Vulvar cancer is a rare disease in which malignant (cancer) cells form in the tissues of the vulva.
Vulvar cancer forms in a woman’s external genitalia. The vulva includes the inner and outer lips of the vagina, the clitoris (sensitive tissue between the lips), and the opening of the vagina and its glands.

Vulvar cancer most often affects the outer vaginal lips. Less often, cancer affects the inner vaginal lips or the clitoris.

Vulvar cancer usually develops slowly over a period of years. Abnormal cells can grow on the surface of the vulvar skin for a long time. This precancerous condition is called vulvar intraepithelial neoplasia (VIN) or dysplasia. Because it is possible for VIN or dysplasia to develop into vulvar cancer, treatment of this condition is very important.

HPV infection and older age can affect the risk of developing vulvar cancer.

Risk factors include the following:

—      Having human papillomavirus (HPV) infection.

—      Older age.

Possible signs of vulvar cancer include bleeding or itching.

Vulvar cancer often does not cause early symptoms. When symptoms occur, they may be caused by vulvar cancer or by other conditions. A doctor should be consulted if any of the following problems occur:

—      A lump in the vulva.

—      Itching that does not go away in the vulvar area.

—      Bleeding not related to menstruation (periods).

—      Tenderness in the vulvar area.

Tests that examine the vulva are used to detect (find) and diagnose vulvar cancer.

The following tests and procedures may be used:

Physical exam and history: An exam of the body to check general signs of health, including checking the vulva for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.

Biopsy: The removal of cells or tissues from the vulva so they can be viewed under a microscope by a pathologist to check for signs of cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

—      The stage of the cancer.

—      The patient’s age and general health.

—      Whether the cancer has just been diagnosed or has recurred (come back).

Stages of vulvar cancer

After vulvar cancer has been diagnosed, tests are done to find out if cancer cells have spread within the vulva or to other parts of the body.

The process used to find out if cancer has spread within the vulva or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process:

Pelvic exam: An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. The doctor or nurse inserts one or two lubricated, 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 uterus and ovaries. A speculum is also inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test or Pap smear of the cervix is usually done. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas.

Cystoscopy: A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope (a thin, lighted tube) is inserted through the urethra into the bladder. Tissue samples may be taken for biopsy.

Proctoscopy: A procedure to look inside the rectum and anus to check for abnormal areas. A proctoscope (a thin, lighted tube) is inserted into the anus and rectum. Tissue samples may be taken for biopsy.

X-rays: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. To stage vulvar cancer, x-rays may be taken of the organs and bones inside the chest, and the pelvic bones.

Intravenous pyelogram (IVP): A series of x-rays of the kidneys, ureters, and bladder to find out if cancer has spread to these organs. A contrast dye is injected into a vein. As the contrast dye moves through the kidneys, ureters and bladder, x-rays are taken to see if there are any blockages. This procedure is also called intravenous urography.

CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

The following stages are used for vulvar cancer:

Stage 0 (Carcinoma in Situ)

In stage 0, abnormal cells are found on the surface of the vulvar skin. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.

Stage I

In stage I, cancer has formed and is found in the vulva only or in the vulva and perineum (area between the rectum and the vagina). The tumor is 2 centimeters or smaller and has spread to tissue under the skin. Stage I vulvar cancer is divided into stage IA and stage IB.

—      Stage IA: The tumor has spread 1 millimeter or less into the tissue of the vulva.

—      Stage IB: The tumor has spread more than 1 millimeter into the tissue of the vulva.

Stage II

In stage II, cancer is found in the vulva or the vulva and perineum (space between the rectum and the vagina), and the tumor is larger than 2 centimeters.

Stage III

In stage III vulvar cancer, the cancer is of any size and either:

—      is found only in the vulva or the vulva and perineum and has spread to nearby lymph nodes on one side of the groin; or

—      has spread to nearby tissues such as the lower part of the urethra and/or vagina or anus, and may have spread to nearby lymph nodes on one side of the groin.

Stage IV

Stage IV is divided into stage IVA and stage IVB, based on where the cancer has spread.

—      Stage IVA: Cancer has spread to nearby lymph nodes on both sides of the groin, or has spread beyond nearby tissues to the upper part of the urethra, bladder, or rectum, or has attached to the pelvic bone and may have spread to lymph nodes.

—      Stage IVB: Cancer has spread to distant parts of the body.

Recurrent Vulvar Cancer

Recurrent vulvar cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the vulva or in other parts of the body.

Treatment option overview

There are different types of treatment for patients with vulvar cancer. Different types of treatments are available for patients with vulvar cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Four types of standard treatment are used:

Laser therapy Laser therapy is a cancer treatment that uses a laser beam (a narrow beam of intense light) to kill cancer cells.

Surgery

Surgery is the most common treatment for cancer of the vulva. The goal of surgery is to remove all the cancer without any loss of the woman’s sexual function. One of the following types of surgery may be done:

Wide local excision: A surgical procedure to remove the cancer and some of the normal tissue around the cancer.

Radical local excision: A surgical procedure to remove the cancer and a large amount of normal tissue around it. Nearby lymph nodes in the groin may also be removed.

Vulvectomy: A surgical procedure to remove part or all of the vulva:

—      Skinning vulvectomy: The top layer of vulvar skin where the cancer is found is removed. Skin grafts from other parts of the body may be needed to cover the area.

—      Simple vulvectomy: The entire vulva is removed.

—      Modified radical vulvectomy: The vulva containing cancer and some of the normal tissue around it is removed.

—      Radical vulvectomy: The entire vulva, including the clitoris, and nearby tissue is removed. Nearby lymph nodes may also be removed.

Pelvic exenteration: A surgical procedure to remove the lower colon, rectum, and bladder. The cervix, vagina, ovaries, and nearby lymph nodes are also removed. Artificial openings (stoma) are made for urine and stool to flow from the body into a collection bag.

Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may have chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to increase the chances of a cure, is called adjuvant therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, a body cavity such as the abdomen, or onto the skin, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Topical chemotherapy for vulvar cancer may be applied to the skin in a cream or lotion.
New types of treatment are being tested in clinical trials.

Treatment options by stage

Stage 0 (Carcinoma in Situ)

Treatment of stage 0 may include the following:

—      Wide local excision and/or laser therapy.

—      Skinning vulvectomy with or without skin grafting.

—      Simple vulvectomy.

—      Topical chemotherapy.

Stage I Vulvar Cancer

Treatment of stage I vulvar cancer may include the following:

—      Wide local excision.

—      Radical local excision with removal of nearby lymph nodes.

—      Radical vulvectomy and either removal of nearby lymph nodes or radiation therapy to the lymph nodes.

—      Radiation therapy.

Stage II Vulvar Cancer

Treatment of stage II vulvar cancer may include the following:

—      Modified radical vulvectomy and removal of nearby lymph nodes or radiation therapy to the lymph nodes. Radiation therapy to the area of surgery may also be given.

—      Radiation therapy.

Stage III Vulvar Cancer

Treatment of stage III vulvar cancer may include the following:

—      Modified radical vulvectomy and removal of nearby lymph nodes, with or without radiation therapy.

—      Radical vulvectomy and removal of nearby lymph nodes, with or without radiation therapy.

—      Radiation therapy followed by surgery.

—      Radiation therapy with or without chemotherapy.

—      A clinical trial of a new treatment.

Stage IV Vulvar Cancer

Treatment of stage IV vulvar cancer may include the following:

—      Radical vulvectomy and pelvic exenteration.

—      Radical vulvectomy followed by radiation therapy.

—      Radiation therapy followed by surgery, with or without chemotherapy.

—      Radiation therapy with or without chemotherapy.

—      A clinical trial of a new treatment.

Treatment options for Recurrent Vulvar Cancer

It is important to have regular follow-up exams to check for recurrent vulvar cancer. Treatment of recurrent vulvar cancer may include the following:

—      Wide local excision with or without radiation therapy.

—      Radical vulvectomy and pelvic exenteration.

—      Radiation therapy and chemotherapy given during the same period of time, with or without surgery.

—      Radiation therapy followed by surgery or chemotherapy.

—      Radiation therapy as palliative treatment to relieve symptoms and improve quality of life.

—      A clinical trial of a new treatment.

For more information from the NCI, please write to this address:

NCI Public Inquiries Office

Suite 3036A

6116 Executive Boulevard, MSC8322

Bethesda, MD 20892-8322

U.S. residents may call the National Cancer Institute’s (NCI’s) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. Information about ongoing clinical trials is available from the NCI Web site www.cancer.gov/clinicaltrials.

Source: National Institutes of Health; National Cancer Institute

It is an abnormal growth of malignant cells (neoplasm, tumor) in the vulva. The vulva is defined as the female external genitalia, and includes the labia majora, labia minora, clitoris, and vestibule, or entryway, of the vagina.

About 70% of vulvar cancers involve the labia, (mainly the labia majora), 15% - 20% involve the clitoris, and another 15% - 20% involve the perineum, which is the area of sensitive skin located between the vagina and the anus. In about 5% of cases, the cancer is present at more than one site.

What are the different types of vulvar cancer?

The vulva is essentially epithelial skin, and so the main tumor types that affect this area are skin-related cancers.

The majority of vulvar cancers are squamous cell carcinomas, which typically develop at the edges of the labia majora/ minora or the vagina. As with vaginal squamous cell carcinomas, vulvar squamous cell cancers are slow-growing and usually develop from “precancerous”, pre-invasive areas called vulvar intraepithelial neoplasia (VIN).

Melanoma is the second most common type and represents about 5% - 10% of vulvar cases; roughly half involve the labia majora.

The most common skin-cancer in sun-exposed areas is basal cell carcinoma, and as expected, this type rarely occurs on the vulva.

Adenocarcinomas of the vulva are also rare, but can develop from glands such as the Bartholin’s glands at the vaginal opening.

How common is vulvar cancer?

Vulvar cancer is a relatively rare diagnosis, representing about 5% of all gynecologic cancers, and only about 1% of all female cancers in general. There are about 3,000 new cases in reported annually in the US, but the incidence has been rising over the past several years. In fact, the American Cancer Society (ACS) is estimating ~3,800 new cases will be diagnosed in 2005. The cause for the growing number of cases is not well-understood.

Who gets vulvar cancer?

Vulvar cancer is most common in women over 50 years of age, with a median age of 65 – 75 years old at diagnosis.

What are the risk factors for vulvar cancer?

In addition to older age, vulvar cancer has been associated with a history of:

—      infection with high-risk HPV types, (ie: HPV 16,18,31)

—      multiple sexual partners/ sexually transmitted diseases

—      cervical cancer

—      immunodeficiency

—      presence of chronic vaginal and vulvar irritation

—      smoking

What are the symptoms of vulvar cancer?

The classic symptom is vulvar itching (pruritus), reported in almost 90% of the women with vulvar cancer. There can also be associated pain, bleeding, vaginal discharge, and/or painful urination (dysuria).

Also, women often develop a visible vulvar mass: the squamous cell subtype can look like elevated white, pink or red bumps, while vulvar melanoma characteristically presents as a colored, ulcerated growth. There can be portions of the tumor that look sore and scaly, or cauliflower-like (similar to HPV-related warts)

How is vulvar cancer diagnosed?

First and foremost, a thorough gynecological examination should be performed using a colposcope (special magnifying instrument) for better visualization. Any suspicious areas should be tested by applying a dilute solution of acetic acid to the region; abnormal areas typically turn white, making them easier to identify. Also, any abnormal-appearing area should be sampled along with surrounding normal tissue using a thick wedge-shaped biopsy (usually under local anesthesia). If the area is small, it should be entirely removed in the process of the biopsy (so-called excisional biopsy).

Chest x-ray and CT scan of the abdomen/pelvis can be done to look for disease spread to lymph nodes and/or distant organs. If spread to bladder or rectum is suspected, endoscopy (cystoscopy andproctoscopy, respectively) should be performed.

Once it is diagnosed, how is vulvar cancer staged?

Unlike vaginal cancer, which is typically clinically staged, the International Federation of Gynecology and Obstetrics (FIGO) uses a surgical staging system for vulvar cancer. This means that the stage of the cancer is not actually determined until after surgery is performed and the specimen is examined by the pathologist.

Like vaginal cancer, vulvar cancer has five main FIGO stages (0, I, II, III, and IV). They are:

—      Stage 0 - Vulvar intraepithelial neoplasia

—      Stage I - cancer is limited to the vulva and perineum, and measures < 2 cm in size

—      Stage II - cancer is limited to the vulva and perineum, but tumor is > 2 cm in size

—      Stage III - cancer spread to vagina, urethra, anus, and/or the lymph nodes in the groin

—      Stage IV - cancer spread to bladder, bowel, pelvic bone, pelvic lymph nodes, and/or other parts of the body

How is vulvar cancer treated?

Surgery, radiation therapy and chemotherapy are the main treatment options, and are typically used in various combinations. As with many cancers, the optimal treatment depends on the disease stage and patient factors such as age and other medical conditions.

Treatment options by stage are as follows:

Stage 0

—      Wide local excision, laser surgery, or a combination of both

—      Skinning vulvectomy

—      Chemotherapy ointment

Stage I

—      Wide local excision

—      Radical local excision with removal of all nearby groin/ upper thigh lymph nodes

—      Radical vulvectomy and removal of nearby groin lymph nodes (and sometimes lymph nodes on opposite side of the body)

—      Radiation therapy alone (in selected patients)

Stage II

—      Radical vulvectomy and removal of groin lymph nodes on both sides of the body, plus postoperative radiation therapy to the pelvis if lymph nodes are positive for cancer

—      Radiation therapy alone (in selected patients)

Stage III

—      Radical vulvectomy and removal of groin/ upper thigh lymph nodes on both sides of the body, plus postoperative radiation therapy to the pelvis and groin if lymph nodes are positive for cancer or if the primary vulvar tumor is very large

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

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

Stage IV

—      Pelvic exenteration, which entails radical vulvectomy and removal of the lower colon, rectum, or bladder (depending on where the cancer has spread), as well as the uterus, cervix, and vagina

—      Radical vulvectomy followed by radiation therapy

—      Radiation therapy followed by radical vulvectomy

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

What is the prognosis?

In early stage disease, when lymph nodes are not involved, the overall 5-year survival rate is 90%.

Once cancer has spread to the lymph nodes, the overall 5-year survival rate drops to 50% - 70%.

Description of 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 and looks much like a pair of lips. 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.

Most women with cancer of the vulva are over age 50. However, it is becoming more common in women under age 40. Women who have constant itching and changes in the color and the way the vulva looks are at a high risk to get cancer of the vulva. You should see your doctor if you have bleeding or discharge not related to your periods (menstruation), severe burning/itching or pain in the vulva, or if the skin of the vulva looks white and feels rough.

Like most cancers, cancer of the vulva is best treated when it is found (diagnosed) early. If you have symptoms, your doctor may do certain tests to see if you have cancer, usually beginning by looking at the vulva and feeling for any lumps. Your 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. You will be given some medicine to numb the area when the biopsy is done. You may feel some pressure, but you usually won’t feel any pain. This test is often done in a doctor’s office.

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

Stages Of Cancer Of The Vulva

Once cancer of the vulva is diagnosed, more tests will be done to find out if the cancer has spread from the vulva to other parts of the body (staging). Your doctor needs to know the stage of your disease to plan treatment. The following stages are used for cancer of the vulva:

Stage 0 Or Carcinoma In Situ Stage 0 cancer of the vulva is a very early cancer. The cancer is found in the
vulva only and is only in the surface of the skin.

Stage I

Cancer is found only in the vulva and/or the space between the opening of the rectum and the vagina (perineum). The tumor is 2 centimeters (about 1 inch) or less in size.

Stage II

Cancer is found in the vulva and/or the space between the opening of the rectum and the vagina (perineum), and the tumor is larger than 2 centimeters (larger than 1 inch).

Stage III

Cancer is found in the vulva and/or perineum and has spread to nearby tissues such as the lower part of the urethra (the tube through which urine passes), the vagina, the anus (the opening of the rectum), and/or has spread to nearby lymph nodes. (Lymph nodes are small bean-shaped structures that are found throughout the body. They produce and store infection-fighting cells.)

Stage IV

Cancer has spread beyond the urethra, vagina, and anus into the lining of the bladder (the sac that holds urine) and the bowel (intestine); or, it may have spread to the lymph nodes in the pelvis or to other parts of the body.

Recurrent

Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the vulva or another place. How Cancer Of The Vulva Is Treated 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 for cancer of the vulva. Your 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.

If the cancer has spread outside the vulva and the other female organs, your 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).

You may need to have skin from another part of your 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 By Stage

Treatment for cancer of the vulva depends on the stage of your disease, the type of disease, your age, and your overall condition.

You may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or you may choose to go into a clinical trial. 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 going on in most parts of the country for stages III and IV of cancer of the vulva. If you want more information, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

STAGE 0 VULVAR CANCER

Your treatment may be one of the following:

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

            Skinning vulvectomy.

            Ointment containing a chemotherapy drug.

STAGE I VULVAR CANCER

Your treatment may be one of the following:

            Wide local excision.

            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.

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

            Radiation therapy alone (in selected patients).

STAGE II VULVAR CANCER

Your treatment may be one of the following:

            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.

            Radiation therapy alone (in selected patients).

STAGE III VULVAR CANCER

Your treatment may be one of the following:

            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.

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

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

STAGE IV VULVAR CANCER

Your treatment may be one of the following:

            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).

            Radical vulvectomy followed by radiation therapy.

            Radiation therapy followed by radical vulvectomy.

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

RECURRENT VULVAR CANCER

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

            Wide local excision with or without radiation therapy.

            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).

            Radiation therapy plus chemotherapy with or without surgery.

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

            Clinical trials of new forms of therapy.

Cancer of the Vulva or known as cancer of the perineum, a rare kind of cancer in women, is a disease in which cancer (malignant) cells are found in the vulva. Most cases are found in women over the age of 50. Although, it’s becoming more and more frequent to be diagnosed under the age of 40. I am one of them, I was 30 years old when diagnosed. 

Symptoms

Some of the Symptoms

Note: Nearly 20% of women with vulvar cancer have no symptoms.

Signs And Tests

A routine pelvic examination may be the best means of noting early changes. Skin changes include any color changes and the presence of lesions that may vary in size and shape.
Excision and biopsy of the lesion is critical for accurate diagnosis.

If any of these symptoms persist a doctor should be seen immediately.  The type of cancer is 80% of the time squamaus epithelium, 10% are malignant melanomas, 5% are glandular, 3% are basal cell carcinoma, and 2% are rare sarcomas. The cause of the cancer of the vulva is still uncertain. Though there is a link with the Human Papilloma Virus (HPV). 

Treatment see main Treatment page

Treatment of vulva cancer may begin with a biopsy or removing a small portion of the lump for further examination. If the growth is malignant, the doctor may choose to do a vulvectomy (vul-vek-toe-me), which entails a surgical procedure which removes part (removal of the growth and the surrounding skin) or all of the vulva. And sometimes the  removal of the glands in the groin and the skin in between. 

See Treatment  See Stages

The chance of recovery and treatment choice depend on the stage of the cancer and your overall heath condition.

Cancer of the Vulva can be beaten with early detection.

The vulva is the skin and fatty tissue between the upper thighs of women, from the area of the anus to about an inch below the pubic hairline. Cancer of the vulva most often affects the two skin folds (or lips) around the vagina, known as the labia.

Vulvar cancer is not very common. However, it is very serious because it can affect a woman’s sexual functioning. It can make sex painful and difficult. This makes some women feel sad and worthless. If found early, vulvar cancer has a high cure rate and the treatment options involve less surgery.

Who is affected?

Vulvar cancer most often affects women 65 to 75 years of age. However, it can also occur in women 40 years of age or younger. Vulvar cancer may be related to genital warts, a sexually transmitted disease caused by the human papillomavirus (HPV).

What are the signs of vulvar cancer?

Tell your doctor if you have any of these warning signs of vulvar cancer:

—      Vulvar itching that lasts more than one month

—      A cut or sore on the vulva that won’t heal

—      A lump or mass on the vulva

—      Vulvar pain

—      Bleeding from the vulva (different from your usual monthly bleeding)

—      Burning in the area that lasts even after your doctor has treated the burning

—      Any change in size, color, or texture of a birthmark or mole in the vulvar area

How is vulvar cancer diagnosed?

If your doctor finds an abnormal area on the vulva, he or she may want to take a small piece of skin to look at under the microscope. This procedure can be done in the doctor’s office. It is called a biopsy. A biopsy is the only way to find out if you have vulvar cancer.

How is vulvar cancer treated?

Vulvar cancer is usually treated with surgery. The type of surgery depends on the size, depth and spread of the cancer. Your doctor will review all the options for surgery and the pros and cons of each option. Some people may also need radiation therapy.

When vulvar cancer is found and treated early, the cure rate is over 90 percent. The key to a cure is to tell your doctor about any warning signs early and to have a biopsy right away.

The premalignant phase of vulvar squamous cell cancer has several different names: carcinoma-in-situ, vulvar intraepithelial neoplasia grade III, (VIN III), severe dysplasia and Bowen’s disease. This condition is diagnosed by tissue biopsy and is characterized by a full thickness disorder of maturation of the squamous epithelium (see Dysplasia). It is usually symptomatic with itching and burning and can be present for years. It is usually misdiagnosed as a yeast infection and a multitude of anti-fungal agents will have been prescribed, none of which will be effective. It is easy to see on examination and will appear as either a raised white or pigmented patch. A simple biopsy will confirm the diagnosis.

It is best treated by excision or sometimes by laser evaporation. If a large area is involved and must be removed, then a skin graft can be applied. These premalignant conditions are likely to recur after treatment so continued follow up is a necessity. Another condition that can occur on the vulva that also causes itching and soreness is called lichen sclerosis. It is not a premalignant change, but an atrophy of the skin. It will not be improved by anti-yeast medications either. It can be diagnosed by biopsy. These two conditions, lichen sclerosis and VIN III, can be present for years and be misdiagnosed as yeast infections.

The most important point about premalignant vulvar changes is that there is usually a long delay in diagnosis. Often these women are not examined properly or the examiner is unfamiliar with this condition and prescribes yet another course of cream, salve, or ointment. Usually the condition is fully visible and simply needs to be biopsied to establish the diagnosis.

INVASIVE VULVAR CANCER

Squamous cell cancer of the vulva usually causes pain, soreness and itching. There is usually an obvious growth on the skin or an ulcerated area. Diagnosis is by simple biopsy. These cancers are usually slow growing and do not spread early. When they do spread it is usually by way of the lymph nodes. The regional lymph nodes are located at the top of the thigh in the groin area. Vulvar cancers are staged by a combination of examination and surgery.

The TNM staging system is used.

T-0          Pre-malignant change.

T-1A       A cancer less than 2.0cm in diameter and less than 1.0mm in depth of invasion.

T-1B       A cancer less than 2.0cm in diameter but greater than 1.0mm in invasion.

T-2          Greater than 2.0cm in diameter.

T-3          Involves vagina, urethra or anus.

T-4          Involves bladder, rectum or pelvic bone.

               

N-0          No lymph nodes involved.

N-1          Lymph node metastases to one groin.

N-2          Lymph node metastases to both groins.

               

M-0         No distant metastases.

M-1         Any distant metastases.

TREATMENT OF VULVAR CANCER

Vulvar cancers are usually treated by surgery with a radical excision of the cancer and removal of the regional lymph nodes. If the cancer is clearly on only one side and small then only that one side may need to be removed. Radical excision means that there must be a good margin of uninvolved tissue removed with the cancer. Usually an acceptable margin is about two centimeters. This will result in some disfigurement if the cancer is larger than about two centimeters in size. Large cancers will also require some sort of plastic surgery technique to close the defect Complications of surgery are closure breakdown with prolonged healing and sometimes a collection of fluid in the groin where the lymph nodes where removed. There may also be leg swelling. If the cancer is very large and a radical resection would require removal of the anus, rectum or urethra then primary treatment can be given by radiation to preserve these vital structures. If there is cancer in the lymph nodes then that groin as well as the pelvic lymph nodes are irradiated upon recovery from surgery. Often when these cancers are being irradiated chemotherapy will also be given to potentiate the effects of the radiation.

The prognosis is in general good. If the lymph nodes are negative then the chance for a cure is excellent. Even with positive lymph nodes a significant number are cured.

Vulvar melanoma is no different from melanomas that occur elsewhere on the body. They are unpredictable and can be very aggressive. They are treated surgically if possible.

William M. Rich, MD

Clinical Professor of Obstetrics and Gynecology

University of California San Francisco

Director of Gynecologic Oncology

Valley Medical Center

Fresno, Ca.

The vulva is the external portion of the female genital organs. It includes:

—      labia majora - two large, fleshy lips, or folds of skin

—      labia minora - small lips that lie inside the labia majora and surround the openings to the urethra and vagina

—      vestibule - space where the vagina opens

—      prepuce - a fold of skin formed by the labia minora

—      clitoris - a small protrusion sensitive to stimulation

—      fourchette - area beneath the vaginal opening where the labia minora meet

—      perineum - area between the vagina and the anus

—      anus - opening at the end of the anal canal

—      urethra - connecting tube to the bladder

What is vulvar cancer?

Vulvar cancer is a malignancy that can occur on any part of the external organs, but most often affects the labia majora or labia minora. Cancer of the vulva is a rare disease, which accounts for half of one percent of all cancers in women, and may form slowly over many years. Nearly 90 percent of vulvar cancers are squamous cell carcinomas. Melanoma is the second most common type of vulvar cancer, usually found in the labia minora or clitoris. Other types of vulvar cancer include:

—      adenocarcinoma

—      Paget’s disease

—      sarcomas

—      verrucous carcinoma

—      basal cell carcinoma

What are risk factors for vulvar cancer?

The following have been suggested as risk factors for vulvar cancer:

—      age - of the women who develop vulvar cancer, three-fourths are over age 50, and two-thirds are over age 70.

—      infection with the human papillomavirus (HPV)

—      smoking

—      human immunodeficiency virus (HIV) infection

—      low socioeconomic status

—      vulvar intraepithelial neoplasia (VIN) - there is an increased risk for vulvar cancer in women with VIN, although most cases do not progr