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Gangliogliomas are rare tumors found in the brain and spinal cord. They grow from groups of nerve cells (ganglion cells) and supportive cells (glial cells) in the central nervous system (CNS). Most gangliogliomas occur in children and young adults. They account for less than 2% of all CNS tumors. Gangliogliomas tend to be small, low grade tumors that do not spread. However, they can sometimes be malignant, higher grade tumors.
The most common place for gangliogliomas to develop is in the cerebrum, usually in the temporal lobe. However, they can occur anywhere in the central nervous system.
Surgery is the main treatment for gangliogliomas. Radiation therapy may be used if the tumor can’t be removed or comes back. Chemotherapy and targeted therapy may also be used. Doctors sometimes recommend watching small, low grade tumors if they do not appear to be causing problems. Children with low grade ganglioglioma usually do well and have a greater than 90% survival rate.
Symptoms depend on tumor size and location. Because gangliogliomas tend to grow slowly, symptoms often have a gradual onset. Seizures are a main symptom of ganglioglioma.
Ganglioglioma symptoms include:
Doctors evaluate ganglioglioma in several ways.
Gangliogliomas are generally low grade tumors. In most cases, they are slow growing and not considered malignant. However, about 5-10% of gangliomas are more aggressive, high grade tumors.
For children with ganglioglioma, the most important factor that affects prognosis is the tumor grade.
Other factors that influence chance of cure include:
The goal of treatment is gross total resection of the tumor. Gangliogliomas can often be managed with surgery alone and then followed with imaging.
Radiation therapy may be used if surgery cannot completely remove the tumor or if the cancer returns. Doctors try to limit the use of radiation in low grade tumors due to the risk of late effects.
Chemotherapy may also be used in certain cases if the tumor is not fully removed with surgery. Chemotherapy depends on the age of the child and location of the tumor. Low grade gangliogliomas are treated with the same chemotherapy treatment plans used for low grade astrocytomas.
Targeted therapies work by acting on, or targeting, specific features of tumor cells. In some gangliogliomas, there appears to be a change in the BRAF gene in cells of the tumor. This change is called a V600E BRAF mutation. The BRAF gene helps to control a protein important for cell growth and function. Certain drugs can block this specific signaling pathway. These V600E BRAF inhibitors include vemurafenib and dabrafenib.
Periodic imaging is used to monitor patients after treatment. Like other low grade gliomas, there is higher risk of recurrence if the tumor was not fully resected. Follow-up care should include appropriate rehabilitation, psychological, and neurological consultations as needed. Many patients will take anti-seizure medication after surgery, but medications can often be tapered and stopped after a period of time.
Reviewed: June 2018