Basal cell carcinoma (BCC) is the most common malignancy among white persons, but its occurrence in the nipple-areola complex is exceedingly rare, having first been described in the literature in 18931and since reported 16 times. The overall incidence of BCC in North America is approximately 10% per year, with an overall lifetime risk of 30%.2Exposure to UV light is felt to be the main causative factor in the development of BCC, along with family history of skin cancer, low intake of certain vitamins, and exposure to ionizing radiation and various chemicals such as arsenic.2Approximately 80% of BCCs are found on the head or neck, and the most common morphology is a pearly ulcerative lesion with a raised, indurated border (“rodent ulcer”). Basal cell carcinoma tends to be an indolent, slow-growing tumor that may spread to local tissue but has low metastatic rates varying from 0.0028% to 0.55%.2Curettage with cautery and surgical excision have both been shown to yield 5-year cure rates of 95% or better,3but excision offers better cosmetic results as well as the opportunity for histological examination of tissue to ensure disease-free margins. Moh micrographic surgery has also been described with 5-year cure rates as high as 99% for primary disease.4
There have been a total of 17 cases of nipple-areola complex BCC described in the literature,5- 7with 9 cases in men and 8 in women. It was first described in a 60-year-old man with a rodent ulcer of the nipple in 1893.1In a 1956 review of 10 000 cases of breast malignancy from the Mayo Clinic, 29 were found to have originated from the nipple; of those, only 2 were BCC (1 man and 1 woman).5Treatment has consisted of simple local excision in all but 3 cases. In 1965, Wyatt reported performing a simple mastectomy in a 71-year-old man for a nipple BCC.8In 1986, Shertz and Balogh8described a 54-year-old man who after being diagnosed with a BCC of the nipple and undergoing simple excision developed axillary node metastases requiring a mastectomy and axillary node dissection. In 2000, Sánchez-Carpintero et al7reported using a Moh micrographic procedure to excise an areolar BCC in a 65-year-old woman. Sauven and Roberts used local radiation therapy in 1983 in addition to local excision.8Although there was 1 case of axillary nodal metastases of the 18 reports (5.5%),8BCC has been documented to have a very low metastatic potential overall.2This seemingly higher prevalence may be owing to an underreporting of the disease, but it does raise the question as to whether any studies of the axillary nodes should be undertaken when confronted with this disease.
In previous case reports, BCC of the nipple-areola complex has been described as a disease primarily of men. This was attributed to the greater likelihood of sun and UV light exposure of the male breast.8However, when including this case report, the literature contains 9 accounts of male cases and 9 accounts of female cases, thus making sex seem less of a contributing factor.
This patient underwent an excisional biopsy given the high suspicion for carcinoma. Pathological findings revealed in situ and invasive carcinoma arising from skin adnexal structures consistent with BCC. The lactiferous ducts showed fibrocystic changes with focal ductal hyperplasia but no atypia, and the surgical resection margins were clear. The patient had an ultrasonographic examination 8 weeks postoperatively, which showed resolution of the edematous changes. She subsequently moved back to India and was lost to follow-up despite multiple attempts to contact her.
Due to the overwhelmingly positive response to the Image of the Month, the Archives of Surgery has temporarily discontinued accepting submissions for this feature. It is anticipated that requests for submissions will resume in mid 2008. Thank you.
Correspondence:Christine B. Teal, MD, Breast Cancer Center, 2150 Pennsylvania Ave NW, Washington, DC 20037 (email@example.com).
Accepted for Publication:June 29, 2006.
Author Contributions:Study concept and design: Roland and Teal. Acquisition of data: Schwartz and Teal. Analysis and interpretation of data: Schwartz. Drafting of the manuscript: Roland and Schwartz. Critical revision of the manuscript for important intellectual content: Teal. Study supervision: Teal.
Financial Disclosure:None reported.
Image of the Month—Diagnosis. Arch Surg. 2007;142(7):688. doi:10.1001/archsurg.142.7.688