Surgical removal has been the historical treatment for subependymal giant-cell astrocytoma (SEGA) in tuberous sclerosis complex (TSC) patients. In the past decade, mTOR inhibitors have shown efficacy in the treatment of SEGA, significantly reducing tumor size. The aim of this study was to assess the safety and efficacy of surgical treatment at a time when mTOR inhibitors have changed standard treatment.

We conducted a single-center retrospective study including all patients treated by surgery for SEGA from October 2003 to September 2019, with a review of all SEGA surgical case series, following PRISMA guidelines. Research focused on demographics, surgical indications, surgical approach, use of CSF shunt, morbidity and mortality, resection quality, recurrence rate and treatment of recurrence, follow-up and long-term clinical status.

Eleven patients were included, with a median age at surgery of 16.0 years. Gross total resection was achieved in 8 patients (72%), with no permanent morbidity. One patient needed further surgery for tumor recurrence. Eighteen studies were reviewed, totaling 263 TSC patients affected by SEGA and 286 surgical procedures. Gross total resection was achieved in 81.1% of cases, mortality was 4.9% and permanent morbidity 6.1%. Tumor recurrence occurred in 11.5% of cases, and was secondary to partial tumor resection at first surgery in the majority of cases.

Surgical treatment of SEGA is still a valid and effective option. Morbidity is low and complete disappearance of SEGA can be achieved in selected cases.