Vulvar Cancer

Vulvar cancer is cancer that begins in the vulva, the outer part of the female genitals. It is a rare cancer that comprises about 6% of gynecologic cancer diagnoses.

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Screening for Vulvar Cancer

There is no standard screening tool for vulvar cancer. Routine pelvic exams, and awareness of possible symptoms, can improve the likelihood of earlier detection, and more effective treatment.

Symptoms of Vulvar Cancer

Different types of vulvar cancer can have different symptoms.

Pre-cancerous changes of the vulva, called vulvar intraepithelial neoplasia (VIN), may be asymptomatic or may cause itching that does not go away or improve, or changes in the skin of the vulva with areas  that are thicker, or a different color from the surrounding area. 

Invasive squamous cell cancer of the vulva may also be asymptomatic or may present with similar symptoms, which can include:

  • An area of the vulva that looks different from surrounding area, either lighter or darker, or reddish or pinkish
  • A bump or lump, which may be red, pink, or white, with a wart-like, raw, rough, or thick surface
  • Thickening vulvar skin
  • Itching
  • Pain or burning
  • Bleeding or discharge that is unrelated to menstruation
  • An open sore, especially lasting for a month or more
  • Cauliflower-like warty growths 

Melanomas can also develop on the vulva.  Symptoms of vulvar melanoma can include:

  • A lump
  • Pain or itching
  • Bleeding or discharge
  • Discolored areas, which are most often black or dark brown, but can also be pink, red, white, or other colors
  • Changes in the appearance of a mole or changes in pigmentation 

Rare vulvar cancers includ Bartholin gland cancer, which maybe associated with a distinct mass or lump on the side of the vaginal opening.  Most often, a lump of this type is caused by a Bartholin gland cyst, which is not cancerous.

Paget disease is a rare cancer that can develop onthe vulva which may be associated with soreness, and a red, scaly area on the skin.

Risk Factors for Vulvar Cancer

There are several risk factors that can increase a person’s likelihood of developing vulvar cancer, including:

  • Increased age, as more than half of women diagnosed with vulvar cancer are over 70, and less than 20% occur in women under age 50.
  • Human papillomavirus (HPV) infection
  • Smoking
  • Human immunodeficiency virus (HIV)
  • Vulvar intraepithelial neoplasia (VIN) is a medical term for the pre-cancerous condition where abnormal cells are found only in the surface layer of the vulvar skin
  • Lichen sclerosis, also called lichen sclerosis et atrophicus (LSA), a condition that causes vulvar skin to be thin and itchy, is associated with higher risk of vulvar cancer, with about 4% of those with LSA eventually developing the disease
  • Other genital cancers
  • Melanoma or atypical moles, or a family history of melanoma

Types of Vulvar Cancer

  • Squamous cell carcinomas, cancers that start in squamous skin cells, are the most common type of vulvar cancer. There are several subtypes of squamous cell carcinomas of the vulva, including keratinizing type which is the most common, basaloid and warty types which are less common, and verrucuous carcinoma which is uncommon but has a better prognosis.
  • Adenocarcinoma, cancer that starts in the gland cells, accounts for about 8 out of 100 vulvar cancers.
  • Melanoma, which starts in pigment-producing skin cells, accounts for about 6 out of 100 vulvar cancers.
  • Sarcoma, which starts in connective tissue or muscle cells, accounts for fewer than 2 in 100 vulvar cancers.
  • Basal cell carcinoma is the most common type off skin cancer, and occurs on the vulva very rarely. 

Diagnosing Vulvar Cancer

If a doctor suspects vulvar cancer, the first step is often to perform a physical and pelvic exam, along with a discussion of the patient’s medical history. After these steps, if the doctor believes a patient may have vulvar cancer, they will perform a biopsy. A biopsy is the only way to confirm a diagnosis of vulvar cancer.

  • A doctor may perform a vulvoscopy, which is an external examination of the vulva using a magnifying lens called a colposcope, to identify a site for biopsy.
  • A doctor may also use vinegar to make any pre-cancerous or cancerous cells more visible.
  • Depending on the size of the abnormal area, doctors may perform an excision biopsy (complete removal of abnormal cells) or a punch biopsy (removal of a small sample of abnormal cells). A local anesthetic is used on the biopsy area.

Stages of Vulvar Cancer

Stages of vulvar cancer range from stage 1 (1) to stage 4 (IV). In general, a lower stage refers to vulvar cancer that is localized, whereas a higher stage means that the cancer has spread. Vulvar cancer staging can be complex, with doctors basing exact stage on the size of the tumor, the spread to nearby lymph nodes, and the spread to distant lymph nodes or parts of the body. The stage of cancer will help guide plans for care and treatment. Learn more about stages of vulvar cancer.

Vulvar Cancer Treatment

The main type of treatment for vulvar cancer is surgery to excise the lesion and a small amount of surrounding tissue.  This may also involve resection of lymph nodes in the groin to look for evidence that the cancer has spread. If cancer has spread, more extensive surgery may be needed, and may be followed by radiation and chemotherapy. . Learn more about treatment for vulvar cancer.

Recurrent Vulvar Cancer

If cancer comes back after treatment, it is called a recurrence. It is sometimes challenging to determine whether a person has a recurrence of a prior vulvar cancer or a new lesion that has developed on the vulva. Close surveillance can help identify evidence of new lesions or recurrence early, which improves outcomes.  

Vulvar Cancer Survival Rates and Incidence

The overall 5-year relative survival rate for those diagnosed with vulvar cancer is 71%. Survival rates differ based on when the cancer was first diagnosed and treated. The 5-year relative survival rate for localized vulvar cancer, when the cancer is only in the vulva and has not spread, is 86%. The 5-year relative survival rate for vulvar cancer with regional spread, to  nearby lymph nodes or tissues but not distant organs, is 53%. The 5-year relative survival rate for vulvar cancer with spread to distant parts of the body is 19%.

The American Cancer Society estimates that in 2022 in the U.S., about 6,330 cancers of the vulva will be diagnosed, and 1,560 women will die of vulvar cancer. Vulvar cancer accounts for <1% of cancers in women, and women have about a 1 in 133 chance of developing vulvar cancer in their lifetime.

For more detailed information about vulvar cancer, visit the American Cancer Society.