Vulvar cancer is a relatively rare type of cancer that affects the vulva, the external genital organs that protect a female’s reproductive system. Early signs include a lump, itching, and bleeding.

The vulva includes the vagina and vaginal opening, the labia majora, labia minora, clitoris, mons pubis, the bulb of the vestibule, and the greater and lesser vestibular glands.

It is most likely to appear in the outer vaginal lips, or labia, and usually forms over several years.

In 2022, vulvar cancer accounted for around 0.3% of all new cancer cases in the United States.

Here, find out how to recognize vulvar cancer and what to do if you have symptoms.

diagram of vulva showing where cancer can formShare on Pinterest
Anatomy of the vulva and areas where cancer might occur. Image credit: wenzdai figueroa

Vulvar cancer most commonly affects the outer lips of the vagina.

Symptoms can vary between types of vulvar cancer and can develop at different rates.

The first sign usually involves changes to the vaginal lips or the sides of the vaginal opening.

Depending on the type, there may be:

  • a warty growth
  • a lump
  • changes in skin color or texture
  • ulceration
  • itching
  • pain and burning
  • irritation
  • bleeding
  • pain during sexual intercourse
  • painful urination
  • rawness and sensitivity

Different types of vulvar cancer may have different symptoms, and some may have no noticeable symptoms in the early stages.

It is essential to seek help if any changes occur around the vulva or vagina. An early diagnosis can significantly improve the outlook.

Cancer that starts in the vulva is called primary vulvar cancer. If it starts in another part of the body and spreads to the vulva, it is called secondary vulvar cancer.

There are several types of vulvar cancer, and the symptoms can differ. The table below shows some types and their features.

TypeDescription
Vulvar intraepithelial neoplasia (VIN)Cell changes may cause no symptoms.
There may be itching or changes in skin color and texture.
Skin color may be lighter, red, pink, or darker than surrounding skin.
Skin may thicken.
Symptoms can resemble those of noncancerous conditions.
VIN is not cancerous but can become malignant.
Invasive squamous cell carcinoma It affects the flat, outer layers of skin.
Skin may be lighter, darker, red, or pink.
Skin may thicken.
There may be itching, pain, or both.
There may be a red or pink bump or lump with a wart-like, raw, rough, or thick surface.
An open sore may appear and last more than 1 month.
It can take several years for noticeable symptoms to develop.
It accounts for about 90% of all vulvar cancers.
It usually affects people aged 65–74 years.
Vulvar melanomaIt presents as discoloration that may be black, dark brown, white, pink, red, or other colors.
Changes can appear anywhere in the vulva, but most are around the clitoris or on the vaginal opening, or labia.
They can start from a mole.
There is a high risk of metastasis.
It accounts for about 5% of all vulvar cancers.
It is most likely to occur at the ages of 50–70 years.
Bartholin gland carcinomaIt originates in the tissue of cells that line the glands in the vulva.
A mass or lump appears on either side of the vaginal opening.
It can resemble a cyst.
Paget disease of the vulvaThis causes soreness and a red, scaly area.
Verrucous carcinoma It is a subtype of squamous cell cancer.
It tends to appear as a slowly growing, cauliflower-like wart.
It can resemble a noncancerous genital wart.

Experts do not know precisely why vulvar cancer happens, but various factors appear to increase the risk:

  • Age: Over 50% of cases occur in females aged 70 years or over, and fewer than 20% are in people aged 50 or under.
  • Human papillomavirus (HPV): Some types of HPV lead to genital warts that can become cancerous. Over half of all vulvar cancers are linked to HPV. They are more likely to occur in younger people.
  • Smoking: Smoking increases the risk of various types of cancer. People who smoke and have the HPV virus have a higher risk.
  • Inflammatory conditions: Some inflammatory conditions that affect the vulva — such as lichen sclerosus et atrophicus (LSA) — appear to increase the risk. Around 4% of females with LSA go on to develop vulvar cancer.
  • Radiation in the pelvic area: People who have previously had radiation therapy sometimes develop a new and different type of cancer in the affected area, as radiation affects the DNA of cells.
  • Immunodeficiency: Some types of immunodeficiency increase the risk of vulvar cancer. It could be due to a genetic cause or a disease such as HIV.
  • Moles and melanoma: People with moles or melanoma anywhere on the body may have a higher chance of developing vulvar cancer.
  • Cervical cancer: People who have previously had precancerous changes in the cervix or cervical cancer may have a higher chance of vulvar cancer.

If a person visits a doctor with concerns about changes to the vulva, the doctor will most likely:

  • ask about signs and symptoms
  • ask about the person’s individual and family medical history
  • carry out a physical examination
  • recommend a biopsy, if anything looks unusual

Depending on the results of the biopsy, there may be further tests:

  • A Pap smear involves taking a sample of cervical cells to assess in a laboratory.
  • Cystoscopy is used to examine the bladder and look for signs of cancer.
  • Proctoscopy is used to the rectum to check if cancer is present.
  • An MRI, CT scan, or X-ray can look for signs of cancer elsewhere in the body.

If a biopsy confirms the presence of vulvar cancer, the doctor will stage it with the help of imaging scans.

There are different ways of staging cancer.

To stage vulvar cancer, a doctor may use the following:

  • Stage 0 or carcinoma in situ: Cell changes are present on the surface of the skin.
  • Stage 1: Cancer is limited to the vulva or perineum.
  • Stage 2: Cancer of any size has reached nearby structures, such as the vagina, but not the lymph nodes.
  • Stage 3: Cancer may or may not have reached nearby structures and has spread to lymph nodes.
  • Stage 4: Cancer has started to reach organs further from the original site.

Early diagnosis and treatment can often prevent the spread of cancer and improve the outlook.

Treatment for vulvar cancer usually involves a combination of approaches.

Surgery

Surgery aims to remove the cancer while leaving sexual function intact. The extent of the surgery will depend on how far cancer has spread.

Here are some types of surgery:

  • Laser surgery uses a laser beam to remove lesions.
  • Excision involves removing cancerous tissue and some healthy tissue around it.
  • Skinning vulvectomy is when a surgeon removes only the layer of skin affected by cancer and repairs it with a skin graft.
  • Radical vulvectomy removes the vulva, including the clitoris, vaginal lips, the opening to the vagina, and usually the nearby lymph nodes as well.

Reconstructive surgery may be possible, depending on how much tissue is removed.

Radiation therapy

Radiation therapy can:

  • shrink deep lesions or tumors before surgery, making them easier to remove
  • treat lymph nodes to remove any remaining cancer cells
  • relieve symptoms and improve quality of life for people with later-stage cancer

Chemotherapy

People may have chemotherapy alongside radiotherapy as part of treatment or palliative care. It can take the form of a cream or lotion to apply to the skin, but the method will depend on the stage and extent of the cancer.

Other therapies

Some drugs target specific functions that appear to boost the development of cancer cells. They can slow or stop cancer from growing by altering these functions.

Biologic therapy is a kind of immunotherapy. It uses either synthetic or natural substances to help the body defend itself against cancer. Imiquimod is an example. It may be applied topically, as a cream, to treat vulvar cancer.

What is the difference between chemotherapy and immunotherapy?

Measures that can reduce the risk of developing vulvar cancer include:

  • using a condom during sex
  • attending scheduled Pap tests
  • having the HPV vaccination
  • not smoking

There is no standard screening for vulvar cancer, but people should attend any recommended checkups and be aware of any changes to their bodies. Doing this can help spot the early signs of vulvar cancer.

Survival rates refer to a person’s chance of surviving another 5 years or longer after a diagnosis of vulvar cancer compared with someone who does not have a diagnosis.

According to the American Cancer Society, the relative survival rates for vulvar cancer are as follows:

StageSurvival rate
Localized (cancer is in the vulva only)86%
Regional (it has spread to nearby tissues and lymph nodes)53%
Distant (it has reached other organs throughout the body)19%
All stages combined71%

However, the outlook will depend on the type of vulvar cancer and other factors. The outlook for vulvar melanoma is less positive than for other types, and there is a 50% chance of a recurrence.

Overall, the chance of vulvar cancer recurring is 37% within 5 years of an initial diagnosis.

Here are some questions people often ask about vulvar cancer.

What are the warning signs of vulvar cancer?

The early signs include:

  • changes in skin color and texture
  • the appearance of warts, lumps, or bumps on the skin
  • itching, burning, and irritation
  • pain during sex or when urinating

Symptoms usually appear first on the lips of the vagina.

What is the first stage of vulvar cancer?

At the first stage, cancer only affects the vulva and perineum and has not spread to other areas.

What is the survival rate for vulvar cancer?

For all stages combined, a person with vulvar cancer has a 71% chance of surviving another 5 years after diagnosis, compared with someone without the disease. However, this may be higher or lower depending on the stage at diagnosis, the type of cancer, and other factors.

Vulvar cancer affects the area in and around the female genitalia, including the labia and clitoris. There are different types of vulvar cancer, but they can all involve skin changes, itching, and pain during sex or urination. Treatment options include surgery, chemotherapy, and radiation therapy.

With early detection, the 5-year survival rate is 86%, but various factors can affect this.

Anyone who notices skin changes or other symptoms around the vulva should seek medical advice, as early treatment can help prevent the progression of vulvar cancer.