Extramammary Paget’s disease of the perianal area: a case report and the summarizing review of last 5-year literature.

Authors

DOI:

https://doi.org/10.26641/1997-9665.2019.3.156-161

Keywords:

Extramammary Paget’s disease, primary, secondary type, perianal region, Toker cells, immunohistochemical study

Abstract

Background. Extramammary Paget’s disease is one of the rarest malignant skin tumors which are mostly apocrine. However, there were described some cases in non-apocrine bearing glands areas. The perianal region, as part of the anogenital site, is the place where extramammary Paget’s disease could manifest. It is used to classified this disease into 2 categories: primary and secondary, where the main differentiating feature is the absence or presence of underlying carcinoma relatively. Due to extramammary Paget’s disease rarity all around the world publishing information about any case could help to evaluate the situation in all. Objective. To report a rare case of the extramammary Paget’s disease and analyze one-case-publications during last 5 years on PubMed and assessed contributions of age, sex, and localizations. Methods. While making a diagnosis histological, immunohistochemical studies and statistical methods were used. Results. The first case of primary noninvasive extramammary Paget’s disease was diagnosed in the immunohistochemical laboratory of the Medical Diagnostic Center of the Medical Academy and described in this article. A 64-year-old Ukranian man suffered within 1 year from big erythematous pruritus unhealed lesion between his buttocks and came to our laboratory to verify suspicious malignant melanoma what was assumed while previous histological study. According to immunohistochemical study, this tumor had positive status with cytokeratin 7, HER-2/Neu, EGFR and p16, while cytokeratin HMW, S100, HMB-45, EBV were negative. Such immunophenotype might point to some ways of the pathogenesis of this problem. The theory about involved Toker cell and preceded Toker cell hyperplasia had both advantages and drawbacks, what we showed with the presented example. There were already published 49 cases with middle age 67,68±1,64; median 67 years, m:f ratio 1,58:1 and utterly predominant location – anogenital region. Conclusion. Received data have mostly corresponded to published information previously.Both clinical studies and immunophenotype gave a possibility to make an accurate diagnose.

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Shponka, I. S., Hrytsenko, P. O., & Skoryk, V. R. (2019). Extramammary Paget’s disease of the perianal area: a case report and the summarizing review of last 5-year literature. Морфологія / Morphologia / Morfologìâ, 13(3), 156–161. https://doi.org/10.26641/1997-9665.2019.3.156-161

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Vol. 13 No. 3 (2019)

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