title: Pediatric and adolescent synovial sarcoma: multivariate analysis of prognostic factors and survival outcomes.
abstract: OBJECTIVE
  Treatment of synovial sarcoma (SS) is challenging because of its unpredictable clinical behavior. We reviewed our institutional experience with pediatric SS to identify prognostic indicators and survival outcomes.
  
  
  METHODS
  We retrospectively reviewed all pediatric/adolescent patients (age<22 years) with confirmed SS treated from 1970 to 2010. Patient and clinical characteristics were evaluated for prognostic significance and survival outcomes.
  
  
  RESULTS
  We identified 111 patients. The median age was 15.4 years. Sixty-seven tumors (60%) were monophasic, 42 (38%) were biphasic, and 2 (2%) were of unknown histology. Median follow-up was 5.3 years (range 0.8-36.8 years), 5-year overall survival (OS) was 73%, and 10-year OS was 65%. Greater tumor size (stratified as ≤5 cm, >5 cm, or ≥10 cm) (P=0.001) and depth (P=0.03) correlated with decreased OS. Primary tumor location in the upper extremity correlated with increased OS when compared with lower-extremity and central lesions (P=0.05). Bone and/or neurovascular invasion negatively impacted survival (P=0.02). Multivariate analysis revealed that tumor size (trichotomized) was the dominant and sole factor in discriminating survival risk. Neither radiotherapy nor chemotherapy correlated with improved 5-year survival.
  
  
  CONCLUSIONS
  Tumor size, depth, invasion, and primary location affect survival in pediatric SS. The role of radiotherapy and chemotherapy for SS warrants future study.
authors.affiliation: Pediatric Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA.
authors.forename: Eric J
authors.initials: EJ
authors.lastname: Stanelle
authors.forename: Emily R
authors.initials: ER
authors.lastname: Christison-Lagay
authors.forename: John H
authors.initials: JH
authors.lastname: Healey
authors.forename: Samuel
authors.initials: S
authors.lastname: Singer
authors.forename: Paul A
authors.initials: PA
authors.lastname: Meyers
authors.forename: Michael P
authors.initials: MP
authors.lastname: La Quaglia
mesh_terms: D000293:Adolescent; D000328:Adult; D000671:Amputation, Surgical; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006258:Head and Neck Neoplasms / Q000473:pathology* / Q000628:therapy; D006801:Humans; D053208:Kaplan-Meier Estimate; D035002:Lower Extremity; D008297:Male; D015999:Multivariate Analysis; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local / Q000473:pathology*; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D013584:Sarcoma, Synovial / Q000473:pathology* / Q000556:secondary / Q000628:therapy*; D012983:Soft Tissue Neoplasms / Q000473:pathology* / Q000628:therapy*; D015996:Survival Rate; D013899:Thoracic Neoplasms / Q000473:pathology* / Q000628:therapy; D034941:Upper Extremity; D055815:Young Adult