Pulmonary Lymphangiomyomatosis: A case report

ABSTRACT

Pulmonary lymphangiomyomatosis is a rare disease of haphazard overproliferation of smooth muscle from perilymphatics, peribronchial and perivascular regions of the lung that leads to chylous effusion, hemoptysis, spontaneous pneumothorax and pulmonary insufficiency. The disease only affects women of child-bearing age. There is presently no successful therapy and most of the patients die within 10 years of diagnosis. We experienced a case of pulmonary lymphangiomyomatosis in a 30-year-old woman who had suffered from three episodes of spontaneous pneumothorax for 4 years. Open lung biopsy was performed. Microscopically, abnormal excess proliferation of smooth muscle tissue was identified in the wall of perivascular lymphatic channels as well as around small bronchi, and more distal air spaces. Varying sized cytic spaces Iined by cuboidal cells were also present. Tamoxifen, given if February 1991, did not influence the course of her disease. She died of respiratory failure at age of 31 in April 1991.