Paget Disease of the Breast
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- 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
- 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)
<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
- Transformation theory (not favored)
- 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
- 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].