ArticleActa neuropathologica2021
Glioblastomas with primitive neuronal component harbor a distinct methylation and copy-number profile with inactivation of TP53, PTEN, and RB1.
Article in Acta neuropathologica, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers.
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Who cites it
36 citing papers in PubMed, 52 citations in OpenAlex.
- Cystic glioblastoma is associated with alterations of KDR and the Rb pathway: a single-center retrospective analysis.Acta neuropathologica communications · 2026Article
- Primitive neuronal component is a frequent finding among IDH-mutant astrocytomas with RB1 alterations.Acta neuropathologica · 2026Article
- ITPKA suppresses glioma progression and predicts patient prognosis.Frontiers in oncology · 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
- Patterns of hypermutation shape tumorigenesis and immunotherapy response in mismatch-repair-deficient glioma.Nature genetics · 2026Article
- High frequency and prognostic significance of TP53 promoter methylation in Pakistani glioblastoma patients.Molecular biology reports · 2025Article
- DNA Methylation-Based Classification of Kidney Neoplasms.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2025Article
- Cut the fat: targeting cholesterol and lipid metabolism in glioblastoma.Cell death & disease · 2025Review
- ATRX loss in adult gliomas lacking H3 alterations or IDH mutations, an exceptional situation for exceptional diagnoses: the experience of Sainte-Anne hospital.Acta neuropathologica communications · 2025Article
- Rare glioblastoma subtype masquerading as a poorly differentiated carcinoma: illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- Clinical characteristics, molecular reclassification trajectories and DNA methylation patterns of long- and short-term survivors of WHO grade II and III glioma.Journal of neurology · 2025Article
- IDH-mutant astrocytomas with primitive neuronal component have a distinct methylation profile and a higher risk of leptomeningeal spread.Acta neuropathologica · 2025Article
- Concurrent RB1 and P53 pathway disruption predisposes to the development of a primitive neuronal component in high-grade gliomas depending on MYC-driven EBF3 transcription.Acta neuropathologica · 2025Article
- Posterior pituitary tumors and other rare entities involving the pituitary gland.Brain pathology (Zurich, Switzerland) · 2025Review
- Rodent models of tumours of the central nervous system.Molecular oncology · 2024Review
- A syngeneic spontaneous zebrafish model ofeLife · 2024Article
- Glioblastoma: An Update in Pathology, Molecular Mechanisms and Biomarkers.International journal of molecular sciences · 2024Review
- Pycallingcards: an integrated environment for visualizing, analyzing, and interpreting Calling Cards data.Bioinformatics (Oxford, England) · 2024Article
- Article
- Molecular diversity in isocitrate dehydrogenase-wild-type glioblastoma.Brain communications · 2024Review
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Authors and funding
40 authors at 20 institutions in 9 countries.
Funding
Abstract
Glioblastoma IDH-wildtype presents with a wide histological spectrum. Some features are so distinctive that they are considered as separate histological variants or patterns for the purpose of classification. However, these usually lack defined (epi-)genetic alterations or profiles correlating with this histology. Here, we describe a molecular subtype with overlap to the unique histological pattern of glioblastoma with primitive neuronal component. Our cohort consists of 63 IDH-wildtype glioblastomas that harbor a characteristic DNA methylation profile. Median age at diagnosis was 59.5 years. Copy-number variations and genetic sequencing revealed frequent alterations in TP53, RB1 and PTEN, with fewer gains of chromosome 7 and homozygous CDKN2A/B deletions than usually described for IDH-wildtype glioblastoma. Gains of chromosome 1 were detected in more than half of the cases. A poorly differentiated phenotype with frequent absence of GFAP expression, high proliferation index and strong staining for p53 and TTF1 often caused misleading histological classification as carcinoma metastasis or primitive neuroectodermal tumor. Clinically, many patients presented with leptomeningeal dissemination and spinal metastasis. Outcome was poor with a median overall survival of only 12 months. Overall, we describe a new molecular subtype of IDH-wildtype glioblastoma with a distinct histological appearance and genetic signature.
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