Shuman R L, Engelman R, Kittle C F
Ann Thorac Surg. 1979 Jan;27(1):70-5. doi: 10.1016/s0003-4975(10)62975-x.
Pulmonary lymphangiomyomatosis is a rare but distinct clinical and pathological entity. It is characterized by hamartomatous proliferation of smooth muscle around the lymphatic vessels of the lung, mediastinum, and retroperitoneum. It occurs only in menstruating women and girls and is manifested by spontaneous pneumothorax and chylous pleural or abdominal effusion. As it progresses, the lungs become increasingly involved with subsequent pulmonary insufficiency and recurrent infection. Eventually death results. This entity has not been reported previously in the surgical literature even though the thoracic surgeon is called on to both establish the diagnosis and aid in palliation. The hypothesis that the disease is estrogen dependent is reviewed and the desirability of estrogen ablation in patients with positive estrogen receptors is suggested.
肺淋巴管平滑肌瘤病是一种罕见但独特的临床和病理实体。其特征是肺、纵隔和腹膜后淋巴管周围平滑肌的错构瘤样增生。它仅发生于育龄期女性和女孩，表现为自发性气胸和乳糜性胸腔或腹腔积液。随着病情进展，肺部受累日益加重，继而出现肺功能不全和反复感染。最终导致死亡。尽管胸外科医生既要负责确立诊断又要协助缓解症状，但此前手术文献中尚未报道过该实体。本文回顾了该疾病依赖雌激素的假说，并提出对雌激素受体阳性患者进行雌激素去除治疗的必要性。
