Cancers and Tumors

Neuroblastoma

What is neuroblastoma?

Neuroblastoma is cancer that starts in young nerve cells called neuroblasts.

In a healthy child, these cells become the nerves that control functions like heartbeat and blood pressure and the way the body reacts to stress. In neuroblastoma, some of the neuroblasts do not mature. Instead, they divide and grow into tumors.

Cancer in children is rare, and neuroblastoma accounts for less than 10% of all childhood cancers.

Most children with this disease get it before age 5. In some kids, the disease starts before birth.

Doctors group childhood neuroblastoma into 3 levels: low, intermediate and high risk. Risk refers to how hard the neuroblastoma is to treat.

  • Doctors do not know what causes the changes in cells that lead to this disease.

    In most cases, parents and doctors don’t know the disease is there until tumors grow large enough to be felt or to cause symptoms.

    Usually, neuroblastoma tumors start in the nerves of the adrenal glands. These glands sit on top of the kidneys. They release hormones that control the part of the nervous system that affects heartbeat, blood pressure and reactions to stress. But these tumors can begin in any part of the body. The chest, neck, pelvis and spinal cord are other common places.

    As the disease goes on, it can spread (metastasize) to other areas of the body. It usually spreads to the lymph nodes, bones, bone marrow, liver or skin.

    For most children, the neuroblastoma has spread to at least 1 of these areas by the time doctors diagnose the disease.

Neuroblastoma at Seattle Children’s

Many families have never heard of neuroblastoma before their child develops it. Doctors at our Neuroblastoma Program focus on curing neuroblastoma in babies, children and teens.

The program is part of the Cancer and Blood Disorders Center at Seattle Children’s. We treat more pediatric cancer than any center in the region.

Please contact the center at 206-987-2106 for more information, a second opinion or to make an appointment.

  • Our doctors help set national standards for neuroblastoma care. They are international leaders in developing new treatments for children at all stages and risk levels of the disease. This includes neuroblastoma that:

    • Does not respond well to treatment (refractory)
    • Comes back after treatment (recurrent)
    • Is high risk, which is very hard to cure

    Treatment for children with high-risk neuroblastoma has changed across North America as a result of a Phase 3 national study led by Dr. Julie R. Park of Seattle Children’s. The research study found that adding a second stem cell transplant improved children’s 3-year survival significantly. The Children’s Oncology Group (COG) sponsored the trial.

    COG is an international organization of childhood cancer specialists that studies many forms of childhood cancer, with the goal of improving treatments. Dr. Park chairs the COG neuroblastoma committee.

    We are the only center in the Pacific Northwest to belong to the NANT (New Approaches to Neuroblastoma Therapy) group. Our doctors are leaders within NANT. The group works to find new treatments for children whose neuroblastoma is not controlled by treatment or comes back.

    Learn about cancer research at Seattle Children’s.

  • Because doctors at Seattle Children’s are leaders in cancer research, our patients have the chance to get the very latest treatments being studied.

    The newest treatment options are especially important for children and teens with neuroblastoma that does not respond well to early treatment or that comes back. At Seattle Children’s, the options include a Phase 1 clinical trial that reprograms the body’s infection-fighting T cells to find and destroy cancer cells with few side effects. This clinical trial is called ENCIT.

    Learn more about cancer clinical trials at Seattle Children’s.

    For children with high-risk neuroblastoma, treatment may involve:

    • A special form of radiation treatment that gets radiation into your child’s cancer cells via the bloodstream. This type of radiation is called I-131-MIBG therapy.
    • A stem cell transplant, also called a hematopoietic (hee-MA-toh-poy-EH-tik) cell transplant. We offer this treatment with our partner, Fred Hutch. Fred Hutch pioneered this lifesaving procedure over 30 years ago.

  • In 2016, U.S. News & World Report ranked Seattle Children’s Cancer Center number 1 in the West and number 6 in the country.

    At Seattle Children’s Cancer and Blood Disorders Center, our outcomes for a wide range of cancers are significantly better than the national average.

    Through our partnership in Seattle Cancer Care Alliance (SCCA), our patients benefit from the work of physician-scientists at Fred Hutch and UW Medicine, as well as at Seattle Children’s. The National Cancer Institute has designated our partnership a comprehensive cancer center.

    Seattle Children’s provides the most advanced treatments available in our region. Our experience helps us recommend the right treatment at the right time to have the best results for your child and your family.

  • Our specialty is treating and curing children’s cancer while helping them grow up to be healthy and productive.

    Children don’t react to illness, injury, pain and medicine in the same way as adults. They need – and deserve – care designed just for them. They need a healthcare team specially trained to understand and meet their needs.

    Our cancer doctors and nurses have special training in how to diagnose and treat children with cancer. Our experts are focused on how treatments today affect growing bodies in the future. They base their treatment plans on years of experience and the newest research on what works best – and most safely – for children.

    We care for your whole child. We don’t just treat their cancer. Your family has a full team behind you, including specialists in nutrition, nursing, pain management, physical therapy, social work and emotional health. Read more about the supportive care we offer.

  • A diagnosis of neuroblastoma can be scary. We help take positive steps right away by offering appointments within 1 to 3 days to children who are suspected of having cancer.

    A team that specializes in your child’s cancer will take care of your child. A doctor specially trained in diagnosing and treating cancer in children (pediatric oncologist) will guide your child’s care. During visits, we take time to explain your child’s condition. We help you fully understand your treatment options and make the choices that are right for your family.

    We work together, and with you and your family’s doctor, to manage your child’s personalized care.

    Our child life specialists and social workers help your child and your family through the challenges of cancer. We connect you to community resources and support groups.

    At Seattle Children’s, we work with many children and families from around the Northwest and beyond. Whether you live nearby or far away, we can help with financial counseling, schooling, housing, transportation, interpreter services and spiritual care. Read about our services for patients and families.

  • People who have been cured of cancer may be affected for months or years by their disease or treatment. Our Cancer Survivor Program provides long-term follow-up to help children and young adults stay healthy after being treated for cancer in childhood.

    Another specialized program through our partners in the SCCA provides follow-up care for those who have had a stem cell transplant.

Symptoms of Neuroblastoma

Symptoms of neuroblastoma depend on where the tumor or tumors are. Tumors may cause a lump that you can see or feel.

Here are some other signs or symptoms that might happen in a child who has neuroblastoma. The same things can also be caused by other health problems. Check with a doctor if your child has any of these:

  • Pain or weakness in some part of the body. This can happen from tumors that press on nerves.
  • Enlarged belly or trouble having a bowel movement. This can happen if tumors are growing inside the belly. They may press against the muscles and skin that cover the belly, or they may press on internal organs, like the intestines.
  • Feeling tired or irritable and being short of breath. Your child may also get dizzy or lightheaded or be paler than usual. These may be signs that your child does not have enough red blood cells to carry oxygen around the body (anemia). Anemia can happen if there are tumors in the bone marrow (where blood cells are made).
  • Bulging eyes or dark circles around the eyes. Tumors around the eyes can cause these symptoms.

Diagnosing Neuroblastoma

  • To diagnose your child, your child’s doctor will:

    • Do an exam to look for signs of cancer
    • Ask about your child’s health

    The doctor may also:

    • Test your child’s pee (urine) or blood to check levels of chemicals that are made or increased by neuroblastoma.
    • Ask about your child’s thinking and movement.
    • Do simple tests to check whether muscles, reflexes and coordination seem healthy. These tell the doctor if there are problems with your child’s nervous system.

    If the doctor thinks that your child may have neuroblastoma, other tests will help tell the type of cancer and whether it has spread.

Risk Levels and Stages of Neuroblastoma

Doctors determine your child’s cancer risk level and stage so they can plan the best treatment.

  • Risk level refers to how hard the neuroblastoma is to treat. Doctors group childhood neuroblastoma into 3 risk levels: low, intermediate and high.

    Low-risk and intermediate-risk neuroblastoma have a good chance of being cured. High-risk neuroblastoma is very hard to cure.

    The risk group is based on:

    • Your child’s age when doctors diagnose the disease
    • The stage of their disease
    • Special features of the cancer cells

    Your child’s doctor can explain your child’s risk level.

  • It is important to find out if cancer is only in 1 place or has spread. This process is called staging.

    Stages are based on whether:

    • There are tumors in more than 1 place
    • Surgeons can remove all of the tumor that they can see
    • The cancer has spread and where
    • Cancer affects the lymph nodes

  • To help tell the stage of your child’s neuroblastoma and plan the best treatment, doctors will:

    • Remove a small sample of tissue to check under a microscope. This is called bone marrow aspiration and biopsy. It shows if neuroblastoma has spread to the soft inner part of bone (bone marrow). Your child’s doctor will:
      • Place a hollow needle into the hip bone and suck out (aspirate) a small sample of liquid bone marrow
      • Push a larger needle into the bone to remove a small amount of bone marrow
      • Look at the samples under a microscope to check for cancer cells
    • Take pictures inside your child’s body (imaging studies) to look for tumors or places where cancer is active. Pictures may be made using:
      • X-rays
      • CT (computed tomography) scan
      • MRI (magnetic resonance imaging) scan
      • MIBG (metaiodobenzylguanidine) scan
      • PET (positron emission tomography) scan

Treatment Options for Neuroblastoma

Our treatment goals are to cure your child’s cancer and prevent serious side effects, both short term and long term. All of the options below are offered through our Neuroblastoma Program.

  • Children with neuroblastoma usually have surgery. The first surgery your child has may be a biopsy. This means doctors remove a small sample of tumor cells to check for cancer.

    Doctors will remove as much of the tumor as is safely possible. This depends on where the cancer is and how high risk it is. Sometimes, the tumor is removed during the same surgery as the biopsy.

    In some children, the tumor has spread to other places in the body or is too large to remove safely during the biopsy. In this case, after the biopsy and diagnosis, your child will receive chemotherapy to help control the tumor. Later, surgeons remove as much of the tumor as possible. Studies have shown excellent results in low-risk and intermediate-risk neuroblastoma even if the whole tumor is not removed.

    Even when doctors can remove the whole tumor, they may suspect or know that cancer cells are still in your child’s body. They often use chemotherapy, radiation or both to kill any cancer cells that are left.

    Your child will get medicine to make them sleep without pain (general anesthesia) during surgery. Surgery is performed at our hospital’s main campus in Seattle.

    Learn more about surgery to treat cancer at Seattle Children’s.

  • Whole body (systemic) chemotherapy means giving medicines that go throughout your child’s body to kill cancer cells. For neuroblastoma, the medicine usually is injected into your child’s vein. This is called an intravenous (IV) infusion.

    Your child’s doctors may suggest whole body chemotherapy:

    • As the main treatment
    • To help shrink a tumor before another type of treatment, such as surgery
    • To kill any cancer cells that have spread to other parts of your child’s body

    Children with neuroblastoma receive chemotherapy at our hospital’s main campus in Seattle:

    The mix of medicines and how long they are given depend on the type of neuroblastoma your child has. Researchers are studying new mixes of medicines to find which work best against each type of the disease.

    See more about getting chemotherapy at Seattle Children’s.

  • Your child’s doctors may suggest radiation therapy as part of their treatment.

    For low-risk or intermediate-risk disease, doctors may use radiation when medicine isn’t helping fast enough and a child has tumors that cause severe problems. Problems include tumors that block breathing or blood flow or that affect how an organ works.

    All children with high-risk neuroblastoma receive radiation to the first tumor. Sometimes radiation also is given to places where the tumor has spread.

    The best radiation therapy depends on your child’s cancer. Options include:

    • X-rays: A machine sends beams of light particles (photons) through your child’s skin and outer structures into deeper parts of their body. The radiation kills cancer cells and shrinks tumors.
    • Proton therapy. This is a more precise type of radiation that uses beams of protons instead of photons.
    • I-131-MIBG therapy. This is a way to deliver radiation directly to tumors via the bloodstream. It may be an option for children with high-risk neuroblastoma.

    Learn more about Seattle Children’s Radiation Therapy Service.

  • If your child has high-risk neuroblastoma, they may get very high doses of chemotherapy medicines and a transplant of their own blood-forming (hematopoietic) stem cells. This is called an autologous stem cell transplant.

    Before high-dose chemotherapy, doctors will remove stem cells from your child. The cells are frozen and stored. After chemotherapy is finished, doctors return the stem cells to your child’s body through a vein.

    High-dose chemotherapy treats tumors more effectively than lower-dose chemotherapy. But the drugs damage your child’s bone marrow’s ability to make new blood cells. That’s why a transplant of the stored healthy stem cells is needed.

    A national Phase 3 COG clinical trial led by Dr. Park showed that adding a second stem cell transplant increases survival in patients with high-risk neuroblastoma. See “From 35 Percent Chance of Survival to Five Years Cancer-Free.

    Our patients who have these cell transplants get care through Seattle Children’s and our partner in the Seattle Cancer Care Alliance, Fred Hutch. Learn more about the Pediatric Blood and Marrow Transplant Program.

    Treatment after transplant

    After transplant, your child will get 6 months of additional treatment. The aim is to kill any cancer cells that may regrow and cause the disease to come back. Treatment includes:

    • A vitamin-like drug (isotretinoin) that slows the cancer’s ability to make more cancer cells. It is taken by mouth.
    • A monoclonal antibody that targets a substance (GD2) on neuroblastoma cells. The antibody then triggers the immune system to kill the neuroblastoma cells. Monoclonal antibodies are man-made versions of immune system proteins that fight disease. Your child receives the antibodies through a tube into their vein.
    • Proteins called cytokines that help the immune system fight cancer. Interleukin-2 and GM-CSF are used against neuroblastoma. Your child may get them through a tube into their vein or as a shot (injection) under the skin.

  • Many of our patients with neuroblastoma take part in clinical trials. These research studies give children the chance to get the very latest treatments being studied.

    Your child’s doctor will talk with you in detail about any new treatment that might help your child. Then you can decide whether you want to try these options.

    To learn more about cancer clinical trials at Seattle Children’s:

    Our national leadership in neuroblastoma research means more options for children with high-risk, refractory or recurrent neuroblastoma. These options are not offered at most treatment centers.

    They include:

    • Reprogramming the body’s infection-fighting T cells to find and destroy neuroblastoma cells. The Phase 1 clinical trial for children and adolescents is called Engineered Neuroblastoma Cellular Immunotherapy (ENCIT).
    • A newer type of radiation therapy called I-131-MIBG therapy for children with newly diagnosed high-risk neuroblastoma. This gets radiation inside your child’s body and into their cancer cells via the bloodstream.

Treating Neuroblastoma by Risk Level

Your child’s neuroblastoma treatment will depend on whether their disease is low risk, intermediate risk or high risk.

Here are the most common treatments based on risk level.

  • Most children with low-risk neuroblastoma have surgery to remove their primary tumor. Some have chemotherapy or radiation, too.

    In some children with a certain form of low-risk disease, tumors go away on their own. For children with this form, doctors may suggest “watchful waiting.” This means closely tracking your child’s cancer, without treatment, to see whether it changes.

  • Children at this risk level most often have surgery and chemotherapy. Some have radiation, too.

  • Treatment for high-risk neuroblastoma involves some mix of:

    • Surgery.
    • Chemotherapy.
    • Radiation, including internal radiation using I-131-MIBG.
    • High-dose chemotherapy and stem cell transplant.
    • A vitamin-like drug that slows formation of new cancer cells.
    • Clinical trials of promising new treatments. These studies include using the immune system to kill the neuroblastoma cells (immunotherapy).

Treating Refractory Neuroblastoma

If your child’s neuroblastoma does not respond well to their first treatment, it is called refractory. The next treatment steps may include:

  • Surgery.
  • Chemotherapy, often using different medicines or doses than your child’s first treatment.
  • Radiation, including internal radiation using I-131-MIBG.
  • Clinical trials of promising new treatments. These studies include using the immune system to kill the neuroblastoma cells (immunotherapy).

Treating Recurrent Neuroblastoma

If your child’s neuroblastoma comes back after treatment, it is called recurrent. The best treatment for your child will depend on:

  • The risk level of their neuroblastoma
  • Whether it has come back in the same area or spread to other parts of the body
  • Whether it involves the brain and spinal cord
  • Features of the cancer cells

Treatment options for recurrent neuroblastoma include:

  • Surgery.
  • Chemotherapy.
  • Radiation, including internal radiation using I-131-MIBG.
  • High-dose chemotherapy and stem cell transplant.
  • Clinical trials of promising new treatments. These studies include using the immune system to kill the neuroblastoma cells (immunotherapy).

Follow-Up Care

Follow-up care is important after treatment ends. The follow-up routine will depend on your child’s cancer and their treatments. Usually visits are every 3 to 6 months for 5 years.

Your child can be seen at Seattle Children’s or by an oncologist in your own community. Your doctor will let you know the schedule that is right for you.

During follow-up visits we will check your child for any signs that neuroblastoma is returning. Your doctor also will check for effects that may happen months or years after treatment.

Our Cancer Survivor Program provides long-term follow-up care to help young people stay healthy after being treated for cancer in childhood.

The Long-Term Follow-Up program for people who have had a stem cell transplant is offered through our partnership in the Seattle Cancer Care Alliance.

Contact Us

Contact the Cancer and Blood Disorders Center at 206-987-2106 for an appointment, a second opinion or more information.

To make an appointment, you can call us directly or get a referral from your child’s primary care provider. We encourage you to coordinate with your pediatrician or family doctor when coming to Seattle Children’s.

Providers, see how to refer a patient (PDF).