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Review
. 2019 Nov 1;8(3):CNS39.
doi: 10.2217/cns-2019-0009. Epub 2019 Sep 19.

Pleomorphic xanthoastrocytoma: a brief review

Nawal Shaikh  1 , Nupur Brahmbhatt  2 , Tim J Kruser  3   4 , Kwok L Kam  5 , Christina L Appin  3   5 , Nitin Wadhwani  6 , James Chandler  3   7 , Priya Kumthekar  2   3 , Rimas V Lukas  2   3
Affiliations
Review

Pleomorphic xanthoastrocytoma: a brief review

Nawal Shaikh et al. CNS Oncol. .

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.

Keywords: BRAF; PXA; pathology; pleomorphic xanthoastrocytoma; radiation; surgery.

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Conflict of interest statement

Financial & competing interests disclosure

PK and RVL are supported by P50CA221747 SPORE for Translational Approaches to Brain Tumors. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Figures

Figure 1.
Figure 1.. Histopathology of pleomorphic xanthoastrocytoma.
(A) The smear preparation shows highly pleomorphic tumor cells with occasional bizarre-looking nuclei (H&E, ×400). (B) There is an admixture of spindelled neoplastic and neoplastic cells with bizarre nuclei or multinucleation (H&E, ×200). (C) The neoplastic cells show multivacuolated cytoplasm (H&E, ×400). (D) An anaplastic pleomorphic xanthogranuloma showing paradoxically monomorphic tumor cells with increased cellularity. Scattered mitotic figures (arrows) are noted (H&E, ×200). (E) Reticulin stain shows a rich reticulin network surrounding nests of tumor cells. Occasional pericellular reticulin deposition is also noted (reticulin stain, ×200). (F) Synaptophysin immunohistochemical stain highlights pleomorphic tumor cells (synaptophysin, ×400). H&E: Hematoxylin and eosin stain.
Figure 2.
Figure 2.. Coronal T1 postcontrast magnetic resonance imaging revealing a left frontal enhancing lesion with cystic components.
A distinct delineation between tumor and surrounding brain parenchyma is noted.
See this image and copyright information in PMC

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