Neuroblastic tumours, also known as neurogenic tumours, are a group of tumours that arise from neuroblast cellsthe basic structural and functional unit of all living things (young nerve cells) and/or a specific type of chromaffin cell called paraganglia cells, which produce catecholamine hormones in nerve tissuea group of cells that work together to perform a function. These tumours often develop within the sympathetic chain in the body, which consists of the bilateralaffecting both sides nerves that run parallel to the spine from the base of the skull to the coccyx (bottom portion of the spine). The nerves in this chain are primarily responsible for the ‘fight or flight’ responses, such as sweat, reflexes and accelerated heart rate.
The nervous system is made up of two primary components: the central nervous system (CNS), and the peripheral nervous system (PNS). The CNS is made up of the brain and spinal cord, and is responsible for all sensory and motor functions in the body. The PNS encompasses all nerves outside of the CNS, and is responsible for all involuntary functions in the body. The PNS is further subdivided into the autonomic nervous system (ANS) and the somatic nervous system (SNS). The ANS controls involuntary processes and glands, such as heart rate, bloodthe red bodily fluid that transports oxygen and other nutrients around the body pressure, respiration, and digestion. Comparatively, the SNS is responsible for voluntary and involuntary muscle movements, as well as transmitting sensory information to the CNS.
Neuroblastic tumours are generally diagnosed equally among the sexes, and are generally diagnosed in children under 10. However, anyone can develop these diseases.
Types of Neuroblastic Tumours
There are three primary types of neuroblastic tumours, which are categorised by the types of cells they develop from.
Ganglioneuroma
Ganglioneuromas are a rare type of neuroblastic tumoura tissue mass that forms from groups of unhealthy cells that contain ganglion cells and neuronal cells without an immature blast cells. Unlike most neuroblastic tumours, ganglioneuromas tend to be slightly more common in females. These tumours are often slow-growing, non-aggressive, and can have a good prognosisto predict how a disease/condition may progress and what the outcome might be.
Ganglioneuroblastoma
Ganglioneuroblastoma is a more common type of neuroblastic tumour that contains ganglion cells, neuronal cells, and immature blast cells. These tumours are often attached to a nerve trunk, which is a bundle of nerve fibres or the main stem of a nerve. These tumours can be aggressive, but can have a good prognosis when found early.
Neuroblastoma
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, abdomenstomach, stomach area, belly or pelvis. For more information on neuroblastomas, please refer to the Rare Cancers Australia Neuroblastoma page.
Treatment
If a neuroblastic tumour is detected, it will be staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs cells look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas 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 (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid.
- 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 localisedaffecting only one area of body 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 advancedat a late stage, far along or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells 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 testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, 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, age, stage of disease and overall health.
Treatment for neuroblastic tumours may include:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence to remove as much of the tumour as possible.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Stem cell transplanta procedure that involves replacing unhealthy blood-forming cells (stem cells) with healthy stem cells.
- Immunotherapya treatment that uses a person's immune system to fight cancer.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Risk factors
While the cause of neuroblastic tumours remain unknown, the following factors may increase the likelihood of developing the disease:
- A family history of ganglioneuromas, ganglioneuroblastomas and/or neuroblastomas.
- Certain genetic conditions, such as:
- Hirschsprung disease.
- Congenital central hypoventilation syndrome (CCHS).
- Neurofibromatosis type 1 (NF1).
Not everyone with these riskthe possibility that something bad will happen 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.
Symptoms
Neuroblastic tumours may present as asymptomatic during the early stages of disease. As the tumour progresses, some of the following symptoms may appear:
- Lump or swelling in the affected area.
- Abdominal pain.
- Unexplained weight loss/loss of appetite.
- Issues urinating and/or with bowelportion of the digestive system that digests food (small bowel) and absorbs salts and water (large bowel); also called intestines movements.
- Proptosisbulging or protrusion of one or both of the eyes out of the eye sockets, also known as exophthalmos.
- Dark circles around the eyes.
- Uncontrolled eye movements.
- Bone and/or other unexplained pain.
- General weakness or paralysis.
- Dyspneadifficulty breathing, shortness of breath.
- Difficulties swallowing.
- Diarrhoeafrequent discharge of watery or loose stools from the body.
- Hypertensionhigh blood pressure.
- Tachycardiaa rapid heart rate; clinically defined as a rate of more than 100 beats per minute.
- Unexplainable sweating.
- Flushing of the skin.
- Haematuriathe presence of blood in urine.
Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.
Diagnosis
If your doctor suspects you have a neuroblastic tumour, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Imagingtests that create detailed images of areas inside the body tests, potentially including:
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Bone marrow aspirationa procedure that involves inserting a needle into the hipbone (or the breastbone in some cases) to remove samples of solid and liquid bone marrow..
- Biopsyremoval of a section of tissue to analyse for cancer cells.