Introduction: The role of surgery for local control in the multimodal management of Ewing’s sarcoma has substantially increased during the past 20 years. However, selection bias due to location (extremities vs axial skeleton) and relatively non-homogeneous treatment received by patients in multi-institutional trials may limit objective evaluation and comparison of the relative role of surgery and radiation therapy in this setting. Purpose of this study was to review a large series of patients homogeneously treated at a single institution.

Methods: 268 patients with non-metastatic Ewing’s sarcoma of the extremities treated by contemporary multimodal management were reviewed. Chemotherapy was administered according to 4 sequential protocols of adjuvant (1) and neoadjuvant (3) treatment. Local control consisted of surgery in 136 patients, surgery and radiation therapy in 70 patients, and radiation therapy in 60 patients. Two patients underwent only chemotherapy.

Results: The 5-year event-free survival (EFS) and overall survival (OS) were 62 and 69 per cent respectively. The rates of 5-year EFS and local control were significantly lower in patients treated with radiation therapy compared to patients treated by surgery or surgery and radiation therapy (48 vs 66 per cent, p=0.002; 80 vs 94 per cent, p= 0,0001). In group 3 (Radiation Therapy only) there were also 6 secondary malignancies.

Conclusion: Surgery was associated with better survival and local control in this series. In our opinion, surgery should always be considered in the local treatment of Ewing’s sarcoma of the extremities. Postoperative Radiation Therapy must be added in cases of inadequate surgical margins.

Introduction: Secondary sarcomas of bone are a rare group of usually high-grade malignant tumors developing over different pre-existing bone conditions, historically associated with worse prognosis than their primary counterpart. Purpose of this study was to investigate the factors affecting the outcome, with emphasis modern multi-modal management.

Methods: From approximately 30000 bone tumor cases filed at our Institution, 87 patients with secondary sarcoma were identified. Secondary chondrosarcomas were not included and all the patients had an high-grade lesion. Patients were divided in 3 groups: radiation induced sarcomas (29), sarcomas in Paget’s disease (34), and sarcomas in benign lesions (24). Of these patients, 67 were admitted for treatment while 20 were consultations; of the 67 patients admitted, 44 had stage II while 23 had stage III disease. There were 57 males and 30 females, average age was 52. Most common histotype was osteosarcoma (81). Treatment varied from none to combined multimodal management including chemotherapy and surgery.

Results: Cumulative survival is 37% at 10 years. Survival was affected by stage of disease (II=54%, III=0%; p< 0.00005), site (extremities 44%, pelvis-spine 0%; p< 0.00005), inclusion of surgery in the treatment plan (yes 51%, no 0%; p< 0.00005), and type of treatment (combined chemotherapy and surgery 56%, surgery 39%; p=0.07). No differences were noted between the 3 main etiologic groups.

Conclusions: Secondary sarcomas are a rare group malignant tumors with heterogeneous clinical features. While stage and site are still the most relevant prognostic factors, modern multimodal management including neoadjuvant chemotherapy and surgery seems to favourably affect the outcome.