What is vulvar cancer

Vulvar cancer also known as cancer of the vulva, most often affects the two skin folds (or lips) around the vagina, the inner edges of the labia majora or the labia minora. Vulvar cancer starts in the clitoris or in the Bartholin glands less often (see Figure 1 below).

Vulvar cancer usually grows slowly over several years. First, precancerous cells grow on vulvar skin. This is called vulvar intraepithelial neoplasia (VIN) or dysplasia. Not all vulvar intraepithelial neoplasia (VIN) cases turn into vulvar cancer, but it is best to treat it early.

Vulvar cancer most often affects women 65 to 75 years of age. However, it can also occur in women 40 years of age or younger. Vulvar cancer may be related to genital warts, a sexually transmitted disease caused by the human papillomavirus (HPV).

In the United States, vulvar cancer accounts for nearly 6% of cancers of the female reproductive organs and 0.7% of all cancers in women. In the United States, women have a 1 in 333 chance of developing vulvar cancer at some point during their life.

The American Cancer Society’s estimates for vulvar cancer in the United States for 2018 are:

• About 6,190 cancers of the vulva will be diagnosed
• About 1,200 women will die of this cancer.

Often, vulvar cancer doesn’t cause symptoms at first. Vulvar cancer can make sex painful and difficult.

See your doctor for testing if you notice:

• A lump in the vulva or mass on the vulva
• Vulvar itching or tenderness that lasts more than one month
• Vulvar pain
• A burning pain when passing urine
• Burning in the genital area that lasts even after your doctor has treated the burning
• Bleeding from the vulva (different from your usual menstrual bleeding)
• Changes in the vulvar skin, such as color changes or growths that look like a wart or ulcer
• A mole on the vulva that changes shape or color
• A cut or sore on the vulva that won’t heal

You are at greater risk if you’ve had a human papillomavirus (HPV) infection or have a history of genital warts. Your health care provider diagnoses vulvar cancer with a physical exam and a biopsy. If found early, vulvar cancer has a high cure rate and the treatment options involve less surgery.

Treatment of  vulvar cancer varies, depending on your overall health and how advanced the cancer is. It might include surgery, radiation therapy, chemotherapy, or biologic therapy or a combination of treatments. Biologic therapy boosts your body’s own ability to fight cancer.

The type of surgery depends on the size, depth and spread of the vulvar cancer. Your doctor will review all the options for surgery and the pros and cons of each option. Some people may also need radiation therapy.

When vulvar cancer is found and treated early, the cure rate is over 90%. The key to a cure is to tell your doctor about any warning signs early and to have a biopsy right away. After treatment, be sure to go to all follow-up appointments that your doctor recommends.

The vulva

The vulva is the outer part of the female genitals. The vulva includes the opening of the vagina (sometimes called the vestibule), the labia majora (outer lips), the labia minora (inner lips), and the clitoris (Figure 1).

Around the opening of the vagina, there are 2 sets of skin folds. The inner set, called the labia minora, are small and hairless. The outer set, the labia majora, are larger, with hair on the outer surface. (Labia is Latin for lips.) The inner and outer labia meet, protecting the vaginal opening and, just above it, the opening of the urethra (the short tube that carries urine from the bladder). The Bartholin glands are found just inside the opening of the vagina — one on each side. These glands produce a mucus-like fluid that acts as a lubricant during sex.

At the front of the vagina, the labia minora meet to form a fold or small hood of skin called the prepuce. The clitoris is beneath the prepuce. The clitoris is an approximately ¾-inch structure of highly sensitive tissue that becomes swollen with blood during sexual stimulation. The labia minora also meet at a place just beneath the vaginal opening, at the fourchette. Beyond the fourchette is the anus, the opening to the rectum. This is where stool comes out of the body. The space between the vagina and the anus is called the perineum.

Figure 1. The vulva – the outer part of the female genitalia

Vulvar cancer types

Squamous cell carcinomas

Most cancers (90%) of the vulva are squamous cell carcinomas. This type of cancer starts in squamous cells, the main type of skin cells. There are several subtypes of squamous cell carcinoma:

• The keratinizing type is most common. It usually develops in older women and is not linked to infection with human papilloma virus (HPV).
• Basaloid and warty types are less common. These are the kinds more often found in younger women with HPV infections.
• Verrucous carcinoma is an uncommon subtype that’s important to recognize because it’s slow-growing and tends to have a good prognosis (outlook). This cancer looks like a large wart and a biopsy is needed to be sure it’s not a benign (non-cancer) growth.

Adenocarcinoma

Cancer that starts in gland cells is called adenocarcinoma. About 8 of every 100 vulvar cancers are adenocarcinomas. Vulvar adenocarcinomas most often start in cells of the Bartholin glands. These glands are found just inside the opening of the vagina. A Bartholin gland cancer is easily mistaken for a cyst (build-up of fluid in the gland), so it’s common to take awhile to get an accurate diagnosis. Most Bartholin gland cancers are adenocarcinomas. Adenocarcinomas can also form in the sweat glands of the vulvar skin.

Paget disease of the vulva is a condition in which adenocarcinoma cells are found in the top layer of the vulvar skin. Up to 25% of patients with vulvar Paget disease also have an invasive vulvar adenocarcinoma (in a Bartholin gland or sweat gland). In the remaining patients, the cancer cells are found only in the skin’s top layer and have not grown into the tissues below.

Melanoma

Melanomas are cancers that start in the pigment-producing cells that give skin color. They are much more common on sun-exposed areas of the skin, but can start in other areas, such as the vulva. Vulvar melanomas are rare, making up about 6 of every 100 vulvar cancers.

Sarcoma

A sarcoma is a cancer that starts in the cells of bones, muscles, or connective tissue. Less than 2 of every 100 vulvar cancers are sarcomas. Unlike other cancers of the vulva, vulvar sarcomas can occur in females at any age, including in childhood.

Basal cell carcinoma

Basal cell carcinoma, the most common type of skin cancer, is more often found on sun-exposed areas of the skin. Develops from cells called basal cells that are found in the deepest layer of the skin of the vulva. It occurs very rarely on the vulva.

Verrucous carcinoma

Looks like a large wart, again very rare.

Vulvar cancer outlook (prognosis)

The outlook for vulval cancer depends on things such as how far the cancer has spread, your age, and your general health. Generally, the earlier the cancer is detected and the younger you are, the better the chances of treatment being successful.

Overall, around 6 in every 10 women diagnosed with vulval cancer will survive at least five years. However, even after successful treatment, the cancer comes back in up to one in every three cases. You’ll need regular follow-up appointments so your doctor can check if this is happening.

What does vulvar cancer look like

Figure 2. Vulvar cancer – squamous cell carcinoma

Figure 3. Vulvar cancer – basal cell carcinoma

Figure 4. Vulvar cancer – melanoma

Vulvar cancer causes

Several risk factors for cancer of the vulva have been identified, and scientists are beginning to understand how these factors can cause cells in the vulva to become cancerous.

Researchers have made a lot of progress in understanding how certain changes in DNA can cause normal cells to become cancerous. DNA is the chemical that carries the instructions for nearly everything our cells do. We usually look like our parents because they are the source of our DNA. However, DNA affects more than our outward appearance. Some genes (parts of your DNA) contain instructions for controlling when our cells grow and divide.

• Certain genes that promote cell division are called oncogenes.
• Others that slow down cell division or cause cells to die at the right time are called tumor suppressor genes.

Cancers can be caused by DNA mutations (defects) that turn on oncogenes or turn off tumor suppressor genes. Usually DNA mutations related to cancers of the vulva occur during life rather than having been inherited before birth. Acquired mutations may result from cancer-causing chemicals in tobacco smoke. Sometimes they occur for no apparent reason.

Studies suggest that squamous cell cancer of the vulva (the most common type ~ 90%) can develop in at least 2 ways. In up to half of cases, human papillomavirus (HPV) infection appears to have an important role. Vulvar cancers associated with HPV infection (the basaloid and warty subtypes) seem to have certain distinctive features. They are often found along with several other areas of vulvar intraepithelial neoplasia (VIN). The women who have these cancers tend to be younger and are often smokers.

The second process by which vulvar cancers develop does not involve HPV infection. Vulvar cancers not linked to HPV infection (the keratinizing subtype) are usually diagnosed in older women (over age 55). These women may have lichen sclerosis and may also have the differentiated type of VIN. DNA tests from vulvar cancers in older women rarely show HPV infection, but often show mutations of the p53 tumor suppressor gene. The p53 gene is important in preventing cells from becoming cancerous. When this gene has undergone mutation, it is easier for cancer to develop. Younger vulvar cancer patients with HPV infection rarely have p53 mutations.

These discoveries have not yet affected treatment. But they may help in finding ways to prevent cancer of the vulva and at some point might lead to changes in treatment.

Because vulvar melanomas and adenocarcinomas are so rare, much less is known about how they develop.

Risk Factors for Vulvar Cancer

A risk factor is anything that changes a person’s chance of getting a disease such as cancer. Different cancers have different risk factors. For example, exposing skin to strong sunlight is a risk factor for skin cancer. Smoking is a risk factor for many cancers.

There are different kinds of risk factors. Some, such as your age or race, can’t be changed. Others may be related to personal choices such as smoking, drinking, or diet. Some factors influence risk more than others. But risk factors don’t tell us everything. Having a risk factor, or even several, does not mean that a person will get the disease. Also, not having any risk factors doesn’t mean that you won’t get it, either.

Although several risk factors increase the odds of developing vulvar cancer, most women with these risks do not develop it. And some women who don’t have any apparent risk factors develop vulvar cancer. When a woman develops vulvar cancer, it is usually not possible to say with certainty that a particular risk factor was the cause.

Age

The risk of vulvar cancer goes up as women age. Less than 20% of cases are in women younger than age 50, and more than half occur in women over age 70. The average age of women diagnosed with invasive vulvar cancer is 70, whereas women diagnosed with non-invasive vulvar cancer average about 20 years younger.

Human papillomavirus

HPV stands for human papillomavirus. HPVs are a large group of related viruses. They are called papillomaviruses because some of them cause a type of growth called a papilloma. Papillomas — more commonly known as warts — are not cancers. Different HPV types can cause different types of warts in different parts of the body. Some types cause common warts on the hands and feet. Other types tend to cause warts on the lips or tongue.

Certain HPV types can infect the outer female and male genital organs and the anal area, causing raised, bumpy warts. These warts may barely be visible or they may be several inches across. The medical term for genital warts is condyloma acuminatum. Two types of HPV (HPV 6 and HPV 11) cause most cases of genital warts, but are seldom linked to cancer and are known as low-risk HPV.

Other HPV types have been linked with cancers of the cervix, vagina, and vulva in women, cancer of the penis in men, and cancers of the anus and throat (in men and women). These are known as high-risk types of HPV and include HPV 16 and HPV 18 as well as others. Infection with a high-risk HPV may produce no visible signs until pre-cancerous changes or cancer develops.

HPV can pass from one person to another during skin-to-skin contact. One way HPV is spread is through sexual activity, including vaginal and anal intercourse and even oral sex.

Some doctors think there are 2 kinds of vulvar cancer. One kind is associated with HPV infection (more than half of all vulvar cancers are linked to infection with the high-risk HPV types) and tends to occur in younger women. The other is not associated with HPV infection, is more often found in older women, and may develop from a precursor lesion called differentiated vulvar intraepithelial neoplasia.

Vaccines have been developed to help prevent infection with some types of HPV.

Smoking

Smoking exposes people to many cancer-causing chemicals that affect more than their lungs. These harmful substances can be absorbed into the lining of the lungs and spread throughout the body. Smoking increases the risk of developing vulvar cancer. Among women who have a history of HPV infection, smoking further increases the risk of developing vulvar cancer. If women are infected with a high-risk HPV, they have a much higher risk of developing vulvar cancer if they smoke.

HIV infection

HIV (human immunodeficiency virus) causes AIDS (acquired immunodeficiency syndrome). Because this virus damages the immune system, it makes women more likely to get and to stay infected with HPV. This could increase the risk of vulvar pre-cancer and cancer. Scientists also believe that the immune system plays a role in destroying cancer cells and slowing their growth and spread.

Vulvar intraepithelial neoplasia (VIN)

Squamous cell carcinoma of the vulva usually forms slowly over many years. Pre-cancerous changes often occur first and can last for several years. The medical term most often used for this pre-cancerous condition is vulvar intraepithelial neoplasia (VIN). Intraepithelial means that the abnormal cells are only found in the surface layer of the vulvar skin (epithelium).

VIN is typed by how the lesions and cells look: usual-type VIN and differentiated-type VIN. It is sometimes graded VIN 2 and VIN 3, with the number 3 indicating furthest progression toward a true cancer. However, many doctors use only one grade of VIN.

• Usual-type VIN occurs in younger women and is caused by HPV infection. When usual-type VIN changes into invasive squamous cell cancer, it becomes the basaloid or warty subtypes.
• Differentiated-type VIN tends to occur in older women and is not linked to HPV infection. It can progress to the keratinizing subtype of invasive squamous cell cancer.

In the past, the term dysplasia was used instead of VIN, but this term is used much less often now. When talking about dysplasia, there is also a range of increasing progress toward cancer — first, mild dysplasia; next, moderate dysplasia; then severe dysplasia; and, finally, carcinoma in situ.

Although women with VIN have an increased risk of developing invasive vulvar cancer, most cases of VIN never progress to cancer. Still, since it is not possible to tell which cases will become cancers, treatment or close medical follow-up is needed.

The risk of progression to cancer seems to be highest with VIN3 and lower with VIN2. This risk can be altered with treatment. In one study, 88% of untreated VIN3 progressed to cancer, but of the women who were treated, only 4% developed vulvar cancer.

In the past, cases of VIN were included in the broad category of disorders known as vulvar dystrophy. Since this category included a wide variety of other diseases, most of which are not pre-cancerous, most doctors no longer use this term.

Lichen sclerosus

This disorder, also called lichen sclerosus et atrophicus, causes the vulvar skin to become very thin and itchy. The risk of vulvar cancer appears to be slightly increased by lichen sclerosus et atrophicus, with about 4% of women having lichen sclerosus et atrophicus later developing vulvar cancer.

Other genital cancers

Women with cervical cancer also have a higher risk of vulvar cancer. This is probably because these cancers share certain risk factors. The same HPV types that are linked to cervical cancer are also linked to vulvar cancer. Smoking is also linked to a higher risk of both cervical and vulvar cancers.

Melanoma or atypical moles

Women who have had melanoma or dysplastic nevi (atypical moles) in other places have an increased risk of developing a melanoma on the vulva. A family history of melanoma also leads to an increased risk.

Vulvar Cancer Prevention

The risk of vulvar cancer can be lowered by avoiding certain risk factors and by having pre-cancerous conditions treated before an invasive cancer develops. Taking these steps cannot guarantee that all vulvar cancers are prevented, but they can greatly reduce your chances of developing vulvar cancer.

Avoid HPV infection

Infection with human papillomavirus (HPV) is a risk factor for vulvar cancer. In women, HPV infections occur mainly at younger ages and are less common in women over 30. The reason for this is not clear.

HPV is passed from one person to another during skin-to-skin contact with an infected area of the body. HPV can be spread during sexual activity — including vaginal intercourse, anal intercourse, and oral sex — but sex doesn’t have to occur for the infection to spread. All that is needed is skin-to-skin contact with an area of the body infected with HPV. The virus can be spread through genital-to-genital contact. It is even possible to spread a genital infection through hand-to-genital contact.

An HPV infection also seems to be able to be spread from one part of the body to another. This means that an infection may start in the cervix and then spread to the vagina and vulva.

It can be very hard to avoid being exposed to HPV. If you are sexually active, limiting the number of sex partners and avoiding sex with people who have had many other sex partners can help lower your risk of exposure to HPV. But again, HPV is very common, so having sex with even one other person can put you at risk.

Infection with HPV is common, and in most cases your body is able to clear the infection on its own. But in some cases, the infection does not go away and becomes chronic. Chronic infection, especially with high-risk HPV types, can eventually cause certain cancers, including vulvar cancer.

A person can be infected with HPV for years and not have any symptoms, so the absence of visible warts cannot be used to tell if someone has HPV. Even when someone doesn’t have warts (or any other symptom), he (or she) can still be infected with HPV and pass the virus to somebody else.

Condoms

Condoms (rubbers) provide some protection against HPV, but they do not completely prevent infection. Condoms cannot protect completely because they don’t cover every possible HPV-infected area of the body, such as the skin on the genital or anal area. Still, condoms do provide some protection against HPV, and they also protect against HIV and some other sexually transmitted diseases.

Get vaccinated

Vaccines that protect against certain HPV infections are available. All of them protect against infection with HPV subtypes 16 and 18. Some can also protect against infections with other HPV subtypes, including some types that cause anal and genital warts.

These vaccines can only be used to prevent HPV infection –they do not help treat an existing infection. To be most effective, the vaccine should be given before a person becomes exposed to HPV (such as through sexual activity).

All of these vaccines can help prevent cervical cancer and pre-cancers. They are also approved to help prevent anal and genital warts, as well as other cancers.

More HPV vaccines are being developed and tested.

Don’t smoke

Not smoking is another way to lower the risk for vulvar cancer. Women who don’t smoke are also less likely to develop a number of other cancers, like those of the lungs, mouth, throat, bladder, kidneys, and several other organs.

Get regular pelvic checkups

Pre-cancerous vulvar conditions that are not causing any symptoms can be found by regular gynecologic checkups. It is also important to see your health care provider if any problems come up between checkups. Symptoms such as vulvar itching, rashes, moles, or lumps that don’t go away could be caused by vulvar pre-cancer and should be checked out. If vulvar intraepithelial neoplasia (VIN) is found, treating it might help prevent invasive squamous cell vulvar cancer. Also, some vulvar melanomas can be prevented by removing atypical moles.

The vulva is examined at the same time a woman has a pelvic examination. Cervical cancer screening with a Pap test (sometimes combined with a HPV test) is often done at the same time. Neither the Pap test nor the HPV test is used to screen for vulvar cancer. The purpose of these tests is to find cervical cancers and pre-cancers early.

How Pap tests and pelvic examinations are done

First, the skin of the outer lips (labia majora) and inner lips (labia minora) is examined for any visible abnormalities. The health care professional then places a speculum inside the vagina. A speculum is a metal or plastic instrument that keeps the vagina open so that the cervix can be seen clearly. Next, using a small spatula, a sample of cells and mucus is lightly scraped from the exocervix (the surface of the cervix that is closest to the vagina). A small brush or a cotton-tipped swab is then inserted into the cervical opening to take a sample from the endocervix (the inside part of the cervix that is closest to the body of the uterus). Then, the speculum is removed. The doctor then will check the organs of the pelvis by inserting 1 or 2 gloved fingers of one hand into the vagina while he or she palpates (feels) the lower abdomen, just above the pubic bone, with the other. The doctor may do a rectal exam at this time also. It is very important to know that a Pap test is not always done when a pelvic exam is done, so if you are uncertain you should ask if one was done.

Self-exam of the vulva

For most women, the best way to find VIN and vulvar cancer is to report any signs and symptoms to their health care provider and have a yearly well-woman exam. If you have an increased risk of vulvar cancer, you may also want to check your vulva regularly to look for any of the signs of vulvar cancer. This is known as self-examination. Some women choose to examine themselves monthly using a mirror. This can allow you to become aware of any changes in the skin of your vulva. If you do this, look for any areas that are white, darkly pigmented, or red and irritated. You should also note any new growths, nodules, bumps, or ulcers (open sores). Report any of these to a doctor, since they could indicate a vulvar cancer or pre-cancer.

Can Vulvar Cancer Be Found Early?

Having pelvic exams and knowing any signs and symptoms of vulvar cancer greatly improve the chances of early detection and successful treatment. If you have any of the problems discussed in signs and symptoms of vulvar cancers and pre-cancers, you should see a doctor. If the doctor finds anything abnormal during a pelvic examination, you may need more tests to figure out what is wrong. This may mean referral to a gynecologist (specialist in problems of the female genital system).

Knowing what to look for can sometimes help with early detection, but it is even better not to wait until you notice symptoms. Get regular well-women exams.

There is no standard screening for this disease.

Vulvar cancer signs and symptoms

Symptoms depend on whether it is a cancer or pre-cancer and what kind of vulvar cancer it is.

Signs and symptoms of vulvar cancer may include:

• A lump in the vulva or mass on the vulva
• Vulvar itching or tenderness that lasts more than one month
• A burning pain when passing urine
• Vulvar pain
• Burning in the genital area that lasts even after your doctor has treated the burning
• Bleeding from the vulva (different from your usual menstrual bleeding)
• Changes in the vulvar skin, such as color changes or growths that look like a wart or ulcer
• A mole on the vulva that changes shape or color
• A cut or sore on the vulva that won’t heal

Vulvar intraepithelial neoplasia

Most women with vulvar intraepithelial neoplasia (VIN) have no symptoms at all. When a woman with VIN does have a symptom, it is most often itching that does not go away or get better. An area of VIN may look different from normal vulvar skin. It is often thicker and lighter than the normal skin around it. However, an area of VIN can also appear red, pink, or darker than the surrounding skin.

Because these changes are often caused by other conditions that are not pre-cancerous, some women don’t realize that they might have a serious condition. Some try to treat the problem themselves with over-the-counter remedies. Sometimes doctors might not even recognize the condition at first.

Invasive squamous cell cancer of the vulva

Almost all women with invasive vulvar cancers will have symptoms. These can include:

• An area on the vulva that looks different from normal – it could be lighter or darker than the normal skin around it, or look red or pink.
• A bump or lump, which could be red, pink, or white and could have a wart-like or raw surface or feel rough or thick
• Thickening of the skin of the vulva
• Itching
• Pain or burning
• Bleeding or discharge not related to the normal menstrual period
• An open sore (especially if it lasts for a month or more)

Verrucous carcinoma, a subtype of invasive squamous cell vulvar cancer, looks like cauliflower-like growths similar to genital warts.

These symptoms are more often caused by other, non-cancerous conditions. Still, if you have these symptoms, you should have them checked by a doctor or nurse.

Vulvar melanoma

Patients with vulvar melanoma can have many of the same symptoms as other vulvar cancers, such as:

• A lump
• Itching
• Pain
• Bleeding or discharge

Most vulvar melanomas are black or dark brown, but they can be white, pink, red, or other colors. They can be found throughout the vulva, but most are in the area around the clitoris or on the labia majora or minora.

Vulvar melanomas can sometimes start in a mole, so a change in a mole that has been present for years can also indicate melanoma. The ABCDE rule can be used to help tell a normal mole from one that could be melanoma.

• Asymmetry: One-half of the mole does not match the other.
• Border irregularity: The edges of the mole are ragged or notched.
• Color: The color over the mole is not the same. There may be differing shades of tan, brown, or black and sometimes patches of red, blue, or white.
• Diameter: The mole is wider than 6 mm (about 1/4 inch).
• Evolving: The mole is changing in size, shape, or color.

The most important sign of melanoma is a change in size, shape, or color of a mole. Still, not all melanomas fit the ABCDE rule.

If you have a mole that has changed, ask your doctor to check it out.

Bartholin gland cancer

A distinct mass (lump) on either side of the opening to the vagina can be the sign of a Bartholin gland carcinoma. More often, however, a lump in this area is from a Bartholin gland cyst, which is much more common (and is not a cancer).

Paget disease

Soreness and a red, scaly area are symptoms of Paget disease of the vulva.

Vulvar cancer diagnosis

Diagnosing vulvar cancer

Tests and procedures used to diagnose vulvar cancer include:

• Examining your vulva. Your doctor will likely conduct a physical exam of your vulva to look for abnormalities.
• Using a special magnifying device to examine your vulva. During a colposcopy exam, your doctor uses a device that works like a magnifying glass to closely inspect your vulva for abnormal areas.
• Removing a sample of tissue for testing (biopsy). To determine whether an area of suspicious skin on your vulva is cancer, your doctor may recommend removing a sample of skin for testing. During a biopsy procedure, the area is numbed with a local anesthetic and a scalpel or other special cutting tool is used to remove all or part of the suspicious area. Depending on how much skin is removed, you may need stitches.

Biopsy

Certain signs and symptoms might strongly suggest vulvar cancer, but many of them can be caused by changes that aren’t cancer. The only way to be sure cancer is present is for the doctor to do a biopsy. To do this, a small piece of tissue from the changed area is removed and examined under a microscope. A pathologist (a doctor specially trained to diagnose diseases with laboratory tests) will look at the tissue sample with a microscope to see if cancer or pre-cancer cells are present and, if so, what type it is.

The doctor might use a colposcope or a hand-held magnifying lens to select areas to biopsy. A colposcope is an instrument that stays outside the body and has magnifying lenses. It lets the doctor see the surface of the vulva closely and clearly. The vulva is treated with a dilute solution of acetic acid (like vinegar) that causes areas of vulvar intraepithelial neoplasia (VIN) and vulvar cancer to turn white. This makes them easier to see through the colposcope. Examining the vulva with magnification is called vulvoscopy.

Less often, the doctor might wipe the vulva with a dye (called toluidine blue) to find areas of abnormal vulvar skin to biopsy. This dye causes skin with certain diseases — including VIN and vulvar cancer — to turn blue.

Once the abnormal areas are found, a numbing medicine (local anesthetic) is injected into the skin so you won’t feel pain. If the abnormal area is small, it may be completely removed (called an excisional biopsy). Sometimes stitches are needed afterward.

If the abnormal area is larger, a punch biopsy is used to take a small piece of it. The instrument used looks like a tiny apple corer and removes a small, cylinder of skin about 4 mm (about 1/6 inch) across. Stitches aren’t usually needed after a punch biopsy. Depending on the results of the punch biopsy, more surgery may be needed.

Determining the extent of the cancer

Once your diagnosis is confirmed, your doctor works to determine the size and extent (stage) of your cancer. Staging tests can include:

Examination of your pelvic area for cancer spread. Your doctor may do a more thorough examination of your pelvis for signs that the cancer has spread.
Imaging tests. Images of your chest or abdomen may show whether the cancer has spread to those areas. Imaging tests may include X-ray, computerized tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET).

Imaging tests

Imaging tests use sound waves, x-rays, magnetic fields, or radioactive substances to create pictures of the inside of your body.

Chest x-ray

An x-ray of your chest may be done to see if cancer has spread to your lungs.

Computed tomography (CT) scan

A CT scan is an x-ray test that makes detailed cross-sectional images of your body. CT scans are not often needed, but they might be done in women with large vulvar tumors or enlarged lymph nodes. They can also be helpful in deciding whether to do a sentinel lymph node procedure to check groin lymph nodes for cancer spread.

Magnetic resonance imaging (MRI)

An MRI uses radio waves and strong magnets instead of x-rays to make images of the body. Like a CT scanner, it produces cross sectional slices of the body. MRI images are very useful in examining pelvic tumors. They can show enlarged lymph nodes in the groin. But, they’re rarely used in patients with early vulvar cancer.

Positron emission tomography (PET) scan

A PET scan uses a form of radioactive sugar that’s put into the blood. Body cells take in different amounts of the sugar, depending on how fast they’re growing. Cancer cells grow quickly and are more likely to take up larger amounts of the sugar than normal cells. A special camera is then used to create a picture of areas of radioactivity in the body.

This test can be helpful for spotting collections of cancer cells, and seeing if the cancer has spread to lymph nodes. The picture from a PET scan is not as detailed as a CT or MRI scan, but it provides helpful information about whether abnormal areas seen on these other tests are likely to be cancer or not.

PET scans are also useful when your doctor thinks the cancer has spread, but doesn’t know where (although they aren’t useful for finding cancer spread in the brain). PET scans can be used instead of several different x-rays because they scan your whole body. Often, a machine that combines a PET scanner and a CT scanner (called a PET/CT) is used, which gives more information about areas of cancer and cancer spread.

Other tests to look for cancer

These tests aren’t often used, but if the doctor suspects the cancer has spread to nearby organs, other tests may be used to look for it. These tests let the doctor directly look inside your body for signs of cancer. You may be given drugs to put you into a deep sleep (general anesthesia) while the test is done.

Cystoscopy

The doctor uses a lighted tube to check the inside lining of your bladder. Some advanced cases of vulvar cancer can spread to the bladder, so any suspicious areas noted during this exam are biopsied. This procedure also can be done using a local anesthetic, where the area is just numbed, but some patients may need general anesthesia.

Proctoscopy

This lets the doctor look at the inside of the rectum using a thin, lighted tube. Some advanced cases of vulvar cancer can spread to the rectum. Any suspicious areas are biopsied.

Examination of the pelvis while under anesthesia

Putting the patient into a deep sleep (under anesthesia) allows the doctor to do a more thorough exam that can better evaluate how much the cancer has spread to internal organs of the pelvis.

Blood tests

Your doctor might also order certain blood tests to help get an idea of your overall health and how well certain organs, like your liver and kidneys, are working.

Vulvar cancer staging

After a woman is diagnosed with vulvar cancer, doctors will try to figure out if it has spread, and if so, how far. This process is called staging. The stage of a cancer describes the amount of cancer in the body. It helps determine how serious the cancer is and how best to treat it. Doctors also use a cancer’s stage when talking about survival statistics.

Vulvar cancer stages range from stage I (1) through IV (4). As a rule, the lower the number, the less the cancer has spread. A higher number, such as stage IV, means cancer has spread more. Although each person’s cancer experience is unique, cancers with similar stages tend to have a similar outlook and are often treated in much the same way.

How is the stage determined?

The 2 systems used for staging vulvar cancer, the FIGO (International Federation of Gynecology and Obstetrics) system and the AJCC (American Joint Committee on Cancer ) TNM staging system are basically the same.

They both stage (classify) this cancer based on 3 pieces of information:

1. The extent (size) of the tumor (T): How large and deep has the cancer grown? Has the cancer reached nearby structures or organs like the bladder or rectum?
2. The spread to nearby lymph nodes (N): How many lymph nodes has the cancer spread to and has it grown outside of those lymph nodes?
3. The spread (metastasis) to distant sites (M): Has the cancer spread to distant lymph nodes or distant organs?

Numbers or letters after T, N, and M provide more details about each of these factors. Higher numbers mean the cancer is more advanced. Once a person’s T, N, and M categories have been determined, this information is combined in a process called stage grouping to assign an overall stage.

The staging system in the table below uses the pathologic stage (also called the surgical stage). It is determined by examining tissue removed during an operation. . Sometimes, if surgery is not possible right away, the cancer will be given a clinical stage instead. This stage is based on the results of a physical exam, biopsy, and imaging tests done before surgery.

The system described below is the most recent AJCC (American Joint Committee on Cancer ) system, effective January 2018.

These systems are not used to stage vulvar melanoma, which is staged like melanoma of the skin.

Vulvar cancer staging can be complex, so ask your doctor to explain it to you in a way you understand.

Your vulvar cancer is assigned a Roman numeral that denotes its stage. Stages of vulvar cancer include:

• Stage I describes a small tumor that is confined to the vulva or the area of skin between your vaginal opening and anus (perineum). This cancer hasn’t spread to your lymph nodes or other areas of your body.
• Stage II tumors are those that have grown to include nearby structures, such as the lower portions of the urethra, vagina and anus.
• Stage III cancer has spread to lymph nodes.
• Stage IV signifies a cancer that has spread more extensively to the lymph nodes, or that has spread to the upper portions of the urethra or vagina, or that has spread to the bladder, rectum or pelvic bone. Cancer may have spread (metastasized) to distant parts of your body.

Vulvar melanoma uses a different staging system.

Table 1. Vulvar cancer stages

* The following additional categories are not listed on the table above:

• TX: Main tumor cannot be assessed due to lack of information.
• T0: No evidence of a primary tumor.
• NX: Regional lymph nodes cannot be assessed due to lack of information.

Vulvar cancer survival rates

The 5-year survival rate refers to the percentage of patients who live at least 5 years after their cancer is diagnosed. Five-year survival rates are used to produce a standard way of discussing prognosis. Of course, many people live much longer than 5 years.

Relative survival rates assume that people will die of other causes and compare the observed survival with that expected for people without vulvar cancer. This is a more accurate way to describe the outlook for patients with a particular type and stage of cancer.

Keep in mind that 5-year survival rates are based on patients diagnosed and initially treated more than 5 years ago. Improvements in treatment often result in a more favorable outlook for women more recently diagnosed with vulvar cancer.

Survival rates are often based on previous outcomes of large numbers of people who had the disease, but they cannot predict what will happen in any particular person’s case. Many other factors may affect a person’s outlook, such as the type of vulvar cancer, the patient’s age and general health, the treatment received, and how well the cancer responds to treatment. Your doctor can tell you how the numbers below may apply to your situation.

These numbers come from the National Cancer Institute’s SEER program. SEER does not list survival rates by FIGO (or AJCC) stage. Instead, it divides vulvar cancers into 3 summary stages:

• Local (stages I and II): The cancer is only in the vulva, without spread to lymph nodes or nearby tissues.
• Regional (stages III and IVA):The cancer has spread to nearby lymph nodes or tissues, but hasn’t spread to distant organs.
• Distant (stage IVB): The cancer has spread to distant organs or tissues.

Table 2. Vulvar cancer survival rates

Vulvar cancer treatment

After the stage of your vulvar cancer is known, your cancer care team will talk with you about treatment options. Think about your options without feeling rushed. If there’s anything you don’t understand, ask to have it explained again.

• The choice of treatment depends largely on the stage of the disease at the time of diagnosis, but other factors can play a part in choosing the best treatment plan, such as your age, your general health, your individual circumstances, and your preferences. Be sure you understand all the risks and side effects of the various options before making a decision.

The 3 main types of treatment used for women with vulvar cancer are

• Surgery
• Radiation therapy
• Chemotherapy

Vulvar pre-cancers (vulvar intraepithelial neoplasia or VIN) can also be treated with topical therapy.

Surgery to remove vulvar cancer

Operations used to treat vulvar cancer include:

• Removing the cancer and a margin of healthy tissue (excision). This procedure, which may also be called a wide local excision or radical excision, involves cutting out the cancer and a small amount of normal tissue that surrounds it. Cutting out what doctors refer to as a margin of normal-looking tissue helps ensure that all of the cancerous cells have been removed.
• More extensive surgery. Surgery to remove part of the vulva (partial vulvectomy) or the entire vulva, including the underlying tissue (radical vulvectomy), may be an option for larger cancers. However, doctors may recommend combining radiation therapy and chemotherapy to try to shrink the tumor before surgery, which may allow for a less extensive operation.

Surgery to remove the entire vulva carries a risk of complications, such as infection and problems with healing around the incision.

Surgery to your vulva may change sensation in your genital area. Depending on the operation, your genital area may feel numb and you may not be able to achieve orgasm during sexual intercourse.

Surgery to remove nearby lymph nodes

Vulvar cancer can spread to the lymph nodes in the groin, so your doctor may remove these lymph nodes at the time you undergo surgery to remove the cancer. Depending on your situation, your doctor may remove only a few lymph nodes or many lymph nodes.

Removing lymph nodes can cause fluid retention and leg swelling, a condition called lymphedema.

In certain situations, surgeons may use a technique that allows them to remove fewer lymph nodes. Called sentinel lymph node biopsy, this procedure involves identifying the lymph node where the cancer is most likely to spread first. The surgeon then removes that lymph node for testing. If cancer cells aren’t found in that lymph node, then it’s unlikely that cancer cells have spread to other lymph nodes.

Radiation therapy

Radiation therapy uses high-powered energy beams, such as X-rays and protons, to kill cancer cells. Radiation therapy for vulvar cancer is usually administered by a machine that moves around your body and directs radiation to precise points on your skin (external beam radiation).

Radiation therapy is sometimes used to shrink large vulvar cancers in order to make it more likely that surgery will be successful. Radiation is sometimes combined with chemotherapy, which can make cancer cells more vulnerable to radiation therapy.

If cancer cells are discovered in your lymph nodes, your doctor may recommend radiation to the area around your lymph nodes to kill any cancer cells that might remain after surgery. Radiation is sometimes combined with chemotherapy in these situations.

Chemotherapy

Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. Chemotherapy drugs are typically administered through a vein in your arm or by mouth.

For those with advanced vulvar cancer that has spread to other areas of the body, chemotherapy may be an option.

Chemotherapy is sometimes combined with radiation therapy to shrink large vulvar cancers in order to make it more likely that surgery will be successful. Chemotherapy may also be combined with radiation if there’s evidence cancer has spread to the lymph nodes.

Follow-up tests after treatment

After completing vulvar cancer treatment, your doctor may recommend periodic follow-up exams to look for a cancer recurrence. Even after successful treatment, vulvar cancer can return. Your doctor will determine the schedule of follow-up exams that’s right for you, but doctors generally recommend exams two to four times each year for the first two years after vulvar cancer treatment.

Coping and support

Living with vulvar cancer can be challenging. Although there are no easy answers for coping with vulvar cancer, the following suggestions may help:

• Learn enough about vulvar cancer to feel comfortable making treatment decisions. Ask your doctor to explain the basics of your cancer, such as what types of cells are involved and what stage is your cancer. Also ask your doctor or nurse to recommend good sources of information. Learn enough about your cancer so that you feel comfortable asking questions and discussing your treatment options with your doctor.
• Talk to someone about your feelings. When you feel ready, consider talking to someone you trust about your hopes and fears as you face cancer treatment. This might be a friend, a family member, your doctor, a social worker, a spiritual adviser or a counselor.
• Connect with other cancer survivors. You may find it helpful to talk to other people with vulvar cancer. They can tell you how they’ve coped with problems similar to the ones you’re facing. Ask your doctor about support groups in your area. The National Cancer Institute ¹⁾ and the American Cancer Society ²⁾ are good places to start.
• Consider joining a support group for people with cancer. You may find strength and encouragement in being with people who are facing the same challenges you are. Ask your doctor, nurse or social worker about groups in your area. Or try online message boards, such as those available through the American Cancer Society ³⁾.
• Don’t be afraid of intimacy. Your natural reaction to changes in your body may be to avoid intimacy. Although it may not be easy, discuss your feelings with your partner. You may also find it helpful to talk to a therapist, either on your own or together with your partner. Remember that you can express your sexuality in many ways. Touching, holding, hugging and caressing may become far more important to you and your partner.