This is a multi-treatment arm study that will be conducted through the Pacific Pediatric Neuro-oncology Consortium (PNOC).The study will assess the safety and efficacy of novel therapies and combinatorial strategies for participants with recurrent or progressive ATRT.
In addition to the below, investigators are to refer to arm-specific inclusion criteria in the appendix.
Participants must have a pathologic diagnosis of central nervous system (CNS) ATRT, with confirmation of SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily b, member 1 (SMARCB1) (INI1) loss by immunohistochemistry (IHC) and/or biallelic loss of function of SMARCB1 by molecular report. Loss of SWI/SNF Related, Matrix Associated, Actin Dependent Regulator of Chromatin, Subfamily A, Member 4 (SMARCA4) as confirmed by IHC or molecular report is also acceptable but requires study chair approval.
Participants must have confirmation of methylation report, co-enrollment on PNOC-030 or sufficient tumor tissue available for methylation-based subgrouping
Participants must have recurrent or progressive ATRT.
Evidence of synchronous tumors or other extra-CNS malignancy
Participants who are receiving any other investigational agents
Participants who are currently receiving other anti-cancer agents
Participants with uncontrolled infection or other uncontrolled systemic illness
Sabine Mueller, MD, PhD
Lead sponsor
University of California, San Francisco
Sponsor institution
Pacific Pediatric Neuro-Oncology Consortium
Collaborator
Rally Foundation
Collaborator