Original Scientific Articles
Paget’s disease of the breast: a 33-year experience

https://doi.org/10.1016/S1072-7515(98)00143-4Get rights and content

Abstract

Background: Paget’s disease (PD) of the breast is an uncommon condition traditionally treated with mastectomy. To assess the natural history and treatment options in PD we have reviewed the experience at the Roswell Park Cancer Institute (RPCI).

Study Design: The medical records of patients treated for PD at RPCI between 1963 and 1996 were reviewed.

Results: Eighty patients were treated in the period reviewed. The median follow up was 61 months. Sixty- eight of 80 patients had nipple changes consistent with PD including 30 who had an associated breast mass (group 1), and 38 who had no associated mass (group 2). Of the 68 patients with clinical PD, 58 (85%) were treated with mastectomy while the remainder were treated with some form of breast conserving therapy. Breast cancer (BC) was found in 56 of 68 patients (82%) with clinical PD, including 28 of 30 patients (93%) in group 1 and 28 of 38 patients (74%) in group 2. Breast cancer was centrally located (within 2 cm of the areolar margin) in 61% of patients, including 71% in group 1 and 50% in group 2. Of the 28 patients with an associated BC in group 1, 21 (75%) had invasive cancer, 6 (21%) had ductal carcinoma in situ (DCIS), and 16 (57%) had pathologic axillary nodes. Of the 28 patients with an associated BC in group 2, 10 (36%) had invasive cancer, 18 (64%) had DCIS, and 6 (21%) had positive axillary nodes. The median survival was significantly shorter for group 1 (42 months) than for group 2 (126 months; p = 0.007).

Conclusion: Most patients with PD have an associated BC (82%) that was centrally located (61%). In those patients without an associated mass, a significant number (26% in this series) may not have an underlying BC and may be overtreated with mastectomy. On the other hand, a significant number of patients with PD and no associated breast mass will be found to have a peripherally located tumor (29% in this series). These non-central BC masses could potentially be missed by a wide local excision that includes the areolar complex.

Section snippets

Methods

The medical records of all patients diagnosed or treated at RPCI for PD between January 1963 and June 1996 were reviewed. Collected data included: age at diagnosis, race, menopausal status, family history, symptoms, physical examination findings, mammographic findings, surgical therapy, pathologic findings/diagnosis, lymph node involvement, surgical therapy, adjuvant therapy, recurrence of cancer, and date of last followup. Persons having one or more first degree relatives with breast cancer

Results

In the 33-year period reviewed, 2 men and 78 women with a histologic diagnosis of PD were seen or treated at RPCI. The median followup for all patients was 61 months (range 2–288 months). The median age at diagnosis was 57 years (range 23–85 years). At the time of diagnosis 76% of the women were postmenopausal. Ten patients (13%) had a first-degree relative with breast cancer.

Patient characteristics and clinical findings are summarized in Table 1. Of the 80 patients with a histologic diagnosis

Discussion

At RPCI, 80 patients with PD were treated over a 33-year period and 82% were found to have a related breast cancer. Sixty four percent of patients presented with nipple ulceration and 31% complained of nipple discharge. Thirty of 68 patients (group 1) were found to have a clinically apparent breast lesion. A cancer was identified in 93% of these patients (75% invasive, 25% DCIS). Fifty-seven percent of the patients in this group had pathologic axillary nodes and their median tumor size was 4.2

Conclusions

This retrospective review of patients with Paget’s disease of the breast treated at a single institution over 33 years reinforces the well described natural history of the disease. It also suggests that an underlying breast cancer is found in most, but not all, patients with PD and identifies differences between PD and DCIS. These differences suggest that treatment of PD, especially in patients with no associated breast mass, cannot simply reflect the treatment of DCIS. The treatment of

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