Title

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

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

Share

COinS