PAGETS-DISEASE OF THE BREAST - A 33-YEAR EXPERIENCE

Background: Paget's disease (PD) of the breast is an uncommon conditio n traditionally treated with mastectomy. To assess the natural history and treatment options in PD we have reviewed the experience at the Ro swell 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 me dian follow up was 61 months. Sixty-eight of 80 patients had nipple ch anges consistent with PD including 30 who had an associated breast mas s (group 1), and 38 who had no associated mass (group 2). Of the 68 pa tients with clinical PD, 58 (85%) were treated with mastectomy while t he 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 patient s (74%) in group 2. Breast cancer was centrally located (within 2 cm o f 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 (DC IS), and 16 (57%) had pathologic axillary nodes. Of the 28 patients wi th an associated BC in group 2, 10 (36%) had invasive cancer, 18 (64%) had DCIS, and 6 (21%) had positive axillary nodes. The median surviva l was significantly shorter for group 1 (42 months) than for group 2 ( 126 months; p = 0.007). Conclusion: Most patients with PD have an asso ciated BC (82%) that was centrally located (61%). In those patients wi thout an associated mass, a significant number (26% in this series) ma y not have an underlying BC and may be overtreated with mastectomy. On the other hand, a significant number of patients with PD and no assoc iated breast mass will be found to have a peripherally located tumor ( 29% in this series). These noncentral BC masses could potentially be m issed by a wide local excision that includes the areolar complex. (J A m Coll Surg 1998;187:171-177. (C) 1998 by the American College of Surg eons)