TY  - JOUR
AU  - Ackermann, Sandra
AU  - Cartolano, Maria
AU  - Hero, Barbara
AU  - Welte, Anne
AU  - Kahlert, Yvonne
AU  - Roderwieser, Andrea
AU  - Bartenhagen, Christoph
AU  - Walter, Esther
AU  - Gecht, Judith
AU  - Kerschke, Laura
AU  - Volland, Ruth
AU  - Menon, Roopika
AU  - Heuckmann, Johannes M
AU  - Gartlgruber, Moritz
AU  - Hartlieb, Sabine
AU  - Henrich, Kai-Oliver
AU  - Okonechnikov, Konstantin
AU  - Altmüller, Janine
AU  - Nürnberg, Peter
AU  - Lefever, Steve
AU  - de Wilde, Bram
AU  - Sand, Frederik
AU  - Ikram, Fakhera
AU  - Rosswog, Carolina
AU  - Fischer, Janina
AU  - Theissen, Jessica
AU  - Hertwig, Falk
AU  - Singhi, Aatur D
AU  - Simon, Thorsten
AU  - Vogel, Wenzel
AU  - Perner, Sven
AU  - Krug, Barbara
AU  - Schmidt, Matthias
AU  - Rahmann, Sven
AU  - Achter, Viktor
AU  - Lang, Ulrich
AU  - Vokuhl, Christian
AU  - Ortmann, Monika
AU  - Büttner, Reinhard
AU  - Eggert, Angelika
AU  - Speleman, Frank
AU  - O'Sullivan, Roderick J
AU  - Thomas, Roman K
AU  - Berthold, Frank
AU  - Vandesompele, Jo
AU  - Schramm, Alexander
AU  - Westermann, Frank
AU  - Schulte, Johannes
AU  - Peifer, Martin
AU  - Fischer, Matthias
TI  - A mechanistic classification of clinical phenotypes in neuroblastoma.
JO  - Science
VL  - 362
IS  - 6419
SN  - 0036-8075
CY  - Cambridge, Mass.
PB  - Moses King
M1  - DKFZ-2018-02198
SP  - 1165 - 1170
PY  - 2018
AB  - Neuroblastoma is a pediatric tumor of the sympathetic nervous system. Its clinical course ranges from spontaneous tumor regression to fatal progression. To investigate the molecular features of the divergent tumor subtypes, we performed genome sequencing on 416 pretreatment neuroblastomas and assessed telomere maintenance mechanisms in 208 of these tumors. We found that patients whose tumors lacked telomere maintenance mechanisms had an excellent prognosis, whereas the prognosis of patients whose tumors harbored telomere maintenance mechanisms was substantially worse. Survival rates were lowest for neuroblastoma patients whose tumors harbored telomere maintenance mechanisms in combination with RAS and/or p53 pathway mutations. Spontaneous tumor regression occurred both in the presence and absence of these mutations in patients with telomere maintenance-negative tumors. On the basis of these data, we propose a mechanistic classification of neuroblastoma that may benefit the clinical management of patients.
LB  - PUB:(DE-HGF)16
C6  - pmid:30523111
DO  - DOI:10.1126/science.aat6768
UR  - https://inrepo02.dkfz.de/record/141968
ER  -