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CNS Embryonal Tumor with PLAGL Amplification, a New Tumor in Children and Adolescents: Insights from a Comprehensive MRI Analysis

Tietze, A
Autor Tietze, A ORCID From the Institute of Neuroradiology (A.T.), Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Germany anna.tietze@charite.de
Bison, B
Autor Bison, B ORCID Department of Neuroradiology (B.B.), University Hospital Augsburg, Augsburg, Germany Faculty of Medicine (B.B.), Neuroradiological Reference Center for the pediatric brain tumor studies of the German Society of Pediatric Oncology and Hematology, University Augsburg, Augsburg, Germany
Engelhardt, J
Autor Engelhardt, J Service de Neurochirurgie B (J.E.), Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France University Bordeaux (J.E.), Bordeaux INP, CNRS, IMB, UMR 5251, Talence, France
Fenouil, T
Autor Fenouil, T Institut de Pathologie Multisite-Site Est (T.F., D.M., A.V.), Groupement Hospitalier Est, Hospices Civils de Lyon, Lyon, France
Figarella-Branger, D
Autor Figarella-Branger, D Institute Neurophysiopathol (D.F.-B.), Aix-Marseille University, APHM, CNRS, INP, centre hospitalier universitaire Timone, Service d'Anatomie Pathologique et de Neuropathologie, Marseille, France
Goebell, E
Autor Goebell, E ORCID Department of Diagnostic and Interventional Neuroradiology (E.G.), University Medical Center Hamburg-Eppendorf, Hamburg, Germany
Hakumäki, J
Autor Hakumäki, J Department of Clinical Radiology (J.H.), Kuopio University Hospital, Kuopio, Finland Institute of Clinical Medicine (J.H.), University of Eastern Finland, Kuopio, Finland
Koscielniak, E
Autor Koscielniak, E Department of Pediatric Oncology/Hematology/Immunology (E.K.), Olgahospital, Klinikum Stuttgart, Stuttgart, Germany
Ludlow, L E
Autor Ludlow, L E Murdoch Children's Research Institute (L.E.L.), and Children's Cancer Centre (L.E.L.), The Royal Children's Hospital, Parkville, Victoria, Australia Department of Paediatrics (L.E.L.), The University of Melbourne, Parkville, Victoria, Australia
Meyronet, D
Autor Meyronet, D Institut de Pathologie Multisite-Site Est (T.F., D.M., A.V.), Groupement Hospitalier Est, Hospices Civils de Lyon, Lyon, France

American journal of neuroradiology. 2025 ; 46 (3) : 536-543. [pub] 20250304

AJNR Am J Neuroradiol
ISSN 1936-959X
Medvik
Zdroj

BACKGROUND AND PURPOSE: CNS embryonal tumor with pleomorphic adenoma gene-like 1 (PLAGL1)/pleomorphic adenoma gene-like 2 (PLAGL2) amplification (ET, PLAGL) is a newly identified, highly malignant pediatric tumor. Systematic MRI descriptions of ET, PLAGL are currently lacking. MATERIALS AND METHODS: MRI data from 19 treatment-naïve patients with confirmed ET, PLAGL were analyzed. Evaluation focused on anatomic involvement, tumor localization, MRI signal characteristics, DWI behavior, and the presence of necrosis and hemorrhage. Descriptive statistics (median, interquartile range, percentage) were assessed. RESULTS: Ten patients had PLAGL1 and nine had PLAGL2 amplifications. The solid components of the tumors were often multinodular with heterogeneous enhancement (mild to intermediate in 47% and intermediate to strong in 47% of cases). Nonsolid components included cysts in 47% and necrosis in 84% of the cases. The tumors showed heterogeneous T2WI hyper- and isointensity (74%), relatively little diffusion restriction (ADC values less than contralateral normal-appearing WM in 36% of cases with available DWI), and tendencies toward hemorrhage/calcification (42%). No reliable distinction was found between PLAGL1- and PLAGL2-amplified tumors or compared with other embryonal CNS tumors. CONCLUSIONS: The study contributes to understanding the imaging characteristics of ET, PLAGL. It underscores the need for collaboration in studying rare pediatric tumors and advocates the use of harmonized imaging protocols for better characterization.

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