Incidence and outcomes of neuroblastoma in Australian children: A population-based study (1983-2015).

AIM: Neuroblastoma predominantly affects younger children and exhibits heterogeneous behaviour. This study describes incidence and outcomes for neuroblastoma using national population-based data from the Australian Childhood Cancer Registry. METHODS: Deidentified data for all children (0-14 years) diagnosed with neuroblastoma and ganglioneuroblastoma from 1983 to 2015 were extracted. Cause-specific (CSS) and event-free survival were estimated using the cohort method. Adjusted hazard ratios were calculated using a multivariable flexible parametric survival model. Other outcomes investigated included recurrence and second primary malignancies (SPMs). RESULTS: The study cohort comprised 1269 patients. Age-standardised incidence rates remained steady across the study period at approximately 9.5 per million children per year. The proportion of patients with metastatic disease at diagnosis decreased from 63% in 1983-1995 to 42% by 2006-2015 (P < 0.001). CSS and event-free survival both improved significantly over time and reached 75% (95% confidence interval (CI) = 71-79%) and 71% (95% CI = 66-75%) at 5 years post-diagnosis, respectively, for children diagnosed between 2004 and 2013. Of patients achieving full remission, 28% relapsed with subsequent 5-year CSS of only 20%. Although SPMs were rare, neuroblastoma survivors carried a fivefold increased risk compared to cancer rates in the general population (standardised incidence ratio = 5.18, 95% CI = 3.01-8.91), with 7 of the 13 patients (54%) who were diagnosed with an SPM dying within 5 years. CONCLUSIONS: CSS for childhood neuroblastoma has improved substantially over time in Australia, but still remains lower than for most other types of childhood cancer. SPMs are uncommon and carry a better prognosis than relapse of the primary tumour.