Paget disease of the breast: Changing patterns of incidence, clinical presentation, and treatment in the U.S.

BACKGROUND: Paget disease is an uncommon presentation of breast cancer that increased in incidence in the U.S. between 1973 and 1987. Characterized by malignant crusting or ulceration of the nipple, Paget disease can present in 1 of 3 ways: 1) in conjunction with an underlying invasive cancer, 2) in conjunction with underlying ductal carcinoma in situ (DCIS), or 3) alone without any underlying invasive breast carcinoma or DCIS. Paget disease can be treated with breast conservation by undergoing central lumpectomy. The objective of this study was to determine how the incidence, presentation, biomarkers, operative approach, and outcome of Paget disease have evolved in the U.S. since 1988. METHODS: Between 1988 and 2002, 1738 women who were diagnosed with Paget disease were reported in the 9 registries of the Surveillance, Epidemiology, and End Results Program. To the authors' knowledge, the current study on tumor characteristics, surgical intervention, and survival represented the largest series of Paget disease ever reported. RESULTS: Although the overall incidence of breast cancer increased between 1988 and 2002, the incidence of Paget disease concomitantly decreased by 45% (95% confidence interval, from -35% to -53%). This decreasing incidence was greatest for Paget disease associated with invasive cancer or DCIS. Invasive cancer associated with Paget disease more commonly was estrogen receptor negative, progesterone receptor negative, and of high histologic grade. Even when 60% of the disease was located centrally, only 293 of 1642 patients with Paget disease (18%) who were treated surgically underwent central lumpectomy. Patients with Paget disease who underwent breast conservation had outcomes equivalent to the outcomes among patients who underwent mastectomy. CONCLUSIONS: The incidence of Paget disease associated with underlying invasive cancer or DCIS decreased since 1988. Patients who underwent central lumpectomy and patients who underwent mastectomy for Paget disease had similar outcomes; nonetheless, most patients who were candidates for preservation underwent mastectomy.