Primary Squamous Cell Carcinoma of the Nipple

Abstract

Squamous cell carcinoma (SCC) is the second most common skin cancer after basal cell carcinoma. Ultraviolet exposure and immunosuppression are major risk factors for SCC of the skin, with chronic sun exposure being the strongest environmental risk factor. As such, skin cancer around the nipple-areola complex (NAC) is not unusual; however, primary SCC, specifically, is indeed incredibly rare, with only four cases having been identified in the English literature. Due to the rarity of SCCs in this region, it is likely that the diagnosis may be delayed for lack of suspicion, as often they will resemble other more common cancers or conditions. This chapter outlines a systematic approach in dealing with such a rare diagnosis through presentation of a rare case report of a primary SCC of the NAC which clinically resembled Paget’s disease.