ArticleBrain pathology (Zurich, Switzerland)2020
Myxoid glioneuronal tumor, PDGFRA p.K385-mutant: clinical, radiologic, and histopathologic features.
Article in Brain pathology (Zurich, Switzerland), 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers, 1 of them a synthesis that pooled it.
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Who cites it
38 citing papers in PubMed, 1 synthesis or guideline pooled it, 74 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
- 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
- Myxoid glioneuronal tumor of the septum pellucidum with concurrent dualFrontiers in oncology · 2026Article
- Case Report: Molecular diagnostics and clinical courses of two adult spinal pilocytic astrocytoma long-term survivors with GTF2I::BRAF fusion.Frontiers in oncology · 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
- Myxoid glioneuronal tumor with PDGFRA p.K385 mutation in the periventricular white matter: a rare case and literature insights.Pathologica · 2025Article
- Rapid resolution of depression and epilepsy following resection of a septal region tumor: 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
- 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
- High-Grade Progression, Sarcomatous Transformation, and/or Metastasis of Pituitary Neuroendocrine Neoplasms (PitNENs): The UCSF Experience.Endocrine pathology · 2024Article
- Myxoid glioneuronal tumor of the septum pellucidum in pediatric patients: a case report and comprehensive review of the literature.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Review
- Imaging of pediatric glioneuronal and neuronal tumors.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Review
- Insulin therapy in type 2 diabetes: Insights into clinical efficacy, patient-reported outcomes, and adherence challenges.World journal of diabetes · 2024Review
- One case of myxoid glioneuronal tumour in the left frontal lobe.BJR case reports · 2024Article
- Imaging of supratentorial intraventricular masses in children: a pictorial review-part 2.Neuroradiology · 2024Review
- PDGFRA K385 mutants in myxoid glioneuronal tumors promote receptor dimerization and oncogenic signaling.Scientific reports · 2024Article
- Enrichment of oligodendrocyte precursor phenotypes in subsets of low-grade glioneuronal tumours.Brain communications · 2024Article
- Imaging of the septum pellucidum: normal, variants and pathology.The British journal of radiology · 2023Review
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Authors and funding
35 authors at 7 institutions in 1 country.
Funding
Abstract
"Myxoid glioneuronal tumor, PDGFRA p.K385-mutant" is a recently described tumor entity of the central nervous system with a predilection for origin in the septum pellucidum and a defining dinucleotide mutation at codon 385 of the PDGFRA oncogene replacing lysine with either leucine or isoleucine (p.K385L/I). Clinical outcomes and optimal treatment for this new tumor entity have yet to be defined. Here, we report a comprehensive clinical, radiologic, and histopathologic assessment of eight cases. In addition to its stereotypic location in the septum pellucidum, we identify that this tumor can also occur in the corpus callosum and periventricular white matter of the lateral ventricle. Tumors centered in the septum pellucidum uniformly were associated with obstructive hydrocephalus, whereas tumors centered in the corpus callosum and periventricular white matter did not demonstrate hydrocephalus. While multiple patients were found to have ventricular dissemination or local recurrence/progression, all patients in this series remain alive at last clinical follow-up despite only biopsy or subtotal resection without adjuvant therapy in most cases. Our study further supports "myxoid glioneuronal tumor, PDGFRA p.K385-mutant" as a distinct CNS tumor entity and expands the spectrum of clinicopathologic and radiologic features of this neoplasm.
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