Definition and Facts
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 is a common and often difficult to treat cancer, the most common cancer in infancy.
- In the United States, about 600 children are diagnosed with neuroblastoma each year.
- It is the most common tumor found in children younger than 1 year of age.
- Neuroblastoma is the most common extra cranial solid tumor cancer in children.
- Every 16 hours a child with neuroblastoma dies.
- There is no known cure for relapsed neuroblastoma.
- Nearly 70% of those children first diagnosed with neuroblastoma have disease that has already metastasized or spread to other parts of the body. When disease has spread at diagnosis and a child is over the age of 2, there is less than a 30% chance of survival.
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:
- Abdominal pain
- A mass under the skin that isn’t tender when touched
- Changes in bowel habits, such as diarrhea
- Swelling in the legs
Neuroblastoma in the chest may cause signs and symptoms such as:
- Chest pain
- Changes to the eyes, including drooping eyelids and unequal pupil size
Other signs and symptoms that may indicate neuroblastoma include:
- Lumps of tissue under the skin
- Eyeballs that seem to protrude from the sockets (proptosis)
- Dark circles, similar to bruises, around the eyes
- Back pain
- Unexplained weight loss
- Bone pain
Note: In Jack’s case, his first symptom was a low grade fever that lasted about 10 days. He the told us that his left armpit hurt him and that is how we initially knew something was wrong. The first time he saw a doctor was when his left eye started to droop and as we checked in to Phoenix Children’s Hospital his neck started to swell on the left side. The physical symptoms happened very fast but the symptoms that made us take him to the doctor was nothing that we could see, but just what he told us was bothering him.
When to see a doctor
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:
- Spread of the cancer (metastasis). Neuroblastoma may spread (metastasize) to other parts of the body, such as the lymph nodes, bone marrow, liver, skin and bones.
- Spinal cord compression. Tumors may grow and press on the spinal cord, causing spinal cord compression. Spinal cord compression may cause pain and paralysis.
- Signs and symptoms caused by tumor secretions. Neuroblastomas may secrete certain chemicals that irritate other normal tissues, causing signs and symptoms called paraneoplastic syndromes. One paraneoplastic syndrome that occurs rarely in people with neuroblastoma causes rapid eye movements and difficulty with coordination. Another rare syndrome causes abdominal swelling and diarrhea.
Tests and diagnosis
Tests and procedures used to diagnose neuroblastoma include:
- Physical exam. Your child’s doctor conducts a physical exam to check out any signs and symptoms. The doctor will ask you questions about your child’s habits and behaviors.
- Urine and blood tests in children. These may indicate the cause of any signs and symptoms your child is experiencing. Urine tests may be used to check for high levels of certain chemicals that result from the neuroblastoma cells producing excess catecholamines.
- Imaging tests. Imaging tests may reveal a mass that can indicate a tumor. Imaging tests may include X-ray, ultrasound, computerized tomography (CT) scan and magnetic resonance imaging (MRI), among others.
- Removing a sample of tissue for testing. If a mass is found, your child’s doctor may want to remove a sample of the tissue for laboratory testing (biopsy). Specialized tests on the tissue sample can reveal what types of cells are involved in the tumor and specific genetic characteristics of the cancer cells. This information helps your child’s doctor devise an individualized treatment plan.
- Removing a sample of bone marrow for testing. Your child may also undergo bone marrow biopsy and bone marrow aspiration procedures to see if neuroblastoma has spread to the bone marrow — the spongy material inside the largest bones where blood cells are formed. In order to remove bone marrow for testing, the surgeon inserts a needle into your child’s hipbone or lower back and draws out the marrow.
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:
- Stage I. Neuroblastoma at this stage is localized, meaning it’s confined to one area, and may be completely removed with surgery. Lymph nodes connected to the tumor may have signs of cancer, but other lymph nodes don’t have cancer.
- Stage IIA. Neuroblastoma at this stage is localized, but may not be as easily removed through surgery.
- Stage IIB. Neuroblastoma at this stage is localized and may or may not be easily removed through surgery. Both the lymph nodes connected to the tumor and the lymph nodes nearby contain cancer cells.
- Stage III. Neuroblastoma at this stage is considered advanced, and it isn’t possible to remove the tumor through surgery. The tumor may be a larger size at this stage. Lymph nodes may or may not contain cancer cells.
- Stage IV. Neuroblastoma at this stage is considered advanced and has spread (metastasized) to other parts of the body.
- Stage IVS. This stage is a special category of neuroblastoma that doesn’t behave like other forms of neuroblastoma, though it isn’t clear why. Stage IVS neuroblastoma applies only to children younger than 1 year old. Stage IVS indicates that neuroblastoma has spread to another part of the body — most commonly the skin, liver or limited bone marrow involvement. Despite the extent of neuroblastoma, babies with this stage have a good chance of recovery. Neuroblastoma at this stage sometimes goes away on its own and often doesn’t require any treatment.
Treatments and drugs
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.
Information provided by MayoClinic.com