Vulvar cancer is a rare type of slow-growing cancer that forms on the exterior area of the female genitals (the vulva). It’s most commonly diagnosed in older adults.

Vulvar cancer doesn’t always cause symptoms in its early stages. Symptoms that may result include:

  • itching in the area that doesn’t subside
  • tenderness, discomfort, or pain
  • a lump on the vulva
  • bleeding in the area that isn’t menstruation
  • painful urination
  • changes in the skin of the vulva, such as thickening or discoloration
  • growths that look like warts
  • an ulcer (open sore) in the area that doesn’t heal after a month

See your doctor if you experience any of these symptoms.

Although doctors aren’t 100 percent clear on what causes vulvar cancer, it’s believed that DNA defects (mutations) play a major role.

These mutations turn on genes that promote cell division (oncogenes) and turn off genes that slow down cell division (tumor suppressor genes). This causes the mutated cells to divide and to grow uncontrollably.

The DNA mutations related to vulvar cancer are believed to happen during life as opposed to being inherited. For example, tobacco smoke could be responsible for acquired DNA mutations.

Vulvar squamous cell carcinoma

According to the American Society of Clinical Oncology (ASCO), squamous cell cancer of the vulva is a skin cancer that appears most of the time on the labia. This type of cancer accounts for about 90 percent of vulvar cancers.

Squamous cell carcinoma appears to develop due to these two factors:

  • Human papillomavirus (HPV). HPV seems to have an important role in about half the cases of vulvar cancer.
  • Tumor suppressor gene p53. DNA tests from vulvar cancers that might not be due HPV infection often show a mutation in the p53 gene.

Other types of vulvar cancers

The following are other types of cancers that can occur in the vulva:

  • adenocarcinoma
  • melanoma
  • sarcoma
  • verrucous carcinoma

While the exact cause of vulvar cancer hasn’t yet been determined, there are factors that could increase risk of developing the disease:

  • Age. The average age at diagnosis is 64 years. Although it can occur at any age, risk does increase as you age.
  • HPV. A sexually transmitted infection, HPV appears to increase the risk of vulvar, cervical, and other cancers.
  • Lichen sclerosus. If you have lichen sclerosus — a skin condition that makes vulvar skin itchy and thin — you are at a higher risk.
  • History. If you’ve ever had a vulvar intraepithelial neoplasia (a type of precancerous change on the surface of the vulva), you are at higher risk.
  • Smoking. Smoking tobacco products like cigarettes increase the risk of a number of cancers, including vulvar cancer.
  • Weakened or suppressed immune system. You’re at a higher risk if you have a weakened immune system from a condition, such as living with HIV, or take medications to suppress your immune system, such as for an organ transplant.

At this time, the best way to protect yourself from vulvar cancer is to reduce your risk of HPV infection. You can do this by:

  • using a condom during sexual encounters
  • getting the HPV vaccine

There are certain steps you can take to lower your risk for getting vulvar cancer, such as not smoking and getting the HPV vaccine.

Other steps you can take include talking with your doctor about your risk factor of cancer in general and specifically vulvar cancer. Among other suggestions, your doctor might recommend an appropriate schedule for pelvic examinations.