ArticleBrain pathology (Zurich, Switzerland)2019
The genetic landscape of anaplastic pleomorphic xanthoastrocytoma.
Article in Brain pathology (Zurich, Switzerland), 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 77 papers.
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Who cites it
77 citing papers in PubMed, 139 citations in OpenAlex.
- Distinct Clinicogenomic Contexts of BRAF-Altered Gliomas and Glioneuronal Tumors: A Nationwide Real-World Study.Cancer science · 2026Article
- MAPK-driven glioma progression and reprogramming of the tumor-associated immune response.Neuro-oncology · 2026Article
- Ganglioglioma and gangliocytoma: a review for pathologists.Journal of pathology and translational medicine · 2026Article
- Association of CDKN2A/B and MTAP deletions in adult-type diffuse gliomas.Journal of neuropathology and experimental neurology · 2026Article
- Clinical potential of [European journal of nuclear medicine and molecular imaging · 2026Article
- Leptomeningeal Dissemination in TERT Promoter-mutant Anaplastic Pleomorphic Xanthoastrocytoma Responding to BRAF-MEK Inhibition: A Case Report.NMC case report journal · 2026Article
- CNS Tumor with BCOR/BCORL1 Fusion: A Rare Tumor Entity.International journal of molecular sciences · 2025Article
- PXA-like tumors: prognostic implications.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Article
- Anaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination presenting with cranial neuropathy in an adult patient: illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- Review
- Targeted therapy for pediatric glioma: RAF(t)ing in the molecular era.World journal of pediatrics : WJP · 2025Review
- Proton Beam Therapy for a Rare Anaplastic Pleomorphic Xanthoastrocytoma: Case Report and Literature Review.International journal of particle therapy · 2025Article
- Cortically Based Brain Tumors in Children: A Decision-Tree Approach in the Radiology Reading Room.AJNR. American journal of neuroradiology · 2025Review
- [Diagnostic challenges of pediatric gliomas; the role of molecular profiling in everyday diagnostics].Magyar onkologia · 2024Review
- Comparing loss of p16 and MTAP expression in detecting CDKN2A homozygous deletion in pleomorphic xanthoastrocytoma.Journal of neuropathology and experimental neurology · 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
- Mechanisms of telomere maintenance and associated therapeutic vulnerabilities in malignant gliomas.Neuro-oncology · 2024Review
- Ganglioglioma with anaplastic/high-grade transformation: Histopathologic, molecular, and epigenetic characterization of 3 cases.Journal of neuropathology and experimental neurology · 2024Article
- The role of CRAF in cancer progression: from molecular mechanisms to precision therapies.Nature reviews. Cancer · 2024Review
17 more citing papers are in PubMed but not listed here.
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Authors and funding
20 authors at 3 institutions in 1 country.
Funding
Abstract
Pleomorphic xanthoastrocytoma (PXA) is an astrocytic neoplasm that is typically well circumscribed and can have a relatively favorable prognosis. Tumor progression to anaplastic PXA (WHO grade III), however, is associated with a more aggressive biologic behavior and worse prognosis. The factors that drive anaplastic progression are largely unknown. We performed comprehensive genomic profiling on a set of 23 PXAs from 19 patients, including 15 with anaplastic PXA. Four patients had tumor tissue from multiple recurrences, including two with anaplastic progression. We find that PXAs are genetically defined by the combination of CDKN2A biallelic inactivation and RAF alterations that were present in all 19 cases, most commonly as CDKN2A homozygous deletion and BRAF p.V600E mutation but also occasionally BRAF or RAF1 fusions or other rearrangements. The third most commonly altered gene in anaplastic PXA was TERT, with 47% (7/15) harboring TERT alterations, either gene amplification (n = 2) or promoter hotspot mutation (n = 5). In tumor pairs analyzed before and after anaplastic progression, two had increased copy number alterations and one had TERT promoter mutation at recurrence. Less commonly altered genes included TP53, BCOR, BCORL1, ARID1A, ATRX, PTEN, and BCL6. All PXA in this cohort were IDH and histone H3 wildtype, and did not contain alterations in EGFR. Genetic profiling performed on six regions from the same tumor identified intratumoral genomic heterogeneity, likely reflecting clonal evolution during tumor progression. Overall, anaplastic PXA is characterized by the combination of CDKN2A biallelic inactivation and oncogenic RAF kinase signaling as well as a relatively small number of additional genetic alterations, with the most common being TERT amplification or promoter mutation. These data define a distinct molecular profile for PXA and suggest additional genetic alterations, including TERT, may be associated with anaplastic progression.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.