NEUROBLASTOMA

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Definition

Neuroblastoma is a malignant (cancerous) tumor that develops from nerve tissue. It 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 (the part of the nervous system that controls body functions, such as heart rate and blood pressure, digestion, and levels of certain hormones).

Neuroblastoma most commonly begins in the abdomen in the tissues of the adrenal gland, but it may also occur in other areas. It can spread to the lymph nodes, liver, bones, and bone marrow.

The cause of the tumor is unknown. Neuroblastoma is most commonly diagnosed in children before age 5. The disorder occurs in approximately 1 out of 100,000 children and is slightly more common in boys.

Symptoms

• Pale skin
• Dark circles around the eyes
• Chronic fatigue, excessive tiredness lasting for weeks to months
• Diarrhea
• Enlarged abdomen (particularly if spread to the liver)
  • Abdominal mass
  • Swollen abdomen
• Bone pain or tenderness (if the cancer spreads to the bones)
• Difficulty breathing (if the cancer spreads to the chest)
• General discomfort or uneasiness (malaise) lasting for weeks or months
• Flushed, red skin
• Profuse sweating
• Rapid pulse (tachycardia)
• Uncontrolled eye movements
• Loss of movement (paralysis) of the hips, legs, or feet (lower extremities)
• Uncoordinated movement
• Irritability or poor temper control

Signs and tests

Signs vary depending on the location of the tumor. Examination of the abdomen with the hands (palpation) may find a mass. The liver may be enlarged, if the tumor has spread to the liver. Adrenal gland tumors can cause high blood pressure and a fast heart rate.

Testing reveals the original (primary) tumor and locations of tumor spread:

• CT scan
• MRI scan
• Chest x-ray
• Bone scan
• Bone marrow biopsy
• Hormone tests (levels of hormones such as epinephrine)
• CBC showing anemia or other abnormality
• Catecholamines - urine
• Catecholamines - blood
• MIBG scan

Reviewer Info: James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc., 06/10/2008