Markedly improving survival of neuroblastoma: a 30-year analysis of 1,646 patients

Abstract

We sought to define current incidence trends and outcomes for pediatric patients with neuroblastoma. The SEER registry was queried from 1973 to 2002. Overall, 1,646 patients with neuroblastoma were identified. The annual incidence has remained unchanged at 0.9 per 100,000. The median age of the population was 1 year, with 42% of patients presenting at <1 year of age. The majority of tumors arose in the retroperitoneum (75.6%) with the remainder located in the mediastinum (15.3%), cervical region (6.6%) and pelvis (2.2%). Markedly improved survival has been noted in each decade (P < 0.002). Sixteen percent of lesions were over 10 cm in greatest dimension, while 84% were high-grade. Disease-specific survival at 1, 2, 5 and 20 years for the entire cohort was 81, 70, 61 and 59%, respectively. Superior survival was observed for infants <1 year of age (P < 0.001). Neuroblastomas in the mediastinum and pelvis had a better prognosis (P < 0.05) while high-grade and lesions over 10 cm carried a worse prognosis (P < 0.022). Surgery but not radiotherapy was associated with improvement in survival (P < 0.001). Multivariate analysis identified age, tumor location, stage, decade of diagnosis and surgical treatment as independent prognostic factors. Neuroblastoma remains a common malignancy with markedly improving patient outcomes. Early diagnosis and surgical therapy continue to provide the best chance for cure. More effective therapies for patients presenting over 1 year of age or those with advanced disease are still needed.