Anogenital Mammary Type Sclerosing Adenosis – Two CaseReports and the Review of Literature

Overview

Objective:
To describe a rare case of mammary type sclerosing adenosis arising in anogenital,mammary-like adnexal glands. Differential diagnosis is discussed and detailed review of literatureis presented.Design: Two case reports.Setting: Private Biopsy Lab s.r.o. and Šikl‘s Department of Pathology, Charles University andFaculty Hospital, Pilsen.Methods: Included are two cases from Breast Registry, Biopsy Lab s.r.o. and from personal consultingregistry of Prof. Michal, Šikl‘s Department of Pathology, Pilsen. Detailed morphology of thelesion is described and documented by pictures. The presence of myoepithelial cell is demonstratedby immunohistochemistry using streptavidin-bioptin detection system (LSAB+, Dako) andthree conventional myoepithelial markers (actin S, calponin and CD10). Expression of estrogenand progesteron receptors and apocrine antigen GCDFP-15 were studied too.Results: There were two women at the age of 54 and 60-years with lesion located on vulva andanus; the maximum tumor diameter was 7 and 10 mm, respectively. The histological picture wasvirtually the same in both cases displaying under hyperplastic epidermis small glands proliferationset in sclerotic fi brous stroma. In spite of relatively sharp circumscription, the chaotic andirregular distribution of the glands imparted the invasive image of the lesion. The luminal cellsshowed no cytological and nuclear atypia and visible was only minimal regular mitotic activity.The signs of luminal, decapitation-type, apocrine secretion were dispersal seen throughout thelesion. By immunohistochemistry the myoepithelial cells regularly and systematically decoratedperiphery of the glands and all the composition was similar to sclerosing adenosis in the breast including massive hormonal receptors expression. In the periphery of one lesion an insolated,single, typical anogenital gland was found. Features of conventional papillary hidradenoma werenot seen.Conclusion: Mammary – type anogenital adnexal glands are regularly present in vulvar and perianalskin; these are considered there as a substrate for analogous lesion occurring in the breast(lactation change, papilloma and fi broadenoma, but also some types of carcinomas including primaryextrammary Paget‘s disease). In these physiological structures there are combined featuresof both eccrine and apocrine adnexal glands creating a primitive caricature of basic tubulolobularunit in the breast. Their existence dispel historical dogma about running of the embryonalmilk lines or mammary ridges and explains the fi ndings of varied scale of neoplasms differingfrom well known lesion derived from conventional adnexal glands.

Key words:
anogenital adnexal glands, mammary-like (mammary-type) sweat glands, vulva, anus,sclerosing adenosis