THE CONSERVATIVE MANAGEMENT OF PAGETS-DISEASE OF THE BREAST WITH RADIOTHERAPY

BACKGROUND. The purpose of this study was to evaluate the feasibility of breast-conserving therapy involving limited surgery and definitive radio-therapy as a treatment for Paget's disease, and to determine the disease free and overall survival associated with this approach. METH ODS, The authors retrospectively reviewed the charts of all patients t reated during the period 1980-1994 for Paget's disease of the breast w ho did not present with a palpable mass or mammographic density. Throu gh a collaborative review, 30 cases were identified. A biopsy confirmi ng the presence of typical Paget's cells was performed on all patients . All patients received external beam radiotherapy to the breast, with a median dose of 50 gray (Gy). Ninety-seven percent received a boost to the remaining nipple or tumor bed, with a median dose to the tumor bed of 61.5 Gy. RESULTS. The median follow-up for surviving patients w as 62 months. Three patients (10%) developed a recurrence in the breas t as the only site of first failure, and 2 additional patients (7%) ex perienced failure in the breast as a component of first failure. The m edian time to local failure was 69 months. The 5- and 8-year actuarial estimates of local failure as the only site of first failure were 9% (95% confidence interval [CI], 0-20%) and 16% (95% CI, 0-31%), respect ively. Of the 5 patients with local failures, 3 were among 22 patients (14%) who underwent complete resection of the nipple or nipple-areola complex, compared with 2 failures among 6 patients (33%) after partia l resection (P = 0.29). There were no failures among 2 patients who ha d a biopsy only. Four of 5 local failures were salvaged by mastectomy, and 3 of these patients were free of disease after a median follow-up of 52 months. The 5- and 8-year estimates of disease free survival fo r the overall series were both 95% (95% CI, 87-100%); cause specific o verall survival was 100% at 8 years. CONCLUSIONS. Breast-conserving th erapy involving complete resection of the nipple-areola complex follow ed by definitive radiotherapy is a viable alternative to mastectomy in the treatment of Paget's disease. High rates of disease free and caus e specific survival, in addition to adequate local control, justify co nsideration of a conservative approach. (C) 1997 American Cancer Socie ty.