Journal Article

DKFZ-2024-01869

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png CNS Embryonal Tumor with PLAGL Amplification, a New Tumor Type in Children and Adolescents: Insights from a Comprehensive MRI Analysis.

Tietze, A. ; Bison, B. ; Oncology, E. S. f. P. (Collaboration Author) ; Engelhardt, J. ; Fenouil, T. ; Figarella-Branger, D. ; Goebell, E. ; Hakumäki, J. ; Koscielniak, E. ; Ludlow, L. E. ; Meyronet, D. ; Nyman, P. ; Øra, I. ; Pesola, J. ; Rauramaa, T. ; Reddingius, R. E. ; Samuel, D. ; Sexton-Oates, A. ; Vasiljevic, A. ; Wefers, A. K. ; Zamecnik, J. ; Jones, D. ; Keck, M. K.DKFZ* ; von Hoff, K. ; Avula, S. (Contributor) ; Faure-Conter, C. (Contributor) ; Bison, B. (Contributor) ; Gojo, J. (Contributor) ; Haberler, C. (Contributor) ; Hayden, J. T. (Contributor) ; Johann, P. D. (Contributor) ; Jones, D. (Contributor)DKFZ* ; Korhonen, L. S. (Contributor) ; Kramm, C. (Contributor) ; Kranendonk, M. (Contributor) ; Lequin, M. (Contributor) ; Perwein, T. (Contributor) ; Schouten-van Meeteren, A. (Contributor) ; Tietze, A. (Contributor) ; von Kalle, T. (Contributor) ; von Zezschwitz, B. (Contributor) ; Wesseling, P. (Contributor) ; Zapotocky, M. (Contributor) ; von Hoff, K. (Contributor)

2025
Soc. Oak Brook, Ill.

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Please use a persistent id in citations: doi:10.3174/ajnr.A8496

Abstract: CNS embryonal tumor with PLAGL1/PLAGL2 amplification (ET, PLAGL) is a newly identified, highly malignant pediatric tumor. Systematic MRI descriptions of ET, PLAGL are currently lacking.MRI data from 19 treatment-naïve patients with confirmed ET, PLAGL were analyzed. Evaluation focused on anatomical involvement, tumor localization, MRI signal characteristics, DWI behavior, and the presence of necrosis and hemorrhage. Descriptive statistics (median, interquartile range, percentage) were assessed.Ten patients had PLAGL1 and nine 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). Non-solid 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 < contralateral normal-appearing WM in 36% of cases with available DWI), and tendencies towards hemorrhage/calcification (42%). No reliable distinction was found between PLAGL1-and PLAGL2-amplified tumors or compared to other embryonal CNS tumors.The study contributes to understanding the imaging characteristics of ET, PLAGL. It underscores the need for collaboration in studying rare pediatric tumors and advocates for the use of harmonized imaging protocols for better characterization.ATRT= atypical teratoid/rhabdoid tumor; ETMR= embryonal tumor with multilayered rosettes; ET, PLAGL= CNS embryonal tumor with PLAGL amplification; EVD= external ventricular drain; IQR: interquartile range; PLAGL1= pleomorphic adenoma gene-like 1; PLAGL2= pleomorphic adenoma gene-like 2; WHO= World Health Organization.

Note: 2025 Mar 4;46(3):536-543

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Contributing Institute(s):

1. Pädiatrische Gliomforschung (B360)

Research Program(s):

1. 312 - Funktionelle und strukturelle Genomforschung (POF4-312) (POF4-312)

Appears in the scientific report 2024

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Database coverage:
; BIOSIS Previews ; Biological Abstracts ; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; Current Contents - Life Sciences ; Essential Science Indicators ; IF < 5 ; JCR ; PubMed Central ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

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