Improved survival of children with neuroblastoma between 1979 and 2005: A report of the Italian neuroblastoma registry

Riccardo Haupt, Alberto Garaventa, Claudio Gambini, Stefano Parodi, Giuliana Cangemi, Fiorina Casale, Elisabetta Viscardi, Maurizio Bianchi, Arcangelo Prete, Alessandro Jenkner, Roberto Luksch, Andrea Di Cataldo, Claudio Favre, Paolo D'Angelo, Giulio Andrea Zanazzo, Giampaolo Arcamone, Gian Carlo Izzi, Anna Rita Gigliotti, Guido Pastore, Bruno De Bernardi

Research output: Contribution to journalArticlepeer-review


Purpose: To describe treatment, clinical course, and survival of a cohort of Italian patients with neuroblastoma. Patients and 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. Conclusion: The outcome of children with neuroblastoma has progressively improved. Long-term survivors bear a significant risk of SMN.

Original languageEnglish
Pages (from-to)2331-2338
Number of pages8
JournalJournal of Clinical Oncology
Issue number14
Publication statusPublished - May 10 2010

ASJC Scopus subject areas

  • Cancer Research
  • Oncology
  • Medicine(all)


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