Vulvar cancer occurs in the vulva, the external genital area of a woman's reproductive system. It can affect any part of the vulva, including the labia, the mons pubis (the skin and tissue that cover the pubic bone), the clitoris, or the vaginal or urethral openings. In most cases, it affects the inner edges of the labia majora or labia minora.
The vast majority of vulvar cancers are squamous cell carcinomas. This cancer starts in squamous cells, the main type of skin cells. Squamous cell cancer usually develops over many years. Before it forms, abnormal cells usually develop in the surface layer of the skin, called the epithelium. This condition is called vulvar intraepithelial neoplasia (VIN).
Another common form of vulvar cancer is melanoma. It usually occurs on the labia minora or the clitoris. Uncommon forms of vulvar cancer include Bartholin's gland adenocarcinoma and non-mammary Paget's disease. Few vulvar cancers are sarcomas. These cancers occur in the connective tissue beneath the skin.
Vulvar cancer is uncommon, accounting for a very small percentage of all cancers in women. Most women diagnosed with vulvar cancer are older than 50; and two-thirds are older than 70.
Recently, younger women have been getting diagnosed with VIN. With early detection and treatment of this precancerous condition, a woman may never develop actual vulvar cancer.
Risk factors for vulvar cancer include:
having abnormal, precancerous vulvar cells
being infected with human papilloma virus (HPV)
having a condition related to immune deficiency (an organ transplant, for example)
having vulvar dystrophy, a condition in which skin looks abnormal and is covered with white bumps
having precancerous changes to the vulva
having precancerous changes on the cervix or a history of cervical cancer
being of northern European ancestry
Common symptoms of vulvar cancer and VIN include:
persistent itching or burning anywhere on the vulva
a red, pink, or white lump with a wart-like or raw surface
a white, rough area on the vulva
painful urination or bleeding
discharge not related to your period
a skin ulcer that lasts more than a month
Signs of vulvar melanoma include a black or brown raised area, or a change in the size, shape, or color of a pre-existing mole.
Signs of a Bartholin's gland adenocarcinoma include a lump at the opening to the vagina. Having a lump doesn't mean you have cancer. It could be a common benign cyst. However, you should have your doctor examine the lump to make sure it is not cancerous.
A sore, red, scaly area on the vulva can be a sign of Paget's disease.
Some signs and symptoms of vulvar cancer can occur with noncancerous conditions, such as infection or trauma. Also, some noncancerous conditions may mimic vulvar cancer. If conservative treatment does not make these problems go away, you will need a biopsy to find out if they are cancerous.
Vulvar cancer is usually diagnosed with a biopsy. During a biopsy, your doctor will remove a small bit of tissue, usually from the center of the abnormal area to be sure that a representative sample is taken. A specialist will examine the tissue under a microscope to check for cancerous and precancerous cells.
Your doctor may use an instrument called a colposcope, which has magnifying lenses, to select the biopsy site. Before the colposcopy, your doctor will apply a vinegar solution to any suspicious-looking areas. It causes abnormal skin to turn white for a short time. Your doctor will be better able to see these areas through the colposcope. Your doctor may look at your cervix and vagina with the colposcope, too.
If your doctor sees abnormalities on different areas of the vulva, he or she may take multiple tissue samples. Small abnormal areas may be removed completely.
If the biopsy detects cancer, your doctor may do additional tests to determine if the cancer has spread beyond the vulva. For example, he or she may use a lighted tube to examine the inside of the bladder and rectum. He or she may also do a more thorough pelvic exam under anesthesia.
You may also need imaging tests. A chest X-ray may be done to see if cancer has spread to your lungs. Another type of imaging test is a computed tomography (CT) scan. It creates detailed pictures of internal organs with a rotating X-ray beam and a computer.
Your doctor may also recommend a sentinel node biopsy to check for cancer in nearby lymph nodes. At some medical centers, this involves injecting a radioactive substance that lymph nodes soak up. If the radioactive substance looks abnormal, it can signal the presence of cancer in the lymph nodes.
These selected nodes (sentinel nodes) may be removed to help doctors predict whether the cancer has spread to nearby pelvic organs or more distant parts of the body. Checking these lymph nodes for cancer will also help doctors determine your treatment options.
If vulvar cancer is diagnosed, it will be "staged." The stages -- stage I to stage IV -- indicate how far the cancer has progressed. Stage 0 indicates VIN. It means that abnormal cells are confined to one spot on the outer surface of the vulva. These abnormal cells have the potential to become cancerous.
Each higher stage means more vulvar cancer progression. Women with stage IV have very advanced cancer that has spread into other organs or to lymph nodes on both sides of the pelvis.
Vulvar cancer will continue to grow until it is treated.
You can take steps to reduce your risk of vulvar cancer. You also can take steps to identify and treat precancerous conditions before they turn into invasive cancer.
HPV infection is found in up to half of vulvar cancers. Almost all HPV infections are transmitted during sexual contact. The most common cancer-producing viruses are HPV types 16, 18, and 33. To lower your risk of HPV:
get the HPV vaccine
use latex condoms (the female condom protects a broader area of the lower genital tract and vulva than the male condom)
limit your number of sex partners
avoid sex with someone who has had many partners
Early detection and treatment of precancerous conditions helps prevent invasive squamous cell vulvar cancer. Precancerous and cancerous conditions can be detected early if you have an annual pelvic exam. Have all vulvar rashes, moles, and lumps checked thoroughly.
The vulva usually is examined when you have a Pap test and pelvic exam. In general, doctors recommend that women start having Pap tests annually when they become sexually active or when they reach age 21 at the latest. After three negative Pap tests at least one year apart, your doctor may do the test every two to three years. This will depend on your age and your risk of cervical cancer.
Removing or odd-looking moles from the vulva may help prevent some vulvar melanomas. Quitting smoking and avoiding the use of tobacco can reduce your risk of many cancers, including vulvar cancer. These steps may also help prevent precancerous changes in the vulva.
The treatment of vulvar cancer depends upon the type of cancer, its stage, and its location. Your age, overall health, and the importance of maintaining sexual function will affect treatment choices, too.
Surgery is the most common treatment for vulvar cancer. The exact type of surgery you have will depend on how much tissue needs to be removed:
Laser surgery burns off the layer of abnormal cells. Doctors treat VIN with laser surgery but not invasive cancer.
Excision (sometimes called wide local excision) removes the cancer and some surrounding normal tissue.
Vulvectomy removes part or all of the vulva and the underlying tissue. A simple vulvectomy removes only the vulva. A partial radical vulvectomy removes part of the vulva and the underlying tissue. A complete vulvectomy removes the entire vulva and the tissue underneath it, including the clitoris. The impact on sexual function depends on how much of the vulva is removed.
Pelvic exenteration is the most extensive surgery. It includes vulvectomy, the removal of the pelvic lymph nodes, and the removal of one or more these structures: the vagina, rectum, lower colon, bladder, uterus, and cervix.
The surgeon will try to remove all of the cancer cells while preserving as much sexual function as possible.
Radiation is usually started after surgery. However, if the cancer affects a large area, radiation may be used before surgery to reduce its size.
Chemotherapy (anticancer drugs) for vulvar cancer is being studied. Researchers are testing a new treatment for women with severe cases of vulvar cancer. This treatment involves receiving chemotherapy intravenously (into a vein) along with radiation therapy prior to surgery.
The greatest challenge is to select a treatment that maximizes the chances of removing all of the cancer while preserving sexual function, which can be lost with aggressive surgery.
Rare forms of vulvar cancer may be linked to cancers elsewhere in the body. That may require more testing, treatment, and monitoring.
It's important to regularly examine your vulva and the area around it. Contact your doctor if you have:
a rash that doesn't go away
itching or pain that doesn't go away
abnormal growths, bumps, or ulcers
any changes in the skin of the vulva
Itchiness, abdominal pain, or fever can signal infection instead of cancer. You should see your doctor right away if you have any abdominal pain with fever.
Patients with vulvar cancer should see a specialist in gynecologic oncology.
If precancerous changes of the vulva and vulvar cancer are detected early, the chances of a cure are excellent. VIN is almost 100% curable. Nearly all patients who have vulvar cancer that hasn't spread to the lymph nodes live at least five years. If vulvar cancer has invaded the lymph nodes, the prognosis depends on the number of lymph nodes involved.
American Cancer Society (ACS)
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National Cancer Institute (NCI)
U.S. National Institutes of Health
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American College of Obstetricians and Gynecologists
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Washington, DC 20090-6920
National Women's Health Information Center (NWHIC)
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Fairfax, VA 22031