Vulvar Cancer

What is vulvar cancer?

Vulvar cancer is a rare cancer that forms in the tissues of your vulva. “Vulva” is the collective name for all the female external sex organs, or genitals. Your vulva includes:

• Opening of your vagina: The muscular canal for sex, childbirth and menstruation (periods).
• Labia minora (inner lips): Tissue folds that surround your vaginal opening and extend above your clitoris.
• Labia majora (outer lips): Fleshy area that surrounds your inner lips.
• The outer part of your clitoris: The sexually sensitive nub of flesh above your vaginal opening.
• Mons pubis: The rounded area in front of your pubic bones that becomes covered with hair at puberty.
• Opening of the urethra: The tube that allows urine (pee) to exit your body.
• Perineum: The patch of skin between your vagina and anus (butthole).

Vulvar cancer usually develops slowly over several years. Precancerous areas of tissue (lesions) typically develop first. Healthcare providers usually discover the abnormal growth in the outermost layer of your skin. These precancerous lesions are called vulvar intraepithelial neoplasia (VIN).

Types of vulvar cancer

Healthcare providers classify vulvar cancer based on the type of cells where the cancer starts. The most common types of vulvar cancer include:

• Vulvar squamous cell carcinoma: Approximately 90% of vulvar cancers are squamous cell carcinomas. They develop in the cells on the surface of your skin.
• Vulvar melanoma: Approximately 5% of vulvar cancers are melanomas. Melanomas develop rapidly and have a high risk of spreading to other areas of your body.

The remaining vulvar cancers are rare. They include:

• Basal cell carcinoma.
• Bartholin gland adenocarcinoma.
• Paget disease of the vulva.
• Sarcoma.
• Verrucous carcinoma.

How common is vulvar cancer?

Vulvar cancer is rare. Healthcare providers diagnose just under 6,500 new cases of vulvar cancer in the U.S. each year.

Nearly 80% of people diagnosed are over age 50, and over half of all diagnoses are in people over age 70. The average age at diagnosis is 68.

What are the symptoms of vulvar cancer?

The first noticeable signs of vulvar cancer are usually skin changes on your outer lips (labia majora) or inner lips (labia minora). But cancer can form anywhere on your vulva.

Vulvar cancer symptoms include:

• Color changes, including skin that looks darker or lighter than usual, or patches of white skin.
• Thickened or rough skin patches.
• Growths, including lumps, wart-like bumps or ulcers that don’t heal.
• Itching or burning that doesn’t improve.
• Bleeding that’s unrelated to menstruation (periods).
• Tenderness and pain, potentially during sex or when you’re peeing.

See your healthcare provider if you have one or more of these symptoms. Vulvar cancer symptoms usually don’t appear in the early stages, so it’s important to get checked as soon as possible.

Still, many of these symptoms are also common in noncancerous conditions. Your provider can tell you whether these changes are signs of vulvar cancer or a different condition.

What causes vulvar cancer?

With vulvar cancer, cells begin multiplying out of control. Without treatment, these cancer cells can spread to other parts of your body.

The most common type of vulvar cancer, vulvar squamous cell carcinoma, arises in association with one of two conditions:

• Human papillomavirus (HPV) infection: A common sexually transmitted infection (STI) that spreads through skin-to-skin contact. Some types of HPV increase your risk of certain cancers, including cervical cancer, anal cancer, rectal cancer and vulvar cancer.
• Lichen sclerosus: A chronic (lifelong) skin condition. Lichen sclerosus causes inflammation and other symptoms, such as skin changes and itching, on your vulva.

Risk factors

Risk factors for vulvar cancer include:

• Age: Your likelihood of developing vulvar cancer increases with age.
• Exposure to HPV: Not all strains of HPV cause cancer, but some can lead to cell changes that eventually become vulvar cancer.
• Skin conditions involving your vulva: Growths associated with lichen sclerosus may progress to vulvar cancer.
• Vulvar intraepithelial neoplasia (VIN): VIN is a precancerous condition that can progress to vulvar cancer if it’s not treated.
• Human immunodeficiency virus (HIV) infection: A weakened immune system from a condition like HIV can make it harder for your body to fight cancer.
• Smoking: Smoking raises your risk of developing multiple cancer types, including vulvar cancer.

How is vulvar cancer diagnosed?

Your healthcare provider will ask about your medical history, potential risk factors and symptoms. Diagnosis often involves multiple tests.

Tests to diagnose vulvar cancer

Tests may include:

• Pelvic exam: Your provider will visually inspect your vulva, checking for unusual skin changes. They’ll insert one or two gloved, lubricated fingers inside your vagina to feel for any lumps or other signs of cancer. They may use a similar technique to check your rectum. They may use a tool called a speculum to widen your vagina so they can check for abnormalities.
• Pap smear: Your provider may take a sample of cells during the pelvic exam and test them for cancerous changes. They may perform an HPV test on the sample to see if you have an infection.
• Colposcopy: Your provider may use a lighted, magnifying instrument called a colposcope to view your vulva, vagina and cervix (the organ between your vagina and uterus) in more detail. They may apply a special solution that can highlight abnormal cells, making them easier to see.
• Biopsy: Your provider may remove a sample of abnormal tissue to test it for cancer cells. A biopsy is the only way to know for sure whether you have vulvar cancer.

Tests to determine cancer spread

If you have cancer, your provider will perform additional tests to see if it’s spread beyond your vulva. Without treatment, vulvar cancer may spread to your vagina or other nearby organs, lymph nodes in your pelvis and eventually your bloodstream. Cancer that’s spread (metastatic cancer) is harder to treat.

Tests may include:

• Scope exams: You may receive a cystoscopy to check for cancer spread in your urethra (the tube that carries your pee) or bladder. A proctoscopy checks for cancer cells in your rectum or anus.
• Imaging tests: X-rays, computed tomography (CT) scans, magnetic resonance imaging (MRI) and positron emission tomography (PET) scans can show if the cancer has spread from your vulva to other tissues.
• Sentinel node biopsy: Your provider may remove the lymph node closest to your tumor (the sentinel node) to test for cancer cells. With vulvar cancer, tumors usually drain into sentinel lymph nodes in your groin.

What are the stages of vulvar cancer?

Vulvar cancer staging allows healthcare providers to determine if your cancer’s spread beyond your vulva. This information guides treatment decisions. There are four main stages:

• Stage I: Early-stage vulvar cancer is only on your vulva or perineum (area between your rectum and vagina). Stage I consists of Stages IA or IB based on tumor size and how far it reaches into nearby tissue.
• Stage II: The tumor (of any size) has spread into the lower part of your urethra, the lower part of your vagina or anus.
• Stage III: Cancer has spread to one or more nearby lymph nodes. Stage III consists of Stages IIIA, IIIB, and IIIC based on the number and size of the lymph nodes involved.
• Stage IV: Cancer has spread into the upper part of your urethra, vagina or other body parts. Stage IV consists of Stages IVA and IVB based on if the spread is localized near your vulva or spread distantly.

Ask your healthcare provider to explain the details of what your cancer stage means for your treatment.

How is vulvar cancer treated?

Your treatment depends on factors like your general health, cancer stage and whether your healthcare provider recently diagnosed your cancer or if it’s recurred (come back). Your provider can explain how your treatment plan is best suited for your diagnosis.

Surgery

Surgery is the most common treatment for cancer of the vulva. The goal is to remove all the cancer while preserving your sexual function. Types of surgery include:

• Laser surgery: This surgery uses a laser beam to make bloodless cuts in tissue or to remove cancerous surface lesions.
• Local excision: This surgery removes the cancer and a small-to-large amount of normal tissue around the cancer. Sometimes, providers remove nearby lymph nodes to test for cancer cells or to remove lymph nodes when there’s evidence of cancer.
• Vulvectomy: This procedure removes part or all of your vulva and possibly some nearby lymph nodes. Your provider may use skin grafts to replace removed skin.
• Pelvic exenteration: This surgery removes your lower colon, rectum, bladder, cervix, vagina, ovaries and nearby lymph nodes. Your healthcare provider will create openings to allow urine and stool to flow from your body into a collection bag.

Radiation therapy

Radiation therapy uses X-rays or other high-energy sources to kill cancer cells. The most common delivery method for vulvar cancer treatment is external beam radiation therapy (EBRT). EBRT uses a machine to deliver radiation through your skin to the targeted cancer site.

Often, people receive radiation therapy and chemotherapy together (chemoradiation). You may receive radiation before surgery to shrink a tumor or after surgery to destroy any remaining cancer cells.

Chemotherapy

Chemotherapy uses drugs to attack cancer cells throughout your body. Your healthcare provider may inject the medicine into a vein or muscle, or you may take a pill. You may receive a lotion that you can apply directly to your vulva. This form of chemotherapy attacks cancer more locally — in the specific area.

Cisplatin (Platinol®, Platinol -AQ®) and fluorouracil (Carac®) are commonly prescribed chemotherapy drugs for vulvar cancer.

Immunotherapy

Immunotherapy helps your body’s immune system identify cancer cells and fight them more effectively. Imiquimod cream (Aldara®, Zyclara®) is a common immunotherapy medication used to treat vulvar cancer.

What follow-up should I expect after vulvar cancer treatment?

Your healthcare provider may perform tests at various checkpoints after treatment to monitor your condition and ensure the cancer hasn’t returned.

Testing often involves the same procedures used to diagnose and stage vulvar cancer.

Can vulvar cancer be prevented?

The best way to reduce your risk is to get the HPV vaccine to prevent infections. In the U.S., adults up to age 45 may receive Gardasil 9® depending on their risk of HPV exposure. Cervarix® and Gardasil® are HPV vaccines available in other countries.

See your healthcare provider right away if you develop any symptoms of vulvar cancer. Schedule regular checkups, including a physical exam, at least annually for your gynecological health.

Is vulvar cancer serious?

It can be. Untreated vulvar cancer is life-threatening. Cancer that’s spread to your lymph nodes or other body parts is much harder to treat than cancer diagnosed early.

While there’s always a risk that cancer may return after treatment (recur), most people who receive treatment in the early stages of the disease remain cancer-free.

What’s the survival rate for vulvar cancer?

The relative five-year survival rate for people with vulvar cancer is approximately 70%. But survival rates are higher when people are diagnosed and treated in the early stages. For example, the five-year survival rate for localized cancer (remaining in the vulva) is approximately 86%. The five-year survival rate drops to approximately 30% once the cancer spreads.

Still, your prognosis depends on factors unique to your diagnosis, including your health and your response to treatment. Ask your healthcare provider about likely outcomes based on your diagnosis.

How quickly does vulvar cancer progress?

Most types of vulvar cancer progress slowly over several years. Less common types, like melanomas, tend to grow and spread more quickly.

How do I take care of myself?

Many people feel self-conscious about visible changes to their vulva. Still, having vulvar cancer doesn’t mean you must abandon physical intimacy. Don’t be ashamed to ask your healthcare provider how your diagnosis may affect your sex life. They can connect you with resources that support your physical and emotional needs as you navigate your diagnosis and treatment.

Where does vulvar cancer usually start?

Vulvar cancer usually starts on the surface of the skin surrounding your vagina, either your outer lips (labia majora) or inner lips (labia minora). Less commonly, it forms on other parts of your vulva, like your Bartholin gland and clitoris.

What is the first stage of vulvar cancer?

Stage I is the first stage of vulvar cancer. In this stage, cancer hasn’t spread beyond your vulva or perineum. Stage IA vulvar cancer is two centimeters (peanut size) or smaller. It hasn’t spread beyond one millimeter (tip of a pencil-size) into nearby tissue beyond one millimeter. Stage IB vulvar cancers involve larger tumors that may have invaded more deeply into nearby tissue.