Introdução
O que você precisa saber de cara
A Classificação da OMS dos Tumores do Sistema Nervoso Central é um Livro Azul da Organização Mundial da Saúde que define, descreve e classifica os tumores do sistema nervoso central (SNC).
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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
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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 glioneuronal formador de rosetas do quarto ventrículo
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
Pesquisa e ensaios clínicos
2 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.
Multinodular and vacuolating neuronal tumor: molecular genetics and DNA methylation analysis of 12 cases.
Multinodular and vacuolating neuronal tumor (MVNT) was recognized as a distinct neuronal tumor entity in the revision of the 2021 World Health Organization (WHO) Classification of Tumors of the CNS. In this study, we retrospectively analyzed 12 surgical cases, two of which exhibited ganglioglioma (GG)-like components. The cohort consisted of eight male and four female patients, with a median age of 31 years (age range: 18-53 years). Seizures were the most common clinical presentation, followed by headache and dizziness. Three patients were incidentally identified during physical examination. Ten tumors were located in the cerebral hemisphere, and the remaining two were found in the cerebellum and thalamus, respectively. Histopathological examination revealed clusters of neuroepithelial cells with large amphophilic vacuolated cytoplasm and eccentrically placed round nuclei containing prominent nucleoli. Immunohistochemically, these vacuolated cells were positive for OLIG2, MAP2, SYN and SOX10, and negative for GFAP and NEUN. DNA sequencing analysis identified no mutations in IDH1, IDH2, BRAF V600E, TERT promoter, or EGFR genes. Among the cohort, three cases harbored FGFR2 mutations, and FGFR2::INA gene fusion was detected in both MVNT and GG-like components. Two cases carried BRAF mutation, and one case exhibited MAP2K1 mutation. A novel BCAN::NTRK1 (exon 12-exon 9) gene fusion was identified in one case. DNA methylation profiling of eight cases revealed that none matched with a known CNS tumor type. Six cases formed a separate methylation cluster, suggesting a potential novel molecular subtype, while the remaining two cases exhibited transcriptional similarities to supratentorial pilocytic astrocytoma and rosette-forming glioneuronal tumor (RGNT), respectively. Postoperatively, all patients remained seizure free with no evidence of tumor progression. Only one patient died 16 months after surgery due to an unrelated traffic accident. © 2026 The Pathological Society of Great Britain and Ireland.
Stereotactic radiosurgery for residual rosette-forming glioneuronal tumor: a case report and literature review.
Rosette-forming glioneuronal tumors (RGNT) are rare and novel World Health Organization grade I neoplasms that typically arise in the fourth ventricle and progress slowly. Surgical resection is the standard treatment. However, owing to their adherence to critical structures, complete resection is often not possible. The role of stereotactic radiosurgery (SRS) in the management of RGNT remains inconclusive. We present a case of tissue-confirmed RGNT successfully treated with SRS. A 24-year-old woman presented with diplopia and dysequilibrium and was subsequently diagnosed with a fourth ventricular tumor. Subtotal resection was performed at another hospital, and a tissue-based diagnosis of RGNT was made. After a multidisciplinary discussion and following the patient's willingness, single-session SRS was prescribed at a marginal dose of 12 Gy. During the subsequent 66-month follow-up period, radiologic regression of the tumor with corresponding resolution of symptoms was noted. She remained neurologically intact at her last official visit. The treatment paradigm for residual RGNT remains elusive due to its scarcity and varied presentation. We have presented our preliminary experience with a residual RGNT that was managed with SRS, attaining long-term freedom from tumor progression. SRS may be a safe, effective, and durable treatment modality for patients with RGNT.
Glioneuronal and Neuronal Tumors in Adults: WHO 2021 Imaging and Molecular Updates.
Glioneuronal and neuronal tumors are defined histologically by the presence of mixed glial and neuronal elements in the case of glioneuronal tumors, and by neuronal elements in the case of neuronal tumors. In 2021, the World Health Organization (WHO) published the latest edition of central nervous system tumor classifications and added three new glioneuronal tumors, as well as further delineated histological, immunohistochemical, and molecular markers associated with the diagnosis of these tumors. This review aims to highlight the key updated features of glioneuronal and neuronal tumors in adults based on the 5th edition of the WHO classification of central nervous system tumors by clinical and epidemiological presentation, imaging appearance, and pathology.ABBREVIATIONS: DGONC = diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters; DLGNT = diffuse leptomeningeal glioneuronal tumor; DNET = dysembryoplastic neuroepithelial tumor; LEAT = long term epilepsy associated tumor; MGNT = myxoid glioneuronal tumor; MVNT = multinodular and vacuolating neuronal tumor; PGNT = papillary glioneuronal tumor; RGNT = rosette-forming glioneuronal tumor; WHO = World Health Organization.
Gamma knife radiosurgery for a rare Rosette-forming glioneuronal tumor in the brainstem region: A case report and literature review.
Rosette-forming glioneuronal tumor (RGNT) is a rare primary nervous system tumor, with limited treatment guidelines due to its rarity, especially in the brainstem. This report presents a unique case of brainstem RGNT treated with gamma knife radiosurgery (GKRS). A 35-year-old woman sought medical attention after sudden syncope and rapid decline in consciousness. Magnetic resonance imaging revealed a mass in the pineal region, extending to the brainstem and thalamus. Due to the critical location, only partial resection of the pineal tumor was possible, leaving most of the residual tumor in the vital brainstem area, requiring urgent intervention to control its growth and prevent sudden complications. Postoperative histopathological results confirmed a diagnosis of RGNT. The patient underwent 25 Gy/5 fractions of GKRS using the frameless Gamma Knife ICON™ (Elekta) device, as confirmed by cone-beam computed tomography scans for precise dose distribution and patient alignment. GKRS was performed successfully and safely. The tumor significantly shrank 3 months post-GKRS, and the patient experienced symptom relief without any adverse effects. GKRS is considered an effective modality for RGNT in high-risk brainstem areas, minimizing risks while controlling tumor growth and alleviating symptoms. In addition, the frameless Gamma Knife ICON™ device enhanced patient comfort and treatment precision. GKRS offers a noninvasive alternative for similar RGNT cases.
Publicações recentes
Multinodular and vacuolating neuronal tumor: molecular genetics and DNA methylation analysis of 12 cases.
Stereotactic radiosurgery for residual rosette-forming glioneuronal tumor: a case report and literature review.
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.
Gamma knife radiosurgery for a rare Rosette-forming glioneuronal tumor in the brainstem region: A case report and literature review.
Rosette-Forming Glioneuronal Tumor Mimicking Foramen Monro Colloid Cyst: Case Presentation and Systematic Literature Review.
📚 EuropePMC98 artigos no totalmostrando 85
Multinodular and vacuolating neuronal tumor: molecular genetics and DNA methylation analysis of 12 cases.
The Journal of pathologyStereotactic radiosurgery for residual rosette-forming glioneuronal tumor: a case report and literature review.
Acta neurochirurgicaMultifocal 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 pathologyGlioneuronal and Neuronal Tumors in Adults: WHO 2021 Imaging and Molecular Updates.
AJNR. American journal of neuroradiologyGamma knife radiosurgery for a rare Rosette-forming glioneuronal tumor in the brainstem region: A case report and literature review.
MedicineRosette-Forming Glioneuronal Tumor Mimicking Foramen Monro Colloid Cyst: Case Presentation and Systematic Literature Review.
World neurosurgeryPosterior Fossa Stereotactic Biopsy with Leksell Vantage Frame-Case Series and Review of Literature.
Journal of clinical medicineA glioneuronal tumor with neurocytic rosettes harboring FGFR1 internal tandem duplication - A report of a unique case.
Neuropathology : official journal of the Japanese Society of NeuropathologyOccipital Interhemispheric Retrocallosal Transtentorial Approach to a Tectal Tumor in an 8-Year-Old Girl: Operative Video.
World neurosurgeryGlioneuronal tumor in an autosomal-dominant polycystic kidney disease patient: a case report and literature review.
Annals of medicine and surgery (2012)Cranio-Orbital Pretemporal Approach for Microsurgical Resection of Hypothalamic Rosette Forming Glioneuronal Tumor with Reversal of Preoperative Blindness: 2-Dimensional Operative Video.
World neurosurgeryLi-Fraumeni Syndrome With Six Primary Tumors-Case Report.
Case reports in oncological medicineClinicopathological analysis of rosette-forming glioneuronal tumors.
Diagnostic pathologyA Rare Lesion in the Anterior Region of the Third Ventricle: Rosette-Forming Glioneuronal Tumor.
CureusH3 K27M mutation in rosette-forming glioneuronal tumors: a potential diagnostic pitfall.
Virchows Archiv : an international journal of pathologyDiffusely invasive supratentorial rosette-forming glioneuronal tumor: illustrative case.
Journal of neurosurgery. Case lessonsStaged Gamma Knife radiosurgery for a rosette-forming glioneuronal tumor of the fourth ventricle: a case report.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryEndoscopic resection of thalamic lesions via supracerebellar infratentorial approach: a case series and technical note.
Neurosurgical reviewTreatment of Pineal Region Rosette-Forming Glioneuronal Tumors (RGNT).
CancersRedefining germline predisposition in children with molecularly characterized ependymoma: a population-based 20-year cohort.
Acta neuropathologica communicationsImmune Microenvironment and Lineage Tracing Help to Decipher Rosette-Forming Glioneuronal Tumors: A Multi-Omics Analysis.
Journal of neuropathology and experimental neurologyMolecular analyses of rosette-forming glioneuronal tumor of the midbrain tegmentum: A report of two cases and a review of the FGFR1 status in unusual tumor locations.
Surgical neurology internationalUncommon Glioneuronal Tumors: A Radiologic and Pathologic Synopsis.
AJNR. American journal of neuroradiologyFrequent FGFR1 hotspot alterations in driver-unknown low-grade glioma and mixed neuronal-glial tumors.
Journal of cancer research and clinical oncologyTectal Rosette-Forming Glioneuronal Tumor - A Case Report Focusing on a Possible Role for Radiotherapy in Inoperable Tumors.
Neurology IndiaSpinal rosette-forming glioneuronal tumor: First case in a young child.
Radiology case reportsRosette-Forming Glioneuronal Tumor in the Pineal Region: A Series of 6 Cases and Literature Review.
Journal of neuropathology and experimental neurologyRosette-forming glioneuronal tumor of the fourth ventricle; A case report and review of the literature.
Clinical case reportsRole of intra-operative squash cytology in rosette-forming glioneuronal tumor of the fourth ventricle: a case report.
The International journal of neuroscienceRosette-Forming Glioneuronal Tumor of the Fourth Ventricle: A Case of Relapse Treated with Proton Beam Therapy.
Diagnostics (Basel, Switzerland)The rosette-forming glioneuronal tumor mimicked cerebral cysticercosis: a case report.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyA 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 pathologyDissemination Patterns and Short-Term Management of Multifocal Rosette-Forming Glioneuronal Tumors.
World neurosurgeryHistological features in pediatric central nervous system tumors with FGFR alterations.
Folia neuropathologicaRosette-forming glioneuronal tumor: an illustrative case and a systematic review.
Neuro-oncology advancesComprehensive analysis of diverse low-grade neuroepithelial tumors with FGFR1 alterations reveals a distinct molecular signature of rosette-forming glioneuronal tumor.
Acta neuropathologica communicationsRosette-forming glioneuronal tumor: A case report.
Revue neurologiqueSupratentorial intraventricular rosette-forming glioneuronal tumors - Case report and review of treatment paradigms.
Surgical neurology internationalA 26-Year-Old Woman With Diplopia and Nystagmus.
Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology SocietySurgical treatment of a rare rosette-forming glioneuronal tumor in the pineal region.
Surgical neurology internationalPediatric rosette-forming glioneuronal tumor of the septum pellucidum.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgerySpinal rosette-forming glioneuronal tumor: A case report.
MedicineClinicopathologic Features of Diencephalic Neuronal and Glioneuronal Tumors.
Journal of neuropathology and experimental neurologyRosette-forming Glioneuronal Tumor in the Optic Pathway of a Child.
Journal of pediatric hematology/oncologyA Multifocal Glioneuronal Tumor with RGNT-Like Morphology Occupying the Supratentorial Ventricular System and Infiltrating the Brain Parenchyma.
World neurosurgery[Rosette-forming glioneuronal tumor in vermis: report of a case].
Zhonghua bing li xue za zhi = Chinese journal of pathologyDisseminated Rosette-Forming Glioneuronal Tumor with Spinal Drop Metastasis, a Uniquely Aggressive Presentation of Rare Tumor.
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 NeuropathologyGlioneuronal Tumor With Features of Ganglioglioma and Neurocytoma Arising in the Fourth Ventricle: A Report of 2 Unusual Cases and a Review of Infratentorial Gangliogliomas.
Journal of neuropathology and experimental neurologyRosette-forming glioneuronal tumor: an update.
Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of MexicoCytological features of rosette-forming glioneuronal tumor of the fourth ventricle.
Diagnostic cytopathologyRosette-forming glioneuronal tumors share a distinct DNA methylation profile and mutations in FGFR1, with recurrent co-mutation of PIK3CA and NF1.
Acta neuropathologicaAn epilepsy-associated glioneuronal tumor with mixed morphology harboring FGFR1 mutation.
Pathology internationalMalignant Transformation of a Rosette-Forming Glioneuronal Tumor with IDH1 Mutation: A Case Report and Literature Review.
World neurosurgery: XMalignant Transformation of a Rosette-Forming Glioneuronal Tumor to Glioblastoma.
World neurosurgeryA case of a rosette-forming glioneuronal tumor arising from the pons with disappearance of contrast enhancement.
Radiology case reportsRosette-Forming Glioneuronal Tumor in Opticochiasmatic Region-Novel Entity in New Location.
World neurosurgeryMesial temporal lobe rosette-forming glioneuronal tumor : an unusual location for a rare tumor.
Acta neurologica BelgicaMRI of intracranial intraventricular lesions.
Clinical imagingRosette-Forming Glioneuronal Tumor of Spinal Cord.
World neurosurgeryMagnetic resonance imaging findings of mixed neuronal-glial tumors with pathologic correlation: a review.
Acta neurologica BelgicaRosette-forming glioneuronal tumor of the fourth ventricle. Two cases report and literature review.
NeurocirugiaHistopathological, molecular, clinical and radiological characterization of rosette-forming glioneuronal tumor in the central nervous system.
OncotargetIntractable epilepsy due to a rosette-forming glioneuronal tumor with a dysembryoplastic neuroepithelial background.
Neuropathology : official journal of the Japanese Society of NeuropathologyRosette-forming Glioneuronal Tumor: A Rare Posterior Fossa Tumor in an Adolescent.
Journal of pediatric neurosciencesRosette-forming glioneuronal tumor of the fourth ventricle.
Neurology IndiaRosette-Forming Glioneuronal Tumor Arising from the Spinal Cord.
World neurosurgeryRosette-Forming Glioneuronal Tumor of the Fourth Ventricle in Children: Case Report and Literature Review.
World neurosurgeryDiagnosis and treatment for pure aqueductal tumor.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaRetrospective Analysis of Molecular and Immunohistochemical Characterization of 381 Primary Brain Tumors.
Journal of neuropathology and experimental neurologyPIK3CA mutation in a mixed dysembryoplastic neuroepithelial tumor and rosette forming glioneuronal tumor, a case report and literature review.
Journal of the neurological sciencesCase Report: A Rosette-forming Glioneuronal Tumor in the Tectal Plate in a Patient with Neurofibromatosis Type I.
CureusRosette-Forming Glioneuronal Tumor Originating From the Spinal Cord: Report of 2 Cases and Literature Review.
World neurosurgeryComprehensive genetic characterization of rosette-forming glioneuronal tumors: independent component analysis by tissue microdissection.
Brain pathology (Zurich, Switzerland)Integrated tumor and germline whole-exome sequencing identifies mutations in MAPK and PI3K pathway genes in an adolescent with rosette-forming glioneuronal tumor of the fourth ventricle.
Cold Spring Harbor molecular case studiesRosette-forming glioneuronal tumor of the thalamus.
Clinical neuropathologyThalamic rosette-forming a glioneuronal tumor in an elderly patient: Case report and literature review.
Neuro-ChirurgieRosette-forming glioneuronal tumor outside the fourth ventricle: a case-based update.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryMolecular Profiling of a Rare Rosette-Forming Glioneuronal Tumor Arising in the Spinal Cord.
PloS oneUncommon pediatric tumors of the posterior fossa: pathologic and molecular features.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgerySLC44A1-PRKCA fusion in papillary and rosette-forming glioneuronal tumors.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaRosette-forming glioneuronal tumor originating in the hypothalamus.
Brain tumor pathologyRosette forming glioneuronal tumor of the fourth ventricle in squash cytology smear.
Journal of cytologyRosette-forming Glioneuronal Tumor: Rare Case Presented with Spontaneous Disappearance of Contrast Enhancement.
NMC case report journalRare pathologies in the posterior third ventricular region in children: case series and review.
Pediatric neurosurgeryAssociações
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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.
- 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.
- Multinodular and vacuolating neuronal tumor: molecular genetics and DNA methylation analysis of 12 cases.
- Stereotactic radiosurgery for residual rosette-forming glioneuronal tumor: a case report and literature review.
- Glioneuronal and Neuronal Tumors in Adults: WHO 2021 Imaging and Molecular Updates.
- Gamma knife radiosurgery for a rare Rosette-forming glioneuronal tumor in the brainstem region: A case report and literature review.
- Rosette-Forming Glioneuronal Tumor Mimicking Foramen Monro Colloid Cyst: Case Presentation and Systematic Literature Review.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:251975(Orphanet)
- MONDO:0016736(MONDO)
- GARD:13593(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q9282951(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
