Paget’s Disease

  • BBSG – Brazilian Breast Study Group


Paget’s disease (PD) is a rare breast impairment whose first cancer association was described by James Paget in 1874. PD is histologically characterized by large malignant cells presenting hyper-chromatic nucleus and pale cytoplasm inside of the epidermis that convers the nipple, exhibiting, in most cases, overexpression of HER2 in immunohistochemical tests (IHC).


Paget disease Carcinoma Eczema Intraductal carcinoma Conservative management 

Recommended Reading

  1. 1.
    Bijker N, Rutgers EJ, Duchateau L, Peterse JL, Julien JP, Cataliotti L. Breast conserving therapy for Paget disease of the nipple: a prospective European Organization for Research and Treatment of Cancer study of 61 patients. Cancer. 2001;91:472–7. A study conducted by the European Organization for Research and Treatment of Cancer (EORTC) encompassed 61 patients submitted to BCT, including women with palpable mass, supporting 7% local recurrence after a mean follow-up of 6.4 years. CrossRefGoogle Scholar
  2. 2.
    Lagios MD, Westdahl PR, Rose MR, Concannon S. Paget’s disease of the nipple. Alternative management in cases without or with minimal extent of underlying breast carcinoma. Cancer. 1984;54:545–51. Retrospectively evaluated a population of 200 women treated for PD over a 25-year period, with 20% of patients undergoing BCT while the rest underwent total mastectomy. The authors concluded that the surgery type performed did not influence disease-free survival. CrossRefGoogle Scholar
  3. 3.
    Marshall JK, Griffith KA, Haffty BG, Solin LJ, Vicini FA, McCormick B, et al. Conservative management of Paget disease of breast with radiotherapy: 10- and 15-year results. Cancer. 2003;97:2142–9. This analysis, performed in patients with no palpable mass, supported local recurrence in 11% after BCT and a mean follow-up of 113 months. CrossRefGoogle Scholar
  4. 4.
    Morrogh M, Morris EA, Liberman L, Van Zee K, Cody HS 3rd, King TA. MRI identifies otherwise occult disease in select patients with Paget disease of the nipple. J Am Coll Surg. 2008;206:316–21. 58 patients evaluation comparing MRI to conventional examinations in PD, supporting that the sensitivity to detect IC in MMG and US were 79% and 74%, respectively. Regarding ISDC, the percentage was even lower (39% and 19%). MRI had, on the other hand, 100% sensitivity for IC and 44% for ISDC. CrossRefGoogle Scholar
  5. 5.
    Wong SM, Freedman RA, Stamell E, Sagara Y, Brock JE, Desantis SD, et al. Modern trends in the surgical management of Paget’s disease. Ann Surg Oncol. 2015;22:3308–16. Supported that PD associated to IC or ISDC incidence declined. Women were more likely to have higher histological grades, more commonly committed by negative hormone receptor (HR), and the axilla was impaired in up to 47.1% of IC cases. CrossRefGoogle Scholar

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • BBSG – Brazilian Breast Study Group
    • 1
  1. 1.BBSGSao PauloBrazil

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