Chordoma | Sarcoma UK

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Chordoma makes up only 6% of all bone sarcoma diagnoses and most commonly affects adults in their 40’s and 50’s.

The majority of chordomas (50%) arise in the sacrum (the bottom of the spine); however, 30% arise at the base of the skull and the remaining 20% arise in other locations in the spine.

Signs and symptoms

Symptoms of bone sarcoma can vary depending on the size and location of your tumour.

  • Bone pain, particularly occurring at night
  • A mass or swelling
  • Restricted movement in a joint

Symptoms can sometimes be confused with more common problems such as a sports injury or in children and young people, growing pains.

Diagnosis

Your doctor will examine you. You may have one or more of these tests:

  • MRI scan – uses magnets to create an image of the tissues of the body
  • CT scan – The Computer Tomography (CT) scan takes a number of x-rays to make a 3D image of an affected area
  • X-ray – uses x-radiation to take images of dense tissues inside the body such as bones or tumours
  • Bone scan – shows up changes or abnormalities in the bones. A small amount of radioactive material is injected into your veins before having a scan

To read more about scans and tests, click here.

Treatment

The first treatment method for chordoma is surgery. The surgeon will remove the tumour and will aim to take out an area of normal tissue around it too; this is known as taking a margin. Sometimes surgery for chordoma cannot involve taking a margin of normal tissue due to the tumour’s location, so in these cases, the margins of the removed tissue will show tumour cells when viewed under a microscope.

Radiotherapy will be given after surgery to kill off any local cancer cells left in the area of your tumour, including those not visible microscopically. If there are nerves and arteries involved and they cannot be removed along with the tumour, then the aim of the surgeon will be to reduce the size of the tumour so radiotherapy treatment after surgery will be more successful. If your tumour cannot be operated on then radiotherapy alone will be the treatment of choice.

Some surgery for sarcoma may have some impact on your quality of life afterwards. Spinal surgery may affect how your bowel or bladder works or how your legs work. Surgery in the sacrum may involve the removal of nerves that work the bowel or bladder. This may lead to some people needing a colostomy or urostomy following surgery. You will be told of any potential risks involved before you have surgery.

The treatment you receive should take into account your individual situation and your doctor or clinical nurse specialist should talk you through all possible treatment alternatives. You should also have the opportunity to ask any questions you may have regarding the future implications of your surgery. Please speak to your doctor or sarcoma clinical nurse specialist if you have any concerns about your treatment.

Radiotherapy
Radiotherapy uses high-energy radiation beams to destroy cancer cells. It is used in the treatment of chordoma after surgery to kill off any local cancer cells that are left in the area. It will also be used after surgery if your tumour has only been partially removed. If your tumour cannot be operated on then radiotherapy alone will be the treatment of choice.

There are newer methods of radiotherapy that are sometime used to treat chordoma. Stereotactic radiotherapy, image-guided radiotherapy (IGRT) and intensity-modulated radiotherapy (IMRT) all allow doctors to direct higher doses of radiation more accurately at the tumour and minimise the side effects of treatment.

Proton beam therapy can be effective in treating chordoma. It is a different type of radiotherapy that uses high-energy protons rather than high-energy radiation to deliver a dose of radiotherapy. Proton beam therapy can be more effective than regular radiotherapy as it can be delivered precisely to where it is needed. This reduces the risk of damage to the surrounding tissue or vital organs. It is not currently available in the UK; however, the NHS will pay for selected patients (including some sarcoma patients) to receive this type of treatment overseas (in the USA or Europe).

Chemotherapy
Chemotherapy uses anti-cancer drugs to destroy cancer cells. It has not been shown to be effective in treating chordoma; however, it is sometimes used to control chordoma that
has come back after initial treatment (recurred) or spread to another part of the body (metastasised).

After treatment

After treatment for sarcoma you may benefit from rehabilitation services, such as occupational therapy, physiotherapy, dieticians, orthotics and prosthetics.

Rehabilitation usually starts after treatment. However, with bone sarcoma you may find that it helps to start rehabilitation earlier.

You may find that you are not automatically offered a referral to rehabilitation services by your medical team, so it is important to ask at the earliest opportunity for a referral.

You can read more about rehabilitation services here.

Living with

A recurrence of cancer may appear in other parts of the body. This is called a metastasis or secondary cancer.

In people with bone sarcoma, these secondary cancers may appear in the lungs. A chest x-ray is taken at follow-up appointments to look for secondary cancers in the lung.

If you have any questions or if you need to talk to someone, our Support Line team are here for you.

 

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