Chordoma

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What do I have?

Chordoma is a primary malignant bone cancer that develops from remnants of embryonic notochord in the skull-base (head) and spine. Chordomas are generally slow growing and are most commonly found within the head at the base of the skull near a bone called the clivus and in the lower portion of the spine. Chordomas are extremely rare with a yearly incidence in the US of approximately 1 in one million (300 new cases each year).

What does it do?

Because they are usually very slow to grow, they are often quite large by the time they are discovered. Usually, symptoms develop and worsen over time. Common symptoms include headache or double vision. Less frequently seen symptoms are visual loss, hearing loss, difficulty swallowing, hoarse voice, facial numbness, incoordination, motor weakness and memory disturbance.

These tumors can be very destructive as they invade through bone and put pressure on vital brain structures.

Can it be cured?

In most cases, aggressive surgical removal followed by radiation therapy to remaining tumor offers the best chance of long-term control. Because chordomas invade the bone, complete removal is often impossible. Additionally, chordomas are relatively resistant to radiation therapy and are located adjacent to important and delicate brain structures, such as the brain stem and cranial nerves, which limits the dose of radiation that can be given. For these reasons, highly-focused radiation must be used to treat these tumors. Stereotactic radiosurgery and proton beam are the two most effective methods.

There are no chemotherapy drugs that are particularly effective in the treatment of chordomas.

Chordomas are malignant and potentially life threatening tumors. Currently the median survival in the United States is about 7 years. The overall survival rates are 68% at 5 years and 40% at 10 years. Complete surgical resection offers the best chance for long-term survival. In many cases, radiation therapy can also increase local control rates and prolong survival. Even after surgery and/or radiation, chordomas tend to return locally - in the same location or in the areas around the original tumor. Many patients undergo multiple surgeries over several years to treat local recurrences. After a local recurrence the chances of achieving a cure are significantly diminished. Distant metastasis (spreading to other body parts) occurs in 20-40% of patients with chordomas of the spine and less than 10% of patients with skull-base tumors. The most common sites of distant metastasis are the lungs, liver, bones, and skin.

What caused it?

There are currently no known genetic or environmental risk factors for chordoma.

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