This page was reviewed under our medical and editorial policy by
Maurie Markman, MD, President, Medicine & Science
This page was updated on June 7, 2022.
Vulvar cancer is relatively rare, representing about 6 percent of all cancers that occur in the female reproductive organs and less than 1 percent of all cancers affecting women. The lifetime risk for developing vulvar cancer is about 1 in 333, according to the American Cancer Society.
While the exact cause of many vulvar cancers may not be known, several factors may increase the risk for developing the disease.
Age: Approximately 80 percent of vulvar cancer cases occur in women who are over the age of 50, and over 50 percent of cases occur in women age 70 or older.
Cervical cancer: A previous diagnosis of cervical cancer may increase the risk of vulvar cancer. This higher risk may be related to compounding risk factors, such as HPV infection and smoking.
Human papillomavirus (HPV): HPV is a group of more than 100 viruses that are sexually transmitted and have been associated with vulvar cancers, as well as other cancers of the reproductive system. Some of the more common HPV strains cause noncancerous warts (papillomas), while other types of HPV infections may have no visible symptoms. HPV types 16, 18 and 31 have been most strongly linked to cancer, and these are known as high-risk HPV viruses. Approximately 70 percent of all vulvar cancers are linked to infection with a high-risk virus, and these are more commonly seen in younger women. Protection against HPV infection may help many women reduce their vulvar cancer risk.
Lichen sclerosus: This condition is characterized by thin, itchy vulvar skin, and it slightly increases a woman’s vulvar cancer risks.
Smoking: Smoking is considered a risk factor for developing vulvar cancer, particularly in women who have already been infected with a high-risk HPV virus.
Vulvar intraepithelial neoplasia (VIN): This is a precancerous condition in which abnormal cells are restricted to the top layer of skin of the vulva (also known as the epithelium). VIN, previously called dysplasia, is generally associated with HPV infection. While the presence of VIN increases the risk for developing invasive vulvar cancer, not all women with VIN will develop cancer. However, it is important to treat the condition and to get regular gynecologic check-ups.
Women who have an inherited disorder called Fanconi anemia have an increased risk for developing vulvar cancer.
Next topic: What are the symptoms of vulvar cancer?