Vulvar Cancer

Vulvar cancer is cancer in your vulva, the collective name for all the female external sex organs (genitals). Vulvar cancer usually develops slowly over several years. It typically starts as an abnormal growth in your labia. Your healthcare provider may say you have vulvar intraepithelial neoplasia (VIN). The growth isn’t cancer. But without treatment, VIN can become vulvar cancer.

Vulvar cancer is rare. Each year, between 2 and 3 women out of 100,000 in the U.S. are diagnosed with it.

Surgery to remove the tumor before it spreads may cure vulvar cancer.

Types of vulvar cancer

Healthcare providers classify this condition by the type of cells that cause it. The most common vulvar cancer types are:

• Vulvar squamous cell carcinoma: This type develops in squamous cells. These cells form the outermost layer of your skin. Vulvar squamous cell carcinoma accounts for 9 out of 10 vulvar cancer cases. This type typically affects your labia, the two sets of skin folds that surround your vulva.
• Vulvar melanoma: This type develops in melanocytes. These cells make pigment that determines your skin tone. Vulvar melanoma can start anywhere in your vulva, but most often affects your labia and clitoris. It accounts for 5 out of 100 cases of the disease.

The other types are rare and include:

• Vulvar basal cell carcinoma
• Bartholin gland adenocarcinoma
• Paget disease of the vulva
• Vulvar sarcoma
• Verrucous carcinoma of the vulva

Symptoms of vulvar cancer

The condition may cause changes in your vulva, including:

• Bleeding that’s not related to your period
• Burning and itching
• Sores, lumps or wart-like bumps
• Pain when you pee or have sex
• Skin changes that make the skin in your vulva look darker or lighter than what’s normal for you
• Skin changes that may include white patches or skin that feels rough or thicker than usual

Vulvar cancer causes

This condition happens when healthy cells change into cancer cells. The cells multiply to form tumors in your vulva. Two common causes are:

• Exposure to certain types of human papillomavirus (HPV)
• Lichen sclerosus

Other issues may increase your risk, including:

• Age: Your likelihood of developing vulvar cancer increases with age. More than half of all cases occur in women over 70.
• HIV infection: A weakened immune system from a condition like HIV can make it harder for your body to fight cancer.
• Smoking: Smoking can affect your immune system and make it hard for your body to get rid of infections, including HPV.

Complications of vulvar cancer

Without treatment, it can spread to places like your anus, cervix, urethra or rectum. It may spread to more distant parts of your body, like your bones, lymph nodes or lungs. Cancer that spreads is more difficult to treat than localized or regional cancer.

How doctors diagnose this condition

A healthcare provider will ask about your symptoms. They’ll do a pelvic exam to look for changes in your vulva, including unusual skin color, sores or bumps. They may do tests including:

• Pap smear: Your provider will check your cervix. They’ll take some cells to check for cancer. They may also do an HPV test.
• Vulvoscopy: Your provider may use a lighted, magnifying instrument called a colposcope to view your vulva, vagina and cervix in more detail. They may apply a solution that highlights abnormal cells.
• Biopsy: Your provider may get a small tissue sample for analysis.
• Imaging tests: You may have CT and MRI scans. The tests may show the tumor’s location and size. They may also detect cancer in nearby lymph nodes.

Your provider may do additional tests to check for cancer in other parts of your body, such as:

• Cystoscopy, to look for vulvar cancer in your bladder or urethra
• Imaging tests, like PET CT scans
• Proctoscopy, to check your anus and rectum

Vulvar cancer stages

Cancer staging allows healthcare providers to determine how far cancer has spread. This information guides treatment decisions. There are four main stages:

• Stage I: There’s cancer in your vulva or perineum (the area between your rectum and vagina). Stage 1A (IA) tumors are small (up to 2 centimeters) and have limited spread. Stage 1B (IB) tumors are larger and invade more deeply into nearby tissue.
• Stage II: The tumor spreads into the lower part of your urethra, the lower part of your vagina or anus.
• Stage III: Cancer has spread to one or more nearby lymph nodes or places like the upper two-thirds of your vagina or urethra or the inner layers of your bladder or rectum. Stage III consists of stages 3A (IIIA) 3B (IIIB) and 3C (IIIC). These stages vary.
• Stage IV: This stage consists of stages 4A (IVA)and 4B (IVB). With stage 4A (IVA), the tumor is in your pelvic bone and nearby lymph nodes. With stage 4B (IVB), the cancer has spread to more distant parts of your body, like your bones or lungs.

How is vulvar cancer treated?

Surgery is the most common treatment for vulvar cancer. The goal is to remove the cancerous tumor while preserving your ability to have sex. A gynecologic oncologist surgeon will do the procedure. Types of surgery include:

• Laser surgery: This treats VIN, the condition that can become vulvar cancer.
• Excision: Your surgeon removes the tumor and some healthy tissue. They may call this a local excision.
• Vulvectomy: This surgery removes part or all of your vulva. Your surgeon may remove some nearby lymph nodes to look for cancer.

Very rarely, your surgeon may remove your vulva, vagina, bladder and rectum. This procedure is an option if vulvar cancer comes back or surgery and other treatments don’t work.

You may have other treatments, too, such as:

• Radiation therapy: You may have external beam radiation therapy before surgery to shrink a tumor or after surgery to kill any remaining cancer cells. Your provider may combine radiation and chemotherapy (chemoradiation).
• Chemotherapy: This treatment kills cancer cells. You may have medication that attacks cancer cells throughout your body. Cisplatin (Platinol^®, Platinol -AQ^®) and fluorouracil (Carac^®) are commonly prescribed chemotherapy drugs for vulvar cancer. Your provider may prescribe a lotion you apply to your vulva to treat cancer in a specific area.
• Immunotherapy: This treatment helps your immune system find and destroy cancer. Your provider may prescribe pembrolizumab (Keytruda^®).

Researchers are studying new treatments for this rare cancer. Ask your cancer care team about clinical trials for vulvar cancer.

Recovery time

Your recovery time will depend on your treatment. It can take up to eight weeks to recover from surgery to remove part or all of your vulva. Your surgeon and cancer care team will explain what you can expect.

When should I see my healthcare provider?

Talk to your cancer care team if you have infection symptoms, like:

• Fever
• Pus draining from the incision

A vulvectomy to remove a cancerous tumor can be life-changing. Research shows that women feel like the surgery affects how they feel about their body and their sexuality. You may feel anxious and depressed after surgery. These are common emotional reactions. Your cancer care team understands all the ways this experience may affect you. They may recommend mental health support.

Is vulvar cancer life-threatening?

It can be if the cancer spreads from your vulva to other areas of your body. The National Cancer Institute (U.S.) tracks the five-year relative survival rate for vulvar and other cancer types. In this case, experts compare five-year survival rates of women who have vulvar cancer and women who don’t. For example, if the five-year relative survival is 80%, women with vulvar cancer are 80% as likely to be alive five years after diagnosis as women who don’t have the condition.

NCI data show that 85% of women with early-stage vulvar cancer were alive five years after their diagnosis. That survival rate drops to 51% when cancer is in nearby lymph nodes and to 21% when it spreads to more distant parts of your body.

How do you check yourself for vulvar cancer?

You should check your vulva monthly, before or after your period. To do a vulva self-examination, sit down or place one leg on a chair. Use a mirror to see your entire vulva. Use your fingers to check for bumps or lumps. Talk to your healthcare provider if you notice a change, like skin that’s lighter or darker than normal.

Can I prevent vulvar cancer?

There’s no clear cause for this condition. But avoiding HPV infections and not smoking may help reduce your risk. And schedule regular checkups with a gynecologist. They may spot changes that could be early signs of vulvar cancer.