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Vulvar cancer is cancer that starts in the vulva. This is the outer part of the female reproductive system. This is also called the external genitalia.
The vulva includes the mons pubis. This is the rounded area in front of the pubic bones that becomes covered with hair at puberty. It also includes the skin folds under the pubic hair that protect the opening of the urethra (the tube that carries urine out of the body) and the vagina.
The vulva has two folds of skin. The outer folds are called the labia majora. The inner folds are called the labia minora.
The inner folds of the vulva form a hood of skin called the prepuce or the hood of the clitoris. Below the prepuce is the clitoris. The clitoris is a sensitive piece of tissue that swells with blood when stimulated.
At the bottom of the inner folds of the vulva is the fourchette. This is where the labia minora meet. Below the fourchette is the perineum. This is also part of the vulva. The perineum ends at the anus. The anus is where stool leaves the body.
Vulvar cancer is rare. It only accounts for about 4 percent of all female reproductive organ cancers. If it’s found in its early stages, vulvar cancer is highly curable. Fortunately, most cases are diagnosed in the earlier stages.
According to the American Cancer Society, about 5,950 women will be diagnosed with vulvar cancer in the U.S. in 2016. Most women with vulvar cancer are older than 50 years. More than half are older than 70 years at the time they learn they have cancer.
Certain factors can make women more likely to get vulvar cancer. These are called risk factors. Just because a woman has one or more risk factors does not mean she will get vulvar cancer. In fact, a woman can have all of the risk factors and still not get the disease. Or, a woman can have no known risk factors and get vulvar cancer. Experts aren’t exactly sure what causes vulvar cancer. However, possible risk factors can include:
Age. Women older than 50 years are more at risk for squamous cell carcinoma. This is the most common type of vulvar cancer.
Human papillomavirus (HPV) infection. HPV is a group of viruses that can cause genital warts. HPV has been linked to certain cancers. Women infected with certain types of HPV may be more at risk for vulvar cancer.
Smoking. Smoking increases your risk for vulvar cancer. If you smoke and have been infected with a high-risk strain of HPV, you’re at an even greater risk for vulvar cancer.
Vulvar intraepithelial neoplasia (VIN). This condition causes a change in the cells on the surface of the vulva. Women with VIN may be more likely to get vulvar cancer.
Lichen sclerosus. Women with this condition have vulvar skin that’s itchy and thin. These women are at a slightly higher risk of getting vulvar cancer.
Family history of melanoma. Women with a family history of melanoma or atypical moles have a higher risk of getting a melanoma of the vulva.
Human immunodeficiency virus (HIV) infection. Women with HIV are more at risk for vulvar cancer.
History of cervical cancer. Having cervical cancer is linked with a higher risk for vulvar cancer. The two types of cancer share some risk factors. These include having certain types of HPV infections and smoking.
Immunosuppression. Women whose immune system is weakened are at higher risk of vulvar cancer. This may include women with HIV. It may also include women who have had a transplant and are taking medicine to prevent rejection.
A woman can have vulvar cancer without having any symptoms. Note that some symptoms of vulvar cancer are similar to the symptoms of VIN. Symptoms of vulvar cancer can include:
Vulvar itching that does not improve
Change in skin color or feel of vulva
Red, white, or wart-like bump or sore on the vulva
Pain when urinating
Burning or bleeding and discharge that’s not related to the menstrual cycle
Enlarged glands (lymph nodes) in the groin
A new mole on the vulva or a change in a mole's size or appearance, including irregular color or borders
An ulcer or cracking of the vulvar skin that does not heal
If you have symptoms of vulvar cancer, see your healthcare provider. He or she may do the following:
Clinical history and physical exam. Your healthcare provider will ask questions to learn about your symptoms, personal and family history, and risk factors. A thorough physical exam includes a pelvic exam and a Pap test. These can also help rule out other problems.
HPV test. Your healthcare provider can test for HPV at the same time as a Pap test.
Colposcopy of the vulva. For this test, your healthcare provider uses a tool called a colposcope. This tool magnifies cells on the surface of the cervix, vagina, and vulva. It allows your healthcare provider to select suspicious looking spots of tissue to remove and examine. This is called a biopsy.
Biopsy. Your healthcare provider may take a small piece of tissue from the vulva. A pathologist looks at it under a microscope to check for cancer cells.
Your treatment depends on the type of vulvar cancer you have. It also depends on the results of lab tests and the stage or extent of the cancer. Your healthcare provider will also consider your age and general health when making treatment recommendations. The most common ways to treat vulvar cancer are with surgery and/or radiation. You may also need chemotherapy.
Many people with cancer get a second opinion from another healthcare provider. There are many reasons to get a second opinion. Here are some of those reasons:
Not feeling comfortable with the treatment decision
Being diagnosed with an invasive or rare type of vulvar cancer
Having several options for how to treat the cancer
Not being able to see a cancer expert
It may help to have a second healthcare provider review the diagnosis and treatment options before starting treatment. Gynecologic oncologists are doctors with advanced training in the diagnosis, treatment, and surveillance of female cancers. These include vulvar cancer. Know that, in most cases, a short delay in treatment will not lower the chance that it will work. Some health insurance companies even require that a person with cancer seek a second opinion. Many companies will pay for a second opinion.
There are many ways to get a second opinion:
Ask a primary care healthcare provider. He or she may be able to recommend a specialist. These can include a gynecologic oncologist, medical oncologist, or radiation oncologist. Sometimes these doctors work together at cancer centers or programs.
Call the Foundation for Women's Cancer. The number is 312-578-1439 They can help callers find a gynecologic oncologist for a second opinion. Or use the Find a Gynecologic Oncologist tool at their website to search for a gynecologic oncologist by zip code. You can also find one at the Society of Gynecologic Oncology.
Call the National Cancer Institute's Cancer Information Service. The number is 800-4-CANCER (800-422-6237). They have information about treatment facilities, including cancer centers and other programs supported by the National Cancer Institute.
Seek other options. Check with a local medical society, a nearby hospital, a medical school, or local cancer advocacy groups. Or talk with other women who've had vulvar cancer to get names of specialists who can give you a second opinion.
Clinical trials are studies of new kinds of cancer treatments. Experts use clinical trials to learn how well new treatments work and what their side effects are. Promising treatments are ones that work better or have fewer side effects than the current treatments. People who participate in these studies get to use treatments before the U.S. Food and Drug Administration (FDA) approves them. People who join trials also help researchers learn more about cancer and help future cancer patients. There is a large searchable database at www.clinicaltrials.gov where you can see what trials might be available for you.
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