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
No comments:
Post a Comment