Neuroblastoma: an enigmatic disease

Neuroblastoma is the most common extra-cranial solid tumor of childhood. 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 features of 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.