Introdução
O que você precisa saber de cara
A Classificação da OMS de Tumores do Sistema Nervoso Central é um Livro Azul da Organização Mundial da Saúde que define, descreve e classifica tumores do sistema nervoso central (SNC).
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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
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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 — Neurocitoma extraventricular
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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
Pesquisa e ensaios clínicos
3 ensaios clínicos encontrados, 1 ativos.
Publicações mais relevantes
Extraventricular Neurocytoma in a Five-Month-Old Infant.
Incidental detection of extraventricular neurocytoma arising from VIII cranial nerve - Unanticipated postmortem finding.
Extraventricular neurocytoma (EVN) is one of the rare tumors of the central nervous system (CNS), which has been reported to arise from various sites within the CNS as well as at extracranial locations. Herein, we report a unique case of EVN arising from the VIII cranial nerve to occupy the cerebellopontine angle that was incidentally detected in an asymptomatic 50-year-old male during postmortem examination. Furthermore, this report gives an insight into various theories that have been postulated regarding the origin of EVN. However, a large number of EVN cases need to be studied to establish the cell of origin of EVN.
Extraventricular Neurocytoma With a Complex Presentation: The Role of Molecular Studies and Technology in Its Management.
Diagnosis and management of central nervous system tumors is challenging, especially when they have unusual presentation and require molecular analysis to confirm the pathology to offer the best treatment with the least risk of morbidity and mortality. This is a 34-year-old man who presented with a hemorrhagic lesion at the corpus callosum and intraventricular level that was initially classified as an ependymoma but was later redefined as a neurocytoma after molecular analysis. The patient had no significant medical history. The initial clinical picture was manifested with intense headache, nausea, and photophobia. Initial management consisted of placement of an external ventriculostomy for acute hydrocephalus. A biopsy of the lesion, aided by navigation, was performed. A ventriculoperitoneal shunt for persistent hydrocephalus was placed. After confirming the molecular diagnosis of neurocytoma, treatment with a gamma knife provided excellent results. The importance of this case centers around the fundamental role of molecular diagnosis in accurately classifying central nervous system (CNS) tumors, specifically in cases where histopathological analysis may be inconclusive, allowing for better directed management. Technology enables effective management of these complex lesions through minimally invasive treatment options like the gamma knife.
Neuroepithelial tumors of the central nervous system with EWSR1::PATZ1 fusion: a case report and literature review.
Neuroepithelial tumors (NEpT) harboring EWSR::PATZ1 fusions remain an enigma. Initially described in sarcomas, these tumors display remarkable histomorphological diversity and unpredictable clinical behavior based on histologic or molecular features, with no established management protocols. To date, this subgroup of neoplasms has not been acknowledged as a sui generis entity by the WHO classification system, and it is currently designated as 'NEC'/'NOS'. This retrospective case series describes two young adults (32-35 years old) without cancer predisposition or risk factors, diagnosed with EWSR1::PATZ1-fused NEpT. Case 1, a female with seizures, presented a heterogeneous left parietal lobe lesion (4.0 × 3.2 × 3.6 cm), classified as high-grade NEpT with MGMT promoter methylation, a calibrated score of 0.95 (≥ 0.9), and a co-occurring somatic MUTYH mutation. Case 2, a male with chronic headaches and mild right-sided paresthesia, had a left frontotemporal lesion (3.0 × 2.8 × 3.4 cm), initially diagnosed as an extraventricular neurocytoma but later reclassified as a NEpT with low-to-intermediate grade features, without MGMT methylation, and a calibrated score of 0.92. Case 1 received upfront resection, followed by Stupp protocol chemoradiation and temozolomide maintenance, resulting in 14 months of progression-free survival (PFS). Case 2 underwent subtotal resection and adjuvant radiotherapy after an 8-month recurrence, achieving 11 months of PFS to date. Both patients are asymptomatic, off corticosteroids, with the latest imaging revealing no disease progression. Our cases emphasize that EWSR1::PATZ1-fused NEpT displays a unique signature (ventricular localization, glioneuronal differentiation, and a distinct methylation cluster), supporting their inclusion in the WHO classification. Moreover, we present the first documented somatic co-mutation involving MUTYH. At present, despite the theoretical risk of temozolomide resistance due to PATZ1 overexpression, our results suggest that conventional glioma therapies remain the preferred approach.
Parasellar Extraventricular Neurocytoma: Magnetic Resonance and Angiographic Findings.
Publicações recentes
Incidental detection of extraventricular neurocytoma arising from VIII cranial nerve - Unanticipated postmortem finding.
🥉 Relato de casoExtraventricular Neurocytoma With a Complex Presentation: The Role of Molecular Studies and Technology in Its Management.
Neuroepithelial tumors of the central nervous system with EWSR1::PATZ1 fusion: a case report and literature review.
Extraventricular Neurocytoma in a Five-Month-Old Infant.
Congenital extraventricular neurocytoma with atypical features in a 3-day-old neonate.
📚 EuropePMC92 artigos no totalmostrando 58
Incidental detection of extraventricular neurocytoma arising from VIII cranial nerve - Unanticipated postmortem finding.
Indian journal of pathology & microbiologyExtraventricular Neurocytoma With a Complex Presentation: The Role of Molecular Studies and Technology in Its Management.
CureusNeuroepithelial tumors of the central nervous system with EWSR1::PATZ1 fusion: a case report and literature review.
Frontiers in oncologyExtraventricular Neurocytoma in a Five-Month-Old Infant.
Pediatric blood & cancerCongenital extraventricular neurocytoma with atypical features in a 3-day-old neonate.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryCase report: A rare case of neurocytoma of the Vth cranial nerve.
Frontiers in oncologyExtraventricular Neurocytoma of the Sellar Region Presenting With Syndrome of Inappropriate Antidiuresis.
JCEM case reportsTumor-Agnostic Genomic and Clinical Analysis of BRAF Fusions Identifies Actionable Targets.
Clinical cancer research : an official journal of the American Association for Cancer ResearchClinical rarity: simultaneous choroid plexus papilloma and extraventricular neurocytoma presenting as intraventricular hemorrhage in an adolescent.
Journal of surgical case reportsAnalysis of a pituitary tumour with histological features of central neurocytoma points towards the emergence of a new entity recognizable by a specific epigenetic signature.
Neuropathology and applied neurobiologySurvival outcomes of intracranial extraventricular neurocytomas: a systematic review and individual patient data meta-analysis.
Journal of neurosurgeryAwake craniotomy in an adolescent patient with an extraventricular neurocytoma.
BMJ case reportsIntraventricular central neurocytoma molecularly defined as extraventricular neurocytoma: a case representing the discrepancy between clinicopathological and molecular classifications.
Brain tumor pathologyParasellar Extraventricular Neurocytoma: Magnetic Resonance and Angiographic Findings.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiquesPediatric atypical extraventricular neurocytoma: Clinical features and survival outcomes.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryExtraventricular Neurocytoma of the Posterior Fossa: A Case Report Written by ChatGPT.
CureusFirst-time identification of a KIF5B-NTRK2 fusion in extraventricular neurocytoma.
Journal of neuropathology and experimental neurologyMR Finding of Extraventricular Neurocytoma.
Journal of the College of Physicians and Surgeons--Pakistan : JCPSPA Clinicopathological and Molecular Analysis of Sellar/Suprasellar Neurocytoma Mimicking Pituitary Adenoma.
Frontiers in endocrinologyExtraventricular neurocytoma at the sellar region: Report of 8 cases and literature review.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaExtraventricular Neurocytoma: Clinical Investigation of Heterogenous Prognosis.
Brain tumor research and treatmentExtraventricular neurocytoma.
Autopsy & case reportsMRI features of an atypical case of extraventricular neurocytoma: A case report.
MedicineA rare diagnosis of an extraventricular neurocytoma.
Surgical neurology internationalCan we use intraoperative high-frequency oscillations to guide tumor-related epilepsy surgery?
EpilepsiaHistological features in pediatric central nervous system tumors with FGFR alterations.
Folia neuropathologicaComprehensive analysis of diverse low-grade neuroepithelial tumors with FGFR1 alterations reveals a distinct molecular signature of rosette-forming glioneuronal tumor.
Acta neuropathologica communicationsCerebellar Neurocytoma with Excellent Response to Radiotherapy.
World neurosurgeryAtypical extraventricular neurocytoma: A case report.
Cellular and molecular biology (Noisy-le-Grand, France)Imaging of extraventricular neurocytoma: a systematic literature review.
La Radiologia medicaFGFR- gene family alterations in low-grade neuroepithelial tumors.
Acta neuropathologica communicationsExtraventricular neurocytoma with ganglioid differentiation of the sellar and parasellar regions in an elderly patient: A case report.
Surgical neurology internationalAtypical Extraventricular Neurocytoma: Imaging Findings of Disregarded Diagnosis.
World neurosurgeryThe epidemiology of central and extraventricular neurocytoma in the United States between 2006 and 2014.
Journal of neuro-oncologyMesial temporal extraventricular neurocytoma (mtEVN): A case report and literature review.
Epilepsy & behavior case reportsMalignant progression of an extraventricular neurocytoma arising from the VIIIth cranial nerve: A case report and literature review.
Neuropathology : official journal of the Japanese Society of NeuropathologyImaging characteristics of cerebral extraventricular neurocytoma with pathological correlation.
Journal of neuro-oncologyFGFR1:TACC1 fusion is a frequent event in molecularly defined extraventricular neurocytoma.
Acta neuropathologicaCongenital Extra-Ventricular (Ganglio)Neurocytoma of the Brain Stem: A Case Report.
Frontiers in pediatricsComplete resection of hypervascularised extraventricular neurocytoma after preoperative embolisation.
NeurocirugiaPatterns of care and survival outcomes in patients with an extraventricular neurocytoma: An individual patient data analysis of 201 cases.
Neurology IndiaIntramedullary central neurocytoma of the thoracic spinal cord: A case report and literature review.
Molecular and clinical oncologyExtraventricular Neurocytoma in the Left Frontal Lobe: A Case Report and Literature Review.
World neurosurgeryPediatric Basal Ganglia Region Tumors: Clinical and Radiologic Features Correlated with Histopathologic Findings.
World neurosurgeryExtraventricular neurocytoma: An uncommon tumor in a young boy. A review of literature.
Neurology IndiaA clinicopathologic study of extraventricular neurocytoma.
Journal of neuro-oncologyDensly calcified cystic extraventricular neurocytoma: Radiological-pathological correlation.
Neurology IndiaProgression of atypical extraventricular neurocytoma to anaplastic ganglioglioma.
Human pathologyCentral Versus Extraventricular Neurocytoma in Children: A Clinicopathologic Comparison and Review of the Literature.
Journal of pediatric hematology/oncologyExtraventricular neurocytoma of the sellar region: case report and literature review.
SpringerPlusExtraventricular neurocytoma in the left temporal lobe: A case report and review of the literature.
Oncology lettersHypothalamic Extraventricular Neurocytoma (EVN) in a Pediatric Patient: A Case of EVN Treated with Subtotal Removal Followed by Adjuvant Radiotherapy.
Brain tumor research and treatmentAtypical extraventricular neurocytoma:A report of two cases.
Journal of cancer research and therapeuticsNeurocytoma arising from a mature ovary teratoma: a case report.
Diagnostic pathologyExtraventricular neurocytoma mimicking bone tumor in thoracic spinal column.
The spine journal : official journal of the North American Spine SocietyAtypical extraventricular neurocytoma in a 3-year-old girl: case report with radiological-pathological correlation.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryExtraventricular neurocytoma treated with 177Lu DOTATATE PRRT induction and maintenance therapies.
Clinical nuclear medicine[Atypical extraventricular neurocytoma: report of a case].
Zhonghua bing li xue za zhi = Chinese journal of pathologyAssociações
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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.
- Extraventricular Neurocytoma in a Five-Month-Old Infant.
- Incidental detection of extraventricular neurocytoma arising from VIII cranial nerve - Unanticipated postmortem finding.
- Extraventricular Neurocytoma With a Complex Presentation: The Role of Molecular Studies and Technology in Its Management.
- Neuroepithelial tumors of the central nervous system with EWSR1::PATZ1 fusion: a case report and literature review.
- Parasellar Extraventricular Neurocytoma: Magnetic Resonance and Angiographic Findings.The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques· 2024· PMID 37577989mais citado
- Congenital extraventricular neurocytoma with atypical features in a 3-day-old neonate.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:251927(Orphanet)
- MONDO:0016727(MONDO)
- GARD:20727(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55786392(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
