ArticleChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2024
Central nervous system embryonal tumor with PLAGL1 amplification: a case report of a novel entity focusing on imaging findings.
Article in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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3 citing papers in PubMed, 4 citations in OpenAlex.
- Adult presentation of a PLAGL2-amplified neuroepithelial tumor mimicking glioblastoma: broadening the spectrum of a proposed entity.Acta neuropathologica communications · 2026Article
- Comparative Clinical and Imaging-Based Evaluation of Therapeutic Modalities in CNS Embryonal Tumours With PLAGL Amplification.Neuropathology and applied neurobiology · 2025Article
- CNS Embryonal Tumor with PLAGL Amplification, a New Tumor in Children and Adolescents: Insights from a Comprehensive MRI Analysis.AJNR. American journal of neuroradiology · 2025Article
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6 authors at 3 institutions in 3 countries.
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Abstract
The embryonal central nervous system (CNS) tumor with PLAGL1 (pleomorphic adenoma gene-like) amplification is a novel type of pediatric neoplasm with a distinct methylation profile, described for the first time in 2022. It may be located anywhere in the neuroaxis and, as its name implies, it is driven by the amplification and overexpression of one of the PLAG family genes. Although the associated clinical, immunohistopathological, and molecular characteristics are well characterized in the seminal report of this entity, data on the radiological features is still lacking. Here, we present a case report of a 4-year-old girl with a biopsy-proven PLAGL1-amplified brainstem tumor and provide a detailed description of the corresponding conventional neuroimaging characteristics, aiming to better delineate this entity and to increase the awareness of this pathology in the radiological community.
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