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

Tietze, Anna ; Bison, B. ; Engelhardt, J. ; Fenouil, T. ; Figarella-Branger, D. ; Goebell, E. ; Hakumäki, J. ; Koscielniak, E. ; Ludlow, L. E. and Meyronet, D. , et al. (2025) In American Journal of Neuroradiology 46(3). p.536-543
Abstract

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... (More)

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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type
Contribution to journal
publication status
published
subject
in
American Journal of Neuroradiology
volume
46
issue
3
pages
8 pages
publisher
American Society of Neuroradiology
external identifiers
  • pmid:39271290
  • scopus:86000609050
ISSN
0195-6108
DOI
10.3174/ajnr.A8496
language
English
LU publication?
yes
id
8ad5f362-a09f-42f3-882f-309dc3b49bed
date added to LUP
2025-06-10 11:11:36
date last changed
2025-07-08 14:12:33
@article{8ad5f362-a09f-42f3-882f-309dc3b49bed,
  abstract     = {{<p>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.</p>}},
  author       = {{Tietze, Anna and Bison, B. and Engelhardt, J. and Fenouil, T. and Figarella-Branger, D. and Goebell, E. and Hakumäki, J. and Koscielniak, E. and Ludlow, L. E. and Meyronet, D. and Nyman, P. and Øra, I. and Pesola, J. and Rauramaa, T. and Reddingius, R. E. and Samuel, D. and Sexton-Oates, A. and Vasiljevic, A. and Wefers, A. K. and Zamecnik, J. and Jones, D. T.W. and Keck, M. K. and von Hoff, K. and Avula, S. and Faure-Conter, C. and Gojo, J. and Haberler, C. and Hayden, J. T. and Johann, P. D. and Jones, D. and Korhonen, L. S. and Kramm, C. M. and Kranendonk, M. E.G. and Lequin, M. and Perwein, T. and van Meeteren, A. Y.N.Schouten and Tietze, A. and von Kalle, T. and von Zezschwitz, B. and Wesseling, P. and Zapotocky, M.}},
  issn         = {{0195-6108}},
  language     = {{eng}},
  number       = {{3}},
  pages        = {{536--543}},
  publisher    = {{American Society of Neuroradiology}},
  series       = {{American Journal of Neuroradiology}},
  title        = {{CNS Embryonal Tumor with PLAGL Amplification, a New Tumor in Children and Adolescents : Insights from a Comprehensive MRI Analysis}},
  url          = {{http://dx.doi.org/10.3174/ajnr.A8496}},
  doi          = {{10.3174/ajnr.A8496}},
  volume       = {{46}},
  year         = {{2025}},
}