Neuroblastoma

Alternative names:

pepper's syndrome

Definition:

A malignant (cancerous) tumor that develops from embryonic (fetal) neural tissue, with the tumor appearing in infancy or childhood.

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 (the part of the nervous system that regulates involuntary body functions by increasing heart rate and blood pressure, constricting blood vessels, and stimulating certain hormones). Neuroblastoma most commonly begins in the abdomen in the tissues of the adrenal gland but may occur in other areas. It usually spreads rapidly to the lymph nodes, liver, lungs, bones, and bone marrow.

The cause of the tumor is unknown. It may be linked to hereditary tendencies, and typical gene abnormalities are seen in persons with the tumor. Neuroblastoma is most commonly diagnosed in children before age 5. The disorder occurs in approximately 1 out of 100,000 people. It is slightly more common in boys.

Pepper's syndrome is a neuroblastoma that originates in the adrenal gland and usually metastasizes to the liver.