Dedifferentiated Epithelial-Myoepithelial Carcinoma: Analysis of a Rare Entity Based on a Case Report and Literature Review

作者：Baker Aaron R; Ohanessian Sara E; Adil Eelam; Crist Henry S; Goldenberg David; Mani Haresh^*

来源：International Journal of Surgical Pathology, 2013, 21(5): 514-519.

DOI：10.1177/1066896912468153

• head and neck cancer
• salivary gland
• dedifferentiated
• epithelial-myoepithelial carcinoma
• DEMC
• high-grade

Dedifferentiated epithelial-myoepithelial carcinoma (DEMC) is very rare salivary gland neoplasm with only anecdotal reports. We present an analysis of DEMC, based on a case and review of literature. Our patient, an 85-year-old woman, presented with a submandibular mass of 5 years duration that was increasing in size over a 5-week period. Histologically, there were areas of typical epithelial-myoepithelial carcinoma, with dedifferentiation of both components, manifesting morphologically as salivary duct carcinoma and areas of myoepithelial carcinoma. A review of literature revealed 21 previously reported cases of DEMC. DEMC occurs at an average age of 72 years, most often in the parotid gland (72%) followed by submandibular gland (17%). Dedifferentiation more often involves the epithelial component (13/15 cases) than the myoepithelial component (5/15 cases). Although typical epithelial-myoepithelial carcinomas are fairly indolent (average disease-free survival of 11.34 years), dedifferentiation confers a poor prognosis (survival reported from 1 to 72 months).

• 出版日期2013-10