). Five-year EFS and overall survival (OS) were, respectively, 43.3% and 55.1% for axial SS, and 69.6% (p=0.0068) and 84.0% (p=0.0004) for extremity SS. Local progression/recurrence was the cause of treatment failure in 75% of relapsing patients axial disease.
  
  
  CONCLUSIONS
  Our findings emphasise that children and adolescents with SS originating at non-extremity locations have a worse prognosis than those with limb SS. Tumour site should be considered when defining a risk-adapted treatment strategy for SS.
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Via G. Venezian 1, Milan, Italy. andrea.ferrari@istitutotumori.mi.it
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Rita
authors.initials: R
authors.lastname: Alaggio
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Paola
authors.initials: P
authors.lastname: Collini
authors.forename: Angelo
authors.initials: A
authors.lastname: Rosolen
authors.forename: Cristina
authors.initials: C
authors.lastname: Meazza
authors.forename: Paolo
authors.initials: P
authors.lastname: Indolfi
authors.forename: Alberto
authors.initials: A
authors.lastname: Garaventa
authors.forename: Luigi
authors.initials: L
authors.lastname: De Sio
authors.forename: Paolo
authors.initials: P
authors.lastname: D'Angelo
authors.forename: Paolo
authors.initials: P
authors.lastname: Tamaro
authors.forename: Michela
authors.initials: M
authors.lastname: Casanova
authors.forename: Modesto
authors.initials: M
authors.lastname: Carli
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy / Q000401:mortality; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D007558:Italy / Q000453:epidemiology; D008297:Male; D009367:Neoplasm Staging; D018365:Neoplasm, Residual; D011446:Prospective Studies; D013584:Sarcoma, Synovial / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome