All issues > Volume 39(2); 1996

Case Report

J Korean Pediatr Soc. 1996;39(2):280-286. Published online February 15, 1996.

A Case of Tuberous Sclerosis Complex Developing with Subependymal Giant-Cell Astrocytoma and Hydrocephalus

Ki Hyun KH Chung¹, Wan Sub WS Kim¹

¹Department of Pediatrics, Presbyterian Medical Center, Chonju, Korea

Abstract

The subependymal giant-cell astrocytoma, a key feature of the tuberous sclerosis complex, has been reported in patients without obvious features of this condition. The tumor is a rare cerebral glioma that characteristically arise in the wall of lateral ventricle and as an intraventricular mass, causing hydrocephalus by obstruction of the foramen of Monro. We experienced a case of tuberous sclerosis in 9-year-old male patient who presented typical clinical manifestations and cortical tuber, subependymal giant-cell astrocytoma developing with hydrocephalus. Diagnosis was confirmed by brain CT and MRI. We applied surgical resection to remove the mass. Brief review and related literatures were also presented.

Keywords :Subependymal giant-cell astrocytoma, Tuberous Sclerosis Complex