Neuroblastoma

Diagnosis

A neuroblastoma diagnosis might start with a physical exam. Urine and blood tests can give clues about what might be causing your child's symptoms. Imaging tests can look for signs of a growth that could be a neuroblastoma.

To be certain of the diagnosis, the healthcare team removes some of the growth for testing. If that test finds neuroblastoma, your child may have tests to look for signs that the cancer has spread. These might include other imaging tests and a procedure to remove some bone marrow for testing.

Physical exam

A healthcare professional may examine your child to check for signs of neuroblastoma. The healthcare professional may ask you questions about your child's symptoms and health history.

Urine and blood tests

A healthcare professional might test your child's urine and blood. The results can help the healthcare professional better understand your child's condition. Urine tests might look for high levels of chemicals made by neuroblastoma cells. For example, neuroblastoma can produce chemicals called catecholamines. As the body breaks these down, they can be detected in the urine.

Ultrasound

Ultrasound is an imaging test that uses sound waves to make pictures of the body. It's usually the first test used to check out symptoms in the belly area in children. Ultrasound isn't the best imaging test to diagnose neuroblastoma, but it gives the healthcare professional clues about what test to do next.

CT scan

A computerized tomography (CT) scan is an imaging test that uses X-ray techniques to create detailed images of the body. It then uses a computer to create cross-sectional images, also called slices, of the bones, blood vessels and soft tissues inside the body.

Healthcare professionals might use CT to show the size of a neuroblastoma in the belly or chest. It also can show cancer that spreads to the lymph nodes, bones, liver or skin.

CT is widely available and results come back quickly. A healthcare professional might choose this test based on your child's symptoms and the location of the neuroblastoma. CT might be the best choice if results are needed quickly.

MRI

Magnetic resonance imaging (MRI) is an imaging test that uses a magnetic field and radio waves to create pictures of the inside of the body. Healthcare professionals use MRI to show the size and location of neuroblastoma in the belly or chest. It's particularly good at showing neuroblastoma in and around the spine. MRI also can show cancer that spreads to the lymph nodes, bones, liver or skin.

Biopsy

A biopsy is a procedure to remove a sample of tissue for testing in a lab. To get the sample, a healthcare professional might put a hollow needle through the skin and into the cancer. The health professional uses the needle to draw out some cells for testing. Sometimes a surgeon removes the tissue sample during surgery.

In the lab, tests can check the tissue for signs of cancer. Other tests might look for changes in the DNA inside the cancer cells. Results from these tests may help your child's healthcare team make a treatment plan.

MIBG scan

Metaiodobenzylguanidine (MIBG) scans use nuclear imaging to make pictures. Nuclear imaging involves using medicines that have tiny amounts of radioactive substances. These medicines are called radiopharmaceuticals. When they're used for imaging, they also might be called radiotracers.

The MIBG scan uses a radiotracer to find neuroblastoma cells. A healthcare professional puts the tracer into a vein. The tracer goes through the body and attaches to the neuroblastoma cells. A camera in the scanner detects the radiation that the tracer gives off. A computer takes the information from the camera and makes pictures that show where neuroblastoma cells are in the body.

Healthcare professionals use MIBG scans to look for signs that neuroblastoma has spread. This test can find neuroblastoma cells anywhere in the body. The healthcare team might recommend this scan after other tests have confirmed the diagnosis of neuroblastoma. MIBG scans might be repeated during and after treatment to see how well treatment is working.

PET scan

Positron emission tomography (PET) scans are nuclear imaging tests that use radioactive tracers to highlight areas of disease. The tracer contains a substance that helps it stick to fast-growing cells, such as cancer cells. The PET images show the places where the tracer builds up.

PET scans aren't often used for neuroblastoma. A PET scan might be used if MIBG scans don't show the neuroblastoma. MIBG scans don't work for about 1 out of every 10 neuroblastomas.

Bone marrow aspiration and biopsy

Bone marrow aspiration and biopsy are procedures that involve collecting cells from the bone marrow. The cells are sent for testing. These procedures are used to check if neuroblastoma has spread to the bone marrow.

Bone marrow is the soft matter inside bones where blood cells are made. Bone marrow has a solid part and a liquid part. In a bone marrow aspiration, a needle is used to draw a sample of the fluid. In a bone marrow biopsy, a needle is used to collect a small amount of the solid tissue. The samples are typically taken from the hip bone.

Treatment

Treatments for neuroblastoma include surgery, radiation therapy and medicines, such as chemotherapy, and others. Healthcare teams consider many things when creating a treatment plan. These include the child's age, the stage of the cancer and the risk group.

Surgery

During surgery for neuroblastoma, surgeons use cutting tools to remove the cancer cells. In children with low-risk neuroblastoma, surgery to remove the cancer may be the only treatment needed.

Whether the cancer can be removed completely depends on its location and size. Cancers that are attached to nearby vital organs may be too risky to remove.

In intermediate-risk and high-risk neuroblastoma, treatment usually starts with chemotherapy to shrink the cancer. Surgeons may try to remove as much of the cancer as possible.

Chemotherapy

Chemotherapy treats cancer with strong medicines. Many chemotherapy medicines exist. Most chemotherapy medicines are given through a vein. Some come in pill form.

Children with intermediate-risk neuroblastoma often receive a combination of chemotherapy medicines before surgery. This improves the chances that the entire cancer can be removed.

Children with high-risk neuroblastoma often receive high doses of chemotherapy medicines to shrink the cancer. Chemotherapy also helps kill any cancer cells that have spread to other parts in the body. Chemotherapy often is used before surgery and before bone marrow transplant.

Bone marrow transplant

A bone marrow transplant, also called a bone marrow stem cell transplant, involves putting healthy bone marrow stem cells into the body. These cells replace cells hurt by chemotherapy and other treatments.

A bone marrow transplant might be an option for children with high-risk neuroblastoma. A bone marrow transplant for neuroblastoma uses the child's own blood stem cells. This kind of transplant is called an autologous stem cell transplant.

Before the transplant, a procedure is done to filter and collect blood stem cells from the child's blood. The stem cells are stored for later use. Next, the child receives high doses of chemotherapy to kill any remaining cancer cells. Then the blood stem cells are put back into the child's body. The transplanted cells can form new, healthy blood cells.

Radiation therapy

Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources.

Children with high-risk neuroblastoma may receive radiation therapy after chemotherapy and surgery. The radiation can help lower the risk that the cancer will come back.

Proton therapy

Proton therapy is a type of radiation therapy that uses energy from protons. Proton beams can be more carefully targeted to the cancer cells. Proton therapy may be less likely to hurt healthy tissue near the cancer. This is a newer form of radiation therapy. It's not available at all medical centers.

For neuroblastoma, proton therapy may be an option for children with high-risk neuroblastoma. Like other forms of radiation therapy, it's typically used after chemotherapy and surgery.

Immunotherapy

Immunotherapy is a treatment with medicine that helps the body's immune system kill cancer cells. The immune system fights off diseases by attacking germs and other cells that shouldn't be in the body. Cancer cells survive by hiding from the immune system. Immunotherapy helps the immune system cells find and kill the cancer cells.

Immunotherapy medicines for neuroblastoma target a substance called glycolipid GD2 on the surface of neuroblastoma cells. Medicines that work in this way are called anti-GD2 immunotherapies. The medicine is given through a vein. It travels through the body and attaches to the cancer cells. This causes the immune system cells to attack the cancer.

Immunotherapy is sometimes used for high-risk neuroblastoma. It's typically used after other treatments. Immunotherapy medicines are usually combined with other medicines, such as chemotherapy and isotretinoin, which used to be sold under the brand name Accutane.

Treatment to prevent relapse

Children with high-risk neuroblastoma sometimes receive extra treatment to reduce the risk that the cancer will start growing again. It's generally used after other treatments have controlled the cancer.

Eflornithine (Iwilfin) comes as a pill that's taken twice a day. It can be taken for as long as two years. This medicine also is called difluoromethylornithine (DFMO).

The medicine reduces the risk of relapse. Relapsed neuroblastoma happens when the cancer starts to get worse after a period of getting better.

Treatment for recurrence or relapse

Other treatments might be needed if the cancer comes back, called recurrence, or if it relapses. Other treatments also might be used if the cancer doesn't respond to the usual treatments, called refractory cancer.

Most neuroblastoma relapses and recurrences happen in the first two years after treatment. Rarely, the cancer can come back many years later.

Treatment usually starts with medicines that may have helped before, such as chemotherapy and immunotherapy. If these are no longer helping, other options might include:

• Radiopharmaceutical therapy. Radiopharmaceutical therapy uses a medicine that contains a radioactive substance. The medicine goes through the body, attaches to target cells and releases the radiation. One neuroblastoma medicine that works in this way is I-131 MIBG, also called MIBG therapy. It's being studied in clinical trials. Researchers are studying other radiopharmaceutical therapies for neuroblastoma, including lutetium Lu-177 dotatate.
• Targeted therapy. Targeted therapy for cancer is a treatment that uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die. Clinical trials are studying targeted therapy medicines for neuroblastoma.
• CAR-T cell therapy. CAR-T stands for chimeric antigen receptor (CAR)-T cell therapy. It's a cancer treatment that takes cells from the body and changes them so that they can fight cancer. It's most often used to treat cancers that affect blood cells, such lymphoma. Clinical trials are studying CAR-T cell therapy for neuroblastoma.

Coping and support

When your child is diagnosed with cancer, it's common to feel a range of emotions. Some parents say they had feelings such as shock, disbelief, guilt and anger. At the time you might be feeling these strong feelings, you also may need to make decisions about your child's treatment. This can feel overwhelming. Here are some ideas to help with coping.

Gather all the information you need

Find out enough about neuroblastoma to feel comfortable making decisions about your child's care. Talk with your child's healthcare team. Keep a list of questions to ask at the next appointment. Ask your child's healthcare team to recommend good sources of information. In the United States, the National Cancer Institute and the American Cancer Society are good organizations to go to for more information.

Organize a support network

Friends and family can provide both emotional and practical support as your child goes through treatment. Your friends and family will likely ask you what they can do to help. Take them up on their offers. Loved ones can go with your child to healthcare visits or sit by your child's bedside in the hospital when you can't be there. When you're with your child, your friends and family can help out by spending time with your other children or helping around your home.

Take advantage of resources for children with cancer

Seek out special resources for families of kids with cancer. Ask your clinic's social workers about what's 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 your usual routines as much as possible

Small children can't understand what's happening to them as they undergo cancer treatment. To help your child cope, try to keep your usual routines as much as possible. Try to arrange appointments so that your child can have a set nap time each day. Have routine mealtimes. 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 your child feel more comfortable.

Ask your healthcare team about other ways to comfort your child through treatment. Some hospitals have recreation therapists or child life workers who can give you more-specific ways to help your child cope.

Preparing for your appointment

Make an appointment with your family healthcare professional if your child has any symptoms that worry you.

Because appointments can be brief it's a good idea to be prepared. Here's some information to help you get ready, and what to expect.

What you can do

• Be aware of any restrictions before your appointment. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as change your child's diet.
• Write down any symptoms your child is experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including any major stresses or recent life changes.
• Make a list of all medicines, vitamins or supplements your child is taking and the doses.
• Consider taking a family member or friend along. Sometimes it can be hard to remember all the information provided during an appointment. Someone who comes with you may remember something that you missed or forgot.
• Write down questions to ask your child's healthcare team.

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

• What may be causing my child's symptoms or condition?
• What kinds of tests does my child need?
• What is the best course of action?
• What are the alternatives to the approach that you're suggesting?
• My child has these other health conditions. How can they best be managed together?
• Are there any restrictions that my child needs to follow?
• Should my child see a specialist?
• Where can I find more information?

In addition to the questions that you've prepared, don't hesitate to ask other questions during your appointment.

What to expect from your child's doctor

Your child's healthcare team is likely to ask you questions, such as:

• When did your child begin experiencing symptoms?
• How have your child's symptoms changed over time?
• What, if anything, seems to improve your child's symptoms?
• What, if anything, appears to worsen your child's symptoms?