From the Guidelines
Paget's disease of the breast should be treated with breast-conserving surgery, including removal of the entire nipple areola complex with a negative margin of underlying breast tissue, and whole-breast radiation therapy, as this approach has been shown to achieve satisfactory local control and is a reasonable option for patients regardless of the absence or presence of an associated breast cancer. The diagnosis of Paget's disease is often delayed due to its rare nature and confusion with other dermatologic conditions, and it is essential to perform a full-thickness skin biopsy of the involved nipple areola complex to confirm the diagnosis 1.
The treatment of Paget's disease depends on the presence and extent of underlying breast cancer, and breast-conserving surgery with radiation therapy is a viable option for patients with Paget's disease and associated DCIS or invasive breast cancer. In cases without detectable underlying cancer, breast-conserving surgery may be sufficient 1. The prognosis depends primarily on the stage and characteristics of any underlying breast cancer rather than the Paget's disease itself.
Key considerations in the treatment of Paget's disease include:
- Removal of the entire nipple areola complex with a negative margin of underlying breast tissue
- Whole-breast radiation therapy
- Axillary lymph node staging is not necessary when breast-conserving therapy is used to treat Paget's disease with underlying DCIS in the absence of evidence of invasive cancer
- Adjuvant systemic therapy should be administered according to the stage of the cancer
- Mastectomy is always an option with any manifestation of Paget's disease.
It is crucial to note that the treatment approach should be individualized based on the patient's specific condition and characteristics, and early detection and appropriate treatment are crucial for optimal outcomes 1.
From the Research
Overview of Paget's Disease of the Breast
- Paget's disease of the breast is a rare disorder of the nipple-areola complex often associated with an underlying in situ or invasive carcinoma 2.
- It typically affects postmenopausal women and is associated with an underlying malignancy 3.
- Skin changes are a common presenting symptom, as well as a lump, nipple discharge, pain, and changes to the nipple shape 3.
Diagnosis and Management
- The definitive diagnostic investigation is a tissue core biopsy 3.
- Imaging options include ultrasound for women under the age of 35 years or mammogram and ultrasound for women over the age of 40 years 3.
- Management options are typically surgical and include breast-conserving surgery or mastectomy in addition to oncoplastic techniques 3.
- Sentinel lymph node biopsy is performed in all patients undergoing surgery 3, 4.
- Adjuvant chemotherapy, radiotherapy, or endocrine therapy can be used to treat concomitant invasive disease or ductal carcinoma in situ 3.
Clinical Presentation and Treatment
- Eczematoid changes of the nipple-areola complex and persistent soreness or itching should raise suspicion of this disease 2.
- A case of a 29-year-old female with erythema and skin excoriations of the left breast nipple-areolar complex was diagnosed with Paget's disease and ductal carcinoma in situ (DCIS) 4.
- Treatment consisted of a left breast skin-sparing simple mastectomy with sentinel lymph node (SLN) biopsy and immediate tissue expander placement for implant reconstruction 4.
Local Recurrence and Risk Factor Analysis
- Paget's disease can occur as a local recurrence after nipple-sparing mastectomy, with an average latency period of 32 months 5.
- Complete nipple-areola complex removal was performed in all seven recurrences, with no locoregional relapse or metastatic event reported 5.
- Primary carcinoma with ductal intraepithelial neoplasia or invasive ductal carcinoma with extensive in situ component, negative hormonal receptor, high pathological grade, overexpression of HER2/neu, and "HER2 positive (nonluminal)" subtype tend to be significantly associated with more Paget's disease local recurrence 5.