Surgical treatment of subependymal giant cell astrocytoma in patients with tuberous sclerosis complex – an institutional experience and results

Abstract

Objective Subependymal giant cell astrocytoma (SEGA) present in patients with tuberous sclerosis complex (TSC), occasionally requiring surgical removal. The study aimed to analyze the results from our series of children undergoing surgery for SEGA. Methods We retrospectively identified children with TSC undergoing resection of SEGA at Oslo University Hospital between 1982 to 2016. Patient charts, radiological images, epilepsy and neuropsychological reports were reviewed. Results Out of 208 patients with TSC, 18 (9%) underwent resection of SEGA. Due to missing data, we could only analyze results from 14 surgeries in 11 children (median age 6 years, range 0–19; male/female ratio 2.7:1). The tumours were bilateral in four (36%) patients. The tumor diameter was median 19 mm (10–104 mm). The surgical approach was transcortical in eight (57%) and transcallosal in six surgeries (43%). Gross total resection was achieved in 12 (86%) of surgeries. There was no mortality or major morbidity related to surgery, but one patient developed complications related to the ventriculoperitoneal shunt. During the follow-up (median 11 years, range 1–21), three patients (27%) underwent repeated surgery. We could not document any significant impact of the surgery on patients` cognitive functioning or the grade of epilepsy. Conclusions Resection of SEGA in children with TSC was associated with a low complication rate. We could not document any impact of surgery on patients` cognitive functioning or grade of epilepsy. However, the neuropsychological data were limited in most cases. Neuropsychological assessment should be performed before the surgery and be a part of follow-up after surgery.