Abstract

Introduction Primary Ewing sarcoma of the spine is a challenging tumor to treat because of a high rate of local recurrence. The optimum role of surgery in the treatment of Ewing sarcoma is not clearly defined. The objective of this study was to analyze factors relevant to survival and local control in surgically treated patients diagnosed with primary Ewing sarcoma of the spine. Materials and Methods A multi-institutional international ambispective with cross-sectional follow-up data was gathered about patients who underwent surgery for primary Ewing sarcoma of the spine. The primary outcomes assessed were 5-year survival and local recurrence. Multiple variables including the Enneking appropriateness of the surgery were evaluated for relationships to the primary outcomes. An Enneking appropriate procedure for Ewing sarcoma is an en bloc resection with negative margins. Survival and recurrence were analyzed using Kaplan–Meier curves and log-rank tests. Results A total of 59 patients diagnosed with primary Ewing sarcoma of the spine underwent surgery. Enneking appropriateness of surgery was known for 55 patients; 30 treated Enneking appropriately (EA) and 25 treated Enneking inappropriately (EI). The 5-year survival was 73% ( n = 22) for EA patients and 48% ( n = 12) for EI patients. Median survival was not achieved within 5-year postoperative for EA patients and was 2.2 years for EI patients ( p = 0.124). Local recurrence occurred in 20% ( n = 6) of patients with an EA procedure versus 48% ( n = 11) of patients with an EI procedure. The rate of local control was favorable for EA-treated patients compared with EI-treated patients ( p = 0.046). Conclusion Primary spinal Ewing sarcoma requires multidisciplinary treatment. An Enneking appropriate surgical procedure is associated with longer survival and better local control. An Enneking appropriate procedure, when feasible, should be the surgical treatment of choice for primary spinal Ewing sarcoma.

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