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Difference between revisions of "Extramammary Paget disease"
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==IHC== | ==IHC== | ||
*Extramammary Paget disease is a 'big' diagnosis in that the diagnosis will have significant clinical consequences. So a large panel of immuno is required to nail down the diangosis. | |||
*Is the lesion epithelial or melanocytic? (S100, Melan A) | |||
*Is the lesion adenocarcinoma or squamous cell carcinoma? | |||
**Low molecular weight (CK7, cam5.2) or high molecular weight keratins (34BE12, CK5/6)? | |||
**Adenocarcinoma markers? - CEA, BerEP4 | |||
**Nuclear differentiation markers? - p63 (squamous) vs GATA3 (adnexal) | |||
*Is the lesion primary or secondary | |||
**Secondary extramammary Paget disease may be CK20 positive (urothelial or rectal | |||
**If CK20 is positive are other organ specific markers positive? - CDX2 - colorectal or GATA3 - urothelial | |||
Panel: | Panel: | ||
*CEA | *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> | |||
*CK7 and CK20 - where does it come from? | |||
*S100 - | *S100 and Melan A - exclude melanoma in situ | ||
*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 you do not need to consider mammary Paget disease or Toker cells in your ddx. | |||
* | 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> | ||
**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> | |||
==See also== | ==See also== | ||