Neuroblastoma

Neuroblastoma

Neuroblastoma is a type of cancer that develops from immature nerve cells, called neuroblasts, found in the adrenal glands, neck, chest, and spinal cord. It is the most common solid tumor outside of the brain in infants and young children and is often diagnosed in children under the age of five.

Neuroblastoma can be either low-risk or high-risk, depending on various factors such as the age of the child, the location and size of the tumor, and certain genetic changes in the tumor cells. 

Low-risk neuroblastomas – tend to grow slowly and may even go away on their own.

High-risk neuroblastomas – grow rapidly and are more likely to spread to other parts of the body.

Symptoms

The symptoms of neuroblastoma can vary depending on the location and size of the tumor. In some cases, neuroblastoma may be discovered during routine screening tests before any symptoms develop. However, in other cases, symptoms may be present and can include –

1. A lump or swelling in the abdomen, neck, or chest
2. Pain or tenderness in the bones or joints
3. Difficulty breathing or wheezing

Neuroblastoma

4. Anemia and fatigue

5. High blood pressure

6. Frequent infections

7. Loss of appetite, weight loss, or abdominal pain

8. Changes in the eyes, such as drooping eyelids or unequal pupils

9. Uncontrolled eye movements or eye bulging

10. A mass or lump in the pelvis, neck, or other areas

These symptoms may be caused by other conditions as well, and not all children with neuroblastoma will experience these symptoms. If you notice any of these symptoms in your child or have any concerns, it’s important to speak with your child’s pediatrician for an early diagnosis

Causes and Risk Factors

The actual causes of neuroblastoma are not yet known, but there are several factors that can increase a child’s risk of developing this type of cancer –

1. Age – Neuroblastoma is most commonly diagnosed in children under the age of five, with nearly 90% of cases occurring in children under the age of 10.
2. Genetic mutations – Certain genetic mutations have been linked to an increased risk of developing neuroblastoma. For example, mutations in the ALK gene have been found in about 10% of neuroblastoma cases.
3. Family history – Children who have a sibling or parent with neuroblastoma or certain other genetic conditions, such as familial cancer syndromes, may have a higher risk of developing the disease.
4. Prenatal exposure to certain substances – Exposure to certain substances during pregnancy, such as alcohol, tobacco, and some chemicals, has been linked to an increased risk of neuroblastoma in some studies.
5. Other medical conditions – Certain medical conditions, such as congenital anomalies and certain hormonal imbalances, may increase the risk of developing neuroblastoma.

Many children with neuroblastoma have no known risk factors, and most children with risk factors do not develop the disease. Ongoing research is aimed at identifying additional risk factors and better understanding the underlying causes of neuroblastoma.

Diagnosis and Tests

Diagnosis of neuroblastoma typically involves a combination of medical history, physical examination, and various tests to evaluate the extent and characteristics of the tumor. The followings are some of the most common tests used to diagnose and stage neuroblastoma –

1. Imaging tests – Imaging tests such as ultrasound, X-ray, CT scan, MRI, or MIBG scan may be used to identify the location and size of the tumor, as well as whether the cancer has spread to other parts of the body.

• Ultrasound – Ultrasound is particularly useful for detecting tumors in the abdomen, where most neuroblastomas occur. During an ultrasound, a transducer (a handheld device) is placed on the skin over the area being examined, and a gel is applied to help transmit the sound waves. The transducer sends and receives sound waves that bounce off the organs and tissues in the body, creating images on a computer screen that can be analyzed by a healthcare provider.
• X-ray – X-rays are not typically used as a primary diagnostic tool for neuroblastoma because they may not provide enough detail to accurately identify the presence and extent of the tumor. However, X-rays may be used as part of the diagnostic process to rule out other possible causes of symptoms or to detect any possible bone involvement of the cancer.
• Computed Tomography (CT) scan – is a commonly used imaging test in the diagnosis and evaluation of neuroblastoma. CT scan uses X-rays and computer technology to produce detailed images of the body’s internal structures, including bones, soft tissues, and organs.
• MRI (Magnetic Resonance Imaging) – is another common imaging test used in the diagnosis and evaluation of neuroblastoma. It uses a powerful magnetic field, radio waves, and a computer to produce detailed images of the body’s internal structures, including bones, soft tissues, and organs.
• MIBG (Meta-iodobenzylguanidine) – is a nuclear medicine imaging test that is used to diagnose and evaluate neuroblastoma. MIBG is a substance that is similar to the neurotransmitter norepinephrine, which is taken up by neuroblastoma cells.

2. Biopsy – A biopsy is a procedure in which a small sample of tissue is removed from the tumor and examined under a microscope to confirm the diagnosis and determine the type of neuroblastoma.

3. Bone marrow aspiration and biopsy – A small amount of bone marrow is removed from the hip bone using a needle and examined under a microscope to determine if cancer has spread to the bone marrow.

4. Blood and urine tests – Blood and urine tests may be done to check for elevated levels of certain hormones or chemicals that can be produced by neuroblastoma cells.

5. Genetic testing – Genetic testing may be performed to look for specific genetic mutations that can be associated with neuroblastoma.

Once the diagnosis and stage of cancer have been determined, the healthcare team can work with the child and their family to develop a treatment plan. Treatment options may include surgery, chemotherapy, radiation therapy, stem cell transplant, and targeted therapy, depending on the extent and characteristics of the tumor.

Treatments

The treatment for neuroblastoma depends on several factors, such as the stage and location of the cancer, the age and overall health of the child, and whether the cancer has spread to other parts of the body. The main treatments for neuroblastoma include –

1. Surgery – Surgery is often the first step in the treatment of neuroblastoma, and it involves the removal of as much of the tumor as possible. Depending on the location and extent of the tumor, surgery may be performed with open surgery or minimally invasive techniques.
2. Chemotherapy – Chemotherapy involves the use of powerful drugs to kill cancer cells. Chemotherapy is often used in combination with surgery and radiation therapy to treat neuroblastoma. The type and duration of chemotherapy depend on the stage and location of the cancer.
3. Radiation therapy – Radiation therapy uses high-energy radiation to kill cancer cells. It is often used after surgery to kill any remaining cancer cells or to treat neuroblastoma that has spread to other parts of the body.
4. Immunotherapy – Immunotherapy is a type of treatment that works by stimulating the body’s immune system to attack cancer cells. It is often used in children with high-risk neuroblastoma.
5. Targeted therapy – Targeted therapy uses drugs that target specific molecules or proteins in cancer cells to stop their growth and spread. It is often used in children with advanced or recurrent neuroblastoma.
6. Stem cell transplant – A stem cell transplant may be used in children with high-risk neuroblastoma who have received high-dose chemotherapy or radiation therapy. The transplant involves the use of stem cells to replace the bone marrow, which has been damaged by the treatment.

The treatment plan for neuroblastoma is often complex and may involve a combination of different treatments. The goal of treatment is to completely remove or destroy the cancer cells and prevent the cancer from coming back. The treatment plan may also involve supportive care to help manage the side effects of treatment and improve the child’s quality of life.

Outlook

The outlook for neuroblastoma depends on several factors, including the stage and location of the cancer, the age and overall health of the child, and the response to treatment.

The 5-year survival rate for children with neuroblastoma ranges from about 90% for low-risk neuroblastoma to about 50% for high-risk neuroblastoma. The survival rate is lower for children with neuroblastoma that has spread to other parts of the body.

The treatment for neuroblastoma is often complex and may involve a combination of different treatments, such as surgery, chemotherapy, radiation therapy, immunotherapy, targeted therapy, and stem cell transplant. The goal of treatment is to completely remove or destroy the cancer cells and prevent the cancer from coming back.

Even after successful treatment, there is a risk of the cancer coming back or developing new tumors. Regular follow-up care, including imaging tests and blood tests, is important to monitor for any signs of recurrence.

Overall, the outlook for neuroblastoma has improved significantly in recent years due to advances in treatment and supportive care. With appropriate treatment and care, many children with neuroblastoma can go on to lead healthy, productive lives.