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

BackgroundThe outcome of patients with non-extremity synovial sarcoma (SS) is generally worse than that of patients with limb tumours.MethodsThe 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).ResultsInitial 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.ConclusionsOur 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.