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British Medical Bulletin 52:787-801 (1996)
© 1996 The British Council

research-article

Neuroblastoma: an enigmatic disease

Josée Brossard*,, Mark L Bernstein{dagger} and Bernard Lemieux*

*Department of Pediatrics, Centre universitaire de santé de l'Estrie, University of Sherbrooke
{dagger}Departments of Pediatrics and Oncology, Montreal Children's, Hospital, McGill University Quebec, Canada

Dr Josée Brossard, Department of Pediatrics, Centre universtaire de santé de l'Estrie, 3001, 12th Avenue North, Sherbrooke, Quebec, Canada J1H 5N4

Abstract

Neuroblastoma is the most common extra-cranial solid tumor of childhood1. It originates in cells of the neural crest, and so can be found anywhere along the paravertebral sympathetic chain or in the adrenal gland. In the last 15 years, new developments in the genetics and biology of neuroblastoma, have led to a better understanding of the natural history and prognostic featuresof this cancer. The presence of identifying biochemical markers detectable in the urine of patients with neuroblastoma, as well as the remarkably inferior survival of children diagnosed at more than 12 months of age, have led some groups to screen infants for neuroblastoma, in the hope of decreasing both overall mortality, as well as the incidence of advanced stage disease. This article reviews some clinical aspects of neuroblastoma, but emphasizes the genetic and biologic features in relation to prognosis and treatment. Finally, we discuss the different screening experiences for this disease, in particular from the Quebec Neuroblastoma Screening Project.


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