Lymphangioleiomyomatosis (LAM) is a rare lung disease traditionally affecting women during their childbearing years. It can be sporadic or be associated with tuberous sclerosis syndrome. It is usually manifested in the lungs, kidneys, and/or lymphatic system. It consists of an overgrowth of abnormal smooth muscle-like cells, usually along the bronchovascular structures, resulting in the formation of cysts and the destruction of the lung parenchyma. We present the case of a 43-year-old woman with a history of pleural effusion and dyspnea. A computed tomographic scan revealed a mediastinal mass, chylothorax, and multiple pulmonary cysts. A diagnosis of LAM was rendered on a pleural fluid sample.
Keywords: Lymphangioleiomyomatosis; pleural effusion; spindle cells; tuberous sclerosis.