Incidence and outcomes of neuroblastoma in Australian children: A population-based study (1983-2015)
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Jones, Brendan C
Cundy, Thomas P
Karpelowsky, Jonathan
Aitken, Joanne F
McBride, Craig A
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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.
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Journal of Paediatrics and Child Health
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56
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7
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© 2020 Paediatrics and Child Health Division (Royal Australasian College of Physicians). This is the peer reviewed version of the following article: Incidence and outcomes of neuroblastoma in Australian children: A population-based study (1983-2015), Journal of Paediatrics and Child Health, 2020, 56 (7), pp. 1046-1052, which has been published in final form at https://doi.org/10.1111/jpc.14810. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions. This article may not be enhanced, enriched or otherwise transformed into a derivative work, without express permission from Wiley or by statutory rights under applicable legislation. Copyright notices must not be removed, obscured or modified. The article must be linked to Wiley’s version of record on Wiley Online Library and any embedding, framing or otherwise making available the article or pages thereof by third parties from platforms, services and websites other than Wiley Online Library must be prohibited.
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Paediatrics
Reproductive medicine
Clinical sciences
Health services and systems
Public health
Science & Technology
Life Sciences & Biomedicine
childhood cancer
incidence
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Youlden, DR; Jones, BC; Cundy, TP; Karpelowsky, J; Aitken, JF; McBride, CA, Incidence and outcomes of neuroblastoma in Australian children: A population-based study (1983-2015), Journal of Paediatrics and Child Health, 2020, 56 (7), pp. 1046-1052