Atypical Teratoid Rhabdoid Tumor (ATRT)

What is an atypical teratoid rhabdoid tumor?

An atypical teratoid rhabdoid tumor (ATRT) is a very rare, aggressive tumor of the central nervous system, occurring mostly in the cerebellum, the part of the brain that controls movement and balance, or the brain stem, the part of the brain that controls basic body functions.

ATRTs usually occur by age 3, but sometimes are found in older children. ATRTs represent only 1 to 2 percent of childhood brain tumors. These tumors are part of a larger group of malignant tumors called rhabdoid tumors, which also can occur outside the brain. ATRTs may be localized to one part of the brain, or they can spread to other locations in the brain, spine or body.

How is ATRT classified?

Successfully treating your child’s ATRT depends on where the tumor is located and whether the tumor has spread.

For many tumors, we use a system of classification called “staging” to evaluate cancers. However, there is currently no standardized classification system for atypical teratoid rhabdoid tumors. An ATRT may be:

  • localized, occurring in only one location in the brain
  • disseminated, spread to multiple locations in the brain, spinal cord or body

How we care for ATRTs

Children with ATRTs are treated at Dana-Farber/Boston Children's through our Brain Tumor Center, a world leader in treating this rare disease. Our brain tumor specialists have extensive expertise in treating all types of neural tumors, including ATRTs.

At Dana-Farber/Boston Children’s, your child will:

  • receive care from neuro-oncologists, neurosurgeons, neurologists, and pediatric subspecialists
  • have access to technological advances such as the intra-operative MRI, which allows pediatric neurosurgeons to see the tumor as they operate and remove as much of it as possible
  • receive expert diagnosis by neuropathologists who use advanced molecular diagnostic testing

Our areas of ATRT research

Because ATRT is so rare, these tumors are difficult to study. As a result, our researchers collaborate with other institutions around the country to improve our current understanding of this condition and offer new treatments to children with ATRT. Currently, researchers at Dana Farber/Boston Children’s are working with the Children’s Hospital of Philadelphia and the Broad Institute of MIT and Harvard to learn more about the molecular characteristics of atypical teratoid rhabdoid tumors.

In addition, researchers in our Brain Tumor Center are collaborating with physicians and researchers that treat other types of rhabdoid tumors in order to study the effectiveness of current treatments and to establish new, more standardized treatments for all types of rhabdoid tumors.

Through clinical trials, our researchers are testing new, targeted treatments that may help improve the survival rate for ATRT.

Most children with a diagnosis of ATRT will be treated as part of a brain tumor clinical trial. In addition to launching our own clinical trials, we also offer trials available through collaborative groups such as the Children's Oncology Group (COG) and the Pacific Pediatric Neuro-Oncology Consortium (PNOC). We are also the New England Phase I Center of the Children's Oncology Group. If your child has a progressive or recurrent tumor, they may be eligible for a number of experimental therapies available through these groups or from one of our independent clinical investigators.