Title

Primary Spinal Epidural/Extramedullary Ewing Sarcoma in Young Female Patients.

Creator(s)

Amanda N. Fletcher
Joanne Abby M. Marasigan
Stephen V. Hiatt
John Anderson, Children's Mercy HospitalFollow
Eugenio Taboada, Children's Mercy HospitalFollow
Richard M. Schwend, Children's Mercy HospitalFollow

Document Type

Article

Publication Date

11-27-2019

Identifier

DOI: 10.5435/JAAOSGlobal-D-19-00072; PMCID: PMC6903810

Abstract

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.

Journal Title

J Am Acad Orthop Surg Glob Res Rev

Volume

3

Issue

11

Recommended Citation

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).