Vulvar cancer is a malignancy that develops on the external female genitalia, including the labia, clitoris, and the tissue surrounding the vaginal and urethral openings. It accounts for a small fraction of all cancers but has been rising in incidence by about 0.7% per year over the past decade. The median age at diagnosis is 69, though it can occur at any age.
Types of Vulvar Cancer
About 90% of vulvar cancers are squamous cell carcinomas, meaning they start in the flat skin cells covering the vulva. This is the same cell type involved in many skin cancers elsewhere on the body. The remaining 10% includes several rarer types: vulvar melanoma makes up roughly 5%, basal cell carcinoma about 8%, and a condition called extramammary Paget disease accounts for 1 to 2%. Each type behaves differently, but squamous cell carcinoma dominates the conversation around vulvar cancer because it is overwhelmingly the most common.
Symptoms and Warning Signs
Vulvar cancer often starts with changes you can see or feel. The most common signs include:
- A lump, wart-like bump, or open sore on the vulva that doesn’t heal
- Persistent itching that won’t go away with typical treatments
- Bleeding from the genital area that isn’t related to your period
- Pain or tenderness in the vulvar area
- Skin changes such as thickening, darkening, lightening, or a rough texture
Many of these symptoms overlap with common, noncancerous conditions like yeast infections or lichen sclerosus, which is part of why vulvar cancer can be missed early. A sore or itch that persists for weeks despite treatment warrants a closer look.
Risk Factors and Causes
Vulvar squamous cell carcinoma develops through two distinct pathways, and understanding this helps clarify who is at risk.
The first pathway involves HPV infection. High-risk strains of HPV drive about 19% of vulvar squamous cell carcinomas, typically in younger women. These cancers tend to arise from a precancerous stage called usual vulvar intraepithelial neoplasia (VIN). The second, more common pathway is HPV-independent and occurs primarily in older women. It is linked to chronic skin conditions, especially lichen sclerosus, a condition that causes thin, itchy, white patches on the vulva.
A systematic review of over 14,000 women with lichen sclerosus found that 2.2% went on to develop vulvar cancer. The risk was highest in the first one to three years after biopsy and increased with age. Importantly, the review also found that consistent use of strong prescription steroid creams appeared to reduce this risk, reinforcing how much regular monitoring and treatment of lichen sclerosus matters.
Other risk factors include smoking, a weakened immune system (from HIV or immunosuppressive medications), and a history of other HPV-related cancers or precancerous conditions.
How Vulvar Cancer Is Diagnosed
Diagnosis starts with a physical exam. Your provider may perform a vulvar colposcopy, which involves applying a vinegar-like solution to the skin and examining it under magnification and bright light. Abnormal areas absorb the solution differently, making them easier to spot.
If anything looks suspicious, a punch biopsy is the standard next step. The area is numbed with a local anesthetic, and a small cylinder of tissue is removed. Silver nitrate or stitches stop the bleeding. The biopsy site is typically sensitive for 7 to 10 days, and if stitches are placed, they dissolve on their own within two to three weeks. Results usually come back in one to two weeks.
Staging
Vulvar cancer is staged using the FIGO system, updated most recently in 2021. Staging determines how far the cancer has spread and guides treatment decisions.
Stage I means the tumor is confined to the vulva. Within this, Stage IA describes a small tumor (2 cm or less) that has barely invaded the deeper tissue, no more than 1 mm deep. Stage IB covers anything larger or deeper. Stage II means the cancer has extended into the lower third of nearby structures like the urethra, vagina, or anus, but lymph nodes are still clear. Stage III involves spread to upper portions of those structures or to nearby lymph nodes. Stage IV means the cancer has reached bone, caused fixed or ulcerated lymph nodes, or spread to distant parts of the body.
The distinction between IA and IB matters a great deal. Stage IA tumors carry a very low risk of lymph node involvement, which changes the surgical approach significantly.
Treatment Options
Surgery is the primary treatment for most vulvar cancers, and the extent of the operation depends on tumor size, location, and stage.
A partial vulvectomy removes only the portion of the vulva containing the tumor and is used for small cancers or precancerous areas. A radical partial vulvectomy removes part of the vulva along with deeper tissue beneath it, giving wider margins for more invasive tumors. A simple vulvectomy removes the entire vulva, including the labia and clitoris, but not deeper structures or lymph nodes. A radical complete vulvectomy is the most extensive option, removing the entire vulva and potentially portions of the vagina or perineum.
For cancers beyond Stage IA, surgeons typically also evaluate the inguinal lymph nodes in the groin, either by removing a sentinel node or performing a more complete lymph node dissection. Radiation therapy and chemotherapy may be used before surgery to shrink larger tumors, after surgery to reduce the chance of recurrence, or as the main treatment when surgery isn’t feasible.
Survival Rates and Prognosis
Prognosis depends heavily on the stage at diagnosis. Vulvar cancer that is caught while still localized to the vulva carries a significantly better outlook than cancer that has reached the lymph nodes or distant organs. Nearly half of all cases are diagnosed at a localized stage, which is encouraging. However, because early symptoms mimic benign conditions, some cancers aren’t caught until they’ve already spread regionally.
Age also plays a role. About 63% of new cases occur in women over 65, and older patients may have additional health factors that influence treatment options and outcomes. The steady rise in incidence over the past decade, at 0.7% per year, makes awareness of symptoms especially important.
The Role of HPV Vaccination
The HPV vaccine offers strong protection against the subset of vulvar cancers driven by HPV. Clinical trials of the nine-valent HPV vaccine showed 100% efficacy against HPV-related high-grade vulvar precancerous lesions in women who received the vaccine before exposure to the virus. Even the earlier four-valent vaccine demonstrated efficacy between 71% and 100% depending on the study population.
For women who have already been treated for HPV-related vulvar precancer, the picture is less clear. Some studies suggested vaccination after treatment reduced recurrence from reactivated HPV infections by as much as 78%, while others found no significant difference. At least one trial was stopped early because the vaccine showed no benefit in preventing recurrent high-grade lesions. The vaccine’s greatest strength is in prevention before HPV exposure, which is why it’s recommended in adolescence.
Who Gets Vulvar Cancer
Vulvar cancer is predominantly a disease of older women, but it isn’t exclusive to them. About 26% of cases are diagnosed in women aged 65 to 74, and another 23% in women 75 to 84. Still, roughly 17% of cases occur in women under 55. The two pathways to the disease help explain this split: HPV-driven cancers tend to appear at younger ages, while lichen sclerosus-related cancers cluster in older women.
Because vulvar cancer is relatively uncommon, there is no routine screening test for it. Detection relies on awareness of symptoms and willingness to seek evaluation for changes that don’t resolve. Women with known risk factors, particularly lichen sclerosus, benefit from regular follow-up with a provider familiar with vulvar conditions. Treatment of lichen sclerosus with prescribed steroid creams is not just about symptom relief; it appears to actively lower the risk of cancer developing over time.