What is an atypical teratoid rhabdoid tumor (ATRT)?
Atypical teratoid rhabdoid tumor (ATRT) is a rare, fast-growing cancer of the central nervous system (the brain and spinal cord). Most often, ATRT affects young children, especially boys under age 3. In some cases, it can occur in older children and adults.
What causes atypical teratoid rhabdoid tumor (ATRT)?
A genetic change (mutation) in the SMARCB1 or SMARCA4 gene causes ATRT. These genes are tumor suppressor genes, which help protect the body from cancer.
Some children inherit this from a parent, which means they may have a higher risk of developing other tumors. But over 90% of children with ATRT have no family history of the condition, and the genetic change happens randomly as the brain develops.
What are the signs and symptoms of atypical teratoid rhabdoid tumor (ATRT)?
Depending on the size and location of the tumors, ATRT can cause a variety of symptoms. Many of these symptoms are similar to symptoms caused by more common childhood conditions or medical problems. Talk to your child's doctor if you have concerns about your child’s symptoms.
Because ATRT tumors grow quickly, symptoms can happen fast — sometimes appearing in a few days or weeks. These symptoms include:
- Headache, usually when the child wakes up in the morning
- Increased head size in infants, caused by hydrocephalus (a buildup of fluid in the brain)
- Low energy (lethargy) and fatigue
- Nausea and vomiting, often worse in the morning
- Trouble with balance and coordination
How do we diagnose atypical teratoid rhabdoid tumor (ATRT)?
We diagnose ATRT using a combination of different exams and tests, including:
- Physical and neurological exams to look for symptoms like increased head size, head tilt or muscle weakness and paralysis on one side of the child’s body
- Imaging studies, including:
How is atypical teratoid rhabdoid tumor (ATRT) treated?
Depending on several factors, our team provides an individualized treatment plan for your child. Those factors may include:
- Your child’s age, overall health and medical history
- The type, location and size of the tumor
- Whether the cancer has spread
Your child’s treatment may include:
- Neurosurgery to remove as much of the tumor as possible.
- Radiation therapy to shrink tumors or kill cancer cells left behind after surgery. In most cases, we use radiation for children over 3 years old. For younger children, we may use a lower, safer dose of radiation that targets a smaller area.
- Chemotherapy drugs to prevent cancer cells from growing or multiplying. There are many ways to give chemotherapy drugs. With ATRT, we sometimes use a technique called intrathecal chemotherapy to deliver the drugs directly into the fluid around the brain and spinal cord. There are two different ways to deliver intrathecal chemotherapy. We may place a small dome-shaped container (called an Ommaya reservoir) under your child’s scalp to send the drugs through a tiny tube into the brain. As an alternative, we may inject the drugs directly into your child’s spinal fluid in the lower part of the spine.
- A stem cell transplant after high doses of chemotherapy. We give your child healthy bone marrow cells to help them recover from chemotherapy and make new, healthy cells. Before the stem cell transplant, your child may need outpatient surgery to put a thin, flexible tube called a central venous catheter (CVC) in their chest. We use the CVC during and after the transplant to draw blood and give your child medicines, stem cells and any blood products they need.
Helpful resources for atypical teratoid rhabdoid tumor (ATRT)
Why choose us for treatment of atypical teratoid rhabdoid tumor (ATRT)?
Rare cancers like ATRT benefit from a team approach to care. Our experienced, child-focused cancer experts, including oncologists, pediatric surgeons, radiologists and pathologists, work together to give your child comprehensive and individualized care.
With us, you can feel confident that your child is getting trusted, high-quality care from nationally recognized experts. U.S. News & World Report consistently ranks our Center for Cancer and Blood Disorders among the top children’s hospitals for cancer treatment. Your child will also benefit from access to the most advanced and effective treatments. Through partnerships, including with the University of Colorado Cancer Center, we lead research and clinical trials to help ensure your child gets the most current and effective ATRT treatments available.
Our team doesn’t work alone. As members of the Children’s Oncology Group (COG), our surgeons and oncologists collaborate with leading childhood cancer specialists from across the U.S. and Canada. This shared expertise helps us create a treatment plan tailored to your child’s specific needs. You can rest assured that the brightest minds in childhood cancer care are making the best choices for your child.