Vulvar Cancer

What vulvar cancer is

The vulva includes the labia majora and minora, clitoris, vaginal opening and the skin between the vagina and anus (perineum). Vulvar cancer accounts for a small share of gynecologic cancers, with several thousand cases a year in the US.

Types

Causes and precancers

Most vulvar squamous cancers develop through one of two pathways:

• HPV-related: persistent infection with high-risk HPV types, especially HPV 16, causes high-grade squamous intraepithelial lesions (HSIL, formerly usual VIN). More common in younger women and smokers
• HPV-independent: linked to lichen sclerosus, a chronic inflammatory skin condition, through a precancer called differentiated VIN. More common in older women

Other risk factors:

• smoking
• weakened immune system, including HIV and organ transplant medicines
• previous cervical or vaginal precancer or cancer
• older age

Symptoms

• itching that does not go away (vaginal itching is often blamed on yeast), often the earliest symptom
• a lump, bump or wart-like growth, sometimes mistaken for genital warts
• an open sore or ulcer that does not heal
• skin changes: white, red, pink, brown or darker patches, or thickened skin
• pain, burning or tenderness
• bleeding or discharge not related to periods
• a lump in the groin from spread to lymph nodes

Many women delay seeking help because of embarrassment or because itching is assumed to be a yeast infection. Itching that persists despite treatment deserves examination.

Diagnosis

• examination of the vulva, vagina, cervix and groin lymph nodes
• vulvoscopy: magnified examination, sometimes with dilute acetic acid
• biopsy: a small skin sample under local anesthetic is the only way to confirm cancer
• imaging: CT, MRI or PET to check for spread
• sentinel lymph node biopsy at surgery to check groin nodes in many cases

Stages

Vulvar cancer is staged with the FIGO system:

• stage I: confined to the vulva or perineum, without node spread
• stage II: spread to the lower urethra, lower vagina or anus
• stage III: spread to nearby lymph nodes or upper parts of nearby organs
• stage IV: spread to the bladder or rectum lining, pelvic bone, fixed nodes or distant organs

Treatment

Precancer (VIN or HSIL)

• surgical excision
• laser treatment
• imiquimod cream for HPV-related lesions
• regular follow-up

Cancer

• surgery: wide local excision or partial vulvectomy for most early cancers, removing the tumor with a margin of healthy tissue; radical vulvectomy is now less often needed
• lymph node surgery: sentinel node biopsy or groin node dissection
• radiation therapy: after surgery if margins or nodes are involved, or as main treatment with chemotherapy for advanced cancers
• chemotherapy: usually with radiation (chemoradiation); also for spread disease
• immunotherapy for some advanced or recurrent cancers
• reconstructive surgery when larger areas are removed

Life after treatment

• lymphedema of the legs after node removal
• changes in sexual function and body image; pelvic floor therapy, counseling and lubricants help
• regular follow-up exams, because recurrence or new lesions can occur

Outlook

Outcome depends on stage. According to the American Cancer Society, 5-year relative survival is about 86 percent when the cancer is confined to the vulva, around 50 percent with regional spread, and about 20 percent with distant spread. Early diagnosis greatly improves results.

Prevention

• HPV vaccination, recommended routinely at ages 11 to 12, with catch-up through age 26 and for some adults up to 45
• not smoking
• treatment and regular follow-up of lichen sclerosus, usually with strong steroid ointments, which lowers cancer risk
• examining the vulva with a mirror and reporting changes
• routine gynecologic care, including cervical screening

Frequently asked questions

What are the early signs of vulvar cancer?

The most common early sign is persistent itching. Others include a lump, a wart-like growth, an open sore that will not heal, a white, red or dark patch, pain or burning, and bleeding not related to periods.

Is vulvar cancer caused by HPV?

Many vulvar cancers, especially in younger women, are caused by high-risk HPV. Others, mainly in older women, develop from lichen sclerosus without HPV. HPV vaccination prevents most HPV-related vulvar precancers and cancers.

Can lichen sclerosus turn into cancer?

Lichen sclerosus carries a small increased risk of vulvar cancer, estimated at a few percent over a lifetime. Long-term treatment with steroid ointment and regular checks reduce this risk. Report any new lump, sore or thickened area.

Is vulvar cancer curable?

Yes, especially when found early. Most early vulvar cancers are treated successfully with surgery, and 5-year survival is high when cancer has not spread. Advanced cancers are treated with surgery, radiation and chemotherapy.