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Reviewed by the Faculty of Harvard Medical School

Vulvar Cancer

What Is It?

Vulvar cancer occurs in the vulva, the external genital area of a woman's reproductive system. Vulvar cancer 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. Most commonly, it affects the inner edges of the labia majora or labia minora.

According to the American Cancer Society, more than 90% of vulvar cancers are squamous cell carcinomas. This type of cancer starts in the squamous cells, the main type of cells that make up the skin. Squamous cell cancer usually develops slowly over many years. Before the cancer forms, abnormal cells usually develop in the surface layer of the skin, called the epithelium. This condition is called vulvar intraepithelial neoplasia.

The second most common form of cancer of the vulva is melanoma. The American Cancer Society estimates that melanoma accounts for about 4% of vulvar cancers and usually occurs on the labia minora or the clitoris. More uncommon forms of vulvar cancer include Bartholin gland adenocarcinoma and non-mammary Paget's disease. Less than 2% of vulvar cancers are sarcomas, cancer of the connective tissue underlying the skin. Sarcomas can occur at any age.

According to the American Cancer Society, vulvar cancer is uncommon, accounting for approximately 4% of cancers of the female reproductive system and 0.6% of all cancers in women. Seventy-five percent of women who have vulvar cancer are older than 50, and two-thirds of women are older than 70 when first diagnosed. However, more cases are occurring in younger women. Fifteen percent of new cases now appear in women younger than 40. A recent study found that the average age of women presenting with vulvar cancer has decreased from 69 to 55. In great part, this is thought to result from the increasing incidence and detection of vulvar intraepithelial carcinoma, which when detected and treated, may prevent the development of invasive vulvar cancer.

One recent review lists the following risk factors for vulvar cancer: pre-existing vulvar intraepithelial carcinoma; infection with human papilloma virus (HPV); tobacco use; conditions associated with immune deficiency, such as transplantation; vulvar dystrophy; and cervical intraepithelial neoplasia. Women who have a northern European ancestry may also be more prone to develop the disease.

Symptoms

Common symptoms of vulvar cancer and vulvar intraepithelial neoplasia include:
  • Persistent itching or burning pain anywhere on the vulva
  • A red, pink, or white lump with a wartlike or raw surface
  • A white and rough area on the vulva
  • Painful urination or bleeding
  • A discharge not associated with your period
  • An ulcer that lasts more than a month

Signs of vulvar melanoma can 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. A lump also can be a more common benign cyst, but you should have an evaluation to make sure it is not cancerous. A sore, red, scaly area of the vulva can be a sign of Paget's disease.

Keep in mind that some signs and symptoms of vulvar cancer also can occur with other, noncancerous conditions, such as infection or trauma.

There are some noncancerous conditions that may mimic vulvar cancer. If conservative treatment of these lesions does not result in resolution, you will need a biopsy to establish the nature of the vulvar abnormality.

Diagnosis

The disease usually is diagnosed with a biopsy. In this procedure, a small sample of tissue is removed and examined by a pathologist under a microscope to identify cancer cells or precancerous cells.

An instrument called a colposcope, which has magnifying lenses, may be used to select the biopsy site. Before colposcopy, the skin is treated with a dilute solution of acetic acid that causes suspicious-looking skin to turn white. This white color is temporary and can best be seen through the colposcope. Because of the close association of vulvar cancer with cervical and vaginal cancer, colposcopy is often done on these anatomic areas as well.

If the doctor suspects abnormalities in different areas of the vulva, more than one biopsy may be required. In excisional biopsy, small areas of abnormality may be removed completely. In punch biopsy, a very small cylinder of skin is removed. The biopsy specimen is sent to a laboratory for evaluation and diagnosis.

If a biopsy detects cancer, additional tests also may be done to determine if it has spread beyond the vulva. These may include:

  • Cystoscopy — An exam with a lighted tube to check the inside surface of the bladder
  • Proctoscopy — An exam of the rectum using a lighted tube
  • Pelvic examination — A more thorough pelvic examination under anesthesia
  • Chest X-ray — To check for any spread to the lungs
  • Computed tomography (CT) scan — An imaging method that uses a rotating X-ray beam and a computer to create a detailed view of internal organs
  • Sentinel node biopsy — Recently, combining lymphoscintigraphy and a sentinel lymph node biopsy has gained popularity in centers that specialize in gynecologic cancers. Lymphoscintigraphy uses an injected radioactive substance that is taken up by the lymph nodes. Abnormalities in the appearance of the radioactive substance can identify the presence of cancer in the lymph nodes. Likewise, it is thought that when vulvar cancer spreads, it may preferentially spread to several nodes before more widespread dissemination occurs. Thus, the surgeon may wish to do a biopsy of these selected nodes (sentinel nodes) and make a determination if others are involved. This helps to decide treatment options. While not the standard in all centers, the combination of lymphoscintigraphy and sentinel node biopsy is promising.

These tests can help to predict whether the vulvar cancer has spread to nearby pelvic organs or to more distant parts of the body.

If vulvar cancer is diagnosed, the cancer is staged according to the following convention, as established by the American Joint Commission on Cancer Staging:

  • Stage 0: Carcinoma in situ, intraepithelial carcinoma
  • Stage Ia: Tumor confined to the vulva or perineum, less than or equal to 2 centimeters in greatest dimension, negative nodes, stromal invasion no greater than 1.0 millimeter
  • Stage Ib: Tumor confined to the vulva or perineum, less than or equal to 2 centimeters in greatest dimension, negative nodes, stromal invasion greater than 1.0 millimeter
  • Stage II: Tumor confined to the vulva and/or perineum, greater than 2 centimeters in greatest dimension, negative nodes
  • Stage III: Tumor of any size with adjacent spread to the lower urethra or anus and/or unilateral regional lymph node metastasis
  • Stage IVa: Tumor invades any of the following: upper urethra, bladder or rectal mucosa, pelvic bone, or bilateral regional node metastasis
  • Stage IVb: Any distant metastasis including pelvic lymph nodes
Expected Duration

Vulvar cancer will continue to grow until it is treated.

Prevention

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.

According to the American Cancer Society and the American College of Obstetrics and Gynecology, human papilloma virus (HPV) infection is found in 20% to 50% of invasive vulvar cancers. Certain types of HPV are transmitted during sexual contact. You can lower your risk of HPV by:

  • Using latex condoms (the female condom protects a broader area of the lower genital tract and vulva compared with the male condom)
  • Minimizing your number of sexual partners
  • Avoiding sexual relations with someone who has had many sexual partners

The risk of HPV and vulvar cancer is also lower if you do not have sex before you are 18 years old. Early detection and treatment of precancerous conditions helps to prevent many cases of invasive squamous cell vulvar cancer. Precancerous and cancerous conditions can be detected early if your reproductive system is examined each year and any problems, such as vulvar rashes, moles and lumps, are examined thoroughly.

Your vulva normally is examined at the same time you have a Pap test and pelvic examination. In general, doctors recommend that women start to have annual pelvic exams and regular Pap tests when they become sexually active or by the age of 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, depending on your age and whether you have certain risks of developing cervical cancer.

Removing atypical or "funny-looking" moles found on the vulva will help to prevent some vulvar melanomas. Quitting smoking and avoiding the use of tobacco can reduce the risk of developing many cancerous conditions of the body, including vulvar cancer and precancerous changes in the vulva.

Treatment

The treatment of vulvar cancer depends upon the type of cancer, its stage at diagnosis and its location on the vulva. Treatment also will be influenced by the patient's age and overall health and the importance of maintaining sexual function balanced against the need to remove all the cancer. The main forms of treatment include surgery, radiation and chemotherapy.

Surgery is the most common treatment for vulvar cancer. It can take many forms, depending on how much tissue must be removed. The surgeon will try to remove all of the cancer cells while preserving as much sexual function as possible. The following procedures are listed in order of the least to most aggressive tissue removal.

  • Laser surgery burns off the layer of abnormal cells. It is used for treating vulvar intraepithelial neoplasia but not invasive cancer.
  • Excision (sometimes called wide local excision) removes the cancer and a small portion of surrounding normal cells.
  • Vulvectomy may involve removing part or all of the vulva and its underlying tissue. A simple vulvectomy removes only the vulva. A partial radical vulvectomy involves the removal of a portion of the vulva and the underlying tissue. A complete vulvectomy removes the entire vulva and the tissue underneath it, including the clitoris. The effects on sexual function depend on how much of the vulva is removed.
  • Pelvic exenteration is an extensive surgery that includes vulvectomy, the removal of the pelvic lymph nodes and removal of one or more of the following: vagina, rectum, lower colon, bladder, uterus and cervix.

In general, for patients with Stage Ia, extensive local excision without a lymph node dissection is generally recommended. For patients with Stage Ib, most experts recommend a lymph node dissection in addition to the extensive local excision, because the incidence of lymph node metastases (spread) is higher in this stage. For patients with Stage II, a modified radical vulvectomy is recommended along with a lymph node dissection. For patients with Stage III and IV, a radical vulvectomy with removal of many of the pelvic organs is often done. However, the integration of radiotherapy and chemotherapy may provide an alternative treatment in those women who do not want to or are medically unable to undergo the more extensive surgery.

Radiation can be given before or after surgery. If cancer has spread to the lymph glands, external-beam radiation therapy may be directed at the lymph nodes after surgery. This type of therapy carefully targets a beam of radiation at the cancer. If tumor cells are found at the edges of the tissue that was removed, radiation therapy directed at these areas may be recommended after surgery. If the cancer affects a large area, radiation may be used before the surgery to reduce its size.

The use of chemotherapy (anticancer drugs) for vulvar cancer still is being investigated. Research is being done on a new method of treatment for severe cases, in which the cancer has spread to other tissues, organs and lymph nodes nearby. In this treatment, chemotherapy drugs are given intravenously (into a vein) along with radiation therapy before surgery. The chemotherapy agents that have shown great promise include cisplatin, fluorouracil, bleomycin, methotrexate and mitomycin.

When To Call A Professional

It's extremely important to examine your vulva and the area around it yourself. If you note any persistent rash, persistent itching or pain of the vulva, any changes in the skin of the vulva or abnormal growths, bumps or ulcers, make an appointment with your doctor for an evaluation. Itchiness, abdominal pain or fever can signal infection instead of cancer. You should see your health care professional the same day if you have any abdominal pain with fever. It is strongly recommended that patients with vulvar cancer seek the medical attention of a specialist in gynecologic oncology.

Prognosis

If vulvar cancer is detected early, chances of a cure are excellent. This is especially true for patients with vulvar intraepithelial neoplasia. For cancers in which the lymph nodes are not affected, 90% of people survive 5 years or more. When vulvar cancer has invaded the lymph nodes, then the 5-year survival rate drops to 30% to 55%. The prognosis depends on the number of lymph nodes involved.

Additional Info

American Cancer Society (ACS)
1599 Clifton Road, NE
Atlanta, GA 30329-4251
Toll-Free: (800) 227-2345
http://www.cancer.org/

National Cancer Institute (NCI)
U.S. National Institutes of Health
Public Inquiries Office
Building 31, Room 10A03
31 Center Drive, MSC 8322
Bethesda, MD 20892-2580
Phone: (301) 435-3848
Toll-Free: (800) 422-6237
TTY: (800) 332-8615
E-Mail: cancergovstaff@mail.nih.gov
http://www.nci.nih.gov/

American College of Obstetricians and Gynecologists
P.O. Box 96920
Washington, DC 20090-6920
Phone: (202) 638-5577
http://www.acog.org/

National Women's Health Information Center (NWHIC)
8550 Arlington Blvd.
Suite 300
Fairfax, VA 22031
Toll-Free: (800) 994-9662
TTY: (888) 220-5446
http://www.4woman.org/

Last updated July 16, 2008




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