Subependymal giant cell astrocytoma in children with tuberous sclerosis.

METHODS

Out of 105 patients with tuberous sclerosis (TS) admitted to the Hospital Nacional de Pediatría "Juan P. Garrahan" (Buenos Aires, Argentina), we surgically treated 17 children between January 1988 and December 2000. Two patients were operated on because of epilepsy and 15 patients because of an intraventricular tumor (subependymal giant cell astrocytoma [SGCA]). In this report we focus on tumors. Twelve of the 15 patients presented with hydrocephalus but none of them had a preoperative shunt. All tumors were surgically resected using frontal transventricular or transcallosal routes. Total removal was achieved in 12 out of 15 and subtotal removal in 3 out of 15 patients (resection of 70-95%).

RESULTS

Exeresis of the tumor was not accompanied by significant morbidity and there was no perioperative mortality. Seizures and mental retardation did not improve after tumor resection. It was necessary to insert a postoperative shunt a long time after surgery in only one patient. There were no recurrences of SGCA that were totally removed after a mean follow-up of 51.7 months.

CONCLUSIONS

We encourage surgery as soon as a lesion is diagnosed as a tumor. The "transformation" of subependymal nodules (SEN) into tumors (SGCA) may be considered controversial.