Pneumocytic adenomyoepithelioma: a distinctive lung tumor with epithelial, myoepithelial, and pneumocytic differentiation.

Pulmonary tumors with epithelial and myoepithelial differentiation are rare, thought to be of bronchial minor salivary gland origin and classified similarly to salivary gland neoplasms. We report a series of a distinctive subtype of pulmonary glandular tumors showing epithelial and myoepithelial differentiation with further pneumocytic specialization. All patients were women, aged 52 to 63 years and presented with single or multiple pulmonary nodules. The tumors were grossly circumscribed, 0.8 to 2.6 cm in greatest dimension, and histologically showed glandular and spindle cell differentiation. Some glands were filled with colloidlike secretion and had an inner, cuboidal epithelial cell layer (pankeratin, epithelial membrane antigen, and thyroid transcription factor-1 positive), surrounded by an outer layer of myoepithelial cells merging with foci of spindled myoepithelial cells (high molecular weight keratin, S100, smooth muscle actin, calponin, caldesmon, and p63 positive). There were also some glands lined by a single layer of plump cells that were positive for surfactant protein-A in addition to the other epithelial cell markers. Electron microscopy confirmed pneumocytic features in these cells and the myoepithelial nature of the spindled cells. The surgery in all cases was wedge resection of the masses. The biologic behavior to date has been benign. This is the first reported series of a distinctive lung tumor with epithelial, myoepithelial, and pneumocytic differentiation that differs histologically from all previously recognized pulmonary salivary gland-type and pneumocytic tumors. It is a unique benign appearing neoplasm for which the designation pneumocytic adenomyoepithelioma is suggested.