Vulvar Cancer

Vulvar cancer is a rare cancer that forms in the vulva, the external female genital organs. The vulva includes the inner and outer lips of the labia, the clitoris, the opening of the vagina and the vaginal glands, which sit in the perineum, the area between the vulva and the anus. Vulvar cancer most often develops in the outer lips of the labia.

About half of all vulvar cancers are linked to an infection with certain types of human papillomavirus (HPV). Vulvar cancer often grows slowly over many years, and precancerous changes may appear first.

Types & Stages

Types of vulvar cancer include:

• Squamous cell carcinoma is the most common type of vulvar cancer. It begins in the flat cells that make up the surface of the vulvar skin. There are several subtypes, including keratinizing, basaloid, warty and verrucous. These subtypes differ in how the cells look and behave.
   
• Adenocarcinoma begins in gland cells. Vulvar adenocarcinoma most often starts in the Bartholin glands, which are found just inside the opening of the vagina. It can also begin in the sweat glands of the vulvar skin.
   
• Paget disease of the vulva is a condition in which adenocarcinoma cells are found in the top layer of the vulvar skin. Some people with vulvar Paget disease also have an invasive adenocarcinoma in a Bartholin gland or sweat gland.

Vulvar cancer stages describe how far the cancer has spread:

• Stage I means the cancer is found only in the vulva or perineum and has not spread to lymph nodes or other areas.
   
• Stage II means the cancer has spread to nearby tissues, such as the lower part of the vagina, urethra or anus, but not to lymph nodes.
   
• Stage III means the cancer has spread to nearby lymph nodes in the groin.
   
• Stage IV means the cancer has spread more deeply into nearby structures, such as the upper urethra, bladder or rectum, or to distant parts of the body.

Your care team will use the cancer type, stage and your overall health to recommend a treatment plan.

Signs & Symptoms

Visit a doctor if you are experiencing symptoms that are new, unusual for you or do not go away.

Symptoms of vulvar cancer may include:

• Itching that does not go away
• Burning, pain or tenderness in the vulva
• Changes in the color or texture of the vulvar skin
• A lump, bump or wart-like growth
• A sore or ulcer that does not heal
• Bleeding or discharge not related to your period
• A thickened or raised patch of skin
• A lump or swelling in the groin

Many of these symptoms can be caused by conditions that are not cancer. Still, it is important to pay attention to your body. If you notice changes that are not normal for you, talk with a healthcare provider.

Diagnosis

Tests and procedures used to diagnose vulvar cancer may include:

• Pelvic and vulvar exam. Your doctor performs a physical exam of the vulva to look for anything concerning, such as a lump, sore or change in the skin. The doctor also checks nearby lymph nodes in the groin.
   
• Magnified exam. Your doctor may use a special magnifying instrument called a colposcope to look closely at the vulva. This exam is called vulvoscopy. During the exam, your doctor may apply a mild solution, such as a dilute vinegar-like liquid or a special dye, to help abnormal areas show up more clearly.
   
• Biopsy. A biopsy removes a small sample of tissue for testing in a lab. For vulvar cancer, a biopsy involves removing a sample of skin, often after the area is numbed with local anesthetic. This can usually be done in your doctor’s office. A pathologist then examines the tissue under a microscope to check for cancer or precancer cells.
   
• Imaging tests. If cancer is found or suspected to have spread, imaging tests such as CT, MRI or PET scans may be used. These tests help your doctor learn the size of the cancer, whether nearby lymph nodes are involved and whether the cancer has reached other parts of the body.

Treatment

Treatment for vulvar cancer depends on the type and stage of cancer, whether it has spread, your overall health and your personal goals. Your care team can help explain the benefits and risks of each option.

Treatment may include:

• Wide local excision or radical excision is a procedure that removes the cancer along with a margin of normal tissue around it. Removing some healthy tissue helps lower the chance that cancer cells are left behind.
   
• Vulvectomy is surgery to remove all or part of the vulva. In a partial vulvectomy, only the part of the vulva where the cancer is located is removed. In a simple vulvectomy, the entire vulva is removed. In a radical vulvectomy, part or all of the vulva and nearby tissue is removed.
   
• Sentinel lymph node dissection removes only the first lymph nodes that drain the area near the cancer. These nodes are checked for cancer cells, which helps show whether the cancer has begun to spread.
   
• Complete lymph node dissection removes all of the lymph nodes near the vulva. This may be done on one or both sides of the perineum, depending on where the cancer is located.
   
• Radiation therapy uses high-energy beams to kill cancer cells. It may be used before surgery to shrink a tumor, after surgery to lower the risk of the cancer returning or together with chemotherapy.
   
• Chemotherapy uses medicines to kill cancer cells or slow their growth. It may be combined with radiation or used for cancer that has spread or returned.
   
• Immunotherapy helps the immune system recognize and attack cancer cells. It may be an option for some advanced or recurrent vulvar cancers with certain tumor features.
   
• Targeted therapy uses medicines that focus on specific features of cancer cells. It may be an option for some advanced or recurrent vulvar cancers.

Clinical trials may also be available. These research studies test new treatments or new ways to use current treatments. Ask your care team whether a clinical trial may be right for you.

Causes & Risk Factors

The exact cause of vulvar cancer is not always known. It often develops slowly, and precancerous changes may appear before cancer forms.

Factors that may increase vulvar cancer risk include:

• Age. The risk of vulvar cancer increases as you get older. More than half of cases occur in women over age 70, though precancers are often found in younger women.
   
• HPV infection. Certain high-risk types of HPV are linked to about half of all vulvar cancers. HPV-associated vulvar cancers tend to develop in younger women.
   
• Smoking. Smoking exposes the body to harmful chemicals and increases the risk of vulvar cancer. The risk is even higher in people who also have an HPV infection.
   
• HIV or a weakened immune system. HIV and other causes of a weakened immune system can make it harder for the body to clear an HPV infection, which may raise the risk of vulvar precancer and cancer.
   
• Vulvar intraepithelial neoplasia (VIN). VIN, also called vulvar squamous intraepithelial lesion, is a precancerous condition in which abnormal cells are found in the surface layer of the vulvar skin. Most cases do not become cancer, but treatment or close follow-up is often recommended.
   
• Lichen sclerosus. This inflammatory condition causes the vulvar skin to become thin and itchy. It is linked to a small increase in vulvar cancer risk.
   
• History of other genital cancers. A history of cervical cancer or other genital cancers may increase the risk of vulvar cancer, partly because these cancers share certain risk factors, such as HPV infection and smoking.

You can take steps that may help lower your risk, such as getting the HPV vaccine, not smoking and treating precancerous changes when they are found. Talk with your healthcare provider about your personal risk.

Screening

There is no routine screening test for vulvar cancer for people who do not have symptoms.

A Pap test screens for cervical cancer, not vulvar cancer. There is currently no simple, reliable test to find vulvar cancer before symptoms appear.

Because there is no screening test, paying attention to your body is one of the most important things you can do. During a routine pelvic exam, your provider may check the vulva for changes, and you can talk with your provider about anything unusual you notice.

People with a higher risk, such as those with VIN, lichen sclerosus, HIV or a history of other genital cancers, should talk with a healthcare provider about close follow-up and how often they should be checked.

If you notice itching, pain, a lump, a sore that does not heal, or any other change in your vulva that is not normal for you, do not wait. Talk with a healthcare provider so the cause can be found and treated early.