What is vulvar cancer?

Vulvar cancer is a rare cancer that forms in the tissues of the vulva.

The vulva is part of a woman's external genitalia. It is the area around the opening of your vagina and includes:

• Inner (labia minora) and outer lips (labia majora) of the vagina.
• Clitoris (sexually sensitive tissue between the lips).
• Opening of the vagina (muscular canal for childbirth, sex and menstruation).
• Mons pubis (the rounded area in front of the pubic bones that becomes covered with hair at puberty).
• Opening of the urethra (where pee exits your body).
• Perineum (the area between the vagina and the anus).

Vulvar cancer most often develops on the inner or outer vaginal lips, but can arise in any location of the vulva.

Vulvar cancer usually develops slowly over several years. Precancerous lesions usually develop first and are discovered as abnormal cell growth in the outer most layer of skin. These precancerous lesions are called vulvar intraepithelial neoplasia (VIN).

Who gets vulvar cancer?

Your chance of getting vulvar cancer is increased if you:

• Have vulvar intraepithelial neoplasia (VIN).
• Have human papillomavirus (HPV) infection.
• Have a history of genital warts.
• Have a skin condition involving the vulva, such as lichen sclerosus.
• Are older.

Other possible risk factors include the following:

• Having many sexual partners.
• Having a history of abnormal Pap tests (Pap smears).
• Having a medical condition that weakens your immune system.
• Being a smoker.

Vulvar cancer is most frequently diagnosed in women aged 65 to 74. However, vulvar cancer is becoming more common in younger women.

How common is vulvar cancer?

Vulvar cancer is rare. Compared with other types of cancer, it ranks 28th in terms of the most common types of cancer. There are about 6,000 new cases of vulvar cancer in the U.S. each year.

Are there different types of vulvar cancer?

About 90% vulvar cancers are squamous cell carcinomas and develop on the surface of the vulva. About 5% of vulvar cancers are melanomas. Melanomas develop rapidly and have a high risk of spreading to other areas of the body. They are more likely to affect younger women than older women. The remaining 5% are other rare types of vulvar cancer.

What are the signs and symptoms of vulvar cancer?

Vulvar cancer may not cause noticeable early symptoms.

If you have one or more of the following symptoms, see your healthcare provider for an examination.

• Changes in the vulvar skin color (more red or white/pale than normal).
• Growths or lumps in the vulva that look like a wart or ulcer; or a rash or other sore that doesn’t heal.
• Itching or burning in the vulvar area that does not go away.
• Bleeding in the vulvar area not related to menstruation (periods).
• Tenderness in the vulvar area.
• Pelvic pain while having sex or peeing.

What causes vulvar cancer?

About half the cases of vulvar squamous cell cancer are caused by human papillomavirus. The remaining half are related to a chronic skin condition called lichen sclerosus.

How is vulvar cancer diagnosed?

First, your healthcare provider will ask about your health, including current and past illnesses, medical conditions, treatments and family history of disease. You’ll also get a physical exam, checking your general overall health. Other exams and tests include:

Vulva and pelvic exam

You’ll have a physical examine of your vulva. Your provider will be checking for signs of disease such as lumps or anything that seems unusual. Next, a biopsy of vulvar tissue might be taken to be examined for signs of cancer.

Your healthcare provider will insert one or two gloved, lubricated fingers inside your vagina with other hand on top of your lower abdomen. Your provider will feel the size and shape of your uterus and ovaries and feel for any lumps or anything unusual. Your vagina and cervix are examined with a speculum inserted into your vagina. A Pap smear is usually done. A sample of cells collected during the Pap smear may be checked for HPV. A manual rectal exam will also be performed, feeling for lumps or anything unusual. Any abnormal areas of the vagina, cervix, or anus may be biopsied as well.

Colposcopy exam

A colposcope is a lighted, magnifying instrument that is used to visualize the vulva, vagina and cervix in more detail. The colposcope may be used during a vulvar and pelvic exam to see the tissue in more detail. Often times when the colposcope is used, a dilute acetic acid solution or a brown-colored paint in applied to the tissue of interest to highlight abnormal cells that cannot be seen with the naked eye. Biopsies, or tissues samples, may be taken of any areas that look abnormal to check for cancer.

What tests may be done to find out if vulvar cancer has spread?

If cancer is found, you’ll undergo other tests to find out if the cancer has spread to other areas of the vulva or your body. These tests may include:

Other internal scope exams

Additional internal scope exams may be ordered, including.

• Bladder and urethra exam: A cystoscope (a lighted tube with camera) will be inserted into your urethra to look for anything abnormal in your urethra and bladder and to take tissue samples if something abnormal is found.
• Rectum and anus exam: A proctoscope (a lighted tube with viewing lens) will be inserted into your rectum to look for anything abnormal in your rectum or anus and take tissue samples if something abnormal is found.

X-rays and scans

• X-rays: X-rays help show if cancer has spread to organs and bones inside the chest, and the pelvic bones.
• Intravenous pyelogram (IVP): This test uses a contrast dye, injected into a vein, and X-rays to look for blockages in your kidneys, ureters or bladder.
• CT scan (CAT scan) and MRI (magnetic resonance imaging): CT and MRI scans take detailed pictures of the inside of your body. A contrast dye can be used along with a CT scan to help see organs and tissues more clearly. MRI scans use a magnet and radio waves to make its pictures.
• PET scan (positron emission tomography scan): This scan takes pictures of the inside of your body after radioactive sugar (glucose) has been injected into a vein. The glucose collects in cancerous cells, which are easily seen in the pictures.

Lymph node biopsy

• Sentinel lymph node biopsy: In this procedure, the first few lymph nodes nearest a cancerous tumor (into which the tumor drains) are removed. A tissue biopsy is taken to find out if the cancer has spread to these lymph nodes. If no cancer is found, this means it’s unlikely that the cancer has spread and removing additional lymph nodes may not be needed.

Are there different stages of vulvar cancer?

Yes. Once vulvar cancer is diagnosed, it is categorized by its stage, which means by how far it has spread. There are four main stages:

• Stage I: In this stage, cancer is only present onthe vulva or perineum (area between the rectum and the vagina). Stage I is divided into stages, IA and IB, based on size of tumor (less or greater than two centimeters [peanut size]) and spread into the tissue (less or more than one millimeter [tip of a pencil]).
• Stage II: In this stage, the tumor (of any size) has spread into the lower part of the urethra, the lower part of the vagina or the anus. Cancer has not spread to the lymph nodes.
• Stage III: In this stage, the cancer has spread to one or more nearby lymph nodes. Stage III is further divided into stages IIIA, IIIB, and IIIC based on number and size of the lymph nodes involved.
• Stage IV: In this stage, the cancer has spread into the upper part of the urethra, the upper part of the vagina, or to other parts of the body. Stage IV is divided into stages IVA and IVB based on if the spread is localized near the vulva or spread distantly

If you’ve been diagnosed with a certain stage of cancer, ask your healthcare provider to explain the details of the specific stage and what it means.

How is vulvar cancer treated?

There are four treatment types for vulvar cancer. Factors that will help guide your healthcare provider’s choice include:

• The stage of your cancer.
• Your age and general health.
• Whether the cancer has just been diagnosed or has recurred (come back).

Your healthcare provider will discuss exactly which type of treatment and order of treatment is best suited for your stage of vulvar cancer.

Vulvar cancer treatment options include the following:

Surgery

Surgery is the most common treatment for cancer of the vulva. The goal of surgery is to remove all the cancer without any loss of 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 nearby lymph nodes are removed.
• Vulvectomy: This surgical procedure removes part or all of the vulva and possibly some nearby lymph nodes. Skin grafts may be used to replace removed skin.
• Pelvic exenteration: In this surgery, your lower colon, rectum, bladder, cervix, vagina, ovaries and nearby lymph nodes are removed. Artificial openings are created to allow urine and stool to flow from your body into a collection bag.

Surgery may be followed by chemotherapy or radiation therapy to kill any remaining cancer cells. Sometimes radiation or chemotherapy is recommended prior to or instead of surgery.

Radiation therapy

Radiation therapy kills cancer cells using high-energy x-rays or other types of radiation. External radiation therapy uses a machine to deliver radiation through your skin to the targeted cancer site. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires or catheters that are placed directly into or near the cancer. Choice of radiation therapy delivery method depends on the type and stage of the cancer being treated.

Chemotherapy

Chemotherapy is cancer-killing medications. When taken by mouth, or injected into a vein or muscle, the chemotherapy can attack cancers throughout your body. When chemotherapy is placed directly into the spinal column, in an organ or a body cavity such as the abdomen, or applied directly to the skin in a cream or lotion, the chemotherapy attacks cancer more locally – in those specific areas. Type of chemotherapy given depends on the stage and type of cancer.

Biologic therapy

Biologic therapy is a type of treatment that uses lab-made substances or substances in your body to help your body’s immune system or fight cancer. Imiquimod cream (Aldara®, Zyclara®) is an example of a biologic therapy used to treat precancerous vulvar lesions.

Are any medications approved to treat vulvar cancer?

Yes, bleomycin sulfate (Blenoxane®) is approved in the U.S. to treat squamous cell carcinoma (cancer) of the cervix and the vulva. This is not a common drug to treat vulvar cancer however, and most medications used today are considered “off-label” use, but have been studied extensively for this purpose. These medications include:

• Cisplatin.
• Carboplatin.
• Paclitaxel (Abraxane®).
• Bevacizumab (Avastin®).
• Vinorelbine (Navelbine®).
• Erlotinib (Tarceva®).
• Gemcitabine (Gemzar®).
• Pembrolizumab (Keytruda®).
• Nivolumab (Opdivo®).
• Larotrectanib (Vitrakvi®).
• Entrectanib (Rozlytrek®).

What follow up should I expect after vulvar cancer treatment?

Some of the tests that were done to diagnose vulvar cancer or determine the stage may be repeated. Some tests are repeated to see how well treatment is working and determine if changes need to be made.

You may continue to be tested at various time points after treatment has ended to see if your condition has changed and to make sure that the cancer has not returned. Please be sure to keep all of your check-up appointments and regularly scheduled exams.

How can vulvar cancer be prevented?

Vaccines are available to prevent HPV, which can cause vulvar cancer. Ask your healthcare provider about these vaccines:

• Recombinant HPV Quadrivalent Vaccine (Gardasil®). This vaccine is approved for individuals nine to 26 years of age.
• Recombinant HPV Nonavalent Vaccine (Gardasil 9®). This vaccine is approved for adults through 26 years of age. It may be given to adults up to 45 years of age if appropriate after discussing risk for new HPV infection.

There are no screening tests for vulvar cancer. The best way to reduce your risk is to be aware of the symptoms and see your healthcare provider right away if you develop any of them. Also, be sure to schedule regular checkups, including a physical exam at least annually, for your gynecological health.

What’s the survival rate for women diagnosed with vulvar cancer?

According to the National Cancer Institute, the relative five-year survival rate for women with vulvar cancer is 71%. Higher five-year survival rates are seen in earlier stage disease (over 90% for stage I) compared with late stage disease (15% for stage IV).

A note from Cleveland Clinic

The earlier a cancer is discovered and treated, the better the chance of a positive, long-term outcome. You play a large role in your own health. Become familiar with all parts of your body, even your own genital area. Knowing what you look like "down there" now can help you easily identify changes. And when you see changes, make an appointment to see your healthcare provider.