Treatment of Paget's Disease of the Breast
Breast-conserving surgery with complete nipple-areolar complex excision plus whole-breast radiation therapy is an appropriate treatment option that achieves local control and survival comparable to mastectomy, though total mastectomy remains a reasonable alternative regardless of whether underlying breast cancer is present. 1
Initial Diagnostic Workup
Before determining treatment, proper staging is essential:
- 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 1, 2
- Complete history and physical examination focusing on eczema, bleeding, ulceration, and itching of the nipple 2
- Bilateral diagnostic mammography with or without ultrasound, though be aware that pure Paget's disease can be occult on mammography 2
Surgical Treatment Algorithm
For Paget's 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 1
- Follow with whole-breast radiation therapy 1
- Axillary lymph node staging is NOT necessary in this scenario 1
Alternative: Total Mastectomy:
- Remains a reasonable option 1
- Consider axillary staging even without invasive disease, as final pathology may reveal occult invasion and mastectomy precludes subsequent sentinel node biopsy 1
For Paget's Disease WITH Associated Cancer:
Breast-Conserving Surgery:
- Remove the nipple-areolar complex with negative margins 1
- Remove the peripheral cancer using standard breast-conserving technique to achieve negative margins 1
- The nipple-areolar complex and peripheral cancer do NOT need to be removed in continuity or through a single incision 1
- Perform axillary surgery according to standard staging guidelines if underlying invasive cancer is present 1
- Follow with whole-breast radiation therapy 1
Alternative: Total Mastectomy:
- Remains appropriate and was historically the standard of care 1
- Axillary staging recommended for invasive disease 1
Radiation Therapy
- Whole-breast radiation is mandatory for all patients treated with breast conservation 1
- Consider a radiation boost to the site of the resected nipple-areolar complex and any associated cancer site 1
- Extended-field radiation to regional lymph nodes should be used if associated invasive cancer has involved lymph nodes 1
Critical Pitfall to Avoid
Do NOT perform breast-conserving surgery without radiation therapy when associated breast cancer is present. A recent study showed that in patients with Paget's disease associated with non-invasive breast cancer treated by breast-conserving surgery without radiotherapy, local recurrence occurred in 50% of cases (3 out of 6 patients), which was significantly higher than those undergoing mastectomy 3. This represents inadequate oncological therapy.
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
- Women with associated invasive cancer have substantial risk of metastases and should receive adjuvant systemic therapy according to cancer stage 1
- Note that tumors associated with Paget's disease are frequently ER-negative and HER2-positive 3
Expected Outcomes
- Breast-conserving surgery with radiation achieves similar ipsilateral breast cancer recurrence rates as standard breast-conserving surgery for typical invasive or in situ cancers 1
- Local control and survival rates with breast conservation are comparable to mastectomy 4, 5
- The 10-year cumulative local recurrence rate is approximately 8% after mastectomy and 16% after breast-conserving surgery 5