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Vulvar Cancer

To view this article in the new Rare Cancers Australia Knowledgebase, click here 

Definition of vulvar cancer:

Cancer of the vulva (the external female genital organs, including the clitoris, vaginal lips, and the opening to the vagina). 

Vulvar cancer is a rare disease in which malignant (cancer) cells form in the tissues of the vulva.

Vulvar cancer forms in a woman's external genitalia. The vulva includes:

  • Inner and outer lips of the vagina.
  • Clitoris (sensitive tissue between the lips).
  • Opening of the vagina and its glands.
  • Mons pubis (the rounded area in front of the pubic bones that becomes covered with hair at puberty).
  • Perineum (the area between the vulva and the anus).

Anatomy of the vulva; drawing shows the mons pubis, clitoris, urethral opening, inner and outer lips of the vagina, and the vaginal opening. Also shown are the perineum and anus.

Anatomy of the vulva. The vulva includes the mons pubis, clitoris, urethral opening, inner and outer lips of the vagina, vaginal opening, and perineum.

Vulvar cancer most often affects the outer vaginal lips. Less often, cancer affects the inner vaginal lips, clitoris, or vaginal glands.

Vulvar cancer usually forms slowly over a number of years. Abnormal cells can grow on the surface of the vulvar skin for a long time. This condition is called vulvar intraepithelial neoplasia (VIN). Because it is possible for VIN to become vulvar cancer, it is very important to get treatment.

Having vulvar intraepithelial neoplasia or HPV infection can affect the risk of vulvar cancer.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for vulvar cancer include the following:

  • Having vulvar intraepithelial neoplasia (VIN).
  • Having human papillomavirus (HPV) infection.
  • Having a history of genital warts.

Other possible risk factors include the following:

  • Having many sexual partners.
  • Having first sexual intercourse at a young age.
  • Having a history of abnormal Pap tests (Pap smears).

Possible signs of vulvar cancer include bleeding or itching.

Vulvar cancer often does not cause early symptoms. When symptoms occur, they may be caused by vulvar cancer or by other conditions. Check with your doctor if you have any of the following problems:

  • A lump or growth on the vulva.
  • Changes in the vulvar skin, such as color changes or growths that look like a wart or ulcer.
  • Itching in the vulvar area, that does not go away.
  • Bleeding not related to menstruation (periods).
  • Tenderness in the vulvar area.

Tests that examine the vulva are used to detect (find) and diagnose vulvar cancer.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking the vulva for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Biopsy: The removal of samples of cells or tissues from the vulva so they can be viewed under a microscope by a pathologist to check for signs of cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

  • The stage of the cancer.
  • The patient's age and general health.
  • Whether the cancer has just been diagnosed or has recurred (come back).

Paget's disease of vulva and vagina

Paget’s disease of the vulva is an unusual kind of skin cancer that arises from glandular cells. This disease appears as a red, velvety area with white islands of tissue on the vulva. At times it may be pink, and occasionally there are moist, oozing ulcerations that bleed easily.

Most people with the condition will experience itching and soreness. Almost all patients are postmenopausal, Caucasian women.

The cause of this disorder is unknown, and it is usually diagnosed by a biopsy. The typical treatment is surgery. In more severe cases, you may need to have skin grafting to close the wound. The tumor will frequently extend into what appears to be normal skin, making the extent of the surgical resection difficult to determine.

Approximately 15% of patients with vulvar Paget’s disease will also have invasive Paget’s disease or an underlying invasive adenocarcinoma of the vulva. This disease requires careful follow-up as it has a high recurrence rate.

Undifferentiated carcinoma of the vulva and vagina

Undifferentiated carcinoma is a form of carcinoma composed of poorly differentiated cells whose type is difficult to establish; these cells tend to be more virulent or aggressive. 

For more information on Vulvar Cancer click here

Information has also been sourced from &

This link is to the National Cancer Institute (NCI) cancer website in the United States. There may be references to drugs and clinical trials that are not available here in Australia.

For information about clinical trials that are available in Australia click here

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