Vulvar cancer is a cancer of the vulva, the outer portion of the female genitals. It most commonly affects the labia majora. Less often, the labia minora, clitoris, or Bartholin's glands are affected. Symptoms include a lump, itchiness, changes in the skin, or bleeding from the vulva. Risk factors include vulvar intraepithelial neoplasia (VIN), HPV infection, genital warts, smoking, and many sexual partners. Most vulvar cancers are squamous cell cancers. Other types include adenocarcinoma, melanoma, sarcoma, and basal cell carcinoma. Diagnosis is suspected based on physical examination and confirmed by tissue biopsy. Routine screening is not recommended. Prevention may include HPV vaccination. Standard treatments may include surgery, radiation therapy, chemotherapy, and biologic therapy. Vulvar cancer newly affected about 44,200 people and resulted in 15,200 deaths globally in 2018. In the United States, it newly occurred in about 6,070 people with 1,280 deaths a year. Onset is typically after the age of 45. The five-year survival rate for vulvar cancer is around 70% as of 2022. Outcomes, however, are affected by whether spread has occurred to lymph nodes.
Signs and symptoms
The signs and symptoms can include:
Itching, burn, or bleeding on the vulva that does not go away. Changes in the color of the skin of the vulva, so that it looks redder or whiter than is normal. Skin changes in the vulva, including what looks like a rash or warts. Sores, lumps, or ulcers on the vulva that do not go away. Pain in the pelvis, especially during urination or sex. Typically, a lesion presents in the form of a lump or ulcer on the labia majora and may be associated with itching, irritation, local bleeding or discharge, in addition to pain with urination or pain during sexual intercourse. The labia minora, clitoris, perineum and mons pubis are less commonly involved. Due to modesty or embarrassment, people may put off seeing a doctor. Melanomas tend to display the typical asymmetry, uneven borders, and dark discoloration, as do melanomas in other parts of the body. Adenocarcinoma can arise from the Bartholin's gland and present with a painful lump.
Causes Two main pathophysiological pathways are currently understood to contribute to the development of vulvar cancer: human papillomavirus (HPV) infection and chronic inflammation or autoimmunity affecting the vulvar area. HPV DNA can be found in up to 87% of vulvar intraepithelial neoplasia (VIN) and 29% of invasive vulvar cancers; HPV 16 is the most commonly detected subtype in VIN and vulvar cancer, followed by HPV 33 and HPV 18. VIN is a superficial lesion of the skin that has not invaded the basement membrane—or a pre-cancer. VIN may progress to carcinoma in situ and, eventually, squamous cell cancer. Chronic inflammatory conditions of the vulva that may be precursors to vulvar cancer include lichen sclerosus, which can predispose to differentiated VIN.
Risk factors Risk factors for vulvar cancer are largely related to the causal pathways above, involving exposure or infection with the HPV virus and/or acquired or innate autoimmunity.
Increasing age History of vulvar or cervical intraepithelial neoplasia Increased number of male sexual partners Prior history of pre-invasive or invasive cervical cancer History of cigarette smoking Infection with human immunodeficiency virus (HIV) Vulvar lichen sclerosus Immunodeficiency syndromes Northern European ancestry
Diagnosis Examination of the vulva is part of the gynecologic evaluation. It should include a thorough inspection of the perineum, including areas around the clitoris and urethra, and palpation of the Bartholin's glands. The exam may reveal an ulceration, lump, or mass in the vulvar region. Any suspicious lesions need to be sampled, or biopsied. This can generally be done in an office setting under local anesthesia. Small lesions can be removed under local anesthesia. Additional evaluation may include a chest X-ray, an intravenous pyelogram, cystoscopy or proctoscopy, as well as blood counts and metabolic assessment.
Types Depending on the cellular origin, different histologic cancer subtypes may arise in vulvar structures.
Squamous cell carcinoma A recent analysis of the Surveillance, Epidemiology and End Results (SEER) registry of the US National Cancer Institute has shown that squamous cell carcinoma accounts for approximately 75% of all vulvar cancers. These lesions originate from epidermal squamous cells, the most common type of skin cell. Carcinoma in situ is a precursor lesion of squamous cell cancer that does not invade through the basement membrane. There are two types of precursor lesions:
Usual-type vulvar intraepithelial neoplasia (uVIN), which is associated with human papillomavirus (HPV) and often affects younger women. This precursor lesion progresses to basaloid or warty squamous cell carcinoma in approximately 6%. Differentiated vulvar intraepithelial neoplasia (dVIN), which is associated with chronic skin conditions including lichen sclerosus and lichen planus and typically affects older women. This lesion progresses to keratinizing squamous cell carcinoma in approximately 33%. Squamous lesions tend to arise in a single site and occur most commonly in the vestibule. They grow by local extension and spread via the local lymph system. The lymphatics of the labia drain to the upper vulva and mons pubis, then to both superficial and deep inguinal and femoral lymph nodes. The last deep femoral node is called the Cloquet's node. Spread beyond this node reaches the lymph nodes of the pelvis. The tumor may also invade nearby organs such as the vagina, urethra, and rectum and spread via their lymphatics. A verrucous carcinoma of the vulva is a rare subtype of squamous cell cancer and tends to appear as a slowly growing wart. Verrucous vulvar cancers tend to have a good overall prognosis, as these lesions hardly ever spread to regional lymph nodes or metastasize.
Basal cell carcinoma Basal cell carcinoma accounts for approximately 8% of all vulvar cancers. It typically affects women in the 7th and 8th decades of life. These tend to be slow-growing lesions on the labia majora but can occur anywhere on the vulva. Their behavior is similar to basal cell cancers in other locations. They often grow locally and have low risk for deep invasion or metastasis. Treatment involves local excision, but these lesions have a tendency to recur if not completely removed.
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