What is neuroblastoma?
Neuroblastoma is a malignant tumor derived from primitive neural crest cells. It belongs to a group of neuroblastic tumors, which include ganglioneuroblastoma (a malignant tumor comprised of mature ganglion cells and nerve fibers, regarded by
many to be a fully differentiated neuroblastoma) and ganglioneuroma (a benign tumor composed of Schwann and ganglion cells).
Neuroblastoma is a very common tumor of childhood, accounting for 7 to 8 percent of all malignancies. About one in 6,000 children will be diagnosed with neuroblastoma by the age of five. There are no gender-related differences in incidence and it is the most common malignant tumor of infancy. Fifty percent of the cases occur in children less than two years of age with 75 percent occurring in children less than four years of age. Neuroblastoma has a wide range of severity. Children with localized neuroblastoma can be cured by surgery alone but those who have disease that has spread to the bone (metastasized) usually have a fatal outcome.
Neuroblastoma can occur anywhere in the body but most often occurs in the adrenal glands, which are above the kidneys. The adrenal glands are specialised glands that release hormones to maintain blood pressure and enable us to respond to stress.
Neuroblastomas usually form in one of two parts of the sympathetic nervous system: the adrenal glands - these glands in the abdomen produce the hormone adrenaline, which is released in an emergency to help the body react. When a neuroblastoma develops in the adrenal gland, it typically grows into a large, firm abdominal mass that can compress nearby organs; the chain of sympathetic ganglia - these are collections of sympathetic nerves that lay near the backbone (spine). When a neuroblastoma develops along the sympathetic chain, it can invade nearby areas of the spine and cause injury to spinal nerves.