Description

• Carcinoma in situ (Bowen disease): Premalignant changes involving the squamous epithelium of the vulva
• Squamous cell carcinoma: Invasive squamous cell carcinoma is the most common malignancy involving the vulva (90% of patients). The malignancy can be well, moderately, or poorly differentiated.
• Other invasive cell types include melanoma, Paget disease, adenocarcinoma, adenoid cystic carcinoma, small cell carcinoma, verrucous carcinoma, and sarcomas. Sarcomas are usually leiomyosarcoma and probably arise at the insertion of the round ligament in the labium major.
• System(s) affected: Reproductive

• Older patients with associated medical problems are at high risk from radical surgery. The surgery, however, is external, usually well tolerated, and is the treatment of choice. Patients who are not surgical candidates can be treated with primary radiotherapy.
• In the very elderly, palliative vulvectomy provides relief of symptoms for ulcerating symptomatic advanced disease.

Epidemiology

• In the US, invasive vulvar malignancy is the 4th most common gynecologic malignancy, accounting for 4,340 new cases and 940 deaths in 2011.
• Predominant age:
  • In situ disease: Mean age 40 years
  • Invasive malignancy: Mean age 60 years, with a range of 20–90 years.

Risk Factors

• VIN or CIN
• Smoking
• Lichen sclerosus (vulvar dystrophy)
• Human papillomavirus (HPV) infection
• Autoimmune processes
• Immunodeficiency syndromes

Genetics
No known genetic pattern

General Prevention

• HPV vaccination has the potential to decrease vulvar cancer by 60%.
• Abstinence from smoking/smoking cessation counseling

Etiology

• Patients with cervical cancer are more likely to develop vulvar cancer at a later date, because if the so-called “field effect” with a carcinogen involving the lower genital tract.
• HPV has been associated with squamous cell abnormalities of the cervix, vagina, and vulva; 60% of vulvar cancers are attributable to oncogenic HPV; 92% of VIN 2/3, VaIN 2/3, and AIN are attributable to HPV.
• Smoking is associated with squamous cell disease of the vulva, possibly from direct irritation of the vulva by the transfer of tars and nicotine on the patient's hands or from systemic absorption of carcinogen.

Commonly Associated Conditions

• Patients with invasive vulvar cancer are often elderly and have associated medical conditions.
• High rate of other gynecologic malignancies; patients should be evaluated for these.