PAGETS-DISEASE OF THE NIPPLE - A 14 YEAR EXPERIENCE

Paget's disease of the nipple represents an unusual hut potentially le thal form of breast cancer, From 1983 to 1996, twenty-eight patients w ith Paget's disease of the nipple were treated at our institution. Pre senting symptoms which included nipple ulceration, nipple discharge an d a palpable lump were present for an average of 31 weeks (range = 1-1 10 weeks) prior to diagnosis. The mean age at diagnosis was 54 years ( range 30-74 years). The mean follow-up was 35 months (range 7-134 mont hs). The histopathologic types were Paget's disease of the nipple alon e in 4 patients, Paget's with ductal carcinoma in situ in 8 patients, Paget's with lobular carcinoma in situ in one patient and Paget's with invasive cancer in 15 patients. Treatment was by mastectomy in 25 cas es and by breast conserving surgery in one case. Two patients did not have operative treatment because of metastatic disease. Patients who p resented without a palpable lump (n=16) are all alive with no evidence of disease. Sis of the twelve patients who presented with a palpable lump have developed recurrent disease and four have died of their dise ase. In Paget's disease of the nipple, there was an average delay of 3 1 weeks for patients to present with their symptoms, The presence of a palpable mass at presentation significantly impairs the long-term out come.