Survival rates

Survival rate means the percentage of people who are alive a certain amount of time after diagnosis, usually five years.

Survival varies by age and tumor stage. The five-year relative survival rate for ATRT is about 48% for children up to age 14, 42% for teenagers and young adults ages 15 to 39, and 25% for adults age 40 and older.

Average survival times reported in studies range from 12 to 24 months, though many children live longer with aggressive treatment and participation in ongoing research trials.

Returning cancer, also called recurrence, is common, especially within the first 2 to 3 years after diagnosis. The chance of the tumor coming back depends on age, tumor spread and how completely the tumor could be removed.

Children who respond well to their first treatments and finish therapy have the best chance of long-term survival. Regular follow-up imaging helps find any regrowth of tumors early.

Prognosis

Prognosis means the expected outcome or course of a disease. It helps describe how likely it is that treatment will control or remove the tumor and what factors may affect long-term health.

ATRT is an aggressive form of brain cancer, but outcomes have improved as treatments advance. Prognosis depends on many factors, including age, tumor size, tumor spread and how much of the tumor can be safely taken out.

Children age 3 or older who have surgery followed by intensive chemotherapy and radiation tend to have better outcomes. Babies and toddlers often face a harder course because treatment must be adjusted to protect their developing brains. Radiation is generally not used on babies and children younger than age 3.

Prognosis also is influenced by whether the cancer has spread through the brain or spinal fluid. Tumors that are found only in one place have a higher chance of long-term control than do tumors that have already spread.

Genetic factors also may affect outcomes. Tumors with SMARCB1 changes can behave differently from those with SMARCA4 changes. Ongoing research continues to study these differences.

Survival is highest for children age 3 or older when the tumor can be fully removed and treated with intensive chemotherapy and radiation.

18/12/2025
  1. Atypical teratoid rhabdoid tumor (ATRT). American Brain Tumor Association. https://www.abta.org/tumor_types/atypical-teratoid-rhabdoid-tumor-atrt/. Accessed Nov. 12, 2025.
  2. Chheda MG, et al. Uncommon brain tumors. https://www.uptodate.com/contents/search. Accessed Nov. 12, 2025.
  3. Baliga S, et al. Brain tumors: Medulloblastoma, ATRT, ependymoma. Pediatric Blood and Cancer. 2021; doi:10.1002/pbc.28395.
  4. Childhood central nervous system atypical teratoid/rhabdoid tumor treatment (PDQ) –Health professional version. National Cancer Institute.https://www.cancer.gov/types/brain/hp/child-cns-atrt-treatment-pdq. Accessed Nov. 14, 2025.
  5. Atypical teratoid/rhabdoid tumor (AT/RT): Diagnosis and treatment. National Cancer Institute. https://www.cancer.gov/rare-brain-spine-tumor/tumors/atrt. Accessed Nov. 14, 2025.
  6. Timmermann B, et al. ESTRO-SIOPE guideline: Clinical management of radiotherapy in atypical teratoid/rhabdoid tumors (AT/RTs). Radiotherapy Oncology: Journal of the European Society for Therapeutic Radiology and Oncology. 2024; doi:10.1016/j.radonc.2024.110227.
  7. Andres S, et al. Rhabdoid tumor predisposition syndrome: A historical review of treatments and outcomes for associated pediatric malignancies. Pediatric Blood & Cancer. 2024; doi:10.1002/pbc.30979.
  8. Baliga S, et al. Brain tumors: Medulloblastoma, ATRT, ependymoma. Pediatric Blood and Cancer. 2021; doi:10.1002/pbc.28395.
  9. Chi SN, et al. Tazemetostat for tumors harboring SMARCB1/SMARCA4 or EZH2 alterations: Results from NCI-COG pediatric MATCH APEC1621C. Journal of the National Cancer Institute. 2023; doi:10.1093/jnci/djad085.
  10. Ronsley R, et al. Pediatric central nervous system embryonal tumors: Presentation, diagnosis, therapeutic strategies, and survivorship — A review. Pediatric Neurology. 2024;doi:10.1016/j.pediatrneurol.2024.09.031.
  11. Abdelbaki MS, et al., eds. Current advances in the management of atypical teratoid rhabdoid tumors (ATRT). In: Advances in Cancer Research. Elsevier; 2025. https://www.sciencedirect.com. Accessed Nov. 12, 2025.
  12. Ashwal S, et al., eds. Atypical teratoid/rhabdoid tumors. In: Swaiman's Pediatric Neurology. 7th ed. Elsevier; 2026. https://www.clinicalkey.com. Accessed Nov. 12, 2025.