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Vulvar Cancer

Definition


Disease: Vulvar Cancer Vulvar Cancer
Category: Tumors

Disease Definition:

Cancer of the outer surface area of the female genitalia is called vulvar cancer, which is an uncommon one. Squamous cell carcinoma is the type of skin cancer that develops slowly over the years. Most of the vulvar cancers are squamous cell carcinomas, but a small number of vulvar cancers begin as melanoma and in some rare cases, vulvar cancer develops in the mucus-producing glands located on the sides of the vaginal opening. The development of this type of vulvar cancer is thought to be somehow influenced by the human papillomavirus (HPV), which is a sexually transmitted infection.

 

Early detection of vulvar cancer means a better chance of successful treatment, which is why getting regular gynecologic exams play an important role. A woman could also control other risk factors and prevent vulvar cancer, such as by engaging in safe sexual practices.
 

Work Group:


Symptoms, Causes

Symptoms:

A doctor should be seen in the case of experiencing any of the below mentioned vulvar signs and symptoms:

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

 

In the case of experiencing irregular bleeding, persistent itching, burning, pain or tenderness in the genital area, or if a woman notices skin changes or a lump or open sore on her vulvar area, she should contact either her primary care doctor or gynecologist, for early diagnosis of vulvar cancer increases the likelihood of successful treatment.

 

To try and avoid recurrence of the disease, a woman who has been treated for vulvar cancer should make sure to see her doctor for regular follow-up exams.
 

Causes:

Vulvar cancer’s actual cause is unknown. A mutation or defect in the p53 tumor suppressor gene may be the cause of vulvar cancers that occur in older women that aren’t related to HPV infections. This gene plays a role in preventing cells from becoming cancerous. Lichen sclerosus is a condition that causes the vulvar skin to become thin and itchy; vulvar cancer may also occur in women with this condition.
The sexually transmitted HPV infection has been associated with about 30 % to 50 % of vulvar cancers. Before developing cancer, these women usually have a precancerous skin condition called vulvar intraepithelial neoplasia in more than one area of the vulva. Vulvar cancer is not a common cancer type.
 

There are certain factors that seem to increase the risk of vulvar cancer, despite the fact that the exact cause is still not known. Some of those factors are:

 

Age:

When first diagnosed with vulvar cancer, about half of the women are older than 70. However, about 15% of new cases occur in women younger than 40, meaning that this cancer isn’t limited to older women.

 

HPV infection:

Most younger women who have vulvar cancer have this sexually transmitted disease. The risk of vulvar cancer increases with having HPV or having unsafe sex practices that put the woman at a higher risk of HPV infection.

 

Smoking:

Women with a history of genital warts or HPV have an even further increased risk of vulvar cancer in case they smoke because smoking exposes a person to chemicals that might increase the risk of vulvar cancer.

 

Human immunodeficiency virus (HIV):

This virus increases the risk of vulvar cancer because it makes a person more susceptible to HPV infections by weakening the immune system.

 

Vulvar intraepithelial neoplasia:

Even though most cases of this precancerous condition won’t turn into vulvar cancer, the condition does increase the person’s risk of vulvar cancer and should be monitored by the doctor. This precancerous condition increases the risk of vulvar cancer and should be monitored by a doctor, despite the fact that most cases don’t turn into vulvar cancer.

 

Lichen sclerosus:

This condition causes the vulva to become itchy and thin. Vulvar cancer develops later in life in about 4% of women with this condition.

 

A history of melanoma or suspicious moles:

A person will be at an increased risk of a vulvar melanoma in the case of having a personal or family history of this serious type of skin cancer anywhere on the body, or in the case of having a family or personal history of unusual moles.
 

Complications

Complications:

None

Treatments:

Surgical removal of the tumor, radiation therapy, chemotherapy or a combination of these is included in the treatment options for vulvar cancer, depending on the kind and stage of the cancer. The patient should discuss with the doctor all of the treatment options and the pros and cons of each treatment. Before starting treatment, the patient could also get a second opinion.

 

SURGERY:

More tissue may need to be surgically removed in case the vulvar cancer is advanced. Some of the types of surgery that could be performed include:

 

Laser surgery:

Laser surgery could be an option in case the cancer is in its early noninvasive stages. To kill the cancer cells, the doctor aims a laser beam at the layer of the vulva containing the cancer.

 

Excision:

The cancer is cut out in this procedure, in addition to about 1.3 centimeters (a half-inch) of normal tissue all the way around it. This margin of normal-looking tissue is cut out to make sure that all of the cancerous cells have been removed. This procedure could also be called radical excision or a wide local excision.

 

Vulvectomy:

Vulvectomy has several types. Only the top layer of the skin where the cancer is located in the method called skinning vulvectomy. The doctor may cover this area by grafting skin from another part of the body. The entire vulva is removed in the method called simple vulvectomy. People who have noninvasive vulvar cancer can undergo these two types of vulvectomies. On the other hand, the doctor may remove the entire vulva, clitoris and nearby tissue in a method called complete radical vulvectomy, or he/she may perform partial vulvectomy by removing the cancer and the deep surrounding tissue.

 

Problems with healing, infection and the ability of the skin grafts to take may be some of the complications that occur with removing large areas of skin and tissue in the vulva. In case the removed tissue is greater, the risk of these complications increases.
Plus, women who’ve undergone vulvectomy might have difficulties achieving orgasm. This problem may sometimes be temporary. Sexual intercourse may become uncomfortable or even painful in case scar tissue narrows the vaginal opening. Devices named vaginal dilators might help stretching the opening, or the surgeon might recommend skin grafts to widen the vaginal opening. In this case, the patient may either be suggested skin grafts to widen the vaginal opening, or the use of devices called vaginal dilators to help stretch the opening.

 

Pelvic exenteration:

The doctor may remove any or all of the below mentioned organs in case the spread of the cancer is extensive:

  • Cervix
  • Uterus
  • Bladder
  • Lower colon
  • Ovaries and nearby lymph nodes
  • Rectum
  • Vagina

 

The doctor will create an artificial opening in the body (stoma) for the woman’s waste to be removed in a bag (ostomy) in case the bladder, rectum or colon is removed.

 

Lymph node removal:

The lymph nodes in the groin may be removed because vulvar cancer usually spreads there.
The doctor might also tie off the saphenous vein, which is a major vein. To avoid the swelling of the leg that may occur with this procedure, some doctors will try to avoid closing this vein. For many days after the procedure, the patient will need a suction drain in the incision.

 

Problems with fluid retention, leg swelling and a high risk of lymphedema, which is the infection of the lymph vessels also called lymphangitis may result from removing the lymph nodes. In this case, to help reduce the symptoms, the patient may be given support stockings or compression devices. In addition to this, because of the increased risk of infection, the patient should avoid sunburns, scratches and other injury to the legs.

 

Sentinel lymph node biopsy:

Some of the side effects of lymph node removal may be avoided with this procedure. A sentinel node is the closest to the area of the tumor draining fluid from the cancerous area. On the day before the procedure, a blue dye or a radioactive tracer is injected into the tumor area. Then, to see where the tumor drains, the doctor scans the area to know which side he/she will focus on during the next day’s surgery. Blue dye or radioactive tracer is once again injected on the day of surgery to make the finding and removal of the sentinel node easier. No additional surgery will be required if no cancer cells are found in the sentinel node. But additional lymph nodes on that side of the groin will need to be removed in case cancerous cells are found. Sentinel node biopsy won’t be performed in case the initial testing reveals an already enlarged lymph node. In this case, the swollen lymph node is removed and biopsied. This type of treatment isn’t widely available yet because it is still an experimental one.

 

The development of blood clots, lymphoceles (cysts) near the wounds, painful urination, loss of sexual desire or pleasure and urinary infections are some of the other complications that may result from vulvar cancer surgery. 

 

RADIATION THERAPY:

Only the lymph nodes in the groin and pelvis are treated with radiation given from outside the body (external beam radiation), and not the vulva itself. The doctor may sometimes choose this method to try and shrink a large tumor, so that it can be removed with less extensive surgery. For six to twelve months, the area of the skin that is treated may look and feel sunburned. The patient may also have problems with urination and premature menopause in case radiation is used on the pelvic area.

 

CHEMOTHERAPY:

To kill cancer cells, chemotherapy uses drugs, usually a combination of drugs, which are given topically (through the skin), orally or intravenously. Before surgery, chemotherapy could be used to shrink a tumor, just like radiation therapy. However, because vulvar cancers that have spread are usually resistant to chemotherapy and because surgery is usually more effective, chemotherapy isn’t used on its own.

 

Hair loss, nausea, fatigue and vomiting are some of the side effects of chemotherapy. These side effects occur because chemotherapy affects fast-growing cells, whether healthy or cancerous ones, particularly those in the digestive tract, bone marrow and hair. But nowadays, there are better ways to control some of the side effects, and not everyone seems to experience these side effects.

 

RECONSTRUCTIVE SURGERY:

Removal of some skin from the vulva is usually included in the treatment of vulvar cancer. Usually without grafting skin from another area of the body, the wound or area that is left behind can be closed. But the doctor may perform reconstructive surgery, grafting skin from another part of the body to cover that area, depending on how much tissue should be removed and how widespread the cancer is.

 

VULVAR INTRAEPITHELIAL NEOPLASIA: 

Before the cells have a chance to turn into cancer, the tissue that contains these precancerous changes is removed. But it seems that the size of these lesions may be reduced with the use of imiquimod, which is an immune system modulating medication. This could prove to be an additional treatment option.

 

FOLLOW-UP CARE: 

After finishing treatment, it is very important to keep seeing the gynecologist at least twice a year because as many as one in ten women experience recurrence.
 

Prognosis:

Not available

Expert's opinion

Expert's Name:
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Expert's opinion:

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Clinical Trials:

Not available

 

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Consultants Corner

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