Journal of Clinical Oncology, Vol 10, 323-329, Copyright © 1992 by American Society of Clinical Oncology
A population-based study of neuroblastoma incidence, survival, and mortality in North America [published erratum appears in J Clin Oncol 1992 Jul;10(7):1202]
ML Bernstein, JM Leclerc, G Bunin, L Brisson, L Robison, J Shuster, T Byrne, D Gregory, G Hill and G Dougherty
Department of Pediatrics, McGill University, Montreal Children's Hospital, Quebec, Canada.
PURPOSE: The purpose of this study was twofold: (1) to provide a
population-based estimate of neuroblastoma incidence, disease stage and age
distribution, and survival and mortality rates in North America; and (2) to
compare these figures in the province of Quebec at a time shortly before
the institution of province-wide screening with those in a population-based
control group, the Greater Delaware Valley (GDV) Pediatric Tumor Registry.
MATERIALS AND METHODS: In Quebec, the four major pediatric teaching
hospital records were searched for children with a diagnosis of
neuroblastoma. Tumor board registry data and information supplied to the
Division of Vital Statistics were also reviewed. Birth statistics were
obtained from the population registry. The GDV Pediatric Tumor Registry is
a population-based registry of pediatric cancer covering all of Delaware
and parts of New Jersey, Pennsylvania, and Maryland. Age, stage of disease,
and follow-up data were obtained through December 31, 1989, with Evans
neuroblastoma staging data used for all comparisons. RESULTS: One hundred
thirty children with neuroblastoma were identified in Quebec and 165 in the
GDV, in a combined population of 3,178,736 children. The annual incidence
of neuroblastoma was 10.95/10(6) under the age of 15 years and 27.75/10(6)
between the ages of 0 and 4 years. The annual mortality rate due to
neuroblastoma was 4.89/10(6) and 9.10/10(6) for the age groups 0 to 14 and
0 to 4, respectively. The overall 10-year survival rate for the 295 cases
of neuroblastoma was 55%. The 10-year survival rates for patients with
Evans stage I-IV and IVS disease were 88%, 90%, 63%, 21%, and 81%. There
was no significant difference observed in the incidence, mortality, or
survival in the two populations. CONCLUSION: These data represent the first
large, population-based description of the clinical presentation and
outcome of patients with neuroblastoma in North America, with no
significant differences noted between Quebec patients and the GDV patients.
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