Breast Pathology

2020 Edition
| Editors: Anna Sapino, Janina Kulka

Paget Disease of the Nipple

  • Janina KulkaEmail author
  • Anna Sapino
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-62539-3_4725

Synonyms

Mammary Paget disease; Paget disease of the breast; Paget disease of the nipple and areola

Definition

Paget disease (PD) is characterized by the presence of malignant glandular epithelial cells (Paget cells) within the squamous epithelium of the nipple that may extend into the areola and adjacent skin (Shousha et al. 2012).

Clinical Features

  • Incidence

    PD was first described by the British surgeon Sir James Paget in 1874. It is a rare disease; its exact incidence in unknown. It is estimated to occur in 1–4% of all breast cancer patients. PD is associated with an underlying in situ or invasive breast carcinoma in 82–94% of cases (Wong et al. 2015). Wong et al. in their large American patient cohort described statistically significant decrease in the age-adjusted incidence of the disease from 2000 to 2011, mainly due to decreasing rates of PD with underlying carcinoma (Wong et al. 2015). PD without underlying malignant lesion is considered pTis.

  • Age

    PD most commonly affects...

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References and Further Reading

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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.2nd Department of PathologySemmelweis UniversityBudapestHungary
  2. 2.Unit of PathologyCandiolo Cancer Institute FPO-IRCCSCandioloItaly
  3. 3.Department of Medical SciencesUniversity of TurinTurinItaly