Atypical Teratoid/Rhabdoid Tumor (ATRT)
A fast-growing tumor that grows in the brain or spinal cord.
What Is an Atypical Teratoid/Rhabdoid Tumor (ATRT)?
An atypical teratoid/rhabdoid tumor (ATRT) is a fast-growing tumor that grows in the brain or spinal cord. ATRTs usually occur in isolation, meaning the tumor does not spread to other parts of the body. Spreading to other parts of the body usually only occurs in rare cases like rabid predisposition syndrome.
It mostly affects children younger than 3 years old, but it can affect anyone. It can be difficult to treat. Results (outcomes) depend many factors, including:
- The location of the tumor in the brain
- How much can be taken out during surgery
- The child’s age
- The treatments used
- Response to therapy
What to Expect With an Atypical Teratoid/Rhabdoid Tumor (ATRT)
ATRT is an aggressive brain cancer. Your child’s chances of survival depend on factors such as:
- Where the tumor is in the brain
- How much it has spread
- How much can be taken out during surgery
- How old your child is
- How the ATRT is treated
Your child’s care team can help you understand what to expect based on your child’s test results.
The treatments used for ATRT may have long-term side effects. They may cause trouble walking, talking, growing or learning. Children who survive ATRT may also be more likely to have cancer again.
All children with ATRT treated at Nationwide Children’s can expect the help of a large care team. During your child’s diagnosis and treatment, the care team will work with your family to make decisions. The team will include medical experts, surgeons, child life specialists, social workers and more. The goal is to get help from experts in many areas of child health, to make sure your child’s needs are met.
After treatment, you can expect regular visits with our team to monitor your child’s health. Your child will have blood tests and imaging every year (or more often) to check for new tumors. We will also help your family adjust to any new care needs your child may have. The new care team may include physical therapists, occupational therapists, speech therapists, behavioral or learning specialists, social workers, psychologists and other experts.
What Causes an Atypical Teratoid/Rhabdoid Tumor (ATRT)?
Most cases of ATRT are related to a change in genes called SMARCB1 and SMARCB4. In cases of ATRT, a problem with one of these genes lets tumors grow uncontrolled.
In most kids, the problem with SMARCB1 or SMARCB4 happens by chance. This means the change is not passed down from parents. In some children, though, the problem with the gene is passed down from parents. In these cases, it is more likely a child will have tumors in more than one part of the body (such as the brain and the kidney).
What Are the Signs and Symptoms of an Atypical Teratoid/Rhabdoid Tumor (ATRT)?
Every child is different, and some symptoms of brain tumors overlap with other health problems. With ATRT, symptoms can come on suddenly and get worse over a few days or weeks. This may be because the tumor is growing quickly. Talk to a doctor if your child has:
How Is an Atypical Teratoid/Rhabdoid Tumor (ATRT) Diagnosed?
Knowing the exact type of cancer is important so doctors can recommend the best treatment. ATRT can be diagnosed with:
- Neurological exam: A neurologist will check your child’s vision, balance, speech, muscles and other senses. This helps find problems that tumors may be causing with the brain, nerves or spinal cord.
- Imaging: Pictures of the tumor using MRI, CT scans or ultrasounds can help show doctors where the tumor is and what it looks like.
- Surgery and tissue analysis: When the tumor is taken out, some of the tumor will be tested to find out for sure what type of cancer it is. By looking at the tumor cells under a microscope and doing blood tests, ATRT can be diagnosed.
- Blood tests: These will check the tumor DNA for specific changes that happen with ATRT (such as changes in SMARCB1 or SMARCB4). Blood tests can also show how the body is reacting to the cancer.
- Spinal fluid tests: Checking the liquid inside the spinal cord can show whether the tumor cells are spreading outside of the tumor.
How Is an Atypical Teratoid/Rhabdoid Tumor (ATRT) Treated?
While ATRTs are difficult to treat, advances in therapy are improving cure rates for children. Survival depends on several factors. Depending upon how old your child is and their condition, your doctor may discuss new therapies, including clinical trials for your child. Your child's doctor will discuss these with you based on the treatment your child receives.
Your child’s treatment plan will depend on their age, the tumor size and location, and whether it has spread. The care team will talk about options with you and discuss the best plan.
When to Seek Help for an Atypical Teratoid/Rhabdoid Tumor (ATRT)
Always talk to a doctor if you are worried about your child’s health. If you child has any of the symptoms listed for ATRT, or if you feel something is wrong, do not wait. Call a doctor or seek care.
If your child has been diagnosed with ATRT, contact your care team for any questions you have. If your child has ATRT and is having a medical problem, come to the hospital. Our surgeons, oncologists, neurologists, social workers, child life specialists and other experts are all available to help you when you need it.
Why Choose Nationwide Children's?
The Neuro-Oncology Program at Nationwide Children’s Hospital offers clinical excellence in treating children, adolescents, and young adults with brain and spine tumors. Your child will have access to doctors who are experts in different types of medical care. We call this multidisciplinary care. Depending on the specific tumor diagnosis and treatment plan, your team may include oncologists, neurosurgeons, radiation oncologists, clinical psychologists, rehabilitation medicine doctors, neurologists, endocrinologists, ophthalmologists, therapists (physical, occupational, speech, art, music), social workers, dietitians and/or pharmacists. These team members all work together with the family to provide the best care for the patient.
Cancer Clinical Research
Nationwide Children’s is a national leader in oncology research and clinical trials. There are many ongoing research studies aimed at improving outcomes for children and young adults with brain/spine tumors—understanding why these tumors develop and how to appropriately target them. We are members of all major pediatric brain tumor clinical trial consortia including Pediatric Brain Tumor Consortium (PBTC), Collaborative Network of Neuro-oncology Clinical Trials (CONNECT), Pacific Neuro-oncology Consortium (PNOC) and the Children’s Oncology Group (COG). We have many open clinical trials testing new drugs in difficult-to-treat tumors, including in DIPG.
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