Difference between revisions of "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]] <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 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 tumour

***Arises in the perineal areas near these organs.

*R/O VIN.

*R/O vulvitis.

Image:Penis PagetDisease MP CTR.jpg|Penis Paget Disease - medium 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)

*[[Malignant melanoma in situ]] - atypical cells are mostly basilar with upward migration.

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)

*Squamous cell carcinoma in situ "[[Bowen disease]]"

**Pagetoid squamous cell carcinoma in situ can look identical to extramammary Paget disease

Image:Skin SCCIS PagetoidandConventional 15BR30103.jpg|An SCCIS from sundamaged skin.  Pagetoid SCCIS on the left, conventional SCCIS on the right. (SKB)

*Pagetoid vulvar/anal/penil HPV associated intraepithelial neoplasia - Pagetoid areas often merge into areas of typical VIN

 

*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.

*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.

**Low molecular weight (CK7, cam5.2) or high molecular weight keratins (34BE12, CK5/6)?

**Adenocarcinoma markers? - CEA, BerEP4

**Primary cutaneous extramammary Paget disease is always CK20 negative.

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

 

*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 and Melan A - exclude melanoma in situ

 

**A CK20 negative urothelial origin EMPD could show a similar immunoprofile as a primary cutaneous EMPD.

*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 this DDX as  VIN, EMPD and urothelial carcioma can 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>

 

*Pagetoid VIN or squamous cell carcinoma in situ          - CK7-, CK20-, BerEP4-, CEA-, S100-, MelanA-, 34BE12+, CK5/6+ p63+, GATA-, CDX2-

 

 

Image:Skin Vulva PagetDisease CK7 15BR***.jpg|Vulva - Primary cutaneous extramammary Paget disease.  CK7 (SKB)