Clinical, Radiologic, and Molecular Characteristics of Anaplastic Pleomorphic Xanthoastrocytomas

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.