Management of subependymal giant cell astrocytoma (SEGA) associated with tuberous sclerosis complex (TSC): Clinical recommendations

Abstract

Subependymal giant cell astrocytoma (SEGA) is a type of brain tumour that develops in 10-15% of individuals with tuberous sclerosis complex (TSC). SEGAs can be unilateral or bilateral, developing from benign subependymal nodules (hamartomas) located near the foramen of Monro. These are usually slow-growing, glialneuronal tumours that develop within the first 2 decades of life. Traditionally, the management of SEGA involved monitoring using periodic neuroimaging, and surgical resection of tumours that exhibited growth and/or caused clinical signs of intracranial hypertension. Recent clinical research has demonstrated that mammalian target of rapamycin (mTOR) inhibitors can induce partial regression of SEGA associated with TSC and so might provide an acceptable alternative to neurosurgery for these tumours. This report summarizes the clinical recommendations for the management of SEGA made by a panel of European experts in March 2012. Current treatment options and outstanding questions are outlined.