Gangliocytomas are rare types of tumours that generally develop in the central nervous system (CNS). More specifically, they develop from ganglia in the CNS, which are a group of neuronal bodies which sends messages to the brain relating to touch, smell, taste, sound, and visual stimuli. While most gangliocytomas are benignnot cancerous, can grow but will not spread to other body parts, in very rare cases they may become malignantcancerous, may grow and spread to other areas of the body.
The brain is a complex organ that is responsible for controlling all functions of the body. It has five main portions: the cerebrum, cerebellum, brainstem, pituitary gland, and hypothalamus. The cerebrum is the biggest part of the brain, and consists of the frontal, parietal, temporal, and occipital lobes. This part of the brain is responsible for voluntary movement, intelligence, and memory. The cerebellum is a small part of the brain located at the back of the head, and regulates posture and balance. The brainstem is a small, stalk-like structure towards the bottom of the brain that connects the brain to the spinal cord. It regulates many vital bodily processes, such as swallowing, breathing, and heart rate. The pituitary gland is a pea sized organ located behind the eyes, and is responsible for the production and secretion of hormones in the body. The hypothalamus is located deep within the brain, and has many important functions, such as producing and secreting different hormones, regulating temperature, and controlling appetite.
The spinal cord is a long, thin, tubular structure that extends down from the brainstem to the lower back. It is made of nerve tissuea group of cells that work together to perform a function, and is surrounded by the bones of the spine (vertebrae). The spinal cord is responsible for transmitting nerve signals from the brain to the body, and vice versa. It has motor functions (such as voluntary movement), sensory functions (such as pressure, touch, temperature, and pain), and autonomous functions (such as regulating digestion, heart rate, and bloodthe red bodily fluid that transports oxygen and other nutrients around the body pressure).
Gangliocytomas are most commonly found in the temporal lobe of the cerebrum, but can also develop in the cerebellum, brainstem, and spinal cord. Initially, they may be confused with ganglioglioma as they are indistinguishable on imagingtests that create detailed images of areas inside the body scans.
Gangliocytomas are generally diagnosed equally among the sexes, and are most commonly found in people between the ages of 10-30. However, anyone can develop this disease.
Types of Gangliocytoma
Because of how rare gangliocytomas are, only one variant of this disease has been identified.
Lhermitte-Duclos Disease
Lhermitte-Duclos disease, also known as dysplastic cerebellar gangliocytoma, is a very rare condition that is characterised by the abnormal development and enlargement of the cerebellum portion of the brain. It is often accompanied by increased intracranial pressure, and has been associated with a hereditary cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs syndrome known as Cowden syndrome. Unlike most gangliocytomas, Lhermitte-Duclos disease is most commonly diagnosed in people between the ages of 30 and 40. This tumoura tissue mass that forms from groups of unhealthy cells is often non-aggressive, slow growing, and can have a good prognosisto predict how a disease/condition may progress and what the outcome might be.
For more information on Lhermitte-Duclos disease, please refer to the Rare Cancers Australia Lhermitte-Duclos disease page.
Treatment
When cancers are detected, they are staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancer cellsthe basic structural and functional unit of all living things 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. However, brain cancers are rarely staged, as they rarely spread to other parts of the body. Instead, they are generally graded from I-IV.
Gangliocytomas are generally benign, and are often classified as a gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells I tumour. Grade I (low grade) tumours have cells that present as slightly abnormal, and are usually slow growing.
Once your tumour has been 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 options for gangliocytomas 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.
- Watch and waitthe close monitoring of a cancer without giving treatment until symptoms appear or worsen.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- 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
Because of how rare gangliocytomas are, there has been limited research done into the riskthe possibility that something bad will happen factors of this disease. However, Lhermitte-Duclos disease has been linked to people with Cowden syndrome.
Symptoms
Gangliocytomas may appear asymptomatic in the early stages of disease. As the tumour progresses, some of the following symptoms may appear:
- Epilepsy (most commonly temporal lobe epilepsy (TLE)).
- Increased intracranial pressure.
- Hydrocephalusa build up of cerebrospinal fluid (CSF) in the brain, causing pressure and potentially damaging brain tissue.
- Headaches.
- Blurred or double vision.
- Nauseato feel sick or likely to vomit and/or vomiting.
- General feeling of weakness.
- Behavioural changes.
- Difficulties with moving and/or talking.
- Lack of energy.
- Fatiguea state of extreme tiredness or exhaustion, can be physical or mental.
- Hypertensionhigh blood pressure.
- Confusion and/or lack of alertness.
- Endocrine disorders.
- Focal signs.
In addition to these symptoms, patients with Lhermitte-Duclos disease may also experience:
- Megalencephalya condition characterised by an abnormally large brain.
- Hydromyeliaabnormal widening of central canal of the spinal cord.
- Polydactylya condition where a person has extra fingers and/or toes.
- Partial giantism.
- Macroglossiaa condition characterised by an abnormally large or oversized tongue.
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 an gangliocytoma, 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.
- Neurological examinationan assessment of sensory and motor functions, such as vision, balance and coordination.
- Imaging 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.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
