Vulvar Cancer

Vulvar cancer at a glance

• Vulvar cancer is a rare disease where cancer, which results from the uncontrolled growth of cells, originates in the external part of a woman’s vagina.
• Symptoms of vulvar cancer include itching and burning.
• The only definitive test for vulvar cancer is a biopsy.
• Surgery to remove all or part of the vulva is the most common cancer treatment option, but it can also be combined with radiation or chemotherapy.
• Women who have had part or all of their genitals removed will experience altered sensations and may have difficulty reaching orgasm.
• Reconstructive surgery is an option for these women.

What is vulvar cancer?

Vulvar cancer forms in the outer part of the female genitals. The vulva includes the opening of the vagina, skin folds around the vagina (the labia majora and the labia minora) and the clitoris. Vulvar cancer most often occurs on the inner edges of the labia.

Vulvar cancer usually forms slowly over many years. Abnormal cells can grow on the surface of the vulvar skin for a long time. This condition is called vulvar intraepithelial neoplasia (VIN). If left untreated, VIN can develop into cancer.

Vulvar cancers are very rare, and while all women are at risk for these cancers, very few will get them. According to the Centers for Disease Control and Prevention, vaginal and vulvar cancers combined account for 6-7 percent of all gynecologic cancers diagnosed in the United States.

There is no reliable way to test for vulvar cancers in women before they experience signs or symptoms. A woman’s best defense is knowing her own body and paying attention to potential signs, listed below, then visiting her doctor for a biopsy.

When found early, vulvar cancers are highly treatable.

Types of vulvar cancer

There are a number of types of vulvar cancer, although squamous cell carcinoma is by far the most common. A gynecologic oncologist can determine the type by performing a biopsy.

Squamous cell carcinoma: This wart-like growth is the most common type of vulvar cancer. It may take several years before growth develops into cancer.

Melanoma: This skin cancer is the second most common type of vulvar cancer, occurring in about 4 out of every 100 vulvar cancers. It is most often found in women older than 50, and Caucasian women are at higher risk of developing vulvar melanoma.

Sarcomas: These fast-growing but rare cancers start in tissue such as muscle or fat under the skin. These cancers tend to grow quite quickly and are found in both children and adults.

Adenocarcinoma: A small number of vulvar cancers called adenocarcinomas (about 8 percent) develop from sweat glands in the vulvar skin. Paget’s disease of the vulva is a condition where adenocarcinoma cells spread out from these glands and across the skin of the vulva.

Basal cell carcinoma: A small number of vulvar cancers are basal cell carcinomas, which develop from basal cells— the deepest layer of skin cells.

Causes of vulvar cancer

As is the case with other cancers, there are few definitive causes for vulvar cancer, though several genetic and lifestyle factors can increase the risk of developing vulvar cancer. These risk factors include:

• Having human papillomavirus (HPV) or vulvar intraepithelial neoplasia (VIN)
• Having pre-cancerous cervical cells or being diagnosed with cervical cancer
• Having a condition that weakens your immune system (such as HIV, the virus that can lead to AIDS)
• Family history or genetic predisposition to vulvar or vaginal cancer
• Smoking
• Chronic vulvar itching or burning.

Vulvar cancer diagnosis and testing

The only way to diagnose vulvar cancer is to perform a biopsy. Even when a woman is showing signs or symptoms, it doesn’t necessarily mean she has vulvar cancer, as many symptoms overlap with other conditions.

During a biopsy, a physician will take a small piece of tissue from the vaginal area to be examined under the microscope. If the cancerous area is small, it may be completely removed (called an excisional biopsy) with a scalpel. Sometimes stitches are needed.

A biopsy determines if cancer or a pre-cancerous condition is present and, if so, what type it is.

Rarely, a doctor may use a dye test (painting the vulva with toluidine blue) to find areas of abnormal vulvar skin and to select the areas to biopsy. This dye causes skin with certain diseases, such as vulvar cancer, to turn blue.

Depending upon the cancerous results, further testing may be necessary. These tests can include:

• Cystoscopy, a procedure using a cystoscope (a thin, lighted tube with a camera) inserted through the urethra to see if the cancer has spread to the bladder.
• Colposcopy, in which a colposcope (a lighted, magnifying instrument) is used to check the vagina and cervix for abnormal areas. Tissue samples may also be taken at this time.
• Proctoscopy, a visual inspection of the rectum using a lighted tube to see if the cancer has spread to the rectum or anus.
• Pelvic exam to evaluate the stage of the cancer and if it has spread to internal organs.
• Intravenous pyelogram (IVP), a series of x-rays using a dye to visualize the kidneys, ureters and bladder to find out if there are any blockages.

Vulvar cancer treatment

Surgery is the most common form of vulvar cancer treatment, although radiation and chemotherapy are often used in conjunction with surgery in advanced stages. The type of surgery used depends on balancing sexual function importance with the need to remove all the cancer. The goal of surgery is to remove all the cancer without any loss of the woman’s sexual function or sensation.

Common surgical procedures to treat vulvar cancer include:

• Laser surgery: Acting as a scalpel, a high-intensity laser beam destroys and removes tumors found on the surface or within the tissue of the vulva.
• Wide local excision: Surgery to remove the cancer and a margin of normal tissue around the cancer as a precaution.
• Radical local excision: Surgery that removes cancer cells, a large amount of surrounding, normal tissue and, in some cases, nearby lymph nodes in the groin.
• Ultrasound surgical aspiration (USA): A concentrated surgery that results in less damage to surrounding healthy cells, a USA uses vibrations to dissolve the tumor into tiny pieces. The area is then washed and the tumor particles removed.
• Vulvectomy: Surgery to remove part or all of the vulva. Variations of a vulvectomy include:
  • Skinning vulvectomy removes the top layer of vulvar skin where the cancer is found. Skin grafts from other parts of the body may be needed to cover the area where the skin was removed.
  • Radical (or modified) vulvectomy removes part of the vulva and nearby lymph nodes may also be removed.
  • Complete radical vulvectomy removes the entire vulva and deep tissues, including the clitoris. This procedure is rarely prescribed.
• Pelvic exenteration: Extensive surgical procedure to remove the vulva and nearby lymph nodes, as well as one or more of the following: lower colon, rectum, uterus, vagina, cervix and bladder. Artificial openings (stoma) are made for urine and stool to flow from the body into a collection bag.

Surgery alone may not be enough in treating some forms of vulvar cancer. Some patients may undergo chemotherapy or radiation following the initial surgery in order to remove any remaining cancer cells. These treatments, known as adjuvant therapy, reduce the chances that the cancer will return.

Recovery time varies according to the type of surgery and how extensive it was. For extensive operations, such as a pelvic exenteration, it may be many weeks or months before a patient fully recovers.

Risks, side effects and complications of vulvar surgery

As with any surgery, possible risks include infection, bleeding, scarring and blood clots.

Women who have had a vulvectomy or pelvic exenteration may also experience altered sensation in their vulva and difficulties reaching orgasm. Reconstructive surgery to rebuild the inner or outer lips of the genitals may also be an option for those women who have had more extensive operations.

Some of the decreased sensitivity is caused by nerve damage during surgery which may heal over time, although very slowly.

A gynecologic oncologist (a special cancer doctor trained to diagnosis and perform surgery on reproductive cancers) will walk patients through possible risks associated with different procedure options. They will also determine the best treatment path for individual patients based on their health, cancer stage and reproductive goals.