What is the treatment for confirmed Paget's disease of the breast?

Treatment for Confirmed Paget's Disease of Breast

The treatment for confirmed Paget's disease of the breast is either mastectomy with axillary staging or breast-conserving surgery (BCS) with excision of the nipple-areolar complex (NAC) followed by adjuvant radiotherapy, with the choice depending on the extent of disease. 1

Treatment Options Based on Disease Presentation

When Paget's Disease is Associated with Underlying Breast Cancer:

• For Paget's with invasive breast cancer:

  • Option 1: Mastectomy + axillary staging 1
  • Option 2: Excision of breast tumor and NAC + axillary staging with whole-breast radiation therapy (consider boost to breast and NAC sites) 1
  • Adjuvant systemic therapy should be administered according to the stage and hormone receptor status of the cancer 1

• For Paget's with DCIS (non-invasive cancer):

  • Option 1: Mastectomy ± axillary staging 1
  • Option 2: Excision of breast tumor and NAC with whole-breast radiation therapy (consider boost to breast and NAC sites) 1
  • Consider tamoxifen 20 mg per day for 5 years 1

When Paget's Disease Occurs Without Underlying Cancer:

• For Paget's disease alone (NAC positive, breast negative):
  • Option 1: Mastectomy + axillary staging 1
  • Option 2: Excision of NAC with whole-breast radiation therapy (consider boost to NAC sites) 1
  • Consider tamoxifen 20 mg per day for 5 years 1

Important Considerations for Treatment Selection

• MRI is recommended to assess the extent of disease and identify additional disease when Paget's disease is diagnosed 1

• Axillary staging is not necessary for Paget's disease with underlying DCIS when using breast-conserving therapy, unless there is evidence of invasive cancer 1

• For patients undergoing mastectomy with DCIS, axillary staging should be considered since the final pathology may reveal invasive cancer 1

• Breast conservation has shown local control and survival rates similar to mastectomy in selected patients, particularly those without palpable masses or mammographic abnormalities 2, 3

• Patients treated with breast conservation should undergo whole-breast radiation with consideration of a boost to the resected NAC site 1

Outcomes and Prognosis

• The risk of ipsilateral breast cancer recurrence after breast-conserving NAC resection and radiation therapy is similar to that associated with typical invasive or in situ cancer treatment 1

• Local recurrence rates after breast conservation are comparable to mastectomy when appropriate patient selection and clear surgical margins are achieved 2, 3

• Patients with Paget's disease associated with invasive cancer have a substantial risk of developing metastases and should receive appropriate systemic therapy 1

Common Pitfalls to Avoid

• Failing to perform adequate preoperative imaging to identify the extent of disease, as Paget's disease is associated with underlying breast cancer in over 90% of cases 4, 2

• Neglecting to consider sentinel lymph node biopsy, which has shown high accuracy in patients with Paget's disease 1, 4

• Incomplete excision of the nipple-areolar complex during breast-conserving surgery, which can lead to positive margins 2

• Omitting radiation therapy after breast-conserving surgery, which is essential for local control 5, 2