Description

Chordoma is a rare type of bone cancer that starts as a growth of the cells in the bones of the spinal column or skull. It most often forms where the skull is located on the top of the spine, called the base of the skull, or in the lower part of the spine, called the sacrum.

The chordoma begins in the cells that help form the backbone before birth. The cells tend to become the discs of the spine. If any of these cells are left behind when a baby is born, that it would normally disappear shortly after birth. But sometimes, some of these cells for the stay and, in rare cases, they may become cancerous.

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

Symptoms

The signs and symptoms of chordoma may include:

  • The pain of a headache.
  • The neck pain.
  • The vision problems, including blurred vision, double vision or loss of vision.
  • Problems with balance.
  • The hearing loss.
  • The Facial paralysis.
  • The muscle weakness.
  • Difficulty swallowing.
  • Shortness of breath.

When to see a doctor

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

Causes

It is not clear what the cause of the majority of chordomas. This type of cancer most often begins in the bones in the skull or spine.

The chordoma occurs when the cells in the bones develop changes in their DNA. A cell's DNA contains the instructions that tell the cell what to do. In healthy cells, the DNA contains the instructions to grow and multiply at a set rate. The instructions that tell cells to die at a set time. In cancer cells, changes in DNA to give different instructions. The changes tell cancer cells to grow and multiply quickly. Cancer cells can continue living when healthy cells would die. This makes too many cells.

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

Risk factors

Factors that may increase the risk of chordoma include:

  • Age. The chordoma can occur at any age. Most of the times, however, this type of cancer occurs in people between the ages of 40 to 60.
  • The history of the family. People with a family history of chordoma may have a greater chance of developing the same.

Diagnosis

To diagnose the chordoma, a health care provider may do a physical exam and ask you questions about your health history. Other tests and procedures may include imaging tests and take a sample of tissue for laboratory analysis.

Imaging tests

Imaging tests create pictures of the body. You can display the size and the location of a cancer. For chordoma, imaging tests may 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 to give more details on the cancer cells. Your health care team uses this information to make a treatment plan.

The decision of how the biopsy should be performed requires careful planning on the part of your health care team. Your health care team needs to perform the biopsy in a way that does not cause problems with the future of the surgery to remove the cancer. For this reason, it is a good idea to seek care at a medical center that you see many people with chordoma.

Treatment

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

Treatment for chordoma in the sacrum of the vertebral column

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

  • Surgery. The goal of surgery for a sacrum of the spine chordoma is to remove all the cancer and some healthy tissue around it. The surgery may be difficult because the cancer is close to critical structures, such as nerves and blood vessels. When the cancer cannot be completely removed, the surgeons may try to remove as much as possible.
  • The radiation therapy.Radiation therapy treats cancer with powerful rays of energy. 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 the cancer and make it easier to remove. It can also be used after surgery to destroy any cancer cells that remain. If surgery is not an option, radiation therapy may be recommended in its place. Some types of radiation therapy allows health care teams to make the treatment more accurately. This reduces the risk of damaging the healthy tissue around the tumor. And allows the health care team the use of higher doses of radiation. Types of radiation therapy that do this include proton therapy and stereotactic body radiation therapy.
  • The targeted therapy. Targeted therapy for cancer is a treatment that uses drugs that attack specific chemicals in cancer cells. By blocking these chemicals, specific treatments can cause cancer cells to die. Targeted therapy is sometimes used to treat chordoma that extends to other areas of the body or the chordoma that comes back after treatment.

The radiation therapy. Radiation therapy treats cancer with powerful rays of energy. 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 the cancer and make it easier to remove. It can also be used after surgery to destroy any cancer cells that remain. If surgery is not an option, radiation therapy may be recommended in its place.

Some types of radiation therapy allows health care teams to make the treatment more accurately. This reduces the risk of damaging the healthy tissue around the tumor. And allows the health care team the use of higher doses of radiation. Types of radiation therapy that do this include proton therapy and stereotactic body radiation therapy.

Treatment for chordoma in the skull base

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

  • Surgery.The treatment usually begins with surgery to remove as much of the cancer as possible without damaging the surrounding healthy tissue. The surgeons work with care to avoid causing new problems, such as injury to the brain or the spinal cord. The complete removal may not be possible if the cancer is located close to important structures. Sometimes, surgeons can go through the nose to get to a skull base chordoma. This type of surgery is called endoscopic surgery of skull base surgery. Using 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.
  • The radiation therapy.Radiation therapy treats cancer with powerful rays of energy. Radiation therapy is often recommended after surgery for skull base chordoma to kill any cancer cells that may remain. If surgery is not an option, radiation therapy may be recommended in its place. Some types of radiation therapy allows health care teams to make the treatment more accurately. This reduces the risk of damaging the healthy tissue around the tumor. And allows the health care team the use of higher doses of radiation. Types of radiation therapy that do this include proton therapy and stereotactic radiosurgery.
  • The targeted therapy. Targeted therapy for cancer is a treatment that uses drugs that attack specific chemicals in cancer cells. By blocking these chemicals, specific treatments can cause cancer cells to die. Targeted therapy is sometimes used to treat chordoma that extends to other areas of the body or the chordoma that comes back after treatment.

Surgery. The treatment usually begins with surgery to remove as much of the cancer as possible without damaging the surrounding healthy tissue. The surgeons work with care to avoid causing new problems, such as injury to the brain or the spinal cord. The complete removal may not be possible if the cancer is located close to important structures.

Sometimes, surgeons can go through the nose to get to a skull base chordoma. This type of surgery is called endoscopic surgery of skull base surgery. Using 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.

The radiation therapy . Radiation therapy treats cancer with powerful rays of energy. Radiation therapy is often recommended after surgery for skull base chordoma to kill any cancer cells that may remain. If surgery is not an option, radiation therapy may be recommended in its place.

Some types of radiation therapy allows health care teams to make the treatment more accurately. This reduces the risk of damaging the healthy tissue around the tumor. And allows the health care team the use of higher doses of radiation. Types of radiation therapy that do this include proton therapy and stereotactic radiosurgery.

Coping and support

With time, you will find what helps you to deal with the uncertainty and fear of a cancer diagnosis. Until then, you can find help for:

Learn enough about chordomas to make decisions about your care

Ask your health care team about your cancer, including your test results, treatment options, and, if you like, your prognosis. As you learn more about your chordoma, you can be more confident in making treatment choices.

Keep friends and family close

Keep your close relationships strong can help you deal with a chordoma. Friends and family can provide the practical support that you may need, such as help to take care of your house if you're in the hospital. And that you can serve as emotional support when you feel overwhelmed by the fact of having cancer.

Find someone to talk to

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

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

Preparing for your appointment

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

Because appointments can be brief, it is a good idea to be prepared. Here's some information to help you prepare.

What you can do

  • Be conscious of any thing you need to do ahead of time. At the time you make the appointment, be sure to ask if there is anything that you need to do in advance, such as restrict your diet.
  • Write down any symptoms you have, including those that do not seem to be related to the reason for which you scheduled the appointment.
  • Write important personal information, including major stresses or recent life changes.
  • Make a list of all the medicines, vitamins, and supplements you are taking and the dose.
  • Have a family member or friend. It can be difficult to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
  • Write questions to ask their health care team.

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

  • I have a chordoma?
  • What is the stage of my chordoma?
  • Has my chordoma spread to other parts of my body?
  • More evidence is needed?
  • What are the treatment options?
  • How much does it cost each treatment to increase my chances of a cure or to prolong my life?
  • What are the possible side effects of each treatment?
  • How will each treatment affect my daily life?
  • There is a treatment option which you think is the best?
  • What would you recommend to a friend or family member in my situation?
  • You should see a specialist?
  • Are there brochures or other printed material that I can take with me? What sites do you recommend?
  • What is going to determine whether I should plan for a follow-up visit?

Do not hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer questions, such as:

  • When did your symptoms begin?
  • The symptoms been continuous or occasional?
  • How severe are the symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
Symptoms and treatment of Chordoma