Diffuse leptomeningeal glioneural tumours (DLGT), also known as diffuse leptomeningeal neuroepithelial tumoura tissue mass that forms from groups of unhealthy cells or primary leptomeningeal oligodendrogliomatosis, are a rare type of central nervous system (CNS) tumour that generally develops from leptomeninges. Meninges are the layers of tissuea group of cells that work together to perform a function that cover the brain and the spinal cord in the CNS, and include the leptomeninges (the inner most layer of meninges). DLGTs are classified as glioneural tumours, as they contain both glial cellsthe basic structural and functional unit of all living things (supportive cells within the central nervous system) and nerve cells/neuronal cells (receive sensory input from internal and external stimuli, and relay information to the brain).
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 tissue 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).
DGLTs are generally considered to be slow-growing and benignnot cancerous, can grow but will not spread to other body parts tumours. They most commonly affect the spinal cord, posterior fossa (a small piece of the skull near the brainstem), brainstem, and base of the brain, however they can develop in other areas of the CNS as well. DGLTs are termed ‘diffuse’, as these tumours are not well contained.
DLGTs are generally more common in males, and tend to affect children between the ages of 5-14. However, anyone can develop this disease.
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 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. However, as most DLGTs are considered benign, these tumours are generally considered low-grade and are not staged.
Once your tumour has been diagnosed, 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 DLGTs 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.
- Shunt insertiona procedure to relieve build-up of cerebrospinal fluid (CSF) in the brain.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Targeted therapymedication that targets specific molecular features of 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 DLGTs are, there has been limited research done into the riskthe possibility that something bad will happen factors of this disease.
Symptoms
Symptoms of DLGTs may include:
- Hydrocephalusa build up of cerebrospinal fluid (CSF) in the brain, causing pressure and potentially damaging brain tissue, which caries its own set of symptoms:
- Headaches.
- Nauseato feel sick or likely to vomit and/or vomiting.
- Difficulties with eye movement.
- Difficulties with balance.
- Difficulties walking.
- Muscle spasms.
- Nerve irritation, which carries its own set of symptoms:
- Numbness and/or decreased sensation in affected area.
- Sharp aching or burning pain, which often radiates outwards from affected area.
- Tingling/pins and needles in affected area.
- Muscle weakness in affected area.
- Eye sensitivity to bright light.
- Neck stiffness.
- Seizures (rare).
- Poor muscle control.
- Spinal cord compression.
- Fatiguea state of extreme tiredness or exhaustion, can be physical or mental.
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 DLGT, 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.
- Imagingtests that create detailed images of areas inside the body tests, most commonly a 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.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
