Paget's disease of the breast.

Twenty patients with histologically proven Paget's disease of the breast are reviewed. They represent an incidence of 1.06% of all breast carcinomas seen over a 12-year period in the Bloemfontein academic hospitals. All cases had ductal carcinoma. Clinically, 20% presented with a mass only, 20% with nipple disease only and 60% with both lesions. More patients with a mass compared with patients with only nipple disease had positive axillary lymph nodes--68.7% v. 25%. Cumulative 5-year survival rates showed a 100% survival rate for patients with only nipple disease, and an 83% survival rate for stage I and II disease. All the patients with stage III and IV disease died within the 4-year follow-up period. Nipple (skin) involvement per se did not worsen the prognosis of patients presenting with both a mass and nipple disease. The main predictors of prognosis were tumour size and lymph node involvement. It is suggested that patients presenting with nipple involvement only and/or small T1 lesions close to the nipple could be treated with wide local excision and axillary dissection in discontinuity followed by radiotherapy to the rest of the breast.