NEUROBLASTOMA

The neuroblastic tumours, derived from primordial neural crest cells w hich ultimately populate the sympathetic ganglia, adrenal medulla and other sites, (Brodeur GM and Castleberry RP. Neuroblastoma. In Pizzo P A, Poplack DG, eds, Principles and Practice of Pediatric Oncology. Phi ladelphia, 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 oc curring as disseminated disease in children. Significant advances in u nderstanding and predicting the natural history of neuroblastoma have resulted from translational studies coupling tumour biology and clinic al features to form prognostic strata and allowing more accurate route ing of patients to risk-related management. While this strategy has cl arified the management for lower risk tumours, little improvement in s urvival for higher risk disease has been realised. Ironically, this la tter patient subset, for which the most innovative therapeutic strateg ies are needed, is also the one from which the least tumour biology is gleaned owing to inadequate tissue sampling. This update will summari se the evolving biology of neuroblastoma and its relationship to curre nt risk-related therapy and future management strategies. Throughout t his report, prognostic grouping by age will be infants (<1 year) versu s children (greater than or equal to 1 year) since the change of risk according to age seems most distinct at this cut-off point. (C) 1997 P ublished by Elsevier Science Ltd.