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Diseases & Conditions

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:

  • 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.

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).
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. Check with your doctor if you have any of the following problems:

  • 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.
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.
  • Colposcopy: A procedure in which a colposcope (a lighted, magnifying instrument) is used to check the vagina and cervix for abnormal areas. Tissue samples may be taken using a curette (spoon-shaped instrument) and checked under a microscope for signs of disease.
  • 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 is inserted into the anus and rectum. A proctoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
  • 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).
  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
  • Sentinel lymph node biopsy: The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. Sentinel lymph node biopsy may be done during surgery to remove the tumor for early-stage vulvar cancer.
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.

In vulvar intraepithelial neoplasia (VIN), abnormal cells are found on the surface of the vulvar skin.

These abnormal cells are not cancer. Vulvar intraepithelial neoplasia (VIN) may become cancer and spread into nearby tissue. VIN is sometimes called stage 0 or carcinoma in situ.

The following stages are used for vulvar cancer:

Stage I

In stage I, cancer has formed. The tumor is found only in the vulva or perineum (area between the rectum and the vagina). Stage I is divided into stages IA and IB.

  • In stage IA, the tumor is 2 centimeters or smaller and has spread 1 millimeter or less into the tissue of the vulva. Cancer has not spread to the lymph nodes.
  • In stage IB, the tumor is larger than 2 centimeters or has spread more than 1 millimeter into the tissue of the vulva. Cancer has not spread to the lymph nodes.

Stage II

In stage II, the tumor is any size and 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 stage III, the tumor is any size and may have spread into the lower part of the urethra, the lower part of the vagina, or the anus. Cancer has spread to one or more nearby lymph nodes. Stage III is divided into stages IIIA, IIIB, and IIIC.

  • In stage IIIA, cancer is found in 1 or 2 lymph nodes that are smaller than 5 millimeters or in one lymph node that is 5 millimeters or larger.
  • In stage IIIB, cancer is found in 2 or more lymph nodes that are 5 millimeters or larger, or in 3 or more lymph nodes that are smaller than 5 millimeters.
  • In stage IIIC, cancer is found in lymph nodes and has spread to the outside surface of the lymph nodes.

Stage IV

In stage IV, the tumor 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.

  • In stage IVA:
    • cancer has spread into the lining of the upper urethra, the upper vagina, the bladder, or the rectum, or has attached to the pelvic bone; or
    • cancer has spread to nearby lymph nodes and the lymph nodes are not moveable or have formed an ulcer.
  • In stage IVB, cancer has spread to lymph nodes in the pelvis or to other 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:

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:

  • 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 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.

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 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.

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.

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

Treatment Options by Stage

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.

Vulvar Intraepithelial Neoplasia (VIN)

Treatment of vulvar intraepithelial neoplasia (VIN) may include the following:

  • Removal of single lesions or wide local excision.
  • Laser surgery.
  • Ultrasound surgical aspiration.
  • Skinning vulvectomy with or without a skin graft.
  • Biologic therapy with topical imiquimod.

Stage I Vulvar Cancer

Treatment of stage I vulvar cancer may include the following:

  • Wide local excision for lesions that are less than 1 millimeter deep..
  • Radical local excision and removal of nearby lymph nodes.
  • Radical local excision and sentinel lymph node biopsy. If cancer is found in the sentinel lymph node, nearby lymph nodes are also removed.
  • Radiation therapy for patients who cannot have surgery.

Stage II Vulvar Cancer

Treatment of stage II vulvar cancer may include the following:

  • Radical local excision and removal of nearby lymph nodes.
  • Modified radical vulvectomy or radical vulvectomy for large tumors. Nearby lymph nodes may be removed. Radiation therapy may be given after surgery.
  • Radical local excision and sentinel lymph node biopsy. If cancer is found in the sentinel lymph node, nearby lymph nodes are also removed.
  • Radiation therapy for patients who cannot have surgery.

Stage III Vulvar Cancer

Treatment of stage III vulvar cancer may include the following:

  • Modified radical vulvectomy or radical vulvectomy. Nearby lymph nodes may be removed. Radiation therapy may be given after surgery.
  • Radiation therapy or chemotherapy and radiation therapy followed by surgery.
  • Radiation therapy with or without chemotherapy for patients who cannot have surgery.

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 or chemotherapy and radiation therapy followed by surgery.
  • Radiation therapy with or without chemotherapy for patients who cannot have surgery.

Treatment Options for Recurrent Vulvar Cancer

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with vulvar cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug.

For more information from the NCI, please write to this address:

NCI Public Inquiries Office
Suite 3036A
6116 Executive Boulevard, MSC8322
Bethesda, MD 20892-8322

U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1.800.332.8615. Information about ongoing clinical trials is available from the NCI website.

Source: National Institutes of Health; National Cancer Institute

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 10/16/2012...#6220

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