Chordomas are rare malignant tumors arising from the embryologic remnant of the notochord. Tumors occur most commonly in middle-aged patients. Approximately one-third of chordomas arise at the skull base in a region called the clivus. This is a critical location in the center of the skull bordering major blood vessels, optic nerves, and the brainstem.

Tumors often cause severe headaches, vision changes, or facial numbness. CT scan imaging may show a mass at the central skull base with surrounding bony destruction. MRI scanning is critical to understand the tumor’s relationship to the adjacent structures.

Treatment Options

Surgical resection of chordomas is a significant challenge given their aggressive nature and proximity to vital structures. Open surgical approaches have been used with mixed success. Endoscopic approaches have also been used for midline clival chordomas with good success rates and low morbidity.

Close collaboration between rhinology and neurosurgery is important to improve outcomes. Local control may be improved by the addition of proton beam radiotherapy. UT Southwestern Medical Center uses a team approach, giving you access to the physicians and specialists necessary to treat your tumor.