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Neuroblastoma

Medically reviewed by Drugs.com. Last updated on Nov 4, 2022.

Overview

Neuroblastoma is a cancer that develops from immature nerve cells found in several areas of the body.

Neuroblastoma most commonly arises in and around the adrenal glands, which have similar origins to nerve cells and sit atop the kidneys. However, neuroblastoma can also develop in other areas of the abdomen and in the chest, neck and near the spine, where groups of nerve cells exist.

Neuroblastoma most commonly affects children age 5 or younger, though it may rarely occur in older children.

Some forms of neuroblastoma go away on their own, while others may require multiple treatments. Your child's neuroblastoma treatment options will depend on several factors.

Adrenal glands

Located on top of each kidney, the adrenal glands make hormones that help regulate metabolism, the immune system, blood pressure and other important functions. Although small, these glands control much of what happens in the body.

Symptoms

Signs and symptoms of neuroblastoma vary depending on what part of the body is affected.

Neuroblastoma in the abdomen — the most common form — may cause signs and symptoms such as:

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

Other signs and symptoms that may indicate neuroblastoma include:

When to see a doctor

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.

Causes

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).

Neuroblastoma begins in neuroblasts — immature nerve cells that a fetus makes as part of its development process.

As the fetus matures, neuroblasts eventually turn into nerve cells and fibers and the cells that make up the adrenal glands. Most neuroblasts mature by birth, though a small number of immature neuroblasts can be found in newborns. In most cases, these neuroblasts mature or disappear. Others, however, form a tumor — a neuroblastoma.

It isn't clear what causes the initial genetic mutation that leads to neuroblastoma.

Risk factors

Children with a family history of neuroblastoma may be more likely to develop the disease. Yet, familial neuroblastoma is thought to comprise a very small number of neuroblastoma cases. In most cases of neuroblastoma, a cause is never identified.

Complications

Complications of neuroblastoma may include:

Diagnosis

Tests and procedures used to diagnose neuroblastoma include:

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. Knowing the cancer's stage helps the doctor decide what treatment is most appropriate.

Imaging tests used to stage cancer include X-rays, bone scans, and CT, MRI and MIBG scans, among others.

The stages of neuroblastoma are indicated by Roman numerals that range from 0 to IV, with the lowest stages indicating cancer that is limited to one area. By stage IV, the cancer is considered advanced and has spread to other areas of the body.

Treatment

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.

Your child's doctor uses this information to categorize the cancer as low risk, intermediate risk or high risk. What treatment or combination of treatments your child receives for neuroblastoma depends on the risk category.

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 destroy 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 intermediate-risk neuroblastoma often receive a combination of chemotherapy drugs 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 transplant.

Radiation therapy

Radiation therapy uses high-energy beams, such as X-rays, to destroy cancer cells.

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.

Radiation therapy primarily affects the area where it's aimed, but some healthy cells may be damaged by the radiation. What side effects your child experiences depends on where the radiation is directed and how much radiation is administered.

Bone marrow transplant

Children with high-risk neuroblastoma may receive a transplant using stem cells collected from bone marrow (autologous stem cell transplant).

Before the bone marrow transplant, also known as stem cell transplant, your child undergoes a procedure that filters and collects stem cells from his or her blood. The stem cells are stored for later use. 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 your child's body, where they can form new, healthy blood cells.

Immunotherapy

Immunotherapy uses drugs that work by signaling your body's immune system to help fight cancer cells. Children with high-risk neuroblastoma may receive immunotherapy drugs that stimulate the immune system to kill the neuroblastoma cells.

Newer treatments

Doctors are studying a newer form of radiation therapy that may help control high-risk neuroblastoma. The treatment uses a radioactive form of the chemical metaiodobenzylguanidine (MIBG). When injected in to the bloodstream, the MIBG travels to the neuroblastoma cells and releases the radiation.

MIBG therapy is sometimes combined with chemotherapy or bone marrow transplant. After receiving an injection of the radioactive MIBG, your child will need to stay in a special hospital room until the radiation leaves his or her body in the urine. MIBG therapy usually takes a few days.

Coping and support

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. It can be overwhelming.

If you're feeling lost, you might try to:

Preparing for an appointment

Make an appointment with your family doctor or pediatrician if your child has any signs or symptoms that worry you. You may be referred to a doctor who specializes in digestive problems (gastroenterologist), respiratory problems (pulmonologist), or problems affecting the brain and nervous system (neurologist).

Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well-prepared for your child's appointment. Here's some information to help you get ready, and what to expect from your child's doctor.

What you can do

Your time with your child's doctor is limited, so preparing a list of questions will help you make the most of your time. List your questions from most important to least important in case time runs out. For neuroblastoma, some basic questions to ask your child's doctor include:

In addition to the questions that you've prepared to ask your child's doctor, don't hesitate to ask questions anytime you don't understand something.

What to expect from your child's doctor

Your child's doctor is likely to ask you a number of questions. Being ready to answer them may allow more time to cover points you want to address. Your child's doctor may ask:

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