subependymal giant cell astrocytoma
Also written as: SEGA
A benign WHO grade I tumor strongly associated with tuberous sclerosis complex, typically occurring near the foramen of Monro.
Full Definition
Subependymal giant cell astrocytoma (SEGA) is a WHO grade I benign brain tumor that occurs almost exclusively in patients with tuberous sclerosis complex (TSC), a genetic disorder characterized by tumor formation in multiple organs. These tumors typically develop from subependymal nodules near the foramen of Monro and can cause obstructive hydrocephalus due to their strategic location. SEGAs are composed of large astrocytic cells with abundant cytoplasm and may contain calcifications. While histologically benign, they can be life-threatening due to their location and propensity to cause CSF obstruction. Treatment options include surgical resection or targeted therapy with mTOR inhibitors such as everolimus.
Usage
Usage note: Virtually pathognomonic for tuberous sclerosis complex when present.
In Context
- "The TSC patient developed a subependymal giant cell astrocytoma requiring urgent surgical intervention for hydrocephalus." — Emergency consultation note
- "Everolimus therapy resulted in significant reduction of the subependymal giant cell astrocytoma volume." — Treatment response assessment