Paget's disease of the male breast

Histologically proved Paget's disease of the breast with negative hormonal receptor assay in a 73-year-old man induced a review of the 27 valuable cases of the literature. Our patient is disease-free, eight years after treatment by modified radical mastectomy and adjuvant irradiation. Peak incidence of the disease is between 50 and 70 years of age. The most frequent presenting signs reported are ulcerations and eczematous lesions of the nipple (71.4%) with discharge and bleeding in 39.3% of the cases. Other findings are a palpable mass (42.8%) and involved axillary lymph nodes (53.5%). The treatment of choice is shown to be radical or modified radical mastectomy with adjuvant radiotherapy. To date, no proof exists of the value of hormone receptors assays and routine adjuvant hormone therapy is not practiced.