Introdução
O que você precisa saber de cara
Papiloma do plexo coroide, também conhecido como papiloma do plexo coroide, é uma lesão neuroepitelial intraventricular rara, benigna e de grau I da OMS, encontrada no plexo coroide. Ela leva ao aumento da produção de líquido cefalorraquidiano, causando assim aumento da pressão intracraniana e hidrocefalia.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
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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 — Papiloma atípico do plexo coroide
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Ensaios clínicos abertos e novidades científicas recentes
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1 ensaios clínicos encontrados.
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.
Spinal drop metastases of choroid plexus papilloma: a brief report and updated literature review.
Choroid plexus papillomas (CPPs) are rare central nervous system benign tumours and spinal metastases are even less frequent. Herein, we describe a case of a 51-year-old man with several episodes of loss of consciousness. Brain magnetic resonance imaging (MRI) displayed a fourth ventricle contrast-enhancing lesion that was totally resected, and histopathological findings showed a papillary neoplasm derived from choroid plexus epithelium, with mild pleomorphism and absence of mitotic activity, consistent with CPP. Two months after the first operation, multiple spinal drop metastases at L4, L5 and S1-S2 were revealed through spine MRI. Histological examination of the spinal lesions fulfilled as well the diagnostic criteria for CPP. Radiological and histological findings are presented, and the relevant literature of spinal spreading of CPPs is discussed. This special case, together with the literature review, helps expanding the spectrum of knowledge on benign CPPs, their potential for disease spread and progression and highlights the need to identify biomarkers that correlate with clinical behaviour.
Extraventricular Choroid Plexus Papilloma: A Case Report.
The patient is a 25-year-old female transferred from an outside facility with week-long complaints of a headache, nausea, and vomiting. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed obstructive hydrocephalus and a large cystic mass of the posterior fossa. Subsequent external ventricular drain placement and surgical resection were performed. The patient was discharged six days postoperatively with resolution of symptoms. Pathological examination of the fleshy component within the cystic structure after surgical resection was relatively inconclusive, with findings suggestive of a choroid plexus tumor. However, the lesion demonstrates the immunohistochemical characteristics of a choroid plexus papilloma (CPP) but with a cystic structure surrounding it. CPPs are rare and typically benign tumors that originate from the choroid plexus, which is responsible for cerebrospinal fluid (CSF) production. These growths can occur throughout the ventricular system of the brain, more commonly in the lateral ventricles and less commonly in the third and fourth ventricles. Differential diagnosis based on imaging is broad due to the shared visual characteristics of various brain tumors. Consequently, immunohistochemical markers are essential for the identification and diagnosis of CPPs. In the case of our patient, the small enhancing nodule is unusual in that it is surrounded by a large cystic lesion. We present the unusual case of a cystic tumor of the posterior fossa with a small nodular component located within, suggestive of a CPP by immunohistochemical and morphological characteristics. A multidisciplinary diagnostic approach is necessary to exact a proper classification of lesions such as this one, including identification by neuroradiology, resection by neurosurgery, and immunohistochemical and morphological analysis by pathology.
An overview of the diagnosis and management of Choroid Plexus tumors.
Choroid Plexus Tumors (CPT) are rare (2-4% of all pediatric CNS tumors), predominantly early childhood brain neoplasms. Due to their rarity and the lack of prospective clinical trials, evidence-based treatment guidelines remain limited. This review provides a comprehensive summary of the current knowledge.\n\nCPTs span a spectrum from mature Choroid Plexus Papillomas (CPP) to malignant Choroid Plexus Carcinomas (CPC), with Atypical Choroid Plexus Papillomas (aCPP) in between. A significant proportion of CPCs are driven by either somatic or germline TP53 mutations (Li-Fraumeni syndrome); however, other molecular drivers of CPT tumorigenesis remain poorly understood. CPTs exhibit distinct DNA methylation profiles, allowing for classification into clinically relevant subgroups. These tumors also display a chromosomal instability phenotype, characterized by multiple copy number alterations. \n\nAdvances in molecular profiling have revealed that TP53-mutated CPCs have significantly worse outcomes. Both retrospective and limited prospective data have shown 5-year event-free survival rates of 0-25% for TP53-mutant CPCs versus 70-80% for TP53-wild-type cases. The extent of surgical resection remains another established prognostic factor, while data on the roles of radiation therapy (RT) and myeloablative chemotherapy with stem cell rescue are still evolving. Preliminary evidence suggests that TP53-mutant patients may be getting less benefit from RT, but greater benefit from myeloablative chemotherapy approach with avoidance of RT.\n\n The emerging insights into CPC biology and long-term outcomes can direct the design of future clinical trials. A new international prospective study led by the Pediatric Neuro-Oncology Consortium is in development, with the goal to stratify patients by molecular subtype to receive different therapy based on individual molecular profiles and patient age.
Predictive factors in the management of hydrocephalus and cerebrospinal fluid-related complications in pediatric choroid plexus tumors: a single-center experience.
The intimate relation between hydrocephalus and pediatric choroid plexus tumors (CPTs) should receive special care owing to the high incidence of permanent cerebrospinal fluid (CSF) diversion in those patients. To our knowledge, this is the first study that analyzes predictive factors linked to permanent CSF diversion in children with CPTs. We included all cases that were treated between January 2012 and December 2022 upfront in our institute, with a follow-up from 12 to 106 months. Children in whom ventriculoperitoneal shunts were placed before tumor excision were excluded. Demographic data, radiological findings, operative details, and postoperative management of hydrocephalus and CSF-related complications were reviewed. A total of 80 children with CPTs were included in our study; 58 children (72.5%) needed permanent CSF diversion methods. CSF permeation in FLAIR/T2 sequence (p-value 0.05), longer duration of external ventricular drainage (p-value 0.032), and horn entrapment (p-value 0.03) were related to permanent CSF diversion. Location of the tumor, maximum tumor diameter, consistency, preoperative Evan's index, and amount of blood in the ventricles were not statistically significant. We found that tumor pathology, parenchymal invasion, and extent of resection were statistically significant factors in relation to postoperative entrapment (p-values = 0.016, 0.001, 0.042). Hydrocephalus has an intimate relationship with pediatric choroid plexus tumors. Placement of a shunt could be inevitable at the end for most of them. However, transependymal CSF permeation in preoperative imaging, duration of EVD placement, and entrapment due to parenchymal invasion or high-grade disease are associated with higher rates of CSF diversion.
Publicações recentes
Risk Factors for Delayed Leptomeningeal Dissemination in Choroid Plexus Papillomas: A Systematic Review and Illustrative Case.
Spinal drop metastases of choroid plexus papilloma: a brief report and updated literature review.
Atypical Choroid Plexus Papilloma in a Newborn, Misdiagnosed by Ultrasound and MRI: A Case Report.
Tractography Navigated Endoscopic En-Bloc Excision of Pediatric Giant Choroid Plexus Tumor.
The composition of choroid plexus tumor research: a bibliometric analysis of the 100 most impactful studies to date.
📚 EuropePMCmostrando 107
Risk Factors for Delayed Leptomeningeal Dissemination in Choroid Plexus Papillomas: A Systematic Review and Illustrative Case.
Current oncology (Toronto, Ont.)Spinal drop metastases of choroid plexus papilloma: a brief report and updated literature review.
PathologicaExtraventricular Choroid Plexus Papilloma: A Case Report.
CureusAn overview of the diagnosis and management of Choroid Plexus tumors.
Advances in cancer researchPredictive factors in the management of hydrocephalus and cerebrospinal fluid-related complications in pediatric choroid plexus tumors: a single-center experience.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryPaediatric cranial ultrasound: abnormalities of the brain in term neonates and young infants.
Insights into imagingOncocytic Features in Choroid Plexus Tumors: An Integrative Clinicopathological Study.
CureusCPT-SIOP registry: evaluation of risk factors for disease progression in pediatric choroid plexus papilloma.
Journal of neuro-oncologyA Rare Case of Atypical Choroid Plexus Papilloma in an Adult Male Patient: A Case Report.
Case reports in pathologyAtypical choroid plexus papilloma arising in a mature ovarian teratoma, a case report.
Pathology internationalLeptomeningeal Dissemination in Choroid Plexus Tumors: Magnetic Resonance Imaging Appearance and Risk Factors.
Children (Basel, Switzerland)Atypical Choroid Plexus Papilloma in a Newborn, Misdiagnosed by Ultrasound and MRI: A Case Report.
The American journal of case reportsAtypical Choroid Plexus Tumor of the Cauda Equina With Metastases to the Spinal Cord and Brain.
CureusDiagnosis of choroid plexus papilloma: Current perspectives and future directions.
Cancer pathogenesis and therapyClinical utility of DNA methylation profiling for choroid plexus tumors.
Neuro-oncology advancesDeveloping a nomogram based on SEER database for predicting prognosis in choroid plexus tumors.
Scientific reportsAtypical choroid plexus papilloma: Diagnosis and differentials: A case report.
SAGE open medical case reportsForamen of Monro choroid plexus papilloma: An extremely rare location managed by endoscopic resection.
Radiology case reportsPrognostic Factors and Nomogram for Choroid Plexus Tumors: A Population-Based Retrospective Surveillance, Epidemiology, and End Results Database Analysis.
CancersTractography Navigated Endoscopic En-Bloc Excision of Pediatric Giant Choroid Plexus Tumor.
World neurosurgeryCerebellopontine angle meningioma originating from choroid plexus of the foramen of Luschka: An extremely rare entity and overlook of literature.
International journal of surgery case reportsThe 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 NeurosurgeryRare Case of Hemorrhagic Atypical Choroid Plexus Papilloma of Fourth Ventricle in Adult: A Diagnostic Dilemma.
World neurosurgeryPure endoscopic ultrasonic removal of choroid plexus papillomas of the third ventricle: technical report of two cases.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryDistinct challenge: the case report of disseminated adult atypical choroid plexus papilloma.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyGenomic profile of two Brazilian choroid plexus tumors by whole-exome sequencing.
Cold Spring Harbor molecular case studiesInfratentorial atypical choroid plexus papilloma in an adult: A case report and literature review.
Indian journal of cancerIncidence and survival of choroid plexus tumors in the United States.
Neuro-oncology practiceA Prospective Study of Conventionally Fractionated Dose Constraints for Reirradiation of Primary Brain Tumors in Adults.
Practical radiation oncologyEmbolization of a third ventricular choroid plexus papilloma: illustrative case.
Journal of neurosurgery. Case lessonsCase Report: Reversible Hyperglycemia Following Rapamycin Treatment for Atypical Choroid Plexus Papilloma in an Infant.
Frontiers in endocrinologyAtypical Choroid Plexus Papilloma of the Fourth Ventricle in an Adult: A Case Report.
CureusGiant Pediatric Supratentorial Tumor: Clinical Feature and Surgical Strategy.
Frontiers in pediatricsDisruption of GMNC-MCIDAS multiciliogenesis program is critical in choroid plexus carcinoma development.
Cell death and differentiationPrognostic Implication of DNA Methylation Signature in Atypical Choroid Plexus Papilloma With Intracranial Dissemination.
Journal of pediatric hematology/oncologyFinal results of the Choroid Plexus Tumor study CPT-SIOP-2000.
Journal of neuro-oncology"Benign" Choroid Plexus Papilloma with "Atypical" Transformation and Spinal Drop Lesions.
Neurology IndiaA case report of atypical choroid plexus papilloma in the cervicothoracic spinal cord.
Radiology case reportsUreaplasma parvum meningitis following atypical choroid plexus papilloma resection in an adult patient: a case report and literature review.
BMC infectious diseasesIsolated Lumbar Atypical Choroid Plexus Papilloma: A Case Report.
Turkish neurosurgeryChoroid Plexus Tumors in Children: Long-Term Follow-Up of Consecutive Single-Institutional Series of 59 Patients Treated over a Period of 8 Decades (1939-2020).
World neurosurgeryThe use of exoscope combined with tubular retractor system for minimally invasive transsulcal resection of an ventricular atrium atypical choroid plexus papilloma: Three-dimensional operative video.
Surgical neurology internationalAtypical choroid plexus papilloma: A case report.
Asian journal of surgerySpinal ectopic choroid plexus papilloma in a cat.
JFMS open reportsPaediatric atypical choroid plexus papilloma: is adjuvant therapy necessary?
Journal of neuro-oncologyIntracranial Choroid Plexus Carcinomas: Report of 11 Cases from a Single Institution.
World neurosurgeryBilateral Choroid Plexus Papillomas Diagnosed by Prenatal Ultrasound and MRI.
CureusMolecular insights into malignant progression of atypical choroid plexus papilloma.
Cold Spring Harbor molecular case studiesManagement of choroid plexus tumours: A comprehensive study from a tertiary hospital.
Clinical neurology and neurosurgeryNot So Benign: A Rare Atypical Ectopic Choroid Plexus Papilloma.
Journal of ophthalmology & visual sciencesSpinal drop of atypical choroid plexus papilloma in an adult patient: A case report and literature review.
Surgical neurology internationalRole of available adjuvant therapies following surgical resection of atypical choroid plexus papilloma-a systematic review and pooled analysis.
Neuro-oncology advancesThe genetic landscape of choroid plexus tumors in children and adults.
Neuro-oncologyPediatric atypical choroid plexus papilloma: Clinical features and diagnosis.
Clinical neurology and neurosurgeryAn atypical presentation of primary central nervous system lymphoma: A case report.
MedicineIntraventricular choroid plexus tumors: clinical characteristics and impact of current management on survival.
Journal of neuro-oncologyEpigenetics impacts upon prognosis and clinical management of choroid plexus tumors.
Journal of neuro-oncologyPrimary papillary epithelial tumour of the sella: expanding the spectrum of TTF-1-positive sellar lesions.
Neuropathology and applied neurobiologyBevacizumab in progressive disseminated atypical choroid plexus papilloma in adults.
Neuro-oncologyInfratentorial choroid plexus tumors in children.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryModerate-to-strong expression of FGFR3 and TP53 alterations in a subpopulation of choroid plexus tumors.
Histology and histopathologyNext generation DNA sequencing of atypical choroid plexus papilloma of brain: Identification of novel mutations in a female patient by Ion Proton.
Oncology lettersThe cerebellar peduncle as localization of a recurrent atypical plexus papilloma: A case report.
Surgical neurology internationalDNA methylation signature is prognostic of choroid plexus tumor aggressiveness.
Clinical epigeneticsClinicopathologic Features, Diagnosis, and Characterization of the Immune Cell Population in Canine Choroid Plexus Tumors.
Frontiers in veterinary scienceChoroid Plexus Papilloma of the Fourth Ventricle: A Pediatric Patient.
Asian journal of neurosurgeryCerebrospinal fluid cytology of choroid plexus tumor: A report of two cases.
CytoJournalPerinatal (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 SocietyRapidly growing, multifocal, benign choroid plexus tumor in an infant: case report.
Journal of neurosurgery. PediatricsManagement of choroid plexus tumors-an institutional experience.
Acta neurochirurgicaClinical Features and Prognostic Risk Factors of Choroid Plexus Tumors in Children.
Chinese medical journalA comparison of pseudo-continuous arterial spin labelling and dynamic susceptibility contrast MRI with and without contrast agent leakage correction in paediatric brain tumours.
The British journal of radiologyENDOSCOPIC ASSISTED, TRANSFONTANELLE EXCISION OF A LARGE THIRD VENTRICULAR ATYPICAL CHOROID PLEXUS PAPILLOMA IN AN INFANT.
Journal of the West African College of SurgeonsManagement of Choroid Plexus Tumors in Infants and Young Children Up to 4 Years of Age: An Institutional Experience.
World neurosurgeryRapid malignant progression of an intraparenchymal choroid plexus papillomas.
Surgical neurology internationalImmunohistochemical Analysis of Cerebral Intraparenchymal Choroid Plexus Tumor: Case Report.
Journal of neurological surgery. Part A, Central European neurosurgeryRevisiting pleuropulmonary blastoma and atypical choroid plexus papilloma in a young child: DICER1 syndrome or not?
Pediatric blood & cancerA unique presentation of an adolescent with disseminated atypical choroid plexus papilloma.
Journal of neuro-oncologyIntracranial Neoplasms in the First Year of Life: Results of a Third Cohort of Patients From a Single Institution.
NeurosurgeryRoles of the apparent diffusion coefficient and tumor volume in predicting tumor grade in patients with choroid plexus tumors.
NeuroradiologyAtypical Teratoid/Rhabdoid Tumor After In Vitro Fertilization: Illustrative Case Report and Systematic Literature Review.
World neurosurgeryGlomeruloid Microvascular Proliferation, Desmoplasia, and High Proliferative Index as Potential Indicators of High Grade Canine Choroid Plexus Tumors.
Veterinary pathology[Central nervous system infection by Bartonella henselae associated with a choroid plexus papilloma].
Revista chilena de infectologia : organo oficial de la Sociedad Chilena de InfectologiaPlasticity of the human visual brain after an early cortical lesion.
NeuropsychologiaSpinal drop metastasis from a benign fourth ventricular choroid plexus papilloma in a pediatric patient: case report.
Journal of neurosurgery. PediatricsIncreased NG2 and SOX2 expression is associated with high-grade choroid plexus tumors.
Oncology lettersAtypical 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-oncologyUltrasonic elastography exploration of the foetal brain: A case of atypical choroid plexus papilloma.
Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and GynaecologyAtypical choroid plexus papilloma: clinicopathological and neuroradiological features.
Acta radiologica (Stockholm, Sweden : 1987)Choroid 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 NeurosurgeryMetachronous Type I pleuropulmonary blastoma and atypical choroid plexus papilloma in a young child.
Pediatric blood & cancerChoroid Plexus Papillomas of the Cerebellopontine Angle.
World neurosurgeryDisseminated choroid plexus papillomas in adults: A case series and review of the literature.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaMethylation profiling of choroid plexus tumors reveals 3 clinically distinct subgroups.
Neuro-oncologyMicrosurgical treatment of atypical choroid plexus papilloma in the fourth ventricle.
Acta neurologica Belgica[Choroid plexus tumours in childhood: Experience in Sant Joan de Déu hospital].
Neurocirugia (Asturias, Spain)Extraventricular Intraparenchymal Choroid Plexus Tumors in Cerebral Hemisphere: A Series of 6 Cases.
World neurosurgeryClinicopathologic study of endolymphatic sac tumor (ELST) and differential diagnosis of papillary tumors located at the cerebellopontine angle.
Neuropathology : official journal of the Japanese Society of NeuropathologyPediatric atypical choroid plexus papilloma reconsidered: increased mitotic activity is prognostic only in older children.
Acta neuropathologicaThe 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 NeurosurgeryDelayed Diagnosis of Enhancing Posterior Fossa Tumors Mimicking the Tela Choroidea of the Fourth Ventricle.
World neurosurgeryCombined rigid and flexible endoscopy for tumors in the posterior third ventricle.
Journal of neurosurgery[Choroid plexus tumour treatment at Hospital Infantil Niño Jesús in Madrid: Our experience over the last three decades].
Neurocirugia (Asturias, Spain)High-resolution genomic analysis does not qualify atypical plexus papilloma as a separate entity among choroid plexus tumors.
Journal of neuropathology and experimental neurologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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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.
- Spinal drop metastases of choroid plexus papilloma: a brief report and updated literature review.
- Extraventricular Choroid Plexus Papilloma: A Case Report.
- An overview of the diagnosis and management of Choroid Plexus tumors.
- Predictive factors in the management of hydrocephalus and cerebrospinal fluid-related complications in pediatric choroid plexus tumors: a single-center experience.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery· 2025· PMID 40844614mais citado
- Atypical Choroid Plexus Papilloma in a Newborn, Misdiagnosed by Ultrasound and MRI: A Case Report.
- Tractography Navigated Endoscopic En-Bloc Excision of Pediatric Giant Choroid Plexus Tumor.
- The composition of choroid plexus tumor research: a bibliometric analysis of the 100 most impactful studies to date.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:251902(Orphanet)
- MONDO:0002684(MONDO)
- GARD:20723(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q758294(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
