Neuroblastoma

Alternate 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.

The condition 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.

Prevention: Prevention is unknown. Awareness of risk (such as a family history) may allow early diagnosis and treatment.

Symptoms: Symptoms vary depending on the site of the tumor.

  • 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
  • abdomen, swollen
  • bone pain or tenderness (if spread to the bones)
  • difficulty breathing (if spread to the chest)
  • general discomfort or uneasiness (malaise) lasting for weeks or months
  • flushed, red skin
  • profuse sweating
  • rapid pulse (tachycardia)
  • eye movements, uncontrollable
  • loss of movement (paralysis) of the hips, legs, or feet (lower extremities)
  • movement, uncoordinated
  • 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 show a mass (aggregation of cells). The liver may be enlarged if the tumor has spread to the liver. Adrenal gland tumor often causes high blood pressure and a fast heart rate. Testing reveals the original tumor and locations of tumor spread:

  • CT scan
  • MRI scan
  • chest X-ray
  • bone scan
  • bone marrow biopsy
  • hormone tests (levels of sympathetic hormones such as epinephrine)
  • CBC showing anemia or other abnormality
  • catecholamines - urine
  • catecholamines - blood

Treatment: Treatment varies depending on the location of the tumor and extent of tumor spread. Surgery is usually indicated. This includes removal of all or part of the tumor and surrounding lymph nodes.

Radiation therapy may also be advised. Anticancer medications (chemotherapy) may be recommended if the tumor is widespread. A bone marrow transplant may be advised if the tumor has spread to the bone marrow.

Expectations (prognosis): The expected outcome varies. Some children with neuroblastoma experience spontaneous regression of the tumor, where the tissues of the tumor mature and develop into a benign ganglioneuroma that can be surgically removed. In other cases, the tumor spreads rapidly.

Response to treatment is variable; response is often quite successful if treatment begins before the tumor has spread. If cancer is widespread, survival is often less than 10%, although bone marrow transplant increases the chance of survival to 25%.

Complications:

  • 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 losses

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