BACKGROUND Basal cell carcinoma (BCC) of the nipple–areola complex is uncommon. It has been suggested that BCCs in this region behave more aggressively, with a higher potential for distant spread, than in other anatomical sites.
OBJECTIVE To address questions about etiology, behavior, optimal treatment, and prognosis of this entity.
METHODS AND MATERIALS A literature search identifying all cases of BCC of the nipple and nipple–areola complex in the English literature from 1893 to 2008.
RESULTS Thirty-four cases of BCC of the nipple, areola, or both were identified, mostly affecting middle-aged men. The majority of patients were treated with tissue-sparing surgery. There was a metastatic rate of 9.1%, and one patient died from the disease (3.0%).
CONCLUSIONS The optimal treatment of this condition should be local excision, but patients with this condition should be followed up for primary site recurrence and axillary metastasis, because there is greater incidence than with BCC at other anatomical sites. Furthermore, proven axillary metastasis should be surgically treated.