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Difference between revisions of "Extramammary Paget disease"
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Extramammary Paget disease (view source)
Revision as of 17:15, 20 December 2016
, 17:15, 20 December 2016no edit summary
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==General== | ==General== | ||
*Two types | *Two types | ||
**Primary Extramammary Paget disease - | **Primary Extramammary Paget disease - thought to be an intraepidermal carcinoma arising from cutaneous apocrine glands | ||
***Arises in apocrine rich areas - usually the vulva but also the groin, inguinal area, perineum, penis<ref name=pmid19126202>{{Cite journal | last1 = Ekwueme | first1 = KC. | last2 = Zakhour | first2 = HD. | last3 = Parr | first3 = NJ. | title = Extramammary Paget's disease of the penis: a case report and review of the literature. | journal = J Med Case Reports | volume = 3 | issue = | pages = 4 | month = | year = 2009 | doi = 10.1186/1752-1947-3-4 | PMID = 19126202 }}</ref> or [[scrotum]] | ***Arises in apocrine rich areas - usually the vulva but also the groin, inguinal area, perineum, penis<ref name=pmid19126202>{{Cite journal | last1 = Ekwueme | first1 = KC. | last2 = Zakhour | first2 = HD. | last3 = Parr | first3 = NJ. | title = Extramammary Paget's disease of the penis: a case report and review of the literature. | journal = J Med Case Reports | volume = 3 | issue = | pages = 4 | month = | year = 2009 | doi = 10.1186/1752-1947-3-4 | PMID = 19126202 }}</ref> or [[scrotum]] <ref name=pmid24349803>{{Cite journal | last1 = Guerra | first1 = R. | last2 = Misra | first2 = S. | title = Management of Extramammary Paget's Disease: A Case Report and Review of the Literature. | journal = Case Rep Dermatol Med | volume = 2013 | issue = | pages = 436390 | month = | year = 2013 | doi = 10.1155/2013/436390 | PMID = 24349803 }}</ref> and rarely axilla or eye. | ||
***Usually entirely intraepidermal but may be associated with an underlying apocrine gland carcinoma (in contrast to mammary Paget disease which is usually associated with underlying mammary carcinoma). | ***Usually entirely intraepidermal but may rarely be associated with an underlying apocrine gland carcinoma (in contrast to mammary Paget disease which is usually associated with underlying mammary carcinoma). | ||
**Secondary Extramammary Paget disease - intraepidermal spread from a distant | **Secondary Extramammary Paget disease - intraepidermal spread from a distant tumour | ||
***Usually of urothelial or colorectal origin. | ***Usually of urothelial or colorectal origin. | ||
***Arises in the perineal areas near these organs | ***Arises in the perineal areas near these organs. | ||
Clinical: | Clinical: | ||
*Pruritis. | *Pruritis. | ||
*R/O VIN | *R/O VIN. | ||
*R/O vulvitis | *R/O vulvitis. | ||
==Gross== | ==Gross== | ||
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Image:Anus PagetsDisease-2 PA.JPG|Anus Pagets Disease - Notice that the neoplastic cells ride above the normal basal cells (SKB) | Image:Anus PagetsDisease-2 PA.JPG|Anus Pagets Disease - Notice that the neoplastic cells ride above the normal basal cells (SKB) | ||
Image:Anus PagetsDisease-3 PA.JPG|Anus Pagets Disease - (SKB) | Image:Anus PagetsDisease-3 PA.JPG|Anus Pagets Disease - (SKB) | ||
Image:Penis PagetDisease MP CTR.jpg|Penis Paget Disease - medium power - (SKB) | |||
Image:Penis PagetDisease HP CTR.jpg|Penis Paget Disease - high power - (SKB) | |||
Image:Vulva PagetDisease LP Mater.JPG|Vulva Paget Disease - low power - (SKB) | |||
Image:Vulva PagetDisease MP3 Mater.JPG|Vulva Paget Disease - medium power - (SKB) | |||
Image:Vulva PagetDisease MP Mater.JPG|Vulva Paget Disease - medium power - (SKB) | |||
</gallery> | </gallery> | ||
===DDx=== | ===DDx=== | ||
*[[Malignant melanoma in situ]] - atypical cells are mostly basilar with upward migration. | *[[Malignant melanoma in situ]] - atypical cells are mostly basilar with upward migration. | ||
*[[Bowen disease]] | <gallery> | ||
*Pagetoid vulvar intraepithelial neoplasia - Pagetoid areas often merge into areas of typical VIN | Image:Skin MMIS HP 14PY***.jpg|Melanoma in situ - notice that the atypical cells are largely down at the basal layer with some upward migration. (SKB) | ||
* | </gallery> | ||
*Squamous cell carcinoma in situ "[[Bowen disease]]" | |||
**Pagetoid squamous cell carcinoma in situ can look identical to extramammary Paget disease | |||
**But should have some areas of typical SCCIS with full thickness cohesive atypia | |||
<gallery> | |||
Image:Skin SCCIS PagetoidandConventional 15BR30103.jpg|An SCCIS from sundamaged skin. Pagetoid SCCIS on the left, conventional SCCIS on the right. (SKB) | |||
</gallery> | |||
*Pagetoid vulvar/anal/penil HPV associated intraepithelial neoplasia - Pagetoid areas often merge into areas of typical VIN | |||
*'Paget disease' associated with an underlying local carcinoma - apocrine, Bartholin's duct etc (rare). | |||
*Notice that mammary Paget disease and Toker cell hyperplasia are not part of this DDX (wrong | *Notice that mammary Paget disease and Toker cell hyperplasia are not part of this DDX (wrong location) except possibly in the rare case of EMPD arising in the axilla where mammary Paget disease arising from a ectopic nipple or ectopic breast tissue may be a consideration. | ||
*Notice that EMPD is not really part of the DDX of pagetoid cutaneous squamous cell carcinoma arising on typical sun damaged skin (arm, cheek, leg) - if it's not from an apocrine rich area (and these are usually in the shade) - it's not EMPD. | *Notice that EMPD is not really part of the DDX of pagetoid cutaneous squamous cell carcinoma arising on typical sun damaged skin (arm, cheek, leg) - if it's not from an apocrine rich area (and these are usually in the shade) - it's not EMPD. | ||
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*Is the lesion adenocarcinoma or squamous cell carcinoma? | *Is the lesion adenocarcinoma or squamous cell carcinoma? | ||
**Low molecular weight (CK7, cam5.2) or high molecular weight keratins (34BE12, CK5/6)? | **Low molecular weight (CK7, cam5.2) or high molecular weight keratins (34BE12, CK5/6)? | ||
***Don't rely entirely on the cytokeratin profile | |||
***Pagetoid squamous cell carcinoma in situ can lose HMW keratins and produce a LMW profile. | |||
**Adenocarcinoma markers? - CEA, BerEP4 | **Adenocarcinoma markers? - CEA, BerEP4 | ||
**Nuclear differentiation markers? - p63 (squamous) vs GATA3 (adnexal) | **Nuclear differentiation markers? - p63 or [[p40]] (squamous) vs GATA3 (adnexal) | ||
*Is the lesion primary or secondary? | *Is the lesion primary or secondary? | ||
**Secondary extramammary Paget disease may be CK20 positive (urothelial or rectal) | **Secondary extramammary Paget disease may be CK20 positive (urothelial or rectal) | ||
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*CK7 and CK20 - where does it come from? | *CK7 and CK20 - where does it come from? | ||
*S100 and Melan A - exclude melanoma in situ | *S100 and Melan A - exclude melanoma in situ | ||
*Differentiation markers; GATA - apocrine and urothelial, p63 - squamous, CDX2 - colorectal | *Differentiation markers; GATA - apocrine and urothelial, p63 and p40 - squamous, CDX2 - colorectal | ||
*Notice urothelial origin Paget can show immunos patterns "transitional" between glandular and squamous epithelium | *Notice urothelial origin Paget can show immunos patterns "transitional" between glandular and squamous epithelium | ||
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<gallery> | <gallery> | ||
Image:Skin Vulva PagetDisease MP 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. | Image:Skin Vulva PagetDisease MP 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. (SKB) | ||
Image:Skin Vulva PagetDisease CK7 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. CK7 (SKB) | Image:Skin Vulva PagetDisease CK7 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. CK7 (SKB) | ||
Image:Skin Vulva PagetDisease CK20 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. CK20(SKB) | Image:Skin Vulva PagetDisease CK20 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. CK20 (SKB) | ||
Image:Skin Vulva PagetDisease 34BE12 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. 34BE12 the benign keratinocytes are positive (SKB) | Image:Skin Vulva PagetDisease 34BE12 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. 34BE12 the benign keratinocytes are positive (SKB) | ||
Image:Skin Vulva PagetDisease CEA 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. CEA(SKB) | Image:Skin Vulva PagetDisease CEA 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. CEA (SKB) | ||
Image:Skin Vulva PagetDisease GATA 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. GATA3(SKB) | Image:Skin Vulva PagetDisease GATA 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. GATA3 (SKB) | ||
Image:Skin Vulva PagetDisease p63 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. p63 - the benign keratinocytes are positive (SKB) | Image:Skin Vulva PagetDisease p63 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease. p63 - the benign keratinocytes are positive (SKB) | ||
</gallery> | </gallery> | ||