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.


=Malignant neoplasms of the vulva=
==A general differential diagnosis==
==Most common malignancies==
Benign:
Most common malignancies of vulva:<ref name=Ref_WMSP459>{{Ref WMSP|459}}</ref>
*[[Condyloma acuminatum]].
#Invasive [[squamous cell carcinoma]].
*[[Bartholin cyst]].
#[[Malignant melanoma]].
*[[Lichen sclerosus]].
*[[Zoon vulvitis]].
*[[Papillary hidradenoma]].
*[[Extramammary Paget disease]].


==Vulvar squamous cell carcinoma==
Other:
{{Main|Squamous cell carcinoma}}
*[[Aggressive angiomyxoma]].
Like SCC elsewhere.
*[[Angiomyofibroblastoma]].
*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>
*Depth from DE junction.


Note:
Premalignant:
*Tumour thickness != depth of invasion.
*[[Differentiated vulvar intraepithelial neoplasia]].
**Thickness = granular layer ''or'' surface (no granular layer present) to deepest tumour.
*[[Classic vulvar intraepithelial neoplasia]].
**Depth of invasion = epithelial-stromal junction in "valley" of papillae.


===Precursor lesions for SCC===
Malignant:
*Vulvar intraepithelial neoplasia (VIN).
*[[Vulvar squamous cell carcinoma]].
*[[Malignant melanoma]] of the vulva.


VIN can be divided into:  
==Normal vulva==
*''Classic VIN'', and
===Microscopic===
*''Differentiated VIN''.
Features:
**''Differentiated VIN'' is mostly irrelevant as it is basically never seen alone, i.e. it usually accompanies cancer.
*Stratified squamous epithelium with maturation.
*No nuclear changes.
*No inflammation.


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>
===Sign out===
====Mildly inflamed====
<pre>
VULVA, BIOPSY:
- SQUAMOUS MUCOSA WITH MILD CHRONIC INFLAMMATION AND REACTIVE CHANGES.
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.
</pre>


==Classic vulvar intraepithelial neoplasia==
=====Micro=====
*Abbreviated ''classic VIN'' or ''cVIN''.
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.


===General===
====Hyperkeratotic====
Epidemiology:
<pre>
*Classic VIN, like CIN, is associated with [[HPV]] and seen in younger women.
VULVA, BIOPSY:
*May be multifocal, i.e. associated with cervical ([[CIN]]) or vaginal ([[VAIN]]) lesions;<ref>{{Cite journal  | last1 = Pai | first1 = K. | last2 = Pai | first2 = S. | last3 = Gupta | first3 = A. | last4 = Rao | first4 = P. | last5 = Renjhen | first5 = P. | title = Synchronous vulvar intraepithelial neoplasia (VIN) of warty type and cervical intraepithelial neoplasia (CIN): case report. | journal = Indian J Pathol Microbiol | volume = 49 | issue = 4 | pages = 585-7 | month = Oct | year = 2006 | doi =  | PMID = 17183865 }}</ref> multifocality has a strongly association with immunosuppression.<ref name=pmid16713062>{{Cite journal  | last1 = Ait Menguellet | first1 = S. | last2 = Collinet | first2 = P. | last3 = Houfflin Debarge | first3 = V. | last4 = Nayama | first4 = M. | last5 = Vinatier | first5 = D. | last6 = Leroy | first6 = JL. | title = Management of multicentric lesions of the lower genital tract. | journal = Eur J Obstet Gynecol Reprod Biol | volume = 132 | issue = 1 | pages = 116-20 | month = May | year = 2007 | doi = 10.1016/j.ejogrb.2006.04.011 | PMID = 16713062 }}</ref>
- SQUAMOUS MUCOSA WITH MILD HYPERGRANULOSIS AND A THIN COMPACT LAYER OF KERATIN.
- NO SIGNIFICANT INFLAMMATION.
- NEGATIVE FOR DYSPLASIA.
</pre>


Classic VIN is graded like cervical pre-cancerous lesions:
=====Micro=====
*VIN I.
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.
**DDx: [[condyloma acuminatum]].<ref name=pmid15870532>{{Cite journal  | last1 = Rufforny | first1 = I. | last2 = Wilkinson | first2 = EJ. | last3 = Liu | first3 = C. | last4 = Zhu | first4 = H. | last5 = Buteral | first5 = M. | last6 = Massoll | first6 = NA. | title = Human papillomavirus infection and p16(INK4a) protein expression in vulvar intraepithelial neoplasia and invasive squamous cell carcinoma. | journal = J Low Genit Tract Dis | volume = 9 | issue = 2 | pages = 108-13 | month = Apr | year = 2005 | doi =  | PMID = 15870532 }}</ref>
**Uncommon.  
*VIN II.
**Not common.
*VIN III.
**Commonly seen.


===Microscopic===
=Benign disease=
Features:
This is grab bag of non-neoplastic stuffs.
*Increased [[NC ratio]].
*Multinucleation.
*Lack of maturation to surface (not very useful -- unlike in the cervix).<ref>LAE. February 2009.</ref>
**May have "vertical streaming" - the long axis of the cells are perpendicular to the free surface & basement membrane.
*Apical mitoses.


DDx:
==Condyloma acuminatum==
*[[Condyloma acuminatum]].
*[[AKA]] ''genital [[wart]]''.
{{Main|Condyloma acuminatum}}


Images:
==Lichen sclerosus==
*[http://www.flickr.com/photos/euthman/2658773592/ VIN III (flickr.com)].
{{Main|Lichen sclerosus}}
*[http://commons.wikimedia.org/wiki/File:Vulvar_intraepithelial_neoplasia3_2.jpg VIN III (WC)].
*[http://www.brown.edu/Courses/Digital_Path/systemic_path/female/warty_VIN.html VIN (brown.edu)].
 
===IHC===
*Classic VIN: p16 +ve, p53 -ve.
*Differentiated VIN: p16 -ve, p53 +ve.<ref name=pmid20062014>{{Cite journal  | last1 = Pinto | first1 = AP. | last2 = Miron | first2 = A. | last3 = Yassin | first3 = Y. | last4 = Monte | first4 = N. | last5 = Woo | first5 = TY. | last6 = Mehra | first6 = KK. | last7 = Medeiros | first7 = F. | last8 = Crum | first8 = CP. | title = Differentiated vulvar intraepithelial neoplasia contains Tp53 mutations and is genetically linked to vulvar squamous cell carcinoma. | journal = Mod Pathol | volume = 23 | issue = 3 | pages = 404-12 | month = Mar | year = 2010 | doi = 10.1038/modpathol.2009.179 | PMID = 20062014 }}</ref>
 
Note:
*p16 can be thought of as a poor man's HPV test.
 
==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>


==Bartholin cyst==
===General===
===General===
Epidemiology:
*Common.
*Associated with [[lichen sclerosus]].
*May become infected.
*Seen in older women.


Note:
Treatment:
*'''Not''' associated with [[HPV]].<ref name=pmid10716158>{{Cite journal  | last1 = Yang | first1 = B. | last2 = Hart | first2 = WR. | title = Vulvar intraepithelial neoplasia of the simplex (differentiated) type: a clinicopathologic study including analysis of HPV and p53 expression. | journal = Am J Surg Pathol | volume = 24 | issue = 3 | pages = 429-41 | month = Mar | year = 2000 | doi =  | PMID = 10716158 }}</ref>
*Drainage.
*Marsupialization.


===Microscopic===
===Microscopic===
Features:<ref name=pmid21057461>{{Cite journal  | last1 = van de Nieuwenhof | first1 = HP. | last2 = Bulten | first2 = J. | last3 = Hollema | first3 = H. | last4 = Dommerholt | first4 = RG. | last5 = Massuger | first5 = LF. | last6 = van der Zee | first6 = AG. | last7 = de Hullu | first7 = JA. | last8 = van Kempen | first8 = LC. | title = Differentiated vulvar intraepithelial neoplasia is often found in lesions, previously diagnosed as lichen sclerosus, which have progressed to vulvar squamous cell carcinoma. | journal = Mod Pathol | volume = 24 | issue = 2 | pages = 297-305 | month = Feb | year = 2011 | doi = 10.1038/modpathol.2010.192 | PMID = 21057461 }}</ref>
Features:
*Thickening of the stratum spinosum (acanthosis).
*Cyst with squamous or urothelial epithelium.<ref>[http://pathologyoutlines.com/vulva.html#bartholincyst http://pathologyoutlines.com/vulva.html#bartholincyst]</ref>
*Elongation of rete ridges with bridging/anastomoses between rete ridges.
*Nuclear atypia - esp. basal layer.
*Nuclei in stratum corneum (parakeratosis).
*+/-Atypical mitoses.


Notes:
Images:
*'''Not''' graded like classic VIN.
*[http://webpathology.com/image.asp?case=540&n=1 Bartholin cyst (webpathology.com)].
*Mnemonic ''BAD LAP'': '''b'''asal cell atypia, '''a'''nastomosing rete ridges (bridging), '''d'''VIN, '''l'''ichen sclerosus, '''a'''canthosis, '''p'''arakeratosis.
*[http://webpathology.com/image.asp?n=2&Case=540 Bartholin cyst - high mag. (webpathology.com)].


DDx:
===Sign out===
*[[Lichen sclerosus]].
====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>


Images:
=====Block letters=====
*www:
<pre>
**[http://www.nature.com/modpathol/journal/v24/n2/fig_tab/modpathol2010192f3.html#figure-title Differentiated VIN (nature.com)].<ref name=pmid21057461>{{Cite journal  | last1 = van de Nieuwenhof | first1 = HP. | last2 = Bulten | first2 = J. | last3 = Hollema | first3 = H. | last4 = Dommerholt | first4 = RG. | last5 = Massuger | first5 = LF. | last6 = van der Zee | first6 = AG. | last7 = de Hullu | first7 = JA. | last8 = van Kempen | first8 = LC. | title = Differentiated vulvar intraepithelial neoplasia is often found in lesions, previously diagnosed as lichen sclerosus, which have progressed to vulvar squamous cell carcinoma. | journal = Mod Pathol | volume = 24 | issue = 2 | pages = 297-305 | month = Feb | year = 2011 | doi = 10.1038/modpathol.2010.192 | PMID = 21057461 }}</ref>
VAGINA, CYST WALL, BIOPSY:
**[http://www.nature.com/modpathol/journal/v23/n3/fig_tab/modpathol2009179f1.html Differentiated VIN with bridging rete ridges (nature.com)].<ref name=pmid20062014>{{Cite journal  | last1 = Pinto | first1 = AP. | last2 = Miron | first2 = A. | last3 = Yassin | first3 = Y. | last4 = Monte | first4 = N. | last5 = Woo | first5 = TY. | last6 = Mehra | first6 = KK. | last7 = Medeiros | first7 = F. | last8 = Crum | first8 = CP. | title = Differentiated vulvar intraepithelial neoplasia contains Tp53 mutations and is genetically linked to vulvar squamous cell carcinoma. | journal = Mod Pathol | volume = 23 | issue = 3 | pages = 404-12 | month = Mar | year = 2010 | doi = 10.1038/modpathol.2009.179 | PMID = 20062014 }}</ref>
- SOFT TISSUE WITH A MIXED INFLAMMATORY INFILTRATE RICH IN NEUTROPHILS,
*[[WC]]:
  NO EPITHELIUM APPARENT; COMPATIBLE WITH DENUDED CYST WALL.
**[http://commons.wikimedia.org/wiki/File:Differentiated_vulvar_intraepithelial_neoplasia_-_low_mag.jpg Differentiated VIN - low mag. (WC)].
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.
**[http://commons.wikimedia.org/wiki/File:Differentiated_vulvar_intraepithelial_neoplasia_-_intermed_mag.jpg Differentiated VIN - intermed. mag. (WC)].
</pre>
**[http://commons.wikimedia.org/wiki/File:Differentiated_vulvar_intraepithelial_neoplasia_-_deep_-_high_mag.jpg Differentiated VIN - deep - high mag. (WC)].


===IHC===
==Zoon vulvitis==
*Differentiated VIN: p16 -ve, p53 +ve.<ref name=pmid20062014/>
*[[AKA]] ''plasma cell vulvitis''.
*Classic VIN: p16 +ve, p53 -ve.
{{Main|Zoon vulvitis}}
 
Note:
*p16 can be thought of as a poor man's [[HPV]] test.


=Neoplasms (non-malignant)=
=Neoplasms (non-malignant)=
Line 116: Line 105:
*[[Leiomyoma]].
*[[Leiomyoma]].
*[[Fibroepithelial polyp]].
*[[Fibroepithelial polyp]].
*[[Paget's disease]].
*[[Extramammary Paget's disease]].


==Hidradenoma papilliferum==
==Hidradenoma papilliferum==
*[[AKA]] ''papillary hidradenoma''.<ref>Hidradenoma papilliferum. Stedman's Medical Dictionary. 27th Ed.</ref>
*[[AKA]] ''papillary hidradenoma''.<ref>Hidradenoma papilliferum. Stedman's Medical Dictionary. 27th Ed.</ref>
===General===
{{Main|papillary hidradenoma}}
*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>
*Looks like ''[[intraductal papilloma]] of the breast''.<ref>{{Ref PBoD|1067}}</ref>


===Microscopic===
=Vulvar neoplasia=
Features:
==Classic vulvar intraepithelial neoplasia==
*Cystic spaces.
*Abbreviated ''classic VIN'' or ''cVIN''.
*Epithelium with apocrine differentiation (as demonstrated by apocrine snouts).
*[[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>
*Well-circumscribed.
{{Main|Classic vulvar intraepithelial neoplasia}}


Images:
==Differentiated vulvar intraepithelial neoplasia==
*[[WC]]:
*Abbreviated ''dVIN''.
**[http://commons.wikimedia.org/wiki/File:Papillary_hidradenoma_-_low_mag.jpg Papillary hidradenoma - low mag. (WC)].
*[[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>
**[http://commons.wikimedia.org/wiki/File:Papillary_hidradenoma_-_very_high_mag.jpg Papillary hidradenoma - very high mag. (WC)].
{{Main|Differentiated vulvar intraepithelial neoplasia}}
*www:
**[http://farm4.static.flickr.com/3019/2646470314_12fb77d3ec_z.jpg Hidradenoma papilliferum (flickr.com)].


Notes:
=Malignant neoplasms of the vulva=
*No attachment to epidermis.
==Overview==
*No nuclear changes suggestive of malignancy.
Most common malignancies of vulva:<ref name=Ref_WMSP459>{{Ref WMSP|459}}</ref>
#Invasive [[squamous cell carcinoma]].
#[[Malignant melanoma]].


=Other=
==Vulvar squamous cell carcinoma==
This is grab bag of non-neoplastic stuffs.
{{Main|Squamous cell carcinoma}}
 
*[[AKA]] ''squamous cell carcinoma of the vulva''.
==Condyloma acuminatum==
*[[AKA]] ''genital [[wart]]''.
===General===
===General===
*Due to [[human papillomavirus]] (HPV).
*Most common vulvar malignancy.
**Transmission: sexual, non-sexual, horizontal (mother to child).<ref name=Ref_APBR280>{{Ref APBR|280 Q29}}</ref>
***Should raise the suspicion of child abuse.


Note:
====Precursor lesions for SCC====  
*Related to [[verruca vulgaris]] (common wart).
*Vulvar intraepithelial neoplasia (VIN).
*The Bethesda system includes this in [[LSIL]].<ref>{{Ref GP|143}}</ref>
===Microscopic===
Features:
*Koilocytes.<ref name=pmid11860848>{{Cite journal  | last1 = Huang | first1 = Z. | last2 = Yang | first2 = S. | last3 = Li | first3 = Q. | last4 = Yan | first4 = P. | last5 = Li | first5 = L. | title = [Evaluation the pathological diagnostic values of koilocyte in condyloma acuminatum]. | journal = Zhonghua Liu Xing Bing Xue Za Zhi | volume = 22 | issue = 1 | pages = 58-60 | month = Feb | year = 2001 | doi =  | PMID = 11860848 }}</ref>
**Cells with an enlarged nucleus and perinuclear clearing.
*Papillomatosis.<ref>{{Ref WMSP|204}}</ref>
**Papillomatosis = surface elevation due to dermal papillae enlargement.<ref>{{Ref PBoD|1230}}</ref>
*+/-Parakeratosis.


DDx:
VIN can be divided into:  
*[[Classic vulvar intraepithelial neoplasia]] - architecture different.
*''Classic VIN'', and
*''Differentiated VIN''.
**''Differentiated VIN'' is mostly irrelevant as it is basically never seen alone, i.e. it usually accompanies cancer.


Images:
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>
*[http://commons.wikimedia.org/wiki/File:Condyloma_acuminatum_-_low_mag.jpg Condyloma acuminatum - low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Condyloma_acuminatum_-_very_high_mag.jpg Condyloma acuminatum - very high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Anal_condyloma_%282%29.jpg Condyloma acuminatum - 2 (WC)].
*[http://commons.wikimedia.org/wiki/File:Anal_condyloma_%284%29.jpg Condyloma acuminatum - 3 (WC)].


===Sign out===
===Microscopic===
<pre>
Like SCC elsewhere.
SKIN LESION ("VULVAR WART"), VULVA, EXCISION:
*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>
- CONDYLOMA ACUMINATUM (GENITAL WART).
*Depth from DE junction.
</pre>


====Seborrheic keratosis-like====
Note:
<pre>
*Tumour thickness != depth of invasion.
SKIN LESION, PERINEUM, BIOPSY:
**Thickness = granular layer ''or'' surface (no granular layer present) to deepest tumour.
- SEBORRHEIC KERATOSIS-LIKE CONDYLOMA ACUMINATUM (GENITAL WART).
**Depth of invasion = epithelial-stromal junction in "valley" of papillae.
- NEGATIVE FOR DYSPLASIA.
</pre>
 
====Micro====
The sections show a polypoid fragment of skin with epithelium on three sides, acanthosis, hyperkeratosis and parakeratosis. Koilocytic changes (mild nuclear enlargement, perinuclear clearing) are seen focally. There is mild basilar nuclear enlargement and hyperchromasia.  The epithelium matures to the surface and a granular layer is present.
 
=====Seborrheic keratosis-like=====
The sections show skin with acanthosis with papillomatous features (round bulbous rete ridges, acanthosis with penetrating fibrovascular cores) pseudohorn cysts, parakeratosis and hyperkeratosis.  There is no significant basal nuclear atypia. There are no mitoses and no melanocytic nests. There is mild dermal inflammation. There is no solar elastosis. Pigment incontinence is present focally.
 
==Lichen sclerosus==
*[[AKA]] ''chronic atrophic vulvitis'' - vulvar lesion.
*[[AKA]] ''balanitis xerotica obliterans'' (BXO) - penile lesion.<ref name=pmid12602704>{{cite journal |author=Finkbeiner AE |title=Balanitis xerotica obliterans: a form of lichen sclerosus |journal=South. Med. J. |volume=96 |issue=1 |pages=7–8 |year=2003 |month=January |pmid=12602704 |doi= |url=}}</ref>
 
===General===
*Associated with [[differentiated vulvar intraepithelial neoplasia]] - '''important'''.
**Approximately 50% of [[vulvar cancer]] associated with lichen sclerosus.
 
Clinical:
*Pruritis -> leads to scratching.
*Chronic condition.
*Usu. post-menopausal women.
*May lead to labial fusion.
 
Treatment:
*Steroids - high dose initially, then a maintenance therapy to prevent relapse.
 
Notes:
*'''Mixed vulvar dystrophy''' = lichen sclerosus + squamous cell hyperplasia.<ref name=pmid9491669>{{Cite journal  | last1 = Kini | first1 = U. | title = Squamous cell carcinoma of the vulva in association with mixed vulvar dystrophy. A brief report with review of literature. | journal = Indian J Cancer | volume = 34 | issue = 2 | pages = 92-5 | month = Jun | year = 1997 | doi =  | PMID = 9491669 }}</ref>
 
===Microscopic===
Features:<ref>URL: [http://www.pathologyoutlines.com/vulva.html#lichensclerosis http://www.pathologyoutlines.com/vulva.html#lichensclerosis]. Accessed on: 19 April 2011.</ref>
*Loss of rete ridges.
*Severe [[hyperkeratosis]].
**Hyperkeratosis = stratum corneum thickened.
*Fibrosis of dermis with loss of adnexal structures - '''key feature'''.
**May appear pale - directly deep to the epidermis.<ref>URL: [http://www.webpathology.com/image.asp?n=2&Case=538 http://www.webpathology.com/image.asp?n=2&Case=538]. Accessed on: 25 August 2011.</ref>
*Inflammation - often with eosinophils.
**May be prominent - in the ''inflammatory phase'' of the disease.<ref name=pmid9537476>{{Cite journal  | last1 = Fung | first1 = MA. | last2 = LeBoit | first2 = PE. | title = Light microscopic criteria for the diagnosis of early vulvar lichen sclerosus: a comparison with lichen planus. | journal = Am J Surg Pathol | volume = 22 | issue = 4 | pages = 473-8 | month = Apr | year = 1998 | doi =  | PMID = 9537476 }}</ref>


DDx:
DDx:
*[[Morphea profunda]] - deep fibrosis.
*[[Classic vulvar intraepithelial neoplasia]] - esp. tangential sections.
*[[Differentiated vulvar intraepithelial neoplasia]] - commonly co-exists with lichen sclerosus.
*[[Differentiated vulvar intraepithelial neoplasia]].
*[[Lichen planus]] (LP) - esp. for the ''inflammatory phase of lichen sclerosus''.
**LP has wedge shaped hypergranulosis, lacks basilar epidermotropism, no epidermal atrophy.<ref name=pmid9537476/>
 
Images:
*WC:
**[http://commons.wikimedia.org/wiki/File:Lichen_sclerosus_-_low_mag.jpg Lichen sclerosus - low mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Lichen_sclerosus_-_high_mag.jpg Lichen sclerosus - high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Lichen_sclerosus_-_very_high_mag.jpg Lichen sclerosus - very high mag. (WC)].
*www:
**[http://www.flickr.com/photos/euthman/2329061374/in/set-72057594114099781 Lichen sclerosus + syringoma (flickr.com)].
**[http://www.webpathology.com/image.asp?n=2&Case=538 Lichen sclerosus (webpathology.com)].


===Sign out===
===Sign out===
<pre>
<pre>
VULVA, BIOPSY:
VULVA, LEFT SIDE, (INCISIONAL) BIOPSY:
- LICHEN SCLEROSUS.
- INVASIVE SQUAMOUS CELL CARCINOMA.
</pre>
-- PLEASE SEE TUMOUR SUMMARY.


<pre>
TUMOUR SUMMARY - VULVA
FORESKIN, CIRCUMCISION:
Specimen Size: multiple fragments - up to 2.5 cm in aggregate.
- BALANITIS XEROTICA OBLITERANS.
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>
</pre>
====Micro====
=====Inflammtory phase of lichen sclerosus=====
The sections show skin with a lymphoplasmacytic predominant interface dermatitis with hyperkeratosis. Spongiosis is present. Scattered inflammatory cell are found with the basal aspect of the epidermis; however, they do not form clusters. No mitotic activity is appreciated.
Focal hypergranulosis and focal parakeratosis is present. Numerous Civatte bodies are identified.
The focal hypergranulosis is not wedge-shaped. There are no pointed rete ridges. There is no basal squamatization.
=====Sclerotic phase of lichen sclerosus=====
The sections show skin with loss of the rete ridges, hyperkeratosis and marked fibrosis of the superficial dermis. Few, scattered lymphocytes are seen in the dermis.
A granular layer is present. There is no basal nuclear atypia. There is no acanthosis.
==Bartholin cyst==
===General===
*Common.
*May become infected.
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>
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)].


=See also=
=See also=
Line 287: Line 190:


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

Latest revision as of 15:43, 8 March 2016

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

A general differential diagnosis

Benign:

Other:

Premalignant:

Malignant:

Normal vulva

Microscopic

Features:

  • Stratified squamous epithelium with maturation.
  • No nuclear changes.
  • No inflammation.

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Mildly inflamed

VULVA, BIOPSY:
- SQUAMOUS MUCOSA WITH MILD CHRONIC INFLAMMATION AND REACTIVE CHANGES.
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.
Micro

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.

Hyperkeratotic

VULVA, BIOPSY:
- SQUAMOUS MUCOSA WITH MILD HYPERGRANULOSIS AND A THIN COMPACT LAYER OF KERATIN.
- NO SIGNIFICANT INFLAMMATION.
- NEGATIVE FOR DYSPLASIA.
Micro

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.

Benign disease

This is grab bag of non-neoplastic stuffs.

Condyloma acuminatum

Main article: Condyloma acuminatum

Lichen sclerosus

Main article: Lichen sclerosus

Bartholin cyst

General

  • Common.
  • May become infected.

Treatment:

  • Drainage.
  • Marsupialization.

Microscopic

Features:

  • Cyst with squamous or urothelial epithelium.[1]

Images:

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Compatible with Bartholin cyst

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.
Block letters
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.

Zoon vulvitis

  • AKA plasma cell vulvitis.
Main article: Zoon vulvitis

Neoplasms (non-malignant)

A short DDx:[2]

Hidradenoma papilliferum

Main article: papillary hidradenoma

Vulvar neoplasia

Classic vulvar intraepithelial neoplasia

  • Abbreviated classic VIN or cVIN.
  • AKA usual VIN or uVIN.[4]
Main article: Classic vulvar intraepithelial neoplasia

Differentiated vulvar intraepithelial neoplasia

  • Abbreviated dVIN.
  • AKA VIN simplex.[5]
Main article: Differentiated vulvar intraepithelial neoplasia

Malignant neoplasms of the vulva

Overview

Most common malignancies of vulva:[6]

  1. Invasive squamous cell carcinoma.
  2. Malignant melanoma.

Vulvar squamous cell carcinoma

Main article: Squamous cell carcinoma
  • AKA squamous cell carcinoma of the vulva.

General

  • Most common vulvar malignancy.

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.[7]

Microscopic

Like SCC elsewhere.

  • Microinvasion: <=1 mm stromal invasion, tumour size <=2 cm (T1a).[8]
  • Depth from DE junction.

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:

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VULVA, LEFT SIDE, (INCISIONAL) BIOPSY:
- INVASIVE SQUAMOUS CELL CARCINOMA.
-- PLEASE SEE TUMOUR SUMMARY.

TUMOUR SUMMARY - VULVA
Specimen Size: multiple fragments - up to 2.5 cm in aggregate.
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).

See also

References

  1. ↑ http://pathologyoutlines.com/vulva.html#bartholincyst
  2. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 456. ISBN 978-0781765275. }}
  3. ↑ Hidradenoma papilliferum. Stedman's Medical Dictionary. 27th Ed.
  4. ↑ Reyes, MC.; Cooper, K. (Jan 2014). "An update on vulvar intraepithelial neoplasia: terminology and a practical approach to diagnosis.". J Clin Pathol. doi:10.1136/jclinpath-2013-202117. PMID 24399036.
  5. ↑ Ruhul Quddus, M.; Xu, C.; Steinhoff, MM.; Zhang, C.; Lawrence, WD.; Sung, CJ. (Jun 2005). "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.". Histopathology 46 (6): 718-20. doi:10.1111/j.1365-2559.2005.02036.x. PMID 15910611.
  6. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 459. ISBN 978-0781765275.
  7. ↑ De Vuyst H, Clifford GM, Nascimento MC, Madeleine MM, Franceschi S (April 2009). "Prevalence and type distribution of human papillomavirus in carcinoma and intraepithelial neoplasia of the vulva, vagina and anus: a meta-analysis". Int. J. Cancer 124 (7): 1626–36. doi:10.1002/ijc.24116. PMID 19115209.
  8. ↑ URL: http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/Vulva_11protocol.pdf. Accessed on: 9 March 2012.
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