Paget's disease of the nipple-areola complex

The histogenesis of Paget's disease has been hotly debated, and only recently has epidermotropic theory become widely accepted. With the evolution of our understanding of breast cancer, it became apparent that the prognosis of Paget's disease was more a reflection of that of the underlying carcinoma, be it intraductal or infiltrating. The standard treatment of Pager's disease remains mastectomy with or without axillary dissection. In this era of breast-conserving surgery, however, there is much evidence to suggest that conservative treatment of Paget's disease of the breast is possible. A breast-conserving algorithm for the treatment of Paget's disease of the breast is proposed. Further refinements or modifications to the algorithm should be made as data from ongoing trials redefine our understanding of breast pathology and treatment.