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Background  The prognosis of children with metastatic stage 4 neuroblastoma (NB) has remained poor in the past decade.    Patients and methods  Using microarray analyses of 342 primary tumors, we here developed and validated an easy to use gene expression-based risk score including 18 genes, which can robustly predict the outcome of stage 4 patients.    Results  This classifier was a significant predictor of overall survival in two independent validation cohorts [cohort 1 (n = 214):  P  = 6.3 × 10 −5 ; cohort 2 (n = 27):  P  = 3.1 × 10 −2 ]. The prognostic value of the risk score was validated by multivariate analysis including the established markers age and  MYCN  status  (P  = 0.027). In the pooled validation cohorts (n = 241), integration of the risk score with the age and/or  MYCN  status identified subgroups with significantly differing overall survival (ranging from 35 to 100 %).    Conclusion  Together, the 18-gene risk score classifier can identify patients with stage 4 NB with favorable outcome and may therefore improve risk assessment and treatment stratification of NB patients with disseminated disease.    Electronic supplementary material  The online version of this article (doi:10.1186/s12967-016-0896-7) contains supplementary material, which is available to authorized users.

An 18 gene expression-based score classifier predicts the clinical outcome in stage 4 neuroblastoma