Treatment for Confirmed Paget's Disease of Breast

Treatment Options

The National Comprehensive Cancer Network recommends treatment for confirmed Paget's disease of the breast with either mastectomy with axillary staging or breast-conserving surgery with excision of the nipple-areolar complex followed by adjuvant radiotherapy, with the choice depending on the extent of disease 1, 2
For Paget's disease with invasive breast cancer, treatment options include mastectomy with axillary staging or excision of the breast tumor and nipple-areolar complex with axillary staging and whole-breast radiation therapy, with adjuvant systemic therapy administered according to the stage and hormone receptor status of the cancer 1, 3, 4, 5
For Paget's disease with ductal carcinoma in situ (DCIS), treatment options include mastectomy with or without axillary staging or excision of the breast tumor and nipple-areolar complex with whole-breast radiation therapy, with consideration of tamoxifen 20 mg per day for 5 years 1, 5, 6

The National Comprehensive Cancer Network recommends MRI to assess the extent of disease and identify additional disease when Paget's disease is diagnosed 1, 7
Axillary staging is not necessary for Paget's disease with underlying DCIS when using breast-conserving therapy, unless there is evidence of invasive cancer 5
Patients treated with breast conservation should undergo whole-breast radiation with consideration of a boost to the resected nipple-areolar complex site 4, 5

The risk of ipsilateral breast cancer recurrence after breast-conserving nipple-areolar complex resection and radiation therapy is similar to that associated with typical invasive or in situ cancer treatment 4, 7
Patients with Paget's disease associated with invasive cancer have a substantial risk of developing metastases and should receive appropriate systemic therapy 5