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

  • Punch/wedge or surface biopsy of nipple tissue: Paget cells confirm disease.

  • Imaging to look for underlying DCIS/IDC (see “Diagnostics” below)

  • Differential diagnosis: mamillary eczema

  • Treatment

  • Breast-conserving surgery with subsequent adjuvant whole-breast radiation (see “Treatment” below)

  • Mastectomy