Pediatric chest wall Ewing's sarcoma

Background: Chest wall tumors of primitive neuroectodermal origin (PNET, Ewing's sarcoma [ES]) are rare and have a poor prognosis. Multimodality therapy has improved survival results, and long-term survival is possible. Whether 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 identified. The median age was 12 years (range, 2.5 to 21 years). Median follow-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 neuropathy. Initial therapy consisted of biopsy and neoadjuvant chemotherapy followed by chest wall resection in 12 patients. Of the remaining 8 patients, 6 underwent biopsy, followed by chest wall resection and adjuvant chemotherapy, 1 underwent biopsy, chemotherapy, and resection of a lung nodule, and 1 underwent biopsy, chemotherapy, and a laminectomy and decompression procedure. All 20 patients were included in institutional-based trials using multiagent chemotherapy. Fifteen patients received radiation therapy with a median dose of 3,000 cGy. At last follow- up, 11 patients are alive and disease 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 wall resection. Seven of 11 (64%) survivors had radiation therapy. There was no surgical mortality. Twelve patients had treatment-related complications, 3 of which were related to surgical resection. There were no survivors among patients with recurrent disease. Three of the patients who died of disease had both local and distant recurrences, 4 patients had distant recurrence only, and one patient had local recurrence only. Only 4 of 9 (44%) patients who died were treated initially with chemotherapy followed by chest wall resection. All but 1 of those that died (89%) received initial radiation therapy. All 9 patients who did not survive received additional salvage radiotherapy as well. Conclusions: Long-term survival is possible with ES-PNET after complete chest wall resection. This may be facilitated by neoadjuvant chemotherapy. Long- term survival without radiation therapy is possible, and consideration of radiation therapy should be made on a case-by-case basis. (C) 2000 by W.B. Saunders Company.