Astrocitoma são tumores (neoplasia) do sistema nervoso central (SNC) originados de um astrócito, uma célula em forma de estrela que sustenta os neurônios. É o tipo de glioma mais comum e podem ser benignos ou malignos. Quanto menor o grau, mais tempo de sobrevida, que pode ultrapassar 10 anos ou serem menor a 1 ano. Afetam 5 em cada 100.000 pessoas.
Introdução
O que você precisa saber de cara
Tumor cerebral raro, de crescimento lento, originado de células gliais. Caracteriza-se por células com núcleos pleomórficos e citoplasma xantomatoso, frequentemente associado a epilepsia.
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
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 — Xantoastrocitoma pleomórfico
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Outros ensaios clínicos
17 ensaios clínicos encontrados, 6 ativos.
Publicações mais relevantes
Multi-omic landscape of human gliomas from diagnosis to treatment and recurrence.
Gliomas are among the most lethal cancers, with limited treatment options. To uncover hallmarks of therapeutic escape and tumor microenvironment (TME) landscape, we applied spatial proteomics, transcriptomics, and glycomics to 670 lesions from 310 adult and pediatric patients. Single-cell analysis shows high B7H3+ tumor cell prevalence in glioblastoma (GBM) and pleomorphic xanthoastrocytoma, while most gliomas, including pediatric cases, express targetable tumor antigens in less than 50% of tumor cells, potentially explaining trial failures. Paired samples of isocitrate dehydrogenase (IDH)-mutant gliomas reveal recurrence driven by tumor-immune spatial reorganization, shifting from T cell and vasculature-associated myeloid cell-enriched niches to microglia and CD206+ macrophage-dominated tumors. Multi-omic integration identified N-glycosylation as the best classifier of grade, while the immune transcriptome best predicted GBM survival. Provided as a community resource, this study offers a framework for glioma targeting, classification, outcome prediction, and a baseline of TME composition across all stages.
Identification of BRAF V600E Mutation in Cerebrospinal Fluid Aids in Diagnosing Leptomeningeal Carcinomatosis Arising From Pleomorphic Xanthoastrocytoma: A Case Report.
Pleomorphic xanthoastrocytomas (PXAs) are rare primary central nervous system (CNS) tumors that appear heterogeneous on imaging and histology and typically cause headaches or seizures on initial presentation. Alongside high rates of favorable prognosis after surgical excision exist similarly high rates of recurrence. Leptomeningeal spread on recurrence is even rarer and more challenging to diagnose. We describe a case of a 40-year-old man with a history of surgically resected PXA presenting 12 years later with persistent headaches and lower back pain. Imaging studies revealed arachnoiditis, and a subsequent brain biopsy was nondiagnostic. Serial CSF studies only revealed the presence of atypical cells too few to further characterize via standard histology studies, with rare small lymphocytes and monocytoid cells. Submitting these cells for next-generation sequencing ultimately revealed a BRAF V600E mutation typically found in PXAs, thereby confirming the diagnosis of leptomeningeal recurrence and revealing a therapeutic target. This case highlights the utility of next-generation sequencing as a means of non-invasively diagnosing leptomeningeal disease in recurrent PXA and potentially in other cancer types as well.
BRAFV600E-mutated central nervous system tumors benefit from treatment with dabrafenib plus trametinib: Results from the Drug Rediscovery Protocol.
Dual MAPK pathway inhibition with dabrafenib and trametinib has demonstrated significant activity across several BRAFV600E-mutated tumor types. The aim of this study was to evaluate the efficacy and safety of dabrafenib plus trametinib in patients with BRAFV600E-mutated progressive or recurrent tumors of the central nervous system (CNS). Adult patients with BRAFV600E-mutated CNS-tumors, including pilocytic astrocytoma, pleomorphic xanthoastrocytoma, and glioblastoma, progressive on their last treatment line, were treated in the Drug Rediscovery Protocol (2023-509152-33-00) with dabrafenib 150 mg twice daily plus trametinib 2 mg once daily, until disease progression or intolerable toxicity. The primary endpoints were clinical benefit (CB: confirmed complete or partial response [PR] or stable disease [SD] ≥ 16 weeks) and safety. Between January 2019 and May 2024, 30 patients started treatment, of whom 25 were evaluable for response after completing at least one full treatment cycle. CB was observed in 19 patients, including 11 with confirmed PR and eight with SD for ≥ 16 weeks, resulting in a CB-rate of 76% (95% CI, 54.9%-90.6%) and an objective response rate of 44% (95% CI, 24.4%-65.1%). After a median follow-up of 40.2 months, the median duration of response was 27.8 months (95% CI, 23.6 months-not reached). The median progression-free and overall survival were 18.1 months (95% CI, 8.4 months-not reached) and 32.3 months (95% CI, 22.0 months-not reached), respectively. No unexpected toxicities were observed. Dabrafenib plus trametinib is highly effective in patients with recurrent or progressive BRAFV600E-mutated CNS-tumors, representing a valuable therapeutic option for these vulnerable patients.
BRAF-mutant high-grade glioma with pleomorphic and pseudopapillary features (HPAP): A PLNTY mimic demonstrating tumor progression during longitudinal follow-up.
High-grade glioma with pleomorphic and pseudopapillary features (HPAP) is a recently recognized glioma subtype defined by DNA methylation profiling. While it exhibits overlapping histological features with various CNS tumors, such as polymorphous low-grade neuroepithelial tumor of the young (PLNTY) and pleomorphic xanthoastrocytoma, its molecular pathogenesis and clinical behavior remain incompletely understood. We report a rare case of HPAP with BRAF p.V600E mutation and PLNTY-like histological features that showed rapid tumor progression during long-term follow-up. A 47-year-old woman harbored a lesion that remained asymptomatic and slow-growing for over 20 years, but later exhibited contrast enhancement and rapid expansion. Partial tumor resection was performed with hippocampal preservation based on intraoperative genetic testing and functional considerations. No regrowth of the residual hippocampal lesion was observed at 12 months postoperatively. Histologically, the tumor showed oligodendroglioma-like morphology, strong CD34 immunopositivity, consistent with PLNTY-like features, but indicated a high proliferative index. Molecular analysis revealed co-occurring BRAF p.V600E and TERT promoter (pTERT, c.-124C>T) mutations, with a lower variant allele frequency for the pTERT mutation. This disparity, confirmed by droplet digital PCR, suggests that the BRAF p.V600E mutation was an early, clonal event, whereas the pTERT mutation likely arose later in a subclonal population. DNA methylation profiling classified the tumor as HPAP with high confidence (NCI-Bethesda score: 0.969), and uniform manifold approximation and projection showed clustering within HPAP reference cases. This case represents a rare example of BRAF p.V600E-mutant HPAP with PLNTY-like features in which a subclonal pTERT mutation likely emerged during tumor evolution, contributing to rapid tumor progression. The combination of a prolonged indolent phase followed by rapid growth, along with the intratumoral genetic heterogeneity observed, provides novel insights into the biological diversity and evolutionary dynamics of HPAP.
Pediatric low-grade epilepsy-associated tumors (LEATS): neuroimaging review and genetics update.
Low-grade epilepsy-associated tumors (LEATs) are a distinct group of tumors commonly encountered in pediatric drug-resistant epilepsy that necessitate surgical intervention. Like tumors elsewhere in the central nervous system, molecular characterization is becoming an increasingly important consideration in pediatric neuro-oncology prognostication and management for LEATs. Thus, familiarity with relevant tumor mutations and radiogenomic features of LEATs is important for radiologists caring for affected patients. This article will review the genetic alterations and imaging characteristics of LEATs, formatted according to the three categories defined by the World Health Organization (WHO): glioneuronal and neuronal tumors (ganglioglioma, dysembryoplastic neuroepithelial tumor, papillary glioneuronal tumor, multinodular and vacuolating neuronal tumor); circumscribed astrocytic gliomas (pilocytic astrocytoma, pleomorphic xanthoastrocytoma); and pediatric-type diffuse low-grade gliomas (diffuse astrocytoma MYB or MYBL1-altered, angiocentric glioma, diffuse low-grade glioma MAPK pathway-altered, polymorphous low-grade neuroepithelial tumor of the young).
Publicações recentes
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.
Role of Surrogate Immunohistochemistry Markers in CNS Tumors in the Era of Molecular Diagnostics With Recent Updates.
Genomic Alteration Patterns Across Histological Grades in BRAF p.V600E-Mutant Gliomas and Glioneuronal Tumors: An Analysis of 15 Cases.
Identification of BRAF V600E Mutation in Cerebrospinal Fluid Aids in Diagnosing Leptomeningeal Carcinomatosis Arising From Pleomorphic Xanthoastrocytoma: A Case Report.
BRAF(V600E)-mutated central nervous system tumors benefit from treatment with dabrafenib plus trametinib: Results from the Drug Rediscovery Protocol.
📚 EuropePMC446 artigos no totalmostrando 196
Identification of BRAF V600E Mutation in Cerebrospinal Fluid Aids in Diagnosing Leptomeningeal Carcinomatosis Arising From Pleomorphic Xanthoastrocytoma: A Case Report.
Cancer reports (Hoboken, N.J.)BRAFV600E-mutated central nervous system tumors benefit from treatment with dabrafenib plus trametinib: Results from the Drug Rediscovery Protocol.
European journal of cancer (Oxford, England : 1990)BRAF-mutant high-grade glioma with pleomorphic and pseudopapillary features (HPAP): A PLNTY mimic demonstrating tumor progression during longitudinal follow-up.
Neuro-oncology advancesPediatric low-grade epilepsy-associated tumors (LEATS): neuroimaging review and genetics update.
Pediatric radiologyClinicopathological, Immunohistochemical, and Molecular Genetic Study on Pleomorphic Xanthoastrocytoma and Epithelioid Glioblastoma.
Current cancer drug targetsDabrafenib in pediatric patients with BRAF V600 mutation-positive high-grade glioma: Results from a phase 1/2a single-arm study.
Neuro-oncology practiceComparative molecular analysis of primary and recurrent pleomorphic xanthoastrocytoma with BRAF mutation treated with combination therapy with dabrafenib and trametinib: a case report and literature review.
International cancer conference journalMulti-omic landscape of human gliomas from diagnosis to treatment and recurrence.
Cancer cellOCD Symptoms and Capgras Syndrome in a 13-Year-Old Girl Following Right-Sided Brain Surgery.
Case reports in psychiatrySuccessful response in advanced leptomeningeal disease from pleomorphic xanthoastrocytoma with BRAF/MEK inhibitors: a case report.
Frontiers in oncologyAn atypical presentation of a pleomorphic xanthoastrocytoma in a 66-year-old woman, a case report.
Radiology case reportsIntraventricular Trigone Pleomorphic Xanthoastrocytoma: A Rare Presentation with Favorable Prognosis in a Young Adult-A Case Report.
Asian journal of neurosurgeryPleomorphic xanthoastrocytoma with anaplastic features.
Brain circulationChallenging CDKN2A assessment in BRAF-altered gliomas: lessons from a pleomorphic xanthoastrocytoma-enriched cohort.
Acta neuropathologica communicationsMetastatic Low-Grade Glioma Successfully Treated in a Pediatric Patient With BRAF A598_T599insI Mutation.
Cancer reports (Hoboken, N.J.)Molecular, histologic, and clinical characterization of methylation class pleomorphic xanthoastrocytoma: An analysis of 469 tumors.
Neuro-oncology advancesSeizure characteristics and outcomes in patients with pleomorphic xanthoastrocytoma.
Neuro-oncology advancesMTAP and p16 as immunohistochemical surrogates of CDKN2A/B homozygous deletion in central nervous system tumors: A multicentre Italian experience.
Annals of diagnostic pathologyPleomorphic xanthoastrocytoma with anaplasia and BEND5-NTRK2 fusion in a young adult with a history of cranial radiation for childhood rhabdomyosarcoma.
Neuro-oncology advancesPleomorphic xanthoastrocytoma with multiple recurrences and continuous malignant progression to bone metastasis: a case report.
Frontiers in surgeryThalamic pleomorphic xanthoastrocytoma central nervous system World Health Organization grade 3.
Radiology case reportsMRI features of pleomorphic xanthoastrocytoma defined by DNA methylation profile.
Revue neurologiquePXA-like tumors: prognostic implications.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryShift of DNA methylation class may occur on recurrence of pleomorphic xanthoastrocytoma.
Journal of neuropathology and experimental neurologyGlioneuronal tumors PATZ1-fused: clinico-molecular and DNA methylation signatures for a variety of morphological and radiological profiles.
Acta neuropathologica communicationsPapillary Glioneuronal Tumor Located in the Subcortical White Matter With a Purely Solid Pattern: A Case Report.
CureusAnaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination presenting with cranial neuropathy in an adult patient: illustrative case.
Journal of neurosurgery. Case lessonsIntraventricular WHO Grade 3 Pleomorphic Xanthoastrocytoma: A Rare Case Report and Review of the Literature.
Case reports in pathologyCase Report: SMARCB1-deficient phenotype may be a new specialized type of pleomorphic xanthoastrocytoma associated with poor prognosis.
Frontiers in oncologyNontumoral Amygdalar Enlargement in Tumoral Epilepsy.
World neurosurgeryPleomorphic xanthoastrocytoma and treatment of epilepsy: a case report.
Translational pediatricsClinical outcomes for pleomorphic xanthoastrocytoma patients.
Neuro-oncology practiceProton Beam Therapy for a Rare Anaplastic Pleomorphic Xanthoastrocytoma: Case Report and Literature Review.
International journal of particle therapyFree-hand Frameless Pinless Electromagnetic-Navigation (AXIEM™)-Guided Brain Lesion Biopsies: An Institution Based Experience from a Low-Middle-Income Country.
Pakistan journal of medical sciencesDrug-resistant BRAF V600E-mutant recurrent pleomorphic xanthoastrocytoma, CNS WHO Grade 3 successfully resolved with incidental discontinuation of combined BRAF and MEK inhibitor therapy.
Surgical neurology internationalIntracranial pleomorphic liposarcoma misclassified as a pleomorphic xanthoastrocytoma by a DNA methylation classifier: illustrative case.
Journal of neurosurgery. Case lessonsEndoscopic transorbital approach for resection of mediobasal temporal lesions using an optic radiation-sparing strategy.
Journal of neurosurgeryBRAF/MEK inhibitors use for pediatric gliomas; real world experience from a resource-limited country.
Frontiers in oncologyDrug-resistant schizophrenia-like psychosis associated with temporal non-anaplastic pleomorphic xanthoastrocytoma: unusual revealing symptom of a rare pathology.
Annals of medicine and surgery (2012)"Recurrent Pleomorphic Xanthoastrocytoma Presenting with Diffuse Leptomeningeal Spread".
The NeurohospitalistNo drug holidays in BRAFV600E glioma patients: An argument for dose reduction of targeted therapies.
Neuro-oncology practiceEpithelioid angiosarcoma arising from pleomorphic xanthoastrocytoma, CNS WHO grade 3.
Journal of neuropathology and experimental neurologyExtra-axial primary meningeal pleomorphic xanthoastrocytoma: a case report.
The Pan African medical journal[Clinicopathological and molecular characteristics of pediatric gliomas: analysis of 111 cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyExtra-temporal pediatric low-grade gliomas and epilepsy.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryJourney through tumorverse: Creating models to decode PXA mysteries.
Molecular therapy. OncologyIntra-axial Cortical-Based Tumour Presented as Homonymous Hemianopia in a Young Patient: A Diagnostic Dilemma.
CureusCell-in-cell phenomena of intracellular neutrophils in a recurrent pleomorphic xanthoastrocytoma.
Free neuropathologyPleomorphic xanthoastrocytoma with NTRK fusion presenting as spontaneous intracranial hemorrhage-case report and literature review.
Frontiers in pediatricsSpinal Cord Astroblastoma With EWSR1-BEND2 Fusion in Female Patients : A Report of Four Cases From China and a Comprehensive Literature Review.
The American journal of surgical pathologyComparing loss of p16 and MTAP expression in detecting CDKN2A homozygous deletion in pleomorphic xanthoastrocytoma.
Journal of neuropathology and experimental neurologySuccessful treatment of pediatric patients with high-grade gliomas featuring leptomeningeal metastases by targeting BRAF V600E mutations with dabrafenib plus trametinib: two illustrative cases.
International cancer conference journalPleomorphic Xanthoastrocytoma: Multi-Institutional Evaluation of Stereotactic Radiosurgery.
NeurosurgeryGermline Co-deletion of CDKN2A and CDKN2B Genes in Pleomorphic Xanthoastrocytoma: Case Report.
In vivo (Athens, Greece)Immunotherapy for pediatric low-grade gliomas.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryLow-grade glioma of the temporal lobe and tumor-related epilepsy in children.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryBRAF V600E in a preclinical model of pleomorphic Xanthoastrocytoma: Analysis of the tumor microenvironment and immune cell infiltration dynamics in vivo.
Molecular therapy. OncologyCase report: complete long-lasting response to multimodal third line treatment with neurosurgical resection, carmustine wafer implantation and dabrafenib plus trametinib in a BRAFV600E mutated high-grade glioma.
Frontiers in oncologyPediatric high grade gliomas: A comprehensive histopathological, immunohistochemical and molecular integrated approach in routine practice.
Pathology, research and practiceDetection of tumor-derived cell-free DNA in cerebrospinal fluid using a clinically validated targeted sequencing panel for pediatric brain tumors.
Journal of neuro-oncologyGanglioglioma with anaplastic/high-grade transformation: Histopathologic, molecular, and epigenetic characterization of 3 cases.
Journal of neuropathology and experimental neurologyPediatric-Like Brain Tumors in Adults.
Advances and technical standards in neurosurgeryPDP type brain tumor in association with multiple endocrine neoplasia type 1.
HeliyonExploring prognostic factors and treatment strategies for long-term survival in pleomorphic xanthoastrocytoma patients.
Scientific reportsIntraventricular Pleomorphic Xanthoastrocytoma: A Case Report and Systemic Review.
CureusClinical and molecular study of radiation-induced gliomas.
Scientific reportsRare AGK-BRAF gene fusion in an adolescent with supratentorial pleomorphic xanthoastrocytoma.
BMJ case reportsRecurrent Pleomorphic Xanthoastrocytoma Presenting With Diffuse Leptomeningeal Spread.
The NeurohospitalistGiant cell glioblastoma with lipogenic differentiation in a patient with neurofibromatosis type 1: A case report.
Neuropathology : official journal of the Japanese Society of NeuropathologyAnalysis of Histomorphologic/Molecular Association and Immune Checkpoint Regulators in Epithelioid Glioblastoma and Pleomorphic Xanthoastrocytoma: Are These Tumors Potential Candidates for Immune Checkpoint Blockade?
Applied immunohistochemistry & molecular morphology : AIMMReference on copy number variations in pleomorphic xanthoastrocytoma: Implications for diagnostic approach.
Free neuropathologyDiagnostic Insights into Pediatric Pleomorphic Xanthoastrocytoma through DNA Methylation Class and Pathological Diagnosis Analysis.
Diagnostics (Basel, Switzerland)A pleomorphic xanthoastrocytoma highlighting the morphological heterogeneity of this uncommon tumor.
Journal of neuropathology and experimental neurologyFreiburg Neuropathology Case Conference : Headache, Mental Confusion and Mild Hemiparesis in a 68-year-old Patient.
Clinical neuroradiologyLi-Fraumeni Syndrome, A Rarity Among Rarities: A Case Report and Review of Literature.
CureusA rare case of cerebellar anaplastic pleomorphic xanthoastrocytoma.
Journal of cancer research and therapeuticsThe Significance of BRAF Mutation in the Epithelioid Glioblastoma Subtype: A Systematic Literature Review and a Case Report with a Unique Intraventricular Topography.
International journal of surgical pathology[Circumscribed Astrocytic Gliomas].
No shinkei geka. Neurological surgeryNomograms Based on MRI Radiomics for Differential Diagnosis and Predicting BRAFV600E Expression in Pleomorphic Xanthoastrocytoma and Ganglioglioma.
Academic radiologyA Rare Case of BRAF-mutated Metastatic Pleomorphic Xanthoastrocytoma Patient who Developed Radiodermatitis After Receiving Anti-BRAF Treatment.
Journal of pediatric hematology/oncologyCytological features of diffuse and circumscribed gliomas.
Cytopathology : official journal of the British Society for Clinical CytologyAnti-N-Methyl-D-Aspartate Receptor Encephalitis With Diffuse Demyelinating Plaques: A Case Report of an Atypical Presentation.
CureusPerisellar Tumor Coexisting with Unruptured Cerebral Aneurysm: Single-Stage or Staged Surgery?
World neurosurgeryA "masked" pleomorphic xanthoastrocytoma.
Pathology, research and practiceFreiburg Neuropathology Case Conference : Recurrent Speech Arrest, Neologistic Jargon Aphasia, and Impaired Memory Function in a 39-year-old Patient.
Clinical neuroradiologyClinical features and surgical outcomes of high grade pleomorphic xanthoastrocytomas: a single-center experience with a systematic review.
Frontiers in oncologyMRI characteristics predict BRAF V600E status in gangliogliomas and pleomorphic xanthoastrocytomas and provide survival prognostication.
Acta radiologica (Stockholm, Sweden : 1987)Differentiating Cerebral Toxoplasmosis and Tumor Recurrence by Thallium-201 Single-Photon Emission Computerized Tomography in a 28-Year-Old Female with Astrocytoma.
Case reports in oncologyEstablishment and characterization of patient-derived xenograft of a rare pediatric anaplastic pleomorphic xanthoastrocytoma (PXA) bearing a CDC42SE2-BRAF fusion.
Scientific reportsDiffusely Infiltrating Gliomas With Poor Prognosis, TERT Promotor Mutations, and Histological Anaplastic Pleomorphic Xanthoastrocytoma-Like Appearance Classify as Mesenchymal Type of Glioblastoma, IDH-wildtype by Methylation Analysis.
Neurosurgery practiceClinical outcome following surgical resection and radiotherapy in adult patients with pleomorphic xanthoastrocytoma as defined by DNA methylation profiling.
Neuro-oncology practiceMEN1 and the brain: Don't just look only at the pituitary gland. A case report of anaplastic pleomorphic xanthoastrocytoma in a MEN1 patient, and systematic review.
Annales d'endocrinologieDetection of human neurotropic JCPyV DNA sequence in pediatric anaplastic xanthoastrocytoma.
Journal of neurovirologyA Rare Case Report of Intraventricular Anaplastic Pleomorphic Xanthoastrocytoma.
CureusRecombinant polio-rhinovirus immunotherapy for recurrent paediatric high-grade glioma: a phase 1b trial.
The Lancet. Child & adolescent healthPleomorphic xanthoastrocytoma in the brainstem with BRAF V600E mutation: A case report.
Asian journal of surgeryA Recurrent Pleomorphic Xanthoastrocytoma in the Cerebellum in a Young Adult: A Case Report and Review of the Literature.
Case reports in neurologyClinical, Morphological, and Molecular Study on Grade 2 and 3 Pleomorphic Xanthoastrocytoma.
Current oncology (Toronto, Ont.)Not All Monstrous Cells Indicate Glioblastoma: A Neuropathological Case Report of Pleomorphic Xanthoastrocytoma Misdiagnoses As Giant Cell Glioblastoma.
CureusClinical outcomes for pleomorphic xanthoastrocytoma patients: an institutional experience.
Research squareImplications of BRAF V600E mutation in gliomas: Molecular considerations, prognostic value and treatment evolution.
Frontiers in oncologyUpfront BRAF/MEK inhibitors for treatment of high-grade glioma: A case report and review of the literature.
Neuro-oncology advancesCorrelation of MTAP immunohistochemical deficiency with CDKN2A homozygous deletion and clinicopathological features in pleomorphic xanthoastrocytoma.
Brain tumor pathologyFluorescein sodium in the surgical treatment of pleomorphic xanthoastrocytomas: Results from a retrospective study.
Frontiers in oncologyBRAF/MEK Dual Inhibitors Therapy in Progressive and Anaplastic Pleomorphic Xanthoastrocytoma: Case Series and Literature Review.
Journal of the National Comprehensive Cancer Network : JNCCNIntravenous Fluorescein Sodium for Resection of Pleomorphic Xanthoastrocytoma: 2-Dimensional Operative Video.
Operative neurosurgery (Hagerstown, Md.)5-ALA fluorescence-guided resection of a recurrent anaplastic pleomorphic xanthoastrocytoma: illustrative case.
Journal of neurosurgery. Case lessonsIntegrated genomic analysis reveals actionable targets in pediatric spinal cord low-grade gliomas.
Acta neuropathologica communicationsLateral ventricle pleomorphic xanthoastrocytoma concurrent with Dandy-Walker complex: A case report.
MedicineA 7-year-old boy with recurrent cyanosis and tachypnea: A case report.
World journal of clinical casesMR Imaging Characteristics and ADC Histogram Metrics for Differentiating Molecular Subgroups of Pediatric Low-Grade Gliomas.
AJNR. American journal of neuroradiologyAnaplastic ganglioglioma-A diagnosis comprising several distinct tumour types.
Neuropathology and applied neurobiologyPleomorphic Xanthoastrocytoma: a single institution retrospective analysis and a review of the literature.
La Radiologia medicapRB immunostaining in the differential diagnosis between pleomorphic xanthoastrocytoma and glioblastoma with giant cells.
HistopathologyPleomorphic xanthoastrocytoma, anaplastic pleomorphic xanthoastrocytoma, and epithelioid glioblastoma: Case series with clinical characteristics, molecular features and progression relationship.
Clinical neurology and neurosurgery5-ALA fluorescence-guided resection of pediatric low-grade glioma using the ORBEYE 3D digital exoscope: a technical report.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryA clinicopathologic study of malignancy in VCP-associated multisystem proteinopathy.
Orphanet journal of rare diseasesTargeted Next-Generation Sequencing Reveals Divergent Clonal Evolution in Components of Composite Pleomorphic Xanthoastrocytoma-Ganglioglioma.
Journal of neuropathology and experimental neurologyLong Non-coding RNA and mRNA Co-expression Network Reveals Novel Players in Pleomorphic Xanthoastrocytoma.
Molecular neurobiologyPleomorphic xanthoastrocytoma in the multiverse of epigenomics: is it time to recognize the variants?
Acta neuropathologica communicationsGliosarcoma with unusual glial components: Two case reports.
Neuropathology : official journal of the Japanese Society of NeuropathologyImpact of Grade on Survival in Pleomorphic Xanthoastrocytoma and Low Prevalence of BRAF V600E Mutation.
World neurosurgeryA case report of a novel NTRK gene fusion in pleomorphic xanthoastrocytoma.
Clinical neuropathologyGenetic Alteration Analysis of IDH1, IDH2, CDKN2A, MYB and MYBL1 in Pediatric Low-Grade Gliomas.
Frontiers in surgeryCircumscribed astrocytic gliomas: Contribution of molecular analyses to histopathology diagnosis in the WHO CNS5 classification.
Indian journal of pathology & microbiologyCXCL14 Promotes a Robust Brain Tumor-Associated Immune Response in Glioma.
Clinical cancer research : an official journal of the American Association for Cancer ResearchAnaplastic Pleomorphic Xanthoastrocytoma: A Rare Variant of Astrocytoma.
CureusCytologic and histologic features of pigmented pleomorphic xanthoastrocytoma: A case report and literature review.
Diagnostic cytopathology[Anaplastic pleomorphic xanthoastrocytoma - single-center analysis of 42 patients].
Zhurnal voprosy neirokhirurgii imeni N. N. BurdenkoGene expression based profiling of pleomorphic xanthoastrocytoma highlights two prognostic subgroups.
American journal of translational researchAnaplastic pleomorphic xanthoastrocytoma with epithelioid morphology misdiagnosed and treated as melanoma.
Neuro-oncology advancesMolecular landscape of IDH-wild-type, H3-wild-type glioblastomas of adolescents and young adults.
Neuropathology and applied neurobiologyHigh-grade glioma with pleomorphic and pseudopapillary features (HPAP): a proposed type of circumscribed glioma in adults harboring frequent TP53 mutations and recurrent monosomy 13.
Acta neuropathologicaThe evolution of pleomorphic xanthoastrocytoma: from genesis to molecular alterations and mimics.
Laboratory investigation; a journal of technical methods and pathologyPleomorphic xanthoastrocytoma is a heterogeneous entity with pTERT mutations prognosticating shorter survival.
Acta neuropathologica communicationsAutobiographical Case Report of Experiences With Pleomorphic Xanthoastrocytoma.
CureusRapid Diffuse Leptomeningeal Dissemination of an Anaplastic Pleomorphic Xanthoastrocytoma in an Adult Patient.
CureusSporadic and Lynch syndrome-associated mismatch repair-deficient brain tumors.
Laboratory investigation; a journal of technical methods and pathologyAn atypical teratoid/rhabdoid tumor (AT/RT) with molecular features of pleomorphic xanthoastrocytoma (PXA) in a 62-year-old patient.
Free neuropathologyIsocitrate Dehydrogenase Wild-type Glial Tumors, Including Glioblastoma.
Hematology/oncology clinics of North AmericaLarge Parenchymal Perianeurysmal Cyst: A Case Report.
Journal of neurosciences in rural practiceIdentification of BRAF p. V600E-Mutant and Wild-Type by MR Imaging in Pleomorphic Xanthoastrocytoma and Anaplastic Pleomorphic Xanthoastrocytoma.
AJNR. American journal of neuroradiologyPrevalence of BRAFV600 in glioma and use of BRAF Inhibitors in patients with BRAFV600 mutation-positive glioma: systematic review.
Neuro-oncologyComplex grade III astrocytoma with granular cells and PXA features in a patient with tuberous sclerosis complex (Bourneville-Pringle syndrome) - case report and review of literature.
Polish journal of pathology : official journal of the Polish Society of PathologistsClinical-pathological study of 28 glial and mixed neuronal-glial tumors diagnosed within the first year of life.
Clinical neuropathologyMalignant transformation of pleomorphic xanthoastrocytoma in pregnant patient: Clinical case and ethical dilemma.
Surgical neurology internationalDurable Progression-Free Survival With the Use of BRAF and MEK Inhibitors in Four Cases With BRAF V600E-Mutated Gliomas.
Cancer control : journal of the Moffitt Cancer CenterCytopathological and histopathological features of cerebral granular cell astrocytoma: A case report.
Cytopathology : official journal of the British Society for Clinical CytologyAnaplastic pleomorphic xanthoastrocytoma misdiagnosed as cerebral sparganosis-identification of the "mirror image".
Quantitative imaging in medicine and surgerySupratentorial non-RELA, ZFTA-fused ependymomas: a comprehensive phenotype genotype correlation highlighting the number of zinc fingers in ZFTA-NCOA1/2 fusions.
Acta neuropathologica communicationsIntegrated genotype-phenotype analysis of long-term epilepsy-associated ganglioglioma.
Brain pathology (Zurich, Switzerland)Sequencing of a central nervous system tumor demonstrates cancer transmission in an organ transplant.
Life science allianceA pediatric cerebral tumor with MN1 alteration and pathological features mimicking carcinoma metastasis: may the terminology "high grade neuroepithelial tumor with MN1 alteration" still be relevant?
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryA Rare Case of a High-Grade Astroblastoma with 5-Year Follow-up.
Asian journal of neurosurgeryRole of Adamts-1 in Pleomorphic Xanthoastrocytoma Tumor Cells Progression.
Turkish neurosurgeryPleomorphic Xanthoastrocytoma of the Frontal Lobe in a Child: A Rare Entity.
CureusCyclin D1 expression in ganglioglioma, pleomorphic xanthoastrocytoma and pilocytic astrocytoma.
Experimental and molecular pathologyDifferences in treatment patterns and overall survival between grade II and anaplastic pleomorphic xanthoastrocytomas.
Journal of neuro-oncologyClinical Features and Surgical Results of Pediatric Pleomorphic Xanthoastrocytoma: Analysis of 17 Cases with a Literature Review.
World neurosurgeryPediatric Pleomorphic Xanthoastrocytoma: A National Database Inquiry on Current Treatment Approaches in the United States.
Cancer reports (Hoboken, N.J.)Diagnostic Utility of the Immunohistochemical Expression of Serine and Arginine Rich Splicing Factor 1 (SRSF1) in the Differential Diagnosis of Adult Gliomas.
CancersPredictors of outcome in pleomorphic xanthoastrocytoma.
Neuro-oncology practiceClinical Characteristics of BRAF V600E Gene Mutation in Patients of Epilepsy-Associated Brain Tumor: a Meta-analysis.
Journal of molecular neuroscience : MNAtypical Teratoid/Rhabdoid Tumor (AT/RT) With Molecular Features of Pleomorphic Xanthoastrocytoma.
The American journal of surgical pathology[Epithelioid glioblastoma with BRAF V600E mutation: a clinicopathological and molecular study].
Zhonghua bing li xue za zhi = Chinese journal of pathologyPaediatric Gliomas: BRAF and Histone H3 as Biomarkers, Therapy and Perspective of Liquid Biopsies.
CancersA case series of pediatric survivors of anaplastic pleomorphic xanthoastrocytoma.
Neuro-oncology advancesAnaplastic Pleomorphic Xanthoastrocytoma: A Case Report and Literature Review.
International journal of general medicineFrameless radiosurgical third ventriculostomy: Technical report.
Surgical neurology internationalConditional reprogramming culture conditions facilitate growth of lower-grade glioma models.
Neuro-oncologyIntradural extramedullary pleomorphic xanthoastrocytoma: A case report.
Surgical neurology internationalCerebellar pleomorphic xanthoastrocytoma in the setting of neurofibromatosis type-I: Does it portend a different prognosis? A case report and systematic review.
Clinical neurology and neurosurgeryCerebrospinal Fluid Spread in a Child with Pleomorphic Xanthoastrocytoma: Report with Cytopathologic Evidence.
World neurosurgeryMolecular Features and Prognostic Factors of Pleomorphic Xanthoastrocytoma: A Collaborative Investigation of the Tohoku Brain Tumor Study Group.
Neurologia medico-chirurgicaCerebral pleomorphic xanthoastrocytoma mimicking inflammatory granuloma: Two case reports.
MedicinePhase 1 study of pomalidomide in children with recurrent, refractory, and progressive central nervous system tumors: A Pediatric Brain Tumor Consortium trial.
Pediatric blood & cancerConcomitant Idiopathic Intracranial Hypertension, Normal Pressure Hydrocephalus, and Pleomorphic Xanthoastrocytoma: A Case Report and Review of the Literature.
Case reports in surgerySubtype and grade-dependent spatial heterogeneity of T-cell infiltration in pediatric glioma.
Journal for immunotherapy of cancerAcquired ATRX Loss and ALT Phenotype Through Tumor Recurrences in a Case of Pleomorphic Xanthoastrocytoma Suggest Their Possible Roles in Tumor Progression.
Journal of neuropathology and experimental neurologyClinical Relevance of BRAF V600E Mutation Status in Brain Tumors with a Focus on a Novel Management Algorithm.
Targeted oncologyBiology and grading of pleomorphic xanthoastrocytoma-what have we learned about it?
Brain pathology (Zurich, Switzerland)Pleomorphic Xanthoastrocytoma of the Pineal Region in a Pediatric Patient With Neurofibromatosis Type 1.
Ochsner journalAssociation between adjuvant radiation therapy and overall survival in Pleomorphic Xanthoastrocytoma.
Clinical neurology and neurosurgeryExploring DNA Methylation for Prognosis and Analyzing the Tumor Microenvironment in Pleomorphic Xanthoastrocytoma.
Journal of neuropathology and experimental neurologyAn anaplastic pleomorphic xanthoastrocytoma with periventricular extension: An autopsy case report and review of the literature.
Neuropathology : official journal of the Japanese Society of NeuropathologyUveitis Induced by Biological Agents Used in Cancer Therapy.
Ocular immunology and inflammationSurgical treatment of low-grade brain tumors associated with epilepsy.
International review of neurobiologySpinal Pleomorphic Xanthoastrocytoma: Case Report and Literature Review.
World neurosurgeryPediatric Low-Grade Gliomas.
CancersCerebellar Pleomorphic Xanthoastrocytoma with BRAF V600E Mutation.
World neurosurgeryClinical impact of combined epigenetic and molecular analysis of pediatric low-grade gliomas.
Neuro-oncologyTPM3-NTRK1 fusion in a pleomorphic xanthoastrocytoma presenting with haemorrhage in a child.
BMJ case reportsUltrastructural evidence for presenсe of gap junctions in rare case of pleomorphic xanthoastrocytoma.
Ultrastructural pathologyPleomorphic xanthoastrocytoma in the posterior fossa: a case report with advanced neuroimaging findings.
Clinical imagingThe efficacy of DNA mismatch repair enzyme immunohistochemistry as a screening test for hypermutated gliomas.
Acta neuropathologica communications[Symptomatic epilepsy with Panayiotopoulos-like onset: the importance of neuroimaging].
Revista de neurologiaAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Comunidades
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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.
- Multi-omic landscape of human gliomas from diagnosis to treatment and recurrence.
- Identification of BRAF V600E Mutation in Cerebrospinal Fluid Aids in Diagnosing Leptomeningeal Carcinomatosis Arising From Pleomorphic Xanthoastrocytoma: A Case Report.
- BRAFV600E-mutated central nervous system tumors benefit from treatment with dabrafenib plus trametinib: Results from the Drug Rediscovery Protocol.
- BRAF-mutant high-grade glioma with pleomorphic and pseudopapillary features (HPAP): A PLNTY mimic demonstrating tumor progression during longitudinal follow-up.
- Pediatric low-grade epilepsy-associated tumors (LEATS): neuroimaging review and genetics update.
- 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.
- Role of Surrogate Immunohistochemistry Markers in CNS Tumors in the Era of Molecular Diagnostics With Recent Updates.
- Genomic Alteration Patterns Across Histological Grades in BRAF p.V600E-Mutant Gliomas and Glioneuronal Tumors: An Analysis of 15 Cases.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:251607(Orphanet)
- MONDO:0016690(MONDO)
- GARD:10631(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q627373(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
