What is atypical teratoid rhabdoid tumor?
Atypical teratoid rhabdoid tumor (ATRT) is a rare and fast-growing cancerous tumor of the brain and spinal cord. About half of these tumors begin in the cerebellum or brain stem:
- The cerebellum, located at the base of the brain, controls movement, balance and posture.
- The brain stem controls breathing, heart rate and all the muscles used in seeing, hearing, walking, talking and eating.
ATRT often appears to result from changes in a gene that normally makes proteins to stop tumor growth. In ATRT, this gene does not function properly, the protein is not made and tumor growth is uncontrolled. More than 90% of cases of ATRT are related to this gene defect. While this defect commonly occurs only within the cancer, this gene defect may be inherited and your doctor can discuss a need for genetic testing.
How common are atypical teratoid rhabdoid tumors?
- ATRT is very rare and is found in fewer than 10% of children with brain tumors.
- The disease is most often seen in children age 3 or younger, but it can also occur in older children and adults.
What are the symptoms of an atypical teratoid rhabdoid tumor?
ATRT symptoms vary widely depending on the patient’s age and the tumor’s location. Because ATRTs are fast-growing, symptoms usually progress rapidly in a short period of time.
Symptoms of ATRT may include the following:
- Morning headaches or headaches that are less painful after vomiting
- Nausea and vomiting
- Changes in activity levels
- Feeling sleepy
- Loss of balance, increasing problems with coordination or trouble walking
- Asymmetric eye movements or face movements
- Increase in head size (in infants)
How is an atypical teratoid rhabdoid tumor treated?
Treatment depends on the size and location of the tumor as well as the patient’s age. Because of the aggressive nature of these tumors, most patients receive several types of treatment, which may include surgery, chemotherapy and radiation.
- Surgery — is used to both diagnose and treat ATRT:
- The surgeon removes part of the patient’s skull, uses a needle to take out a sample (biopsy) of the tumor and sends it to the laboratory. There, scientists examine the sample under the microscope.
- If cancer is confirmed, the surgeon continues the operation by taking out as much of the tumor as it is safe to remove.
- Chemotherapy (“chemo”) — uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells. Chemo is usually given after surgery.
- Chemo may be injected into the bloodstream, so that it can travel throughout the body.
- Some chemo may be given by mouth.
- Combination therapy uses more than one type of chemo at a time.
- Radiation therapy — uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing.
What are the survival rates for atypical teratoid rhabdoid tumor?
ATRT is an aggressive form of cancer and is difficult to cure. Survival is poor, but treatment advances are being made. Current advances in therapy have helped older children, and children with tumors that can be completely removed have an improved survival. New therapy is being investigated in clinical trials. The goal is to increase the cure rates and improve survival in very young children and in children who have disease that cannot be surgically removed.
Why choose St. Jude for your child’s atypical teratoid rhabdoid tumor treatment?
- St. Jude has one of the largest pediatric brain tumor programs in the country.
- The St. Jude staff is dedicated to treating children who have serious illnesses. Here, you will find a hospital and staff that has only one focus—your child.
- St. Jude is committed to not only finding cures, but also helping children hold on to their quality of life. Children are not just small people. Their bodies and systems are unique, along with the disorders that affect them. For the best care, they need a specialized team. That is what you get from the St. Jude Brain Tumor Program.
- By working closely with the pediatric neurosurgeons at Le Bonheur Children’s Hospital in Memphis, the St. Jude Brain Tumor Program offers an expert team of doctors from many medical fields. The team includes: neuro-oncologists, who study and treat cancer in the brain and nervous system; neurosurgeons, who operate on the brain, skull, scalp and spinal cord; and radiation oncologists, who treat cancer with radiation. All of these doctors have devoted their careers to finding cures and providing treatment for children with brain tumors. This diverse knowledge is vital to the complete care of children with brain tumors.
- Our brain tumor doctors also work closely with support staff in nursing, rehabilitation services, neuropathology, pharmacy, neuroradiology, neuropsychology, clinical nutrition, child life and social work, along with many others. Support staff members have been specially trained to care for children with brain tumors.
- The nurse-to-patient ratio at St. Jude is unmatched— averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
- The St. Jude Brain Tumor Program has made great advances in treatments for brain tumor patients. Some of the world’s best neuroscientists, cancer biologists and doctors from the St. Jude Neurobiology and Brain Tumor Divisions combine their efforts to seek cures. In this interactive program, discoveries about the biology of both normal and harmful cell growth are rapidly tested in clinic studies. These studies include Phase I, II and III trials of the most common and deadly forms of childhood brain tumors.
- Being able to take a drug directly from the lab to your child is one of the major advantages of centers like St. Jude. Before treating a patient with any new drug, the St. Jude staff does many tests in the lab. The tests show if the drug is safe, if it works, and the best way to use it to help patients.
- St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
- 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, which are large tests carried out at centers across the United States.
- St. Jude practices patient family-centered care. Patient family-centered care is health care that focuses on the family as a child’s primary source of strength, support and well-being. At the heart of patient family-centered care is the belief that health care staff and the family are partners, working together to best meet the needs of the child. Excellence in health care happens when we work together and honor the expertise each of us brings to every health care encounter.
- St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.
Contact the Brain Tumor Team any time, 24 hours a day, 7 days a week.
Call our Brain Tumor Coordinator, Tabatha E. Doyle, RN:
(901) 595-2544 or
Or email us at firstname.lastname@example.org
Associated Clinical Trials
Phase II Study of Alisertib as a Single Agent in Recurrent or Progressive Central Nervous System (CNS) Atypical Teratoid Rhabdoid Tumors (ATRT) and Extra-CNS Malignant Rhabdoid Tumors (MRT) and in Combination Therapy in Newly Diagnosed ATRT
- Has one of these types of tumors:
- Newly diagnosed atypical teratoid rhabdoid tumors (ATRTs) or synchronous extraneural ATRTs
- ATRTs or malignant rhabdoid tumors (MRTs) that have come back after previous treatment (recurrent disease)
- ATRTs or MRTs that are growing after previous treatment (progressive disease)
- 21 years of age or younger
SJDAWN: Molecular-Based Therapy for Aggressive Brain and Spinal Cord Tumors in Children and Young Adults
Molecularly-Driven Doublet Therapy for All Children with Refractory or Recurrent CNS Malignant Neoplasms and Young Adults with Refractory or Recurrent SHH Medulloblastoma
Ages for Study Enrollment
- Stratum A (ribociclib and gemcitabine): Between 1 and 24 years old with recurrent, progressive or refractory non-WNT, non-sonic hedgehog (SHH) (NWNS) medulloblastoma or ependymoma
- Stratum B (ribociclib and trametinib): Between 1 and 24 years old with recurrent, progressive, or refractory central nervous system (CNS) tumors, including:
- High-grade glioma
- Atypical teratoid rhabdoid tumor (ATRT)
- SHH and WNT medulloblastoma
- CNS embryonal tumors [previously called PNET])
- Stratum C (ribociclib and sonidegib): Between 10 and 39 years old with recurrent, progressive, or refractory SHH medulloblastoma and copy number loss of 9q or PTCH1 mutation. (This stratum is only open to patients with SHH medulloblastoma who are fully grown. Patients younger than 18 years old will have bone age to determine if fully grown)
Screening Phase Eligibility
- Participants with recurrent, progressive, or refractory brain tumors
- At least 1 year old and younger than 25 years old at the time of screening. Exception: Participants with recurrent, progressive or refractory medulloblastoma who are at least 1 year old and younger than 40 years of age at the time of study screening are eligible for screening.
- Participants and/or guardian have the ability to understand and the willingness to sign a written informed consent document according to institutional guidelines.
- Recurrent, progressive or refractory low grade glioma (LGG)
- Prior exposure to a CDK4/6 inhibitor
- History of clinically significant, uncontrolled heart disease and/or repolarization abnormalities
- History of QTc prolongation
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