Paget Disease of the Breast

Abstract

Paget disease of the breast is an in situ malignancy located within the epidermal layer of the nipple-areolar complex. It accounts for 0.5% of all breast cancers in women and 1 to 3% of new breast cancers diagnosed annually in the United States. Patients most likely present with erosion at the tip or base of the nipple. These symptoms are often confused with the symptoms of eczema. Persistent itching can occur. As the disease progresses, ulceration of the nipple expands outward over the areola. Full-thickness biopsy of the nipple or involved skin is needed to confirm diagnosis. Further workup includes mammography and, frequently, breast magnetic resonance imaging to rule out an associated breast parenchymal malignancy. Surgical excision requires removal of the nipple and involved areolar complex as well as any underlying malignancy. Mastectomy is also an option and may be required if additional multicentric disease is present. This review contains 10 figures, 6 tables, and 52 references. Key words: breast conservation, breast magnetic resonance imaging, eczema, intraepithelial adenocarcinoma, nipple abnormalities, nipple biopsy, occult malignancy, Paget disease