ArticleNeuro-oncology2019
Septal dysembryoplastic neuroepithelial tumor: a comprehensive clinical, imaging, histopathologic, and molecular analysis.
Article in Neuro-oncology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
43 citing papers in PubMed, 1 synthesis or guideline pooled it, 87 citations in OpenAlex.
- cIMPACT-NOW update 6: new entity and diagnostic principle recommendations of the cIMPACT-Utrecht meeting on future CNS tumor classification and grading.Brain pathology (Zurich, Switzerland) · 2020Guideline
- Imaging diagnosis of cystic intraparenchymal brain neoplasms.Japanese journal of radiology · 2026Review
- Intraoperative diagnosis of myxoid glioneuronal tumor guiding safe limited resection: a case report.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026Article
- Pediatric low-grade epilepsy-associated tumors (LEATS): neuroimaging review and genetics update.Pediatric radiology · 2026Review
- Pediatric H3 G34-mutant diffuse hemispheric glioma: clinical, imaging and molecular prognostic factors, MGMT expression, and temozolomide response.Acta neuropathologica · 2026Article
- A Case of Myxoid Glioneuronal Tumor in the Lateral Ventricle.NMC case report journal · 2026Article
- Activated signaling pathways and metabolic processes in disseminating myxoid glioneuronal tumors.Journal of neuropathology and experimental neurology · 2025Article
- FOXR2 activation is not exclusive of CNS neuroblastoma.Neuro-oncology · 2025Article
- Diffuse leptomeningeal glioneuronal tumor (DLGNT): a comprehensive clinical and molecular analysis.Acta neuropathologica · 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
- Myxoid Glioneuronal Tumours with PDGFRA p.K385 Mutations Causing Hydrocephalus and Successfully Treated with Neuroendoscopic Surgery: A Case Report and Literature Review.NMC case report journal · 2025Article
- Novel insights toward diagnosis and treatment of glioneuronal and neuronal tumors in young adults.CNS oncology · 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
- Topographical anatomy of the septum verum and its white matter connections.Scientific reports · 2024Article
- Comprehensive analysis of MYB/MYBL1-altered pediatric-type diffuse low-grade glioma.Neuro-oncology · 2024Article
- Solid pilocytic astrocytoma in cavum septum pellucidum: a description of two cases and an analysis of misdiagnosis.Quantitative imaging in medicine and surgery · 2024Article
- PDGFRA K385 mutants in myxoid glioneuronal tumors promote receptor dimerization and oncogenic signaling.Scientific reports · 2024Article
- High-grade glioma in infants and young children is histologically, molecularly, and clinically diverse: Results from the SJYC07 trial and institutional experience.Neuro-oncology · 2024Article
- Enrichment of oligodendrocyte precursor phenotypes in subsets of low-grade glioneuronal tumours.Brain communications · 2024Article
- Clinicopathological features of dysembryoplastic neuroepithelial tumor: a case series.Journal of medical case reports · 2023Review
Corrections and comments
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Authors and funding
25 authors at 13 institutions in 2 countries.
Funding
Abstract
backgroundDysembryoplastic neuroepithelial tumors (DNETs) are uncommon neural tumors presenting most often in children and young adults and associated with intractable seizures. Rare midline neoplasms with similar histological features to those found in DNETs have been described near the septum pellucidum and termed "DNET-like neoplasms of the septum pellucidum." Due to their rarity, these tumors have been described in just a few reports and their genetic alterations sought only in small series.
methodsWe collected 20 of these tumors for a comprehensive study of their clinical, radiological, and pathological features. RNA sequencing or targeted DNA sequencing was undertaken on 18 tumors, and genome-wide DNA methylation profiling was possible with 11 tumors. Published cases (n = 22) were also reviewed for comparative purposes.
resultsThe commonest presenting symptoms and signs were related to raised intracranial pressure; 40% of cases required cerebrospinal fluid diversion. Epilepsy was seen in approximately one third of cases. All patients had an indolent disease course, despite metastasis within the neuraxis in a few cases. Radiologically, the septum verum/septal nuclei were involved in all cases and are the proposed site of origin for septal DNET (sDNET). Septal DNET showed a high frequency (~80%) of mutations of platelet derived growth factor receptor A (PDGFRA), and alterations in fibroblast growth factor receptor 1 (FGFR1) and neurofibromatosis type 1 (NF1) were also identified. In a genomic DNA methylation analysis alongside other neural tumors, sDNETs formed a separate molecular group.
conclusionsGenetic alterations that are different from those of cerebral DNETs and a distinct methylome profile support the proposal that sDNET is a distinct disease entity.
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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.