Neuroblastoma

What Is Neuroblastoma?

Neuroblastoma is a type of cancer that starts in early nerve cells called neuroblasts. Normally, these immature cells grow into working nerve cells. But in neuroblastoma (nur-oh-bla-STOW-muh), they grow uncontrollably and become cancer cells that form a solid tumor. 

Often, neuroblastoma starts in the tissue of the adrenal glands. These triangular glands sit on top of the kidneys and make hormones that control heart rate, blood pressure, and other important body functions. Neuroblastoma also can start in other areas of the body with clusters of nerve cells, like in the belly, chest, or neck. The cancer can spread through the blood and start growing (metastasize) in other parts of the body, such as the lymph nodes, bones, lungs, and liver.

Almost all cases of neuroblastoma happen in infants and children younger than 5 years old. The success of treatment depends on many things, including the child’s age, how much disease there is, and characteristics of the tumor.

What Are the Signs & Symptoms of Neuroblastoma?

The signs of neuroblastoma can be different depending on where the disease began, how much the cancer has grown, and if has spread to other parts of the body.

The first symptoms are often vague and may include:

• irritability
• pain
• constipation or diarrhea
• a swollen belly
• being very tired
• loss of appetite, weight loss
• dark circles around the eyes
• weakness
• fever

Because these early signs can develop slowly and be similar to symptoms of other common childhood illnesses, neuroblastoma can be hard to diagnose.

If the growing tumor presses on nearby tissues or the cancer spreads to other areas, different symptoms can show up.

In young children, neuroblastoma often is discovered when a parent or doctor feels an unusual lump or mass somewhere in the child's body — most often in the belly, though tumors also can be in the neck, chest, and elsewhere.

What Causes Neuroblastoma?

Neuroblastoma happens when neuroblasts grow and divide out of control instead of developing into nerve cells. Experts believe that a defect in the genes of a neuroblast lets it divide like this. Rarely, the tendency to get this type of cancer can be passed from a parent to a child.

How Is Neuroblastoma Diagnosed?

If they suspect neuroblastoma, doctors will order tests to confirm the diagnosis and rule out other causes of symptoms. These tests may include:

• urine tests and blood tests
• imaging studies (such as X-rays, CT scans, MRIs, and ultrasounds)
• a biopsy (taking a small tissue sample to check in a lab)
• a  bone marrow aspiration and biopsy

If the test results show it is neuroblastoma, doctors classify the disease as low-risk, intermediate-risk, or high-risk. This is called staging. To do this, they consider the child’s age, the area affected by the cancer, and the results of tests done on the neuroblastoma cells. 

The doctor also will order an MIBG scan. In this test, a low-dose radioactive material is attached to a molecule (MIBG), then injected into the child. Neuroblastoma cells will absorb the MIBG. During the scan, doctors can see where the cancer cells are in the body. Doctors also can use this scan to see how well a child responds to treatment because it will show how much cancer is left.

MIBG given with higher-dose radioactive iodine can treat neuroblastoma. After it’s injected, it delivers the radiation specifically to the neuroblastoma cells and kill them.

How Is Neuroblastoma Treated?

How doctors treat neuroblastoma depends on its staging and whether the cancer has spread.

Doctors will closely watch a child with low-risk disease, and do tests often to make sure the tumor doesn’t get bigger. Sometimes, they’ll do surgery to remove the tumor.

A child with intermediate-risk disease will likely need chemotherapy and may also have surgery. Some may need radiation therapy.

A child with high-risk disease needs longer, more aggressive treatments, including surgery to remove the tumor, radiation therapy, chemotherapy, stem cell transplants, and immunotherapy. Studies are being done to see if MIBG treatment will improve a child’s chances of being cured.

How Can Parents Help?

Being told your child has neuroblastoma can be overwhelming, and cancer treatment can take a toll on any family.

Parents often struggle with how much to tell a child who's diagnosed with cancer. There's no perfect answer, but experts agree that it's best to be honest. Use terms your child can understand and consider their emotional maturity. Give as much information as your child needs, but not more. Make it clear that the disease is not due to anything your child did or didn’t do.

When explaining treatment, breaking things down into steps can make the big picture less scary. For example:

• We’ll visit different doctors.
• A special machine will take pictures of your body.
• You might need an operation, but we’ll be there when you wake up.

At times, you might feel helpless. But you play a big role in your child's treatment. Learn as much as you can about neuroblastoma and its treatment. This will help you work with the care team to make informed decisions and better help your child cope with the tests and treatments. Don't be afraid to ask the doctors questions.

Take care of yourself too. Parents who get the support they need are better able to support their children. And you don't have to go it alone. The doctors, nurses, social workers, and other members of the care team are there to help you and your child.

During and after treatment, it can help to find a support group for parents whose kids are coping with cancer. There are groups specifically for parents of children with neuroblastoma. Ask the care team for recommendations. You also can find support and more information online at:

• American Cancer Society
• The Children's Neuroblastoma Cancer Foundation