title: Outcome of children with neuroblastoma after progression or relapse. A retrospective study of the Italian neuroblastoma registry.
abstract: The Italian Neuroblastoma Registry was investigated to describe 781 children with neuroblastoma experiencing tumour 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 dehydrogenase (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.
authors.affiliation: Department of Pediatric Hematology-Oncology, Giannina Gaslini Children's Hospital, Genova, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Garaventa
authors.forename: Stefano
authors.initials: S
authors.lastname: Parodi
authors.forename: Bruno
authors.initials: B
authors.lastname: De Bernardi
authors.forename: Daniela
authors.initials: D
authors.lastname: Dau
authors.forename: Carla
authors.initials: C
authors.lastname: Manzitti
authors.forename: Massimo
authors.initials: M
authors.lastname: Conte
authors.forename: Fiorina
authors.initials: F
authors.lastname: Casale
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Viscardi
authors.forename: Maurizio
authors.initials: M
authors.lastname: Bianchi
authors.forename: Paolo
authors.initials: P
authors.lastname: D'Angelo
authors.forename: Giulio Andrea
authors.initials: GA
authors.lastname: Zanazzo
authors.forename: Roberto
authors.initials: R
authors.lastname: Luksch
authors.forename: Claudio
authors.initials: C
authors.lastname: Favre
authors.forename: Angela
authors.initials: A
authors.lastname: Tamburini
authors.forename: Riccardo
authors.initials: R
authors.lastname: Haupt
mesh_terms: D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D007558:Italy / Q000453:epidemiology; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000401:mortality* / Q000628:therapy; D009447:Neuroblastoma / Q000401:mortality* / Q000628:therapy; D012189:Retrospective Studies; D012307:Risk Factors; D016879:Salvage Therapy / Q000401:mortality; D016019:Survival Analysis