Paget disease of the breast
Paget disease of the breast is an intraepidermal adenocarcinoma of the nipple that signals an underlying ductal carcinoma in nearly every case. Diagnosis is confirmed by full-thickness nipple biopsy, and first-line treatment is surgical excision of the nipple-areolar complex with radiation and, for hormone-receptor-positive disease, adjuvant tamoxifen or an aromatase inhibitor.
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What this article covers
- Overview
Paget disease of the breast is an intraepidermal adenocarcinoma of the nipple and areola in which malignant cells migrate from an underlying ductal carcinoma into the overlying epidermis.
- Epidemiology
Paget disease of the breast is an uncommon presentation of breast cancer that occurs mainly in perimenopausal and postmenopausal women; it can rarely occur in men, in whom diagnosis is often delayed…
- Risk factors
Age: age 50 or older is a recognized threshold, since Paget disease is a manifestation of the same ductal carcinoma whose risk rises with age.
- Pathophysiology
The lesion arises from an underlying DCIS or invasive ductal carcinoma centered in the major lactiferous ducts beneath the nipple.
- Clinical features
Onset and course: insidious nipple pruritus, burning, or tingling over weeks to months, progressing to visible skin change; the lesion persists and slowly enlarges rather than clearing.
- Differential diagnosis
Atopic or irritant contact dermatitis of the areola: typically bilateral or exposure-linked, often spares the nipple itself, and clears within 2 to 4 weeks of topical corticosteroids, unlike the…
- Diagnosis
First, confirm the diagnosis with a full-thickness punch or wedge biopsy of the nipple lesion, or scrape or touch-prep cytology, showing Paget cells: large, pale, intraepidermal adenocarcinoma cells…
- Management
First, excise the nipple-areolar complex once biopsy and imaging define the extent of the underlying carcinoma; this excision is required in every patient and dictates the rest of the plan.
- Complications
Nipple-areolar complex loss or necrosis: a risk of central lumpectomy, with delayed healing and cosmetic deformity.
- Prognosis
Prognosis tracks the stage of the underlying ductal carcinoma, not the appearance of the skin lesion.
- Prevention
Population screening mammography: age 50 to 74, every 2 years per USPSTF guidance, so an underlying carcinoma is less likely to be advanced when nipple skin changes appear; screening under age 50 is reserved for high-risk individuals.
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