Pulmonary mucoepidermoid carcinoma (PMEC) is the most common malignant salivary gland tumor in the lungs and accounts for 0.1-0.2% of all lung malignancies in adults. It has no specific epidemiological or clinical characteristics. Correct diagnosis requires the combined examinations of images, laboratories, pathology, and immunohistochemistry (IHC) as well as molecular characteristics. PMEC tumors are characterized by squamous, intermediate, and mucus-secreting cells. Currently, histological appearance, mitotic frequency, cellular atypia, and necrocytosis allow the classification of PMEC into low grade or high grade. Molecular changes are crucial to pathological diagnosis. The driver of PMEC seems to be the fusion protein MECT1-MAML2 that is generated from a genetic mutation in t (11; 19) (q21; p13), while other gene mutations are also reported. However, no treatment of PMEC exists so far; surgical excision is still the primary treatment, while the efficacies of chemotherapy or radiotherapy are undefined. Tyrosine kinase inhibitor (TKI) therapy and immunotherapy showed to have significant therapeutic effects but require more investigation and better understanding. This review focuses on the clinical characteristics, imaging and pathologic features, immunohistochemical examination, mutation analysis, differential diagnosis, prognosis, and treatment of PMEC.
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