To mark Childhood Cancer Awareness Month, we’re taking a look at childhood cancer neuroblastoma and the groundbreaking research being carried out by NSW scientist Dr Orazio Vittorio that has the potential to change how neuroblastoma is treated globally.
Every year, around 40 children are diagnosed with neuroblastoma in Australia. The disease is most common in under 5s and rarely occurs in anyone aged over 10 years. Although it is rare, neuroblastoma accounts for 15% of total childhood cancer deaths.
Neuroblastoma is a type of cancer that forms in cells in the nerve tissues of the body, found most commonly in the adrenal glands and along the tissues around the spinal cord in the neck, chest, abdomen and pelvis.
The brain, spinal cord, and the nerves that reach out from them to all areas of the body make up the nervous system. The nervous system is needed for thinking, feeling, and movement, as well as body functions like heart rate, breathing, blood pressure and digestion.
When matured properly, nerve cells process and transmit information throughout the nervous system. These cells interact with other types of cells in the body by releasing hormones.
Neuroblastoma starts in very early forms of nerve cells (called neuroblasts), often found in an embryo or fetus. When these cells multiply and grow in an uncontrolled way, neuroblastoma can occur. Neuroblastoma cells often release hormones that can cause symptoms of the disease
The rate of progression of neuroblastoma can vary greatly. Occasionally, in very young children, the cancer cells die for no reason and the tumor goes away on its own.
Who is at risk of developing Neuroblastoma?
Neuroblastoma is most commonly found in very young children. There are some factors that can increase the risk of incidence:
Familial
1% to 2% of all neuroblastomas are a result of children who have inherited an increased risk of developing neuroblastoma from a parent . Children with the familial form of neuroblastoma usually come from families with one or more members who had neuroblastoma as infants.
When tumors develop in several places at once it suggests a familial form. However, both familial and neuroblastoma that is not inherited can spread to other organs.
Birth Defects
If cell growth and development don’t happen normally in a developing fetus, birth defects may occur. Some studies have shown a link between birth defects and neuroblastoma , which may relate to genetic changes during fetal development.
Symptoms of Neuroblastoma in Children:
Neuroblastoma can cause a number of symptoms in children , including:
- Non-tender lumps/swelling in the abdomen or neck
- Swelling of the legs, chest, neck, or face
- Bloated looking belly
- Problems swallowing
- Weight/appetite loss
- Problems with bowel movements or urinating
- Bone pain
- Blue tinged lumps/bumps in the skin
- Drooping eyelid and small pupil in one eye
- Problems with movement in specific body parts
- Bulging/bruising around the eyes
There are lots of reasons why your child might exhibit the above symptoms that are not related to neuroblastoma. However, if you have any concerns you should speak to your GP.
If your child is experiencing bloating, problems swallowing, swelling in the legs, a loss of weight or appetite, or other symptoms that may be associated with neuroblastoma, schedule an appointment to see a doctor .
Even if it’s nothing, it’s better to be safe than sorry. The fastest and easiest way to search for and book healthcare appointments is through MyHealth1st.
Diagnosing Neuroblastoma in Children:
If your child is exhibiting symptoms of neuroblastoma, your doctor will recommend a series of tests. These could include simple urine and blood tests to check for different types of hormones, blood counts, liver and kidney function and electrolytes in the body.
Imaging tests such as x-rays and scans may also be recommended. This will help your child’s medical team understand if suspicious areas might be cancerous and if the cancer has spread. If a child is very young, an ultrasound may be one of the first tests carried out as it is quick and easy. It is often used to look for tumours in the abdomen.
If neuroblastoma is highly suspected, an MRI or CT scan will likely be recommended.
Exams and imaging tests might strongly suggest a child has neuroblastoma but removing some of the tumor for viewing under a microscope and lab testing is usually needed to be sure. This is called a biopsy and may require a general anaesthetic in young children.
Treatment for Neuroblastoma in Children
Children diagnosed with neuroblastoma will be treated according to risk factors including age and general wellness. Treatment will also depend on how far the cancer has spread.
Treatments may include:
- Surgery
- Chemotherapy
- Radiation Therapy
- Stem Cell Transplant
- Retinoid Therapy
- Immunotherapy
Research into Neuroblastoma
Based at Children’s Cancer Institute in NSW, Dr Orazio Vittorio is focused on studying and developing drugs that target neuroblastoma cells, and which have fewer side effects than conventional chemotherapies. His team have discovered that by removing copper from the blood you can destroy neuroblastomas and glioblastomas (which have only a 5% survival rate past five years of diagnosis) that have shown to be resistant to immunotherapy.
Having recently published the findings in the prestigious Cancer Research , a journal of the American Association for Cancer Research, Dr Vittorio has shown that cancer cells ‘feed’ on copper, often having up to six times the normal levels of the metal inside the tumour cells.
According to Dr Vittorio, these cancers are able to hide from the immune system, so cannot be fought against even when using immunotherapy. The researchers have discovered that their ability to hide may be related to the high levels of copper within the cells. Reducing the levels of copper in the blood may therefore mean the cancer cells can no longer survive.
Quality of life is a huge issue for survivors, who deal with short and long-term side effects of treatment for the rest of their lives, including increased risk of heart disease. Dr Vittorio aims to establish a therapeutic strategy to increase survival rates and improve the quality of life of patients.
Dr Vittorio is now looking to take his findings to clinical trial. If he is successful, this may alter the way neuroblastoma is treated in children around the world.
The team needs to raise an additional $90,000 to take this research to trial so that they can start saving lives.
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Resources:
Cancer Australia AIHW