摘要篇首： Lymphangiomyomatosis(LAM)is a progressive disease affecting women of childbearing age.It is microscopically characterized by abnormal smooth muscle proliferation that causes gradual obstruction of small airways,lymphatics and vasculature.The proliferation eventually results in a characteristic clinical syndrome of progressive dyspnea,recurrent pneumothorax,chylous effusion and hemoptysis.The first cases of LAM were reported by Von Stosse1 in 1937.Comog and Enterline2 first recognized lAM as a distinct syndrome in 1966.Less than 200 cases of LAM have been reported thus far,of which 6 cases have been reported in China,to our knowledge.LAM is a rare disease unfamiliar to many physicians,and it may pose a diagnostic dilemma for the primary physician,possibly resulting in delayed or missed diagnosis,unnecessary surgical intervention,and inappropriate therapy.This study investigates the clinical,radiographic,pathological and therapeutic features of 2 LAM cases and reviews the literature on LAM.