Neuroblastoma: Signs, Symptoms, & Treatments

Neuroblastoma is a form of cancer in children that develops in immature nerve tissue called neuroblasts found in the adrenal glands, chest, neck, and spinal cord.

Symptoms of this condition include severe pain and paralysis that affect the patient’s daily life. Proper treatment of neuroblastoma can help to relieve the symptoms and reduce the risk of complications.

Continue reading to learn more about Neuroblastoma, its causes, symptoms, and treatments.

What is neuroblastoma?

Neuroblastoma is an abnormal cancerous growth that develops from improperly developed nerve cells in several areas of the body.

Neuroblastoma usually affects children below 5 years of age, although it may occur in older children in rare cases. It is the most common tumor affecting the sympathetic nervous system and the most common solid malignant tumor in infants, with 17 months as the median age of diagnosis.

Neuroblastoma can spread to the adrenal glands, bones, lymph nodes, liver, and skin in children and infants. In adolescents, the common sites of metastasis include the lungs and brain.

The neuroblastoma treatment options for a child depend on several factors, including the type of growth, the age of the child, general health, severity of symptoms, and other pre-existing conditions.

How do neuroblastoma cells develop?

This condition most commonly arises in the adrenal glands situated at the top of the kidneys. However, neuroblastoma may also develop in other parts of the body, including the abdomen, chest, spine, and neck, where groups of nerve cells called neurons exist.

In most cases, neuroblastoma begins in infancy and is diagnosed between one month of birth and the age of 5 years.

What are the stages of neuroblastoma?

Neuroblastoma is classified based on the stage of cancer and its rate of growth. It is also classified based on the extent of the spread of cancer cells and rate of growth to other parts of the body.

Imaging studies such as MRI and CT scans can assess the spread and current stage of neuroblastoma. This is called “image-defined risk factors (INRG).”

The stages of neuroblastoma based on INRG are:

Stage L1

At this stage, the tumors are confined to one part of the body and have not spread. The tumor also does not involve any vital organs and tissues of the body, suggesting the absence of image-defined risk factors for the patient.

Stage L2

This stage is marked by at least 1 image-defined risk factor.

The tumor is confined to one compartment of the body. However, this stage is also marked by the spread of abnormal cells to regional lymph nodes and other close structures. The tumor mass may also wrap around large blood vessels or other vital organs and tissues.

Stage M

At this stage, the cancer cells have spread to more than one part of the body, leading to the development of distant metastatic disease.

Stage M carries the highest risk due to the involvement of multiple vital organs.

Stage MS

Stage MS is a special form of neuroblastoma affecting children below 18 months of age. At this stage, the cancer cells have spread to either the liver, bone marrow, or skin only.

Children diagnosed with stage MS neuroblastoma generally have a good prognosis. Hence, it is usually considered a low-risk disease.

What are the symptoms of neuroblastoma?

The symptoms of neuroblastoma depend on the part of the body affected. For example, children who have a diagnosis of neuroblastoma in the abdomen may have symptoms such as pain in the abdomen and a non-tender mass under the skin. They also experience changes in bowel movements, leading to constipation or diarrhea.

Neuroblastoma in the chest causes symptoms such as chest pain, wheezing, and changes in the eyes, like unequal pupil size or drooping eyelids.

Some other symptoms caused by neuroblastoma include:

Formation of lumps of tissue under the skin
Fever
Proptosis (appearance of the protrusion of the eyeballs from the sockets)
Dark circles that appear like bruises under the eyes
Bone pain
Back pain
Unexplained weight loss
Trouble breathing
Changes in blood pressure

What are the causes of neuroblastoma?

Neuroblastoma develops as a result of a genetic mutation that causes healthy cells to grow continuously without responding to the signal to stop. Unlike normal cells, these cells continue to grow and multiply uncontrollably. The accumulation of such rapidly multiplying cells leads to the formation of a tumor mass.

The exact factors that trigger the initial genetic mutation responsible for the development of neuroblastoma are not known. However, this abnormal growth is found to begin in immature nerve cells called neuroblasts during the development of the fetus in the womb. In normal cases, as the fetus matures, these neuroblasts mature and are converted into nerve cells.

But, in some cases, they fail to mature, thus forming a tumor called neuroblastoma.

What are the risk factors for neuroblastoma?

Children with a family history of neuroblastoma are at a higher risk for this condition. However, familial neuroblastoma is found to comprise only a small number of cases. In most patients, the cause of neuroblastoma cannot be identified.

What are the complications of neuroblastoma?

Some common complications of neuroblastoma include:

The cancer may spread to other parts of the body, including the lymph nodes, liver, skin, bones, and marrow.
A tumor close to the tissues in the back can exert pressure on the spinal cord as well as nerves, causing spinal cord compression. This condition can result in moderate to severe pain and even paralysis.
Neuroblastoma cells sometimes secrete chemicals that can irritate normal tissues. This can result in symptoms referred to as paraneoplastic syndromes. For example, a form of paraneoplastic syndrome found in rare cases of neuroblastoma causes difficulty in coordination and rapid eye movements. Another rare form of paraneoplastic syndrome causes recurrent diarrhea and abdominal swelling.

Cancer diagnosis for neuroblastoma

There are various medical tests and procedures done to determine a diagnosis of neuroblastoma.

Physical examination

Your child's healthcare provider will conduct a physical examination to check for the signs of neuroblastoma. The doctor will also ask questions about the child's habits and behaviors to assess the possibility of neuroblastoma or other conditions that cause similar symptoms.

Laboratory tests

Laboratory tests, such as blood or urine tests, can check the chemical levels that tend to rise due to the production of excess catecholamines by the neuroblastoma cells.

Imaging tests

Your child's physician may recommend imaging tests to detect a mass that indicates the presence of a tumor.

Imaging tests for the diagnosis of neuroblastoma include an X-ray, ultrasound, MIBG (metaiodobenzylguanidine) scan, CT (computerized tomography) scan, and MRI (magnetic resonance imaging).

Biopsy

If a mass is detected during an imaging test, your child's doctor might want to perform a biopsy that involves the removal of a small sample of the tissue to be sent for testing in order to identify the presence of cancer cells.

In addition, the physician may also recommend a bone marrow biopsy if neuroblastoma has spread to the marrow where blood cells are formed.

Treatment of neuroblastoma

Some forms of neuroblastoma resolve on their own, while some require prolonged treatments.

The treatment plan for neuroblastoma is based on several factors, including the child's age, the type of cells involved, the stage of cancer, and the presence of specific abnormalities in the genes or chromosomes.

The staging of neuroblastoma can help to categorize neuroblastoma as low, intermediate, or high risk. The treatment or plan is then determined based on the risk category.

Surgery for neuroblastoma

Surgery to remove the tumor is typically performed on children with low-risk neuroblastoma. The tumor may be removed completely or partially depending on its size and location.

In children with intermediate or high-risk neuroblastoma, the surgeon might try to remove as much of the mass as possible. The surgery is often done in part with other therapies, such as chemotherapy and radiation, for eliminating the remaining abnormal cells.

However, tumors that are too close to or attached to vital organs, such as the lungs or spinal cord, might be too risky to remove.

Radiation therapy

Radiation treatment involves high-energy beams, such as X-rays, to destroy cancer cells.

Children with intermediate and low-risk neuroblastoma usually need radiation therapy, especially when surgery and chemotherapy have not been helpful. Children with high-risk neuroblastoma are also given radiation therapy after surgery and chemotherapy to prevent a relapse of the condition.

Chemotherapy

Chemotherapy involves the administration of medications to destroy abnormal cells. This therapy targets rapidly growing cells, including cancer cells.

Children with intermediate-risk neuroblastoma usually receive chemotherapy before surgery to improve the prognosis, especially when the entire mass cannot be removed.

Bone marrow transplant

Advanced treatments such as bone marrow transplant and immunotherapy may be recommended in some cases depending on the severity of symptoms and the development of complications.

Neuroblastoma: Frequently Asked Questions

How do you know if your child has neuroblastoma?

If your child has symptoms such as a mass under the skin, protrusion of the eyes, chest pain, or wheezing, it could be due to neuroblastoma, especially if there is a family history of this condition.

How early can neuroblastoma cancer cells be detected?

The nerve tumor is detected when it begins to grow and causes symptoms. In some cases, it is formed before birth and detected when an ultrasound of the mother is performed. Genetic testing and genetic counseling are recommended in these cases to assess the possibility of recurrence in future pregnancies.

In most cases, the cancer mass has already metastasized or spread to healthy organs by the time it is diagnosed.

What are the different types of treatments?

Neuroblastoma can be treated with surgery, chemotherapy, and radiation. Some children need immunotherapy (treatment for modifying immune system response) and a bone marrow transplant.

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