Observing a brain scan

Chordoma

Home Brain and Skull Base Tumors Chordoma

What is a Chordoma?

A chordoma is a rare type of malignant tumor that arises from remnants of the notochord, a structure that is present during early fetal development and provides support for the developing spine. Chordomas typically occur in the bones of the skull base, spine, or sacrum (the triangular bone at the base of the spine). These tumors grow slowly but are locally invasive and have a tendency to recur after treatment.

Clival chordomas can be considered as brain tumors or pituitary tumors. Clival chordomas are locally invasive tumors of the midline skull base. Chordomas are locally invasive slow-growing malignant tumors that arise from the remnant of the primitive notochord. They occur most commonly in the skull base (clivus) and lower spine. Approximately 40% of chordomas and chondrosarcomas arise in the clivus (directly below the sella turcica and pituitary gland).

Endoscopic endonasal surgery for Clival Cordoma
Endoscopic endonasal surgery for clival chordoma

Treatment for Chordomas

Endoscopic endonasal surgery is the optimal initial treatment for most Clival Chordomas

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.

At PNI, we have one of the world’s largest experiences with endonasal surgery for clival chordomas including patients who have had prior surgery and radiation. By incorporating cutting edge technology and instrumentation with proven surgical experience, our highly collaborative team provides safe, less invasive and effective chordoma surgery.

Given that many chordomas can be resistant to both surgery and radiosurgery, tumor genomic profiling is routinely performed in our chordoma patients to find alternative targeted therapies. We also have a protocol with pemetrexed (Alimpta) for patients with chordoma which we can offer for some patients.

Chordoma Overview

Chordoma are locally invasive slow-growing malignant tumors that arise from the remnant of the primitive notochord. They occur most commonly in the skull base (clivus) and lower spine. Approximately 40% of chordomas and chondrosarcomas arise in the clivus (directly below the sella turcica and pituitary gland).

Chordomas can behave quite aggressively and are often extensively invasive into surrounding skull base structures. A small subset of patients (~10%) may develop metastases to distant sites beyond their site of origin.

Symptoms of Chordoma

The most common presenting symptom of a clival chordoma is double vision.

Less common symptoms may include:

  • Facial numbness or tingling
  • Loss of vision
  • Hearing loss
  • Difficulty swallowing
  • Coordination
  • Motor weakness
  • Nasal congestion
  • Pituitary gland dysfunction
  • Headache

Diagnosis of Chordoma

These skull base tumors are best diagnosed by MRI and CT scans which will clearly show the extent of tumor and bony destruction.

Focused MRIs of the pituitary region, sinuses, temporal bones or internal auditory canals may be indicated to obtain better anatomical detail of a chordoma.

Other tests may also be needed prior to surgery such as angiography (typically now performed as a CT angiogram or an MR angiogram), visual field tests, an audiogram or pituitary hormonal tests.

Treatment for Chordoma

The initial treatment for a clival chordoma is maximal safe surgical removal.

Given their midline location, the great majority of clival chordomas and chondrosarcomas are best removed via an endoscopic endonasal approach.

However, some extensive and/or laterally placed chordomas may require different skull base 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 a residual tumor is common.

Extensive surgery can certainly improve long term survival but over-aggressive tumor removal can be associated with significant neurological complications.

After maximal safe removal, most chordoma patients will require Stereotactic Radiotherapy (SRT), stereotactic radiosurgery (SRS) or proton beam radiation. Chemotherapy is generally considered ineffective for treating chordomas although clinical trials of some experimental chemotherapies and immunotherapies are available. At Pacific Neuroscience Institute our innovative precision neuro-oncology approach has been successful in controlling tumor growth in many cases where surgical options have been exhausted.

Multidisciplinary Chordoma Specialist Team

Stories Related to Chordoma

Brain Tumor Clinic Locations

The Brain Tumor Center’s state-of-the-art facilities are located at:

Pacific Neuroscience Institute
2125 Arizona Ave., Santa Monica, CA 90404
310-582-7450

PNI – South Bay
5215 Torrance Blvd., #300, Torrance, CA 90503
424-212-5361

Providence Saint John’s Health Center
2200 Santa Monica Blvd., Santa Monica, CA 90404
310-829-8265 

PNI – Burbank
501 S. Buena Vista St., Burbank, CA 90505
818-847-6049

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Written and reviewed by:
The Pacific Neuroscience medical and editorial team
We are a highly specialized team of medical professionals with extensive neurological and cranial disorder knowledge, expertise and writing experience.
Last updated: September 21, 2023