Paget’s Disease of the Female Breast: Clinical Findings and Management in ۵۳ Cases at a Single Institution

Background: Mammary Paget’s disease is an uncommon form of primary breast cancer. The aim of this study is to assess our institution’s experience in its management.Methods:We retrospectively reviewed the medical records of ۵۳ female patients with histologically confirmed Paget’s disease, treated at the Salah Azaïz Institute between ۲۰۰۱ and ۲۰۱۰.Results: There were palpable masses in ۷۱.۷% of cases, of which ۹۰% revealed invasive carcinoma. Approximately ۴۸% of underlying malignancies were multifocal/multicentric. Overall, invasive carcinoma accounted for ۶۹.۸% with a median tumor size of ۴۰ mm, high grade in ۶۲.۲%, and negative hormone receptor in ۴۷.۶% of cases. There was only one case with direct dermis invasion among those with no underlying invasive carcinoma. After a median follow-up of ۴۵ months, ۴۹% of patients presented with relapse/progression and ۴۷.۱% died from their disease. Median overall survival was ۶۷ months, whereas disease-free survival was ۶۵ months. Tumor and node advanced clinical stages correlated with poor survival, as well as the presence of invasive carcinoma with additional negative impacts of large tumor size and lymph node involvement. Tumor stage was the only independent indicator on multivariate analysis. Conclusion: The general trend for decreased incidence of Paget’s disease is noted parallel to earlier breast cancer diagnosis. Paget’s disease is at high risk of multifocal/multicentric underlying tumors. The presence of a palpable mass is almost pathognomonic of invasive neoplasm. The major challenge concerns aggressiveness of surgical procedures with breast and axilla preservation perspectives. Prognosis is mainly determined by that of an eventual underlying breast tumor.