Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Extramammary Paget Disease

  • Chiara Trambaiolo Antonelli
  • Francesca PortelliEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_4868-1



Paget disease is a form of intraepithelial adenocarcinoma with primary involvement of external genitalia epidermis. It is classified as primary or secondary. Primary extramammary Paget disease can be restricted to the epidermis and derive from intraepidermal sweat duct or can be related to an underlying sweat gland adenocarcinoma, more often with apocrine differentiation. Dermal invasion can also be observed. Secondary extramammary Paget disease represents an epidermotropic metastasis from an underlying visceral malignancy, especially urogenital and colorectal carcinoma, and it is far less common.

Clinical Features

  • Incidence

    Extramammary Paget disease represents 21% of primary scrotal cancers in the USA with tendency to increase (3.2% per year). It has been observed a higher incidence rate among Asian population. Diagnosis increment likely represents recognition of disease at an earlier stage (Herrel et al. 2015).

  • Age

    Paget disease commonly involves...

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References and Further Reading

  1. Amin, A., Griffith, C., & Chaux, A. (2014). Penile intraepithelial neoplasia with pagetoid features: Report of an unusual variant mimicking Paget disease. Human Pathology, 45, 889–892.CrossRefGoogle Scholar
  2. Herrel, L. A., Weiss, A. D., Goodman, M., Johnson, T. V., Osunkoya, A. O., Delman, K. A., & Master, V. A. (2015). Extramammary Paget’s disease in males: Survival outcomes in 495 patients. Annals of Surgical Oncology, 22, 1625–1630.CrossRefGoogle Scholar
  3. Kang, Z., Xu, F., Zhu, Y., Fu, P., Zhang, Q., Hu, T., Li, X., Zhang, Q., Wu, Z., Zhang, X., Wang, H., Xu, J., Fang, Z., & Guan, M. (2016). Genetic analysis of mismatch repair genes alterations in Extramammary Paget disease. American Journal of Surgical Pathology, 40, 1517–1525.CrossRefGoogle Scholar
  4. Zhang, N., Gong, K., Zhang, X., Yang, Y., & Na, Y. (2010). Extramammary Paget’s disease of scrotum – Report of 25 cases and literature review. Urologic Oncology, 28, 28–33.CrossRefGoogle Scholar
  5. Zhao, M., Zhou, L., Sun, L., Song, Y., Guo, Y., Zhang, X., Zhao, F., Wang, P., Yue, J., Niu, D., Li, Z., Huang, X., Kang, Q., Jia, L., Lai, J., & Cao, D. (2017). GATA3 is a sensitive marker for primary genital extramammary Paget disease: An immunohistochemical study of 72 cases with comparison to gross cystic disease fluid protein 15. Diagnostic Pathology, 12, 51.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Chiara Trambaiolo Antonelli
    • 1
  • Francesca Portelli
    • 2
    Email author
  1. 1.Departments of Anatomical Pathology and of Oncology, Ospedale Policlinico San MartinoUniversity of GenoaGenoaItaly
  2. 2.Section of Anatomical Pathology, Department of Health SciencesAzienda Ospedaliero-Universitaria Careggi, University of FlorenceFlorenceItaly