Pediatric and adolescent synovial sarcoma: multivariate analysis of prognostic factors and survival outcomes

Treatment of synovial sarcoma (SS) is challenging because of its unpredictable clinical behavior. We reviewed our institutional experience with pediatric SS to identify prognostic indicators and survival outcomes.

We retrospectively reviewed all pediatric/adolescent patients (age<22 years) with confirmed SS treated from 1970 to 2010. Patient and clinical characteristics were evaluated for prognostic significance and survival outcomes.

We identified 111 patients. The median age was 15.4 years. Sixty-seven tumors (60%) were monophasic, 42 (38%) were biphasic, and 2 (2%) were of unknown histology. Median follow-up was 5.3 years (range 0.8-36.8 years), 5-year overall survival (OS) was 73%, and 10-year OS was 65%. Greater tumor size (stratified as ≤5 cm, >5 cm, or ≥10 cm) (P=0.001) and depth (P=0.03) correlated with decreased OS. Primary tumor location in the upper extremity correlated with increased OS when compared with lower-extremity and central lesions (P=0.05). Bone and/or neurovascular invasion negatively impacted survival (P=0.02). Multivariate analysis revealed that tumor size (trichotomized) was the dominant and sole factor in discriminating survival risk. Neither radiotherapy nor chemotherapy correlated with improved 5-year survival.

Tumor size, depth, invasion, and primary location affect survival in pediatric SS. The role of radiotherapy and chemotherapy for SS warrants future study.