Clival Chordoma: Symptoms, Diagnosis, and Treatment

A clival chordoma is a rare, slow-growing tumor that develops along the clivus — a bone at the center of the skull base, just in front of the brainstem. Chordomas arise from remnants of the notochord, a structure present during early development. While they are usually not aggressive in the way some cancers are, their location near the brainstem, cranial nerves, and major blood vessels makes them serious and complex to treat.

What is a clival chordoma?

Chordomas grow from tiny leftover cells of the notochord that can persist in the bones of the skull base and spine. At the clivus, a chordoma sits in a deep, central location surrounded by vital structures. They tend to grow slowly but can recur locally, which is why thorough treatment and long-term follow-up are so important.

Symptoms

Symptoms depend on the tumor’s size and exactly which structures it affects, and may include:

• Headaches
• Double vision or other eye-movement problems (a common early sign)
• Difficulty swallowing or speaking
• Facial numbness
• Balance or coordination problems

Because these symptoms develop gradually and overlap with many other conditions, chordomas are sometimes discovered only after imaging is done for another reason.

How is it diagnosed?

Diagnosis relies on detailed MRI and CT imaging, which show the tumor and the bone it involves and help distinguish a chordoma from other skull base tumors. A biopsy or surgery is typically needed to confirm the diagnosis.

Treatment options

Treatment usually combines surgery and radiation, delivered by an experienced skull base team:

• Surgery. The mainstay of treatment is maximal safe removal of the tumor. Many clival chordomas can be reached through endoscopic endonasal surgery — an approach that removes the tumor through the nose, without an external incision, while protecting the brainstem, nerves, and blood vessels.
• Radiation therapy. Because chordomas can recur, focused high-dose radiation — often proton therapy — is commonly recommended after surgery to reduce the chance of regrowth.

Outlook and follow-up

With modern surgical and radiation techniques, many patients do well, but because chordomas can come back over time, long-term follow-up with periodic imaging is an essential part of care.

Why an experienced team matters

The clivus is one of the most challenging areas in all of neurosurgery. Achieving a safe, thorough removal while protecting the brainstem and cranial nerves requires specific skull base expertise and a coordinated team of neurosurgery, ENT, and radiation oncology.

Dr. Arnaout has subspecialized training in skull base and endoscopic neurosurgery and treats these tumors as part of a multidisciplinary team.

When to see a specialist

If imaging has identified a clival tumor, or you’ve been diagnosed with a chordoma, an evaluation by a skull base neurosurgeon can help you understand the diagnosis and the full range of treatment options.

To discuss a clival chordoma diagnosis or get a second opinion, request an appointment.