General Information About Vulvar Cancer

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 the inner and outer lips of the vagina, the clitoris (sensitive tissue between the lips), and the opening of the vagina and its glands.

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

Vulvar cancer usually develops slowly over a period of years. Abnormal cells can grow on the surface of the vulvar skin for a long time. This precancerous condition is called vulvar intraepithelial neoplasia (VIN) or dysplasia. Because it is possible for VIN or dysplasia to develop into vulvar cancer, treatment of this condition is very important.

HPV infection and older age can affect the risk of developing vulvar cancer.

Risk factors include the following:

• Having human papillomavirus (HPV) infection.

• Older age.

Possible signs of vulvar cancer include bleeding or itching.

Vulvar cancer often does not cause early symptoms. When symptoms occur, they may be caused by vulvar cancer or by other conditions. A doctor should be consulted if any of the following problems occur:

• A lump in the vulva.

• Itching that does not go away in the vulvar area.

• Bleeding not related to menstruation (periods).

• Tenderness in the vulvar area.

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 cells or tissues from the vulva so they can be viewed under a microscope by a pathologist to check for signs of cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

• The stage of the cancer.

• The patient's age and general health.

• Whether the cancer has just been diagnosed or has recurred (come back).

Stages of Vulvar Cancer

After vulvar cancer has been diagnosed, tests are done to find out if cancer cells have spread within the vulva or to other parts of the body.

The process used to find out if cancer has spread within the vulva or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process:

• Pelvic exam: An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. The doctor or nurse inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. A speculum is also inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test or Pap smear of the cervix is usually done. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas.

• Cystoscopy: A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope (a thin, lighted tube) is inserted through the urethra into the bladder. Tissue samples may be taken for biopsy.

• Proctoscopy: A procedure to look inside the rectum and anus to check for abnormal areas. A proctoscope (a thin, lighted tube) is inserted into the anus and rectum. Tissue samples may be taken for biopsy.

• X-rays: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. To stage vulvar cancer, x-rays may be taken of the organs and bones inside the chest, and the pelvic bones.

• Intravenous pyelogram (IVP): A series of x-rays of the kidneys, ureters, and bladder to find out if cancer has spread to these organs. A contrast dye is injected into a vein. As the contrast dye moves through the kidneys, ureters and bladder, x-rays are taken to see if there are any blockages. This procedure is also called intravenous urography.

• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

• Through tissue. Cancer invades the surrounding normal tissue.

• Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.

• Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.

The following stages are used for vulvar cancer:

Stage 0 (Carcinoma in Situ)

In stage 0, abnormal cells are found on the surface of the vulvar skin. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.

Stage I

In stage I, cancer has formed and is found in the vulva only or in the vulva and perineum (area between the rectum and the vagina). The tumor is 2 centimeters or smaller and has spread to tissue under the skin. Stage I vulvar cancer is further divided into stage IA and stage IB.

• Stage IA: The tumor has spread 1 millimeter or less into the tissue of the vulva.

• Stage IB: The tumor has spread more than 1 millimeter into the tissue of the vulva.

Stage II

In stage II, cancer is found in the vulva or the vulva and perineum (space between the rectum and the vagina), and the tumor is larger than 2 centimeters.

Stage III

In stage III vulvar cancer, the cancer is of any size and either:

• is found only in the vulva or the vulva and perineum and has spread to tissue under the skin and to nearby lymph nodes on one side of the groin; or

• has spread to nearby tissues such as the lower part of the urethra and/or vagina or anus, and may have spread to nearby lymph nodes on one side of the groin.

Stage IV

Stage IV is divided into stage IVA and stage IVB, based on where the cancer has spread.

• Stage IVA: Cancer has spread to nearby lymph nodes on both sides of the groin, or has spread beyond nearby tissues to the upper part of the urethra, bladder, or rectum, or has attached to the pelvic bone and may have spread to lymph nodes.

• Stage IVB: Cancer has spread to distant parts of the body.

Recurrent Vulvar Cancer

Recurrent vulvar cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the vulva or in other parts of the body.

Treatment Option Overview

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:

Laser therapy

Laser therapy is a cancer treatment that uses a laser beam (a narrow beam of intense light) to kill cancer cells.

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 the woman's sexual function. One of the following types of surgery may be done:

• 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.

• 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.
  • Simple vulvectomy: The entire vulva is removed.
  • Modified radical vulvectomy: The vulva containing cancer and some of the normal tissue around it is removed.
  • Radical vulvectomy: The entire vulva, including the clitoris, and nearby tissue is removed. Nearby lymph nodes may also be 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 increase the chances of a cure, 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 spinal column, 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.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI Web site.

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 clinical trials database.

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. This is sometimes called re-staging.

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.

Treatment Options by Stage

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Stage 0 (Carcinoma in Situ)

Treatment of stage 0 may include the following:

• Wide local excision and/or laser therapy.

• Skinning vulvectomy with or without skin grafting.

• Simple vulvectomy.

• Topical chemotherapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage 0 vulvar cancer.

Stage I Vulvar Cancer

Treatment of stage I vulvar cancer may include the following:

• Wide local excision.

• Radical local excision with removal of nearby lymph nodes.

• Radical vulvectomy and either removal of nearby lymph nodes or radiation therapy to the lymph nodes.

• Radiation therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage I vulvar cancer.

Stage II Vulvar Cancer

Treatment of stage II vulvar cancer may include the following:

• Modified radical vulvectomy and removal of nearby lymph nodes or radiation therapy to the lymph nodes. Radiation therapy to the area of surgery may also be given.

• Radiation therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage II vulvar cancer.

Stage III Vulvar Cancer

Treatment of stage III vulvar cancer may include the following:

• Modified radical vulvectomy and removal of nearby lymph nodes, with or without radiation therapy.

• Radical vulvectomy and removal of nearby lymph nodes, with or without radiation therapy.

• Radiation therapy followed by surgery.

• Radiation therapy with or without chemotherapy.

• A clinical trial of a new treatment.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III vulvar cancer.

Stage IV Vulvar Cancer

Treatment of stage IV vulvar cancer may include the following:

• Radical vulvectomy and pelvic exenteration.

• Radical vulvectomy followed by radiation therapy.

• Radiation therapy followed by surgery, with or without chemotherapy.

• Radiation therapy with or without chemotherapy.

• A clinical trial of a new treatment.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage IV vulvar cancer.

Treatment Options for Recurrent Vulvar Cancer

It is important to have regular follow-up exams to check for recurrent vulvar cancer. Treatment of recurrent vulvar cancer may include the following:

• Wide local excision with or without radiation therapy.

• Radical vulvectomy and pelvic exenteration.

• Radiation therapy and chemotherapy given during the same period of time, with or without surgery.

• Radiation therapy followed by surgery or chemotherapy.

• Radiation therapy as palliative treatment to relieve symptoms and improve quality of life.

• A clinical trial of a new treatment.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent vulvar cancer.

To Learn More About Vulvar Cancer

For more information from the National Cancer Institute about vulvar cancer, see the following:

• Vulvar Cancer Home Page

• Human Papillomaviruses and Cancer: Questions and Answers

• Lasers in Cancer Treatment: Questions and Answers

For general cancer information and other resources from the National Cancer Institute, see the following:

• What You Need to Know About™ Cancer - An Overview

• Understanding Cancer Series: Cancer

• Staging: Questions and Answers

• Chemotherapy and You: Support for People With Cancer

• Radiation Therapy and You: Support for People With Cancer

• Coping with Cancer

• Support and Resources

• Cancer Library

• Information For Survivors/Caregivers/Advocates

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Changes to This Summary (07/31/2008)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made to this summary.

2005-09-23 Last Modified: 2008-07-31