Skip to Main Content

Atypical Teratoid Rhabdoid Tumor (ATRT) Treatment

Also called: ATRT or AT/RT

Atypical teratoid/rhabdoid tumor (also called AT/RT or ATRT) is an aggressive malignant tumor. It can be in the brain or spinal cord. AT/RT is rare and is most often seen in children 3 years old or younger. It is found in fewer than 10% (1 out of 10) children with brain tumors.

AT/RT often appears to result from either a loss or a mutation in a gene that slows down growth. Without the normal function of this gene, tumor growth can be uncontrolled. Most cases of AT/RT are related to this gene, and your child’s tumor should be tested for this to confirm the diagnosis. This gene defect commonly occurs only with AT/RT, but it may also be inherited (passed down from family members). Your doctor will discuss the need for genetic testing.

Find out more about AT/RT on the Together by St. Jude online resource.

Treatment of atypical teratoid/rhabdoid tumor

Treatment depends on:

  • The size of the tumor
  • The location of the tumor
  • Your child’s age

These tumors can be aggressive. Most patients receive several treatment types, including:

  • Surgery is used to diagnose AT/RT and to remove all or part of the tumor.
  • Chemotherapy (chemo) uses powerful medicines to kill cancer cells or stop them from spreading. The order of chemotherapy and radiation can depend on your child’s age.
  • Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or stop them from growing.

Atypical teratoid rhabdoid tumor clinical trials

St. Jude offers clinical trials and cancer research studies for children, teens, and young adults with AT/RT and other brain tumors.  Learn more about clinical research at St. Jude.

PEPN2121: Tiragolumab and Atezolizumab for SMARCB1- or SMARCA4-deficient Tumors

Study goal:

Tiragolumab and atezolizumab will be used to treat pediatric and young adult patients with SMARCB1- and SMARCA4-deficient tumors.


Phase 1: ages 1–17 years; Phase 2: age 18 and older

Atypical teratoid/rhabdoid tumor care at St. Jude

St. Jude provides the highest quality of care for patients with AT/RT:

  • We bring together medical experts and specially trained staff to care for your child. We also work with brain surgeons (neurosurgeons) at Le Bonheur Children’s Hospital.
  • We have a special team that helps understand the makeup of the tumor. This team includes doctors and scientists who study:
    • Cancer cells
    • How the cancer happened
    • Current medicines and treatments to kill cancer cells
    • What makes cancer cells grow and what new medicines can stop them
    • New medicines and other therapies to improve how treatments work
  • Our brain tumor clinical trials have led to better therapies in children. These improvements include lower-dose therapies, targeted therapy, and proton therapy. These treatments kill cancer cells while sparing healthy cells and may lessen the severity of side effects that affect brain function after treatment for some types of brain tumors.
  • The St. Jude Proton Therapy Center is the first center in the world that treats only children with this therapy.
  • Along with the treatment studies constantly being developed at St. Jude, the staff also treats patients on studies developed by the Children’s Oncology Group, the Pediatric Brain Tumor Consortium, and the Pacific Pediatric Neuro-Oncology Consortium. Many St. Jude staff members play key roles in developing these studies. They are large tests carried out at multiple centers across the United States. 
  • St. Jude offers a dedicated team of specialists to meet the needs of children with brain tumors, including:

More reasons to choose St. Jude for care include:

  • We are consistently ranked among the best childhood cancer centers in the nation by US News & World Report.
  • At St. Jude, we have created an environment where children can be children and families can be together.
  • We lead more clinical trials for childhood cancer than any other hospital in the U.S. 
  • St. Jude is the only National Cancer Institute–designated Comprehensive Cancer Center just for children. A Comprehensive Cancer Center meets rigorous standards for research that develops new and better approaches to prevent, diagnose, and treat cancer.
  • The nurse-to-patient ratio at St. Jude is about 1:3 in hematology and oncology and 1:1 in the Intensive Care Unit.
  • Patients may be able to get expert, compassionate care and treatment closer to their homes through our St. Jude Affiliate clinics.

Get more information about the Brain Tumor Treatment program at St. Jude.

A statue of children running and holding hands

Seeking treatment at St. Jude

Patients accepted to St. Jude must have a disease we treat and must be referred by a physician or other qualified medical professional. We accept most patients based on their ability to enroll in an open clinical trial.

How to seek treatment

Contact us

You can start the referral process by letting your physician know you would like a second opinion from St. Jude. They can contact our 24-hour beeper service or contact our brain tumor coordinator. You may also contact the brain tumor coordinator directly with questions.

Learn more about the types of brain tumors we treat at St. Jude and our brain tumor eligibility requirements.

Physician / Patient Referral Office

Call: 1-888-226-4343 (toll-free) or 901-595-4055 (local)  | Email:
Fax: 901-595-4011 | 24-hour pager: 1-800-349-4334

Contact the Brain Tumor Team directly:

Submit brain tumor contact form

Contact the Surgery Team directly:


Learn more