Neuroblastomas are neuroendocrine tumours that develop from neuroblast cells (young nerve cells) in nerve tissue. Neuroblasts are most commonly found in the adrenal glands, which are located on top of the kidneys; however, they can also be found in the nerve tissues of the neck, chest, abdomen or pelvis. 

Neuroendocrine cancers are a complex group of tumours that develop in the neuroendocrine system, which is responsible for regulating important bodily functions such as heart rate, blood pressure and metabolism. They most commonly develop in the gastro-intestinal tract, pancreas and the lungs; however, they can develop anywhere in the body. These tumours develop from neuroendocrine cells, which are responsible for receiving signals from the nervous system and producing hormones and peptides (small proteins) in response. 

Neuroblastomas are the most common type of solid childhood tumour and is usually diagnosed between birth and five years of age. However, it can be diagnosed in people at any age.

Treatment 

If a neuroblastoma is detected, it will be staged and graded based on size, metastasis (whether the cancer has spread to other parts of the body) and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.

Cancers can be staged using the TNM staging system:

• T (tumour) indicates the size and depth of the tumour.
• N (nodes) indicates whether the cancer has spread to nearby lymph nodes.
• M (metastasis) indicates whether the cancer has spread to other parts of the body.

This system can also be used in combination with a numerical value, from stage 0-IV:

• Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
• Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
• Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
• Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
• Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.
  

Cancers can also be graded based on the rate of growth and how likely they are to spread:

• Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
• Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour. 
• Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour. 

Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you. 

Treatment is dependent on several factors, including location, stage of disease and overall health.

Treatmento options for neuroblastoma may include:

• Surgery to remove as much of the tumour as possible.
• Radiation therapy.
• Chemotherapy.
• Immunotherapy.
• Clinical trials, potentially including vaccine therapies.
• Palliative care. 

For more information on treatment options, please refer to the Rare Cancers Australia treatment options page. 

Risk factors

While the exact cause of neuroblastomas remains unknown, the following factors have been linked to a higher chance of developing the disease:

• Family history of neuroblastoma.
• Certain genetic conditions, such as Hirschsprung disease, congenital central hypoventilation syndrome (CCHS) and neurofibromatosis type 1 (NF1).

Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.

Early symptoms

The symptoms of neuroblastomas will vary depending on stage, location and whether the tumour has metastasised (spread to other parts of the body). Some of these symptoms include:

• Lump or swelling in the affected area.
• Abdominal pain.
• Unexplained weight loss/loss of appetite. 
• Issues urinating and/or with bowel movements.
• Exophthalmos (bulging eyes).
• Dark circles around the eyes. 
• Uncontrolled eye movements.
• Bone and/or other unexplained pain.
• General weakness or paralysis.
• Difficulties breathing and/or swallowing.

In some cases, neuroblastomas release hormones that causes additional symptoms:

• Diarrhoea.
• High blood pressure.
• Rapid heartbeat.
• Unexplainable sweating.
• Flushing of the skin.

Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.

Diagnosis/diagnosing

If your doctor suspects you have a neuroblastoma, they will order a range of diagnostic tests. 

Imaging & blood tests 

The doctor will take images of your body using magnetic resonance imaging (MRI), a computed tomography scan (CT scan), x-ray, bone scan, metaiodobenzylguanidine (MIBG) scan and/or positron emission tomography (PET scan), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and look for signs of metastasis. Additionally, a blood and urine test may also be taken to assess your overall health and help guide treatment decisions. 

Biopsy 

Once the location(s) of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. In most cases, this will be done by a bone marrow aspiration and biopsy. This process involves inserting the needle into the hipbone (or the breastbone in some cases) to remove samples of solid and liquid bone marrow. These samples will then be analysed for cancer cells. 

Prognosis (Certain factors affect the prognosis and treatment options)

While it is not possible to predict the exact course of the disease, your doctor may be able to give you a general idea based on stage, rate/depth of tumour growth, susceptibility to treatment, age, overall fitness and medical history. Generally, early-stage neuroblastomas have good prognosis and survival rates. However, if the cancer is advanced and has spread, the prognosis may not be as good and there may be a higher risk of cancer recurrence.  

References

• Cancer Australia
• Neuroendocrine Cancer Australia

^{Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.}