Subependymal Giant Cell Astrocytomas in Children Homepage

Authors

Chandrashekhar E. Deopujari, M.Ch.
Dattatreya Muzumdar, M.Ch.
Varun Badami, B.S., B.A.

Section Editor

Paul Steinbok, M.D.

Editor in Chief

Rick Abbott, M.D.

Introduction

SEGA is a benign intraventricular tumor, associated with TSC. SEGA is an intraventricular astrocytoma of the circumscribed type that occurs in 5–15% of patients with TSC, usually in the second decade of life (3, 5, 8, 10, 22, 28) and is considered a low-grade, W.H.O. grade I tumor. Isolated cases are extremely rare (24). Although SEGAs are neither cancerous nor malignant, their location, typically adjacent to the foramen of Monro, can cause hydrocephalus.

TSC, also called Pringle-Bourneville disease, is an autosomal dominant neurocutaneous syndrome (phakomatosis) characterized by the triad of seizures, mental retardation, and facial adenoma sebaceum (32). There is also widespread development of hamartomas in multiple organs, including the heart, kidneys, and brain. In the brain, the lesions typically manifest as cortical or subependymal hamartomas or tubers (9, 24).

Key Points

  • Benign tumor projecting into ventricle: SEGA is a benign intraventricular tumor associated with TSC. It occurs usually adjacent to the foramen of Monro and causes hydrocephalus by blocking the foramen.
  • Surgery curative but chemotherapy now available: Complete surgical resection is usually curative. SEGAs are responsive to medical treatment with mTOR inhibitors, such as rapamycin.