Neuroblastoma: biological insights into a clinical enigma.
Neuroblastoma is a tumour derived from primitive cells of the sympathetic nervous system and is the most common solid tumour in childhood. Interestingly, most infants experience complete regression of their disease with minimal therapy, even with metastatic disease. However, older patients frequently have metastatic disease that grows relentlessly, despite even the most intensive multimodality therapy. Recent advances in understanding the biology and genetics of neuroblastomas have allowed classification into low-, intermediate- and high-risk groups. This allows the most appropriate intensity of therapy to be selected - from observation alone to aggressive, multimodality therapy. Future therapies will focus increasingly on the genes and biological pathways that contribute to malignant transformation or progression.
Brodeur
Garrett M
GM
Division of Oncology, The Children's Hospital of Philadelphia and the University of Pennsylvania, Philadelphia, Pennsylvania 19104-4318, USA. Brodeur@email.chop.edu
D000782:Aneuploidy; D002471:Cell Transformation, Neoplastic / Q000235:genetics; D002675:Child, Preschool; D002877:Chromosomes, Human / Q000235:genetics / Q000648:ultrastructure; D005544:Forecasting; D005729:Ganglioneuroma / Q000235:genetics / Q000473:pathology; D005784:Gene Amplification; D015972:Gene Expression Regulation, Neoplastic; D016259:Genes, myc; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D019656:Loss of Heterozygosity; D008957:Models, Genetic; D009363:Neoplasm Proteins / Q000235:genetics / Q000502:physiology; D009447:Neuroblastoma / Q000145:classification / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D020917:Receptor, trkA / Q000235:genetics / Q000502:physiology; D020813:Receptor, trkB / Q000235:genetics / Q000502:physiology; D012075:Remission, Spontaneous; D012306:Risk
D009363:Neoplasm Proteins; D020917:Receptor, trkA; D020813:Receptor, trkB