Abstract

BackgroundAs chordomas are slow growing and locally invasive with high recurrence rates, initial recommendations include complete surgical resection with or without radiation therapy. A large proportion of recurrences occur years after initial resection necessitating lengthy follow-up. The novel biomarker brachyury and the repurposing of pharmaceutical products have the potential to substantially impact long-term recurrence rates.Case presentationA 43-year-old woman presented with an isolated, cervical extraosseous intradural extramedullary chordoma attached to a nerve root underwent a C3-5 laminectomy, C3-5 lateral mass screw instrumentation, and mass resection. All symptoms resolved by the 12-month postoperative follow-up visit.ConclusionsThis is the first report of an isolated, cervical extraosseous intradural extramedullary chordoma attached to a nerve root, and this case adds to the previous six Type IV chordomas in the literature. Unfortunately, the very rare form of extraosseous intradural chordoma is poorly understood: the lack of detailed knowledge in how they are differentiated from other forms of chordoma confounds the development of optimal treatment strategies and follow-up guidelines.

Highlights

  • As chordomas are slow growing and locally invasive with high recurrence rates, initial recommendations include complete surgical resection with or without radiation therapy

  • Cervical spine Magnetic resonance imaging (MRI) (Fig. 1) demonstrated a C3-5 right paracentral extramedullary homogenous 2.7 × 1.1 × 1.6 cm lesion that displaced the spinal cord left of midline

  • Hypothesized as originating from notochordal remnants, recent research revealed that some developed from benign notochordal cell tumors (BCNT) [2]

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Summary

Conclusions

This is the first report of an isolated, cervical extraosseous intradural extramedullary chordoma attached to a nerve root, and this case adds to the previous six Type IV chordomas in the literature.

Findings
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Discussion and conclusions
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