Extramammary Paget's disease causes: Difference between revisions

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Latest revision as of 13:01, 10 April 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

The direct cause of extramammary Paget's disease has not been identified.

Causes

The direct cause of Extramammary Paget's disease (EMPD) is unknown.

References

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 ==Overview==
-The cause of extramammary Paget's disease has not been identified.
+The direct cause of extramammary Paget's disease has not been identified.
 ==Causes==
-The cause of  Extramammary Paget's disease (EMPD) is unknown.
+*The direct cause of  Extramammary Paget's disease (EMPD) is unknown.
-The histogenesis of EMPD remains controversial. An apocrine origin is suggested by its predilection for apocrine gland-bearing sites and its staining with markers of apocrine differentiation such as CEA and GCDFP. However, it can occur in apocrine poor (ectopic) sites and the abovementioned markers are not specific for apocrine glands (e.g. GCDFP has been found in eccrine glands). Other suggested origins include eccrine glands, ‘mammary-like’ glands, pluripotent keratinocyte stem cells or direct spread from an underlying adenocarcinoma.
-At present, the most popular theory is that EMPD may arise either as a primary intraepidermal neoplasm of the epidermis (primary EMPD) or less commonly as a result of spread from an underlying internal malignancy (secondary EMPD).24 In primary EMPD, Paget's cells probably originate from intraepidermal portions of sweat/apocrine glands or from primitive basal cells within the epidermis. Primary Paget's disease may progress from in situ intraepidermal neoplasia to dermally invasive adenocarcinoma, which may in turn metastasise to local lymph nodes and distant sites.8 Paget's disease may also arise following epidermotropic spread of malignant cells from an underlying neoplasm in a dermal adnexal gland or a local internal organ with contiguous epithelium (secondary EMPD).25
-Some investigators believe that EMPD may be associated with a generalised tendency to neoplasia, especially adenocarcinoma, as there is a high rate of synchronous and metachronous cancers in these patients.8 A spatial and temporal association of EMPD of the vulva and high grade vulval intraepithelial neoplasia (VIN) has been reported by some authors, although this is extremely rare.
 ==References==
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Extramammary Paget's disease causes: Difference between revisions

Latest revision as of 13:01, 10 April 2019

Overview

Causes

References

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