Vulval Cancer

Vulval cancer is a rare cancer that begins in the external female sex organs, most commonly appearing as a lump or sore that causes itching. While it can occur at any age, it is usually diagnosed in older adults, with most cases found in women over 50.

Table of contents

• What is vulval cancer
• Anatomy of the vulva
• Types of vulval cancer
• How common is vulval cancer
• Signs and symptoms
• Causes and risk factors
• Diagnosis
• Treatment options
• Coping with vulval cancer

What is vulval cancer

Vulval cancer is a disease where cells in the vulva grow out of control^[1]. The vulva is the area of skin that surrounds the opening of the vagina and the tube where urine leaves the body, called the urethra^[1]. This type of cancer is rare, accounting for about 7% of all gynecologic cancers diagnosed in the United States^[3].

Vulval cancer commonly forms as a lump or sore on the vulva that often causes itching^[1]. Though it can happen at any age, vulval cancer is usually diagnosed in older adults^[1]. Healthcare providers diagnose just under 6,500 new cases of vulval cancer in the United States each year^[2].

Vulval cancer usually forms slowly over several years^[2]. Before cancer develops, changes to cells of the vulva can cause a condition that is not yet cancer but may become cancer if not treated. The most common condition of this type is called vulvar intraepithelial neoplasia (VIN)^[7]. Healthcare providers usually discover abnormal growth in the outermost layer of skin during this stage^[2].

Anatomy of the vulva

The vulva is the collective name for all the female external sex organs^[2]. Your vulva includes several parts:

• Opening of your vagina, which is the muscular canal for sex, childbirth and periods^[2]
• Labia minora (inner lips), which are tissue folds that surround your vaginal opening and extend above your clitoris^[2]
• Labia majora (outer lips), which are the fleshy area that surrounds your inner lips^[2]
• The outer part of your clitoris, which is the sexually sensitive nub of flesh above your vaginal opening^[2]
• Mons pubis, which is the rounded area in front of your pubic bones that becomes covered with hair at puberty^[2]
• Opening of the urethra, which is the tube that allows urine to exit your body^[2]
• Perineum, which is the patch of skin between your vagina and anus^[2]

About 50% of vulval cancers occur in the labia majora, the most common site. The labia minora are the site of 15% to 20% of vulval cancer cases. The clitoris and glands are less frequently involved^[15].

Types of vulval cancer

Healthcare providers classify vulval cancer based on the type of cells where the cancer starts^[2]. More than 90% of invasive vulval cancers are squamous cell carcinomas^[15]. The most common types of vulval cancer include:

Vulvar squamous cell carcinoma is the most common type. Approximately 90% of vulval cancers are squamous cell carcinomas. They develop in the cells on the surface of your skin^[2].

Vulvar melanoma is the second most common type. Approximately 5% of vulval cancers are melanomas. Melanomas develop rapidly and have a high risk of spreading to other areas of your body^[2].

The remaining vulval cancers are rare. They include basal cell carcinoma, Bartholin gland adenocarcinoma, Paget disease of the vulva, sarcoma, and verrucous carcinoma^[2].

How common is vulval cancer

Vulval cancer is rare. Healthcare providers diagnose just under 6,500 new cases of vulval cancer in the United States each year^[2]. According to recent statistics, there are approximately 7,480 new cases and 1,770 deaths from vulval cancer in the United States annually^[10].

Nearly 80% of people diagnosed are over age 50, and over half of all diagnoses are in people over age 70. The average age at diagnosis is 68^[2]. Vulval cancer is most commonly diagnosed at ages 65 to 74, with the median age at diagnosis of 69 years^[8].

Vulval cancer accounts for 0.3% of all new cancer cases annually in the United States^[8]. Together with vaginal cancer, these cancers account for about 7% of all gynecologic cancers diagnosed in the United States^[3].

Signs and symptoms

Vulval cancer often does not cause early signs or symptoms^[10]. The first noticeable signs of vulval cancer are usually skin changes on your outer lips (labia majora) or inner lips (labia minora). But cancer can form anywhere on your vulva^[2].

Signs and symptoms of vulval cancer may include^[1]:

• 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

Additional symptoms may include color changes, including skin that looks darker or lighter than usual, or patches of white skin. Thickened or rough skin patches may appear. Growths, including lumps, wart-like bumps or ulcers that don’t heal, are common signs. Itching or burning that doesn’t improve may occur. Bleeding that’s unrelated to menstruation can happen. Tenderness and pain, potentially during sex or when you’re urinating, may be present^[2].

See your healthcare provider if you have one or more of these symptoms. Vulval cancer symptoms usually don’t appear in the early stages, so it’s important to get checked as soon as possible^[2]. Still, many of these symptoms are also common in noncancerous conditions. Your provider can tell you whether these changes are signs of vulval cancer or a different condition^[2].

Causes and risk factors

With vulval cancer, cells begin multiplying out of control. Without treatment, these cancer cells can spread to other parts of your body^[2].

Anything that increases a person’s chance of getting a disease is called a risk factor. Not every person with one or more of these risk factors will develop vulval cancer, and it will develop in some people who don’t have any known risk factors^[10].

Risk factors for the development of vulval cancer include^[8]:

Increasing age is the most important risk factor for most cancers^[15]. Nearly 80% of people diagnosed are over age 50, and over half of all diagnoses are in people over age 70^[2].

Infection with human papillomavirus (HPV) is a major risk factor. In many cases, the development of vulval cancer is preceded by other conditions or abnormal cell growth. The prevailing evidence favors HPV infection as a causative factor in many genital tract cancers^[15]. About 30 to 40% of vulval cancer cases are associated with high-risk human papillomavirus^[8].

Smoking is another risk factor for vulval cancer^[8].

Inflammatory conditions of the vulva can increase risk. A skin condition called lichen sclerosus is associated with vulval cancer^[2].

Prior pelvic radiation increases the risk of developing vulval cancer^[8].

Immunodeficiency, when the body’s immune system is weakened, is a risk factor^[8].

Having vulvar intraepithelial neoplasia (VIN) can increase the risk of vulval cancer. Because it is possible for VIN to become vulval cancer, it is important to get treatment^[10].

Other possible risk factors include having a history of genital warts, having many sexual partners, having first sexual intercourse at a young age, and having a history of abnormal Pap tests^[10].

Diagnosis

Vulval cancer diagnosis often begins with a physical exam and a discussion of your health history^[11]. Tests that examine the vulva are used to diagnose vulval cancer^[10].

In addition to asking about your personal and family health history and doing a physical exam, your doctor may perform the following tests and procedures^[10]:

Pelvic exam: An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A special instrument called a speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. The doctor or nurse also inserts one or two gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the organs^[10].

Colposcopy: Your healthcare professional may use a special magnifying instrument to look closely at the vulva. This instrument is called a colposcope. It may also be used to look at the vagina and cervix^[11].

Biopsy: A biopsy is a procedure to remove a sample of tissue for testing in a lab. For vulval cancer, a biopsy involves removing a sample of skin. A vulval 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^[11].

If you’re diagnosed with vulval 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^[11]. Staging tests may include examination of your pelvic area for cancer spread, and 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^[11].

Treatment options

Vulval cancer is often treatable. The treatment you have will depend on the size and type of vulval cancer you have, where the cancer is, if it has spread, and your general health^[12]. The main treatment for vulval cancer is usually surgery. You may also have radiotherapy or chemotherapy^[12].

Vulval cancer treatment often starts with surgery to remove the cancer and a small amount of surrounding healthy tissue. Sometimes vulval cancer surgery requires removing the entire vulva. The earlier vulval cancer is diagnosed, the less likely an extensive surgery is needed for treatment^[1].

Surgery

Surgery is usually the main treatment for vulval cancer, especially if the cancer is found early^[12]. Most people with vulval cancer need surgery. Your surgeon removes the cancer and a margin of healthy tissue around it. This is a wide local excision. For larger cancers, your surgeon may need to remove a larger part of your vulva. This is a partial vulvectomy. More rarely, you might need to have your whole vulva removed. This is a radical vulvectomy^[17].

Surgery may involve removing the cancer and an area of tissue around it, part of your vulva, or the whole of your vulva, including the labia and sometimes the clitoris^[12].

You may also need to have some lymph nodes in your groin removed. These are part of the body’s drainage system^[12]. Vulval cancer can spread to the lymph nodes in the groin. So your surgeon may recommend checking the lymph nodes for cancer cells^[17].

After removing the cancer, the surgeon will repair your vulva using the remaining skin. In some cases a specialist doctor (plastic surgeon) will need to reconstruct your vulva. They’ll do this by using skin or muscle from other parts of your body^[12].

Radiotherapy

Radiotherapy uses high-energy rays of radiation to kill cancer cells^[12]. You may have radiotherapy for vulval cancer before surgery, to shrink the cancer; after surgery, to help stop the cancer coming back; if you cannot have surgery; or if the cancer has spread to other parts of your body^[12].

You may have radiotherapy after surgery to try to stop the cancer from coming back. This is adjuvant radiotherapy. You usually have adjuvant radiotherapy if one or more lymph nodes from the groin contain cancer cells, the cancer has broken through the capsule that surrounds the lymph node, you have cancer cells in the sentinel lymph node, or the cancer is too close to the border of healthy tissue removed during surgery^[17].

Chemotherapy

Chemotherapy uses medicines to kill cancer cells^[12]. You may have chemotherapy for vulval cancer before surgery, to shrink the cancer; after surgery, to help stop the cancer coming back; if you cannot have surgery; or to treat your symptoms if the cancer has spread^[12].

You usually have chemotherapy in combination with radiotherapy. This is called chemoradiotherapy^[17]. You might have chemoradiotherapy if you can’t have surgery for any reason^[17].

Most often, your doctor will recommend that you start treatment within a few weeks of your diagnosis. If surgery is all that is required, treatment and recovery should be completed within six to eight weeks. If radiation or chemotherapy is required, this process may take longer^[13].

Coping with vulval cancer

Vulval cancer can be a very difficult cancer to cope with emotionally and physically. The vulva is one of the most private parts of a woman’s body so it’s normal to feel anxious and upset when you find out you have vulval cancer^[18].

Everyone reacts in their own way. Sometimes it’s hard to take in the fact that you have cancer at all. You may feel a range of powerful emotions at first such as feeling shocked, upset and find it difficult to take in anything else that is being said to you. Other emotions include feeling numb, frightened and uncertain, confused, angry and resentful, guilty, or sad^[18].

You may feel that you don’t want to know much information straight away. Tell your doctor or nurse. You will always be able to ask for more information when you feel ready^[18].

Talking to your friends and relatives about your cancer can help and support you. Some of those close to you might be scared of the emotions this could bring up and could be reluctant to talk. Help your family and friends by letting them know if you would like to talk about what’s happening^[18].

Adjusting to life after treatment is different for everyone. Your recovery will depend on many different factors, such as where the cancer was in your body, the stage of the cancer, the organs and tissues removed during surgery, the type of treatment and side effects^[22].

Vulval cancer and its treatments can affect your self-esteem and body image. Often this is because cancer or cancer treatments change your body in different ways, such as scars, hair loss, skin problems, change in the shape or appearance of the vulva or a complete removal of the vulva, urinary incontinence, or sexual problems. Some of these changes can be temporary. Others will last for a long time or be permanent^[22].

Many people continue to have strong, supportive relationships and a satisfying sex life after vulval cancer. If sexual problems occur because of vulval cancer treatment, there are ways to manage them^[22].