Difference between revisions of "Paget's disease of the breast"

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Paget's disease of the breast (view source)

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#redirect [[Paget_disease_of_the_breast]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Extramammary_Paget_disease_-_high_mag.jpg
| Width      =
| Caption    = Paget's disease. [[H&E stain]].
| Synonyms  =
| Micro      = large epithelioid cells - nested or single - in the epidermis, clear/pale cytoplasm (occasionally eosinophilic), large nucleoli
| Subtypes  =
| LMDDx      = benign Toker cell hyperplasia, [[malignant melanoma]], [[Bowen's disease]], [[apocrine carcinoma of the skin]]
| Stains    =
| IHC        = CK7 +ve, CEA +ve, S-100 -ve, CK5/6 -ve, HER2 +ve
| EM        =
| Molecular  =
| IF        =
| Gross      = erythema, +/-weeping, +/-crusted
| Grossing  =
| Site      = [[breast]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = usu. associated with invasive breast carcinoma
| Other      =
| ClinDDx    = [[nipple adenoma]], contact dermatitis
| Tx        =
}}
'''Paget disease of the breast''', also '''[[Paget's disease]] of the breast''' and '''Paget disease of the nipple''', is a thingy seen in the [[breast]].  It is abbreviated '''PDB'''.<ref name=pmid19919588>{{Cite journal  | last1 = Ellis | first1 = PE. | last2 = Maclean | first2 = AB. | last3 = Crow | first3 = JC. | last4 = Wong Te Fong | first4 = LF. | last5 = Rolfe | first5 = KJ. | last6 = Perrett | first6 = CW. | title = Expression of cyclin D1 and retinoblastoma protein in Paget's disease of the vulva and breast: an immunohistochemical study of 108 cases. | journal = Histopathology | volume = 55 | issue = 6 | pages = 709-15 | month = Dec | year = 2009 | doi = 10.1111/j.1365-2559.2009.03434.x | PMID = 19919588 }}</ref>
 
There is also a ''[[Paget disease of the bone]]'' - just to make things confusing.  This is dealt with in the ''[[bone]]'' article and has nothing (from a pathologic perspective) to do with the Paget disease discussed in this article; these two things just happened to be discovered by the same guy.
 
Non-bone Paget disease is subdivided into:
#Mammary Paget disease - dealt with in this article.
#[[Extramammary Paget disease]].
 
Histologically, i.e. under the [[microscope]], the above are essentially identically; however, the associations (and prognosis) are quite different!
 
==General==
*Cells in the epithelium, i.e. skin, that look like they don't belong.
*Associated with underlying invasive [[invasive breast cancer|breast carcinoma]].<ref name=emed_pagets>URL: [http://emedicine.medscape.com/article/1101235-diagnosis http://emedicine.medscape.com/article/1101235-diagnosis]</ref>
 
Note:
*[[Extramammary Paget's disease]] is '''not''' usually associated with malignancy.
 
==Gross==
Features:<ref>{{Ref APBR|294 & 306 Q15}}</ref>
*Erythematous, i.e. red.
*+/-Crusted.
*+/-Weeping (wet).
 
DDx - clinical:
*[[Contact dermatitis]].<ref>URL: [http://www.danderm-pdv.is.kkh.dk/atlas/2-38.html http://www.danderm-pdv.is.kkh.dk/atlas/2-38.html]. Accessed on: 30 May 2012.</ref><ref name=pmid19882019>{{Cite journal  | last1 = Feroze | first1 = K. | last2 = Manoj | first2 = J. | last3 = Venkitakrishnan | first3 = S. | title = Allergic contact dermatitis mimicking mammary paget's disease. | journal = Indian J Dermatol | volume = 53 | issue = 3 | pages = 154-5 | month =  | year = 2008 | doi = 10.4103/0019-5154.43210 | PMID = 19882019 }}</ref>
 
Images:
*[http://www.sciencephoto.com/media/252126/enlarge Paget disease of the breast (sciencephoto.com)].
*[http://img.medscape.com/pi/emed/ckb/dermatology/1048885-1101235-864.jpg Paget disease of breast (medscape.com)].<ref>URL: [http://www.gfmer.ch/selected_images_v2/search_result_list.php?offset=15&param1=Paget%20disease%20of%20breast&param2=and http://www.gfmer.ch/selected_images_v2/search_result_list.php?offset=15&param1=Paget%20disease%20of%20breast&param2=and]. Accessed on: 30 May 2012.</ref>
 
==Microscopic==
Features:<ref name=emed_pagets/>
*Epitheliod morphology (round/ovoid).
*Cells nested or single.
*Clear/pale cytoplasm '''key feature''' - may also be eosinophilic.
*Large nucleoli.
 
Note:
*The neoplastic cells of Paget's disease may contain melanin.<ref name=Ref_APBR306-7>{{Ref APBR|306-7 Q15}}</ref>
 
===DDx===
*Benign Toker cell hyperplasia.
*[[Malignant melanoma]].
*[[Bowen disease]].
*[[Nipple adenoma|Nipple (duct) adenoma]] (clinical DDx).
*[[Apocrine carcinoma of the skin]].<ref>URL: [http://derm101.com/searchResults.aspx?searchStr=apocrine+carcinoma&rootTerm=apocrine+carcinoma&searchType=2&rootID=12687 http://derm101.com/searchResults.aspx?searchStr=apocrine+carcinoma&rootTerm=apocrine+carcinoma&searchType=2&rootID=12687]. Accessed on: 9 September 2011.</ref>
**Images: [http://commons.wikimedia.org/wiki/File:Apocrine_carcinoma_-_intermed_mag.jpg apocrine carcinoma - intermed. mag. (WC)], [http://commons.wikimedia.org/wiki/File:Apocrine_carcinoma_-_high_mag.jpg apocrine carcinoma - high mag. (WC)].
 
===Images===
<gallery>
Image:Extramammary_Paget_disease_-_low_mag.jpg | Paget disease - low mag. (WC/Nephron)
Image:Extramammary_Paget_disease_-_high_mag.jpg | Paget disease - high mag. (WC/Nephron)
</gallery>
www:
*[http://path.upmc.edu/cases/case331.html Paget disease of the breast - several good pictures (upmc.edu)].
*[http://www.webpathology.com/image.asp?case=303&n=3 Paget disease of the nipple (webpathology.com)].
 
==IHC==
Panel:<ref name=emed_pagets/>
*S-100 -ve, HMB-45 -ve (both typically +ve in melanoma).
*CK7 +ve. (???)
**Toker cells CK7 +ve.<ref name=pmid19601945>{{Cite journal  | last1 = Nofech-Mozes | first1 = S. | last2 = Hanna | first2 = W. | title = Toker cells revisited. | journal = Breast J | volume = 15 | issue = 4 | pages = 394-8 | month =  | year =  | doi = 10.1111/j.1524-4741.2009.00743.x | PMID = 19601945 }}</ref>
*CEA +ve (-ve in Bowen's disease, -ve in Toker cells).
 
Additional:
*HER2/neu - usually +ve.
*CK5/6 -ve.<ref>RS. May 2010.</ref>
**Usu. +ve in squamous cell carcinoma.<ref name=pmid19170169>{{Cite journal  | last1 = Khayyata | first1 = S. | last2 = Yun | first2 = S. | last3 = Pasha | first3 = T. | last4 = Jian | first4 = B. | last5 = McGrath | first5 = C. | last6 = Yu | first6 = G. | last7 = Gupta | first7 = P. | last8 = Baloch | first8 = Z. | title = Value of P63 and CK5/6 in distinguishing squamous cell carcinoma from adenocarcinoma in lung fine-needle aspiration specimens. | journal = Diagn Cytopathol | volume = 37 | issue = 3 | pages = 178-83 | month = Mar | year = 2009 | doi = 10.1002/dc.20975 | PMID = 19170169 }}</ref>
 
===Tabular comparison===
IHC features of Paget disease and its DDx:<ref>URL: [http://www.histopathology-india.net/EPD.htm http://www.histopathology-india.net/EPD.htm]. Accessed on: 4 December 2011.</ref>
{| class="wikitable sortable"
!Entity
!CK5/6
!CK7
!CAM5.2
!EMA
!CEA
!HER2
!S100
!HMB-45
|-
|Paget disease
|CK5/6 -ve
|CK7 +ve (?)
|CAM5.2 +ve
|EMA +ve
|CEA +ve
|HER2 '''+ve'''
|S100 -ve
|HMB-45 -ve
|-
|Bowen disease
|CK5/6 '''+ve'''
|CK7 -ve
|CAM5.2 -ve
|EMA -ve
|CEA -ve
|HER2 -ve (?)
|S100 -ve
|HMB-45 -ve
|-
|Toker cell hyperplasia
|CK5/6 ?
|CK7 '''+ve'''
|CAM5.2 ?
|EMA ?
|CEA '''-ve'''
|HER2 ?
|S100 ?
|HMB-45 ?
|-
|Melanoma
|CK5/6 -ve
|CK7 -ve
|CAM5.2 -ve
|EMA -ve
|CEA -ve
|HER2 -ve
|S100 '''+ve'''
|HMB-45 '''+ve'''
|-
|}
 
====Mini-table comparison====
{| class="wikitable sortable"
!Entity
!CK7
!CEA
!HER2
|-
|Paget disease
|CK7 '''+ve'''
|CEA '''+ve'''
|HER2 '''+ve'''
|-
|Toker cell hyperplasia
|CK7 '''+ve'''
|CEA -ve
|HER2 -ve
|-
|Bowen disease
|CK7 -ve
|CEA -ve
|HER2 -ve
|-
|}
 
==See also==
*[[Breast]].
*[[Invasive breast cancer]].
*[[Paget disease]].
*[[Extramammary Paget disease]].
 
==References==
{{Reflist|2}}
 
[[Category:Breast pathology]]
[[Category:Diagnosis]]
Michael
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