Difference between revisions of "Extramammary Paget disease"

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**If CK20 is positive are other organ specific markers positive? - CDX2 - colorectal or GATA3 - urothelial
**If CK20 is positive are other organ specific markers positive? - CDX2 - colorectal or GATA3 - urothelial


Panel:
Panel: You can not rely on any one marker - a panel is required
*A carcinoma marker - CEA or BerEP4 or both
*A carcinoma marker - CEA or BerEP4 or both
*Differential keratins - low molecular weight (glandular) cam5.2, CK7 vs high molecular weight (squamous) 34BE12, CK5/6<ref>RS. May 2010.</ref>
*Differential keratins - low molecular weight (glandular) cam5.2, CK7 vs high molecular weight (squamous) 34BE12, CK5/6<ref>RS. May 2010.</ref>
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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 that a CK20 negative urothelial origin EMPD will show the same 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.
You can not rely on any one marker - a panel is required
**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. <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>
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