Atypical Teratoid/Rhabdoid Tumor



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Summary of

Atypical Teratoid/Rhabdoid Tumor

Central nervous system (CNS) atypical teratoid/rhabdoid tumor (AT/RT) is a very rare, fast-growing tumor of the brain and spinal cord. It usually occurs in children aged three years and younger, although it can occur in older children and adults. About half of these tumors form in the cerebellum or brain stem. The cerebellum is the part of the brain that controls movement, balance, and posture. The brainstem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating. AT/RT may also be found in other parts of the central nervous system (brain and spinal cord). The WHO 2016 classification indicates Embryonal tumor.

Available CBTN Biospecimens

participants with flash-frozen tissue available97

participants with flash-frozen tissue available

participants with match blood62

participants with match blood

participants with match parental specimens5

participants with match parental specimens

participants with cerebral spinal fluid8

participants with cerebral spinal fluid

Available CBTN Pre-clinical Models

genomically characterized cell lines with data available11

genomically characterized cell lines with data available

genomically characterized pdx with data available0

genomically characterized pdx with data available

How do I get access to the specimens?

To request any of the biospecimens, or pre-clinical model please fill out this form.
Request take approx. 3 months and will require review by the scientific committee and MTA.
Need help? Contact us at

Explore the data in these informatics portals

How do I get access to the data?

To access the data please follow these instructions. Need help? Contact us at
You can access processed data today here by simply logging in to PedcBioPortal
You can request raw data by completing this form. The review normally takes 1 week. Once approved you can access the raw data by creating cohorts of interest on the Kids First Data Resource Portal and performing analysis on the cloud in Cavatica


Scientific Projects

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Impeding LIN28 Function in ATRT

A lack of atypical teratoid rhabdoid tumor samples representing all subtypes has stalled the understanding of this complex disease. Identifying subtypes based on molecular characteristics may provide therapeutic strategies for suppressing growth, proliferation, and development of ATRT. Using cell lines provided by the Children’s Brain Tumor Network, researchers will investigate subtype-dependent responses to known treatments.


Giselle Saulnier Sholler

Giselle Saulnier Sholler

Levine Children's Cancer & Blood Disorders
Charlotte, NC
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Oncolytic Virus to Potentiate Immune-checkpoint Blockade in Immunologically Cold Pediatric Brain Tumors

The use of an oncolytic virus refers to the use of a virus to stimulate immune response in a tumor. Researchers will utilize carefully catalogued samples provided by the Children’s Brain Tumor Network to further explore the use of oncolytic viruses in the treatment of pediatric brain cancers.



Aaron Diaz

UCSF Benioff Children's Hospital
San Francisco and Oakland, CA, USA
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Using Whole Genome Bisulfite Sequencing to Identify Novel Therapeutic Targets in DIPG and ATRT

Diffuse intrinsic pontine gliomas (DIPG) and atypical teratoid rhabdoid tumors (ATRT) develop some tumor forms that are particularly resistant to therapies. To better understand these differences, researchers will analyze DIPG and ATRT samples provided by the Children’s Brain Tumor Network.



Eric Raabe

Johns Hopkins Medicine
Baltimore, MD, USA