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.

Types of Vulvar Cancer

Ninety percent of vulvar cancers are squamous cell carcinomas, or more simply put, a skin cancer of the genital area. Adenocarcinomas can arise in the Bartholin's Glands which are tiny glands located near the opening of the vagina on both sides. This type of represents 1-2% of vulvar cancers. Verrucous carcinoma is a very rare type of vulvar cancer that looks like a huge genital wart. Basal Cell cancer can arise in the vulva just as it can in other areas of the body. Two percent of vulvar cancers are the basal cell type. Melanoma represents about 5% of vulvar cancers. Melanomas of the vulva develop in pigmented lesions of the vulva (junctional nevi) just as they do in other areas of the body. Most pigmented lesions (moles) on the vulvar are pre-malignant and should be removed by biopsy. Sarcomas are extremely rare and arise in the muscle tissue of the vulva.

Where It Develops

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

Risk factors include the following:

• Having human papillomavirus (HPV) infection.
• Older age.
• Cigarette smoking may be a risk factor.

Possible signs of vulvar cancer include bleeding or itching. Most patients complain of a non-healing sore.

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.
• A lesion or "sore" that does not heal.
• Itching or irritation 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).

=Stages

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 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 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. Before starting treatment, patients may want to think about taking part in a clinical trial. 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.

Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

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 ongoing clinical trials is available from the NCI Web site.

Treatment Options by Stage

Find Clinical Trials in your area here

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 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 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 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.

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

Check for 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.

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

Check for 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.

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

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

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