neuroblastoma

Neuroblastoma is a malignant (cancerous) tumor that develops from nerve tissue. It occurs in infants and children.

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.

• 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 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

Treatment varies depending on the location of the tumor, the extent of tumor spread and the age of the patient. In certain cases, surgery alone is enough, but often other therapies are needed. Anticancer medications (chemotherapy) may be recommended if the tumor is widespread. Radiation therapy may also be used.

The expected outcome varies. In very young children with neuroblastoma, the tumor may go away on its own, without any treatment, or the tissues of the tumor may mature and develop into a benign ganglioneuroma that can be surgically removed. In other cases, the tumor spreads rapidly.

Response to treatment is variable. Treatment is often successful if the cancer has not spread, but if there has been spread to other areas, neuroblastoma is much harder to cure.

• Spread (metastasis) of the tumor
• Damage and loss of function of involved organ(s)
  • Kidney failure
  • Liver failure
  • Loss of blood cells produced by the bone marrow
  • Decreased resistance to infection
  • Other organ system failures

Call your health care provider if symptoms indicate neuroblastoma may be present. Early diagnosis and treatment improves the chance of a good outcome.