Paget Disease of the Nipple
Mammary Paget disease; Paget disease of the breast; Paget disease of the nipple and areola
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).
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.
PD most commonly affects...
References and Further Reading
- Anderson, J. M., Ariga, R., Govil, H., Bloom, K. J., Francescatti, D., Reddy, V. B., et al. (2003). Assessment of Her-2/Neu status by immunohistochemistry and fluorescence in situ hybridization in mammary Paget disease and underlying carcinoma. Applied Immunohistochemistry and Molecular Morphology, 11, 120–124.PubMedCrossRefGoogle Scholar
- Jacobs, T. W. Clear cells of Toker in the Nipple Epidermis. USCAP 2010 Annual Meeting, Specialty Conference, Breast Pathology, Case 2. http://uscapknowledgehub.org/newindex.htm?99th/specbreah2.htm
- Mai, R., Zhou, S., Zhou, S., Zhong, W., Hong, L., Wang, Y., Lu, S., Pan, J., Huang, Y., Su, M., Crawford, R., Zhou, Y., & Zhang, G. (2018). Transcriptome analyses reveal FOXA1 dysregulation in mammary and extramammary Paget’s disease. Human Pathology, 77, 152–158.PubMedCrossRefPubMedCentralGoogle Scholar
- Sanders, M. A. G. Paget disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/breastmalignantpaget.html. Accessed 13 Jan 2019.
- Shousha, S., Eusebi, V., & Lester, S. (2012). Paget disease of the nipple. In S. R. Lakhani, I. O. Ellis, S. J. Schnitt, P. H. Tan, & M. J. van de Vijver (Eds.), WHO classification of tumours of the breast (4th ed., pp. 152–153). Lyon: IARC.Google Scholar
- Wachter, D. L., Wachter, P. W., Fasching, P. A., Beckmann, M. W., Hack, C. C., Riener, M. O., Hartmann, A., & Strehl, J. D. (2019). Characterization of molecular subtypes of Paget disease of the breast using immunohistochemistry and in situ hybridization. Archives of Pathology and Laboratory Medicine, 143, 206–211.PubMedCrossRefPubMedCentralGoogle Scholar
- Zhang, G., Zhou, S., Zhong, W., Hong, L., Wang, Y., Lu, S., Pan, J., Huang, Y., Su, M., Crawford, R., Zhou, Y., & Mai, R. (2019). Whole-exome sequencing reveals frequent mutations in chromatin remodeling genes in mammary and Extramammary Paget’s diseases. Journal of Investigative Dermatology, 139, 789–795.PubMedCrossRefPubMedCentralGoogle Scholar