OUTCOMES OF PAEDIATRIC NON-RHABDOMYOSARCOMA SOFT-TISSUE SARCOMAS OF THE EXTREMITIES

Soft tissue sarcoma (STS) is rare, accounting for 1% of all malignancies and 7% of pediatric cancers. Treatment and outcomes have been described in adults, but pediatric data is lacking. The purpose of this study is to review our institutions treatment and outcome of STS in children. Eighty-four (46 male:38 female) pediatric patients with musculoskeletal STS of the extremity at our institution since 1980 were reviewed. Rhabdomyosarcoma was excluded. Mean age at diagnosis was 11±5 years, and mean follow-up was 13±10 years. The most common diagnosis was synovial sarcoma (26%), and 65% were high grade. Treatment included surgery alone (49%), surgery and radiation (11%), surgery and chemotherapy/immunotherapy (20%), or a combination of the three (20%). Limb salvage was performed in 83%. Margins were negative in 99%. Complications occurred in 12%, and reoperation was performed in the post-operative period in 15% while 5% underwent long term reconstruction. 10-year local and distant recurrence-free survival were 84% and 88%, respectively, with a 90% disease specific survival (Figure 1). There was no difference in survival based on systemic therapy (HR 3.4; 95% CI 0.9–12), and no difference in local recurrence based on radiation therapy (HR 0.7; 95% CI 0.2–2). STS in the pediatric population is rare and includes heterogenous pathologies. A multidisciplinary approach to treatment including surgery, radiation, and systemic therapy leads to definitive local control and durable survival in most patients with few complications. For any figures or tables, please contact authors directly.