ArticleClinical neuroradiology2019
MRI Phenotype of RELA-fused Pediatric Supratentorial Ependymoma.
Article in Clinical neuroradiology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 40 citations in OpenAlex.
- Review
- Recurrence patterns in pediatric intracranial ependymal neoplasm: a systematic imaging work-up.Neuroradiology · 2025Article
- Optimizing outcomes in intracranial ependymoma: a contemporary review.Frontiers in oncology · 2025Review
- Supra-tentorial Ependymomas with ZFTA Fusion, YAP1 Fusion, and Astroblastomas, MN1-altered: Characteristic Imaging Features.Clinical neuroradiology · 2024Article
- Ependymal Tumors: Overview of the Recent World Health Organization Histopathologic and Genetic Updates with an Imaging Characteristic.AJNR. American journal of neuroradiology · 2024Review
- Approaches to supratentorial brain tumours in children.Neuroradiology · 2024Review
- Imaging of supratentorial ependymomas with radio-pathological correlation.Exploration of targeted anti-tumor therapy · 2024Article
- Pediatric CNS tumors and 2021 WHO classification: what do oncologists need from pathologists?Frontiers in molecular neuroscience · 2024Review
- Ependymoma from Benign to Highly Aggressive Diseases: A Review.Advances and technical standards in neurosurgery · 2024Review
- Cyclin-Dependent Kinase Inhibitor 2A/B Homozygous Deletion Prediction and Survival Analysis.Brain sciences · 2023Article
- Changes to pediatric brain tumors in 2021 World Health Organization classification of tumors of the central nervous system.Pediatric radiology · 2023Review
- Pediatric diencephalic tumors: a constellation of entities and management modalities.Frontiers in oncology · 2023Review
- 2021 WHO classification of tumours of the central nervous system: a review for the neuroradiologist.Neuroradiology · 2022Review
- The role of artificial intelligence in paediatric neuroradiology.Pediatric radiology · 2022Review
- Driver mutations in ADGRL3 are involved in the evolution of ependymoma.Laboratory investigation; a journal of technical methods and pathology · 2022Article
- Review
- Pre- and Post-Treatment Imaging of Primary Central Nervous System Tumors in the Molecular and Genetic Era.Korean journal of radiology · 2021Review
- Pediatric ependymoma: an overview of a complex disease.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2021Review
- Supratentorial ependymoma in childhood: more than just RELA or YAP.Acta neuropathologica · 2021Article
- Diagnostics of pediatric supratentorial RELA ependymomas: integration of information from histopathology, genetics, DNA methylation and imaging.Brain pathology (Zurich, Switzerland) · 2019Article
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Authors and funding
10 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeEpigenetic profiling has recently identified clinically and molecularly distinct subgroups of ependymoma. The 2016 World Health Organization (WHO) classification recognized supratentorial ependymomas (ST-EPN) with REL-associated protein/p65 (RELA) fusion as a clinicopathological entity. These tumors represent 70% of pediatric ST-EPN characterized by recurrent C11orf95-RELA fusion transcripts, which lead to pathological activation of the nuclear factor 'kappa-light-chain-enhancer' of activated B-cells (NF-κB) signaling pathway. Cyclin-dependent kinase inhibitor 2A (CDKN2A) inactivation has also been reported to correlate with poor prognosis. Here, we systematically describe magnetic resonance imaging (MRI) characteristics of RELA-fused ST-EPN, with respect to CDKN2A deletion status.
methodsOur cohort of patients with ST-EPN (n = 57) was obtained from the database of the German Brain Tumor Reference Center of the German Society for Neuropathology and Neuroanatomy (DGNN), and tumors were diagnosed according to the 2016 WHO classification. Molecular characterization identified 47 RELA-fused tumors. We analyzed the preoperative MRI according to standardized criteria, and comparison was performed between CDKN2A altered (n = 21) and CDKN2A wild type (n = 26) tumors.
resultsThe RELA-fused ST-EPN showed a spectrum of predominantly hemispheric tumors with cysts and necrosis. Statistical analysis on CDKN2A status revealed significant differences in terms of younger manifestation age (p =0.002) and more intratumoral hemorrhage in T2-weighted imaging (T2WI) (p =0.010) in wild type tumors; however, the location was not a parameter for differentiation.
conclusionThis study first provides comprehensive MRI data for RELA-fused ST-EPN as a distinct entity, with further interest on CDKN2A genomic status. Patient stratification by morphological MRI alone seems difficult at present. The results may support ongoing research in ST-EPN within the framework of the radiogenomics concept.
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