What is Neuroblastoma? Symptoms, Causes, & Treatment

Neuroblastoma is a type of cancer that develops from immature nerve cells called neuroblasts. These cells are supposed to mature into normal nerve cells, but in neuroblastoma, they grow out of control and form tumors instead.

This cancer almost exclusively affects children, with most cases occurring before age 5. While the word "cancer" can feel overwhelming, it's important to know that many children with neuroblastoma respond well to treatment, especially when caught early.

What are the symptoms of neuroblastoma?

The symptoms of neuroblastoma can vary quite a bit depending on where the tumor grows and how large it becomes. Since this cancer can develop in different parts of your child's body, the signs might seem unrelated at first.

Here are the symptoms you might notice, grouped by where they commonly appear:

General symptoms that affect the whole body:

• Persistent fever that doesn't respond well to usual treatments
• Unexplained weight loss or poor appetite
• Fatigue or unusual tiredness that doesn't improve with rest
• Irritability or changes in your child's usual behavior
• Pale skin that might indicate anemia

Abdominal symptoms (since many tumors start in the belly area):

• A firm lump or swelling in the abdomen that you can feel
• Abdominal pain or discomfort
• Feeling full quickly during meals
• Constipation or changes in bowel habits

Chest-related symptoms:

• Persistent cough that doesn't seem to be from a cold
• Difficulty breathing or shortness of breath
• Chest pain or discomfort

Less common but important symptoms to watch for:

• Bone pain, especially in the legs, which might cause limping
• Bruising or small red spots on the skin
• Swollen lymph nodes
• Eye problems like dark circles, droopy eyelids, or pupils of different sizes
• High blood pressure
• Diarrhea that persists

These symptoms can develop gradually over weeks or months. Many of these signs can also be caused by much more common childhood illnesses, so try not to worry if you notice one or two. However, if several symptoms appear together or persist despite treatment, it's worth discussing with your pediatrician.

What are the types of neuroblastoma?

Doctors classify neuroblastoma in several ways to help determine the best treatment approach. The main way they categorize it is by risk level, which helps predict how the cancer might behave.

By risk level:

• Low-risk neuroblastoma: These tumors grow slowly and often respond very well to treatment, sometimes even shrinking on their own
• Intermediate-risk neuroblastoma: These require more intensive treatment but still have good outcomes with proper care
• High-risk neuroblastoma: These are more aggressive and need the most intensive treatment, but many children still do well with comprehensive care

By location in the body:

• Adrenal neuroblastoma: Starts in the adrenal glands above the kidneys (most common location)
• Abdominal neuroblastoma: Develops in nerve tissue in the belly area
• Thoracic neuroblastoma: Grows in the chest area
• Pelvic neuroblastoma: Forms in the pelvic region (less common)

Your child's medical team will use these classifications along with other factors like age and specific tumor characteristics to create the most effective treatment plan. Each type responds differently to treatment, which is why this classification system is so helpful.

What causes neuroblastoma?

The exact cause of neuroblastoma isn't fully understood, but researchers believe it happens when something goes wrong during normal fetal development. During pregnancy, special cells called neural crest cells are supposed to develop into mature nerve cells, but sometimes this process doesn't complete properly.

Most cases of neuroblastoma occur randomly, meaning there's nothing parents did or didn't do that caused it. This is called a "sporadic" cancer, and it accounts for about 98% of all neuroblastoma cases.

In rare cases (about 1-2% of the time), neuroblastoma can be inherited, meaning it's passed down through families. This happens when there are changes in specific genes that control how nerve cells develop. Families with hereditary neuroblastoma often have multiple family members affected and may develop the cancer at younger ages.

Some factors that researchers are studying include exposure to certain chemicals during pregnancy, but no definitive environmental causes have been proven. The important thing to understand is that neuroblastoma is not caused by anything you could have prevented or controlled.

When to see a doctor for neuroblastoma?

You should contact your child's pediatrician if you notice any combination of the symptoms mentioned earlier, especially if they persist for more than a week or two. While most of these symptoms are usually caused by common childhood illnesses, it's always better to have them checked.

Seek medical attention more urgently if your child has:

• A firm lump in the abdomen that you can feel
• Persistent fever along with weight loss
• Severe abdominal pain
• Difficulty breathing
• Severe bone pain or inability to walk normally
• Any eye changes like droopy eyelids or different pupil sizes

Trust your instincts as a parent. If something feels "off" about your child's health or behavior, don't hesitate to call your doctor. Pediatricians are used to concerned parents and would much rather check something that turns out to be minor than miss something important.

Early detection can make a significant difference in treatment outcomes, so being alert to changes in your child's health is one of the most valuable things you can do.

What are the risk factors for neuroblastoma?

Unlike many adult cancers, neuroblastoma doesn't have many clear risk factors that parents can control. Most children who develop this cancer have no known risk factors at all.

The main risk factors that doctors have identified include:

Age: This is the most significant risk factor. About 90% of neuroblastoma cases occur in children under 5 years old, with the highest risk being in the first year of life. The risk decreases significantly as children get older.

Gender: Boys are slightly more likely to develop neuroblastoma than girls, but the difference is small.

Family history: In very rare cases (1-2% of all cases), neuroblastoma can run in families. Children with a parent or sibling who had neuroblastoma have a higher risk, but this accounts for a tiny fraction of cases.

Genetic conditions: Some rare genetic disorders can increase the risk slightly, but these conditions themselves are extremely uncommon.

It's crucial to understand that most children with neuroblastoma have none of these risk factors. The cancer typically develops randomly during fetal development, and there's nothing parents could have done differently to prevent it. This isn't caused by diet, lifestyle, or environmental factors that you could control.

What are the possible complications of neuroblastoma?

Complications from neuroblastoma can arise from the tumor itself or from the treatments used to fight it. Understanding these possibilities can help you know what to watch for and feel more prepared for your child's care journey.

Complications from the tumor:

• Compression of nearby organs: As tumors grow, they can press on kidneys, lungs, or other organs, affecting their function
• Spinal cord compression: If the tumor grows near the spine, it can press on the spinal cord, potentially causing weakness or paralysis
• Hormone-related problems: Some tumors release hormones that can cause high blood pressure or persistent diarrhea
• Spread to other parts of the body: Advanced neuroblastoma can spread to bones, bone marrow, liver, or lymph nodes

Treatment-related complications:

• Infection risk: Chemotherapy can lower white blood cell counts, making infections more likely
• Hearing problems: Some chemotherapy drugs can affect hearing
• Growth and development changes: Intensive treatments can sometimes affect normal growth patterns
• Secondary cancers: Very rarely, treatment can increase the risk of developing other cancers later in life

While this list might seem overwhelming, remember that your child's medical team is experienced in preventing and managing these complications. Many children go through treatment without experiencing serious complications, and those who do often recover completely with proper care.

Modern treatment protocols are designed to be as effective as possible while minimizing these risks. Your medical team will monitor your child closely throughout treatment to catch and address any complications early.

How is neuroblastoma diagnosed?

Diagnosing neuroblastoma involves several steps, and your child's medical team will work systematically to get a complete picture. The process typically starts with your pediatrician's examination and then moves to specialized tests.

Initial evaluation:

Your doctor will start with a thorough physical exam, feeling for any lumps or swelling, especially in the abdomen. They'll ask detailed questions about your child's symptoms and when they started.

Imaging tests:

• Ultrasound: Often the first imaging test, especially for abdominal masses
• CT scan: Provides detailed pictures of the inside of the body to show tumor size and location
• MRI: Gives even more detailed images, particularly useful for tumors near the spine
• MIBG scan: A special nuclear medicine test that can detect neuroblastoma cells throughout the body

Laboratory tests:

• Urine tests: Check for specific chemicals that neuroblastoma cells often produce
• Blood tests: Look for tumor markers and assess overall health
• Bone marrow biopsy: Checks if cancer has spread to the bone marrow

Tissue biopsy:

The definitive diagnosis requires examining actual tumor tissue under a microscope. This is usually done through a small surgical procedure to remove a piece of the tumor.

The entire diagnostic process typically takes several days to a few weeks. While waiting for results can be stressful, this thorough evaluation helps ensure your child gets exactly the right treatment for their specific situation.

What is the treatment for neuroblastoma?

Treatment for neuroblastoma is highly individualized based on your child's age, the tumor's characteristics, and how far it has spread. The good news is that treatment approaches have improved dramatically over the years, and many children with neuroblastoma go on to live healthy, normal lives.

Surgery:

Surgery is often the first treatment, especially for tumors that haven't spread. The goal is to remove as much of the tumor as possible while protecting nearby organs and structures. Sometimes complete removal isn't possible initially, so surgery might be done after other treatments shrink the tumor.

Chemotherapy:

These are powerful medicines that target cancer cells throughout the body. Your child will likely receive several different chemotherapy drugs over a period of months. The treatment is usually given through a central line (a special IV) to make it more comfortable.

Radiation therapy:

High-energy beams are used to destroy cancer cells in specific areas. This treatment is carefully planned to target the tumor while protecting healthy tissue. Not all children with neuroblastoma need radiation therapy.

Stem cell transplant:

For high-risk cases, doctors might recommend collecting your child's healthy stem cells before giving very high doses of chemotherapy, then returning the stem cells to help rebuild the immune system.

Immunotherapy:

These newer treatments help your child's immune system recognize and fight cancer cells more effectively. This has become an important part of treatment for many children with neuroblastoma.

Targeted therapy:

These medications target specific features of cancer cells while leaving normal cells mostly alone.

Treatment typically lasts 12-18 months, though this varies considerably. Your child's oncology team will create a detailed treatment plan and adjust it as needed based on how well your child responds.

How to provide home care during neuroblastoma treatment?

Caring for your child at home during neuroblastoma treatment involves managing both the physical and emotional aspects of their journey. Your medical team will provide specific instructions, but here are some general guidelines that can help.

Managing treatment side effects:

• Infection prevention: Keep your child away from crowds and sick people when their immune system is low
• Nutrition support: Offer small, frequent meals and work with a nutritionist if appetite becomes a problem
• Hydration: Encourage plenty of fluids unless your doctor advises otherwise
• Mouth care: Gentle brushing and special mouth rinses can prevent painful mouth sores
• Skin care: Keep skin clean and moisturized, especially around the central line site

Monitoring for complications:

Keep a daily log of your child's temperature, appetite, and energy levels. Contact your medical team immediately if you notice fever, signs of infection, unusual bleeding, or severe nausea and vomiting.

Emotional support:

Maintain as much normalcy as possible in your child's routine. Continue with favorite activities when they feel well enough. Many hospitals have child life specialists who can provide resources for helping children cope with treatment.

School and social connections:

Work with your child's school to arrange for continued education during treatment. Many children can continue some schoolwork from home or return to school between treatment cycles.

Remember that you don't have to manage everything alone. Your medical team, social workers, and other families going through similar experiences can provide tremendous support and practical advice.

How should you prepare for your doctor appointments?

Being well-prepared for appointments can help you make the most of your time with your child's medical team and ensure you get all the information you need. Here's how to get ready for both initial consultations and ongoing care visits.

Before the appointment:

• Write down all symptoms you've noticed, including when they started and how they've changed
• List all medications, vitamins, and supplements your child takes
• Prepare a list of questions - write them down so you don't forget in the moment
• Gather any previous medical records or test results
• Consider bringing a family member or friend for support and to help remember information

Questions you might want to ask:

• What type and stage of neuroblastoma does my child have?
• What are the treatment options and what do you recommend?
• What are the potential side effects of treatment?
• How will we know if the treatment is working?
• What should I watch for at home?
• Are there any activities my child should avoid?
• What support services are available for our family?

During the appointment:

Don't hesitate to ask for clarification if something isn't clear. Take notes or ask if you can record important parts of the conversation. Make sure you understand next steps before leaving.

What to bring:

Bring your insurance cards, a list of current medications, comfort items for your child, snacks, and entertainment for potentially long visits.

Remember that your medical team wants to partner with you in your child's care. They expect questions and want you to feel informed and confident about the treatment plan.

What's the key takeaway about neuroblastoma?

Neuroblastoma is a childhood cancer that, while serious, has seen tremendous improvements in treatment outcomes over the past decades. Many children with neuroblastoma go on to live healthy, normal lives after treatment.

The most important things to remember are that early detection makes a difference, treatment is highly individualized based on your child's specific situation, and you're not alone in this journey. Modern medical teams are experienced in treating neuroblastoma and supporting families through the process.

While the diagnosis can feel overwhelming, focus on taking things one step at a time. Your child's medical team will guide you through each phase of treatment and help you understand what to expect. Many families find that connecting with other families who have been through similar experiences provides valuable support and perspective.

Trust in your medical team, stay connected with your support network, and remember that children are remarkably resilient. With proper treatment and care, many children with neuroblastoma go on to thrive.