ArticleActa neuropathologica2020
Isomorphic diffuse glioma is a morphologically and molecularly distinct tumour entity with recurrent gene fusions of MYBL1 or MYB and a benign disease course.
Article in Acta neuropathologica, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 76 papers.
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Who cites it
76 citing papers in PubMed, 129 citations in OpenAlex.
- Article
- From Classical to Emerging Biomarkers of Brain and Central Nervous System Tumors. An Evidence-Based Review with a Focus on Gliomas.Cellular and molecular neurobiology · 2026Review
- CircFAT1(e2) acts as a sponge for miR-30e-5p and inhibits glioma progression by regulating MYBL2-mediated cell proliferation.Discover oncology · 2025Article
- Illuminating radiogenomic signatures in pediatric-type diffuse gliomas: insights into molecular, clinical, and imaging correlations. Part II: low-grade group.La Radiologia medica · 2025Review
- The malignant transformation of an atypical angiocentric glioma, MYB-altered.Acta neuropathologica communications · 2025Article
- Quantifying the non-isomorphism of global urban road networks using GNNs and graph kernels.Scientific reports · 2025Article
- Spatially resolved transcriptomics and graph-based deep learning improve accuracy of routine CNS tumor diagnostics.Nature cancer · 2025Article
- Cortically Based Brain Tumors in Children: A Decision-Tree Approach in the Radiology Reading Room.AJNR. American journal of neuroradiology · 2025Review
- Clinical characteristics and detection of MYB-QKI fusions in patients with angiocentric glioma.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Article
- [Diagnostic challenges of pediatric gliomas; the role of molecular profiling in everyday diagnostics].Magyar onkologia · 2024Review
- Metabolism: an important player in glioma survival and development.Discover oncology · 2024Review
- MYB/MYBL1-altered gliomas frequently harbor truncations and non-productive fusions in the MYB and MYBL1 genes.Acta neuropathologica · 2024Article
- Extra-temporal pediatric low-grade gliomas and epilepsy.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Review
- 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
- Molecular markers for pediatric low-grade glioma.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Review
- Second-look surgery in postoperative pediatric low-grade glioma.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Review
- Pediatric-type diffuse low-grade gliomas.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Review
- Approaches to supratentorial brain tumours in children.Neuroradiology · 2024Review
- Comprehensive analysis of MYB/MYBL1-altered pediatric-type diffuse low-grade glioma.Neuro-oncology · 2024Article
- T2-FLAIR Mismatch: An Imaging Biomarker for Children'sAJNR. American journal of neuroradiology · 2024Article
16 more citing papers are in PubMed but not listed here.
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Authors and funding
52 authors at 20 institutions in 10 countries.
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Abstract
The "isomorphic subtype of diffuse astrocytoma" was identified histologically in 2004 as a supratentorial, highly differentiated glioma with low cellularity, low proliferation and focal diffuse brain infiltration. Patients typically had seizures since childhood and all were operated on as adults. To define the position of these lesions among brain tumours, we histologically, molecularly and clinically analysed 26 histologically prototypical isomorphic diffuse gliomas. Immunohistochemically, they were GFAP-positive, MAP2-, OLIG2- and CD34-negative, nuclear ATRX-expression was retained and proliferation was low. All 24 cases sequenced were IDH-wildtype. In cluster analyses of DNA methylation data, isomorphic diffuse gliomas formed a group clearly distinct from other glial/glio-neuronal brain tumours and normal hemispheric tissue, most closely related to paediatric MYB/MYBL1-altered diffuse astrocytomas and angiocentric gliomas. Half of the isomorphic diffuse gliomas had copy number alterations of MYBL1 or MYB (13/25, 52%). Gene fusions of MYBL1 or MYB with various gene partners were identified in 11/22 (50%) and were associated with an increased RNA-expression of the respective MYB-family gene. Integrating copy number alterations and available RNA sequencing data, 20/26 (77%) of isomorphic diffuse gliomas demonstrated MYBL1 (54%) or MYB (23%) alterations. Clinically, 89% of patients were seizure-free after surgery and all had a good outcome. In summary, we here define a distinct benign tumour class belonging to the family of MYB/MYBL1-altered gliomas. Isomorphic diffuse glioma occurs both in children and adults, has a concise morphology, frequent MYBL1 and MYB alterations and a specific DNA methylation profile. As an exclusively histological diagnosis may be very challenging and as paediatric MYB/MYBL1-altered diffuse astrocytomas may have the same gene fusions, we consider DNA methylation profiling very helpful for their identification.
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