Vulva

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

Malignant neoplasms overview

Most common malignancies

Most common malignancies of vulva:[1]

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

Squamous cell carcinoma

Like SCC elsewhere.

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.[2]

Classic vulvar intraepithelial neoplasia

Epidemiology

  • Classic VIN, like CIN, is associated with HPV and seen in younger women.

General

  • Classic VIN is graded like cervical pre-cancerous lesions:
    • VIN I.
      • Not common.
    • VIN II.
      • Not common.
    • VIN III.
      • Commonly seen.

DDx:

  • Condyloma (genital wart).
    • Most caused by HPV.

Microscopic

Features:

  • Increased NC ratio.
  • Multinucleation.
  • Lack of maturation to surface (not very useful -- unlike in the cervix).[3]
  • Apical mitoses.

Differentiated vulvar intraepithelial neoplasia

Epidemiology

  • 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

  • Classic VIN: p16+, p53-.
  • Differentiated VIN: p16-, p53+.

ASIDE: p16 can be thought of as a poor man's HPV test.

Neoplasms (non-malignant)

A short DDx:[4]

Hidradenoma papilliferum

General

Microscopic

Features:

  • Cystic spaces.
  • Epithelium with apocrine differentiation (as demonstrated by apocrine snouts).
  • Well-circumscribed.

Images:

Notes:

  • No attachment to epidermis.
  • No nuclear changes suggestive of malignancy.

Other

This is grab bag of non-neoplastic stuffs.

Condyloma acuminatum

  • AKA genital wart.

General

Note:

Microscopic

Features:

  • Koilocytes.[8]
    • Cells with an enlarged nucleus and perinuclear clearing.

Images:

Lichen sclerosus

  • AKA chronic atrophic vulvitis - vulvar lesion.
  • AKA balanitis xerotica obliterans (BXO) - penile lesion.[9]

General

  • Associated with differentiated vulvar intraepithelial neoplasia.
    • 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.[10]

Microscopic

Features:[11]

  • 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.[12]
  • Inflammation - often with eosinophils.

Images:

Bartholin cyst

General

  • Common.

Microscopic

Features:

  • Cyst with squamous or urothelial epithelium.[13]

See also

References

  1. 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.
  2. 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.
  3. LAE. February 2009.
  4. 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. }}
  5. Hidradenoma papilliferum. Stedman's Medical Dictionary. 27th Ed.
  6. URL: http://medical-dictionary.thefreedictionary.com/hidradenoma. Accessed on: 14 April 2011.
  7. Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1067. ISBN 0-7216-0187-1.
  8. Huang, Z.; Yang, S.; Li, Q.; Yan, P.; Li, L. (Feb 2001). "[Evaluation the pathological diagnostic values of koilocyte in condyloma acuminatum].". Zhonghua Liu Xing Bing Xue Za Zhi 22 (1): 58-60. PMID 11860848.
  9. Finkbeiner AE (January 2003). "Balanitis xerotica obliterans: a form of lichen sclerosus". South. Med. J. 96 (1): 7–8. PMID 12602704.
  10. Kini, U. (Jun 1997). "Squamous cell carcinoma of the vulva in association with mixed vulvar dystrophy. A brief report with review of literature.". Indian J Cancer 34 (2): 92-5. PMID 9491669.
  11. URL: http://www.pathologyoutlines.com/vulva.html#lichensclerosis. Accessed on: 19 April 2011.
  12. URL: http://www.webpathology.com/image.asp?n=2&Case=538. Accessed on: 25 August 2011.
  13. http://pathologyoutlines.com/vulva.html#bartholincyst