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000168539 0247_ $$2doi$$a10.1093/neuonc/noab100
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000168539 0247_ $$2ISSN$$a1523-5866
000168539 037__ $$aDKFZ-2021-00965
000168539 041__ $$aEnglish
000168539 082__ $$a610
000168539 1001_ $$aJuhnke, B-Ole$$b0
000168539 245__ $$aTreatment of Embryonal Tumours with Multilayered Rosettes with Carboplatin/Etoposide Induction and High-dose Chemotherapy within the Prospective P-HIT Trial.
000168539 260__ $$aOxford$$bOxford Univ. Press$$c2022
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000168539 500__ $$a2022 Jan 5;24(1):127-137
000168539 520__ $$aEmbryonal tumours with multilayered rosettes (ETMR) are highly aggressive tumours occurring in early childhood. Published clinical data refer to retrospective, heterogeneously treated cohorts. Here, we describe the outcome of patients treated according to the prospective P-HIT trial and subsequent HIT2000-interim-registry.Age-stratified treatment included carboplatin/etoposide-induction, tandem-high-dose chemotherapy ('CARBO/ETO+HDCT') and response-stratified radiotherapy. Patients with centrally reviewed neuropathological and molecularly confirmed diagnosis of ETMR recruited within the P-HIT trial (2001-2011; n=19), the HIT2000-interim-registry (2012-2014; n=12) and earlier HIT-trials (n=4) were selected for analysis.Age-adjusted incidence rate was 1.35 per 1 million children (aged 1-4 years) in the years 2012-2014. Median age at diagnosis for 35 patients was 2.9 years. Metastases at diagnosis were detected in 9 patients. One patient died due to postoperative complications. For 30 patients with non-brainstem tumour location, 5-year progression-free (PFS) and overall survival (OS) were 35% and 47% after treatment with CARBO/ETO+HDCT (n=17), compared to 0% and 8% with other treatments (n=13, p[OS]=0.011). All 4 patients with brainstem tumour died within 10 months after diagnosis. By multivariable analysis, supratentorial location: (HR[PFS]:0.07 [95%CI:0.01-0.38], p=0.003), localised disease (M0): (HR[OS] M0, no residual tumor:0.30 [95%CI:0.009-1.09], p=0.068; M0, residual tumor:0.18 [95%CI: 0.04-0.76], p=0.020) and CARBO/ETO+HDCT treatment (HR[OS]:0.16 [95%CI:0.05-054], p=0.003) were identified as independent prognostic factors. Of 9 survivors, 6 were treated with radiotherapy (craniospinal 4; local 2).Our data indicate improved survival with intensified chemotherapy (CARBO/ETO+HDCT). However, despite intensive treatment, the outcome was poor. Thus, innovative therapies need to be evaluated urgently in an upfront setting.
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000168539 650_7 $$2Other$$aETMR
000168539 650_7 $$2Other$$aclinical trial
000168539 650_7 $$2Other$$ahigh-dose chemotherapy
000168539 650_7 $$2Other$$aincidence
000168539 650_7 $$2Other$$aoutcome
000168539 7001_ $$aGessi, Marco$$b1
000168539 7001_ $$aGerber, Nicolas U$$b2
000168539 7001_ $$aFriedrich, Carsten$$b3
000168539 7001_ $$aMynarek, Martin$$b4
000168539 7001_ $$avon Bueren, André O$$b5
000168539 7001_ $$aHaberler, Christine$$b6
000168539 7001_ $$aSchüller, Ulrich$$b7
000168539 7001_ $$aKortmann, Rolf-Dieter$$b8
000168539 7001_ $$0P:(DE-HGF)0$$aTimmermann, Beate$$b9
000168539 7001_ $$aBison, Brigitte$$b10
000168539 7001_ $$aWarmuth-Metz, Monika$$b11
000168539 7001_ $$aKwiecien, Robert$$b12
000168539 7001_ $$0P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aPfister, Stefan M$$b13$$udkfz
000168539 7001_ $$aSpix, Claudia$$b14
000168539 7001_ $$aPietsch, Torsten$$b15
000168539 7001_ $$0P:(DE-He78)4c28e2aade5f44d8eca9dd8e97638ec8$$aKool, Marcel$$b16$$udkfz
000168539 7001_ $$aRutkowski, Stefan$$b17
000168539 7001_ $$avon Hoff, Katja$$b18
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