결절성 경화증에 동반된 폐림프관평활근종증과 소결절성폐포세포증식:1예 보고
Pulmonary Lymphangioleiomyomatosis and Micronodular Pneumocyte Hyperplasia associated with Tuberous Sclerosis;A Case Report
김교영, 이주희, 박용구, 김윤화, 박재훈, 양문호,
소속 상세정보
김교영 ( Kim Gou-Young )
경희대학교 의과대학 병리학교실
이주희 ( Lee Ju-Hie )
경희대학교 의과대학 병리학교실
박용구 ( Park Yong-Koo )
경희대학교 의과대학 병리학교실
김윤화 ( Kim Youn-Hwa )
경희대학교 의과대학 병리학교실
박재훈 ( Park Jae-Hoon )
경희대학교 의과대학 병리학교실
양문호 ( Yang Moon-Ho )
경희대학교 의과대학 병리학교실
KMID : 0357920020360010051
Abstract
Lymphangioleiomyomatosis (LAM) is characterized by a hamartomatous proliferation of smooth muscle cells in the lung, mediastium, and abdomen. In the lung, an abnormal proliferation of smooth muscle is seen along the airways, blood vessels, and lymphatics, resuliting in honeycombing of the lung. It occurs in 0.1-1% of tuberous sclerosis (TSC) patients. Micronodular pneumocyte hyperplasia (MNPH)is a rare but distinctive pumonary manifestation of TSC, and appears to be a hamartomatous proliferation of the type Ⅱ pneumocytes. We report a case of pulmonary LAM and MNPH associated with TSC and bilateral renal angiomyolipoma in a 26-year-old woman. Immunohistochemicalty, the spindle cells of LAM were positive for HMB-45, but the type Ⅱ pneumocytes of MNPH were negative.
키워드
Lung Neoplasms;Tuberous Sclerosis
원문 및 링크아웃 정보
등재저널 정보