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Neuroblastoma

Definition

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

Causes

Neuroblastoma can occur in many areas of the body. It develops from the tissues that form the sympathetic nervous system. This is the part of the nervous system that controls body functions, such as heart rate and blood pressure, digestion, and levels of certain hormones.

Most neuroblastomas begin in the abdomen in the adrenal gland or next to the spinal cord, or in the chest. Neuroblastomas can spread to the bones. These include the face, skull, pelvis, shoulders, arms, and legs. It can also spread to the bone marrow, liver, lymph nodes, skin, and around the eyes (orbits).

The cause of the tumor is not known. Neuroblastoma is commonly diagnosed in children before age 5. Each year there are around 700 new cases in the United States. The disorder occurs in approximately 1 out of 100,000 children and is slightly more common in boys.

In most patients, the tumor has spread when it is first diagnosed.

Symptoms

The first symptoms are usually fever, a general sick feeling (malaise), and pain. There may also be loss of appetite, weight loss, and diarrhea.

Other symptoms depend on the site of the tumor, and may include:

  • Bone pain or tenderness (if the cancer has spread to the bones)
  • Difficulty breathing or a chronic cough (if the cancer has spread to the chest)
  • Enlarged abdomen (from a large tumor or excess fluid)
  • Flushed, red skin
  • Pale skin and bluish color around the eyes
  • Profuse sweating
  • Rapid heart rate (tachycardia)

Brain and nervous system problems may include:

  • Inability to empty the bladder
  • Loss of movement (paralysis) of the hips, legs, or feet (lower extremities)
  • Problems with balance
  • Uncontrolled eye movements or leg and feet movements (called opsoclonus-myoconus syndrome, or "dancing eyes and dancing feet")

Exams and Tests

Signs vary, depending on the location of the tumor.

  • Examination of the abdomen with the hands (palpation) may reveal a lump or 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.
  • Lymph nodes may be swollen.

X-ray or other imaging tests are done to locate the main (primary) tumor and to see where it has spread. These include:

Other tests include:

Treatment

Treatment depends on:

  • Location of the tumor
  • How much and where the tumor has spread
  • The patient's age

In certain cases, surgery alone is enough. Often, though, other therapies are needed. Anticancer medicines (chemotherapy) may be recommended if the tumor has spread. Radiation therapy may also be used.

High-dose chemotherapy, followed by autologous stem cell transplantation, is being studied for use in children with very high-risk tumors.

Support Groups

You can ease the stress of illness by joining a cancer support group. Sharing with others who have common experiences and problems can help you and your child not feel alone.

Outlook (Prognosis)

Outcome varies. In very young children, the tumor may go away on its own, without treatment. Or, the tissues of the tumor may mature and develop into a non-cancerous (benign) tumor called a ganglioneuroma, which can be surgically removed. In other cases, the tumor spreads quickly.

Response to treatment also varies. Treatment is often successful if the cancer has not spread. If it has spread, neuroblastoma is harder to cure. Younger children often do better than older children.

Children treated for neuroblastoma may be at risk of getting a second, different cancer in the future.

Possible Complications

  • Spread (metastasis) of the tumor
  • Damage and loss of function of involved organ(s)

When to Contact a Medical Professional

Call your health care provider if your child has symptoms of neuroblastoma. Early diagnosis and treatment improves the chance of a good outcome.

References

Dome JS, Rodriguez-Galindo C, Spunt SL, Santana VM. Pediatric solid tumors. In: Niederhuber JE, Armitage JO, Doroshow JH, et al., eds. Abeloff's Clinical Oncology. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2013:chap 95.

National Cancer Institute: PDQ neuroblastoma treatment. Bethesda, Md: National CancerInstitute. Date last modified 9/6/2013. Available at: http://www.cancer.gov/cancertopics/pdq/treatment/neuroblastoma/HealthProfessional. Accessed November 8, 2013.


Review Date: 10/30/2013
Reviewed By: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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