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  • • Infiltrating ductal carcinoma involving the nipple epithelium

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Epidemiology

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  • • 1% of all breast cancers

    • No age group predilection

    • Changes may be limited to the nipple, extend to the areola, or to the skin around the areola

    • 50-60% have a palpable tumor

    • If lesion is confined to nipple only, axillary metastases present in only 5% of patients

    • Paget disease of the breast has been associated with breast carcinoma developing in males who had Klinefelter syndrome

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Symptoms and Signs

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  • • Burning and pruritus of the nipple

    • Superficial erosion or ulceration of the nipple

    • Serous or bloody nipple discharge

    • Nipple retraction

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Imaging Findings

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  • • Mammography may show thickening of the nipple, calcifications, or lesion anywhere in the breast

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Rule Out

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  • • Inflammatory breast carcinoma

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  • • Complete history and physical exam

    • Bilateral mammogram

    • Biopsy of the nipple erosion

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  • • Multimodality treatment is the same as for carcinoma of the female breast

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Surgery

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Indications

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  • • May consider excision of nipple-areola complex alone if no palpable tumor and no extensive disease visualized on mammogram

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Treatment Monitoring

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  • • Self breast exams

    • Semiannual clinical breast exam

    • Annual mammogram

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Complications

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  • • Edema of the arm

    • Metastatic spread

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Prognosis

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  • • Disease is manifestation of mammary carcinoma, and thus prognosis is determined by extent of associated carcinoma

    • No underlying mass and treated by modified radical mastectomy, 10-year survival is 82-100%

    • Palpable invasive tumor, but node negative, treated with modified radical mastectomy, 10-year survival is 70%

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References

Chen CY et al: Paget disease of the breast: changing patterns of incidence, clinical presentation, and treatment in the U.S. Cancer 2006;107:1448.  [PubMed: 16933329]

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Practice Guidelines

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