Outcome of children with neuroblastoma after progression or relapse. A retrospective study of the Italian neuroblastoma registry.
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.
Garaventa
Alberto
A
Department of Pediatric Hematology-Oncology, Giannina Gaslini Children's Hospital, Genova, Italy.
Parodi
Stefano
S
De Bernardi
Bruno
B
Dau
Daniela
D
Manzitti
Carla
C
Conte
Massimo
M
Casale
Fiorina
F
Viscardi
Elisabetta
E
Bianchi
Maurizio
M
D'Angelo
Paolo
P
Zanazzo
Giulio Andrea
GA
Luksch
Roberto
R
Favre
Claudio
C
Tamburini
Angela
A
Haupt
Riccardo
R
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