• Raja K Kutty, Anilkumar Peethambaran, Balakrishnan S Sunilkumar, Krishna G Balachandran Nair, Paresh Korde, and Sourabh Kumar Jain.
• Department of Neurosurgery, Government Medical College, Thiruvananthapuram, Kerala, India. Electronic address: drrajakkutty@gmail.com.
• World Neurosurg. 2017 Nov 1; 107: 1046.e9-1046.e15.

BackgroundEwing sarcoma (ES) is among the most frequented extremity osseous tumor in childhood. It was first described by James Ewing as diffuse endotheliomas in 1921. The name Ewing sarcoma was coined by Oberling in 1928 as a tribute to the legend who described this disease. ES exists in osseous and extraosseous forms. It shares much of its molecular typing with primitive neuroectodermal tumor (PNET); hence, they are regarded as different ends of the same molecular spectrum. ES, extraosseous ES, PNET, and Askins tumor are the other members of this family termed the Ewing sarcoma family of tumors. Extraosseous ES has been described in various locations of the spine, but its occurrence in the cervical epidural region is uncommon.Case DescriptionA 12-year-old child presented to us with neck pain and progressive weakness of all 4 limbs. She was investigated and found to have a highly vascular lesion in the cervical epidural region extending from C2 to the C4 region. Embolization therapy was unsuccessful. She deteriorated and eventually had to undergo emergency surgery to decompress the spinal cord. The lesion was biopsied and diagnosed as extraosseous ES. After radiotherapy and chemotherapy, the patient regained most of the power in her limbs.ConclusionExtraosseous ES is a rare tumor of the cervical cord in this age group. We report this case to highlight the difficulties encountered in the management of this variant.Copyright © 2017 Elsevier Inc. All rights reserved.

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