Journal Article

DKFZ-2018-02198

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png A mechanistic classification of clinical phenotypes in neuroblastoma.

Ackermann, S. ; Cartolano, M. ; Hero, B. ; Welte, A. ; Kahlert, Y. ; Roderwieser, A. ; Bartenhagen, C. ; Walter, E. ; Gecht, J. ; Kerschke, L. ; Volland, R. ; Menon, R. ; Heuckmann, J. M. ; Gartlgruber, M.DKFZ* ; Hartlieb, S.DKFZ* ; Henrich, K.-O.DKFZ* ; Okonechnikov, K.DKFZ* ; Altmüller, J. ; Nürnberg, P. ; Lefever, S. ; de Wilde, B. ; Sand, F. ; Ikram, F. ; Rosswog, C. ; Fischer, J. ; Theissen, J. ; Hertwig, F.DKFZ* ; Singhi, A. D. ; Simon, T. ; Vogel, W. ; Perner, S. ; Krug, B. ; Schmidt, M. ; Rahmann, S. ; Achter, V. ; Lang, U. ; Vokuhl, C. ; Ortmann, M. ; Büttner, R. ; Eggert, A. ; Speleman, F. ; O'Sullivan, R. J. ; Thomas, R. K.DKFZ* ; Berthold, F. ; Vandesompele, J. ; Schramm, A. ; Westermann, F. (Last author)DKFZ* ; Schulte, J. (Last author)DKFZ* ; Peifer, M. ; Fischer, M.et al. Show all 50 authors

2018
Moses King Cambridge, Mass.

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Please use a persistent id in citations: doi:10.1126/science.aat6768

Abstract: 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.

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Contributing Institute(s):

1. B062 Pädiatrische Neuroonkologie (B062)
2. B087 Neuroblastom Genomik (B087)
3. DKTK Heidelberg (L101)

Research Program(s):

1. 312 - Functional and structural genomics (POF3-312) (POF3-312)

Appears in the scientific report 2018

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Database coverage:
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