Anaplastic pleomorphic xanthoastrocytomas (PXAs) account for less than 1% of all brain tumors, and they are often diagnosed during transformation from lower grade tumors. The rate of malignant transformation of grade II tumors to grade III PXAs is between 10% and 25%. Previous studies have found common molecular abnormalities such as BRAF V600E mutations in PXAs. The use of BRAF inhibitors has shown promising activity in patients with BRAF V600E-mutated tumors, including anaplastic PXAs, but tumor progression still occurs. The prognosis of patients with anaplastic PXAs is worse than those with grade II tumors and a better understanding of their molecular characteristics is greatly needed to propel research into improved therapies. Researchers will perform detailed molecular analysis of PXA samples through access to the Pediatric Brain Tumor Atlas. PXAs are a rare and deadly cancer, but results from this comprehensive analysis will further characterize these tumors and could find new avenues for patient care.
What are the goals of this project?
Researchers will perform comprehensive molecular analysis of the rare and deadly PXAs in a pursuit of therapies.
What is the impact of this project?
PXAs are a rare cancer with poor outcomes, but detailed analysis of PXA specimens will provide detailed characterization of this tumor type and could pave the way for improved clinical care and therapies for patients.
Why is the CBTN request important to this project?
Due to the rarity of PXA, specimens of PXA suitable for comprehensive analysis are also rare. The Pediatric Brain Tumor Atlas provides researchers with the rare specimens necessary to complete this research.
The Children's Brain Tumor Network contributed to this project by providing access to the Pediatric Brain Tumor Atlas.
PI: Alberto Broniscer
UPMC Children's Hospital of PittsburghJoined on
The Neuro-Oncology Program at UPMC Children’s Hospital is at the center of finding new and improved treatments for pediatric brain and nervous system cancers. As one of the leading sites in the United States for childhood neurologic cancer research and clinical studies, UPMC Children’s brain tumor p