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.
Introdução
O que você precisa saber de cara
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.
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Tumor neuroepitelial disembrioplásico
Selecione um estado ou use sua localização para ver resultados.
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
🟢 Recrutando agora
1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
4 ensaios clínicos encontrados, 1 ativos.
Publicações mais relevantes
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.
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.
Dysembryoplastic neuroepithelial tumor: Malignant transformation following radiation.
Endoscopic Transorbital Approach for Select Recurrent and Newly Diagnosed Anteromedial Temporal Lobe Gliomas.
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.
A Case of Myxoid Glioneuronal Tumor in the Lateral Ventricle.
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.
PTPN11-related Noonan syndrome predisposes to multifocal low-grade CNS tumors harboring FGFR1 variants.
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
Long-term radiological progression after resection of dysembryoplastic neuroepithelial tumors: patterns and prognostic factors.
Low-grade Epilepsy-associated Tumors and Other Acquired Causes of Epilepsy: MR Imaging.
Endoscopic Transorbital Approach for Select Recurrent and Newly Diagnosed Anteromedial Temporal Lobe Gliomas.
A Case of Myxoid Glioneuronal Tumor in the Lateral Ventricle.
PTPN11-related Noonan syndrome predisposes to multifocal low-grade CNS tumors harboring FGFR1 variants.
📚 EuropePMC185 artigos no totalmostrando 178
Endoscopic Transorbital Approach for Select Recurrent and Newly Diagnosed Anteromedial Temporal Lobe Gliomas.
Journal of neurological surgery. Part B, Skull baseA Case of Myxoid Glioneuronal Tumor in the Lateral Ventricle.
NMC case report journalPTPN11-related Noonan syndrome predisposes to multifocal low-grade CNS tumors harboring FGFR1 variants.
Journal of neuro-oncologyDysembryoplastic neuroepithelial tumor: Malignant transformation following radiation.
Journal of neuropathology and experimental neurologyA Case of Dysembryoplastic Neuroepithelial Tumor in an HIV-Positive Adult: Diagnostic Lessons for Clinicians.
CureusMagnetic resonance imaging findings of dysembryoplastic neuroepithelial tumors and low-grade astrocytomas.
Journal of the Chinese Medical Association : JCMAPediatric low-grade epilepsy-associated tumors (LEATS): neuroimaging review and genetics update.
Pediatric radiologyMultifocal 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 pathologyCase Report: FGFR1 mutation and massive chromosome loss drive malignant transformation of low-grade gliomas.
Frontiers in oncologyMRI-based radiomics model for differentiating focal cortical dysplasia from dysembryoplastic neuroepithelial tumor in epileptic children.
Frontiers in neurologyGlioneuronal and Neuronal Tumors in Adults: WHO 2021 Imaging and Molecular Updates.
AJNR. American journal of neuroradiologyRuptured intratumoral arteriovenous malformation in a patient with dysembryoplastic neuroepithelial tumor: A case report.
Surgical neurology internationalCo-Occurrence of Dysembryoplastic Neuroepithelial Tumor and Hodgkin Lymphoma in a Patient with Noonan Syndrome and Mutation in the PTPN11 Gene.
Hormone research in paediatricsDifferentiation of multinodular and vacuolating neuronal tumor and dysembryoplastic neuroepithelial tumor based on MRI.
Turkish journal of medical sciencesUltrahigh-field imaging (7 Tesla) in DNET: Unmasking microstructural imaging characteristics - A case report.
Epilepsy & behavior reportsAtypical Lesions in Virchow-Robin Spaces: A Case Report.
CureusAre 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 SocietyMyxoid 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 NeurosurgeryA glioneuronal tumor with neurocytic rosettes harboring FGFR1 internal tandem duplication - A report of a unique case.
Neuropathology : official journal of the Japanese Society of NeuropathologyDisseminated ependymal dysembryoplastic neuroepithelial tumor: a case report and literature review.
Zhurnal voprosy neirokhirurgii imeni N. N. BurdenkoDysembryoplastic Neuroepithelial Tumor: A Case Report of A Benign Intracranial Lesion Masquerading as Seizure Disorder.
CureusImaging of pediatric glioneuronal and neuronal tumors.
Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery[Differential diagnosis of epileptogenic substrates of unknown etiology using a modification of the routine MRI protocol].
Zhurnal nevrologii i psikhiatrii imeni S.S. KorsakovaLong-term seizure outcome after epilepsy surgery of neuroglial tumors.
Frontiers in neurologyLow-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 NeurosurgeryPediatric Neuroglial Tumors: A Review of Ependymoma and Dysembryoplastic Neuroepithelial Tumor.
Pediatric neurologyAppearance of intracranial cottonoids on intraoperative magnetic resonance imaging.
The neuroradiology journalEmerging insights into cephalic neural crest disorders: A single center experience.
Journal of clinical imaging scienceBright diffusion sign: A sensitive and specific radiologic biomarker for multinodular and vacuolating neuronal tumor.
Journal of neuroradiology = Journal de neuroradiologieEpileptogenic focal lesions in Dravet syndrome: A warning to investigators.
Epileptic disorders : international epilepsy journal with videotapeThe T2-FLAIR mismatch sign in oncologic neuroradiology: History, current use, emerging data, and future directions.
The neuroradiology journalAn imaging review of the hippocampus and its common pathologies.
Journal of neuroimaging : official journal of the American Society of NeuroimagingAtypical presentation of dysembryoplastic neuroepithelial tumor in an adult without epilepsy: a case report.
The International journal of neuroscienceCharacterization of low-grade epilepsy-associated tumor from implanted stereoelectroencephalography electrodes.
Epilepsia openPolymorphous 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 NeurosurgeryEpileptic versus neuro-oncological focus of management in pediatric patients with concurrent primary brain lesion and seizures: a systematic review.
Journal of neurosurgery. PediatricsSurgical treatment of epilepsy - Initial experience from a comprehensive epilepsy program in coastal South India.
Journal of neurosciences in rural practiceClinicopathological features of dysembryoplastic neuroepithelial tumor: a case series.
Journal of medical case reports[Surgical Pathological Features of Epileptogenic Brain Lesions: Cortical Dysplasia and Tumors].
Brain and nerve = Shinkei kenkyu no shinpoStereo-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 SocietyAssociation between features of intraoperative ultrasound and magnetic resonance imaging in the diagnosis of dysembryoplastic neuroepithelial tumor.
Quantitative imaging in medicine and surgeryValue of quantitative apparent diffusion coefficients in differentiating low-grade gliomas from mixed neuronal-glial tumors.
World neurosurgery: XPosterior Fossa Dysembryoplastic Neuroepithelial Tumor: A Neuropathological Report.
CureusLow-grade epilepsy-associated neuroepithelial tumors: Tumor spectrum and diagnosis based on genetic alterations.
Frontiers in neuroscienceClinico-pathological and epigenetic heterogeneity of diffuse gliomas with FGFR3::TACC3 fusion.
Acta neuropathologica communicationsGenomic analysis as a tool to infer disparate phylogenetic origins of dysembryoplastic neuroepithelial tumors and their satellite lesions.
Scientific reportsAssociation of CT and MRI Manifestations with Pathology in Dysembryoplastic Neuroepithelial Tumors.
Journal of the Belgian Society of RadiologyCase Report: A novel LHFPL3::NTRK2 fusion in dysembryoplastic neuroepithelial tumor.
Frontiers in oncologyCentral nervous system involvement in individuals with RASopathies.
American journal of medical genetics. Part C, Seminars in medical geneticsEarly Epilepsy Surgery in Benign Cerebral Tumors: Avoid Your 'Low-Grade' Becoming a 'Long-Term' Epilepsy-Associated Tumor.
Journal of clinical medicinePreoperative MRI for postoperative seizure prediction: a radiomics study of dysembryoplastic neuroepithelial tumor and a systematic review.
Neurosurgical focusNovel FGFR2::ZCCHC24 Fusion in Dysembryoplastic Neuroepithelial Tumor.
Journal of neuropathology and experimental neurologyInfiltration in Pilocytic Astrocytoma: A Diagnostic Pitfall.
CureusA Constellation of Atypical Findings in a Nine-Year-Old Child With Dysembryoplastic Neuroepithelial Tumors: A Case Report and Review of the Literature.
CureusDysembryoplastic Neuroepithelial Tumor: A Benign but Complex Tumor of the Cerebral Cortex.
Brain tumor research and treatmentElectroconvulsive Therapy in a Young Adult Patient With Bipolar Depression and Dysembryoplastic Neuroepithelial Tumor: A Case Report.
The journal of ECT5-Aminolevulinic acid-assisted resection of pediatric dysembryoplastic neuroepithelial tumor: illustrative case.
Journal of neurosurgery. Case lessonsThe 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 NeuropathologySURGICAL AND SEIZURE TREATMENT OUTCOMES IN ADULT DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMORS: A CASE SERIES.
Acta clinica CroaticaBilateral oligodendroglial hamartomas: A rare cause of drug-resistant epilepsy in a pediatric patient.
Journal of clinical imaging scienceDysembryoplastic Neuroepithelial Tumor of the Infratentorial Multiple Lesions: A Case Report and Review of the Literature.
NMC case report journalUncommon Glioneuronal Tumors: A Radiologic and Pathologic Synopsis.
AJNR. American journal of neuroradiologyFrom a dysembryoplastic neuroepithelial tumor to a glioblastoma multiforme: Pitfalls of initial diagnosis on biopsy material, a case report.
Surgical neurology internationalMalignant transformation of a dysembryoplastic neuroepithelial tumor presenting with intraventricular hemorrhage.
Radiology case reportsPreoperative and Postoperative Gait Analysis and Video for Selective Dorsal Rhizotomy in Focal Hemiplegic Spasticity: 2-Dimensional Operative Video.
World neurosurgeryMultinodular and Vacuolating Neuronal Tumor: Incidental Diagnosis of a Rare Brain Lesion.
CureusFrequent FGFR1 hotspot alterations in driver-unknown low-grade glioma and mixed neuronal-glial tumors.
Journal of cancer research and clinical oncologyChallenges in the Intraoperative Consultation of Low-Grade Epilepsy-Associated Neuroepithelial Tumors by Cytomorphology in Squash Preparations.
Acta cytologicaMagnetic 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 neurologyPrevalence of BRAFV600 in glioma and use of BRAF Inhibitors in patients with BRAFV600 mutation-positive glioma: systematic review.
Neuro-oncologyClinical and histopathological profile of dysembryoplastic neuroepithelial tumor: An experience from a tertiary care center.
Journal of cancer research and therapeuticsIntegrated genotype-phenotype analysis of long-term epilepsy-associated ganglioglioma.
Brain pathology (Zurich, Switzerland)The spectrum of mitochondrial DNA (mtDNA) mutations in pediatric CNS tumors.
Neuro-oncology advancesIntraventricular dysembryoplastic neuroepithelial tumor in the temporal horn with Broad involvement of the ependyma.
Clinical case reportsA Case of Dysembryoplastic Neuroepithelial Tumor in an Adolescent Male.
CureusA 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 pathologyFrom Neurosurgical Planning to Histopathological Brain Tumor Characterization: Potentialities of Arcuate Fasciculus Along-Tract Diffusion Tensor Imaging Tractography Measures.
Frontiers in neurologyCan we use intraoperative high-frequency oscillations to guide tumor-related epilepsy surgery?
EpilepsiaThe 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[Features of the neuroradiological picture of ganglioglioma on the example of 20 clinical cases].
Zhurnal nevrologii i psikhiatrii imeni S.S. KorsakovaCognitive impairment in temporal lobe epilepsy: contributions of lesion, localization and lateralization.
Journal of neurologyDysembryoplastic neuroepithelial tumor and temporal arachnoid cyst on a child with epilepsy. Which one to operate? Case report and literature review.
Cirugia y cirujanosCoupling between infraslow activities and high-frequency oscillations precedes seizure onset.
Epilepsia openNon-invasive therapeutic brain stimulation for treatment of resistant focal epilepsy in a teenager.
Clinical neurophysiology practiceComprehensive analysis of diverse low-grade neuroepithelial tumors with FGFR1 alterations reveals a distinct molecular signature of rosette-forming glioneuronal tumor.
Acta neuropathologica communicationsOrbitofrontal epilepsy: distinct neuronal networks underlying electroclinical subtypes and surgical outcomes.
Journal of neurosurgeryEpilepsy in NF1: a systematic review of the literature.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryCorrigendum to "T2-FLAIR mismatch sign in dysembryoplastic neuroepithelial tumor" [Eur. J. Radiol. 126 (2020) 108924].
European journal of radiologyBreakpoint Mapping of Symptomatic Balanced Translocations Links the EPHA6, KLF13 and UBR3 Genes to Novel Disease Phenotype.
Journal of clinical medicineMalignant transformation of a dysembryoplastic neuroepithelial tumor verified by a shared copy number gain of the tyrosine kinase domain of FGFR1.
Brain tumor pathologyDysembryoplastic neuroepithelial tumor presenting as a hypothalamic hamartoma in a child with gelastic seizures: case report.
Journal of neurosurgery. PediatricsPediatric septal dysembryoplastic neuroepithelial tumor (sDNT): case-based update.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryT2-FLAIR mismatch sign in dysembryoplasticneuroepithelial tumor.
European journal of radiologyLow-grade developmental and epilepsy associated brain tumors: a critical update 2020.
Acta neuropathologica communicationsJunior-Real Time neuropsychological testing (j-RTNT) for a young patient undergoing awake craniotomy.
Brain and cognitionA Multi-Institutional Analysis of Factors Influencing Surgical Outcomes for Patients with Newly Diagnosed Grade I Gliomas.
World neurosurgeryMultinodular and vacuolating neuronal tumor (MVNT): A presumably incidental and asymptomatic case in an intractable epilepsy patient.
Clinical neurophysiology practice[Natural History of Patients with Asymptomatic FLAIR High-signal Lesions Suspected of Lower-grade Gliomas].
No shinkei geka. Neurological surgeryRapid fully-automated assay for routine molecular diagnosis of BRAF mutations for personalized therapy of low grade gliomas.
Pediatric hematology and oncologyGenomic Analysis of Dysembryoplastic Neuroepithelial Tumor Spectrum Reveals a Diversity of Molecular Alterations Dysregulating the MAPK and PI3K/mTOR Pathways.
Journal of neuropathology and experimental neurologyMyxoid glioneuronal tumor, PDGFRA p.K385-mutant: clinical, radiologic, and histopathologic features.
Brain pathology (Zurich, Switzerland)A Multifocal Glioneuronal Tumor with RGNT-Like Morphology Occupying the Supratentorial Ventricular System and Infiltrating the Brain Parenchyma.
World neurosurgeryPolymorphous Low-Grade Neuroepithelial Tumor of the Young: A Case Report with Genomic Findings.
World neurosurgeryA 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 NeuropathologyDysembryoplastic Neuroepithelial Tumor with Enlarging Heterogeneously Enhancing Organizing Hematoma.
World neurosurgerySynchronous identification of a dysembryoplastic neuroepithelial tumor (DNET) and an oligodendroglioma in a patient: A case report.
Clinical neuropathologyMalignant Glial Neuronal Tumors After West Nile Virus Neuroinvasive Disease: A Coincidence or a Clue?
The NeurohospitalistAn epilepsy-associated glioneuronal tumor with mixed morphology harboring FGFR1 mutation.
Pathology internationalExpression-based decision tree model reveals distinct microRNA expression pattern in pediatric neuronal and mixed neuronal-glial tumors.
BMC cancerClinical Pearls and Advances in Molecular Researches of Epilepsy-Associated Tumors.
Journal of Korean Neurosurgical SocietySatellite lesions of DNET: implications for seizure and tumor control after resection.
Journal of neuro-oncologyDysembryoplastic Neuroepithelial Tumors: What You Need to Know.
World neurosurgerySubependymoma and dysembryoplastic neuroepithelial collision tumor in the foramen of Monro: case report.
Journal of neurosurgery. PediatricsPapillary glioneuronal tumor (PGNT) exhibits a characteristic methylation profile and fusions involving PRKCA.
Acta neuropathologicaSeptal dysembryoplastic neuroepithelial tumor: a comprehensive clinical, imaging, histopathologic, and molecular analysis.
Neuro-oncologyOccurrence of high-grade glioma in Noonan syndrome: Report of two cases.
Pediatric blood & cancerA Novel Missense LIG4 Mutation in a Patient With a Phenotype Mimicking Behçet's Disease.
Journal of clinical immunologyEpilepsy Surgery for Children With Low-Grade Epilepsy-Associated Tumors: Factors Associated With Seizure Recurrence and Cognitive Function.
Pediatric neurology[BRAF V600E mutation and clinicopathologic characteristics in 250 cases of brain tumors associated with epilepsy].
Zhonghua bing li xue za zhi = Chinese journal of pathologyPreoperative evaluation and surgical management of infants and toddlers with drug-resistant epilepsy.
Neurosurgical focusFreiburg Neuropathology Case Conference : Tumor of the Mesiotemporal Lobe in a Child.
Clinical neuroradiologySurgical Treatment of Lesional Mesial Temporal Lobe Epilepsy.
Journal of epilepsy researchLethal disseminated dysembryoplastic neuroepithelial tumor following West Nile virus: Report of a very unusual combination.
Radiology case reportsDysembryoplastic neuroepithelial tumor-like pilocytic astrocytoma: A case report.
MedicineIntraoperative monitoring with visual evoked potentials for brain surgeries.
Journal of neurosurgeryAdapting a memory fMRI research protocol in clinical routine: Feasibility and results.
Epilepsy & behavior : E&BLesional Temporal Lobe Epilepsy: Beware the Deceitful "Panic Attack".
World neurosurgeryNeuroimaging of Dilated Perivascular Spaces: From Benign and Pathologic Causes to Mimics.
Journal of neuroimaging : official journal of the American Society of NeuroimagingSurgical treatment of thalamic tumors in children.
Journal of neurosurgery. PediatricsIntractable epilepsy due to a rosette-forming glioneuronal tumor with a dysembryoplastic neuroepithelial background.
Neuropathology : official journal of the Japanese Society of NeuropathologyAtypical presentations of dysembryoplastic neuroepithelial tumors.
EpilepsiaOccipital dysembryoplastic neuroepithelial tumor presenting as adult-onset temporal epilepsy.
Epilepsy & behavior case reports[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 sciencesSurgical 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 polskaRole of the temporal pole in temporal lobe epilepsy seizure networks: an intracranial electrode investigation.
Journal of neurosurgeryCerebellar dysembryoplastic neuroepithelial tumor: report of a case and review of the literature.
Journal of neurologyUtility of Stereoelectroencephalography in Children with Dysembryoplastic Neuroepithelial Tumor and Cortical Malformation.
The Neurodiagnostic journalLong-term recurrence of dysembryoplastic neuroepithelial tumor: Clinical case report.
Surgical neurology internationalSurgery for dysembryoplastic neuroepithelial tumors and gangliogliomas in eloquent areas. Functional results and seizure control.
Neuro-ChirurgiePediatric Basal Ganglia Region Tumors: Clinical and Radiologic Features Correlated with Histopathologic Findings.
World neurosurgeryNoonan syndrome, PTPN11 mutations, and brain tumors. A clinical report and review of the literature.
American journal of medical genetics. Part AA 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. PediatricsVolumetric growth analysis of an insular dysembryoplastic neuroepithelial tumor over a 10-year follow-up.
Surgical neurology internationalPIK3CA mutation in a mixed dysembryoplastic neuroepithelial tumor and rosette forming glioneuronal tumor, a case report and literature review.
Journal of the neurological sciencesFDOPA PET-CT of Nonenhancing Brain Tumors.
Clinical nuclear medicineFocal Epileptogenic Lesions in Adult Patients with Epilepsy and Generalized Epileptiform Discharges.
Journal of epilepsy researchPosterior cortex epilepsy surgery in childhood and adolescence: Predictors of long-term seizure outcome.
EpilepsiaEarly and late epilepsy surgery in focal epilepsies associated with long-term epilepsy-associated tumors.
Journal of neurosurgeryA rare case of dysembryoplastic neuroepithelial tumor combined with encephalocraniocutaneous lipomatosis and intractable seizures.
Korean journal of pediatricsSeizures caused by brain tumors in children.
SeizureA comprehensive analysis identifies BRAF hotspot mutations associated with gliomas with peculiar epithelial morphology.
Neuropathology : official journal of the Japanese Society of NeuropathologyDroplet digital PCR is a powerful technique to demonstrate frequent FGFR1 duplication in dysembryoplastic neuroepithelial tumors.
OncotargetAlterations in BRAF gene, and enhanced mTOR and MAPK signaling in dysembryoplastic neuroepithelial tumors (DNTs).
Epilepsy researchSubcortical DNET in a Patient With an Enzymatic Deficiency: A Rare Case and Review of the Literature.
Journal of pediatric hematology/oncologyGlioneuronal 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 NeurosurgeryRadiologic response to radiation therapy concurrent with temozolomide for progressive simple dysembryoplastic neuroepithelial tumor.
Acta neurochirurgicaRemitted epilepsy with dysembryoplastic neuroepithelial tumor involving the thalamus.
Pediatrics international : official journal of the Japan Pediatric SocietyPediatric epilepsy surgery: could age be a predictor of outcomes?
Journal of neurosurgery. PediatricsLong-term drug-resistant temporal lobe epilepsy associated with a mixed ganglioglioma and dysembryoplastic neuroepithelial tumor in an elderly patient.
Surgical neurology internationalDysembryoplastic neuroepithelial tumor: A rare brain tumor not to be misdiagnosed.
Asian journal of neurosurgeryAtypical Teratoid Rhabdoid Tumor Diagnosis after Partial Resection of Dysembryoplastic Neuroepithelial Tumor: Case Report and Review of the Literature.
Pediatric neurosurgeryBeneficial impact of high-field intraoperative magnetic resonance imaging on the efficacy of pediatric low-grade glioma surgery.
Neurosurgical focusMalignant Transformation of a Dysembryoplastic Neuroepithelial Tumor (DNET) Characterized by Genome-Wide Methylation Analysis.
Journal of neuropathology and experimental neurologyGermline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors.
Acta neuropathologicaDysembryoplastic Neuroepithelial Tumor of the Septum Pellucidum and the Supratentorial Midline: Histopathologic, Neuroradiologic, and Molecular Features of 7 Cases.
The American journal of surgical pathology[Epilepsy-associated tumors of the central nervous system: Epilepsy surgery and oncological aspects].
Der NervenarztMultinodular and vacuolating neuronal tumor affecting amygdala and hippocampus: A quasi-tumor?
Pathology internationalReview of seizure outcomes after surgical resection of dysembryoplastic neuroepithelial tumors.
Journal of neuro-oncologyReal-time magnetic resonance imaging-guided frameless stereotactic brain biopsy: technical note.
Journal of neurosurgeryDysembryoplastic Neuroepithelial Tumors.
Journal of pathology and translational medicineOccurrence of DNET and other brain tumors in Noonan syndrome warrants caution with growth hormone therapy.
American journal of medical genetics. Part AEpilepsy surgery related to pediatric brain tumors: Miami Children's Hospital experience.
Journal of neurosurgery. PediatricsResection of a dysembryoplastic neuroepithelial tumor in the precentral gyrus.
World journal of pediatrics : WJPImpact of epilepsy surgery on development of preschool children: identification of a cohort likely to benefit from early intervention.
Journal of neurosurgery. PediatricsCerebral sparganosis: case report and review of the European cases.
Acta neurochirurgica"Unusual brain stone": heavily calcified primary neoplasm with some features suggestive of angiocentric glioma.
Journal of neurosurgery[Clinical and histological characteristics of ictal onset zone in cases of intractable epilepsy associated with dysembryoplastic neuroepithelial tumor].
Brain and nerve = Shinkei kenkyu no shinpoSemiological and electroencephalographic features of epilepsy with amygdalar lesion.
Epilepsy researchAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Tumor neuroepitelial disembrioplásico.
É de uma associação que acompanha esta doença? Fale com a gente →
Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Tumor neuroepitelial disembrioplásico
Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.
Tire suas dúvidas
Perguntas, dicas e experiências compartilhadas aqui na página
Participe da discussão
Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.
Fazer loginDoenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Ainda não achamos doenças com sintomas parecidos o suficiente.
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.
- 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.
- Dysembryoplastic neuroepithelial tumor: Malignant transformation following radiation.
- Endoscopic Transorbital Approach for Select Recurrent and Newly Diagnosed Anteromedial Temporal Lobe Gliomas.
- A Case of Myxoid Glioneuronal Tumor in the Lateral Ventricle.
- PTPN11-related Noonan syndrome predisposes to multifocal low-grade CNS tumors harboring FGFR1 variants.
- Long-term radiological progression after resection of dysembryoplastic neuroepithelial tumors: patterns and prognostic factors.
- Low-grade Epilepsy-associated Tumors and Other Acquired Causes of Epilepsy: MR Imaging.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:251946(Orphanet)
- MONDO:0005505(MONDO)
- GARD:10640(GARD (NIH))
- Busca completa no PubMed(PubMed)
- 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
