Pleomorphic xanthoastrocytomas (PXAs) are rare tumors having a heterogenous morphology and biological behavior. Recent genomic advances have discovered BRAFp.V600E mutations, CDKN2A/B deletions and TERTp mutations to be the most frequent alterations in PXAs. These tumors can present a diagnostic challenge as they share overlapping histopathological, genomic as well as methylation profile with various other tumor types. This review provides the spectrum of evolution of PXAs from their genesis to recent molecular insights and attempts to review pathogenesis and relationship to other tumors that they mimic.
- Swati Mahajan
- Iman Dandapath
- Chitra Sarkar