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Comparative Clinical and Imaging-Based Evaluation of Therapeutic Modalities in CNS Embryonal Tumours With PLAGL Amplification
MK. Keck, A. Tietze, B. Bison, S. Avula, J. Engelhardt, C. Faure-Conter, T. Fenouil, D. Figarella-Branger, E. Goebell, J. Gojo, C. Haberler, J. Hakumäki, JT. Hayden, LS. Korhonen, E. Koscielniak, CM. Kramm, MEG. Kranendonk, M. Lequin, LE. Ludlow,...
Language English Country England, Great Britain
Document type Journal Article, Comparative Study
Grant support
001
World Health Organization - International
GN-000707
The Brain Tumour Charity
PubMed
40196918
DOI
10.1111/nan.70015
Knihovny.cz E-resources
- MeSH
- Gene Amplification MeSH
- Child MeSH
- DNA-Binding Proteins * genetics MeSH
- Adult MeSH
- Neoplasms, Germ Cell and Embryonal * genetics therapy pathology diagnostic imaging MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Central Nervous System Neoplasms * genetics therapy pathology diagnostic imaging MeSH
- Brain Neoplasms * genetics therapy MeSH
- Child, Preschool MeSH
- Retrospective Studies MeSH
- Check Tag
- Child MeSH
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Child, Preschool MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Comparative Study MeSH
AIMS: Embryonal tumours with PLAGL1 or PLAGL2 amplification (ET, PLAGL) show substantial heterogeneity regarding their clinical characteristics and have been treated inconsistently, resulting in diverse outcomes. In this study, we aimed to evaluate the clinical behaviour of ET, PLAGL and elucidate their response pattern across the different applied treatment regimens. METHODS: We conducted an in-depth retrospective analysis of clinical and serial imaging data of 18 patients with ET, PLAGL (nine each of PLAGL1 and PLAGL2 amplified). RESULTS: Patients with PLAGL1-amplified tumours (ET, PLAGL1) had fewer relapses (3/9), while PLAGL2-amplified tumours (ET, PLAGL2) were prone to early relapse or progression (8/9) and to distant, leptomeningeal and intraventricular relapses. Progression-free survival differed significantly between the subtypes (log-rank test, p = 0.0055). Postoperative treatment included chemotherapy (n = 17, various protocols), alone (n = 8) or combined with radiotherapy (n = 9). Responses to chemotherapy were observed in both subtypes, and incomplete resection was not associated with inferior survival. All three survivors with ET, PLAGL2 were treated with induction and high-dose chemotherapy with (n = 1-low-dose CSI and boost) or without (n = 2) radiotherapy, whereas five patients with less intensive chemotherapy relapsed. All six survivors with ET, PLAGL1 were treated with conventional chemotherapy regimens, with (n = 4-local radiotherapy n = 3; CSI and boost n = 1) or without (n = 2) radiotherapy. Two patients with ET, PLAGL1 relapsed after 8 years. CONCLUSIONS: Adjuvant therapy should be considered for all ET, PLAGL patients: Patients with ET, PLAGL2 might benefit from intensified chemotherapy regimens. In contrast, patients with ET, PLAGL1 showed superior outcomes without high-dose chemotherapy or craniospinal irradiation.
Children's Cancer Centre The Royal Children's Hospital Parkville Victoria Australia
Crown Princess Victoria Children's Hospital Linköping University Hospital Linköping Sweden
Department of Biomedical and Clinical Sciences Linköping University Linköping Sweden
Department of Clinical Radiology Kuopio University Hospital Kuopio Finland
Department of Neuro Oncology Princess Máxima Center for Pediatric Oncology Utrecht the Netherlands
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 Pediatric Hematology Oncology Valley Children's Hospital Madera California USA
Department of Radiology Alder Hey Children's NHS Foundation Trust Liverpool UK
Department of Radiology Olgahospital Klinikum Stuttgart Stuttgart Germany
Division of Imaging and Oncology Department of Radiology UMC Utrecht the Netherlands
Division of Pediatric Glioma Research Hopp Children's Cancer Center Heidelberg Heidelberg Germany
Division of Pediatric Hematology and Oncology University Medical Center Göttingen Göttingen Germany
German Cancer Research Center Heidelberg Germany
Institut d'Hemato oncologie Pediatrique Lyon 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
Princess Máxima Center for Pediatric Oncology Utrecht The Netherlands
Service de Neurochirurgie B CHU de Bordeaux Bordeaux France
Université de Bordeaux Bordeaux INP CNRS IMB UMR 5251 Talence France
References provided by Crossref.org
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