Vulvar Cancer: Causes, Symptoms, and Treatments

Cancer can develop in any part of the body, including the outer part of the female genitalia, also called the vulva. At some point during their life, 1 out of 333 people with a female reproductive system will develop vulvar cancer.

Key takeaways:
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    Vulvar cancer develops in the external female genitalia and is a rare disease.
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    Older people, those who smoke, or with HPV infection have an increased chance of developing vulvar cancer, but vulvar cancer can be found in any person with a female reproductive system at any age.
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    Warning signs of vulvar cancer include itching in the vulvar area that doesn’t improve and bleeding not associated with your menstrual periods.
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    Be observant about changes in your body and talk to your healthcare provider immediately if you have any concerns.
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    Discussing cancer that affects the sex organs can be difficult and uncomfortable. But don’t let that stop you from getting the emotional, physical, and social help you need.

Vulvar cancer is a rare type of gynecologic cancer that tends to grow slowly, usually over several years. In the U.S., about 6,330 people will be diagnosed with vulvar cancer in 2022, and an estimated 1,560 people are expected to die from it.

What is vulvar cancer?

The vulva is made up of the:

Mons pubis: The fatty area is shaped like an upside-down triangle and is usually covered with hair at puberty.

Labia majora: This is Latin for “larger lips.” It’s the outer, fleshy skin fold you see by looking at yourself naked.

Labia minora: This term means “smaller lips.” You see these smaller, inner, thinner lips if you pull the labia majora apart.

Vaginal opening: The hole through which a penis or a tampon goes; it also contains the Bartholin glands.

Clitoris: The sensitive tissue that becomes swollen during sex.

Vulvar cancer occurs when cells grow out of control in any part of the vulva. It most often develops in the labia and less in the clitoris and Bartholin glands. It’s commonly found in people over age 70.

Squamous cell carcinoma accounts for 90% of vulvar cancer. Adenocarcinoma, melanoma, sarcoma, and basal cell carcinoma can also be found in the vulva.

Who’s at risk for vulvar cancer?

Research is being done to understand the causes of vulvar cancer. Still, scientists know the following can increase your chance of developing vulvar cancer:

  • Being older – the odds of getting vulvar cancer increase as you age.
  • Carrying the human papillomavirus (HPV) – about half of the vulvar squamous cell carcinomas are associated with HPV infection.
  • Smoking.
  • Having human immunodeficiency virus (HIV) infection or a weak immune system.
  • Having precancerous cell changes like vulvar intraepithelial neoplasia (VIN).
  • Having a condition called lichen sclerosus.
  • Having a personal history of cervical cancer or Paget’s disease of the vulva.
  • Having had melanoma elsewhere in the body or a family history of melanoma.

How to reduce your risk of vulvar cancer

Avoiding infection with HPV is one way to reduce your risk of vulvar cancer. This includes limiting the number of sexual partners, using condoms every time you have sex and getting the HPV vaccine.

You may also reduce your risk of vulvar cancer by not smoking. If you smoke, it’s not too late to quit to lower your risk of vulvar cancer, other types of cancer, and other diseases.

Get your gynecologic checkups done regularly to increase your chance of catching potential signs or precancerous cell changes before they become cancer.

Vulvar cancer symptoms to watch for

You may not have any warning signs in the early stages of vulvar cancer. Some people with VIN may develop persistent vulvar itching.

People with squamous cell carcinoma of the vulva may experience:

  • A change in how the vulva looks – like redness, a lighter or darker area.
  • A bump or lump anywhere in the vulva.
  • Thickening of the skin.
  • Vulvar itching that doesn’t get better.
  • Pain, soreness, or burning of the vulva.
  • Bleeding or discharge unrelated to the menstrual period.
  • A growth that looks like a genital wart.
  • An open sore or wound that doesn’t heal.

You may think of sun exposure when you hear the word melanoma, but melanoma can develop in areas like the eyes, nails, and genitals. Vulvar melanoma can appear as a brown or dark mole and is most often found around the clitoris or on the labia majora or minora.

If you notice a mole in your vulva, use the ABCDE rule of melanoma to help determine if it could be melanoma. But it’s always a good idea to see a healthcare professional, as some melanoma may not have all the ABCDE features.

The rule of thumb is to seek medical help if you notice any warning signs or are concerned about anything unusual with your body.

How is vulvar cancer diagnosed?

During your visit, your gynecologist will inspect the vulva and do a pelvic exam.

If abnormalities are found, you may undergo a biopsy, where pieces of tissue are removed and sent to the lab to check for cancer cells. If the biopsy reveals vulvar cancer, you will likely be referred to a gynecologic oncologist – a healthcare provider specializing in gynecologic cancers.

Stages of vulvar cancer

Knowing the stage of your cancer helps your oncologist determine if the tumor has spread and is essential in selecting the appropriate treatment. Staging involves additional tests like a colposcopy, computed tomography (CT) scan, magnetic resonance imaging (MRI), and positron emission tomography (PET) scan.

Vulvar cancer is described in four stages:

Stage I: Cancer is found in the vulva or the perineum (the area between the opening of the vagina and the rectum). In this stage, the tumor has not spread to the lymph nodes.

Stage II: Cancer has spread to the vagina, lower part of the urethra, or anus but has not spread to the lymph nodes.

Stage III: The tumor has reached nearby organs like the vagina, urethra, or anus and the lymph nodes in the groin, but it has not been found in distant organs.

Stage IV: Cancer has spread beyond the vulva and nearby organs and has reached distant organs like the lung or bones.

Each stage is further characterized by a substage.

For example, stage IA means that the tumor is only in the vulva or perineum, is less than 2 centimeters (about the size of a peanut) in size, has not spread to nearby lymph nodes, and hasn’t spread more than 1 millimeter (about the size of a sharp pencil point) deep into the vulvar tissue.

In stage IB, the tumor is larger than 2 centimeters and deeper than 1 millimeter, but it has not spread beyond the vulva or perineum and is not found in the lymph nodes.

How is vulvar cancer treated?

Once the stage is known, your providers will discuss the appropriate treatment recommendation.

VIN is frequently treated with laser surgery or chemotherapy medicine applied directly to the skin.

For invasive vulvar cancer, surgery is the primary treatment. Sometimes, radical local excision of the vulva is performed; this involves the removal of the tumor and healthy tissue around it.

Part of the vulva may be removed for larger tumors; this is called a partial or modified radical vulvectomy. Rarely a complete radical vulvectomy, which involves the removal of the entire vulva, including the clitoris, is needed. The groin lymph nodes may also be removed in a lymphadenectomy. A more extensive surgery – pelvic exenteration – may be performed for more advanced cases of vulvar cancer.

A vulvar reconstruction in the form of a skin graft or skin flap may be necessary. Talk to your providers about your options, benefits, potential complications, and how surgery may affect your quality of life.

Chemotherapy may be combined with radiation therapy to shrink a very large vulvar tumor before surgery. This is referred to as neoadjuvant chemoradiation.

Radiation therapy may be used after surgery to destroy any remaining cancer cells. To help boost your immune system, your cancer care team may recommend adding immunotherapy. Your healthcare provider may also discuss participation in a clinical trial.

During and after treatment for vulvar cancer, you may experience an emotional rollercoaster, especially if surgery was part of your treatment. You may feel less attractive, less feminine, and embarrassed. Whether your cancer care team or a mental health therapist, a healthcare professional is an excellent resource to help you manage these emotions and regain your self-confidence. Don’t hesitate to talk to them about how you feel, even if it’s an uncomfortable conversation. Your provider may encourage you to join a cancer support group where you can discuss your experiences with people who can relate.

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