paget’s disease of the female breast: clinical findings and management in 53 cases at a single institution

Background: mammary paget’s disease is an uncommon form of primary breastcancer. the aim of this study is to assess our institution’s experience in its management.methods:we retrospectively reviewed the medical records of 53 female patientswith histologically confirmed paget’s disease, treated at the salah azaïz institutebetween 2001 and 2010.results: there were palpable masses in 71.7% of cases, of which 90% revealedinvasive carcinoma. approximately 48% of underlying malignancies weremultifocal/multicentric. overall, invasive carcinoma accounted for 69.8% with amedian tumor size of 40 mm, high grade in 62.2%, and negative hormone receptor in47.6% of cases. there was only one case with direct dermis invasion among those withno underlying invasive carcinoma. after a median follow-up of 45 months, 49% ofpatients presented with relapse/progression and 47.1% died from their disease. medianoverall survival was 67 months, whereas disease-free survival was 65 months. tumorand node advanced clinical stages correlated with poor survival, as well as the presenceof invasive carcinoma with additional negative impacts of large tumor size and lymphnode involvement. tumor stage was the only independent indicator on multivariateanalysis.conclusion: the general trend for decreased incidence of paget’s disease is notedparallel to earlier breast cancer diagnosis. paget’s disease is at high risk ofmultifocal/multicentric underlying tumors. the presence of a palpable mass is almostpathognomonic of invasive neoplasm. the major challenge concerns aggressivenessof surgical procedures with breast and axilla preservation perspectives. prognosis ismainly determined by that of an eventual underlying breast tumor.