• What is Cancer of the Vulva?
• Detecting Cancer of Vulva
• Treatmant for Vulva
• General Questions

What is cancer of the vulva?

Cancer of the vulva, a rare kind of cancer in women accounting for 3 to 5% of all female genital cancers, is a disease in which cancer (malignant) cells are found in the vulva. The vulva is the outer part of a woman’s vagina. The vulva includes two prominent skin folds known as the labia majora, and two more delicate, barely visible, hairless skin folds called the labia minora. Cancer of the vulva (also known as vulvar cancer) is a malignancy that can occur on any part of the female external reproductive system but most often affects the inner edges of the labia majora or the labia minora. Less often, cancer occurs on the clitoris or in Bartholin's glands (small mucus-producing glands on either side of the vaginal opening).

Over 90% of cancers of the vulva are squamous cell carcinomas, which means they begin in squamous cells, the main cell type of the skin. This type of cancer usually forms slowly over many years, and is usually preceded by precancerous changes that may last for several years. The medical term most often used for this precancerous condition is vulvar intraepithelial neoplasia, or VIN. VIN is often divided into three categories - VIN1, VIN2, and VIN3, with the last indicating furthest progression toward a true cancer. Dysplasia is often used as another term for VIN. Using this terminology there is also a spectrum of increasing progress toward cancer: first mild dysplasia; next moderate dysplasia; then severe dysplasia; and finally, carcinoma in situ. Not all women with VIN or dysplasia will develop vulvar cancer.

The second most common type of vulvar cancer (about 4%) is melanoma. Melanomas develop from the pigment-producing cells that determine the skin’s color. About 5% to 8% of melanomas in women occur on the vulva, usually on the labia minora and clitoris.

Less than 2% of vulvar cancers are sarcomas, tumors of the connective tissues under the skin that tend to grow rapidly. Unlike other cancers of the vulva, vulvar sarcomas can occur at any age.

Detection of Cancer of the Vulva

The best method of detection for cancer of the vulva is by having pelvic examinations and being alert to any signs and symptoms of vulvar cancer. Early detection greatly improves the chances of successful treatment.

Signs and symptoms of vulvar intraepithelial neoplasia (VIN):

The most common symptom of VIN is persistent itching that does not improve. Areas of VIN usually have skin that is thicker and lighter in color than the surrounding skin. However, some cases of VIN can appear red, pink, or darker than the surrounding skin.

Signs and symptoms of invasive vulvar squamous cell cancer of the vulva:

The signs and symptoms of early invasive vulvar cancer is a distinct tumor. The most common symptoms are a red, pink, or white bump or bumps with a wart-like and/or raw surface. An area of the vulva may appear white and feel rough. About half of the women with vulvar cancer complain of persistent itching and a growth in the genitalia. Some also complain of pain, burning, painful urination, bleeding, and discharge not associated with the normal menstrual period. An ulcer that persists for more than a month is another sign.

Signs and symptoms of vulvar melanoma:

The appearance of a darkly pigmented lesion or a change in a mole that has been present for years may indicate melanoma.

Knowing what to look for can sometimes helps with early detection, but it is even better not to wait until you notice symptoms. Have a regular Pap test and pelvic examination. Although certain signs and symptoms may be highly suggestive of a vulvar cancer, many of them can be caused by benign conditions. The only way to be certain a vulvar cancer is present is to have a biopsy.

The chance of recovery (prognosis) and choice of treatment depend on the stage of the cancer (whether it is just in the vulva or has spread to other places) and the patient's general state of health.

Treatment of Vulvar Cancer

There are treatments for all patients with cancer of the vulva. Three kinds of treatment are used:

• Surgery (taking out the cancer in an operation)

• Radiation therapy (using high-dose x-rays or other high-energy rays to kill cancer cells)

• Chemotherapy (using drugs to kill cancer cells)

Surgery is the most common treatment of cancer of the vulva. A doctor may take out the cancer using one of the following operations:

• Wide local excision takes out the cancer and some of the normal tissue around the cancer.

• Radical local excision takes out the cancer and a larger portion of normal tissue around the cancer. Lymph nodes may also be removed.

• Laser surgery uses a narrow beam of light to remove cancer cells.

• Skinning vulvectomy takes out only the skin of the vulva that contains the cancer.

• Simple vulvectomy takes out the entire vulva, but no lymph nodes.

• Partial vulvectomy takes out less than the entire vulva.

• Radical vulvectomy takes out the entire vulva. The lymph nodes around it are usually removed as well.

Chemotherapy combined with irradiation is most often used in patients with extensive local spread of vulvar cancer. This combination of therapy has been very successful in minimizing the proportion of patients that require the ultraradical procedure termed "pelvic exenteration." Since treatment may inflove multimodality therapy, gynecologic oncologists are well suited to direct the care of patients with vulvar cancer.

The standard therapy for cancer localized to the vulva include radical surgery resection of the primary lesion and inguinal lymphadenectomy. Inadequate local surgical excision results in a high local recurrence rate. Radical excision with bilateral groin node dissection has been the recommended treatment for larger vulvar lesions. When vulvar cancers are diagnosed earlier, smaller localized, less traumatic surgeries with unilateral lymph node dissection can be performed.

The consequences of inadequate surgical therapy may be a recurrent, incurable lesion. However, the consequences of curative surgery can be psychologically devastating as vulvar surgery can result in lifelong anatomic alterations. Sexual dysfunction is common because of loss of clitoris in some clinical situations and in general because of alteration in body image. Lower extremity lymphedema (leg swelling) can occur causing difficulty walking, pain, recurrent infections and disfigurement. Therefore, all therapy includes pretreatment counseling about sexual and physical function.

If the cancer has spread outside the vulva and the other female organs, the doctor may take out the lower colon, rectum, or bladder (depending on where the cancer has spread) along with the cervix, uterus, and vagina (pelvic exenteration).

A patient may need to have skin from another part of the body added (grafted) and plastic surgery to make an artificial vulva or vagina after these operations.

Radiation therapy uses x-rays or other high-energy rays to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external radiation) or from putting materials that contain radiation through thin plastic tubes into the area where the cancer cells are found (internal radiation). Radiation may be used alone or before or after surgery.

Chemotherapy uses drugs to kill cancer cells. Drugs may be given by mouth, or they may be put into the body by a needle in the vein or muscle. Chemotherapy is called systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells throughout the body.

Treatment of cancer of the vulva depends on the stage of the disease, the type of disease, and the patient's age and overall condition.

Standard treatment may be considered because of its effectiveness in patients in past studies, or participation in a clinical trial may be considered. Not all patients are cured with standard therapy and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to find better ways to treat cancer patients and are based on the most up-to-date information. Clinical trials are ongoing in most parts of the country for stages III and IV of cancer of the vulva.

Treatment may be one of the following:

1.Wide local excision or laser surgery or a combination of both.

2.Skinning vulvectomy.

3.Ointment containing a chemotherapy drug.

Treatment may be one of the following:

1.Wide local excision.

2.Radical local excision plus taking out all nearby lymph nodes in the groin and upper part of the thigh on the same side as the cancer.

3.Radical vulvectomy and removal of the lymph nodes in the groin on one or both sides of the body.

4.Radiation therapy alone (in selected patients).

Treatment may be one of the following:

1.Radical vulvectomy and removal of the lymph nodes in the groin on both sides of the body. Radiation may be given to the pelvis following the operation if

cancer cells are found in the lymph nodes.

2.Radiation therapy alone (in selected patients).

Treatment may be one of the following:

1.Radical vulvectomy and removal of the lymph nodes in the groin and upper part of the thigh on both sides of the body. Radiation may be given to the pelvis

and groin following the operation if cancer cells are found in the lymph nodes or only to the vulva if the tumor is large but has not spread.

2.Radiation therapy and chemotherapy followed by radical vulvectomy and removal of lymph nodes on both sides of the body.

3.Radiation therapy (in selected patients) with or without chemotherapy.

Treatment may be one of the following:

1.Radical vulvectomy and removal of the lower colon, rectum, or bladder (depending on where the cancer has spread) along with the uterus, cervix, and vagina

(pelvic exenteration).

2.Radical vulvectomy followed by radiation therapy.

3.Radiation therapy followed by radical vulvectomy.

4.Radiation therapy (in selected patients) with or without chemotherapy, and possibly following surgery.

If the cancer has come back, treatment may be one of the following:

1.Wide local excision with or without radiation therapy.

2.Radical vulvectomy and removal of the lower colon, rectum, or bladder (depending on where the cancer has spread) along with the uterus, cervix, and vagina (pelvic exenteration).

3.Radiation therapy plus chemotherapy with or without surgery.

4.Radiation therapy for local recurrences or to reduce symptoms such as pain, nausea, or abnormal body functions.

5.Clinical trials of new forms of therapy.

General Questions

1. What is vulvar cancer?

   Cancer of the vulva, a rare kind of cancer in women, is a disease in which cancer (malignant) cells are found in the vulva. The vulva is the outer part of a woman’s vagina. The vagina is the passage between the uterus (the hollow, pear-shaped organ where a baby grows) and the outside of the body. It is also called the birth canal. Vulvar malignancies are rare and account 3 to 5% of all female genital cancers.

2. What are the causes and risk factors for vulvar cancer?

   It is difficult to discover what actually causes cancer from one person to another, but researchers have found several specific factors that increase a woman’s likelihood of developing vulvar cancer. Some risk factors for vulvar cancer include:

   Age: Of women who develop vulvar cancer, three-fourths are over 50, and two-thirds are over 70.The average age at diagnosis is 65 years old; however, vulvar carcinoma is becoming more common in women under 40 years old.

   Human papillomas (HPV infection): Human papillomavirus infection is thought to be responsible for about 30% to 50% of vulvar cancers. HPVs are a group of more than 70 types of viruses called papilloma viruses because they can cause papillomas (warts). This abnormal cell growth increases the risk of squamous cell cancer.
   

   Tobacco use: Smoking exposes the body to many cancer-causing chemicals that affect more than the lungs. These harmful substances can be absorbed into the lining of the lungs and spread throughout the body. Among women who have a history of genital warts, smoking further increases the risk of developing vulvar cancer.
   

   Vulvar intraepithelial neoplasia (VIN): Women with VIN have an increased risk of progression to invasive vulvar cancer. Although most cases of VIN never progress to cancer, it is not possible to tell which will, so treatment and/or close medical follow-up is needed.

   Immunosuppression is also a risk factor for vulvar cancer.

3. What are the symptoms for vulvar cancer?

A doctor should be seen if a woman observes any of the following:

• vulvar mass or lump

• pruritus (itching)

• pain

• burning

• bleeding

• dysuria

• discharge
  
  How will my doctor know if I have vulvar cancer?

If there are symptoms, a doctor may do certain tests to see if there is cancer, usually beginning by looking at the vulva and feeling for any lumps. The doctor may then go on to cut out a small piece of tissue (called a biopsy) from the vulva and look at it under a microscope. A patient will be given some medicine to numb the area when the biopsy is done. Some pressure may be felt, but usually with no pain. This test is often done in a doctor's office.

What about treatment, what should I ask?

There are treatments for all patients with cancer of the vulva. Three kinds of treatment are used:

• surgery (taking out the cancer in an operation)

• radiation therapy (using high-dose x-rays or other high-energy rays to kill cancer cells)

• chemotherapy (using drugs to kill cancer cells)

A doctor may use just one method or combine methods to treat the cancer most effectively; however, surgery is the most common treatment of cancer of the vulva.

The standard therapy for cancer localized to the vulva include radical surgery resection of the primary lesion and inguinal lymphadenectomy. Inadequate local surgical excision results in a high local recurrence rate. Radical excision with bilateral groin node dissection has been the recommended treatment for larger vulvar lesions. When vulvar cancers are diagnosed earlier, smaller localized, less traumatic surgeries with unilateral lymph node dissection can be performed.

Here are some questions a woman may want to ask her doctor before treatment begins:

• What is my diagnosis?

• What is the stage of the disease?

• What are my treatment choices? Which do you recommend for me? Why?

• What are the chances that the treatment will be successful?

• Would a clinical trial be appropriate for me?

• What are the risks and possible side effects of each treatment?

• How long will my treatment last?

• Will I have to change my normal activities?

• What is the treatment likely to cost?

6. What are the side effects of treatment?

   It is hard to limit the effects of therapy so that only cancer cells are destroyed. Because treatment often damages healthy cells and tissues, it can cause unpleasant side effects.

   The side effects of cancer treatment vary, depending on the type of treatment. Also, each woman reacts differently. Doctors try to keep side effects to a minimum, but problems may occur.

   The consequences of curative surgery can be psychologically devastating, as vulvar surgery can result in lifelong anatomic alterations. Sexual dysfunction is common because of loss of clitoris in some clinical situations and in general because of alteration in body image. Lower extremity lymphedema (leg swelling) can occur causing difficulty walking, pain, recurrent infections and disfigurement. Therefore, all therapy includes pretreatment counseling about sexual and physical function.

   If the cancer has spread outside the vulva and the other female organs, the doctor may take out the lower colon, rectum, or bladder (depending on where the cancer has spread) along with the cervix, uterus, and vagina (pelvic exenteration). Physicians have developed ways for patients to store and eliminate wastes after these procedures, and it is sometimes possible to reconstruct or reattach these organs so no external appliances are needed. A patient may need to have skin from another part of the body added (grafted) and plastic surgery to make an artificial vulva or vagina after these operations.

   Radiation therapy is delivered by exposing cancer cells to high-energy rays or particles to destroy them. The most common method of radiation therapy is known as external beam radiation or teletherapy. With this method, a beam from a machine outside the body is focused in the area of the cancer. Treatment usually involves receiving teletherapy for five days a week for about six weeks. The side effects for this method of radiation therapy include a skin reaction like a sunburn on the outside of the skin, fatigue, nausea, and diarrhea. When delivered to the pelvis, premature menopause and problems with urination may also occur.

   Chemotherapy is the use of anti-cancer drugs to fight the disease. Chemotherapy is a systemic therapy, which means the anti-cancer drugs travel through the bloodstream, reaching all parts of the body. In general, chemotherapy drugs affect rapidly dividing cells. The drugs kill cancer cells, but also affect other cells in the body, like cells in hair roots and cells that line the digestive tract. As a result, chemotherapy can cause hair loss, nausea, vomiting, or mouth sores. Doctors can suggest diet changes or medication to ease these problems, and most side effects of chemotherapy gradually disappear during the recovery period or after treatment stops.

    

7. Will I be able to adjust to this disease well?

   Each cancer survivor’s recovery is different, and a person’s adjustment after cancer treatment depends on a number of factors. Vular cancer can cause major life changes in its survivors. Treatments often affect a woman’s ability to have intercourse and to have children. It is important for women to seek support during and after cancer treatment. In fact, behavioral scientists have found that women who took advantage of a social support system, such as a cancer support group, survived with a better quality of life. Maintain an open dialogue with your cancer care team to address any concerns you have.

    

8. Are there clinical trials available for vulvar cancer?

Yes. For some vulvar cancer patients, treatment may involve a clinical trial. Clinical trials are studies conducted with the consent of patients to evaluate a new treatment. Speak with your doctor.