Raras
Buscar doenças, sintomas, genes...
Tumor teratoide, atípico
ORPHA:99966CID-10 · C49.9CID-11 · 2A00.1YDOENÇA RARA

O ATRT (tumor rabdoide teratoide atípico) é um tumor rabdoide (TR) muito agressivo do sistema nervoso central (SNC), que afeta quase exclusivamente crianças.

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Introdução

O que você precisa saber de cara

📋

O ATRT (tumor rabdoide teratoide atípico) é um tumor rabdoide (TR) muito agressivo do sistema nervoso central (SNC), que afeta quase exclusivamente crianças.

Pesquisas ativas
23 ensaios
134 total registrados no ClinicalTrials.gov
Publicações científicas
837 artigos
Último publicado: 2026 Mar 23

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Antenatal
+ childhood, infancy, neonatal
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C49.9
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

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

Partes do corpo afetadas

🧠
Neurológico
6 sintomas
🫘
Rins
1 sintomas
💪
Músculos
1 sintomas
🦴
Ossos e articulações
1 sintomas
🫃
Digestivo
1 sintomas

+ 8 sintomas em outras categorias

Características mais comuns

90%prev.
Irritabilidade
Muito frequente (99-80%)
90%prev.
Apatia
Muito frequente (99-80%)
90%prev.
Neoplasia maligna do sistema nervoso central
Muito frequente (99-80%)
90%prev.
Náusea e vômito
Muito frequente (99-80%)
55%prev.
Ataxia
Frequente (79-30%)
55%prev.
Fraqueza muscular
Frequente (79-30%)
18sintomas
Muito frequente (4)
Frequente (9)
Ocasional (3)
Sem dados (2)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 18 características clínicas mais associadas, ordenadas por frequência.

IrritabilidadeIrritability
Muito frequente (99-80%)90%
ApatiaApathy
Muito frequente (99-80%)90%
Neoplasia maligna do sistema nervoso centralMalignant neoplasm of the central nervous system
Muito frequente (99-80%)90%
Náusea e vômitoNausea and vomiting
Muito frequente (99-80%)90%
Ataxia
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico837PubMed
Últimos 10 anos200publicações
Pico202560 papers
Linha do tempo
2026Hoje · 2026🧪 1992Primeiro ensaio clínico📈 2025Ano 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

2 genes identificados com associação a esta condição. Padrão de herança: Not applicable.

SMARCB1SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1Disease-causing somatic mutation(s) inAltamente restrito
FUNÇÃO

Core component of the BAF (hSWI/SNF) complex. This ATP-dependent chromatin-remodeling complex plays important roles in cell proliferation and differentiation, in cellular antiviral activities and inhibition of tumor formation. The BAF complex is able to create a stable, altered form of chromatin that constrains fewer negative supercoils than normal. This change in supercoiling would be due to the conversion of up to one-half of the nucleosomes on polynucleosomal arrays into asymmetric structures

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (8)
RUNX1 interacts with co-factors whose precise effect on RUNX1 targets is not knownRMTs methylate histone argininesFormation of neuronal progenitor and neuronal BAF (npBAF and nBAF)Formation of the embryonic stem cell BAF (esBAF) complexFormation of the polybromo-BAF (pBAF) complex
MECANISMO DE DOENÇA

Rhabdoid tumor predisposition syndrome 1

A familial cancer syndrome predisposing to renal or extrarenal malignant rhabdoid tumors and to a variety of tumors of the central nervous system, including choroid plexus carcinoma, medulloblastoma, and central primitive neuroectodermal tumors. Rhabdoid tumors are the most aggressive and lethal malignancies occurring in early childhood.

EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
136.4 TPM
Testículo
110.5 TPM
Cérebro - Hemisfério cerebelar
98.7 TPM
Cerebelo
93.5 TPM
Ovário
93.2 TPM
OUTRAS DOENÇAS (9)
rhabdoid tumor predisposition syndrome 1intellectual disability, autosomal dominant 15familial multiple meningiomaschwannomatosis
HGNC:11103UniProt:Q12824
SMARCA4SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily A member 4Candidate gene tested inAltamente restrito
FUNÇÃO

ATPase involved in transcriptional activation and repression of select genes by chromatin remodeling (alteration of DNA-nucleosome topology). Component of SWI/SNF chromatin remodeling complexes that carry out key enzymatic activities, changing chromatin structure by altering DNA-histone contacts within a nucleosome in an ATP-dependent manner (PubMed:15075294, PubMed:29374058, PubMed:30339381, PubMed:32459350). Component of the CREST-BRG1 complex, a multiprotein complex that regulates promoter ac

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (10)
Formation of the beta-catenin:TCF transactivating complexNegative Regulation of CDH1 Gene TranscriptionRUNX1 interacts with co-factors whose precise effect on RUNX1 targets is not knownRMTs methylate histone argininesFormation of neuronal progenitor and neuronal BAF (npBAF and nBAF)
MECANISMO DE DOENÇA

Rhabdoid tumor predisposition syndrome 2

A familial cancer syndrome predisposing to renal or extrarenal malignant rhabdoid tumors and to a variety of tumors of the central nervous system, including choroid plexus carcinoma, medulloblastoma, and central primitive neuroectodermal tumors. Rhabdoid tumors are the most aggressive and lethal malignancies occurring in early childhood.

EXPRESSÃO TECIDUAL(Ubíquo)
Testículo
81.7 TPM
Cérebro - Hemisfério cerebelar
65.7 TPM
Cerebelo
62.6 TPM
Linfócitos
61.0 TPM
Esôfago - Mucosa
57.8 TPM
OUTRAS DOENÇAS (7)
intellectual disability, autosomal dominant 16otosclerosis 12Coffin-Siris syndromeovarian small cell carcinoma
HGNC:11100UniProt:P51532

Variantes genéticas (ClinVar)

1,111 variantes patogênicas registradas no ClinVar.

🧬 SMARCA4: NM_003072.5(SMARCA4):c.1849del (p.Pro616_Val617insTer) ()
🧬 SMARCA4: NM_003072.5(SMARCA4):c.4318C>T (p.Gln1440Ter) ()
🧬 SMARCA4: NM_003072.5(SMARCA4):c.1761+2T>G ()
🧬 SMARCA4: NM_003072.5(SMARCA4):c.768del (p.Asn259fs) ()
🧬 SMARCA4: NM_003072.5(SMARCA4):c.3229dup (p.Arg1077fs) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 10 variantes classificadas pelo ClinVar.

8
1
1
Patogênica (80.0%)
VUS (10.0%)
Benigna (10.0%)
VARIANTES MAIS SIGNIFICATIVAS
NF1: NM_001042492.3(NF1):c.1721G>C (p.Ser574Thr) [Pathogenic]
SMARCB1: NM_003073.5(SMARCB1):c.1148del (p.Pro383fs) [Pathogenic]
LOC110806263: NM_198253.3(TERT):c.-124C>T [Conflicting classifications of pathogenicity]
SMARCB1: NM_003073.5(SMARCB1):c.601C>T (p.Arg201Ter) [Pathogenic]
TP53: NM_000546.6(TP53):c.560-1G>C [Pathogenic]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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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.
2Fase 29
1Fase 111
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 20 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Tumor teratoide, atípico

🗺️

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

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

Outros ensaios clínicos

134 ensaios clínicos encontrados, 23 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
537 papers (10 anos)
#1

Longitudinal Detection of Tumor-Specific Peptides in Cerebrospinal Fluid for Pediatric Brain Tumor Surveillance.

Cells2026 Mar 05

Pediatric brain tumor survivors remain at high risk of recurrence, yet current surveillance strategies relying on neuroimaging and cerebrospinal fluid (CSF) cytology have limited sensitivity for early or minimal disease. Tumor-specific peptides (TSPs) derived from individual tumors represent a promising class of highly specific biomarkers for longitudinal disease monitoring through CSF-based proteomic analysis. In this study, tumor tissue and serial CSF samples from six pediatric brain tumor patients (five medulloblastomas and one atypical teratoid/rhabdoid tumor (ATRT)) were analyzed using an integrated proteogenomic workflow combining discovery and targeted mass spectrometry. TSPs were identified from resected tumor tissue and matched against shotgun CSF proteomic datasets to nominate candidate biomarkers. High-confidence peptides were synthesized as isotopically labeled standards and quantified longitudinally using targeted multiple reaction monitoring. Two TSP biomarkers derived from individualized pediatric brain tumors (one medulloblastoma and one ATRT) demonstrated robust detection in serial CSF samples and exhibited temporal concordance with radiographic disease course, declining with treatment response and increasing during disease progression. These findings establish the feasibility of detecting and longitudinally quantifying TSPs in CSF and support further investigation of individualized proteomic biomarkers for treatment response monitoring and disease surveillance in pediatric brain tumors.

#2

A Rare Thoracolumbar Spinal Atypical Teratoid/Rhabdoid Tumor in a 7-Year-Old Child: Diagnostic Challenges and Therapeutic Considerations.

International journal of surgical pathology2026 Mar 23

Atypical teratoid/rhabdoid tumor (AT/RT) is a rare, highly aggressive embryonal neoplasm of the central nervous system, primarily diagnosed in children under three years of age, with spinal presentations accounting for fewer than 2% of patients. This report describes an unusual primary thoracolumbar spinal AT/RT in a 7-year-old girl who presented with severe, rapidly progressive pain impairing mobility. Magnetic resonance imaging revealed a large intradural extramedullary mass extending from T11 to L3 with marked compression of the conus medullaris and cauda equina. Surgical resection was performed, and histopathological evaluation confirmed rhabdoid morphology with characteristic immunophenotypic features, including loss of nuclear SMARCB1 (INI1) expression and elevated proliferative index, consistent with AT/RT. This case report highlights the diagnostic complexity due to overlapping features with other pediatric spinal tumors, necessitating comprehensive histological, immunohistochemical, and molecular analyses for accurate classification. Discussion integrates the patient tumor profile within the framework of WHO 2021 molecular subgroups, underscoring the clinical heterogeneity and poor prognosis associated with spinal AT/RT, particularly the aggressive AT/RT-MYC subtype. Despite maximal surgical and multimodal therapy, outcomes remain dismal, emphasizing the urgent need for collaborative research exploring targeted molecular and immunotherapeutic strategies. This case report expands limited literature, offering valuable insights for early recognition and management of spinal AT/RT in older pediatric patients.

#3

Atypical Teratoid/Rhabdoid Tumor of the Posterior Fossa Mimicking Medulloblastoma in an Infant: A Case Report.

Cureus2026 Feb

Atypical teratoid/rhabdoid tumor (AT/RT) is a rare and highly aggressive central nervous system (CNS) neoplasm that predominantly affects children under three years of age. In the posterior fossa, AT/RT frequently mimics medulloblastoma (MB) on both radiological and histopathological evaluation. Owing to significant morphological overlap, a definitive diagnosis cannot be established on histology alone and requires immunohistochemical (IHC) confirmation, particularly demonstration of loss of nuclear integrase interactor 1 (INI1) expression. We report the case of a seven-month-old male infant who presented with signs of intracranial hypertension and rapidly progressive symptoms attributable to posterior fossa mass effect. Neuroimaging revealed a posterior fossa mass causing marked compression of the fourth ventricle and brainstem, resulting in obstructive hydrocephalus. Magnetic resonance imaging demonstrated heterogeneous contrast enhancement, initially suggestive of MB. Cerebrospinal fluid diversion was performed, followed by tumor biopsy and partial surgical resection. Although intraoperative findings and initial histopathological examination were suggestive of MB, subsequent IHC analysis demonstrated complete loss of nuclear INI1 expression, confirming the diagnosis of AT/RT. The patient subsequently received adjuvant chemotherapy. AT/RT should be strongly considered in infants presenting with posterior fossa tumors, particularly when radiological and histopathological features resemble MB. Early tissue diagnosis and routine use of INI1/SMARCB1 IHC are essential for accurate differentiation from morphologically similar entities, ensuring appropriate therapeutic management and prognostic assessment.

#4

Enhanced Delivery of Aurora Kinase A Inhibitor Alisertib via Tumor-Targeting Immunoliposome Nanocomplex for Improved Treatment of Cancers Including Atypical Teratoid/Rhabdoid Tumor.

International journal of nanomedicine2026

This study explores a nanoparticle-based delivery system for alisertib, a selective aurora kinase A (AURKA) inhibitor. This nanocomplex is designed to enhance tumor-specific delivery. AURKA is overexpressed in various cancers, including atypical teratoid/rhabdoid tumors (ATRTs) and is associated with poor clinical outcomes. Alisertib has demonstrated promising efficacy in multiple preclinical studies, which supported its advancement into clinical trials across various cancer types. However, alisertib has not met key efficacy endpoints primarily due to its poor biodistribution to tumors and inadequate penetration of biological barriers, including the blood-brain barrier (BBB). Our study aims to address these issues. To address the limitations of alisertib, we developed scL-ALI, a nanocomplex formulation designed to improve BBB penetration and to enhance tumor uptake of alisertib, to achieve higher effective intratumoral doses. Our new formulation of alisertib comprises a cationic liposome encapsulating the kinase inhibitor. This formulation incorporates an anti-transferrin receptor antibody fragment to enable efficient delivery into the central nervous system and facilitate tumor targeting. Here, we assessed the therapeutic efficacy of nano-formulated scL-ALI versus conventional alisertib utilizing patient-derived tumor cells and xenograft tumor models of various cancer types, including ATRT. Additionally, we investigated the potential synergistic effects of combining scL-ALI with radiation therapy, a standard treatment modality for brain tumors. Compared to conventional alisertib, scL-ALI more effectively inhibited AURKA activity and enhanced tumor cell killing of multiple cancer cell lines. In mouse models of ATRT, glioblastoma, and lung cancer, scL-ALI significantly improved tumor growth inhibition relative to conventional unencapsulared alisertib. Furthermore, when combined with radiation therapy, scL-ALI produced further improved antitumor effects, leading to extended survival in ATRT-bearing mice. These findings highlight the superiority of scL-ALI in overcoming delivery barriers and enhancing therapeutic efficacy of alisertib, while possibly minimizing undesirable side effects in normal tissues. The scL-ALI nanocomplex shows enhanced therapeutic potential for treating AURKA-driven malignancies, particularly in combination with radiation therapy.

#5

Management of Pediatric Brain Tumors with the Aid of PET/MR Imaging.

PET clinics2026 Apr

The review highlights clinical use cases of PET/MR imaging in a pediatric multispecialty neuro-oncology practice with a focus on the role of hybrid imaging with PET/MR imaging, the application of the clinically available tracer fluorodeoxyglucose (18F-FDG), and opportunities in the implementation of amino acid-based tracers. Clinical examples of 18F-FDG hybrid PET/MR imaging use in pediatric diffuse gliomas, medulloblastomas, atypical teratoid rhabdoid tumor, metastatic disease with spine involvement, and cases where a brain tumor presents as a second malignancy are highlighted.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC471 artigos no totalmostrando 197

2026

A Rare Thoracolumbar Spinal Atypical Teratoid/Rhabdoid Tumor in a 7-Year-Old Child: Diagnostic Challenges and Therapeutic Considerations.

International journal of surgical pathology
2026

Atypical Teratoid/Rhabdoid Tumor of the Posterior Fossa Mimicking Medulloblastoma in an Infant: A Case Report.

Cureus
2026

Enhanced Delivery of Aurora Kinase A Inhibitor Alisertib via Tumor-Targeting Immunoliposome Nanocomplex for Improved Treatment of Cancers Including Atypical Teratoid/Rhabdoid Tumor.

International journal of nanomedicine
2026

Longitudinal Detection of Tumor-Specific Peptides in Cerebrospinal Fluid for Pediatric Brain Tumor Surveillance.

Cells
2026

Management of Pediatric Brain Tumors with the Aid of PET/MR Imaging.

PET clinics
2026

Atypical Teratoid Rhabdoid Tumor With TTF-1 Expression: A Case Report and Possible Insight Into Its Embryologic Origin.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2025

Beyond Aqueductal Stenosis: A Case of an Atypical Teratoid/Rhabdoid Tumor Presenting With Neonatal Hydrocephalus.

Cureus
2026

Preclinical assessment of checkpoint blockade combined with DNA methyltransferase inhibition in high-risk pediatric brain tumors reveals limited therapeutic synergy.

Neuro-oncology advances
2026

Proton-Based Re-Irradiation of Locally Recurrent Pediatric Brain Tumors in Close Proximity to the Brainstem.

International journal of particle therapy
2025

Radiotherapy Utilization and Treatment Continuity of Pediatric Patients With Brain Tumor in Egypt: A Decade of Data.

JCO global oncology
2025

Disordered DNA methylation leads to targetable transcriptional plasticity in ATRT.

Acta neuropathologica communications
2025

Malignant transformation of low-grade diffusely infiltrative tumor (LGDIT), SMARCB1-mutant to atypical teratoid/rhabdoid tumor (AT/RT).

NPJ genomic medicine
2025

A case report of atypical teratoid/rhabdoid tumor and pituitary adenoma collision tumor in the sellar region.

Frontiers in surgery
2025

Atypical teratoid rhabdoid tumor with extraneural metastases: a case report with genomic analysis.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

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

Cancers
2025

[Fertility Preservation for Pediatric Brain Tumor Patients: Current Status and Challenges].

No shinkei geka. Neurological surgery
2025

[Current Status and Prospects of Clinical Trials for Medulloblastoma and Atypical Teratoid/Rhabdoid Tumor].

No shinkei geka. Neurological surgery
2025

Pediatric Oncology Patients With Germline Pathogenic Variants in Adult-Onset Cancer Predisposition Genes.

JCO precision oncology
2025

Atypical Teratoid/Rhabdoid Tumor of the Sellar Region in an Elderly Patient: A Case Report and Literature Review.

Cureus
2026

From Traditional Therapies to Nanomedicine: A Thorough Examination of Atypical Teratoid Rhabdoid Tumor Treatment Methods.

Molecular pharmaceutics
2026

Central Nervous System Embryonal Tumors.

Neuroimaging clinics of North America
2025

Role of immunohistochemistry in the molecular classification of atypical teratoid/rhabdoid tumor.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Atypical teratoid/rhabdoid tumor of the third ventricle in a pediatric patient: a case report.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Current advances in the management of atypical teratoid rhabdoid tumors (ATRT).

Advances in cancer research
2025

A rare case of atypical teratoid rhabdoid tumor (AT/RT) with homozygous SMARCB1 loss and one concurrent somatic heterozygous SMARCA4 variant.

Acta neuropathologica communications
2025

Unusual Dual Brain Tumor Morphologies in an MEN1 Patient: A Case Report of Diagnostic Challenges and Methylation Insights.

International journal of molecular sciences
2025

Potential clinical value of circulating tumor cells in predicting progression for atypical teratoid rhabdoid tumor in young children.

Journal of neuro-oncology
2025

Rare encounter of spinal atypical teratoid/rhabdoid tumor in an adult: A case report and review of clinicopathological diagnostic pitfalls.

Clinical neuropathology
2025

Interhemispheric-Transcortical Approach for Resection of an Atypical Teratoid/Rhabdoid Tumor (AT/RT) of the Left Lateral and Third Ventricle.

Journal of neurological surgery reports
2025

Clinical and Molecular Characteristics of 100 Atypical Teratoid Rhabdoid Tumor Patients from Low- and Middle-Income Countries.

Cancers
2025

Outcome of Infantile Malignant Solid Tumors: A Single-Center Experience.

Children (Basel, Switzerland)
2025

Aggressive sellar-suprasellar atypical teratoid rhabdoid tumor in a 38-year-old pregnant woman: a case report and literature review.

Annals of medicine and surgery (2012)
2025

Outcomes of Infants and Young Children With CNS Embryonal Tumors Using Pre-Irradiation Chemotherapy: A Decade Long Experience.

Cancer medicine
2025

Asymmetric Total Synthesis of 4,9,10-Trihydroxyguaia-11(13)en-12,6-olide and Discovery of Its Anticancer Activity against Atypical Teratoid Rhabdoid Tumor.

ACS central science
2025

A cycling, progenitor-like cell population at the base of atypical teratoid rhabdoid tumor subtype differentiation trajectories.

Neuro-oncology
2025

Old Tools in a New Era: The Continued Relevance of Chemotherapy in Pediatric Neuro-Oncology.

Current oncology (Toronto, Ont.)
2025

Expression of LTR and LINE1 transposable elements defines atypical teratoid/rhabdoid tumor subtypes.

Acta neuropathologica communications
2025

Spinal Atypical Teratoid/Rhabdoid Tumor with Metastatic Lesions in the Bilateral Acoustic Nerves.

Pediatric blood &amp; cancer
2025

Measles Oncolytic Virus as an Immunotherapy for Recurrent/Refractory Pediatric Medulloblastoma and Atypical Teratoid/Rhabdoid Tumor: Results from PNOC005.

Clinical cancer research : an official journal of the American Association for Cancer Research
2025

Specific imaging features of sellar atypical teratoid/rhabdoid tumor or the lack of thereof.

World journal of radiology
2025

Importance of symptoms acuity for clinical diagnosis of primary sellar atypical teratoid/rhabdoid tumor.

World journal of clinical oncology
2025

Malignant Rhabdoid Tumors of Cranial Nerves-A Clinically Distinct Group With Characteristic Neuroradiological, Histopathological, and Molecular Features.

Pediatric blood &amp; cancer
2025

Elevated Bromide Levels Due to Tazemetostat Therapy Treated With Salt and Water Supplementation in Two Children With Atypical Teratoid Rhabdoid Tumor.

Pediatric blood &amp; cancer
2025

Pediatric Atypical Teratoid Rhabdoid Tumor of Central Nervous System: A Case Series with Review of Literature.

Indian journal of surgical oncology
2025

Navigating the complexity of atypical teratoid/rhabdoid tumor (ATRT) in pediatric neuro-oncology: Insights from clinical spectrum to therapeutic challenges.

International journal of surgery case reports
2025

A Case of Adult-Onset Atypical Teratoid/Rhabdoid Tumor With Extracranial Metastasis.

Cureus
2025

High-throughput screening of FDA-approved drugs identifies colchicine as a potential therapeutic agent for atypical teratoid/rhabdoid tumors (AT/RTs).

RSC advances
2025

Case Report: SMARCB1-deficient phenotype may be a new specialized type of pleomorphic xanthoastrocytoma associated with poor prognosis.

Frontiers in oncology
2025

Effects of Induced Pluripotent Stem Cell-Derived Astrocytes on Cisplatin Sensitivity in Pediatric Brain Cancer Cells.

Cancers
2025

Co-occurrence of congenital anomalies and childhood brain tumors in 22 million live births.

Neuro-oncology
2025

SWI/SNF complexes govern ontology-specific transcription factor function in MYC-subtype atypical teratoid rhabdoid tumor.

Neuro-oncology
2025

Clinical characteristics of patients with atypical teratoid/rhabdoid tumors: a monocentric retrospective analysis.

Frontiers in pediatrics
2025

Characterizing the Natural History of Pediatric Brain Tumors Presenting with Metastasis.

Cancers
2025

Locoregional infusion of EGFR806-CAR T cells for recurrent or refractory pediatric CNS tumors: Results of the completed BrainChild02 phase 1 clinical trial.

Neuro-oncology
2025

Effect of tandem autologous stem cell transplantation on survival in pediatric patients with high-risk solid tumors in South China.

World journal of stem cells
2025

Pediatric isolated oculomotor nerve palsy induced by peduncular atypical teratoid/rhabdoid tumor: case report and literature review.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
2025

Case report: Molecular characterization of adult atypical teratoid rhabdoid tumor and review of the literature.

Frontiers in oncology
2025

Novel SMARCA4 Variant in an Infant With Atypical Teratoid Rhabdoid Tumor.

Journal of pediatric hematology/oncology
2025

An in vitro pharmacogenomic approach reveals subtype-specific therapeutic vulnerabilities in atypical teratoid/rhabdoid tumors (AT/RT).

Pharmacological research
2025

SIRT2 Regulates the SMARCB1 Loss-Driven Differentiation Block in ATRT.

Molecular cancer research : MCR
2025

Development of a Nomogram and Risk Grouping System for Predicting 1-Year Overall Survival of Patients With Atypical Teratoid/Rhabdoid Tumors.

Journal of child neurology
2025

Genetic ancestry superpopulations show distinct prevalence and outcomes across pediatric central nervous system tumors from the Pediatric Brain Tumor Atlas and Pediatric Neuro-Oncology Consortium.

Neuro-oncology
2025

Descriptive epidemiology and prognostic factors of atypical teratoid/rhabdoid tumors in the United States, 2001-2021.

Neurosurgical review
2024

The CoREST complex inhibitor, corin, leads to decreased tumor growth, increased cellular differentiation and extended lifespan in atypical teratoid rhabdoid tumor xenograft models.

bioRxiv : the preprint server for biology
2024

Pediatric skull base tumors: Clinical features and surgical outcomes; a single center retrospective study with a review of literature.

Brain &amp; spine
2024

Adult Primary Central Nervous System Atypical Teratoid/Rhabdoid Tumor Metastasizing to the Cervical Lymph Node.

Cureus
2025

Designing a time-dependent therapeutic strategy using CDK4/6 inhibitors in an intracranial ATRT model.

Neuro-oncology
2024

A rare case of atypical teratoid rhabdoid tumor at the sellar region in an adult: Case report and review of literature.

Brain &amp; spine
2024

Differential Replication and Oncolytic Effects of Zika Virus in Aggressive CNS Tumor Cells: Insights from Organoid and Tumoroid Models.

Viruses
2025

Atypical Teratoid Rhabdoid Tumor of the Brain in a Young Adult With Down Syndrome: Case Report and Literature Review.

Journal of pediatric hematology/oncology
2024

Treatment of cerebral radiation necrosis using hyperbaric oxygen therapy in a child: illustrative case.

Journal of neurosurgery. Case lessons
2025

Brachyury Expression in Atypical Teratoid/Rhabdoid Tumor.

International journal of surgical pathology
2025

Proximal and Classic Epithelioid Sarcomas are Distinct Molecular Entities Defined by MYC/GATA3 and SOX17/Endothelial Markers, Respectively.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2024

Neuropathology Entities Involving the Sinonasal Tract.

Surgical pathology clinics
2024

Re-programming by a six-factor-secretome in the patient tumor ecosystem during nutrient stress and drug response.

iScience
2024

Inhibiting EZH2 targets atypical teratoid rhabdoid tumor by triggering viral mimicry via both RNA and DNA sensing pathways.

Nature communications
2024

Pediatric Central Nervous System Embryonal Tumors: Presentation, Diagnosis, Therapeutic Strategies, and Survivorship-A Review.

Pediatric neurology
2024

RRM2 inhibition alters cell cycle through ATM/Rb/E2F1 pathway in atypical teratoid rhabdoid tumor.

Neoplasia (New York, N.Y.)
2024

Approaches for prevention of tumors in patients with rhabdoid tumor predisposition syndrome.

Neuro-oncology advances
2025

Posterior pituitary tumors and other rare entities involving the pituitary gland.

Brain pathology (Zurich, Switzerland)
2024

Cerebrospinal fluid liquid biopsy by low-pass whole genome sequencing for clinical disease monitoring in pediatric embryonal tumors.

Neuro-oncology advances
2024

Direct Administration of Chemotherapy and Other Agents into the Fourth Ventricle to Treat Recurrent Malignant Brain Tumors in Children.

Advances and technical standards in neurosurgery
2024

Prediction of intraoperative blood loss in pediatric posterior fossa tumors by neuroradiological evaluation: preliminary study.

Neuro-Chirurgie
2024

Decoding pediatric spinal tumors: a single-center retrospective case series on etiology, presentation, therapeutic strategies, and outcomes.

Neurosurgical review
2024

Gemcitabine therapeutically disrupts essential SIRT1-mediated p53 repression in atypical teratoid/rhabdoid tumors.

Cell reports. Medicine
2024

Pediatric atypical teratoid/rhabdoid tumor in the cauda equina with rapid tumor progression: illustrative case.

Journal of neurosurgery. Case lessons
2024

Autologous stem cell transplantation in adults with atypical teratoid rhabdoid tumor: a case report and review.

CNS oncology
2024

Outcomes for children with recurrent/refractory atypical teratoid rhabdoid tumor: A single-institution study with molecular correlation.

Pediatric blood &amp; cancer
2024

A kinome drug screen identifies multi-TKI synergies and ERBB2 signaling as a therapeutic vulnerability in MYC/TYR subgroup atypical teratoid rhabdoid tumors.

Neuro-oncology
2024

Spinal Atypical Teratoid Rhabdoid Tumor in a 14-Year-old Child With Down Syndrome: A Case Report.

Journal of pediatric hematology/oncology
2024

SMARCB1 Gene Therapy Using a Novel Tumor-Targeted Nanomedicine Enhances Anti-Cancer Efficacy in a Mouse Model of Atypical Teratoid Rhabdoid Tumors.

International journal of nanomedicine
2024

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

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

Retrospective experience of children with relapsed brain tumors treated with oral combination of axitinib and metronomic etoposide.

Pediatric blood &amp; cancer
2024

Detection of tumor-derived cell-free DNA in cerebrospinal fluid using a clinically validated targeted sequencing panel for pediatric brain tumors.

Journal of neuro-oncology
2024

Plasma miRNA expression profile in pediatric pineal pure germinomas.

Frontiers in oncology
2024

Atypical Teratoid/Rhabdoid Tumor with Retained SMARCB1 (INI1) Expression and Rare SMARCA4 Gene Mutation: A Case Report of a Pediatric Patient.

Reports (MDPI)
2024

Central nervous system embryonal tumors with EWSR1-PLAGL1 rearrangements reclassified as INI-1 deficient tumors at relapse.

Journal of neuro-oncology
2024

Rare Coexistence: Pilocytic Astrocytoma With Atypical Teratoid/Rhabdoid Tumor Features in an Infant.

Cureus
2024

Clinical and histopathological spectrum of cranial small round cell tumors: An experience from a tertiary care center.

Journal of cancer research and therapeutics
2024

Rhabdoid tumor predisposition syndrome: A historical review of treatments and outcomes for associated pediatric malignancies.

Pediatric blood &amp; cancer
2024

Atypical teratoid/rhabdoid tumor of the central nervous system: Clinicopathological features of two challenging cases.

International journal of surgery case reports
2024

Feeder-free differentiation of human iPSCs into natural killer cells with cytotoxic potential against malignant brain rhabdoid tumor cells.

Bioactive materials
2024

ESTRO-SIOPE guideline: Clinical management of radiotherapy in atypical teratoid/rhabdoid tumors (AT/RTs).

Radiotherapy and oncology : journal of the European Society for Therapeutic Radiology and Oncology
2024

High-Grade Atypical Teratoid/Rhabdoid Tumor in the Pituitary Region.

Cureus
2024

Dynamic Survival Risk Prognostic Model and Genomic Landscape for Atypical Teratoid/Rhabdoid Tumors: A Population-Based, Real-World Study.

Cancers
2024

Recurrence Patterns and Surveillance Imaging in Pediatric Brain Tumor Survivors.

Journal of pediatric hematology/oncology
2024

Two Cases of Atypical Teratoid/Rhabdoid Tumor in the Spinal Cord: Loss of SMARCB1 in a Child and Loss of SMARCA4 in an Adult.

NMC case report journal
2024

An adult with recurrent atypical teratoid rhabdoid tumor of the spine.

CNS oncology
2024

A 7-year-old boy presented with temporal lobe lesion.

Brain pathology (Zurich, Switzerland)
2024

Atypical teratoid rhabdoid tumor in a lower middle‑income country: Challenges to cure.

Oncology letters
2024

Targeting cholesterol biosynthesis for AT/RT: comprehensive expression analysis and validation in newly established AT/RT cell line.

Human cell
2024

Epidemiology, Characteristics, and Prognostic Factors of Primary Atypical Teratoid/Rhabdoid Tumors in the Spinal Canal: A Systematic Review.

Neurospine
2024

Multifocal atypical teratoid/rhabdoid tumour in an infant-a rare case report.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2024

Rapid, economical diagnostic classification of ATRT molecular subgroup using NanoString nCounter platform.

Neuro-oncology advances
2024

Radiation Therapy Plays an Important Role in the Treatment of Atypical Teratoid/Rhabdoid Tumors: Analysis of the EU-RHAB Cohorts and Their Precursors.

International journal of radiation oncology, biology, physics
2024

Recurrent spinal atypical teratoid/rhabdoid tumor with pulmonary metastasis.

Cancer reports (Hoboken, N.J.)
2024

METRO-PD1: Phase 1 study of nivolumab in combination with metronomic chemotherapy in children and adolescents with relapsing/refractory solid tumors.

European journal of cancer (Oxford, England : 1990)
2024

Successful peripheral blood stem cell harvesting for two pediatric cases of atypical teratoid/rhabdoid tumor with low bodyweight.

Pediatric blood &amp; cancer
2023

Targeting of RRM2 suppresses DNA damage response and activates apoptosis in atypical teratoid rhabdoid tumor.

Journal of experimental &amp; clinical cancer research : CR
2023

Current Molecular and Clinical Landscape of ATRT - The Link to Future Therapies.

Cancer management and research
2023

Tazemetostat in the therapy of pediatric INI1-negative malignant rhabdoid tumors.

Scientific reports
2023

Sellar Mass in 2 Patients With Acute-Onset Headache and Visual Symptoms: Not Your Usual Pituitary Adenoma.

AACE clinical case reports
2023

Sellar Suprasellar Surprise: A Rare Case of Atypical Teratoid/Rhabdoid Tumor in an Infant.

Cureus
2023

Malignant rhabdoid tumor of kidney in an adult patient with positive family history of rhabdoid tumor: A case report and review of literature.

International journal of surgery case reports
2024

SWI/SNF-deficient tumors of the central nervous system: An update.

Clinical neuropathology
2023

Evaluation of INI1 Protein Expression Through IHC Study in Pediatric High-Grade Brain Tumors in South of Iran in 2008-2021.

Iranian journal of pathology
2023

Clinical diagnostic and radiographic features of primary spinal atypical teratoid rhabdoid tumors tumor in a pediatric patient: A case report and review of the literature.

Journal of central nervous system disease
2024

Management of Atypical Teratoid/Rhabdoid Tumors in the Pediatric Population: A Systematic Review and Meta-Analysis.

World neurosurgery
2024

Systematic characterization of antibody-drug conjugate targets in central nervous system tumors.

Neuro-oncology
2023

Spinal ATRT and Radiotherapy Case Report in an Adult Man.

Oncology (Williston Park, N.Y.)
2023

Early death from childhood cancer: First medical record-level analysis reveals insights on diagnostic timing and cause of death.

Cancer medicine
2023

Sellar atypical teratoid/rhabdoid tumor in adults: survival analysis of treatment strategies. Illustrative case.

Journal of neurosurgery. Case lessons
2023

Synchronous congenital malignant rhabdoid tumor of the orbit and cerebellar atypical teratoid/rhabdoid tumor: Case report.

Radiology case reports
2023

Atypical Teratoid/Rhabdoid Tumor of the Nervous System in Adults: Location-Related Features and Outcome.

World neurosurgery
2024

Modeling human brain rhabdoid tumor by inactivating tumor suppressor genes in induced pluripotent stem cells.

Bioactive materials
2024

Survival Benefit from Multimodal Treatment for Patients with Atypical Teratoid Rhabdoid Tumor in a Surveillance, Epidemiology, and End Results Database Analysis.

Oncology
2023

B7-H3 in Brain Malignancies: Immunology and Immunotherapy.

International journal of biological sciences
2023

Rhabdoid tumors in patients conceived following ART: is there an association?

Human reproduction (Oxford, England)
2024

Constitutional balanced translocations involving SMARCB1: A rare cause of rhabdoid tumor predisposition syndrome.

Genes, chromosomes &amp; cancer
2023

Infantile (congenital) anaplastic intracranial solitary fibrous tumor/hemangiopericytoma-A case report with brief literature review.

Indian journal of pathology &amp; microbiology
2023

Novel Strategy Involving High-Dose Chemotherapy with Stem Cell Rescue Followed by Intrathecal Topotecan Maintenance Therapy without Whole-Brain Irradiation for Atypical Teratoid/Rhabdoid Tumors.

Pediatric hematology and oncology
2023

Focal glioneuronal differentiation in an atypical teratoid/rhabdoid tumor after chemoradiotherapy.

Pathology international
2023

Current advances in immunotherapy for atypical teratoid rhabdoid tumor (ATRT).

Neuro-oncology practice
2023

Embryonal Tumors of the Central Nervous System with Multilayered Rosettes and Atypical Teratoid/Rhabdoid Tumors.

Advances in experimental medicine and biology
2023

The Role of Immunotherapy in the Treatment of Rare Central Nervous System Tumors.

Current oncology (Toronto, Ont.)
2023

Current Open Trials and Molecular Update for Pediatric Embryonal Tumors.

Pediatric neurosurgery
2023

Pediatric Posterior Fossa ATRT: A Case Report, New Treatment Strategies and Perspectives.

Brain sciences
2023

Tazemetostat for tumors harboring SMARCB1/SMARCA4 or EZH2 alterations: results from NCI-COG pediatric MATCH APEC1621C.

Journal of the National Cancer Institute
2023

Survival benefit of postoperative radiotherapy for pediatric patients with primary intracranial atypical teratoid/rhabdoid tumors: Propensity score analysis and prediction model construction in a multi-registry based cohort.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2023

Prognostic factors of pediatric pineal region tumors at a single institution.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2023

Epigenetic Regulation in Primary CNS Tumors: An Opportunity to Bridge Old and New WHO Classifications.

Cancers
2023

Spinal atypical teratoid rhabdoid tumor-narrative review and report of a rare case managed with multimodality approach.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2023

Posterior fossa tumors in children: An update and new concepts.

Surgical neurology international
2023

Outcomes and surgical approaches for pineal region tumors in children: 30 years' experience.

Journal of neurosurgery. Pediatrics
2023

Large intracranial atypical teratoid / rhabdoid tumor with preserved INI-1 expression: A case report.

Asian journal of surgery
2023

Atypical Teratoid/Rhabdoid Tumor of the Sellar Region in an Adult Male: A Case Report.

Cureus
2023

An infantile pineal embryonal tumor showing pathological features of a CNS ganglioneuroblastoma and the methylation profiling of an atypical teratoid/rhabdoid tumor and SMARCA4 mutation.

Journal of neuropathology and experimental neurology
2024

Atypical Teratoid Rhabdoid Tumor Presenting as Acute Optic Neuritis--A Rare Case in an Adult Chinese Patient.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2023

Focal versus craniospinal radiation for disseminated atypical teratoid/rhabdoid tumor following favorable response to systemic therapy.

Pediatric blood &amp; cancer
2023

Characterization of Switch/Sucrose Nonfermenting Complex Proteins and Nestin Expression in a Cohort of Pediatric Central Nervous System Tumors.

Applied immunohistochemistry &amp; molecular morphology : AIMM
2023

Radiotherapy for Atypical Teratoid/Rhabdoid Tumor (ATRT) on the Pediatric Proton/Photon Consortium Registry (PPCR).

Journal of neuro-oncology
2023

MRI features of pediatric atypical teratoid rhabdoid tumors and medulloblastomas of the posterior fossa.

Cancer medicine
2023

The PARP inhibitor Rucaparib synergizes with radiation to attenuate atypical teratoid rhabdoid tumor growth.

Neuro-oncology advances
2023

Efficacy and safety of bevacizumab, irinotecan, and temozolomide combination for relapsed or refractory pediatric central nervous system embryonal tumor: a single-institution study.

Journal of neurosurgery. Pediatrics
2023

Increased Radiation Sensitivity in Patients with Phelan-McDermid Syndrome.

Cells
2023

Successful stem cell collection for atypical teratoid rhabdoid tumor in an extremely low-body weight child: A case report.

Journal of clinical apheresis
2023

Modeling nervous system tumors with human stem cells and organoids.

Cell regeneration (London, England)
2023

Spinal epidural lipomatosis in a pediatric patient with a malignant brain tumor: illustrative case.

Journal of neurosurgery. Case lessons
2023

Prolonged remission achieved with maintenance intraventricular chemotherapy in young patient with recurrent atypical teratoid rhabdoid tumor.

Pediatric blood &amp; cancer
2023

Recent progress and novel approaches to treating atypical teratoid rhabdoid tumor.

Neoplasia (New York, N.Y.)
2023

Atypical Teratoid Rhabdoid Tumor: Proposal of a Diagnostic Pathway Based on Clinical Features and Neuroimaging Findings.

Diagnostics (Basel, Switzerland)
2023

Endoscopic Resection of Intraspinal Malignant Atypical Teratoid/Rhabdoid Tumor: 2-Dimensional Operative Video.

Operative neurosurgery (Hagerstown, Md.)
2023

Tacedinaline (CI-994), a class I HDAC inhibitor, targets intrinsic tumor growth and leptomeningeal dissemination in MYC-driven medulloblastoma while making them susceptible to anti-CD47-induced macrophage phagocytosis via NF-kB-TGM2 driven tumor inflammation.

Journal for immunotherapy of cancer
2023

Cell of Origin of Brain and Spinal Cord Tumors.

Advances in experimental medicine and biology
2023

A rare case of multifocal atypical teratoid rhabdoid tumor.

Radiology case reports
2022

[Pediatric Posterior Fossa Tumors].

No shinkei geka. Neurological surgery
2022

Magnetic Resonance Imaging (MRI) and Positron Emission Tomography (PET)/Computed Tomography Features of Atypical Teratoid/Rhabdoid Tumors: Case Series and Review.

Journal of child neurology
2022

Case report: Atypical teratoid/rhabdoid tumor of the lateral ventricle in a male adolescent (case-based review and diagnostic challenges in developing countries).

Frontiers in oncology
2023

A phase I trial of metformin in combination with vincristine, irinotecan, and temozolomide in children with relapsed or refractory solid and central nervous system tumors: A report from the national pediatric cancer foundation.

Cancer medicine
2022

Primary cilia contribute to the aggressiveness of atypical teratoid/rhabdoid tumors.

Cell death &amp; disease
2022

[Analysis of 18 cases of malignant rhabdoid tumor in children].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2022

Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System in Children: Case Reports and Literature Review.

Frontiers in surgery
2022

Sellar Atypical Teratoid/Rhabdoid Tumors (AT/RT): A Systematic Review and Case Illustration.

Cureus
2022

Sustained and durable response with Alisertib monotherapy in the treatment of relapsed Atypical Teratoid Rhabdoid Tumor (ATRT).

Neuro-oncology advances
2022

An unusual case of atypical teratoid/rhabdoid tumor, initially diagnosed as atypical pituitary adenoma in a 13-year-old male patient.

Neuro-oncology advances
2022

Human Malignant Rhabdoid Tumor Antigens as Biomarkers and Potential Therapeutic Targets.

Cancers
2022

Molecular Heterogeneity in Pediatric Malignant Rhabdoid Tumors in Patients With Multi-Organ Involvement.

Frontiers in oncology
2022

SMARCB1-Deficient Cancers: Novel Molecular Insights and Therapeutic Vulnerabilities.

Cancers
2022

Recent Advances in Renal Medullary Carcinoma.

International journal of molecular sciences
2022

Primary adult sellar SMARCB1/INI1-deficient tumor represents a subtype of atypical teratoid/rhabdoid tumor.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2022

[Clinicopathological and molecular genetic characterization of 2 cases of atypical teratoid/rhabdoid tumor of central nervous system in adult patients].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2022

Whole-genome sequencing analysis of an atypical teratoid/rhabdoid tumor in a patient with Phelan-McDermid syndrome: a case report and systematic review.

Brain tumor pathology
2022

Bromodomain and Extra-Terminal Protein Inhibitors: Biologic Insights and Therapeutic Potential in Pediatric Brain Tumors.

Pharmaceuticals (Basel, Switzerland)
2022

Pituitary Stalk Thickening: Causes and Consequences. The Children's Memorial Health Institute Experience and Literature Review.

Frontiers in endocrinology
2023

Phase II study of alisertib as a single agent for treating recurrent or progressive atypical teratoid/rhabdoid tumor.

Neuro-oncology
2022

Embryonal tumors in the WHO CNS5 classification: A Review.

Indian journal of pathology &amp; microbiology
2022

Giant Pediatric Supratentorial Tumor: Clinical Feature and Surgical Strategy.

Frontiers in pediatrics
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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.

  1. Longitudinal Detection of Tumor-Specific Peptides in Cerebrospinal Fluid for Pediatric Brain Tumor Surveillance.
    Cells· 2026· PMID 41827907mais citado
  2. A Rare Thoracolumbar Spinal Atypical Teratoid/Rhabdoid Tumor in a 7-Year-Old Child: Diagnostic Challenges and Therapeutic Considerations.
    International journal of surgical pathology· 2026· PMID 41869689mais citado
  3. Atypical Teratoid/Rhabdoid Tumor of the Posterior Fossa Mimicking Medulloblastoma in an Infant: A Case Report.
    Cureus· 2026· PMID 41841066mais citado
  4. Enhanced Delivery of Aurora Kinase A Inhibitor Alisertib via Tumor-Targeting Immunoliposome Nanocomplex for Improved Treatment of Cancers Including Atypical Teratoid/Rhabdoid Tumor.
    International journal of nanomedicine· 2026· PMID 41836727mais citado
  5. Management of Pediatric Brain Tumors with the Aid of PET/MR Imaging.
    PET clinics· 2026· PMID 41813365mais citado
  6. Aggressive atypical teratoid/rhabdoid tumor with extensive leptomeningeal metastasis in an 8-year-old boy: a case report and narrative review.
    Transl Pediatr· 2026· PMID 41982946recente
  7. Pediatric Spinal Atypical Teratoid Rhabdoid Tumor: Recent Advances in Biology and Management Options.
    Cancers (Basel)· 2026· PMID 41976393recente
  8. Hemispherotomy for atypical teratoid rhabdoid tumor-associated drug-resistant epilepsy: illustrative case.
    J Neurosurg Case Lessons· 2026· PMID 41974062recente
  9. Revisiting preoperative diagnostic clues for adult primary sellar atypical teratoid/rhabdoid tumor: a rare entity mimicking pituitary adenoma.
    World Neurosurg· 2026· PMID 41966303recente
  10. Role of Surrogate Immunohistochemistry Markers in CNS Tumors in the Era of Molecular Diagnostics With Recent Updates.
    Adv Anat Pathol· 2026· PMID 41954087recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:99966(Orphanet)
  2. MONDO:0020560(MONDO)
  3. GARD:16926(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q631904(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

Tumor teratoide, atípico
Compêndio · Raras BR

Tumor teratoide, atípico

ORPHA:99966 · MONDO:0020560
Prevalência
Unknown
Herança
Not applicable
CID-10
C49.9 · Neoplasia maligna do tecido conjuntivo e tecidos moles, não especificados
CID-11
Ensaios
23 ativos
Início
Antenatal, Childhood, Infancy, Neonatal
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C1266184
EuropePMC
Wikidata
Papers 10a
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