Pulmonary epithelial-myoepithelial carcinoma (P-EMC) is an exceptionally rare subtype of salivary gland lung tumor originating from tracheobronchial glands. P-EMC is a biphasic tumor consisting of an inner layer of epithelial cells and an outer layer of spindle-shaped, clear-cell-like myoepithelial cells. Bronchial obstruction symptom is the main clinical characteristic for P-EMC. Because its clinical and imaging characteristics are highly similar to other types of non-small-cell lung cancer (NSCLC), it is easy to cause missed diagnosis and misdiagnosis. The diagnosis is mainly based on the pathology and immunohistochemistry with an inner layer of epithelial cells immunoreactive for cytokeratin and an outside layer of myoepithelial cells immunoreactive for S100 protein (S-100) and smooth muscle actin (SMA). Therefore, positive for cytokeratin, S-100 and SMA can assist in the diagnosis. Although in general, P-EMC is a low-grade malignant neoplasm, it may occasionally recur and metastasize. The optimal method for P-EMC treatment has not been established, and surgical resection is still the main clinical method. Radiotherapy and chemotherapy have been shown not sensitive for P-EMC treatment, whereas targeted therapy and immunotherapy have not evaluated in clinical practice. This review focuses on the pathological characteristics, molecular characteristics, diagnosis, treatment, and prognosis of P-EMC.
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