Neuroblastoma

Definition
Neuroblastoma is a malignant (cancerous) tumor of infants and children that develops from nerve tissue.

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)       o Abdominal mass       o 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

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

Signs and tests
Signs vary depending on the location of the tumor. Examination of the abdomen with the hands (palpation) may show 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 like epinephrine)  
  • CBC showing anemia or other abnormality.  
  • catecholamines - urine  
  • catecholamines - blood  
  • MIBG scan

Treatment

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.

Support Groups
The stress of illness can often be helped by joining a support group where members share common experiences and problems.

Expectations (prognosis)
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.

Complications

     
  • spread (metastasis) of the tumor  
  • damage and loss of function of involved organ(s)       o Kidney failure       o liver failure       o loss of blood cells produced by the bone marrow       o decreased resistance to infection       o other organ system losses

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

Johns Hopkins patient information

Last revised: December 3, 2012
by Gevorg A. Poghosian, Ph.D.

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