Artikel
Rapid progressive primary extraosseous Ewing sarcoma of the cervical intra- and epidural space
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Veröffentlicht: | 28. April 2011 |
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Gliederung
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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.