Outcome of children with neuroblastoma after progression or relapse. A retrospective study of the Italian neuroblastoma registry.

The Italian Neuroblestoma Registry was invetigated to describe 781 children with neuroblastoma experiencing tumor recurrence (424 progressions and 357 relapses). Ten-year overall survival (OS) was 6.8% (95% confidence interval(CI) 4.3-10.0) after progression and 14.4% (95% CI 10.5%-18.9) after relapse: For both circumstances, OS was better for age at diagnosis<18 months, less advanced International Neuroblastoma Staging System (INSS) stage, normal lactate deydrogenase (LDH) serum level, normal MYCN gene status (P<0.001) and a non-abdominal primary site (P=0.034 for progression, and P=0.004 for relapses). A local type of recurrence had a significantly better outcome only in case of relapse (P<0.001). Probability of survival increased by era of diagnosis.Survival of children with recurrent neuroblastoma is very poor. A small cohort of patients, mainly represented by children with stages 1 and 2 who underwent local recurrence or developed late relapse may still benefit from further conventional treatment. For the remaining larger proportion of patients, experimental therapies should be proposed.