[A case of mammary Paget's disease with early systemic recurrence after operation]

A 66-year-old woman visited our hospital because of redness and erosion of her right breast, and was diagnosed with mammary Paget's disease by histological examination of erosive skin. The patient had a simple mastectomy. The pathological diagnosis was mammary Paget's disease (8x7.5 cm) showing intraductal spread. The resected skin margin was negative. Partially dermal microinvasion and lymphatic involvement were found. Immunohistochemical analysis of this tumor showed ER negative, PgR negative, and HER2 positive (3+). Eighteen months after the operation, she presented with redness of the chest wall with edema of the right arm. Incisional biopsy on the chest mass revealed a local recurrence of mammary Paget's disease. In addition, she had carcinomatous lymphangiosis in the bilateral lung, nodal metastases in the ipsilateral axillary and mediastinal space, and contralateral breast metastases on CT. She was treated with twelve courses of weekly paclitaxel in combination with trastuzumab. A complete response was obtained eleven months after initiation of chemotherapy. A rare case of early systemic recurrence of mammary Paget's disease after curative operation is reported with a review of the literature.