Atypical Teratoid Rhabdoid Tumor: Causes, Symptoms, and Treatment
Medically reviewed by Achuta Uppu, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Atypical Teratoid Rhabdoid Tumor?
A care team treats this tumor. A pediatric neurosurgeon, a children's brain surgeon, may do the surgery. A pediatric oncologist, a doctor who treats children with cancer, oversees treatment. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is atypical teratoid rhabdoid tumor?
Atypical teratoid rhabdoid tumor (ATRT) is a very rare, fast-growing cancer that begins in the brain and spinal cord. It usually occurs in children aged 3 years and younger, but it can also occur in older children and adults. ATRT is a grade 4 tumor, which means it is cancerous and fast-growing. About half of these tumors form in the cerebellum or brain stem. The cerebellum controls movement, balance, and posture. The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating.
Symptoms
Symptoms depend on the child's age and where the tumor has formed. Because this tumor is fast growing, symptoms may start quickly and get worse over days or weeks. Check with a doctor if a child has:
- A morning headache, or a headache that stops after vomiting.
- Nausea and vomiting.
- Unusual sleepiness, or a change in activity level.
- Loss of balance, or trouble walking.
- An increase in head size in infants.
- Numbness or paralysis in the face.
These symptoms may have other causes. The only way to know is to see a doctor.
Causes and who gets it
Most ATRTs are caused by changes in a gene called SMARCB1 (also called INI1), and less often by changes in a gene called SMARCA4. These genes normally help stop tumor growth. The changes may be inherited, meaning passed on from parents to children. When inherited, tumors may form in two parts of the body at once, for example the brain and the kidney. Not every child with these risk factors gets ATRT. Some children with ATRT have no known risk factor. Genetic counseling may be recommended.
How doctors diagnose it
Doctors start with a physical exam and a neurological exam, which tests how the brain and nerves work. Tests may include: MRI, which uses a magnet and radio waves to make detailed pictures of the brain and spinal cord; lumbar puncture, also called a spinal tap, which collects fluid from the spine to check for tumor cells; and gene testing. An ultrasound may check the kidney for tumors. A biopsy confirms the diagnosis. A biopsy removes a small sample of tumor tissue. The tumor may be removed in the same surgery.
Treatment
The first treatment is surgery, if possible. The goal is to remove as much tumor as safely possible. After surgery, most children get chemotherapy, drugs that kill cancer cells or stop them from growing, and possibly radiation therapy. Drugs used for ATRT include carboplatin, cisplatin, cyclophosphamide, cytarabine, etoposide, methotrexate, and thiotepa. Radiation can affect a young child's growth and brain development, so the dose may be lower. Other options are high-dose chemotherapy with stem cell transplant, and clinical trials. A team of children's cancer experts plans treatment.
When to see a doctor
Take a child to a doctor right away if the child has morning headaches, vomiting, unusual sleepiness, loss of balance, an increase in head size in an infant, or new numbness or paralysis in the face. This tumor grows fast, so symptoms can get worse quickly.
Call 911 or go to the emergency room right away if a child has a seizure, trouble breathing, or cannot be woken up.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. Either can check symptoms and send a child to the right cancer doctors. They can also help the family plan care.
Common questions
How rare is ATRT?
ATRT is very rare. It usually occurs in children aged 3 years and younger, and it can also occur in older children and adults.
Who treats it?
A pediatric oncologist, a doctor who treats children with cancer, oversees treatment. The team may include a pediatric neurosurgeon, a radiation oncologist, a neurologist, and a geneticist or genetic counselor.
What is a biopsy?
A biopsy removes a sample of tumor tissue. A pathologist, a doctor who studies tissue under a microscope, looks for cancer cells.
Does it spread?
ATRTs often spread to other areas of the brain and spinal cord through the cerebrospinal fluid, the fluid around the brain and spinal cord. Staging tests show whether it has spread.
Sources
- National Cancer Institute - Atypical Teratoid/Rhabdoid Tumors (AT/RT): Diagnosis and Treatment
- National Cancer Institute - Childhood Atypical Teratoid/Rhabdoid Tumor Treatment
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
