O astrocitoma pilocítico é um subtipo raro de glioma de baixo grau do sistema nervoso central, caracterizado por um tumor cerebral bem circunscrito, muitas vezes cístico, com um nódulo mural discreto e longas projeções semelhantes a cabelos que se estendem dos astrócitos neoplásicos. Dependendo da localização primária e do tamanho do tumor, os pacientes podem apresentar sinais de aumento da pressão intracraniana (cefaléia, vômito, papiledema), visão turva, diminuição da acuidade visual, ataxia e/ou nistagmo, entre outros. Está mais comumente localizado no cerebelo, mas também foi relatada ocorrência no hipotálamo, tronco cerebral, quiasma óptico e hemisférios.
Introdução
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O astrocitoma pilocítico é um subtipo raro de glioma de baixo grau do sistema nervoso central, caracterizado por um tumor cerebral bem circunscrito, muitas vezes cístico, com um nódulo mural discreto e longas projeções semelhantes a cabelos que se estendem dos astrócitos neoplásicos. Dependendo da localização primária e do tamanho do tumor, os pacientes podem apresentar sinais de aumento da pressão intracraniana (cefaléia, vômito, papiledema), visão turva, diminuição da acuidade visual, ataxia e/ou nistagmo, entre outros. Está mais comumente localizado no cerebelo, mas também foi relatada ocorrência no hipotálamo, tronco cerebral, quiasma óptico e hemisférios.
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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
Genes associados
7 genes identificados com associação a esta condição. Padrão de herança: Not applicable.
Tyrosine-protein kinase that acts as a cell-surface receptor for fibroblast growth factors and plays an essential role in the regulation of embryonic development, cell proliferation, differentiation and migration. Required for normal mesoderm patterning and correct axial organization during embryonic development, normal skeletogenesis and normal development of the gonadotropin-releasing hormone (GnRH) neuronal system. Phosphorylates PLCG1, FRS2, GAB1 and SHB. Ligand binding leads to the activati
Cell membraneNucleusCytoplasm, cytosolCytoplasmic vesicle
Pfeiffer syndrome
A syndrome characterized by the association of craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly of the fingers and toes. Three subtypes are known: mild autosomal dominant form (type 1); cloverleaf skull, elbow ankylosis, early death, sporadic (type 2); craniosynostosis, early demise, sporadic (type 3).
Protein kinase involved in the transduction of mitogenic signals from the cell membrane to the nucleus (Probable). Phosphorylates MAP2K1, and thereby activates the MAP kinase signal transduction pathway (PubMed:21441910, PubMed:29433126). Phosphorylates PFKFB2 (PubMed:36402789). May play a role in the postsynaptic responses of hippocampal neurons (PubMed:1508179)
NucleusCytoplasmCell membrane
May play a role in photoreceptor function
MembraneCell projection, cilium
Retinitis pigmentosa 86
A form of retinitis pigmentosa, a retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. RP86 is an autosomal recessive form.
Receptor tyrosine kinase involved in the development and the maturation of the central and the peripheral nervous systems through regulation of neuron survival, proliferation, migration, differentiation, and synapse formation and plasticity (By similarity). Receptor for BDNF/brain-derived neurotrophic factor and NTF4/neurotrophin-4. Alternatively can also bind NTF3/neurotrophin-3 which is less efficient in activating the receptor but regulates neuron survival through NTRK2 (PubMed:15494731, PubM
Cell membraneEndosome membraneEarly endosome membraneCell projection, axonCell projection, dendriteCytoplasm, perinuclear regionPostsynaptic density
Developmental and epileptic encephalopathy 58
A form of epileptic encephalopathy, a heterogeneous group of severe early-onset epilepsies characterized by refractory seizures, neurodevelopmental impairment, and poor prognosis. Development is normal prior to seizure onset, after which cognitive and motor delays become apparent. DEE58 is an autosomal dominant condition characterized by onset of refractory seizures in the first days or months of life.
Serine/threonine-protein kinase that acts as a regulatory link between the membrane-associated Ras GTPases and the MAPK/ERK cascade, and this critical regulatory link functions as a switch determining cell fate decisions including proliferation, differentiation, apoptosis, survival and oncogenic transformation. RAF1 activation initiates a mitogen-activated protein kinase (MAPK) cascade that comprises a sequential phosphorylation of the dual-specific MAPK kinases (MAP2K1/MEK1 and MAP2K2/MEK2) and
CytoplasmCell membraneMitochondrionNucleus
Noonan syndrome 5
A form of Noonan syndrome, a disease characterized by short stature, facial dysmorphic features such as hypertelorism, a downward eyeslant and low-set posteriorly rotated ears, and a high incidence of congenital heart defects and hypertrophic cardiomyopathy. Other features can include a short neck with webbing or redundancy of skin, deafness, motor delay, variable intellectual deficits, multiple skeletal defects, cryptorchidism, and bleeding diathesis. Individuals with Noonan syndrome are at risk of juvenile myelomonocytic leukemia, a myeloproliferative disorder characterized by excessive production of myelomonocytic cells.
Ras proteins bind GDP/GTP and possess intrinsic GTPase activity (PubMed:20949621, PubMed:39809765). Plays an important role in the regulation of cell proliferation (PubMed:22711838, PubMed:23698361). Activates MAPK1/MAPK3 resulting in phosphorylation and ultimately degradation of GJA1 (By similarity). Plays a role in promoting oncogenic events by inducing transcriptional silencing of tumor suppressor genes (TSGs) in colorectal cancer (CRC) cells in a ZNF304-dependent manner (PubMed:24623306)
Cell membraneEndomembrane systemCytoplasm, cytosol
Leukemia, acute myelogenous
A subtype of acute leukemia, a cancer of the white blood cells. AML is a malignant disease of bone marrow characterized by maturational arrest of hematopoietic precursors at an early stage of development. Clonal expansion of myeloid blasts occurs in bone marrow, blood, and other tissue. Myelogenous leukemias develop from changes in cells that normally produce neutrophils, basophils, eosinophils and monocytes.
GTPase-activating protein for RAC1 and perhaps Cdc42, but not for RhoA small GTPase. May attenuate RAC1 signaling in neurons
Medicamentos e terapias
Mecanismo: Interferon alpha/beta receptor agonist
Mecanismo: DNA topoisomerase II inhibitor
Mecanismo: DNA topoisomerase II alpha inhibitor
Mecanismo: Dual specificity mitogen-activated protein kinase kinase; MEK1/2 inhibitor
Mecanismo: Dual specificity mitogen-activated protein kinase kinase 2 inhibitor
Mecanismo: RAF serine/threonine protein kinase inhibitor
Mecanismo: FK506-binding protein 1A inhibitor
Mecanismo: Epidermal growth factor receptor erbB1 inhibitor
Variantes genéticas (ClinVar)
507 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 7 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
83 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 — Astrocitoma pilocítico
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Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 1.114
High-Grade Astrocytoma With Piloid Features: An Aggressive Clinicogenomic Entity Distinct From Pilocytic Astrocytoma.
High-grade astrocytoma with piloid features (HGAP) has recently emerged as an aggressive glioma entity with distinct molecular alterations, yet its clinicogenomic distinction from pilocytic astrocytoma (PA) remains to be fully elucidated. This study aims to clarify the clinical, pathological, and genomic differences between pediatric PA, adult PA, and HGAP, and to provide evidence supporting the recognition of HGAP as a new, aggressive entity. We retrospectively analyzed 100 genetically and histopathologically confirmed PA cases (87 pediatric, 13 adult) and 25 HGAP cases (all > 19 years old) diagnosed at Seoul National University Hospital between 2015 and 2024. Next-generation sequencing using a brain tumor-specific gene panel and immunohistochemistry evaluation. Pediatric PAs (median age 7 years) were predominantly cerebellar (61%) and showed classic biphasic histology (72%) with frequent KIAA1549-BRAF fusion (72%) and BRAF V600E mutations (13%) and rarely KRAS mutation (2.3%). Adult PAs (median age 35 years), when HGAP was excluded, were less often cerebellar (53.8%) and rarely KRAS mutation (2.3%), more frequently supratentorial (23%) or spinal (15%) than pediatric PAs, and showed a higher incidence of KRAS mutations (23.1%), and more patternless or diffuse oligoastrocytic histology (31%), but did not differ in recurrence rate or prognosis compared to pediatric PA. In contrast, HGAPs predominantly affected adults (median age 53 years, ranges: 19-87 years), frequently involved cerebellum (40%), and exhibited high-grade histopathological features. Molecular profiling revealed HGAPs harbored frequent CDKN2A/B deletions (76%) and mutations of NF1 (64%), ATRX (52%), PTPN11 (28%), FGFR1/FGFR4 (20%), TERTp (16%), and TP53 (16%). Patients with HGAP had significantly shorter progression-free and overall survival compared to both pediatric and adult PA. HGAP represents a clinically aggressive and molecularly distinct high-grade glioma, clearly separable from pediatric and adult PA. Its poor prognosis and unique genetic drivers justify its recognition as a new entity. Accurate molecular profiling is essential for diagnosis and management of these tumors, and the poor survival outcomes observed in HGAP highlight the need for further larger cohort studies to identify optimal therapeutic strategies.
Fourth ventricular pilocytic astrocytoma mimicking an arachnoid cyst: A case report and review of literature.
Arachnoid cysts and pilocytic astrocytomas (PAs) are pathologically distinct lesions. Arachnoid cysts are essentially cerebrospinal fluid (CSF)-filled collections lined by the arachnoid membrane, mostly congenital but sometimes developing later in life. In contrast, PAs are low-grade, solid-cum-cystic brain tumors arising from astrocytes that mainly affect children and young adults. Fourth ventricular PAs are exceptionally rare, with approximately 56 cases reported in the literature since the early 1990s. We report a case of a 20-year-old female who presented with progressive headache, nausea, and gait imbalance. Magnetic resonance imaging (MRI) revealed a nonenhancing cystic lesion in the fourth ventricle, initially suspected to be an arachnoid cyst due to the absence of mural nodules or enhancing solid components. Given her symptomatic presentation, she underwent a midline suboccipital craniotomy with cyst fenestration. Intraoperatively, the appearance of the cyst did not resemble an arachnoid cyst; hence, the cyst wall was sent for histopathological evaluation, which confirmed the diagnosis of PA. Postoperatively, the patient experienced significant improvement in her symptoms, and her neurological examination normalized. Follow-up MRI at 1 year demonstrated no evidence of progression. This case emphasizes the diagnostic difficulty of distinguishing cystic, nonenhancing PAs from arachnoid cysts on imaging alone, particularly in the fourth ventricle of adults. Our findings highlight the critical role of surgical exploration both for symptom relief and for obtaining tissue to establish a definitive diagnosis, as well as the importance of long-term radiological surveillance due to the limited resection achievable in this anatomically constrained region.
Detection of Tumor-Specific Variants in CSF of Children With Brain Tumors, Including Low-Grade and Cytology-Negative Cases.
The genetic diagnosis of brain tumors necessitates the direct collection of tumor tissue. Recently, genetic diagnosis using cell-free DNA (cfDNA) from plasma has been clinically applied to some somatic cancers, but it has not been established for brain tumors. This study conducted a genetic diagnosis via liquid biopsy (LB) using cfDNA extracted from the plasma and cerebrospinal fluid (CSF) of pediatric brain tumor patients. The study included children who underwent surgical resection or biopsy for brain tumors between January 2019 and December 2020. Tumor-specific variants were identified using whole-exome sequencing of genomic DNA from tumor samples and normal blood cells. Subsequently, CSF and plasma collected during surgery were analyzed for these specific variants using droplet digital PCR. Tumor-specific variants were detected in six of 13 (46.2%) CSF samples. In contrast, no tumor-specific variants were detected in plasma in any patient. Among the six CSF-positive cases, two had negative or inconclusive CSF cytology (Class I or II), and one was a case of low-grade pilocytic astrocytoma. The median variant allele frequency in the positive CSF samples was 16.0% (range: 0.6%-86.1%). In certain pediatric brain tumors, genetic diagnosis using cfDNA from CSF demonstrated promising potential. This approach has the potential for diagnosis in cases with negative cytology or low-grade tumors. However, several issues must be addressed before this method can achieve clinical utility, including its low sensitivity and the extended turnaround time for analysis.
FGFR1:TACC1-Fused Pilocytic Astrocytoma in a Child With Muenke Syndrome: A Case Report.
Molecularly Informed Combination Therapy for BRAF-KIAA1549 Fusion-Positive Pilocytic Astrocytoma in a Resource-Limited Setting.
Publicações recentes
Intraoperative Differentiation of Pilocytic Astrocytoma and Hemangioblastoma with Indocyanine Green Video Angiography.
A case report of postoperative atlantoaxial rotatory subluxation following craniotomy for pilocytic astrocytoma in a pediatric patient.
Radiotherapy for Refractory Cystic Progression in Basal Ganglia Pilocytic Astrocytoma: A Case Report.
Bridging surgery and radiotherapy: the role of stereotactic radiosurgery in pediatric brain tumor care.
Intrinsic pontine tumors: Pathology of brainstem tumors and exploration of diffuse intrinsic pontine glioma.
📚 EuropePMC812 artigos no totalmostrando 197
Nested pediatric low-grade glioma cerebral organoid avatars reveal glutamatergic neuron stromal growth dependency.
Genes & developmentStereotactic radiosurgery for pilocytic astrocytoma: A single center retrospective study.
Neurosurgical reviewPilocytic astrocytoma surgery via the pineal region in a 9-year-old boy via the infratentorial-supracerebellar approach.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryCase Report: Molecular diagnostics and clinical courses of two adult spinal pilocytic astrocytoma long-term survivors with GTF2I::BRAF fusion.
Frontiers in oncologyFGFR1:TACC1-Fused Pilocytic Astrocytoma in a Child With Muenke Syndrome: A Case Report.
Pediatric blood & cancerBRAFV600E-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)High-Grade Astrocytoma With Piloid Features: An Aggressive Clinicogenomic Entity Distinct From Pilocytic Astrocytoma.
Journal of Korean medical scienceDifferentiating medulloblastoma and pilocytic astrocytoma in children based on multimodal MRI radiomics model.
NeuroradiologyPrimary Pediatric Brain Tumors in a Tertiary Referral Center in Iran: A 13-Year Retrospective Analysis.
Archives of Iranian medicineMultifocal cerebral and orbital neoplastic lesions in a 2-year-old child: a case report and literature review emphasizing diagnostic uncertainty and ethical decision-making.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryA Systematic Review and Meta-Analysis of Executive Function Outcomes in Pediatric Central Nervous System Tumor Survivors.
Actas espanolas de psiquiatriaPediatric Pilocytic Astrocytoma, Rich in Oligodendrocyte-Like Cells: A Diagnostic Challenge.
Journal of pediatric hematology/oncologySurgical Management of Optic Pathway-Hypothalamic Gliomas: Institutional Experience and Systematic Review.
Operative neurosurgery (Hagerstown, Md.)Pilocytic astrocytoma in pediatric and adult patients: a single-center analysis of 650 cases.
Virchows Archiv : an international journal of pathologySpontaneous contrast enhancement fluctuation in adult pilocytic astrocytoma.
RadiologiaFourth ventricular pilocytic astrocytoma mimicking an arachnoid cyst: A case report and review of literature.
Surgical neurology internationalCase Report: Novel ASAP1::BRAF fusion in a young adult with low-grade temporal lobe glioma.
Frontiers in oncologyNeuro-Ophthalmic Manifestations of Suprasellar Pilomyxoid Astrocytoma.
Neuro-ophthalmology (Aeolus Press)Pediatric low-grade epilepsy-associated tumors (LEATS): neuroimaging review and genetics update.
Pediatric radiologyPhase II Study of Dabrafenib and Trametinib in Patients With Tumors With BRAFV600E Mutations: Updated Results From NCI-MATCH ECOG-ACRIN Trial (EAY131) Subprotocol H.
JCO precision oncologyPilocytic Astrocytoma: An institutional perspective over the last decade.
Pakistan journal of medical sciences[Polymorphous low-grade neuroepithelial tumor of the young: a molecular pathological study].
Zhonghua bing li xue za zhi = Chinese journal of pathologyUnusual localization of pilocytic astrocytoma at the foramen of Monro mimicking a colloid cyst: a case report and literature review.
Frontiers in oncologyThe contribution of methylation profiling in neuropathological diagnosis of central nervous system tumors in children, adolescent and young adults.
PathologicaCerebellar pilocytic astrocytoma in a patient with autism spectrum disorder and psychotic symptoms: a case report.
BMC psychiatryDetection of Tumor-Specific Variants in CSF of Children With Brain Tumors, Including Low-Grade and Cytology-Negative Cases.
Pediatric blood & cancerBiopsy and laser ablation as a treatment strategy for pediatric brain incidentalomas.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryUnpredictable Evolution of Pilocytic Astrocytoma in Adults: A Case Series and Diagnostic Challenges.
The American journal of case reportsUnexpected [18F] Fluciclovine Uptake in Pilocytic Astrocytoma: A Pediatric Case Report.
Clinical nuclear medicineMismatch Repair Deficiency in Gliomas: A Rare Insight into Microsatellite Instability and Its Diagnostic Implications.
Asian journal of neurosurgeryCase Report: FGFR1 mutation and massive chromosome loss drive malignant transformation of low-grade gliomas.
Frontiers in oncologyPrecise diagnosis of pediatric posterior cranial fossa neoplasms based on 2.5D MRI deep learning.
Frontiers in oncologyTen years of pediatric low-grade glioma care in Kenya: Outcomes, progress, and gaps toward achieving global childhood cancer equity.
Neuro-oncology pediatricsCircumscribed Pilocytic Astrocytoma Including Pilomyxoid and Intermediate Pilomyxoid Variants - A Single Institutional, Retrospective Study.
Indian journal of surgical oncologyNeuro-ophthalmic clues affecting the surgical approach to a brain tumor: the case of a craniopharyngioma masquerader.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryGlutamate promotes glioma growth via a non-excitable, receptor tyrosine kinase-mediated mechanism.
NeuronExpanding morphological spectrum of pilocytic astrocytomas: insights from integrated histopathological and multiomic profiling.
Journal of neuro-oncologyMolecularly Informed Combination Therapy for BRAF-KIAA1549 Fusion-Positive Pilocytic Astrocytoma in a Resource-Limited Setting.
Pediatric blood & cancerHigh-grade gliomas associated with neurofibromatosis type 1: analysis of imaging features and literature review.
Polish journal of radiologyChemotherapy in adult patients with pilocytic astrocytoma: a retrospective multicenter cohort study.
Journal of neuro-oncologyThe transient external granular layer in human foetal cerebellum: insights into neurogenesis, genetic regulation, and developmental disorders.
Anatomy & cell biologyExamining the Impact of Time-to-Treatment on Disease-Specific Survival in Pediatric Pilocytic Astrocytoma: A Multi-Institutional Retrospective Cohort Analysis.
Pediatric blood & cancerCHALLENGES IN RADIOLOGICAL DIAGNOSIS: CRANIOPHARYNGIOMA VS ASTROCYTOMA.
Georgian medical newsEvolution of pilocytic astrocytoma to diffuse leptomeningeal glioneuronal tumor (DLGNT): bridging two distinct tumor types.
Acta neuropathologicaSustained response to larotrectinib in a pediatric patient with recurrent STRN3::NTRK2 fusion-positive pilocytic astrocytoma.
CNS oncologyTargeted tumor resection to reestablish cerebrospinal fluid flow in the setting of a pediatric third ventricular glioma with obstructive hydrocephalus.
Surgical neurology internationalBilateral Pterygium in a Paediatric Cancer Patient Taking Vemurafenib: A Case Report.
Case reports in ophthalmologyAdult pilocytic astrocytomas: challenging the benign paradigm with surgical risks, recurrence dynamics, and molecular insights.
Neurosurgical reviewCoexistence of IDH1 Mutation and KIAA1549::BRAF Fusion in a Diffuse Glioma: A Case Report With Clinical, Radiological, and Pathological Correlation.
Neuropathology : official journal of the Japanese Society of NeuropathologySurgical Strategy for Superior Cerebellar Peduncle Lesions: Utility of the Subtemporal Transtentorial Approach.
World neurosurgeryHigh-grade astrocytoma with piloid features resected with an exoscopic supracerebellar infratentorial approach: illustrative case.
Journal of neurosurgery. Case lessonsThe Transventricular Preforniceal Approach Can Be Used in Cases Where the Space between the Anterior Commissure and the Venous Angle Is Enlarged.
Neurologia medico-chirurgicaAberrant coupling of glutamate and tyrosine kinase receptors enables neuronal control of brain-tumor growth.
NeuronImpact 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 NeurosurgeryVolumetric amide-proton-transfer weighted imaging histogram analysis to differentiate pediatric high-grade and low-grade gliomas.
BMC cancerStrabismus fixus convergens secondary to protracted hydrocephalus.
BMJ case reportsClinical and genomic factors differ in pediatric and adult pilocytic astrocytoma: a single-center experience with over 350 patients.
Journal of neuro-oncologyA Single Motor Donor Nerve for Two Functional Free Flaps in Facial Reanimation.
Plastic and reconstructive surgery. Global openChallenges in diagnosing pediatric pilocytic Astrocytoma around the foramen of monro: the potential imaging pitfalls.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyMultimodal analysis of pediatric pilocytic astrocytomas reveals tumor location-associated cellular and transcriptional heterogeneity.
Neuro-oncologyDoes D-wave predict laterality? A case report and video demonstrating use of D-wave intraoperative neuromonitoring in surgery of intramedullary spinal cord tumor to predict laterality of post-operative neurological deficit.
European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research SocietyMolecular diagnostic factors, prognostic implications, and long-term outcomes in pediatric pilocytic astrocytoma.
Journal of neurosurgery. PediatricsCauses of death among patients with primary malignant brain tumors in the US from 2000 to 2021.
European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical OncologyMulti-pronged analysis of pediatric low-grade glioma and ganglioglioma reveals a unique tumor microenvironment associated with BRAF alterations.
Brain pathology (Zurich, Switzerland)Pineal region collision tumor.
Journal of surgical case reportsPseudoprogression in Pediatric Spinal Pilocytic Astrocytoma and Myxopapillary Ependymoma after Proton Therapy: A Case Series.
Pediatric blood & cancerMinimally Invasive and Cost-Effective Access to Deep-Seated Intracranial Lesions Using 19F Peel-Away Sheath Introducer and "Dynamic" Retraction: Technical Note and a Case Series.
Operative neurosurgery (Hagerstown, Md.)38-Year Delayed Spinal Leptomeningeal Dissemination of a Paediatric Pilocytic Astrocytoma: A Case Report.
Case reports in oncologyPituitary Blastoma: Expanding the Spectrum of Histopathological Findings in a Young Adult With DICER1 Mutation.
Neuropathology : official journal of the Japanese Society of NeuropathologyComprehensive transcriptomic profiling of fusions and abnormal variant transcripts in pilocytic astrocytoma using NanoString nCounter technology.
Pathology, research and practiceAggressive behavior in a molecularly benign tumor: The diagnostic odyssey of a pediatric pilocytic astrocytoma with initial high-grade histologic features and rapid recurrence.
Journal of neuropathology and experimental neurologyYield of MRI in patients with spontaneous deep intracerebral hemorrhage.
Emergency radiologyInclusion of Optic Pathway and Hypothalamic Tumors in Prognostic Studies of Pilocytic Astrocytoma: Is It Justified?
Pediatric blood & cancerEndoscopic transorbital approach with electrocorticography for resection of anterior temporal lobe mass.
Neurosurgical focus: VideoStereotactic laser ablation for pediatric central nervous system tumors: a systematic review and meta-analysis of the literature.
Journal of neurosurgery. PediatricsReclassification of pineal tumor as high-grade astrocytoma with piloid features through methylation profiling: illustrative case.
Journal of neurosurgery. Case lessonsPilocytic astrocytoma with BARD1 mutation: oncogenic pathways via DNA damage repair mechanism and MAPK/BRAF.
PathologyMibrain cystic pilocytic astrocytoma.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaPulmonary embolism of haemostatic material during paediatric neurosurgery.
Anaesthesia reportsHypertrophic olivary degeneration after posterior cranial fossa tumor resection in adults: illustrative case.
Journal of neurosurgery. Case lessonsMR Imaging Features of Juvenile Pilocytic Astrocytoma in the Suprasellar Region: A Study on 11 Patients.
Current medical imagingA phase 2 PBTC study of selumetinib for recurrent/progressive pediatric low-grade glioma: Strata 2, 5, and 6 with long-term outcomes on strata 1, 3, and 4.
Neuro-oncologyIRX2 and NPTX1 differential regulation of β-catenin underlies MEK-mediated proliferation in human neuroglial cells.
Genes & developmentSurvival outcomes and prognostic factors in surgically treated adult pilocytic astrocytomas.
Frontiers in oncologyEpilepsy in patients with pediatric brain tumors: Etiology, treatment & management.
Seminars in pediatric neurologyDeciphering Metabolic Alterations Associated with Glioma Grading Using Hyperspectral Stimulated Raman Scattering Imaging.
Analytical chemistryHigh-grade astrocytoma with piloid features: case report and systematic review.
Zhurnal voprosy neirokhirurgii imeni N. N. BurdenkoIntraventricular Pilocytic Astrocytoma in a 75-Year-Old Adult Patient: A Case Report.
CureusSudden Unexpected Death Due to Undiagnosed Pilocytic Astrocytoma.
The American journal of forensic medicine and pathologyDiagnostic accuracy of MRI without gadolinium for follow-up of pilocytic astrocytoma in the paediatric population.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaSpontaneous regression of epileptogenic pilocytic astrocytoma with FGFR1-TACC1 fusion.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryCharacterizing the Natural History of Pediatric Brain Tumors Presenting with Metastasis.
CancersHigh-grade astrocytoma with piloid features: A case report and review of literature.
Surgical neurology internationalA case report on a late diagnosis of pilocytic astrocytoma in a Dandy-Walker complex.
NeurocirugiaPilocytic astrocytoma: A rare case report.
Radiology case reportsPilocytic astrocytoma in a child with spinal muscular atrophy treated with onasemnogene abeparvovec.
Molecular therapy : the journal of the American Society of Gene TherapyEndoscopic-Assisted Microsurgical Removal of a Tectal Plate Pilocytic Astrocytoma Through the Occipital Inter Hemispheric Transtentorial Approach: 2-Dimensional Operative Video.
Operative neurosurgery (Hagerstown, Md.)The tumour microenvironment of pilocytic astrocytoma evolves over time via enrichment for microglia.
Acta neuropathologica communicationsThe Case Series of Contralateral Interhemispheric Transfalcine Approach to Medial Parietooccipital Pathologies: Surgical Technique and Results.
Operative neurosurgery (Hagerstown, Md.)Chronic headache and obstructive hydrocephalus: A case of pilocytic astrocytoma in the third ventricle with detailed radiological and histopathological findings.
Radiology case reportsHigh-Grade Astrocytoma With Piloid Features: Case Series and Review of a Recently Described Brain Tumor Entity.
Archives of pathology & laboratory medicineMotor mapping-guided resection of a brainstem recurrent pilocytic astrocytoma.
Neurosurgical focus: VideoBRAF inhibitor monotherapy in BRAFV600E-mutated pediatric low-grade glioma: a single center's experience.
Frontiers in oncologyIdiopathic Optic Nerve Glioma: A Case Report.
CureusEndoscopic endonasal approach for optic Pathway-Hypothalamic Glioma: A pediatric case Report and a Systematic review.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaLow-grade gliomas presenting with hemorrhage: Are intratumoral blood products associated with malignant transformation?
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaOptic nerve glioma successfully treated with definitive intensity-modulated radiotherapy in a limited-resource setting: a case report.
EcancermedicalscienceIncreased SOX10, p16, and Cyclin D1 Immunoreactivity Differentiates MAP Kinase-activated Low-grade Gliomas From Piloid Gliosis.
The American journal of surgical pathologyImpact of Pediatric Posterior Fossa Tumor Treatments on Working Memory Tracts Using Resting-State fMRI and Tractography.
Journal of neuroimaging : official journal of the American Society of NeuroimagingIntracranial hydatid cyst in a pediatric patient: a rare mimic of brain malignancy.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryCerebellar pilocytic astrocytoma: predictors of recurrence based on MRI morphology-a single-centre experience.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryA bibliometric analysis of research trends and hotspots of pilocytic astrocytoma from 2004 to 2023.
Neurosurgical reviewIntegrating molecular analyses with the 2021 WHO classification of adult pilocytic astrocytomas.
Journal of neuropathology and experimental neurologyImpact of molecular classification on prognosis in children and adolescents with spinal ependymoma: Results from the HIT-MED database.
Neuro-oncology advancesA Fusion Model of MRI Deep Transfer Learning and Radiomics for Discriminating between Pilocytic Astrocytoma and Adamantinomatous Craniopharyngioma.
Academic radiologyHolocord intramedullary pilocytic astrocytoma mimicking holocord spinal abscess: a case report and literature review.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryRadiosurgery for Hypothalamic Gliomas: A Case Report and Clinical Guidelines Form a Neurosurgical Center of Excellence.
Journal of personalized medicineTowards a Risk-Based Follow-Up Surveillance Imaging Schedule for Children and Adolescents with Low-Grade Glioma.
Current oncology (Toronto, Ont.)A glioneuronal tumor with neurocytic rosettes harboring FGFR1 internal tandem duplication - A report of a unique case.
Neuropathology : official journal of the Japanese Society of NeuropathologyGamma Knife Radiosurgery for symptomatic eloquently deep-seated cystic pilocytic astrocytoma mural nodules: Retrospective case series of effective outcomes.
Acta neurochirurgicaDiagnostic Utility of Diffusion-Weighted Imaging in Distinguishing Common Pediatric Posterior Fossa Tumors: A Single Center Retrospective Study.
Turkish archives of pediatricsCerebellopontine angle pilocytic astrocytoma in adults: A systematic review.
Surgical neurology internationalHigh-grade astrocytoma with piloid features in the conus medullaris: a rare presentation of a new World Health Organization diagnosis. Illustrative case.
Journal of neurosurgery. Case lessonsCervical Pilocytic Astrocytoma Mimicking Spondylotic Myelopathy: A Case Report and Literature Review.
CureusFreiburg Neuropathology Case Conference : Posterior fossa tumour 15 years after microsurgical resection of a cerebellar pilocytic astrocytoma.
Clinical neuroradiologyRadiological Hypertrophic Olivary Degeneration Following Posterior Fossa Tumor Surgery.
World neurosurgeryInfantile pilocytic astrocytoma with persisting external granular layer of the cerebellum: a potential diagnostic pitfall.
Free neuropathologyTectal glioma: clinical, radiological, and pathological features, and the importance of molecular analysis.
Brain tumor pathologyEfficacy of various extent of resection on survival rates of patients with pilocytic astrocytoma: based on a large population.
Scientific reportsIntracranial tumor in a patient with mucopolysaccharidosis type 1 (Scheie syndrome): An extremely rare combination.
HeliyonMolecular markers for pediatric low-grade glioma.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryAll-in-one bimodal DNA and RNA next-generation sequencing panel for integrative diagnosis of glioma.
Pathology, research and practiceA High-Grade Glioma, Not Elsewhere Classified in an Older Adult with Discordant Genetic and Epigenetic Analyses.
BiomedicinesA pilocytic astrocytoma with novel ATG16L1::NTRK2 fusion responsive to larotrectinib: a case report with genomic and functional analysis.
The oncologistSymptomatic cerebral vasospasm after posterior fossa surgery in pediatric patients: single-center study and systematic literature review.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryAnalysis of Spinal Pilocytic Astrocytoma in 12 Case Reports and Literature Review.
Journal of the Belgian Society of RadiologyPilomyxoid Astrocytoma Presenting With Developmental Regression: A Case Report.
CureusMedulloblastoma in a 13-Year-Old Female: A Comprehensive Case Report.
CureusThe Role of Radiotherapy, Chemotherapy, and Targeted Therapies in Adult Intramedullary Spinal Cord Tumors.
CancersBrazilian pediatric patients with gliomas: treatment characteristics and survival outcomes.
Reports of practical oncology and radiotherapy : journal of Greatpoland Cancer Center in Poznan and Polish Society of Radiation OncologyHistopathological Spectrum of Gliomas and Its Immunohistochemical Correlation in a Tertiary Care Setup.
CureusPrimary mismatch repair-deficient IDH-mutant astrocytoma in child: Unusual entity.
International journal of surgery case reportsAn Extremely Rare Case of Collision Tumor: A Craniopharyngioma Coexists Pilocytic Astrocytoma.
World neurosurgeryPediatric glioma immune profiling identifies TIM3 as a therapeutic target in BRAF fusion pilocytic astrocytoma.
The Journal of clinical investigationRapid postradiation malignant transformation of a pilocytic astrocytoma in an adult with neurofibromatosis type 1: illustrative case.
Journal of neurosurgery. Case lessonsIdentification and validation of miRNA-target genes network in pediatric brain tumors.
Scientific reportsBeyond childhood: exploring the state of transitional care in pediatric pilocytic astrocytoma.
Neurosurgical focusMachine Learning Analysis in Diffusion Kurtosis Imaging for Discriminating Pediatric Posterior Fossa Tumors: A Repeatability and Accuracy Pilot Study.
CancersDissecting the Natural Patterns of Progression and Senescence in Pediatric Low-Grade Glioma: From Cellular Mechanisms to Clinical Implications.
CellsDoing more with less: surgical results of pediatric posterior fossa tumors from a single center in Latin America.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryUnraveling morphology, methylation profiling, and diagnostic challenges in BRAF-Mutant pediatric glial and glioneuronal tumors.
Neurosciences (Riyadh, Saudi Arabia)Critical domains for NACC2-NTRK2 fusion protein activation.
PloS oneHypothalamic Hemangioma-like Pilocytic Astrocytoma in an Adult Patient: A Systematic Review with a Focus on Differential Diagnosis and Neurological Presentation.
Journal of clinical medicineArterial Spin-Labeling Perfusion Lightbulb Sign: An Imaging Biomarker of Pediatric Posterior Fossa Hemangioblastoma.
AJNR. American journal of neuroradiologyCase report: Pediatric low-grade gliomas: a fine balance between treatment options, timing of therapy, symptom management and quality of life.
Frontiers in oncologyHolocord pilocytic astrocytoma in a young woman with intracranial extension: case report and review of the MRI characteristics.
Spinal cord series and casesMEK Inhibition in a Pilocytic Astrocytoma With a Rare KRAS Q61R Mutation in a Young Adult Patient: A Case Report.
JCO precision oncologyA comprehensive evaluation of imaging features in pediatric spinal gliomas and their value in predicting tumor grade and histology.
NeuroradiologySolid pilocytic astrocytoma in cavum septum pellucidum: a description of two cases and an analysis of misdiagnosis.
Quantitative imaging in medicine and surgerySuprasellar pilocytic astrocytoma with leptomeningeal dissemination.
SA journal of radiologySpinal cord anaplastic Pilocytic Astrocytoma - two stage resection with elsberg and beer technique. Case report and literature review.
British journal of 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 NeurosurgeryDabrafenib for Pilocytic Astrocytoma With BRAF V599ins.
JCO precision oncologyObstructive Hydrocephalus Due to Aggressive Posterior Fossa Tumor Exhibiting Histological Characteristics of Pilocytic Astrocytoma in Two Adult Neurofibromatosis Type 1 (NF1) Cases.
CureusThe role of reoperation in pediatric cerebellar pilocytic astrocytoma.
Journal of neurosurgery. PediatricsDetection of tumor-derived cell-free DNA in cerebrospinal fluid using a clinically validated targeted sequencing panel for pediatric brain tumors.
Journal of neuro-oncologyRapid identification of pediatric brain tumors with differential mobility spectrometry.
Frontiers in oncologyEvaluation of KIAA1549::BRAF fusions and clinicopathological insights of pilocytic astrocytomas.
Annals of diagnostic pathologyThe role of focused ultrasound for pediatric brain tumors: current insights and future implications on treatment strategies.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryThe Spectrum of Central Nervous System Tumors at a Tertiary Care Center Primarily Serving a Rural Population.
Cureus[Pilocytic astrocytoma with KRAS gene mutation: a clinicopathological analysis of two cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyHuman-Level Differentiation of Medulloblastoma from Pilocytic Astrocytoma: A Real-World Multicenter Pilot Study.
CancersModular Hub Genes in DNA Microarray Suggest Potential Signaling Pathway Interconnectivity in Various Glioma Grades.
BiologyRole of sodium fluorescein in pediatric low-grade glioma surgery: an update.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryJuvenile pilocytic astrocytoma in a child with a prior normal neonatal brain MRI.
BMJ case reportsMulti-pronged analysis of pediatric low-grade glioma reveals a unique tumor microenvironment associated with BRAF alterations.
bioRxiv : the preprint server for biologyRare vermian pilocytic astrocytoma with recurrent spontaneous hemorrhage in the elderly: A case report and review of literature.
Surgical neurology internationalComparison of MRI findings of hypothalamic-optic chiasmatic gliomas and craniopharyngiomas.
Acta radiologica (Stockholm, Sweden : 1987)Pilocytic Astrocytoma of the fourth ventricle: A case report.
Asian journal of surgeryPediatric-Like Brain Tumors in Adults.
Advances and technical standards in neurosurgeryRare Coexistence: Pilocytic Astrocytoma With Atypical Teratoid/Rhabdoid Tumor Features in an Infant.
CureusIntraventricular Pilocytic Astrocytoma in Adults: A 25-year Single-Center Case Series and Systematic Review of the Literature.
World neurosurgeryResection of intradural spinal lesions with concomitant instrumented fusion in children: a systematic review and representative cases.
Journal of neurosurgery. PediatricsEpidemiology and Anatomical Distribution of Primary Brain Tumors Among Children in Palestine: A 6-Year National Referral Institution Study.
World neurosurgeryEndoscopic transorbital approach in children: surgical technique and early results.
Neurosurgical focusComprehensive Transcriptomic Profiling of Diverse Brain Tumor Types Uncovers Complex Structures of the Brain Tumor Microenvironment.
BiomedicinesProteomic Profiling of Cerebrospinal Fluid and Its Extracellular Vesicles from Extraventricular Drainage in Pediatric Pilocytic Astrocytoma, towards Precision Oncology.
CancersRadiological characterization of pediatric intramedullary astrocytomas: Do they differ from adults?
Brain & spineNoise suppression of proton magnetic resonance spectroscopy improves paediatric brain tumour classification.
NMR in biomedicineGene Expression of CSF3R/CD114 Is Associated with Poorer Patient Survival in Glioma.
International journal of molecular sciencesSocio-demographic characteristics of children and young people with primary brain tumours: comparison between a public and private sector tertiary hospital in Karachi, Pakistan.
JPMA. The Journal of the Pakistan Medical AssociationA case report of a child with pulmonary hypertension associated with SARS-CoV-2 infection.
Frontiers in pediatricsClinicopathological analysis of rosette-forming glioneuronal tumors.
Diagnostic pathologyPilocytic astrocytoma: The paradigmatic entity in low‑grade gliomas (Review).
Oncology lettersThe Endonasal Endoscopic Transsphenoidal Approach to Paediatric Optic Chiasma Glioma: A Case Report and Literature Review.
CureusBrain stem tumors in children less than 3 months: Clinical and radiologic findings of a rare disease.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryThe potential of liquid biopsy for detection of the KIAA1549-BRAF fusion in circulating tumor DNA from children with pilocytic astrocytoma.
Neuro-oncology advancesAssociações
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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.
- High-Grade Astrocytoma With Piloid Features: An Aggressive Clinicogenomic Entity Distinct From Pilocytic Astrocytoma.
- Fourth ventricular pilocytic astrocytoma mimicking an arachnoid cyst: A case report and review of literature.
- Detection of Tumor-Specific Variants in CSF of Children With Brain Tumors, Including Low-Grade and Cytology-Negative Cases.
- FGFR1:TACC1-Fused Pilocytic Astrocytoma in a Child With Muenke Syndrome: A Case Report.
- Molecularly Informed Combination Therapy for BRAF-KIAA1549 Fusion-Positive Pilocytic Astrocytoma in a Resource-Limited Setting.
- Intraoperative Differentiation of Pilocytic Astrocytoma and Hemangioblastoma with Indocyanine Green Video Angiography.
- A case report of postoperative atlantoaxial rotatory subluxation following craniotomy for pilocytic astrocytoma in a pediatric patient.
- Radiotherapy for Refractory Cystic Progression in Basal Ganglia Pilocytic Astrocytoma: A Case Report.
- Bridging surgery and radiotherapy: the role of stereotactic radiosurgery in pediatric brain tumor care.
- Intrinsic pontine tumors: Pathology of brainstem tumors and exploration of diffuse intrinsic pontine glioma.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:251612(Orphanet)
- MONDO:0016691(MONDO)
- GARD:9808(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q2095252(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
