결절성 경화증에서 발생한 비장의 과오종 1례

• 영문명: A Case of a Sp lenic Hamartoma in Tuberous Sclerosis
• 발행기관: 대한소아신경학회
• 저자명: 진혜영(Hye Young Jin) 김선영(Sun Young Kim) 이건수(Keon Su Lee)
• 간행물 정보: 『Annals of Child Neurology(구 대한소아신경학회지)』대한소아신경학회지 제14권 제1호, 164~168쪽, 전체 5쪽
• 주제분류: 의약학 > 의학일반
• 파일형태: PDF
• 발행일자: 2006.05.30

국문 초록

저자들은 좌상복부 종괴를 주소로 내원한 8세 여아에서 결절성 경화증에 동반된 비장의 과오종을 보인 증례를 경험하였기에 보고하는 바이다.

영문 초록

Tuberous sclerosis is a neurocutaneous syndrome with autosomal dominant inheritance with variable penetrances and the incidence is approximately one in 10,000 live births. It is described as a clinical triad of mental retardation, seizures and adenoma sebaceum. Tuberous sclerosis is characterized by the development of hamartomas and benign neoplasms involving the brain and other organs. Typical lesions include cortical tubers and subependymal hamartomas in the brain, rhabdomyomas in the heart, angiofibromas and periungual fibromas of the skin and angiomyolipomas in the kidney. A splenic hamartoma is a rare benign lesion. Only about 100 cases have been reported since its first description by Rokitansky in 1861. We report a case of child with a splenic hamartoma associated with tuberous sclerosis.

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키워드

Tuberous sclerosis Splenic hamartoma