A rare case of primary intradural spinal chordoma with a systematic review of the literature

Abstract

Background: Pure intradural spinal chordomas are rare notochordal tumors with limited clinical characterization. Their intradural location often mimics more common extramedullary lesions, posing diagnostic challenges. We report a thoracolumbar intradural chordoma and present a systematic review of the literature. Case Description: A 45-year-old woman presented with progressive lower-limb weakness and urinary dysfunction. Magnetic resonance imaging revealed an intradural extramedullary lesion at D12–L2. The patient underwent gross total resection through posterior laminectomy. Histopathology demonstrated classic physaliphorous cells within a myxoid stroma, with immunopositivity for cytokeratin AE1/AE3, epithelial membrane antigen, and brachyury. The Ki-67 index was approximately 20%. Postoperatively, the patient showed complete neurological recovery, with no evidence of recurrence at 18-month follow-up. A systematic review identified 11 studies comprising 12 patients. Gross total resection was the primary treatment and was associated with favorable outcomes. Conclusion: Primary intradural spinal chordomas are rare lesions that frequently mimic other intradural tumors. Definitive diagnosis relies on histopathological and immunohistochemical confirmation, particularly brachyury expression. Gross total resection remains the cornerstone of management, and long-term follow-up is essential due to the risk of late recurrence.