When Your Child Has Neuroblastoma

What is neuroblastoma?

Neuroblastoma is a rare type of childhood cancer. It often forms in the area of the nerves that control blood pressure and some hormones. Children of any age can get neuroblastoma. Children age 5 and younger are affected most often. Different kinds of treatment can help shrink the cancer and make your child feel better. Your child's healthcare team will work closely with you to make decisions about your child's health.

Neuroblastoma most often begins in the adrenal glands. These glands sit on top of the kidneys. It can also start in the nerve tissue in the abdomen, chest, or spinal cord. It can start in nerve tissue near the spine in the neck. When the cancer cells group together, they form a tumor. The tumor can spread (metastasize) to another part of the body. It may spread to the lungs, bone marrow, lymph nodes, or liver. It can also spread to the area behind the eyes. In infants, it can spread beneath the skin. Even though the cells can spread in the body, the disease is not contagious. It is not spread from person to person.

What causes neuroblastoma?

Neuroblastoma occurs when certain cells grow abnormally (mutate). The cause of this is not fully known. Changes in certain genes may affect the way your child’s cells grow. These gene changes are random. There is no known way to prevent them. Most often, the cause of cancer in children is unknown. In a small number of cases, the child has a family history of the tumor.

What are the symptoms of neuroblastoma?

These are some common symptoms of neuroblastoma:

• Dark circles around the eyes

• Bone pain

• Bulging eyes

• Swelling, a lump, or pain in the abdomen

In babies, these are also common symptoms:

• Trouble breathing

• Painless, blue lumps under the skin

Less common symptoms include:

• Fever

• Trouble breathing

• Easy bleeding or bruising

• High blood pressure

• Severe, watery diarrhea

• Uncontrolled eye movements and muscle twitching (opsoclonus myoclonus), also known as “dancing eyes–dancing feet”

How is neuroblastoma diagnosed?

Your child’s healthcare provider will give your child a physical exam. Your child will also have a neurological exam. You will be asked about your child’s health history. Your child may also have tests such as:

• Blood or urine tests. These are done to look for certain chemicals in the blood and urine.

• Imaging tests. These are done to take detailed pictures of areas inside the body. These may include X-ray, MRI, CT scan, MIBG scan, or ultrasound.

• Biopsy. This is done to take tiny pieces of tissue from a tumor. The tissue samples are then looked at with a microscope and tested for cancer.

• Bone marrow aspiration and biopsy. This is done to take samples of bone marrow from the hipbones. The samples are then looked at with a microscope and checked for cancer cells.

Staging and grading of neuroblastoma

Staging is the process to note the size of the cancer and how much it has spread. The stage is important in making treatment decisions and predicting outcomes.

Stage

There are 2 systems used to stage neuroblastomas. The International Neuroblastoma Staging System (INSS) and the International Neuroblastoma Risk Group Staging System (INRGSS).

The INSS system divides tumors into stages 1 through 4. It's assigned after surgery has been done to remove the tumor.

Stage 1 is early cancer. It's only in the place where it started and only on one side of the body. For neuroblastoma, this means the tumor can be fully seen and removed by surgery. Stages 2 and 3 are more complex. The tumor is harder to remove and cancer cells may have spread. Stage 4 means cancer cells from the tumor have spread to other parts of the body far from the primary tumor.

The INRGSS system divides tumors into 4 stages labeled L1, L2, M, and MS. Surgery does not need to be done before assigning one of these stages. They are based on how the tumor looks based on scans (called image-defined risk factors or IDRFs) and biopsies. It is used to predict how much of the tumor can be taken out with surgery. The "L" groups mean the cancer is confined to one part of the body. The "M" group means it has spread beyond where it first started to distant organs. MS is used in children younger than 18 months with cancer that has spread only to the skin, liver, or less than 10% of the bone marrow.

Grade

Grading is used to describe how abnormal the cancer cells look when seen through a microscope. The more abnormal the cells are, the faster they grow. Staging and grading help the healthcare team plan treatment for your child.

Other factors considered include:

• Location of the main tumor

• Genetic changes in the tumor cells

• The age of your child

Your child’s healthcare team will tell you more about the stage and grade of your child’s cancer. Be sure to ask the healthcare provider if you have any questions.

How is neuroblastoma treated?

Your child will be treated by several types of healthcare providers who specialize in caring for children with cancer. You child may see a:

• Pediatric surgeon

• Pediatric cancer specialist (oncologist/hematologist)

• Endocrinologist

• Neurologist

• Neuroradiologist

• Pediatrician

• Pediatric nurse specialist

• Social worker, counselor, or psychologist

The goal of treatment is to remove or destroy cancer cells. The kind of treatment your child gets depends on the type and stage of tumor your child has. Infants younger than 6 months  old may not need treatment. Sometimes the tumors go away on their own. But if it is needed, your child may need one or more of these:

• Surgery. This is done to remove all or part of a tumor.

• Watchful waiting. Your child may be closely watched and not treated until his or her signs and symptoms change.

• Chemotherapy (“chemo”). This is the use of medicine to destroy cancer cells. More than one chemo medicines may be used. Chemo is given through a tube (IV) that’s put into a vein in the arm or chest. Or, it may be given by mouth or an injection.

• Radiation therapy. This uses high-energy X-rays to destroy cancer cells and shrink a tumor.

Supportive treatments

While being treated for cancer, your child will also have supportive treatments. The goal of these is to protect your child from infection, prevent discomfort, and bring the body’s blood counts to a healthy range. Your child may be given:

• Antibiotics. These help to prevent and fight infection.

• Anti-nausea and other medicines. These help ease side effects caused by treatment.

• A blood transfusion. This is done to restore the blood cells destroyed by treatment.

Long-term care

Treatment for neuroblastoma can be hard on the body. Your child may need physical therapy. This is to get the body working normally after treatment. Also, chemotherapy and radiation may cause some problems. These may include damage to certain organs. Your child’s health will need to be watched for life. This may include clinic visits, blood tests, imaging tests, and ultrasounds of the heart.

Getting support

It can be scary and confusing when your child is diagnosed with cancer. Remember that you are not alone. Your child’s healthcare team will work with you during your child’s illness and care. You may also want to seek out other kinds of support. This can help you cope with the changes that cancer can bring. Contact these organizations for information and resources:

• Children’s Neuroblastoma Cancer Foundation, www.nbhope.org

• Children’s Oncology Group, www.childrensoncologygroup.org