What is Neuroblastoma?

Neuroblastoma is a rare cancer that develops from immature nerve cells called neuroblasts. It primarily affects infants and young children, with the majority of cases diagnosed in children under the age of 5. Around 100 children between 0 and 14 are diagnosed with neuroblastoma each year in the UK. Very rarely it can develop in older children, teenagers and adults.  

Neuroblastoma can occur anywhere in the body, but often starts in the adrenal glands, which are located on top of the kidneys. The cancer can also develop in other parts of the body where nerve tissue is present, such as the neck, chest, or spinal cord. 

Neuroblastoma is the most common form of embryonal tumour. Embryonal tumours are formed from tissue that is normally only seen in the developing embryo. The exact cause of neuroblastoma is not well understood. 

What are the symptoms?

Symptoms of neuroblastoma can vary depending on the location and size of the tumour, as well as if it has spread to other parts of the body. Some children with neuroblastoma may not show any obvious symptoms initially, while others may experience a variety of signs.  

Common symptoms can include, but are not limited to:

  • A visible lump or mass, often in the abdomen
  • Tiredness
  • Loss of appetite and weight loss
  • Bone pain or limping
  • Weakness in the legs
  • Swelling, especially in the legs or abdomen
  • Difficulty breathing or swallowing
  • Small bluish-purple skin nodules (called "blueberry muffin spots ")

What is the prognosis?

The prognosis for neuroblastoma varies depending on several factors, including the stage of the disease, the age of the patient, and the specific characteristics of the tumour. In general, those who were diagnosed under 12 months of age and those with lower-stage disease have better survival rates. Girls also tend to have a better outlook than boys.

70% of children (aged 0-14) in the UK diagnosed with neuroblastoma and other peripheral nervous cell tumours survive five years or more (2012-2016).

It's important to note that these statistics are based on data from large groups of patients and may not be reflective of an individual's specific prognosis. Survival rates can also change as new treatments become available and as researchers continue to learn more about the disease.

What are the stages for neuroblastoma?

Staging is a measure of how far the tumour has spread beyond its original site, and can help determine the correct treatment. The following describes the staging system that is used in the UK.

Stage 1: The tumour is in one location and can be removed completely with surgery.

Stage 2A: The tumour is in one location but cannot be completely removed by surgery.

Stage 2B: The tumour is localised but has spread to local lymph nodes.

Stage 3: The tumour cannot be removed fully by surgery and it is present on both sides of the body (either side of spine), or the tumour is present on one side of the body and cancer cells are found in lymph nodes on the other.  

Stage 4: The tumour has spread to distant lymph nodes, bone marrow, bone, liver and/or other organs.

Stage 4S: The tumour is localised (stage I, IIA or IIB), with spread limited to liver, skin and/or bone marrow in an infant under one year of age. 4S is unique compared to the other stages as it has much better outlook, children with 4S generally get better with very little, or no treatment. 

How common is neuroblastoma is children?

Neuroblastoma is considered a relatively rare childhood cancer. It accounts for a small percentage of all pediatric cancers. The incidence of neuroblastoma varies by country and population, but globally, it is estimated to occur in about 1 out of every 7,000 to 10,000 live births. 

In some countries, such as the United States, neuroblastoma is one of the most common solid tumours in children. It is typically diagnosed in early childhood, with the majority of cases occurring in children under the age of 5. Neuroblastoma is rare in older children, adolescents, and adults. 

It's important to note that the rarity of neuroblastoma does not diminish its significance. Despite being relatively uncommon, neuroblastoma can have a significant impact on affected children and their families. Early detection, appropriate diagnosis, and specialized treatment are essential in providing the best possible outcomes for children with neuroblastoma. 

If you have concerns about neuroblastoma or want more specific information on its incidence in a particular region, it is advisable to consult official sources such as cancer registries or reach out to healthcare professionals or organizations specializing in paediatric oncology. They can provide more accurate and up-to-date information based on the specific population and region of interest. 

Frequently asked questions

What is the leading cause of neuroblastoma?

The exact cause of neuroblastoma, like many cancers, is not fully understood. However, several factors have been identified that may contribute to its development. 

Neuroblastoma arises from abnormal growth and development of immature nerve cells called neuroblasts. Here are some factors that have been associated with an increased risk of developing neuroblastoma: 

Genetic factors: Certain genetic mutations and alterations have been linked to an increased susceptibility to neuroblastoma. For example, mutations in the ALK (anaplastic lymphoma kinase) gene and MYCN (v-Myc avian myelocytomatosis viral oncogene neuroblastoma derived homolog) amplification are found in a subset of neuroblastoma cases. 

Inherited predisposition: Around 1-2% of neuroblastoma cases are hereditary and can be passed down through families. Mutations in specific genes, such as the ALK gene, PHOX2B gene, and others, can increase the risk of developing neuroblastoma. 

Age: Neuroblastoma is primarily a pediatric cancer, with the majority of cases diagnosed in children under the age of 5. The reason for this age distribution is not completely understood. 

Environmental factors: Some environmental factors have been suggested to play a role in neuroblastoma development, but their contribution is not yet well-defined. Factors such as maternal exposure to certain chemicals or substances during pregnancy or early childhood exposure to certain infections or toxins have been studied, but their direct influence is not fully established. 

It's important to note that neuroblastoma is a complex disease, and individual cases can have unique characteristics. Ongoing research aims to uncover more about the underlying causes and risk factors associated with neuroblastoma. 

What is the treatment for neuroblastoma?

The treatment of neuroblastoma depends on the age of the child, the size and position of the tumour, the tumour biology and whether it has spread. It is a decision that will be made on an individual basis with the child’s treatment team.

The main treatments include surgery, chemotherapy, radiotherapy and immunotherapy (currently available in clinical trials only).

Please note:

Symptoms and prognosis will be different from person to person and a thorough evaluation by a healthcare professional is necessary for an accurate diagnosis. If you suspect that your child may have neuroblastoma or any other health concern, it is crucial to consult a paediatrician or medical specialist for an appropriate evaluation and diagnosis.

Is neuroblastoma curable or not?

Neuroblastoma is considered curable in some cases, while in others, it can be more challenging to cure. The potential for a cure depends on several factors, including the stage of the disease, age of the patient, genetic features, and response to treatment. 

Low-risk and intermediate-risk neuroblastoma cases, which are typically diagnosed at an early stage and have favorable characteristics, often have a high cure rate. With appropriate treatment, the majority of children with low-risk neuroblastoma can be cured, and the long-term survival rate is generally very good. 

High-risk neuroblastoma, on the other hand, is more aggressive and has a greater tendency to spread to other parts of the body. It can be more challenging to cure and requires more intensive and complex treatment approaches. The prognosis for high-risk neuroblastoma is generally poorer compared to low- and intermediate-risk cases. However, advances in treatment options, including chemotherapy, radiation therapy, surgery, immunotherapy, and targeted therapies, have improved outcomes for some patients with high-risk neuroblastoma. 

It's important to consult with a medical professional who specializes in pediatric oncology for a comprehensive evaluation and personalized information about the specific case and available treatment options. They will be able to provide the most accurate and up-to-date information regarding the potential for cure in a particular situation. 

How common is neuroblastoma in UK?

Neuroblastoma is a relatively rare childhood cancer, and its incidence can vary by country. In the United Kingdom (UK), neuroblastoma is considered one of the most common solid tumours in children, accounting for around 8% of all childhood cancers. 

The exact number of new cases of neuroblastoma diagnosed in the UK each year can vary, but it is estimated that there are around 100 new cases diagnosed annually. However, it's important to note that these numbers can fluctuate and may not reflect the most recent data. 

Neuroblastoma can occur in children of all ages, but it is most commonly diagnosed in infants and young children, typically under the age of 5. It affects slightly more boys than girls. 

Diagnosis and treatment of neuroblastoma in the UK are primarily carried out by specialist pediatric oncology centers. These centers provide multidisciplinary care and access to the latest treatment options and clinical trials for children with neuroblastoma. 

If you have specific concerns or need more accurate and up-to-date information on the incidence and prevalence of neuroblastoma in the UK, I would recommend consulting official sources such as cancer registries or speaking with a healthcare professional or organization specializing in pediatric oncology in the UK.

What is the survival rate for neuroblastoma?

The survival rate for neuroblastoma varies depending on several factors, including the stage of the disease, age of the patient, genetic features, and response to treatment. Survival rates are often reported in terms of the percentage of patients who are alive at a certain time point after diagnosis. It's important to note that these statistics are general estimates and individual outcomes may differ. 

For neuroblastoma, survival rates are typically categorized based on risk groups: 

Low-risk neuroblastoma: The survival rate for low-risk neuroblastoma is generally very high, with estimates ranging from around 95% to 99%. The majority of low-risk cases can be successfully treated, and long-term survival is common. 

Intermediate-risk neuroblastoma: Intermediate-risk neuroblastoma has a somewhat lower survival rate compared to low-risk cases. The estimated survival rate for intermediate-risk neuroblastoma is typically around 90% to 95%. Although the prognosis is generally favourable, some intermediate-risk cases may require more intensive treatment. 

High-risk neuroblastoma: High-risk neuroblastoma is the most aggressive form of the disease and poses the greatest challenges in terms of treatment and prognosis. The survival rate for high-risk neuroblastoma is lower compared to low- and intermediate-risk cases. Despite advances in treatment, the estimated survival rate for high-risk neuroblastoma is typically around 40% to 50%. 

It's important to remember that these survival rates are based on average outcomes and can vary from individual to individual. The treatment of neuroblastoma is continually evolving, and newer therapies, such as immunotherapy and targeted treatments, are being developed to improve outcomes for high-risk cases. Consulting with a healthcare professional who specializes in pediatric oncology can provide more specific and up-to-date information based on the individual case and available treatment options.