Info
Paget disease
- Definition: a rare type of breast cancer that affects the lactiferous ducts and the skin of the nipple and areola
- Pathogenesis
- Not fully understood
- The following two hypotheses have been postulated: [14]
- Migratory/epidermotropic theory: neoplastic ductal epithelial cells from an underlying DCIS or IDC move through the lactiferous ducts and invade the surrounding epidermis of the nipple.
- In situ malignant transformation of existing cells into Paget cells, meaning that the condition is a carcinoma in situ, without preexisting DCIS/IDC.
Clinical features-of-Paget disease
- Erythematous, scaly, or vesicular rash affecting the nipple and areola
- Pruritus; burning sensation
- Nipple retraction
- Ulceration that causes blood-tinged nipple discharge
Diagnostics
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Punch/wedge or surface biopsy of nipple tissue: Paget cells confirm disease.
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Imaging to look for underlying DCIS/IDC (see “Diagnostics” below)
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Differential diagnosis: mamillary eczema
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Treatment
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Breast-conserving surgery with subsequent adjuvant whole-breast radiation (see “Treatment” below)
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Mastectomy