About Vulvar Cancer

Vulvar cancer is a rare type of cancer that forms on the outer part of a woman's genitals, known as the vulva. The vulva includes labia (the inner and outer lips around the opening of the vagina), the clitoris (small, sensitive, nerve-dense bud of tissue, located between the labia and in front of the urethra and vagina) and the opening of the vagina and its glands. Vulvar cancer most often affects the outer labia.

The cancer usually develops slowly over several years, with abnormal cells growing on the surface of the vulvar skin - a precancerous condition called vulvar intraepithelial neoplasia (VIN). It is possible for VIN to turn into cancer, therefore early treatment is critical.

The majority of vulvar cancers are squamous cell carcinomas, or cancer that begins in squamous cells, the main type of skin cells found on the surface of the skin.

Other rare types of vulvar cancer include:

• Adenocarcinoma - cancer that begins in gland cells
• Melanoma - cancer that develops from the pigment-producing cells that give skin color
• Sarcoma - cancer that begins in the cells of connective tissue
• Basal cell carcinoma - cancer that begins from a deeper layer of the skin, below the squamous layer

While the cause of vulvar cancer is not yet known, factors that may increase a woman's risk include:

• Human papillomavirus (HPV) infection
• Age 50 or older
• Smoking
• HIV infection
• History of Vulvar intraepithelial neoplasia (VIN)
• Lichen sclerosus
• Chronic vulvar inflammation
• Genital warts (may be associated with risk of exposure to high risk HPV) 

Often there are no symptoms in the early stages of the disease. If symptoms do occur, they may include:

• A lump in the vulva
• Vulvar itching that doesn't go away
• Vulvar pain or tenderness
• Bleeding that is not your period
• Skin changes, such as color changes or thickening
• A lump, wart-like bumps or an open sore (ulcer)

Evaluation may include:

• A thorough medical history and physical exam
• Pelvic exam, including vulvar and groin exam to check for lumps or anything else unusual
• Biopsy - removal of a small piece of tissue for examination under a microscope, to look for cancerous or precancerous cells

If the diagnosis is vulvar cancer, more tests may be run to determine the extent or "stage" of the disease - how far the cancer cells have spread and the best treatment strategy.

These tests may include:

• Imaging tests such as x-rays, computed tomography (CT) scans, MRIs (magnetic resonance imaging), and PET scans (positron emission tomography)
• Additional tests that are occasionally performed are: 
  • Examination of the pelvis under anesthesia
  • Cystoscopy - to determine if the cancer has spread to the bladder
  • Proctoscopy - to determine if the cancer has spread to the rectum

Treatment depends on the individual patient and their cancer, but may include one or more of the following:

• Surgery - to remove or destroy the cancerous tissue and to assess lymph nodes in the groin 
• Radiation - Radiation treatment uses high-energy x-rays to kill cancer cells
• Chemotherapy - uses drugs, typically given intravenously (through a vein), to stop the growth of cancer cells
• Immunotherapy. Some patients with vulvar cancers are candidates for immunotherapy. Immunotherapy is also given through an infusion into a vein but works by stimulating the immune system against cancer.