Vulvar cancer is uncommon, accounting for roughly 5% to 8% of all gynecological cancers in the United States. About 6,470 new cases are diagnosed each year, with approximately 1,670 deaths attributed to the disease annually. By the National Cancer Institute’s working definition, any cancer with fewer than 40,000 new cases per year qualifies as rare, so vulvar cancer falls well within that threshold. Still, “rare” doesn’t mean insignificant. The 5-year survival rate sits at 71% overall, and outcomes depend heavily on how early it’s caught.
How Common Is Vulvar Cancer by the Numbers?
To put 6,470 annual cases in perspective, compare it to breast cancer (roughly 300,000 new cases per year) or even cervical cancer (about 13,000). Vulvar cancer is a small fraction of female cancers overall. The incidence rate averages about 2.25 per 100,000 women.
That said, the numbers have been creeping upward. Between 1992 and 2014, vulvar cancer rates rose by 14.3%. During that same period, cervical cancer death rates dropped by more than a third, while vulvar cancer death rates stayed essentially flat. Part of the explanation is demographic: the proportion of cases in women over 65 grew from about 37% in the early 1990s to nearly 50% by 2012 to 2015, while cases in women under 50 dropped sharply. The median age at diagnosis is 67.
What Causes It
There are two main pathways to vulvar cancer, and they tend to affect different age groups. The first involves infection with high-risk strains of human papillomavirus (HPV). A large meta-analysis covering more than 7,700 cases found HPV present in about 34% of vulvar cancers overall. These HPV-related tumors tend to appear in younger women and are often preceded by precancerous changes that can be detected on examination.
The second pathway is unrelated to HPV and more common in older women. It’s often associated with chronic inflammatory skin conditions, particularly lichen sclerosus, a condition that causes the vulvar skin to become thin, white, and intensely itchy. About 4% of women with lichen sclerosus eventually develop vulvar cancer. Other risk factors include smoking, a weakened immune system, and a history of cervical or vaginal cancer.
Types of Vulvar Cancer
Squamous cell carcinoma dominates, making up roughly 95% of all vulvar cancers. It develops from the flat surface cells of the vulvar skin and comes in several subtypes: warty, basaloid, and keratinizing. The warty and basaloid forms are more closely linked to HPV, while the keratinizing type is more common in older women without HPV.
Melanoma is the second most common type, though it’s far less frequent. Other rare subtypes include basal cell carcinoma, cancers arising from Bartholin’s glands, and Paget’s disease of the vulva.
Symptoms to Recognize
Vulvar cancer does produce noticeable signs in most cases, but the symptoms overlap with common, benign conditions, which can delay diagnosis. The most frequently reported symptoms include persistent itching, burning, or bleeding on the vulva that doesn’t resolve on its own. You might also notice color changes in the skin, areas that appear redder or whiter than usual, or what looks like a rash or warts.
Sores, lumps, or ulcers that don’t heal are another key warning sign. Pelvic pain, particularly during urination or sex, can develop as the disease progresses. Because itching and skin changes are so common in benign conditions like yeast infections or lichen sclerosus, any persistent vulvar symptom lasting more than a few weeks warrants a closer look. A biopsy is the only way to confirm or rule out cancer.
Survival Rates by Stage
Stage at diagnosis makes an enormous difference in outcomes. Data from the SEER database (based on women diagnosed between 2008 and 2014) breaks it down clearly:
- Localized (cancer confined to the vulva): 86% five-year survival
- Regional (spread to nearby lymph nodes or tissues): 53% five-year survival
- Distant (spread to other parts of the body): 19% five-year survival
The gap between localized and regional disease is striking. Once cancer reaches the lymph nodes, survival drops by more than 30 percentage points. This is why early detection matters so much, even for a cancer that’s statistically rare.
How It’s Treated
Surgery is the primary treatment for most vulvar cancers, especially in earlier stages. The extent of the operation depends on the size and location of the tumor. For smaller cancers, a wide local excision (removing the tumor with a margin of healthy tissue) may be sufficient. Larger or more advanced tumors may require removal of a bigger portion of the vulva.
One major advance in surgical treatment has been sentinel lymph node biopsy, where surgeons identify and remove just the first lymph node or nodes that drain the tumor site, rather than removing all the groin lymph nodes. This approach significantly reduces the risk of chronic leg swelling, infection, and blood loss that can follow more extensive lymph node surgery, without compromising cancer outcomes in appropriate candidates.
Radiation and chemotherapy may be used alongside surgery for more advanced disease, or as the primary treatment when surgery isn’t feasible. Recovery timelines vary considerably depending on the extent of the procedure, but wound healing in the vulvar area can take several weeks, and some women experience long-term changes in sensation or appearance that affect quality of life.
Why a Rare Cancer Still Matters
The rarity of vulvar cancer creates a paradox. Because it’s uncommon, many women and even some healthcare providers don’t think of it first when symptoms appear. Persistent itching gets treated as a yeast infection. A skin change gets attributed to irritation. Meanwhile, vulvar cancer death rates have remained stubbornly unchanged over two decades, even as cervical cancer mortality has fallen sharply, partly because of effective screening programs that don’t exist for vulvar cancer.
HPV vaccination offers one layer of protection, since it targets the viral strains responsible for roughly a third of vulvar cancers. For the HPV-unrelated cases, awareness of risk factors like lichen sclerosus and attention to persistent symptoms remain the best tools for catching the disease early, when the odds of survival are strongest.