Tumor grau II da OMS composto por uma mistura notável de dois tipos distintos de células neoplásicas que se assemelham morfologicamente às células tumorais do oligodendroglioma e do astrocitoma difuso. (QUEM)
Introdução
O que você precisa saber de cara
Tumor grau II da OMS composto por uma mistura notável de dois tipos distintos de células neoplásicas que se assemelham morfologicamente às células tumorais do oligodendroglioma e do astrocitoma difuso. (QUEM)
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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
Genes associados
1 gene identificado com associação a esta condição.
Plays a role in intermediary metabolism and energy production (PubMed:19228619, PubMed:22416140). It may tightly associate or interact with the pyruvate dehydrogenase complex (PubMed:19228619, PubMed:22416140)
Mitochondrion
D-2-hydroxyglutaric aciduria 2
A neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features. Both a mild and a severe phenotype exist. The severe phenotype is homogeneous and is characterized by early infantile-onset epileptic encephalopathy and cardiomyopathy. The mild phenotype has a more variable clinical presentation. Diagnosis is based on the presence of an excess of D-2-hydroxyglutaric acid in the urine.
Variantes genéticas (ClinVar)
82 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
4 vias biológicas associadas aos genes desta condição.
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 — Oligoastrocitoma
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.
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2 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
91 ensaios clínicos encontrados, 6 ativos.
Publicações mais relevantes
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.
Risk factors for postoperative malignant progression of lower-grade gliomas: a systematic review and meta-analysis.
The primary aim was to evaluate the risk factors for postoperative malignant progression of lower-grade glioma (LGG) in patients. PubMed, EMBASE, Web of Science and Cochrane Library were searched from database inception to August 2024. Quantitative and original studies reporting risk factors for postoperative malignant progression of LGGs were included. 17 observational studies with 3810 glioma patients met the inclusion criteria. Factors including advanced age (HR 1.011, p = 0.042), contrast enhancement (HR 1.540, p = 0.001), rapid expanding speed (HR 4.525, p < 0.001), location in insular lobe (HR 1.514, p = 0.020), eloquence involved (HR 2.413, p < 0.001) and corpus callosum involved (HR 1.695, p = 0.002) were identified as risk factors of postoperative malignant progression of LGGs. High Karnofsky Performance Status (KPS) score (HR 0.955, p = 0.001), oligodendroglioma (HR 0.603, p < 0.001), oligoastrocytoma (HR 0.693, p = 0.016), isocitrate dehydrogenase (IDH) mutation (HR 0.406, p = 0.004), 1p19q codeletion (HR 0.534, p < 0.001), O6-methylguanine-DNA methyltransferase promoter (MGMTP) methylation (HR 0.539, p = 0.007), resection operation (HR 0.277, p < 0.001) and high extent of resection (EOR) (HR 0.972, p = 0.038) were identified as factors that decreased the risk of postoperative malignant progression of LGGs. This review identified multiple factors associated with the risk of postoperative malignant progression of LGGs, with moderate to high certainty of evidence supporting several key risk and protective factors. Surgeons should be aware of these factors and consider implementing more active treatment and surveillance measures for high-risk patients to improve prognosis.
Investigation of mismatch repair protein expression in glioma.
Mismatch repair (MMR) proteins are essential for maintaining genomic stability, and their dysfunction contributes to tumorigenesis in several malignancies. However, their role in glioma remains insufficiently defined. This study evaluated MMR protein expression in Tunisian glioma patients and examined its clinicopathological and prognostic significance. Ninety-five glioma samples were retrospectively analyzed. MMR protein expression was assessed by immunohistochemistry on formalin-fixed, paraffin-embedded tissues. Survival outcomes were evaluated using Kaplan-Meier analysis with Log-Rank testing. MMR expression was retained in 65 tumors (68.4 %) and lost in 30 (31.6 %). Loss of MLH1/PMS2 was observed in 16 tumors, MSH2/MSH6 loss in 12, and complete loss of all MMR proteins in two tumors. The distribution of MMR deficiency varied by histological subtype. Among pilocytic astrocytomas, 7 cases exhibited MMR deficiency, predominantly MLH1/PMS2 (n = 5). Two astrocytomas IDH mutant showed either MLH1/PMS2 or MSH2/MSH6 loss. Altered MMR profiles were also identified in oligodendroglioma IDH-mutant, 1p/19q co-deleted (n = 3), oligoastrocytoma NOS (n = 1), and anaplastic oligodendroglioma NOS (n = 1). In glioblastoma, IDH-wildtype, 14 cases showed heterodimer loss (MLH1/PMS2 or MSH2/MSH6), and two tumors demonstrated complete loss of all MMR proteins. Survival analysis revealed a significant prognostic effect exclusively in glioblastomas IDH-wildtype, where MMR deficiency was associated with worse survival (Log-rank test, p < 0.0001). MMR deficiency is relatively frequent in gliomas and carries prognostic significance, particularly in glioblastoma IDH-wildtype. Incorporating MMR status into molecular profiling may enhance risk stratification and inform therapeutic decision-making in glioma management.
Oligodendrogliomas: findings after classifying the same cohort using pre- and post-World Health Organization (WHO) 2021 criteria.
The 2021 World Health Organization (WHO) classification includes the presence of isocitrate dehydrogenase (IDH) mutation and 1p/19q codeletion for oligodendrogliomas. The objective of this study was to evaluate the impact of the introduction of this classification in a cohort of oligodendrogliomas. A total of 182 cases with an initial diagnosis of oligodendroglioma by histological criteria were identified, including Grades 2 and 3 and oligoastrocytoma (initial cohort). Subsequently, IDH mutation and 1p/19q codeletion were determined and were present in a total of 91 cases (reclassified cohort). The clinical evolution of both cohorts was analyzed. The mean age was 45 years (14-75), 65% were Grade 2 and 22% were oligoastrocytomas. Complete resection was performed in 47% and biopsy in 7%. After surgery, 50% received radiotherapy, 30% chemotherapy and 36% did not receive adjuvant therapy. In the reclassified cohort, there were no statistically significant differences between Grade 2 and Grade 3 oligodendrogliomas, the median overall survival (OS) in Grade 2 was 13.3 years [95% confidence interval (CI) 8.2-18.4] and 12 years in Grade 3 (95% CI 5.6-18.3). However, in the initial cohort, significant differences were found according to tumour grade. Even in cases without adjuvant treatment, the median OS was 12 years. Compared with this data, the median OS for the cohort that did not meet IDH mutation and 1p/19q codeletion criteria was 7.52 years (95% CI 4.67-10.38). Molecular classification allows a more accurate selection of oligodendrogliomas and implies cases with a better prognosis, regardless of the grade and treatment received. These data should be taken into account in clinical practice and clinical trials.
A dual-genotype IDH-mutant infiltrating glioma, a real oligoastrocytoma in cerebral hemisphere.
Since the 5th edition of CNS WHO classification. the categorization of oligoastrocytoma has been discontinued. It is now understood that the majority of tumors previously identified as oligoastrocytomas can be reclassified into either astrocytomas or oligodendrogliomas based on molecular characteristics. In this report, we present a rare case of true oligoastrocytoma characterized by the coexistence of two distinct cell types within a single tumor mass, as evidenced in imaging findings and histological examination. The left frontal infiltrating glioma displayed calcification, and histological analysis revealed two morphologically distinct regions corresponding to oligodendroglioma and astrocytoma. Immunohistochemical and molecular pathology analyses, including IDH1, ATRX, TP53 mutations, H3K27me3 status, Tert promoter mutations, and 1p/19q co-deletion, are consistent with oligodendroglioma and astrocytoma, respectively. Post-surgery, the patient opted against radiotherapy and chemotherapy and showed no signs of recurrence at a 4-month follow-up, but was subsequently lost to follow-up. This case prompts questions about the prognosis and potential grading criteria for true oligoastrocytoma. It underscores the need for further case studies to potentially re-establish it as a distinct tumor type in future CNS WHO classifications.
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Risk factors for postoperative malignant progression of lower-grade gliomas: a systematic review and meta-analysis.
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Oligodendrogliomas: findings after classifying the same cohort using pre- and post-World Health Organization (WHO) 2021 criteria.
📚 EuropePMC103 artigos no totalmostrando 183
Endoscopic Transorbital Approach for Select Recurrent and Newly Diagnosed Anteromedial Temporal Lobe Gliomas.
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American journal of clinical oncologyThe usefulness of 18F-fluorocholine PET/CT in the detection of recurrence of central nervous system primary neoplasms.
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Journal of oncology practiceNeurosurgery concepts: Key perspectives on intrathecal fluorescein for detecting intraoperative cerebrospinal fluid leak during endoscopic endonasal surgery, spinal intraarterial chemotherapy, oligoastrocytoma classification by in situ molecular genetics, and prenatal myelomeningocele closure and the need for cerebrospinal fluid shunt placement.
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Journal of neuro-oncologyMolecular and histologic characteristics of pseudoprogression in diffuse gliomas.
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The Lancet. OncologyHealth-related quality of life in patients with high-risk low-grade glioma (EORTC 22033-26033): a randomised, open-label, phase 3 intergroup study.
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Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaA case of medulloblastoma in adult patient affected by anaplastic oligoastrocytoma.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyDevelopment of a transplantable glioma tumour model from genetically engineered mice: MRI/MRS/MRSI characterisation.
Journal of neuro-oncologyImpact of histopathological transformation and overall survival in patients with progressive anaplastic glioma.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaIDH1 Mutation in Gliomas in Mosul City - Iraq.
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Journal of neurosurgeryAnaplastic astrocytoma and non-1p/19q co-deleted anaplastic oligoastrocytoma: long-term survival, employment, and performance status of survivors.
Neuro-oncology practicePre-operative Embolization of Intracranial and Extracranial Tumors: A Review of 37 Cases.
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Veterinary pathology[Glioneuronal tumor with neuropil-like island: report of four cases and review of literature].
Zhonghua bing li xue za zhi = Chinese journal of pathologyPrimary Spinal Oligoastrocytoma.
The Journal of craniofacial surgeryRadiation plus Procarbazine, CCNU, and Vincristine in Low-Grade Glioma.
The New England journal of medicine18F-fluoroethyl-L-tyrosine positron emission tomography for the differential diagnosis of tumefactive multiple sclerosis versus glioma: A case report.
Oncology lettersOverview of Transgenic Glioblastoma and Oligoastrocytoma CNS Models and Their Utility in Drug Discovery.
Current protocols in pharmacologyLow-grade and anaplastic oligodendroglioma.
Handbook of clinical neurologyDetection of ATRX and IDH1-R132H immunohistochemistry in the progression of 211 paired gliomas.
OncotargetA rare case of oligoastrocytoma with atypical symptoms initially diagnosed as multiple sclerosis: A case report.
Molecular and clinical oncologyPrimary spinal cord anaplastic oligoastrocytoma presenting with paraplegia.
The spine journal : official journal of the North American Spine SocietyPresence of neural progenitors in spontaneous canine gliomas: A histopathological and immunohistochemical study of 20 cases.
Veterinary journal (London, England : 1997)Clinicopathological factors predictive of postoperative seizures in patients with gliomas.
SeizureEpidermal growth factor receptor (EGFR) gene amplification in high-grade gliomas: Western Indian tertiary cancer center experience.
Neurology IndiaPrognostic significance of histomolecular subgroups of adult anaplastic (WHO Grade III) gliomas: applying the 'integrated' diagnosis approach.
Journal of clinical pathologyThe effect of pregnancy on survival in a low-grade glioma cohort.
Journal of neurosurgeryA case of osteoclast-like giant cell-rich epithelioid glioblastoma with BRAF V600E mutation.
Brain tumor pathologyPrimary spinal oligoastrocytoma mimicking longitudinally extensive transverse myelitis.
Multiple sclerosis and related disordersOligodendrogliomas: a short history of clinical developments.
CNS oncologyProcarbazine, lomustine and vincristine or temozolomide: which is the better regimen?
CNS oncologyThe role of surgery in the management of patients with diffuse low grade glioma: A systematic review and evidence-based clinical practice guideline.
Journal of neuro-oncologyManagement of patients with recurrence of diffuse low grade glioma: A systematic review and evidence-based clinical practice guideline.
Journal of neuro-oncologyThe role of initial chemotherapy for the treatment of adults with diffuse low grade glioma : A systematic review and evidence-based clinical practice guideline.
Journal of neuro-oncologyGenetics and imaging of oligodendroglial tumors.
CNS oncologyThe role of surgery in grade II/III oligodendroglial tumors.
CNS oncologyDoes morphological assessment have a role in classifying oligoastrocytoma as 'oligodendroglial' versus 'astrocytic'?
HistopathologyCorrelation of diffusion tensor and dynamic susceptibility contrast MRI with DNA ploidy and cell cycle analysis of gliomas.
Clinical neurology and neurosurgerySurvival of adults with primary malignant brain tumours in Europe; Results of the EUROCARE-5 study.
European journal of cancer (Oxford, England : 1990)PC or PCV, That Is the Question: Primary Anaplastic Oligodendroglial Tumors Treated with Procarbazine and CCNU With and Without Vincristine.
Anticancer researchA multicenter study of anaplastic oligodendroglioma: the Korean Radiation Oncology Group Study 13-12.
Journal of neuro-oncologySolitary "aggressive" leptomeningeal anaplastic oligoastrocytoma.
Revue neurologiqueStereotactic interstitial brachytherapy for the treatment of oligodendroglial brain tumors.
Strahlentherapie und Onkologie : Organ der Deutschen Rontgengesellschaft ... [et al]Genetic and epigenetic characterization of low-grade gliomas reveals frequent methylation of the MLH3 gene.
Genes, chromosomes & cancerCombined IDH1 mutation and MGMT methylation status on long-term survival of patients with cerebral low-grade glioma.
Clinical neurology and neurosurgeryDendritic cell-based immunotherapy targeting Wilms' tumor 1 in patients with recurrent malignant glioma.
Journal of neurosurgeryBiopsy Proven Tumefactive Multiple Sclerosis with Concomitant Glioma: Case Report and Review of the Literature.
Frontiers in neurologyIDH mutation, 1p19q codeletion and ATRX loss in WHO grade II gliomas.
OncotargetHypomethylated Rab27b is a progression-associated prognostic biomarker of glioma regulating MMP-9 to promote invasion.
Oncology reportsQuantitative fluorescence using 5-aminolevulinic acid-induced protoporphyrin IX biomarker as a surgical adjunct in low-grade glioma surgery.
Journal of neurosurgeryApplication of technical strategies for surgical management of adult intrinsic pontine gliomas: a retrospective series.
International journal of clinical and experimental medicineIDH1 and IDH2 mutations in different histologic subtypes and WHO grading gliomas in a sample from Northern Brazil.
Genetics and molecular research : GMRPhase II trial of pre-irradiation and concurrent temozolomide in patients with newly diagnosed anaplastic oligodendrogliomas and mixed anaplastic oligoastrocytomas: long term results of RTOG BR0131.
Journal of neuro-oncologyImpending Impact of Molecular Pathology on Classifying Adult Diffuse Gliomas.
Cancer control : journal of the Moffitt Cancer CenterCombined ATRX/IDH1 immunohistochemistry predicts genotype of oligoastrocytomas.
HistopathologyImmunohistochemical profiles of IDH1, MGMT and P53: practical significance for prognostication of patients with diffuse gliomas.
Neuropathology : official journal of the Japanese Society of NeuropathologyOligodendroglioma: pathology, molecular mechanisms and markers.
Acta neuropathologicaAnaplastic glioma: current treatment and management.
Expert review of neurotherapeuticsRadiotherapy and temozolomide for anaplastic astrocytic gliomas.
Journal of neuro-oncologyOpsoclonus-myoclonus syndrome in a patient with an anaplastic oligoastrocytoma.
Journal of neuro-oncologyPrognostic value of volume-based measurements on (11)C-methionine PET in glioma patients.
European journal of nuclear medicine and molecular imagingDynamic study of methionine positron emission tomography in patients with glioblastoma with oligodendroglial components.
Brain tumor pathologyBevacizumab and irinotecan in recurrent malignant glioma, a single institution experience.
Radiology and oncologySemiological and electroencephalographic features of epilepsy with amygdalar lesion.
Epilepsy researchAstroblastoma: beside being a tumor entity, an occasional phenotype of astrocytic gliomas?
OncoTargets and therapyHealth-related quality of life in stable, long-term survivors of low-grade glioma.
Journal of clinical oncology : official journal of the American Society of Clinical OncologyPossible induction of multiple seizure foci due to parietal tumour and anti-NMDAR antibody.
Epileptic disorders : international epilepsy journal with videotapeImpact of 1p/19q codeletion and histology on outcomes of anaplastic gliomas treated with radiation therapy and temozolomide.
International journal of radiation oncology, biology, physicsThe impact of adjuvant radiation therapy for high-grade gliomas by histology in the United States population.
International journal of radiation oncology, biology, physicsAnaplastic oligoastrocytoma: is molecular stratification based on 1p/19q status alone appropriate?
Journal of neuro-oncologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
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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.
- Endoscopic Transorbital Approach for Select Recurrent and Newly Diagnosed Anteromedial Temporal Lobe Gliomas.
- Risk factors for postoperative malignant progression of lower-grade gliomas: a systematic review and meta-analysis.
- Investigation of mismatch repair protein expression in glioma.
- Oligodendrogliomas: findings after classifying the same cohort using pre- and post-World Health Organization (WHO) 2021 criteria.
- A dual-genotype IDH-mutant infiltrating glioma, a real oligoastrocytoma in cerebral hemisphere.
- Revisiting pediatric HGGs and PNETs according to the WHO CNS5 criteria: A clinical and genomic retrospective analysis.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:251656(Orphanet)
- MONDO:0016702(MONDO)
- GARD:9769(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q1938668(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
