Paget's disease of the breast

Paget's disease of the breast is rare (1% of breast cancers), more often affects postmenopausal women and frequently shows a considerable diagnostic delay. Between 1973 and 1994, 38 female patients with Paget's disease, mean age 60 years, were observed at Surgery and Oncology Institute of Cagliari University. In 14 patients diagnostic delay was more than 12 months. Twenty-eight patients were operated of radical mastectomy, 4 of total mastectomy and 4 of conservative surgery. In 22 cases invasive ductal and in 7 intraductal carcinoma was associated to Paget's disease. Axillary node metastases were present in 12 patients. Four local recurrences occurred (2 after radical mastectomy, 1 after total mastectomy and 1 after conservative surgery). Actuarial overall survival was 67.47% at 5 years and 57.83% at 10 years. In patients with Paget's disease confined to the nipple and in patients with associated intraductal carcinoma survival was 100% at 5 and 10 years, in those with associated invasive ductal carcinoma 56.64% at 5 years and 45.31% at 10 years. In patients without axillary node metastases survival was 91.67% at 5 and 10 years, in those with lymph node metastases 31.17% at 5 years and 15.58% at 10 years. Authors conclude that Paget's disease, if confined to the nipple, has a good prognosis, whilst, if associated with an underlying breast carcinoma, its stage is the main predictor of prognosis; early diagnosis could considerably improve survival.