PAGETS-DISEASE OF THE MALE BREAST

A histopathologically proven case of Paget's disease of the breast in a 47-year-old male induced a review of the 32 cases in the world's lit erature. Paget's disease usually presents in the 5th and 6th decades o f life without any obvious racial predilections. The mast frequent pre senting signs include ulceration, eczema, nipple discharge, bleeding, and crust formation. At the time of presentation, 50 per cent of the p atients have a palpable breast mass, positive lymph nodes, or both. Wh en entertaining the diagnosis of Paget's disease of the breast using m orphologic characteristics of the cells, the diagnosis of malignant me lanoma and Bowen's disease (intraepithelial squamous cell carcinoma) m ust be included in the differential diagnosis. Certain differences wil l be seen between these three entities based on specific cell staining and the uptake of certain tumor markers. There is no evidence that th is disease behaves differently in males versus females, but the 5-year survival for males is worse: 20 to 30 per cent in males versus 30 to 50 per cent in females. The treatment of this disease must focus on th e pathology of the underlying tumor. Current treatment involves modifi ed radical mastectomy or radical mastectomy for Stage I and II tumors. Adjuvant chemotherapy, radiation, and tamoxifen are also used dependi ng on the nodal and receptor status of the tumor.