title: Improved survival of children with neuroblastoma between 1979 and 2005: a report of the Italian Neuroblastoma Registry.
abstract: 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.
authors.affiliation: 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
authors.forename: Riccardo
authors.initials: R
authors.lastname: Haupt
authors.forename: Alberto
authors.initials: A
authors.lastname: Garaventa
authors.forename: Claudio
authors.initials: C
authors.lastname: Gambini
authors.forename: Stefano
authors.initials: S
authors.lastname: Parodi
authors.forename: Giuliana
authors.initials: G
authors.lastname: Cangemi
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: Arcangelo
authors.initials: A
authors.lastname: Prete
authors.forename: Alessandro
authors.initials: A
authors.lastname: Jenkner
authors.forename: Roberto
authors.initials: R
authors.lastname: Luksch
authors.forename: Andrea
authors.initials: A
authors.lastname: Di Cataldo
authors.forename: Claudio
authors.initials: C
authors.lastname: Favre
authors.forename: Paolo
authors.initials: P
authors.lastname: D'Angelo
authors.forename: Giulio Andrea
authors.initials: GA
authors.lastname: Zanazzo
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Arcamone
authors.forename: Gian Carlo
authors.initials: GC
authors.lastname: Izzi
authors.forename: Anna Rita
authors.initials: AR
authors.lastname: Gigliotti
authors.forename: Guido
authors.initials: G
authors.lastname: Pastore
authors.forename: Bruno
authors.initials: B
authors.lastname: De Bernardi
mesh_terms: 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