Neuroblastoma is a malignant (cancerous) tumor that develops from nerve tissue. It usually occurs in infants and children.
Causes, incidence, and risk factors
Neuroblastoma can occur in many areas of the body. It develops from the tissues that form the sympathetic nervous system. This is the part of the nervous system that controls body functions, such as heart rate and blood pressure, digestion, and levels of certain hormones.
Most neuroblastomas begin in the abdomen in the adrenal gland or next to the spinal cord, or in the chest. Neuroblastomas can spread to the bones, such as in the face, skull, pelvis, shoulders, arms, and legs. It can also spread to bone marrow, liver, lymph nodes, skin, and around the eyes (orbits).
The cause of the tumor is unknown. Neuroblastoma is most commonly diagnosed in children before age 5. Each year there are around 700 new cases in the United States. The disorder occurs in approximately 1 out of 100,000 children and is slightly more common in boys.
In most patients, the neuroblastoma has already spread when it is first diagnosed.
The first symptoms are usually fever, a general sick feeling (malaise), and pain. There may also be loss of appetite, weight loss, and diarrhea.
Other symptoms depend on the site of the tumor, and may include:
Urine 24-hour test for catecholamines, homovanillic acid (HVA), and vanillymandelic acid (VMA)
Treatment varies depending on:
The location of the tumor
How much and where the tumor has spread
The patient's age
In certain cases, surgery alone is enough. Often, though, other therapies are needed. Anticancer medications (chemotherapy) may be recommended if the tumor has spread. Radiation therapy may also be used.
High-dose chemotherapy, followed by autologous stem cell transplantation, is being studied for use in children with very high-risk tumors.
The stress of illness can be eased by joining a cancer support group. Sharing with others who have common experiences and problems and help you and your child not feel alone.
The outcome varies. In very young children with neuroblastoma, the tumor may go away on its own, without treatment. Or, the tissues of the tumor may mature and develop into a non-cancerous (benign) tumor called a ganglioneuroma, which can be surgically removed. In other cases, the tumor spreads quickly.
Response to treatment also varies. Treatment is often successful if the cancer has not spread. If it has spread, neuroblastoma is harder to cure. Younger children often do better than older children.
Tumors with certain genetic characteristics may be harder to cure.
Children treated for neuroblastoma may be at risk of getting a second, different cancer in the future.
NationalCancer Institute: PDQ neuroblastoma treatment. Bethesda, MD: National CancerInstitute. Date last modified 11/16/2012. Available at: http://www.cancer.gov/cancertopics/pdq/treatment/neuroblastoma/HealthProfessional. Accessed November 17, 2012.
Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, David R. Eltz, Stephanie Slon, and Nissi Wang.