A Case of Tuberous Sclerosis with Suspicious Malignancy

J Korean Neurol Assoc > Volume 5(1); 1987 > Article

Journal of the Korean Neurological Association 1987;5(1): 97-102.

악성변화가 의심되는 결절성 경화증 1례

이창훈, 김승현, 김주한, 김명호

한양대학교 신경과.

A Case of Tuberous Sclerosis with Suspicious Malignancy

Chang-Hoon Kim, M.D., Seung-Hyun Kim, M.D., Ju-Han Kim, M.D., Myung-Ho Kim, M.D.

Department of Neurology, College of Medicine, Han Yang University

Abstract

This case, a 15-year-old female, is peculiar because she had more numerous characteristic lesions of tuberous sclerosis than other previous cases in Korea. In addition to the typical triad of tuberous sclerosis, she had shagreen patches, cafe-au-lait spot, angiomyolipomas in both kidneys, phacomas in both eyes, periventricular calcifications, and hydrocephalus due to a mass around the foramen of Monro. Biopsy, taken after complete removal of a mass around the foramen of Monro, revealed hamartoma which was made up of subependymal giant cell astrocytoma, gliosis, and spongioblastoma. With reviewing some of literatures, we present a case of the tuberous sclerosis.