Synonyms
Definition
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
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.
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.
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.
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.
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.
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Trambaiolo Antonelli, C., Portelli, F. (2019). Extramammary Paget Disease. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4868-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_4868-1
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