Understanding Paget's Disease of the Nipple

Paget’s disease of the nipple is an eczema-like lesion which was first described in 1874 by Sir James Paget (1). It is characterized by the presence of large malignant cells with clear cytoplasm and prominent nucleolus within the epidermis (2).

Paget’s disease is present in 2% of breast carcinomas, and in almost all cases, results from an intraepithelial spread of malignant cells from an underlying intraductal carcinoma of the breast, which may or may not be associated with an invasive component (2,3,4,5). Paget’s disease is not associated with in situ lobular carcinoma (5).

Interestingly, if multiple sections from the nipple are examined, a direct connection between the epidermal lesion and the underlying carcinoma will be noted in the majority of cases (2).

The histologic differential diagnosis includes malignant melanoma and Bowen’s disease, which can be distinguished by immunohistochemical studies (2,3,5).

Paget’s disease of nipple is not an independent pathologic process but an epidermal manifestation of ductal carcinoma of the breast and its prognosis is usually determined by the underlying neoplasm (3,4).

References

1. Paget, J. On disease of mammary areola preceding cancer of mammary gland. St Barth Hosp Rep 1874;10:87-89.

2. Rosai, J (ed). Ackerman’s surgical pathology 8th ed. Mosby, St. Louis, Missouri, 1996.

3. Fletcher, CDM (ed). Diagnostic histopathology of tumors 1st ed. Churchill Livingstone, Edinburg, United Kingdom, 1995.

4. Paone, JF, Baker, RR. Pathogenesis and treatment of Paget’s disease of the breast. Cancer 1981;48:825-29.

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