Melanoma is an aggressive (fast-growing) form of cancer that develops in melanocytes, the cells responsible for skin color. The condition commonly develops in parts of the body exposed to ultraviolet (UV) light, such as sunlight. However, it can also develop in less expected places, including the skin of the vulva (external female genitalia).

Research shows that vulvar melanoma differs from more common types of skin melanoma — and also from mucosal melanoma, which affects moist mucus membranes in areas like the mouth and nose. Although there is still a lot to learn about vulvar melanoma, here are key insights into its appearance and diagnosis.

How Common Is Melanoma of the Vulva?

Up to 10 percent of women have some pigmented lesions (areas of skin with a different color) on the vulva, according to a 2021 research article in the American Journal of Clinical Dermatology. The vast majority of these lesions aren’t cancerous. Among cancers that develop on the vulva, only about 5 percent are melanomas. Over 75 percent of vulvar cancers are squamous cell carcinomas, a separate type of skin cancer.

Overall, melanoma is the sixth most common form of cancer in women in the U.S., affecting 2.4 percent of that population, according to the research. Vulvar melanomas account for up to 1 percent of the total types of melanomas that may develop in women, per the 2021 article.

Risk Factors

Women who have gone through menopause (the end of monthly periods) are more likely to get vulvar melanoma than those who have not, according to Cancer Research UK. The median age for diagnosis is 68. In comparison, the average age of diagnosis for other types of melanoma is 65.

According to Cancer Research UK, vulvar melanoma is more common in white women than in Black women. However, the racial and ethnic differences in vulvar melanoma rates is less drastic than for other melanoma types. While scientists don’t fully understand what increases the risk for vulvar melanoma in some people, having a family history of melanoma or other cancers like breast cancer may increase the chance of developing this cancer. This suggests that genetic factors (inherited traits) might play a role.

What Are the Symptoms of Vulvar Cancer?

Some symptoms of vulvar melanoma overlap with other types of vulvar cancer. These include:

• Open sores
• Bleeding
• Discharge
• Itching
• Lumps
• Pain

Vulvar melanoma usually grows near the clitoris or labia. The spots are typically black or dark brown, but other colors, like white, pink, or red, are also possible. Sometimes, a preexisting mole can turn cancerous. Watching for changes in the color, shape, or size of moles can help identify spots that warrant a closer look.

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People with vulvar melanoma don’t usually recognize the condition right away because symptoms can vary or be mistaken for other issues. For example:

• Bartholin gland carcinomas or cysts can create a lump on the outside of the vagina.
• Paget disease of the vulva can cause soreness and scaly patches.
• Human papillomavirus (HPV), genital warts, and yeast infections can also cause symptoms around the vulva that are unrelated to skin cancer.

How Is Vulvar Melanoma Diagnosed?

Diagnosing vulvar melanoma usually involves a few tests, including a physical exam, blood tests, and imaging scans to get a detailed view of the internal organs and pelvic area. To confirm a diagnosis, a biopsy (removing a tissue sample for lab testing) is required.

During a biopsy, a surgeon removes part or all of the suspicious area on the vulva. This tissue sample is then sent to a lab, where specialists examine it on a molecular level to check for cancer cells. Ideally, the surgeon will remove the full tumor for testing. However, if this isn’t possible because of the tumor’s location or other concerns, they may take a smaller sample instead.

What Are the Treatment Options for Vulvar Melanoma?

Treating vulvar melanoma comes with a few challenges. Unfortunately, it’s often diagnosed in later stages, after it may have already spread to other parts of the body. Because vulvar melanoma is rare, there aren’t specific treatment guidelines, so doctors usually follow the same approach as they do for skin melanomas.

Surgery is generally the first treatment option for early-stage vulvar melanoma. Usually, surgeons remove the affected area and some surrounding healthy tissue. This procedure is called a wide local excision. They’ll also perform a sentinel lymph node biopsy, removing one or more nearby lymph nodes (small glands that filter harmful substances and help the body fight infection). This type of biopsy can help determine the cancer stage and show if the cancer has spread beyond the vulva.

Other cancer treatments include:

• Chemotherapy
• Radiation therapy
• Immunotherapy
• Targeted cancer drugs

The oncology team must consider each case carefully to decide which treatments will most likely improve survival while balancing the risk of side effects.

What Is the Prognosis for Vulvar Melanoma?

The five-year survival rate for cutaneous melanoma — the most common type of melanoma — is 92 percent. This means that 92 out of 100 people diagnosed with the condition are still alive five years later. This rate has improved significantly thanks to better screening and treatment options. However, for vulvar melanoma, the five-year survival rate is much lower, at about 47 percent. Unfortunately, this rate hasn’t improved much in recent years.

As researchers learn more about how the immune system and genetics affect specific types of cancer, they may develop better screening tools to detect cancer in the early stages and treat it in more targeted ways. Clinical trials are one way to contribute to melanoma research while also potentially gaining access to promising new treatments before they become widely available. Ask your oncologist or health care professional if joining a clinical trial might be a good option for your situation.

Following Up After Treatment

After treatment, regular checkups are essential, as vulvar melanoma can return within the five years. Doctors typically recommend a physical exam every three to four months during the first two years after diagnosis. For those who remain in remission (cancer-free) after two years, follow-up exams may be reduced to twice a year, as risk of recurrence (cancer returning) often decreases over time.

Being diagnosed with a rare cancer like vulvar melanoma can feel overwhelming. It may help to connect with support groups or people who have had similar experiences, even with other types of cancer. Sharing experiences and support can provide strength and hope when you need it most.

Talk With Others Who Understand

MyMelanomaTeam is the social network for people with melanoma and their loved ones. Members come together to ask questions, give advice, and share their stories with others who understand life with melanoma.

Have you or a loved one been diagnosed with vulvar melanoma? What symptoms did you notice? Share your experience in the comments below, or start a conversation by posting on your Activities page.