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Abstract

The incidence and mortality of neuroblastoma was reviewed in the general context of childhood cancer in Canada for the periods 1982-86 and 1987-91. This was done to complement the preliminary work of the Quebec Neuroblastoma Screening Project that is studying the impact of screening North American infants for the preclinical detection of neuroblastoma on population-based mortality. Annual age-standardized incidence rates for all childhood cancer in Canada appear to have declined slightly (nonsignificantly) from 155.1 to 150.8 per million, between 1982-86 and 1987-91; the rates for neuroblastoma were stable between the two five-year periods (11.8 per million in 1982-86 and 11.4 per million in 1987-91). With respect to mortality, the age-standardized rates for childhood cancer in Canada have shown a declining trend between the first and second halves of the decade, from 43.4 to 34.7 per million, while the rates for neuroblastoma have not changed (4.4 and 4.2 per million). The age-specific distributions of incident cancers indicate that neuroblastoma accounts for the greatest proportion of all cancers in children less than one year of age. Similarly, neuroblastoma is the leading cause of cancer deaths in children aged one to four years. Theoretically, infants less than one year of age could benefit most from effective preventive interventions, treatment, and research.

Association Between Participation in Clinical Trials and Overall Survival Among Children With Intermediate- or High-risk Neuroblastoma

“…After activation of the COG biology study (ANBL00B1) in 2001, a median of 561 patients (IQR, 542-629 patients) were enrolled each year between 2001 and 2019. In the United States and Canada, approximately 700 to 800 new cases of neuroblastoma are diagnosed annually . Thus, approximately 50% to 57% of all patients with neuroblastoma who received a diagnosis in the 1990s and 70% to 80% of patients who received a diagnosis since 2001 are included in the INRG Data Commons.…”

“…In addition, we excluded 845 patients for whom risk group assignment could not be determined owing to unknown stage States and Canada, approximately 700 to 800 new cases of neuroblastoma are diagnosed annually. 26,27 Thus, approximately 50% to 57% of all patients with neuroblastoma who received a diagnosis in the 1990s and 70% to 80% of patients who received a diagnosis since 2001 are included in the INRG Data Commons. Of the 3058 patients with intermediate-risk disease, 41 (1.3%) were enrolled in a high-risk clinical trial and were excluded from survival analyses.…”

High-risk neuroblastoma with NF1 loss of function is targetable using SHP2 inhibition

“…Maintenance therapy consists of high-dose 13-cis retinoic acid (RA) (isotretinoin) ( Matthay et al, 2009 ); in addition, the inclusion of the anti-GD2 antibody dinutuximab with interleukin-2 (IL-2) and granulocyte macrophage-colony-stimulating factor (GM-CSF) during maintenance therapy improves outcomes in some patients ( McGinty and Kolesar, 2017 ; Sano et al, 2019b ; Yu et al, 2010 ). Still, half of all high-risk patients succumb to their disease, and NB accounts for the most cancer-related deaths in children aged 5 years and younger ( Gao et al, 1997 ). While there have been intentional investigations into new therapies for NB, including the dedicated new approaches to NB therapy ( Morris et al, 2013 ) consortium, major breakthroughs significantly altering the fate of high-risk NBs have been elusive.…”

Exploitation of the Apoptosis-Primed State of MYCN-Amplified Neuroblastoma to Develop a Potent and Specific Targeted Therapy Combination

“…Neuroblastoma is a neural-crest-derived cancer and is the leading cause of cancer-related deaths in children aged 1–4 years ( Gao et al., 1997 ). High-level amplifications of the MYCN oncogene is observed in about 20% of cases, and has long been associated with high-risk disease and poor outcome in neuroblastoma ( Huang and Weiss, 2013 ).…”