Neuroblastoma - Early Signs, Risk Factors, Diagnosis, and Treatment Explained

Neuroblastoma is a rare type of cancer that most often affects young children, developing from immature nerve cells found in the adrenal glands, spine, or other areas of the body. Because its symptoms—such as abdominal pain, lumps, or changes in behavior—can be similar to common childhood illnesses, understanding the condition is especially important. This comprehensive guide provides clear, compassionate, and easy-to-understand information about neuroblastoma—covering its symptoms, how it is diagnosed, treatment options, and what to expect throughout the journey. Our goal is to help you feel informed, supported, and empowered to face this condition with confidence and hope.

What Is Neuroblastoma?

Neuroblastoma is a rare cancer that starts in very early nerve cells called neuroblasts. These neuroblasts are a part of the nervous system that controls involuntary functions like heart rate, blood pressure, and digestion. In a healthy child, these neuroblasts mature into functioning nerve cells. In neuroblastoma, they multiply uncontrollably to form a solid tumor.

This cancer is almost exclusively found in infants and young children. It is the most common cancer in infants and the most common extracranial (outside the brain) solid tumor in childhood. Neuroblastoma most often begins in the adrenal glands, which are located on top of the kidneys, but it can also start in nerve tissue in the neck, chest, or spine. While it is a serious disease, recent advances in treatment have dramatically improved the prognosis for many children with neuroblastoma.

Types of Neuroblastoma

Neuroblastoma is a single disease, but it has a very wide range of behaviors. It can be classified based on its risk group, which is a key factor in determining the best treatment plan and predicting the long-term outlook.

• Low-Risk Neuroblastoma: These tumors are often localized (confined to one area) and have a very high chance of a cure. In some cases, low-risk tumors can even regress (shrink or disappear) on their own without any treatment.
• Intermediate-Risk Neuroblastoma: These tumors are also localized but have a higher risk of recurrence. They require a moderate course of chemotherapy in addition to surgery.
• High-Risk Neuroblastoma: These tumors are the most aggressive and have a high potential to metastasize (spread) to distant parts of the body, such as the bones, bone marrow, and liver. High-risk neuroblastoma requires very intensive and aggressive treatment.

Doctors also classify neuroblastoma based on its location and how far it has spread, from Stage L1 (localized and easily removable with surgery) to Stage M (metastatic and spread to distant organs).

What Are the Causes and Risk Factors for Neuroblastoma?

In the vast majority of cases, the exact cause of neuroblastoma is unknown. It is not caused by a single factor, but rather a specific genetic mutation that occurs during a child's lifetime.

• Genetic Change: In most cases, neuroblastoma is the result of a random genetic mutation in the neuroblast cells that happens during a child's development, sometimes even before birth. This genetic change is not inherited.
• Hereditary Neuroblastoma: In very rare cases (1-2%), neuroblastoma can be inherited. This is caused by a gene mutation that is passed down from a parent to a child. Children with hereditary neuroblastoma are more likely to have tumors in multiple places.
• Age: Neuroblastoma is a disease of early childhood, with the average age of diagnosis being around 17 months. Most cases are diagnosed in children under the age of 5.

It is important to remember that most children with neuroblastoma have no known risk factors, and parents should not blame themselves for their child's diagnosis.

What Are the Symptoms of Neuroblastoma?

The symptoms of neuroblastoma can be vague and are often mistaken for other common childhood illnesses. However, if you notice these symptoms, especially if they are persistent or getting worse, it is important to see a doctor for a proper evaluation. The symptoms depend on the tumor's location, size, and how far it has spread.

Common Early Signs:

• A Lump or Swelling: The most common sign of neuroblastoma is a painless lump or swelling, often in the abdomen, neck, or chest.
• Changes in the Eyes: A tumor near the eyes can cause bulging eyes, dark circles around the eyes, or a drooping eyelid.
• Bone Pain: A persistent bone pain that may cause a child to limp or refuse to walk.
• Swollen Abdomen: A large tumor in the abdomen can cause swelling and a feeling of fullness.
• Painful, Bluish Lumps Under the Skin: In infants, small, bluish lumps may appear under the skin.

Advanced Symptoms:

As the cancer progresses and spreads, other symptoms may appear:

• Unexplained Fever: A persistent, unexplained fever.
• Unexplained Weight Loss: Losing weight without trying.
• Fatigue: Feeling unusually tired or a general lack of energy.
• Diarrhea or High Blood Pressure: In some cases, neuroblastoma cells can produce hormones called catecholamines that can cause these symptoms.
• Neurological Symptoms: If the tumor presses on the spinal cord, it can cause weakness, numbness, or paralysis in the legs or arms.

If you have a gut feeling that something is wrong with your child, do not hesitate to seek medical attention.

How Is Neuroblastoma Diagnosed?

Diagnosing neuroblastoma requires a series of specialized tests to confirm the presence of a tumor, determine its type and location, and see if it has spread. The process often begins when a parent or a pediatrician notices a suspicious symptom.

Diagnostic Steps and Tests:

1. Physical and Neurological Exam: Your child's doctor will perform a thorough physical and neurological exam, checking for things like coordination, reflexes, and a lump.

2. Imaging Scans: Imaging scans are the most important tool for finding a neuroblastoma.

• Ultrasound: A non-invasive scan that can be used to see a tumor in the abdomen.
• MRI (Magnetic Resonance Imaging): An MRI provides very detailed images of the tumor and its relationship to nearby nerves, blood vessels, and the spinal cord.
• CT (Computed Tomography) Scan: A CT scan can provide a detailed look at the tumor and surrounding structures.
• MIBG Scan: This is a very important scan for neuroblastoma. A small amount of a radioactive substance is injected into the body. This substance is absorbed by neuroblastoma cells, which makes them light up on the scan.

3. Blood and Urine Tests: Blood and urine tests are crucial for diagnosis and monitoring. Neuroblastoma cells often produce high levels of hormones called catecholamines. These can be detected in a urine test.

4. Biopsy (The Definitive Test): A biopsy is the only way to definitively diagnose neuroblastoma. A small tissue sample from the suspicious area is removed and sent to a lab for examination.

5. Bone Marrow Aspiration and Biopsy: A small sample of bone marrow is removed from the hip bone to check if the cancer has spread to the bone marrow. This is an important step for staging.

Staging and Grading of Neuroblastoma

The staging of neuroblastoma is a key factor in determining the treatment plan and predicting a patient's prognosis. The stage describes how far the cancer has spread. The most common staging system is a four-stage system with additional letters to provide more information.

• Localized Neuroblastoma (L1 and L2): The cancer is confined to one area.
• Metastatic Neuroblastoma (M): The cancer has spread to distant lymph nodes or other organs.
• Stage MS: This is a unique stage for infants under 18 months. It means the cancer has spread to the skin, liver, or bone marrow, but it often has a good prognosis and can spontaneously regress.

In addition to the stage, doctors use a risk group system (low, intermediate, or high risk) to help guide treatment.

What Are the Treatment Options for Neuroblastoma?

The treatment plan for neuroblastoma is highly personalized and depends on the tumor's risk group and the child's age and overall health. A multidisciplinary team of specialists, including a pediatric neurosurgeon, a pediatric oncologist, and a radiation oncologist, will work together to create a comprehensive treatment plan.

1. Surgery

Surgery is the primary treatment for low-risk and some intermediate-risk neuroblastomas. The goal is to remove the entire tumor. If the tumor cannot be completely removed, a surgeon may remove as much as possible to relieve pressure on nearby organs.

2. Medical Treatment (Chemotherapy, Targeted Therapy, Immunotherapy)

• Chemotherapy: Chemotherapy is the primary treatment for high-risk neuroblastoma. It is given in intensive cycles to kill cancer cells throughout the body.
• High-Dose Chemotherapy and Stem Cell Transplant: For high-risk neuroblastoma, a child may receive very high doses of chemotherapy to kill all the cancer cells. Because this also kills healthy blood-forming cells, the child will then receive their own healthy stem cells (autologous stem cell transplant) that were collected earlier to restore their blood cell production.
• Targeted Therapy: Targeted therapies are designed to attack specific cancer cells with less harm to normal cells. Retinoids are a type of targeted therapy that are often used after intensive treatment for high-risk neuroblastoma to prevent the cancer from coming back.
• Immunotherapy: Immunotherapy is a major advance in neuroblastoma treatment. A specific type of immunotherapy, an antibody called dinutuximab, is used for high-risk neuroblastoma and has been shown to improve survival.

3. Radiation Therapy

Radiation therapy uses high-energy rays to kill cancer cells. It is often used for intermediate and high-risk neuroblastoma, especially to treat the primary tumor site after a stem cell transplant or to relieve pain from a tumor.

4. Proton Therapy

Proton therapy is a highly advanced form of radiation that uses proton beams instead of X-rays. Because protons can be precisely aimed to deliver a high dose of radiation directly to the tumor while sparing nearby healthy tissues and organs, it may be a good option for neuroblastomas located near sensitive structures.

Prognosis and Survival Rates for Neuroblastoma

The prognosis (the likely outcome of the disease) for neuroblastoma has improved dramatically in recent decades. The outlook depends on the tumor's risk group and the child's age.

Prognostic Factors: The most important factors affecting prognosis are the risk group (low, intermediate, or high), the child's age at diagnosis, and the specific genetic features of the tumor.

Survival Rates: The survival rate for neuroblastoma varies widely.

• Low-Risk: The 5-year survival rate is over 95%.
• Intermediate-Risk: The 5-year survival rate is around 90-95%.
• High-Risk: The 5-year survival rate is around 50%, but with new treatments, the prognosis is improving.

It is important to discuss your child's specific prognosis with your oncology team, as they can provide a more accurate picture based on the unique characteristics of the tumor.

Screening and Prevention of Neuroblastoma

There are no routine screening tests for neuroblastoma in the general population. The best way to ensure early detection is for parents and pediatricians to be aware of the symptoms.

Prevention Strategies:

• Since the causes are not fully known, there is no known way to prevent neuroblastoma.
• If your child has a family history of neuroblastoma, it is important to talk to a genetic counselor.

For International Patients: Your Seamless Journey to Apollo Hospitals

Apollo Hospitals is a leading medical destination for international patients seeking high-quality and affordable cancer care. Our dedicated International Patient Services team is here to ensure your entire experience is as smooth and comfortable as possible. We have extensive experience treating children with complex conditions, including neuroblastoma.

Our Services for International Patients Include:

• Travel and Visa Assistance: We will provide you with a visa invitation letter and help with travel arrangements.
• Airport Transfers: We will arrange for a car to pick you up from the airport.
• Personalized Care: A dedicated patient coordinator will be your single point of contact, assisting with hospital admission, language interpretation, and any other needs you may have.
• Accommodation: We can assist you with booking suitable accommodation for you and your family near the hospital.
• Child Life Specialists: Our team includes child life specialists who can help your child and your family cope with the emotional and psychological challenges of a cancer diagnosis.
• Post-Treatment Follow-up: We will stay in touch with you after your return home to ensure a smooth recovery.

Frequently Asked Questions (FAQs) About Neuroblastoma

Q1: Is Neuroblastoma curable?

A: Yes, neuroblastoma is curable. The cure rate for low and intermediate-risk neuroblastoma is very high. While high-risk neuroblastoma is more difficult to cure, a combination of intensive treatments can lead to a long-lasting remission or a complete cure.

Q2: What is the survival rate for Neuroblastoma?

A: The survival rate varies significantly by the risk group. The 5-year survival rate for low-risk neuroblastoma is over 95%. For high-risk neuroblastoma, it is around 50%, but new treatments are improving these numbers. Your doctor can provide a more accurate prognosis based on your child's specific case.

Q3: What are the side effects of Neuroblastoma treatment?

A: Side effects vary with the type of treatment. Chemotherapy can cause fatigue, nausea, hair loss, and a weakened immune system. Surgery can cause pain and a long recovery time. Radiation may cause skin irritation. Your medical team will work closely with you to manage these side effects.

Q4: Can Neuroblastoma come back (recurrence)?

A: Yes, there is a risk of recurrence, especially for high-risk tumors. This is why regular follow-up appointments and monitoring are crucial for early detection of any relapse.

Q5: What is the typical recovery time after Neuroblastoma treatment?

A: Recovery time depends on the complexity of the surgery and the intensity of the chemotherapy. For high-risk neuroblastoma, the recovery from each cycle of chemotherapy can take a few weeks. The recovery from a stem cell transplant is much longer, often taking several months or more. Your medical team will provide a detailed recovery plan.

Q6: Can a baby be born with neuroblastoma?

A: Yes. In very rare cases, neuroblastoma can be found in a fetus during a prenatal ultrasound.

Q7: Can neuroblastoma be a "growing pain"?

A: No. While neuroblastoma bone pain can be mistaken for growing pains, it is important to know that neuroblastoma pain is persistent, often gets worse at night, and does not go away. Any persistent pain should be checked by a doctor.

Q8: What kind of support is available for families?

A: There are many support organizations for families of children with cancer. Many hospitals also have social workers and child life specialists who can provide emotional support and guidance.

Q9: Is there any way to prevent neuroblastoma?

A: No. Because the cause is a random genetic change, there is no known way to prevent neuroblastoma.