What is ATRT? (Atypical Teratoid Rhabdoid Tumor)

Atypical teratoid rhabdoid tumor (ATRT) is a rare and fast-growing cancerous tumor of the brain and spinal cord. About half of these tumors 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 tumor growth. In ATRT, this gene does not function properly, the protein is not made and tumor 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 tumors?

ATRT is very rare and is found in fewer than 10% of children with brain tumors.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 tumor?

ATRT symptoms vary widely depending on the patient’s age and the tumor’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 vomitingChanges 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 tumor treated?

Treatment depends on the size and location of the tumor as well as the patient’s age. Because of the aggressive nature of these tumors, 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 tumor 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 tumor 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.

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