management and prognosis

Last reviewed 01/2018

Management is concentrated on detecting and treating any associated malignancy.

  • total mastectomy with axillary dissection may be preferable in older patients as it is rapid and reliable. In those who wish to avoid mastectomy, radiotherapy alone or after cone excision of the nipple, areola and underlying ducts may be offered
    • NICE suggest that breast conserving surgery with removal of the nipple-areolar complex should be offered as an alternative to mastectomy for patients with Paget's disease of the nipple that has been assessed as localised (1)
  • wide local excision without radiotherapy is unsatisfactory; it is associated with a 50% recurrence rate. Adjuvant therapy is indicated only for established invasive carcinoma
  • Paget's disease with associated intraduct carcinoma only and no palpable lump, has an excellent prognosis, with a cure rate approaching 100%

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