Paget's disease of the nipple

A total of 48 women with Paget's disease of the nipple (nipple eczema containing Paget cells but without a palpable lump) presented to one surgeon over a 13-year period. Temporary healing of the nipple eczema occurred in six patients. In all, 21 of 34 patients with in situ (DCIS) or invasive ductal carcinoma had mammographic abnormalities. Treatment was by simple mastectomy (37 cases), cone excision of the nipple-areola complex (ten cases) and tamoxifen (one case). DCIS was found in 45 operative specimens (96 per cent); eight had associated invasion. The DCIS was predominantly large cell solid/comedo in type and was multifocal in seven cases (19 per cent). At a median (range) follow-up of 56 (18-96) months, four of the ten patients treated by cone excision have developed a local recurrence, two of these patients have also developed metastases. Two of the 37 patients who underwent mastectomy developed loco-regional recurrences; both had invasive foci at their first operation and remain disease free at 8 years. We no longer feel that cone excision is appropriate treatment.