In 1930, Courville defined the term ganglioglioma as tumors consisting of astrocytic neuronal components with rare mitotic figures (see Figure 8).140 Approximately 50% show focal areas of calcification. Generally, they are benign growths that occur over a long clinical course and present with no specific radiologic characteristics.144

Ganglioglioma Neuro 8

 

Gangliogliomas make up between 0.3% and 8.0% of primary brain tumors, with the higher rates in children. The mean age at presentation is 9.5 years.30,141,142

Distribution and presentation

Most are located supratentorially, often in the temporal lobes.143  In one study of 137 patients, 65% were located in the frontal, temporal, or frontotemporal regions, versus only 8% in the midline or deep structures.141(See Figure 2.)

In a study by Otsubo and colleagues, 59% of patients presented with seizures. Of these, 64% had complex partial seizures, 32% had secondarily generalized seizures, and 4% presented with generalized seizures.

Treatment and prognosis

Optimal treatment includes gross total resection. Adjuvant chemotherapy or radiation therapy is generally not indicated.

Postoperative 5-year survival of hemispheric gangliogliomas has been reported as 93%, although seizure relief statistics were lower than expected.145 Morris and colleagues reported freedom from seizures in 79% (30 of 38) of patients at 6 months, and 72% at 1 year postoperatively.137 Favorable prognostic factors included younger patient age, benign histopathology, stable clinical status at admission, decreased duration of seizure disorder, and lack of generalized features of seizures.137,141

Another group reported that among patients who presented with neurologic deficits or symptoms of increased intracranial pressure, those who were given adjuvant radiation had a postoperative 5-year survival of 53% versus 11% for those who did not receive radiation.141

Rarely, these tumors (as well as dysembryoplastic neuroepithelial tumors [DNETs]) may be associated with a rare postoperative disorder, with schizophreniform features of paranoia, depression, and psychosis. This psychosis does not seem to accompany other tumor types resected for intractable epilepsy.151,152

Adapted from: Mangano FT, McBride AE, and Schneider SJ. Brain tumors and epilepsy. In: Ettinger AB and Devinsky O, eds. Managing epilepsy and co-existing disorders. Boston: Butterworth-Heinemann; 2002;175–194.
With permission from Elsevier (www.elsevier.com). 

Authored By: 
Steven C. Schachter, MD
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Authored Date: 
03/2004