Improved survival of children with neuroblastoma between 1979 and 2005: a report of the Italian Neuroblastoma Registry.
OBJECTIVE
To describe treatment, clinical course, and survival of a cohort of Italian patients with neuroblastoma.


METHODS
The study includes data from 2,216 children (age 0 to 14 years) diagnosed between 1979 and 2005. Overall survival (OS) was analyzed by clinical and biologic features at presentation and periods of diagnosis: 1979 to 1984, 1985 to 1991, 1992 to 1998, and 1999 to 2005. The relative risk of second malignant neoplasm (SMN) was assessed by the standardized incidence ratio (SIR), with the Italian population selected as referent.


RESULTS
Yearly patient accrual increased over time from 58 to 102. Patients age 0 to 17 months represented 45.6% of the total population, and their incidence increased over time from 36.5% to 48.5%. The incidence of stage 1 patients increased over time from 5.8% to 23.2%. A total of 898 patients (40.5%) developed disease progression or relapse, 19 patients developed SMN, and two patients developed myelodysplasia. The cumulative risk of SMN at 20 years was 7.1%, for an SIR of 8.4 (95% CI, 5.1 to 13.2). A total of 858 patients (39%) died (779 of disease, 71 of toxicity, six of SMN, and two of tumor-unrelated surgical complications). Ten-year OS was 55.3% (95% CI, 53.0% to 57.6%) and increased over time from 34.9% to 65.0%; it was significantly better for females and patients age 0 to 17 months at diagnosis, with extra-abdominal primary, and stage 1 and 2 disease. OS improved significantly over time in stage 1 and 3 patients. In patients with stage 4 disease, the improvement occurred between the first and second time cohorts (6.7% v 23.5%), but not afterward.


CONCLUSIONS
The outcome of children with neuroblastoma has progressively improved. Long-term survivors bear a significant risk of SMN.
Haupt
Riccardo
R
Epidemiology and Biostatistics Section, Scientific Directorate, Department of Hematology-Oncology, and Laboratories of Diagnostic Pathology and Clinical Chemistry, Giannina Gaslini Children's Hospital, Genova, Italy. brunodebernardi@ospedale-gaslini.ge.it
Garaventa
Alberto
A
Gambini
Claudio
C
Parodi
Stefano
S
Cangemi
Giuliana
G
Casale
Fiorina
F
Viscardi
Elisabetta
E
Bianchi
Maurizio
M
Prete
Arcangelo
A
Jenkner
Alessandro
A
Luksch
Roberto
R
Di Cataldo
Andrea
A
Favre
Claudio
C
D'Angelo
Paolo
P
Zanazzo
Giulio Andrea
GA
Arcamone
Giampaolo
G
Izzi
Gian Carlo
GC
Gigliotti
Anna Rita
AR
Pastore
Guido
G
De Bernardi
Bruno
B
D000293:Adolescent; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007558:Italy / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy; D016016:Proportional Hazards Models; D012008:Recurrence; D012042:Registries; D018570:Risk Assessment; D012307:Risk Factors; D015996:Survival Rate; D017741:Survivors / Q000706:statistics & numerical data*; D013997:Time Factors; D016896:Treatment Outcome