Abstract: |
Purpose: 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. Methods: 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. Results: 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. Conclusions: Tumor size, depth, invasion, and primary location affect survival in pediatric SS. The role of radiotherapy and chemotherapy for SS warrants future study. © 2012 Society of Surgical Oncology. |