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Neuroblastoma - Cause, Stages, Diagnosis, Complications, Treatment and Prevention

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Neuroblastoma is a rare type of pediatric cancer that usually occurs in the immature nerve cells of babies and children. Read the article to know more.

Written by

Dr. Preetha. J

Medically reviewed by

Dr. Pawar Satyajit Jalinder

Published At April 22, 2022
Reviewed AtMay 6, 2024

What Is Neuroblastoma?

Neuroblastoma is an uncommon type of cancer that grows in the nerves. Children under the age of five are commonly affected, and it is less commonly seen in children above the age of 10. It affects the nervous system of the babies and the little children. They usually grow on the immature nerve tissues called the neuroblast. Neuroblastoma can occur in babies even before they are born.

What Could Cause Neuroblastoma?

Neuroblastoma can be formed due to the uncontrolled growth of the immature nerve tissues. Due to genetic mutation, the cells may grow out of control and divide abnormally. It usually affects the neuroblasts present in the adrenal glands on the top of the kidneys.

How Does a Neuroblastoma Patient Feel?

Patients with neuroblastoma may have symptoms that range from mild to severe, and they may vary depending on the tumor location, the child's age, and the stage of the disease. Before the signs and symptoms could appear, the tumor would have spread to other parts of the body.

Symptoms may include:

  • Lump or bump in the neck, pelvis, chest, or abdomen, or numerous lumps under the skin that may appear purple or blue.

  • Bulging eyes or darkish circles under the eyes, which may look like a black eye.

  • Constipation, diarrhea (watery and loose stool), upset stomach, or loss of appetite.

  • Cough, fatigue, and fever.

  • Bone pain occurs if cancer has spread to the bones.

  • Pale skin can be a sign of anemia (lack of enough healthy red blood cells in the bloodstream).

  • Painful and bloated belly.

  • Breathing difficulty (usually in young babies).

  • Weakness, a problem in movement, or insensibility in the legs and feet.

  • High blood pressure and a fast pulsation (tachycardia).

  • Horner's syndrome causes small pupils, droopy eyelids, and sweating on only one side of the face.

  • Pain in the bones, legs, or back.

  • Problems with coordination, balance, and movement.

  • Shortness of breath.

  • Uncontrollable eye movements.

  • Unexplained weight loss.

What Are the Four Stages of Neuroblastoma?

The different stages of neuroblastoma are:

Stage 1 - Surgical methods can entirely remove the tumor since it has not spread.

Stage 2A - In this stage, the tumor cannot be removed entirely by surgical methods even though it has not spread.

Stage 2B - The entire tumor cannot be removed by surgical methods even though it has not spread. The adjacent lymph nodes may contain some neuroblastoma cells.

Stage 3 - The tumor has started to spread.

  • 3A - Cancer has crossed the midline and has spread to the other sides of the body. Surgical methods cannot remove it.

  • 3B - Cancer has not spread to the other sides of the body except the lymph nodes.

  • 3C - The tumor is present in the middle of the body and starts growing on both sides directly or by the lymph nodes. It cannot be removed entirely by surgery.

Stage 4 - Cancer has spread to the body's distant parts like bones, lymph nodes, bone marrow, liver, skin, and other organs.

Stage 4S - It applies only to children under the age of one. Cancer has spread to the liver, skin, bone marrow, and lymph nodes but only on one side of the body, and it has not spread to the bones.

How to Prevent Neuroblastoma?

Many adult cancer risks can be prevented by changing their lifestyles, like quitting smoking and diet planning, but many cancers cannot be avoided in children. Neuroblastoma cannot be controlled by any means, but the following methods are helpful:

  • Close observation of newborns in a state of family history of neuroblastoma.

  • Early diagnosis and treatment.

  • Mothers taking Folic acid and prenatal neuroblastoma can reduce the risk of neuroblastoma in children.

  • Getting proper care from the doctor during pregnancy to ensure the health of the fetus.

How to Diagnose Neuroblastoma?

  • Physical Examination - The doctor may conduct a physical examination to check out any symptoms and ask questions about the child's habits and behaviors.

  • Urine and Blood Tests - These tests can indicate the cause of any signs and symptoms the child is experiencing. Urine tests are used to check the high levels of certain chemicals like catecholamines produced from the neuroblastoma cells.

  • Imaging Tests - Imaging tests can be used to explain a mass that can indicate a tumor and can be used to stage cancer. Imaging tests may include an X-ray, ultrasound, magnetic resonance imaging (MRI), computerized tomography (CT) scan, and metaiodobenzylguanidine (MIBG) scan.

  • Biopsy of Tissues - Removing a sample of tissue for testing may be recommended by the doctor for laboratory testing. Specialized tests on the tissue sample can reveal the types of cells involved in the cancer cells' tumor and specific genetic characteristics. This information helps the doctor to prepare an individualized treatment plan.

  • Biopsy of Bone Marrow - Removing a bone marrow sample for testing may be recommended by the doctor by aspiration procedures to detect if neuroblastoma has spread to the bone marrow (the spongy substance inside the largest bones where blood cells are produced). Bone marrow is removed for testing by inserting a needle into the hipbone or lower back of the child to draw out the marrow.

How to Treat Neuroblastoma?

The treatment plan for neuroblastoma is based on factors like:

  • Age of the child.

  • Stage of cancer.

  • Whether the abnormalities are on the genes or chromosomes.

  • Types of cells involved.

Treatment options for neuroblastoma include:

  • Surgery.

  • Chemotherapy.

  • Radiation therapy.

  • Bone-marrow transplant.

  • Immunotherapy.

  • Retinoid therapy for neuroblastoma.

  • High-dosage radiation therapy or chemotherapy.

  • Stem cell transplant for neuroblastoma.

What Are the Complications of Neuroblastoma?

  • Paralysis due to compression of the spinal cord.

  • Hormonal imbalance due to the hormone-secreting neuroblastoma cells.

  • Metastasis to the other organs.

How Parents Can Help Children Deal With Neuroblastoma?

Parents of children diagnosed with neuroblastoma often feel overwhelmed, and the treatment often sounds scary to them. They mostly struggle with how to tell their child about this condition, how much to tell them about their cancer, and many more such questions arise, but they have no answer to it. However, most doctors suggest that one should be honest in revealing things to their children and tell them all the necessary details they should know about their current health status. Also, parents should make the child understand that the condition did not occur because of something the child has done or has not done.

The treatment of neuroblastoma can be explained to the child by breaking the process into small steps as parents can tell them they need to visit multiple doctors, and will get their whole body pictures clicked by a special machine, and they will also have to undergo an operation, and when they will wake up parents will be in front of them.

Sometimes, the patient's attendants feel helpless; however, they can be the biggest support to their child and also to the doctors treating the patient. Parents, too, need emotional support, and they are not alone. Doctors, social workers, and nurses all help them get through this situation and stay strong for their children.

Conclusion

Neuroblastoma is a type of cancer that develops in early cells of the nervous tissue, mostly involving the adrenal glands. The majority of the survivors have long-term complications due to the treatment, and relapse of the disease after initial treatment is also common. One can also consult an online doctor for more information regarding this disease and its treatment.

Frequently Asked Questions

1.

How to Diagnose Neuroblastoma?

The various ways of diagnosing neuroblastoma include:
Physical examination.
Blood tests and urine tests.
Imaging tests.
Biopsy (removal of a small amount of tissue for examination under a microscope).
Genetic studies. Tests of neuroblastoma cell DNA are used to find a change in the oncogene.
Bone marrow aspiration and biopsy.

2.

What Do You Mean by Neuroblastoma Cancer?

Neuroblastoma is a cancer that develops in immature nerve cells. It usually arises in and around one of the adrenal glands. But it can also develop in the neck, chest, abdomen, or spine. Neuroblastoma occurs due to an inherited genetic mutation.

3.

How Prevalent Is Neuroblastoma?

Neuroblastoma is considered the most common cancer in babies younger than one year. Children less than 5 years of age are commonly affected by the incidence rate of 90 percent.

4.

How Fast Does Neuroblastoma Grow?

A few neuroblastomas may grow and spread rapidly, and the others may develop slowly. In some cases, the cancer cells may die and go away on their own, but the other requires treatment.

5.

How Is Neuroblastoma Diagnosed in Children?

The various tests used to diagnose neuroblastoma in children are:
Urine test to detect raised levels of a hormone called catecholamine.
Blood tests.
CT (computed tomography).
MRI (magnetic resonance imaging).
PET scans (positron emission tomography).
X-rays.
Bone scans.
Bone marrow tests.

6.

What Are the Chances of Survival From Neuroblastoma?

In the case of low-risk children with neuroblastoma, the five-year survival rate is 95 %. It is about 90 % to 95 % in intermediate-risk cases, and in high-risk patients, the chances are about 40 % to 50 %.

7.

Is Neuroblastoma Cancer a Fatal Condition?

Neuroblastoma cancer is a fatal condition because it is the most common cancer in babies and the third most common cancer next to leukemia and brain cancer. Therefore, it requires proper diagnosis and appropriate treatment.

8.

Can Neuroblastoma Cancer Be Cured?

Neuroblastoma cancer can be cured when diagnosed early and treated on time before cancer spreads to the other parts of the body. Low-risk and intermediate-risk neuroblastoma might regrow after chemotherapy or surgery, but it will usually cure these children, and the prognosis is also good.

9.

Can Neuroblastoma Cure on Its Own?

Neuroblastoma is considered to be the third most common cancer in children after brain cancer and leukemia. In some cases of neuroblastoma, the cancer cells will die and cure on their own, but in other cases, it will grow and spread rapidly or sometimes slowly; therefore, it requires treatment.

10.

Who Can Be Affected by Neuroblastoma?

Children younger than 5 years can be affected by neuroblastoma. It is rarely diagnosed in children over the age of 10 years. Babies between 12 months to 18 months will have a more aggressive form of neuroblastoma.

11.

Where Will Neuroblastoma Occur?

Neuroblastoma can occur in immature nerve cells found in several body areas, but it is commonly seen in the nerve cells present in and around the adrenal glands. The other areas where the neuroblastoma can be seen are the neck, chest, abdomen, or spine.

12.

Can Neuroblastoma Be Seen in Blood Tests?

As other cells in the body produce hormones, the sympathetic nerve cells produce a certain hormone called catecholamines, such as epinephrine and norepinephrine. These hormones will be released and broken down into two common metabolites called homovanillic acid (HVA) and vanillylmandelic acid (VMA). Since neuroblastoma also produces the same hormones, the amount of HVA and VMA released in urine or blood will be higher than expected. Therefore, children who undergo blood tests can reveal neuroblastoma.

13.

Can Adults Be Affected by Neuroblastoma?

Neuroblastoma is very rare in people older than ten years of age because it is an embryonal malignancy of the autonomic nervous system. But still, even though neuroblastoma occurs in adults, the overall incidence will be only 1 in 10 million adults/year.

14.

How Do Children Get Neuroblastoma?

The cause of neuroblastoma is usually unknown. Most cases are sporadic and nonfamilial. However, it occurs due to a specific genetic mutation called familial neuroblastoma in some instances. The rare germline mutation occurs in the anaplastic lymphoma kinase (ALK) gene.

15.

Where Will the Neuroblastoma Lump Develop in the Body?

The neuroblastoma lumps can develop in the body's nerve cells in and around the adrenal glands located on the top of the kidneys and in the chest, neck, spine or spinal cord, abdomen, and bones.
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Dr. Pawar Satyajit Jalinder
Dr. Pawar Satyajit Jalinder

Medical oncology

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