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- Premalignant lesions of the vulva are collectively known as vulvar intraepithelial neoplasia (VIN). Exposure to human papillomavirus (HPV) has been linked to >70% of VIN.
- Invasive squamous cell carcinoma is the most common malignancy involving the vulva (90% of patients); can be well, moderately, or poorly differentiated and derives from keratinized skin covering the vulva and perineum
- Melanoma is the second most common type of vulvar malignancy (8%) and sarcoma is the third.
- Other invasive cell types include basal cell carcinoma, Paget disease, adenocarcinoma arising from Bartholin gland or apocrine sweat glands, 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; however, sarcoma can arise from any structure of the vulva, including blood vessels, skeletal muscle, and fat.
- Rarely, breast carcinoma has been reported in the vulva and is thought to arise from ectopic breast tissue.
- System(s) affected: reproductive
- Older patients with associated medical problems are at high risk from radical surgery. The surgery, however, is usually well tolerated.
- Patients who are not surgical candidates can be treated with combination chemotherapy and/or radiation.
- In the very elderly, palliative vulvectomy provides relief of symptoms for ulcerating symptomatic advanced disease.
- In 2015, 5,150 women were diagnosed with vulvar cancer and 1,080 women died from vulvar cancer in the United States (1); accounting for approximately 4% of all gynecologic malignancies (2)
- Estimated 6,020 new cases and 1,150 deaths in 2017
- Surveillance, epidemiology, and end result (SEER) data showed that the incidence of in situ vulvar carcinoma increased by >400% between 1973 and 2000.
- Mean age at diagnosis 65 years; in situ disease: mean age 40 years; invasive malignancy: mean age 60 years
- Ethnic distribution: more common in Caucasian women than in any other race
Etiology and Pathophysiology
- Patients with cervical cancer are more likely to develop vulvar cancer later in life, secondary to “field effect” phenomenon with a carcinogen involving the lower genital tract.
- HPV has been associated with squamous cell abnormalities of the cervix, vagina, and vulva; 55% of vulvar cancers are attributable to oncogenic HPV, predominantly HPV 16 and 33; 92% of VIN 2/3, vaginal intraepithelial neoplasia (VAIN) 2/3, and anal intraepithelial neoplasia (AIN) are attributable to HPV.
- Squamous cell carcinoma
- The warty basaloid type, also known as bowenoid type, is related to HPV infection and occurs in younger women. Although traditionally graded in a three-level system, the International Society for the Study of Vulvovaginal Disease voted not to use a grading system for VIN in 2004. This system eliminated VIN 1 and combined VIN 2 and VIN 3. This is based on the fact that VIN 1 is not reproducible. VIN 2 and VIN 3 were combined because VIN 2 is rare and carries the same risk of progression to malignancy as VIN 3.
- The more common variant is simplex or differentiated and does not appear to be related to HPV, occurs in older age groups, and associated with lichen sclerosus and chronic venereal diseases. It is thought to carry a higher risk of progression to malignancy.
- Melanoma, second most common histology, often identified in postmenopausal women; often pigmented but can be amelanotic, arising de novo, often found on clitoris or labia minora
- Cases not associated with HPV infection are typically associated with vulvar dystrophies causing chronic inflammation, such as lichen sclerosus or squamous cell hyperplasia.
- 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.
No known genetic pattern
- VIN or cervical intraepithelial neoplasia (CIN)
- Lichen sclerosus (vulvar dystrophy)
- HPV infection, condylomata or sexually transmitted diseases (STD) in the past
- Low economic status
- Autoimmune processes
- Immunodeficiency syndromes or immunosuppression
- Northern European ancestry
- HPV vaccination has the potential to decrease vulvar cancer by 60% (3).
- Abstinence from smoking/smoking cessation counseling
Commonly Associated Conditions
- Patients with invasive vulvar cancer are often elderly and have associated medical conditions.
- High rate of other gynecologic malignancies