Definition
Extramammary Paget disease (EMPD) can be classified into primary (EMPD in sensu stricto) and secondary variants, with the latter representing secondary involvement from an underlying carcinoma originating most commonly in the lower gastrointestinal or urinary tract. Primary EMPD is considered an analogue of mammary Paget disease (MPD). Both are characterized histopathologically by a characteristic pattern of an intraepithelial proliferation of carcinoma cells. In MPD, intraepidermal Paget cells are assumed to be the result of upward migration and epidermotropism from the underlying carcinoma which is almost always a ductal carcinoma, either in situ or invasive, or, rarely, lobular carcinoma. In extremely rare cases lacking an associated neoplasm, Toker cells of the nipple are assumed to be the source of the lesion. In contrast to MPD, the histogenesis of primary EMPD is less clear. Skin adnexae (eccrine or apocrine glands), anogenital mammary-like glands, vulval Toker cells...
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References and Further Reading
Belousova, I. E., Kazakov, D. V., Michal, M., & Suster, S. (2006). Vulvar toker cells: The long-awaited missing link: a proposal for an origin-based histogenetic classification of extramammary paget disease. American Journal of Dermatopathology, 28(1), 84–86.
Feuer, G. A., Shevchuk, M., & Calanog, A. (1990). Vulvar Paget’s disease: The need to exclude an invasive lesion. Gynecologic Oncology, 38(1), 81–89.
Kazakov, D. V., Spagnolo, D. V., Kacerovska, D., & Michal, M. (2011). Lesions of anogenital mammary-like glands: An update. Advances in Anatomic Pathology, 18(1), 1–28.
Lee, M. W., Jee, K. J., Gong, G. Y., Choi, J. H., Moon, K. C., & Koh, J. K. (2005). Comparative genomic hybridization in extramammary Paget’s disease. British Journal of Dermatology, 153(2), 290–294.
van der Putte, S. C. (1991). Anogenital “sweat” glands. Histology and pathology of a gland that may mimic mammary glands. American Journal of Dermatopathology, 13(6), 557–567.
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Kazakov, D.V. (2016). Extramammary Paget’s Disease. In: Massi, D. (eds) Dermatopathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-30006-1_421
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DOI: https://doi.org/10.1007/978-3-319-30006-1_421
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