A 76-year-old male with no past medical history of interest was presented with a thirty-year history of pruritus and unilateral enlargement of the left nipple.

Cutaneous examination showed a one-centimeter enlargement of the left nipple (Fig. 1A). Dermoscopy revealed milky-red areas with central ulceration and polymorphous vessels (Fig. 1B). No other breast lesions were detected and no axillary lymphadenopathy was palpated.

Figure 1

(0.13MB).

A punch biopsy was performed. Hematoxylin-eosin staining showed a non-encapsulated proliferation of small irregular clumps of basaloid cells with peripheral palisading and a highly infiltrative growth pattern (Fig. 2A). In the immunohistochemical study, the cells stained positive for Ber-EP4 (Fig. 2B).

Figure 2.

(A) Hematoxylin-eosin, 20×. (B) Staining with Ber-EP4, 11×.

(0.19MB).

The mammography and breast magnetic resonance imaging did not reveal significant alterations.

What is your diagnosis?

Basal Cell Carcinoma (BCC) of the nipple-areola complex (NAC).

The NAC was excised, confirming the presence of infiltrating BCC. After 10 months of clinical follow-up, no recurrences have been detected.

BCC of the NAC is extremely rare and it seems to be more prevalent in men. Based on our literature review, 59 cases have been described. However, its incidence may be underestimated.1–5 Clinically, it tends to present as a scaly or ulcerated plaque or nodule.2 Previous studies have suggested that ultraviolet irradiation might be the main causal factor.3

BCC of the NAC has been suggested to have a higher metastatic potential than other locations, showing a more aggressive course due to the presence of ducts and lymphatic plexus in the NAC.2

The differential diagnosis of the BCC of the NAC would also be with eczema, Paget's disease, Bowen's disease, erosive adenomatosis, melanoma, leiomyoma, adenoma, fibroma, and papilloma of the nipple.4,6 Despite histopathology is the gold standard for making the diagnosis, clinical suspect is essential.

Treatment of choice is surgical excision, the most commonly used options are simple excision, wide local excision and Mohs micrographic surgery, nevertheless simple mastectomy has been also reported. Tumor recurrence is uncommon after successful treatment.2

The authors declare they have no conflict of interest.