É um tumor de crescimento lento, Grau II da OMS (Organização Mundial da Saúde), que afeta crianças e adultos jovens. Geralmente está localizado dentro dos ventrículos cerebrais (espaços cheios de líquido no cérebro). É o tipo mais comum de tumor ependimário. Frequentemente causa sintomas ao bloquear as vias do líquido cefalorraquidiano (o líquido que envolve o cérebro e a medula espinhal). As características principais observadas ao microscópio incluem pseudorrosetas perivasculares e rosetas ependimárias.
Introdução
O que você precisa saber de cara
É um tumor de crescimento lento, Grau II da OMS (Organização Mundial da Saúde), que afeta crianças e adultos jovens. Geralmente está localizado dentro dos ventrículos cerebrais (espaços cheios de líquido no cérebro). É o tipo mais comum de tumor ependimário. Frequentemente causa sintomas ao bloquear as vias do líquido cefalorraquidiano (o líquido que envolve o cérebro e a medula espinhal). As características principais observadas ao microscópio incluem pseudorrosetas perivasculares e rosetas ependimárias.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 11 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 16 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
1 gene identificado com associação a esta condição. Padrão de herança: Not applicable.
Medicamentos aprovados (FDA)
1 medicamento encontrado nos registros da FDA americana.
Variantes genéticas (ClinVar)
8 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 9 variantes classificadas pelo ClinVar.
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 — Ependimona
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
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Outros ensaios clínicos
313 ensaios clínicos encontrados, 34 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 2.349
Multidimensional profiling of heterogeneity in supratentorial ependymomas.
Supratentorial ependymomas are aggressive childhood brain cancers that retain features of neurodevelopmental cell types1 and segregate into molecularly and clinically distinct subgroups2,3, suggesting different developmental roots. The developmental signatures, as well as microenvironmental factors, underlying aberrant cellular transformation and behaviour across each supratentorial ependymoma subgroup are unclear. Here we integrated single-cell and spatial transcriptomics, as well as in vitro and in vivo live-cell imaging, to define supratentorial ependymoma cell states, spatial organization and dynamic behaviour within the neural microenvironment. We find that individual tumour subgroups have two distinct progenitor-like cell states-neuroepithelial-like and embryonic-like-that are reminiscent of early human brain development and diverge in the extent of their neuronal or ependymal differentiation. We further identify several modes of spatial organization of these tumours, including a high-order architecture that is influenced by mesenchymal and hypoxia signatures, and local neighbourhood structures. Finally, we identify a role for brain-resident cells in shifting supratentorial ependymoma cellular heterogeneity towards neuronal-like cells that co-opt immature neuronal morphology and migratory mechanisms, and a subset of neuroepithelial-like cells that are both proliferative and highly migratory. Collectively, these findings provide a multidimensional framework to integrate transcriptional and phenotypic characterization of tumour heterogeneity in supratentorial ependymoma and its potential clinical implications.
Ventriculoperitoneal shunt-associated cystic lesion with perilesional cerebral edema 45 years after placement for posterior fossa tumor: A case report.
Ventriculoperitoneal (VP) shunts are widely used to treat hydrocephalus in children with posterior fossa tumors. While many patients gain shunt independence after tumor control, indwelling shunts are often not removed. Shunt-related intracranial cysts are extremely rare and typically develop along the intracranial catheter tract within days to several years after shunt insertion; to date, the longest reported interval has been 19 years. Here, we report a case a cystic lesion with marked perilesional edema developing 45 years after childhood VP shunt placement for tumor-related hydrocephalus. We report the case of a 58-year-old man who presented with acute severe left-sided hemiparesis. At the age of 13 years, he underwent resection of a posterior fossa ependymoma, VP shunt placement, and whole-brain radiotherapy. Neuroimaging revealed a large right frontal low-density lesion with vasogenic edema and a cystic cavity surrounding the intracranial shunt catheter. Retrospective review of magnetic resonance imaging performed 3 months earlier showed a smaller preexisting cyst at the same site. The shunt catheter was removed without resistance, and the membranous material adhering to the distal tip caused partial intraluminal obstruction. Postoperatively, the hemiparesis improved markedly; follow-up imaging demonstrated resolution of the edema and reduction in the cyst size. As hydrocephalus did not recur, shunt reinsertion was not required. This case represents the longest latency reported in the literature for VP shunt-related intracranial cyst formation, occurring 45 years after shunt implantation, exceeding the previously reported maximum interval of 19 years. The acute symptoms closely resemble stroke or infection, underscoring the diagnostic challenge. Clinicians should be vigilant of such delayed complications even decades after shunt placement.
Modeling pediatric brain tumors with human stem cells.
With recent advances in stem cell technology, there has been an expansion of human stem and progenitor cell models of pediatric brain tumors, including use of human pluripotent and embryonic stem cells both in organoid cultures and following xenotransplantation in mice. In this review, we discuss the current approaches to modelling pediatric brain cancers using stem cells. While brain tumors describe a broad set of disease entities, we focus on glioma, medulloblastoma and ependymoma, as these are not only the most common malignant brain tumor types but also have the most stem cell models currently available. We examine human stem cell-based modeling approaches and discuss the biological questions that are being addressed using these state-of-the-art tools. Specifically, we focus on the unique advantage of using these cells to understand the functional consequences of gene mutations and their downstream growth-promoting pathways within the cell in a human context. These approaches are needed to ascertain the key players that are functionally relevant in the initiation and propagation of these tumors at the gene and protein level and to identify new drug targets. Moreover, human stem cell-based modeling approaches may complement studies in genetically engineered mouse models to address fundamental questions in tumor biology, particularly the early stages of tumorigenesis.
A systems approach identifies MERTK as a therapeutic vulnerability in ZFTA-RELA-driven ependymomas.
Ependymomas (EPN) are rare central nervous system tumors that account for approximately 10% of intracranial tumors in children and 4% in adults. Despite their clinical and molecular heterogeneity, spanning supratentorial, posterior fossa, and spinal subtypes, treatment remains limited to surgery and radiotherapy, with chemotherapy offering minimal benefit. Here, we performed transcriptomic analysis of 370 human ependymoma samples and identified two distinct molecular subgroups: EPN-E1 and EPN-E2. The EPN-E1 cluster is enriched for supratentorial tumors harboring ZFTA-RELA fusions (ZFTA-RELAfus), which occur in over 70% of cases and are associated with poor prognosis. To identify targeted therapies for this aggressive subtype, we validated a ZFTA-RELAfus mouse model that recapitulates the human EPN-E1 transcriptome and used it for target discovery. Through Kinome Regularization, a machine learning-driven polypharmacology approach, we identified MERTK as a critical regulator of tumor cell viability. Genetic depletion or pharmacologic inhibition of Mertk reduced cell growth ex vivo, and treatment with a clinical-grade MERTK inhibitor significantly suppressed tumor proliferation in vivo. Both human EPN-E1 tumors and ZFTA-RELAfus mouse tumors exhibited elevated expression of MERTK and its ligand GAS6, and MERTK inhibition led to suppression of pro-survival signaling pathways including MEK/ERK (Mitogen-Activated Protein Kinase Kinase/Extracellular Signal-Regulated Kinase) and PI3K/AKT (Phosphoinositide 3-Kinase/Protein Kinase B). Notably, over 80% of genes upregulated in ZFTA-RELAfus tumors were downregulated following MERTK inhibition, indicating a strong dependency on this pathway for tumor maintenance. These findings define a signaling vulnerability in ZFTA-RELA-driven ependymomas and support the clinical development of MERTK-targeted therapies for patients with the high-risk EPN-E1 subtype.
Thoracic tanycytic ependymoma with anaplastic progression after subtotal resection and repeated radiotherapy: A 17-year follow-up case report.
Tanycytic ependymomas (TEs) are rare spindle-cell variants of spinal ependymomas. They are classified as central nervous system World Health Organization grade II, and they usually behave like ordinary spinal ependymomas especially following gross total resection (GTR). A 43-year-old male presented with a progressive paraparesis and sensory level at T4 (pain/ temperature loss). The magnetic resonance imaging showed an intradural intramedullary tumor extending from Th2 to Th5. Only a subtotal resection was performed as a central portion was tightly adherent to the spinal cord itself. Histology revealed a predominantly grade II TE; the main component showed a low proliferative index, whereas the anaplastic focus had a markedly elevated index. Despite craniospinal tomotherapy (36 Gy), tumor recurred 6 years 7 months later. Local tomotherapy was repeated 3 times (41.4, 40, and 30 Gy), but each treatment produced only transient shrinkage followed by regrowth at shorter intervals. Further irradiation was contraindicated due to cumulative dose constraints. Seventeen years after the first surgery, rapid enlargement over 3 months caused paraplegia and bladder-bowel dysfunction. Reoperation achieved GTR, and at this point the pathology showed a diffuse anaplastic ependymoma; the MR 6 months later showed no tumor recurrence. Although TE is usually indolent, it can harbor an anaplastic subclone and progress to malignancy after subtotal resection. Long-term surveillance and early reoperation when regrowth is first observed should be considered and is likely due to the development of anaplastic features.
Publicações recentes
Intracranial ependymoma grade II with lipomatous metaplasia: a case report and literature review.
Clinical Characteristics, Management, and Outcomes of Intramedullary Spinal Cord Ependymomas: A Systematic Review and Meta-Analysis.
Clinical and Magnetic Resonance Features of Spinal Myxopapillary Ependymoma.
CNS tumor type prevalence according to age group: an analysis of 21,000 cases confirmed by methylation profiling, with a focus on adolescents and young adults.
Ependymoma group-specific blood-brain barrier differences uncovered by a multi-omics approach.
📚 EuropePMC2.727 artigos no totalmostrando 198
Reverse brain herniation following CSF diversion in a 2-year-old child-case report and review of literature.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryDiscovery of genetic susceptibility variants in pediatric and adult ependymoma.
Neuro-oncology advancesDevelopment and internal validation of a Cox-based nomogram for predicting cancer-specific survival in central nervous system ependymoma: a population-based study.
Journal of the Formosan Medical Association = Taiwan yi zhiIs maternal smoking during pregnancy associated with childhood brain tumors? A systematic literature review.
Toxicology lettersCase Report: Molecular diagnostics and clinical courses of two adult spinal pilocytic astrocytoma long-term survivors with GTF2I::BRAF fusion.
Frontiers in oncologyNivolumab-Associated Autoimmune Encephalopathy in a Child With Multiply Relapsed Anaplastic Ependymoma.
Pediatric blood & cancerAI-driven label-free Raman spectromics for intraoperative spinal tumor assessment.
NPJ digital medicineA Parental Request for an Unproven Treatment for a Rare Pediatric Cancer: Sound Reasons for Not Going Off-Label.
The Hastings Center reportThe Case for Prescribing Metformin Off-Label for a Child in Remission from Ependymoma.
The Hastings Center reportPhase I/II Study of the CDK2/9 Inhibitor Fadraciclib in Combination with Chemotherapy in Children with Advanced Malignancies: Arm K of the AcSé-ESMART Trial.
Targeted oncologyThoracic intramedullary spinal cord astrocytoma associated with syringomyelia: a case report of gross total resection.
International journal of surgery case reportsNeuroendocrine Tumor of the Cauda Equina: A Report of a Rare Case With Histopathological and Immunohistochemical Correlation.
CureusExploiting Metabolic Dependencies for Therapeutic Targeting of Brain Cancers.
Cancer lettersMetabolic vulnerability in fusion-driven ependymoma.
Nature reviews. CancerMultidimensional profiling of heterogeneity in supratentorial ependymomas.
NatureBack to square one: Treatment for ependymoma in the post-ACNS0831 era.
Neuro-oncologyAdnexal Endometrioid Carcinomas With Sex Cord-Like Morphology are Frequently PAX8-Negative, SOX17-Positive, and Enriched for CTNNB1 Alterations.
The American journal of surgical pathologyEffect of radiation on patients with grade 2 intramedullary spine ependymoma.
European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research SocietyTargeted and systemic therapies for recurrent adult ependymomas: real-world outcomes from a single institution and concise literature review.
Frontiers in oncologyAnterior Fibular Graft and Posterior Fusion for Proximal Cervical Kyphosis Following Resection of Posterior Fossa Ependymoma: A Case Report.
JBJS case connectorVentriculoperitoneal shunt-associated cystic lesion with perilesional cerebral edema 45 years after placement for posterior fossa tumor: A case report.
Surgical neurology internationalPrimary Pediatric Brain Tumors in a Tertiary Referral Center in Iran: A 13-Year Retrospective Analysis.
Archives of Iranian medicineModeling pediatric brain tumors with human stem cells.
Frontiers in cellular neuroscienceRestricting Itaconate Suppresses ZFTA-RELA Ependymoma Growth.
Cancer discoveryProton Radiotherapy Outcomes in Pediatric Ependymoma: a Long-term meta-analysis (PROPEL).
Critical reviews in oncology/hematologyNuances in the management of cerebellopontine angle ependymomas: illustrative case.
Journal of neurosurgery. Case lessonsThe Impact of Hearing Loss on Cognitive Outcomes among Children Treated with Radiation Therapy for Ependymoma.
Neuro-oncology"Endocrine Disorders After Treatment for Pediatric Brain Tumors: Long-Term Outcomes and Risk Factors".
The Journal of clinical endocrinology and metabolismA rare constellation of bilateral progressive visual and auditory loss in neurofibromatosis type 2: a multimodal diagnostic approach.
Annals of medicine and surgery (2012)Memantine to preserve memory and neurocognition following craniospinal irradiation (MEMENTO): a phase 3 randomized controlled trial.
BMC cancerA systems approach identifies MERTK as a therapeutic vulnerability in ZFTA-RELA-driven ependymomas.
Proceedings of the National Academy of Sciences of the United States of AmericaThoracic tanycytic ependymoma with anaplastic progression after subtotal resection and repeated radiotherapy: A 17-year follow-up case report.
Surgical neurology internationalAccurate classification of ependymomas and medulloblastomas using Raman spectroscopy and pilot transcriptomic profiling.
Spectrochimica acta. Part A, Molecular and biomolecular spectroscopyClinical predictors of overall survival in pediatric patients with intramedullary spinal tumors: an analysis of the SEER database.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryTwo pediatric supratentorial ependymal tumors with novel PLAG1 fusions.
Acta neuropathologica communicationsChildhood brain tumors instruct cranial hematopoiesis and immunotolerance.
Nature geneticsEn-bloc resection achieves higher GTR rates with similar neurological outcomes in grade-2 intramedullary spinal ependymomas: single-centre cohort study.
British journal of neurosurgeryUnraveling the miRNA-EMT-stemness interplay in fusion-positive supratentorial ependymomas: Identifying therapeutic vulnerabilities.
Biochemical and biophysical research communicationsA brief history of ependymoma.
Neuro-oncologySurgical treatment of myxopapillary ependymoma: an institutional case series of 56 patients.
Journal of neurosurgery. SpineAn opportunity for fertility preservation in neurosurgical spinal ependymoma patients: a single center review.
Frontiers in reproductive healthEZHIP in Pediatric Brain Tumors: From Epigenetic Mimicry to Therapeutic Vulnerabilities.
International journal of molecular sciencesThoracic metastasis of a pediatric posterior fossa group A ependymoma: a rare case and literature review.
World journal of pediatric surgeryRadiological analysis correlated with neurological outcomes following resection of myxopapillary ependymoma: patient series.
Journal of neurosurgery. Case lessonsRecurrent pediatric posterior fossa anaplastic ependymoma managed with multiple resections: illustrative case.
Journal of neurosurgery. Case lessonsPosterior fossa ependymoma harboring H3K27M mutation: A rare case report with clinical follow-up and diagnostic challenges.
Clinical neuropathologyIntraoperative Ultrasound Imaging Features Guiding Surgical Strategy in Spinal Cord Ependymoma Resection.
Neurosurgery practiceA highly abundant circular RNA from the RMST locus plays a role in posterior fossa ependymoma pathogenesis.
Brain pathology (Zurich, Switzerland)Spinal deformity following surgery for paediatric intramedullary spinal cord tumours.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryClinical and Prognostic Implications of Isocitrate Dehydrogenase Mutation in Gliomas Within a Latin American Public Oncology System: Insights From a Retrospective Panamanian Cohort.
CureusExtraspinal sacrococcygeal anaplastic ependymoma: A case report.
International journal of surgery case reportsBrain Mapping for Surgical Resection of Left Dominant Supramarginal and Angular Gyrus Grade II Ependymoma: 2-Dimensional Operative Video.
Operative neurosurgery (Hagerstown, Md.)Multimodal-based analysis of single-cell ATAC-seq data enables highly accurate delineation of clinically relevant tumor cell subpopulations.
Genome medicineImpact of molecular subtypes on postoperative hydrocephalus following resection of posterior fossa medulloblastoma and ependymoma in children: A systematic review and meta-analysis.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryRE: Comments on cIMPACT-NOW Update 11.
Brain pathology (Zurich, Switzerland)A rare case of supratentorial intraparenchymal ependymoma in a 13 years old girl: Diagnostic and therapeutic challenges.
Pakistan journal of medical sciencesA Systematic Review of Evidence on the Clinical Effectiveness of Surveillance Imaging in Children With Medulloblastoma and Ependymoma.
Pediatric blood & cancerCerebello-Pontine Angle Tumors in Children: An Update on Challenging Neoplasms.
Diagnostics (Basel, Switzerland)Proton-Based Re-Irradiation of Locally Recurrent Pediatric Brain Tumors in Close Proximity to the Brainstem.
International journal of particle therapyMethylation profiling in neuropathological tumors diagnosis: a comprehensive review.
Frontiers in oncologyHistopathological Assessment of Cellular Heterogeneity in Pediatric Ependymomas.
Diagnostics (Basel, Switzerland)Clinical Features and Prognostic Indicators for Brainstem Ependymomas : A Population-Based Retrospective Surveillance, Epidemiology, and End Results Database Analysis.
Journal of Korean Neurosurgical SocietyPhase 1 study of mebendazole therapy for refractory/progressive or recurrent pediatric brain tumors.
Neuro-oncology practiceLongitudinal neuropathologic and molecular characterization of a retroperitoneal ependymoma.
Journal of neuropathology and experimental neurologyPhase 3 randomized trial of post-irradiation chemotherapy in patients with newly diagnosed ependymoma, a report from the Children's Oncology Group.
Neuro-oncologyPosterior Fossa Group A Ependymoma in Trisomy 21.
Neuropathology : official journal of the Japanese Society of NeuropathologySurgery in NF2-Schwannomatosis.
Familial cancerAlgorithm-based assessment of T-cell dysfunction and exclusion to forecast ICB sensitivity in pediatric brain ependymoma.
Journal of neuro-oncologyA rare case of cauda equina paraganglioma histologically simulating ependymoma.
Surgical neurology internationalOutcomes of initially chosen non-operative management for spinal ependymoma.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaBenchmarking nazionale della sopravvivenza per stadio alla diagnosi dei tumori infantili in Italia (BENCHISTA-ITA): protocollo di studio.
Epidemiologia e prevenzioneMultifocal Spinal Intradural Myxopapillary Ependymoma.
Indian journal of orthopaedicsIntraspinal collision tumor comprising a schwannoma and ependymocytoma: a rare case study and literature review.
Folia neuropathologica[Fertility Preservation for Pediatric Brain Tumor Patients: Current Status and Challenges].
No shinkei geka. Neurological surgery[Current Status and Prospects of Clinical Trials for Ependymoma].
No shinkei geka. Neurological surgeryClinical and Magnetic Resonance Findings for the Diagnosis of Recurrent Tumors versus Radiation-Induced Secondary Tumors of Malignant Pediatric Brain Tumors of the Posterior Fossa.
World neurosurgeryAscending Transtentorial Herniation: A Rare Complication of Posterior Cranial Fossa Tumors and Review of Literature.
Asian journal of neurosurgeryUtility of Immunohistochemistry in Subtyping Posterior Fossa Group A Ependymoma: A Retrospective Study.
Asian journal of neurosurgeryNon-invasive prediction of Ki-67 and p53 biomarkers in spinal ependymoma via deep learning: using multimodal magnetic resonance imaging and clinical data.
Biomarker researchDevelopment and validation of a multi-modal MRI-based deep learning framework for differentiation of intraspinal tumors (ISMF-Net).
EClinicalMedicinePrecise diagnosis of pediatric posterior cranial fossa neoplasms based on 2.5D MRI deep learning.
Frontiers in oncologySyncope in a Patient With an Extensive Thoracolumbosacral Myxopapillary Ependymoma.
CureusLate Morbidity and Mortality in Survivors of Childhood Ependymoma: A Report from the Childhood Cancer Survivor Study (CCSS).
CancersPatterns of care in primary brain tumour Reirradiation: A survey by the ESTRO CNS focus Group.
Radiotherapy and oncology : journal of the European Society for Therapeutic Radiology and OncologyCAR T cells targeting the glycoprotein GD2 show potent antitumor efficacy in high-risk ependymoma models.
The Journal of clinical investigationThe clinical spectrum of paediatric NF2- related schwannomatosis.
Familial cancerDNA Methylation Profiling Classifies and Reveals Origin of Gynecologic Central Nervous System-Like Tumors.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncPan-cancer 3D genomic analysis revealed extremely long Polycomb loops as the biomarker for sensitivity to Polycomb inhibition.
bioRxiv : the preprint server for biologyA rare case of supratentorial ependymosarcoma harboring ZFTA::RELA fusion.
Brain tumor pathologySingle-nucleus transcriptomics of spinal ependymoma subtypes recognizes intratumoral heterogeneity.
Neuro-oncologyMatrix viscoelasticity regulates dendritic cell migration and immune priming.
bioRxiv : the preprint server for biologyTumor size and treatment factors as correlates of 10-year mortality in grade III spinal ependymomas: a nationwide analysis.
Scientific reportsFatal ornithine transcarbamylase deficiency presentation postresection of fourth ventricle ependymoma: illustrative case.
Journal of neurosurgery. Case lessonsMaternal Neurosurgical Management of Central Nervous System Tumors During Pregnancy: A Case Series with Long-Term Offspring Neurodevelopmental Follow-Up.
World neurosurgeryEZHIP boosts neuronal-like synaptic gene programs and depresses polyamine metabolism.
Acta neuropathologica communicationsThree decades of radiotherapy advancements for pediatric ependymoma.
Advances in cancer researchAdult ependymoma: results from the Dutch Brain Tumour Registry on primary treatment and survival.
Journal of neuro-oncologyThe current status of immune checkpoint inhibitors in pediatric CNS tumors: a systematic review with a representative case of CMMRD-associated glioma.
Journal of neuro-oncologyMulti-omics analysis delineates molecular signatures of spinal ependymal tumor.
Cellular oncology (Dordrecht, Netherlands)Integrated transcriptomic landscape of medulloblastoma and ependymoma reveals novel tumor subtype-specific biology.
Neuro-oncologyTandem Detethering: A Novel One-Stage Approach Combining Cervicothoracic Cord Release Followed by Filum Terminale Sectioning.
Journal of clinical medicineGenetic Markers and Mutations in Primary Spinal Cord Tumors and Their Impact on Clinical Management.
Brain sciencesIntegrated Single-Nucleus and Spatial Transcriptomics Elucidate Heterogeneity and Hypoxia-Driven Organization of Supratentorial Ependymoma.
Cancer researchThree-year insights from a proton therapy centre: Evolving strategies in ependymoma treatment planning.
Technical innovations & patient support in radiation oncologyClinical profile and outcomes of pediatric ependymoma: a 10-year retrospective analysis from a resource-limited setting.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryPresenting symptoms and outcomes of cervical and thoracic ependymomas compared to conus and filum ependymomas.
Journal of neurosurgery. SpineIntraoperative neurophysiological monitoring in surgery for intramedullary spinal cord lesions - workflow, setup and outcomes.
Acta neurochirurgicaA predictive model for differentiating PFA and PFB subtypes of posterior fossa ependymoma using multi-sequence MRI radiomics: a two-center study.
NeuroradiologyEpendymoma: Advances in Systems Treatment Strategies.
Current neurology and neuroscience reportsHigh-grade astroblastoma initially misdiagnosed as papillary meningioma: a case report.
The Pan African medical journalProfiling extracellular vesicles from cerebrospinal fluid for classification of intradural spinal tumors.
Scientific reportsSpinal myxopapillary ependymoma with anaplastic features: A case report and review of the literature.
Surgical neurology internationalLaparoscopic anchoring of peritoneal catheters using surgical mesh - a promising approach for recurrent extrinsic ventriculoperitoneal shunt migration.
British journal of neurosurgeryThe effect of oncolytic virotherapy on pediatric brain tumor- a systematic review.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryThe Role of D-Wave Monitoring in Motor-Evoked Potential Loss During Intramedullary Spinal Cord Tumors Resection.
Neurospine[Intramedullary ependymoma of spinal canal:a case report].
Zhongguo gu shang = China journal of orthopaedics and traumatologyInvestigation of DNA Damage Response Genes Validates the Role of DNA Repair in Pediatric Cancer Risk and Identifies SMARCAL1 as a Novel Osteosarcoma Predisposition Gene.
Journal of clinical oncology : official journal of the American Society of Clinical OncologyEUS Guided FNA Cell Block Cytology and Intraoperative Squash Cytology in the Diagnostic Approach of Unfamiliar Malignant Neoplastic Disorders.
Acta medica (Hradec Kralove)Surgical Outcomes and Prognostic Factors for Resectability of Intramedullary Spinal Cord Tumors Incorporating Intraoperative Neurophysiological Monitoring In Vietnam.
Medical archives (Sarajevo, Bosnia and Herzegovina)Surgical technique for executing an en bloc resection of a multilevel cervical intramedullary ependymoma: A 2D operative video.
Surgical neurology internationalA rare case of posterior fossa collision tumor involving low-grade astrocytoma and meningioma.
Journal of surgical case reportsDeveloping an advanced risk stratification model for pediatric intracranial ependymoma based on the prospective trial E-HIT2000 and subsequent registries.
Neuro-oncologyAqueductal posterior fossa type B ependymoma in Li-Fraumeni syndrome.
Journal of neuropathology and experimental neurologyIntramedullary Schwannoma of the Conus Medullaris Presenting With Progressive Paraparesis: A Rare Case From a Resource-Limited Setting.
Clinical case reportsDelay in the diagnosis of pediatric central nervous system tumors: data from a single tertiary care center.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryImaging Differentiation of Supratentorial Ependymoma and High-Grade Glioma in Children Using VASARI Features.
AJNR. American journal of neuroradiologyConditionally Reprogrammed Cells as Preclinical Model for Rare Cancers.
CancersHuman Polyomavirus BK Genome Analysis in BKPyV Induced Rodent Cell Lines.
MicrobiologyOpenCentral nervous system tumors with BCOR internal tandem duplications: a systematic review of clinical, radiological, and pathological features in 69 cases.
Journal of pathology and translational medicineCurrent Approach and Predictors of Clinical Outcomes in Adults With Spinal Ependymomas.
CureuscIMPACT-NOW update 11: Proposal on adaptation of diagnostic criteria for IDH- and H3-wildtype diffuse high-grade gliomas and for posterior fossa ependymal tumors.
Brain pathology (Zurich, Switzerland)Impact of the COVID-19 pandemic on diagnosis and management of new brain tumours in children and young people (aged < 16 years old) in the UK.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgerySurvival Patterns and Mortality Causes in Patients with Invasive Ependymoma: A Retrospective Cohort Analysis from 2000 to 2019.
Medical sciences (Basel, Switzerland)Microscopic Minimal Invasive Resection of Spinal Tumor with Tubular Retractor System: Case Studies of 70 Patients with Literature Review.
Asian journal of neurosurgeryMicroscopic Resection of Intracranial Lesions with Tubular Retractor of Plastic Syringe: A Single-Center Experience of 157 Cases.
Asian journal of neurosurgeryMulticenter basket trial for central nervous system tumors identifies activity of the CDK4/6 inhibitor abemaciclib in recurrent meningioma.
Neuro-oncologyRadiomics in pediatric brain tumors: from images to insights.
Discover oncologyNationwide Brain Tumor Registry-based Study of Adult Intracranial Ependymoma in Japan.
Neurologia medico-chirurgicaTargeted Therapy with Dalpiciclib in a Pediatric Patient with Anaplastic Ependymoma: A Case Study and Literature Review.
Recent patents on anti-cancer drug discoveryMicrosurgical removal of a C1-C5 laterally located intramedullary subependymoma through a postero-lateral sulcus approach: case report, surgical video and critical review of diagnostic and surgical challenges.
European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research SocietyCentral Nervous System Tumors in Adolescents and Young Adults.
Current neurology and neuroscience reportsHow is rosette formation in brain tumours linked with cerebrospinal fluid spread?
Brain tumor pathologyIntracranial low-grade tumors with ALK rearrangement: diverse tumor types with shared molecular alterations.
Human pathologySpinal Myxopapillary Ependymoma Presenting With Isolated Intracranial Hypertension: A Case Report.
CureusAn anaplastic ependymoma with chondroid metaplasia after stereotactic radiosurgery: illustrative case.
Journal of neurosurgery. Case lessonsEffect of the TGF-b signaling pathway on spinal cord ependymoma: A study based on bioinformatics analysis and clinical trials.
Folia neuropathologicaPLAG1 fusions define a third subtype of CNS embryonal tumor with PLAG family gene alteration.
Acta neuropathologicaBuilding a dynamic web calculator for individualized conditional survival estimation in brainstem ependymoma.
Scientific reportsMetastatic Supratentorial Ependymoma: A Case Presentation and Systematic Review of the Literature.
Neuropathology : official journal of the Japanese Society of NeuropathologyCNS Tumor with BCOR/BCORL1 Fusion: A Rare Tumor Entity.
International journal of molecular sciencesCorrelation Analysis of Imaging and Pathological Features of Ependymomas.
International journal of general medicineUncharted Territory: The First Case of Cardiac Myxoma in a Patient With NF2.
JACC. Case reportsDifferent nuances between patient- and surgeon-reported outcomes in intramedullary ependymoma surgery.
Journal of neurosurgery. SpineFreiburg Neuropathology Case Conference: Transition from Chronic Dizziness to Acute Headache and Nausea in a 30-Year-Old.
Clinical neuroradiologySyringobulbia and Syringomyelia Associated with Intramedullary Ependymoma.
World neurosurgeryPractice Pearls: Spinal Myxopapillary Ependymoma Masquerading as Charcot-Marie Tooth (CMT) Disease.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiquesExtraventricular Neurocytoma With a Complex Presentation: The Role of Molecular Studies and Technology in Its Management.
CureusThe role of MRI patterns in predicting neurologic deficits in spinal ependymomas.
Journal of neuro-oncologyAcute paraplegia following epidural anesthesia for cesarean delivery due to asymptomatic spinal cord ependymoma hemorrhage: A case report and literature review.
MedicineLong-level Intramedullary Spinal Cord Tumor: A Case Series.
JNMA; journal of the Nepal Medical AssociationHistopathological Types, Clinical Presentation, Imaging Studies, Treatment Strategies, and Prognosis of Posterior Pituitary Tumors: An Updated Review.
Journal of clinical medicineSurvival and Functional Outcomes Following Surgical Resection of Intramedullary Spinal Cord Tumors: A Series of 253 Patients over 22 Years.
CancersAre dorsal column deficits inevitable in intramedullary spine tumor resection?
Journal of neurosurgery. SpinePrimary pediatric brain tumors in Africa: a systematic review and comparative meta-analysis.
Journal of neurosurgery. PediatricsAdjuvant Etoposide for Very High-risk PFA Ependymoma: A Case Report.
Journal of pediatric hematology/oncologyB7-H3 CAR T Cells Are Effective against Ependymomas but Limited by Tumor Size and Immune Response.
Clinical cancer research : an official journal of the American Association for Cancer ResearchMyxopapillary Ependymoma: A Case Report.
CureusPulsed reduced dose rate radiotherapy: a narrative review.
Chinese clinical oncologyPseudoprogression in Pediatric Spinal Pilocytic Astrocytoma and Myxopapillary Ependymoma after Proton Therapy: A Case Series.
Pediatric blood & cancerOptimizing outcomes in intracranial ependymoma: a contemporary review.
Frontiers in oncologyMachine learning-driven national analysis for predicting adverse outcomes in intramedullary spinal cord tumor surgery.
European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research SocietyRadiosurgery for pediatric central nervous system lesions - initial report and insights from a multicenter registry.
Radiotherapy and oncology : journal of the European Society for Therapeutic Radiology and OncologyRecurrent parietal lobe supratentorial ependymoma, ZFTA fusion-positive, CNS WHO grade 3, with new dural and calvarial reactive changes in a child: illustrative case.
Journal of neurosurgery. Case lessonsHow long should survivors of pediatric medulloblastoma and ependymoma be screened for recurrence? A retrospective cohort study.
International journal of cancerIntramedullary schwannoma mimicking ependymoma: illustrative case.
Journal of neurosurgery. Case lessonsComprehensive Genome Analysis Facilitates the Diagnosis of Ependymoma With ZFTA::NCOA2 Fusion.
Pediatric blood & cancerDiagnostic Accuracy of MRI and CT Scan Features in Differentiation of Pediatric Ependymoma from Medulloblastoma.
Iranian journal of child neurologyAssessment of Clinical and Neurological Alterations Before Radiation Therapy in Children With Malignant Brain Tumours.
Clinical oncology (Royal College of Radiologists (Great Britain))Extra-neural metastases of recurrent myxopapillary ependymoma: A patient case and literature review.
Surgical neurology internationalNon-Neoplastic Lesions of the Ependyma: A Neuropathological Overview.
Neuropathology : official journal of the Japanese Society of NeuropathologyDynamic Magnetic Resonance Imaging Changes in Spinal Ependymomas and Their Impact on Surgical Planning.
Neurologia medico-chirurgicaAn adult patient with initial presentation of myxopapillary ependymoma at the cerebellopontine angle: illustrative case.
Journal of neurosurgery. Case lessonsHow many cases of spinal intramedullary ependymoma surgery are required to achieve stability? - Analysis using X-bar charts.
Spinal cordSupra-sellar clear cell ependymoma in a 2-year-old female: A case report.
International journal of surgery case reportsRadiosurgery for central nervous system ependymomas: a systematic review and meta-analysis.
Journal of neuro-oncologyMultiple system biology approaches reveals the role of the hsa-miR-21 in increasing risk of neurological disorders in patients suffering from hypertension.
Journal of human hypertensionEstablishment of Orthotopic Patient-derived Xenograft Models for Brain Tumors using a Stereotaxic Device.
Journal of visualized experiments : JoVEThe immunopeptidomic landscape of ependymomas provides actionable antigens for T-cell-based immunotherapy.
Neuro-oncology advancesFreiburg Neuropathology Case Conference: Gait Ataxia, Segmental Hypoesthesia, and Combined Incontinence in a 57-Years-Old Patient.
Clinical neuroradiologyMRI-based deep learning with clinical and imaging features to differentiate medulloblastoma and ependymoma in children.
Frontiers in molecular biosciencesDosimetric comparison of intensity-modulated proton therapy and proton arc therapy for pediatric ependymoma.
Acta oncologica (Stockholm, Sweden)Expanding clinicopathologic knowledge in high-grade glioma with pleomorphic and pseudopapillary features (HPAP): a report of two cases.
Acta neuropathologica communicationsThree cases of pituicytoma with a review of the literature and insight into a rare variant of ependymal pituicytoma.
Clinical neuropathologyClinical Characteristics, Histopathological Profile, and Postoperative Outcomes of Tumoral Radiculomedullary Compressions.
CureusDissecting the immune landscape in pediatric high-grade glioma reveals cell state changes under therapeutic pressure.
Cell reports. MedicineResection of a Cerebellopontine Angle Ependymoma Using a Far Lateral Approach: 2-Dimensional Operative Video.
Operative neurosurgery (Hagerstown, Md.)Magnetic resonance imaging (MRI) radiomics in paediatric neuro-oncology: A systematic review of clinical applications, feature interpretation, and biological insights in the characterisation and management of childhood brain tumours.
Digital healthAutomatic smart brain tumor classification and prediction system using deep learning.
Scientific reportsAssociações
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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.
- Multidimensional profiling of heterogeneity in supratentorial ependymomas.
- Ventriculoperitoneal shunt-associated cystic lesion with perilesional cerebral edema 45 years after placement for posterior fossa tumor: A case report.
- Modeling pediatric brain tumors with human stem cells.
- A systems approach identifies MERTK as a therapeutic vulnerability in ZFTA-RELA-driven ependymomas.Proceedings of the National Academy of Sciences of the United States of America· 2026· PMID 41665993mais citado
- Thoracic tanycytic ependymoma with anaplastic progression after subtotal resection and repeated radiotherapy: A 17-year follow-up case report.
- Intracranial ependymoma grade II with lipomatous metaplasia: a case report and literature review.
- Clinical Characteristics, Management, and Outcomes of Intramedullary Spinal Cord Ependymomas: A Systematic Review and Meta-Analysis.
- Clinical and Magnetic Resonance Features of Spinal Myxopapillary Ependymoma.
- CNS tumor type prevalence according to age group: an analysis of 21,000 cases confirmed by methylation profiling, with a focus on adolescents and young adults.
- Ependymoma group-specific blood-brain barrier differences uncovered by a multi-omics approach.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:251636(Orphanet)
- MONDO:0016698(MONDO)
- GARD:6353(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q1346753(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
