Published 2021 March 18
Relevance of Molecular Groups in Children with Newly Diagnosed Atypical Teratoid Rhabdoid Tumor: Results from Prospective St. Jude Multi-Institutional Trials

An article published online in Clinical Cancer Research offers some insight into differing outcomes associated with the three molecular subtypes of ATRT. The study included 74 newly diagnosed ATRT patients, from infants to adolescents, who were treated in two clinical trials at multiple institutions using protocols established through St. Jude Children’s Research Hospital. Infants with ATRT-TYR had the best overall survival. ATRT-SHH was associated with metastatic disease and poorer outcomes regardless of age.  Among children – regardless of molecular subtype – those whose ATRT had not spread beyond the tumor site benefited from post-surgical chemotherapy and radiation therapy of the brain and spine.

Published 2021 Feb 3
Current recommendations for clinical surveillance and genetic testing in rhabdoid tumor predisposition: a report from the SIOPE Host Genome Working Group

Atypical Teratoid Rhabdoid Tumor can occur in some children who have rare genetic conditions known as rhabdoid tumor predisposition syndrome making young children vulnerable to an increased risk of developing Rhabdoid tumors. Tumor surveillance protocols for these rare families have not been established. The European Society for Pediatric Oncology (SIOPe) Host Genome working group invited pediatric oncologists and geneticists to contribute to an expert meeting that culminated in a consensus statement to guide clinicians and patient families.

Checkpoint inhibitor drugs offer new hope for many types of cancer by unleashing the body’s immune system to attack cancer cells. To date, however, these drugs have not shown much success against pediatric cancers. A new study from researchers at the Dana Farber Institute and Boston Children’s Hospital may challenge that finding when it comes to patients with ATRT.
READ MORE for the full publication summary. 

Study of Nivolumab and Ipilimumab in Children and Young Adults With INI1-Negative Cancers
A clinical trial based on this study’s results is already underway. 
The main purpose of this study is to determine the safety and effectiveness of nivolumab and ipilimumab for patients with relapsed or refractory INI1- negative tumors.  Patients with multiple types of solid tumors and brain tumors will be included as part of this study.  Patients will only be eligible for this study if their tumor has shown a mutation/deletion of a gene that is known to suppress tumor growth called INI1. LEARN MORE about Nivolumab-Ipiliumab | Clinical Trials | Hope4ATRT.