신혈관근지방종을 동반한 결절성 경화증에서 발생한 폐림프관평활근종증 1예

A Case of Pulmonary Lymphangioleiomyomatosis Developed in Tuberous Sclerosis with Renal Angiomyolipoma

결절성 경화증의 폐침윤은 드문 것으로 알려져 있는데 저자들은 결절성 경화증과 신장의 혈관근지방종으로 추적 관찰중인 환자에서 폐의 기흉이 발생하여 고해상 전산화단층촬영과 병리조직소견에서 HMB-45 양성인 평활근의 증식을 보인 폐의 림프관평활근종증을 진단받은 1예를 경험하였기에 보고하는 바이다.

Tuberous sclerosis is a rare hereditary anomaly of variable penetrance characterized by mental retardation, epilepsy, and adenoma sebaceum. Pulmonary involvement is uncommon, but, when involved by tuberous sclerosis, it shows characteristic reticulonodular infiltration and cystic changes. Lymphangioleiomyomatosis is a rare disease of unknown cause that affects women of reproductive age. It is characterized by progressive proliferation of smooth muscle in the lung. We experienced one case of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis in a 26-year-old female patient. This case is reported with a brief review of the literature.