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000300291 1001_ $$0P:(DE-He78)9f1196ab1abb80483e1f69e8c0c4172d$$aKeck, Michaela-Kristina$$b0$$eFirst author$$udkfz
000300291 245__ $$aComparative Clinical and Imaging-Based Evaluation of Therapeutic Modalities in CNS Embryonal Tumours With PLAGL Amplification.
000300291 260__ $$aOxford [u.a.]$$bWiley-Blackwell$$c2025
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000300291 520__ $$aEmbryonal 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.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).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.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.
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000300291 650_7 $$2Other$$aPLAGL1
000300291 650_7 $$2Other$$aPLAGL2
000300291 650_7 $$2Other$$aET, PLAGL
000300291 650_7 $$2Other$$aembryonal CNS tumour
000300291 650_7 $$2Other$$atreatment
000300291 650_7 $$2NLM Chemicals$$aDNA-Binding Proteins
000300291 650_2 $$2MeSH$$aHumans
000300291 650_2 $$2MeSH$$aMale
000300291 650_2 $$2MeSH$$aFemale
000300291 650_2 $$2MeSH$$aAdult
000300291 650_2 $$2MeSH$$aAdolescent
000300291 650_2 $$2MeSH$$aChild
000300291 650_2 $$2MeSH$$aChild, Preschool
000300291 650_2 $$2MeSH$$aGene Amplification
000300291 650_2 $$2MeSH$$aMiddle Aged
000300291 650_2 $$2MeSH$$aDNA-Binding Proteins: genetics
000300291 650_2 $$2MeSH$$aBrain Neoplasms: genetics
000300291 650_2 $$2MeSH$$aBrain Neoplasms: therapy
000300291 7001_ $$aTietze, Anna$$b1
000300291 7001_ $$aBison, Brigitte$$b2
000300291 7001_ $$aAvula, Shivaram$$b3
000300291 7001_ $$aEngelhardt, Julien$$b4
000300291 7001_ $$aFaure-Conter, Cécile$$b5
000300291 7001_ $$aFenouil, Tanguy$$b6
000300291 7001_ $$aFigarella-Branger, Dominique$$b7
000300291 7001_ $$aGoebell, Einar$$b8
000300291 7001_ $$aGojo, Johannes$$b9
000300291 7001_ $$aHaberler, Christine$$b10
000300291 7001_ $$aHakumäki, Juhana$$b11
000300291 7001_ $$aHayden, James T$$b12
000300291 7001_ $$aKorhonen, Laura S$$b13
000300291 7001_ $$aKoscielniak, Ewa$$b14
000300291 7001_ $$aKramm, Christof M$$b15
000300291 7001_ $$aKranendonk, Mariëtte E G$$b16
000300291 7001_ $$aLequin, Maarten$$b17
000300291 7001_ $$aLudlow, Louise E$$b18
000300291 7001_ $$aMeyronet, David$$b19
000300291 7001_ $$aNyman, Per$$b20
000300291 7001_ $$aØra, Ingrid$$b21
000300291 7001_ $$aPerwein, Thomas$$b22
000300291 7001_ $$aPesola, Jouni$$b23
000300291 7001_ $$aRauramaa, Tuomas$$b24
000300291 7001_ $$aReddingius, Roel$$b25
000300291 7001_ $$aSamuel, David$$b26
000300291 7001_ $$aSchouten-van Meeteren, Antoinette Y N$$b27
000300291 7001_ $$aSexton-Oates, Alexandra$$b28
000300291 7001_ $$aVasiljevic, Alexandre$$b29
000300291 7001_ $$avon Kalle, Thekla$$b30
000300291 7001_ $$aWefers, Annika K$$b31
000300291 7001_ $$aWesseling, Pieter$$b32
000300291 7001_ $$aZamecnik, Josef$$b33
000300291 7001_ $$aZapotocky, Michal$$b34
000300291 7001_ $$avon Hoff, Katja$$b35
000300291 7001_ $$0P:(DE-He78)551bb92841f634070997aa168d818492$$aJones, David$$b36$$eLast author$$udkfz
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