Epithelial-myoepithelial carcinoma affects children and adults and presents at a mean age of approximately 60 years. Women are involved slightly more than men. The majority of cases involve major salivary glands, presenting as single or multiple masses. Most cases of epithelial-myoepithelial carcinoma behave as low- to intermediate-grade malignancies; however, a subset of cases with high-grade transformation (necrosis, elevated mitotic activity, and cytomorphologic atypia) are predisposed to recurrence, lymph node involvement, and distant metastasis. Epithelial-myoepithelial carcinoma may be well-circumscribed or infiltrative, comprised of multiple cell types (ductal and myoepithelial), and exhibit a variety of architectural patterns (cystic and solid) (Figs. 13.1, 13.2, 13.3, 13.4, 13.5, 13.6, 13.7, 13.8, 13.9, 13.10, 13.11, 13.12, 13.13, 13.14, 13.15, 13.16, 13.17, 13.18, 13.19, 13.20, 13.21, 13.22, 13.23, 13.24, 13.25, 13.26, 13.27, 13.28, 13.29, 13.30, 13.31, 13.32, 13.33, 13.34, 13.35 and 13.36).
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