Abstract

Paget's disease of the nipple is a distinct pathologic entity associated with intraductal and/or invasive carcinoma of the breast. Paget's change in the nipple is observed in 2.5% of breast malignancies. It is associated with progressive clinical changes corresponding to progressive microscopic infiltration of the nipple epidermis by “Paget” cells. The underlying intraductal carcinoma typically involves the lacteriferous sinuses and is often multifocal. Theories regarding the histogenesis of Paget's disease include: 1) ductal carcinoma cells migrating into the nipple epidermis; 2) melanocytes; 3) squamous cells of the nipple; and 4) keratinocytes which differentiate into glandular malignant cells. The histologic characteristics of Paget's disease include nests of large pale cells with granular cytoplasm and hyperchromatic nuclei with prominent nucleoli, in the epidermis of the nipple. The tumors may show continuity with underlying carcinoma of the lacteriferous ducts. Paget cells are characterized ultrastructurally by an electron lucent cytoplasm with numerous polyribosomes, extensive rough endoplasmic reticulum and golgi, numerous large mitochondria, lysosome-like inclusions, lipid bodies, and glycogen particles.

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