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vulvar-cancer

Vulvar Cancer

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What is vulvar cancer?

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

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. These inner and outer labia (Latin for lips) 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 thefourchette. Beyond the fourchette is the anus, the opening to the rectum. The space between the vagina and the anus is called the perineum.

Types of vulvar cancer

Squamous cell carcinomas : Most cancers of the vulva are squamous cell carcinomas. This type of cancer begins in squamous cells, the main type of skin cells. There are several subtypes of squamous cell carcinoma. The keratinizing type is most common, and usually develops in older women, and is not linked to infection with human papilloma virus (HPV). Basaloid and warty types are less common, and are the kinds more often found in younger women with HPV infections. Verrucous carcinoma is an uncommon subtype that is important to recognize because it is slow-growing and tends to have a good prognosis (outlook). This cancer looks like a large wart and a biopsy is needed to determine it is not a benign (non-cancerous) growth.

Adenocarcinoma :Cancer that begins 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 (accumulation of fluid in the gland), so a delay in accurate diagnosis is common. 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 develop from 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. Around 5 of every 100 melanomas in women occur on the vulva, usually on the labia minora and clitoris. They make up about 6 of every 100 vulvar cancers. 

Sarcoma : A sarcoma is a cancer that begins 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. It occurs very rarely on the vulva. 

What are the 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 without 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 papilloma virus : Human papilloma virus (HPV) is a group of more than 100 types of viruses. They are called papilloma viruses 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 iscondyloma acuminatum. 2 types of HPV, HPV 6 and HPV 11, cause most cases of genital warts. These 2 types are seldom linked to cancer, and so are called low-risk types of HPV.

Other HPV types have been linked with cancers of the cervix 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, HPV 18, HPV 31, as well as others. Infection with a high-risk HPV may produce no visible signs until pre-cancerous changes or cancer develops.

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

About half of all vulvar cancers are linked to infection with the high-risk HPV types. HPV linked vulvar cancer is more common in younger women and is seen less often in older women. Some doctors think there are 2 kinds of vulvar cancer. One is associated with HPV infection and tends to occur in younger women. The other kind is not associated with HPV infection, and more often is found in older women.

Vaccines have been developed to help prevent infection with some types of HPV. Right now, 2 different HPV vaccines have been approved for use in the United States by the Food and Drug Administration (FDA): Gardasil® and Cervarix®. 

Smoking : Smoking exposes the body to many cancer-causing chemicals that affect more than the 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.

Human immunodeficiency virus : Human immunodeficiency virus (HIV) causes the acquired immunodeficiency syndrome (AIDS). Because this virus damages the body's immune system, it makes women more likely to get HPV and to stay infected with it. This may, in turn, 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 : Squamous cell carcinoma of the vulva usually forms slowly over many years. Often, pre-cancerous changes occur first and may 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).

There are 2 types of VIN based on how the lesions and cells look: usual-type VIN and differentiated-type VIN. Usual-type VIN occurs in younger women and is caused by HPV infection. It is sometimes split into 2 grades, VIN2 and VIN3, with the number 3 indicating furthest progression toward a true cancer. However, many doctors use only one grade of VIN. 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 VIN 3 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 (LSA), causes the vulvar skin to become very thin and itchy. The risk of vulvar cancer appears to be slightly increased by LSA, with about 4% of women having LSA 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) elsewhere on the body have an increased risk of developing a melanoma on the vulva. A family history of melanoma also leads to an increased risk.

Do we know what causes vulvar cancer?

Several risk factors for cancer of the vulva have been identified, and we 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 our 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) can develop in at least 2 ways. In up to half of cases, human papilloma virus (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 rarely 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.

Can vulvar cancer be prevented?

The risk of vulvar cancer can be reduced by avoiding certain risk factors and by treating pre-cancerous conditions before an invasive cancer develops. These steps cannot guarantee prevention but can greatly reduce your chances of developing vulvar cancer.

Avoid HPV infection:  Infection with human papilloma virus (HPV) is a risk factor for vulvar cancer. In women, genital 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 sex -- 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 for there to be 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 for a genital infection to spread 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. It might be possible to prevent genital HPV infection by not allowing others to have contact with your anal or genital area, but even then there could be other ways to become infected that aren’t yet clear.

Infection with HPV is common, and in most cases the 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.

Certain types of sexual behavior increase a woman's risk of getting a genital HPV infection, such as having sex at an early age and having many sexual partners. Although women who have had many sexual partners are more likely to get infected with HPV, a woman who has had only one sexual partner can still get infected. This is more likely if she has a partner who has had many sex partners or if her partner is an uncircumcised male.

Delaying sex until you are older can help you avoid HPV. It also helps to limit your number of sexual partners and to avoid having sex with someone who has had many other sexual partners.

A person can be infected with HPV for years without 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.

HPV in men: The 2 main factors influencing the risk of genital HPV infection in men are circumcision and the number of sexual partners. Men who are circumcised (have had the foreskin of the penis removed) have a lower chance of becoming and staying infected with HPV. Men who have not been circumcised are more likely to be infected with HPV and pass it on to their partners. The reasons for this are unclear. It may be that after circumcision the skin on the glans (of the penis) goes through changes that make it more resistant to HPV infection. Another theory is that the surface of the foreskin (which is removed by circumcision) is more easily infected by HPV. Still, circumcision does not completely protect against HPV infection - men who are circumcised can still get HPV and pass it on to their partners.

The risk of a man being infected with HPV is also strongly linked to having many sexual partners (over a man's lifetime).

Condoms and HPV : Condoms ("rubbers") provide some protection against HPV, but they do not completely prevent infection. Men who use condoms are less likely to be infected with HPV and pass it on to their female partners. One study found that when condoms are used correctly every time sex occurs, they can lower the HPV infection rate in women by about 70%. 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. Condoms (when used by the male partner) also seem to help genital HPV infections clear (go away) faster in both women and men.

Get vaccinated against HPV : A vaccine called Gardasil can help protect against infection with HPV subtypes 16 and 18 (as well as 6 and 11). In studies, this vaccine was found to prevent anal and genital warts caused by HPV types 6 and 11 and to prevent anal, vulvar, vaginal, and cervical cancers and pre-cancers caused by types 16 and 18.

This vaccine can only be used to prevent HPV infection -- it does 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).

Gardasil was originally only approved for use in women to prevent cervical cancer, but it is now also approved to prevent anal, vulvar, and vaginal cancers and pre-cancers. It is also approved to prevent anal and genital warts.

Cervarix, another HPV vaccine available in the United States, can also be used to prevent infection with HPV types 16 and 18, but so far it has only been shown to help prevent cervical cancers and pre-cancers and not any of the other cancers linked to HPV infection (such as vulvar cancer). Cervarix also seems to protect against some high risk HPV types besides types 16 and 18.

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.

Find and treat pre-cancerous conditions :Pre-cancerous vulvar conditions that are not causing any symptoms can be found through 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 may help prevent invasive squamous cell vulvar cancer. Also, some vulvar melanomas can be prevented by removing atypical moles.

Examination of the vulva is done 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. For more information about these tests and the American Cancer Society guidelines for the early detection of cervical cancer, see our documents Cervical Cancer, and Cervical Cancer: Prevention and Early Detection.

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. Then the health care professional inserts a speculum, a metal or plastic instrument that keeps the vagina open so that the cervix and vagina can be seen clearly. Next, for the Pap test, a sample of cells and mucus is lightly scraped from the exocervix (outer part) using a spatula or a broom. A small brush is used to sample 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 : 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 the next section, 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).

There is no standard screening for this disease other than routine physical examinations.

How is vulvar cancer diagnosed?

Signs and symptoms of vulvar cancers and pre-cancers

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 than 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. The symptoms of early invasive vulvar cancer are similar to those that can be seen with VIN. As the cancer grows, a distinct tumor may be seen. The cancer might appear as a red, pink, or white bump (or bumps) with a wart-like or raw surface. The area could also appear white and feel rough.

About half of the women with vulvar cancer complain of persistent itching and a growth. Some also complain of pain, burning, painful urination, and bleeding and discharge not associated with the normal menstrual period. An open sore (ulcer) that persists for more than a month is another sign.

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

Vulvar melanoma

 Melanoma can appear as a darkly pigmented growth. A change in a mole that has been present for years can also indicate melanoma. The ABCD 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).

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

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.

Paget disease

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

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.

Medical history and physical exam

The first step is for the doctor to take your complete medical history to check for risk factors and symptoms. Then your doctor will give you a complete physical exam, including a pelvic exam. He or she will feel your uterus, ovaries, cervix, and vagina for anything irregular. Your doctor will also use a speculum to look at your vagina and cervix and will take a Pap smear.

Biopsy : Certain signs and symptoms might strongly suggest vulvar cancer, but many of them can be caused by conditions that aren't cancer. The only way to be certain that cancer is present is to do a biopsy. In this procedure, a small piece of tissue from the suspicious area is removed and examined under the microscope. A pathologist (a doctor specializing in diagnosing diseases by laboratory tests) will look at the tissue sample under a microscope to see if cancer or a pre-cancerous condition is present and, if so, what type it is.

Rarely, the doctor will paint the vulva with toluidine blue dye to find all areas of abnormal vulvar skin and to select the best areas to biopsy. This dye causes skin with certain diseases -- including vulvar intraepithelial neoplasia (VIN) and vulvar cancer -- to turn blue.

The doctor might use a colposcope (an instrument with binocular magnifying lenses) or a hand-held magnifying lens to select areas to biopsy. The vulva is treated with a dilute solution of acetic acid (which is also the main ingredient in vinegar) that causes areas of VIN and cancer to turn white, making them easier to see through the colposcope. Examining the vulva with magnification is called vulvoscopy.

Once the abnormal areas are found, local anesthetic is injected into the skin to make it numb. If the abnormal area is small, it may be completely removed by an excisional biopsy. In this procedure, the doctor removes a small area of skin with a scalpel. Sometimes stitches may be needed..

If the abnormal area is larger, a punch biopsy is used to take a small sample. The instrument used looks like a tiny apple corer and removes a small, cylindrical piece of skin 4 mm (about 1/6 inch) across. No stitches are usually needed after the punch biopsy. Depending on the results of the punch biopsy, additional surgery may be necessary.

Further testing : If you have cancer, tests will be done to see how far it has spread. The results of your physical examination and certain diagnostic tests will be used to determine the size of the tumor, how deeply it has grown into tissues at the site where it originated, if it has grown into nearby organs, and if it has metastasized (spread to lymph nodes or distant organs). This is called staging. The stage of your cancer is the most important factor in selecting the right treatment plan. 

If your biopsy shows that you have vulvar cancer, your health care professional will refer you to a gynecologic oncologist, a specialist in female reproductive system cancers. The specialist will also look at your complete personal and family medical history to learn about related risk factors and symptoms of vulvar cancer.

The doctor will perform a complete physical examination to evaluate your general state of health, paying special attention to the lymph nodes, particularly those in your groin region, to check for signs of cancer spread. Depending on the biopsy results, several more tests may be done to determine if the vulvar cancer has spread to other areas.

Cystoscopy : This examination uses a lighted tube to check the inside surface of the bladder. Some advanced cases of vulvar cancer can spread to the bladder, so any suspicious areas noted by this exam are removed for biopsy. This procedure can be done using a local anesthetic, but some patients may need general anesthesia. Your doctor will let you know what to expect before and after the procedure. This procedure was used more often in the past, but is no longer a standard part of the work-up of a woman with vulvar cancer.

Proctoscopy : This is a visual inspection of the rectum using a lighted tube. Some advanced cases of vulvar cancer can spread to the rectum. Any suspicious areas are biopsied. This test was used more often in the past, but is no longer a standard part of the work-up of a woman with vulvar cancer.

Examination of the pelvis under anesthesia : This permits a more thorough exam that can better evaluate how much the cancer has spread to internal organs of the pelvis.

Imaging tests

Chest x-ray: A plain x-ray of your chest might be done to check for other health problems that might make certain treatments difficult to tolerate. This x-ray can be done in any outpatient setting.

Computed tomography (CT) : The computed tomography (CT) scan is an x-ray test that produces detailed cross-sectional images of your body. Instead of taking one picture, like a standard x-ray, a CT scanner takes many pictures as it rotates around you. A computer then combines these pictures into an image of a slice of your body. A CT scan can provide information about the size, shape, and position of tumors and can be helpful to see if the cancer has spread to other organs. It can also help find enlarged lymph nodes that might have cancer cells.

A CT scanner has been described as a large donut, with a narrow table in the middle opening. You will need to lie still on the table while the scan is being done. CT scans take longer than regular x-rays, and you might feel a bit confined by the ring while the pictures are being taken.

Before the test, you may be asked to drink 1 to 2 pints of a liquid called oral contrast. This helps outline the intestine so that certain areas are not mistaken for tumors. You may also receive an IV line through which a different kind of contrast dye (IV contrast) is injected. This helps better outline structures such as blood vessels in your body.

The injection can cause some flushing (redness and warm feeling). A few people are allergic to the dye and get hives, or rarely, have more serious reactions like trouble breathing and low blood pressure. Be sure to tell the doctor if you have ever had a reaction to any contrast material used for x-rays.

CT scans are not often needed in vulvar cancer patients. They might be done in patients with large 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) : Magnetic resonance imaging (MRI) scans use radio waves and strong magnets instead of x-rays to make images of the body. The energy from the radio waves is absorbed by the body and then released in a specific pattern formed by the type of tissue and by certain diseases. A computer translates the pattern into a very detailed image of parts of the body. Like a CT scanner, this produces cross sectional slices of the body. An MRI can also produce slices that are parallel with the length of your body. As with a CT scan, a contrast material might be used, but it is not needed as often.

MRI scans are more uncomfortable than CT scans. They take longer -- often up to an hour. You have to be placed inside tube-like equipment, which is confining and can upset people with claustrophobia (a fear of close spaces). If you have trouble with close spaces, let your doctor know before the MRI scan. Sometimes medication can be given just before the scan to reduce anxiety. Another option is to use a special "open" MRI machine that is less confining and more comfortable for such people, the drawback being that the images from these machines are not as good. The machine also makes a buzzing or clanging noise that some people find disturbing. Some places will provide headphones with music to block this sound.

MRI images are particularly useful in examining pelvic tumors. They may often detect enlarged lymph nodes in the groin. They are also helpful in detecting cancer that has spread to the brain or spinal cord. However, they are rarely used in patients with early vulvar cancer.

Positron emission tomography (PET) : Positron emission tomography (PET) uses glucose (a form of sugar) that contains a low-level radioactive atom. Because cancers use glucose at a higher rate than normal tissues, the radioactivity tends to concentrate in the cancer. A special camera is used to detect the radioactivity. This test can be helpful for spotting small collections of cancer cells, and can be useful in seeing if the cancer has spread to lymph nodes. PET scans are also useful when your doctor thinks the cancer has spread, but doesn’t know where. PET scans can be used instead of several different x-rays because they scan your whole body. Special devices combine a CT scan and a PET scan to even better pinpoint the tumor. However, these scans are rarely used in patients with early vulvar cancer.

How is vulvar cancer staged?

The FIGO/AJCC system for staging vulvar cancer

The 2 systems used for staging most types of vulvar cancer -- the FIGO (International Federation of Gynecology and Obstetrics) system and the American Joint Committee on Cancer TNM staging system -- are very similar. They both classify vulvar cancer on the basis of 3 factors: the extent of the tumor (T), whether the cancer has spread to lymph nodes (N) and whether it has spread to distant sites (M). The system described below is the most recent AJCC system, which went into effect January 2010. Any differences between the AJCC system and the FIGO system are explained in the text.

These systems are not used to stage vulvar melanoma, which is staged like melanoma of the skin. Information about melanoma staging can be found in our document, Melanoma Skin Cancer.

Tumor extent (T)

Tis: The cancer is not growing into the underlying tissues. This stage, also known as carcinoma in situ, is not included in the FIGO system.

T1: The cancer is growing only in the vulva or perineum

T1a: The cancer has grown no more than 1 mm into underlying tissue (stroma) and is 2 cm or smaller in size. (about 0.8 inches).

T1b: The cancer is either more than 2 cm or it has grown more than 1 mm into underlying tissue (stroma).

T2: The tumor can be any size. The cancer is growing into the anus or the lower third of the vagina or urethra (the tube that drains urine from the bladder). (This is called stage 2/3 in the FIGO system)

T3: The tumor can be any size. The cancer is growing into the upper urethra, bladder or rectum or into the pubic bone. (This is called stage 4 in the FIGO system)

Lymph node spread of cancer (N)

N0: No lymph node spread

N1: The cancer has spread to 1 or 2 lymph nodes in the groin with the following features:

N1a: The cancer has spread to 1 or 2 lymph nodes and the areas of cancer spread are both less than 5 mm (about 1/5th of an inch) in size

N1b: The cancer has spread to one lymph node and the area of cancer spread is 5 mm or greater

N2: The cancer has spread to groin lymph nodes with the following features:

N2a: The cancer has spread to 3 or more lymph nodes, but each area of spread is less than 5 mm

N2b: The cancer has spread to 2 or more lymph nodes with each area of spread 5 mm or greater

N2c: The cancer has spread to lymph nodes and has started growing through the outer covering of at least one of the lymph nodes (called extracapsular spread)

N3: The cancer has spread to the lymph nodes causing open sores (ulceration) or causing the lymph node to be stuck (fixed) to the tissue below it.

Distant spread of cancer (M)

M0: No distant spread

M1: The cancer has spread to distant sites (includes spread to pelvic lymph nodes)

Stage grouping

The grouping of T, N, and M determines the stage:

Stage 0 (Tis, N0, M0): This is a very early cancer found on the surface of the skin of the vulva only. It is also known as carcinoma in situ and as Bowen disease. This stage is not included in the FIGO system.

Stage I (T1, N0, M0): The cancer is in the vulva or the perineum (the space between the rectum and the vagina) or both. The tumor has not spread to lymph nodes or distant sites.

Stage IA (T1a, N0, M0): These are stage I cancers with tumors that are 2 cm or less that have grown into the underlying tissue no deeper than 1 mm (about 1/25 inch).

Stage IB (T1b, N0, M0): These are stage I cancers that have invaded deeper than 1 mm and/or are larger than 2 cm.

Stage II (T2, N0, M0): The cancer has grown outside the vulva or perineum to the anus or lower third of the vagina or urethra (T2). It has not spread to lymph nodes (N0) or distant sites (M0). In FIGO, this grouping is T2/T3, N0, M0, but it is still stage II.

Stage IIIA (T1 or T2, N1a or N1b, M0): Cancer is found in the vulva or perineum or both (T1) and may be growing into the anus, lower vagina, or lower urethra (T2). Either it has spread to a single nearby lymph node with the area of cancer spread 5 mm or greater in size (N1a); OR it has spread to 1 or 2 nearby lymph nodes with both areas of cancer spread less than 5 mm in size (N1b). It has not spread to distant sites (M0). In FIGO, this stage is also called IIIA.

Stage IIIB (T1 or T2, N2a or N2b, M0): Cancer is found in the vulva or perineum or both (T1) and may be growing into the anus, vagina, or lower urethra (T2). Either, the cancer has spread to 3 or more nearby lymph nodes, with all areas of cancer spread less than 5 mm in size (N2a); OR the cancer has spread to 2 or more lymph nodes with each area of spread 5 mm or greater in size (N2b). The cancer has not spread to distant sites (M0). In FIGO, this stage is also called IIIB.

Stage IIIC (T1 or T2, N2c, M0): Cancer is found in the vulva or perineum or both (T1) and may be growing into the anus, lower vagina, or lower urethra (T2). The cancer has spread to nearby lymph nodes and has started growing through the outer covering of at least one of the lymph nodes (called extracapsular spread; N2c). The cancer has not spread to distant sites (M0). In FIGO, this stage is also called IIIC.

Stage IVA: Either of the following:

T1 or T2, N3, M0: Cancer is found in the vulva or perineum or both (T1) and may be growing into the anus, vagina, or lower urethra (T2). Cancer spread to nearby lymph nodes has caused them to be stuck (fixed) to the underlying tissue or caused open sores (ulceration) (N3). It has not spread to distant sites. In FIGO, this stage is also called IVA.

OR

T3, any N, M0: The cancer has spread beyond nearby tissues to the bladder, rectum, pelvic bone, or upper part of the urethra (T3). It may or may not have spread to nearby lymph nodes (any N). It has not spread to distant sites (M0). In FIGO, this stage is also called IVA.

Stage IVB (any T, any N, M1): Cancer has spread to distant organs or lymph nodes (M1). This is the most advanced stage of cancer. In FIGO, this stage is also called IVB.

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