ArticleActa neuropathologica2022
High-grade glioma with pleomorphic and pseudopapillary features (HPAP): a proposed type of circumscribed glioma in adults harboring frequent TP53 mutations and recurrent monosomy 13.
Article in Acta neuropathologica, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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Who cites it
28 citing papers in PubMed, 53 citations in OpenAlex.
- Gliomas with pleomorphic and pseudopapillary features (GPAP) are circumscribed tumors with targetable mutations, prolonged survival, and frequent tumor predisposition.Brain pathology (Zurich, Switzerland) · 2026Article
- Methylation profiling in CNS tumor diagnostics: a single-centre real-world experience from Central Europe.Virchows Archiv : an international journal of pathology · 2026Article
- CNS tumor type prevalence according to age group: An analysis of 21 000 cases confirmed by methylation profiling, with a focus on adolescents and young adults.Neuro-oncology · 2026Article
- RB1 Loss as a Diagnostic Clue in a High-Grade Glioma With Pleomorphic and Pseudopapillary Features (HPAP): The First Reported Case With Spinal Metastasis and a Review of the Literature.Pathology international · 2026Review
- MGMT promoter methylation across glioma subtypes: biological relevance, treatment response, and survival outcomes.Molecular biology reports · 2026Review
- Clinicoradiological differentiation of TERT promoter-mutant molecular glioblastoma versus histological glioblastoma: a single-center cohort study of 105 IDH-wildtype tumors.Scientific reports · 2026Article
- The therapeutic effect and specific mechanism involved active Chinese medicine component biochaninA in glioma.Frontiers in immunology · 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
- High-grade glioma with pleomorphic and pseudopapillary features: a single-institution series of three cases.Acta neuropathologica communications · 2025Article
- Mutational accumulation drives pathway dysregulation to predict glioma patient survival.Scientific reports · 2025Article
- Role of lysine acetylation-related genes in the diagnosis and prognosis of glioma.Scientific reports · 2025Article
- Expanding clinicopathologic knowledge in high-grade glioma with pleomorphic and pseudopapillary features (HPAP): a report of two cases.Acta neuropathologica communications · 2025Article
- Diffuse astrocytoma, AYA-type, frequently MAPK-altered: report of 45 patients.Acta neuropathologica · 2025Article
- Evaluation of ploidy and the DNA index by flow cytometry in central nervous system tumors: a review.Molecular biology reports · 2024Review
- A High-Grade Glioma, Not Elsewhere Classified in an Older Adult with Discordant Genetic and Epigenetic Analyses.Biomedicines · 2024Article
- An incidental finding of a high-grade glioma with pleomorphic and pseudopapillary features (HPAP) with PBRM1 mutation.Journal of neuropathology and experimental neurology · 2024Article
- When a dermatopathologist encounters the ultra-rare: A case series of superficial soft tissue/cutaneous myxopapillary ependymomas.Journal of cutaneous pathology · 2024Article
- Diagnostic Insights into Pediatric Pleomorphic Xanthoastrocytoma through DNA Methylation Class and Pathological Diagnosis Analysis.Diagnostics (Basel, Switzerland) · 2023Article
- Glioneuronal tumor with ATRX alteration, kinase fusion and anaplastic features (GTAKA): a molecularly distinct brain tumor type with recurrent NTRK gene fusions.Acta neuropathologica · 2023Article
- Signaling pathways in brain tumors and therapeutic interventions.Signal transduction and targeted therapy · 2023Review
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Authors and funding
22 authors at 8 institutions in 1 country.
Funding
Abstract
Tumors of the central nervous system (CNS) often display a wide morphologic spectrum that has, until recently, been the sole basis for tumor classification. The introduction of the integrated histomolecular diagnostic approach in CNS tumors has facilitated a classification system that is increasingly data-driven and with improved alignment to clinical outcome. Here, we report a previously uncharacterized glioma type (n = 31) using unsupervised clustering analysis of DNA methylation array data from approximately 14,000 CNS tumor samples. Histologic examination revealed circumscribed growth and morphologic similarities to pleomorphic xanthoastrocytoma (PXA), astroblastoma, ependymoma, polymorphous neuroepithelial tumor of the young (PLNTY), and IDH-wildtype glioblastoma (GBM). Median age (46.5 years) was significantly older than other circumscribed gliomas and younger than GBM. Dimensionality reduction with uniform manifold approximation and projection (UMAP) and hierarchical clustering confirmed a methylation signature distinct from known tumor types and methylation classes. DNA sequencing revealed recurrent mutations in TP53 (57%), RB1 (26%), NF1 (26%), and NF2 (14%). BRAF V600E mutations were detected in 3/27 sequenced cases (12%). Copy number analysis showed increased whole chromosome aneuploidy with recurrent loss of chromosome 13 (28/31 cases, 90%). CDKN2A/B deletion (2/31, 6%) and MGMT promoter methylation (1/31, 3%) were notably rare events. Most tumors showed features of a high-grade glioma, yet survival data showed significantly better overall survival compared to GBM (p < 0.0001). In summary, we describe a previously uncharacterized glioma of adults identified by a distinct DNA methylation signature and recurrent loss of chromosome 13.
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