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62nd Annual Meeting of the German Society of Neurosurgery (DGNC)
Joint Meeting with the Polish Society of Neurosurgeons (PNCH)

German Society of Neurosurgery (DGNC)

7 - 11 May 2011, Hamburg

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Rapid progressive primary extraosseous Ewing sarcoma of the cervical intra- and epidural space

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  • R. Bostelmann - Neurochirurgische Klinik der Heinrich Heine Universität, Düsseldorf, Deutschland
  • M. Leimert - Klinik für Neurochirurgie am Universitätsklinikum Carl Gustav Carus, Dresden, Deutschland
  • H.J. Steiger - Neurochirurgische Klinik der Heinrich Heine Universität, Düsseldorf, Deutschland
  • S. Eicker - Neurochirurgische Klinik der Heinrich Heine Universität, Düsseldorf, Deutschland
  • J.F. Cornelius - Neurochirurgische Klinik der Heinrich Heine Universität, Düsseldorf, Deutschland

Deutsche Gesellschaft für Neurochirurgie. Polnische Gesellschaft für Neurochirurgen. 62. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit der Polnischen Gesellschaft für Neurochirurgen (PNCH). Hamburg, 07.-11.05.2011. Düsseldorf: German Medical Science GMS Publishing House; 2011. DocP 080

doi: 10.3205/11dgnc301, urn:nbn:de:0183-11dgnc3018

Published: April 28, 2011

© 2011 Bostelmann et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.

Outline

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Objective: Primary extraosseous Ewing sarcomas (EESs) are a rare entity indeed. In the epidural space of the spine less than 20 cases have been reported in the literature. Here, we report about a very uncommon and unique case with rapid progression in the cervical region.

Methods: A previously healthy 29-year-old man complained of right-sided radiculopathy at C7. Magnetic resonance imaging showed a typical enhancing foraminal, sandglass-shaped neurinoma-like lesion. However, the operation revealed an intra- and extradural histologically proven Ewing sarcoma. Immunohistochemically, bright expression for MIC-2 was present. Before the diagnosis could be confirmed, there was a massive progression in the first postoperative weeks with hemiparesis, so that reoperation had to be performed.

Results: The patient had been treated according to the EURO E.W.I.N.G.-Protocol (VIDE) and the patient recovered very well. During therapy there was a progression free survival (clinically and MRI) for nearly six months. Afterwards the tumor recurred. This time, massive metastatic spread along the whole spinal axis occurred. To improve the patient’s quality of life we performed a decompressive spinal surgery and combined radiochemotherapy. Up till now there is clinically and radiologically partial remission.

Conclusions: This case illustrates that despite extensive therapeutic efforts, the progression-free survival in case of rapid recurrence of Ewing sarcoma can be very short, but nevertheless patients can profit with respect to quality of life.