폐의 임파관 평활근종 -1예 보고-
Lymphangiomyomatosis of the Lung -A report of a case-
소속 상세정보
지미경/Mi-Kyung Jae
양기화/김병기/김선무/Ki-Hwa Yang/Byoung-Kee Kim/Sun-Moo Kim
KMID : 0357919840180040437
Abstract
서 론
임파관평활근종증은 후복막강과 종격동내의 임파관과 폐에 평활근 증식을 나타내는 비교적
드문 질환으로서 폐의 병변된이 후복막강의 임파계에만 병변이 생긴 예도 보고되어 있다.
Cornog등은 과거에 임파관종, 임파관평활근종, 평활근종증, 임파관종성 기형, 임파관주세포
종등으로 보고되었던 예들이 이 병변과 동일한 것임을 주장하면서 임파관평활근종증이 라는
명칭이 합당하다고 했다.
저자들은 1983년 4월 반복되는 기흉을 주소로 입원한 23세 여자환자의 우폐에서 발견한 낭
성종괴가 임파관평활근종의 소견과 일치하여 보고하고저 한다.
#초록#
Pulmonary lymphangiomyomatosis is a rare but distinct condition caused by progressive
widespread smooth muscle proliferation in the perilymphatic regions throughout the lung.
The disease is clinically manifested by recurrent chylous pleural effusion,
pneumothoraxor ascites and striking female sex predominance. A relationship is noted
between lymphangiomyomatosis and certain secondary lesions associated with tuberous
sclerosis.
The authors experienced a case of pulmonary lymphangiomyomatosis in 23 years o1d
female patient who had been suffered due to recurrent pneumothorax for two weeks.
During explorative thoracotomy, a huge cystic mass, 18.5×8.0×3.0㎝ and weighing 100
gm, was noted in the lower lobe of the right lung. The external surface was tan brown
and dirty with rubbery consistency. The inner surface was also tan brown and dirty
with fibrous meshwork. Microscopically, there were varying sized cysts lined by
flattened cuboidal cells. There were irregular and nodular proliferation of smooth muscle
bundles in the wall of the cysts.
키워드
원문 및 링크아웃 정보
등재저널 정보