Raras
Buscar doenças, sintomas, genes...
Carcinoma do plexo coroide
ORPHA:251899CID-10 · C71.7CID-11 · 2A00.22DOENÇA RARA

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.

Mantido por Agente Raras·Colaborar como especialista →

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.

Pesquisas ativas
4 ensaios
13 total registrados no ClinicalTrials.gov
Publicações científicas
411 artigos
Último publicado: 2026 Apr 6

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Childhood
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C71.7
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico411PubMed
Últimos 10 anos181publicações
Pico202423 papers
Linha do tempo
2026Hoje · 2026🧪 1994Primeiro ensaio clínico📈 2024Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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.

TP53Cellular tumor antigen p53Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

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

LOCALIZAÇÃO

CytoplasmNucleusNucleus, PML bodyEndoplasmic reticulumMitochondrion matrixCytoplasm, cytoskeleton, microtubule organizing center, centrosome

VIAS BIOLÓGICAS (10)
TP53 Regulates Metabolic GenesRegulation of TP53 ExpressionRegulation of TP53 DegradationOncogene Induced SenescenceOxidative Stress Induced Senescence
EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
73.0 TPM
Skin Sun Exposed Lower leg
37.0 TPM
Skin Not Sun Exposed Suprapubic
35.2 TPM
Fibroblastos
32.9 TPM
Ovário
32.4 TPM
OUTRAS DOENÇAS (29)
Li-Fraumeni syndromenasopharyngeal carcinoma, susceptibility to, 1hepatocellular carcinomafamilial pancreatic carcinoma
HGNC:11998UniProt:P04637

Variantes genéticas (ClinVar)

1,725 variantes patogênicas registradas no ClinVar.

🧬 TP53: NM_000546.6(TP53):c.377del (p.Tyr126fs) ()
🧬 TP53: NM_000546.6(TP53):c.494dup (p.Ser166fs) ()
🧬 TP53: NM_000546.6(TP53):c.184_187dup (p.Ala63fs) ()
🧬 TP53: NM_000546.6(TP53):c.549dup (p.Asp184fs) ()
🧬 TP53: NM_000546.6(TP53):c.43del (p.Ser15fs) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 6 variantes classificadas pelo ClinVar.

6
Patogênica (100.0%)
VARIANTES MAIS SIGNIFICATIVAS
TP53: NM_000546.6(TP53):c.799C>G (p.Arg267Gly) [Pathogenic]
TP53: NM_000546.6(TP53):c.396G>C (p.Lys132Asn) [Likely pathogenic]
TP53: NM_000546.6(TP53):c.808T>G (p.Phe270Val) [Pathogenic]
TP53: NM_000546.6(TP53):c.413C>T (p.Ala138Val) [Likely pathogenic]
TP53: NM_000546.6(TP53):c.854A>T (p.Glu285Val) [Pathogenic]

Vias biológicas (Reactome)

30 vias biológicas associadas aos genes desta condição.

Activation of NOXA and translocation to mitochondria Activation of PUMA and translocation to mitochondria Pre-NOTCH Transcription and Translation Oxidative Stress Induced Senescence Formation of Senescence-Associated Heterochromatin Foci (SAHF) Oncogene Induced Senescence DNA Damage/Telomere Stress Induced Senescence SUMOylation of transcription factors Autodegradation of the E3 ubiquitin ligase COP1 Association of TriC/CCT with target proteins during biosynthesis Pyroptosis TP53 Regulates Metabolic Genes Ub-specific processing proteases Ovarian tumor domain proteases Recruitment and ATM-mediated phosphorylation of repair and signaling proteins at DNA double strand breaks Interleukin-4 and Interleukin-13 signaling TP53 Regulates Transcription of DNA Repair Genes TP53 Regulates Transcription of Genes Involved in Cytochrome C Release TP53 regulates transcription of several additional cell death genes whose specific roles in p53-dependent apoptosis remain uncertain TP53 Regulates Transcription of Caspase Activators and Caspases TP53 Regulates Transcription of Death Receptors and Ligands TP53 Regulates Transcription of Genes Involved in G2 Cell Cycle Arrest TP53 regulates transcription of additional cell cycle genes whose exact role in the p53 pathway remain uncertain TP53 Regulates Transcription of Genes Involved in G1 Cell Cycle Arrest Regulation of TP53 Expression Regulation of TP53 Activity through Phosphorylation Regulation of TP53 Degradation Regulation of TP53 Activity through Acetylation Regulation of TP53 Activity through Association with Co-factors Regulation of TP53 Activity through Methylation

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
1Fase 17
·Pré-clínico2
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 9 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Carcinoma do plexo coroide

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

2 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

13 ensaios clínicos encontrados, 4 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
🧪 Está conduzindo uma pesquisa?
Divulgue para pacientes e familiares que acompanham esta doença.
Divulgar pesquisa →

Publicações mais relevantes

Timeline de publicações
166 papers (10 anos)
#1

Risk Factors for Delayed Leptomeningeal Dissemination in Choroid Plexus Papillomas: A Systematic Review and Illustrative Case.

Current oncology (Toronto, Ont.)2026 Feb 13

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.

#2

Neoadjuvant Chemotherapy in Choroid Plexus Carcinoma: Improving Surgical Safety and Resection Outcomes.

JPMA. The Journal of the Pakistan Medical Association2026 Jan

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.

#3

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 Research2026 Jan 27

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.

#4

Gene context drift identifies drug targets to mitigate cancer treatment resistance.

Cancer cell2025 Sep 08

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.

#5

The role of preoperative chemotherapy in pediatric neuro-oncology: a single-center experience and a systematic review of the literature.

Journal of neurosurgery. Pediatrics2025 Jul 01

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

Ver todas no PubMed

📚 EuropePMC212 artigos no totalmostrando 181

2026

Risk Factors for Delayed Leptomeningeal Dissemination in Choroid Plexus Papillomas: A Systematic Review and Illustrative Case.

Current oncology (Toronto, Ont.)
2026

Neoadjuvant Chemotherapy in Choroid Plexus Carcinoma: Improving Surgical Safety and Resection Outcomes.

JPMA. The Journal of the Pakistan Medical Association
2025

Choroid Plexus Mimicry: Metastatic Papillary Lung Adenocarcinoma in the Fourth Ventricle.

Cureus
2026

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
2025

Atypical Teratoid Rhabdoid Tumor: How Tumor Diagnostic Methods in the Laboratory Have Evolved over the Past 40 Years.

Cancers
2025

Clinical 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 Neurosurgery
2025

An overview of the diagnosis and management of Choroid Plexus tumors.

Advances in cancer research
2025

Superselective anterior choroidal artery preoperative embolization of a giant choroid plexus carcinoma in an infant: case report and systematic review.

Neuroradiology
2025

A 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 radiology
2025

Choroid plexus metastasis of a renal cell carcinoma-A case report and review of the literature.

Neuro-oncology advances
2025

Gene context drift identifies drug targets to mitigate cancer treatment resistance.

Cancer cell
2025

Gemcitabine-oxaliplatin as a bridge therapy toward autologous hematopoietic stem cell transplantation in infant-type brain tumors.

Frontiers in oncology
2025

The role of preoperative chemotherapy in pediatric neuro-oncology: a single-center experience and a systematic review of the literature.

Journal of neurosurgery. Pediatrics
2025

A Rare Case of Atypical Choroid Plexus Papilloma in an Adult Male Patient: A Case Report.

Case reports in pathology
2025

OTX-2 Expression as a Diagnostic Marker for Choroid Plexus Tumors.

Neuropathology : official journal of the Japanese Society of Neuropathology
2025

Leptomeningeal Dissemination in Choroid Plexus Tumors: Magnetic Resonance Imaging Appearance and Risk Factors.

Children (Basel, Switzerland)
2024

Obstructive hydrocephalus due to choroid plexus carcinoma of third ventricle in pediatric: A rare case report.

Radiology case reports
2024

Epigenetic silencing of ZIC4 unveils a potential tumor suppressor role in pediatric choroid plexus carcinoma.

Scientific reports
2024

Freiburg Neuropathology Case Conference : Mild Disorientation and Mild Anomic Aphasia in a 79-Year-Old Female.

Clinical neuroradiology
2024

Role 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 India
2024

Marrow-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 advances
2024

Molecular genetics and diversity of choroid plexus tumors.

Neuro-oncology advances
2024

Diagnosis of choroid plexus papilloma: Current perspectives and future directions.

Cancer pathogenesis and therapy
2024

Recurrent choroid plexus carcinoma treated with CyberKnife radiosurgery: illustrative case.

Journal of neurosurgery. Case lessons
2024

Molecular insights into TP53 mutation (p. Arg267Trp) and its connection to Choroid Plexus Carcinomas and Li-Fraumeni Syndrome.

Genes &amp; genomics
2024

Comprehensive multiomics analysis reveals distinct differences between pediatric choroid plexus papilloma and carcinoma.

Acta neuropathologica communications
2024

[Multiple primary tumors in children: a clinicopathological analysis of four cases].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2024

Developing a nomogram based on SEER database for predicting prognosis in choroid plexus tumors.

Scientific reports
2024

LC3A-mediated autophagy elicits PERK-eIF2α-ATF4 axis activation and mitochondrial dysfunction: Exposing vulnerability in aggresome-positive cancer cells.

The Journal of biological chemistry
2024

Atypical choroid plexus papilloma: Diagnosis and differentials: A case report.

SAGE open medical case reports
2024

Clinical 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 cancer
2024

Recurrence Patterns and Surveillance Imaging in Pediatric Brain Tumor Survivors.

Journal of pediatric hematology/oncology
2024

Prognostic Factors and Nomogram for Choroid Plexus Tumors: A Population-Based Retrospective Surveillance, Epidemiology, and End Results Database Analysis.

Cancers
2024

Correlation 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 Neurosurgery
2024

Laser 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 lessons
2023

Congenital Tumors-Magnetic Resonance Imaging Findings with Focus on Rare Tumors.

Cancers
2024

Multi-omics analyses of choroid plexus carcinoma cell lines reveal potential targetable pathways and alterations.

Journal of neuro-oncology
2023

Paediatric choroid plexus carcinoma: a retrospective case series from Karachi.

JPMA. The Journal of the Pakistan Medical Association
2023

Primary adult choroid plexus carcinomas: a single-center experience with a systematic review.

Frontiers in oncology
2024

Management of Choroid Plexus Tumors and the Benefit of Preoperative Embolization in Pediatric Patients: Report of 46 Cases from a Single Institution.

World neurosurgery
2024

Current practice of cancer predisposition testing in pediatric patients with CNS tumors in the United States.

Pediatric blood &amp; cancer
2023

Safety 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 therapy
2023

Approaches to Minimise the Neurodevelopmental Impact of Choroid Plexus Carcinoma and Its Treatment.

Life (Basel, Switzerland)
2024

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 Neurosurgery
2023

Stereotactic radiotherapy outcomes for intraventricular brain tumours in 11 dogs.

Veterinary and comparative oncology
2023

Novel Adjuvant Targeted Approach for PTEN-Mutated Choroid Plexus Carcinoma in Adults: Case Report and Literature Review.

JCO precision oncology
2023

Tumors of Choroid Plexus and Other Ventricular Tumors.

Advances in experimental medicine and biology
2023

Preclinical validation of a novel therapeutic strategy for choroid plexus carcinoma.

Journal of controlled release : official journal of the Controlled Release Society
2023

Choroid plexus carcinoma with meningeal carcinomatosis in a dog.

The Journal of small animal practice
2023

Genomic profile of two Brazilian choroid plexus tumors by whole-exome sequencing.

Cold Spring Harbor molecular case studies
2023

Transcallosal 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 Neurosurgery
2023

Durability of an endoscopic management strategy for recurrent choroid plexus carcinoma with a comprehensive molecular characterization: illustrative case.

Journal of neurosurgery. Case lessons
2023

Infratentorial atypical choroid plexus papilloma in an adult: A case report and literature review.

Indian journal of cancer
2023

Corrigendum to: DDEL-17. TRIPLE INTRAVENTRICULAR CHEMOTHERAPY FOR TREATMENT OF RELAPSED CHOROID PLEXUS CARCINOMA.

Neuro-oncology
2023

dSmad2 differentially regulates dILP2 and dILP5 in insulin producing and circadian pacemaker cells in unmated adult females.

PloS one
2023

A novel cardiac arrest severity score for the early prediction of hypoxic-ischemic brain injury and in-hospital death.

The American journal of emergency medicine
2022

Intracranial Subdural Fluid Accumulation Associated with a Choroid Plexus Carcinoma in a Dog.

Veterinary sciences
2023

Incidence and survival of choroid plexus tumors in the United States.

Neuro-oncology practice
2023

Hollow CoP@N-Carbon Nanospheres: Heterostructure and Glucose-Enhanced Charge Separation for Sonodynamic/Starvation Therapy.

ACS applied materials &amp; interfaces
2022

Elevated TAF12 Expression Predicts Poor Prognosis in Glioma Patients: Evidence from Bioinformatic and Immunohistochemical Analyses.

Biomolecules
2023

Molecular heterogeneity of pediatric choroid plexus carcinomas determines the distinctions in clinical course and prognosis.

Neuro-oncology
2023

A rare, giant, lateral intraventricular gliosarcoma.

Radiology case reports
2022

Validation of the revised diagnostic criteria for primary plasma cell leukemia by the Korean Multiple Myeloma Working Party.

Blood cancer journal
2022

Delayed Recurrence of Choroid Plexus Carcinoma in the Sacral Spinal Cord 17 Years after Its Initial Presentation.

NMC case report journal
2022

Choroid plexus carcinoma in two siblings, with a novel genetic mutation in TP53 - A case report and review of literature.

Surgical neurology international
2022

Nervous system (NS) Tumors in Cancer Predisposition Syndromes.

Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics
2022

Intermittent Hydrostatic Pressure Promotes Cartilage Repair in an Inflammatory Environment through Hippo-YAP Signaling In Vitro and In Vivo.

BioMed research international
2022

[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'urologie
2022

Bovine intracranial neoplasia: A retrospective case series.

Veterinary pathology
2022

Asymptomatic choroid plexus carcinoma in an infant: Report of one case.

Annals of medicine and surgery (2012)
2022

Giant Pediatric Supratentorial Tumor: Clinical Feature and Surgical Strategy.

Frontiers in pediatrics
2022

Quantifying intraventricular drug delivery utilizing programmable ventriculoperitoneal shunts as the intraventricular access device.

Journal of neuro-oncology
2022

Disruption of GMNC-MCIDAS multiciliogenesis program is critical in choroid plexus carcinoma development.

Cell death and differentiation
2022

Final results of the Choroid Plexus Tumor study CPT-SIOP-2000.

Journal of neuro-oncology
2021

Case Report : Li-Fraumeni Syndrome with Central Nervous System Tumors in Two Siblings.

BMC pediatrics
2022

Long-term survival of a patient with metastatiс choroid plexus carcinoma, de novo germline TP53 mutated, after craniospinal proton irradiation.

Pediatric blood &amp; cancer
2021

Choroid Plexus Carcinomas With TP53 Germline Mutations: Management and Outcome.

Frontiers in oncology
2022

Atypical choroid plexus papilloma: A case report.

Asian journal of surgery
2021

Freiburg Neuropathology Case Conference : A 6-year-old Girl Presenting with Vomiting and Right-sided Facial Paresis.

Clinical neuroradiology
2021

Choroid plexus carcinoma with leptomeningeal spread in an adult: a case report and  review of the literature.

Journal of medical case reports
2021

Triple intraventricular chemotherapy for treatment of relapsed choroid plexus carcinoma.

Pediatric blood &amp; cancer
2021

Preoperative Devascularization of Choroid Plexus Tumors: Specific Issues about Anatomy and Embolization Technique.

Brain sciences
2021

Intracranial Choroid Plexus Carcinomas: Report of 11 Cases from a Single Institution.

World neurosurgery
2021

Choroid plexus carcinoma in an adolescent male: a case report.

Journal of medical case reports
2021

Cerebellopontine Angle Primary Choroid Plexus Carcinoma Present in an Adult: Case Report and Literature Review.

Cureus
2022

A choroid plexus metastasis of a prostatic adenocarcinoma mimicking a choroid plexus carcinoma: A case report.

Neuro-Chirurgie
2021

Molecular insights into malignant progression of atypical choroid plexus papilloma.

Cold Spring Harbor molecular case studies
2021

Benefit of Static FET PET in Pretreated Pediatric Brain Tumor Patients with Equivocal Conventional MRI Results.

Klinische Padiatrie
2021

Outcome and molecular analysis of young children with choroid plexus carcinoma treated with non-myeloablative therapy: results from the SJYC07 trial.

Neuro-oncology advances
2021

Management of choroid plexus tumours: A comprehensive study from a tertiary hospital.

Clinical neurology and neurosurgery
2021

Synchronous choroid plexus papilloma and Wilms tumor in a girl, disclosing a Li-Fraumeni syndrome.

Hereditary cancer in clinical practice
2021

Pediatric atypical choroid plexus papilloma: Clinical features and diagnosis.

Clinical neurology and neurosurgery
2021

A Unique Case of Cerebellar Choroid Plexus Carcinoma.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2022

Choroid Plexus Carcinoma with Hyaline Globules: An Uncommon Histological Finding.

Fetal and pediatric pathology
2020

Intraventricular choroid plexus tumors: clinical characteristics and impact of current management on survival.

Journal of neuro-oncology
2020

Frequency of the TP53 p.R337H mutation in a Brazilian cohort of pediatric patients with solid tumors.

Molecular biology reports
2020

Delayed 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 oncology
2020

Late recurrence of choroid plexus carcinoma.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2020

Epigenetics impacts upon prognosis and clinical management of choroid plexus tumors.

Journal of neuro-oncology
2020

An update on the CNS manifestations of brain tumor polyposis syndromes.

Acta neuropathologica
2020

Case 2: A Rare Cause of Intraventricular Hemorrhage in a Term Neonate.

Pediatrics in review
2019

Multiple High Grade Rhabdoid Papillary Meningiomas Mimicking Choroid Plexus Carcinoma: A Case Report.

Iranian journal of pathology
2020

Moderate-to-strong expression of FGFR3 and TP53 alterations in a subpopulation of choroid plexus tumors.

Histology and histopathology
2020

Choroid Plexus Carcinoma in Adults: Literature Review and First Report of a Location into the Third Ventricle.

World neurosurgery
2019

Next generation DNA sequencing of atypical choroid plexus papilloma of brain: Identification of novel mutations in a female patient by Ion Proton.

Oncology letters
2019

Transventricular Migration of Choroid Plexus Carcinoma Causing an Intraoperative Conundrum: A Case Report with a Review of the Literature.

Pediatric neurosurgery
2020

An update on the central nervous system manifestations of Li-Fraumeni syndrome.

Acta neuropathologica
2019

DNA methylation signature is prognostic of choroid plexus tumor aggressiveness.

Clinical epigenetics
2020

Subgroup-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 Neurosurgery
2019

Structure-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 chemistry
2019

Choroid Plexus Carcinoma in Adults: Two Case Reports.

Brain tumor research and treatment
2020

An Intraventricular Type of Chondroma: A Case Report.

Turkish neurosurgery
2019

Clinical 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 biology
2019

Perinatal (fetal and neonatal) choroid plexus tumors: a review.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2019

Choroid plexus tumours on MRI: similarities and distinctions in different grades.

Cancer imaging : the official publication of the International Cancer Imaging Society
2021

Multimodality characteristics of multifocal choroid plexus carcinoma with bilateral calvarial defects in a dog.

Veterinary radiology &amp; ultrasound : the official journal of the American College of Veterinary Radiology and the International Veterinary Radiology Association
2019

Myc and Loss of p53 Cooperate to Drive Formation of Choroid Plexus Carcinoma.

Cancer research
2019

Management of choroid plexus tumors-an institutional experience.

Acta neurochirurgica
2018

Rare TP53 variant associated with Li-Fraumeni syndrome exhibits variable penetrance in a Saudi family.

NPJ genomic medicine
2018

Clinical Features and Prognostic Risk Factors of Choroid Plexus Tumors in Children.

Chinese medical journal
2019

Management of Choroid Plexus Tumors in Infants and Young Children Up to 4 Years of Age: An Institutional Experience.

World neurosurgery
2018

MRI of intracranial intraventricular lesions.

Clinical imaging
2018

Rapid malignant progression of an intraparenchymal choroid plexus papillomas.

Surgical neurology international
2018

Widely Metastatic Choroid Plexus Carcinoma Associated with Novel TP53 Somatic Mutation.

World neurosurgery
2019

Report of a bi-allelic truncating germline mutation in TP53.

Familial cancer
2019

Intracranial Neoplasms in the First Year of Life: Results of a Third Cohort of Patients From a Single Institution.

Neurosurgery
2017

Increasing genomic instability during cancer therapy in a patient with Li-Fraumeni syndrome.

Clinical and translational radiation oncology
2018

Comparative Cytogenetic Analysis of Dog and Human Choroid Plexus Tumors Defines Syntenic Regions of Genomic Loss.

Journal of neuropathology and experimental neurology
2018

Roles of the apparent diffusion coefficient and tumor volume in predicting tumor grade in patients with choroid plexus tumors.

Neuroradiology
2018

Mice Expressing Myc in Neural Precursors Develop Choroid Plexus and Ciliary Body Tumors.

The American journal of pathology
2018

Pediatric choroid plexus carcinoma: Biologically and clinically in need of new perspectives.

Pediatric blood &amp; cancer
2018

Choroid plexus carcinoma with neuronal and glial differentiation in a 7-week-old male Sprague-Dawley rat.

The Journal of veterinary medical science
2018

Glomeruloid Microvascular Proliferation, Desmoplasia, and High Proliferative Index as Potential Indicators of High Grade Canine Choroid Plexus Tumors.

Veterinary pathology
2018

A new genetically engineered mouse model of choroid plexus carcinoma.

Biochemical and biophysical research communications
2018

A Case of Giant Supratentorial Choroid Plexus Carcinoma in a Young Girl.

Pediatric neurosurgery
2018

Establishing a Preclinical Multidisciplinary Board for Brain Tumors.

Clinical cancer research : an official journal of the American Association for Cancer Research
2018

Screening with whole-body magnetic resonance imaging in pediatric subjects with Li-Fraumeni syndrome: A single institution pilot study.

Pediatric blood &amp; cancer
2018

Revisiting tumor patterns and penetrance in germline TP53 mutation carriers: temporal phases of Li-Fraumeni syndrome.

Current opinion in oncology
2017

Constitutional mosaicism of a de novo TP53 mutation in a patient with bilateral choroid plexus carcinoma.

Cancer genetics
2017

Molecular Guided Therapy Provides Sustained Clinical Response in Refractory Choroid Plexus Carcinoma.

Frontiers in pharmacology
2017

Effect 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, physics
2017

Successful 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 Neurosurgery
2017

Successful Treatment of Recurrent Li-Fraumeni Syndrome-related Choroid Plexus Carcinoma.

Journal of pediatric hematology/oncology
2017

Spinal drop metastasis from a benign fourth ventricular choroid plexus papilloma in a pediatric patient: case report.

Journal of neurosurgery. Pediatrics
2017

LC3A Silencing Hinders Aggresome Vimentin Cage Clearance in Primary Choroid Plexus Carcinoma.

Scientific reports
2017

Magnetic Resonance Imaging and Clinical Findings Associated with Choroid Plexus Spinal Cord "Drop" Metastases.

Journal of the American Animal Hospital Association
2017

Increased NG2 and SOX2 expression is associated with high-grade choroid plexus tumors.

Oncology letters
2017

Marrow-ablative chemotherapy followed by tandem autologous hematopoietic cell transplantation in pediatric patients with malignant brain tumors.

Bone marrow transplantation
2017

Atypical choroid plexus papilloma: spontaneous resolution of diffuse leptomeningeal contrast enhancement after primary tumor removal in 2 pediatric cases.

Journal of neurosurgery. Pediatrics
2017

A French retrospective study on clinical outcome in 102 choroid plexus tumors in children.

Journal of neuro-oncology
2017

A rare case of choroid plexus carcinoma that led to the diagnosis of Lynch syndrome (hereditary nonpolyposis colorectal cancer).

Clinical neurology and neurosurgery
2017

Targeted next-generation sequencing of pediatric neuro-oncology patients improves diagnosis, identifies pathogenic germline mutations, and directs targeted therapy.

Neuro-oncology
2017

Neoadjuvant chemotherapy for atypical teratoid rhabdoid tumors: case report.

Journal of neurosurgery. Pediatrics
2017

Choroid plexus tumors in adult and pediatric populations: the Cleveland Clinic and University Hospitals experience.

Journal of neuro-oncology
2017

Clinicopathological 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 Neurosurgery
2017

Concurrent Gliosarcoma and Choroid Plexus Carcinoma in a Cow.

Journal of comparative pathology
2016

Risks of first and subsequent cancers among TP53 mutation carriers in the National Cancer Institute Li-Fraumeni syndrome cohort.

Cancer
2016

Infant brain tumours: a tale of two cities.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2016

Kir7.1 immunoreactivity in canine choroid plexus tumors.

Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, Inc
2016

Choroid plexus carcinoma with hyaline globules: An unusual histological feature.

Indian journal of pathology &amp; microbiology
2016

Methylation profiling of choroid plexus tumors reveals 3 clinically distinct subgroups.

Neuro-oncology
2015

USE 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 Croatica
2016

Preclinical studies of 5-fluoro-2'-deoxycytidine and tetrahydrouridine in pediatric brain tumors.

Journal of neuro-oncology
2015

Choroid Plexus Tumors: Experience of 10 Cases with Special References to Adult Cases.

Neurologia medico-chirurgica
2015

Occurrence of Neuroblastoma among TP53 p.R337H Carriers.

PloS one
2015

Choroid Plexus Carcinoma in the External Ventricle of an Adult.

The Journal of craniofacial surgery
2016

A 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 Neuropathology
2016

Pre-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
2016

[Choroid plexus tumours in childhood: Experience in Sant Joan de Déu hospital].

Neurocirugia (Asturias, Spain)
2015

Choroid plexus carcinoma in adults: an extremely rare case.

The Pan African medical journal
2015

Choroid Plexus Neoplasms: Toward a Distinction between Carcinoma and Papilloma Using Arterial Spin-Labeling.

AJNR. American journal of neuroradiology
2015

Concurrent Gain of Oncogenes Drives Choroid Plexus Carcinoma.

Cancer discovery
2015

Cross-Species Genomics Identifies TAF12, NFYC, and RAD54L as Choroid Plexus Carcinoma Oncogenes.

Cancer cell
2015

Radiation therapy for choroid plexus carcinoma patients with Li-Fraumeni syndrome: advantageous or detrimental?

Anticancer research
2015

Neoadjuvant chemotherapy reduces blood loss during the resection of pediatric choroid plexus carcinomas.

Journal of neurosurgery. Pediatrics
2015

Phase 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 Neurosurgery
2015

The 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 Neurosurgery
2015

A child with Li-Fraumeni syndrome: Modes to inactivate the second allele of TP53 in three different malignancies.

Pediatric blood &amp; cancer
2015

[Choroid plexus tumour treatment at Hospital Infantil Niño Jesús in Madrid: Our experience over the last three decades].

Neurocirugia (Asturias, Spain)
2015

Neoadjuvant chemotherapy for brain tumors in infants and young children.

Journal of neurosurgery. Pediatrics
2015

Choroid plexus carcinoma in children: the Head Start experience.

Pediatric blood &amp; cancer
Ver todos os 212 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Carcinoma do plexo coroide.

É de uma associação que acompanha esta doença? Fale com a gente →

Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Carcinoma do plexo coroide

Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.

Tire suas dúvidas

Perguntas, dicas e experiências compartilhadas aqui na página

Participe da discussão

Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.

Fazer login

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ainda não achamos doenças com sintomas parecidos o suficiente.

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.

  1. Risk Factors for Delayed Leptomeningeal Dissemination in Choroid Plexus Papillomas: A Systematic Review and Illustrative Case.
    Current oncology (Toronto, Ont.)· 2026· PMID 41744878mais citado
  2. Neoadjuvant Chemotherapy in Choroid Plexus Carcinoma: Improving Surgical Safety and Resection Outcomes.
    JPMA. The Journal of the Pakistan Medical Association· 2026· PMID 41736349mais citado
  3. Phase I/II study of the PARP inhibitor olaparib and irinotecan in children and young adults with recurrent/refractory malignancies: Arm D of the AcS&#xe9;-ESMART trial.
    Clinical cancer research : an official journal of the American Association for Cancer Research· 2026· PMID 41591981mais citado
  4. Gene context drift identifies drug targets to mitigate cancer treatment resistance.
    Cancer cell· 2025· PMID 40578362mais citado
  5. The role of preoperative chemotherapy in pediatric neuro-oncology: a single-center experience and a systematic review of the literature.
    Journal of neurosurgery. Pediatrics· 2025· PMID 40279718mais citado
  6. Choroid plexus carcinoma: state of the field and emerging directions.
    Oncogenesis· 2026· PMID 41942479recente
  7. Choroid Plexus Mimicry: Metastatic Papillary Lung Adenocarcinoma in the Fourth Ventricle.
    Cureus· 2025· PMID 41625814recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:251899(Orphanet)
  2. MONDO:0016718(MONDO)
  3. GARD:8238(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. 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

Carcinoma do plexo coroide
Compêndio · Raras BR

Carcinoma do plexo coroide

ORPHA:251899 · MONDO:0016718
Prevalência
1-9 / 1 000 000
Herança
Autosomal dominant
CID-10
C71.7 · Neoplasia maligna do tronco cerebral
CID-11
Ensaios
4 ativos
Início
Childhood
Prevalência
0.0 (Europe)
MedGen
UMLS
C0346289
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

Nenhuma novidade ainda. O agente esta monitorando.

0membros
0novidades