ABSTRACT

Based on the combination of slow evolution, the extended period of symptomatology prior to diagnosis, and multiple histopathological studies, ganglioglioma was postulated to have a hamartomatous or maldevelopmental origin. It is rare, accounting for only 0.4"–1.3" of all central nervous system neoplasms but about 70" of neuronal and mixed neuronal-glial tumors. Most recently, the PI3K-mTOR pathway, which is responsible for cell size, growth control, cortical development, and neuronal migration, has been shown to play a critical role in the specific pathogenesis of gangliogioma. Surgical intervention with the goal of gross total resection is the first modality of treatment for ganglioglioma. Early surgical resection of ganglioglioma reduces both morbidity and mortality as it pertains to seizures as well as recurrence. Features associated with poor prognosis of ganglioglioma are older age at time of surgery, subtotal resection, extratemporal location, and absence of chronic epilepsy.