Vulvar cancer, usually a skin cancer, develops in the area around the opening of the vagina.
The vulva refers to the area around the opening of the vagina. It contains the external female reproductive organs.
In the United States, cancer of the vulva (vulvar carcinoma) is the fourth most common gynecologic cancer, accounting for 5% of these cancers. Vulvar cancer usually occurs after menopause. The average age at diagnosis is 70 years. As more women live longer, this cancer is likely to become more common. Recent evidence suggests that vulvar cancer is becoming more common among younger women.
The risk of developing vulvar cancer is increased by the following:
Precancerous changes (dysplasia) in vulvar tissues
Lichen sclerosus, which causes persistent itching and scarring of the vulva
Human papillomavirus (HPV) infection
Cancer of the vagina or cervix
Heavy cigarette smoking
Chronic granulomatous disease (a hereditary disease that impairs the immune system)
Most vulvar cancers are skin cancers that develop near or at the opening of the vagina. About 90% of vulvar cancers are squamous cell carcinomas, which develop in the flat cells that form the outermost layer of skin, and 5% are melanomas, which develop in the pigment-producing cells of the skin (melanocytes). The remaining 5% include adenocarcinomas (which develop from gland cells), basal cell carcinomas (which rarely spread), and rare cancers such as Bartholin gland cancer.
Vulvar cancer begins on the surface of the vulva. Most of these cancers grow slowly, remaining on the surface for years. However, some (for example, melanomas) grow quickly. Untreated, vulvar cancer can eventually invade the vagina, the urethra, or the anus and spread into lymph nodes in the area.
Redness or a change in skin color of the vulva may be precancerous (indicating that cancer is likely to eventually develop). Vulvar cancer usually causes unusual lumps or flat, red, or flesh-colored sores that can be seen and felt and that do not heal. Sometimes the flat sores become scaly, discolored, or both. The surrounding tissue may contract and pucker. Melanomas may be bluish black or brown and raised. Some sores look like warts. Typically, vulvar cancer causes little discomfort, but itching is common. Eventually, the lump or sore may bleed or produce a watery discharge (weep). These symptoms should be evaluated promptly by a doctor.
Doctors diagnose vulvar cancer by taking a sample of the abnormal skin and examining it (biopsy). The biopsy enables doctors to determine whether the abnormal skin is cancerous or just infected or irritated. The type of cancer, if present, can also be identified, helping doctors develop a treatment plan. If the skin abnormalities are not well-defined, doctors apply stains to the abnormal area to help determine where to take a sample of tissue for a biopsy. Alternatively, they may use an instrument with a binocular magnifying lens (colposcope) to examine the surface of the vulva.
Doctors stage vulvar cancer based on how large it is, where it is, and whether it has spread to nearby lymph nodes, which is determined during surgery to remove the cancer. Stages range from I (the earliest) to IV (advanced).
If vulvar cancer is detected and treated early, about 3 of 4 women have no sign of cancer 5 years after diagnosis. The percentage of women who are alive 5 years after diagnosis and treatment depends on whether and how far the cancer has spread:
Melanomas are more likely to spread than squamous cell carcinomas.
Depending on the extent and type of the cancer, all or part of the vulva is surgically removed (a procedure called vulvectomy). Nearby lymph nodes are usually also removed. But sometimes doctors can instead do a sentinel lymph node dissection (removal of the first lymph node that would be affected by the cancer—see Figure: What Is a Sentinel Lymph Node?). This lymph node is checked for cancer. If it is cancer-free, no other lymph nodes need to be removed. For early-stage cancers, such treatment is usually all that is needed.
For more advanced cancers, radiation therapy, often with chemotherapy (with cisplatin or fluorouracil), may be used before vulvectomy. Such treatment can shrink very large cancers, making them easier to remove. Sometimes the clitoris and other organs in the pelvis must be removed.
For very advanced cancers, treatment may include surgery to remove all pelvic organs (pelvic exenteration—see Later stages (late stage III through early stage IV)), radiation therapy, and/or chemotherapy.
After the cancer is removed, surgery to reconstruct the vulva and other affected areas (such as the vagina) may be done. Such surgery can improve function and appearance.
Doctors work closely with the woman to develop a treatment plan that is best suited to her and takes into account her age, sexual lifestyle, and any other medical problems. Sexual intercourse is usually possible after vulvectomy.
Because basal cell carcinoma of the vulva does not tend to spread (metastasize) to distant sites, surgery usually involves removing only the cancer. The whole vulva is removed only if the cancer is extensive.