Breast-conserving therapy for Paget disease of the nipple - A prospective European Organization for Research and Treatment of Cancer Study of 61 patients

BACKGROUND. The purpose of the current study was to assess the outcome of b reast-conserving therapy by means of a cone excision and radiotherapy in pa tients with Paget disease of the nipple without associated invasive breast carcinoma. METHODS. Between 1987 and 1998, 61 eligible patients were registered in the European Organization for Research and Treatment of Cancer Study 10873. Th e majority of patients (97%) presented without an associated palpable mass. At histologic examination, the majority (93%%) of patients had an underlyi ng ductal carcinoma in situ (DCIS); in the remaining 7%, only Paget disease was found. Treatment was comprised of a complete excision of the nipple-ar eolar complex including the underlying breast tissue with tumor free margin s, followed by external irradiation to the whole breast (50 gray in 25 frac tions). The primary endpoint was local recurrence. RESULTS. At a median follow-up of 6.4 years, 4 of the 61 patients developed a recurrence in the treated breast (1 patient with DCIS and 3 patients wit h invasive disease). One patient with an invasive local recurrence died of disseminated breast carcinoma. The 5-year local recurrence rate was 5.2%, ( 95% confidence interval, 1.8-14.1%). CONCLUSIONS. Breast-conserving therapy is a feasible alternative for patien ts with Paget disease and a limited extent of underlying DCIS. To achieve g ood local control, treatment should be comprised of a complete excision of the nipple-areolar complex including the underlying disease, followed by ir radiation to the whole breast. (C) 2001 American Cancer Society.