Synovial sarcoma in childhood and adolescence: a retrospective series of 77 patients registered by the Children's Cancer and Leukaemia Group between 1991 and 2006.

BACKGROUND: The analysis of a contemporary multi-centre series of synovial sarcoma in children and young people describes the clinical characteristics, examines the effect of known prognostic factors and looks at outcome with particular emphasis on a conservative approach to treatment of localised disease. PROCEDURE: Data from 77 young patients with synovial sarcoma registered or treated by the Children's Cancer and Leukaemia Group (CCLG) between 1991 and 2006 were reviewed. The outcome of 21 patients with localised favourable disease who were treated with surgery only is described.
RESULTS: Five-year event-free survival (EFS) and overall survival (OS) for all 77 patients were 72% and 76%, respectively. In multivariate analysis survival was influenced by tumour invasiveness and size (T stage) and IRS group (T1b, P = 0.002; T2a, P = 0.041; T2b, P < 0.001; IRS III, P = 0.012). Patients with metastatic disease (IRS group IV) had a very poor prognosis; all but one has died. In IRS III patients, response to chemotherapy was seen in 38%. In a subgroup of 21 patients with small completed resected tumours no further primary treatment was given. Nineteen of this group were alive in first complete remission (CR).
CONCLUSION: The outcome of treatment for young patients in this national, multi-centre series of synovial sarcoma cases compares favourably to that in other contemporary series and presents novel information that demonstrates the validity of a conservative approach to further therapy in patients with small completely resected tumours.