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Mammary and extramammary Paget’s disease are intra-epidermal adenocarcinomas associated with the presence of large cells with a voluminous nucleus, known as Paget cells, with immunohistochemical features of glandular cells. They are rare diseases and though the clinical, histologic and immunohistochemical features are well defined, the etiology is still uncertain. Their prognosis is closely related to their association with an underlying adenocarcomina. However, while mammary Paget’s disease (MPD) is almost always associated with an underlying adenocarcinoma and therefore has an unfavorable prognosis, this association is much less frequent in extramammary Paget’s disease which more often occurs in isolation.
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