Reappraisal and literature review of primary cutaneous cribriform apocrine carcinoma

Primary cutaneous cribriform apocrine carcinoma (PCCAC) is an exceedingly rare sweat gland carcinoma. Clinically, it most often presents as a singular, asymptomatic nodule or cyst on the extremities of middle-aged patients and follows an indolent course. Both visceral and cutaneous cribriform tumors exist in nature. While a cribriform pattern is well recognized in tumors of visceral organs, there is a paucity in the literature on the defining characteristics for primary cutaneous tumors. Consensus regarding diagnostic criteria, etiology, and management protocols has yet to be achieved. We conducted an extensive literature review using pre-determined search criteria, resulting in 12 identified case reports and series on PCCAC that were subsequently analyzed. All data were compiled to provide a comprehensive update on the existing information regarding clinical presentation, histopathology, and management of reported PCCACs, as well as differential diagnosis, controversial issues, and recommendations for future considerations.