The histogenesis of mammary and extramammary Paget's disease

Abstract

The histogenesis of mammary and extramammary Paget's disease has been studied by immunohistochemical staining of paraffin-embedded tissue using a panel of epithelial cell markers, which react with secretory or ductal epithelium, but not stratified epithelium. These markers included a monoclonal antibody E29 to epithelial membrane antigen EMA, the cytokeratin marker CAM 5.2 and three new monoclonal antibodies raised to human milk fat globule membrane (LICR-LON-TW19 and H.10.A) and a human bladder cell cancer line (3.77). The findings demonstrate that both mammary and extramammary Paget's disease are of epithelial cell origin and share antigens expressed by simple epithelia. Some antigens, such as EMA and low molecular weight cytokeratins are consistently present in both diseases, whereas other antigens, identified by H.10.A and TW19 are found more frequently in cases of extramammary Paget's disease. This panel of monoclonal antibodies also proved useful in distinguishing Paget's disease from pagetoid melanoma and clear cell Bowen's disease.

The pagetoid variant of bladder urothelial carcinoma in situ

“…Immunostaining against EMA and LEA 135 stained rare pagetoid cells (<5%). We failed to demonstrate any pagetoid cell positive with Male 69 Secondary Cystectomy multifocal Cystectomy G3/pT3a NED (24) antibodies against CEA and HMWCK (Fig. 1).…”

“…Cells comprising EMPD may not be associated with carcinomas in adjacent sites [24]. They are generally considered to differentiate toward glandular rather than squamous epithelium, although detailed studies documenting this hypothesis have been performed in only a few instances [24]. The immunohistochemical profile of EMPD has been recently addressed in a number of publications [2,10,12,15,18,19,22,25,28,29], and an immunohistochemical profile characterized by CK7+ and CK20-has been considered specific of EMPD [18]; meanwhile, other regional internal malignancies with pagetoid growth, such as pagetoid urothelial CIS, are CK7+ and CK20+ [18].…”

“…Most authors agreed that pagetoid changes should not be labeled as urothelial dysplasia, and cases misdiagnosed as dysplasia that remain untreated are at higher risk for recurrence and progression [3, 8,26] Pagetoid changes at extramammary sites (EMPD) are rare alterations most often recorded in the anogenital region [1,3,4,7,9,11,21,23,24,26,27]. Cells comprising EMPD may not be associated with carcinomas in adjacent sites [24]. They are generally considered to differentiate toward glandular rather than squamous epithelium, although detailed studies documenting this hypothesis have been performed in only a few instances [24].…”

“…In addition, its recognition in a small biopsy specimen can alert the pathologist to the presence of a conventional urothelial CIS elsewhere in the bladder, even in cases with extensive bladder denudation in which the lesion may be present in normal-looking urothelium [5]. Some cases of pagetoid CIS may extend to the urethra, ureter, and beyond to the external genitalia where they represent an important differential diagnostic problem, given that primary extramammary Paget disease (EMPD) of the external genitalia may also extend to the bladder [1,3,4,5,7,9,11,21,23,24,26,27]. An appropriate approach to differential diagnosis in this case, may need immunohistochemical analysis in addition to a complete patient's work-up.…”

Axillary apocrine carcinoma with benign apocrine tumours: a case report involving a pathological and immunohistochemical study and review of the literature

“…Staining for EMA is consistently positive in both extramammary and mammary Paget's disease. 15 Some cases of apocrine adenocarcinoma were positive for EMA, 5 16 but the results were not described in detail. During the neoplastic transformation of breast cancer cells, the morphological expression of EMA may change.…”