Now Know All about Childhood Neuroblastoma: Cause, Symptoms, Diagnosis, Treatment

Childhood neuroblastoma is a rare type of cancer that primarily affects children under the age of 5 and originates in immature nerve cells called neuroblasts. These cells are found in several areas of the body, primarily in the adrenal glands, which sit above the kidneys. Neuroblastoma can also develop in the nerve tissue of the spine, chest, abdomen, or neck. It accounts for about 6-10% of all childhood cancers and is the most common solid tumor found outside of the brain in children. The disease can be difficult to diagnose early because its symptoms are often mistaken for those of other common childhood conditions.

What is Childhood Neuroblastoma?

Neuroblastoma is a type of cancer that begins in neuroblasts, which are immature nerve cells that should mature into functioning nerve cells. These cells are part of the sympathetic nervous system, which controls bodily functions like heart rate and digestion. During normal development, neuroblasts become mature nerve cells or adrenal gland cells. However, in neuroblastoma, these cells grow uncontrollably and form tumors.

Neuroblastoma can vary in its behavior; some cases grow slowly, while others can grow and spread rapidly. It is most commonly diagnosed in the adrenal glands but can also form in nerve tissues along the spine, in the chest, or in the pelvis. (Source)

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Causes of Childhood Neuroblastoma

The exact cause of neuroblastoma is unknown, but a combination of genetic and environmental factors is believed to play a role. Neuroblastoma occurs when genetic mutations cause normal neuroblasts to develop into cancerous cells.

• Genetic Mutations: Most cases of neuroblastoma are thought to occur sporadically due to random mutations in the cell’s DNA, which lead to uncontrolled cell growth. These mutations can arise early in life or before birth, but they are usually not inherited from parents.
• Genetic Syndromes: In a small percentage of cases (about 1-2%), neuroblastoma is linked to inherited mutations in the ALK or PHOX2B genes. Children with these hereditary mutations have a higher risk of developing neuroblastoma, often at a younger age, and with tumors occurring in multiple areas of the body. (Source)

How Does Neuroblastoma Spread?

Neuroblastoma can spread from its original site to other parts of the body through a process known as metastasis. The most common sites for the cancer to spread are:

• Lymph Nodes: Cancer cells can spread to the lymph nodes, which are part of the immune system.
• Bone Marrow: The cancer can infiltrate the bone marrow, affecting blood cell production.
• Bones: Neuroblastoma frequently spreads to the bones, causing bone pain and fractures.
• Liver: In infants, neuroblastoma can spread to the liver.
• Skin: The spread of neuroblastoma to the skin may cause bluish lumps.

Neuroblastoma can metastasize before it is even diagnosed, making it harder to treat in some cases.

Symptoms of Childhood Neuroblastoma

Symptoms of neuroblastoma depend on the location of the tumor and whether it has spread to other parts of the body. Early symptoms may be vague and nonspecific, often resembling common childhood illnesses. Some common signs and symptoms include:

• Abdominal swelling or mass: Often painless, but can cause a visible bulge or discomfort.
• Bone pain: If the cancer has spread to the bones, children may experience bone pain or fractures.
• Fever: Persistent or recurring fever without a clear cause.
• Loss of appetite or weight loss: Children may lose interest in eating, leading to weight loss.
• Weakness or paralysis: If the tumor compresses the spinal cord, it may result in weakness or paralysis in certain body parts.
• Bruising around the eyes: When neuroblastoma spreads to the bones around the eyes, it can cause dark circles or bruising.
• Difficulty breathing: Tumors in the chest can compress the airways, making breathing difficult.
• High blood pressure or rapid heartbeat: Caused by tumors in the adrenal glands that release hormones.

These symptoms can be caused by conditions other than neuroblastoma, so it is important to seek medical attention for a proper diagnosis. (Source)

Diagnosis of Childhood Neuroblastoma

Neuroblastoma is diagnosed using a combination of tests that help identify the location of the tumor, determine the stage of the cancer, and check whether it has spread to other parts of the body. Diagnostic tests include:

1. Physical Examination and Medical History: The doctor will perform a physical exam and ask about the child’s symptoms and medical history.
2. Imaging Tests: Ultrasound, CT scans, MRI, and MIBG scans are used to identify the tumor and check for metastasis.
3. Urine or Blood Tests: Children with neuroblastoma often have elevated levels of certain chemicals, like catecholamines, that can be detected through urine or blood tests.
4. Biopsy: A sample of the tumor is taken and analyzed to confirm the diagnosis of neuroblastoma.
5. Bone Marrow Aspiration and Biopsy: If the cancer has spread, a bone marrow biopsy may be done to check for cancer cells in the bone marrow. (Source)

Treatment of Childhood Neuroblastoma

Treatment options for neuroblastoma depend on the child’s age, the stage of cancer, and the tumor’s genetic features. Neuroblastoma is classified into low-risk, intermediate-risk, and high-risk categories, which guide treatment.

1. Surgery: In some cases, especially for low-risk neuroblastoma, surgery to remove the tumor may be the only treatment needed.
2. Chemotherapy: Chemotherapy uses drugs to kill cancer cells and is commonly used to shrink tumors before surgery or to treat high-risk neuroblastoma.
3. Radiation Therapy: Radiation may be used if the tumor cannot be completely removed with surgery or if the cancer has spread to other areas of the body.
4. Stem Cell Transplant: High-risk neuroblastoma patients may undergo a stem cell transplant following high-dose chemotherapy to replace damaged bone marrow with healthy cells.
5. Immunotherapy: For high-risk neuroblastoma, immunotherapy uses the body’s immune system to target cancer cells, often combined with other treatments.
6. Targeted Therapy: Certain drugs, like ALKS inhibitors, target specific genetic mutations in neuroblastoma cells. (Source)

Can Childhood Neuroblastoma Be Prevented?

There are currently no known ways to prevent neuroblastoma, as most cases occur sporadically without a clear environmental cause. Preventive measures cannot be recommended because the majority of cases are due to random genetic mutations.

Is Childhood Neuroblastoma Hereditary?

While most cases of neuroblastoma occur randomly, about 1-2% of cases are linked to inherited genetic mutations. Children with a family history of neuroblastoma or certain genetic syndromes, such as mutations in the ALK gene, have an increased risk. However, most children with neuroblastoma do not have a family history of the disease. (Source)

Can Childhood Neuroblastoma Be Cured?

The prognosis for neuroblastoma varies depending on the risk category:

• Low-risk neuroblastoma: Many children with low-risk neuroblastoma are cured with surgery or minimal treatment. Survival rates are very high, around 90-95%.
• Intermediate-risk neuroblastoma: With chemotherapy and other treatments, the survival rate for intermediate-risk neuroblastoma is around 70-90%.
• High-risk neuroblastoma: High-risk neuroblastoma is more difficult to treat and has a lower survival rate, around 50%. However, aggressive treatment, including stem cell transplants and immunotherapy, has improved outcomes. (Source)

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Conclusion

Childhood neuroblastoma is a rare but serious cancer that can vary in its behavior. Early diagnosis and treatment are essential for improving outcomes. While the exact cause is still unclear, advancements in medical research have led to improved treatments and survival rates, especially for low- and intermediate-risk cases. For high-risk patients, emerging therapies like immunotherapy and stem cell transplants offer hope for better long-term outcomes.