Neuroblastoma is a cancer that develops from nerve cells found in several areas of the body. Neuroblastoma most commonly affects children age 5 or younger, though it may rarely occur in older children and adults. Neuroblastoma is the most common cancer in babies.

Neuroblastoma develops in tissue that makes up the sympathetic nervous system — the system of nerves that automatically regulates your heart rate, blood pressure and digestion. Neuroblastoma most commonly arises in and around the adrenal glands, which sit atop the kidneys. However, neuroblastoma can also develop in other areas of the abdomen and in the chest, neck and pelvis.

About 650 people are diagnosed with neuroblastoma each year in the United States, according to the American Cancer Society. For those diagnosed at an early stage, the chance for a cure is good. For older children and those with advanced neuroblastoma, outcomes generally aren't good. However, research into new treatments continues and may one day improve survival rates for children with neuroblastoma.

Signs and symptoms of neuroblastoma vary depending on what part of the body is affected. About two-thirds of all neuroblastomas occur in the abdomen, usually in or near the adrenal glands. About 15 percent of neuroblastomas arise in the chest. Less frequently, neuroblastoma occurs in the neck or pelvis. Occasionally the site of origin of a neuroblastoma can't be determined.

Neuroblastoma in the abdomen may cause signs and symptoms such as:

• Abdominal pain
• A feeling of fullness in the abdomen
• A mass under the skin that isn't tender when touched
• Changes in bowel habits, such as constipation
• Changes in urinary habits, such as having to go to the bathroom more often
• Swelling in the legs

Neuroblastoma in the chest may cause signs and symptoms such as:

• Wheezing
• Swelling in the face
• Changes to the eyes, including drooping eyelids and unequal pupil size

Other signs and symptoms that may indicate neuroblastoma include:

• Lumps of tissue under the skin
• Eyeballs that seem to protrude from the sockets (proptosis)
• Back pain
• Curvature of the spine (scoliosis)
• Fever
• Unexplained weight loss
• Bone pain

In general, cancer begins with a genetic mutation that allows normal, healthy cells to continue growing without responding to the signals to stop, which normal cells do. Cancer cells grow and multiply out of control. The accumulating abnormal cells form a mass (tumor). Cancer cells may invade nearby tissues and can break off from an initial tumor to spread elsewhere in the body (metastasize).

Neuroblastoma begins in neuroblasts — immature nerve cells that a fetus makes as part of its development process in the womb. As the fetus matures, neuroblasts eventually turn into nerve cells and fibers and the cells that make up the adrenal glands. The majority of neuroblasts mature by birth, though studies have found a small number of immature neuroblasts in newborns. In most cases, these neuroblasts will mature or disappear. Others, however, form a tumor — a neuroblastoma.

It isn't clear what causes the initial genetic mutation that leads to neuroblastoma. However, because neuroblastoma usually affects very young children, researchers believe the mutation occurs during pregnancy, or possibly even before conception.

The only factor known to increase the risk of neuroblastoma is a family history of the cancer. However, only 1 percent to 2 percent of all neuroblastomas are believed to be related to family history. Babies with inherited neuroblastoma usually develop the disease around 9 months — earlier than do children with no family history, who are diagnosed at 17 months on average.

Contact your child's doctor if your child has any signs or symptoms that worry you. Mention any changes in your child's behavior or habits.

Your child's doctor conducts a physical exam to check out any signs and symptoms. He or she asks you questions about your child's habits and behaviors. Your child's doctor may also order blood and urine tests to look for clues that may indicate the cause of any signs and symptoms. Urine tests may be used to check for high levels of certain chemicals that result from the neuroblastoma cells being unable to process catecholamines.

In rare cases, neuroblastoma may be detected before a baby is born. Fetal ultrasounds may detect neuroblastoma after 32 weeks of development and sometimes earlier. Analysis of the mother's urine can give other clues that may lead doctors to suspect neuroblastoma.

Biopsy and bone marrow aspiration
Your child's doctor may order a biopsy of the mass to confirm the diagnosis. An incisional biopsy is a surgical procedure to remove a sample of tissue from the tumor to test for cancer. Doctors examine the sample under a microscope and may use other tests on the tissue to spot specific abnormalities. If the cancer is small and confined to one area, the surgeon may remove the entire tumor and send it to a laboratory for examination (excisional biopsy).

Your child may also undergo bone marrow biopsy and bone marrow aspiration procedures to see if neuroblastoma has spread to the bone marrow — the spongy material inside the largest bones where blood cells are formed. In order to remove bone marrow for testing, the surgeon inserts a needle into your child's hipbones or lower back and draws out the marrow.

Staging
Once neuroblastoma is diagnosed, your child's doctor may order further testing to determine the extent of the cancer and whether it has spread to distant organs — a process called staging. Tests and procedures to determine the stage of your child's neuroblastoma include:

• X-rays
• Bone scans
• Computerized tomography (CT) scans
• Magnetic resonance imaging (MRI) scans

Using the information from those procedures, your child's doctor assigns a stage to the neuroblastoma. Stages of neuroblastoma include:

• I. Neuroblastoma at this stage is localized, meaning it's confined to one area, and may be totally removed with surgery. Lymph nodes connected to the tumor may have signs of cancer, but other lymph nodes don't have cancer.
• IIA. Neuroblastoma at this stage is localized, but may not be as easily removed through surgery. Lymph nodes connected to the tumor may have signs of cancer, but other lymph nodes don't have cancer.
• IIB. Neuroblastoma at this stage is localized and may or may not be easily removed through surgery. Both the lymph nodes connected to the tumor and the lymph nodes nearby contain cancer cells.
• III. Neuroblastoma at this stage is considered advanced, and it isn't possible to remove the tumor through surgery. The tumor may be a larger size at this stage. Lymph nodes may or may not contain cancer cells.
• IV. Neuroblastoma at this stage is considered advanced and has spread (metastasized) to other parts of the body.
• IVS. This stage is a special category of neuroblastoma that doesn't behave like other forms of neuroblastoma, though it isn't clear why. Stage IVS neuroblastoma applies only to children younger than 1 year old. Stage IVS indicates that neuroblastoma has spread to another part of the body — most commonly the skin, liver or bone marrow. Despite the extent of neuroblastoma, babies with this stage have a good chance of recovery. Neuroblastoma at this stage sometimes goes away on its own and often doesn't require treatment.

Complications of neuroblastoma may include:

• Spread of the cancer (metastasis). Neuroblastoma that advances may spread (metastasize) to other parts of the body. Common places for neuroblastoma to spread include the lymph nodes, bone marrow, eyes, liver, skin and the sac that surrounds the spinal cord (dura).
• Spinal cord compression. Tumors may grow and press on the spinal cord, causing spinal cord compression. Spinal cord compression may cause pain and paralysis. Treating the cancer may resolve these signs and symptoms.
• Signs and symptoms caused by tumor secretions. Neuroblastomas may secrete certain chemicals that irritate certain other normal tissues, causing signs and symptoms called paraneoplastic syndromes. One paraneoplastic syndrome that occurs rarely in people with neuroblastoma causes rapid eye movements and difficulty with coordination. Another rare syndrome causes abdominal swelling and diarrhea.

• Long-term and late side effects of cancer treatment. As more and more children are surviving cancer and living into adulthood, doctors are increasingly aware of the long-term side effects of cancer treatment. Doctors recommend childhood cancer survivors be seen every year by a doctor who understands the long-term and late side effects that can affect children after cancer treatment.

  Side effects vary depending on treatment, but may include impaired growth, thyroid problems, second cancers and infertility, which may be caused by chemotherapy drugs. Difficulty thinking and trouble processing thoughts may be long-term side effects of radiation, especially brain radiation or total body irradiation (TBI).

Your child's doctor selects a treatment plan based on several factors that affect your child's prognosis. Factors include your child's age, the stage of the cancer, the type of cells involved in the cancer, and whether there are any abnormalities in the chromosomes and genes. Using this information, your child's doctor categorizes the cancer as low risk, intermediate risk or high risk.

What treatment your child receives for neuroblastoma depends on the risk category. Treatment may include surgery, chemotherapy, radiation, bone marrow stem cell transplant or a combination of these treatments.

Surgery
Surgeons use scalpels and other surgical tools to remove cancer cells. In children with low-risk neuroblastoma, surgery to remove the tumor may be the only treatment needed. Whether the tumor can be completely removed depends on its location and its size. Tumors that are attached to nearby vital organs — such as the lungs or the spinal cord — may be too risky to remove. In intermediate-risk and high-risk neuroblastoma, surgeons may try to remove as much of the tumor as possible. Other treatments, such as chemotherapy and radiation, may then be used to kill remaining cancer cells.

Chemotherapy
Chemotherapy uses chemicals to kill cancer cells. Chemotherapy targets rapidly growing cells in the body, including cancer cells. Unfortunately, chemotherapy also damages healthy cells that grow quickly, such as cells in the hair follicles and in the gastrointestinal system, which can cause side effects. Children with low-risk neuroblastoma that can't be removed surgically may receive low doses of chemotherapy. Sometimes chemotherapy is administered before surgery (neoadjuvant chemotherapy) to shrink the tumor to a size that's more easily removed. In other cases, chemotherapy may be the only treatment.

Children with intermediate-risk neuroblastoma often receive a combination of moderate-intensity chemotherapy drugs. Chemotherapy is often given before surgery to improve the chances that the entire tumor can be removed.

Children with high-risk neuroblastoma usually receive high doses of chemotherapy drugs to shrink the tumor and to kill any cancer cells that have spread elsewhere in the body. Chemotherapy is usually used before surgery and before bone marrow stem cell transplant.

Radiation therapy
Radiation therapy uses high doses of energy particles to destroy cancer cells. Radiation therapy primarily affects the area where it's aimed. Your child's radiation therapy team tries to protect the healthy cells near the cancer, but some healthy cells may be damaged by the radiation. What side effects your child experiences depends on where the radiation is directed.

Children with low-risk or intermediate-risk neuroblastoma may receive radiation therapy if surgery and chemotherapy haven't been helpful. Children with high-risk neuroblastoma may receive radiation therapy after chemotherapy and surgery to prevent cancer from recurring.

Stem cell transplant
Children with high-risk neuroblastoma may receive a transplant using their own blood stem cells (autologous stem cell transplant). The bone marrow makes stem cells, which mature and develop into the red and white cells and platelets that make up the blood. Your child undergoes a procedure that filters and collects stem cells from his or her blood. Then high doses of chemotherapy are used to kill any remaining cancer cells in your child's body. Your child's stem cells are then injected into his or her body, where they can form new, healthy blood cells.

When your child is diagnosed with cancer, it's common to feel a range of emotions — from shock and disbelief to guilt and anger. In the midst of this emotional roller coaster, you're expected to make decisions about your child's treatment. And while you're trying to sort it all out, friends and family may be calling for updates and to find out if they can help. It's overwhelming, to say the least. Everyone finds his or her own way of coping with stressful situations, but if you're feeling lost, you might try to:

• Gather all the information you can. Learn all you can about neuroblastoma. Talk with your health care team. Keep a list of questions to ask at your child's next appointment. Visit your local library and ask a librarian for help searching for information. Consult the National Cancer Institute (NCI) and the American Cancer Society (ACS) for more information. Both organizations can provide you with information through their Web sites or toll-free telephone information lines. Call the NCI at 800-4-CANCER, or 800-422-6237. Call the ACS at 800-ACS-2345, or 800-227-2345. Knowing more about your child's condition may make you more confident when choosing treatment options.
• Organize a support network. In the coming weeks and months, your child will undergo a number of tests and procedures that will be time-consuming and may require traveling to a specialized medical center. If you want to be with your child through this time, you may have to take time off from work. Some people will be able to organize this with their employers. For others, time off from work and time without income won't be possible. Find friends and family you can count on in this situation. Loved ones can accompany your child to doctor visits or sit by his or her bedside in the hospital when you can't be there. If you can take time away from work to be with your child, your friends and family can help out by spending time with your other children or helping around your house. Don't feel guilty about asking for help. Your friends and family feel helpless, too, and they want to help.
• Take advantage of resources for kids with cancer. Seek out special resources for families of kids with cancer. Social workers and other members of your health care team can tell you what programs are available. Support groups for parents and siblings put you in touch with people who understand what you're feeling. Your family may be eligible for summer camps, temporary housing and other support.
• Maintain normalcy as much as possible. Small children can't understand what is happening to them as they undergo treatment for their cancer. To help your child cope, try to maintain your normal routine as much as possible. Try to arrange appointments so that your child can have a set nap time each day. Have routine meal times. Allow time for play when your child feels up to it. If your child must spend time in the hospital, bring items from home that help him or her feel more comfortable. Ask your health care team about other ways to comfort your child through his or her treatment. Some hospitals have recreation therapists or child-life workers who can give you more specific ways to help your child to cope with all that's happening.