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68th Annual Meeting of the German Society of Neurosurgery (DGNC)
7th Joint Meeting with the British Neurosurgical Society (SBNS)

German Society of Neurosurgery (DGNC)

14 - 17 May 2017, Magdeburg

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Surgery as part of a multimodal therapy in a rapid progressive extraosseus Ewing sarcoma of the cervical spine

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  • Athanasios K. Petridis - Heinrich Heine University Duesseldorf, Department of Neurosurgery, Duesseldorf, Deutschland
  • Jan Frederick Cornelius - Neurochirurgische Klinik, Universitätsklinikum Düsseldorf, Heinrich Heine Universität, Duesseldorf, Deutschland
  • Hans-Jakob Steiger - Universitätsklinikum Düsseldorf, Neurochirurgische Klinik, Düsseldorf, Deutschland
  • Richard Bostelmann - Neurochirurgische Klinik, Universitätsklinik Düsseldorf, Duesseldorf, Deutschland

Deutsche Gesellschaft für Neurochirurgie. Society of British Neurological Surgeons. 68. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), 7. Joint Meeting mit der Society of British Neurological Surgeons (SBNS). Magdeburg, 14.-17.05.2017. Düsseldorf: German Medical Science GMS Publishing House; 2017. DocP 073

doi: 10.3205/17dgnc636, urn:nbn:de:0183-17dgnc6365

Published: June 9, 2017

© 2017 Petridis et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.

Outline

Text

Objective: Primary extraosseous Ewing sarcomas (EESs) are an extremely rare pathological entity. Less than 32 cases have been reported in the literature. Here we report an uncommon case with very rapid progression in the cervical region with extra- and intradural involvement.

Methods: Case presentation.

Results: A previously healthy 29-year-old man complained of right-sided radiculopathy (C7). Magnetic resonance imaging showed an enhancing foraminal, sandglass shaped neurinoma-like lesion. Surgery revealed an intra- and extra-dural lesion, which was histologically diagnosed as Ewing sarcoma. Despite gross total resection, there was a massive symptomatic tumor recurrence within 6 weeks. A second gross total resection was realized. The patient was treated according to the EURO E.W.I.N.G.-Protocol (VIDE) and recovered very well (progression-free interval during therapy). Several decompressive re-surgeries were realized with adjuvant radio-chemotherapy. At the last follow-up (17 months after initial surgery) the patient was in remission with a good quality of live.

Conclusion: This case is to illustrate that despite extensive therapeutic efforts, the progression-free survival in case of primary EES may be very short. To maintain neurological function and good quality of live as long as possible, a multimodal strategy with surgery and chemo-, radiotherapy seems to be adequate.