Intradural extramedullary Ewing's sarcoma: A case report and review of the literature

Neurol Neurochir Pol. 2017 Jan-Feb;51(1):106-110. doi: 10.1016/j.pjnns.2016.11.006. Epub 2016 Nov 30.


Introduction: Extra-skeletal Ewing's sarcomas are very rare lesions to the spine surgeon, with the intradural, extramedullary lesions being even rarer. Herein we present a patient with an intradural, extramedullary form of Ewing's sarcoma and review the relevant literature. The medical records, operative reports, radiographical studies and histological examinations of a single patient are retrospectively reviewed.

Case report: A 31-year old male presented with back-pain, right-leg progressive paraparesis, and inability to walk. Both motor and sensory disturbances were revealed on the right leg at the clinical examination. Lumbar MRI showed two lesions. The first one was an intradural, extramedullary lesion at the L2-L3 level, while the second was smaller, located at the bottom of the dural sac. The patient underwent gross total resection of the L2-L3 lesion after a bilateral laminectomy. Histological examination was compatible with Ewing's sarcoma, and was verified by molecular analysis. No other extra-skeletal or skeletal lesion was found. A chemotherapy scheme was tailored to the patients' histological diagnosis. The patient presented with local recurrence and bone metastasis 2 years after his initial diagnosis. A second operation was performed and the follow up of the patient showed no disease progression 18 months after revision surgery.

Conclusion: The spine surgeon should be aware of the existence of such rare entities, in order to timely fulfill the staging process and institute the proper therapy. The management of patients with extra-skeletal Ewing's sarcomas involves professionals as members of a multidisciplinary team, all of which should co-operate for the patient's optimal outcome.

Keywords: Intradural extramedullary Ewing's sarcoma; Lumbar spine; Management; Surgery.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Humans
  • Male
  • Neoplasm Recurrence, Local / diagnostic imaging*
  • Neoplasm Recurrence, Local / surgery
  • Sarcoma, Ewing / diagnostic imaging*
  • Sarcoma, Ewing / drug therapy
  • Sarcoma, Ewing / surgery
  • Spinal Cord Neoplasms / diagnostic imaging*
  • Spinal Cord Neoplasms / drug therapy
  • Spinal Cord Neoplasms / surgery
  • Spinal Neoplasms / diagnostic imaging*
  • Spinal Neoplasms / drug therapy
  • Spinal Neoplasms / surgery