The vulva is a woman’s external genitalia and is made up of the skin and fatty tissue that surround the clitoris and the openings of the vagina and urethra. The fatty tissue makes up two folds, called the labia majora and labia minora. Cancer of the vulva occurs most often in or on the labia. Less frequently, it can occur on the clitoris or in the glands on the sides of the vaginal opening, called Bartholin’s glands, which produce a mucus­­-like lubricating fluid.

Vulvar cancer is classified into three main types, named for the type of tissue where the cancer started.

Squamous cell carcinoma. Squamous cell carcinoma is a type of skin cancer that accounts for about 90% of vulvar cancers. It is usually found on the labia.

Adenocarcinoma. Adenocarcinoma starts in the Bartholin’s glands or vulvar sweat glands and accounts for a small percentage of vulvar cancers. It is usually found on the sides of the vaginal opening.

Melanoma. Melanoma is another type of skin cancer that accounts for about 2% to 4% of vulvar cancers. It occurs most often on the clitoris or the labia minora. Women with melanoma on other parts of their body have an increased risk of developing vulvar melanoma. For more information, read the Cancer.Net Guide to Melanoma.

Other, less common vulvar cancers include Paget’s disease of the vulva, in which adenocarcinoma cells are found in the vulvar skin; sarcoma, a tumor of the connective tissues beneath the skin; and verrucous carcinoma, a slow-growing subtype of squamous cell carcinoma that resembles a wart.

Statistics

In 2009, an estimated 3,580 women in the United States will be diagnosed with vulvar cancer. It is estimated that 900 deaths from this disease will occur this year. Vulvar cancer accounts for about 4% of cancers in female reproductive organs and 0.6% of all cancers in women. Its incidence is increasing in young women because of its association with the human papillomavirus (HPV). See the Risk Factors section for more information on HPV.

Cancer statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer in the United States each year, but the actual risk for a particular individual may differ. It is not possible to tell a woman how long she will live with vulvar cancer.

Statistics adapted from the American Cancer Society's publication, Cancer Facts & Figures 2009.

Find out more about basic cancer terms used in this section.

A risk factor is anything that increases a person’s chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and communicating them to your doctor may help you make more informed lifestyle and health-care choices.

The following factors may raise a woman’s risk of developing vulvar cancer:

Age. The majority of women diagnosed with vulvar cancer are older than 50. However, a significant percentage (15%) of women under 40 develop vulvar cancer. Generally, vulvar cancer in younger women is associated with HPV infection and smoking. Vulvar cancer in older women is most often associated with lichen sclerosus (a rare skin condition; see below) or mutations (changes) in certain genes.

HPV infection. HPV is most often spread from one person to another during sexual intercourse. There are many types or strains of HPV, and some are linked with genital or anal cancer in men and women. HPV may be responsible for an about one-third to two-thirds of vulvar cancers. Many types of cancer caused by HPV are associated with precancerous conditions (changes in cells that may, but do not always, become cancer) that develop before the cancer.

Smoking. Smoking may increase a woman’s risk of developing vulvar cancer if she has HPV.

Immune system deficiency. Women with lowered immune systems have a higher risk of developing vulvar cancer. A lowered immune system can be caused by immune suppression from corticosteroid medications, organ transplantation, or treatment for other types of cancer or from human immunodeficiency virus (HIV), the virus that causes acquired immune deficiency syndrome (AIDS). When a woman has a lowered immune system, her body is more likely to develop infections, including HPV.

Lichen sclerosus. This condition affects the vulvar skin, making it thin and itchy. About 4% of women with lichen sclerosus develop vulvar cancer.

Precancerous conditions. Precancerous conditions of the vulva, cervix, or vagina, or melanoma elsewhere on the body, can increase a woman’s risk of developing vulvar cancer.

Prevention and Early Detection

All women should have an annual gynecologic examination. During this exam, the doctor will take a family medical history and perform a general physical examination of the pelvis, during which the doctor will feel a woman’s uterus, vagina, cervix, and other reproductive organs to check for any unusual changes. Regular pelvic examinations can help detect cancer or precancerous conditions at an early stage. In addition, research has shown that certain factors can help prevent vulvar cancer.

• Delaying first sexual intercourse until the late teens or older
  
  
• Avoiding sexual intercourse with multiple partners
  
  
• Avoiding sexual intercourse with someone who has had many partners
  
  
• Practicing safe sex, including condom use (although condoms cannot fully protect against HPV)
  
  
• Having regular gynecologic examinations to detect and treat precancerous conditions
  
  
• Not starting to smoke
  
  
• Quitting smoking, if a smoker

In 2008, the vaccine used to prevent cervical cancer for girls and women between ages 9 and 26 was also approved to prevent vulvar cancer. It prevents infection by the four most common strains (types) of HPV. The vaccine does not protect people who are already infected with HPV. For more information on the HPV vaccine, read the ASCO Expert Corner: HPV Vaccination for Cervical Cancer. People are encouraged to talk with their doctors for more information about the HPV vaccine.

Women with vulvar cancer may experience the following symptoms. Sometimes, women with vulvar cancer do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom on this list, please talk with your doctor.

• A lump or growth in or on the vulvar area
  
  
• A patch of skin that is differently textured or colored than the rest of the vulvar area
  
  
• Persistent itching, pain, soreness, or burning in the vulvar area
  
  
• Painful urination
  
  
• Bleeding or discharge that is not menstrual blood
  
  
• An ulcer that persists for more than one month
  
  
• A change in the appearance of an existing mole (specific to vulvar melanoma)
  
  
• Wart-like growths (similar to genital warts)

Doctors use many tests to diagnose cancer and determine if it has metastasized (spread).

Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:

• Age and medical condition
  
  
• The type of cancer suspected
  
  
• Severity of symptoms
  
  
• Previous test results

In addition to a physical examination, the following tests may be used to diagnose vulvar cancer:

Pelvic examination. The doctor feels the uterus, vagina, ovaries, fallopian tubes, bladder, and rectum to check for any unusual changes.

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). The type of biopsy performed will depend on the location of the tissue being biopsied.

Lymph node sampling. The lymph nodes are tiny, bean-shaped organs that help fight infection. To determine whether a cancer has spread, it may be necessary to remove lymph nodes for biopsy. The procedure for determining if cancer has spread to the lymph nodes is the sentinel lymph node biopsy. In this procedure, the first, or sentinel, lymph node is sampled. Recent research has shown that if the first lymph node is free of cancer, then the cancer most likely has not spread.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail.

Endoscopy. This test allows the doctor to see inside the body. The person may be sedated, and the doctor inserts a thin, lighted, flexible tube called an endoscope through the mouth, anus, vagina, urethra, or a small surgical opening.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture.

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient’s body and absorbed by the organs or tissues being studied. This substance gives off energy that is detected by a scanner, which produces the images.

X-ray. An x-ray is a picture of the inside of the body. For instance, a chest x-ray can help doctors determine if the cancer has spread to the lungs.

To learn more about what to expect during common diagnostic tests, read Cancer.Net: Tests and Procedures.

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: Newly Diagnosed.

Staging is a way of describing a cancer, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of cancer.

One tool that doctors use to describe the stage is the TNM system. This system uses three criteria to judge the stage of the cancer: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to other parts of the body. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.

TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:

• How large is the primary tumor and where is it located? (Tumor, T)
  
  
• Has the tumor spread to the lymph nodes? (Node, N)
  
  
• Has the cancer metastasized to other parts of the body? (Metastasis, M)

Tumor. Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the size and location of the tumor. Some stages are also divided into smaller groups that help describe the tumor in even more detail. Specific tumor stage information is listed below.

TX: The primary tumor cannot be evaluated.

T0: There is no tumor.

Tis: The tumor is carcinoma in situ (early cancer on the surface of the skin and has not spread to nearby tissue).

T1: The tumor is only in the vulva, or the vulva and perineum (the area of skin between the anus and vagina), and is 2 centimeters (cm) or smaller.

T1a: The tumor is only in the vulva, or the vulva and perineum, is 2 cm or smaller, and has spread no more than 1 millimeter (mm) into nearby structures.

T1b: The tumor is only in the vulva, or the vulva and perineum, is 2 cm or smaller, and has spread more than 1 mm into nearby structures.

T2: The tumor is only in the vulva, or the vulva and perineum, and is larger than 2 cm.

T3: The tumor, of any size, has spread to the lower urethra and/or the vagina or anus.

T4: The tumor has spread to any of the following: upper urethra, bladder mucosa, rectal mucosa, or is attached to the pubic bone.

Node. The “N” in the TNM staging system stands for lymph nodes. Lymph nodes near the pelvis and groin are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes.

NX: The regional lymph nodes cannot be evaluated.

N0 (N plus zero): Cancer has not spread to the lymph nodes.

N1: Cancer has spread to lymph nodes on the same side of the body as the tumor.

N2: Cancer has spread to lymph nodes on both sides of the body.

Distant metastasis. The “M” in the TNM system indicates whether the cancer has spread to other parts of the body.

MX: Distant metastasis cannot be evaluated.

M0 (M plus zero): There is no distant metastasis.

M1: There is metastasis to other parts of the body.

Cancer stage grouping

Doctors assign the stage of the cancer by combining the T, N, and M classifications.

Stage 0: The cancer has not spread from where it started and is on the surface of the skin of the vulva only (Tis, N0, M0).

Stage I: The tumor is smaller than 2 cm and has not spread (T1, N0, M0).

Stage IA: The tumor is smaller than 2 cm, has not spread, and is no deeper than 1 mm (T1a, N0, M0).

Stage IB: The tumor is smaller than 2 cm, has not spread, and is deeper than 1mm (T1b, N0, M0).

State II: The tumor is larger than 2 cm, is in the vulva or perineum or both, but has not spread to nearby tissue (T2, N0, M0).

Stage III: The cancer has spread to nearby tissue (vagina, anus, urethra) and/or lymph nodes on one side of the body, but there is no distant metastasis (T1 or T2, N1, M0; T3, N0 or N1, M0).

Stage IVA: The cancer has spread to lymph nodes on both sides of the body or spread into the upper part of the urethra, bladder, rectum, or pelvic bone (T1, T2, T3; N2, M0; or T4, any N, M0).

Stage IVB: The cancer has spread to distant parts of the body (Any T, any N, M1).

Recurrent: Recurrent cancer is cancer that comes back after treatment.

Grading

Tumor grade (G). Doctors also describe a primary tumor by its grade, which is determined by using a microscope to examine tissue from a tumor. The doctor compares the tumor tissue with normal tissue. Healthy tissue contains many different types of cells grouped together, which is called differentiated. Tissue from a tumor usually has cells that look more alike, called poorly differentiated. Generally, the more differentiated the tissue, the better the prognosis.

GX: The tumor grade cannot be evaluated.

G1: The tumor cells are well differentiated (contains many healthy-looking cells).

G2: The tumor cells are moderately differentiated (more cells appear abnormal than healthy).

G3: The tumor cells are poorly differentiated (most of the cells appear abnormal).

G4: The tumor cells are undifferentiated (the cells barely resemble healthy cells).

Used with permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Sixth Edition (2002)published by Springer-Verlag New York, www.springer-ny.com.

The treatment of vulvar cancer depends on the size and location of the tumor, whether the cancer has spread, and the woman’s overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan. Women with vulvar cancer may have concerns about if and how these treatments may affect their sexual function and fertility, and these topics should be discussed with the health care team before treatment begins.

This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, visit the Clinical Trials section.

The main treatment for vulvar cancer is surgery. Radiation therapy and chemotherapy may be used if the cancer cannot be entirely removed with surgery. Each treatment option is described below.

Surgery

Due to the location and sensitivity of vulvar tissue, the type of surgery is carefully considered. Surgical options for vulvar cancer include:

Laser surgery. Laser surgery is the use of a focused beam of light that burns the cancer off the skin. It can be used to treat precancerous conditions, but cannot be used to treat an invasive tumor.

Excision. This is the removal of the tumor and some of the surrounding healthy tissue (called a margin). If the procedure is extensive, this may be called a partial vulvectomy (see below).

Vulvectomy. This is the removal of part or all of the vulva. In a skinning vulvectomy, only the top layer of skin is removed. A simple vulvectomy is the removal of the entire vulva. A radical vulvectomy is the removal of part or all of the vulva and deep tissue, including the clitoris.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. Radiation therapy may be used before surgery to shrink the size of the tumor or after surgery to destroy any remaining cancer cells. The most common type of radiation treatment is called external-bean radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy.

General side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most of these side effects go away soon after treatment is finished. Specific side effects may include narrowing of the vagina, damage to healthy vaginal tissue, and irritation of the intestines. The vagina may shorten and narrow so much that sexual intercourse is not possible. To prevent this, the vagina may need to be stretched with a plastic tube called a vaginal dilator several times a week.

For more information about radiation therapy, see the American Society for Therapeutic Radiology and Oncology's pamphlet, Radiation Therapy for Gynecologic Cancers.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. The side effects of chemotherapy depend on the individual and the dose used, but can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.

The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions through Cancer.Net's Drug Information Resources, which provides links to searchable drug databases.

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: During Treatment.

Doctors and scientists are always looking for better ways to treat patients with vulvar cancer. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. Patients who participate in clinical trials are among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that finding new drugs and other therapies is the only way to make progress in treating vulvar cancer. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with vulvar cancer.

To join a clinical trial, patients must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about Clinical Trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.

Cancer and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of cancer treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur.

Fear of treatment side effects is common after a diagnosis of cancer, but it may be helpful to know that preventing and controlling side effects is a major focus of your health-care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and the person’s overall health.

Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health-care team if they do happen. Also, be sure to communicate with your doctor about side effects you experience during and after treatment. For more information on the most common side effects of cancer and different treatments, along with ways to prevent or control them, visit Cancer.Net’ s section on Managing Side Effects .

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing these needs in Cancer.Net’s section on Caring for the Whole Patient .

For more information on late effects or long-term side effects, please read the After Treatment section or talk with your doctor.

After treatment for vulvar cancer ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. Although there are no specific guidelines, follow-up care is essential for women who have finished vulvar cancer treatment. Women should visit their doctors regularly for physical and pelvic examinations. The doctor may also recommend other tests, including x-rays, CT scans, ultrasound studies, or MRI scans. Tell your doctor about any new symptoms.

Women recovering from vulvar cancer are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about Healthy Living After Cancer.

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: After Treatment.

Research for vulvar cancer is ongoing. The following advances may still be under investigation in clinical trials and may not be approved or available at this time. Always discuss all diagnostic and treatment options with your doctor.

Different surgical techniques. New techniques, including reconstruction, to reduce the effects of vulvar surgery or to reconstruct the fatty tissue of the vulva after surgery are being studied.

HPV/vulvar cancer link. Research continues regarding the link between HPV and vulvar cancer, leading to a better understanding of the types of vulvar cancer and how they grow.

Regular communication with your doctor is important in making informed decisions about your health care. Consider asking the following questions of your doctor:

• What type of vulvar cancer do I have?
  
  
• What is the stage of my cancer? What does this mean?
  
  
• Can you explain my pathology report (laboratory test results) to me?
  
  
• What are my treatment options?
  
  
• What clinical trials are open to me?
  
  
• What treatment do you recommend? Why?
  
  
• Who will be doing the surgery? How experienced is this person with this type of cancer?
  
  
• Will I need reconstructive surgery?
  
  
• What are the possible side effects of treatment, both in the short term and the long term?
  
  
• How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?
  
  
• Will my sex life be affected during treatment? After treatment?
  
  
• Will the treatment affect my ability to have children? If so, should I speak to a fertility specialist before treatment begins?
  
  
• How can I keep myself as healthy as possible during treatment?
  
  
• What are the chances that the cancer will recur?
  
  
• What follow-up tests will I need, and how often will I need them?
  
  
• What support services are available for me? For my family?

Gynecologic Cancer Foundation
230 W. Monroe, Ste. 2528
Chicago, IL  60606
Toll Free: 800-444-4441
Phone: 312-578-1439
www.thegcf.org

Rare Cancer Alliance
1649 N. Pacana Way
Green Valley, AZ  85614
www.rare-cancer.org

National Organization for Rare Disorders
55 Kenosia Ave.
P.O. Box 1968
Danbury, CT  06813-1968
Toll Free: 800-999-6673
Phone: 203-744-0100
www.rarediseases.org

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