Vulvar cancer

Overview

Vulvar cancer is cancer that starts as a growth of cells on the vulva. The vulva is the area of skin that surrounds the urethra and vagina. It includes the clitoris and labia.

Vulvar cancer commonly forms as a lump or sore on the vulva that often causes itching. Though it can happen at any age, vulvar cancer is usually diagnosed in older adults.

Vulvar cancer treatment often starts with surgery to remove the cancer and a small amount of surrounding healthy tissue. Sometimes vulvar cancer surgery requires removing the entire vulva. The earlier vulvar cancer is diagnosed, the less likely an extensive surgery is needed for treatment.

Symptoms

Signs and symptoms of vulvar cancer may include:

• A lump, wartlike bump or an open sore on the vulva.
• Bleeding in the genital area that isn't from menstruation.
• Itching of the skin of the vulva that doesn't go away.
• Pain and tenderness that affects the vulva.
• Skin changes, such as changes in the color of the skin of the vulva or thickening of the skin.

When to see a doctor

Make an appointment with a doctor, gynecologist or other healthcare professional if you have any symptoms that worry you.

Causes

It's not always clear what causes vulvar cancer. This cancer starts on the area of skin that surrounds the urethra and vagina. This area of skin is called the vulva.

Vulvar cancer happens when cells in the vulva develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to make many more cells quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells.

The cancer cells might form a growth called a tumor. The tumor can grow to invade and destroy healthy body tissue. In time, cancer cells can break away and spread to other parts of the body. When cancer spreads, it's called metastatic cancer.

Exactly what causes the DNA changes that lead to vulvar cancer isn't always known. Healthcare professionals believe some vulvar cancers are caused by human papillomavirus. Human papilloma virus, also called HPV, is a common virus passed through sexual contact. It's associated with the most common type of vulvar cancer, which is vulvar squamous cell carcinoma.

Types of vulvar cancer

The type of cell in which the cancer begins tells your healthcare team what type of vulvar cancer you have. Knowing your vulvar cancer type helps your healthcare team plan the most effective treatment. Some types of vulvar cancer include:

• Vulvar squamous cell carcinoma. Squamous cell carcinoma of the skin is cancer that starts in cells called squamous cells. Squamous cell carcinoma of the skin happens most often in skin exposed to the sun. But it can happen on any skin, including the skin of the vulva. Most vulvar cancers are squamous cell carcinomas. This type of vulvar cancer is associated with HPV infection.
• Vulvar melanoma. Melanoma is cancer that starts in the pigment-producing cells called melanocytes. Melanoma most often happens on skin that's exposed to the sun. But it can happen anywhere on the skin, including the skin of the vulva.
• Extramammary Paget's disease of the vulva. Extramammary Paget's disease is a cancer that starts near sweat glands in the skin. It most often affects the skin of the vulva. Sometimes it happens at the same time as another kind of cancer. This can include a cancer in the breast, colon, urinary system or female reproductive system.

Risk factors

Factors that increase the risk of vulvar cancer include:

• Older age. The risk of vulvar cancer increases with age, though it can happen at any age. The average age at diagnosis is 65.
• Being exposed to human papillomavirus. Human papillomavirus, also called HPV, is a common virus that's passed through sexual contact. HPV increases the risk of several cancers, including vulvar cancer and cervical cancer. Many young, sexually active people are exposed to HPV. For most the infection goes away on its own. For some, the infection causes cell changes and increases the risk of cancer in the future.
• Smoking tobacco. Smoking tobacco increases the risk of vulvar cancer.
• Having a weakened immune system. If the body's germ-fighting immune system is weakened by medicines or illness, there might be a higher risk of vulvar cancer. People with a weakened immune system include those taking medicines to control the immune system, such as after an organ transplant. Certain medical conditions, such as infection with HIV, can weaken the immune system.
• Having a history of a precancerous condition of the vulva. Vulvar intraepithelial neoplasia is a precancerous condition that increases the risk of vulvar cancer. Most instances of vulvar intraepithelial neoplasia will never develop into cancer. But a small number do go on to become invasive vulvar cancer.
• Having a skin condition involving the vulva. Lichen sclerosus causes the vulvar skin to become thin and itchy. It also increases the risk of vulvar cancer.

Prevention

To lower the risk of vulvar cancer, don't smoke tobacco. Take steps to protect yourself from human papillomavirus infection. Human papillomavirus, also called HPV, is associated with the most common type of vulvar cancer.

Don't use tobacco

Smoking tobacco increases the risk of vulvar cancer. If you don't smoke, don't start. If you smoke, talk with a healthcare professional about things that can help you quit. This might include medicines and counseling.

Lower your risk of HPV infection

HPV is a common virus that's passed through sexual contact. It increases the risk of vulvar cancer. To help reduce the risk of HPV infection:

• Use a condom every time you have sex. Condoms may reduce the risk of contracting HPV but can't fully protect against it.
• Get the HPV vaccine. The vaccine protects against the strains of the virus that are thought to cause vulvar cancer. Talk with a healthcare professional about whether the HPV vaccine is right for you.

Diagnosis

Vulvar cancer diagnosis often begins with a physical exam and a discussion of your health history. A special magnifying device may be used to closely inspect the area. A sample of tissue may be taken for lab testing.

Examining the vulva

Your healthcare professional will likely conduct a physical exam of your vulva to look for anything concerning.

The healthcare professional may use a special magnifying instrument to look closely at the vulva. This instrument is called a colposcope. It also may be used to look at the vagina and cervix.

Biopsy

A biopsy is a procedure to remove a sample of tissue for testing in a lab. For vulvar cancer, a biopsy involves removing a sample of skin.

A vulvar biopsy may be done in a healthcare professional's office. Medicine is used to numb the area. The health professional may use a blade or a circular cutting tool to remove some skin.

Sometimes the sample is removed in an operating room. During this kind of biopsy, you receive medicine to put you in a sleep-like state so that you're not aware during the procedure.

Vulvar cancer staging

If you're diagnosed with vulvar cancer, the next step is to determine the cancer's extent, called the stage. Your healthcare team uses the cancer staging test results to help create your treatment plan.

Staging tests may include:

• Examination of your pelvic area for cancer spread. Your healthcare professional may do a more thorough examination of your pelvis for signs that the cancer has spread.
• Imaging tests. Images of your chest, abdomen or pelvis may show whether the cancer has spread to those areas. Tests might include X-ray, MRI, CT and positron emission tomography, which also is called a PET scan.

The stages of vulvar cancer range from 1 to 4. A stage 1 vulvar cancer is small and confined to the vulva. As the cancer gets larger or spreads beyond the area where it started, the stages get higher. A stage 4 vulvar cancer has grown into the pelvic bone or spread to other parts of the body.

Treatment

Treatment for vulvar cancer usually begins with surgery to remove the cancer. Other treatments may include radiation therapy, chemotherapy, targeted therapy and immunotherapy.

Your healthcare team considers many factors when creating a treatment plan. These factors may include your overall health, the type and stage of your cancer, and your preferences.

Surgery

For most vulvar cancers, surgery is the first treatment. Procedures used to treat vulvar cancer include:

• Removing the cancer and some healthy tissue. An excision involves cutting out the cancer and a small amount of healthy tissue that surrounds it, called a margin. Cutting out a margin of healthy-looking tissue helps ensure that all of the cancer cells have been removed. This procedure also might be called a wide local excision or a radical excision.
• Removing part of the vulva or the entire vulva. Vulvectomy is a surgery to remove the vulva. When part of the vulva is removed, it's called a partial vulvectomy. When the entire vulva and the underlying tissue are removed, it's called a radical vulvectomy. Vulvectomy may be an option for larger cancers. Radiation therapy and chemotherapy may be used before surgery to shrink the cancer. This may allow for a less extensive operation.
• Removing a few nearby lymph nodes. A sentinel node biopsy looks for signs of cancer in the nearby lymph nodes. This procedure identifies the lymph nodes most likely to contain cancer. Those lymph nodes are removed and tested. If no cancer is found, it's unlikely that the cancer has spread. For vulvar cancer, the sentinel lymph nodes may be removed from one or more areas.
• Removing many lymph nodes. If the cancer has spread to the lymph nodes, many lymph nodes may be removed to reduce the risk that cancer will spread to other parts of the body.

Surgery carries a risk of complications. These may include infection and problems with healing around the incision. Removing lymph nodes can cause fluid retention and leg swelling, a condition called lymphedema.

Radiation therapy

Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body.

Radiation therapy is sometimes used to shrink large vulvar cancers before surgery. Sometimes radiation therapy is combined with chemotherapy. Using a low dose of chemotherapy medicine during radiation treatments makes the radiation more effective.

If cancer cells are found in your lymph nodes, radiation therapy may be used on the area around your lymph nodes. This treatment may kill any cancer cells that might remain after surgery. Radiation is sometimes combined with chemotherapy in these situations.

Chemotherapy

Chemotherapy treats cancer with strong medicines. Many chemotherapy medicines exist. Most chemotherapy medicines are given through a vein. Some come in pill form.

For those with vulvar cancer that has spread to other areas of the body, chemotherapy may be an option.

Chemotherapy is sometimes combined with radiation therapy to shrink large vulvar cancers before surgery. Chemotherapy also may be combined with radiation to treat cancer that has spread to the lymph nodes.

Targeted therapy

Targeted therapy for cancer is a treatment that uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die. For vulvar cancer, targeted therapy may be used for treating advanced vulvar cancer.

Immunotherapy

Immunotherapy for cancer is a treatment with medicine that helps the body's immune system kill cancer cells. The immune system fights off diseases by attacking germs and other cells that shouldn't be in the body. Cancer cells survive by hiding from the immune system. Immunotherapy helps the immune system cells find and kill the cancer cells. For vulvar cancer, immunotherapy may be used for treating advanced vulvar cancer.

Follow-up tests after treatment

After completing vulvar cancer treatment, your healthcare professional will recommend periodic follow-up exams to check if the cancer has come back. Even after successful treatment, vulvar cancer can return. Your healthcare professional will determine the schedule of follow-up exams that's right for you. Exams are generally recommended 2 to 4 times each year for the first two years after vulvar cancer treatment.

Coping and support

With time, you'll find what helps you cope with the uncertainty and worry of a vulvar cancer diagnosis. Until then, you may find it helps to:

Learn enough about vulvar cancer to make decisions about your care

Ask your healthcare team about your cancer, including your test results, treatment options and, if you like, your prognosis. As you learn more about vulvar cancer, you may become more confident in making treatment decisions.

Keep friends and family close

Keeping your close relationships strong can help you deal with vulvar cancer. Friends and family can provide the practical support you may need, such as helping take care of your home if you're in the hospital. And they can serve as emotional support when you feel overwhelmed by having cancer.

Find someone to talk with

Find someone who is willing to listen to you talk about your hopes and worries. This may be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group also may be helpful.

Ask your healthcare team about support groups in your area. In the United States, other sources of information include the National Cancer Institute and the American Cancer Society.

Don't be afraid of intimacy

A natural reaction to changes in your body may be to avoid intimacy. Although it may not be easy, talk about your feelings with your partner. You also may find it helpful to talk to a therapist, either on your own or together with your partner. Remember that you can express your sexuality in many ways. Touching, holding, hugging and caressing may become far more important to you and your partner.

Preparing for an appointment

Make an appointment with your usual doctor, gynecologist or other healthcare professional if you have any symptoms that worry you.

If you're found to have vulvar cancer, your health professional may refer you to a doctor who specializes in cancers of the female reproductive system. This doctor is called a gynecologic oncologist.

It's a good idea to prepare for your appointment. Here's some information to help you get ready.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down symptoms you have, including any that may not seem related to the reason for which you scheduled the appointment.
• Write down important personal information, including major stresses or recent life changes.
• Make a list of all medicines, vitamins or supplements you're taking and the doses.
• Take a family member or friend along. Sometimes it can be very hard to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

Your time with your healthcare team is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For vulvar cancer, some basic questions to ask include:

• What kinds of tests will I need?
• Do I need to do anything to prepare for these tests?
• Other than vulvar cancer, are there any other possible causes for these symptoms?
• What type of vulvar cancer do I have?
• What stage is my cancer?
• What types of surgical options are available to me?
• What kind of success rates does each type of surgery have?
• What are the drawbacks to each type of surgery?
• Will I need to wear an ostomy bag?
• What about radiation or chemotherapy? Are those options available to me?
• What kind of success rates do those therapies have?
• What types of side effects does each treatment have?
• How will these treatments affect my sexuality?
• Will I be able to have children after treatment?
• How should I prepare for treatment?
• Which course of action do you recommend?
• What are the odds of recurrence?
• What is my prognosis?

Don't hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer questions, such as:

• When did your symptoms begin?
• Have your symptoms been continuous or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?
• Have you ever been diagnosed with lichen sclerosus?
• Have you ever had a concerning Pap test result?
• Have you ever been diagnosed with HPV?