Isolated cervical extraosseous intradural chordoma attached to the C5 nerve root: a case report

BACKGROUND: As 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 PRESENTATION: A 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. 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. 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.