Delayed recurrence of intracranial chordoma

Although a chordoma is extremely rare, it commonly presents with ocular symptoms, often impacting one or more cranial nerves.

The authors describe a unique case of rapidly developing intracranial chordoma that recurred 9 years after the original mass was successfully diagnosed and treated. It is noteworthy that, although the tumor originated within the cranium at the base of the skull, it presented primarily with clinical signs of a unilateral orbital mass because of tumor extension.

The lifetime recurrence rate for chordoma is high after treatment; therefore, a high level of suspicion is warranted in any patient with new symptoms and a history of intracranial chordoma.