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Please use this identifier to cite or link to this item: http://hdl.handle.net/10400.18/794

Título: Segmental chromosomal alterations lead to a higher risk of relapse in infants with MYCN-non-amplified localised unresectable/disseminated neuroblastoma (a SIOPEN collaborative study).
Autor: Schleiermacher, G.
Michon, J.
Ribeiro, A.
Pierron, G.
Mosseri, V.
Rubie, H.
Munzer, C.
Bénard, J.
Auger, N.
Combaret, V.
Janoueix-Lerosey, I.
Pearson, A.
Tweddle, D.A.
Bown, N.
Gerrard, M.
Wheeler, K.
Noguera, R.
Villamon, E.
Cañete, A.
Castel, V.
Marques, B.
de Lacerda, A.
Tonini, G.P.
Mazzocco, K.
Defferrari, R.
de Bernardi, B.
di Cataldo, A.
van Roy, N.
Brichard, B.
Ladenstein, R.
Ambros, I.
Ambros, P.
Beiske, K.
Delattre, O.
Couturier, J.
Palavras-chave: Doenças Genéticas
Neuroblastoma
Segmental Chromosome Alterations
High Risk
Issue Date: Dec-2011
Editora: Nature Publishing Group
Citação: Br J Cancer. 2011 Dec 6;105(12):1940-8. Epub 2011 Nov 10
Resumo: BACKGROUND: In neuroblastoma (NB), the presence of segmental chromosome alterations (SCAs) is associated with a higher risk of relapse. METHODS: In order to analyse the role of SCAs in infants with localised unresectable/disseminated NB without MYCN amplification, we have performed an array CGH analysis of tumours from infants enrolled in the prospective European INES trials. RESULTS: Tumour samples from 218 out of 300 enroled patients could be analysed. Segmental chromosome alterations were observed in 11%, 20% and 59% of infants enroled in trials INES99.1 (localised unresectable NB), INES99.2 (stage 4s) and INES99.3 (stage 4) (P<0.0001). Progression-free survival was poorer in patients whose tumours harboured SCA, in the whole population and in trials INES99.1 and INES99.2, in the absence of clinical symptoms (log-rank test, P=0.0001, P=0.04 and P=0.0003, respectively). In multivariate analysis, a SCA genomic profile was the strongest predictor of poorer progression-free survival. CONCLUSION: In infants with stage 4s MYCN-non-amplified NB, a SCA genomic profile identifies patients who will require upfront treatment even in the absence of other clinical indication for therapy, whereas in infants with localised unresectable NB, a genomic profile characterised by the absence of SCA identifies patients in whom treatment reduction might be possible. These findings will be implemented in a future international trial.
Arbitragem científica: yes
URI: http://hdl.handle.net/10400.18/794
ISSN: 0007-0920
Versão do Editor: http://www.nature.com/bjc/journal/v105/n12/full/bjc2011472a.html
Appears in Collections:DGH - Artigos em revistas internacionais

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