Chordoma is a rare cancer that affects spinal tissue. It can occur anywhere along the spine but is most common near the tailbone and the base of the skull. Treatment includes surgery, chemotherapy, and radiation therapy.

Chordoma is a slow-growing and extremely rare cancer of the spine that typically affects older adults.

About 1 in 1 million people receive a chordoma diagnosis each year. Most of these people are in their 50s or 60s, although chordoma can develop at any age.

Chordoma can cause a range of symptoms depending on its location. As chordoma grows slowly, people may not experience symptoms for some time. Treatment includes surgery and radiation therapy.

This article discusses chordoma, including common symptoms, causes, and treatment options.

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Symptoms depend on the person and the cancer’s location.

Tumors growing near the base of the skull may cause:

  • headaches
  • vision issues
  • difficulty swallowing

Tumors that occur near the tailbone may lead to:

  • bladder or bowel issues
  • tingling or numbness in the legs
  • weakness in the legs
  • numbness around the groin
  • difficulty walking

When tumors grow in other areas along the spine, they may cause symptoms such as:

  • pain around the tumor
  • numbness
  • weakness
  • tingling in the legs or arms

As chordoma develops slowly, symptoms may not occur right away.

Experts are not sure what causes chordoma. Most people who develop this form of cancer do not have a family history.

Chordoma may result from genetic changes. About 80% of individuals with chordoma have a genetic variation in a gene responsible for spinal development.

However, many people in the general population also have this variant. And most individuals with this variant do not go on to develop chordoma. Ongoing studies may shed more light on why some people develop chordoma, and others do not.

If a doctor suspects chordoma, they will order imaging tests to examine the area along the spine.

These tests typically include an MRI and CT scan. An MRI allows doctors to determine the location of a chordoma. This scan can also help determine whether a tumor has invaded tissues around the spine.

A CT scan can provide additional information about whether a tumor has spread. Doctors may recommend scanning the pelvis, chest, and abdomen to check whether the cancer has spread to these areas.

If MRI and CT scans indicate the presence of chordoma, a doctor may perform a biopsy to confirm the diagnosis. This procedure involves removing a small tumor sample and examining it under a microscope.

The proper chordoma treatment depends on the person and whether the cancer has spread. Some chordoma treatments include:

Another emerging chordoma treatment is proton therapy. This type of radiation therapy uses protons to attack cancerous tumors. One 2018 study suggests that proton therapy is an effective treatment for people with chordoma.

There is no single treatment that is right for every person with chordoma. A doctor will help find a suitable treatment plan for an individual’s needs.

On average, people with chordoma live for about 10 years after diagnosis. However, a person’s outlook varies widely depending on the tumor’s location and how far the cancer spread.

The type of treatment a person receives can also affect the disease outlook.

One 2019 study compared the 10-year survival rates of people with chordoma after surgery alone versus surgery combined with radiation.

Researchers found that the 10-year survival rate was 58% for people who received surgery and 73% for those who received surgery and radiation.

In some cases, chordoma tumors may return after surgical treatment. A 2021 study showed the location of chordoma affects the likelihood that it will recur. Researchers found that tumors along the length of the spine had a greater risk of returning than those at the base of the spine.

Each person has a different experience with chordoma. An individual can speak with their medical team about their personal outlook.

Chordoma is a form of cancer that occurs along the spine. It is a rare and slow-growing disease typically affecting people in their 50s and 60s.

Symptoms may develop slowly and can vary depending on tumor location. Tumors along the spine may cause weakness or tingling in the limbs. Tumors near the base of the skull may cause headaches or issues with vision.

Prompt testing and early diagnosis can improve a person’s outlook.