Synovial sarcoma of children and adolescents: the prognostic role of axial sites.
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.
Ferrari
Andrea
A
Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Via G. Venezian 1, Milan, Italy. andrea.ferrari@istitutotumori.mi.it
Bisogno
Gianni
G
Alaggio
Rita
R
Cecchetto
Giovanni
G
Collini
Paola
P
Rosolen
Angelo
A
Meazza
Cristina
C
Indolfi
Paolo
P
Garaventa
Alberto
A
De Sio
Luigi
L
D'Angelo
Paolo
P
Tamaro
Paolo
P
Casanova
Michela
M
Carli
Modesto
M
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