Multifocal micronodular pneumocyte hyperplasia: a distinctive pulmonary manifestation of tuberous sclerosis

We report a peculiar multifocal micronodular proliferation of pneumocytes occurring in a 24-yr-old woman with tuberous sclerosis and lymphangioleiomyomatosis. A computed tomographic scan of the chest demonstrated multiple minute nodules present throughout both lung fields. Histologically, the nodules were well demarcated, measured up to 1.6 mm in diameter, and were composed of thickened, fibrotic, alveolar septa lined by pleomorphic, type II pneumocytes. Positive immunohistochemical stains for keratin, BER-EP4, and surfactant, and negative immunohistochemical staining with an antibody recognizing Clara cells support an epithelial origin from type II pneumocytes. The absence of immunohistochemical staining for HMB45 suggests a histogenesis separate than the lesions of lymphangioleiomyomatosis. We failed to detect estrogen or progesterone receptors in either the lesions of lymphangioleiomyomatosis or the micronodular proliferations. Recognition of these unique lesions facilitates their distinction from other epithelial proliferations, particularly atypical bronchioloalveolar cell hyperplasia. This lesion appears to be a distinctive manifestation of tuberous sclerosis. It is probably hamartomatous.