About half of these tumors form in the cerebellum
or brain stem. The cerebellum is the part of the brain that controls movement, balance, and posture. The brain stem controls breathing, heart rate, and the nerves
and muscles used in seeing, hearing, walking, talking, and eating. AT/RT may also be found in other parts of the central nervous system (brain and spinal cord).
Brain tumors can occur in both children and adults; however,
treatment for children may be different than treatment for adults. See
the PDQ treatment summary on Adult Central Nervous System Tumors Treatment for more information.
Certain genetic changes may increase the risk of atypical teratoid/rhabdoid tumor.
Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk.
Atypical teratoid/rhabdoid tumor may be linked to a change in a tumor suppressor gene
called SMARCB1. This type of gene
makes a protein
that helps control cell growth. Changes in the DNA
of tumor suppressor genes like SMARCB1 may lead to cancer.
Changes in the SMARCB1 gene may be inherited
(passed on from parents to offspring). When the SMARCB1 gene change is inherited, tumors may form in two parts of the body at the same time (for example, in the brain and the kidney). For patients with AT/RT, genetic counseling
(a discussion with a trained professional about inherited diseases and a possible need for gene testing) may be recommended.
The signs and symptoms of atypical teratoid/rhabdoid tumor are not the same in every patient.
Because atypical teratoid/rhabdoid tumor is fast growing, signs and symptoms may develop quickly and get worse over a period of days or weeks. Signs and symptoms may be caused by AT/RT or by other conditions. Check with your child's doctor if your child has any of the following:
Morning headache or headache that goes away after vomiting.
Loss of balance, lack of coordination, or trouble walking.
Increase in head size (in infants).
Tests that examine the brain and spinal cord are used to detect (find) CNS atypical teratoid/rhabdoid tumor.
The following tests and procedures may be used:
and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve
function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
(magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Lumbar puncture: A procedure used to collect cerebrospinal fluid
(CSF) from the spinal column. This is done by placing a needle between two bones in the spine
and into the CSF around the spinal cord and removing a sample of fluid. The sample of CSF is checked under a microscope
for signs of tumor cells. The sample may also be checked for the amounts of protein
and glucose. A higher than normal amount of protein or lower than normal amount of glucose may be a sign of a tumor. This procedure is also called an LP or spinal tap.
Childhood atypical teratoid/rhabdoid tumor is diagnosed and may be removed in surgery.
If doctors think there might be a brain tumor, a biopsy
may be done to remove a sample of tissue. For tumors in the brain, the biopsy is done by removing part of the skull
and using a needle to remove a sample of tissue. A pathologist
views the tissue under a microscope
to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery. The pathologist checks the cancer cells to find out the type of brain tumor. It is often difficult to completely remove AT/RT because of where the tumor is in the brain and because it may already have spread at the time of diagnosis.
The following test may be done on the sample of tissue that is removed:
Immunohistochemistry: A test that uses antibodies
to check for certain antigens
in a sample of tissue. The antibody is usually linked to a radioactive
substance or a dye that causes the tissue to light up under a microscope. This test is used to tell the difference between AT/RT and other brain tumors.
Certain factors affect prognosis (chance of recovery) and treatment options.