Difference between revisions of "Vulva"

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This article addresses the basics of '''vulva''', from a pathologic perspective.
This article addresses the basics of '''vulva''', from a pathologic perspective.


==Neoplasms of the vulva==
==A general differential diagnosis==
Low grade pre-cancerous lesions (VIN) are typically HPV positive, while high grade pre-cancerous lesions and cancer are less often HPV positive.<ref name=pmid19115209>{{cite journal |author=De Vuyst H, Clifford GM, Nascimento MC, Madeleine MM, Franceschi S |title=Prevalence and type distribution of human papillomavirus in carcinoma and intraepithelial neoplasia of the vulva, vagina and anus: a meta-analysis |journal=Int. J. Cancer |volume=124 |issue=7 |pages=1626–36 |year=2009 |month=April |pmid=19115209 |doi=10.1002/ijc.24116 |url=}}</ref>
Benign:
*[[Condyloma acuminatum]].
*[[Bartholin cyst]].
*[[Lichen sclerosus]].
*[[Zoon vulvitis]].
*[[Papillary hidradenoma]].
*[[Extramammary Paget disease]].


Most common malignancy of vulva:
Other:
*Invasive [[squamous cell carcinoma]].
*[[Aggressive angiomyxoma]].
*[[Angiomyofibroblastoma]].


Precursor lesion:  
Premalignant:
*Vulvar intraepithelial neoplasia (VIN).
*[[Differentiated vulvar intraepithelial neoplasia]].
*[[Classic vulvar intraepithelial neoplasia]].


VIN can be divided into:  
Malignant:
*''Classic VIN'', and
*[[Vulvar squamous cell carcinoma]].
*''Differentiated VIN''.
*[[Malignant melanoma]] of the vulva.
**''Differentiated VIN'' is mostly irrelevant as it is basically never seen alone, i.e. it usually accompanies cancer.


==Classic VIN==
==Normal vulva==
===Epidemiology===
===Microscopic===
*Classic VIN, like CIN, is associated with [[HPV]] and seen in younger women.
Features:
*Stratified squamous epithelium with maturation.
*No nuclear changes.
*No inflammation.


===General===
===Sign out===
*Classic VIN is graded like cervical pre-cancerous lesions:
====Mildly inflamed====
**VIN I.
<pre>
***Not common.
VULVA, BIOPSY:
**VIN II.
- SQUAMOUS MUCOSA WITH MILD CHRONIC INFLAMMATION AND REACTIVE CHANGES.
***Not common.
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.
**VIN III.
</pre>
***Commonly seen.


DDx:
=====Micro=====
*Condyloma (genital wart).
The sections show squamous mucosa with a mild chronic inflammatory infiltrate that consists predominantly of lymphocytes. There is mild nuclear enlargement and intracellular edema. The nuclear membranes are regular. No nuclear hyperchromasia is apparent. No mitotic activity is readily apparent.
**Most caused by HPV.


===Microscopic of classic VIN===
====Hyperkeratotic====
*Increased NC ratio.
<pre>
*Multinucleation.
VULVA, BIOPSY:
*Lack of maturation to surface (not very useful -- unlike in the cervix).<ref>LAE. February 2009.</ref>
- SQUAMOUS MUCOSA WITH MILD HYPERGRANULOSIS AND A THIN COMPACT LAYER OF KERATIN.
*Apical mitoses.
- NO SIGNIFICANT INFLAMMATION.
- NEGATIVE FOR DYSPLASIA.
</pre>


==Differentiated VIN==
=====Micro=====
===Epidemiology===
The sections show a small piece of squamous mucosa with mild hypergranulosis and a compact keratin layer. The epithelial component is not significantly thickened but contains rare intraepithelial lymphocytes and has minimal edema. The subepithelial tissue has rare scattered lymphocytes and a mild prominence of small blood vessels. No subepithelial fibrosis is appreciated. The epithelium has no atypia. No mitotic figures are readily apparent.
*Associated with [[lichen sclerosis]].
*NOT associated with HPV and seen in older women.
 
===Microscopic===
Features:
*NOT graded like classic VIN.
*Acanthosis (thickening of stratum spinosum) + elongation of rete ridges.


==IHC for VIN==
=Benign disease=
*Classic VIN: p16+, p53-.
This is grab bag of non-neoplastic stuffs.
*Differentiated VIN: p16-, p53+.


ASIDE: [[p16]] can be thought of as a poor man's HPV test.
==Condyloma acuminatum==
*[[AKA]] ''genital [[wart]]''.
{{Main|Condyloma acuminatum}}


==Lichen sclerosus==
==Lichen sclerosus==
===General===
{{Main|Lichen sclerosus}}
*Pruritis -> leads to scratching.
 
===Microscopic===
Features:<ref>[http://www.pathologyoutlines.com/vulva.html#lichensclerosis http://www.pathologyoutlines.com/vulva.html#lichensclerosis]</ref>
*Loss of rete ridges - '''key feature'''.
*Severe hyperkeratosis.
*Fibrosis of dermis with loss of adnexal structures.
*Inflammation - often with eosinophils.


==Bartholin cyst==
==Bartholin cyst==
===General===
===General===
*Common.
*Common.
*May become infected.


===Micro===
Treatment:
*Drainage.
*Marsupialization.
 
===Microscopic===
Features:
*Cyst with squamous or urothelial epithelium.<ref>[http://pathologyoutlines.com/vulva.html#bartholincyst http://pathologyoutlines.com/vulva.html#bartholincyst]</ref>
*Cyst with squamous or urothelial epithelium.<ref>[http://pathologyoutlines.com/vulva.html#bartholincyst http://pathologyoutlines.com/vulva.html#bartholincyst]</ref>
Images:
*[http://webpathology.com/image.asp?case=540&n=1 Bartholin cyst (webpathology.com)].
*[http://webpathology.com/image.asp?n=2&Case=540 Bartholin cyst - high mag. (webpathology.com)].
===Sign out===
====Compatible with Bartholin cyst====
<pre>
Submitted as "Bartholin's cyst wall - left", Excision:
- Connective tissue with overlying urothelium that is focally
  denuded and associated with a lymphohistiocytic response,
  compatible with Bartholin's cyst.
- NEGATIVE for dysplasia and NEGATIVE for malignancy.
</pre>
=====Block letters=====
<pre>
VAGINA, CYST WALL, BIOPSY:
- SOFT TISSUE WITH A MIXED INFLAMMATORY INFILTRATE RICH IN NEUTROPHILS,
  NO EPITHELIUM APPARENT; COMPATIBLE WITH DENUDED CYST WALL.
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.
</pre>
==Zoon vulvitis==
*[[AKA]] ''plasma cell vulvitis''.
{{Main|Zoon vulvitis}}
=Neoplasms (non-malignant)=
A short DDx:<ref>{{Ref WMSP|456}}}}</ref>
*[[Granular cell tumour]].
*[[Leiomyoma]].
*[[Fibroepithelial polyp]].
*[[Extramammary Paget's disease]].


==Hidradenoma papilliferum==
==Hidradenoma papilliferum==
*[[AKA]] ''papillary hidradenoma''.<ref>Hidradenoma papilliferum. Stedman's Medical Dictionary. 27th Ed.</ref>
{{Main|papillary hidradenoma}}
=Vulvar neoplasia=
==Classic vulvar intraepithelial neoplasia==
*Abbreviated ''classic VIN'' or ''cVIN''.
*[[AKA]] ''usual VIN'' or ''uVIN''.<ref name=pmid24399036>{{Cite journal  | last1 = Reyes | first1 = MC. | last2 = Cooper | first2 = K. | title = An update on vulvar intraepithelial neoplasia: terminology and a practical approach to diagnosis. | journal = J Clin Pathol | volume =  | issue =  | pages =  | month = Jan | year = 2014 | doi = 10.1136/jclinpath-2013-202117 | PMID = 24399036 }}</ref>
{{Main|Classic vulvar intraepithelial neoplasia}}
==Differentiated vulvar intraepithelial neoplasia==
*Abbreviated ''dVIN''.
*[[AKA]] ''VIN simplex''.<ref name=pmid15910611>{{Cite journal  | last1 = Ruhul Quddus | first1 = M. | last2 = Xu | first2 = C. | last3 = Steinhoff | first3 = MM. | last4 = Zhang | first4 = C. | last5 = Lawrence | first5 = WD. | last6 = Sung | first6 = CJ. | title = Simplex (differentiated) type VIN: absence of p16INK4 supports its weak association with HPV and its probable precursor role in non-HPV related vulvar squamous cancers. | journal = Histopathology | volume = 46 | issue = 6 | pages = 718-20 | month = Jun | year = 2005 | doi = 10.1111/j.1365-2559.2005.02036.x | PMID = 15910611 }}</ref>
{{Main|Differentiated vulvar intraepithelial neoplasia}}
=Malignant neoplasms of the vulva=
==Overview==
Most common malignancies of vulva:<ref name=Ref_WMSP459>{{Ref WMSP|459}}</ref>
#Invasive [[squamous cell carcinoma]].
#[[Malignant melanoma]].
==Vulvar squamous cell carcinoma==
{{Main|Squamous cell carcinoma}}
*[[AKA]] ''squamous cell carcinoma of the vulva''.
===General===
===General===
*Dermal thingy; hidradenoma = tumour of sweat duct epithelium.<ref>URL: [http://medical-dictionary.thefreedictionary.com/hidradenoma http://medical-dictionary.thefreedictionary.com/hidradenoma]. Accessed on: 14 April 2011.</ref>
*Most common vulvar malignancy.
*[[AKA]] ''papillary hidradenoma''.<ref>Hidradenoma papilliferum. Stedman's Medical Dictionary. 27th Ed.</ref>
 
*Looks like ''intraductal papilloma of the breast''.<ref>{{Ref PBoD|1067}}</ref>
====Precursor lesions for SCC====  
*Vulvar intraepithelial neoplasia (VIN).
 
VIN can be divided into:  
*''Classic VIN'', and
*''Differentiated VIN''.
**''Differentiated VIN'' is mostly irrelevant as it is basically never seen alone, i.e. it usually accompanies cancer.
 
Low grade pre-cancerous lesions (VIN) are typically [[HPV]] positive, while high grade pre-cancerous lesions and [[cancer]] are less often HPV positive.<ref name=pmid19115209>{{cite journal |author=De Vuyst H, Clifford GM, Nascimento MC, Madeleine MM, Franceschi S |title=Prevalence and type distribution of human papillomavirus in carcinoma and intraepithelial neoplasia of the vulva, vagina and anus: a meta-analysis |journal=Int. J. Cancer |volume=124 |issue=7 |pages=1626–36 |year=2009 |month=April |pmid=19115209 |doi=10.1002/ijc.24116 |url=}}</ref>


===Microscopic===
===Microscopic===
Features:
Like SCC elsewhere.
*Cystic spaces.
*Microinvasion: <=1 mm stromal invasion, tumour size <=2 cm (T1a).<ref>URL: [http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/Vulva_11protocol.pdf http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/Vulva_11protocol.pdf]. Accessed on: 9 March 2012.</ref>
*Epithelium with apocrine differentiation (as demonstrated by apocrine snouts).
*Depth from DE junction.
*Well-circumscribed.
 
Note:
*Tumour thickness != depth of invasion.
**Thickness = granular layer ''or'' surface (no granular layer present) to deepest tumour.
**Depth of invasion = epithelial-stromal junction in "valley" of papillae.
 
DDx:
*[[Classic vulvar intraepithelial neoplasia]] - esp. tangential sections.
*[[Differentiated vulvar intraepithelial neoplasia]].


Images:
===Sign out===
*[http://farm4.static.flickr.com/3019/2646470314_12fb77d3ec_z.jpg Hidradenoma papilliferum (flickr.com)].
<pre>
VULVA, LEFT SIDE, (INCISIONAL) BIOPSY:
- INVASIVE SQUAMOUS CELL CARCINOMA.
-- PLEASE SEE TUMOUR SUMMARY.


Notes:
TUMOUR SUMMARY - VULVA
*No attachment to epidermis.
Specimen Size: multiple fragments - up to 2.5 cm in aggregate.
*No nuclear changes suggestive of malignancy.
Tumour site: left vulva - around Bartholin's gland.
Tumour size: at least 10 mm, cannot be determined due to fragmentation.
Tumour focality: cannot be determined.
Histologic type: squamous cell carcinoma with focal keratinization.
Histologic Grade: G2 - moderately differentiated.
Microscopic tumour extension: greater than 2 mm, assessment limited by
fragmentation and tissue orientation.
Tumour border: infiltrating.
Lymph-Vascular Invasion: present.
Additional findings:
Vulvar intraepithelial neoplasia (VIN) 3 (severe dysplasia/carcinoma in situ).
</pre>


==See also==
=See also=
*[[Gynecologic pathology]].
*[[Gynecologic pathology]].
*[[Uterus]].
*[[Uterus]].
*[[Cervix]] - cervical intraepithelial neoplasia.
*[[Cervix]] - cervical intraepithelial neoplasia (CIN).
*[[Vagina]].
*[[Vagina]].
*[[Anus]] - anal intraepithelial neoplasia.
*[[Anus]] - anal intraepithelial neoplasia.


==References==
=References=
{{reflist|2}}
{{reflist|2}}


[[Category:Gynecologic pathology]]
[[Category:Gynecologic pathology]]
[[Category:Vulva|Vulva]]
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