Microcystic Adnexal Carcinoma (MAC) and Eccrine Cutaneous Mixed Tumor (ECMT): 2 Cases of Rare HPV-independent Vulvar Cutaneous Adnexal Tumors

Microcystic adnexal carcinoma (MAC) and eccrine cutaneous mixed tumor (ECMT) are both cutaneous adnexal tumors that may occur in the vulvar region, but are very rare at this site. Consequently, they may not enter the differential diagnosis of vulvar lesions for gynecologic pathologists in a subspecialized practice setting. Here we report a case of MAC and a case of ECMT recently diagnosed at our institution and underscore key histologic and immunophenotypic features of each lesion that can assist in their correct identification. Both MAC and ECMT have a tubular to corded pattern of lesional cells within a desmoplastic to chondromyxoid stroma. However, MAC shows true eccrine sweat duct differentiation, characterized by 2 SOX10 negative cell layers, including an outer p63+/p40+/EMA- basal cell layer and an inner p63-/p40-/EMA+ ductal layer. The main differential diagnostic considerations for vulvar MAC include other cutaneous adnexal tumors with true eccrine sweat duct differentiation, namely syringoma and squamoid eccrine ductal carcinoma (SEDC). Conversely, ECMT is characterized by a single SOX10+ cell population without immunoreactivity for p63 or p40. The main differential diagnostic considerations for ECMT include the apocrine variant of cutaneous mixed tumor (ACMT)-the cutaneous analog of salivary gland pleomorphic adenoma-and other SOX10+ salivary gland-type neoplasms. Unlike the recently described vulvar analog of HPV-associated multiphenotypic sinonasal carcinoma, neither MAC nor ECMT are HPV-associated and both are therefore p16 negative. In summary, we report one case each of vulvar MAC and ECMT and discuss the key histologic features and ancillary testing results that can help to differentiate these lesions from their morphologic mimics.