What Is It?

Of all the childhood cancers, neuroblastoma is the most common tumor to develop outside the brain. It accounts for 8 percent to 10 percent of childhood cancers. The average age at diagnosis is 2 years old.

Neuroblastomas form in the nerve cells that are responsible for the body’s ability to react to a life-threatening emergency, called the “fight-or-flight” response. This nerve network is called 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 sympathetic nerves that lay 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 less commonly develop in other areas of the body, including the chest, neck and pelvis.

As a neuroblastoma grows, it has the potential to spread (metastasize) to areas away from where the cancer began, 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 of a neuroblastoma can include:

• A firm mass in the abdomen, with or without abdominal pain or discomfort
• Difficulty breathing when the abdominal mass presses upwards on the infant’s chest and lungs
• Weight loss or 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 watery 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.

Your doctor will review your child’s symptoms and examine him or her. He or she 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 diagnostic 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. This may include a biopsy of the tumor, in which a small piece of the tumor is removed and examined in a laboratory. A sample of bone marrow also may be removed to be examined.

In most cases, a neuroblastoma will continue to grow until it is treated. Without treatment, this cancer eventually can spread to the bone marrow, bones, liver, skin and other parts of the body. There are some types of neuroblastoma, usually in children less than 1 year old, that do not require treatment and that decrease in size over time.

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.

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 by 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, it’s possible no further treatment will be needed. If the tumor is localized but cannot be removed completely, the child will need chemotherapy. After chemotherapy, surgery may be done a second time to remove any remaining tumor. This may be followed by radiation therapy.



• Regional neuroblastoma — A regional neuroblastoma is one that has spread to local lymph nodes or to nearby tissues or organs but has not metastasized to area further away. In infants, regional neuroblastoma is treated with a combination of surgery and chemotherapy. In older children, it is treated with surgery and chemotherapy, with or without radiation.



• 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 neuroblastoma tumor itself 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. In some cases, the tumors even go away on their own. Treatment is controversial, with some doctors giving only mild chemotherapy and other doctors choosing a watch-and-wait approach.

Call your doctor if your child shows symptoms of neuroblastoma, especially if your child has an abnormal fullness or swelling in the abdomen, with or without discomfort.

With treatment, up to 90 percent of children with localized neuroblastoma or special neuroblastoma can be cured. An oncologist can use special genetic tests to better estimate the prognosis for an individual. 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.

Diseases and Conditions Center

All ArmMed Media material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.