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).
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through tissue. Cancer invades the surrounding normal tissue.
- Through the lymph system. Cancer invades the lymph system and travels
through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels
through the blood to other places in the body.
When cancer cells break away from the primary (original) tumor
and travel through the lymph or blood to other places in the body, another
(secondary) tumor may form. This process is called metastasis. The secondary
(metastatic) tumor is the same type of cancer as the primary tumor. For example,
if breast cancer spreads to the bones, the cancer cells in the bones are
actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
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
further 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
tissue under the skin and 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. 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. Patients may want to think about taking part in a
clinical trial. Some clinical trials are open only to patients who have not started 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 part of the vulva that
contains cancer and some of the normal tissue around it are removed.
-
Radical vulvectomy: The entire vulva, including the clitoris,
and nearby tissue are 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 lower the risk that the cancer will come back, 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.
Information about clinical trials is available from the NCI Web site.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the
best treatment choice. Clinical trials are part of the cancer research process.
Clinical trials are done to find out if new cancer treatments are safe and
effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on
earlier clinical trials. Patients who take part in a clinical trial may receive
the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the
way cancer will be treated in the future. Even when clinical trials do not lead
to effective new treatments, they often answer important questions and help move
research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet
received treatment. Other trials test treatments for patients whose cancer has
not gotten better. There are also clinical trials that test new ways to stop
cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to
find out the stage of the cancer may be repeated. Some tests will be repeated in
order to see how well the treatment is working. Decisions about whether to
continue, change, or stop treatment may be based on the results of these tests.
This is sometimes called re-staging.
Some of the tests will continue to be done from time to time
after treatment has ended. The results of these tests can show if your condition
has changed or if the cancer has recurred (come back). These tests are sometimes
called follow-up tests or check-ups.
Treatment Options by Stage
For some types or stages of cancer, there may not be any trials
listed. Check with your doctor for clinical trials that are not listed here but
may be right for you.
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
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical
Trials Registry that are now accepting patients with vulvar cancer. For more
specific results, refine the search by using other search features, such as the
location of the trial, the type of treatment, or the name of the drug.
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.
Source: National Institutes of Health; National Cancer Institute
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 8/27/2009...#6220
