Pediatric chest wall Ewing's sarcoma

Background: Chest wall tumors of primitive neuroectodermal origin (PNET, Ew ing's sarcoma [ES]) are rare and have a poor prognosis. Multimodality thera py has improved survival results, and long-term survival is possible. Wheth er adjuvant radiation therapy is uniformly beneficial remains unclear. Methods: A retrospective analysis of the authors' institutional experience between 1979 and 1998 was performed. Results: Twenty consecutive patients with PNET-ES of the chest wall were id entified. The median age was 12 years (range, 2.5 to 21 years). Median foll ow-up was 3 years (range, 7 months to 19.4 years). Seven patients presented with a mass, 12 with pain, 1 with respiratory distress, and 1 with a neuro pathy. Initial therapy consisted of biopsy and neoadjuvant chemotherapy fol lowed by chest wall resection in 12 patients. Of the remaining 8 patients, 6 underwent biopsy, followed by chest wall resection and adjuvant chemother apy, 1 underwent biopsy, chemotherapy, and resection of a lung nodule, and 1 underwent biopsy, chemotherapy, and a laminectomy and decompression proce dure. All 20 patients were included in institutional-based trials using mul tiagent chemotherapy. Fifteen patients received radiation therapy with a me dian dose of 3,000 cGy. At last follow-up, 11 patients are alive and diseas e free, with a median survival of 7.5 years (range, 7 months to 19.4 years) . Seven of 11 (64%) survivors had neoadjuvant therapy followed by chest wal l resection. Seven of 11 (64%) survivors had radiation therapy. There was n o surgical mortality. Twelve patients had treatment-related complications, 3 of which were related to surgical resection. There were no survivors amon g patients with recurrent disease. Three of the patients who died of diseas e had both local and distant recurrences, 4 patients had distant recurrence only, and one patient had local recurrence only. Only 4 of 9 (44%) patient s who died were treated initially with chemotherapy followed by chest wall resection. All but 1 of those that died (89%) received initial radiation th erapy. All 9 patients who did not survive received additional salvage radio therapy as well. Conclusions: Long-term survival is possible with ES-PNET after complete che st wall resection. This may be facilitated by neoadjuvant chemotherapy. Lon g-term survival without radiation therapy is possible, and consideration of radiation therapy should be made on a case-by-case basis. Copyright (C) 20 00 by W.B. Saunders Company.