An Overview of Vulvar Cancer

Vulvar cancer is a rare type of cancer that affects the vulva of women. Not to be confused with the vagina, the vulva is the external female anatomy. It encompasses the labia majora and labia minora (folds of skin that protect the vaginal opening), clitoris, and urethra.

Squamous cell vulvar carcinoma is the most common type of vulvar cancer. It accounts for more than 95% of diagnosed cases. Melanoma of the vulva is the second most common and represents about 5% of women with vulvar cancer. Other types include Paget's Disease of the vulva, vulvar adenocarcinoma, and basal cell carcinoma. However, these are much less common.

This article reviews the causes, risk factors, symptoms, diagnosis, treatment, and prevention of vulvar cancer.

Causes and Risk Factors

Although researchers have yet to pinpoint the exact cause of vulvar cancer, they have identified known risk factors for the disease. Vulvar cancer risk factors include:

• Being infected with the human papillomavirus (HPV) (specifically the subtypes 16 and 18)
• Being infected with human immunodeficiency virus (HIV)
• Having lichen sclerosis, a skin condition that affects the vulva
• Smoking
• Family history of melanoma
• Being 70 or over (vulvar cancer, however, can be diagnosed in younger women)
• Other genital cancers

Symptoms

In the early stages, vulvar cancer rarely has any symptoms. As the disease progresses, vulvar cancer symptoms include:

• Vulvar itching (most common)
• Lump or bump on the vulva
• Vulvar burning, or sensitivity
• Pain during urination
• Change in the texture of the vulva
• Mole on the vulva or change on existing mole
• Bleeding or discharge not related to your menstrual cycle

These vulvar cancer symptoms are not exclusive to vulvar cancer and can be related to another, less serious condition. Reporting symptoms early to your healthcare provider may aid in early detection.

Diagnosis

A biopsy is needed to confirm the presence or absence of cancer. During a vulvar biopsy, the healthcare provider removes a small sample of vulvar tissue to be sent to a pathology lab for screening.

If a biopsy reveals cancer, the disease is then staged. Staging refers to how far cancer has spread to nearby tissues or organs. Other tests used in diagnosing vulvar cancer may include:

• Cystoscopy
• Proctoscopy
• X-ray examination or other imaging tests
• Intravenous urography (as needed)

Treatment

Vulvar cancer treatment plans heavily depend on the stage of cancer and general health. Three methods are used to treat vulvar cancer: surgery, chemotherapy, and radiation therapy.

Surgery

Surgery is the most common type of vulvar cancer treatment. One type of surgery, local excision, involves the removal of cancerous tissue along with a margin of healthy tissue surrounding it. Occasionally, lymph nodes may be removed.

Another surgical procedure, a vulvectomy, could be an option for some women. A vulvectomy removes all or part of the vulva and is reserved for more advanced cases. For women undergoing aggressive vulvectomy surgeries, vaginal reconstruction surgery may be available using plastic surgery techniques and skin grafts.

Chemotherapy

Chemotherapy is sometimes prescribed along with radiation therapy to increase the effectiveness of radiation therapy or to help shrink a large tumor before surgery. It may also be given as adjuvant therapy to prevent a recurrence.

Radiation Therapy

Radiation therapy is also an option for treating vulvar cancer. This type of treatment uses certain types of high-energy beams of radiation to shrink tumors or eliminate cancer cells. Radiation therapy damages a cancer cell's DNA, making it unable to multiply.

Although radiation therapy can damage nearby healthy cells, cancer cells are highly sensitive to radiation and typically die when treated. Healthy cells that are damaged during radiation are resilient and are often able to recover fully.

Prevention

While there are no guaranteed prevention methods for vulvar cancer, there are several things you can do to reduce your risk of developing the disease. Ways to reduce our risk include the following.

Reduce Your HPV and HIV Risk

Limit your exposure to sexually transmitted infections (STIs) such as human papillomavirus (HPV) and human immunodeficiency virus (HIV).

HPV subtypes 16 and 18 are known risk factors for vulvar cancer. HIV also increases the risk for vulvar cancer and other STIs. Practice safe sex and avoid sharing needles or other drug injection equipment.

Avoid Smoking

Since we know that tobacco use is linked to some types of gynecologic cancer, avoiding smoking is a good risk reduction strategy. Quitting smoking may reduce your risk of not only gynecologic cancers like vulvar cancer but many other types of diseases and conditions.

Getting Regular Pelvic Exams

Having a regular pelvic exam is extremely important, even if you are not experiencing any symptoms. A pelvic exam allows your healthcare provider to look for abnormalities that may require further evaluation.

If you are experiencing symptoms, report them to your healthcare provider immediately. Do not wait until your next pelvic exam to do so. A precancerous vulvar condition called vulvar intraepithelial neoplasia (VIN) may be able to be detected and treated before progressing to vulvar cancer, but only if screened by a healthcare provider.

Summary

Vulvar cancer is a rare type of genital cancer in women. It affects the area outside the vagina, including the labia, clitoris, and urethra. 

The human papillomavirus (HPV) subtypes 16 and 18 are often the contributing factor to the most common type of vulvar cancer. Advanced age and a family history of melanoma increase the risk for another type of vulvar cancer. Other risk factors include HIV, lichen sclerosis, smoking, and other genital cancers.

Vulvar cancer may not always show symptoms. When symptoms are present, vulvar itching is the most common. Other symptoms include sores, warts, lumps, painful urination, and more.

A needle biopsy is the most accurate diagnostic tool. Other tests are performed to stage cancer or see how far it may have spread.

Treatment may involve surgery, chemotherapy, or radiation therapy. It could also include a combination of these treatments.

Limiting exposure to STIs, quitting smoking, performing vulvar self-exams, and getting regular pelvic exams greatly reduce your risk.

A Word From Verywell

Getting any type of cancer, including vulvar cancer, can be frightening. While you can't control all risk factors, you can employ various prevention strategies to reduce risk. These include practicing safe sex, not sharing needles or syringes, doing regular vulvar exams, staying current with pelvic exams, and quitting smoking.

Keep in mind that vulvar cancer is very rare. Notify your healthcare provider if you have any signs or symptoms of vulvar cancer as soon as possible. It is highly treatable with early detection and treatment.