Baker Aaron R, Ohanessian Sara E, Adil Eelam, Crist Henry S, Goldenberg David, Mani Haresh
1Penn State College of Medicine & Penn State Milton S. Hershey Medical Center, Hershey, PA, USA.
Int J Surg Pathol. 2013 Oct;21(5):514-9. doi: 10.1177/1066896912468153. Epub 2012 Dec 5.
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).
去分化上皮-肌上皮癌（DEMC）是一种非常罕见的涎腺肿瘤，仅有零星报道。我们基于一例病例并结合文献复习对DEMC进行分析。我们的患者是一位85岁女性，下颌下肿物已有5年，在5周内逐渐增大。组织学上，存在典型上皮-肌上皮癌区域，两种成分均有去分化，形态学上表现为涎腺导管癌和肌上皮癌区域。文献复习发现之前报道了21例DEMC。DEMC发病的平均年龄为72岁，最常发生于腮腺（72%），其次是下颌下腺（17%）。去分化更常累及上皮成分（13/15例）而非肌上皮成分（5/15例）。虽然典型的上皮-肌上皮癌生长相对缓慢（平均无病生存期为11.34年），但去分化会导致预后不良（报道的生存期为1至72个月）。
