What is a Chordoma?A chordoma is a rare type of tumour that usually occurs along the spine or at the base of the skull. These tumours arise from what is left of the notochord, a flexible, rod-like structure that provides support to the developing embryo. Chordomas account for approximately 1-4% of all bone tumours. This makes them a very rare form of cancer. Overall, around 1 in 1,000,000 people will be affected by chordomas. Although chordomas are usually slow growing, they can be difficult to treat due to the problematic locations in which they occur. Ideally, the tumour will be able to be removed via surgery. However, this is not always a viable option due to high risks to the patient.
Causes and Risk Factors of ChordomasNobody knows what causes chordomas to occur. However, there are several known risk factors. A risk factor is something that increases the likelihood of a chordoma occurring. Risk factors do not guarantee that cancer will occur, especially considering the rarity of this form of cancer. Below are some of the most common risk factors associated with chordomas:
- Gender – Chordomas occur twice as frequently in males as opposed to females.
- Age – Chordomas can occur at any age, but are most common in adults aged between 40 and 80.
- Ethnicity – Some reports state that chordomas are more common in people of European ethnicity.
- Genetic abnormalities – A common theory is that acquired genetic abnormalities or mutations result in chordomas occurring. These genetic abnormalities may or may not be inherited.
How are Chordomas Diagnosed?The most common way for chordomas to be diagnosed is via a biopsy. This is when a sample of tissue is removed from the suspected site and examined for signs of cancer. Your doctor may also recommend imaging tests, which help them to determine the location, size and spread of a tumour.
Symptoms associated with Chordomas:It is important to remember that symptoms may vary depending on the size and location of the tumour. Below are some of the most common symptoms associated with the growth of a chordoma. Symptoms of a chordoma on the skull base:
- Double vision
- Paralysis of facial nerves – resulting in difficulty with speaking, difficulty swallowing and unusual facial muscle behaviour.
- Back pain, weakness or numbness
- Loss of bowel or bladder control
- Weakness in legs
- A mass that can felt on the back
- Neck pain
- Difficulty talking and swallowing
- Bleeding from the larynx
Treatment for ChordomasThere are several different types of treatment that can help combat and destroy chordomas. The type of treatment that doctors recommend varies depending on the size and location of a tumour. Below are some of the most common treatments: Surgery: The goal of surgery is to remove the tumour in its entirety and preventing it from growing back over time. During surgery, surgeons will remove what they can without causing damage to critical structures such as the spine or carotid artery. Surgery may be followed by another form of treatment such as radiation therapy. This will target parts of the tumour that surgeons could not fully remove. Sometimes, the risks associated with surgery are too high. This happens when the tumour is located in a position which poses a high risk to surrounding organs or arteries. In these cases, other forms of treatment are recommended. Radiation therapy Radiation therapy uses high-energy beams to target and destroy cancer cells. It is often used as an alternative to surgery to treat chordomas. Newer forms of radiation therapy such as proton therapy allow doctors to use more powerful radiation while protecting surrounding tissue. However, radiation therapy does still come with symptoms. These include:
- Tiredness and fatigue
- Loss of hair around the targeted area
- Redness and irritation in the skin around the affected area.