TY  - JOUR
AU  - Juhnke, B-Ole
AU  - Gessi, Marco
AU  - Gerber, Nicolas U
AU  - Friedrich, Carsten
AU  - Mynarek, Martin
AU  - von Bueren, André O
AU  - Haberler, Christine
AU  - Schüller, Ulrich
AU  - Kortmann, Rolf-Dieter
AU  - Timmermann, Beate
AU  - Bison, Brigitte
AU  - Warmuth-Metz, Monika
AU  - Kwiecien, Robert
AU  - Pfister, Stefan M
AU  - Spix, Claudia
AU  - Pietsch, Torsten
AU  - Kool, Marcel
AU  - Rutkowski, Stefan
AU  - von Hoff, Katja
TI  - Treatment of Embryonal Tumours with Multilayered Rosettes with Carboplatin/Etoposide Induction and High-dose Chemotherapy within the Prospective P-HIT Trial.
JO  - Neuro-Oncology
VL  - 24
IS  - 1
SN  - 1523-5866
CY  - Oxford
PB  - Oxford Univ. Press
M1  - DKFZ-2021-00965
SP  - 127-137
PY  - 2022
N1  - 2022 Jan 5;24(1):127-137
AB  - 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
KW  - ETMR (Other)
KW  - clinical trial (Other)
KW  - high-dose chemotherapy (Other)
KW  - incidence (Other)
KW  - outcome (Other)
LB  - PUB:(DE-HGF)16
C6  - pmid:33908610
DO  - DOI:10.1093/neuonc/noab100
UR  - https://inrepo02.dkfz.de/record/168539
ER  -