Purpose To describe treatment, clinical course, and survival of a cohort of Italian patients with neuroblastoma. Patiens and Methods The study includes data from 2.216 children(age 0 to 14 years)diagnosed between 1979 and 2005. Overal survival (OS) was analyzed by clinical and biologic featurres 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 patientes 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 56,3% (95% CI, 53.0% to 57.6%) and incresed 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:
Improved survival of children with neuroblastoma between 1979 and 2005: a report of the Italian Neuroblastoma Registry
CASALE, Fiorina;
2010
Abstract
Purpose To describe treatment, clinical course, and survival of a cohort of Italian patients with neuroblastoma. Patiens and Methods The study includes data from 2.216 children(age 0 to 14 years)diagnosed between 1979 and 2005. Overal survival (OS) was analyzed by clinical and biologic featurres 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 patientes 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 56,3% (95% CI, 53.0% to 57.6%) and incresed 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:I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
