Vulvar Cancer
General Information About Vulvar Cancer (Symptoms and Diagnosis)
Stages of Vulvar Cancer
Treatment Options
The vulva is the external part of the female genitals,
including the clitoris, the vaginal lips, the opening to the vagina,
and the surrounding skin and tissue.
Most vulvar cancers are squamous cell carcinoma. This type of
cancer begins in squamous cells (thin, flat skin cells) and is usually
found on the vaginal lips.
A small number of vulvar cancers are adenocarcinomas (cancers
that begin in cells that make mucus and other fluids). This type of
cancer is usually found on the sides of the vaginal opening.
Vulvar cancer usually forms slowly over a number of years.
Abnormal cells can grow on the surface of the vulvar skin for a long
time. This condition is called vulvar intraepithelial neoplasia (VIN).
Because it is possible for VIN to become vulvar cancer, it is important
to get treatment. Signs and symptoms of vulvar cancer include a lump,
bleeding, or itching.
Infection with certain types of human papillomavirus (HPV)
causes about half of all vulvar cancers. Vaccines that protect against
infection with these types of HPV may reduce the risk of vulvar cancer.
General Information About Vulvar Cancer
Key Points
- Vulvar cancer is a rare disease in which malignant (cancer)
cells form in the tissues of the vulva.
- Having vulvar intraepithelial neoplasia or HPV infection
can affect the risk of vulvar cancer.
- Signs of vulvar cancer include bleeding or itching.
- Tests that examine the vulva are used to detect (find) and
diagnose vulvar cancer.
- Certain factors affect prognosis (chance of recovery) and
treatment options.
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:
- Inner and outer lips of the vagina.
- Clitoris (sensitive tissue
between the lips).
- Opening of the vagina and its glands.
- Mons pubis (the rounded area in front of the pubic bones
that becomes covered with hair at puberty).
- Perineum
(the area between the vulva and the anus).
Anatomy of the vulva. The
vulva includes the mons pubis, clitoris, urethral opening, inner and
outer lips of the vagina, vaginal opening, and perineum.
Vulvar cancer most often
affects the outer vaginal
lips. Less often, cancer affects the inner vaginal lips, clitoris, or
vaginal glands.
Vulvar cancer usually
forms slowly over a number of years. Abnormal
cells can grow on
the surface of the vulvar
skin for a long time. This condition
is called vulvar intraepithelial
neoplasia (VIN). Because it is possible for VIN to become
vulvar cancer, it is very important to get treatment.
Having
vulvar intraepithelial neoplasia or HPV infection can affect the risk
of vulvar cancer.
Anything that increases
your risk of getting a disease is called a risk
factor. Having a risk factor does not mean that you will
get cancer; not
having risk factors doesn't mean that you will not get cancer. Talk
with your doctor if you think you may be at risk. Risk factors for
vulvar cancer include the following:
- Having vulvar intraepithelial neoplasia (VIN).
- Having human
papillomavirus (HPV) infection.
- Having a history
of genital warts.
Other possible risk
factors include the following:
- Having many sexual partners.
- Having first sexual intercourse at a young age.
- Having a history of abnormal Pap
tests (Pap smears).
Signs
of vulvar cancer include bleeding or itching.
Vulvar cancer often does
not cause early signs
or symptoms. Signs
and symptoms may be caused by vulvar cancer or by other conditions.
Check with your doctor if you have any of the following:
- A lump or growth on the vulva.
- Changes in the vulvar skin, such as color changes or
growths that look like a wart or ulcer.
- Itching in the vulvar area, that does not go away.
- 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 samples 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
Key Points
- 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.
- There are three ways that cancer spreads in the body.
- Cancer may spread from where it began to other parts of the
body.
- In vulvar intraepithelial neoplasia (VIN), abnormal cells
are found on the surface of the vulvar skin.
- The following stages are used for vulvar cancer:
- Stage I
- Stage II
- Stage III
- Stage IV
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. A speculum is inserted into
the vagina and the doctor or nurse
looks at the vagina and cervix for signs of disease. A Pap test of the cervix is
usually done. The doctor or nurse also 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. The doctor or nurse also
inserts a lubricated, gloved finger into the rectum to feel for lumps
or abnormal areas.
Pelvic exam. A doctor
or nurse inserts one or two lubricated, gloved fingers of one hand into
the vagina and presses on the lower abdomen with the other hand. This
is done to feel the size, shape, and position of the uterus and
ovaries. The vagina, cervix, fallopian tubes, and rectum are also
checked.
- Colposcopy
: A procedure in which a colposcope
(a lighted, magnifying instrument) is used to check the vagina and
cervix for abnormal areas. Tissue
samples may be taken using a curette
(spoon-shaped instrument) or a brush and checked under a microscope for signs of
disease.
- Cystoscopy
: A procedure to look inside the bladder and urethra to check for
abnormal areas. A cystoscope
is inserted through the urethra into the bladder. A cystoscope is a
thin, tube-like instrument with a light and a lens for viewing. It may
also have a tool to remove tissue samples, which are checked under a
microscope for signs of cancer.
- Proctoscopy
: A procedure to look inside the rectum and anus to check for abnormal
areas. A proctoscope
is inserted into the anus and rectum. A proctoscope is a thin,
tube-like instrument with a light and a lens for viewing. It may also
have a tool to remove tissue samples, which are checked under a
microscope for signs of cancer.
- 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).
- PET
scan (positron emission tomography scan): A
procedure to find malignant
tumor cells in the body. A small
amount of radioactive
glucose (sugar)
is injected into a vein. The PET scanner
rotates around the body and makes a picture of where glucose is being
used in the body. Malignant tumor cells show up brighter in the picture
because they are more active and take up more glucose than normal cells
do.
- Sentinel
lymph node biopsy : The removal of the sentinel lymph node during surgery. The sentinel lymph
node is the first lymph node
to receive lymphatic drainage
from a tumor. It is the first lymph node the cancer is likely to spread
to from the tumor. A radioactive substance and/or blue dye is injected
near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes.
The first lymph node to receive the substance or dye is removed. A pathologist views the tissue
under a microscope to look for cancer cells. If cancer cells are not
found, it may not be necessary to remove more lymph nodes. Sentinel
lymph node biopsy may be done during surgery to remove the tumor for
early-stage vulvar cancer.
There
are three ways that cancer spreads in the body.
Cancer can spread
through tissue, the
lymph system, and
the blood:
- Tissue. The cancer spreads from where it began by growing
into nearby areas.
- Lymph system. The cancer spreads from where it began by
getting into the lymph system. The cancer travels through the lymph vessels to other parts
of the body.
- Blood. The cancer spreads from where it began by getting
into the blood. The cancer travels through the blood vessels to other parts
of the body.
Cancer
may spread from where it began to other parts of the body.
When cancer spreads
to another part of the body, it is called metastasis.
Cancer cells break
away from where they began (the primary
tumor) and travel through the lymph system or blood.
- Lymph system. The cancer gets into the lymph system,
travels through the lymph vessels, and forms a tumor (metastatic tumor) in another
part of the body.
- Blood. The cancer gets into the blood, travels through the
blood vessels, and forms a tumor (metastatic tumor) in another part of
the body.
The metastatic tumor is
the same type of cancer as the primary tumor. For example, if vulvar
cancer spreads to the lung,
the cancer cells in the lung are actually vulvar cancer cells. The
disease is metastatic vulvar cancer, not lung cancer.
In
vulvar intraepithelial neoplasia (VIN), abnormal cells are found on the
surface of the vulvar skin.
These abnormal cells are not cancer. Vulvar intraepithelial neoplasia
(VIN) may become cancer and spread into nearby tissue. VIN is sometimes
called stage 0 or carcinoma in
situ.
The
following stages are used for vulvar cancer:
Stage
I
In stage I, cancer has formed. The tumor is found only in the vulva or perineum (area between the rectum and the vagina). Stage I is divided
into stages IA and IB.
Pea, peanut, walnut, and
lime show tumor sizes.
Stage
II
In stage II, the tumor is any size and has
spread into the lower part of the urethra,
the lower part of the vagina,
or the anus. Cancer has not spread to the
lymph nodes.
Stage
III
In stage III, the tumor is any size and may
have spread into the lower part of the urethra,
the lower part of the vagina,
or the anus. Cancer has spread to one or
more nearby lymph nodes.
Stage III is divided into stages IIIA, IIIB, and IIIC.
- In stage IIIA,
cancer is found in 1 or 2 lymph
nodes that are smaller than 5 millimeters
or in one lymph node that is 5 millimeters or larger.
- In stage IIIB,
cancer is found in 2 or more lymph
nodes that are 5 millimeters
or larger, or in 3 or more lymph nodes that are smaller than 5
millimeters.
- In stage IIIC,
cancer is found in lymph nodes
and has spread to the outside surface of the lymph nodes.
Stage
IV
In stage IV, the tumor has spread into the
upper part of the urethra,
the upper part of the vagina,
or to other parts of the body. Stage IV is divided into stages IVA and
IVB.
- In stage IVA:
- cancer
has spread into the lining of the upper urethra,
the upper vagina,
the bladder, or the
rectum, or
has attached to the pelvic
bone; or
- cancer has spread to nearby lymph
nodes and the lymph nodes are not moveable or have formed
an ulcer.
- In stage IVB,
cancer has spread to lymph nodes
in the pelvis or to
other 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
Key Points
- There are different types of treatment for patients with
vulvar cancer.
- Four types of standard treatment are used:
- Surgery
- Radiation therapy
- Chemotherapy
- Biologic therapy
- New types of treatment are being tested in clinical trials.
- Patients may want to think about taking part in a clinical
trial.
- Patients can enter clinical trials before, during, or after
starting their cancer treatment.
- Follow-up tests may be needed.
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:
Surgery
Surgery is the most common
treatment for vulvar cancer. 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:
- Laser surgery:
A surgical
procedure that uses a laser beam
(a narrow beam of intense light) as a knife to make bloodless cuts in tissue or to remove a
surface lesion such
as a tumor.
- 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.
- Ultrasound surgical aspiration (USA): A surgical procedure
to break the tumor up into small pieces using very fine vibrations. The
small pieces of tumor are washed away and removed by suction. This
procedure causes less damage to nearby tissue.
- 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 where the skin was removed.
- Modified
radical vulvectomy: Surgery to remove part of the vulva.
Nearby lymph nodes may also be removed.
- Radical
vulvectomy: Surgery to remove the entire vulva. Nearby
lymph nodes are also 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 or keep them from growing. 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. External and internal radiation therapy
are used to treat vulvar cancer, and external radiation therapy may
also be used as palliative
therapy to relieve symptoms
and improve quality of life.
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 cerebrospinal
fluid, 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.
See Drugs Approved to
Treat Vulvar Cancer for more information.
Biologic
therapy
Biologic therapy is a
treatment that uses the patient's immune
system to fight cancer. Substances made by the body or
made in a laboratory are used to boost, direct, or restore the body's
natural defenses against cancer. This type of cancer treatment is also
called biotherapy or immunotherapy.
Imiquimod is a biologic
therapy that may be used to treat vulvar lesions
and is applied to the skin in a cream.
New
types of treatment are being tested in clinical trials.
Information about
clinical trials is available from the NCI website.
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. See the Treatment Options
section that follows for links to current treatment clinical trials.
These have been retrieved from NCI's
listing of clinical trials.
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.
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.
It is important to have
regular follow-up
exams to check for recurrent
vulvar cancer.
Treatment Options by Stage
Vulvar Intraepithelial Neoplasia (VIN)
Treatment of vulvar intraepithelial neoplasia
(VIN) may include the following:
- Removal of single lesions
or wide local excision.
- Laser surgery.
- Ultrasound surgical
aspiration.
- Skinning vulvectomy
with or without a skin graft.
- Biologic therapy
with topical imiquimod.
Stage I Vulvar Cancer
Treatment of stage I vulvar cancer may
include the following:
- Wide local excision
for lesions that
are less than 1 millimeter
deep..
- Radical local
excision and removal of nearby lymph
nodes.
- Radical local excision and sentinel
lymph node biopsy. If cancer
is found in the sentinel lymph
node, nearby lymph nodes are also removed.
- Radiation therapy
for patients who cannot have surgery.
Check the
list of NCI-supported cancer clinical trials that are now accepting
patients with stage I 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. Talk with your
doctor about clinical trials that may be right for you. General
information about clinical trials is available from the NCI website.
Stage II Vulvar Cancer
Treatment of stage II vulvar cancer may
include the following:
- Radical local
excision and removal of nearby lymph
nodes.
- Modified radical
vulvectomy or radical
vulvectomy for large tumors.
Nearby lymph nodes may be removed. Radiation
therapy may be given after surgery.
- Radical local excision and sentinel
lymph node biopsy. If cancer
is found in the sentinel lymph
node, nearby lymph nodes are also removed.
- Radiation therapy for patients who cannot have surgery.
Check the
list of NCI-supported cancer clinical trials that are now accepting
patients with stage II 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. Talk with your
doctor about clinical trials that may be right for you. General
information about clinical trials is available from the NCI website.
Stage III Vulvar Cancer
Treatment of stage III vulvar cancer may
include the following:
- Modified radical
vulvectomy or radical
vulvectomy. Nearby lymph
nodes may be removed. Radiation
therapy may be given after surgery.
- Radiation therapy or chemotherapy
and radiation therapy followed by surgery.
- Radiation therapy with or without chemotherapy for patients
who cannot have surgery.
Check the
list of NCI-supported cancer clinical trials that are now accepting
patients with stage III 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. Talk with
your doctor about clinical trials that may be right for you. General
information about clinical trials is available from the NCI website.
Stage IV Vulvar Cancer
Treatment of stage IVA vulvar cancer may
include the following:
- Radical vulvectomy
and pelvic exenteration.
- Radical vulvectomy followed by radiation
therapy.
- Radiation therapy or chemotherapy
and radiation therapy followed by surgery.
- Radiation therapy with or without chemotherapy for patients
who cannot have surgery.
There is no
standard treatment
for
stage IVB vulvar cancer.
Treatment may include a
clinical
trial of a new treatment.
Treatment Options for Recurrent Vulvar Cancer
Treatment of recurrent vulvar cancer may include
the following:
- Wide local excision
with or without radiation therapy
to treat cancer
that has come back in the same area.
- Radical vulvectomy
and pelvic exenteration
to treat cancer that has come back in the same area.
- Chemotherapy
and radiation therapy 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.
-NIH
