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?
- We are 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..
- By working closely with the pediatric neurosurgeons 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 atkids oncocare s unmatched— averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
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- 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.
- We 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.
