Cases reported "Paget's Disease, Mammary"

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1/8. Male Paget's disease of the breast.

    BACKGROUND: Paget's disease is an extremely rare condition of the male breast that presents as an eczematous change of the nipple and areola. It is almost always associated with an underlying intraductal carcinoma. OBJECTIVE: A case of Paget's disease of the male breast with extension into adnexal structures is reported. MATERIALS AND methods: A medline search for cases of Paget's disease of the male breast was performed and the cases were reviewed. The following antibodies were used in immunohistochemical staining: Rabbit Anti-Human c-erbB-2 Oncoprotein, monoclonal Mouse Anti-Human Estrogen Receptor (IgG1, kappa), and monoclonal Mouse Anti-Human progesterone Receptor (Clone PgR 636). RESULTS: This is only the 43rd histologically proven case of Paget's disease of the male breast in the world literature and, to our knowledge, the first to document extension of Paget's cells into adnexal structures, including eccrine glands. CONCLUSION: Mammary Paget's disease is a rare phenomenon among men.
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2/8. Bilateral paget disease of the nipple associated with lobular carcinoma in situ.

    We report synchronous bilateral Paget disease derived from lobular carcinoma in situ in a 53-year-old woman who underwent bilateral mastectomy. The epidermis of both nipples contained small cells with a moderate amount of pale-staining cytoplasm. The nuclei had fine chromatin and identifiable nucleoli. The cells were strongly immunoreactive with cytokeratin 7 and displayed nuclear estrogen receptor reactivity. The underlying mammary gland showed involvement by lobular carcinoma in situ with pagetoid spread into lactiferous ducts, which was confirmed by lack of immunoreactivity for E-cadherin.
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3/8. Treatment of mammary and extramammary Paget's skin disease with topical imiquimod.

    BACKGROUND: Paget's disease is an uncommon epidermal cancer, affecting all skin regions wherever apocrine glands are present. It is divided into extramammary (EMPD) and mammary Paget's disease (MPD). It can be confined to the epithelium or may be associated with an underlying adenocarcinoma. The diagnosis is confirmed by skin biopsy and the treatment depends on characteristics of the underlying cancer. Surgery is the first-line treatment. Imiquimod, a topical immunomodulator, approved its efficiency in several skin neoplasms and has been shown as a safe treatment for EMPD. However, it has never been reported for the treatment of MPD. OBSERVATIONS: We report on two cases of EMPD and MPD successfully treated with imiquimod 5% cream. CONCLUSION: This non-surgical method may be considered as a primary treatment when Paget's disease is not associated with an underlying malignancy. The good prognosis with a long-term survival, the anatomic localization and the poor general condition of elderly people may favor imiquimod as an alternative treatment. On the other hand, it will reduce the extent of excision when it anticipates surgery.
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4/8. Development of carcinoma of the breast at the site of an implanted pacemaker in two patients.

    We report here on two female patients who had permanent pacemakers implanted in their chests and who developed carcinoma of the breast subsequently. An association is suspected between the breast cancer and the pacemaker, which is implanted in an area which borders with the mammary gland or is even right within it. This suspicion led us lately to change in female patients the site of the subcutaneous pocket for the implantation of the pacemaker to a position higher in the chest than before. Moreover, we advocate frequent breast examinations in all female patients with implanted pacemakers.
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5/8. Cytological diagnosis of Paget's disease of the nipple by scrape smears: a report of five cases.

    Five cases of mammary Paget's disease were diagnosed by nipple scrape cytology. The neoplastic material obtained was abundant in two cases, moderate in one, but in two, only a few cell aggregates feature in three cases and this, together with dense tumour cell cytoplasm and the background of keratinous debris, may impart a squamous-like appearance; however, three-dimensional cell aggregates, papillary-like groups, acinar-like collections, and some cells with vacuolated cytoplasm and eccentric nuclei allowed glandular differentiation to be inferred in three cases. In four cases a combination of clinical evaluation, and cytological findings of malignant cells compatible with Paget's disease was used so that mastectomy could proceed, and in one case there was frozen section confirmation of an underlying invasive carcinoma. In the single case in which it was performed, there was positive immunoperoxidase staining for c-erb B2 oncoprotein. Demonstration of this protein, CEA, or mucin, helps distinguish Paget's disease from melanoma or squamous cell carcinoma in-situ.
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6/8. Mammary Paget's disease confined to the areola and associated with multifocal Toker cell hyperplasia.

    A hitherto unreported variant of mammary Paget's disease (MPD) limited to the areola, leaving the nipple unaffected, has been analyzed by serial sectioning of the whole areola and nipple. This otherwise characteristic MPD proved to be confined to the epidermis. There was no underlying carcinoma. This MPD was associated with a multifocal presence of monomorphic but otherwise similar cells in small collections surrounding the ostia of areolar mammary glands in the clinically unaffected area. This condition was interpreted as hyperplasia of mammary gland-related cells also found in normal nipples (so-called Toker cells). The observations hint at a possible derivation of some cases of mammary and extramammary Paget's disease from such Toker cells.
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7/8. Expression of desmogleins in Paget cells of mammary and extramammary Paget's disease.

    Sagebiel (1969) electronmicroscopically observed that desmosomes are present between adjacent Paget cells, as well as between Paget cells and adjacent keratinizing epidermal cells. desmosomes contain the proteins desmoglein (Dsg) and desmocollin as their transmembrane components, and Dsg has three isotypes. Among the three isotypes of Dsg, Dsg1 and Dsg3 are autoantigens for pemphigus foliaceus and pemphigus vulgaris (PV), respectively. We examined the expression of Dsgs in Paget cells of mammary and extramammary Paget's disease. Skin samples were obtained from one patient with mammary Paget's disease, and from 2 with extramammary Paget's disease. One part of the samples was cut into small pieces and epidermal sheets were separated with dispase, then treated with a mixture of EDTA and trypsin. The resulting cell suspensions were cultured in low Ca medium, then the cells were incubated in high Ca medium. Paget cells identified with anti-epithelial membrane antigen (EMA) antibody were positive for serum from a PV patient which was confirmed to react with both Dsg1 and Dsg3. However, the intensity of fluorescence of Paget cells was weaker than that of EMA-negative keratinocytes. In the cryostat sections, Paget cells identified with anti-EMA antibody showed the same staining pattern as cultured cells in high Ca medium. The data presented in this study confirm that Paget cells express Dsgs, and are consistent with the electronmicroscopical data by Sagebiel (1969). However, our data do not support the hypothesis that Paget cells are of keratinocyte origin, because sweat ducts or sweat glands could be positive for sera from PV patients. It is necessary to confirm whether or not sweat ducts or sweat glands express Dsgs, because sera from PV patients exhibit high background in the dermis.
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8/8. Paget's disease versus Toker cell hyperplasia in a supernumerary nipple.

    We report the second case of mammary Paget's disease arising in a supernumerary nipple of a 29-year-old woman. The epithelium of the nipple was infiltrated by large cells with abundant and pale-staining cytoplasm. The nuclei had a vesicular chromatin pattern and identifiable nucleoli. The cells were strongly immunoreactive with KL1, CEA and EMA, but did not show reactivity with PS100, HMB45, or erb-B2. The pathogenesis of Paget cells is unclear. In our case, the lesion showed nearly all the clinical, histological and histochemical characteristics of Paget's disease, though without involvement of mammary gland epithelium and underlying carcinoma. The possibility of an intraepidermal origin, either by transformation from epidermal keratinocytes or by derivation from intraepidermal precursor cells, has to be considered. The differential diagnosis against Toker cell hyperplasia is also discussed.
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