From the Guidelines
Extra mammary Paget's disease of the nipples should be treated with surgical excision with wide margins to ensure complete removal of the cancerous tissue, and in some cases, breast-conserving surgery with resection of the nipple areolar complex followed by whole-breast radiation therapy may be considered. 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 thorough evaluation, including imaging studies and biopsies, to identify any potential primary cancer 1. The treatment approach should be based on the stage and biologic characteristics of any underlying cancer, and adjuvant systemic therapy should be administered according to the stage of the cancer 1.
Key Considerations
- The skin of the nipple areolar complex should undergo surgical biopsy that includes the full thickness of the epidermis and at least a portion of any clinically involved nipple areola complex 1.
- Breast MRI is recommended to define the extent of disease and identify additional disease 1.
- 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 based on clinical examination, imaging evaluation, and full-thickness skin biopsy of the involved nipple areola complex 1.
- Patients treated with breast conservation should undergo whole-breast radiation, and extended-field radiation to regional lymph nodes should be used in cases of an associated invasive breast cancer with involved lymph nodes 1.
Treatment Options
- Surgical excision with wide margins to ensure complete removal of the cancerous tissue
- Breast-conserving surgery with resection of the nipple areolar complex followed by whole-breast radiation therapy
- Mastectomy with axillary dissection
- Topical treatments like imiquimod cream or 5-fluorouracil for cases where surgery isn't possible
- Radiation therapy as an adjuvant treatment or primary therapy for patients who cannot undergo surgery 1.
From the Research
Extra Mammary Paget's Disease of the Nipples
- Extra mammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands, such as the vulvar area, perianal region, scrotum, penis, and axillae 2.
- The primary location of extra mammary Paget's disease is not typically the nipples, but rather the vulvar area, followed by the perianal region, scrotum, penis, and axillae 2.
- Mammary Paget's disease, on the other hand, is a rare type of cancer that affects the nipple-areolar complex and is often associated with an underlying breast cancer 3, 4, 5.
Diagnosis and Treatment
- Diagnosis of extra mammary Paget's disease involves clinical evaluation, imaging, and histopathological confirmation of Paget cells 4, 6.
- Treatment options for extra mammary Paget's disease include surgical excision, such as Mohs surgery, and nonsurgical treatments like radiotherapy, imiquimod, and photodynamic therapy 4, 6.
- For mammary Paget's disease, treatment options typically involve surgical removal of the tumor, such as mastectomy or breast-conserving surgery, and may also include adjuvant chemotherapy, radiotherapy, or endocrine therapy 3, 4, 5.