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.

Clinical Sciences Pediatric Oncology and Hematology Lund University Lund Sweden

Crown Princess Victoria Children´s Hospital Linköping University Hospital Linköping Sweden

Department of Biomedical and Clinical Sciences Linköping University Linköping Sweden

Department of Clinical Pathology Kuopio University Hospital and Unit of Pathology Institute of Clinical Medicine University of Eastern Finland Kuopio Finland

Department of Clinical Pathology Kuopio University Hospital Kuopio Finland

Department of Clinical Radiology Kuopio University Hospital Kuopio Finland

Department of Diagnostic and Interventional Neuroradiology University Medical Center Hamburg Eppendorf Hamburg Germany

Department of Neuroradiology University Hospital Augsburg Augsburg Germany

Department of Paediatric and Adolescent Medicine Aarhus University Hospital Aarhus Denmark

Department of Paediatrics The University of Melbourne Parkville Victoria Australia

Department of Pathology and Molecular Medicine 2nd Faculty of Medicine Charles University and University Hospital Motol Prague Czech Republic

Department of Pediatric Hematology Oncology Valley Children's Hospital Madera California

Department of Pediatric Oncology and Hematology Charité Universitätsmedizin Berlin Corporate Member of Freie Universität Berlin Humboldt Universität zu Berlin and Berlin Institute of Health Berlin Germany

Department of Pediatric Oncology Hematology Immunology Olgahospital Klinikum Stuttgart Stuttgart Germany

Department of Pediatrics Pediatric Hematology and Oncology Ward Kuopio University Hospital Kuopio Finland

Division of Pediatric Glioma Research Heidelberg Germany

Faculty of Medicine Neuroradiological Reference Center for the pediatric brain tumor studies of the German Society of Pediatric Oncology and Hematology University Augsburg Augsburg Germany

From the Institute of Neuroradiology Charité Universitätsmedizin Berlin corporate member of Freie Universität Berlin and Humboldt Universität zu Berlin Germany

German Cancer Research Center Heidelberg Germany

Institut de Pathologie Multisite Site Est Groupement Hospitalier Est Hospices Civils de Lyon Lyon France

Institute Neurophysiopathol Aix Marseille University APHM CNRS INP centre hospitalier universitaire Timone Service d'Anatomie Pathologique et de Neuropathologie Marseille France

Institute of Clinical Medicine University of Eastern Finland Kuopio Finland

Institute of Neuropathology University Medical Center Hamburg Eppendorf Hamburg Germany

Murdoch Children's Research Institute The Royal Children's Hospital Parkville Victoria Australia

National Center for Tumor Diseases NCT Heidelberg a partnership between DKFZ and Heidelberg University Hospital Heidelberg Germany

Rare Cancers Genomics Team Genomic Epidemiology Branch International Agency for Research on Cancer World Health Organization Lyon France

Service de Neurochirurgie B Centre Hospitalier Universitaire de Bordeaux Bordeaux France

University Bordeaux Bordeaux INP CNRS IMB UMR 5251 Talence France

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Comparative Clinical and Imaging-Based Evaluation of Therapeutic Modalities in CNS Embryonal Tumours With PLAGL Amplification