Ganglioglioma

Ganglioglioma is a rare, slow-growing brain tumor classified as a WHO grade I neoplasm, though higher-grade variants (anaplastic ganglioglioma, WHO grade III) can occur. It is composed of a mixture of neoplastic ganglion cells (mature neurons) and neoplastic glial cells, distinguishing it from other central nervous system tumors. Gangliogliomas most commonly arise in the temporal lobe but can occur anywhere in the central nervous system, including the cerebral hemispheres, brainstem, spinal cord, and cerebellum. They are among the most common tumors associated with chronic, drug-resistant epilepsy, particularly in children and young adults. The hallmark clinical feature of ganglioglioma is seizures, especially temporal lobe epilepsy, which may be present for years before diagnosis. Other symptoms depend on tumor location and may include headaches, focal neurological deficits, increased intracranial pressure, visual disturbances, and cognitive or behavioral changes. In spinal cord gangliogliomas, patients may experience pain, weakness, or sensory changes in the extremities. The primary treatment for ganglioglioma is surgical resection, which is often curative when gross total removal is achieved. Patients who undergo complete resection generally have an excellent long-term prognosis, with five-year survival rates exceeding 90% for grade I tumors. For incompletely resected or recurrent tumors, adjuvant radiation therapy may be considered. Chemotherapy has a limited role but may be used in anaplastic (grade III) variants. A significant proportion of gangliogliomas harbor the BRAF V600E mutation, which has opened the door to targeted molecular therapies in select cases. Long-term follow-up with neuroimaging is recommended to monitor for recurrence.

Common questions about Ganglioglioma