Neuroblastoma

What Is It?

Neuroblastoma is the most common childhood cancer to develop outside the brain. It accounts for about 8% childhood cancers. The average age at diagnosis is 2 years old.

Neuroblastomas form in the nerve cells responsible for the body's "fight-or-flight" response, which is the ability to react to a life-threatening emergency. These nerve cells form the sympathetic nervous system.

Neuroblastomas usually form in one of two parts of the sympathetic nervous system:

• The adrenal glands -- These glands in the abdomen produce the hormone adrenaline, which is released in an emergency to help the body react. When a neuroblastoma develops in the adrenal gland, it typically grows into a large, firm abdominal mass that can compress nearby organs.
• The chain of sympathetic ganglia -- These are collections of nerves near the backbone (spine). When a neuroblastoma develops along the sympathetic chain, it can invade nearby areas of the spine and cause injury to spinal nerves.

Neuroblastomas can develop in other areas of the body, including the chest, neck and pelvis, but this is less common.

As a neuroblastoma grows, it has the potential to spread (metastasize) to other areas, most commonly to the bone marrow, bones, liver and skin.

There is no conclusive evidence that neuroblastoma is related to any toxic chemical or environmental risk factor. Genetic (inherited) factors seem to influence the development of some neuroblastomas.

Symptoms

Symptoms of a neuroblastoma can include:

• A firm mass in the abdomen, with or without abdominal pain or discomfort
• Difficulty breathing (due to the abdominal mass pressing on the infant's chest and lungs)
• Weight loss or failure to gain weight (called failure to thrive)
• Anemia (a low number of red blood cells)
• Fever and irritability
• Bone pain
• Neurological symptoms if the tumor damages nearby nerves, including paralysis, difficulty swallowing, difficulty walking, uncontrolled eye movements, a drooping eyelid, or jerky or uncontrolled limb movements
• Protruding eyes or dark circles around the eyes ("panda eyes")
• Symptoms caused by chemicals produced by the tumor, including high blood pressure, rapid pulse, flushing, sweating or diarrhea

In some cases, a child's neuroblastoma is diagnosed by chance before it causes symptoms. The tumor is discovered in the chest or abdomen when the child has an X-ray to check for some unrelated illness.

Diagnosis

Your doctor will review your child's symptoms and examine the child. The doctor will order blood and urine tests and X-rays. A computed tomography (CT) scan or magnetic resonance imaging (MRI) often is needed to provide further detail.

If any of these tests show signs of a cancerous (malignant) tumor, your doctor will refer you to a medical center that has the facilities, personnel and experience to treat childhood cancer. There, your child will have further tests to confirm the diagnosis of neuroblastoma. These may include a biopsy, in which a small piece of the tumor is removed and examined in a laboratory. A sample of bone marrow also may be taken.

Expected Duration

In most cases, a neuroblastoma will continue to grow until it is treated. Without treatment, the cancer can spread to the bone marrow, bones, liver, skin and other parts of the body. A small number of neuroblastomas, usually in children less than 1 year old, decrease in size over time and do not need aggressive treatment.

Prevention

There is no way to prevent neuroblastoma. However, because genetic factors seem to play some role in the development of this tumor, people with a strong family history of cancer -- especially childhood cancer -- might want to ask their doctors about the need for genetic testing before starting a family.

Treatment

How a neuroblastoma is treated depends on how much it has spread. This is called the tumor stage. Tumor stage is determined by how much of the tumor can be removed surgically and whether the cancer has spread to nearby lymph nodes or to distant organs. Here are the stages of neuroblastoma:

• Localized neuroblastoma -- This means the tumor has not spread. If the tumor is localized and can be removed completely with surgery, no additional treatment may be needed. If the tumor is localized but cannot be removed completely, the child will need chemotherapy. After chemotherapy, the child may need a second surgery to remove any remaining tumor. This may be followed by radiation therapy.
• Regional neuroblastoma -- A regional neuroblastoma has spread to local lymph nodes or to nearby tissues or organs but has not spread to areas further away. A regional neuroblastoma is treated with a combination of surgery and chemotherapy. Older children also may receive radiation therapy.
• Disseminated neuroblastoma -- A disseminated neuroblastoma has spread to distant organs or tissues. This is the most common stage at which children are diagnosed. This advanced stage can be treated with intensive chemotherapy, with or without surgery or radiation.
• Special neuroblastoma, also called stage IVS neuroblastoma -- In this stage, the tumor is fairly localized, but some cancer has spread to the liver, skin or bone marrow. This stage usually appears in infants younger than 12 months. In most cases, children with tumors of this stage do very well. Treatment is controversial, however, with some doctors giving only mild chemotherapy and other doctors choosing a watch-and-wait approach.

When To Call A Professional

Call your doctor if your child shows symptoms of neuroblastoma, especially if your child has an abnormal fullness or swelling in the abdomen.

Prognosis

With treatment, up to 90% of children with localized neuroblastoma or special neuroblastoma can be cured. A genetic test is available to better estimate a child's prognosis. Neuroblastoma that has spread to the surrounding area or to other areas has a worse outlook. However, most children respond to treatment of this cancer even in advanced stages. Children with special neuroblastoma may do extremely well without any treatment, but they continue to need close observation by a specialist.

Additional Info

National Cancer Institute (NCI)

U.S. National Institutes of Health

Public Inquiries Office

Building 31, Room 10A03

31 Center Drive, MSC 8322

Bethesda, MD 20892-2580

Phone: 301-435-3848

Toll-Free: 1-800-422-6237

TTY: 1-800-332-8615

Email: cancergovstaff@mail.nih.gov

http://www.nci.nih.gov/

American Cancer Society (ACS)

1599 Clifton Road, NE

Atlanta, GA 30329-4251

Toll-Free: 1-800-227-2345

http://www.cancer.org/

American Academy of Pediatrics (AAP)

141 Northwest Point Blvd.

Elk Grove Village, IL 60007-1098

Phone: 847-434-4000

Fax: 847-434-8000

Email: kidsdocs@aap.org

http://www.aap.org/

American Society of Clinical Oncology

1900 Duke St.

Suite 200

Alexandria, VA 22314

Phone: 703-299-0150

Fax: 703-299-1044

Email: asco@asco.org

http://www.asco.org/