Treatment of Paget's Disease of the Breast
Introduction to Treatment Options
- The National Comprehensive Cancer Network recommends breast-conserving surgery with complete nipple-areolar complex excision plus whole-breast radiation therapy as an appropriate treatment option that achieves local control and survival comparable to mastectomy 1, 2
Initial Diagnostic Workup
- Obtain breast MRI after biopsy confirmation to define the full extent of disease and identify any additional occult malignancies, as up to 80-90% of cases have associated breast cancer elsewhere that may not be adjacent to the nipple-areolar complex 2, 3
Surgical Treatment Algorithm
- Remove the entire nipple-areolar complex with negative margins of underlying breast tissue, and follow with whole-breast radiation therapy for Paget's disease without associated cancer 1, 2, 3
- The nipple-areolar complex and peripheral cancer do not need to be removed in continuity or through a single incision for Paget's disease with associated cancer 1, 2
- Perform axillary surgery according to standard staging guidelines if underlying invasive cancer is present 3, 6
Radiation Therapy
- Whole-breast radiation is mandatory for all patients treated with breast conservation 5
- Consider a radiation boost to the site of the resected nipple-areolar complex and any associated cancer site 5
Systemic Therapy
- Base systemic therapy decisions on the stage and biologic characteristics of any underlying cancer, not on the presence of Paget's disease itself 1, 2
- Women with associated invasive cancer have substantial risk of metastases and should receive adjuvant systemic therapy according to cancer stage 5