Pulmonary lymphangioleiomyomatosis: a case report with immunohistochemical details and DNA analysis.

A 47-year-old woman is presented with pulmonary lymphangioleiomyomatosis (PLAM) involving the bilateral lung and slight pulmonary function abnormality. Computed tomography scan showed bilateral microcyst formation in the lung. Histologically, proliferating spindle shaped cells with centrilobular emphysema were main findings. Immunohistochemically, these proliferating spindle shaped cells were positive for alpha-smooth muscle actin, desmin, vimentin, HMB45, estrogen receptor and progesterone receptor, but negative for S-100, cytokeratin. Single strand conformation polymorphism (SSCP) and DNA analysis for tuberous sclerosis 1 and 2 showed no significant abnormality.