Clivus chordoma: is it enough to image the primary site?

Chordomas are rare malignant tumors arising from embryonic remnants of the primitive notochord, around which the skull base and vertebral column develop. They are locally aggressive but metastasize rarely. To our knowledge, this is the first reported case of synchronous intraosseous chordomas. A 32-year-old man presented with intermittent double vision secondary to a right-side abducent nerve palsy. Imaging revealed a clivus chordoma and an asymptomatic synchronous second primary chordoma in the fifth lumbar vertebra. Both chordomas were surgically excised: the clivus using the endonasal, endoscopic route and the L5 vertebra by total vertebral excision and replacement with a titanium prosthesis. The patient made an uneventful and complete recovery. We have modified our departmental practice as we believe that all patients diagnosed with chordoma should have magnetic resonance imaging of their entire spinal tract to exclude a second primary chordoma.