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:

• 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).

Anatomy of the vulva. The vulva includes the mons pubis, clitoris, urethral opening, inner and outer lips of the vagina, vaginal opening, and perineum.

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 many 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 important to get treatment.

Having vulvar intraepithelial neoplasia or HPV infection can increase 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:

• Older age.
• Having human papillomavirus (HPV) infection.
• Having vulvar intraepithelial neoplasia (VIN).
• 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).

Signs of vulvar cancer include bleeding or itching in the vulvar area.

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 that looks like a wart or ulcer.
• Itching in the vulvar area that does not go away.
• Bleeding not related to menstruation (periods).
• Pain in the vulvar area.

Tests that examine the vulva are used to diagnose vulvar cancer.

The following tests and procedures may be used:

• Physical exam and health 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.
• Pelvic exam: An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test of the cervix is usually done. The doctor or nurse also 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. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas.
  
  
  
  Pelvic exam. A doctor or nurse inserts one or two lubricated, gloved fingers of one hand into the vagina and presses on the lower abdomen with the other hand. This is done to feel the size, shape, and position of the uterus and ovaries. The vagina, cervix, fallopian tubes, and rectum are also checked.
• Pap test: A procedure to collect cells from the surface of the cervix and vagina. A piece of cotton, a brush, or a small wooden stick is used to gently scrape cells from the cervix and vagina. The cells are viewed under a microscope to find out if they are abnormal.
• Human papillomavirus (HPV) test: A laboratory test used to check DNA or RNA for certain types of HPV infection. Cells are collected from the vulva and DNA or RNA from the cells is checked to find out if an infection is caused by a type of human papillomavirus that is linked to vulvar cancer. This test may be done using the sample of cells removed during a Pap test. This test may also be done if the results of a Pap test show certain abnormal vulvar cells.
• 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.
• 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) or a brush and checked under a microscope for signs of disease.
• 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).
• 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.
• 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.

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

The prognosis and treatment options depend on the following:

• Whether the cancer has spread to nearby areas or other parts of the body.
• Whether the cancer has spread to the lymph nodes.
• 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:

• Cystoscopy: A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope is inserted through the urethra into the bladder. A cystoscope 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 for signs of cancer.
• Proctoscopy: A procedure to look inside the rectum and anus to check for abnormal areas, using a proctoscope. A proctoscope is a thin, tube-like instrument with a light and a lens for viewing the inside of the rectum and anus. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
• Chest x-ray: 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.
• 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.
• Biopsy: The removal of cells or tissues from the bladder or rectum so they can be viewed under a microscope by a pathologist to check for signs of cancer, if it is suspected that cancer has spread there.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

• Tissue. The cancer spreads from where it began by growing into nearby areas.
• Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
• Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

• Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
• Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if vulvar cancer spreads to the lung, the cancer cells in the lung are actually vulvar cancer cells. The disease is metastatic vulvar cancer, not lung 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.

Tumor sizes are often measured in centimeters (cm) or inches. Common food items that can be used to show tumor size in cm include: a pea (1 cm), a peanut (2 cm), a grape (3 cm), a walnut (4 cm), a lime (5 cm or 2 inches), an egg (6 cm), a peach (7 cm), and a grapefruit (10 cm or 4 inches).

• In stage IA, the tumor is 2 centimeters or smaller and has spread 1 millimeter or less into the tissue of the vulva or perineum. 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 or perineum. Cancer has not spread to the lymph nodes.
  
  
  
  Millimeters (mm). A sharp pencil point is about 1 mm, a new crayon point is about 2 mm, and a new pencil eraser is about 5 mm.

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 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 vulvar intraepithelial neoplasia (VIN) and vulvar cancer.

One of the following types of surgery may be done to treat VIN:

• Separate excision of a lesion: A surgical procedure to remove a lesion of concern.
• Wide local excision: A surgical procedure to remove the area of skin affected by VIN and some of the normal tissue around it.
• 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.
• Ultrasound surgical aspiration: 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.
• Skinning vulvectomy: The top layer of vulvar skin where the VIN is found is removed. Skin grafts from other parts of the body may be needed to cover the area where the skin was removed.

The goal of surgery for vulvar cancer 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 to treat vulvar cancer:

• 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:
  • Modified radical vulvectomy: Surgery to remove most 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.

After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy and/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 or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward area of the body with cancer.

External radiation therapy may also be used as palliative therapy to relieve symptoms and improve quality of life.

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). Topical chemotherapy for vulvar cancer may be applied to the skin in a cream or lotion. The way the chemotherapy is given depends on the type and stage of the cancer being treated.

See Drugs Approved to Treat Vulvar Cancer for more information.

Immunotherapy

Immunotherapy 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 biologic therapy.

Imiquimod is an immune response modifier 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.

Treatment for vulvar cancer may cause side effects.

For information about side effects caused by treatment for cancer, see our Side Effects page.

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. Information about clinical trials supported by NCI can be found on NCI's clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

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.

Treatment of Vulvar Intraepithelial Neoplasia (VIN)

For information about the treatments listed below, see the Treatment Option Overview section.

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

• Surgery may be one of the following:
  • Separate excision of lesions.
  • Wide local excision.
  • Laser surgery.
  • Ultrasound surgical aspiration.
  • Skinning vulvectomy.
• Immunotherapy with topical imiquimod.

Treatment of Stages I and II Vulvar Cancer

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of stage I vulvar cancer and stage II vulvar cancer may include the following:

• Surgery (wide local excision).
• Surgery (radical local excision with removal of lymph nodes in the groin and upper thigh).
• Surgery (modified radical vulvectomy or radical vulvectomy with removal of lymph nodes in the groin and upper thigh). Radiation therapy may be given.
• Surgery (radical local excision and removal of sentinel lymph node) followed by radiation therapy in some cases.
• Radiation therapy alone.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Stage III Vulvar Cancer

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of stage III vulvar cancer may include the following:

• Surgery (modified radical vulvectomy or radical vulvectomy with removal of lymph nodes in the groin and upper thigh) with or without radiation therapy.
• Radiation therapy or chemotherapy and radiation therapy followed by surgery.
• Radiation therapy with or without chemotherapy.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Stage IVA Vulvar Cancer

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of stage IVA vulvar cancer may include the following:

• Surgery (radical vulvectomy or pelvic exenteration).
• Surgery and radiation therapy.
• Radiation therapy or chemotherapy and radiation therapy followed by surgery.
• Radiation therapy with or without chemotherapy.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Stage IVB Vulvar Cancer

There is no standard treatment for stage IVB vulvar cancer. Chemotherapy has been studied and may be used if the patient can tolerate it.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Recurrent Vulvar Cancer

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of locally recurrent vulvar cancer may include the following:

• Surgery (wide local excision) with or without radiation therapy.
• Surgery (radical vulvectomy and pelvic exenteration).
• Chemotherapy and radiation therapy with or without surgery.
• Radiation therapy with or without chemotherapy.
• Radiation therapy and surgery.
• Radiation therapy as palliative treatment to relieve symptoms and improve quality of life.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

To Learn More About Vulvar Cancer

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

• Vulvar Cancer Home Page
• Lasers in Cancer Treatment
• Drugs Approved to Treat Vulvar Cancer
• HPV and Cancer
• Computed Tomography (CT) Scans and Cancer
• Immunotherapy to Treat Cancer

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

• About Cancer
• Staging
• Chemotherapy and You: Support for People With Cancer
• Radiation Therapy and You: Support for People With Cancer
• Coping with Cancer
• Questions to Ask Your Doctor about Cancer
• For Survivors and Caregivers

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

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This PDQ cancer information summary has current information about the treatment of vulvar cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

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Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.

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Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).

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The best way to cite this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Vulvar Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389324]

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Last Revised: 2020-04-17

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