Primary Spinal Epidural/Extramedullary Ewing Sarcoma in Young Female Patients.
DOI: 10.5435/JAAOSGlobal-D-19-00072; PMCID: PMC6903810
Primary spinal epidural/extramedullary ewing sarcoma (ES) is a rare extraosseous lesion. Extraosseous ES has a similar demographic as osseous ES, primarily affecting adolescents and young adults and male propensity. Reported 5-year survival is 0% to 37.5% for spinal extraosseous ES.
Methods: Two girls, 19 and 14 months old, presented with progressive lower extremity paraplegia and incontinence. Both had a compressive epidural/extramedullary mass without metastases and underwent decompression with multilevel laminectomy and tumor excision. Primary spinal epidural/extramedullary ES was diagnosed.
Results: Case 1 received 34 weeks of chemotherapy and radiation therapy, and case 2 received 14 cycles of chemotherapy and autologous stem cell rescue without radiation therapy. After more than 5- and 8-year follow-up, case 1 and case 2 are walking and disease-free, respectively.
Conclusion: These cases are the youngest presentation reported for primary spinal epidural/extramedullary ES and suggest that toddlers have a better prognosis for survival than older children and adolescents.
J Am Acad Orthop Surg Glob Res Rev
Fletcher, A. N., Marasigan, J. M., Hiatt, S. V., Anderson, J., Taboada, E., Schwend, R. M. Primary Spinal Epidural/Extramedullary Ewing Sarcoma in Young Female Patients. J Am Acad Orthop Surg Glob Res Rev 3, (2019).