Chordoma: Symptoms, Diagnosis, Treatment


Chordoma is a rare tumor that can be found anywhere in the neck, back, and skull. Under most conditions, it occurs at the skull base, spine or bottom of the spine.

The disease can also be called notochordoma, notochordal sarcoma, chordoepithelioma, and chordocarcinoma.

It is estimated that nearly 50% of all chordomas happen at the base of the spine, around 33% in the base of the skull and about 20% in the neck area.

Although it is a slow-growing tumor, chordoma is a type of disease that is very difficult to treat because the tumor tends to invade its nearby structures in the skull base and spinal cord and the affected area is often near critical structures such as the carotid artery and brain tissue. Therefore, it is difficult to do surgery.

Also, chordoma often tends to recur after surgical treatment. If the chordoma spreads to other parts of the body, the most common places are the lungs, liver, bones and lymph nodes.

When it comes to chordoma, most patients are between 40 to 70 years old. Also, men are affected about twice as often as women.

According to statistics, only 1 out of 1 million people get the disease. In the United States, around 300 people are diagnosed with chordoma each year. In Europe, as many as 700 people are diagnosed with the condition on an annual basis.

Though the disease can occur at any age, only 5% of patients with chordomas are children.


When a baby is still in the womb, he or she has a notochord in his back, which provides support to the bones of the spine. As the baby grows older, the notochord tends to disappear before he or she is born.

However, in some cases, some of the notochord cells are left behind in the spine and skull, which might lead to genetic changes. The specific gene called brachyury is responsible for making a protein that is helpful for the formation of the spine. Under such circumstances, chordomas may grow from the remnants of the notochord.

However, the brachyury gene cannot be defined as the exact reason for a person to have chordoma. Many people carry the specific gene, but they do not develop the condition their whole life.

Right now, researches on this topic are still ongoing. The real cause of chordoma is still yet to be found.


For patients with chordoma, their nerves in the spine or brain may be compressed. In that case, the patients with the disease may have the following symptoms:

  • Pain
  • Numbness
  • Weakness
  • Abnormal eye movements
  • Changes to the voice or speech
  • Double vision
  • Headache
  • Loss of feeling and movement in the face
  • Neck pain
  • Trouble swallowing
  • Loss of control over the bowels
  • Lump in the lower part of the back
  • Pain in the lower back
  • Problems controlling the bladder
  • Aching
  • Tingling
  • Weakness of the arms and legs

Basically, according to the location and size of the tumor, the signs and symptoms of chordoma may be different for every individual. If you have the above-described symptoms, you’d better go to the hospital and get a detailed diagnosis from the doctor.


Getting an early diagnosis plays a significant role in later treatment. So, if you are suspicious about your condition, you should consult an experienced doctor and get an accurate diagnosis.

However, since the symptoms of chordoma may be similar to some other diseases, it is very easy to confuse the condition with other types of tumors. Therefore, before you receive any treatment, you may want to get a second or third opinion on this matter.

Apart from physical examination, the patients often need to go through imaging tests such as magnetic resonance imaging (MRI) and Computed tomography (CT) scans. These scans can help the doctor to better visualize the situation.

To confirm whether the individual has chordoma or not, a biopsy is a must as well. By taking a sample of cells to test in the laboratory, the doctor can be in a better position to decide whether there are cancer cells present or not.


Depending on where the tumor is, how large the tumor is and whether the tumor has invaded the nerves and other tissues, the doctor may tailor specific treatment methods for different patients.

The main treatment approaches may include:

  • Surgery.

The surgical method aims at removing the tumors. Due to the location of the chordoma, the tumors in often cases can’t be removed completely so the doctor may try to remove them as many as possible. Overall, the surgery is a very difficult one.

  • Radiation therapy.

This therapy uses high-energy beams like X-rays and protons to kill cancer cells.

  • Stereotactic radiosurgery.

This treatment targets cancer cells with multiple beams of radiation.

  • Chemotherapy and targeted drug therapy.
  • New therapy.

There are new studies focusing on chordoma that may be available for the patients to have a try. However, the new therapy also may carry higher risk and other possible unknown side effects.

Keywords: chordoma; notochordoma; notochordal sarcoma; chordoepithelioma; chordocarcinoma.

Related Post:

Life Expectancy for People with Chordoma

* The Content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.