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

Abstract

Background: 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 1983-2015 were extracted. Cause-specific (CSS) and event-free (EFS) survival were estimated using the cohort method. Adjusted hazard ratios (HRs) were calculated using a multivariable flexible parametric survival model. Other outcomes investigated included recurrence and second primary malignancies (SPMs). Results:The study cohort comprised 1,269 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 EFS both improved significantly over time and reached 75% (95% CI=71%-79%) and 71% (95% CI=66%-75%) at 5 years postdiagnosis, respectively, for children diagnosed between 2004-2013. Of patients achieving full remission, 28% relapsed with subsequent 5-year CSS of only 20%. Although SPMs were rare, neuroblastoma survivors carried a 5-fold increased risk compared to cancer rates in the general population (standardised incidence ratio [SIR]=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 inAustralia, 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.

A comprehensive analysis of neuroblastoma incidence, survival, and racial and ethnic disparities from 2001 to 2019

“…als aged 0-12 months, noting a varied range in the age-standardized incidence rate of cases per million: 9.5 in Australia, 10.1 in Southern-Eastern Europe countries, 13.7 in Germany, 13.8 in Italy, and 14.3 in France. [14][15][16][17][18][19] These differences were shown to be driven primarily by variation of incidence rates in the first year of life as well as variation in the human development index (HDI). It is hypothesized that higher HDI results in increased detection of neuroblastoma because of the availability of modern imaging technology and increased access to health care services, 20 the difference found in comparing our comprehensive US data with that of other nations may be in part due to higher HDI in the United States.…”

“…In line with prior publications, we found that the overall incidence of neuroblastoma was stable. European data ranging from 1979 to 2016 of over 9000 patients demonstrate increased incidence in individuals aged 0 – 12 months, noting a varied range in the age‐standardized incidence rate of cases per million: 9.5 in Australia, 10.1 in Southern‐Eastern Europe countries, 13.7 in Germany, 13.8 in Italy, and 14.3 in France 14–19 . These differences were shown to be driven primarily by variation of incidence rates in the first year of life as well as variation in the human development index (HDI).…”

Suppression of endoplasmic reticulum stress-dependent autophagy enhances cynaropicrin-induced apoptosis via attenuation of the P62/Keap1/Nrf2 pathways in neuroblastoma

“…Neuroblastoma (NB), a malignant cancer originating from sympathetic nerves, is known to be the most common extracranial solid cancer in childhood, accounting for approximately 15% of all cancer-related paediatric deaths (Matthay et al, 2016;Youlden et al, 2020). Currently, there are multimodal strategies to treat NB, including surgery, radiotherapy, induction chemotherapy, immunotherapy and autologous stem cell transplantation either in combination or separately depending on the clinical features and disease stage (Cañete, 2020;Tas et al, 2020).…”

Anti-tumor effect of Huaier extract against neuroblastoma cells in vitro

“…Neuroblastoma (NB), an aggressive neural crest derived malignancy, is the most common pediatric solid tumor with the majority of cases presenting by 5 years of age, accounting for approximately 15% deaths of total pediatric cancers [1]. NB exerts a wide range of clinical behaviors from spontaneous regressions especially in children less than 1 year to lethal outcome even with intense multimodal therapy due to biologic heterogeneity [2,3].…”