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INTRODUCTION: Intramedullary metastasis of Ewing sarcoma is extremely rare. Here, we report an adult case of cervical intramedullary recurrent Ewing sarcoma after a 10-year disease-free survival after the initial surgery for a thoracic lesion. CASE PRESENTATION: A 39-year-old man with a history of surgery and chemoradiotherapy for thoracic Ewing sarcoma ten years ago presented with neck pain and incomplete motor paralysis in the right upper extremity, which had suddenly appeared three months before. Cervical magnetic resonance imaging revealed a tear-drop-shaped intramedullary lesion at the C3 level accompanied by diffuse edematous change. Because of the rapid progression of his myelopathy, he underwent surgery for this intramedullary lesion. Intraoperatively, the tumor exhibited an orangish exophytic appearance. The unclearness of the tumor boundary compelled us to perform a partial resection. The histopathology showed the tumor comprised small round atypical cells with immunoreactivity for Nkx2.2 and CD99, diagnosing a metastatic Ewing sarcoma. Postoperatively, although his myelopathy improved transiently and adjuvant chemotherapy radiation was undergone, he died of cranial dissemination of the tumor two months and a half later. DISCUSSION: To our knowledge, 31 cases of primary and only 4 cases of recurrent intramedullary spinal Ewing sarcoma have been reported to date; however, this is the first case of recurrent intramedullary Ewing sarcoma with a 10-year disease-free survival. Sadly, the prognosis of the current case was extremely poor. There is no clear treatment guideline for recurrent intramedullary Ewing sarcoma because of its rarity, and further collection of similar cases would be required.

Ewing sarcoma is a rare connective tissue tumor characterized by the translocation of the EWS gene, mainly between chromosome 11 and 22, giving rise to gene re-arrangements between the EWS gene and various members of the ETS gene family. Multi-agent chemotherapy has improved the outcome for patients with localized Ewing sarcoma, but survival of patients with recurrent/metastatic disease remains poor. An exploratory two-stage, single-arm Phase II multicenter trial of the synthetic alkaloid, PM00104, was conducted in patients with recurrent Ewing sarcoma. The primary end point of the trial was objective response rate. PM00104 was administered at a dose of 2 mg/m(2) on Days 1, 8 and 15 of a 4 week cycle. Seventeen patients were recruited. No objective responses were reported in the 16 patients evaluable for efficacy. Recruitment was closed without proceeding to the second stage of the trial. Four patients achieved stable disease as best response, and in two of these patients the stabilization was longer than 4 months. The median progression-free survival was 1.8 months (95 % CI, 0.9-3.5 months) and median overall survival was not reached (95%CI, 56.2 % at censored data). Pharmacokinetics in patients with Ewing sarcoma was similar to that previously reported in other patient populations. PM00104 showed modest activity in Ewing sarcoma at 2 mg/m(2) on a weekly schedule. There remains an unmet need for effective therapies for patients with advanced/metastatic Ewing sarcoma.