TY - JOUR
T1 - Improved survival of children with neuroblastoma between 1979 and 2005
T2 - A report of the Italian neuroblastoma registry
AU - Haupt, Riccardo
AU - Garaventa, Alberto
AU - Gambini, Claudio
AU - Parodi, Stefano
AU - Cangemi, Giuliana
AU - Casale, Fiorina
AU - Viscardi, Elisabetta
AU - Bianchi, Maurizio
AU - Prete, Arcangelo
AU - Jenkner, Alessandro
AU - Luksch, Roberto
AU - Di Cataldo, Andrea
AU - Favre, Claudio
AU - D'Angelo, Paolo
AU - Zanazzo, Giulio Andrea
AU - Arcamone, Giampaolo
AU - Izzi, Gian Carlo
AU - Gigliotti, Anna Rita
AU - Pastore, Guido
AU - De Bernardi, Bruno
PY - 2010/5/10
Y1 - 2010/5/10
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=77952518375&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77952518375&partnerID=8YFLogxK
U2 - 10.1200/JCO.2009.24.8351
DO - 10.1200/JCO.2009.24.8351
M3 - Article
C2 - 20351331
AN - SCOPUS:77952518375
VL - 28
SP - 2331
EP - 2338
JO - Journal of Clinical Oncology
JF - Journal of Clinical Oncology
SN - 0732-183X
IS - 14
ER -