Clinical Implications of Solitary Subependymal Giant Cell Astrocytoma in the Absence of Tuberous Sclerosis Complex: Case Series and Comprehensive Literature Review.

Abstract

Subependymal giant cell astrocytoma (SEGA) is a slow-growing glial or glioneuronal tumor that almost exclusively occurs in patients with Tuberous Sclerosis Complex (TSC), a rare autosomal dominant condition that causes growth of benign tumors throughout the body. Herein, we present 4 cases of isolated SEGA in patients with negative germline testing for TSC alterations and present a comprehensive literature review of other cases of sporadic SEGA. This case series emphasizes the importance of considering SEGA on the differential diagnosis for periventricular tumors even in the absence of other sequelae of TSC and illustrates the importance of long-term monitoring for tuberous sclerosis-related complications.