ArticleBrain pathology (Zurich, Switzerland)2018
Multinodular and vacuolating neuronal tumors in epilepsy: dysplasia or neoplasia?
Article in Brain pathology (Zurich, Switzerland), 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers, 2 of them syntheses that pooled it.
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Who cites it
27 citing papers in PubMed, 2 syntheses or guidelines pooled it, 83 citations in OpenAlex.
- Multinodular and vacuolating neuronal tumor in the thalamus: case report and systematic review of literature.Acta neurochirurgica · 2024Pooled it
- 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
- Beyond Oligodendroglioma: An Integrated Diagnostic Approach to CNS Tumors with Oligodendroglioma-like Morphology, with a Focus on Morphological Pitfalls, Immunoprofiles, and Molecular Signatures.International journal of molecular sciences · 2026Review
- A pathologically diagnosed multinodular and vacuolating neuronal tumor without typical multiple nodules: a case report.Frontiers in radiology · 2026Article
- The Benign Impostor: Recognizing a Multinodular and Vacuolating Neuronal Tumor to Prevent Unnecessary Intervention.Cureus · 2025Article
- The Mickey Mouse's hand sign in brain MRI points out multinodular and vacuolating neuronal tumors in mesial temporal lobe structures.Journal of neuro-oncology · 2025Article
- Multinodular and Vacuolating Neuronal Tumors: Imaging Features, Diagnosis, and Management Challenges.Diagnostics (Basel, Switzerland) · 2025Review
- Novel insights toward diagnosis and treatment of glioneuronal and neuronal tumors in young adults.CNS oncology · 2024Review
- Multinodular and vacuolating posterior fossa lesions of unknown significance: a long-term observational study and literature review.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Observational
- Phenotypic and epigenetic heterogeneity in FGFR2-fused glial and glioneuronal tumours.Neuropathology and applied neurobiology · 2024Article
- Imaging of pediatric glioneuronal and neuronal tumors.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Review
- Changes to pediatric brain tumors in 2021 World Health Organization classification of tumors of the central nervous system.Pediatric radiology · 2023Review
- Rare Neuronal, Glial and Glioneuronal Tumours in Adults.Cancers · 2023Review
- Newly recognised Tumour Types in Glioneuronal tumours according to the 5th edition of the CNS WHO Classification.Pathologica · 2022Review
- Emerging glioneuronal and neuronal tumors: case-based review.Brain tumor pathology · 2022Review
- Low-grade epilepsy-associated neuroepithelial tumors: Tumor spectrum and diagnosis based on genetic alterations.Frontiers in neuroscience · 2022Review
- A multinodular and vacuolating neuronal tumor of the cerebrum (MVNT) with glioma-like appearance.Acta neurologica Belgica · 2021Article
- Developmental malformations in Huntington disease: neuropathologic evidence of focal neuronal migration defects in a subset of adult brains.Acta neuropathologica · 2021Article
- Multinodular and vacuolating neuronal tumor associated with focal cortical dysplasia in a child with refractory epilepsy: a case report and brief review of literature.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2020Review
- Low-grade developmental and epilepsy associated brain tumors: a critical update 2020.Acta neuropathologica communications · 2020Review
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Authors and funding
18 authors at 6 institutions in 4 countries.
Funding
Abstract
Multinodular and vacuolating neuronal tumor (MVNT) is a new pattern of neuronal tumour included in the recently revised WHO 2016 classification of tumors of the CNS. There are 15 reports in the literature to date. They are typically associated with late onset epilepsy and a neoplastic vs. malformative biology has been questioned. We present a series of ten cases and compare their pathological and genetic features to better characterized epilepsy-associated malformations including focal cortical dysplasia type II (FCDII) and low-grade epilepsy-associated tumors (LEAT). Clinical and neuroradiology data were reviewed and a broad immunohistochemistry panel was applied to explore neuronal and glial differentiation, interneuronal populations, mTOR pathway activation and neurodegenerative changes. Next generation sequencing was performed for targeted multi-gene analysis to identify mutations common to epilepsy lesions including FCDII and LEAT. All of the surgical cases in this series presented with seizures, and were located in the temporal lobe. There was a lack of any progressive changes on serial pre-operative MRI and a mean age at surgery of 45 years. The vacuolated cells of the lesion expressed mature neuronal markers (neurofilament/SMI32, MAP2, synaptophysin). Prominent labelling of the lesional cells for developmentally regulated proteins (OTX1, TBR1, SOX2, MAP1b, CD34, GFAPδ) and oligodendroglial lineage markers (OLIG2, SMI94) was observed. No mutations were detected in the mTOR pathway genes, BRAF, FGFR1 or MYB. Clinical, pathological and genetic data could indicate that MVNT aligns more with a malformative lesion than a true neoplasm with origin from a progenitor neuro-glial cell type showing aberrant maturation.
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Registered trials
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.