Synovial sarcoma of children and adolescents: the prognostic role of axial sites

Eur J Cancer. 2008 Jun;44(9):1202-9. doi: 10.1016/j.ejca.2008.03.016. Epub 2008 Apr 25.

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.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Child
  • Child, Preschool
  • Combined Modality Therapy / mortality
  • Disease-Free Survival
  • Female
  • Humans
  • Infant
  • Italy / epidemiology
  • Male
  • Neoplasm Staging
  • Neoplasm, Residual
  • Prospective Studies
  • Sarcoma, Synovial / mortality
  • Sarcoma, Synovial / pathology
  • Sarcoma, Synovial / therapy*
  • Treatment Outcome