Neuroblastoma

neuroblastoma, a tumour of the sympathetic nervous system (the branch of the autonomic nervous system that is best known for producing the fight-or-flight response) that affects young children. It is the most-common pediatric solid tumour that occurs outside the brain, with an annual incidence of about 11 cases per one million children between ages 4 and 15 and 30 cases per one million children under age 4. Neuroblastoma often arises in the abdomen, usually within the adrenaline-producing adrenal gland, which is located immediately above the kidney. Other common sites of tumour formation include the chest and along the spinal column in the neck or pelvis. Disease may be extensive and metastatic (spreading to other areas of the body) at diagnosis, with cancerous cells typically found throughout the bones and in the bone marrow. Neuroblastoma is unique to pediatric tumours in its genetic and clinical heterogeneity; tumours of infants may spontaneously regress without any therapy, whereas tumours of older children are very difficult to cure. Much is known about the genetics and biology of neuroblastoma, and the development of therapies targeting the underlying biological mechanisms responsible for tumour growth is promising.