Epithelial-myoepithelial carcinomas are very rare in the lung, and little i
s known about the relationship of their histologic features to prognosis. W
e describe five primary pulmonary epithelial-myoepithelial carcinomas with
details on clinical presentation, histology, and immunohistochemical profil
es. We also reviewed the literature to detail further their prognosis. The
patients' ages ranged from 33 to 57 years (average 51 years), The tumors we
re all endobronchial and the patients presented with symptoms or imaging fe
atures of airway obstruction. The tumors were completely resected none show
ed nodal involvement. All five patients are alive and free of disease 4 mon
ths to 8 years (average 4.2 years) after surgery. Four tumors showed a mixe
d pattern of glands lined by a dual layer of cells and solid sheets of eith
er spindle cells or clear cells, the glandular and solid components being p
resent in variable proportions. The fifth tumor comprised purely spindle ce
lls. The mitotic rate was <1/20 high power fields in both the glandular and
spindle/clear cell components. In one case there was focal nuclear pleomor
phism. The inner layer of the glands stained for cytokeratins and epithelia
l membrane antigen, and the outer layer for S-100 and smooth muscle actin.
In one case the spindle cells stained for CD34. A review of published cases
shows the majority of tumors behave in an indolent fashion, the rare aggre
ssive tumors being predominantly myoepitheliomatous. Nevertheless, the term
epithelial-myoepithelial carcinoma is preferred because of their malignant
potential. A high mitotic rate, tumoral necrosis. and nuclear pleomorphism
appear to be adverse prognostic factors.