Summary

Key Points

• Vulvar cancer is a rare disease in which malignant (cancer) cells form in the tissues of the vulva.
• Having vulvar intraepithelial neoplasia or HPV infection can affect the risk of vulvar cancer.
• Signs of vulvar cancer include bleeding or itching.
• Tests that examine the vulva are used to detect (find) and diagnose vulvar cancer.
• Certain factors affect prognosis (chance of recovery) and treatment options.

Vulvar cancer is a rare disease in which malignant (cancer) cells form in the tissues of the vulva.

Vulvar cancer forms in a woman’s external genitalia. The vulva includes:

• Inner and outer lips of the vagina.
• Clitoris (sensitive tissue between the lips).
• Opening of the vagina and its glands.
• Mons pubis (the rounded area in front of the pubic bones that becomes covered with hair at puberty).
• Perineum (the area between the vulva and the anus).

Vulvar cancer most often affects the outer vaginal lips. Less often, cancer affects the inner vaginal lips, clitoris, or vaginal glands.

Vulvar cancer usually forms slowly over a number of years. Abnormal cells can grow on the surface of the vulvar skin for a long time. This condition is called vulvar intraepithelial neoplasia (VIN). Because it is possible for VIN to become vulvar cancer, it is very important to get treatment.

Risk Factors

Having vulvar intraepithelial neoplasia or HPV infection can affect the risk of vulvar cancer.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for vulvar cancer include the following:

• Having vulvar intraepithelial neoplasia (VIN).
• Having human papillomavirus (HPV) infection.
• Having a history of genital warts.
• Other possible risk factors include the following:
• Having many sexual partners.
• Having first sexual intercourse at a young age.
• Having a history of abnormal Pap tests (Pap smears).

Screening and Detection

Tests that examine the vulva are used to detect (find) and diagnose vulvar cancer.

The following tests and procedures may be used:

• Physical exam and history: An exam of the body to check general signs of health, including checking the vulva for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
• Biopsy: The removal of samples of cells or tissues from the vulva so they can be viewed under a microscope by a pathologist to check for signs of cancer.

Signs and Symptoms

Signs of vulvar cancer include bleeding or itching.

Vulvar cancer often does not cause early signs or symptoms. Signs and symptoms may be caused by vulvar cancer or by other conditions. Check with your doctor if you have any of the following:

• A lump or growth on the vulva.
• Changes in the vulvar skin, such as color changes or growths that look like a wart or ulcer.
• Itching in the vulvar area, that does not go away.
• Bleeding not related to menstruation (periods).
• Tenderness in the vulvar area.

Treatment Options

There are different types of treatment for patients with vulvar cancer.

Different types of treatments are available for patients with vulvar cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Four types of standard treatment are used:

Surgery

Surgery is the most common treatment for vulvar cancer. The goal of surgery is to remove all the cancer without any loss of the woman’s sexual function. One of the following types of surgery may be done:

• Laser surgery: A surgical procedure that uses a laser beam (a narrow beam of intense light) as a knife to make bloodless cuts in tissue or to remove a surface lesion such as a tumor.
• Wide local excision: A surgical procedure to remove the cancer and some of the normal tissue around the cancer.
• Radical local excision: A surgical procedure to remove the cancer and a large amount of normal tissue around it. Nearby lymph nodes in the groin may also be removed.
• Ultrasound surgical aspiration (USA): A surgical procedure to break the tumor up into small pieces using very fine vibrations. The small pieces of tumor are washed away and removed by suction. This procedure causes less damage to nearby tissue.
• Vulvectomy: A surgical procedure to remove part or all of the vulva:
  • Skinning vulvectomy: The top layer of vulvar skin where the cancer is found is removed. Skin grafts from other parts of the body may be needed to cover the area where the skin was removed.
  • Modified radical vulvectomy: Surgery to remove part of the vulva. Nearby lymph nodes may also be removed.
  • Radical vulvectomy: Surgery to remove the entire vulva. Nearby lymph nodes are also removed.
• Pelvic exenteration: A surgical procedure to remove the lower colon, rectum, and bladder. The cervix, vagina, ovaries, and nearby lymph nodes are also removed. Artificial openings (stoma) are made for urine and stool to flow from the body into a collection bag.

Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may have chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, a body cavity such as the abdomen, or onto the skin, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Topical chemotherapy for vulvar cancer may be applied to the skin in a cream or lotion.

See Drugs Approved to Treat Vulvar Cancer for more information.

Biologic therapy

Biologic therapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.

Imiquimod is a biologic therapy that may be used to treat vulvar lesions and is applied to the skin in a cream.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today’s standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI’s listing of clinical trials.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

It is important to have regular follow-up exams to check for recurrent vulvar cancer.