Paget Disease of the Breast

• The clinical hallmarks of Paget’s disease of the breast are:
  • Scaling, erythema, and / or ulceration of the nipple sometimes extending to the areola

• Because the main differential diagnosis for this clinical presentation is eczema:
  • A short course of topical steroids is an appropriate initial step:
    • Failure to resolve should prompt tissue biopsy by punch or wedge technique and not additional steroid therapy
• Pathology revealing adenocarcinoma cells within the epidermis (Paget cells):
  • Confirms the diagnosis

• HER2 amplification:
  • Is found in 60% to 90% of cases of Paget’s disease of the breast:
    • But the patient should be fully evaluated prior to making decisions regarding the need for targeted therapy
• Appropriate diagnostic imaging includes:
  • Mammography, ultrasound, and / or breast MRI:
    • As Paget’s disease is associated with an underlying malignancy 85% of the time
• The appropriate surgical management of Paget’s disease is:
  • Breast conservation with central mastectomy (resection of the nipple-areolar complex) with resection of the primary tumor and irradiation or mastectomy
• References
  • Chen CY, Sun LM, Anderson BO. Paget disease of the breast: changing patterns of incidence, clinical presentation, and treatment in the U.S. Cancer. 2006;107(7):1448-1458.
  • Killelea BK, Chagpar AB, Horowitz NR, Lannin DR. Characteristics and treatment of human epidermal growth factor receptor 2 positive breast cancer: 43,485 cases from the National Cancer Database treated in 2010 and 2011. Am J Surg. 2017;213(2):426-432.
  • Caliskan M, Gatti G, Sosnovskikh I, et al. Paget’s disease of the breast: the experience of the European Institute of Oncology and review of the literature. Breast Cancer Res Treat. 2008;112(3):513-521.

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