Vulval cancer is a rare cancer affecting the external female genital organs, but with early detection and proper treatment, many women have positive outcomes and can lead fulfilling lives after diagnosis.
Understanding Vulval Cancer
Vulval cancer, sometimes spelled vulvar cancer, is a disease where cells in the vulva—the outer part of the female genital organs—begin to grow out of control. The vulva includes all the visible external structures such as the inner and outer lips (called the labia), the clitoris, the opening of the vagina, the opening where urine exits the body (the urethra), and the area of skin between the vagina and the anus. This cancer typically develops slowly over several years, giving healthcare providers opportunities to detect abnormal changes before cancer fully develops.[1][2]
When doctors talk about vulval cancer, they are most often referring to cancers that start in the skin cells of the vulva. Before cancer develops, abnormal cells may appear in the surface layers of the skin. This condition is called vulvar intraepithelial neoplasia, or VIN for short. VIN is not cancer itself, but it can turn into cancer if left untreated, although this process may take many years. Because of this slow progression, regular checkups and early detection can make a significant difference in preventing the disease from advancing.[6][7]
How Common Is This Cancer?
Vulval cancer is considered rare when compared to other types of cancer. In the United States, healthcare providers diagnose approximately 6,500 new cases each year. Together with vaginal cancer, vulval cancers account for about 7% of all gynecologic cancers diagnosed in the country. This means that while vulval cancer does affect thousands of women annually, it is not among the most frequently occurring cancers.[2][3][8]
The disease predominantly affects older women. Nearly 80% of people diagnosed are over the age of 50, and more than half of all diagnoses occur in women over age 70. The average age at diagnosis is 68 years. Most commonly, the cancer is diagnosed between the ages of 65 and 74. This age pattern suggests that vulval cancer is largely a condition that develops later in life, although younger women are not entirely exempt from the disease, especially when certain risk factors are present.[2][8]
What Causes Vulval Cancer?
The exact cause of vulval cancer is not fully understood, but researchers have identified two main pathways through which the disease typically develops. One pathway involves infection with certain strains of the human papillomavirus, commonly known as HPV. This is a virus that spreads through skin-to-skin contact, particularly during sexual activity. High-risk types of HPV can cause changes in the cells of the vulva that eventually lead to cancer. Between 30% and 40% of vulval cancer cases are linked to HPV infection. The virus produces proteins that interfere with the body’s natural tumor suppression mechanisms, allowing cells to multiply without proper control.[2][8]
The second pathway involves chronic inflammation and skin conditions of the vulva that are not related to HPV. One such condition is lichen sclerosus, which causes patches of thin, white skin on the vulva. Women with lichen sclerosus or other long-term inflammatory conditions of the vulva face an increased risk of developing vulval cancer over time. This type of vulval cancer tends to occur in older women and is not associated with viral infections.[2]
It is important to understand that having one of these conditions does not mean cancer will definitely develop. Many women with HPV infections or lichen sclerosus never develop vulval cancer. However, these conditions do increase the likelihood, which is why regular monitoring and treatment of precancerous changes are so important.
Who Is at Higher Risk?
Several factors can increase a woman’s chances of developing vulval cancer. The most significant risk factor is advancing age. As women get older, their risk increases substantially, particularly after age 50. This is likely because cellular changes accumulate over time, and the body’s ability to repair damaged cells may decline with age.[8][10]
Infection with high-risk strains of HPV is another major risk factor. Women who have had multiple sexual partners or who began having sexual intercourse at a young age have a higher chance of being exposed to HPV. Having a history of genital warts, which are also caused by HPV, increases risk as well. Similarly, women with a history of abnormal Pap smears may be at higher risk because these tests can indicate the presence of HPV-related changes in the cervix, suggesting possible exposure to the virus.[8][10]
Smoking is another important risk factor. Women who smoke are more likely to develop vulval cancer than non-smokers. Tobacco use can weaken the immune system and make it harder for the body to fight off HPV infections and other cellular changes. Women with weakened immune systems due to conditions like HIV or because they are taking immunosuppressive medications also face increased risk.[8][9]
Having inflammatory conditions of the vulva, such as lichen sclerosus, increases risk, particularly in older women. Women who have previously received radiation therapy to the pelvic area for other cancers may also have a higher chance of developing vulval cancer later in life.[8]
Recognizing the Symptoms
Vulval cancer often does not cause symptoms in its earliest stages, which is why regular checkups are so important. However, as the cancer develops, several signs may appear. The most common symptom is persistent itching in the vulvar area that does not go away with standard treatments. This itching can be intense and bothersome, affecting daily activities and quality of life.[1][2]
Women may also notice changes in the appearance of their vulvar skin. These changes can include patches of skin that look darker or lighter than usual, or areas of white, thickened, or rough skin. Some women develop visible lumps or growths on the vulva that may resemble warts or moles. Open sores or ulcers that do not heal are another warning sign. These growths or sores most commonly appear on the outer lips of the vagina, but they can develop anywhere on the vulva.[1][2][6]
Bleeding that is not related to menstrual periods is another symptom that should prompt a visit to a healthcare provider. This bleeding might occur spontaneously or after activities like sexual intercourse. Pain and tenderness in the vulvar area, particularly during sex or while urinating, can also be signs of vulval cancer. Some women experience a burning sensation in the vulva that persists despite treatment attempts.[1][2]
It is crucial to remember that many of these symptoms can also be caused by conditions that are not cancer, such as infections or other benign skin conditions. However, any persistent or concerning changes in the vulvar area should be evaluated by a healthcare provider. Early detection significantly improves treatment outcomes, so it is always better to get unusual symptoms checked rather than waiting to see if they resolve on their own.[2]
Preventing Vulval Cancer
While not all cases of vulval cancer can be prevented, there are several steps women can take to reduce their risk. One of the most effective preventive measures is vaccination against HPV. HPV vaccines protect against the high-risk strains of the virus that are most commonly linked to vulval and other genital cancers. These vaccines are most effective when given before a person becomes sexually active, but they can still provide benefits to those who have already been exposed to some strains of the virus. The vaccination is typically recommended for preteens and young adults, but older individuals may also benefit from discussing vaccination with their healthcare provider.
Quitting smoking or never starting is another important preventive step. Smoking not only increases the risk of vulval cancer but also makes it harder for the body to clear HPV infections and repair damaged cells. Women who smoke should consider seeking support to quit, as this will improve their overall health in addition to reducing cancer risk.
Practicing safe sex by using condoms and limiting the number of sexual partners can reduce the risk of HPV infection, though it cannot eliminate the risk entirely since HPV spreads through skin-to-skin contact. Regular gynecological examinations are also important, as healthcare providers can detect early changes in the vulvar skin during routine checkups. Women with conditions like lichen sclerosus should work closely with their healthcare providers to manage these conditions and monitor for any concerning changes.[10]
Being aware of your body and performing self-examinations can also help with early detection. If you notice any unusual changes in the appearance or feel of your vulvar skin, or if you experience persistent symptoms like itching, burning, or pain, seek medical attention promptly. Early detection of precancerous changes or early-stage cancer significantly improves the chances of successful treatment.
How Vulval Cancer Affects the Body
In vulval cancer, the normal process of cell growth and death becomes disrupted. Healthy cells in the body grow, divide, and die in an orderly fashion, with old or damaged cells being replaced by new ones. In cancer, however, cells begin to multiply out of control and do not die when they should. These abnormal cells accumulate and form tumors or growths on the vulva.[2]
In HPV-related vulval cancer, the virus introduces proteins into the cells that disable important tumor suppressor mechanisms. Specifically, HPV produces proteins called E6 and E7 that interfere with the p53 and RB genes, which normally prevent cells from growing uncontrollably. When these protective mechanisms are disabled, cells can divide without regulation, leading to the development of precancerous changes and eventually cancer.[8]
In non-HPV-related vulval cancer, chronic inflammation and irritation of the vulvar skin can lead to genetic changes in the cells over time. This prolonged irritation causes cells to repeatedly repair themselves, and errors can occur during this repair process. Eventually, these errors may result in cells that grow abnormally and form cancerous tumors.
Most vulval cancers develop in the squamous cells, which are the flat, thin cells that make up the outer layer of the skin. About 90% of vulval cancers are squamous cell carcinomas. A smaller percentage, about 5%, are melanomas, which develop in the pigment-producing cells of the skin. Melanomas tend to grow more rapidly and have a higher risk of spreading to other parts of the body compared to squamous cell carcinomas.[2][7]
If left untreated, vulval cancer cells can spread beyond the vulva to nearby structures such as the vagina, urethra, or anus. Cancer cells can also travel through the body’s drainage system, called the lymphatic system, to lymph nodes in the groin area. From there, they may spread to more distant parts of the body, including other organs. This is why early detection and treatment are so important—they can prevent the cancer from advancing to more difficult-to-treat stages.[2]
