É um tipo de câncer agressivo que nasce no plexo coroide, uma estrutura que existe dentro do cérebro. As células desse tumor são muito alteradas e crescem rapidamente, demonstrando grande agressividade. Geralmente, ele invade as estruturas cerebrais próximas. É comum que o câncer se espalhe para outras áreas através do líquido cefalorraquidiano (o líquor), que envolve o cérebro e a medula espinhal.
Introdução
O que você precisa saber de cara
É um tipo de câncer agressivo que nasce no plexo coroide, uma estrutura que existe dentro do cérebro. As células desse tumor são muito alteradas e crescem rapidamente, demonstrando grande agressividade. Geralmente, ele invade as estruturas cerebrais próximas. É comum que o câncer se espalhe para outras áreas através do líquido cefalorraquidiano (o líquor), que envolve o cérebro e a medula espinhal.
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
1 gene identificado com associação a esta condição. Padrão de herança: Autosomal dominant.
Multifunctional transcription factor that induces cell cycle arrest, DNA repair or apoptosis upon binding to its target DNA sequence (PubMed:11025664, PubMed:12524540, PubMed:12810724, PubMed:15186775, PubMed:15340061, PubMed:17317671, PubMed:17349958, PubMed:19556538, PubMed:20673990, PubMed:20959462, PubMed:22726440, PubMed:24051492, PubMed:24652652, PubMed:35618207, PubMed:36634798, PubMed:38653238, PubMed:9840937). Acts as a tumor suppressor in many tumor types; induces growth arrest or apop
CytoplasmNucleusNucleus, PML bodyEndoplasmic reticulumMitochondrion matrixCytoplasm, cytoskeleton, microtubule organizing center, centrosome
Variantes genéticas (ClinVar)
1,725 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 6 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
30 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 — Carcinoma do plexo coroide
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Outros ensaios clínicos
13 ensaios clínicos encontrados, 4 ativos.
Publicações mais relevantes
Risk Factors for Delayed Leptomeningeal Dissemination in Choroid Plexus Papillomas: A Systematic Review and Illustrative Case.
Choroid plexus papilloma (CPP) is a rare, benign intraventricular tumor that typically has an excellent long-term survival rate after gross total resection. Nevertheless, some patients develop delayed leptomeningeal dissemination (LMD) years after the initial diagnosis, a phenomenon that remains poorly understood. The authors encountered a case of CPP with delayed LMD 14 years after resection, which prompted this systematic review to identify potential risk factors. A systematic review was conducted according to PRISMA guidelines to identify CPP reports with subsequent delayed LMD. Studies of atypical CPP, choroid plexus carcinoma, or concurrent LMD at diagnosis were excluded. Extracted variables included demographics, tumor location, extent of resection, recurrence, proliferative index, and latency to dissemination. Thirty patients developed delayed LMD after initial diagnosis. Seventeen patients underwent gross total resection, ten underwent subtotal resection, and three were unreported. Delayed LMD occurred with tumor recurrence in 14 patients. Histological transformation was observed in nine patients: eight progressed to aCPP and one to CPC. The extent of resection, recurrence, and tumor transformation were not significantly associated with delayed LMD. The pathogenesis and optimal treatment strategy of this phenomenon remain unclear. No significant risk factors for delayed LMD development in CPP were identified. Future studies incorporating molecular characterization are needed to clarify the mechanisms of LMD in patients with CPP and to improve risk stratification, underscoring the importance of lifelong surveillance and the integration of molecular profiling in clinical management.
Neoadjuvant Chemotherapy in Choroid Plexus Carcinoma: Improving Surgical Safety and Resection Outcomes.
Choroid plexus carcinoma (CPC) is a rare and highly vascular malignant brain tumour that occurs mainly in children. Complete surgical resection offers the best chance of long-term survival, but this is often difficult due to excessive intraoperative bleeding. Preoperative (neoadjuvant) chemotherapy has been explored as a strategy to shrink the tumour and reduce its vascularity before surgery. In this review, we have summarized the available literature on preoperative chemotherapy in CPC and its effect on surgical outcomes. Reported cases and small series show that platinum- and etoposide-based regimens can decrease tumour size and intraoperative blood loss, allowing safer and more complete resections. However, due to the rarity of CPC and limited data, larger multicentre studies are required to validate these findings and establish standardized treatment protocols.
Phase I/II study of the PARP inhibitor olaparib and irinotecan in children and young adults with recurrent/refractory malignancies: Arm D of the AcSé-ESMART trial.
Arm D of the AcSé-ESMART proof-of-concept phase I/II platform trial aimed to define the recommended phase II dose (RP2D), pharmacokinetics, activity, and biomarkers of the PARP inhibitor olaparib with irinotecan in pediatric patients with recurrent/refractory malignancies. Olaparib was administered orally twice daily on Days 1-10 and irinotecan intravenously on Days 4-8 of a 21-day cycle. Dose-escalation followed the continuous reassessment method; activity was assessed in diverse tumor types (cohort 1) and Ewing sarcoma (cohort 2) according to a minimax Simon 2-stage design. Cohorts were enriched for alterations in homologous recombination repair (HRR) pathways. Seventy patients (median age: 14.9 years, range 5.0-23.8) were included, 34 with diverse tumor types (25 with HRR gene alterations) and 36 with Ewing sarcoma. Sixty-six patients received 348 treatment cycles (median, 2; range, 1-51) over four dose levels. Main toxicities were gastrointestinal and myelosuppression; the RP2D was olaparib 90 mg/m2 twice daily and irinotecan 20 mg/m2/day. Olaparib exposure in children was equivalent to those in adults. Overall response rate was 9.1%; cohort 1: 11.8%; cohort 2: 6.3%. Four patients with osteosarcoma, pineoblastoma, choroid plexus carcinoma, and neuroblastoma experienced a partial response and were treated for 9 to 51 cycles. Two patients with Ewing sarcoma experienced a complete and a partial response for 10 and 42 cycles, respectively. Genetic analyses suggest high aneuploidy score possibly being associated with objective response and prolonged stable disease. Olaparib combined with irinotecan demonstrated activity in pediatric tumors which was enriched among tumors that exhibited aneuploidy.
Gene context drift identifies drug targets to mitigate cancer treatment resistance.
Cancer treatment often fails because combinations of different therapies evoke complex resistance mechanisms that are hard to predict. We introduce REsistance through COntext DRift (RECODR): a computational pipeline that combines co-expression graph networks of single-cell RNA sequencing profiles with a graph-embedding approach to measure changes in gene co-expression context during cancer treatment. RECODR is based on the idea that gene co-expression context, rather than expression level alone, reveals important information about treatment resistance. Analysis of tumors treated in preclinical and clinical trials using RECODR unmasked resistance mechanisms -invisible to existing computational approaches- enabling the design of highly effective combination treatments for mice with choroid plexus carcinoma, and the prediction of potential new treatments for patients with medulloblastoma and triple-negative breast cancer. Thus, RECODR may unravel the complexity of cancer treatment resistance by detecting context-specific changes in gene interactions that determine the resistant phenotype.
The role of preoperative chemotherapy in pediatric neuro-oncology: a single-center experience and a systematic review of the literature.
Brain tumors in infants and young children may constitute a challenge to achieve a gross-total resection (GTR) due to their large size, high vascularity, and patient's low vascular reserve. Preoperative (neoadjuvant) chemotherapy has been reported to increase rates of GTR, reduce complications, and improve long-term tumor-free survival outcomes by decreasing tumor size and vascularity. This study evaluates the role of preoperative chemotherapy in pediatric patients with brain tumors through a systematic review and a single tertiary center experience. A systematic review was conducted using three databases (i.e., MEDLINE, Embase, and Web of Science) to identify reported cases of pediatric brain tumor treated with preoperative chemotherapy. Furthermore, the records of 6 pediatric brain tumor patients treated with preoperative chemotherapy at Texas Children's Hospital between 2018 and 2023 were reviewed. Descriptive characteristics and clinical outcomes were assessed in compliance with the local ethics committee protocol. Twenty-one studies with 124 patients were included in the systematic review. The mean age at presentation was 32 months (range 0.17-144 months), with a male predominance (n = 73, 58.9%). The mean age of the current single-center cohort was 50.3 months (range 2-180 months), with a mean follow-up of 45 months (range 4-45 months). Within the systematic review, 10 different tumor pathologies were identified, the most common being medulloblastoma (n = 61), followed by choroid plexus carcinoma (n = 26). Overall, 82.3% (102/124) of the patients showed any radiographic tumor reduction after chemotherapy in the literature review versus 50% (3/6) in the current case series. GTR after preoperative chemotherapy was achieved in 78.2% (97/124) of the patients, similar to the current patient series experience in which GTR was achieved in 80% (4/5). No complications from preoperative chemotherapy that impacted the surgery or the immediate postoperative course were identified. Preoperative chemotherapy is a safe and effective strategy to increase maximum and safe resection rates in children and infants with pediatric brain tumors.
Publicações recentes
Choroid plexus carcinoma: state of the field and emerging directions.
Risk Factors for Delayed Leptomeningeal Dissemination in Choroid Plexus Papillomas: A Systematic Review and Illustrative Case.
Neoadjuvant Chemotherapy in Choroid Plexus Carcinoma: Improving Surgical Safety and Resection Outcomes.
Choroid Plexus Mimicry: Metastatic Papillary Lung Adenocarcinoma in the Fourth Ventricle.
Phase I/II Study of the PARP Inhibitor Olaparib and Irinotecan in Children and Young Adults with Recurrent/Refractory Malignancies: Arm D of the AcSé-ESMART Trial.
📚 EuropePMC212 artigos no totalmostrando 181
Risk Factors for Delayed Leptomeningeal Dissemination in Choroid Plexus Papillomas: A Systematic Review and Illustrative Case.
Current oncology (Toronto, Ont.)Neoadjuvant Chemotherapy in Choroid Plexus Carcinoma: Improving Surgical Safety and Resection Outcomes.
JPMA. The Journal of the Pakistan Medical AssociationChoroid Plexus Mimicry: Metastatic Papillary Lung Adenocarcinoma in the Fourth Ventricle.
CureusPhase I/II study of the PARP inhibitor olaparib and irinotecan in children and young adults with recurrent/refractory malignancies: Arm D of the AcSé-ESMART trial.
Clinical cancer research : an official journal of the American Association for Cancer ResearchAtypical Teratoid Rhabdoid Tumor: How Tumor Diagnostic Methods in the Laboratory Have Evolved over the Past 40 Years.
CancersClinical challenges of cancer predisposition syndromes with pediatric central nervous system tumors: a single-center study.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryAn overview of the diagnosis and management of Choroid Plexus tumors.
Advances in cancer researchSuperselective anterior choroidal artery preoperative embolization of a giant choroid plexus carcinoma in an infant: case report and systematic review.
NeuroradiologyA rare pediatric case of Li-Fraumeni syndrome presenting with virilization symptoms and dual primary malignancies on magnetic resonance imaging and [18F]-fluorodeoxyglucose positron emission tomography/computed tomography.
Pediatric radiologyChoroid plexus metastasis of a renal cell carcinoma-A case report and review of the literature.
Neuro-oncology advancesGene context drift identifies drug targets to mitigate cancer treatment resistance.
Cancer cellGemcitabine-oxaliplatin as a bridge therapy toward autologous hematopoietic stem cell transplantation in infant-type brain tumors.
Frontiers in oncologyThe role of preoperative chemotherapy in pediatric neuro-oncology: a single-center experience and a systematic review of the literature.
Journal of neurosurgery. PediatricsA Rare Case of Atypical Choroid Plexus Papilloma in an Adult Male Patient: A Case Report.
Case reports in pathologyOTX-2 Expression as a Diagnostic Marker for Choroid Plexus Tumors.
Neuropathology : official journal of the Japanese Society of NeuropathologyLeptomeningeal Dissemination in Choroid Plexus Tumors: Magnetic Resonance Imaging Appearance and Risk Factors.
Children (Basel, Switzerland)Obstructive hydrocephalus due to choroid plexus carcinoma of third ventricle in pediatric: A rare case report.
Radiology case reportsEpigenetic silencing of ZIC4 unveils a potential tumor suppressor role in pediatric choroid plexus carcinoma.
Scientific reportsFreiburg Neuropathology Case Conference : Mild Disorientation and Mild Anomic Aphasia in a 79-Year-Old Female.
Clinical neuroradiologyRole of Multimodal Monitoring in the Management of Massive Blood Transfusion in Pediatric Patients Undergoing Excision of Choroid Plexus Carcinoma - A Case-Based Review of a Novel Perioperative Protocol.
Neurology IndiaMarrow-ablative consolidation chemotherapy and molecular targeted therapy delivered in a risk-adapted manner for newly diagnosed children with choroid plexus carcinoma: A work in progress.
Neuro-oncology advancesMolecular genetics and diversity of choroid plexus tumors.
Neuro-oncology advancesDiagnosis of choroid plexus papilloma: Current perspectives and future directions.
Cancer pathogenesis and therapyRecurrent choroid plexus carcinoma treated with CyberKnife radiosurgery: illustrative case.
Journal of neurosurgery. Case lessonsMolecular insights into TP53 mutation (p. Arg267Trp) and its connection to Choroid Plexus Carcinomas and Li-Fraumeni Syndrome.
Genes & genomicsComprehensive multiomics analysis reveals distinct differences between pediatric choroid plexus papilloma and carcinoma.
Acta neuropathologica communications[Multiple primary tumors in children: a clinicopathological analysis of four cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyDeveloping a nomogram based on SEER database for predicting prognosis in choroid plexus tumors.
Scientific reportsLC3A-mediated autophagy elicits PERK-eIF2α-ATF4 axis activation and mitochondrial dysfunction: Exposing vulnerability in aggresome-positive cancer cells.
The Journal of biological chemistryAtypical choroid plexus papilloma: Diagnosis and differentials: A case report.
SAGE open medical case reportsClinical and genetic characteristics of carriers of the TP53 c.541C > T, p.Arg181Cys pathogenic variant causing hereditary cancer in patients of Arab-Muslim descent.
Familial cancerRecurrence Patterns and Surveillance Imaging in Pediatric Brain Tumor Survivors.
Journal of pediatric hematology/oncologyPrognostic Factors and Nomogram for Choroid Plexus Tumors: A Population-Based Retrospective Surveillance, Epidemiology, and End Results Database Analysis.
CancersCorrelation between choroid plexus carcinoma and Li-Fraumeni syndrome: implications of TP53 mutations and management strategies-a case-based narrative review.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryLaser interstitial thermal therapy as a radiation-sparing approach for central nervous system tumors in children with cancer predisposition syndromes: report of a child with Li-Fraumeni syndrome. Illustrative case.
Journal of neurosurgery. Case lessonsCongenital Tumors-Magnetic Resonance Imaging Findings with Focus on Rare Tumors.
CancersMulti-omics analyses of choroid plexus carcinoma cell lines reveal potential targetable pathways and alterations.
Journal of neuro-oncologyPaediatric choroid plexus carcinoma: a retrospective case series from Karachi.
JPMA. The Journal of the Pakistan Medical AssociationPrimary adult choroid plexus carcinomas: a single-center experience with a systematic review.
Frontiers in oncologyManagement of Choroid Plexus Tumors and the Benefit of Preoperative Embolization in Pediatric Patients: Report of 46 Cases from a Single Institution.
World neurosurgeryCurrent practice of cancer predisposition testing in pediatric patients with CNS tumors in the United States.
Pediatric blood & cancerSafety and clinical efficacy of sintilimab (anti-PD-1) in pediatric patients with advanced or recurrent malignancies in a phase I study.
Signal transduction and targeted therapyApproaches to Minimise the Neurodevelopmental Impact of Choroid Plexus Carcinoma and Its Treatment.
Life (Basel, Switzerland)The composition of choroid plexus tumor research: a bibliometric analysis of the 100 most impactful studies to date.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryStereotactic radiotherapy outcomes for intraventricular brain tumours in 11 dogs.
Veterinary and comparative oncologyNovel Adjuvant Targeted Approach for PTEN-Mutated Choroid Plexus Carcinoma in Adults: Case Report and Literature Review.
JCO precision oncologyTumors of Choroid Plexus and Other Ventricular Tumors.
Advances in experimental medicine and biologyPreclinical validation of a novel therapeutic strategy for choroid plexus carcinoma.
Journal of controlled release : official journal of the Controlled Release SocietyChoroid plexus carcinoma with meningeal carcinomatosis in a dog.
The Journal of small animal practiceGenomic profile of two Brazilian choroid plexus tumors by whole-exome sequencing.
Cold Spring Harbor molecular case studiesTranscallosal and endoscopic hybrid approach to a rare entity of pediatric intraventricular tumors-cribriform neuroepithelial tumor: a case report and literature review.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryDurability of an endoscopic management strategy for recurrent choroid plexus carcinoma with a comprehensive molecular characterization: illustrative case.
Journal of neurosurgery. Case lessonsInfratentorial atypical choroid plexus papilloma in an adult: A case report and literature review.
Indian journal of cancerCorrigendum to: DDEL-17. TRIPLE INTRAVENTRICULAR CHEMOTHERAPY FOR TREATMENT OF RELAPSED CHOROID PLEXUS CARCINOMA.
Neuro-oncologydSmad2 differentially regulates dILP2 and dILP5 in insulin producing and circadian pacemaker cells in unmated adult females.
PloS oneA novel cardiac arrest severity score for the early prediction of hypoxic-ischemic brain injury and in-hospital death.
The American journal of emergency medicineIntracranial Subdural Fluid Accumulation Associated with a Choroid Plexus Carcinoma in a Dog.
Veterinary sciencesIncidence and survival of choroid plexus tumors in the United States.
Neuro-oncology practiceHollow CoP@N-Carbon Nanospheres: Heterostructure and Glucose-Enhanced Charge Separation for Sonodynamic/Starvation Therapy.
ACS applied materials & interfacesElevated TAF12 Expression Predicts Poor Prognosis in Glioma Patients: Evidence from Bioinformatic and Immunohistochemical Analyses.
BiomoleculesMolecular heterogeneity of pediatric choroid plexus carcinomas determines the distinctions in clinical course and prognosis.
Neuro-oncologyA rare, giant, lateral intraventricular gliosarcoma.
Radiology case reportsValidation of the revised diagnostic criteria for primary plasma cell leukemia by the Korean Multiple Myeloma Working Party.
Blood cancer journalDelayed Recurrence of Choroid Plexus Carcinoma in the Sacral Spinal Cord 17 Years after Its Initial Presentation.
NMC case report journalChoroid plexus carcinoma in two siblings, with a novel genetic mutation in TP53 - A case report and review of literature.
Surgical neurology internationalNervous system (NS) Tumors in Cancer Predisposition Syndromes.
Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeuticsIntermittent Hydrostatic Pressure Promotes Cartilage Repair in an Inflammatory Environment through Hippo-YAP Signaling In Vitro and In Vivo.
BioMed research international[Congenital penile curvature: Guidelines from the Andrology and Sexual Medicine Committee of the French Association of Urology (CAMS-AFU)].
Progres en urologie : journal de l'Association francaise d'urologie et de la Societe francaise d'urologieBovine intracranial neoplasia: A retrospective case series.
Veterinary pathologyAsymptomatic choroid plexus carcinoma in an infant: Report of one case.
Annals of medicine and surgery (2012)Giant Pediatric Supratentorial Tumor: Clinical Feature and Surgical Strategy.
Frontiers in pediatricsQuantifying intraventricular drug delivery utilizing programmable ventriculoperitoneal shunts as the intraventricular access device.
Journal of neuro-oncologyDisruption of GMNC-MCIDAS multiciliogenesis program is critical in choroid plexus carcinoma development.
Cell death and differentiationFinal results of the Choroid Plexus Tumor study CPT-SIOP-2000.
Journal of neuro-oncologyCase Report : Li-Fraumeni Syndrome with Central Nervous System Tumors in Two Siblings.
BMC pediatricsLong-term survival of a patient with metastatiс choroid plexus carcinoma, de novo germline TP53 mutated, after craniospinal proton irradiation.
Pediatric blood & cancerChoroid Plexus Carcinomas With TP53 Germline Mutations: Management and Outcome.
Frontiers in oncologyAtypical choroid plexus papilloma: A case report.
Asian journal of surgeryFreiburg Neuropathology Case Conference : A 6-year-old Girl Presenting with Vomiting and Right-sided Facial Paresis.
Clinical neuroradiologyChoroid plexus carcinoma with leptomeningeal spread in an adult: a case report and review of the literature.
Journal of medical case reportsTriple intraventricular chemotherapy for treatment of relapsed choroid plexus carcinoma.
Pediatric blood & cancerPreoperative Devascularization of Choroid Plexus Tumors: Specific Issues about Anatomy and Embolization Technique.
Brain sciencesIntracranial Choroid Plexus Carcinomas: Report of 11 Cases from a Single Institution.
World neurosurgeryChoroid plexus carcinoma in an adolescent male: a case report.
Journal of medical case reportsCerebellopontine Angle Primary Choroid Plexus Carcinoma Present in an Adult: Case Report and Literature Review.
CureusA choroid plexus metastasis of a prostatic adenocarcinoma mimicking a choroid plexus carcinoma: A case report.
Neuro-ChirurgieMolecular insights into malignant progression of atypical choroid plexus papilloma.
Cold Spring Harbor molecular case studiesBenefit of Static FET PET in Pretreated Pediatric Brain Tumor Patients with Equivocal Conventional MRI Results.
Klinische PadiatrieOutcome and molecular analysis of young children with choroid plexus carcinoma treated with non-myeloablative therapy: results from the SJYC07 trial.
Neuro-oncology advancesManagement of choroid plexus tumours: A comprehensive study from a tertiary hospital.
Clinical neurology and neurosurgerySynchronous choroid plexus papilloma and Wilms tumor in a girl, disclosing a Li-Fraumeni syndrome.
Hereditary cancer in clinical practicePediatric atypical choroid plexus papilloma: Clinical features and diagnosis.
Clinical neurology and neurosurgeryA Unique Case of Cerebellar Choroid Plexus Carcinoma.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiquesChoroid Plexus Carcinoma with Hyaline Globules: An Uncommon Histological Finding.
Fetal and pediatric pathologyIntraventricular choroid plexus tumors: clinical characteristics and impact of current management on survival.
Journal of neuro-oncologyFrequency of the TP53 p.R337H mutation in a Brazilian cohort of pediatric patients with solid tumors.
Molecular biology reportsDelayed Granulocyte Colony-Stimulating Factor (G-CSF) Administration after Chemotherapy Reduces Total G-CSF Doses without Affecting Neutrophil Recovery in a Randomized Clinical Study in Children with Solid Tumors.
Pediatric hematology and oncologyLate recurrence of choroid plexus carcinoma.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryEpigenetics impacts upon prognosis and clinical management of choroid plexus tumors.
Journal of neuro-oncologyAn update on the CNS manifestations of brain tumor polyposis syndromes.
Acta neuropathologicaCase 2: A Rare Cause of Intraventricular Hemorrhage in a Term Neonate.
Pediatrics in reviewMultiple High Grade Rhabdoid Papillary Meningiomas Mimicking Choroid Plexus Carcinoma: A Case Report.
Iranian journal of pathologyModerate-to-strong expression of FGFR3 and TP53 alterations in a subpopulation of choroid plexus tumors.
Histology and histopathologyChoroid Plexus Carcinoma in Adults: Literature Review and First Report of a Location into the Third Ventricle.
World neurosurgeryNext generation DNA sequencing of atypical choroid plexus papilloma of brain: Identification of novel mutations in a female patient by Ion Proton.
Oncology lettersTransventricular Migration of Choroid Plexus Carcinoma Causing an Intraoperative Conundrum: A Case Report with a Review of the Literature.
Pediatric neurosurgeryAn update on the central nervous system manifestations of Li-Fraumeni syndrome.
Acta neuropathologicaDNA methylation signature is prognostic of choroid plexus tumor aggressiveness.
Clinical epigeneticsSubgroup-specific outcomes of children with malignant childhood brain tumors treated with an irradiation-sparing protocol.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryStructure-based design generated novel hydroxamic acid based preferential HDAC6 lead inhibitor with on-target cytotoxic activity against primary choroid plexus carcinoma.
Journal of enzyme inhibition and medicinal chemistryChoroid Plexus Carcinoma in Adults: Two Case Reports.
Brain tumor research and treatmentAn Intraventricular Type of Chondroma: A Case Report.
Turkish neurosurgeryClinical spectrum of Li-Fraumeni syndrome/Li-Fraumeni-like syndrome in Brazilian individuals with the TP53 p.R337H mutation.
The Journal of steroid biochemistry and molecular biologyPerinatal (fetal and neonatal) choroid plexus tumors: a review.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryChoroid plexus tumours on MRI: similarities and distinctions in different grades.
Cancer imaging : the official publication of the International Cancer Imaging SocietyMultimodality characteristics of multifocal choroid plexus carcinoma with bilateral calvarial defects in a dog.
Veterinary radiology & ultrasound : the official journal of the American College of Veterinary Radiology and the International Veterinary Radiology AssociationMyc and Loss of p53 Cooperate to Drive Formation of Choroid Plexus Carcinoma.
Cancer researchManagement of choroid plexus tumors-an institutional experience.
Acta neurochirurgicaRare TP53 variant associated with Li-Fraumeni syndrome exhibits variable penetrance in a Saudi family.
NPJ genomic medicineClinical Features and Prognostic Risk Factors of Choroid Plexus Tumors in Children.
Chinese medical journalManagement of Choroid Plexus Tumors in Infants and Young Children Up to 4 Years of Age: An Institutional Experience.
World neurosurgeryMRI of intracranial intraventricular lesions.
Clinical imagingRapid malignant progression of an intraparenchymal choroid plexus papillomas.
Surgical neurology internationalWidely Metastatic Choroid Plexus Carcinoma Associated with Novel TP53 Somatic Mutation.
World neurosurgeryReport of a bi-allelic truncating germline mutation in TP53.
Familial cancerIntracranial Neoplasms in the First Year of Life: Results of a Third Cohort of Patients From a Single Institution.
NeurosurgeryIncreasing genomic instability during cancer therapy in a patient with Li-Fraumeni syndrome.
Clinical and translational radiation oncologyComparative Cytogenetic Analysis of Dog and Human Choroid Plexus Tumors Defines Syntenic Regions of Genomic Loss.
Journal of neuropathology and experimental neurologyRoles of the apparent diffusion coefficient and tumor volume in predicting tumor grade in patients with choroid plexus tumors.
NeuroradiologyMice Expressing Myc in Neural Precursors Develop Choroid Plexus and Ciliary Body Tumors.
The American journal of pathologyPediatric choroid plexus carcinoma: Biologically and clinically in need of new perspectives.
Pediatric blood & cancerChoroid plexus carcinoma with neuronal and glial differentiation in a 7-week-old male Sprague-Dawley rat.
The Journal of veterinary medical scienceGlomeruloid Microvascular Proliferation, Desmoplasia, and High Proliferative Index as Potential Indicators of High Grade Canine Choroid Plexus Tumors.
Veterinary pathologyA new genetically engineered mouse model of choroid plexus carcinoma.
Biochemical and biophysical research communicationsA Case of Giant Supratentorial Choroid Plexus Carcinoma in a Young Girl.
Pediatric neurosurgeryEstablishing a Preclinical Multidisciplinary Board for Brain Tumors.
Clinical cancer research : an official journal of the American Association for Cancer ResearchScreening with whole-body magnetic resonance imaging in pediatric subjects with Li-Fraumeni syndrome: A single institution pilot study.
Pediatric blood & cancerRevisiting tumor patterns and penetrance in germline TP53 mutation carriers: temporal phases of Li-Fraumeni syndrome.
Current opinion in oncologyConstitutional mosaicism of a de novo TP53 mutation in a patient with bilateral choroid plexus carcinoma.
Cancer geneticsMolecular Guided Therapy Provides Sustained Clinical Response in Refractory Choroid Plexus Carcinoma.
Frontiers in pharmacologyEffect of Surgery, Adjuvant Therapy, and Other Prognostic Factors on Choroid Plexus Carcinoma: A Systematic Review and Individual Patient Data Analysis.
International journal of radiation oncology, biology, physicsSuccessful multimodal treatment of a gigantic choroid plexus carcinoma (CPC) in an 8-year-old girl.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgerySuccessful Treatment of Recurrent Li-Fraumeni Syndrome-related Choroid Plexus Carcinoma.
Journal of pediatric hematology/oncologySpinal drop metastasis from a benign fourth ventricular choroid plexus papilloma in a pediatric patient: case report.
Journal of neurosurgery. PediatricsLC3A Silencing Hinders Aggresome Vimentin Cage Clearance in Primary Choroid Plexus Carcinoma.
Scientific reportsMagnetic Resonance Imaging and Clinical Findings Associated with Choroid Plexus Spinal Cord "Drop" Metastases.
Journal of the American Animal Hospital AssociationIncreased NG2 and SOX2 expression is associated with high-grade choroid plexus tumors.
Oncology lettersMarrow-ablative chemotherapy followed by tandem autologous hematopoietic cell transplantation in pediatric patients with malignant brain tumors.
Bone marrow transplantationAtypical choroid plexus papilloma: spontaneous resolution of diffuse leptomeningeal contrast enhancement after primary tumor removal in 2 pediatric cases.
Journal of neurosurgery. PediatricsA French retrospective study on clinical outcome in 102 choroid plexus tumors in children.
Journal of neuro-oncologyA rare case of choroid plexus carcinoma that led to the diagnosis of Lynch syndrome (hereditary nonpolyposis colorectal cancer).
Clinical neurology and neurosurgeryTargeted next-generation sequencing of pediatric neuro-oncology patients improves diagnosis, identifies pathogenic germline mutations, and directs targeted therapy.
Neuro-oncologyNeoadjuvant chemotherapy for atypical teratoid rhabdoid tumors: case report.
Journal of neurosurgery. PediatricsChoroid plexus tumors in adult and pediatric populations: the Cleveland Clinic and University Hospitals experience.
Journal of neuro-oncologyClinicopathological features and microsurgical outcomes for giant pediatric intracranial tumor in 60 consecutive cases.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryConcurrent Gliosarcoma and Choroid Plexus Carcinoma in a Cow.
Journal of comparative pathologyRisks of first and subsequent cancers among TP53 mutation carriers in the National Cancer Institute Li-Fraumeni syndrome cohort.
CancerInfant brain tumours: a tale of two cities.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryKir7.1 immunoreactivity in canine choroid plexus tumors.
Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, IncChoroid plexus carcinoma with hyaline globules: An unusual histological feature.
Indian journal of pathology & microbiologyMethylation profiling of choroid plexus tumors reveals 3 clinically distinct subgroups.
Neuro-oncologyUSE OF RECOMBINANT ACTIVATED FACTOR VIIa IN A-SIX-MONTH-OLD CHILD DUE TO MASSIVE HEMORRHAGE DURING ELECTIVE SURGERY FOR CHOROID PLEXUS CARCINOMA: CASE REPORT.
Acta clinica CroaticaPreclinical studies of 5-fluoro-2'-deoxycytidine and tetrahydrouridine in pediatric brain tumors.
Journal of neuro-oncologyChoroid Plexus Tumors: Experience of 10 Cases with Special References to Adult Cases.
Neurologia medico-chirurgicaOccurrence of Neuroblastoma among TP53 p.R337H Carriers.
PloS oneChoroid Plexus Carcinoma in the External Ventricle of an Adult.
The Journal of craniofacial surgeryA 12-year-old boy with a mass located at the left parietal lobe involving the left lateral ventricle.
Neuropathology : official journal of the Japanese Society of NeuropathologyPre-operative embolization of a choroid plexus carcinoma: review of the vascular anatomy.
Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery[Choroid plexus tumours in childhood: Experience in Sant Joan de Déu hospital].
Neurocirugia (Asturias, Spain)Choroid plexus carcinoma in adults: an extremely rare case.
The Pan African medical journalChoroid Plexus Neoplasms: Toward a Distinction between Carcinoma and Papilloma Using Arterial Spin-Labeling.
AJNR. American journal of neuroradiologyConcurrent Gain of Oncogenes Drives Choroid Plexus Carcinoma.
Cancer discoveryCross-Species Genomics Identifies TAF12, NFYC, and RAD54L as Choroid Plexus Carcinoma Oncogenes.
Cancer cellRadiation therapy for choroid plexus carcinoma patients with Li-Fraumeni syndrome: advantageous or detrimental?
Anticancer researchNeoadjuvant chemotherapy reduces blood loss during the resection of pediatric choroid plexus carcinomas.
Journal of neurosurgery. PediatricsPhase I trial of weekly MK-0752 in children with refractory central nervous system malignancies: a pediatric brain tumor consortium study.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryThe role of chemotherapy and surgical removal in the treatment of Choroid Plexus carcinomas and atypical papillomas.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryA child with Li-Fraumeni syndrome: Modes to inactivate the second allele of TP53 in three different malignancies.
Pediatric blood & cancer[Choroid plexus tumour treatment at Hospital Infantil Niño Jesús in Madrid: Our experience over the last three decades].
Neurocirugia (Asturias, Spain)Neoadjuvant chemotherapy for brain tumors in infants and young children.
Journal of neurosurgery. PediatricsChoroid plexus carcinoma in children: the Head Start experience.
Pediatric blood & cancerAssociações
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
Ainda não existe comunidade no Raras para Carcinoma do plexo coroide
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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.
- Risk Factors for Delayed Leptomeningeal Dissemination in Choroid Plexus Papillomas: A Systematic Review and Illustrative Case.
- Neoadjuvant Chemotherapy in Choroid Plexus Carcinoma: Improving Surgical Safety and Resection Outcomes.
- Phase I/II study of the PARP inhibitor olaparib and irinotecan in children and young adults with recurrent/refractory malignancies: Arm D of the AcSé-ESMART trial.Clinical cancer research : an official journal of the American Association for Cancer Research· 2026· PMID 41591981mais citado
- Gene context drift identifies drug targets to mitigate cancer treatment resistance.
- The role of preoperative chemotherapy in pediatric neuro-oncology: a single-center experience and a systematic review of the literature.
- Choroid plexus carcinoma: state of the field and emerging directions.
- Choroid Plexus Mimicry: Metastatic Papillary Lung Adenocarcinoma in the Fourth Ventricle.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:251899(Orphanet)
- MONDO:0016718(MONDO)
- GARD:8238(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q1524940(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
