Advances in the management of subependymal giant cell astrocytoma

Abstract

Subependymal giant cell astrocytoma (SEGA) is the most common central nervous system tumor in patients with tuberous sclerosis complex (TSC). Although these lesions are generally benign and non-infiltrative, they commonly arise in the region of the foramen of Monro, where they can cause obstructive hydrocephalus and sudden death. Surgical resection has been, and presently remains, the standard treatment for SEGAs demonstrating serial growth on neuroimaging in the setting of symptomatic hydrocephalus or progressive ventriculomegaly. Surgery can be curative; however, not all SEGAs are amenable to safe and complete resection. Gamma Knife stereotactic radiosurgery provides another treatment option but has highly variable response rates with limited data demonstrating its efficacy. Newer medical therapy targeting mammalian target of rapamycin (mTOR), the key protein kinase that is constitutively activated in TSC, has demonstrated promising results in recent clinical trials. In both case reports and clinical trials, treatment with mTOR inhibitors results in a significant reduction in SEGA volume and improvement or resolution of ventriculomegaly. This has led to the approval of everolimus for the treatment of SEGA in tuberous sclerosis patients who are not candidates for surgery. This review summarizes the surgical and medical management of SEGA in patients with TSC.