Ewing Sarcoma of the Chest Wall: Prognostic Factors of Multimodal Therapy Including En Bloc Resection

Abstract

Background: Radiotherapy has long been the treatment of choice for local control of Ewing sarcoma of the chest wall (ESCW). However, there is debate regarding the use of surgery versus radiotherapy. The objective of this study was to identify risk factors that may affect long-term outcomes of nonmetastatic ESCW treated with preoperative chemotherapy (CT) followed by en bloc resection and adjuvant CT or chemoradiation.

Methods: Between 1996 and 2014, 30 patients with a median age of 25 years (SD ± 8.9 years) were treated at Marie-Lannelongue Hospital in Le Plessis-Robinson, France. Adjuvant therapy was used in 27 patients: CT for 6, chemoradiation for 20, and radiotherapy for 1. Patients' demographics, treatment data, tumor features, and outcomes were collected.

Results: In this cohort of patients who received multimodal therapy, including neoadjuvant CT and en bloc resection, there was no postoperative mortality. Eight patients (27%) experienced postoperative complications. Resection included at least one rib (n = 27) and the sternum (n = 1) or the spine (n = 8). Negative and microscopic disease resections were achieved in 28 and 2 patients, respectively. Tumor viability (TV) was ≤5% in 18 patients (60%). In patients with TV >5% at definitive histologic examination, adjuvant chemoradiation was associated with a better long-term outcome than was treatment with adjuvant CT alone. The 5-year overall survival and disease-free survival rates were 60.7% and 41.0%, respectively, with a median survival of 87 months. By univariate analysis, TV >5% and pleural extension at diagnosis were associated with poorer long-term survival (p < 0.05).

Conclusions: Multimodality treatment of ESCW, including neoadjuvant CT followed by en bloc resection and adjuvant CT or chemoradiation, is associated with excellent long-term outcomes.