Paget Disease of the Breast

Paget Disease of the Breast is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • Rare disease of the nipple-areola complex (NAC) typically associated with underlying in situ or invasive carcinoma
  • Characterized by eczematous changes of the nipple, erythema, ulceration, crusting, bleeding, and/or itching
  • Divided into three categories (1)
    • Paget disease of the nipple without ductal carcinoma in situ (DCIS)
    • Paget disease of the nipple with associated DCIS in the underlying lactiferous ducts of the NAC
    • Paget disease of the nipple with associated DCIS in the underlying lactiferous ducts of the NAC and associated DCIS or invasive breast cancer elsewhere in the breast at least 2 cm from the NAC
  • System(s) affected: skin/exocrine

Epidemiology

Incidence
  • 1–3% of breast cancers in females (2)
  • 0.4% of invasive female breast cancer (3)
  • Incidence of Paget disease of the breast has been decreasing since 1988, despite an increased incidence of breast cancer (4,5).
  • Median age at diagnosis = 64 years (5)
  • Extremely uncommon in males but prognosis is worse in men (6)

Prevalence
<1% of population

Etiology and Pathophysiology

  • Cause is unknown, but risk factors for Paget disease are similar to those for developing breast cancer in general (see below).
  • Epidermotropic theory
    • Ductal carcinoma cells migrate from underlying mammary ducts to epidermis of the nipple to become Paget cells (4,6,7).
  • Transformation theory (not favored)
    • Epidermal cells of nipple/areola transform into Paget cells that can invade the basement membrane into the dermis (4,6,7).

Genetics
No known genetic pattern, although studies suggest up to 88% display HER2/neu overexpression (2)[B]

Risk Factors

  • Same risk factors apply as for noninherited breast cancers.
  • Female gender
  • Age >40 years
  • Previous breast cancer
  • Benign breast disease (atypical ductal/lobular hyperplasia, fibroadenoma, sclerosing adenosis, intraductal papilloma)
  • First-degree relative with history of breast cancer
  • Caucasian
  • Menarche <12 years of age
  • Menopause >50 years of age
  • Nulliparity or first child after age 34 years
  • History of ionizing radiation exposure
  • History of alcohol abuse
  • Hormone replacement
  • Excess weight gain

Commonly Associated Conditions

  • Largest study, using surveillance, epidemiology, and end results (SEER) data representing 1,763 women with confirmed Paget disease, reports an underlying in situ or invasive breast cancer in 87% of patients, although there is often no associated breast mass or mammographic abnormality (5).
  • The underlying carcinomas are multifocal/multicentric in 32–41% of patients (2)[B].

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