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By Mayo Clinic staffThe exact cause of each type of vulvar cancer isn't known. Vulvar cancers that occur in older women that aren't linked to HPV infection may be related to a mutation or defect in the p53 tumor suppressor gene. This gene plays a role in keeping cells from becoming cancerous. This type of cancer may also be seen in women with lichen sclerosus — a condition that causes the vulvar skin to become thin and itchy.
As many as 30 percent to 50 percent of vulvar cancers have been linked to the sexually transmitted HPV infection. Many times these women have a precancerous skin condition called vulvar intraepithelial neoplasia in more than one area of the vulva before developing cancer.
Vulvar cancer is uncommon, accounting for less than 1 percent of all cancers in American women.