Chordoma

What is a Chordoma?

Overview

A chordoma is a rare, slow-growing but malignant tumor that develops from remnants of the notochord—a structure present during early fetal development that helps form the spine. Normally, the notochord disappears before birth, but in rare cases, small areas remain and, over time, can become cancerous.

Approximately 40% of chordomas develop at the skull base (especially the clivus, located just below the pituitary gland), along the spine, or in the sacrum (the triangular bone at the bottom of the spine). Although they grow slowly, chordomas are locally aggressive, meaning they can invade nearby structures such as the spinal cord, brain, major arteries, and important nerves.

Treatment can be challenging because these tumors often occur near critical structures and are resistant to both surgery and radiation. Even after treatment, chordomas have a tendency to come back, and about 10% may spread (metastasize) to other parts of the body.

Clival chordomas are ideally treated with maximal safe surgical removal followed by focused radiotherapy. Fortunately, the majority of clival chordomas can be removed via the nose using an endoscopic endonasal approach. Occasionally, these tumors require multiple keyhole operations including the retrosigmoid and eyebrow craniotomy approaches.

Given that many chordomas can be resistant to both surgery and radiosurgery, tumor genomic profiling is being increasingly used to find alternative targeted therapies.

Who is Affected?

Chordomas can occur in people of any age but are most often diagnosed in adults between 40 and 70 years old. They are slightly more common in men than women.

Chordoma Symptoms & Causes

Symptoms

Symptoms depend on the tumor’s size and location. For clival chordomas, the most common presenting symptom is double vision. Other possible symptoms include:

• Visual loss
• Hearing loss
• Difficulty swallowing
• Facial numbness
• Incoordination or motor weakness
• Nasal congestion

Causes

Chordomas develop from notochord remnants that fail to disappear after birth. In most cases, the exact reason these cells become cancerous is unknown.

When to See a Doctor

Early detection improves treatment options and outcomes. Seek medical evaluation if you experience persistent double vision, changes in hearing or balance, difficulty swallowing, or new neurological symptoms.

Chordoma Diagnosis

Chordomas are best diagnosed with MRI and CT scans, which can reveal the extent of tumor growth and bony destruction.

Additional tests may include:

• Focused MRI of the pituitary region, sinuses, or temporal bones for detailed anatomy
• CT or MR angiography to evaluate blood vessels
• Visual field testing
• Hearing tests (audiogram)
• Hormonal bloodwork if pituitary involvement is suspected

Chordoma Treatment & Outcomes

Treatment Options

Surgery: Initial treatment for a clival chordoma is with surgical removal or debulking.

• Endoscopic endonasal approach: Given their midline location, most clival chordomas and chondrosarcomas are best removed via an endoscopic endonasal approach.
• Alternate approaches (retrosigmoid and eyebrow craniotomy): Some extensive and/or laterally placed chordomas may require different skull base surgical approaches.

Because chordomas typically invade the bone and dura of the skull base as well as cavernous sinus area, complete surgical resection is often not possible and continued growth of residual tumor is common. Extensive surgery can certainly improve long term survival but over aggressive tumor removal can be associated with significant neurological complications.

Following surgery, most patients undergo focused radiation therapy such as:

• Stereotactic Radiotherapy (SRT)
• Stereotactic Radiosurgery (SRS)
• Proton beam therapy (effective for delivering high doses to the tumor while sparing surrounding tissue)

Additionally, while traditional chemotherapy has been relatively ineffective for chordomas, some new targeted agents are now available and showing promise in treating chordomas. By using comprehensive tumor histological subtyping and genomic sequencing, we are able to provide a personalized therapeutic approach for each patient. Our neuro-oncology specialists can facilitate clinical trial participation with novel medical therapies where appropriate.

Patient Outcomes

While chordomas have a high recurrence rate, outcomes are improving with advances in surgical techniques, precision radiation, and targeted treatments. Long-term follow-up with regular imaging is essential.

Managing A Chordoma

Management includes:

Meet our Expert Specialists & Surgeons

Garni Barkhoudarian, MD

Neurosurgery

Jose Carrillo, MD

Neuro-Oncology

Adi Iyer, MD, MS

Neurosurgery, Neuro-interventional Surgery

Daniel F. Kelly, MD

Neurosurgery

Howard R. Krauss, MD

Neuro-ophthalmology

Nouzhan Sehati, MD

Neurosurgery

Walavan Sivakumar, MD

Neurosurgery

Naveed Wagle, MD

Neuro-Oncology

Robert Wollman, MD

Radiation Oncology

April Vassantachart, MD

Radiation Oncology

Experience Compassionate, Expert Care

At Pacific Neuroscience Institute we have one of the world’s largest experiences with endoscopic endonasal surgery for clival chordomas and a highly experienced surgical team. By incorporating leading-edge technology and instrumentation with proven surgical experience, we make chordoma surgery safer, less invasive and more effective.

Watch Andrea’s Clival Chordoma Story

Learn More About a Chordoma

Resources