Overview

Chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. It most often forms where the skull sits atop the spine (skull base) or at the bottom of the spine (sacrum).

Chordoma begins in cells that once made up a collection of cells in the developing embryo that go on to become the disks of the spine. Most of these cells go away by the time you're born or soon after. But sometimes a few of these cells remain and, rarely, they can become cancerous.

Chordoma happens most often in adults between 40 and 60, though it can happen at any age.

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

Diagnosis

Tests and procedures used to diagnose chordoma include:

• Removing a sample of cells for laboratory testing (biopsy). A biopsy is a procedure to remove a sample of suspicious cells for laboratory testing. In the lab, specially trained doctors called pathologists examine the cells under microscopes to determine whether cancer cells are present.

  Determining how the biopsy should be performed requires careful planning by the medical team. Doctors need to perform the biopsy in a way that won't interfere with future surgery to remove the cancer. For this reason, ask your doctor for a referral to a team of experts with extensive experience in treating chordoma.

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

After you receive a diagnosis of chordoma, your doctor will develop a treatment plan tailored to your needs in consultation with experts in ear, nose and throat medicine (otolaryngology), cancer (oncology), and radiation therapy (radiation oncology) or surgery. Your care team may also involve experts in endocrinology, ophthalmology and rehabilitation, as needed.

Treatment

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

Treatment for chordoma in the sacral spine

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

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

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

  Radiation therapy may be used before surgery to shrink a cancer and make it easier to remove. It can also be used after surgery to kill any cancer cells that remain. If surgery isn't an option, radiation therapy may be recommended instead.

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

• Radiosurgery. Stereotactic radiosurgery uses multiple beams of radiation to kill the cancer cells in a very small area. Each beam of radiation isn't very powerful, but the point where all the beams meet — at the chordoma — receives a large dose of radiation to kill the cancer cells. Radiosurgery can be used before or after surgery for chordoma. If surgery isn't an option, radiosurgery may be recommended instead.
• Targeted therapy. Targeted therapy uses drugs that focus on specific abnormalities present within cancer cells. By attacking these abnormalities, targeted drug treatments can cause cancer cells to die. Targeted therapy is sometimes used to treat chordoma that spreads to other areas of the body.

Treatment for chordoma in the skull base

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

• Surgery. Treatment usually begins with an operation to remove as much of the cancer as possible without harming nearby healthy tissue or causing new problems, such as injury to the brain or spinal cord. Complete removal might not be possible if the cancer is near important structures, such as the carotid artery.

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

  Rarely, surgeons might recommend an additional operation in order 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 beams, such as X-rays or protons, to kill cancer cells. Radiation therapy is often recommended after surgery for skull base chordoma to kill any cancer cells that might remain. If surgery isn't an option, radiation therapy may be recommended instead.

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

• New treatments. Clinical trials are studying new treatments for skull base chordoma, including new treatments that target specific weaknesses in the chordoma cells. If you're interested in trying these newer treatments, discuss the options with your doctor.