ArticleActa neuropathologica communications2022
Pleomorphic xanthoastrocytoma is a heterogeneous entity with pTERT mutations prognosticating shorter survival.
Article in Acta neuropathologica communications, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers, 1 of them a synthesis that pooled it.
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Who cites it
33 citing papers in PubMed, 1 synthesis or guideline pooled it, 38 citations in OpenAlex.
- DNA Methylation-Based Classification for Central Nervous System Tumours: A Health Technology Assessment.Ontario health technology assessment series · 2025Pooled it
- MAPK-driven glioma progression and reprogramming of the tumor-associated immune response.Neuro-oncology · 2026Article
- Article
- Identification of BRAF V600E Mutation in Cerebrospinal Fluid Aids in Diagnosing Leptomeningeal Carcinomatosis Arising From Pleomorphic Xanthoastrocytoma: A Case Report.Cancer reports (Hoboken, N.J.) · 2026Article
- cIMPACT-NOW update 11: Proposal on adaptation of diagnostic criteria for IDH- and H3-wildtype diffuse high-grade gliomas and for posterior fossa ependymal tumors.Brain pathology (Zurich, Switzerland) · 2026Article
- Glioblastoma in adults: A Society for Neuro-Oncology (SNO) and European Society of Neuro-Oncology (EANO) consensus review on current management and future directions.Neuro-oncology · 2025Review
- Clinical Outcome of Patients with Epithelioid Glioblastoma Harboring BRAFV600E Mutation; A Single Institution Experience.South Asian journal of cancer · 2025Article
- PXA-like tumors: prognostic implications.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Article
- Alternative lengthening of telomere-based immortalization renders H3G34R-mutant diffuse hemispheric glioma hypersensitive to PARP inhibitor combination regimens.Neuro-oncology · 2025Article
- Cortically Based Brain Tumors in Children: A Decision-Tree Approach in the Radiology Reading Room.AJNR. American journal of neuroradiology · 2025Review
- Intraventricular WHO Grade 3 Pleomorphic Xanthoastrocytoma: A Rare Case Report and Review of the Literature.Case reports in pathology · 2025Article
- Prognosis prediction via histological evaluation of cellular heterogeneity in glioblastoma.Scientific reports · 2024Article
- Paediatric low-grade glioma: the role of classical pathology in integrated diagnostic practice.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Review
- Noninvasive prediction of BRAF V600E mutation status of pleomorphic xanthoastrocytomas with MRI morphologic features and diffusion-weighted imaging.BMC cancer · 2024Article
- Ganglioglioma with anaplastic/high-grade transformation: Histopathologic, molecular, and epigenetic characterization of 3 cases.Journal of neuropathology and experimental neurology · 2024Article
- Glioma.Nature reviews. Disease primers · 2024Review
- A rare case of atypical intradural extramedullary glioblastoma diagnosed utilizing next-generation sequencing and methylation profiling: illustrative case.Journal of neurosurgery. Case lessons · 2024Article
- Cell-in-cell phenomena of intracellular neutrophils in a recurrent pleomorphic xanthoastrocytoma.Free neuropathology · 2024Article
- Current status of DNA methylation profiling in neuro-oncology as a diagnostic support tool: A review.Neuro-oncology practice · 2023Review
- Establishment and characterization of patient-derived xenograft of a rare pediatric anaplastic pleomorphic xanthoastrocytoma (PXA) bearing a CDC42SE2-BRAF fusion.Scientific reports · 2023Article
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39 authors at 14 institutions in 3 countries.
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Abstract
Pleomorphic xanthoastrocytoma (PXA) in its classic manifestation exhibits distinct morphological features and is assigned to CNS WHO grade 2 or grade 3. Distinction from glioblastoma variants and lower grade glial and glioneuronal tumors is a common diagnostic challenge. We compared a morphologically defined set of PXA (histPXA) with an independent set, defined by DNA methylation analysis (mcPXA). HistPXA encompassed 144 tumors all subjected to DNA methylation array analysis. Sixty-two histPXA matched to the methylation class mcPXA. These were combined with the cases that showed the mcPXA signature but had received a histopathological diagnosis other than PXA. This cohort constituted a set of 220 mcPXA. Molecular and clinical parameters were analyzed in these groups. Morphological parameters were analyzed in a subset of tumors with FFPE tissue available. HistPXA revealed considerable heterogeneity in regard to methylation classes, with methylation classes glioblastoma and ganglioglioma being the most frequent mismatches. Similarly, the mcPXA cohort contained tumors of diverse histological diagnoses, with glioblastoma constituting the most frequent mismatch. Subsequent analyses demonstrated the presence of canonical pTERT mutations to be associated with unfavorable prognosis among mcPXA. Based on these data, we consider the tumor type PXA to be histologically more varied than previously assumed. Histological approach to diagnosis will predominantly identify cases with the established archetypical morphology. DNA methylation analysis includes additional tumors in the tumor class PXA that share similar DNA methylation profile but lack the typical morphology of a PXA. DNA methylation analysis also assist in separating other tumor types with morphologic overlap to PXA. Our data suggest the presence of canonical pTERT mutations as a robust indicator for poor prognosis in methylation class PXA.
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