Vulvar cancer occurs in the vulva, which is the exterior of the female genitals. The vulva is composed of fatty tissue and skin that surround the openings of the urethra and vagina as well as the clitoris. Vulvar cancer is most common on or in the labia (the folds of skin around the vaginal opening). It can sometimes occur in the glands on the sides of the vaginal opening (the Bartholin’s glands) or on the clitoris.

Half of all vulvar cancers are caused by infection with certain types of human papillomavirus (HPV). Vulvar cancer usually grows slowly. It can sometimes develop from vulvar intraepithelial neoplasia (VIN), which is the growth of abnormal cells on the vulvar skin.

The most common form of vulvar cancer is squamous cell carcinoma, a type of skin cancer. It is responsible for 90% of vulvar cancer cases. It can result from VIN.

Less common forms of vulvar cancer include:

  • Verrucous carcinoma: A slow-growing, wart-like subtype of squamous cell carcinoma.
  • Adenocarcinoma: This cancer starts in the vulvar sweat glands or the Bartholin’s glands.
  • Melanoma: This type of skin cancer causes 2 to 4% of vulvar cancers. It occurs most often on the labia minora or the clitoris.
  • Sarcoma: This is a tumor that develops in the connective tissue under the skin.
There are no screening tests for vulvar cancer, though regular pelvic exams can help your doctor find vulvar cancer in earlier, more treatable stages.

The following factors may increase your risk of vulvar cancer:

  • Being age 50 or older
  • Smoking
  • HPV infection
  • An immunodeficiency, such as HIV
  • VIN
  • Cervical precancer or cervical cancer
  • Vaginal cancer
  • Melanoma elsewhere on the body
  • Lichen sclerosus, a condition that makes the vulvar skin itchy and thin

Getting the HPV vaccine early may reduce your risk of vulvar cancer. Research has also shown the following factors may reduce your risk of vulvar cancer:

  • Getting regular Pap tests
  • Not smoking (or quitting if you currently smoke)
  • Not having sexual intercourse until your late teens or older
  • Limiting your number of sexual partners
  • Avoiding sexual intercourse with someone who has had many partners
  • Using condoms during sexual intercourse

Signs of vulvar cancer include:

  • A lump or growth in or on the vulva or groin
  • Color changes on the vulva
  • An ulcer or open wound for a month or more
  • Itching, bleeding or burning on the vulva
  • Wart-like growths that look like genital warts
  • Pain during sexual intercourse or urination
  • A changing appearance of an existing mole
Tell your doctor immediately if you have abnormal vaginal bleeding. Otherwise, seek medical care if you experience any of the other symptoms for two weeks or more. They may be caused by less serious conditions, but it is best to see your doctor if you notice any changes in your body.

Your gynecologic oncologist may use one or more of the following tests to diagnose vulvar cancer:

  • Pelvic exam: During this exam, your doctor will check your vulva, vagina, cervix, uterus, ovaries, bladder and rectum for changes.
  • Colposcopy: This test is used to look for abnormal or cancerous cells in or on the vulva, vagina and cervix. During this test, your doctor will use an instrument called a colposcope to get a better view of the tissues in your vagina and surrounding areas. The colposcope never goes inside your body; rather, it just gives your doctor a better view of potentially abnormal cells.
  • Chest X-ray: A chest X-ray may be used to see if the cancer has spread to the lungs.
  • Biopsy: If your doctor suspects that you have abnormal or cancerous cells, they may take a small tissue sample through a biopsy. Only a biopsy can tell your doctor for sure if you have vulvar cancer.
  • Endoscopy: This procedure allows your physician to look inside your body with a thin, flexible tube with a light at its end. During the procedure, you’re sedated and the tube is inserted through the vagina, urethra, anus, mouth or small surgical opening.
  • Computed tomography (CT) scan: A CT scan takes X-rays of the body from different angles to create a three-dimensional image that shows tumors or abnormalities.
  • Positron emission tomography (PET) scan: During a PET scan, a small amount of a radioactive sugar substance is injected into the body. Cells that use the most energy, such as cancer cells, absorb more of this substance than other cells. Then a scanner creates images of your organs and tissues.
  • Magnetic resonance imaging (MRI): This test uses magnetic fields instead of X-rays to create images of the inside of the body. An MRI can also help your doctor measure the size of the tumor.

Staging is based on where your cancer started, if or where it has spread, and if or how it’s affecting other areas of the body. Here are the stages of vulvar cancer:

  • Stage I: The cancer is only found in the vulva or on the vulva and perineum (the area between the vulva and the anus).
    • Stage IA: The tumor is only found on the vulva or on the vulva and perineum and is 2 centimeters (cm) or smaller and less than 1 millimeter (mm) deep. 
    • Stage IB: The tumor is more than 2 cm in size or deeper than 1 mm and is only found on the vulva or on the vulva and perineum.
  • Stage II: The tumor has spread to nearby locations, including the lower parts of the vagina, urethra or anus. It hasn’t spread to the lymph nodes or more distant parts of the body.
  • Stage III: The cancer has spread to nearby areas, such as the urethra, anus or vagina, and to the lymph nodes in the groin. It hasn’t spread to distant places in the body.  
    • Stage IIIA: The tumor has spread to nearby areas and there are only one to two metastases on the lymph nodes that are 5 mm or less or only one metastasis that is 5 mm or less.
    • Stage IIIB: The cancer has spread to nearby areas and there are three or more metastases to the lymph nodes that are 5 mm or smaller or two or more metastases that are 5 mm. 
    • Stage IIIC: The tumor has spread to nearby tissue, one or more lymph nodes and the lymph node covering. 
  • Stage IV: The cancer has spread to the upper portion of the vagina or urethra or to a distant part of the body.
    • Stage IVA: The cancer has spread to the upper urethra, vagina or anus, spread to the regional lymph nodes and caused an ulcer, or caused the lymph node to stick to the tissue beneath it.
    • Stage IVB: The cancer has spread to a distant area in the body. 

Your doctor will use staging to determine what type of treatment is right for you.

During uterine cancer treatment, you’ll be cared for by a multidisciplinary team. This team may include:

  • A gynecologic oncologist
  • A radiation oncologist
  • A nurse navigator
  • Nurse practitioners
  • Physician assistants
  • Oncology nurses
  • Social workers
  • Dietitians
  • Counselors
  • Pharmacists

The treatment will depend on the type and the stage of the disease and may include:

  • Surgery: You may need surgery to remove the tumor and surrounding tissue. Surgery is most often the first treatment for vulvar cancer. Common surgical procedures include:
    • Vulvectomy: During this procedure, all or part of the vulva is removed, depending on the size of the tumor.
    • Laser surgery: During this procedure, a laser beam destroys a precancerous skin lesion. However, laser surgery cannot be used to treat an invasive tumor.
    • Lymphadenectomy: This is a procedure to remove groin lymph nodes.
  • Radiation therapy: Radiation therapy may be used before surgery to help shrink the tumor, after surgery to kill any remaining cancer cells or instead of surgery if you aren’t eligible for surgery. Radiation therapy can be administered either as external radiation therapy (from outside the body) or internal radiation therapy (a radioactive substance is placed in or near the tumor).
  • Chemotherapy: Chemotherapy uses specific drugs to slow or kill cancer cells. With systemic chemotherapy, the medication is injected into a vein or given orally for specific types of vulvar cancer. The goal of chemotherapy is to shrink the tumor and slow its growth.
  • Immunotherapy: Immunotherapy can help your body’s natural defenses fight cancer. It may be recommended if you have vulvar cancer that has spread to another part of the body.

Robotic Surgery for Vulvar Cancer Treatment

Your gynecologic oncologist may recommend robotic surgery, a type of minimally invasive surgery. Robotic surgery gives your doctor more control, a better view and high-definition 3D vision during the procedure. Because this type of surgery requires just a few small incisions, you may experience:

  • Quicker recovery
  • Less blood loss and transfusions
  • Reduced pain
  • Less scarring
  • Lower risk of infection

Ask your doctor if you’re eligible for robotic surgery.

If you have vulvar cancer, you may be wondering if you’ll still be able to have children after treatment. If you’re pre-menopausal, talk with your physician about possible ways to preserve your fertility. You may be referred to a fertility specialist called a reproductive endocrinologist before you begin cancer treatment. Be sure to ask which fertility treatments (if any) your insurance will cover.

See Sarah Cannon’s blog on fertility preservation for more information.