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Tumor neuroepitelial disembrioplásico
ORPHA:251946CID-10 · D33.0CID-11 · 2A00.21DOENÇA RARA

O glioma angiocêntrico (GA) refere-se a um tumor neuroepitelial raro em que as células malignas superficiais do cérebro envolvem os vasos cerebrais; é mais comummente encontrado em crianças e adultos jovens. Inicialmente identificado em 2005 pelo Dr. Ming-Tseh Wang e a sua equipa da Universidade do Texas em Austin, o GA foi classificado como um tumor de Grau I na Classificação de Tumores do Sistema Nervoso Central da OMS de 2007 devido ao seu comportamento clínico benigno, baixo índice de proliferação e propriedades curativas. O GA afeta principalmente crianças e adultos jovens com uma idade média de diagnóstico inicial de 16 anos. Mais de 85% dos pacientes com GA sofrem de crises convulsivas intratáveis desde a infância, especialmente epilepsia parcial.

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Introdução

O que você precisa saber de cara

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Tumor neuroepitelial disembrioplásico é uma malformação congênita rara do cérebro, geralmente benigna, que se manifesta como epilepsia de difícil controle, especialmente em crianças e adolescentes. Apresenta-se como lesões multicísticas com características histológicas únicas.

Pesquisas ativas
1 ensaio
4 total registrados no ClinicalTrials.gov
Publicações científicas
424 artigos
Último publicado: 2026 Apr 7
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SUS: Sem cobertura SUSScore: 0%
CID-10: D33.0
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Total histórico424PubMed
Últimos 10 anos180publicações
Pico201622 papers
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Publicações por ano (últimos 10 anos)

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Publicações mais relevantes

Timeline de publicações
183 papers (10 anos)
#1

Multifocal rosette-forming glioneuronal tumor-like low-grade glioneuronal tumor with dysembryoplastic neuroepithelial tumor features associated with drug-resistant epilepsy: a case report and literature review.

Brain tumor pathology2026 Jan 06

Rosette-forming glioneuronal tumors (RGNTs) are rare, World Health Organization grade 1 tumors that typically arise around the fourth ventricle. However, cerebral hemisphere RGNTs have recently been reported, with some exhibiting clinical features resembling low-grade epilepsy-associated tumor (LEAT). We report a case of multifocal RGNT in a patient with drug-refractory epilepsy. A 14-year-old woman was incidentally found to have multifocal brain tumor involving the left temporal lobe and bilateral thalamus, she developed drug-resistant epilepsy ten years later and underwent surgery. Partial tumor resection and anterior temporal lobectomy were performed. Histopathology revealed a glioneuronal tumor with oligodendroglia-like cells, neurocytic rosette, and perivascular pseudorosette, exhibiting an infiltrative growth pattern extending into the white matter. Genetic analysis revealed Fibroblast Growth Factor Receptor 1 mutation. The methylation profile analysis matched the low-grade glioneuronal tumor class but did not yield to any subclass category. Finally, the tumor was diagnosed as RGNT-like low-grade glioneuronal tumor with dysembryoplastic neuroepithelial tumor (DNT) features. Cases presenting with a LEAT-like clinical course and exhibiting histopathological features of RGNT are often difficult to definitively distinguish from DNT based on histological and genetic findings. Epilepsy-associated RGNT may harbor genetic profiles distinct from those of prototypical RGNTs, highlighting the need for further investigation.

#2

Dysembryoplastic neuroepithelial tumor: Malignant transformation following radiation.

Journal of neuropathology and experimental neurology2026 Feb 14
#3

Endoscopic Transorbital Approach for Select Recurrent and Newly Diagnosed Anteromedial Temporal Lobe Gliomas.

Journal of neurological surgery. Part B, Skull base2026 Apr

The endoscopic lateral transorbital approach (eLTOA) has emerged as a minimal access technique for accessing the middle fossa and periorbital region. This study explores the applicability of eLTOA in the management of recurrent and newly diagnosed medial temporal lobe gliomas. Forty patients at our institution underwent eLTOA between 2016 and 2024. There were five surgeries for glioma. Patients were assessed for demographic and clinical data, radiographic characteristics, and histopathological and molecular findings. Four recurrent gliomas required biopsy for diagnosis and molecular genotyping, and one newly diagnosed tumor aimed for gross total resection (GTR). Histologic diagnoses remained unchanged in three recurrent cases (two glioblastoma multiforme and one xanthoastrocytoma). One case was upgraded from oligoastrocytoma Grade II to glioblastoma Grade IV. GTR was achieved in the newly diagnosed tumor, with histology confirming dysembryoplastic neuroepithelial tumor. Average length of stay was 3 days. There was one case of transient diplopia. eLTOA offers a minimally invasive, direct approach to medial temporal lobe gliomas that minimizes the risks of infection and temporalis muscle atrophy associated with reoperation through a previously irradiated incision. It is most useful for restaging recurrent tumors, although GTR can be obtained in well-selected newly diagnosed tumors.

#4

A Case of Myxoid Glioneuronal Tumor in the Lateral Ventricle.

NMC case report journal2026

Myxoid glioneuronal tumor is a newly included entity in the 2021 World Health Organization classification of central nervous system tumors, based on both pathological and molecular evidence, characterized by platelet-derived growth factor receptor alpha mutations. A 23-year-old female presented with intermittent visual field abnormalities and dizziness. Magnetic resonance imaging revealed a 2-cm mass occupying both foramens of Monro, leading to non-communicating hydrocephalus. Endoscopic surgery via the transcortical approach achieved gross total resection of the tumor. Postoperatively, she developed cognitive dysfunction involving recent memory disturbance and progressive ventricular dilation, necessitating a ventriculoperitoneal shunt, which led to significant cognitive recovery. Histopathological analysis confirmed myxoid glioneuronal tumor with a platelet-derived growth factor receptor alpha p.K385L mutation. A review of 23 cases, including ours, indicates that surgical resection is the preferred treatment and is generally associated with a favorable prognosis. However, recurrence and meningeal dissemination have been reported in some cases, emphasizing the need for long-term follow-up. Myxoid glioneuronal tumor is frequently located close to critical structures like the fornix, so careful surgical planning is essential to balance maximal resection with functional preservation. Endoscopic techniques offer advantages for deep-seated lesions by minimizing cortical damage while allowing adequate tumor resection. Further studies are needed to establish the optimal treatment strategies and clarify the long-term prognosis of myxoid glioneuronal tumor.

#5

PTPN11-related Noonan syndrome predisposes to multifocal low-grade CNS tumors harboring FGFR1 variants.

Journal of neuro-oncology2026 Mar 05

To characterize the clinical, radiological, and molecular characteristics of CNS tumors associated with Noonan syndrome (NS) and other non-Neurofibromatosis type 1 RASopathies. Twenty-four patients with concern for NS underwent clinical and central radiological review in this multi-institutional study. Whole-exome sequencing, RNA sequencing, and methylation analyses of peripheral blood and/or tumor specimens were performed. Nineteen (79%) of 24 participants had NS, 17/19 (89%) of which had a germline PTPN11 variant; Nineteen of 24 participants (79%) were male. Seventeen of 19 (89%) patients with NS developed CNS tumors, including low-grade glioma, (LGG; pilocytic/pilomyxoid astrocytoma; n = 9) and dysembryoplastic neuroepithelial tumor (DNET; n = 6). Five patients incidentally diagnosed did not undergo histological confirmation. Radiological review showed multifocal parenchymal tumors in 9 patients with NS, including histologically confirmed neoplasm (n = 2), radiologic progression (n = 6), or typical tumoral imaging (n = 1). Fourteen of 15 (93%) tumors collected from 13 patients with NS and germline PTPN11 variants harbored somatic FGFR1 abnormalities. RNA sequencing of 12 tumors detected FGFR1 internal tandem duplication in one patient. Comparison with published data showed a statistically significant association between brain tumor occurrence and PTPN11-related NS, driven by two genotypes: NM_002834.5(PTPN11):c.182 A > G (p.Asp61Gly) and c.417G > T (p.Glu139Asp). Ten patients with CNS tumors, including 7/17 (41%) with PTPN11 variants, required chemotherapy. After median follow-up of 7.5 years, one patient died of CNS tumor. PTPN11-related NS predisposes to multifocal low-grade glial and glioneuronal tumors confirmed by radiological, histological, and molecular characteristics. Targeting FGFR1-related pathways may provide new treatment approaches for patients with NS and low-grade CNS tumors. The online version contains supplementary material available at 10.1007/s11060-026-05478-7. Multifocal low-grade glial and glioneuronal tumors are common in patients with Noonan syndrome, especially in males. Concurrent PTPN11 and FGFR1 variants exist in nearly all patients with Noonan syndrome and low-grade CNS tumors. Targeting FGFR1 may provide novel treatment options for patients with Noonan syndrome and low-grade CNS tumors. The online version contains supplementary material available at 10.1007/s11060-026-05478-7. Noonan syndrome (NS) has been recognized as a genetic predisposition to CNS tumors. However, no long-term clinical, radiological, or molecular patient data have previously been reported in a large patient cohort. Herein, we show that individuals with NS and germline PTPN11 variants, particularly those with NM_002834.5(PTPN11):c.182 A > G (p.Asp61Gly) and NM_002834.5(PTPN11):c.417G > C (p.Glu139Asp), are predisposed to develop low-grade tumors, including low-grade gliomas (LGGs) and dysembryoplastic neuroepithelial tumors (DNETs), which may have multifocal distant brain involvement in up to 50% of patients. An overwhelming male predominance was confirmed, for which the biological underpinnings remain unknown. Somatic FGFR1 variants were detected in 93% of tumors in individuals with PTPN11-related NS. Therefore, concurrent variants in both genes seem to be required to drive tumorigenesis. Most tumors exhibited indolent behavior, even in the presence of residual disease, and the affected patients experienced long-term survival. However, aggressive monitoring and treatment may be necessary in a subset of patients. The online version contains supplementary material available at 10.1007/s11060-026-05478-7.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC185 artigos no totalmostrando 178

2026

Endoscopic Transorbital Approach for Select Recurrent and Newly Diagnosed Anteromedial Temporal Lobe Gliomas.

Journal of neurological surgery. Part B, Skull base
2026

A Case of Myxoid Glioneuronal Tumor in the Lateral Ventricle.

NMC case report journal
2026

PTPN11-related Noonan syndrome predisposes to multifocal low-grade CNS tumors harboring FGFR1 variants.

Journal of neuro-oncology
2026

Dysembryoplastic neuroepithelial tumor: Malignant transformation following radiation.

Journal of neuropathology and experimental neurology
2026

A Case of Dysembryoplastic Neuroepithelial Tumor in an HIV-Positive Adult: Diagnostic Lessons for Clinicians.

Cureus
2026

Magnetic resonance imaging findings of dysembryoplastic neuroepithelial tumors and low-grade astrocytomas.

Journal of the Chinese Medical Association : JCMA
2026

Pediatric low-grade epilepsy-associated tumors (LEATS): neuroimaging review and genetics update.

Pediatric radiology
2026

Multifocal rosette-forming glioneuronal tumor-like low-grade glioneuronal tumor with dysembryoplastic neuroepithelial tumor features associated with drug-resistant epilepsy: a case report and literature review.

Brain tumor pathology
2025

Case Report: FGFR1 mutation and massive chromosome loss drive malignant transformation of low-grade gliomas.

Frontiers in oncology
2025

MRI-based radiomics model for differentiating focal cortical dysplasia from dysembryoplastic neuroepithelial tumor in epileptic children.

Frontiers in neurology
2025

Glioneuronal and Neuronal Tumors in Adults: WHO 2021 Imaging and Molecular Updates.

AJNR. American journal of neuroradiology
2025

Ruptured intratumoral arteriovenous malformation in a patient with dysembryoplastic neuroepithelial tumor: A case report.

Surgical neurology international
2025

Co-Occurrence of Dysembryoplastic Neuroepithelial Tumor and Hodgkin Lymphoma in a Patient with Noonan Syndrome and Mutation in the PTPN11 Gene.

Hormone research in paediatrics
2025

Differentiation of multinodular and vacuolating neuronal tumor and dysembryoplastic neuroepithelial tumor based on MRI.

Turkish journal of medical sciences
2025

Ultrahigh-field imaging (7 Tesla) in DNET: Unmasking microstructural imaging characteristics - A case report.

Epilepsy & behavior reports
2025

Atypical Lesions in Virchow-Robin Spaces: A Case Report.

Cureus
2025

Are PDFGRA Dinucleotide Alterations Definitional for Myxoid Glioneuronal Tumor? Report of PDFRA p. K385L Mutation in a Neonatal High-Grade Glioma.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2024

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
2025

A glioneuronal tumor with neurocytic rosettes harboring FGFR1 internal tandem duplication - A report of a unique case.

Neuropathology : official journal of the Japanese Society of Neuropathology
2024

Disseminated ependymal dysembryoplastic neuroepithelial tumor: a case report and literature review.

Zhurnal voprosy neirokhirurgii imeni N. N. Burdenko
2024

Dysembryoplastic Neuroepithelial Tumor: A Case Report of A Benign Intracranial Lesion Masquerading as Seizure Disorder.

Cureus
2024

Imaging of pediatric glioneuronal and neuronal tumors.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2024

[Differential diagnosis of epileptogenic substrates of unknown etiology using a modification of the routine MRI protocol].

Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova
2024

Long-term seizure outcome after epilepsy surgery of neuroglial tumors.

Frontiers in neurology
2024

Low-grade glioma of the temporal lobe and tumor-related epilepsy in children.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2024

Pediatric Neuroglial Tumors: A Review of Ependymoma and Dysembryoplastic Neuroepithelial Tumor.

Pediatric neurology
2025

Appearance of intracranial cottonoids on intraoperative magnetic resonance imaging.

The neuroradiology journal
2024

Emerging insights into cephalic neural crest disorders: A single center experience.

Journal of clinical imaging science
2024

Bright diffusion sign: A sensitive and specific radiologic biomarker for multinodular and vacuolating neuronal tumor.

Journal of neuroradiology = Journal de neuroradiologie
2024

Epileptogenic focal lesions in Dravet syndrome: A warning to investigators.

Epileptic disorders : international epilepsy journal with videotape
2024

The T2-FLAIR mismatch sign in oncologic neuroradiology: History, current use, emerging data, and future directions.

The neuroradiology journal
2024

An imaging review of the hippocampus and its common pathologies.

Journal of neuroimaging : official journal of the American Society of Neuroimaging
2024

Atypical presentation of dysembryoplastic neuroepithelial tumor in an adult without epilepsy: a case report.

The International journal of neuroscience
2024

Characterization of low-grade epilepsy-associated tumor from implanted stereoelectroencephalography electrodes.

Epilepsia open
2024

Polymorphous low-grade neuroepithelial tumour of young (PLNTY): the new kid on the block.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2023

Epileptic versus neuro-oncological focus of management in pediatric patients with concurrent primary brain lesion and seizures: a systematic review.

Journal of neurosurgery. Pediatrics
2023

Surgical treatment of epilepsy - Initial experience from a comprehensive epilepsy program in coastal South India.

Journal of neurosciences in rural practice
2023

Clinicopathological features of dysembryoplastic neuroepithelial tumor: a case series.

Journal of medical case reports
2023

[Surgical Pathological Features of Epileptogenic Brain Lesions: Cortical Dysplasia and Tumors].

Brain and nerve = Shinkei kenkyu no shinpo
2023

Stereo-electroencephalography (SEEG)-Guided Surgery in Epilepsy With Cingulate Gyrus Involvement: Electrode Implantation Strategies and Postoperative Seizure Outcome.

Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society
2023

Association between features of intraoperative ultrasound and magnetic resonance imaging in the diagnosis of dysembryoplastic neuroepithelial tumor.

Quantitative imaging in medicine and surgery
2023

Value of quantitative apparent diffusion coefficients in differentiating low-grade gliomas from mixed neuronal-glial tumors.

World neurosurgery: X
2023

Posterior Fossa Dysembryoplastic Neuroepithelial Tumor: A Neuropathological Report.

Cureus
2022

Low-grade epilepsy-associated neuroepithelial tumors: Tumor spectrum and diagnosis based on genetic alterations.

Frontiers in neuroscience
2023

Clinico-pathological and epigenetic heterogeneity of diffuse gliomas with FGFR3::TACC3 fusion.

Acta neuropathologica communications
2023

Genomic analysis as a tool to infer disparate phylogenetic origins of dysembryoplastic neuroepithelial tumors and their satellite lesions.

Scientific reports
2022

Association of CT and MRI Manifestations with Pathology in Dysembryoplastic Neuroepithelial Tumors.

Journal of the Belgian Society of Radiology
2022

Case Report: A novel LHFPL3::NTRK2 fusion in dysembryoplastic neuroepithelial tumor.

Frontiers in oncology
2022

Central nervous system involvement in individuals with RASopathies.

American journal of medical genetics. Part C, Seminars in medical genetics
2022

Early Epilepsy Surgery in Benign Cerebral Tumors: Avoid Your 'Low-Grade' Becoming a 'Long-Term' Epilepsy-Associated Tumor.

Journal of clinical medicine
2022

Preoperative MRI for postoperative seizure prediction: a radiomics study of dysembryoplastic neuroepithelial tumor and a systematic review.

Neurosurgical focus
2022

Novel FGFR2::ZCCHC24 Fusion in Dysembryoplastic Neuroepithelial Tumor.

Journal of neuropathology and experimental neurology
2022

Infiltration in Pilocytic Astrocytoma: A Diagnostic Pitfall.

Cureus
2022

A Constellation of Atypical Findings in a Nine-Year-Old Child With Dysembryoplastic Neuroepithelial Tumors: A Case Report and Review of the Literature.

Cureus
2022

Dysembryoplastic Neuroepithelial Tumor: A Benign but Complex Tumor of the Cerebral Cortex.

Brain tumor research and treatment
2023

Electroconvulsive Therapy in a Young Adult Patient With Bipolar Depression and Dysembryoplastic Neuroepithelial Tumor: A Case Report.

The journal of ECT
2021

5-Aminolevulinic acid-assisted resection of pediatric dysembryoplastic neuroepithelial tumor: illustrative case.

Journal of neurosurgery. Case lessons
2022

The landscape of common genetic drivers and DNA methylation in low-grade (epilepsy-associated) neuroepithelial tumors: A review.

Neuropathology : official journal of the Japanese Society of Neuropathology
2021

SURGICAL AND SEIZURE TREATMENT OUTCOMES IN ADULT DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMORS: A CASE SERIES.

Acta clinica Croatica
2022

Bilateral oligodendroglial hamartomas: A rare cause of drug-resistant epilepsy in a pediatric patient.

Journal of clinical imaging science
2022

Dysembryoplastic Neuroepithelial Tumor of the Infratentorial Multiple Lesions: A Case Report and Review of the Literature.

NMC case report journal
2022

Uncommon Glioneuronal Tumors: A Radiologic and Pathologic Synopsis.

AJNR. American journal of neuroradiology
2022

From a dysembryoplastic neuroepithelial tumor to a glioblastoma multiforme: Pitfalls of initial diagnosis on biopsy material, a case report.

Surgical neurology international
2022

Malignant transformation of a dysembryoplastic neuroepithelial tumor presenting with intraventricular hemorrhage.

Radiology case reports
2022

Preoperative and Postoperative Gait Analysis and Video for Selective Dorsal Rhizotomy in Focal Hemiplegic Spasticity: 2-Dimensional Operative Video.

World neurosurgery
2021

Multinodular and Vacuolating Neuronal Tumor: Incidental Diagnosis of a Rare Brain Lesion.

Cureus
2022

Frequent FGFR1 hotspot alterations in driver-unknown low-grade glioma and mixed neuronal-glial tumors.

Journal of cancer research and clinical oncology
2022

Challenges in the Intraoperative Consultation of Low-Grade Epilepsy-Associated Neuroepithelial Tumors by Cytomorphology in Squash Preparations.

Acta cytologica
2021

Magnetic Resonance-Guided Laser Interstitial Thermal Therapy (MR-gLiTT) in Pediatric Epilepsy Surgery: State of the Art and Presentation of Giannina Gaslini Children's Hospital (Genoa, Italy) Series.

Frontiers in neurology
2022

Prevalence of BRAFV600 in glioma and use of BRAF Inhibitors in patients with BRAFV600 mutation-positive glioma: systematic review.

Neuro-oncology
2021

Clinical and histopathological profile of dysembryoplastic neuroepithelial tumor: An experience from a tertiary care center.

Journal of cancer research and therapeutics
2022

Integrated genotype-phenotype analysis of long-term epilepsy-associated ganglioglioma.

Brain pathology (Zurich, Switzerland)
2021

The spectrum of mitochondrial DNA (mtDNA) mutations in pediatric CNS tumors.

Neuro-oncology advances
2021

Intraventricular dysembryoplastic neuroepithelial tumor in the temporal horn with Broad involvement of the ependyma.

Clinical case reports
2021

A Case of Dysembryoplastic Neuroepithelial Tumor in an Adolescent Male.

Cureus
2021

A case of a rosette-forming glioneuronal tumor with clinicopathological features of a dysembryoplastic neuroepithelial tumor and fibroblast growth factor receptor 1 internal tandem duplication.

Brain tumor pathology
2021

From Neurosurgical Planning to Histopathological Brain Tumor Characterization: Potentialities of Arcuate Fasciculus Along-Tract Diffusion Tensor Imaging Tractography Measures.

Frontiers in neurology
2021

Can we use intraoperative high-frequency oscillations to guide tumor-related epilepsy surgery?

Epilepsia
2021

The T2-FLAIR mismatch sign as a predictor of IDH-mutant, 1p/19q-noncodeleted lower-grade gliomas: a systematic review and diagnostic meta-analysis.

European radiology
2020

[Features of the neuroradiological picture of ganglioglioma on the example of 20 clinical cases].

Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova
2021

Cognitive impairment in temporal lobe epilepsy: contributions of lesion, localization and lateralization.

Journal of neurology
2020

Dysembryoplastic neuroepithelial tumor and temporal arachnoid cyst on a child with epilepsy. Which one to operate? Case report and literature review.

Cirugia y cirujanos
2020

Coupling between infraslow activities and high-frequency oscillations precedes seizure onset.

Epilepsia open
2020

Non-invasive therapeutic brain stimulation for treatment of resistant focal epilepsy in a teenager.

Clinical neurophysiology practice
2020

Comprehensive analysis of diverse low-grade neuroepithelial tumors with FGFR1 alterations reveals a distinct molecular signature of rosette-forming glioneuronal tumor.

Acta neuropathologica communications
2021

Orbitofrontal epilepsy: distinct neuronal networks underlying electroclinical subtypes and surgical outcomes.

Journal of neurosurgery
2020

Epilepsy in NF1: a systematic review of the literature.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2020

Corrigendum to "T2-FLAIR mismatch sign in dysembryoplastic neuroepithelial tumor" [Eur. J. Radiol. 126 (2020) 108924].

European journal of radiology
2020

Breakpoint Mapping of Symptomatic Balanced Translocations Links the EPHA6, KLF13 and UBR3 Genes to Novel Disease Phenotype.

Journal of clinical medicine
2020

Malignant transformation of a dysembryoplastic neuroepithelial tumor verified by a shared copy number gain of the tyrosine kinase domain of FGFR1.

Brain tumor pathology
2020

Dysembryoplastic neuroepithelial tumor presenting as a hypothalamic hamartoma in a child with gelastic seizures: case report.

Journal of neurosurgery. Pediatrics
2020

Pediatric septal dysembryoplastic neuroepithelial tumor (sDNT): case-based update.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2020

T2-FLAIR mismatch sign in dysembryoplasticneuroepithelial tumor.

European journal of radiology
2020

Low-grade developmental and epilepsy associated brain tumors: a critical update 2020.

Acta neuropathologica communications
2020

Junior-Real Time neuropsychological testing (j-RTNT) for a young patient undergoing awake craniotomy.

Brain and cognition
2020

A Multi-Institutional Analysis of Factors Influencing Surgical Outcomes for Patients with Newly Diagnosed Grade I Gliomas.

World neurosurgery
2019

Multinodular and vacuolating neuronal tumor (MVNT): A presumably incidental and asymptomatic case in an intractable epilepsy patient.

Clinical neurophysiology practice
2019

[Natural History of Patients with Asymptomatic FLAIR High-signal Lesions Suspected of Lower-grade Gliomas].

No shinkei geka. Neurological surgery
2020

Rapid fully-automated assay for routine molecular diagnosis of BRAF mutations for personalized therapy of low grade gliomas.

Pediatric hematology and oncology
2019

Genomic Analysis of Dysembryoplastic Neuroepithelial Tumor Spectrum Reveals a Diversity of Molecular Alterations Dysregulating the MAPK and PI3K/mTOR Pathways.

Journal of neuropathology and experimental neurology
2020

Myxoid glioneuronal tumor, PDGFRA p.K385-mutant: clinical, radiologic, and histopathologic features.

Brain pathology (Zurich, Switzerland)
2020

A Multifocal Glioneuronal Tumor with RGNT-Like Morphology Occupying the Supratentorial Ventricular System and Infiltrating the Brain Parenchyma.

World neurosurgery
2019

Polymorphous Low-Grade Neuroepithelial Tumor of the Young: A Case Report with Genomic Findings.

World neurosurgery
2019

A case of recurrent epilepsy-associated rosette-forming glioneuronal tumor with anaplastic transformation in the absence of therapy.

Neuropathology : official journal of the Japanese Society of Neuropathology
2019

Dysembryoplastic Neuroepithelial Tumor with Enlarging Heterogeneously Enhancing Organizing Hematoma.

World neurosurgery
2019

Synchronous identification of a dysembryoplastic neuroepithelial tumor (DNET) and an oligodendroglioma in a patient: A case report.

Clinical neuropathology
2019

Malignant Glial Neuronal Tumors After West Nile Virus Neuroinvasive Disease: A Coincidence or a Clue?

The Neurohospitalist
2019

An epilepsy-associated glioneuronal tumor with mixed morphology harboring FGFR1 mutation.

Pathology international
2019

Expression-based decision tree model reveals distinct microRNA expression pattern in pediatric neuronal and mixed neuronal-glial tumors.

BMC cancer
2019

Clinical Pearls and Advances in Molecular Researches of Epilepsy-Associated Tumors.

Journal of Korean Neurosurgical Society
2019

Satellite lesions of DNET: implications for seizure and tumor control after resection.

Journal of neuro-oncology
2019

Dysembryoplastic Neuroepithelial Tumors: What You Need to Know.

World neurosurgery
2019

Subependymoma and dysembryoplastic neuroepithelial collision tumor in the foramen of Monro: case report.

Journal of neurosurgery. Pediatrics
2019

Papillary glioneuronal tumor (PGNT) exhibits a characteristic methylation profile and fusions involving PRKCA.

Acta neuropathologica
2019

Septal dysembryoplastic neuroepithelial tumor: a comprehensive clinical, imaging, histopathologic, and molecular analysis.

Neuro-oncology
2019

Occurrence of high-grade glioma in Noonan syndrome: Report of two cases.

Pediatric blood & cancer
2019

A Novel Missense LIG4 Mutation in a Patient With a Phenotype Mimicking Behçet's Disease.

Journal of clinical immunology
2019

Epilepsy Surgery for Children With Low-Grade Epilepsy-Associated Tumors: Factors Associated With Seizure Recurrence and Cognitive Function.

Pediatric neurology
2018

[BRAF V600E mutation and clinicopathologic characteristics in 250 cases of brain tumors associated with epilepsy].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2018

Preoperative evaluation and surgical management of infants and toddlers with drug-resistant epilepsy.

Neurosurgical focus
2018

Freiburg Neuropathology Case Conference : Tumor of the Mesiotemporal Lobe in a Child.

Clinical neuroradiology
2018

Surgical Treatment of Lesional Mesial Temporal Lobe Epilepsy.

Journal of epilepsy research
2018

Lethal disseminated dysembryoplastic neuroepithelial tumor following West Nile virus: Report of a very unusual combination.

Radiology case reports
2018

Dysembryoplastic neuroepithelial tumor-like pilocytic astrocytoma: A case report.

Medicine
2018

Intraoperative monitoring with visual evoked potentials for brain surgeries.

Journal of neurosurgery
2018

Adapting a memory fMRI research protocol in clinical routine: Feasibility and results.

Epilepsy & behavior : E&B
2018

Lesional Temporal Lobe Epilepsy: Beware the Deceitful "Panic Attack".

World neurosurgery
2018

Neuroimaging of Dilated Perivascular Spaces: From Benign and Pathologic Causes to Mimics.

Journal of neuroimaging : official journal of the American Society of Neuroimaging
2018

Surgical treatment of thalamic tumors in children.

Journal of neurosurgery. Pediatrics
2018

Intractable epilepsy due to a rosette-forming glioneuronal tumor with a dysembryoplastic neuroepithelial background.

Neuropathology : official journal of the Japanese Society of Neuropathology
2018

Atypical presentations of dysembryoplastic neuroepithelial tumors.

Epilepsia
2017

Occipital dysembryoplastic neuroepithelial tumor presenting as adult-onset temporal epilepsy.

Epilepsy & behavior case reports
2017

[Clinicopathologic features of infant dysembryoplastic neuroepithelial tumor: a case report and literature review].

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
2018

Surgical treatment of neuronal-glial tumors of mesial-basal part of temporal lobe: Long term outcome and control of epilepsy in pediatric patients.

Neurologia i neurochirurgia polska
2018

Role of the temporal pole in temporal lobe epilepsy seizure networks: an intracranial electrode investigation.

Journal of neurosurgery
2017

Cerebellar dysembryoplastic neuroepithelial tumor: report of a case and review of the literature.

Journal of neurology
2017

Utility of Stereoelectroencephalography in Children with Dysembryoplastic Neuroepithelial Tumor and Cortical Malformation.

The Neurodiagnostic journal
2017

Long-term recurrence of dysembryoplastic neuroepithelial tumor: Clinical case report.

Surgical neurology international
2017

Surgery for dysembryoplastic neuroepithelial tumors and gangliogliomas in eloquent areas. Functional results and seizure control.

Neuro-Chirurgie
2017

Pediatric Basal Ganglia Region Tumors: Clinical and Radiologic Features Correlated with Histopathologic Findings.

World neurosurgery
2017

Noonan syndrome, PTPN11 mutations, and brain tumors. A clinical report and review of the literature.

American journal of medical genetics. Part A
2017

A systematic evaluation of intraoperative white matter tract shift in pediatric epilepsy surgery using high-field MRI and probabilistic high angular resolution diffusion imaging tractography.

Journal of neurosurgery. Pediatrics
2016

Volumetric growth analysis of an insular dysembryoplastic neuroepithelial tumor over a 10-year follow-up.

Surgical neurology international
2017

PIK3CA mutation in a mixed dysembryoplastic neuroepithelial tumor and rosette forming glioneuronal tumor, a case report and literature review.

Journal of the neurological sciences
2017

FDOPA PET-CT of Nonenhancing Brain Tumors.

Clinical nuclear medicine
2016

Focal Epileptogenic Lesions in Adult Patients with Epilepsy and Generalized Epileptiform Discharges.

Journal of epilepsy research
2017

Posterior cortex epilepsy surgery in childhood and adolescence: Predictors of long-term seizure outcome.

Epilepsia
2017

Early and late epilepsy surgery in focal epilepsies associated with long-term epilepsy-associated tumors.

Journal of neurosurgery
2016

A rare case of dysembryoplastic neuroepithelial tumor combined with encephalocraniocutaneous lipomatosis and intractable seizures.

Korean journal of pediatrics
2017

Seizures caused by brain tumors in children.

Seizure
2017

A comprehensive analysis identifies BRAF hotspot mutations associated with gliomas with peculiar epithelial morphology.

Neuropathology : official journal of the Japanese Society of Neuropathology
2017

Droplet digital PCR is a powerful technique to demonstrate frequent FGFR1 duplication in dysembryoplastic neuroepithelial tumors.

Oncotarget
2016

Alterations in BRAF gene, and enhanced mTOR and MAPK signaling in dysembryoplastic neuroepithelial tumors (DNTs).

Epilepsy research
2016

Subcortical DNET in a Patient With an Enzymatic Deficiency: A Rare Case and Review of the Literature.

Journal of pediatric hematology/oncology
2016

Glioneuronal tumors of cerebral hemisphere in children: correlation of surgical resection with seizure outcomes and tumor recurrences.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2016

Radiologic response to radiation therapy concurrent with temozolomide for progressive simple dysembryoplastic neuroepithelial tumor.

Acta neurochirurgica
2016

Remitted epilepsy with dysembryoplastic neuroepithelial tumor involving the thalamus.

Pediatrics international : official journal of the Japan Pediatric Society
2016

Pediatric epilepsy surgery: could age be a predictor of outcomes?

Journal of neurosurgery. Pediatrics
2016

Long-term drug-resistant temporal lobe epilepsy associated with a mixed ganglioglioma and dysembryoplastic neuroepithelial tumor in an elderly patient.

Surgical neurology international
2016

Dysembryoplastic neuroepithelial tumor: A rare brain tumor not to be misdiagnosed.

Asian journal of neurosurgery
2016

Atypical Teratoid Rhabdoid Tumor Diagnosis after Partial Resection of Dysembryoplastic Neuroepithelial Tumor: Case Report and Review of the Literature.

Pediatric neurosurgery
2016

Beneficial impact of high-field intraoperative magnetic resonance imaging on the efficacy of pediatric low-grade glioma surgery.

Neurosurgical focus
2016

Malignant Transformation of a Dysembryoplastic Neuroepithelial Tumor (DNET) Characterized by Genome-Wide Methylation Analysis.

Journal of neuropathology and experimental neurology
2016

Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors.

Acta neuropathologica
2016

Dysembryoplastic Neuroepithelial Tumor of the Septum Pellucidum and the Supratentorial Midline: Histopathologic, Neuroradiologic, and Molecular Features of 7 Cases.

The American journal of surgical pathology
2016

[Epilepsy-associated tumors of the central nervous system: Epilepsy surgery and oncological aspects].

Der Nervenarzt
2016

Multinodular and vacuolating neuronal tumor affecting amygdala and hippocampus: A quasi-tumor?

Pathology international
2016

Review of seizure outcomes after surgical resection of dysembryoplastic neuroepithelial tumors.

Journal of neuro-oncology
2016

Real-time magnetic resonance imaging-guided frameless stereotactic brain biopsy: technical note.

Journal of neurosurgery
2015

Dysembryoplastic Neuroepithelial Tumors.

Journal of pathology and translational medicine
2016

Occurrence of DNET and other brain tumors in Noonan syndrome warrants caution with growth hormone therapy.

American journal of medical genetics. Part A
2015

Epilepsy surgery related to pediatric brain tumors: Miami Children's Hospital experience.

Journal of neurosurgery. Pediatrics
2015

Resection of a dysembryoplastic neuroepithelial tumor in the precentral gyrus.

World journal of pediatrics : WJP
2015

Impact of epilepsy surgery on development of preschool children: identification of a cohort likely to benefit from early intervention.

Journal of neurosurgery. Pediatrics
2015

Cerebral sparganosis: case report and review of the European cases.

Acta neurochirurgica
2015

"Unusual brain stone": heavily calcified primary neoplasm with some features suggestive of angiocentric glioma.

Journal of neurosurgery
2015

[Clinical and histological characteristics of ictal onset zone in cases of intractable epilepsy associated with dysembryoplastic neuroepithelial tumor].

Brain and nerve = Shinkei kenkyu no shinpo
2015

Semiological and electroencephalographic features of epilepsy with amygdalar lesion.

Epilepsy research
Ver todos os 185 no EuropePMC

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Multifocal rosette-forming glioneuronal tumor-like low-grade glioneuronal tumor with dysembryoplastic neuroepithelial tumor features associated with drug-resistant epilepsy: a case report and literature review.
    Brain tumor pathology· 2026· PMID 41495347mais citado
  2. Dysembryoplastic neuroepithelial tumor: Malignant transformation following radiation.
    Journal of neuropathology and experimental neurology· 2026· PMID 41689548mais citado
  3. Endoscopic Transorbital Approach for Select Recurrent and Newly Diagnosed Anteromedial Temporal Lobe Gliomas.
    Journal of neurological surgery. Part B, Skull base· 2026· PMID 41808971mais citado
  4. A Case of Myxoid Glioneuronal Tumor in the Lateral Ventricle.
    NMC case report journal· 2026· PMID 41799219mais citado
  5. PTPN11-related Noonan syndrome predisposes to multifocal low-grade CNS tumors harboring FGFR1 variants.
    Journal of neuro-oncology· 2026· PMID 41784910mais citado
  6. Long-term radiological progression after resection of dysembryoplastic neuroepithelial tumors: patterns and prognostic factors.
    J Neurooncol· 2026· PMID 41944976recente
  7. Low-grade Epilepsy-associated Tumors and Other Acquired Causes of Epilepsy: MR Imaging.
    Neuroimaging Clin N Am· 2026· PMID 41932777recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:251946(Orphanet)
  2. MONDO:0005505(MONDO)
  3. GARD:10640(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q824595(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Tumor neuroepitelial disembrioplásico
Compêndio · Raras BR

Tumor neuroepitelial disembrioplásico

ORPHA:251946 · MONDO:0005505
CID-10
D33.0 · Neoplasia benigna do encéfalo, supratentorial
CID-11
Ensaios
1 ativos
Início
All ages
MedGen
UMLS
C1266177
EuropePMC
Wikidata
Papers 10a
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