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What is Paget's Disease of the Nipple?

Paget's disease of the nipple (Sir James Paget, 1814 - 1899, English surgeon) is an eczematous lesion of the nipple, occasionally involving the areola and adjacent skin of the breast, almost always associated with underlying carcinoma of the breast.

Paget's disease occurs in 1 - 2% of female patients with breast cancer and may be the only abnormality indicating breast cancer. The involved area of the nipple is typically scaling and erythematous, and occasionally ulcerated with crusting. The microscopic appearance consists of an epidermal infiltrate of large cells with abundant cytoplasm and prominent nuclei with large nucleoli.

The condition is best considered as involvement of the nipple by ductal carcinoma in situ of high nuclear grade. Ductal carcinoma in situ, most often with a comedo necrosis growth pattern, is commonly seen in a subareolar duct or deeper in the breast and invasive ductal carcinoma is present in the breast in approximately half of the cases (see breast cancer histological classification). Invasive carcinoma rarely originates directly from the epidermal Paget's disease rarely originates. The neoplastic cells are most numerous in the basal portion of the nipple epidermis; therefore, a full thickness biopsy of the skin, rather than a shave biopsy, should be used for diagnosis.

The findings on mammography and ultrasonography mainly depend on the extent of underlying breast cancer. As many as 50% of patients with Paget's disease may be negative radiographically. In other cases a thickening of the nipple areolar complex may be the only finding (Fig.1). In still other cases there may be an obvious malignant tumour combined with calcifications of intraductal carcinoma. The cancer can be located anywhere in the breast on the mammogram.

Imaging Examples

Paget's disease of the nipple, Fig.1

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Last Updated: Feb 3, 2014

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