Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome.
OBJECTIVE
To identify prognostic factors related to outcome in 219 children and adolescents with synovial sarcoma.


METHODS
We combined the experiences of the four following research groups: Cooperative Weichteilsarkomastudie Group, Germany (n = 95); St. Jude Children's Research Hospital, Memphis, TN (n = 49); Istituto Nazionale dei Tumori, Milan, Italy (n = 33); and The University of Texas M.D. Anderson Cancer Center, Houston, TX (n = 42). Kaplan-Meier and Cox proportional hazard analyses were performed.


RESULTS
The median age at diagnosis was 13 years (range, 1 to 20 years), and the median follow-up was 6.6 years (range, 0.5 to 30.7 years). The estimated 5-year overall survival and event-free survival rates for the entire group were 80% +/- 3% (SE) and 72% +/- 3%, respectively. A previously unreported interaction between tumor size and invasiveness was observed that statistically significantly related to outcome. In multivarible analysis, patients with T1B and T2B disease (hazard ratio [HR] = 5.6, 95% confidence interval (CI), 1.9 to 16.2; and HR = 5.9, 95% CI, 2.1 to 16.4, respectively) or Intergroup Rhabdomyosarcoma Study (IRS) Clinical Group III and IV disease (HR = 2.7, 95% CI, 1.2 to 6.5; and HR = 14.1, 95% CI, 4.3 to 31.3, respectively) had poor overall survival. Treatment with radiotherapy was related to improved overall survival (HR = 0.4; 95% CI, 0.2 to 0.7). In IRS Group III patients, objective response to chemotherapy (18 of 30, 60%) correlated with improved survival.


CONCLUSIONS
Clinical group, tumor size, and invasiveness are important prognostic factors. Multicenter randomized clinical trials are needed to determine both the effect of chemotherapy on survival and the necessity of local radiotherapy in patients with completely resected tumors.
Okcu
Mehmet Fatih
MF
Baylor College of Medicine, Texas Children's Cancer Center, 6621 Fannin, CC 1510.00, Houston, TX 77030-2399, USA. mfokcu@txccc.org
Munsell
Mark
M
Treuner
Joern
J
Mattke
Adrian
A
Pappo
Alberto
A
Cain
Alvida
A
Ferrari
Andrea
A
Casanova
Michela
M
Ozkan
Alp
A
Raney
Beverly
B
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005858:Germany; D006801:Humans; D007223:Infant; D007558:Italy; D008297:Male; D015999:Multivariate Analysis; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012307:Risk Factors; D013584:Sarcoma, Synovial / Q000175:diagnosis / Q000401:mortality* / Q000532:radiotherapy; D016019:Survival Analysis; D013714:Tennessee; D013781:Texas; D016896:Treatment Outcome; D014481:United States