Chordoma

Chordoma is a rare type of bone cancer that starts as a growth of cells in the bones of the spine or the skull. It most often forms where the skull sits atop the spine, called the skull base, or at the bottom of the spine, called the sacrum.

Chordoma begins in cells that help form the spine before birth. The cells typically become the disks of the spine. If any of these cells are left over when a baby is born, they typically go away soon after birth. But sometimes a few of these cells stay and, rarely, they can become cancerous.

Chordoma usually grows slowly. It can be difficult to treat because a chordoma can grow very close to the spinal cord and other important structures, such as arteries, nerves or the brain. This cancer often comes back after treatment.

Signs and symptoms of chordoma may include:

• Headache.
• Neck pain.
• Vision trouble, including blurred or double vision or vision loss.
• Trouble with balance.
• Hearing loss.
• Facial paralysis.
• Muscle weakness.
• Trouble swallowing.
• Shortness of breath.

When to see a doctor

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

It’s not clear what causes most chordomas. This cancer most often starts in the bones in the skull or spine.

Chordoma happens when cells in the bones develop changes in their DNA. A cell’s DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells.

The cancer cells form a mass called a tumor. The tumor can grow to invade and destroy healthy body tissue. In time, cancer cells can break away and spread to other parts of the body. When cancer spreads, it’s called metastatic cancer.

Factors that may increase the risk of chordoma include:

• Age. Chordoma can happen at any age. Most often, however, this cancer happens in people ages 40 to 60.
• Family history. People with a family history of chordoma may have a greater chance of developing it.

To diagnose chordoma, a healthcare professional might do a physical exam and ask questions about your health history. Other tests and procedures might include imaging tests and taking a sample of tissue for lab testing.

Imaging tests

Imaging tests make pictures of the body. They can show the size and location of a cancer. For chordoma, imaging tests might include CT scans and MRIs.

Biopsy

A biopsy is a procedure to remove a sample of tissue for testing in a lab. The sample is tested to see if it is cancer. Other tests give more details about the cancer cells. Your healthcare team uses this information to make a treatment plan.

Deciding how the biopsy should be performed requires careful planning by your healthcare team. Your care team needs to perform the biopsy in a way that won’t cause problems with future surgery to remove the cancer. For this reason, it’s a good idea to seek care at a medical center that sees many people with chordoma.

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 and targeted therapies.

Treatment for chordoma in the sacral spine

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

• Surgery. The goal of surgery for a sacral spine chordoma 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 treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body.

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

  Some types of radiation therapy allow healthcare teams to aim the treatment more precisely. This lowers the risk of hurting healthy tissue around the cancer. And it lets healthcare teams use higher doses of radiation. Types of radiation therapy that do this include proton therapy and stereotactic body radiotherapy.

• Targeted therapy. Targeted therapy for cancer is a treatment that uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die. Targeted therapy is sometimes used to treat chordoma that spreads to other areas of the body or chordoma that comes back after treatment.

Treatment for chordoma in the skull base

If the chordoma affects the area where the spine joins with the skull, called the 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. Surgeons work carefully to avoid 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.

  Sometimes surgeons can go through the nose to get to a skull base chordoma. This kind of surgery is called endoscopic skull base surgery. It uses a long, thin tube called an endoscope. The endoscope is inserted through the nose to access the cancer. Special tools can be passed through the endoscope to remove the cancer.

• Radiation therapy. Radiation therapy treats cancer with powerful energy beams. 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.

  Some types of radiation therapy allow healthcare teams to aim the treatment more precisely. This lowers the risk of hurting healthy tissue around the cancer. And it lets healthcare teams use higher doses of radiation. Types of radiation therapy that do this include proton therapy and stereotactic radiosurgery.

• Targeted therapy. Targeted therapy for cancer is a treatment that uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die. Targeted therapy is sometimes used to treat chordoma that spreads to other areas of the body or chordoma that comes back after treatment.

With time, you’ll find what helps you cope with the uncertainty and worry of a cancer diagnosis. Until then, you may find it helps to:

Learn enough about chordomas to make decisions about your care

Ask your healthcare team about your cancer, including your test results, treatment options and, if you want, your prognosis. As you learn more about your chordoma, you may become more confident in making treatment decisions.

Keep friends and family close

Keeping your close relationships strong can help you deal with a chordoma. Friends and family can provide the practical support you may need, such as helping take care of your home if you’re in the hospital. And they can serve as emotional support when you feel overwhelmed by having cancer.

Find someone to talk with

Find someone who is willing to listen to you talk about your hopes and worries. This may be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group also may be helpful.

Ask your healthcare team about support groups in your area. Other sources of information include the National Cancer Institute and the American Cancer Society.

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you. If your healthcare professional thinks you might have a chordoma, you may be referred to a specialist.

Because appointments can be brief, it’s a good idea to be prepared. Here’s some information to help you get ready.

What you can do

• Be aware of anything you need to do ahead of time. At the time you make the appointment, be sure to ask if there’s anything you need to do in advance, such as restrict your diet.
• Write down symptoms you have, including any that may not seem related to the reason for which you scheduled the appointment.
• Write down important personal information, including major stresses or recent life changes.
• Make a list of all medicines, vitamins and supplements you’re taking and the doses.
• Take a family member or friend along. It can be hard to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

Your time with your healthcare team is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For chordoma, some basic questions to ask include:

• Do I have a chordoma?
• What is the stage of my chordoma?
• Has my chordoma spread to other parts of my body?
• Will I need more tests?
• 治療の選択肢は何ですか?
• How much does each treatment increase my chances of a cure or prolong my life?
• What are the potential side effects of each treatment?
• How will each treatment affect my daily life?
• Is there one treatment option you believe is the best?
• What would you recommend to a friend or family member in my situation?
• Should I see a specialist?
• Are there any brochures or other printed material that I can take with me? What websites do you recommend?
• What will determine whether I should plan for a follow-up visit?

Don’t hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer questions, such as:

• When did your symptoms begin?
• Have your symptoms been continuous or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?