Neuroblastoma.

Abstract

The neuroblastic tumours, derived from primordial neural crest cells which ultimately populate the sympathetic ganglia, adrenal medulla and other sites, (Brodeur GM and Castleberry RP. Neuroblastoma. In Pizzo PA, Poplack DG, eds, Principles and Practice of Pediatric Oncology. Philadelphia, J. B. Lippincott Co., 1997, 761-797) are an enigmatic group of neoplasms which have the highest rate of spontaneous regression of all human malignant neoplasms yet one of the poorest outcomes when occurring as disseminated disease in children. Significant advances in understanding and predicting the natural history of neuroblastoma have resulted from translational studies coupling tumour biology and clinical features to form prognostic strata and allowing more accurate routeing of patients to risk-related management. While this strategy has clarified the management for lower risk tumours, little improvement in survival for higher risk disease has been realised. Ironically, this latter patient subset, for which the most innovative therapeutic strategies are needed, is also the one from which the least tumour biology is gleaned owing to inadequate tissue sampling. This update will summarise the evolving biology of neuroblastoma and its relationship to current risk-related therapy and future management strategies. Throughout this report, prognostic grouping by age will be infants (< 1 year) versus children (> or = 1 year) since the change of risk according to age seems most distinct at this cut-off point.