Neuroblastoma is a cancerous tumor. It grows in nerve tissue of babies and young children. The cancer cells grow in young nerve cells of a baby growing in the womb. These cells are called neuroblasts. It’s is the most common cancer in babies under age 1. It’s rare in children older than age 10.
- Nerve fibers along the spinal cord
- Clusters of nerve cells (ganglia) along the nerve fibers
- Nerve-like cells in the adrenal glands
In most cases, neuroblastoma starts in the adrenal glands or the nerve fibers in the abdomen. Other common places for it to grow include the nerve fibers near the spine in the chest, neck, or lower belly (pelvis).
The cancer is caused by changes in the DNA of the cells. In most children, this happens by chance. A small number of children inherit the risk from their parents.
The symptoms vary depending on the size and location of the tumor and if it has spread. Symptoms can also occur a bit differently in each child.
Symptoms of a tumor in the belly (abdomen) can include:
- A lump in the abdomen
- Swelling in the abdomen
- Loss of appetite
- Weight loss
- Swelling of the legs
- Swelling of the scrotum
- Feeling full
Symptoms of a tumor in the chest can include:
- A lump in the chest
- Swelling in the face, neck, arms, or chest
- A change in mental state
- Coughing or trouble breathing
- Trouble swallowing
- Drooping eye lids and other eye changes
- Changes in feeling or movement of the arms or legs
Symptoms of a neuroblastoma that has spread to other parts of the body can include:
- Enlarged lymph nodes
- Bone pain or limping
- Weakness, numbness, or inability to move arms or legs
- Bruising around the eyes
- Bulging eyes
- Lumps on the head
- Feeling tired or weak
- Frequent infections
- Easy bruising or bleeding
A neuroblastoma may release hormones. This is called paraneoplastic syndrome. It can cause symptoms such as:
- Constant diarrhea
- High blood pressure
- Fast heart rate
- Flushing or redness of skin
A neuroblastoma can also cause opsoclonus-myoclonus-ataxia syndrome. This can lead to symptoms such as:
- Quick eye movements
- Muscle twitching
- Trouble with coordination
The symptoms of neuroblastoma can be like other health conditions. Make sure your child sees a healthcare provider for a diagnosis.
You may take your child to the healthcare provider because of a lump, swelling, or other symptoms. Most of the time, neuroblastoma has spread by the time it is diagnosed. Your child's healthcare provider will ask about your child's medical history and symptoms. He or she will examine your child. Your child may be referred to a specialist in diagnosing and treating cancer in children (pediatric oncologist).Your child may have tests such as:
- Blood and urine tests. Blood tests check for signs of disease in the body. Your child may also have urine and blood tests to check for hormones released by the tumor.
- Ultrasound exam (US). Sound waves and a computer are used to create images. An ultrasound may be done to check your child's belly (abdomen), kidneys, or to guide a needle in a biopsy
- X-ray. X-rays may be done to check your child's chest or bones
- CT scan. X-rays and a computer are used to make images of the body. Scans of the belly (abdomen), lower belly (pelvis), or chest may be done. Contrast dye may be used. A CT scan may also be used to guide the needle in a biopsy.
- MRI scan. An MRI uses large magnets, radio waves, and a computer to make detailed pictures of the body. This test is used to check the brain and spinal cord.
- Bone scans. A small amount of dye is injected into a vein. The whole body is scanned. The dye shows up in areas of bone where there may be cancer.
- Positron emission tomography (PET) scan. For this test, a radioactive sugar is injected into the bloodstream. Cancer cells use more sugar than normal cells, so the sugar will collect in cancer cells. A special camera is used to see where the radioactive sugar is in the body. A PET scan can sometimes spot cancer cells in different areas of the body, even when they can’t be seen by other tests. This test is often used in combination with a CT scan. This is called a PET/CT scan.
- Metaiodobenzylguanidine (MIBG) scan. A small amount of radioactive iodine called MIBG is injected into a vein. The dye travels through the blood and attaches to cancer cells. Scans may be taken over a few days.
- Bone marrow aspiration or biopsy. Bone marrow is found in the center of some bones. It’s where blood cells are made. A small amount of bone marrow fluid may be taken. This is called aspiration. Or solid bone marrow tissue may be taken. This is called a core biopsy. Bone marrow is usually taken from the pelvic bone. This test may be done to see if cancer cells have reached the bone marrow.
- Tumor biopsy. A sample of the tumor is taken. It may be taken with a needle or by a surgical cut (incision). It is checked under a microscope for cancer cells. A biopsy is needed to diagnose neuroblastoma.
Staging and grading of neuroblastoma
Once neuroblastoma has been diagnosed, a stage is assigned. Staging is the process of seeing if the cancer has spread, and where it has spread. Staging also helps to decide the treatment. Doctors use two different staging systems for neuroblastoma: the International Neuroblastoma Staging System (INSS) and the International Neuroblastoma Risk Group Staging System (INRGSS).
The INSS system divides tumors into stages 1 through 4. It’s assigned after surgery has been done to remove the tumor. Stage 1 is early cancer. It's only in the place where it started and only on one side of the body. For neuroblastoma, this means the tumor can be fully seen and removed by surgery. Stages 2 and 3 are more complex. The tumor is harder to remove and cancer cells may have spread. Stage 4 means cancer cells from the tumor have spread to other parts of the body far from the primary tumor.
The INRGSS system divides tumors into 4 stages labeled L1, L2. M, and MS. Surgery does not need to be done before assigning one of these stages. They are based on how the tumor looks based on scans called image-defined risk factors (IDRFs) and biopsies. They are used to predict how much of the tumor can be taken put with surgery. The L groups means the cancer is confined to one part of the body. The M group means it has spread beyond where it first started to distant organs. MS is used in children younger than 18 months with cancer that has spread only to the skin, liver, or less than 10% of the bone marrow, or all three.
Grading is used to describe how abnormal the cancer cells look when seen through a microscope. The more abnormal the cells are, the faster they grow. Staging and grading help the healthcare team plan treatment for your child.
Other factors considered
The healthcare team also looks at:
- The location of the main tumor
- Genetic changes in the tumor cells
- The age of your child
Your child’s healthcare team will tell you more about the stage and grade of your child’s cancer. The stages of neuroblastoma are very complex. Be sure to ask the healthcare provider to explain the stage of your child's cancer to you in a way you can understand. Also be sure to ask the healthcare provider if you have any questions.
Treatment will depend on the stage and other factors. The cancer can be treated with any of the below:
- Surgery. Surgery is often done to remove as much of the tumor as possible (resection). Surgery may not be possible if the cancer has spread.
- Chemotherapy. These are medicines that kill cancer cells or stop them from growing. They may be given into the vein (IV), injected into tissue, or taken by mouth. They may be given before or after surgery. Or they may be the main treatment if surgery is not possible. The medicine is given in cycles, with rest periods in between.
- Radiation therapy. These are high-energy X-rays or other types of radiation. They are used to kill cancer cells or stop them from growing. Radiation may be outside of the body (external) or inside the body (internal). Radioactive iodine (MIBG) is given into a vein (IV). Your child will stay in the hospital for this treatment.
- High-dose chemotherapy/radiation with a stem cell transplant. Young blood cells (stem cells) are taken from the child or from someone else. This is followed by a large amount of chemotherapy medicine and/or radiation. This causes damage to the bone marrow. After the chemotherapy or radiation, your child will get stem cells to renew the bone marrow.
- Immunotherapy. This treatment helps the body's immune system attack cancer cells. It is also used when there is a high risk of cancer returning.
- Retinoid therapy. Retinoid medicine may be given after high-dose chemotherapy/radiation and stem cell transplant. It may be used when there is a high risk of cancer returning. It will reduce the chance of relapse or recurrence.
- Supportive care. Treatment can cause side effects. Medicines and other treatments can be used for pain, fever, infection, and nausea and vomiting.
- Clinical trials. Ask your child's healthcare provider if there are any treatments being tested that may work well for your child.
With any cancer, how well a child is expected to recover (prognosis) varies. Keep in mind:
- Getting medical treatment right away is important for the best prognosis.
- Ongoing follow-up care during and after treatment is needed.
- New treatments are being tested to improve outcome and to lessen side effects.
A child may have complications from the tumor or from treatment, such as:
- Bleeding or infection after surgery
- Hair loss, mouth sores, nausea, vomiting, diarrhea, increased infections, easy bruising and bleeding, and feeling tired from chemotherapy
- Burns, hair loss, and feeling tired from radiation
- Nausea and diarrhea from radiation to the belly (abdomen)
- Problems with growth and development
- Heart and lung problems
- Changes in sexual development
- Problems with the ability to have children (fertility) in the future
- Return of the cancer
- Growth of other cancers
- A high risk of serious bleeding (consumption coagulopathy) (rare)
A child with a neuroblastoma needs ongoing care. Your child will be seen by oncologists and other healthcare providers to treat any late effects of treatment and to watch for signs or symptoms of the tumor returning. Your child will be checked with imaging tests and other tests. And your child may see other healthcare providers for problems from the tumor or from treatment. Your child may need therapy to help with movement and muscle strength. This may be done by physical and occupational therapists.
You can help your child manage his or her treatment in many ways. For example:
- Your child may have trouble eating. A dietitian may be able to help.
- Your child may be very tired. He or she will need to balance rest and activity. Encourage your child to be active. This is good for overall health. And it may help to lessen tiredness.
- Get emotional support for your child. Find a counselor or child support group can help.
- Make sure your child attends all follow-up appointments.
When to Call a Healthcare Provider
Call the healthcare provider if your child has:
- Symptoms that get worse
- New symptoms
- Side effects from treatment
- Neuroblastoma is a cancerous (malignant) tumor that begins in nerve tissue of infants and very young children.
- The symptoms of neuroblastoma vary greatly depending on size, location of the tumor and whether it has spread. Common symptoms are a lump or swelling.
- Neuroblastoma is diagnosed with blood and urine tests, imaging tests, and biopsy.
- Treatment of neuroblastoma may include surgery, chemotherapy, radiation therapy, high-dose chemotherapy/radiation with stem cell transplant, and other medicines.
- Continuous follow-up care during and after treatment is necessary
Tips to help you get the most from a visit to your child’s healthcare provider:
- Know the reason for the visit and what you want to happen.
- Before your visit, write down questions you want answered.
- At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
- Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
- Ask if your child’s condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if your child does not take the medicine or have the test or procedure.
- If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
- Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.