Myoepithelial tumors of the soft tissues represent a rare group of
neoplasms that vary in their clinical behavior, pathologic features and genetics. They are histopathologically typified by a myoepithelial immunohistochemical phenotype, of expression of one or more epithelial markers,
S100 protein and smooth muscle actin. Because of their rarity and occurrence over a wide age range and at a variety of anatomic sites, they can be difficult to diagnose due to the lack of familiarity by physicians, which is compounded by their spectrum of histologic features and morphologic overlap with several other
neoplasms. Recent genetic insights have aided classification, and it is increasingly understood that soft tissue myoepithelial
neoplasms can be stratified into two distinct morphologic and genetic subgroups. We describe a case of a 44-year-old man who was diagnosed with a primary myoepithelial
neoplasm of the paracecal mesentery, which showed aggressive local recurrence after four years. The
tumor was composed of cords of ovoid cells within chondromyxoid stroma, and displayed a characteristic pancytokeratin,
S100 protein and smooth muscle actin-positive myoepithelial immunoprofile. Primary
myoepithelioma has not been previously described at this site, and this case highlights this varied family of
tumors, emphasizes the need to consider
myoepithelial tumor in the differential diagnoses of
carcinoma variants occurring in the bowel or mesentery, and also adds to the number of reported myoepithelial
neoplasms showing markedly aggressive behavior.