Danny R Youlden1,2, Brendan C Jones3,4, Thomas P Cundy5,6, Jonathan Karpelowsky7,8,9, Joanne F Aitken1,2,10,11, Craig A McBride2,3,4. 1. Australian Childhood Cancer Registry, Cancer Council Queensland, Brisbane, Queensland, Australia. 2. Menzies Health Institute Queensland, Griffith University, Gold Coast, Queensland, Australia. 3. Department of Paediatric Surgery, Children's Health Queensland Hospital and Health Service, Queensland Children's Hospital, Brisbane, Queensland, Australia. 4. Discipline of Paediatrics and Child Health, University of Queensland, Brisbane, Queensland, Australia. 5. Department of Paediatric Surgery, Women's and Children's Hospital, Adelaide, South Australia, Australia. 6. Discipline of Surgery, University of Adelaide, Adelaide, South Australia, Australia. 7. Department of Paediatric Surgery, The Children's Hospital at Westmead, Sydney, New South Wales, Australia. 8. Division of Child and Adolescent Health, University of Sydney, Sydney, New South Wales, Australia. 9. Children's Cancer Research Unit, Kids Research Institute, Sydney, New South Wales, Australia. 10. School of Public Health, University of Queensland, Brisbane, Queensland, Australia. 11. Institute for Resilient Regions, University of Southern Queensland, Brisbane, Queensland, Australia.
Abstract
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.
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.