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Ganglioglioma

First described by CB Courville in 1930, Ganglioglioma comes from the Greek work 'ganglion' meaning know, and the Greek word glia meaning glue and the Greek word oma meaning tumor. Strung together, the words mean 'glue knot tumor'. Ganglioglioma is a rare type of tumor that originates from nerve cells and develops in the central nervous system. The growth of the tumor and its symptom may vary depending on its exact location and size.


Ganglioglioma accounts for 2% of all primary intracranial tumors and up to 10% of primary cerebral tumors in children. Ganglioglioma can occur anywhere in the brain and rarely in the spine as well. Most Gangliogliomas are located on the sides of the brain by the ears and in children about 70% are located above in a part in the brain known as the tentorium - a membrane that separates an area in the back, lower part of the brain known as cerebellum, from an area in the back, upper part of the brain known as occipital lobes. Ganglioglioma can also occur in the brain stem, located below the cerebellum that controls many important motor, sensory and reflex actions.


Symptoms

The signs and symptoms depend on the location of the tumor and how fast it can spread, depending upon the age of the patient. In those above ages 10-20, symptoms include:


  • Seizures
  • Involuntary muscle movements
  • Decreased awareness of the environment.

If Gangliogliomas appear in the ear lobes, it can lead to seizures and symptoms such as:


  • Loss of awareness
  • Abnormal sensations
  • Feeling of being detached from oneself
  • Involuntary muscle movements

If Gangliogliomas are present in the cerebellum, signs and symptoms include:


  • Impaired motor coordination
  • Headache
  • Buildup of cerebrospinal fluid in the brain

Causes

Gangliogliomas are caused by abnormal glial cells. Glial cells support and maintain other cells. Gangliogliomas are partly made of neurons or nerve cells in various degrees of abnormality. In Gangliogliomas are also found supportive tissue known as stroma, which contain fibers and blood vessels. Fibers are flexible, threadlike objects found outside the cells. In Gangliogliomas are neurons, glial cells and stroma in abnormal shape, size and appearance. The more the cells change in structure, size and appearance; the more harmful the tumor is.


Diagnosis

Initially the seizures associated with Ganglioglioma lead a patient to seek medical help. The doctor uses various techniques to diagnose the cause of the seizures. Pictures of the brain are taken and CT scans done to get clear and detailed pictures. MRI scans are sensitive and specific at detaching a tumor and when contrast is injected into the person's body during the scan. About 50% of Gangliogliomas are detected when contrast is used. MRI scans can also detect if there are any cysts in an abnormal lump, swelling or sac that contains fluid and is covered with a membrane.


But it is not until a sample of the tumor is removed during surgery that it can be tested in a laboratory and definitively stated that it is Ganglioglioma. In a laboratory abnormal neurons are stimulated and the irritation produces a protein called synaptophysin and glial fibrillary acidic protein. There are special stains applied to the neurons in the lab to detect the presence of these proteins. However regular x rays do not help in diagnosing Gangliogliomas as they are best at detecting bones and can only detect if calcium is near the Ganglioglioma.

Gangliogliomas are common before age 20 and about 60% of people who are diagnosed with Ganglioglioma are adolescents and adults younger than age 30. However, even those as young as 2 or as old as 70 have been known to develop Ganglioglioma. It affects equal numbers of males and females. In the US approximately 1 to 2% of brain tumors are Gangliogliomas. About 10% of all primary brain tumors in children are Gangliogliomas.


Treatment

Surgery is most often done to remove Ganglioglioma tumor. After the tumor is removed, radiation therapy is not needed unless the tumor shows signs of growing back, which is rare. Radiation is aimed at tumors to destroy or weaken them. Prognosis of people with Ganglioglioma is excellent as it is easy for surgeons to remove them as they are typically found in one place and can be separated from the brain tissue. Most get cured after surgery and chance of tumor coming back after it has been totally removed is rare.


Myelography

Myelography or Myelogram is used to make a diagnosis of spinal canal and spinal cord disorders like nerve compression that in turn causes pain and weakness. A special dye is introduced into the spinal sac that in turn shows up in the x-ray to signify any deformities. The dye acts as an exclusive agent to outline the nerve roots and the spinal cord thus helping the doctor determine if there are any abnormal shapes in the spinal cord. Before CT scans and MRI scans were introduced, it was myelography that was used to study any abnormalities in the spinal cord. Current medical world uses myelography only for complicated revision spine surgeries and for patients who have metal plates or screws in their spine. Myelography is primarily used to identify slipped disk by exactly locating the disk or disks concerned. Spinal arthritis can develop bone spurs that may press against the nerves and cause pain, myelography can help identify if surgery is required and can also help spot if the spinal canal is narrowed. Tumors can be accurately located. A patient preparing for myelogram must stop a solid diet for a day and drink plenty of clear fluids.


MRI scan

During a MRI scan, pictures of almost all the tissue in the body are taken. The tissue that has the least hydrogen atoms, such as the bones turns out dark. The tissues that have many hydrogen atoms, such as fatty tissue looks much brighter. The timing of the radio wave pulses is constantly changed to gain information about the different types of tissues present. An MRI scan is normally used as an extremely accurate method of disease detection and structural abnormalities throughout the body.

Patient with any metallic materials within the body must notify their physician prior to the MRI scan. This is because, metallic chips, materials, metal implants, surgical clips or foreign materials (artificial joints, metallic bone plates or prosthetic devices, etc) can significantly distort the images obtained by an MRI scanner. Patients with pacemakers, metal chips or clips in and around the eyeballs cannot be scanned by an MRI because of the risk that the magnet may move the metal in these areas. Patients with artificial heart valves, metallic ear implants, bullet fragments and chemotherapy or insulin pumps should not undergo MRI scanning.

MRI scan can cause a small risk to the fetus in the first 12 weeks of pregnancy. Therefore scans are not performed on pregnant women. The MRI machine produces loud thumping and humming noise when the procedure is performed. Therefore ear plugs are usually given to the patients to reduce the noise. MRI scans are a useful aid in the assessment of certain back conditions. MRI is an aid to detail studies of nerve root injuries like lumbar disc herniation and lumbar spinal stenosis. It is also extensively used in spinal disc and lumbar disc diseases and isthmic spondylolisthesis. It is also useful to rule out tumors and spinal infections.

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Bibliography / Reference

Collection of Pages - Last revised Date: October 21, 2019