Patient Update / Ben

Patient Update / Atypical teratoid/rhabdoid tumor (ATRT


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Atypical teratoid/rhabdoid tumor (ATRT)

This rare, high-grade tumor occurs most commonly in children younger than 2 years of age. It is generally found in the cerebellum, which is the lower, back part of the brain that controls balance. These tumors tend to be aggressive and frequently spread through the central nervous system. This diagnosis has been classified only in the last three to five years.

Even after surgery and chemotherapy treatment, the survival rate for children younger than 3 years old at diagnosis is less than 10 percent. It appears that older children, when treated with chemotherapy and radiation therapy after surgery, do somewhat better long-term, nearing a 70 percent survival rate.

Treatment generally involves surgical removal of the tumor followed by chemotherapy. Radiation therapy may be considered depending on the age of the child and whether the tumor has recurred.

Researchers are investigating new, more effective methods of treating brain tumors in infants and young children. High doses of radiation can’t be used with very young children because it may cause permanent problems with thinking, learning, and growing. It has been standard therapy to administer chemotherapy in an attempt to delay radiation therapy until the child is older and thus allowing the brain more time to develop; however, chemotherapy alone has not been effective in fighting brain tumors.

New radiation techniques at St. Jude that minimize damage to healthy tissue that surrounds brain tumor tissue are under investigation. Stem cell transplantation as a part of treatment continues to be studied.