Vulval Cancer – Symptoms, Causes, Stages & Treatment

Vulval cancer

Vulval cancer is a type of cancer that occurs on the outer surface area of the female genitalia. The vulva is the area of skin that surrounds the urethra and vagina, including the clitoris and labia.

Vulval cancer commonly forms as a lump or sore on the vulva that often causes itching. Though it can occur at any age, vulval cancer is most commonly diagnosed in older women.

Vulval cancer treatment usually involves 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.

Symptoms

Signs and symptoms of vulval cancer may include:

  • Itching that doesn’t go away
  • Pain and tenderness
  • Bleeding that isn’t from menstruation
  • Skin changes, such as color changes or thickening
  • A lump, wart-like bumps or an open sore (ulcer)

When to see a doctor

Make an appointment with your primary care doctor or gynecologist if you experience any vulvar signs or symptoms that worry you, such as:

  • Abnormal bleeding
  • Burning
  • Itching
  • Pain

Causes

It’s not clear what causes vulval cancer.

In general, doctors know that cancer begins when a cell develops mutations in its DNA. The mutations allow the cell to grow and divide rapidly. The cell and its offspring go on living when other normal cells would die. The accumulating cells form a tumor that may be cancerous, invading nearby tissue and spreading to other parts of the body.

Types of vulval cancer

The type of cell in which vulvar cancer begins helps your doctor plan the most effective treatment. The most common types of vulval cancer include:

  • Vulval squamous cell carcinoma. This cancer begins in the thin, flat cells that line the surface of the vulva. Most vulval cancers are squamous cell carcinomas.
  • Vulval melanoma. This cancer begins in the pigment-producing cells found in the skin of the vulva.

Risk factors

Although the exact cause of vulval cancer isn’t known, certain factors appear to increase your risk of the disease, including:

  • Increasing age. The risk of vulval cancer increases with age, though it can occur at any age. The average age at diagnosis is 65.
  • Being exposed to human papillomavirus (HPV). HPV is a sexually transmitted infection that increases the risk of several cancers, including vulval cancer and cervical cancer. Many young, sexually active women are exposed to HPV, but 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. Smoking cigarettes increases the risk of vulvar cancer.
  • Being infected with the human immunodeficiency virus (HIV). This sexually transmitted virus weakens the immune system, which may make you more susceptible to HPV infections, thereby increasing your risk of vulval cancer.
  • Having a history of precancerous conditions of the vulva.Vulval intraepithelial neoplasia is a precancerous condition that increases the risk of vulval cancer. Most women with vulval intraepithelial neoplasia will never develop cancer, but a small number do go on to develop invasive vulval cancer. For this reason, your doctor may recommend treatment to remove the area of abnormal cells and periodic follow-up checks.
  • Having a skin condition involving the vulva. Lichen sclerosus, which causes the vulvar skin to become thin and itchy, increases the risk of vulval cancer.

Tests and diagnosis

Diagnosing vulval cancer

Tests and procedures used to diagnose vulval cancer include:

  • Examining your vulva.The doctor will likely conduct a physical exam of your vulva to look for abnormalities.
  • Using a special magnifying device to examine your vulva.During a colposcopy exam, your doctor uses a device that works like a magnifying glass to closely inspect your vulva for abnormal areas.
  • Removing a sample of tissue for testing (biopsy). To determine whether an area of suspicious skin on your vulva is cancer, your doctor may recommend removing a sample of skin for testing. During a biopsy procedure, the area is numbed with a local anesthetic and a scalpel or other special cutting tool is used to remove all or part of the suspicious area. Depending on how much skin is removed, you may need stitches.

Determining the extent of the cancer

Once your diagnosis is confirmed, your doctor works to determine the size and extent (stage) of your cancer. Staging tests can include:

  • Examination of your pelvic area for cancer spread. Your doctor may do a more thorough examination of your pelvis for signs that the cancer has spread.
  • Imaging tests. Images of your chest or abdomen may show whether the cancer has spread to those areas. Imaging tests may include X-ray, computerized tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET).

Vulval cancer stages

  • Stage I describes a small tumor that is confined to the vulva or the area of skin between your vaginal opening and anus (perineum). This cancer hasn’t spread to your lymph nodes or other areas of your body.
  • Stage II tumors are those that have grown to include nearby structures, such as the lower portions of the urethra, vagina and anus.
  • Stage III cancer has spread to lymph nodes.
  • Stage IVA signifies a cancer that has spread more extensively to the lymph nodes, or that has spread to the upper portions of the urethra or vagina, or that has spread to the bladder, rectum or pelvic bone.
  • Stage IVB is a cancer that has spread (metastasized) to distant parts of your body

Treatments and drugs

Treatment options for vulvar cancer depend on the type and stage of your cancer, your overall health and your preferences.

Surgery to remove vulval cancer

  • Removing the cancer and a margin of healthy tissue (excision). This procedure, which may also be called a wide local excision or radical excision, involves cutting out the cancer and a small amount of normal tissue that surrounds it. Cutting out what doctors refer to as a margin of normal-looking tissue helps ensure that all of the cancerous cells have been removed.
  • Removing a portion of the vulva (partial vulvectomy).During a partial vulvectomy, a portion of the vulva is removed, along with its underlying tissues.
  • Removing the entire vulva (radical vulvectomy). Radical vulvectomy involves removal of the entire vulva, including the clitoris and underlying tissues.
  • Extensive surgery for advanced cancer. If cancer has spread beyond the vulva and involves nearby organs, your doctor may recommend removing all of the vulva and the involved organs in a procedure called pelvic exenteration.

Depending on where your cancer has spread, your surgeon may remove the lower colon, rectum, bladder, cervix, uterus, vagina, ovaries and nearby lymph nodes. If your bladder, rectum or colon is removed, your doctor will create an artificial opening in your body (stoma) for your waste to be removed in a bag (ostomy).

Reconstructive surgery. Treatment of vulval cancer often involves removal of some skin from your vulva. The wound or area left behind can usually be closed without grafting skin from another area of your body. However, depending on how widespread the cancer is and how much tissue your doctor needs to remove, your doctor may perform reconstructive surgery — grafting skin from another part of your body to cover this area.

Surgery to remove the entire vulva carries a risk of complications, such as infection and problems with healing around the incision. In addition, with part or all of the vulval padding gone, it can be uncomfortable to sit for long periods. Your genital area may feel numb, and it may not be possible to achieve orgasm during sexual intercourse.

Surgery to remove nearby lymph nodes

Vulval cancer often spreads to the lymph nodes in the groin, so your doctor may remove these lymph nodes at the time you undergo surgery to remove the cancer. Depending on your situation, your doctor may remove only a few lymph nodes or many lymph nodes.

Removing lymph nodes can cause fluid retention and leg swelling, a condition called lymphedema.

In certain situations, surgeons may use a technique that allows them to remove fewer lymph nodes. Called sentinel lymph node biopsy, this procedure involves identifying the lymph node where the cancer is most likely to spread first. The surgeon then removes that lymph node for testing. If cancer cells aren’t found in that lymph node, then it’s unlikely that cancer cells have spread to other lymph nodes.

Radiation therapy

Radiation therapy uses high-powered energy beams, such as X-rays, to kill cancer cells. Radiation therapy for vulval cancer is usually administered by a machine that moves around your body and directs radiation to precise points on your skin (external beam radiation).

Radiation therapy is sometimes used to shrink large vulval cancers in order to make it more likely that surgery will be successful. Radiation is sometimes combined with chemotherapy, which can make cancer cells more vulnerable to radiation therapy.

If cancer cells are discovered in your lymph nodes, your doctor may recommend radiation to the area around your lymph nodes to kill any cancer cells that might remain after surgery.

Chemotherapy

Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. Chemotherapy drugs are typically administered through a vein in your arm or by mouth.

For women with advanced vulval cancer that has spread to other areas of the body, chemotherapy may be an option. Sometimes chemotherapy is combined with radiation therapy to shrink large vulval cancers in order to make it more likely that surgery will be successful.

Follow-up tests after treatment

After completing vulval cancer treatment, your doctor may recommend periodic follow-up exams to look for a cancer recurrence. Even after successful treatment, vulvar cancer can return. Your doctor will determine the schedule of follow-up exams that’s right for you, but doctors generally recommend exams two to four times each year for the first two years after vulvar cancer treatment.

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