All issues > Volume 34(7); 1991

Original Article

J Korean Pediatr Soc. 1991;34(7):1027-1033. Published online July 31, 1991.

A case of tuberous sclerosis developing with subependymal giant-cell astrocytoma.

Young Chul Ahn¹, Yong Suk Choi¹, Pyoung Han Hwang², Jung Soo Kim²

¹Department of Pediatrics, Chungju LeeRha Hospital, Chungju, Korea
²Department of Pediatrics, College of Medicine, Chonbuk National University, Chonju, Korea

Received: March 4, 1991;  Accepted: April 24, 1991.

Abstract

Subependymal giant-cell astrocytoma is a rare cerebral glioma that characteristically arises in the wall of the lateral ventricle and presents as an intraventricular mass, causing hydrocephalus by obstruction of the foramen of Monro. The tumor mainly occurs in patients with tuberous sclerosis and is slow growing and well circumscribed, so that long survival after total surgical resection is often achieved. We experienced a cas^ of tuberous sclerosis in 9-year-old male patient who presented typical clinical manifestations and subependymal giant-cell astrocytoma developing with tuberous sclerosis were biologically benign. Diagnosis was confirmed by brain CT, facil skin biopsy and tumor mass biopsy. We applied surgical resection in order to remove the mass. Tumor removal relieved attending symptoms and achieved survival without notable functional impairment. Herein, we presented the case with a brief review of the related literatures.

Keywords :Subependymal giant-cell astrocytoma;Tuberous Sclerosis