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Chordoma

What Is Chordoma?

Chordomas, also referred to as Notochordal Sarcoma, are uncommon, slow growing tumours of the spine. Chordoma can happen anywhere along the spine but are often found near the tailbone (called a Sacral tumour) or where the spine meets the skull (called a Clival tumour).

Who Does Chordoma Affect?

Every year, approximately one in one million people are affected by these tumours. These tumours are more commonly found in adults aged between 50 and 60 years of age . Chordomas are twice as common in many men.

 

While Chordoma can be hereditary, this is very rare.

What Causes Chordoma?

Chordomas are tumours that develop from tissue called the notochord. The notochord is a structure in an embryo that helps the development of the spine.

 

The notochord normally disappears after birth however some cells may remain in the bones of the spine and skull. These cells rarely turn into cancer, which is a Chordoma.


Types of Chordomas

There are three types of Chordoma 


Classic/Conventional Chordoma

Classic Chordomas are the most common type of Chordoma - accounting for around 80-90% of all cases


Chondroid Chordoma

Chondroid Chordomas are most commonly found at the base of the skull . This Chordoma has some similarities to chondrosarcoma, a more common type of primary bone cancer (which is a tumour developing from the cartilage).


Dedifferentiated Chordoma

Dedifferentiated Chordomas account for less than 5% of all Chordoma cases reported.


They are, however, a more aggressive form of this tumour type. Although dedifferentiated Chordomas can occur anywhere on the spine or in the skull, they are most commonly found at the base of the spine, in a region known as the sacrum.


Symptoms of Chordomas 

The symptoms associated with Chordomas are determined by the location of the Chordoma. Sacral Chordomas may present with

  • localised pain,
  • nerve symptoms or
  • bladder / bowel problems.

 

Skull base Chordomas often present with headaches and cranial neuropathies (weakness of nerves in the head and neck region).


How is Chordoma Diagnosed

Patients who are suspected of having a Chordoma require further investigation with imaging tests and a biopsy. Diagnostic Testing includes:


Imaging Tests

An MRI is the best investigation to look at the size of the tumour, its location and possible nerve involvement. A CT may also be performed to see if the tumour has spread elsewhere.


Biopsy

To check that the tumour is a Chordoma a biopsy may be performed. A small sample of the tumour is removed by a needle, often inserted using CT guidance. An expert, called a pathologist, will study the cells from the biopsy under a microscope to confirm the diagnosis.

 

Once a final diagnosis has been made your doctor will discuss with you treatment options available based on your particular case. 


Surgical Treatment for Chordoma

Chordomas at the sacrum and in the spine

Are best treated surgically involving complete surgical removal tends to give good local control at the tumours original site and are associated with a higher chance of survival. The tumour with some surrounding tissue is removed during these procedures. Radiotherapy is often administered after this.


Clival Chordomas and other skull-base Chordomas.

Are also treated surgically, this frequently entails performing surgery with specialized instruments and entering through the nose. It is possible that the tumour will not be completely removed. Following surgery, radiotherapy is often used.


Treatments After Surgery

Radiotherapy Treatment for Chordoma

Radiotherapy is often used following surgical resection of the Chordoma. This has been shown to reduce the risk of the Chordoma re-occurring. When surgery cannot be performed for a Chordoma due to the side effects, radiotherapy can be used instead. This has been shown to slow the growth of the tumour and delay onset of symptoms.


Chemotherapy and Medical Treatment for Chordoma

Chordoma is notoriously insensitive to traditional chemotherapy. New research is being undertaken using immunotherapy to treat Chordomas. Immunotherapy is a cancer treatment that uses the body’s immune system to fight the disease. 

 

At present there is no strong evidence for its use.


How long does it take to recover from Chordoma?

Recovery is variable, dependent on the site and size of the Chordoma, and the need for reconstruction of the defect. Early mobilisation and physiotherapy are essential for a good recovery.

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