Modern multimodality therapy for pediatric nonorbital parameningeal sarcomas

Background Nonorbital parameningeal sarcomas are rare, aggressive tumors that arise predominantly in the pediatric population. The purpose of this study was to review their management and to clarify the role of surgery in their treatment. Methods Treatment, prognostic factors, and outcomes for patients over a 10-year period were reviewed. Results In all, 34 patients were identified. The most common tumor site was the maxillary sinus, and 73% were rhabdomyosarcoma. Six patients (18%) presented with positive nodal metastases and 3 patients (10%) presented with distant metastases. Overall, 18 patients were treated surgically. Locoregional recurrence rate was 43%; no patients developed distant recurrence. Five-year overall survival was 66.8%, disease-specific survival was 68.1%, and disease-free survival was 31.9%. Conclusions Pediatric nonorbital parameningeal sarcomas require aggressive multimodality treatment. Although chemotherapy and radiation constitute the treatment of choice in most cases, surgery may have a role as salvage therapy and in cases that fail to respond to chemoradiation.