Background: The Ewing sarcoma family of tumors (ESFT) is a rare but curable bone neoplastic entity. The current standard of care involves chemotherapy and local disease control with surgery or radiation regardless of the extent of disease at presentation. Data that document the effectiveness of the current approach in the adult patient population are limited.
Methods: We performed a retrospective review including all ESFT patients older than 19 years of age who received systemic therapy between January 2002 and December 2013 at our institution. The main study outcome was overall survival; secondary outcomes were objective response rate, disease-free survival, and progression-free survival.
Results: Eighteen patients with ESFT were identified. The median overall survival for the entire group was 20.65 months (range 0.43-114.54). In patients with localized disease, the 1-, 2-, and 3-year survival rates were 90, 80, and 70%, respectively. Age was not correlated with overall survival (r = 0.58, p = 0.76). The 3-year disease-free survival rate was 70%. In patients with metastatic disease, the 1-year survival rate was 40%. In patients treated in the neoadjuvant and palliative setting with chemotherapy, we observed an objective response rate of 61.54%. The time to progression in patients with metastatic disease treated with chemotherapy ranged from 0.69 to 4.93 months.
Conclusion: In this group of adult patients with ESFT treated with multimodality therapy, the outcomes were similar to those reported in well-known larger clinical trials that typically included younger patients. Age was not associated with worse survival.
Keywords: Adult; Bone cancer; Chemotherapy; Ewing sarcoma; Ewing sarcoma family of tumors; Peripheral primitive neuroectodermal tumor; Systemic therapy.