Rib Ewing Sarcomas in Children and Young Adults: A Large National Retrospective Series

Abstract

Background and Purpose: Ewing sarcoma (ES) is the most prevalent malignant thoracic tumor in childhood and young adults. This study reports the outcome of a national cohort treated in an international prospective trial for a localized rib ES, with a long follow-up. Material and Methods: All the patients treated in a prospective trial (December 1999–April 2013) were included. Data were collected from a prospective database and supplemented by a retrospective review of the patients’ files. Local control alone rate, progression-free survival (PFS), and overall survival (OS) were assessed. Multivariate analyses were conducted to adjust for classical prognosis factors. Results: Eighty-two patients, median age 13.6 years, were included. All received induction chemotherapy. Surgery was completed (R0) in 88%. Overall, 66% had good histological response (GR). Radiotherapy (RT) was performed on the primary tumor in 28%, on the hemithorax in 13%, or on both in 32%. With a median follow-up of 12 years, 28 patients (34%) relapsed. Five-year local control rate was 95% (95%CI [89%–95%]), 5-year PFS was 73% (95%CI [62%–81%]), and 5-year OS was 79% (95%CI [69%–87%]). In multivariate analysis, pleural effusion was significantly associated with poor PFS (p = 0.049). In the case of R0 and GR (n = 41), no significant difference in PFS/OS (p = 0.16/p = 0.36) was reported according to adjuvant local RT. Overall, 38% of scoliosis and 7.3% of secondary cancer were reported. Conclusion: A multidisciplinary approach in high-risk patients can provide good local control in rib ES. In patients who have undergone R0 surgery and are GR, omitting adjuvant RT of the surgical bed may be discussed.