Pleomorphic xanthoastrocytoma: a brief review

Abstract

Pleomorphic xanthoastrocytoma (PXA) is a rare primary CNS tumor. Recent advances in the molecular characterization are helping to define subtypes of tumor. The discovery of BRAF mutations within a substantial percentage of PXA fosters a clearer understanding of the pathophysiology of these tumors with clear prognostic and therapeutic implications. These findings are expected to provide insight into the spectrum of clinical behavior observed in PXA, ranging from cure with surgery to diffuse dissemination throughout the neuraxis. This review details the clinical presentation including radiographic appearance of PXA. Pathology, including molecular pathology is discussed. Therapeutic management including surgical resection, radiotherapy and systemic therapies are reviewed.

Blood-Based Detection of BRAF V600E in Gliomas and Brain Tumor Metastasis

“…BRAF mutations have also been implicated in glioma tumorigenesis [ 21 , 44 , 45 ] prompting investigation of the use of BRAF targeted therapies in glioma [ 23 , 46 , 47 , 48 , 49 ]. In our cohort, P4 and P5 had gliomas positive for the BRAF V600E mutation with P4 having a PXA with MAF of 0.452% at the time of resection.…”

Pleomorphic Xanthoastrocytoma of the Frontal Lobe in a Child: A Rare Entity

“…Microscopically, this lesion presented moderated cellularity, predominantly pleomorphic tumor with no necrosis and rare mitoses, and occasional presence of bizarre multinucleate cells [ 16 , 17 ]. In spite of this appearance, the tumor presents a favorable prognosis, and the overall survival was 209 months [ 6 ].…”

“…In spite of this appearance, the tumor presents a favorable prognosis, and the overall survival was 209 months [ 6 ]. Currently, the molecular definition of BRAF or wild-type mutation has prognostic importance, and BRAF positive patients had a better outcome than wild-type mutation [ 17 ].…”

Imaging of brain tumors in children: the basics—a narrative review

“…Over two-thirds of pleomorphic xanthoastrocytomas harbor mutations in BRAF, which conveys a more favorable natural history for this tumor (31). Grade subclassifications based on the presence of wild or mutated BRAF have been considered for pleomorphic xanthoastrocytoma since this molecular feature may predict the outcome.…”