What is AR/RT Cancer?

The Jordan Reid Trust

What is atypical teratoid rhabdoid tumour?

Atypical teratoid rhabdoid tumour (ATRT) is a rare and fast-growing cancerous tumour of the brain and spinal cord. About half of these tumours begin in the cerebellum or brain stem:

  • The cerebellum, located at the base of the brain, controls movement, balance and posture.
  • The brain stem controls breathing, heart rate and all the muscles used in seeing, hearing, walking, talking and eating.

ATRT often appears to result from changes in a gene that normally makes proteins to stop tumour growth. In ATRT, this gene does not function properly, the protein is not made and tumour growth is uncontrolled. More than 90% of cases of ATRT are related to this gene defect. While this defect commonly occurs only within the cancer, this gene defect may be inherited and your doctor can discuss a need for genetic testing.

How common are atypical teratoid rhabdoid tumours?

  • ATRT is very rare and is found in fewer than 10% of children with brain tumours.
  • The disease is most often seen in children age 3 or younger, but it can also occur in older children and adults.

What are the symptoms of an atypical teratoid rhabdoid tumour?

ATRT symptoms vary widely depending on the patient’s age and the tumour’s location. Because ATRTs are fast-growing, symptoms usually progress rapidly in a short period of time.

Symptoms of ATRT may include the following:

  • Morning headaches or headaches that are less painful after vomiting
  • Nausea and vomiting
  • Changes in activity levels
  • Feeling sleepy
  • Loss of balance, increasing problems with coordination or trouble walking
  • Asymmetric eye movements or face movements
  • Increase in head size (in infants)

How is an atypical teratoid rhabdoid tumour treated?

Treatment depends on the size and location of the tumour as well as the patient’s age. Because of the aggressive nature of these tumours, most patients receive several types of treatment, which may include surgery, chemotherapy and radiation.

  • Surgery — is used to both diagnose and treat ATRT:
    • The surgeon removes part of the patient’s skull, uses a needle to take out a sample (biopsy) of the tumour and sends it to the laboratory. There, scientists examine the sample under the microscope.
    • If cancer is confirmed, the surgeon continues the operation by taking out as much of the tumour as it is safe to remove.
  • Chemotherapy (“chemo”) — uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells. Chemo is usually given after surgery.
    • Chemo may be injected into the bloodstream, so that it can travel throughout the body.
    • Some chemo may be given by mouth.
    • Combination therapy uses more than one type of chemo at a time.
  • Radiation therapy — uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing.

Further information

For more information visit the Grace Kelly Ladybird Trust website  whose main mission is to raise awareness of childhood cancer, fund research, provide education and support families.