Paget's Disease of the Female Breast: Clinical Findings and Management in 53 Cases at a Single Institution

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.
We retrospectively reviewed the medical records of 53 female patients with histologically confirmed Paget’s disease, treated at the Salah Azaïz Institute between 2001 and 2010.
There were palpable masses in 71.7% of cases, of which 90% revealed invasive carcinoma. Approximately 48% of underlying malignancies were multifocal/multicentric. Overall, invasive carcinoma accounted for 69.8% with a median tumor size of 40 mm, high grade in 62.2%, and negative hormone receptor in 47.6% of cases. There was only one case with direct dermis invasion among those with no underlying invasive carcinoma. After a median follow-up of 45 months, 49% of patients presented with relapse/progression and 47.1% died from their disease. Median overall survival was 67 months, whereas disease-free survival was 65 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.
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.