What is the best management approach for Paget's disease of the breast?

Management of Paget's Disease of the Breast

For Paget's disease of the breast, both breast-conserving surgery with radiation therapy and mastectomy are appropriate treatment options, with the choice depending on the presence and extent of underlying cancer. 1

Diagnostic Workup

• Clinical presentation: Nipple or areolar eczema, ulceration, bleeding, and itching 2, 1
• Required imaging:
  • Diagnostic bilateral mammogram
  • Ultrasound as necessary
  • Breast MRI (recommended to define extent of disease and identify additional disease) 2, 1, 3
• Pathological confirmation:
  • Full-thickness skin biopsy of involved nipple-areolar complex (NAC)
  • Core biopsy of any breast lesion identified on imaging 2, 1

Treatment Algorithm

1. Paget's Disease Without Underlying Cancer ("Pure" Paget's)

• Options:
  • Breast-conserving approach: Excision of entire NAC with negative margins + whole-breast radiation therapy (consider boost to NAC site) 2, 1
  • Mastectomy with or without axillary staging 2, 1
• Axillary management: Not necessary with breast-conserving therapy 2
• Adjuvant therapy: Consider tamoxifen 20 mg daily for 5 years 2, 1

2. Paget's Disease With DCIS

• Options:
  • Breast-conserving approach: Excision of breast tumor and NAC with negative margins + whole-breast radiation therapy (consider boost to breast and NAC sites) 2, 1
  • Mastectomy with consideration of axillary staging 2, 1
• Axillary management: Not necessary with breast-conserving therapy; consider with mastectomy as final pathology may reveal invasive component 2
• Adjuvant therapy: Consider tamoxifen 20 mg daily for 5 years 2, 1

3. Paget's Disease With Invasive Cancer

• Options:
  • Breast-conserving approach: Excision of breast tumor and NAC with negative margins + axillary staging + whole-breast radiation therapy (consider boost to breast and NAC sites) 2, 1
  • Mastectomy with axillary staging 2, 1
• Axillary management: Perform according to standard breast cancer guidelines 2
• Adjuvant therapy: Based on stage and biological characteristics of the underlying cancer 2, 1

Evidence for Treatment Approaches

• Long-term data support breast-conserving therapy with radiation as an alternative to mastectomy with equivalent outcomes:
  • 10-year and 15-year local control rates of 87% for breast-conserving surgery with radiation 4
  • No significant differences in overall, disease-specific, or recurrence-free survival between mastectomy and breast-conserving approaches 5

Important Considerations

• Risk of underlying malignancy: 80-90% of Paget's disease cases have an underlying breast carcinoma (in situ or invasive), even without evident breast mass or mammographic abnormality 3
• Radiation importance: Breast-conserving surgery without radiation therapy has significantly higher local recurrence rates, particularly when associated with non-invasive breast cancer 6
• Surgical margins: All surgical conservative approaches should include complete removal of the nipple-areolar complex with negative margins 7
• Sentinel lymph node considerations:
  • Not necessary for pure Paget's or Paget's with DCIS treated with breast conservation
  • Recommended for Paget's with invasive cancer
  • Should be considered with mastectomy even for DCIS, as invasive component may be found in final pathology 2, 3

Common Pitfalls to Avoid

1. Delayed diagnosis: Median time from symptoms to diagnosis can be 20 weeks; don't dismiss persistent nipple changes as benign dermatitis 6
2. Inadequate imaging: Always include MRI to detect occult malignancy 3
3. Undertreatment: Breast-conserving surgery without radiation therapy is inadequate when associated malignancy is present 6
4. Inadequate margins: Ensure complete removal of the nipple-areolar complex with negative margins 7
5. Overlooking axillary staging with mastectomy: Consider sentinel node biopsy with mastectomy even for DCIS, as subsequent sentinel node biopsy would be precluded if invasive cancer is found 2, 3