Definition and research guide
This page combines a MeSH definition, publication statistics and selected research abstracts. It is a starting point for reading the literature, not a medical textbook or a systematic evidence review.
Content available: 4 complete abstracts (751 words), 10 references. Full article text is not stored or reproduced here; follow the PMID, DOI or PMC links to read the original sources and their access terms.
Definition
Glioma derived from ependymoglial cells that tend to present as malignant intracranial tumors in children and as benign intraspinal neoplasms in adults. It may arise from any level of the ventricular system or central canal of the spinal cord. Intracranial ependymomas most frequently originate in the fourth ventricle and histologically are densely cellular tumors which may contain ependymal tubules and perivascular pseudorosettes. Spinal ependymomas are usually benign papillary or myxopapillary tumors. (From DeVita et al., Principles and Practice of Oncology, 5th ed, p2018; Escourolle et al., Manual of Basic Neuropathology, 2nd ed, pp28-9)
Definition: NLM Medical Subject Headings (MeSH) scope note (Public domain (U.S. National Library of Medicine); attribution requested). View the MeSH record.
Also known as: Ependymomas; Ependymoma, Myxopapillary; Ependymomas, Myxopapillary; Myxopapillary Ependymoma; Myxopapillary Ependymomas; Ependymoma, Papillary; Ependymomas, Papillary; Papillary Ependymomas.
Where it fits in the MeSH classification
- Broader topics
- Glioma
- Narrower topics
- Glioma, Subependymal
- See also
- Ependymoglial Cells
Related concepts
- Ependymoglial Cells
- Fourth Ventricle
Research landscape
PubMed indexes 5,953 records tagged with the MeSH heading “Ependymoma” (counts retrieved 2026-10-29). 787 of them (13.2%) were published from 2020 onward, and the busiest period so far is through 1999 with 2,865 records.
Reviews account for 739 records (12.4%) and meta-analyses for 28. Randomized controlled trials number 14 (0.2%) as a share of all records on the topic. Randomized trials make up only a small share of this literature, so much of what is known comes from laboratory, observational or descriptive work.
Show the data
| Period | Records |
|---|---|
| through 1999 | 2,865 |
| 2000-2009 | 1,058 |
| 2010-2019 | 1,243 |
| 2020 onward | 787 |
Publication-type chart data
| Type | Records | Share |
|---|---|---|
| Reviews | 739 | 12.4% |
| Meta-analyses | 28 | 0.5% |
| Randomized controlled trials | 14 | 0.2% |
Method: PubMed query "Ependymoma"[MeSH Terms] with publication-type and date filters, retrieved 2026-10-29.
Reading the evidence in cancer and oncology
Cancer research spans very different kinds of evidence. Laboratory studies in cell lines and animal models reveal mechanisms but often do not translate to patients. Early-phase trials mainly test safety and dosing, while phase 3 randomized trials and their meta-analyses are the usual basis for treatment conclusions. Outcomes also differ in meaning: tumour shrinkage, progression-free survival and overall survival are not interchangeable, and screening studies can be distorted by lead-time and overdiagnosis. When reading a result, check the cancer type and stage studied, the comparison treatment, and which outcome was measured.
Evidence from open-access studies
These open-access studies come from PubMed’s best-match results for this heading (papers whose main topic it is), ranked here by citation count, with one recent addition. Each abstract is reproduced in full under its recorded open licence so that you can read the authors’ own words. They illustrate the literature; they are not a systematic summary of it. Citation counts measure attention, not reliability or clinical benefit.
| Study / authors | Year | Source label / design | Europe PMC citations | Licence / source |
|---|---|---|---|---|
| Single-Cell RNA-Seq Reveals Cellular Hierarchies and Impaired Developmental Trajectories in Pediatric Ependymoma | 2020 | Research article Design not recorded in structured metadata | 151 | cc by PMID 32663469 · DOI · PMC7479515 |
| Ependymoma: Evaluation and Management Updates | 2022 | Review article Design not recorded in structured metadata | 98 | cc by PMID 35384591 · DOI · PMC9249684 |
| Significance of molecular classification of ependymomas: C11orf95-RELA fusion-negative supratentorial ependymomas are a heterogeneous group of tumors | 2018 | Research article Design not recorded in structured metadata | 84 | cc by PMID 30514397 · DOI · PMC6278135 |
| Integrated transcriptomic landscape of medulloblastoma and ependymoma reveals novel tumor subtype-specific biology | 2026 | Research article Design not recorded in structured metadata | 6 | cc by PMID 41159380 · DOI · PMC12979040 |
Single-Cell RNA-Seq Reveals Cellular Hierarchies and Impaired Developmental Trajectories in Pediatric Ependymoma [1]
Abstract reading summary — source excerpts
- Opening excerpt
- Ependymoma is a heterogeneous entity of central nervous system tumors with well-established molecular groups.
- Closing excerpt
- Taken together, our analyses reveal a developmental hierarchy underlying ependymomas relevant to biological and clinical behavior.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (109 words)
Ependymoma is a heterogeneous entity of central nervous system tumors with well-established molecular groups. Here, we apply single-cell RNA sequencing to analyze ependymomas across molecular groups and anatomic locations to investigate their intratumoral heterogeneity and developmental origins. Ependymomas are composed of a cellular hierarchy initiating from undifferentiated populations, which undergo impaired differentiation toward three lineages of neuronal-glial fate specification. While prognostically favorable groups of ependymoma predominantly harbor differentiated cells, aggressive groups are enriched for undifferentiated cell populations. The delineated transcriptomic signatures correlate with patient survival and define molecular dependencies for targeted treatment approaches. Taken together, our analyses reveal a developmental hierarchy underlying ependymomas relevant to biological and clinical behavior.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 32663469 · DOI · PMC7479515
Ependymoma: Evaluation and Management Updates [2]
Abstract reading summary — source excerpts
- Opening excerpt
- Purpose of reviewTo review state of art and relevant advances in the molecular genetics and management of ependymomas of children and adults.
- Closing excerpt
- Standard treatments are still the mainstay of treatment: the discovery of new druggable pathways will hopefully increase the therapeutic armamentarium in the near future.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (176 words)
Purpose of reviewTo review state of art and relevant advances in the molecular genetics and management of ependymomas of children and adults.Recent findingsEpendymomas may occur either in the brain or in the spinal cord. Compared with intracranial ependymomas, spinal ependymomas are less frequent and exhibit a better prognosis. The new WHO classification of CNS tumors of 2021 has subdivided ependymomas into different histomolecular subgroups with different outcome. The majority of studies have shown a major impact of extent of resection; thus, a complete resection must be performed, whenever possible, at first surgery or at reoperation. Conformal radiotherapy is recommended for grade 3 or incompletely resected grade II tumors. Proton therapy is increasingly employed especially in children to reduce the risk of neurocognitive and endocrine sequelae. Craniospinal irradiation is reserved for metastatic disease. Chemotherapy is not useful as primary treatment and is commonly employed as salvage treatment for patients failing surgery and radiotherapy. Standard treatments are still the mainstay of treatment: the discovery of new druggable pathways will hopefully increase the therapeutic armamentarium in the near future.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 35384591 · DOI · PMC9249684
Significance of molecular classification of ependymomas: C11orf95-RELA fusion-negative supratentorial ependymomas are a heterogeneous group of tumors [3]
Abstract reading summary — source excerpts
- Opening excerpt
- Extensive molecular analyses of ependymal tumors have revealed that supratentorial and posterior fossa ependymomas have distinct molecular profiles and are likely to be different diseases.
- Closing excerpt
- RELA fusion-negative ST-EPN appear to be a heterogeneous group of tumors that do not fall into any of the existing molecular subgroups and are unlikely to form a single category.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (276 words)
Extensive molecular analyses of ependymal tumors have revealed that supratentorial and posterior fossa ependymomas have distinct molecular profiles and are likely to be different diseases. The presence of C11orf95-RELA fusion genes in a subset of supratentorial ependymomas (ST-EPN) indicated the existence of molecular subgroups. However, the pathogenesis of RELA fusion-negative ependymomas remains elusive. To investigate the molecular pathogenesis of these tumors and validate the molecular classification of ependymal tumors, we conducted thorough molecular analyses of 113 locally diagnosed ependymal tumors from 107 patients in the Japan Pediatric Molecular Neuro-Oncology Group. All tumors were histopathologically reviewed and 12 tumors were re-classified as non-ependymomas. A combination of RT-PCR, FISH, and RNA sequencing identified RELA fusion in 19 of 29 histologically verified ST-EPN cases, whereas another case was diagnosed as ependymoma RELA fusion-positive via the methylation classifier (68.9%). Among the 9 RELA fusion-negative ST-EPN cases, either the YAP1 fusion, BCOR tandem duplication, EP300-BCORL1 fusion, or FOXO1-STK24 fusion was detected in single cases. Methylation classification did not identify a consistent molecular class within this group. Genome-wide methylation profiling successfully sub-classified posterior fossa ependymoma (PF-EPN) into PF-EPN-A (PFA) and PF-EPN-B (PFB). A multivariate analysis using Cox regression confirmed that PFA was the sole molecular marker which was independently associated with patient survival. A clinically applicable pyrosequencing assay was developed to determine the PFB subgroup with 100% specificity using the methylation status of 3 genes, CRIP1, DRD4 and LBX2. Our results emphasized the significance of molecular classification in the diagnosis of ependymomas. RELA fusion-negative ST-EPN appear to be a heterogeneous group of tumors that do not fall into any of the existing molecular subgroups and are unlikely to form a single category.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 30514397 · DOI · PMC6278135
Integrated transcriptomic landscape of medulloblastoma and ependymoma reveals novel tumor subtype-specific biology [4]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundMedulloblastoma and ependymoma are common pediatric central nervous system tumors with significant molecular and clinical heterogeneity.
- Closing excerpt
- The landscape is publicly available via Oncoscape, an interactive platform for global exploration and application.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (190 words)
BackgroundMedulloblastoma and ependymoma are common pediatric central nervous system tumors with significant molecular and clinical heterogeneity. While molecular subgrouping has enabled classification into molecular subtypes, the extent of heterogeneity within these subgroups remains poorly defined.MethodsWe collected bulk RNA sequencing data from 888 medulloblastoma and 370 ependymoma tumors to establish a comprehensive reference landscape. After rigorous batch effect correction, normalization, and dimensionality reduction, we generated a unified landscape to explore gene expression, signaling pathways, RNA fusions, and copy number variations.ResultsOur transcriptional analysis revealed distinct clustering patterns, including two primary ependymoma compartments, EPN-E1 and EPN-E2, each with specific RNA fusions and molecular signatures. In medulloblastoma, we observed precise stratification of Group 3/4 tumors by subtype and in Sonic Hedgehog (SHH) tumors by patient age. We also identified subtype-specific pathways and gene fusions, enriched in each group.ConclusionsThis transcriptomic landscape serves as a resource for biomarker discovery, diagnostic refinement, and prediction of tumor biology and outcome. By enabling projection of new patients' bulk RNA-seq data onto the reference map using nearest neighbor analysis, the framework supports accurate subtype classification. The landscape is publicly available via Oncoscape, an interactive platform for global exploration and application.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 41159380 · DOI · PMC12979040
Key reviews and meta-analyses
The most cited review-type papers among PubMed’s best-match results for this heading, listed with bibliographic details only. Follow the links for the full text and access terms.
- Research article EANO guidelines for the diagnosis and treatment of ependymal tumors [5]
- Research article cIMPACT-NOW update 7: advancing the molecular classification of ependymal tumors [6]
- Research article Pediatric ependymoma: biological perspectives [7]
- Research article Biology and management of ependymomas [8]
- Research article Ependymoma [9]
- Research article Ependymomas in adults [10]
References
- Gojo J, Englinger B, Jiang L, Hübner JM, Shaw ML, Hack OA, et al.. Single-Cell RNA-Seq Reveals Cellular Hierarchies and Impaired Developmental Trajectories in Pediatric Ependymoma. . 2020. PMID 32663469 · DOI · PMC7479515
- Rudà R, Bruno F, Pellerino A, Soffietti R. Ependymoma: Evaluation and Management Updates. . 2022. PMID 35384591 · DOI · PMC9249684
- Fukuoka K, Kanemura Y, Shofuda T, Fukushima S, Yamashita S, Narushima D, et al.. Significance of molecular classification of ependymomas: C11orf95-RELA fusion-negative supratentorial ependymomas are a heterogeneous group of tumors. . 2018. PMID 30514397 · DOI · PMC6278135
- Arora S, Nuechterlein N, Jensen M, Glatzer G, Sievers P, Varadharajan S, et al.. Integrated transcriptomic landscape of medulloblastoma and ependymoma reveals novel tumor subtype-specific biology. . 2026. PMID 41159380 · DOI · PMC12979040
- Rudà R, Reifenberger G, Frappaz D, Pfister SM, Laprie A, Santarius T, et al.. EANO guidelines for the diagnosis and treatment of ependymal tumors. Neuro Oncol. 2018. PMID 29194500 · DOI · PMC5909649
- Ellison DW, Aldape KD, Capper D, Fouladi M, Gilbert MR, Gilbertson RJ, et al.. cIMPACT-NOW update 7: advancing the molecular classification of ependymal tumors. Brain Pathol. 2020. PMID 32502305 · DOI · PMC8018155
- Kilday JP, Rahman R, Dyer S, Ridley L, Lowe J, Coyle B, et al.. Pediatric ependymoma: biological perspectives. Mol Cancer Res. 2009. PMID 19531565 · DOI
- Wu J, Armstrong TS, Gilbert MR. Biology and management of ependymomas. Neuro Oncol. 2016. PMID 27022130 · DOI · PMC4896548
- Reni M, Gatta G, Mazza E, Vecht C. Ependymoma. Crit Rev Oncol Hematol. 2007. PMID 17482475 · DOI
- Gilbert MR, Ruda R, Soffietti R. Ependymomas in adults. Curr Neurol Neurosci Rep. 2010. PMID 20425040 · DOI
About this article
- Sources
- NLM Medical Subject Headings (definition, synonyms); NCBI PubMed (counts, identifiers); Europe PMC (open-access abstracts and citation counts).
- Verification
- Every reference was resolved against PubMed on 2026-10-31; title, year and DOI matched.
- Review state
- automated source verified; no human expert review has been recorded yet.
- Version
- 1, last updated 2026-10-29.
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Evidence strength
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