GANGLIOGLIOMA

OVERVIEW

A gangliogliomas is a glioneuronal tumor that is cortically based, and may have a solid and/or multicystic appearance. Histologically, gangliogliomas are characterized by large ganglion-like cells, intermixed with glial (predominantly astrocytic) cells. They are commonly found in the temporal lobes, but can be found elsewhere. They may co-occur with adjacent focal cortical dysplasia (FCD IIIb), suggesting a common developmental etiology for both structural abnormalities.

Clinical context

The clinical presentation of a ganglioglioma depends on the location of the tumor and the presence of co-occurring structural abnormality such as focal cortical dysplasia. Onset of seizures can be at any age, but is most commonly in childhood. Males and females are equally affected. Patients do not initially suffer from significant developmental, neurological or cognitive impairment, but this can occur in association with uncontrolled seizures.

CAUTION developmental and cognitive outcome may be worse in children with uncontrolled seizures in early life, especially if epileptic spasms or generalized seizure types appear, therefore proactive seizure control is important in these patients.

CAUTION as epilepsy surgery is expected to be associated with a high chance of permanent seizure freedom, this should be pursued in a timely fashion if seizures are uncontrolled with medication, if there are emerging developmental, cognitive or psychiatric impairments, or if epileptic spasms or generalized seizure types are seen.