Current aspects of biology, risk assessment, and treatment of neuroblastoma

Neuroblastoma is one of the most intensely studied solid malignancies that affect the pediatric age groups; its clinical presentation, treatment strat egies and ultimate prognosis vary greatly. The biologic and genetic charact er of each tumor has an important impact on disease behavior, and clinical staging now incorporates these factors to generate an overall therapy plan. The clinical presentation of neuroblastoma is related to primary tumor loc ation, production of metabolically active substances, and the presence of m etastatic disease. There are also prognostically important associated syndr omes including opsoclonus- myoclonus, Horner's syndrome, neurofibromatosis, and a variety of other neurocristopathies. The histologic features of the tumor are of prognostic significance and are utilized in treatment stratifi cation. The International Neuroblastoma Staging System (INSS) has unified c lassic clinical staging. Features at diagnosis and those determined by init ial operation are combined with biologic prognostic factors to achieve risk group assignment for virtually all patients. There are groups of children in which limited therapy is curative and intermediate-risk situations where standard multimodality treatment provides favorable outcomes. Unfortunatel y, there are many patients with high-risk disease that require intensive st rategies, but success is still limited. It is in these most resistant patie nts that innovative approaches are being undertaken and novel strategies ar e being investigated. (C) 1999 Wiley-Liss, Inc.