The aim of this study is to present a rare case of chordoma in the odontoid process in which the tumor involved the odontoid process and compressed the spinal cord at the craniocervical junction. We report on the effectiveness and successful outcome of anterior microscopic tumor resection combined with posterior occipitocervical fixation and review the current standard treatment. A 39-year-old man presented with sudden dyspnea and quadriparesis caused by an unknown tumor compression at C2. Radiographic examination revealed a large destructive mass at C2 and heterogeneous enhancement. The patient received urgent surgical intervention by microscopic-assisted anterior tumor resection and posterior spinal fixation from the occiput to the C5 level. The pathohistologic reports for cytokeratins, epithelial membrane antigen, and S-100 protein were positive. The final diagnosis was chordoma of the odontoid process. At the 2-year follow-up, the patient's condition had improved, and a postoperative MRI showed no indication of tumor regrowth. Chordoma of the odontoid process or C2 body is very rare. The current standard management is wide tumor resection to prevent recurrence. The combined approach of anterior tumor resection with microscopic assistance and posterior stabilization of the occiput to C5 is the optimal treatment for this condition.
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