Pathological and radiological assessments of Paget’s disease

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Background: Pure Paget’s disease (PD) of the nipple is a non-invasive breast carcinoma. However, choosing the optimal surgical procedure is difficult due to tumour location and spread into the breast. Thus, in the current study, we retrospectively investigated PD cases in our institution to clarify the details of this disease. We also considered optimal surgical procedures. Methods: Among 4,341 breast cancer cases in the last 11 years at our institution, we retrospectively investigated 17 PD cases with no mass lesion in the breast. Results: Total mastectomy was performed in 15 patients. One patient initially requested central lumpectomy (CL) but the procedure was switched to mastectomy due to a positive surgical margin, demonstrated intraoperatively. On final pathological diagnosis, two patients had PD alone (12%). PD with ductal carcinoma in situ (PD + DCIS) and invasive ductal carcinoma (PD + IDC) were observed in 10 (59%) and 5 (29%) patients, respectively. As to oestrogen receptor (ER) and epidermal growth factor receptor 2 (HER2) statuses, ER-positive/HER2-negative, ER-positive/HER2-positive, ER-negative/HER2-positive and ER-negative/HER2-negative tumours were observed in 6%, 0%, 76% and 18%, respectively, of patients. Disease spread into the breast was pre-operatively suspected in 8 cases, based on micro-calcifications on MMG, and in 8 cases with segmental enhancement on MRI. Sensitivity and specificity of intra-breast disease detection were 62% and 100%, respectively, in 14 patients who had both MMG and MRI findings of PD. Conclusions: Our data confirm that breast conserving surgery is difficult in patients with PD. The surgical procedure might have to be determined in consideration of a balance between curability and cosmesis.