Increasing Incidence and Improved Survival for Neuroblastoma and Ganglioneuroblastoma in Australian Children: Data from the Australian Childhood Cancer Registry (1983-2014)

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Jones, B
Youlden, D
Cundy, T
Karpelowsky, J
Aitken, J
McBride, C
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2018
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Kyoto, Japan

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Abstract

Background/Objectives: To describe the incidence and outcomes, including second primary malignancy (SPM), for childhood neuroblastoma and ganglioneuroblastoma in Australia using national data from the Australian Childhood Cancer Registry (ACCR).

Design/Methods: All children (0‐14 years) diagnosed with neuroblastoma and ganglioneuroblastoma from 1983‐2014 inclusive were identified in the ACCR. Demographic, tumour characteristic, incidence, mortality, and treatment data were extracted. Cause‐specific (CS) and event‐free (EFS) survival up to 20 years from diagnosis were estimated using the cohort method. Adjusted excess mortality hazard ratios (HR) were calculated using a multivariate flexible parametric survival model. Cumulative incidence, standardised incidence ratios (SIR), and mortality relating to SPMs were estimated relative to the general population.

Results: A total of 1,227 children were diagnosed with neuroblastoma or ganglioneuroblastoma during the study period with an age‐standardised incidence rate of 9.9 per million children per year between 2010‐2014. Incidence rates increased by an estimated 15% from 1983 to 2014. The proportion of patients with non‐metastatic disease at diagnosis increased from 36% in 1983‐1994 to 53% in 2005‐2014 (p<0.001). Cause‐specific survival was 66% and 64% at 5 and 20 years, respectively. The mortality risk due to neuroblastoma and ganglioneuroblastoma more than halved from 1983‐1992 to 2003‐2012 (HR = 0.48, p<0.001). Five‐year EFS was 60%. Of patients achieving full remission, 28% relapsed with subsequent 5‐year CS of only 21%. Thirteen patients were diagnosed with a SPM (cumulative incidence at 20 years = 2.4%), giving an AER of 9.6 per 10,000 person‐years, and a SIR of 5.39. Leukaemias were the most common SPM (6 patients), and 54% of those with an SPM died within 5 years of the SPM diagnosis.

Conclusions: Diagnosis of non‐metastatic neuroblastoma and ganglioneuroblastoma, and cause‐specific survival, have substantially improved over time in Australia. Relapse portends a poor prognosis. SPMs are uncommon and carry a better prognosis than a relapse of the primary tumour.

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Pediatric Blood & Cancer

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65

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S2

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Clinical sciences

Oncology and carcinogenesis

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Life Sciences & Biomedicine

Hematology

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Jones, B; Youlden, D; Cundy, T; Karpelowsky, J; Aitken, J; McBride, C, Increasing Incidence and Improved Survival for Neuroblastoma and Ganglioneuroblastoma in Australian Children: Data from the Australian Childhood Cancer Registry (1983-2014), Pediatric Blood & Cancer, 2018, 65, pp. S665-S665