Probable initial pulmonary lymphangioleiomyomatosis and mediastinal lymphangioleiomyoma

A 68 year old woman was submitted to a mediastinal lymphangioleiomyoma resection found in a follow-up study of lower left lung resection due to bronchiectasis complicated by chylothorax. This led to a revaluation of the pulmonary specimen that revealed, in addition to inflammatory bronchiectasis, small spindle cell nodules in the lung parenchyma, similar to minute pulmonary meningothelial-like nodules, but with smooth muscle actin immunohistochemical positivity. The possibility of initial pulmonary development of lymphangioleiomyomatosis is discussed.