Advances in the diagnosis and treatment of neuroblastoma.
Neuroblastoma, a childhood neoplasm arising from neural crest cells, is characterized by a diversity of clinical behavior ranging from spontaneous remission to rapid tumor progression and death. To a large extent, outcome can be predicted by the stage of disease and the age at diagnosis. However, the molecular events responsible for the variability in response to treatment and the rate of tumor growth remain largely unknown. Over the past decade, transformation-linked genetic changes have been identified in neuroblastoma tumors that have contributed to the understanding of tumor predisposition, metastasis, treatment responsiveness, and prognosis. The Children's Oncology Group recently developed a Neuroblastoma Risk Stratification System that is currently in use for treatment stratification purposes, based on clinical and biologic factors that are strongly predictive of outcome. This review discusses the current risk-based treatment approaches for children with neuroblastoma and recent advances in biologic therapy.
Weinstein
Joanna L
JL
Department of Pediatrics and Robert H. Lurie Comprehensive Cancer Center, Northwestern University, The Feinberg School of Medicine, Chicago, Illinois, USA.
Katzenstein
Howard M
HM
Cohn
Susan L
SL
D000293:Adolescent; D000328:Adult; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D002648:Child; D002669:Child Welfare; D002675:Child, Preschool; D018450:Disease Progression; D006801:Humans; D015994:Incidence; D007223:Infant; D007229:Infant Welfare; D007231:Infant, Newborn; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D014481:United States / Q000453:epidemiology