Paget's disease of the breast

Paget's disease of the breast is a rare disorder of the nipple-areola compl ex that is often associated with an underlying in situ or invasive carcinom a. A change in sensation of the nipple-areola, such as itching and burning, is a common presenting symptom. Objectively eczematoid changes of-the nipp le-areola complex are common. The later stages of Paget's disease of the br east are characterized by ulceration and destruction of the nipple-areola c omplex. Eczematoid changes of the nipple-areola complex and persisting sore ness or itching, without obvious reason, is a suspicious symptom for Paget' s disease of the breast and calls for thorough evaluation, including mammog raphy. Exfoliative cytology with demonstration of Paget's cells may be usef ul, but a negative finding does not exclude Paget's disease of the breast. Surgical biopsy is the diagnostic standard and therefore the diagnosis shou ld always be confirmed by open (surgical) biopsy. The histogenesis of Pager 's disease of-the breast continues to be debated. The epidermotropic theory holds that Paget's cells are ductal carcinoma cells that have migrated fro m the underlying breast parenchyma to the nipple epidermis. According to th e in situ transformation theory. the Paget's cells arise as malignant cells in the nipple epidermis independent from any other pathologic process with in the breast parenchyma. This theory has been proposed to explain those ca ses in which there is no underlying mammary carcinoma or when there is a ca rcinoma remote from the nipple-areola complex. Each of these theories is pl ausible; however, treatment approaches differ markedly depending on the the ory of histogenesis. Mastectomy has been considered the standard of care in the management of patients with Paget's disease of the breast. Nowadays, h owever; some patients with Paget's disease of the breast are candidates for breast-conserving therapy. Patients must be selected carefully on an indiv idual basis. Until there is a better understanding of the relationship of P aget's disease of the breast to the underlying cancer the surgeon should un derstand the natural history and behaviour of this lesion and be aware of b oth the risks of under- and over-treating patients with Paget's disease of the breast. (C) 2001 Harcourt Publishers Ltd.