primary cutaneous adenoid cystic carcinoma of the lower limb rare tumor in a rare location a case report and brief review of literature

Abstract Adenoid Cystic Carcinoma (ACC) is a rare entity with a predilection site at the salivary glands. Cutaneous localization is rather exceptional. Despite its rarity, it has a very distinctive histology consisting of cribriform epithelial structures which form pseudoglandular spaces giving a characteristic ‘swiss cheese’ appearance. Those pseudoglandular areas contain basement membrane-like material. The tumor has also the tendency for perineural invasion, that is responsible for its aggressiveness. We describe a case of a 60-year old male presenting with a pretibial asymptomatic pigmented dermal nodule on the lower leg. Histological examination was consistent with the characteristics of an ACC. After thorough examination we excluded localization elsewhere in the body and confirmed the primary cutaneous origin. In this case report we also briefly refer to the histogenesis, the morphological and molecular profile, the differential diagnosis, the therapeutic options and the management of the Primary Cutaneous Adenoid Cystic Carcinomas (PCACCs). Keywords Primary cutaneous adenoid cystic carcinoma; Skin cancer; Tibia