Vulvar cancer, the name for cancer that occurs in the vulva, is fairly uncommon, accounting for about 1% of all cancers that are diagnosed in women. About 6,100 women are diagnosed with vulvar cancer each year.

Vulvar cancer is sometimes misdiagnosed as an inflammatory skin condition—such as eczema or psoriasis—or even as a yeast infection. This leads to delayed treatment and, thus, may affect outcomes. It’s most common among menopausal women between the ages of 65 and 75, but it’s possible for younger and older women to have the condition.

As with other cancers, when caught early, vulvar cancer responds well to treatment.

Put simply, it is cancer of the vulva, the external portion of the female genital tract. The vulva includes the mons pubis (pubic mound), the inner and outer labia (lips), the clitoris, various glands, the opening of the urethra (where urine leaves the body), and the opening of the vagina.

Most cases—around 90%—of vulvar cancer are classified as squamous cell carcinoma, a common form of skin cancer; about 10% are melanoma, a serious form of skin cancer.

Sometimes vulvar cancer extends to neighboring body parts, including the vagina, anus, and perineum, the strip of skin between the vagina and anus. (Cancer of the clitoris is classified as vulvar cancer.) It also may spread to the lymph nodes and, in advanced disease, to distant areas, like the lungs or bones.

Vulvar cancer has been on the rise over the past few decades, especially among younger women who have been exposed to human papillomavirus (HPV).

Doctors believe that there are two main causes of vulvar cancer: HPV and vulvar dystrophies, conditions in which the skin cells of the vulva grow abnormally.

• HPV. HPV is the most common sexually transmitted infection. Most people who are sexually active are exposed to the virus at some point. Many HPV subtypes are harmless and don’t cause symptoms or long-term health problems. Other HPV subtypes may lie dormant in the body and, years later, cause genital warts, vulvar cancer, or other cancers of the female (and male) genital tracts, anus, or oral cavity. Vulvar cancer is commonly associated with the HPV subtypes HPV-16 and HPV-18.

Vulvar cancer linked to HPV tends to occur in younger, sexually active women who smoke. Researchers believe that HPV may lead to inflammation in the body, which may encourage cells to proliferate, leading to precancerous cell changes and/or vulvar cancer.

• Vulvar dystrophies. Vulvar cancers that are linked to vulvar dystrophies tend to occur in older women. Two common types of vulvar dystrophy are squamous hyperplasia, which is associated with itchy, reddish skin and raised white areas; and lichen sclerosus, which may cause white patches of thinned skin.

Women who are prone to vulvar dystrophies may have gene mutations that impact tumor formation. Healthy women tend to have properly working tumor suppressor genes, which prevent cells from growing uncontrollably or surviving longer than they should. Doctors believe that vulvar dystrophies may be caused by mutations to the p53 tumor suppressor gene. When this gene doesn’t work properly, vulvar cells may grow out of control or survive longer than their intended lifespans. Both of these abnormalities may lead to cancer.

Some women don’t experience any symptoms of vulvar cancer. A doctor may discover signs of the condition during a pelvic exam.

Other women who have vulvar cancer may notice:

• Vulvar itchiness
• Vulvar pain
• Vulvar bleeding
• Vaginal discharge with an unpleasant odor
• Whitish skin discoloration in the vulvar region
• A sore on the vulva
• A mass on the vulva, which may be raised or resemble warts

People who have, or have had, these health conditions may be at greater risk of vulvar cancer:

• HPV
• Genital warts
• HIV
• Vulvar intraepithelial neoplasia
• Squamous hyperplasia
• Lichen sclerosus
• Cervical cancer
• Paget disease of the vulva
• A compromised immune system
• Organ transplantation
• Physical trauma to the vulva
• Past radiation to the pelvic region to treat other cancers
• Tobacco use

Having multiple sexual partners over the years increases the risk of HPV, which may increase the risk of vulvar cancer. Additionally, smoking may increase the risk of vulvar cancer.

To diagnose vulvar cancer, doctors use the information they obtain about a person’s medical history, what they observe during a physical exam, and what diagnostic test results show.

When obtaining a health history, doctors may ask about a person’s sexual history, whether they smoke, and whether they have had other types of cancer.

During a physical exam, the doctor will do a pelvic exam to look and feel for abnormalities. They will also feel the lymph nodes in the groin area to see if they seem swollen or abnormal.

Doctors may also order tests to check for abnormalities, including:

• Blood tests
• X-rays
• Imaging studies of the pelvis, such as CT scan, MRI, or PET scan

When a doctor spots a lesion or mass that appears to be possible vulvar cancer, a biopsy is performed to confirm the mass is cancerous. Biopsy is the gold standard for diagnosing vulvar cancer and is typically done during an office visit.

• Surgery. When possible, surgery is the preferred treatment for vulvar cancer. During surgery, part of the vulva, a margin of healthy adjacent tissue, neighboring lymph nodes, and lymph nodes in the groin are removed. (If vulvar cancer has spread to lymph nodes in the groin, it may be more difficult to treat the cancer.) Doctors take care to avoid removing tissue too close to the clitoris, urethra, and anus during the procedure to avoid damaging their function.
  
  Some people may receive chemotherapy and/or radiation after surgery to kill any cancer cells that remain. After the lymph nodes are removed, doctors test them for cancer. (They also use sentinel lymph node mapping and dissection to prevent some side effects from surgery, such as leg swelling [called lymphedema]. By using this technique, they remove only selected lymph nodes.)
  
  When some or all of the vulva is surgically removed, a woman may have vulvar reconstructive surgery. This improves the look of the site after surgery and allows a woman to remain sexually active once she heals. Vulvar cancer surgery may cause nerve damage, resulting in numbness in areas that previously had sensation.

• Chemoradiation. If the area with vulvar cancer is too large to remove surgically, doctors may recommend chemotherapy combined with radiation to shrink the tumor or lesion so that it may be surgically removed after treatment.
  
  Other times, chemotherapy plus radiation is the only treatment provided. This may be appropriate when a patient isn’t eligible for surgery because the disease is too advanced or because other health problems make them a poor candidate for surgery.

When a person is treated for vulvar cancer early, the outlook is good—nearly 87% of women whose vulvar cancer has not spread outside the vulva survive for five years or longer. If the cancer spreads to nearby body parts, about half of people with vulvar cancer survive for at least five years. That number drops to about 22% when the cancer spreads to distant areas, including the lungs and bones. For these reasons, early detection and a proper diagnosis are crucial for ensuring a good chance of a positive outcome.

“The gynecologic oncology practice at Smillow Cancer Hospital is specialized in treating patients with vulvar cancer,” says Yale Medicine gynecologic oncologist Vaagn Andikyan, MD. “Every day, we address the needs of many patients with precancerous vulvar lesions and vulvar cancer. We utilize a multidisciplinary approach and perform state-of-the-art surgery with the goal of cure. We work closely with our colleagues in the Radiation Oncology department, Nuclear Medicine, Pathology, and Radiology to improve outcomes for our patients. In addition, our patients have access to clinical trials and new targeted therapies for vulvar cancer.”