Outcomes of Pediatric Non‐Rhabdomyosarcoma Soft‐Tissue Sarcomas of the Extremities

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BACKGROUND AND OBJECTIVES: Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) account for less than 4% of pediatric cancers. Treatment and outcomes have been described in adults, but pediatric data are lacking. The purpose of this study is to review our institution's outcomes of extremity NRSTS in pediatric patients. METHODS: Ninety-eight (56 male: 42 female) pediatric patients with NRSTS of the extremity at our institution were reviewed. Mean age at diagnosis was 11 ± 5 years, and mean follow-up was 12 ± 10 years. The most common diagnosis was synovial sarcoma (27%), and 60% were high-grade. RESULTS: Treatment included surgical resection in all patients, with 33% receiving radiation and 37% receiving systemic therapy. 20-year local recurrence- (LRFS) and metastatic disease-free survival (MDFS) were 91% and 91%, respectively, with 92% disease specific survival (DSS). High grade tumors were associated with lower LRFS (p = 0.02) and DSS (p = 0.03). Tumor size < 5 cm demonstrated improved MDFS (p = 0.03), and stage 3 tumors showed worse MDFS (p < 0.01). CONCLUSIONS: Treatment of pediatric NRSTS was variable, with large, deep, high-grade tumors more likely to receive neoadjuvant and adjuvant treatments. Multidisciplinary treatment was associated with high rates of local control and durable survival. This study provides an extended follow-up of pediatric patients to identify late complications.

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