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 sclerosus]].
*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]]

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.

Sign out

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:

Sign out

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:

Sign out

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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