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The results of the European Organization for Research and Treatment of Cancer (EORTC) 10873 prospective study demonstrated that cone excision and radiotherapy (RT) of the conserved breast is an appropriate treatment for women with Paget disease of the nipple without underlying invasive carcinoma, with a 5-year actuarial local recurrence rate of 5.2%.1 Retrospective series also confirmed that patients with Paget disease can be treated effectively with limited surgery and RT.2, 3

The database of the National Institute of Oncology in Budapest, Hungary recently was reviewed and 62 women with mammary Paget disease were identified. Thirty-three of 62 patients with or without underlying ductal carcinoma in situ (DCIS) confined to the nipple-areolar complex were treated with cone excision without RT between 1980–1996. To our knowledge, it represents the largest group of patients treated by conservative surgery alone. The remaining 29 women were treated with mastectomy with or without axillary lymph node dissection due to a more extensive in situ component (12 patients) and/or an underlying invasive carcinoma (17 patients). Clinicopathologic characteristics and treatment results of our series of women who were treated with conservative surgery are summarized in Table 1 and compared with the EORTC prospective study.1 The two series were similar with regard to patient age, the median follow-up time, and the histology of the primary tumor, as well as the percentage of patients with a palpable mass. The mean time to local recurrence was found to be approximately 34 months in both groups. There also was no apparent significant difference between the two series with regard to the distribution of invasive and noninvasive recurrences (P = 0.4762, Fisher exact test). Conversely, the crude local recurrence rate reported in the current study was significantly higher than that reported in the EORTC study (33.3% vs. 6.6%; P = 0.0012). Dixon et al. also reported a local recurrence rate of 40% (4 of 10 cases) after cone excision alone.4 Six of 10 patients in the current study with an invasive recurrence developed distant metastasis, all of whom died of breast carcinoma. Other authors also emphasized that invasive recurrence is associated with a poor prognosis after breast-conserving therapy in patients with Paget disease of the nipple.1, 4

Table 1. Clinicopathologic Characteristics and Outcome of Patients in the Two Series
 Cone excision + RTa (n = 61)Cone excision aloneb (n = 33) 
  • RT: radiotherapy; UK: unknown; LR: local recurrence; Paget: Paget disease; DCIS: ductal carcinoma in situ.

  • a

    European Organization for Research and treatment of Cancer.

  • b

    Current study.

  • c

    Determined using the Fisher exact test.

Median age (yrs) (range)58 (31–74)65 (35–80)
Median follow-up (yrs) (range)6.4 (UK–12.5)6.0 (2–14)
Mean time to LR (mos) (range)33.5 (11–68)34.4 (8–83)
 No. of patients (%) P valuec
PremenopausalUK7 (21)
Palpable mass
 No59 (97)30 (91)0.2317
 Yes2 (3)3 (9)
Histology
 Paget without DCIS4 (7)3 (9)0.8067
 Paget with DCIS57 (93)30 (91)
Histology of LR
 Invasive3 (75)10 (91)0.4762
 DCIS1 (25)1 (9)
LR rate
 Crude4 (6.6)11 (33.3)0.0012
 5-yr actuarial3 (5.2)9 (28.4)-
Distant metastasis1 (1.6)6 (18.2)0.0070
Breast carcinoma death1 (1.6)6 (18.2)0.0070

Menopausal status (premenopausal vs. postmenopausal), palpable mass (yes vs. no), and histology (underlying DCIS vs. no underlying DCIS) were tested as potential risk factors for local recurrence in a Cox regression model. However, to our knowledge, none of these variables demonstrated significance. Other authors also stated that the small sample sizes and number of events in their series precluded the performance of a meaningful multivariate analysis.1, 3

Our conclusion is that Paget disease of the nipple cannot be treated successfully with cone excision alone. RT is mandatory after breast-conserving surgery to maintain adequate local control. Taking into account the relative frequency and aggressive behavior of invasive local recurrences after breast conservation in patients with Paget disease of the nipple, we believe tamoxifen also should be considered in the adjuvant setting.

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Csaba Polgár M.D., Ph.D.*, Zsolt Orosz M.D., Ph.D.†, Tibor Kovács M.D.‡, János Fodor M.D., Ph.D.§, * Department of Radiotherapy, National Institute of Oncology, Budapest, Hungary, † Department of Pathology, National Institute of Oncology, Budapest, Hungary, ‡ Department of General, and Thoracic Surgery, National Institute of Oncology, Budapest, Hungary, § Department of Radiotherapy, National Institute of Oncology, Budapest, Hungary