Chordoma is a rare, slow-growing tumor that can occur in the bones of the skull and spine. These tumors are believed to stem from remnants of the embryonic notochord, which is a rod-shaped structure surrounding the spinal column as it develops. Chordomas are found most frequently at the base of the spine (the sacrum) and intracranially at the clivus (see figure), but they may occur anywhere along the spine. They fall in the category of cancers called sarcoma, which encompasses bone, cartilage and muscle tumors.

At Mount Sinai, our head and neck surgeons and neurosurgeons use a multidisciplinary approach to provide patients with the highest possible comprehensive care. This includes personalizing each patient’s treatment plan, utilizing the collective expertise from multiple specialty leaders and taking into account medical history and lifestyle. Surgical approaches to the craniocervical junction have historically been extremely challenging, but our surgeons have been at the forefront of the full spectrum, which includes anterior, anterolateral and posterolateral approaches.

Causes of Chordoma

No real cause for chordomas is known but a genetic basis has been described for some chordomas. In these few cases, a duplication of the brachyury gene (called a SNP) was determined to be the cause of chordoma.

Chordomas have also been linked to families with tuberous sclerosis, and certain types of childhood brain tumors. However, no environmental factors, trauma, or diet are known to be associated with chordomas.

Risks

Risk factors are things associated with an increased chance of developing a disease or condition. No known dietary, environmental, or lifestyle risk factors exist for chordomas.

While chordomas are generally slow-growing tumors, they tend to cause local destruction of the bone and grow into nearby tissues and organs. They frequently come back after initial treatment. Distant spread (metastasis) is rare but not unknown.

Symptoms

Symptoms depend on the location of the chordoma. Clival chordomas most commonly present with the following:

• double vision
• headaches
• facial pain or paralysis
• hearing changes
• swallowing problems
• hoarseness

Sacral chordomas may not cause symptoms until they are quite large, but common symptoms include:

• back and/or lower extremity pain
• leg weakness
• rectal dysfunction
• urinary incontinence
• erectile dysfunction
• palpable sacral mass

Diagnosis

Chordomas may be diagnosed via the following:

• MRI and/or CT of the brain
• Neurological examination
• Tissue biopsy

Treatment

Surgery is the primary treatment. Sometimes this requires an open cranial procedure. As minimally invasive, transnasal approaches have evolved, many can now be removed through the nose using endoscopic assistance. Mount Sinai surgeons have extensive experience with this transnasal approach and have consistently been at the forefront of developments associated with it.

In many cases, as complete a surgical resection as is possible within the limits of safety, offers the best chance of long-term control. Chordomas are fairly resistant to radiation and high doses are often needed for disease control. Since chordomas are usually close to critical neurological structures, such as the brain stem and cranial nerves, there is a limit as to how much radiation can safely be delivered. Therefore, highly focused radiation such as sterotactic radiosurgery or proton beam therapy may be more effective than conventional x-ray radiation.

Frequent follow-up is required because of potential recurrence of these tumors.

Prevention

There is no known way to prevent chordomas.

Chordoma Case Study

A 66-year-old woman began to develop double vision, which was worse when she looked to the right. She also noticed a muffled sound in the right ear with diminished hearing and discomfort in the right side of her jaw. Her work up led to MRI and CT which showed a lesion in the lateral clivus. Various management options were considered. As the diagnosis was unclear, a tissue biopsy was strongly recommended. Dr. Joshua Bederson and Dr. Eric Genden performed an extended transnasal microendoscopic partial resection. The final pathology revealed a chordoma and the patient was referred for radiation to treat the residual tumor. Aside from some post-operative headaches, changes in her sense of smell, and radiation related fatigue she has recovered well and resumed most of her former activities. She continues to be monitored on a regular basis.