Treatments for Atypical Teratoid Rhabdoid Tumor (ATRT) in Children

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How is treatment for AT/RT determined?

If your child has been diagnosed with AT/RT, you'll naturally want to know how your child's physician may treat the tumor. Specific treatment to cure AT/RT will depend on:

  • type, location, and size of the tumor
  • extent of the disease
  • your child's age, overall health and medical history
  • how your child's doctors expect the disease to progress
  • your child's tolerance for specific medications, procedures or therapies 

What are the treatments for AT/RT?

There are a number of treatments that your child's physician may recommend. Some of them help to treat the tumor while others are intended to complications of the disease or side effects of the treatment. If your child is diagnosed with AT/RT, treatment may include:


Surgery

Surgery may be performed by one of our experienced pediatric neurosurgeons to confirm the diagnosis of AT/RT (biopsy) or to remove as much of the tumor as possible (resection).

  • In the case of biopsy, tumor specimens are examined by one of our neuropathologists to determine the exact diagnosis.
  • Due to the rapid growth and spread of AT/RTs, complete removal of the tumor by resection is generally not possible.

Radiation therapy

In addition to surgery, your child may receive precisely targeted and dosed radiation therapy in order to kill cancer cells left behind after surgery. This treatment is important to control the local growth of tumor. If the AT/RT has spread, we may recommend radiation to the whole brain and spinal cord, depending on your child's age and the tumor location.

  • Because AT/RT often occurs in children younger than 3, we may recommend reducing the intensity of radiation therapy or avoiding it all together.
  • Children over 3 generally receive radiation therapy as a part of treatment.
  • Radiation therapy can cause long-term effects. Your child's physician will work with our pediatric radiation oncologists to determine the optimal dose of radiation that will be effective in treating the tumor while also minimizing side effects. 

Chemotherapy

Chemotherapy is the use of drugs that interfere with the cancer cell's ability to grow or reproduce. Your child's physician may recommend chemotherapy before surgery in order to help shrink the tumor and make complete removal more likely.

While chemotherapy can be quite effective in treating certain cancers, the agents don't differentiate healthy cells from cancer cells. Because of this, there can be side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring.

Chemotherapy is systemic treatment, meaning it is introduced into your child's bloodstream and travels throughout the body to kill cancer cells. Your child may be given chemotherapy:

  • orally, as a pill to swallow
  • intramuscularly, as an injection into the muscle or fat tissue
  • intravenously, directly to the bloodstream (also called IV)
  • intrathecally, directly delivering the chemotherapy into the spinal column with a needle

In the case of AT/RT and other brain tumors, intrathecal chemotherapy is often an important part of treatment as it allows these medications to be delivered directly to the central nervous system to treat the tumor.

Stem cell transplant

Inside many of the body's bones is a special tissue called bone marrow that produces a number of important blood cells. Bone marrow transplantation (also called a stem cell transplant)[LINK: Stem cell transplantation condition page] is a therapy for children with cancer or other diseases that affect the bone marrow. In some cases, your child's physician may recommend stem cell transplant as a treatment for AT/RT.

  • The goal of stem cell transplant is to transfuse healthy bone marrow cells into your child after his unhealthy bone marrow has been eliminated.
  • The use of stem cell transplantation allows children with aggressive cancers, such as AT/RT, to receive higher doses of chemotherapy.

What is the expected outcome (prognosis) after treatment?

AT/RT is a very difficult tumor to cure and, unfortunately, the outcomes remain poor. In general, older children with AT/RT tend to do better than young children. Cure rates for children over 3 are about 70 percent, while cure rates for children younger than 3 remain below 15 percent.

Coping and support

We understand that you may have a lot of questions if your child is diagnosed with a brain tumor. Will it affect my child long term? What do we do next? We've tried to provide some answers to those questions in the following pages, but there are also a number of other resources to help you and your family through this difficult time.
 
Patient education: From the first visit through follow-up care, our nurses will be on hand to walk you through your child's treatment and help answer any questions you may have — How long will my child be in the hospital? How often will my child require follow-up? They will also reach out to you by phone, continuing the care and support you received while at Children's.
 
Parent to parent: Want to talk with someone whose child has been treated for a pediatric brain tumor? We can put you in touch with other families who have been through the same experience that you and your child are facing, and share with you their experience at Children's.
 
Faith-based support: If you are in need of spiritual support, we will help connect you with the Children's chaplaincy. Our program includes nearly a dozen clergy representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian and United Church of Christ traditions who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.
 
Social work: Our clinical social workers have helped many other families in your situation. Your social worker can offer counseling and assistance with issues such as coping with your child's diagnosis, stresses relating to coping with illness and dealing with financial difficulties.

Click here for more information on our pediatric support services.

Long-term follow-up

Survivors of pediatric brain tumors may face physical, psychological, social and intellectual challenges related to their treatment and will require ongoing assessment and specialized care.

To address the needs of these patients, Dana-Farber/Children's Hospital Cancer Care established the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic.

Today, more than 900 pediatric brain tumor survivors of all ages are followed by the Outcomes Clinic, a multi-disciplinary program designed to address long-term health and social issues for families and survivors of childhood brain tumors. Some of the post-treatment services provided by the Outcomes Clinic include:

  • MRI scans to monitor for tumor recurrences
  • intellectual function evaluation
  • endocrine evaluation and treatment
  • neurologic assessment
  • psychosocial care
  • hearing, vision monitoring
  • ovarian dysfunction evaluation and treatment
  • motor function evaluation and physical therapy
  • complementary medicine

As a result of treatment, children may experience changes in intellectual and motor function. Among several programs addressing these needs are the School Liaison and Back to School Programs, which provide individualized services to ease children's return to school and maximize their ability to learn.

In addition to providing thorough and compassionate care, our Outcomes Clinic specialists conduct innovative survivorship research and provide continuing education for staff, patients and families.

To learn more about our services or to schedule an appointment, please click here, or call us at 617-632-2680.

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

Boston Children's Hospital 300 Longwood Avenue, Boston, MA 02115 617-355-6000 | 800-355-7944

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