Clival Chordoma

A clival chordoma is a rare, slow-growing and locally aggressive bone tumor that arises from the cellular remnants of the notochord at the base of the skull (the clivus). Because of its critical location near the brainstem and cranial nerves, it requires a highly specialized, multidisciplinary approach for safe removal and long-term control.

What Is Clival Chordoma?

Chordomas are rare malignant bone tumors that develop from remnants of the embryonic notochord. About one-third of all chordomas occur at the base of the skull, specifically in a bone called the clivus. Although they are typically slow-growing and technically low-grade, clival chordomas are considered locally aggressive because they invade surrounding bone and can compress vital structures like the brainstem, internal carotid arteries, and cranial nerves.

At a Glance

Rare bone tumor at the skull base arising from notochord remnants
Accounts for about 1% to 4% of all primary bone tumors
Locally aggressive and frequently involves cranial nerves and the brainstem
Maximal safe surgical resection is the foundation of treatment
Usually followed by high-dose radiation therapy (such as proton beam therapy) to reduce the risk of recurrence

Symptoms

Diagnosis

MRI of the brain and skull base — the gold standard; provides highly detailed images of the tumor, its relationship to the brainstem and cranial nerves, and exact tumor margins.
CT scan of the skull base — essential for evaluating the extent of bone destruction and planning the surgical approach.
Endoscopic biopsy — occasionally performed to confirm the tissue diagnosis before definitive surgery, though often diagnosis and resection occur simultaneously.

Types

Treatment

Endoscopic Endonasal Approach (EEA) — a minimally invasive surgical technique where neurosurgeons and ENT surgeons remove the tumor through the nose and sinuses. This provides direct access to the clivus without manipulating the brain.
Open Skull Base Surgery — traditional craniotomy approaches may be necessary for tumors that extend laterally beyond the reach of the endoscope.
Proton Beam Therapy — the standard adjuvant radiation treatment. Protons can deliver very high doses of radiation precisely to the tumor bed while sparing the adjacent brainstem and optic nerves from damage.

Outcomes

Treatment Approach	5-Year Survival Rate	Notes
Gross total resection + Proton therapy	~70-80%	Best chance for long-term local control and survival
Subtotal resection + Radiation	~50-60%	Used when tumor involves vital structures prohibiting complete removal
Surgery alone (no radiation)	Lower control rates	High rate of local recurrence; adjuvant radiation is standard of care

References

Our Specialists

Peleg Horowitz, MD, PhD

Neurosurgery

Bakhtiar Yamini, MD

Neurosurgery

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