Neuroblastoma is a cancer that develops from 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 pelvis, 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.
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:
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.
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). Cancer cells may invade nearby tissues and can break off from an initial tumor to spread elsewhere in the body (metastasize).
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. The majority of neuroblasts mature by birth, though studies have found a small number of immature neuroblasts in newborns. In most cases, these neuroblasts will mature or disappear. Others, however, form a tumor – a neuroblastoma.
It isn't clear what causes the initial genetic mutation that leads to neuroblastoma. However, because neuroblastoma usually affects very young children, researchers believe the mutation occurs during pregnancy or soon after birth.
The only factor known to increase the risk of neuroblastoma is a family history of the cancer. 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 of neuroblastoma may include:
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) or respiratory problems (pulmonologist).
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 any time 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:
What you can do in the meantime Try to keep your child comfortable by avoiding things that seem to worsen signs and symptoms. Not feeling well and an approaching trip to the doctor may make your child feel anxious. To reduce your child's anxiety, try to:
Tests and procedures used to diagnose neuroblastoma include:
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. Imaging tests used to stage cancer include X-rays, bone scans, and CT and MRI scans, among others.
Using the information from those procedures, your child's doctor assigns a stage to the neuroblastoma. Stages of neuroblastoma include:
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. Using this information, your child's doctor categorizes 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.
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 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 low-risk neuroblastoma that can't be removed surgically may undergo chemotherapy. Sometimes chemotherapy is administered before surgery (neoadjuvant chemotherapy) to shrink the tumor to a size that's more easily removed. In other cases, chemotherapy may be the only treatment.
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 stem cell transplant.
Radiation therapy uses high doses of energy particles to destroy cancer cells. Radiation therapy primarily affects the area where it's aimed. Your child's radiation therapy team tries to protect the healthy cells near the cancer, 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.
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.
Stem cell transplant
Children with high-risk neuroblastoma may receive a transplant using their own blood stem cells (autologous stem cell transplant). The bone marrow produces stem cells, which mature and develop into the red and white cells and platelets that make up the blood. Your child undergoes a procedure that filters and collects stem cells from his or her blood. 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.
Long-term and late side effects of cancer treatment As more and more children are surviving cancer and living into adulthood, doctors are increasingly aware of the long-term side effects of cancer treatment. Doctors recommend childhood cancer survivors be seen regularly by a doctor who understands the long-term and late side effects that can affect children after cancer treatment.
Side effects vary depending on treatment, but may include impaired growth, thyroid problems, second cancers and infertility, which may be caused by chemotherapy drugs. Difficulty thinking and trouble processing thoughts may be long-term side effects of radiation, especially brain radiation or total body irradiation.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. And while you're trying to sort it all out, friends and family may be calling for updates and to find out if they can help. It can be overwhelming. If you're feeling lost, you might try to: