Doctors use several tests to diagnose neuroblastoma. They ask about your child’s symptoms and medical history and do a physical exam. They may also order blood and urine tests.
This cancer develops in immature nerve cells. It usually starts in the adrenal glands (above each kidney) or in the nerve tissue that surrounds the spinal cord.
What is neuroblastoma?
Neuroblastoma is a type of cancer that forms in nerve cells called neuroblasts. Neuroblasts normally turn into working nerve cells that help control things like heart rate and blood pressure, but in neuroblastoma they form tumors instead. Most often, neuroblastoma begins in the adrenal glands (one on top of each kidney). It may also begin in nerve tissue in the neck or chest. It most commonly affects children younger than 5 years, and most cases are diagnosed before a child is 18 months old. It occurs slightly more often in boys than girls.
Neuroblastomas are caused by changes to genes that affect how the cell grows. The change may be a result of something that happens in the womb, or it could happen as the cells are developing inside the body. The cause of most neuroblastomas isn’t known.
Some children with neuroblastoma have no symptoms at all. Other children have mild or severe symptoms that depend on the size of the tumor and whether it has spread. Symptoms include low energy, fever, headaches, lumps under the skin, or feeling sick (nausea). Neuroblastoma can be difficult to diagnose because its symptoms can be similar to other health problems.
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A doctor can diagnose neuroblastoma by doing tests to find out if there are cancer cells in the body. These tests can include an imaging test, like a CT scan or an MRI, and a blood test to check for certain hormones. A biopsy can be done to get a small piece of tissue (called a sample) for testing. This is usually done with a needle or by making a surgical cut (incision). The sample is then tested to see if it has cancer cells in it.
Children are classified into risk groups based on the results of their blood and imaging tests. Children with lower-risk neuroblastoma have a better chance of being cured. High-risk neuroblastoma is harder to cure.
Diagnosis
Children who have neuroblastoma may need a lot of tests to find it. These tests help doctors see if the cancer has spread, and if it is in other parts of the body. These include blood and bone marrow tests, CT or MRI scans, and a special nuclear medicine scan called an MIBG scan.
These tests look for certain substances, such as catecholamines (substances made when neuroblastoma cells break down). Your child may also have urine and blood tests to check their general health and organ function. They may also have a chest X-ray, an ultrasound of the belly or kidneys, and a bone scan.
Bone marrow is the spongy tissue inside bones that makes blood cells. To test it, a doctor or nurse gives your child sedation and then inserts a needle into a hipbone to withdraw a sample of bone marrow. Then the doctor uses a microscope to check the marrow for signs of neuroblastoma. A swab of skin in the neck or arm may also be tested to check for neuroblastoma.
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Genetic tests may show whether your child has a gene change that increases their chance of getting neuroblastoma. This is an inherited risk factor, and it’s important for families to know if they have this type of family history.
The doctors may diagnose a child with neuroblastoma before they are born by using an ultrasound scan while the mother is still pregnant. They can also find out if your child has the condition after they are born, but it’s rare for a baby to be diagnosed with neuroblastoma at birth.
Once a diagnosis is confirmed, the doctors will use imaging tests to find out how far the cancer has spread and what other parts of the body it may be in. This is called staging. Staging helps the doctor plan treatment. More and more children are surviving cancer now than ever before. This is partly because more and better treatments are available. But it is also because more and more doctors are familiar with the different kinds of cancer in children, and how to treat them.
Treatment
The treatment options for neuroblastoma are based on the age of your child, how far the tumour has spread (if at all) and how aggressive it is. Children with low-risk neuroblastoma can be cured by surgery alone or together with chemotherapy and/or radiotherapy. Children with higher-risk neuroblastoma may require more intensive treatment. Recent advances in understanding how cancer develops and treatment is improving the outlook for all children with neuroblastoma.
X-rays, CT or MRI scans and a special nuclear medicine test called a MIBG scan are usually used to help diagnose neuroblastoma and see how far it has spread (known as staging). A urine test that measures levels of substances called vanillylmandelic acid and homovanillic acid may be done. These substances are taken up by tumour cells and can be detected in the blood. If they fall, this shows that the tumour is responding to treatment.
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Most children with neuroblastoma have a combination of treatments. These include chemotherapy and/or radiation therapy to shrink the tumour and kill any remaining cancer cells. In some cases, surgery may be needed to remove the tumour and surrounding tissue. Neuroblastomas have a rich blood supply and often wrap around important structures such as major blood vessels. As a result, they can be difficult to remove safely.
Bone marrow transplant (also known as cellular therapy) is sometimes used in high-risk neuroblastoma cases. This procedure filters and collects blood stem cells from your child’s body before giving him or her high doses of chemotherapy to kill any cancer cells left in the bone marrow or soft tissues. Your child’s healthy blood stem cells are then returned to the body, where they replace the damaged ones and form new, healthy blood cells.
Immunotherapy is a type of treatment that uses drugs to stimulate your child’s own immune system to fight the cancer cells. Recent research has shown that this can improve the outcome for some children with neuroblastoma, especially those who have a specific gene mutation called MYCN amplification.
You and your child’s doctors will discuss the different treatment options. It is important to ask questions if you don’t understand something or have any concerns. You can also get a second opinion from another doctor.
Symptoms
Cancer is a disease that starts when cells grow out of control and crowd out normal cells. They can also spread (metastasize) to other parts of the body. The signs and symptoms of neuroblastoma depend on where the tumor is, how big it is, and whether it has spread.
Most children with neuroblastoma are younger than 5 years old. They usually get the diagnosis before age 1. Sometimes healthcare providers can find this tumor in unborn babies during a prenatal ultrasound.
This cancer starts in immature nerve cells, called neuroblasts, in the sympathetic nervous system. These nerves are part of the automatic “fight or flight” response that controls involuntary body functions. Neuroblastoma can begin in the nerve tissues of the adrenal glands (on top of each kidney) or in the spinal cord, neck, chest, or pelvis. The adrenal glands make hormones that help control blood pressure, heart rate, and the way the body reacts to stress.
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The signs and symptoms of neuroblastoma include a lump or mass in the abdomen, armpits, groin, or neck; pain in the bones or legs; trouble moving; and bruising around the eyes. Some of these symptoms may also be caused by other health problems. If you notice any of these symptoms, see your child’s healthcare provider.
In most cases, a healthcare provider will do a physical exam and ask about your child’s past health. He or she will also do a blood test to check for high levels of certain substances, such as catecholamines. Catecholamines are chemicals that help control nerve activity. If they are too high, it can cause many of the symptoms of neuroblastoma.
Other tests will be done to find out how far the cancer has spread. These can include a CT, PET, or MRI scan to find out if the cancer is in bone or soft tissue. A radioactive substance called metaiodobenzylguanidine, or MIBG, is sometimes used to find out if the cancer has spread to the bone marrow or other organs. A nuclear medicine test that uses a slightly radioactive compound, called a positron emission tomography (PET) scan, can also show if the cancer has spread to these areas.
