Primary Intradural Extramedullary Extra-OsseousEwing’s Sarcoma of Cervical Spine (C5-C7)

A Case Report and Review of Literature

  • Srinivas DD Gubbala Kamineni Institute of Medical Sciences (KIMS) Sreepuram, Narketpalli, Telangana, India
  • Sridevi Mattaparti St Theresa Hospital. Telangana, India
  • Bhavani . Kamineni Institute of Medical Sciences (KIMS) Sreepuram, Narketpalli, Telangana, India
Keywords: Intradural, extra-skeletal Ewing’s sarcoma, spine, nerve sheath tumor


Ewing's sarcoma is a rare aggressive tumor common in bone or skeletal component. Primary Intradural Extra-medullary Extra-Osseous Ewing sarcoma of cervical spine is extremely rare malignant neoplasm and only few cases have been reported in the literature. There is a paucity of data regarding the clinical features, natural course, pre-operative diagnosis, treatment modalities and post-operative management.  Herein, we report a case of a 22-year-old man who presented with inability to walk and numbness in both lower limbs. MRI findings suggested a possibility of nerve sheath tumor but was diagnosed as Intradural Extra-skeletal Ewing sarcoma on histopathological analysis. The literature with regard to primary spinal intradural extra-skeletal Ewing sarcoma was reviewed.

Author Biographies

Srinivas DD Gubbala, Kamineni Institute of Medical Sciences (KIMS) Sreepuram, Narketpalli, Telangana, India
Department of Pathology
Sridevi Mattaparti, St Theresa Hospital. Telangana, India
Department of Pathology
Bhavani ., Kamineni Institute of Medical Sciences (KIMS) Sreepuram, Narketpalli, Telangana, India
Department of Pathology


1.Hufana V, Tan JSH, Tan KK. Microsurgical treatment for spinal tumors. Singapore med J. 2005; 46:74-77.
2. Porchet F, Sajadi A, Villemure JG: [Spinal tumors: clinical aspects, classification and surgical treatment]. Praxis (Bern 1994). 2003 Nov 5;92(45):1897-905.
3.Osbom AG. Handbook of neuroradiology. St Louis: Mosby; 1991. p. 380‑2.
4.Abul‑Kasim K, Thurnher MM, McKeever P, Sundgren PC. Intradural spinal tumours: Current classification and MRI features. Neuroradiology 2008;50:301‑14.
5.Duong LM, McCarthy BJ, McLendon RE, Dolecek TA, Kruchko C, Douglas LL, et al. Desc riptive epidemiology of malignant and nonmalignant primary spinal cord, spinal meninges, and cauda equina tumors, United States, 2004–2007. Cancer 2012;118:4220‑7.
6.Seppälä MT, Haltia MJ, Sankila RJ, Jääskeläinen JE, Heiskanen O. Long‑term outcome after removal of spinal schwannoma: A clinicopathological study of 187 cases. J Neurosurg 1995;83:621‑6.
7. Stawicki SP, Guarnaschelli JJ: Intradural extramedullary spinal cord tumors: A retrospective study of tumor types, locations, and surgical outcomes. The Internet Journal of Neurosurgery ISSN: 1528-8285 4:2, 2007.
8. Haresh KP, Chinikkatti SK, Prabhakar R, Rishi A, Rath GK, Sharma DN, Julka PK : A rare case of intradural extramedullary Ewing's sarcoma with skip metastasis in the spine. Spinal Cord. 2008 Aug;46(8):582-4.
9. Histologic Features and Prognosis of Spinal Intradural Extramedullary Ewing Sarcoma: Case Report, Literature Review, and Analysis of Prognosis. World Neurosurg. 2018 Jul;115:448-452.e2. doi: 10.1016/j.wneu.2018.04.015. Epub 2018 Apr 11.
10. Uesaka T, Amano T, Inamura T, Ikezaki K, Inoha S, Takamatsu M et al. Intradural, extramedullary spinal Ewing’s sarcoma in childhood. J Clin Neurosci 2003; 10: 122–125.
11. Bouffet E, Marec-Berard P, Thiesse P, Carrie C, Risk T, Jouvet A et al. Spinal cord compression by secondary epi- and intradural metastases in childhood. Childs Nerv Syst 1997; 13: 383–387.
12. Ewing Sarcoma of the Cervical Epidural Space Presenting with Tetraplegia: Case Report and Review of Literature. World Neurosurg. 2017 Nov;107:1046.e9-1046.e15. doi: 10.1016/j.wneu.2017.07.182. Epub 2017 Aug 7.
13. Goktepe AS, Alaca R, Mohur H, Coskun U. Paraplegia: an unusual presentation of Ewing's sarcoma. Spinal Cord 2002;40:367-9.
14. Kaspers GJ, Kamphorst W, van de Graaff M, van Alphen HA, Veerman AJ. Primary spinal epidural extraosseous Ewing’s sarcoma.
15. 17. Song X, Choi J, Rao C, Nallu S, Nicastri AD. Primary Ewing sarcoma of lumbar spine with massive intraspinal extension. Pediatr Neurol. 2008;38:58–60.
16. Harimaya K, Oda Y, Matsuda S, et al. Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature. Spine 2003;28:e408-12.
17. Kim SW, Shin H. Primary Intradural Extraosseous Ewing's Sarcoma. J Korean Neurosurg Soc 2009;45:179-81.
Case Report