What Is Neuroblastoma?
Neuroblastoma is a rare childhood cancer of what’s called the "sympathetic nervous system" — the network of nerves that carries messages from your brain to the rest of your body.
This cancer most often begins around the adrenal glands, a pair of hormone-producing organs that sit atop your kidneys and have cells similar to nerve cells. But neuroblastoma can also start in other areas of your body where groups of nerve cells are clustered.
Doctors most often diagnose it in children younger than 5. It's rarely seen in children older than 10.
Several types of treatments can help children with neuroblastoma survive and lead healthy lives. There are also resources parents can turn to for support and other help.
What Causes Neuroblastoma?
In general, cancers begin with changes ("mutations") in some cells of your body that allow them to grow out of control. They can form tumors and stop the cells from working as they should.
In neuroblastoma, the mutations affect immature nerve cells in a baby still in the womb. These cells are called neuroblasts. As the baby continues to grow before birth, the neuroblasts mature into functioning nerve cells.
In a healthy baby, the neuroblasts go away completely as the nervous system matures. But in babies with this condition, the mutated neuroblasts stick around and form a tumor.
Neuroblastoma Symptoms
Symptoms vary widely, depending on where the tumor is located, how big it is, and how much it's grown. "If it's in the abdomen, usually the first symptom is a firm lump that the parents or the pediatrician can feel when they press on the abdomen of a child," explains Mustafa Barbour, MD, a pediatric oncologist at Norton Children's inKentucky. "Later on, especially if the tumor spreads to other places, it can be bone pain and fever. Some kids look very pale because it can cause anemia." Many of the symptoms might point to conditions other than neuroblastoma.
In a child’s abdomen, neuroblastoma may cause:
- Lumps or swelling in the belly
- Stomach pain or a constant feeling of being full, which can lead to weight loss
- Swelling in their legs or scrotum caused by tumors pressing against their blood and lymph vessels
- Trouble peeing or having bowel movements
In the cheek or neck, it might cause:
- Swelling in their face, neck, arms, and chest
- Headaches and dizziness
- Coughing, trouble breathing, or swallowing
- Changes to their eyes, including drooping eyelids, unequal pupil sizes, bulging of the eyes, and discoloration under the eyes
Neuroblastoma that's spread can cause symptoms including:
- Enlarged lymph nodes. They're felt as hard lumps in the armpits, neck, or groin. Although most often a sign of infection, they can be a result of cancer that's spread to the lymph system.
- Bone pain, weakness in the legs or arms, and bruising around the eyes may come from cancer that's spread to the bones.
- If a neuroblastoma affects bone marrow, which makes blood cells, a child may be tired, irritable, and weak, and get a lot of bruises and infections.
Two other signs to watch for:
- A special, widespread type of neuroblastoma that happens only during the first few months often causes blue or purple bumps that look like small blueberries. That’s a sign the cancer has possibly spread to the skin. This is very treatable and often shrinks or goes away on its own.
- Neuroblastomas that release hormones may cause other symptoms, such as constant diarrhea, fever, high blood pressure, sweating, and reddening of the skin.
Neuroblastoma Diagnosis
Many neuroblastoma symptoms can look like more common conditions, which can make diagnosis difficult. "The biggest challenge is diagnosing it before it's advanced," Barbour says. "Some of the symptoms that children with neuroblastoma present with are very common things, like the child isn't eating well or acting achy and tired. Those are common with things like a cold or simple infection."
Your doctor will need to run lab tests, scans, and biopsies to be sure that your child has this rare cancer. These include:
- Blood and urine tests. These measure the levels of hormones in your blood that may be made by neuroblastomas.
- Imaging tests. These can help doctors figure out how far the cancer has spread.
- Ultrasound. This can look for tumors in your abdomen.
- X-rays. These can find cancer in the chest and bones.
- Other types of scans. You might get CT, PET, or MRI scans. These can help your doctor figure out where else the neuroblastoma might be or whether a treatment is working.
- Biopsy. A doctor can take a sample from a tumor or bone marrow. A lab will run a test on the sample to detect cancer.
- Meta-iodobenzylguanidine (MIBG) scan. This nuclear imaging test, which uses a radioactive substance called MIBG, helps doctors look at and diagnose tumors that start in neuroendocrine cells.
You might also get lab tests that measure your blood cell counts, liver and kidney function, and the balance of salts in your body.
Neuroblastoma is a cancer that can be treated. How much the cancer has spread is the most important factor in whether a child can be cured. The younger their age at diagnosis, the better their survival rate.
Neuroblastoma Staging
Doctors group neuroblastoma into three stages using the International Neuroblastoma Risk Group (INRG):
- Localized neuroblastoma (L1 or L2). This is when doctors find neuroblastoma in one part of the body, and it hasn't spread.
- Metastatic neuroblastoma (stage M). At this stage, neuroblastoma has spread to the bones, bone marrow, and other parts of the body.
- Stage MS neuroblastoma. Babies get this type of neuroblastoma. Although it mostly appears in one part of the body, some cancer cells have spread to the liver, skin, or bone marrow.
Neuroblastoma Treatment
The kind of treatment your child gets will depend on their risk group. Doctors figure out the risk group based on the following:
- The "stage" of the cancer (based on tumor size and whether it's spread)
- The age of your child
- Where the tumor started
- How the tumor is expected to respond to treatment
There are three risk groups: low, intermediate, and high. Low- and intermediate-risk neuroblastomas have a higher chance of being cured than high-risk ones.
The types of treatment your child might get include:
Observation. Doctors will closely watch your child for symptoms without giving treatment.
Surgery. This takes out a tumor and lymph nodes to test for cancer.
Chemotherapy. Your child gets rounds of chemicals to kill cancer cells or stop them from dividing. They'll take chemo as an IV or by mouth.
Radiation therapy. This uses high-energy beams, such as X-rays, to kill cancer cells. There are different ways of giving radiation therapy, including using a machine outside of the body, placing a radioactive substance into or near the cancer, or injecting into the body with a monoclonal antibody.
Stem cell transplant. Your doctor collects your child’s blood stem cells, uses chemotherapy to kill cancerous cells, and then injects the healthy cells back into their body.
Iodine 131-MIBG therapy. This treatment uses radioactive iodine given through an IV, which allows the radiation to reach the tumor cells directly. Doctors often use it for neuroblastoma that returns after the first treatment.
Targeted therapy. Doctors use drugs or other substances to block specific enzymes, proteins, or molecules that help cancer cells grow and spread.
Other drug therapies. Drugs such as difluoromethylornithine and isotretinoin (retinoid therapy) kill cancer cells or stop them from growing.
Newer therapies. These include immunotherapies, which work by signaling your child’s immune system to help fight cancer cells.
Can Neuroblastoma Be Prevented?
In some neuroblastoma cases, children inherit a genetic problem from their parents that raises their risk.
However, there are no other known causes of neuroblastoma.
Unlike many adult cancers, issues such as body weight, diet, exercise, and exposure to toxins and chemicals aren't thought to be linked to the disease.
Neuroblastoma Prognosis
Your child's neuroblastoma survival rate depends on a few things, including:
- Their age at diagnosis
- The tumor's structure
- If the tumor has spread beyond the original area or to the lymph nodes
Younger kids with low- or intermediate-risk neuroblastomas have a 90%-95% five-year survival rate. Older kids and those in the high-risk group have a 60% survival rate.
Takeaways
- Neuroblastoma is a rare cancer that usually affects very young children and begins in immature nerve cells, often near the adrenal glands above the kidneys.
- Genetic mutations in nerve cells before birth cause this type of cancer.
- Doctors use blood and urine tests, scans (such as ultrasound, X-ray, MRI), and biopsies to diagnose neuroblastoma and figure out how far it has spread.
- Treatments include observation, surgery, chemotherapy, radiation, stem cell transplants, and newer therapies, such as immunotherapy and targeted drugs.
Neuroblastoma FAQs
What's the survival rate for neuroblastoma?
Children with low- or intermediate-risk neuroblastoma have a five-year survival rate of 90%-95%. Older children and those classified as high-risk have a survival rate of 60%.
Can neuroblastoma be cured?
Usually, yes. With treatment, doctors can often cure this type of cancer, especially low- and intermediate-risk neuroblastomas in younger children.
Can neuroblastoma come back?
Yes. Sometimes, the condition doesn't respond to treatment (called refractory neuroblastoma) or returns after treatment (called recurrent neuroblastoma). The cancer can return to the same area of the body or a different one.
Can adults get neuroblastoma?
It's rare for adults to get neuroblastoma (more than 90% of cases happen in children under age 5), but it can happen.