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

Journal of the Korean Pediatric Society 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 Chung, Wan Sub Kim
Department of Pediatrics, Presbyterian Medical Center, Chonju, Korea
뇌수두증과 상의하 거대세포 성상세포증이 동반된 결절성 경화증 1례
정기현, 김완섭
전주예수병원 소아과
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.
Key Words: Subependymal giant-cell astrocytoma, Tuberous Sclerosis Complex


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