Paget disease of the nipple

BACKGROUND The treatment of Paget disease by mastectomy has been challenged recently in favor of breast‐conserving techniques. A large series of patients treated with mastectomy has been reviewed to assess the feasibility of less radical surgery. METHODS The cases of 70 women with a clinical diagnosis of Paget disease were reviewed. The type, grade, receptor and node status, and the mammographic and pathologic extent of the underlying breast malignancy were determined. The survival of patients with invasive disease was compared with matched controls without Paget disease. RESULTS The underlying malignancy was invasive in 58% of cases. Despite the fact that only one third of women presented with a palpable mass, the malignancy was frequently extensive, being confined to the retroareolar region in only 25% of cases. The true extent of the disease was underestimated by mammography in 43% of cases. Of the patients with ductal in situ carcinoma, 96.5% had high‐grade carcinomas and 100% had invasive carcinomas of high cytonuclear grade. Overexpression of the c‐erb‐B2 oncogene was detectable in 83% of cases. Patients with Paget disease had a significantly worse survival than matched controls, but this difference was eliminated if they were also matched for c‐erb‐B2 status. CONCLUSIONS Paget disease is often associated with extensive underlying malignancy, which is difficult to assess accurately either clinically or mammographically. As a consequence, cone excision of the nipple would have resulted in incomplete excision in 75% of cases. The underlying disease is of high grade and is frequently c‐erb‐B2 positive with a resulting poor prognosis. Aggressive local and systemic treatment would seem to be merited. Cancer 2002;95:1–7. © 2002 American Cancer Society. DOI 10.1002/cncr.10638 The characteristics of 70 women with Paget disease were reviewed to determine whether a cone excision would have provided effective surgical clearance of the underlying malignancy. In 43% of cases the disease was underestimated by mammography and in 75% of cases it was multicentric precluding cone excision. In addition, 96.5% of cases of ductal carcinoma in situ and 100% of cases of invasive carcinomas were of high cytonuclear grade. Survival of patients with Paget disease was significantly worse than that of age‐matched controls. This was largely attributable to the preponderance of c‐erb‐B2 overexpression, which was demonstrated in 83% of cases.