Ganglioglioma comes from the Greek word “ganglion” meaning “knot,” the Greek word “glia” meaning “glue,” and the Greek word “oma” meaning “tumor.” Ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently occurs in the temporal lobes of children and young adults.
Gangliogliomas were first described by CB Courville in his 1930 paper, “Ganglioglioma; tumor of central nervous system. Review of the literature and report of 2 cases,” published in the Archives of Neurology and Psychiatry, Volume 24, pages 439-491. He described gangliogliomas as abnormal growths of new tissues that contained neurons (nerve cells) and types of glial cells known as astrocytes
A ganglioglioma is a rare type of brain tumor. It accounts for approximately 1 percent of all brain tumors. Gangliogliomas occur when a single cell in the brain starts to divide into more cells, forming a tumor. This can occur when the cell randomly acquires changes (mutations) in genes that regulate how a cell divides. Most gangliogliomas grow slowly and are considered benign. However, up to 10 percent of gangliogliomas may grow more rapidly and become malignant, meaning the tumor affects the surrounding brain tissue.
These tumors occur most commonly in the cerebrum, the part of the brain that controls motor, sensory and higher mental function. They rarely spread to other parts of the brain or transform into higher-grade tumors. Children with certain genetic syndromes, including neurofibromatosis 1 and tuberous sclerosis, are at high risk of developing glial tumors, including gangliogliomas; however, most of these tumors develop spontaneously. Gangliogliomas are associated with a high rate of survival. The survival rate is not as high if the tumor can’t be removed completely during surgery or if the tumor returns.
The main treatment for ganglioglioma is removal of the entire tumor during surgery. If the entire tumor is not removed, it has the potential to recur and may require additional surgery or treatments, such as radiation therapy or chemotherapy. Gangliogliomas are associated with a high rate of survival. The survival rate is not as high if the tumor can’t be removed completely during surgery or if the tumor returns.
What is Ganglioglioma Tumor?
Gangliogliomas are tumors that start from groups of nerve cells (ganglion and glial cells) and grow in the brain. They are usually small and noncancerous, and they do not spread to other parts of the body. Gangliogliomas are generally benign WHO grade I tumors; the presence of anaplastic changes in the glial component is considered to represent WHO grade III (anaplastic ganglioglioma). Criteria for WHO grade II have been suggested, but are not established. Malignant transformation of spinal ganglioglioma has been seen in only a select few cases. Poor prognostic factors for adults with gangliogliomas include older age at diagnosis, male sex, and malignant histologic features.
Ganglioglioma Tumor Symptoms
Gangliogliomas grow relatively slowly, a person may have been having symptoms for many months prior to diagnosis, or symptoms may appear more suddenly. Many people with gangliogliomas first present with seizures. Other symptoms are associated with increased pressure in the brain, including:
- Vision problems
- Cognitive problems
- Behavioral problems
- Balance problems
- Abnormal eye movements
- Personality changes
- Impaired memory
- Speech problems
- Increased intracranial pressure
- Abnormal sensations
Ganglioglioma Tumor Treatment
Ganglioglioma recommended treatment for progressive or recurrent ganglioglioma is radiation therapy. Clinical trials and experimental therapies are also available for children with recurrent tumors that are not responsive to standard therapies.
1. Neurosurgery to remove as much of the tumor as safely possible; surgery is often limited due to the deep, central placement of these tumors within the brain
2. Chemotherapy either before surgery to shrink the tumor or to kill cancer cells
3. Radiation therapy precisely targeted treatment to control local growth of tumors; not recommended unless the child’s tumor has re-grown due to potential long-term side effects of therapy
Ganglioglioma Tumor Prognosis
The prognosis of people with gangliogliomas is excellent if it has been totally removed. The excellent prognosis in people with gangliogliomas is helped by the fact that most of these tumors are no aggressive, meaning that they do not spread very fast. Thus, it is usually easy for surgeons to remove these tumors because they are typically found in one place and can be separated from the brain tissue. Most people are cured after such surgery and the chances of the tumor coming back after it has been totally removed is rare. The ten-year survival rate in children who have been treated with ganglioglioma is about 90%. Prognosis of people with gangliogliomas is better the earlier it is diagnosed and treated.