Raras
Buscar doenças, sintomas, genes...
Tumores rabdoides
ORPHA:69077CID-10 · C49.9DOENÇA RARA

Neoplasia embrionária maligna agressiva que geralmente ocorre durante a infância. É caracterizada pela presença de células grandes com citoplasma abundante, grande núcleo excêntrico e nucléolo proeminente e está associada a anomalias do cromossomo 22. Pode surgir do sistema nervoso central, rim e tecidos moles. O prognóstico é ruim.

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Introdução

O que você precisa saber de cara

📋

Neoplasia embrionária maligna agressiva que geralmente ocorre durante a infância. É caracterizada pela presença de células grandes com citoplasma abundante, grande núcleo excêntrico e nucléolo proeminente e está associada a anomalias do cromossomo 22. Pode surgir do sistema nervoso central, rim e tecidos moles. O prognóstico é ruim.

Pesquisas ativas
36 ensaios
138 total registrados no ClinicalTrials.gov
Publicações científicas
1.985 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
Casos conhecidos
500
pacientes catalogados
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
🩸
Sangue
3 sintomas
🫘
Rins
3 sintomas
🫃
Digestivo
3 sintomas
📏
Crescimento
1 sintomas
🧬
Pele e cabelo
1 sintomas

+ 20 sintomas em outras categorias

Características mais comuns

55%prev.
Irritabilidade
Frequente (79-30%)
55%prev.
Paralisia do nervo oculomotor
Frequente (79-30%)
55%prev.
Paralisia cerebral
Frequente (79-30%)
55%prev.
Febre
Frequente (79-30%)
55%prev.
Neoplasia renal
Frequente (79-30%)
55%prev.
Cefaleia
Frequente (79-30%)
40sintomas
Frequente (20)
Ocasional (4)
Sem dados (16)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 40 características clínicas mais associadas, ordenadas por frequência.

IrritabilidadeIrritability
Frequente (79-30%)55%
Paralisia do nervo oculomotorOculomotor nerve palsy
Frequente (79-30%)55%
Paralisia cerebralCerebral palsy
Frequente (79-30%)55%
FebreFever
Frequente (79-30%)55%
Neoplasia renalRenal neoplasm
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico1.985PubMed
Últimos 10 anos200publicações
Pico2025126 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 germline 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 5,352 variantes classificadas pelo ClinVar.

535
2141
2676
Patogênica (10.0%)
VUS (40.0%)
Benigna (50.0%)
VARIANTES MAIS SIGNIFICATIVAS
SMARCA4: NM_003072.5(SMARCA4):c.1849del (p.Pro616_Val617insTer) [Pathogenic]
SMARCA4: NM_003072.5(SMARCA4):c.4318C>T (p.Gln1440Ter) [Pathogenic]
SMARCA4: NM_003072.5(SMARCA4):c.1246-20_1246-19insCTGTGCA [Uncertain significance]
SMARCA4: NM_003072.5(SMARCA4):c.3817C>T (p.His1273Tyr) [Uncertain significance]
SMARCA4: NM_003072.5(SMARCA4):c.388G>T (p.Ala130Ser) [Uncertain significance]

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.
3Fase 31
2Fase 29
1Fase 19
·Pré-clínico1
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 — Tumores rabdoides

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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

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

Outros ensaios clínicos

138 ensaios clínicos encontrados, 36 ativos.

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

Timeline de publicações
1.035 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 1.035

#1

Impact of Radiation Therapy on Malignant Rhabdoid Tumor of the Kidney: A National Cancer Database Analysis.

Pediatric blood &amp; cancer2026 Mar 15

Malignant rhabdoid tumor of the kidney (MRTK) is a rare and aggressive tumor, primarily affecting infants and young children. Intensive multimodal treatment is standard, entailing chemotherapy, surgery, and radiation therapy (RT). Although previous studies have demonstrated the importance of chemotherapy and surgery, the impact of RT on patient outcomes is unclear. Patients diagnosed with MRTK from 2004 to 2019 were analyzed from the National Cancer Database. Patients who did not receive surgery or chemotherapy or died within 30 days after surgery were excluded. Kaplan-Meier survival analyses were conducted to evaluate overall survival (OS) of the entire cohort, stratified by age, metastatic disease, and RT utilization. Log-rank tests were used to compare survival distributions. The cohort included 103 patients. Median age was <1 year (range <1-15, interquartile range: 0-1 year). Forty-eight (47%) patients presented with metastatic disease at diagnosis. Sixty-six (64%) patients received RT. One- and three-year OS were 55% and 45%, respectively. Median OS for the entire cohort was 12.2 months. Patients aged <1 year exhibited significantly worse 3-year OS compared to those aged ≥1 year (28% vs. 64%, p < 0.001). Metastatic disease was associated with worse 3-year OS compared to localized disease (25% vs. 62%, p < 0.001). Patients who received RT demonstrated significantly better 3-year OS than those who did not receive RT (58% vs. 19%, p < 0.001). In patients with localized disease, RT to the primary tumor bed was associated with improved 3-year OS compared to no RT (74% vs. 33%, p = 0.008). Among patients with metastatic disease, treatment with RT to either primary and/or metastatic site was also associated with a 3-year OS benefit compared to no RT (38% vs. 10%, p < 0.001). Although MRTK remains highly lethal in infants and particularly those with metastatic disease, RT was independently associated with improved survival in patients with and without metastatic disease.

#2

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.

#3

Rhabdoid tumors as a novel target for PSMA-directed CAR T cell therapy.

Molecular therapy. Oncology2026 Mar 19

Rhabdoid tumor is an ultra-rare and highly aggressive pediatric malignancy with a poor prognosis and limited therapeutic options. To identify novel immunotherapeutic targets, transcriptomic data from the Cancer Cell Line Encyclopedia were analyzed, and we found that two rhabdoid tumor cell lines exhibit high expression of prostate-specific membrane antigen (PSMA), with levels comparable to well-established PSMA-positive prostate cancer cell lines. PSMA expression in rhabdoid tumors was subsequently validated in cell lines and in a subset of primary clinical rhabdoid tumor specimens. While PSMA-directed therapies have primarily been explored in prostate cancer, we evaluated their potential in rhabdoid tumors by employing PSMA-directed chimeric antigen receptor (CAR) T cells. These CAR T cells demonstrated potent and antigen-specific cytotoxicity against PSMA-positive rhabdoid tumor cells in vitro. In addition, the in vivo efficacy was also assessed in xenograft mouse models of non-CNS tumors, where PSMA CAR T cell treatment resulted in significant tumor regression and robust accumulation of CAR T cells within the tumor microenvironment. Together, these findings establish PSMA as a promising surface antigen beyond prostate cancer and provide preclinical evidence supporting the development of PSMA-directed therapies for this highly lethal pediatric cancer.

#4

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.

#5

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.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC1.092 artigos no totalmostrando 198

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

Phase I/II Study of the CDK2/9 Inhibitor Fadraciclib in Combination with Chemotherapy in Children with Advanced Malignancies: Arm K of the AcSé-ESMART Trial.

Targeted oncology
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

Impact of Radiation Therapy on Malignant Rhabdoid Tumor of the Kidney: A National Cancer Database Analysis.

Pediatric blood &amp; cancer
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

Rare localization of a rhabdoid tumor in the elbow of a 6-year-old child: A case report.

Radiology case reports
2025

Synchronous malignant rhabdoid tumor of the kidney and adrenal neuroblastoma in an infant treated with proton beam therapy: a case report and literature review.

Frontiers in oncology
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
2026

Rhabdoid tumors as a novel target for PSMA-directed CAR T cell therapy.

Molecular therapy. Oncology
2025

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

Cureus
2026

Targeting CDK11 in Rhabdoid Tumor of the Kidney.

Cancers
2026

SMARCB-1-Positive Rhabdoid Squamous Cell Carcinoma Following Liver Transplant: Systematic Review and Unique Case Report.

The American Journal of dermatopathology
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

Desmoplastic Small Round Cell Tumor: Study of Cytomorphologic and Immunophenotypical Features in Seven Cases, One With Unusual Rhabdoid Morphology.

Diagnostic cytopathology
2025

Histogenesis of Atypical Teratoid Rhabdoid Tumors: Anatomical and Embryological Perspectives.

Cancers
2026

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

International journal of particle therapy
2025

Malignant rhabdoid tumor of the kidney in a 27-Year-Old adult: a rare case with favorable outcomes following surgery and adjuvant radiotherapy.

World journal of surgical oncology
2025

An update on the role of SMARCA4 deficiency in gynecological neoplasms: how and where.

Virchows Archiv : an international journal of pathology
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

Malignant extrarenal rhabdoid tumor: a case report.

Journal of medical case reports
2025

Defective Microhomology-Mediated End-joining in SMARCB1-Deficient Tumors.

bioRxiv : the preprint server for biology
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

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

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

Deciphering metastasis in pediatric Wilms' and rhabdoid tumors: IMPA2 as a key molecular player and promising therapeutic target.

Biochemical and biophysical research communications
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

Claudin 6 is a suitable target for CAR T-cell therapy in atypical teratoid/rhabdoid brain tumors and other pediatric solid tumors.

Journal for immunotherapy of cancer
2025

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

Clinical neuropathology
2025

Pediatric renal rhabdoid tumor: Imaging features and diagnostic challenges of a rare and aggressive neoplasm.

Radiology case reports
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

Cancer Predisposition Syndromes With Involvement of the Head and Neck Regions in Children: An Imaging Guide.

Journal of neuroimaging : official journal of the American Society of Neuroimaging
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

Case Report: Multidisciplinary management and nursing care for a preterm infant with rare extrarenal malignant rhabdoid tumor: a clinical case study.

Frontiers in pediatrics
2025

Malignant Rhabdoid Tumors of the Liver Are Associated With Inferior Outcomes Compared to Other Extracranial Rhabdoid Tumors.

Pediatric blood &amp; cancer
2025

Revisiting the Incidence of Hypercalcemia in Contemporary Diagnoses of Pediatric Patients With Solid Tumors.

Pediatric blood &amp; cancer
2025

Congenital Extrarenal Malignant Rhabdoid Tumor Involving the Right Shoulder in a Newborn Baby.

Journal of Indian Association of Pediatric Surgeons
2025

Subcapsular effusion in a pediatric renal rhabdoid tumor.

Pediatric radiology
2025

Spatial and multi-omic profiling reveals genes and pathways associated with cytotoxic lymphocyte infiltration in malignant rhabdoid tumor.

Research square
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)
2026

INI1-Deficient Carcinoma of the Gallbladder.

International journal of surgical pathology
2025

Epigenetic targeting of PGBD5-dependent DNA damage in SMARCB1-deficient sarcomas.

The Journal of clinical investigation
2025

SMARCB1-related schwannomatosis and other SMARCB1-associated phenotypes: clinical spectrum and molecular pathogenesis.

Familial cancer
2025

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

Cancer medicine
2025

Small bowel metastatic SWI/SNF-deficient undifferentiated carcinoma may be predictive of lung primary-a rare presentation with novel SMARCA2 mutation findings in a study of three cases.

Virchows Archiv : an international journal of pathology
2025

Comprehensive mRNA Expressional Analysis of SMARCB1-Deficient Renal Cell Carcinoma in Patients Without Hemoglobinopathies.

Genes, chromosomes &amp; cancer
2025

Undifferentiated Adenocarcinoma With Rhabdoid Features of the Stomach: A Case Report.

Cureus
2025

Mucin 16-Directed Therapy in Pediatric Sarcomas: Case Evidence of Ubamatamab Efficacy in Epithelioid Sarcoma and Its Implications for Other Sarcoma Subtypes.

JCO precision oncology
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

The Role of the Surgical Pathologist in the Recognition of Hereditary Mesenchymal Neoplasms.

Advances in anatomic pathology
2025

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

Current oncology (Toronto, Ont.)
2026

Intrathoracic Rhabdoid Tumors Showing Rapid Progress in Patients with a History of Immune-suppressive Therapies for Hematological Diseases.

Internal medicine (Tokyo, Japan)
2025

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

Acta neuropathologica communications
2025

Primary non-hepatoblastoma liver tumors in children-Defining the profile of a very rare subset of childhood tumors.

Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology
2025

MYC Regulates a DNA Repair Gene Expression Program in Small Cell Carcinoma of the Ovary, Hypercalcemic Type.

Cancers
2025

Evaluating the consistency of SMARCB1 variant classification and assertions of genotype-phenotype relationships in ClinVar.

Cancer genetics
2025

Novel Findings in Pediatric and Adolescent Patients With Cancer and a Germline SMARCA4 Variant.

Pediatric blood &amp; cancer
2025

Ketone Bodies Inhibit Growth of Hepatoblastoma and Rhabdoid Tumor of the Kidney Cells.

Anticancer research
2025

Paediatric renal tumors: An insight into molecular characteristics, histomorphology and syndromic association.

World journal of nephrology
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

Loss of YTHDF1 suppresses the progression of malignant rhabdoid tumor of the kidney by regulating Glutathione S-transferase Mu 2 (GSTM2).

Cell biology and toxicology
2025

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

Pediatric blood &amp; cancer
2025

What is New in Pediatric Hepatic Neoplasms.

Surgical pathology clinics
2025

Image-guided targeting of mitochondrial metabolism sensitizes pediatric malignant rhabdoid tumors to low-dose radiotherapy.

Science advances
2025

Spatiotemporal T-cell tracking for personalized T-cell receptor T-cell therapy designs in childhood cancer.

Annals of oncology : official journal of the European Society for Medical Oncology
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

A Novel Conditionally Replicative Oncolytic Adenovirus under the Control of the SALL4 Promoter Inhibits the Growth of Rhabdoid Tumors.

Molecular cancer therapeutics
2025

SMARCB1-Deficient Pulmonary Mesenchymal Tumor Without Malignant Histological Features.

Pathology international
2025

Whole Abdominal Radiotherapy Is Tolerable and Effective in Children and Young Adults With Intra-Abdominal Sarcoma: A Canadian Sarcoma Research and Clinical Collaboration (CanSaRCC) Study.

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

Diagnostic challenge of malignant rhabdoid tumor presenting as a forehead mass in an infant: A case report.

International journal of surgery case reports
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

Long-Term Outcomes of Radiation Therapy for Pediatric Brain Tumors: A Single-Center Study.

Cureus
2025

A Rare Case of Recurrent Infant Mediastinal Malignant Rhabdoid Tumor With MSH2 Germline Mutation.

Pediatric blood &amp; cancer
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

Multi-omics analysis reveals key immunogenic signatures induced by oncolytic Zika virus infection of paediatric brain tumour cells.

Scientific reports
2025

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

Frontiers in oncology
2025

Intrathoracic Rhabdoid Tumor: A Rare Site in a Jordanian Infant.

Cureus
2025

Optimized zymogram protocol from 3D spheroid cultures to study MMP-2 and -9 activities in tumor cells.

BMC biotechnology
2025

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

Cancers
2025

Rhabdoid Tumor Predisposition Syndrome : A Comprehensive Review of Genetics, Clinical Manifestations, and Management.

Journal of Korean Neurosurgical Society
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

A Complex Case of Diffused Malignant Extra-Renal Rhabdoid Tumor in a Newborn.

Ear, nose, &amp; throat journal
2025

Associating Liver Partition with Portal Vein Ligation and Staged Hepatectomy (ALPPS): Feasibility of Performing in Infants with Large Hepatic Tumor-Case Report.

Healthcare (Basel, Switzerland)
2025

Untangling the Role of MYC in Sarcomas and Its Potential as a Promising Therapeutic Target.

International journal of molecular sciences
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

Mesenteric SMARCA2-Deficient Yet SMARCA4-Preserved Aggressive Undifferentiated Tumor: A Case Report.

Surgical case reports
2025

Crucial role of preserving umbilical cord stumps for genetic decoding of a family with rhabdoid tumor predisposition syndrome.

Gene
2025

Gem-TIP as a salvage therapy for pediatric extracranial malignant rhabdoid tumors: insights from a retrospective case series.

BMC cancer
2025

A Retrospective Review of 28 Cases of Pediatric Malignant Renal Tumors at a Single Institution.

Diseases (Basel, Switzerland)
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

Paediatric Renal Tumors: A State-of-the-Art Review.

Current oncology reports
2025

Extra-renal non-cerebral malignant rhabdoid tumor in children: does maintenance chemotherapy play a role in survival?

Medicina
2025

Mesenchymal Neoplasms of the Kidney and Perinephric Soft Tissue.

Surgical pathology clinics
2025

Pediatric Genitourinary Tumors: The Developmental Angle.

Surgical pathology clinics
2025

Hallmark discoveries in the biology of non-Wilms tumour childhood kidney cancers.

Nature reviews. Urology
2025

A case of rhabdoid meningioma originating from the optic nerve.

Neuropathology : official journal of the Japanese Society of Neuropathology
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

SMARCB1-deficient malignant melanocytic uveal tumours: a new neural crest-derived tumour entity with SMARCB1-related germline predisposition.

The Journal of pathology
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

Primary diffuse leptomeningeal atypical teratoid/rhabdoid tumours (ATRT) of childhood: a molecularly characterised case report and literature review.

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

"Update on pediatric primary liver tumors".

Virchows Archiv : an international journal of pathology
2025

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

Neurosurgical review
2025

Paediatric renal tumours: an update on challenges and recent developments.

Virchows Archiv : an international journal of pathology
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

Spectrum of Renal Tumors Other Than Renal Cell Carcinoma with Emphasis on Cytomorphological, Immunohistochemical, and Cytogenetic Study.

Journal of cytology
2024

Imaging Findings of an Unusual Malignant Renal Tumor in an Infant.

Cureus
2024

Primary classical seminoma of the testis with rhabdoid differentiation.

BMJ case reports
2025

Metabolic profiling of patient-derived organoids reveals nucleotide synthesis as a metabolic vulnerability in malignant rhabdoid tumors.

Cell reports. Medicine
2024

AGGRESSIVE RHABDOID TUMOR IN THE RIGHT LUNG: A CASE REPORT.

Experimental oncology
2025

Rhabdoid Tumor of the Kidney and Soft Tissues: Results from National Wilms Tumor Study-5 and Children's Oncology Group Study AREN0321.

Pediatric blood &amp; cancer
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

Characterization of malignant kidney tumors in childhood by 18F-FDG PET/CT.

World journal of urology
2024

Functional screening reveals genetic dependencies and diverging cell cycle control in atypical teratoid rhabdoid tumors.

Genome biology
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
2024

Absence of SMARCB1 in rhabdoid tumor cells increases sensitivity to translation inhibition and alters translation efficiency of specific mRNAs.

The Journal of biological chemistry
2025

Switch/Sucrose Nonfermentable-Deficient Tumors-Morphology, Immunophenotype, Genetics, Epigenetics, Nosology, and Therapy.

Laboratory investigation; a journal of technical methods and pathology
2024

Successful treatment of a 4-year-old girl with pure malignant rhabdoid tumor of the bladder: a case report.

Translational pediatrics
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

Nodular Melanoma Presenting with Cutaneous Horn and Displaying Rhabdoid Features: An Unusual Presentation.

Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
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
2024

SALL4 and C-kit positive malignant extrarenal rhabdoid tumor of the pelvis in a child: A diagnostic and therapeutic challenge.

Journal of cancer research and therapeutics
2024

SMARCA4 and SMARCA2 co-deficiency: An uncommon molecular signature defining a subset of rare, aggressive and undifferentiated malignancies associated with defective chromatin remodeling.

Cancer letters
2024

[Pediatric soft tissue tumors : Tumors of uncertain origin].

Pathologie (Heidelberg, Germany)
2025

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

Brain pathology (Zurich, Switzerland)
2024

Role of Fine Needle Aspiration Cytology in Paratesticular Neoplasms-A Case Reports-Based Systematic Review.

Diagnostic cytopathology
2024

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

Neuro-oncology advances
2025

Constitutional mosaicism of pathogenic variants in SMARCB1 in a subset of patients with sporadic rhabdoid tumors.

Neuro-oncology
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

Elimination of mutant SWI/SNF complexes by protein quality control: new opportunities targeting aggressive rhabdoid tumours.

Signal transduction and targeted therapy
2024

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

Cell reports. Medicine
2024

PHF6 cooperates with SWI/SNF complexes to facilitate transcriptional progression.

Nature communications
2024

Effect of emergent nephrostomy on long-term total and split renal function in patients with upper urinary tract obstruction due to pelvic malignant tumors.

Pediatric surgery international
2024

Resection of a primary mediastinal malignant rhabdoid tumor: A study in multidisciplinary collaboration and 3-dimensional printing.

JTCVS techniques
2024

Uterine tumors mimicking ovarian sex cord tumors with rhabdoid differentiation: a clinicopathologic study of 4 cases: A case series analysis.

Medicine
2024

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

Journal of neurosurgery. Case lessons
2024

NRP1 knockdown inhibits the invasion and migration of rhabdoid tumor of the kidney cells.

Pediatric surgery international
2024

Discovery of CW-3308 as a Potent, Selective, and Orally Efficacious PROTAC Degrader of BRD9.

Journal of medicinal chemistry
2024

Clinical protocol phase II study of tumor infiltrating lymphocytes in advanced tumors with alterations in the SWI/SNF complex: the TILTS study.

Future oncology (London, England)
2024

Clinical and Molecular Risk Factors in Extracranial Malignant Rhabdoid Tumors: Toward an Integrated Model of High-Risk Tumors.

Clinical cancer research : an official journal of the American Association for Cancer Research
2024

Late-onset tumors in rhabdoid tumor predisposition syndrome type-1 (RTPS1) and implications for surveillance.

European journal of human genetics : EJHG
2025

SMARCA4-deficient primary bone sarcoma with "teratoid" features in a rhabdoid tumor predisposition syndrome patient.

Virchows Archiv : an international journal of pathology
2024

Postzygotic mosaicism of SMARCB1 variants in patients with rhabdoid tumors: A not-so-rare condition exposing to successive tumors.

Neuro-oncology
2024

Malignant Rhabdoid Tumor and Related Pediatric Tumors: Multimodality Imaging Review with Pathologic Correlation.

Radiographics : a review publication of the Radiological Society of North America, Inc
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

Malignant rhabdoid tumor of the urinary bladder in a 1-year and 9-month-old girl: A case report and literature review.

Pediatrics and neonatology
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

Tumoral EIF4EBP1 regulates the crosstalk between tumor-associated macrophages and tumor cells in MRTK.

European journal of pharmacology
2024

SMARCB1/INI1-deficient undifferentiated carcinoma of the ileum with features of rhabdoid tumor: A case report.

Asian journal of surgery
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

Synergistic Antitumor Activity of Talazoparib and Temozolomide in Malignant Rhabdoid Tumors.

Cancers
2024

Mesenchymal neoplasms of the tongue: A clinicopathologic study of 93 cases.

Human pathology
2024

Knocking Out CD70 Rescues CD70-Specific NanoCAR T Cells from Antigen-Induced Exhaustion.

Cancer immunology research
2024

Targeting dependency on a paralog pair of CBP/p300 against de-repression of KREMEN2 in SMARCB1-deficient cancers.

Nature communications
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Doenças relacionadas

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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. Impact of Radiation Therapy on Malignant Rhabdoid Tumor of the Kidney: A National Cancer Database Analysis.
    Pediatric blood &amp; cancer· 2026· PMID 41834080mais citado
  2. Longitudinal Detection of Tumor-Specific Peptides in Cerebrospinal Fluid for Pediatric Brain Tumor Surveillance.
    Cells· 2026· PMID 41827907mais citado
  3. Rhabdoid tumors as a novel target for PSMA-directed CAR T cell therapy.
    Molecular therapy. Oncology· 2026· PMID 41624404mais citado
  4. 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
  5. Atypical Teratoid/Rhabdoid Tumor of the Posterior Fossa Mimicking Medulloblastoma in an Infant: A Case Report.
    Cureus· 2026· PMID 41841066mais 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:69077(Orphanet)
  2. MONDO:0002728(MONDO)
  3. GARD:7572(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q6743515(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

Tumores rabdoides
Compêndio · Raras BR

Tumores rabdoides

ORPHA:69077 · MONDO:0002728
Prevalência
Unknown
Casos
500 casos conhecidos
Herança
Not applicable
CID-10
C49.9 · Neoplasia maligna do tecido conjuntivo e tecidos moles, não especificados
Ensaios
36 ativos
Início
Antenatal, Childhood, Infancy, Neonatal
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0206743
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

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