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Vulvar and vaginal cancer

Last updated: March 4, 2021

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Vulvar cancer is a malignancy of the outer female genitalia that predominantly occurs in postmenopausal women. Major risk factors are HPV infection, smoking, vulvar dystrophy, and vulvar or cervical intraepithelial neoplasia. Clinical manifestations of vulvar cancer include new lumps or lesions, itching, a burning sensation and, less frequently, vulvar bleeding. Suspicious lesions must be biopsied for histological analysis and to rule out other similar conditions, such as vulvar dermatoses or vulvar intraepithelial neoplasia, which both increase the risk of vulvar cancer. Vulvar cancer is staged based on the depth of the lesion and the involvement of neighboring structures. Surgical resection (radical vulvectomy) is the first-line treatment, but advanced stages may require radiotherapy and/or palliative chemotherapy. Vulvar cancer is usually associated with a poor prognosis.

Vaginal cancer is closely related to vulvar cancer in terms of etiology and histology, but it occurs inside the vagina (typically the posterior third of the vaginal wall), rather than the vulva.

Epidemiological data refers to the US, unless otherwise specified.

Risk factors [3]

  • Pathology
    • Adenocarcinoma; carcinoma in situ
    • Low risk (< 15%) of underlying invasive Paget disease/invasive adenocarcinoma (unlike Paget disease of the breast which is always associated with underlying carcinoma)
  • Clinical features

  • May initially be asymptomatic [2]
  • Local pruritus, possibly with burning sensation and pain
  • Reddish, blackish, and/or whitish patches of discoloration
  • Lumps or growths of various shapes, often wart-like lesions or ulcers
  • Vulvar bleeding or discharge (less common)
  • Dysuria, dyspareunia
  • Lymphadenopathy in the groin area

All suspicious lesions must be biopsied for histological analysis.

Vulvar dermatoses

Vulvar dermatoses are not inherently precancerous, but they do increase the risk of squamous cell carcinoma.

Vulvar intraepithelial neoplasia (VIN)

  • Definition: precancerous lesion caused by dysplasia of squamous cells
  • Classification [4]
    • VIN, usual type (most common)
      • Associated with HPV
      • Commonly multifocal
    • VIN, differentiated type
      • Associated with lichen sclerosis and other dermatoses
      • Commonly unifocal
    • VIN, unclassified type
  • Diagnosis: tissue biopsy
  • Treatment: : depending on severity, excision or ablation may become necessary
  • Prognosis: may progress to vulvar carcinoma despite treatment (in < 10% of cases)

The differential diagnoses listed here are not exhaustive.

  • The average 5-year survival rates range from 30–50%.
  • Survival rates vary greatly depending on the stage of the disease.

Overview

Subtypes

Symptoms

Diagnosis

Therapy

Senile vaginitis should also be considered in patients presenting with vaginal pruritus, burning, and pain.

  1. Insinga RP, Liaw K-L, Johnson LG, Madeleine MM. A Systematic Review of the Prevalence and Attribution of Human Papillomavirus Types Among Cervical, Vaginal and Vulvar Pre-cancers and Cancers in the United States. Cancer Epidemiol Biomarkers Prev. 2008; 17 (7): p.1611–1622. doi: 10.1158/1055-9965.EPI-07-2922 . | Open in Read by QxMD
  2. Alkatout I, Günther V, Schubert M, et al. Vulvar cancer: epidemiology, clinical presentation, and management options. Int J Womens Health. 2015 : p.305. doi: 10.2147/ijwh.s68979 . | Open in Read by QxMD
  3. Qualman SJ, Bowen J, Parham DM, et al. Protocol for the examination of specimens from patients (children and young adults) with rhabdomyosarcoma.. Arch Pathol Lab Med. 2003; 127 (10): p.1290-7. doi: 10.1043/1543-2165(2003)127<1290:PFTEOS>2.0.CO;2 . | Open in Read by QxMD
  4. Ognjanovic S, Linabery AM, Charbonneau B, Ross JA. Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975-2005. Cancer. 2009; 115 (18): p.4218-4226. doi: 10.1002/cncr.24465 . | Open in Read by QxMD
  5. Preti M. VIN usual type—from the past to the future. ecancermedicalscience. 2015; 9 . doi: 10.3332/ecancer.2015.531 . | Open in Read by QxMD
  6. Koh WJ, Greer BE, Abu-Rustum NR, et al. Vulvar Cancer, Version 1.2017, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2017; 15 (1): p.92-120. doi: 10.6004/jnccn.2017.0008 . | Open in Read by QxMD
  7. Key Statistics for Vulvar Cancer. https://www.cancer.org/cancer/vulvar-cancer/about/key-statistics.html. Updated: November 3, 2017. Accessed: March 22, 2020.