What Is a Chordoma?

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Chordomas are malignant but slow-growing bone tumors that can occur along the spine or lower portion of the skull. Most commonly, they appear on the lower back or base of the skull.

Chordomas grow from the remnants of embryonic tissue that help form the spine during prenatal development. When these cells remain after birth, they can develop into tumors, which can metastasize and become aggressive.

This article will provide an overview of chordomas and their causes, as well as symptoms, diagnosis, and treatment options.

Spine exam

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Types of Chordomas

Chordomas account for about 3% of all bone tumors and 20% of all spinal tumors. They occur in one in one million people in the world each year.

Chordomas are most commonly found in people between the ages of 40 and 70, though they can occur at any age. They occur in men almost twice as often as women.

There are three main types of chordomas, which are classified based on their appearance and characteristics:

  • Conventional chordoma: This is the most common type of chordoma. These are characterized by their “bubbly” cell structure.
  • Dedifferentiated chordoma: This is the least common type, occurring in less than 5% of total cases. These grow faster and metastasize more quickly than the other two types.
  • Poorly differentiated chordoma: A very rare and aggressive type, these are unique from other chordomas because of the deletion of specific genes. This type of chordoma is more common in younger people and patients with cervical spine and skull base tumors.

Chordoma Symptoms

Due to the location where chordomas occur—the spine and base of the skull—symptoms can include back pain and nerve problems. As chordomas grow, they compress nerves connected to the part of the brain where they reside. If they become large enough, it may be possible to feel the tumor.

Resulting symptoms can include:

  • Tingling
  • Weakness
  • Numbness
  • Lack of bowel or bladder control
  • Vision problems
  • Difficulty swallowing
  • Sexual dysfunction

Causes

Chordomas originate from leftover embryonic cells that helped form the spinal cord during prenatal development, called notochord cells. If notochord cells remain after birth, they can become chordomas. The underlying reason this occurs is unknown, and there is no way to prevent a chordoma from happening.

There is evidence to suggest that there is a genetic component to why these cells turn into cancerous tumors. However, there is insufficient research to determine the specific mechanisms responsible for leftover notochord cells and how they form into chordomas.

Diagnosis

Because chordomas grow so slowly, it may take years for symptoms to appear. Sometimes patients might feel a lump and only then see their physician for further testing. In other cases, a patient may feel pain or compression from other structures, such as the trachea or esophagus, affected by the growing tumor.

Confirming a chordoma diagnosis may require a biopsy and diagnostic imaging.

A biopsy is a collection of cells from a suspicious area, which are sent for laboratory testing to determine if cancerous cells are present. Biopsies are helpful to distinguish a chordoma from another type of tumor.

Diagnostic imaging can help doctors visualize the chordoma and see if it has spread to other areas. Two types of imaging tests that may be used include:

Treatment

Upon diagnosis, your physician will discuss treatment options with you. They will likely refer you to other specialists for consultation, including an oncologist, a radiologist, or an ear, nose, and throat doctor (ENT).

Some of the main treatment options for chordomas include:

  • Surgery to remove the whole tumor with surrounding tissues containing cancer cells is usually the first step, as it provides the best chance for survival. If complete tumor removal is not possible, the goal is to remove as much as possible. Depending on the location of the tumor, surgery may require a collaboration between specialists.
  • Radiation therapy targets the tumor directly, or residual tumor cells, to deform the tumor’s DNA and cause it to shrink over time. This type of therapy targets large areas at a lower intensity.
  • Stereotactic radiosurgery also uses radiation to destroy tumor cells. However, this option treats small tumor areas at high intensities, all at one time.

Radiation therapy is often recommended after surgery and sometimes before surgery to destroy any remaining chordoma cells. Radiation is also used as a primary treatment when surgical removal is not possible.

Speed of Treatment

Treatment for chordomas can be complex because they form close to the spinal cord, brain, nerves, or arteries. The goal is to treat chordomas quickly before they have a chance to metastasize or cause damage to other parts of the body. For example, chordomas on the base of the skull can press against the brain and become life-threatening.

Prognosis

Studies show that the estimated median overall survival of individuals diagnosed with chordoma is about six to seven years. However, survival rates depend on several factors and whether the chordoma has metastasized to other areas of the body. With early chordoma detection, prognosis can be more positive.

Over half of all chordomas recur at some point after treatment. Recurrence can occur several years later, and many happen more than once. 

Your Prognosis

A chordoma prognosis will depend on your specific circumstances and the location of the tumor. You should discuss with your healthcare provider which treatment options are best for your particular situation.

A Word From Verywell

A chordoma diagnosis can be overwhelming. However, there are successful treatments and specialists who can help. Talk with your healthcare provider about the details of your diagnosis and which treatment options are best for you.

If you or a loved one is experiencing any symptoms of a chordoma or other neurological issue, meet with your healthcare provider as soon as possible. Early detection offers the best treatment options and improves the overall prognosis.

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5 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
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  2. Chordoma Foundation. Understanding chordoma.

  3. Cleveland Clinic. Chordomas. Updated September 5, 2018.

  4. Heery CR. Chordoma: the quest for better treatment optionsOncol Ther. 2016;4(1):35-51. doi:10.1007/s40487-016-0016-0

  5. Chordoma Foundation. Treatment guidelines for locally recurrent chordoma.