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Atypical Teratoid/Rhabdoid Tumor (AT/RT)

An atypical teratoid/rhabdoid tumor (AT/RT) is a fast-growing, aggressive and malignant tumor. It affects your central nervous system (brain and spinal cord). It’s more common among children than adults. Your child’s outlook varies based on the size and location of the tumor. Treatment options are available.

Overview

What is AT/RT?

An atypical teratoid/rhabdoid tumor (AT/RT) is a fast-growing central nervous system cancer. Cancer cells begin growing in your brain or spinal cord. Around half of all AT/RT cases start in your cerebellum or brainstem. It most often affects children.

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Symptoms of AT/RT can come on suddenly. It may seem like your child has a common illness at first, but their symptoms don’t get better with time or traditional therapies. A healthcare provider will recommend testing to understand why your child isn’t feeling well. After diagnosing AT/RT, they’ll offer treatment options specific to your child’s situation.

This type of tumor has a generally poor outcome. It spreads quickly and can be hard to remove. As a result, it may shorten your child’s life expectancy. However, not all cases have a poor outcome. Researchers are studying new treatment options every day to help improve your child’s chance of survival.

There are a lot of uncertainties when you learn about a cancer diagnosis, especially a rare cancer like AT/RT. Remember that your child’s care team will be with you throughout their journey. Support is available for families and caregivers as well.

What type of cancer is AT/RT?

AT/RT is a rare type of brain cancer that affects your central nervous system (CNS). Your CNS includes your brain and spinal cord.

How rare is AT/RT?

In the United States, one study estimated that 470 people are living with AT/RT. This equals about 73 people who receive this diagnosis each year. Only 4 people out of 73 are adults.

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Symptoms and Causes

What are the symptoms of AT/RT?

The signs and symptoms of AT/RT vary based on your age and where the tumor is in your body. The following symptoms may happen suddenly and get worse quickly:

  • Headaches (usually in the morning, which go away after vomiting).
  • Nausea and vomiting.
  • Fatigue.
  • Activity level changes (low energy).
  • Difficulty with balance, coordination and walking.

Parents or caregivers may notice that infants have a larger head size because of this tumor, but it may not be as noticeable in older children.

What causes AT/RT?

A genetic change (mutation) to either the SMARCB1 or SMARCA4 gene causes AT/RT. These are tumor suppressor genes. They make a protein that regulates when and how often cells grow. A change to one of these genes can cause cells to grow uncontrollably, which leads to tumors.

Is AT/RT genetic?

Yes, some cases of AT/RT are genetic. You can inherit the gene change (germline mutation) that causes the tumor from your biological parents. But most cases aren’t inherited. Instead, the gene change happens randomly (sporadically) without a history in your biological family.

What are the risk factors for AT/RT?

Most AT/RTs affect children younger than 3 years old. But it can happen to both children and adults at any age.

Diagnosis and Tests

How is AT/RT diagnosed?

A healthcare provider will diagnose AT/RT after a physical exam, a neurological exam and testing. During the exams, your child’s provider will learn more about their symptoms, medical history and family medical history.

Testing may include:

Management and Treatment

How is AT/RT treated?

Your child’s healthcare provider may recommend the following to treat AT/RT:

  • Removal surgery: A neurosurgeon will surgically remove as much of the tumor as possible. Your child may need chemotherapy and radiation therapy after surgery to destroy any remaining cancer cells.
  • Chemotherapy: Chemotherapy is a medication that destroys cancer cells or stops them from dividing (creating more cells). Your child’s provider may treat AT/RT with oral (by mouth) medications or medicine injected into your child’s vein or muscle (systemic chemotherapy). Or your child’s provider will inject chemotherapy medication into the fluid surrounding your child’s spinal cord (intrathecal chemotherapy).
  • Radiation therapy: Radiation therapy uses high-powered X-rays to destroy cancer cells or prevent them from growing. A machine will direct beams of radiation toward the tumor in your child’s body. Children younger than 3 years old may need a low dose of radiation, as it can affect their growth and development.
  • Stem cell transplantation: After high-dose chemotherapy, your child may undergo stem cell transplantation to replace cells they lost. Before chemotherapy, your child’s provider will remove and store some of your child’s stem cells. They’ll intravenously (through a needle into a vein) put these cells back into your child’s body after chemotherapy.
  • Targeted therapy: This is a newer type of treatment currently in clinical trials for AT/RT. Certain medications prevent components of a cell from doing their job in your child’s body, which can stop cancer cells from growing and multiplying.
  • Immunotherapy: Immunotherapy is in clinical trials for AT/RT. It helps your child’s immune system fight cancer.
  • Palliative care: Palliative care provides symptom relief, comfort and support to your child to help improve their quality of life.

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A healthcare provider will determine what type of treatment they recommend after reviewing diagnostic and imaging tests. Your child may need more than one type of treatment. Follow-up care will be important throughout your child’s life. After treatment, they’ll likely need additional testing to ensure the treatment worked and that cancer hasn’t returned.

Who is on my child’s care team?

You’ll likely see a lot of different healthcare providers during treatment for AT/RT. Your child’s care team may include:

Are there side effects of the treatment?

Side effects are possible with all types of AT/RT treatment. Your child’s provider will explain what the potential side effects are and what to look out for during treatment. Some side effects may happen during treatment, while others may not appear until months after. If you have any questions, let your child’s provider know.

Prevention

Can AT/RT be prevented?

There isn’t a way to prevent AT/RT. If you plan on expanding your family, you may choose to speak with a counselor about genetic testing to learn more about your risk of having a child with a gene change that could cause AT/RT.

Outlook / Prognosis

What’s the outlook for AT/RT?

As AT/RT is an aggressive type of cancer, it may lead to early death if treatment doesn’t remove it from your child’s body. This varies significantly from person to person. For example, a cure is possible if surgeons can completely remove the tumor during a procedure.

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The outlook varies based on several factors, including:

  • Your child’s age.
  • Their genetic inheritance.
  • How much of the tumor can safely be removed during surgery.
  • If the cancer spread to other parts of your child’s body.

Your healthcare provider will give you the most accurate information about your child’s outlook (prognosis). If you have any questions about what to expect, talk to your child’s care team.

What is the survival rate and cure rate for AT/RT?

Because AT/RT is a rare type of cancer, there isn’t enough data available to determine the survival rate and cure rate for this condition. Your child’s provider will have the most up-to-date information for your child’s situation.

Living With

What questions should I ask my child’s healthcare provider?

You probably have a lot of questions after learning about AT/RT. Be sure to ask your child’s provider:

  • Where is the tumor in my child’s body?
  • What type of treatment do you recommend?
  • Are there side effects of treatment?
  • How will treatment affect my child’s growth and development?
  • What’s my child’s prognosis?

A note from Cleveland Clinic

It’s one of the most difficult things you can hear: Your child has cancer. You may be worried about what comes next and what your child’s future may look like. While there are many unknowns to a cancer diagnosis, know that your child’s care team will be with you every step of the way. They’re available to help you navigate treatment, manage symptoms and care for your child throughout their life. Research is ongoing to learn more about atypical teratoid/rhabdoid tumors (AT/RT) and ways to treat it, so don’t give up hope.

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Medically Reviewed

Last reviewed on 09/27/2024.

Learn more about the Health Library and our editorial process.

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