Chordomas

Chordoma is a rare type of bone cancer that most often occurs in the bones of the spine or skull. It mostly occurs where the skull sits above the spine (skull base) or below the spine (sacrum).

Chordoma begins in cells that form a collection of cells in the developing embryo that once became the discs of the spine. Most of these cells disappear when you are born or shortly after. Sometimes, however, a few of these cells remain and they rarely become cancerous.

Although chordoma can occur at any age, it is most common in adults between the ages of 40 and 60.

Chordoma usually grows slowly. It can be difficult to treat because it is often very close to the spinal cord and other important structures such as arteries, nerves or the brain.

Diagnosis

Tests and procedures used to diagnose chordoma include:

Removal of a cell sample for laboratory testing (biopsy). A biopsy is a procedure to remove a sample of suspect cells for laboratory testing. In the lab, specially trained doctors called pathologists examine the cells under a microscope to determine if cancer cells are present.

Determining how the biopsy should be performed requires careful planning by the medical team. Doctors need to do the biopsy in a way that doesn't preclude future surgery to remove the cancer. Therefore, ask your doctor to refer you to a team of experts with extensive experience in treating chordoma.

Obtaining a more detailed view. Your doctor may recommend imaging tests to help visualize your chordoma and determine if it has spread beyond the spine or base of the skull. Tests may include an MRI or CT scan.

After diagnosing a chordoma, your doctor will consult with specialists in ear, nose and throat medicine (otolaryngology), cancer (oncology), and radiation therapy (radiation oncology) or surgery to develop a treatment plan that suits your needs. Your care team may also include endocrinology, ophthalmology and rehabilitation specialists as needed.

Treatment

Chordoma treatment depends on the size and location of the cancer, as well as whether it has invaded nerves or other tissues. Options may include surgery, radiation therapy, radiosurgery, and targeted therapies.

Chordoma treatment in the sacral spine

If the chordoma affects the lower part of the spine (sacrum), treatment options may include:

Operation. The goal of sacral spine cancer surgery is to remove all of the cancer and some of the healthy tissue that surrounds it. Surgery can be difficult because the cancer is close to critical structures such as nerves and blood vessels. When cancer cannot be completely removed, surgeons may try to remove as much as possible.

Radiation therapy. Radiation therapy uses high-energy rays such as X-rays or protons to kill cancer cells. During radiation therapy, you lie on a table while a machine moves around you directing radiation beams to specific points on your body.

Radiation therapy can be used before surgery to shrink a cancer and make it easier to remove. It can also be used to kill remaining cancer cells after surgery. If surgery is not an option, radiation therapy may be recommended instead.

Treatment with newer types of radiation therapy, such as proton therapy, allows doctors to use higher doses of radiation while sparing healthy tissue, which may be more effective in treating chordoma.

Radiosurgery. Stereotactic radiosurgery uses multiple beams of radiation to kill cancer cells in a very small area. Not every beam of radiation is very strong, but the point where all the beams meet - in the chordoma - receives high doses of radiation to kill cancer cells. It can be used before or after surgery for radiosurgery chordoma. If surgery is not an option, radiosurgery may be recommended instead.

Targeted therapy. Targeted therapy uses drugs that focus on specific abnormalities found in cancer cells. By attacking these abnormalities, targeted drug treatments can cause cancer cells to die. Targeted therapy is sometimes used to treat chordoma that has spread to other parts of the body.

Treatment of chordoma at the base of the skull

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If the chordoma affects the area where the spine meets the skull (base of the skull), treatment options may include:

Operation. Treatment usually begins with an operation to remove as much of the cancer as possible without damaging nearby healthy tissue or causing new problems such as brain or spinal cord injury. If the cancer is near important structures such as the carotid artery, complete removal may not be possible.

In some cases, surgeons may use special techniques, such as endoscopic surgery, to access the cancer. Endoscopic skull base surgery, long nose inserted to access cancer. It is a minimally invasive technique that involves using a thin tube (endoscope). Special instruments can be passed through the tube to remove the cancer.

Rarely, surgeons may recommend an additional operation to remove as much of the cancer as possible or to stabilize the area where the cancer once was.

Radiation therapy. Radiation therapy uses high-energy rays such as X-rays or protons to kill cancer cells. Radiation therapy is often recommended after surgery for the skull base chordoma to kill any cancer cells that may remain. If surgery is not an option, radiation therapy may be recommended instead.

Radiation therapy techniques that target treatment more precisely allow doctors to use higher doses of radiation that can be more effective for chordoma. These include proton therapy and stereotactic radiosurgery.