Um tumor embrionário raro do sistema nervoso central caracterizado por células embrionárias dispostas em rosetas multicamadas e exibindo um de três padrões morfológicos: tumor embrionário com neurópilo abundante e rosetas verdadeiras, ependimoblastoma ou meduloepitelioma. Os tumores apresentam tipicamente uma alteração no C19MC ou (raramente) uma mutação no DICER1 e correspondem ao grau IV da OMS. Eles localizam-se predominantemente no interior do crânio, raramente na medula espinhal, e comumente causam sinais e sintomas de pressão intracraniana elevada, algumas vezes convulsões e sinais neurológicos focais. A maioria dos casos ocorre em crianças durante os primeiros dois anos de vida.
Introdução
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Visão geral
O tumor embrionário com rosetas multicamadas é uma neoplasia embrionária rara do sistema nervoso central, classificada como grau IV pela OMS. O quadro se caracteriza histologicamente por células embrionárias organizadas em rosetas multicamadas, exibindo um de três padrões morfológicos: tumor embrionário com neurópilo abundante e rosetas verdadeiras, ependimoblastoma ou meduloepitelioma. A maioria dos casos acomete crianças durante os primeiros dois anos de vida.[1]
Sinais e sintomas
Esses tumores localizam-se predominantemente no interior do crânio e, de forma rara, na medula espinhal. Comumente, manifestam-se por meio de sinais e sintomas associados à pressão intracraniana elevada, além de poderem apresentar convulsões e sinais neurológicos focais.[1]
Causas genéticas
Do ponto de vista genético, as alterações características desses tumores envolvem tipicamente modificações no C19MC ou, mais raramente, mutações no gene DICER1.[1]
Tratamento e manejo
No âmbito do Sistema Único de Saúde (SUS), o PCDT vigente relacionado a 'Tumor Cerebral no Adulto' (Portaria Conjunta SAES-SCTIE/MS nº 07, de 13 de abril de 2020) prevê a conduta terapêutica conforme a autonomia do serviço médico responsável, sem a determinação de medicamentos dispensados padronizados especificamente para esta condição rara nos dados catalogados.[2]
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
O tumor embrionário com rosetas multicamadas (ETMR) é um tumor cerebral raro e de alto grau que atinge principalmente bebês e crianças nos primeiros dois anos de vida. Ele costuma provocar sintomas decorrentes da pressão alta na cabeça, além de possíveis **convulsões** (crises epilépticas) e alterações neurológicas. O diagnóstico é confirmado pela análise das células em rosetas e pela verificação de alterações genéticas como no C19MC. No Sistema Único de Saúde (SUS), o atendimento a tumores cerebrais segue o protocolo clínico com condutas dispensadas conforme a autonomia do serviço médico.
Um tumor embrionário raro do sistema nervoso central caracterizado por células embrionárias dispostas em rosetas multicamadas e exibindo um de três padrões morfológicos: tumor embrionário com neurópilo abundante e rosetas verdadeiras, ependimoblastoma ou meduloepitelioma. Os tumores apresentam tipicamente uma alteração no C19MC ou (raramente) uma mutação no DICER1 e correspondem ao grau IV da OMS. Eles localizam-se predominantemente no interior do crânio, raramente na medula espinhal, e comumente causam sinais e sintomas de pressão intracraniana elevada, algumas vezes convulsões e sinais neurológicos focais. A maioria dos casos ocorre em crianças durante os primeiros dois anos de vida.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
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Genética e causas
O que está alterado no DNA e como passa nas famílias
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Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
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In the latest version of the World Health Organization Classification (WHO) of Central Nervous System Tumors, the terminology "medulloepithelioma" has disappeared as a distinct tumor type in favor of "embryonal tumor with multilayered rosettes (ETMR)", characterized by frequent alterations of C19MC. However, medulloepithelioma persists in the current WHO classification of eye tumors, where two variants exist (teratoid and non-teratoid), harboring frequent DICER1 mutations. DNA-methylation profiling has demonstrated that ETMR and intraocular medulloepitheliomas represented two distinct methylation classes. Outside the CNS and the eye, exceptional observations of medulloepitheliomas have been reported, most of them before the genetic and epigenetic eras. Herein, we report a pediatric case of a presacral tumor classified as a teratoid medulloepithelioma with genetic and epigenetic charaterizations. Using DNA-methylation profiling analysis, the tumor was classified between ETMR, non C19MC-altered, and intra-ocular medulloepithelioma, arguing for a potential distinct cell origin, not represented in the current epigenetic classifiers.
DNA Methylation Profiling Classifies and Reveals Origin of Gynecologic Central Nervous System-Like Tumors.
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Central Nervous System Embryonal Tumors.
Central nervous system (CNS) embryonal tumors are a subset of aggressive pediatric brain tumors, more frequently seen in infants and young children. They share an aggressive imaging appearance with frequent disseminated disease and guarded prognosis; however, patient age, tumor location, and imaging characteristics such as enhancement pattern and peritumoral edema can help with differential diagnosis. In this article, we have reviewed medulloblastoma, atypical teratoid rhabdoid tumor, embryonal tumor with multilayered rosettes as well as newly recognized CNS neuroblastoma, FOXR2-activated and CNS tumor with BCOR internal tandem duplication.
ETMR stem-like state and chemo-resistance are supported by perivascular cells at single-cell resolution.
Embryonal tumor with multilayered rosettes (ETMR) is a lethal embryonal brain tumor entity. To investigate the intratumoral heterogeneity and cellular communication in the tumor microenvironment (TME), we analyze in this work single-cell RNA sequencing of about 250,000 cells of primary human and murine ETMR, in vitro cultures, and a 3D forebrain organoid model of ETMR, supporting the main findings with immunohistochemistry and spatial transcriptomics of human tumors. We characterize three distinct malignant ETMR subpopulations - RG-like, NProg-like and NB-like - positioned within a putative neurodevelopmental hierarchy. We reveal PDGFRβ+ pericytes as key communication partners in the TME, contributing to stem cell signaling through extracellular matrix-mediated interactions with tumor cells. PDGF signaling is upregulated in chemoresistant RG-like cells in vivo and plays a role in recruiting pericytes to ETMR TME by finalizing a signaling cascade which promotes the differentiation of non-malignant radial glia cells, derived from our 3D model, into pericyte-like cells. Selective PDGFR-inhibition blocked the lineage differentiation into pericytes in vitro and reduced the tumor cell population in vivo. Targeting ETMR-pericyte interactions in the TME presents a promising therapeutic approach.
Cellular hierarchies of embryonal tumors with multilayered rosettes are shaped by oncogenic microRNAs and receptor-ligand interactions.
Embryonal tumor with multilayered rosettes (ETMR) is a pediatric brain tumor with dismal prognosis. Characteristic alterations of the chromosome 19 microRNA cluster (C19MC) are observed in most ETMR; however, the ramifications of C19MC activation and the complex cellular architecture of ETMR remain understudied. Here we analyze 11 ETMR samples from patients using single-cell transcriptomics and multiplexed spatial imaging. We reveal a spatially distinct cellular hierarchy that spans highly proliferative neural stem-like cells and more differentiated neuron-like cells. C19MC is predominantly expressed in stem-like cells and controls a transcriptional network governing stemness and lineage commitment, as resolved by genome-wide analysis of microRNA-mRNA binding. Systematic analysis of receptor-ligand interactions between malignant cell types reveals fibroblast growth factor receptor and Notch signaling as oncogenic pathways that can be successfully targeted in preclinical models and in one patient with ETMR. Our study provides fundamental insights into ETMR pathobiology and a powerful rationale for more effective targeted therapies.
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Neuropathology : official journal of the Japanese Society of NeuropathologyAssociações
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É um tumor embrionário raro e agressivo do sistema nervoso central, classificado como grau IV pela OMS. Ele acomete principalmente crianças pequenas nos dois primeiros anos de vida e caracteriza-se por células em rosetas multicamadas.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
4 fontes citadas no texto · 7 publicações do grafo RarasNet (PubMed) · 3 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (2026). Tumor embrionário com rosetas multicamadas. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/tumor-embrionario-com-rosetas-multicamadas
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Tumor embrionário com rosetas multicamadas
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Ensaios clínicos
- fonte: ClinicalTrials.gov
