Disease Information

Brain Tumor: Atypical Teratoid / Rhabdoid Tumor (ATRT)

Alternate Names: ATRT

Definition

This rare, high-grade tumor occurs most commonly in children younger than 2. It can be found anywhere in the brain. These tumors tend to be aggressive and frequently spread through the central nervous system.

Incidence

This diagnosis has been classified only in the last five to ten years; the tumor was once thought to be a subset of medulloblastomas, but now we know that is not true. They occur in about 1-2 percent of children with brain tumors.

Survival Rates

Even after surgery and chemotherapy treatment, the survival rate for children younger than 3 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 70 percent.

Treatment Strategies

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.

Current Research

 

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