Basal Cell Carcinoma of the Nipple-Areola Complex: A Comprehensive Review of the World Literature

BACKGROUND: Basal cell carcinomas (BCCs) usually occur in sun-exposed areas. However, they may also occur—albeit infrequently—in unusual locations, such as the nipple-areola complex. METHODS: Using the PubMed database, an extensive literature search was performed for the following keywords: areola, basal cell carcinoma, and nipple. Papers and references cited in those papers were reviewed to accumulate reports of patients with BCC of the areola and nipple. RESULTS: BCC of the areola and nipple has been described in 55 individuals: 35 males and 20 females. The onset age ranged from 35 to 86 years. The median onset age in males was 61 years, whereas the median onset age in females was 66 years. BCC of the NAC predominantly occurred in Caucasians (75.7%). BCC of the nipple-areola complex (NAC) was observed on the left (54.9%) more frequently than the right (45.1%). Clinical presentation was variable and commonly included scaly or ulcerated plaques and nodules. This tumor was typically associated with the nodular (42.9%) or superficial (30.9%) subtype of BCC. The most common treatment was excision. There were three reported patients who had metastatic disease to their lymph nodes; one of the patients died from his tumor. CONCLUSION: The nipple and areola are uncommon sites of BCC. BCC of the nipple-areola complex is less frequently observed in females (36.4%), as this is more commonly a photo-protected site. BCC of the NAC has been considered to behave more aggressively than BCCs at other anatomical sites; however, the BCCs are frequently associated with a non-aggressive histologic subtype. Treatment usually involves complete excision of the BCC. Tumor recurrence was uncommon following successful treatment of the primary neoplasm.