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.
