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.