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Atypical Teratoid Rhabdoid Tumor (ATRT) Diagnosis and Treatment


MRI of an Atypical Teratoid Rhabdoid Tumor (ATRT) in the brain.

Credit: NCI-CONNECT Staff

ATRT is a primary central nervous system (CNS) tumor. This means it begins in the brain or spinal cord.

To get an accurate diagnosis, a piece of tumor tissue will be removed during surgery, if possible. A neuropathologist should then review the tumor tissue.

What is the grade of ATRTs?

Primary CNS tumors are graded based on the tumor location, tumor type, extent of tumor spread, genetic findings, the patient’s age, and tumor remaining after surgery, if surgery is possible.

ATRTs are all classified as grade 4 (also written as grade IV) tumors. This means they are malignant (cancerous) and fast-growing.

What do ATRTs look like on an MRI?

ATRTs usually appear very large with fluid-filled areas that often brightens with contrast. You can often see areas of bleeding or dead tissue.

What causes ATRTs?

Cancer is a genetic disease – that is, cancer is caused by certain changes to genes that control the way our cells function. Genes may be mutated (changed) in many types of cancer, which can increase the growth and spread of cancer cells. Most ATRTs are caused by changes in a gene known as SMARCB1 (also called INI1). This gene normally signals proteins to stop tumor growth. But in ATRTs, SMARCB1 doesn’t function properly and tumor growth is uncontrolled. SMARCB1 can sometimes be found in a person’s DNA, which means they are born with it.

Where do ATRTs form?

ATRTs can form anywhere in the CNS. They often occur in the brain and often spread to the spinal cord. But ATRTs rarely begin in the spinal cord. ATRTs can form outside the CNS, such as in the kidney, but this is rare. ATRTs develop from several different very young cells called embryonal cells. These cells include rhabdoid, neuroepithelial, epithelial and mesenchymal.

Do ATRTs spread?

ATRTs can be very fast-growing. They often spread to other areas of the CNS through cerebrospinal fluid (CSF).

What are the symptoms of an ATRT?

Symptoms related to an ATRT depend on the tumor’s location and the person’s age. Here are some possible symptoms that can occur.

ATRT Symptoms
  • Morning headaches
  • Vomiting
  • Changes in activity levels
  • Loss of balance
  • Increase in head size (in infants)

Since ATRTs are fast-growing, symptoms usually get worse quickly.

Who is diagnosed with ATRTs?

ATRTs occur in both children and adults and are very rare in both age groups. There have been only 50 reported cases in adults. An estimated 596 people are living with this tumor in the United States. ATRTs occur slightly more often in males than females.

ATRT Statistical Chart

What is the prognosis of ATRTs?

The likely outcome of the disease or chance of recovery is called prognosis.

ATRT Prognosis

The relative 5-year survival rate for ATRTs is 32.2% but know that many factors can affect prognosis. This includes the tumor grade and type, traits of the cancer, the person’s age and health when diagnosed, and how they respond to treatment. If you want to understand your prognosis, talk to your doctor.

What are treatment options for ATRTs?

The first treatment for an ATRT is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and to remove as much tumor as possible without causing more symptoms for the person.

People with ATRTs usually receive further treatments after surgery, which may include radiation, chemotherapy or clinical trials. Clinical trials, with new chemotherapy, targeted therapy, or immunotherapy drugs, may also be available and can be a possible treatment option. Treatments are decided by the patient’s healthcare team based on the patient’s age, remaining tumor after surgery, tumor type, and tumor location.

Open Clinical Studies for ATRTs

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