ReviewBrain pathology (Zurich, Switzerland)2022
Updates in the classification of ependymal neoplasms: The 2021 WHO Classification and beyond.
Review in Brain pathology (Zurich, Switzerland), 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 68 papers, 1 of them a synthesis that pooled it.
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Who cites it
68 citing papers in PubMed, 1 synthesis or guideline pooled it, 105 citations in OpenAlex.
- EANS-EANO guidelines on the extent of resection in gliomas.Neuro-oncology · 2026Guideline
- AOSNP-ADAPTR resource level-based recommendations on practical diagnostic strategies for ependymomas.Brain pathology (Zurich, Switzerland) · 2026Article
- Supratentorial ependymoma: a comprehensive review of molecular classification, management strategies, and clinical outcomes (Part II of pediatric ependymomas across compartments).Journal of neuro-oncology · 2026Review
- Spinal ependymoma: a comprehensive review of molecular classification, management guidelines, and clinical outcomes with a focus on the pediatric population (Part III of ependymomas across compartments).Journal of neuro-oncology · 2026Review
- Challenging anatomical paradigms: unexpected supratentorial localization of posterior fossa B ependymomas identified by DNA methylation.Acta neuropathologica communications · 2026Article
- Outcomes after surgical resection of spinal ependymomas: extent of resection, imaging phenotype, and survival.European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society · 2026Article
- Feasibility of long-read nanopore sequencing for methylation-based classification of posterior fossa ependymomas.Brain tumor pathology · 2026Article
- A Nationwide Central Diagnosis for Pediatric CNS Tumors in Japan: The JCCG Brain Tumor and Pathology Committees.Cancer science · 2026Article
- Clinical, surgical, and molecular features of pediatric supratentorial ependymomas: a single-institution experience.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026Article
- Clinical and Magnetic Resonance Features of Spinal Myxopapillary Ependymoma.Neurosurgery practice · 2026Article
- A rare case of intradural extramedullary anaplastic ependymoma: a 35-month survival update following resection and adjuvant proton beam radiation therapy. Illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- Precision diagnosis and therapy for pediatric central nervous system tumors: consensus from the Brazilian Society of Pediatric Oncology (SOBOPE).Lancet regional health. Americas · 2026Review
- Genomic and mutational landscape of anaplastic ependymoma: Insights from the AACR Project GENIE Consortium.Biomolecules & biomedicine · 2026Article
- Two pediatric supratentorial ependymal tumors with novel PLAG1 fusions.Acta neuropathologica communications · 2026Article
- Report from the National Pediatric Cancer Foundation - infantile glioma and other non-embryonal central nervous system tumors: evolving molecular advances and current treatment landscape.Frontiers in oncology · 2026Review
- Predictors of Long-Term Outcome and Recurrence of Intramedullary Spinal Ependymomas: A multicenter study.Neurosciences (Riyadh, Saudi Arabia) · 2026Article
- Targeted and systemic therapies for recurrent adult ependymomas: real-world outcomes from a single institution and concise literature review.Frontiers in oncology · 2026Article
- Ependymal enhancement on MRI: imaging patterns and diagnostic algorithms - a pictorial essay.Neuroradiology · 2026Review
- Cerebello-Pontine Angle Tumors in Children: An Update on Challenging Neoplasms.Diagnostics (Basel, Switzerland) · 2026Article
- Intracranial ependymoma grade II with lipomatous metaplasia: a case report and literature review.Frontiers in oncology · 2026Article
8 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Ependymal neoplasms occur at all ages and encompass multiple tumor types and subtypes that develop in the supratentorial compartment, the posterior fossa, or the spinal cord. Clinically, ependymomas represent a very heterogeneous group of tumors from rather benign subependymomas to very aggressive and often deadly childhood ependymomas of the posterior fossa. Newly identified biological markers and classification schemes, e. g. based on global DNA methylation profiling, have led to the definition of 10 types of ependymal tumors and an improved prediction of patients' outcome by applying the new classification system. While the exact genetic basis for several ependymoma types still remains unclear, the knowledge about ependymoma driving events has significantly increased within the last decade and contributed to a classification based on molecular characteristics and localization rather than histological features alone. Convincing evidence is now pointing towards gene fusions involving ZFTA or YAP1 causing the development of supratentorial ependymomas. Also, H3, EZHIP, or TERT mutations have been detected in a fraction of infratentorial ependymal tumors. Finally, MYCN amplifications have recently been identified in spinal ependymomas, in addition to the previously known mutations in NF2. This review summarizes how recent findings regarding biology, molecular tumor typing, and clinical outcome have impacted the classification of ependymomas as suggested by the updated 2021 WHO CNS tumor classification system. We focus on changes compared to the previous classification of 2016 and discuss how a formal grading could evolve in the future and guide clinicians to treat ependymoma patients.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.