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  • Atypical Teratoid Rhabdoid Tumor (AT/RT)

    Atypical teratoid rhabdoid tumors (AT/RT) are very rare, aggressive tumors of the central nervous system, occurring mostly in the cerebellum (the part of the brain that controls movement and balance) and the brain stem (the part of the brain that controls basic body functions). AT/RT usually occurs by age 3, but arises in older children, as well.

    These tumors are part of a larger group of malignant tumors called rhabdoid tumors which can occur outside the brain. AT/RT may be localized to one part of the brain or spread to other locations in the brain, central nervous system or body.

    AT/RT Treatment at Dana-Farber/Boston Children's

    Children with atypical teratoid rhabdoid tumors (AT/RT) are treated through the Brain Tumor Center at Dana-Farber/Boston Children's Cancer and Blood Disorders Center, a world leader in treating this extremely rare disease. Through clinical trials, our researchers are testing new, targeted treatments that may help improve the survival rate for AT/RT.

    Symptoms & Diagnosis

    AT/RT can grow very rapidly, with symptoms developing quickly over a period of days to weeks. The most common symptoms are:

    • Headache (generally upon awakening in the morning)
    • Nausea and vomiting
    • Lethargy
    • Fatigue
    • Balance and coordination difficulties
    • Hydrocephalus (swelling inside the brain)

    Doctors diagnose AT/RT with:

    • A physical examination, including a neurological function test of reflexes, muscle strength, eye and mouth movement, coordination and alertness
    • Diagnostic imaging 
    • Biopsy
    • Lumbar puncture (spinal tap)
    • Genetic testing to look for the specific genetic defect associated with AT/RT

    After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    The most common treatments for AT/RT are:

    • Neurosurgery – due to the rapid growth of these tumors, complete surgical removal is generally not possible
    • Radiation therapy – because AT/RT occurs in children under 3, reduced intensity radiation therapy is used or avoided altogether; children over 3 generally receive radiation therapy as standard treatment
    • Chemotherapy – intrathecal chemotherapy is an important part of treatment as it allows medicine to be delivered directly to the central nervous system to treat the tumor
    • Stem cell transplant – this allows children with aggressive AT/RT to receive high doses of chemotherapy

    Progressive or Recurrent Disease

    There is no standard treatment for recurrent AT/RT. Children with recurrent disease are often referred to clinical trials for the latest experimental therapies.

    Long-term Outlook

    AT/RT is very difficult to cure and outcomes remain poor. In general, older children with AT/RT tend to do better than younger children. The cure rate for children over 3 is about 70 percent, but below 15 percent for those under 3.