Chordoma tumor
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What is Chordoma?

Chordoma is a rare type of cancer that affects the bones near the base of the skull and the spine. It belongs to the sarcoma subgroup of malignant bone and soft tissue cancers.

Chordoma develops in cells that were originally part of a group of cells in the developing embryo that went on to produce the spine’s discs. The majority of these cells die before or soon after birth. A few of these cells, however, may remain and, in rare situations, become cancerous.

These tumors are cancerous and can spread, though they normally grow slowly. Even slow-growing chordomas can become aggressive and grow extremely large locally, placing pressure on or penetrating into key regions of the brain or spine, causing discomfort, nerve issues, and even death.

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There are three forms of chordoma depending on how the cells appear under a microscope:

  • Classic/conventional chordoma –  It is made up of a special sort of cell that has a “bubbly” look. The expression of the brachyury protein is a characteristic of chordoma and separates it from other tumors such as chondrosarcoma.
  • Dedifferentiated chordoma –  This type emerges as a mixture of normal chordoma cells that express brachyury and high-grade sarcoma cells that do not exhibit brachyury. It is more aggressive, grows faster in general, and is more likely to metastasize than traditional chordoma.
  • Poorly differentiated chordoma – This kind is quite uncommon. The absence of a gene known as SMARCB1 or INI1 is a defining feature of poorly differentiated chordoma.The deletion of the gene can be discovered by genomic testing, and pathologists can determine the lack of the SMARCB1/INI1 protein by staining tumor cells.


Researchers are unsure of the precise cause of chordoma. Doctors believe chordoma develops as a result of a mutation in the gene that codes for a protein that helps in the formation of the spine. This mutation causes notochord cells that remain in the brain or spinal cord to divide excessively fast.

This transformation occurs at random; no one in your family needs to have chordoma for you to have it. It can run in families in rare situations.

Risk factors

Chordomas develop on their own. It is not known if they are caused by trauma, environmental factors, or food. Chordomas do not run in families. They are unrelated to any medical issues or the usage of drugs or supplements.

The vast majority of chordomas arise at random and are not caused by an inherited genetic characteristic; nonetheless, some genetic factors have been related to chordomas.


Chordomas can irritate nerves in the spine or brain. This can cause discomfort, numbness, or weakness. Your symptoms are determined by the location and size of the malignancy:

  • Inconsistent eye motions
  • Alterations to your voice or speech
  • Dual vision
  • Headache
  • Your face stops moving and feeling
  • A sore neck
  • Difficulty in  swallowing
  • Arm or leg numbness, tingling, or weakness
  • Back pain in the lower back
  • Controlling your bladder issues
Chordoma tumor


Your doctor can use imaging tests to determine where the cancer is located within your body and how large it has grown. These include:

  • The brain or spinal cord can be imaged using low levels of radiation like X-rays.
  • A CT (computed tomography) scan creates comprehensive images of the injured area by combining X-rays taken at various angles.
  • Using strong magnets and radio waves, MRI (magnetic resonance imaging) creates images of the organs and other internal structures in your body.
  • A biopsy is a process that removes a sample of abnormal cells for examination in a laboratory. Pathologists, who are specially trained doctors, analyze the cells under microscopes in the lab to establish whether cancer cells are present.


Chordoma treatment is determined by cancer’s size and location, as well as whether it has invaded nerves or other tissue. Treatment includes:

  • Surgery to treat sacral spine cancer aims to completely remove the malignancy as well as part of the surrounding healthy tissue.
  • To kill cancer cells, radiation therapy employs high-energy beams such as X-rays or protons. Before surgery, radiation therapy may be used to reduce a malignancy and make it easier to remove.
  • Stereotactic radiosurgery employs many radiation beams to eliminate cancer cells in a very tiny area. Each radiation beam isn’t extremely powerful, but the point where all the beams come together – the chordoma — receives a high dose of radiation to destroy the cancer cells.
  • Targeted therapy employs medications that target certain abnormalities found in cancer cells. Cancer cells can be killed by targeted medication treatments that target these abnormalities. Source

When do we see Doctor?

If you have any of the signs or symptoms of chordoma, you should seek medical assistance right once. Early detection and treatment are crucial for the best possible outcome.


There is nothing you can do to stop chordoma from growing. The majority of cases occur at random. 

Visit your healthcare professionals frequently so they can keep an eye out for chordoma symptoms if you have a family history of the disease or tuberous sclerosis. It’s best if they can catch it as soon as possible.

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