%0 Journal Article
%A Juhnke, B-Ole
%A Gessi, Marco
%A Gerber, Nicolas U
%A Friedrich, Carsten
%A Mynarek, Martin
%A von Bueren, André O
%A Haberler, Christine
%A Schüller, Ulrich
%A Kortmann, Rolf-Dieter
%A Timmermann, Beate
%A Bison, Brigitte
%A Warmuth-Metz, Monika
%A Kwiecien, Robert
%A Pfister, Stefan M
%A Spix, Claudia
%A Pietsch, Torsten
%A Kool, Marcel
%A Rutkowski, Stefan
%A von Hoff, Katja
%T Treatment of Embryonal Tumours with Multilayered Rosettes with Carboplatin/Etoposide Induction and High-dose Chemotherapy within the Prospective P-HIT Trial.
%J Neuro-Oncology
%V 24
%N 1
%@ 1523-5866
%C Oxford
%I Oxford Univ. Press
%M DKFZ-2021-00965
%P 127-137
%D 2022
%Z 2022 Jan 5;24(1):127-137
%X Embryonal 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
%K ETMR (Other)
%K clinical trial (Other)
%K high-dose chemotherapy (Other)
%K incidence (Other)
%K outcome (Other)
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:33908610
%R 10.1093/neuonc/noab100
%U https://inrepo02.dkfz.de/record/168539