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Paget’s disease of the nipple

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Abstract

Paget’s disease of the breast is a disorder of the nipple–areola complex that, while rare, is often associated with an underlying carcinoma. It is characterized by eczematoid changes of the nipple. Two theories have been proposed to explain the pathogenesis of Paget’s disease. The Epidermotropic, which is the most accepted theory, suggests that Paget’s cells originate from ductal cancer cells that had migrated from the underlying breast parenchyma. It is supported by the predominance of breast cancer markers found in Paget’s disease. This article provides an overview of Paget’s disease of the breast with special attention to immunohistochemistry and raises the question of new therapeutic approaches.

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Abbreviations

PD:

Paget’s disease

EMPD:

Extra-mammary Paget’s disease

NAC:

Nipple–areolar complex

ER:

Estrogen receptor

PR:

Progesterone receptor

AR:

Androgen receptor

CK:

Cytokeratin

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Authors and Affiliations

  1. Department of Medicine, Division of Hematology/Oncology, University of Miami Miller School of Medicine, Miami, FL, 33136, USA

    Ana C. Sandoval-Leon, Katherine Drews-Elger & Marc E. Lippman

  2. Department of Pathology, University of Miami Miller School of Medicine, Miami, FL, 33136, USA

    Carmen R. Gomez-Fernandez

  3. Department of Radiology, University of Miami Miller School of Medicine, Miami, FL, 33136, USA

    Monica M. Yepes

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  1. Ana C. Sandoval-Leon
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  2. Katherine Drews-Elger
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  3. Carmen R. Gomez-Fernandez
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Correspondence to Katherine Drews-Elger.

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Ana C. Sandoval-Leon and Katherine Drews-Elger contributed equally to this work.

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Sandoval-Leon, A.C., Drews-Elger, K., Gomez-Fernandez, C.R. et al. Paget’s disease of the nipple. Breast Cancer Res Treat 141, 1–12 (2013). https://doi.org/10.1007/s10549-013-2661-4

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  • DOI: https://doi.org/10.1007/s10549-013-2661-4

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