Difference between revisions of "Extramammary Paget disease"

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*Differentiation markers GATA - apocrine and urothelial; p63 - squamous, CDX2 - colorectal
*Differentiation markers GATA - apocrine and urothelial; p63 - squamous, CDX2 - colorectal


**Notice that a CK20 negative urothelial origin EMPD will show the same immunoprofile as a primary cutaneous EMPD.
**Notice urothelial origin Paget can show immunos patterns "transitional" between glandular and squamous epithelium
***A CK20 negative urothelial origin EMPD could show a similar immunoprofile as a primary cutaneous EMPD.
**Notice that you do not need to include markers for mammary Paget disease or Toker cells.
**Notice that you do not need to include markers for mammary Paget disease or Toker cells.
**Do not rely on CK7 alone as CK7 may be positive in pagetoid squamous cell carcinoma in situ or extramammary Paget disease <ref>{{Cite journal  | last1 = Raju | first1 = RR. | last2 = Goldblum | first2 = JR. | last3 = Hart | first3 = WR. | title = Pagetoid squamous cell carcinoma in situ (pagetoid Bowen's disease) of the external genitalia. | journal = Int J Gynecol Pathol | volume = 22 | issue = 2 | pages = 127-35 | month = Apr | year = 2003 | doi =  | PMID = 12649666 }}</ref>
**Do not rely on CK7 alone as CK7 may be positive in pagetoid squamous cell carcinoma in situ or extramammary Paget disease <ref>{{Cite journal  | last1 = Raju | first1 = RR. | last2 = Goldblum | first2 = JR. | last3 = Hart | first3 = WR. | title = Pagetoid squamous cell carcinoma in situ (pagetoid Bowen's disease) of the external genitalia. | journal = Int J Gynecol Pathol | volume = 22 | issue = 2 | pages = 127-35 | month = Apr | year = 2003 | doi =  | PMID = 12649666 }}</ref>
**p16 is not helpful in distinguishing between VIN and EMPD as may be positive in either. <ref>{{Cite journal  | last1 = Sah | first1 = SP. | last2 = McCluggage | first2 = WG. | title = Florid vulval Paget disease exhibiting p16 immunoreactivity and mimicking classic VIN. | journal = Int J Gynecol Pathol | volume = 32 | issue = 2 | pages = 221-7 | month = Mar | year = 2013 | doi = 10.1097/PGP.0b013e31825909f6 | PMID = 23370646 }}</ref>
**p16 is not helpful in distinguishing between VIN and EMPD as may be positive in either. Urothelial carcioma can also be positive for this marker <ref>{{Cite journal  | last1 = Sah | first1 = SP. | last2 = McCluggage | first2 = WG. | title = Florid vulval Paget disease exhibiting p16 immunoreactivity and mimicking classic VIN. | journal = Int J Gynecol Pathol | volume = 32 | issue = 2 | pages = 221-7 | month = Mar | year = 2013 | doi = 10.1097/PGP.0b013e31825909f6 | PMID = 23370646 }}</ref>
 
Roughly and with caveats........
Primary cutaneous extramammary Paget disease -            CK7+, CK20-, BerEP4+, CEA+, S100-, MelanA-, 34BE12-, CK5/6-, p63-, GATA+, CDX2-
Secondary extramammary Paget disease - colorectal origin - CK7-, CK20+, BerEP4+, CEA+, S100-, MelanA-, 34BE12-, CK5/6-, p63-, GATA-, CDX2+
Secondary extramammary Paget disease - urothelial origin - CK7+, CK20+/-, BerEP4-/+, CEA-/+, S100-, MelanA-, 34BE12+, CK5/6+/-, p63+, GATA+, CDX2-
Pagetoid VIN or squamous cell carcinoma in situ          - CK7-, CK20-, BerEP4-, CEA-, S100-, MelanA-, 34BE12+, CK5/6+ p63+, GATA-, CDX2-
Melanoma in situ                                        - CK7-, CK20-, BerEP4-, CEA-, S100+, MelanA+, 34BE12-, CK5/6- p63-, GATA-, CDX2-


==See also==
==See also==
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