What is the management approach for Paget disease of the nipple?

Management of Paget Disease of the Nipple

Breast-conserving surgery with complete excision of the nipple-areolar complex followed by whole-breast radiation therapy is now an appropriate alternative to mastectomy, achieving equivalent local control and survival outcomes. 1, 2

Initial Diagnostic Workup

When Paget disease is confirmed by full-thickness skin biopsy of the nipple-areolar complex, obtain breast MRI to define the full extent of disease and identify occult malignancies elsewhere in the breast, as 80-90% of cases have associated breast cancer that may not be adjacent to the nipple. 3, 1, 2

Complete the workup with:

• Full history and physical examination focusing on nipple changes (eczema, ulceration, itching, burning sensation) and palpable breast masses 3, 4
• Diagnostic breast imaging (mammography and ultrasound) to identify any underlying breast lesions 3
• Full-thickness surgical biopsy of the nipple-areolar complex including epidermis and underlying tissue 3

Surgical Treatment Algorithm

For Paget Disease WITHOUT Associated Cancer (No Mass or Imaging Abnormality)

Breast-conserving surgery:

• Remove the entire nipple-areolar complex with negative margins of underlying breast tissue 3, 2
• No axillary staging required when only DCIS is present without evidence of invasive cancer 3
• Follow with mandatory whole-breast radiation therapy 3, 2
• Consider radiation boost to the resected nipple-areolar complex site 3, 2

Alternative: Total mastectomy remains reasonable, with consideration of axillary staging even for DCIS (since final pathology may reveal invasive cancer and mastectomy precludes subsequent sentinel node biopsy) 3

For Paget Disease WITH Associated Cancer

Breast-conserving surgery:

• Remove the nipple-areolar complex with negative margins 3, 1
• Remove the peripheral cancer using standard breast-conserving technique with negative margins 3
• The nipple-areolar complex and peripheral cancer do not need to be removed in continuity or through a single incision 3, 2
• Perform axillary staging according to standard guidelines if invasive cancer is present 3, 1, 2
• Follow with whole-breast radiation therapy plus boost 3, 2

Alternative: Mastectomy with axillary staging 3, 1

Radiation Therapy

• Whole-breast radiation is mandatory for all patients treated with breast conservation 3, 2
• Consider radiation boost to the resected nipple-areolar complex site and any associated cancer site 3, 2
• Use extended-field radiation to regional lymph nodes for invasive cancer with involved lymph nodes 3

Systemic Therapy

Base systemic therapy decisions on the stage and biologic characteristics of any underlying cancer, not on the presence of Paget disease itself: 3, 1, 2

• For Paget disease with invasive cancer: Administer adjuvant systemic therapy according to cancer stage and hormone receptor status, as these patients have substantial risk of metastases 3, 1, 2
• For Paget disease with DCIS only or no associated cancer: Consider tamoxifen 20 mg daily for 5 years for risk reduction 3, 1

Expected Outcomes

Breast-conserving surgery with radiation achieves ipsilateral breast cancer recurrence rates similar to standard breast-conserving surgery for typical invasive or in situ cancers. 3, 1, 2 The 5-year local recurrence rate is approximately 5% with proper technique. 5

Critical Pitfalls to Avoid

• Do not delay diagnosis by treating as dermatologic condition—any persistent nipple eczema or ulceration requires full-thickness biopsy 3, 6
• Do not skip MRI after diagnosis—it identifies additional occult disease in the majority of cases 3, 1, 2
• Do not perform axillary staging for DCIS treated with breast conservation unless invasive cancer is suspected 3
• Do not omit radiation therapy after breast-conserving surgery—it is mandatory for local control 3, 2