Neuroblastoma: Meaning, Causes, Symptoms, Treatment

Written by Medicover Team and Medically Reviewed by Dr Rahul Gosavi , Pediatricians

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Neuroblastoma is a rare type of childhood cancer that develops from immature nerve cells (neuroblasts). It most often starts in the adrenal glands on top of the kidneys but can also occur in the chest, abdomen, spine, pelvis or other areas of the body.

This cancer mainly affects infants and young children under the age of 5 and is very rare in older children or adults. Neuroblastoma can behave in different ways, sometimes it grows slowly and may even shrink on its own, while in other cases it spreads quickly and requires urgent treatment.

Types of Neuroblastoma

Doctors classify Neuroblastoma based on how the tumor behaves and whether it has spread. The main types include:

• Localized Neuroblastoma: The cancer is found in one area and has not spread. It is usually easier to treat and has a better outcome.
• Regional Neuroblastoma: The tumor has spread to nearby tissues or lymph nodes but has not reached distant parts of the body.
• Metastatic Neuroblastoma: The cancer has spread to distant organs such as the liver, bones, or bone marrow. This type is more aggressive and needs intensive treatment.
• INSS Staging: Categorizes neuroblastoma from Stage 1 (localized) to Stage 4 (widespread metastasis), with specific criteria like tumor size and lymph node involvement.
• Histopathological Classification: Based on tumor cell appearance under a microscope, predicting behavior and treatment response includes favorable and unfavorable histology subtypes.

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Stages of Neuroblastoma

Doctors use staging to describe how much the cancer has grown and spread. The stages of Neuroblastoma are:

• Stage 1: The tumor is only in one area and can be completely removed with surgery.
• Stage 2A: The tumor is in one area but cannot be fully removed with surgery. It has not spread to nearby lymph nodes.
• Stage 2B: The tumor may or may not be fully removed and it has spread to nearby lymph nodes on the same side of the body.
• Stage 3: The tumor cannot be removed and has spread to the opposite side of the body or nearby lymph nodes.
• Stage 4: The cancer has spread to distant organs like bones, bone marrow, liver or skin.
• Stage 4S (special case): Found only in infants under 1 year old. The tumor spreads to the liver, skin or bone marrow, but often behaves less aggressively and can shrink on its own.

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Neuroblastoma Symptoms

The signs of Neuroblastoma depend on where the tumor is located and whether it has spread. Some common symptoms include:

• Abdomen: A swollen belly, pain or a lump that can be felt.
• Chest:Breathing problems, chest pain or coughing.
• Bones: Bone pain, limping or difficulty walking if cancer spreads to the bones.
• Spinal cord: Weakness, numbness or trouble moving parts of the body.
• Eyes: Bulging eyes, dark circles under the eyes or vision problems.
• General symptoms: Fever, weight loss, tiredness or high blood pressure.

Because these symptoms can look like other childhood illnesses, it's important to get medical advice if they last or worsen.

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Causes of Neuroblastoma

The exact cause of Neuroblastoma is not fully understood. It happens when immature nerve cells (neuroblasts) grow out of control and form a tumor. In most cases, this occurs by chance and is not inherited.

But some possible causes include:

• Genetic changes (mutations): Certain genes that control cell growth may stop working properly, leading to cancer.
• Problems in early development: Sometimes, nerve cells in the baby's body don't mature the way they should during pregnancy.
• Inherited changes (rare): In a few families, gene changes passed from parents to children may cause Neuroblastoma.

Most cases, however, happen by chance and are not linked to anything the parents did or did not do.

Risk Factors of Neuroblastoma

• Age: Most cases are found in children under 5 years old.
• Genetics: A small number of children inherit gene changes that increase the risk.
• Family history: Rarely, Neuroblastoma runs in families if a close relative had the disease.
• Developmental factors: Problems during early growth of nerve cells may increase the risk.

While parents cannot usually prevent Neuroblastoma, early detection and treatment improve the chances of recovery.

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Complications of Neuroblastoma

Neuroblastoma can cause different problems depending on where the tumor is and if it spreads:

• Pain and swelling: Large tumors in the abdomen or chest may cause discomfort, bloating or breathing problems.
• Bone problems: If cancer spreads to the bones, it can cause pain, fractures or trouble walking.
• Spinal cord pressure: Tumors near the spine can press on nerves, leading to weakness, numbness or even paralysis.
• Eye problems: Tumors near the eyes may cause drooping eyelids, vision changes or dark circles.
• Infections and anemia: Chemotherapy and other treatments can weaken immunity and lower blood counts.
• Emotional stress: Both children and families may face anxiety, stress and emotional challenges.

With early diagnosis, proper treatment and ongoing care, many of these complications can be managed.

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When to See a Doctor for Neuroblastoma?

See a doctor right away if your child has:

• A lump or swelling in the abdomen, chest or neck that doesn't go away
• Constant pain in bones, back or legs
• Unexplained weight loss, tiredness or loss of appetite
• Vision changes, drooping eyelids or dark circles around the eyes
• Weakness, trouble walking or numbness in legs

Early medical care improves the chances of successful treatment and helps prevent serious complications.

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Diagnosis of Neuroblastoma

Doctors use several tests to find out if a child has Neuroblastoma and how far it has spread.

• Physical exam: The doctor checks for lumps, swelling or other unusual signs.
• Imaging tests: Ultrasound, CT scans or MRI are used to see the size and location of the tumor.
• MIBG scan: A special test that shows where Neuroblastoma cells are in the body.
• Blood and urine tests: These check for substances tumor markers, released by Neuroblastoma cells.
• Biopsy: A small piece of the tumor is removed and looked at under a microscope to confirm the diagnosis.
• Bone marrow tests: To check if cancer has spread to the bone marrow.

These tests help doctors decide the stage of the cancer and plan the best treatment.

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Neuroblastoma Treatment

Treatment for Neuroblastoma depends on the stage of the cancer, the child's age and how aggressive the tumor is. Doctors often use a mix of treatments to get the best results.

Surgery

• If the tumor is small and in one place, doctors may try to remove it completely with surgery.
• In some cases, only part of the tumor can be removed safely and other treatments are used afterward.
• Surgery helps reduce pressure on nearby organs and improves the chances of cure.

Chemotherapy

• Uses strong medicines to kill cancer cells or stop them from growing.
• May be given before surgery to shrink the tumor and make removal easier.
• In advanced stages, chemotherapy helps control cancer that has spread to other parts of the body.

Radiation Therapy

• High-energy rays are directed at the tumor to kill cancer cells.
• Often used when surgery or chemotherapy alone is not enough.
• Helps shrink tumors, relieve pain or treat areas where cancer has spread.

Immunotherapy

• Boosts the body's own immune system to fight Neuroblastoma cells.
• Special medicines called monoclonal antibodies attach to cancer cells, making it easier for the immune system to destroy them.
• This is usually used in high-risk cases after chemotherapy and surgery.

Stem Cell Transplant

• In some children with aggressive Neuroblastoma, doctors use very high doses of chemotherapy to destroy cancer cells.
• Since this also damages the bone marrow, healthy stem cells (from the child or a donor) are given back to rebuild new, healthy blood cells.

Supportive Care

• Along with cancer treatment, supportive care helps manage pain, side effects of treatment and emotional stress.
• Nutrition, physiotherapy and counseling play an important role in recovery and improving quality of life.

With the right treatment plan, many children can recover and live healthy lives, especially when Neuroblastoma is found early.

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Conclusion

Neuroblastoma is a rare but serious childhood cancer that starts in immature nerve cells, most often in the adrenal glands. It can behave differently in each child, sometimes growing slowly and other times spreading quickly. With early diagnosis, proper treatment and ongoing care, many children can recover and live healthy lives. Support from doctors, family and therapy is very important in helping both the child and family cope during treatment and beyond.

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