Updated: 8/8/2020

Paget Disease of the Breast

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Snapshot
  • A 56-year-old woman presents to the physician with complaint of redness and itching in her left nipple. She says she has noticed that her areola has felt "rough and bumpy" and that there is occasional oozing of yellow pus from the skin around the nipple. Physical examination shows the finding seen in the image. The physician orders a mammogram and a punch biopsy of the skin around the nipple.
Introduction
  • Overview
    • Paget disease of the breast is a chronic, eczematous rash on the nipple and adjacent areolar skin
  • Epidemiology
    • incidence
      • occurs in 1-4% of cases of female breast cancer
        • underlying carcinoma is present in nearly 100% of cases
      • almost exclusively seen in women
        • < 2% of cases are seen in men
    • demographics
      • mean age at diagnosis is 55 years
  • Pathophysiology 
    • associated with an underlying breast adenocarcinoma extension of malignant epithelial (Paget) cells through the lactiferous ducts and ductules into the epidermis
    • proliferation of malignant epithelial cells causes thickening of the nipple and surrounding areolar skin
  • Prognosis
    • dependent upon presence of the underlying invasive ductal carcinoma or axillary node metastases
    • Paget disease of the breast that presents with a palpable mass is usually associated with a worse prognosis than cases without a palpable mass
      • palpable mass and invasive ductal carcinoma: 5-year survival around 35-45%
      • no mass and invasive ductal carcinoma: 5-year survival around 90-100%
Presentation
  • Symptoms
    • erythema
    • scaling
    • itching and burning sensation
    • ulceration
    • oozing
    • bleeding
  • Physical exam
    • eczematous lesion involving the nipple and areolar areas
Imaging
  • Mammography
    • indications
      • locate underlying breast carcinoma
    • findings
      • subareolar microcalcifications
      • thickening of the nipple and areola
Studies
  • Scrape cytology
    • rapid diagnostic screening method
    • findings
      • presence of malignant Paget cells
  • Biopsy
    • punch, wedge, or excisional biopsy of the skin lesion in the nipple and areola
      • histologic features of Paget disease
        • hyperkeratosis, parakeratosis, and acanthosis of the epidermis
        • epidermal cells stain with antibodies against carcinoembryonic antigen
        • nests of malignant Paget cells in layers of the epidermis
          • clear cells with large, pleomorphic nuclei that spread individually into the epidermis ("pagetoid")
Differential
  • Bowen disease
    • key distinguishing factor
      • epidermal cells stain with antibodies to keratin and NOT to carcinoembryonic antigen
  • Cutaneous melanoma
    • key distinguishing factor
      • epidermal cells stain with antibodies to S-100 and NOT to carcinoembryonic antigen
  • Contact dermatitis
    • key distinguishing factor
      • onset of symptoms occurs after exposure to an irritant
Treatment
  • Surgical
    • mastectomy and lymph node dissection
      • indications
        • patients with Paget disease of the breast and underlying invasive breast carcinoma

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