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Table 1 Proposed morphological variants of PXA

From: Pleomorphic xanthoastrocytoma in the multiverse of epigenomics: is it time to recognize the variants?

Morphological pattern

Histological description

Differential diagnosis

Classic

Variably pleomorphic cells with eosinophilic cytoplasm, areas of spindling and fascicular growth, and at least focal lipidized neoplastic cells with EGBs

Ganglioglioma, Pilocytic astrocytoma, Giant cell GBM

Ganglioglioma-like

Compact and fasciculated tumor with ganglion cell differentiation and EGBs

Ganglioglioma and other low-grade glial neoplasms with ganglion cell differentiation

Glioblastoma-like

Features resembling conventional glioblastoma, including mitotically active tumor with pseudopalisading necrosis and microvascular proliferation

Conventional GBM IDH-wt and HGG IDH/H3-wt

Epithelioid glioblastoma-like

Predominantly epithelioid cells with distinct cell borders and round nuclei with prominent nucleoli arranged in a solid pattern

GBM IDH-wt and HGG IDH/H3-wt with epithelioid features: non-flat copy-number profile, including gene amplifications (CDK4, CDK6, EGFR, MDM2, MDM4, MET)

Astroblastoma-like

Astroblastic rosettes might be found at least focally in tumors with areas of classic PXA and absence of MN1 fusions

Astroblastoma (ABM): presence of MN1 alterations

Spindle cell

Spindle cell pattern in the majority or entirety of the specimen

Meningothelial and mesenchymal, non-meningothelial neoplasms

Embryonal-like

Monomorphic undifferentiated and/or rhabdoid cells reminiscent of true embryonal neoplasms, including INI-1 (SMARCB1) loss

Atypical teratoid/rhabdoid tumor and other Embryonal tumors