Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast.
Sometimes breast cancer occurs in women who are pregnant or have just given birth.
Signs of breast cancer include a lump or change in the breast.
It may be difficult to detect (find) breast cancer early in pregnant or nursing women.
Breast exams should be part of prenatal and postnatal care.
Tests that examine the breasts are used to diagnose breast cancer.
If cancer is found, tests are done to study the cancer cells.
Certain factors affect prognosis (chance of recovery) and treatment options.
Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast.
The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called lobes. Each lobe has many smaller sections called lobules. Lobules end in dozens of tiny bulbs that can make milk. The lobes, lobules, and bulbs are linked by thin tubes called ducts.
EnlargeThe female breast contains lobes, lobules, and ducts that produce and transport milk to the nipple. Fatty tissue gives the breast its shape, while muscles and the chest wall provide support. The lymphatic system, including lymph nodes, filter lymph and store white blood cells that help fight infection and disease.
Each breast also has blood vessels and lymph vessels. The lymph vessels carry an almost colorless, watery fluid called lymph. Lymph vessels carry lymph between lymph nodes. Lymph nodes are small, bean-shaped structures found throughout the body. They filter lymph and store white blood cells that help fight infection and disease. Groups of lymph nodes are found near the breast in the axilla (under the arm), above the collarbone, and in the chest.
Sometimes breast cancer occurs in women who are pregnant or have just given birth.
Breast cancer occurs about once in every 3,000 pregnancies. It occurs most often in women aged 32 to 38 years. Because many women are choosing to delay having children, it is likely that the number of new cases of breast cancer during pregnancy will increase.
Signs of breast cancer include a lump or change in the breast.
These and other signs may be caused by breast cancer or by other conditions. Check with your doctor if you have any of the following:
A lump or thickening in or near the breast or in the underarm area.
Fluid, other than breast milk, from the nipple, especially if it’s bloody.
Scaly, red, or swollen skin on the breast, nipple, or areola (the dark area of skin around the nipple).
Dimples in the breast that look like the skin of an orange, called peau d’orange.
It may be difficult to detect (find) breast cancer early in pregnant or nursing women.
The breasts usually get larger, tender, or lumpy in women who are pregnant, nursing, or have just given birth. This occurs because of normal hormone changes that take place during pregnancy. These changes can make small lumps difficult to detect. The breasts may also become denser. It is more difficult to detect breast cancer in women with dense breasts using mammography. Because these breast changes can delay diagnosis, breast cancer is often found at a later stage in these women.
Breast exams should be part of prenatal and postnatal care.
To detect breast cancer, pregnant and nursing women should examine their breasts themselves. Women should also receive clinical breast exams during their regular prenatal and postnatal check-ups. Talk to your doctor if you notice any changes in your breasts that you do not expect or that worry you.
Tests that examine the breasts are used to diagnose breast cancer.
The following tests and procedures may be used:
Physical exam and health history: An exam of the body to check general signs of health, including checking 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.
Clinical breast exam (CBE): An exam of the breast by a doctor or other health professional. The doctor will carefully feel the breasts and under the arms for lumps or anything else that seems unusual.
Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to look at later.
Mammogram: An x-ray of the breast. A mammogram can be done with little risk to the fetus. Mammograms in pregnant women may appear negative even though cancer is present. EnlargeMammography is an imaging test used to screen for and diagnose breast cancer. It can detect abnormal breast tissue, including cancer, sometimes before symptoms appear.
Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. If a lump in the breast is found, a biopsy may be done.
Multigene tests: Tests in which samples of tissue are studied to look at the activity of many genes at the same time. These tests may help predict whether cancer will spread to other parts of the body or recur (come back).
Oncotype DX: This test helps predict whether stage I or stage II breast cancer that is estrogen receptor positive and node-negative will spread to other parts of the body. If the risk of the cancer spreading is high, chemotherapy may be given to lower the risk.
MammaPrint: A laboratory test in which the activity of 70 different genes is looked at in the breast cancer tissue of women who have early-stageinvasive breast cancer that has not spread to lymph nodes or has spread to 3 or fewer lymph nodes. The activity level of these genes helps predict whether breast cancer will spread to other parts of the body or come back. If the test shows that the risk that the cancer will spread or come back is high, chemotherapy may be given to lower the risk.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis and treatment options depend on the following:
The stage of the cancer (the size of the tumor and whether it is in the breast only or has spread to other parts of the body).
After breast cancer has been diagnosed, tests are done to find out if cancer cells have spread within the breast 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 breast cancer, stage is based on the size and location of the primary tumor, the spread of cancer to nearby lymph nodes or other parts of the body, tumor grade, and whether certain biomarkers are present.
The TNM system is used to describe the size of the primary tumor and the spread of cancer to nearby lymph nodes or other parts of the body.
Tumor (T). The size and location of the tumor.
Lymph Node (N). The size and location of lymph nodes where cancer has spread.
Metastasis (M). The spread of cancer to other parts of the body.
The grading system is used to describe how quickly a breast tumor is likely to grow and spread.
Biomarker testing is used to find out whether breast cancer cells have certain receptors.
The TNM system, the grading system, and biomarker status are combined to find out the breast cancer stage.
Talk to your doctor to find out what your breast cancer stage is and how it is used to plan the best treatment for you.
After breast cancer has been diagnosed, tests are done to find out if cancer cells have spread within the breast or to other parts of the body.
The process used to find out if the cancer has spread within the breast 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.
Some procedures may expose the fetus to harmful radiation or dyes. These procedures are done only if absolutely necessary. Certain actions, such as using a lead-lined shield to cover the abdomen, are used to help protect the fetus from radiation as much as possible.
Chest x-ray: An x-ray of the organs and bones inside the chest. 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.
Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in bones with cancer and is detected by a scanner.
Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the liver, and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
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, such as the brain. This procedure is also called nuclear magnetic resonance imaging (NMRI).
There are three ways that cancer spreads in the body.
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 breast cancer spreads to the bone, the cancer cells in the bone are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.
In breast cancer, stage is based on the size and location of the primary tumor, the spread of cancer to nearby lymph nodes or other parts of the body, tumor grade, and whether certain biomarkers are present.
To plan the best treatment and understand your prognosis, it is important to know the breast cancer stage.
Pathological Prognostic Stage is then used for patients who have surgery as their first treatment. The Pathological Prognostic Stage is based on all clinical information, biomarker status, and laboratory test results from breast tissue and lymph nodes removed during surgery.
Anatomic Stage is based on the size and the spread of cancer as described by the TNM system. The Anatomic Stage is used in parts of the world where biomarker testing is not available. It is not used in the United States.
The TNM system is used to describe the size of the primary tumor and the spread of cancer to nearby lymph nodes or other parts of the body.
For breast cancer, the TNM system describes the tumor as follows:
Tumor (T). The size and location of the tumor.
EnlargeTumor sizes are often measured in millimeters (mm) or centimeters. Common items that can be used to show tumor size in mm include: a sharp pencil point (1 mm), a new crayon point (2 mm), a pencil-top eraser (5 mm), a pea (10 mm), a peanut (20 mm), and a lime (50 mm).
TX: Primary tumor cannot be assessed.
T0: No sign of a primary tumor in the breast.
Tis: Carcinoma in situ. There are 2 types of breast carcinoma in situ:
Tis (DCIS): DCIS is a condition in which abnormal cells are found in the lining of a breast duct. The abnormal cells have not spread outside the duct to other tissues in the breast. In some cases, DCIS may become invasive breast cancer that is able to spread to other tissues. At this time, there is no way to know which lesions can become invasive.
Tis (Paget disease): Paget disease of the nipple is a condition in which abnormal cells are found in the skin cells of the nipple and may spread to the areola. It is not staged according to the TNM system. If Paget disease AND an invasive breast cancer are present, the TNM system is used to stage the invasive breast cancer.
T1: The tumor is 20 millimeters or smaller. There are 4 subtypes of a T1 tumor depending on the size of the tumor:
T1mi: the tumor is 1 millimeter or smaller.
T1a: the tumor is larger than 1 millimeter but not larger than 5 millimeters.
T1b: the tumor is larger than 5 millimeters but not larger than 10 millimeters.
T1c: the tumor is larger than 10 millimeters but not larger than 20 millimeters.
T2: The tumor is larger than 20 millimeters but not larger than 50 millimeters.
T3: The tumor is larger than 50 millimeters.
T4: The tumor is described as one of the following:
T4b: the tumor has grown into the skin—an ulcer has formed on the surface of the skin on the breast, small tumor nodules have formed in the same breast as the primary tumor, and/or there is swelling of the skin on the breast.
T4c: the tumor has grown into the chest wall and the skin.
Lymph Node (N). The size and location of lymph nodes where cancer has spread.
When the lymph nodes are removed by surgery and studied under a microscope by a pathologist, pathologic staging is used to describe the lymph nodes. The pathologic staging of lymph nodes is described below.
NX: The lymph nodes cannot be assessed.
N0: No sign of cancer in the lymph nodes, or tiny clusters of cancer cells not larger than 0.2 millimeters in the lymph nodes.
N1a: cancer has spread to 1 to 3 axillary lymph nodes and the cancer in at least one of the lymph nodes is larger than 2 millimeters.
N1b: cancer has spread to lymph nodes near the breastbone on the same side of the body as the primary tumor, and the cancer is larger than 0.2 millimeters and is found by sentinel lymph node biopsy. Cancer is not found in the axillary lymph nodes.
N1c: cancer has spread to 1 to 3 axillary lymph nodes and the cancer in at least one of the lymph nodes is larger than 2 millimeters. Cancer is also found by sentinel lymph node biopsy in the lymph nodes near the breastbone on the same side of the body as the primary tumor.
N2: Cancer is described as one of the following:
N2a: cancer has spread to 4 to 9 axillary lymph nodes and the cancer in at least one of the lymph nodes is larger than 2 millimeters.
N2b: cancer has spread to lymph nodes near the breastbone and the cancer is found by imaging tests. Cancer is not found in the axillary lymph nodes by sentinel lymph node biopsy or lymph node dissection.
N3: Cancer is described as one of the following:
N3a: cancer has spread to 10 or more axillary lymph nodes and the cancer in at least one of the lymph nodes is larger than 2 millimeters, or cancer has spread to lymph nodes below the collarbone.
N3b: cancer has spread to 1 to 9 axillary lymph nodes and the cancer in at least one of the lymph nodes is larger than 2 millimeters. Cancer has also spread to lymph nodes near the breastbone and the cancer is found by imaging tests;
or
cancer has spread to 4 to 9 axillary lymph nodes and cancer in at least one of the lymph nodes is larger than 2 millimeters. Cancer has also spread to lymph nodes near the breastbone on the same side of the body as the primary tumor, and the cancer is larger than 0.2 millimeters and is found by sentinel lymph node biopsy.
N3c: cancer has spread to lymph nodes above the collarbone on the same side of the body as the primary tumor.
When the lymph nodes are checked using mammography or ultrasound, it is called clinical staging. The clinical staging of lymph nodes is not described here.
Metastasis (M). The spread of cancer to other parts of the body.
M0: There is no sign that cancer has spread to other parts of the body.
M1: Cancer has spread to other parts of the body, most often the bones, lungs, liver, or brain. If cancer has spread to distant lymph nodes, the cancer in the lymph nodes is larger than 0.2 millimeters. The cancer is called metastatic breast cancer.
The grading system is used to describe how quickly a breast tumor is likely to grow and spread.
The grading system describes a tumor based on how abnormal the cancer cells and tissue look under a microscope and how quickly the cancer cells are likely to grow and spread. Low-grade cancer cells look more like normal cells and tend to grow and spread more slowly than high-grade cancer cells. To describe how abnormal the cancer cells and tissue are, the pathologist will assess the following three features:
How much of the tumor tissue has normal breast ducts.
The size and shape of the nuclei in the tumor cells.
How many dividing cells are present, which is a measure of how fast the tumor cells are growing and dividing.
For each feature, the pathologist assigns a score of 1 to 3; a score of “1” means the cells and tumor tissue look the most like normal cells and tissue, and a score of “3” means the cells and tissue look the most abnormal. The scores for each feature are added together to get a total score between 3 and 9.
Total score of 6 to 7: G2 (Intermediate grade or moderately differentiated).
Total score of 8 to 9: G3 (High grade or poorly differentiated).
Biomarker testing is used to find out whether breast cancer cells have certain receptors.
Healthy breast cells, and some breast cancer cells, have receptors (biomarkers) that attach to the hormonesestrogen and progesterone. These hormones are needed for healthy cells, and some breast cancer cells, to grow and divide. To check for these biomarkers, samples of tissue containing breast cancer cells are removed during a biopsy or surgery. The samples are tested in a laboratory to see whether the breast cancer cells have estrogen or progesterone receptors.
Another type of receptor (biomarker) that is found on the surface of all breast cancer cells is called HER2. HER2 receptors are needed for the breast cancer cells to grow and divide.
For breast cancer, biomarker testing includes the following:
Estrogen receptor (ER). If the breast cancer cells have estrogen receptors, the cancer cells are called ER positive (ER+). If the breast cancer cells do not have estrogen receptors, the cancer cells are called ER negative (ER-).
Progesterone receptor (PR). If the breast cancer cells have progesterone receptors, the cancer cells are called PR positive (PR+). If the breast cancer cells do not have progesterone receptors, the cancer cells are called PR negative (PR-).
Human epidermal growth factor type 2 receptor (HER2/neu or HER2). If the breast cancer cells have larger than normal amounts of HER2 receptors on their surface, the cancer cells are called HER2 positive (HER2+). If the breast cancer cells have a normal amount of HER2 on their surface, the cancer cells are called HER2 negative (HER2-). HER2+ breast cancer is more likely to grow and divide faster than HER2- breast cancer.
Triple negative. If the breast cancer cells do not have estrogen receptors, progesterone receptors, or a larger than normal amount of HER2 receptors, the cancer cells are called triple negative.
Triple positive. If the breast cancer cells do have estrogen receptors, progesterone receptors, and a larger than normal amount of HER2 receptors, the cancer cells are called triple positive.
It is important to know the estrogen receptor, progesterone receptor, and HER2 receptor status to choose the best treatment. There are drugs that can stop the receptors from attaching to the hormones estrogen and progesterone and stop the cancer from growing. Other drugs may be used to block the HER2 receptors on the surface of the breast cancer cells and stop the cancer from growing.
The TNM system, the grading system, and biomarker status are combined to find out the breast cancer stage.
Here are 3 examples that combine the TNM system, the grading system, and the biomarker status to find out the Pathological Prognostic breast cancer stage for a woman whose first treatment was surgery:
If the tumor size is 30 millimeters (T2), has not spread to nearby lymph nodes (N0), has not spread to distant parts of the body (M0), and is:
Grade 1
HER2+
ER-
PR-
The cancer is stage IIA.
If the tumor size is 53 millimeters (T3), has spread to 4 to 9 axillary lymph nodes (N2), has not spread to other parts of the body (M0), and is:
Grade 2
HER2+
ER+
PR-
The tumor is stage IIIA.
If the tumor size is 65 millimeters (T3), has spread to 3 axillary lymph nodes (N1a), has spread to the lungs (M1), and is:
Grade 1
HER2+
ER-
PR-
The cancer is stage IV (metastatic breast cancer).
Talk to your doctor to find out what your breast cancer stage is and how it is used to plan the best treatment for you.
After surgery, your doctor will receive a pathology report that describes the size and location of the primary tumor, the spread of cancer to nearby lymph nodes, tumor grade, and whether certain biomarkers are present. The pathology report and other test results are used to determine your breast cancer stage.
You are likely to have many questions. Ask your doctor to explain how staging is used to decide the best options to treat your cancer and whether there are clinical trials that might be right for you.
Treatment Option Overview
Key Points
Treatment options for pregnant women depend on the stage of the disease and the trimester of the pregnancy.
Three types of standard treatment are used:
Surgery
Radiation therapy
Chemotherapy
Ending the pregnancy does not seem to improve the mother’s chance of survival.
Treatment for breast cancer may cause side effects.
Treatment options for pregnant women depend on the stage of the disease and the trimester of the pregnancy.
Modified radical mastectomy: Surgery to remove the whole breast that has cancer. This may include removal of the nipple, areola (the dark-colored skin around the nipple), and skin over the breast. Most of the lymph nodes under the arm are also removed. EnlargeModified radical mastectomy. The whole breast and most of the lymph nodes under the arm are removed.
Breast-conserving surgery: Surgery to remove the cancer and some normal tissue around it, but not the breast itself. Part of the chest wall lining may also be removed if the cancer is near it. This type of surgery may also be called lumpectomy, partial mastectomy, segmental mastectomy, quadrantectomy, or breast-sparing surgery. EnlargeLumpectomy. The tumor and some normal tissue around it are removed, but not the breast itself. Some lymph nodes under the arm may also be removed. If the cancer is near the chest wall, part of the chest wall lining may be removed as well.
After the doctor removes all of the cancer that can be seen at the time of surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. For pregnant women with early-stage breast cancer, radiation therapy and hormone therapy are given after the baby is born. Treatment given after 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. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.
External radiation therapy may be given to pregnant women with early stage (stage I or II) breast cancer after the baby is born. Women with late stage (stage III or IV) breast cancer may be given external radiation therapy after the first 3 months of pregnancy or, if possible, radiation therapy is delayed until after the baby is born.
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).
Chemotherapy is usually not given during the first 3 months of pregnancy. Chemotherapy given after this time does not usually harm the fetus but may cause early labor or low birth weight.
Pregnant women with early-stage breast cancer (stage I and stage II) are usually treated in the same way as patients who are not pregnant, with some changes to protect the fetus. Treatment may include the following:
Modified radical mastectomy or breast-conserving surgery during pregnancy. After the first 3 months of pregnancy, certain types of chemotherapy may be given before or after surgery.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment of Late-Stage Breast Cancer During Pregnancy
There is no standard treatment for patients with late-stage breast cancer (stage III or stage IV) during pregnancy. Treatment may include the following:
Radiation therapy and chemotherapy should not be given during the first 3 months of pregnancy.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Special Issues About Breast Cancer During Pregnancy
Key Points
Lactation (breast milk production) and breast-feeding should be stopped if surgery or chemotherapy is planned.
Breast cancer does not appear to harm the fetus.
Pregnancy does not seem to affect the survival of women who have had breast cancer in the past.
Lactation (breast milk production) and breast-feeding should be stopped if surgery or chemotherapy is planned.
If surgery is planned, breast-feeding should be stopped to reduce blood flow in the breasts and make them smaller. Many chemotherapydrugs, especially cyclophosphamide and methotrexate, may occur in high levels in breast milk and may harm the nursing baby. Women receiving chemotherapy should not breast-feed.
Stopping lactation does not improve the mother’s prognosis.
Pregnancy does not seem to affect the survival of women who have had breast cancer in the past.
For women who have had breast cancer, pregnancy does not seem to affect their survival. However, some doctors recommend that a woman wait 2 years after treatment for breast cancer before trying to have a baby, so that any early return of the cancer would be detected. This may affect a woman’s decision to become pregnant.
To Learn More About Breast Cancer During Pregnancy
For more information from the National Cancer Institute about breast cancer during pregnancy, see the following:
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Purpose of This Summary
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Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
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Male breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast.
A family history of breast cancer and other factors can increase a man’s risk of breast cancer.
Male breast cancer is sometimes caused by inherited gene mutations (changes).
Men with breast cancer usually have lumps that can be felt.
Tests that examine the breasts are used to diagnose breast cancer in men.
If cancer is found, tests are done to study the cancer cells.
Survival for men with breast cancer is similar to survival for women with breast cancer.
Certain factors affect prognosis (chance of recovery) and treatment options.
Male breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast.
Breast cancer may occur in men. Breast cancer may occur in men at any age, but it usually occurs in men between 60 and 70 years of age. Male breast cancer makes up less than 1% of all cases of breast cancer.
The following types of breast cancer are found in men:
Lobular carcinoma in situ (abnormal cells found in one of the lobes or sections of the breast), which sometimes occurs in women, has not been seen in men.
EnlargeAnatomy of the male breast. The nipple and areola are shown on the outside of the breast. The lymph nodes, fatty tissue, ducts, and other parts of the inside of the breast are also shown.
A family history of breast cancer and other factors can increase a man’s risk of breast 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 breast cancer in men may include the following:
Male breast cancer is sometimes caused by inherited gene mutations (changes).
The genes in cells carry the hereditary information that is received from a person’s parents. Hereditary breast cancer makes up about 5% to 10% of all breast cancer. Some mutated genes related to breast cancer, such as BRCA2, are more common in certain ethnic groups. Men who have a mutated gene related to breast cancer have an increased risk of this disease.
There are tests that can detect (find) mutated genes. These genetic tests are sometimes done for members of families with a high risk of cancer. See the following PDQ summaries for more information:
Scaly, red, or swollen skin on the breast, nipple, or areola (the dark area of skin around the nipple).
Dimples in the breast that look like the skin of an orange, called peau d’orange.
Tests that examine the breasts are used to diagnose breast cancer in men.
The following tests and procedures may be used:
Physical exam and health history: An exam of the body to check general signs of health, including checking 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.
Clinical breast exam (CBE): An exam of the breast by a doctor or other health professional. The doctor will carefully feel the breasts and under the arms for lumps or anything else that seems unusual.
Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of both breasts. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. There are four types of biopsies to check for breast cancer:
HER2 test: A laboratory test to measure how many HER2/neu genes there are and how much HER2/neu protein is made in a sample of tissue. If there are more HER2/neu genes or higher levels of HER2/neu protein than normal, the cancer is called HER2/neu positive. This type of breast cancer may grow more quickly and is more likely to spread to other parts of the body. The cancer may be treated with drugs that target the HER2/neu protein, such as trastuzumab and pertuzumab.
Survival for men with breast cancer is similar to survival for women with breast cancer.
Survival for men with breast cancer is similar to that for women with breast cancer when their stage at diagnosis is the same. Breast cancer in men, however, is often diagnosed at a later stage. Cancer found at a later stage may be less likely to be cured.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis and treatment options depend on the following:
The stage of the cancer (the size of the tumor and whether it is in the breast only or has spread to lymph nodes or other places in the body).
The type of breast cancer.
Estrogen-receptor and progesterone-receptor levels in the tumor tissue.
Whether the cancer is also found in the other breast.
The man’s age and general health.
Whether the cancer has just been diagnosed or has recurred (come back).
Stages of Male Breast Cancer
Key Points
After breast cancer has been diagnosed, tests are done to find out if cancer cells have spread within the breast 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 breast cancer, stage is based on the size and location of the primary tumor, the spread of cancer to nearby lymph nodes or other parts of the body, tumor grade, and whether certain biomarkers are present.
The TNM system is used to describe the size of the primary tumor and the spread of cancer to nearby lymph nodes or other parts of the body.
Tumor (T). The size and location of the tumor.
Lymph Node (N). The size and location of lymph nodes where cancer has spread.
Metastasis (M). The spread of cancer to other parts of the body.
The grading system is used to describe how quickly a breast tumor is likely to grow and spread.
Biomarker testing is used to find out whether breast cancer cells have certain receptors.
The TNM system, the grading system, and biomarker status are combined to find out the breast cancer stage.
Talk to your doctor to find out what your breast cancer stage is and how it is used to plan the best treatment for you.
The treatment of male breast cancer depends partly on the stage of the disease.
After breast cancer has been diagnosed, tests are done to find out if cancer cells have spread within the breast or to other parts of the body.
After breast cancer has been diagnosed, tests are done to find out if cancercells have spread within the breast or to other parts of the body. This process 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. Breast cancer in men is staged the same as it is in women. The spread of cancer from the breast to lymph nodes and other parts of the body appears to be similar in men and women.
The following tests and procedures may be used in the staging process:
Sentinel lymph node biopsy: The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node in a group of lymph nodes to receive lymphatic drainage from the primary tumor. It is the first lymph node the cancer is likely to spread to from the primary 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. Sometimes, a sentinel lymph node is found in more than one group of nodes.
Chest x-ray: An x-ray of the organs and bones inside the chest. 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.
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.
Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner.
PET scan (positron emission tomography scan): A procedure to find malignanttumor 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.
There are three ways that cancer spreads in the body.
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 breast cancer spreads to the bone, the cancer cells in the bone are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.
In breast cancer, stage is based on the size and location of the primary tumor, the spread of cancer to nearby lymph nodes or other parts of the body, tumor grade, and whether certain biomarkers are present.
To plan the best treatment and understand your prognosis, it is important to know the breast cancer stage.
Pathological Prognostic Stage is then used for patients who have surgery as their first treatment. The Pathological Prognostic Stage is based on all clinical information, biomarker status, and laboratory test results from breast tissue and lymph nodes removed during surgery.
Anatomic Stage is based on the size and the spread of cancer as described by the TNM system. The Anatomic Stage is used in parts of the world where biomarker testing is not available. It is not used in the United States.
The TNM system is used to describe the size of the primary tumor and the spread of cancer to nearby lymph nodes or other parts of the body.
For breast cancer, the TNM system describes the tumor as follows:
Tumor (T). The size and location of the tumor.
EnlargeTumor sizes are often measured in millimeters (mm) or centimeters. Common items that can be used to show tumor size in mm include: a sharp pencil point (1 mm), a new crayon point (2 mm), a pencil-top eraser (5 mm), a pea (10 mm), a peanut (20 mm), and a lime (50 mm).
TX: Primary tumor cannot be assessed.
T0: No sign of a primary tumor in the breast.
Tis: Carcinoma in situ. There are 2 types of breast carcinoma in situ:
Tis (DCIS): DCIS is a condition in which abnormal cells are found in the lining of a breast duct. The abnormal cells have not spread outside the duct to other tissues in the breast. In some cases, DCIS may become invasive breast cancer that is able to spread to other tissues. At this time, there is no way to know which lesions can become invasive.
Tis (Paget disease): Paget disease of the nipple is a condition in which abnormal cells are found in the skin cells of the nipple and may spread to the areola. It is not staged according to the TNM system. If Paget disease AND an invasive breast cancer are present, the TNM system is used to stage the invasive breast cancer.
T1: The tumor is 20 millimeters or smaller. There are 4 subtypes of a T1 tumor depending on the size of the tumor:
T1mi: the tumor is 1 millimeter or smaller.
T1a: the tumor is larger than 1 millimeter but not larger than 5 millimeters.
T1b: the tumor is larger than 5 millimeters but not larger than 10 millimeters.
T1c: the tumor is larger than 10 millimeters but not larger than 20 millimeters.
T2: The tumor is larger than 20 millimeters but not larger than 50 millimeters.
T3: The tumor is larger than 50 millimeters.
T4: The tumor is described as one of the following:
T4b: the tumor has grown into the skin—an ulcer has formed on the surface of the skin on the breast, small tumor nodules have formed in the same breast as the primary tumor, and/or there is swelling of the skin on the breast.
T4c: the tumor has grown into the chest wall and the skin.
Lymph Node (N). The size and location of lymph nodes where cancer has spread.
When the lymph nodes are removed by surgery and studied under a microscope by a pathologist, pathologic staging is used to describe the lymph nodes. The pathologic staging of lymph nodes is described below.
NX: The lymph nodes cannot be assessed.
N0: No sign of cancer in the lymph nodes, or tiny clusters of cancer cells not larger than 0.2 millimeters in the lymph nodes.
N1a: cancer has spread to 1 to 3 axillary lymph nodes and the cancer in at least one of the lymph nodes is larger than 2 millimeters.
N1b: cancer has spread to lymph nodes near the breastbone on the same side of the body as the primary tumor, and the cancer is larger than 0.2 millimeters and is found by sentinel lymph node biopsy. Cancer is not found in the axillary lymph nodes.
N1c: cancer has spread to 1 to 3 axillary lymph nodes and the cancer in at least one of the lymph nodes is larger than 2 millimeters. Cancer is also found by sentinel lymph node biopsy in the lymph nodes near the breastbone on the same side of the body as the primary tumor.
N2: Cancer is described as one of the following:
N2a: cancer has spread to 4 to 9 axillary lymph nodes and the cancer in at least one of the lymph nodes is larger than 2 millimeters.
N2b: cancer has spread to lymph nodes near the breastbone and the cancer is found by imaging tests. Cancer is not found in the axillary lymph nodes by sentinel lymph node biopsy or lymph node dissection.
N3: Cancer is described as one of the following:
N3a: cancer has spread to 10 or more axillary lymph nodes and the cancer in at least one of the lymph nodes is larger than 2 millimeters, or cancer has spread to lymph nodes below the collarbone.
N3b: cancer has spread to 1 to 9 axillary lymph nodes and the cancer in at least one of the lymph nodes is larger than 2 millimeters. Cancer has also spread to lymph nodes near the breastbone and the cancer is found by imaging tests;
or
cancer has spread to 4 to 9 axillary lymph nodes and cancer in at least one of the lymph nodes is larger than 2 millimeters. Cancer has also spread to lymph nodes near the breastbone on the same side of the body as the primary tumor, and the cancer is larger than 0.2 millimeters and is found by sentinel lymph node biopsy.
N3c: cancer has spread to lymph nodes above the collarbone on the same side of the body as the primary tumor.
When the lymph nodes are checked using mammography or ultrasound, it is called clinical staging. The clinical staging of lymph nodes is not described here.
Metastasis (M). The spread of cancer to other parts of the body.
M0: There is no sign that cancer has spread to other parts of the body.
M1: Cancer has spread to other parts of the body, most often the bones, lungs, liver, or brain. If cancer has spread to distant lymph nodes, the cancer in the lymph nodes is larger than 0.2 millimeters. The cancer is called metastatic breast cancer.
The grading system is used to describe how quickly a breast tumor is likely to grow and spread.
The grading system describes a tumor based on how abnormal the cancer cells and tissue look under a microscope and how quickly the cancer cells are likely to grow and spread. Low-grade cancer cells look more like normal cells and tend to grow and spread more slowly than high-grade cancer cells. To describe how abnormal the cancer cells and tissue are, the pathologist will assess the following three features:
How much of the tumor tissue has normal breast ducts.
The size and shape of the nuclei in the tumor cells.
How many dividing cells are present, which is a measure of how fast the tumor cells are growing and dividing.
For each feature, the pathologist assigns a score of 1 to 3; a score of “1” means the cells and tumor tissue look the most like normal cells and tissue, and a score of “3” means the cells and tissue look the most abnormal. The scores for each feature are added together to get a total score between 3 and 9.
Total score of 6 to 7: G2 (Intermediate grade or moderately differentiated).
Total score of 8 to 9: G3 (High grade or poorly differentiated).
Biomarker testing is used to find out whether breast cancer cells have certain receptors.
Healthy breast cells, and some breast cancer cells, have receptors (biomarkers) that attach to the hormonesestrogen and progesterone. These hormones are needed for healthy cells, and some breast cancer cells, to grow and divide. To check for these biomarkers, samples of tissue containing breast cancer cells are removed during a biopsy or surgery. The samples are tested in a laboratory to see whether the breast cancer cells have estrogen or progesterone receptors.
Another type of receptor (biomarker) that is found on the surface of all breast cancer cells is called HER2. HER2 receptors are needed for the breast cancer cells to grow and divide.
For breast cancer, biomarker testing includes the following:
Estrogen receptor (ER). If the breast cancer cells have estrogen receptors, the cancer cells are called ER positive (ER+). If the breast cancer cells do not have estrogen receptors, the cancer cells are called ER negative (ER-).
Progesterone receptor (PR). If the breast cancer cells have progesterone receptors, the cancer cells are called PR positive (PR+). If the breast cancer cells do not have progesterone receptors, the cancer cells are called PR negative (PR-).
Human epidermal growth factor type 2 receptor (HER2/neu or HER2). If the breast cancer cells have larger than normal amounts of HER2 receptors on their surface, the cancer cells are called HER2 positive (HER2+). If the breast cancer cells have a normal amount of HER2 on their surface, the cancer cells are called HER2 negative (HER2-). HER2+ breast cancer is more likely to grow and divide faster than HER2- breast cancer.
Triple negative. If the breast cancer cells do not have estrogen receptors, progesterone receptors, or a larger than normal amount of HER2 receptors, the cancer cells are called triple negative.
Triple positive. If the breast cancer cells do have estrogen receptors, progesterone receptors, and a larger than normal amount of HER2 receptors, the cancer cells are called triple positive.
It is important to know the estrogen receptor, progesterone receptor, and HER2 receptor status to choose the best treatment. There are drugs that can stop the receptors from attaching to the hormones estrogen and progesterone and stop the cancer from growing. Other drugs may be used to block the HER2 receptors on the surface of the breast cancer cells and stop the cancer from growing.
The TNM system, the grading system, and biomarker status are combined to find out the breast cancer stage.
Here are 3 examples that combine the TNM system, the grading system, and the biomarker status to find out the Pathological Prognostic breast cancer stage for a woman whose first treatment was surgery:
If the tumor size is 30 millimeters (T2), has not spread to nearby lymph nodes (N0), has not spread to distant parts of the body (M0), and is:
Grade 1
HER2+
ER-
PR-
The cancer is stage IIA.
If the tumor size is 53 millimeters (T3), has spread to 4 to 9 axillary lymph nodes (N2), has not spread to other parts of the body (M0), and is:
Grade 2
HER2+
ER+
PR-
The tumor is stage IIIA.
If the tumor size is 65 millimeters (T3), has spread to 3 axillary lymph nodes (N1a), has spread to the lungs (M1), and is:
Grade 1
HER2+
ER-
PR-
The cancer is stage IV (metastatic breast cancer).
Talk to your doctor to find out what your breast cancer stage is and how it is used to plan the best treatment for you.
After surgery, your doctor will receive a pathology report that describes the size and location of the primary tumor, the spread of cancer to nearby lymph nodes, tumor grade, and whether certain biomarkers are present. The pathology report and other test results are used to determine your breast cancer stage.
You are likely to have many questions. Ask your doctor to explain how staging is used to decide the best options to treat your cancer and whether there are clinical trials that might be right for you.
The treatment of male breast cancer depends partly on the stage of the disease.
For treatment options for stage IV (metastatic) breast cancer or breast cancer that has recurred in other parts of the body, see Treatment of Metastatic Male Breast Cancer.
Inflammatory Male Breast Cancer
In inflammatory breast cancer, cancer has spread to the skin of the breast and the breast looks red and swollen and feels warm. The redness and warmth occur because the cancer cells block the lymph vessels in the skin. The skin of the breast may also show the dimpled appearance called peau d’orange (like the skin of an orange). There may not be any lumps in the breast that can be felt. Inflammatory breast cancer may be stage IIIB, stage IIIC, or stage IV.
Treatment Option Overview
Key Points
There are different types of treatment for men with breast cancer.
Five types of standard treatment are used to treat men with breast cancer:
Surgery
Chemotherapy
Hormone therapy
Radiation therapy
Targeted therapy
Treatment for male breast cancer may cause side effects.
There are different types of treatment for men with breast cancer.
Different types of treatment are available for men with breast 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.
For some patients, taking part in a clinical trial may be the best treatment choice. 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.
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. Information about clinical trials is available from the NCI website. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.
Five types of standard treatment are used to treat men with breast cancer:
Surgery
Surgery for men with breast cancer is usually a modified radical mastectomy, surgery to remove the whole breast that has cancer. This may include removal of the nipple, areola (the dark-colored skin around the nipple), and skin over the breast. Most of the lymph nodes under the arm are also removed.
EnlargeModified radical mastectomy. The whole breast and most of the lymph nodes under the arm are removed.
Breast-conserving surgery, an operation to remove the cancer but not the breast itself, is also used for some men with breast cancer. A lumpectomy is done to remove the tumor (lump) and a small amount of normal tissue around it. Radiation therapy is given after surgery to kill any cancer cells that are left.
EnlargeLumpectomy. The tumor and some normal tissue around it are removed, but not the breast itself. Some lymph nodes under the arm may also be removed. If the cancer is near the chest wall, part of the chest wall lining may be removed as well.
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them 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).
Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working.
Hormone therapy with an aromatase inhibitor is given to some men who have metastatic breast cancer. Aromatase inhibitors decrease the body’s estrogen by blocking an enzyme called aromatase from turning androgen into estrogen. Anastrozole, letrozole, and exemestane are types of aromatase inhibitors.
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. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.
Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.
Types of monoclonal antibody therapy include the following:
How do monoclonal antibodies work to treat cancer? This video shows how monoclonal antibodies, such as trastuzumab, pembrolizumab, and rituximab, block molecules cancer cells need to grow, flag cancer cells for destruction by the body’s immune system, or deliver harmful substances to cancer cells.
Tyrosine kinase inhibitors are targeted therapy drugs that block signals needed for tumors to grow. Lapatinib is a tyrosine kinase inhibitor that may be used to treat men with metastatic breast cancer.
Cyclin-dependent kinase inhibitors are targeted therapy drugs that block proteins called cyclin-dependent kinases, which cause the growth of cancer cells. Palbociclib is a cyclin-dependent kinase inhibitor used to treat men with metastatic breast cancer.
Mammalian target of rapamycin (mTOR) inhibitors block a protein called mTOR, which may keep cancer cells from growing and prevent the growth of new blood vessels that tumors need to grow.
Node-negative: For men whose cancer is node-negative (cancer has not spread to the lymph nodes), adjuvant therapy should be considered on the same basis as for a woman with breast cancer because there is no evidence that response to therapy is different for men and women.
Node-positive: For men whose cancer is node-positive (cancer has spread to the lymph nodes), adjuvant therapy may include the following:
Chemotherapy.
Hormone therapy with tamoxifen (to block the effect of estrogen) or less often, aromatase inhibitors (to reduce the amount of estrogen in the body).
These treatments appear to increase survival in men as they do in women. The patient’s response to hormone therapy depends on whether there are hormonereceptors (proteins) in the tumor. Most breast cancers in men have these receptors. Hormone therapy is usually recommended for male breast cancer patients, but it can have many side effects, including hot flashes and impotence (the inability to have an erection adequate for sexual intercourse).
Treatment of Locoregional Recurrent Male Breast Cancer
Treatment options for metastaticbreast cancer (cancer that has spread to distant parts of the body) may include the following:
Hormone therapy
In men who have just been diagnosed with metastatic breast cancer that is hormone receptor positive or if the hormone receptor status is not known, treatment may include:
In men whose tumors are hormone receptor positive or hormone receptor unknown, with spread to the bone or soft tissue only, and who have been treated with tamoxifen, treatment may include:
Aromatase inhibitor therapy with or without LHRH agonist.
In men with metastatic breast cancer that is hormone receptor positive and has not responded to other treatments, options may include targeted therapy such as:
Cyclin-dependent kinase inhibitor therapy (palbociclib) combined with letrozole.
In men with metastatic breast cancer that is HER2/neu positive, treatment may include:
Targeted therapy such as trastuzumab, pertuzumab, ado-trastuzumab emtansine, or lapatinib.
Chemotherapy
In men with metastatic breast cancer that is hormone receptor negative, has not responded to hormone therapy, has spread to other organs or has caused symptoms, treatment may include:
Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of male breast cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” 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.
Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Male Breast Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/breast/patient/male-breast-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389417]
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The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
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Breast cancer is the most common cancer in pregnant and postpartum women and occurs in about 1 in 3,000 pregnant women. The average patient is between the ages of 32 years and 38 years. Because many women are choosing to delay childbearing, it is likely that the incidence of breast cancer during pregnancy will increase.
Anatomy
EnlargeThe female breast contains lobes, lobules, and ducts that produce and transport milk to the nipple. Fatty tissue gives the breast its shape, while muscles and the chest wall provide support. The lymphatic system, including lymph nodes, filter lymph and store white blood cells that help fight infection and disease.
Diagnostic Evaluation
The natural tenderness and engorgement of the breasts of pregnant and lactating women may hinder detection of discrete masses and early diagnosis of breast cancer. Delays in diagnosis are common, with an average reported delay of 5 to 15 months from the onset of symptoms.[1–4] Because of this delay, cancers are typically detected at a later stage than in a nonpregnant, age-matched population.[5]
The following tests and procedures may be used to diagnose breast cancer during pregnancy:
Breast self-examination.
Clinical breast examination.
Ultrasound.
Biopsy and hormone receptor assays.
Mammography.
To detect breast cancer, pregnant and lactating women should consider practicing self-examination and undergo a clinical breast examination as part of the routine prenatal examination by a doctor. If an abnormality is found, diagnostic approaches such as ultrasound and mammography may be used. With proper shielding, mammography poses little risk of radiation exposure to the fetus.[6] However, mammograms are only used to evaluate dominant masses and to locate occult carcinomas in the presence of other suspicious physical findings.[6]
Because at least 25% of mammograms in pregnancy may be negative in the presence of cancer, a biopsy is essential for the diagnosis of any palpable mass. Diagnosis may be safely accomplished with a fine-needle aspiration, core biopsy, or excisional biopsy under local anesthesia. To avoid a false-positive diagnosis as a result of misinterpretation of pregnancy-related changes, the pathologist should be advised that the patient is pregnant.[7]
Breast cancer pathology is similar in age-matched pregnant and nonpregnant women. Hormone receptor assays using a competitive binding assay are usually negative in pregnant patients with breast cancer, but this may be the result of receptor binding by high serum estrogen levels associated with the pregnancy. Enzyme immunocytochemical receptor assays are more sensitive than competitive binding assays. A study that used both assay methods indicated similar receptor positivity between pregnant and nonpregnant women with breast cancer.[8] The study concluded that increased estrogen levels during pregnancy could result in a higher incidence of receptor positivity detected with immunohistochemistry than is detected by radiolabeled ligand-binding assay because of competitive inhibition by high levels of endogenous estrogen.
For more information, see the Diagnosis section in Breast Cancer Treatment.
Prognosis
The overall survival of pregnant women with breast cancer may be worse than that of nonpregnant women at all stages.[6] However, this discrepancy may be primarily the result of delayed diagnosis.[9] Termination of pregnancy has not shown any beneficial effect on breast cancer outcome and is not usually considered as a therapeutic option.[1,2,4,10,11]
References
Hoover HC: Breast cancer during pregnancy and lactation. Surg Clin North Am 70 (5): 1151-63, 1990. [PUBMED Abstract]
Gwyn K, Theriault R: Breast cancer during pregnancy. Oncology (Huntingt) 15 (1): 39-46; discussion 46, 49-51, 2001. [PUBMED Abstract]
Moore HC, Foster RS: Breast cancer and pregnancy. Semin Oncol 27 (6): 646-53, 2000. [PUBMED Abstract]
Rugo HS: Management of breast cancer diagnosed during pregnancy. Curr Treat Options Oncol 4 (2): 165-73, 2003. [PUBMED Abstract]
Clark RM, Chua T: Breast cancer and pregnancy: the ultimate challenge. Clin Oncol (R Coll Radiol) 1 (1): 11-8, 1989. [PUBMED Abstract]
Yang WT, Dryden MJ, Gwyn K, et al.: Imaging of breast cancer diagnosed and treated with chemotherapy during pregnancy. Radiology 239 (1): 52-60, 2006. [PUBMED Abstract]
Middleton LP, Amin M, Gwyn K, et al.: Breast carcinoma in pregnant women: assessment of clinicopathologic and immunohistochemical features. Cancer 98 (5): 1055-60, 2003. [PUBMED Abstract]
Elledge RM, Ciocca DR, Langone G, et al.: Estrogen receptor, progesterone receptor, and HER-2/neu protein in breast cancers from pregnant patients. Cancer 71 (8): 2499-506, 1993. [PUBMED Abstract]
Petrek JA, Dukoff R, Rogatko A: Prognosis of pregnancy-associated breast cancer. Cancer 67 (4): 869-72, 1991. [PUBMED Abstract]
Barnavon Y, Wallack MK: Management of the pregnant patient with carcinoma of the breast. Surg Gynecol Obstet 171 (4): 347-52, 1990. [PUBMED Abstract]
Gallenberg MM, Loprinzi CL: Breast cancer and pregnancy. Semin Oncol 16 (5): 369-76, 1989. [PUBMED Abstract]
Stage Information for Breast Cancer Treatment and Pregnancy
Staging Evaluation
The following procedures are used to determine the extent of the cancer:
Chest x-ray.
Bone scan.
Ultrasound of the liver.
Magnetic resonance imaging (MRI) of the brain.
Procedures used for determining the stage of breast cancer are modified for pregnant women to avoid radiation exposure to the fetus. Nuclear scans cause fetal radiation exposure.[1] If such scans are essential for evaluation, hydration and Foley catheter drainage of the bladder can be used to prevent retention of radioactivity. Timing of the exposure to radiation relative to the gestational age of the fetus may be more critical than the actual dose of radiation delivered.[2] Radiation exposure during the first trimester (>0.1 Gy) may lead to congenital malformations, intellectual disability, and increased relative risk of carcinogenesis.
Chest x-rays with abdominal shielding are considered safe, but as with all radiological procedures, they are used only when essential for making treatment decisions.[1,3] A chest x-ray delivers 0.00008 Gy.[4]
For the diagnosis of bone metastases, a bone scan is preferable to a skeletal series because the bone scan delivers a smaller amount of radiation and is more sensitive. A bone scan delivers 0.001 Gy.[5]
Evaluation of the liver can be performed with ultrasound, and brain metastases can be diagnosed with an MRI scan. Data on MRI during pregnancy are not available, but gadolinium crosses the placenta and is associated with fetal abnormalities in rats.[5]
American Joint Committee on Cancer (AJCC) Stage Groupings and Definitions of TNM
Gwyn K, Theriault R: Breast cancer during pregnancy. Oncology (Huntingt) 15 (1): 39-46; discussion 46, 49-51, 2001. [PUBMED Abstract]
Barnavon Y, Wallack MK: Management of the pregnant patient with carcinoma of the breast. Surg Gynecol Obstet 171 (4): 347-52, 1990. [PUBMED Abstract]
Nicklas AH, Baker ME: Imaging strategies in the pregnant cancer patient. Semin Oncol 27 (6): 623-32, 2000. [PUBMED Abstract]
Gallenberg MM, Loprinzi CL: Breast cancer and pregnancy. Semin Oncol 16 (5): 369-76, 1989. [PUBMED Abstract]
Yang WT, Dryden MJ, Gwyn K, et al.: Imaging of breast cancer diagnosed and treated with chemotherapy during pregnancy. Radiology 239 (1): 52-60, 2006. [PUBMED Abstract]
Treatment of Early/Localized/Operable Breast Cancer During Pregnancy
Generally, pregnant women with stage I or stage II breast cancer are treated in the same way as nonpregnant patients, with some modifications to protect the fetus.
Treatment options for early/localized/operable breast cancer in pregnant women include:
Surgery. Postpartum radiation therapy may also be given to women diagnosed with breast cancer late in pregnancy.
The use of trastuzumab during pregnancy is contraindicated.
Surgery
Surgery is recommended as the primary treatment of breast cancer in pregnant women.
The data regarding safety of sentinel lymph node biopsy in pregnant patients are limited to several retrospective case series. One study examined sentinel lymph node biopsy in eight patients in the first trimester, nine patients in the second trimester, and eight patients in the third trimester. Technetium Tc 99m alone was used in 16 patients, methylene blue dye alone was used in seven patients, and two patients had unknown mapping methods. All 25 patients had live-born infants, of whom 24 were healthy, and one had a cleft palate (in the setting of other maternal risk factors).[1]
Because radiation in therapeutic doses may expose the fetus to potentially harmful scatter radiation,[2] modified radical mastectomy is the treatment of choice if the breast cancer was diagnosed early in pregnancy. If diagnosed late in pregnancy, breast-conserving surgery with postpartum radiation therapy has been used for breast preservation.[3] An analysis has been performed that helps to predict the risk of waiting to have radiation.[4,5]
Chemotherapy
Data suggest that it is safe to administer certain chemotherapeutic drugs after the first trimester, with most pregnancies resulting in live births with low rates of morbidity in the newborns.
Anthracycline-based chemotherapy (doxorubicin plus cyclophosphamide or fluorouracil, doxorubicin, and cyclophosphamide [FAC]) appears to be safe to administer during the second and/or third trimester on the basis of limited prospective data.[6–8] Safety data on the use of taxanes during pregnancy are limited.
Evidence (use of chemotherapy during the second and/or third trimester of pregnancy):
A multicenter case-control study compared pediatric outcomes of 129 children whose mothers had breast cancer with matched children of women without cancer. In the pregnancy study group, 96 children (74.4%) were exposed to chemotherapy, 11 (8.5%) to radiation therapy, 13 (10.1%) to surgery alone, 2 (1.7%) to other drug treatments, and 14 (10.9%) to no treatment.[9]
The study showed that there was no significant difference in birth weight below the 10th percentile (22% in the breast cancer treatment‒exposed group vs. 15.2% in the control group, P = .16) or in cognitive development based on the Bayley score (P = .08). The gestational age at birth was correlated with cognitive outcome in the two study groups.
Evaluation of cardiac function among 47 children, who were age 36 months in the study group, showed normal cardiac findings.
In a prospective single-arm study, 57 pregnant patients with breast cancer were treated with FAC in the adjuvant or neoadjuvant setting.[6]
Survey data collected when the children were aged 2 months to 157 months revealed that no stillbirths, miscarriages, or perinatal deaths occurred.
One child born vaginally at a gestational age of 38 weeks had a subarachnoid hemorrhage on day 2 postpartum, one child had Down syndrome, and two children had congenital anomalies (club foot and bilateral ureteral reflux).
The findings of the prospective single-arm study above were consistent with other smaller retrospective series of anthracycline-based chemotherapy.[7,8]
A systematic review studied 40 case reports of taxane administration during the second or third trimesters of pregnancy.[10]
Minimal maternal, fetal, or neonatal toxicity was observed.
Fluorouracil dosing
The DPYD gene encodes an enzyme that catabolizes pyrimidines and fluoropyrimidines, like capecitabine and fluorouracil. An estimated 1% to 2% of the population has germline pathogenic variants in DPYD, which lead to reduced DPD protein function and an accumulation of pyrimidines and fluoropyrimidines in the body.[11,12] Patients with the DPYD*2A variant who receive fluoropyrimidines may experience severe, life-threatening toxicities that are sometimes fatal. Many other DPYD variants have been identified, with a range of clinical effects.[11–13] Fluoropyrimidine avoidance or a dose reduction of 50% may be recommended based on the patient’s DPYD genotype and number of functioning DPYD alleles.[14–16] DPYD genetic testing costs less than $200, but insurance coverage varies due to a lack of national guidelines.[17] In addition, testing may delay therapy by 2 weeks, which would not be advisable in urgent situations. This controversial issue requires further evaluation.[18]
Endocrine Therapy
Endocrine therapy is generally avoided until after delivery. Case reports and a literature review of tamoxifen during pregnancy show that tamoxifen administration during pregnancy is associated with vaginal bleeding, miscarriage, congenital abnormalities such as Goldenhar syndrome, and fetal death.[19–21] Breastfeeding is also not recommended concurrently with endocrine therapy.[22]
Targeted Therapy
The use of trastuzumab during pregnancy is contraindicated based on results of a systematic review of 17 studies (18 pregnancies, 19 newborns).[23] Of the fetal complications noted, occurrence of oligohydramnios/anhydramnios was the most common (61.1%) adverse event. Of the pregnancies exposed to trastuzumab during the second or third trimester, 73.3% of the pregnancies were complicated with oligohydramnios/anhydramnios. Of the pregnancies exposed to trastuzumab exclusively during the first trimester, 0% (P = .043) of the pregnancies were complicated with oligohydramnios/anhydramnios. The mean gestational age at delivery was 33.8 weeks, and the mean weight of newborns at delivery was 2,261 grams or 4.984 pounds. In 52.6% of cases, a healthy neonate was born. At the long-term evaluation, all children who were without complications at birth were healthy, with a median follow-up of 9 months, and four of nine children with complications at birth had died within an interval ranging from birth to 5.25 months. All children exposed to trastuzumab in utero exclusively in the first trimester were completely healthy at birth. The data suggest that for women who become pregnant during trastuzumab administration and wish to continue pregnancy, trastuzumab should be stopped and pregnancy would be allowed to continue.
References
Gropper AB, Calvillo KZ, Dominici L, et al.: Sentinel lymph node biopsy in pregnant women with breast cancer. Ann Surg Oncol 21 (8): 2506-11, 2014. [PUBMED Abstract]
Kal HB, Struikmans H: Radiotherapy during pregnancy: fact and fiction. Lancet Oncol 6 (5): 328-33, 2005. [PUBMED Abstract]
Gwyn K, Theriault R: Breast cancer during pregnancy. Oncology (Huntingt) 15 (1): 39-46; discussion 46, 49-51, 2001. [PUBMED Abstract]
Nettleton J, Long J, Kuban D, et al.: Breast cancer during pregnancy: quantifying the risk of treatment delay. Obstet Gynecol 87 (3): 414-8, 1996. [PUBMED Abstract]
Kuerer HM, Gwyn K, Ames FC, et al.: Conservative surgery and chemotherapy for breast carcinoma during pregnancy. Surgery 131 (1): 108-10, 2002. [PUBMED Abstract]
Hahn KM, Johnson PH, Gordon N, et al.: Treatment of pregnant breast cancer patients and outcomes of children exposed to chemotherapy in utero. Cancer 107 (6): 1219-26, 2006. [PUBMED Abstract]
Turchi JJ, Villasis C: Anthracyclines in the treatment of malignancy in pregnancy. Cancer 61 (3): 435-40, 1988. [PUBMED Abstract]
Zemlickis D, Lishner M, Degendorfer P, et al.: Fetal outcome after in utero exposure to cancer chemotherapy. Arch Intern Med 152 (3): 573-6, 1992. [PUBMED Abstract]
Amant F, Vandenbroucke T, Verheecke M, et al.: Pediatric Outcome after Maternal Cancer Diagnosed during Pregnancy. N Engl J Med 373 (19): 1824-34, 2015. [PUBMED Abstract]
Mir O, Berveiller P, Goffinet F, et al.: Taxanes for breast cancer during pregnancy: a systematic review. Ann Oncol 21 (2): 425-6, 2010. [PUBMED Abstract]
Sharma BB, Rai K, Blunt H, et al.: Pathogenic DPYD Variants and Treatment-Related Mortality in Patients Receiving Fluoropyrimidine Chemotherapy: A Systematic Review and Meta-Analysis. Oncologist 26 (12): 1008-1016, 2021. [PUBMED Abstract]
Lam SW, Guchelaar HJ, Boven E: The role of pharmacogenetics in capecitabine efficacy and toxicity. Cancer Treat Rev 50: 9-22, 2016. [PUBMED Abstract]
Shakeel F, Fang F, Kwon JW, et al.: Patients carrying DPYD variant alleles have increased risk of severe toxicity and related treatment modifications during fluoropyrimidine chemotherapy. Pharmacogenomics 22 (3): 145-155, 2021. [PUBMED Abstract]
Amstutz U, Henricks LM, Offer SM, et al.: Clinical Pharmacogenetics Implementation Consortium (CPIC) Guideline for Dihydropyrimidine Dehydrogenase Genotype and Fluoropyrimidine Dosing: 2017 Update. Clin Pharmacol Ther 103 (2): 210-216, 2018. [PUBMED Abstract]
Henricks LM, Lunenburg CATC, de Man FM, et al.: DPYD genotype-guided dose individualisation of fluoropyrimidine therapy in patients with cancer: a prospective safety analysis. Lancet Oncol 19 (11): 1459-1467, 2018. [PUBMED Abstract]
Lau-Min KS, Varughese LA, Nelson MN, et al.: Preemptive pharmacogenetic testing to guide chemotherapy dosing in patients with gastrointestinal malignancies: a qualitative study of barriers to implementation. BMC Cancer 22 (1): 47, 2022. [PUBMED Abstract]
Brooks GA, Tapp S, Daly AT, et al.: Cost-effectiveness of DPYD Genotyping Prior to Fluoropyrimidine-based Adjuvant Chemotherapy for Colon Cancer. Clin Colorectal Cancer 21 (3): e189-e195, 2022. [PUBMED Abstract]
Baker SD, Bates SE, Brooks GA, et al.: DPYD Testing: Time to Put Patient Safety First. J Clin Oncol 41 (15): 2701-2705, 2023. [PUBMED Abstract]
Cullins SL, Pridjian G, Sutherland CM: Goldenhar’s syndrome associated with tamoxifen given to the mother during gestation. JAMA 271 (24): 1905-6, 1994 Jun 22-29. [PUBMED Abstract]
Tewari K, Bonebrake RG, Asrat T, et al.: Ambiguous genitalia in infant exposed to tamoxifen in utero. Lancet 350 (9072): 183, 1997. [PUBMED Abstract]
Isaacs RJ, Hunter W, Clark K: Tamoxifen as systemic treatment of advanced breast cancer during pregnancy–case report and literature review. Gynecol Oncol 80 (3): 405-8, 2001. [PUBMED Abstract]
Helewa M, Lévesque P, Provencher D, et al.: Breast cancer, pregnancy, and breastfeeding. J Obstet Gynaecol Can 24 (2): 164-80; quiz 181-4, 2002. [PUBMED Abstract]
Zagouri F, Sergentanis TN, Chrysikos D, et al.: Trastuzumab administration during pregnancy: a systematic review and meta-analysis. Breast Cancer Res Treat 137 (2): 349-57, 2013. [PUBMED Abstract]
Treatment of Advanced Breast Cancer During Pregnancy
There is no standard treatment for patients with advanced (stage III or stage IV) breast cancer during pregnancy. Most studies show a 5-year survival rate of 10% in pregnant patients with stage III or IV disease.
Because the mother’s life span may be limited, and there is a risk of fetal damage with treatment during the first trimester,[1,2] issues regarding continuation of the pregnancy should be discussed with the patient and her family. Therapeutic abortion does not improve prognosis.[1–5]
References
Hoover HC: Breast cancer during pregnancy and lactation. Surg Clin North Am 70 (5): 1151-63, 1990. [PUBMED Abstract]
Rugo HS: Management of breast cancer diagnosed during pregnancy. Curr Treat Options Oncol 4 (2): 165-73, 2003. [PUBMED Abstract]
Gwyn K, Theriault R: Breast cancer during pregnancy. Oncology (Huntingt) 15 (1): 39-46; discussion 46, 49-51, 2001. [PUBMED Abstract]
Clark RM, Chua T: Breast cancer and pregnancy: the ultimate challenge. Clin Oncol (R Coll Radiol) 1 (1): 11-8, 1989. [PUBMED Abstract]
Barnavon Y, Wallack MK: Management of the pregnant patient with carcinoma of the breast. Surg Gynecol Obstet 171 (4): 347-52, 1990. [PUBMED Abstract]
Special Considerations for Pregnancy and Breast Cancer
Lactation
Suppression of lactation does not improve prognosis. If surgery is planned, however, lactation is suppressed to decrease the size and vascularity of the breasts. If chemotherapy is to be given, lactation is also suppressed because many antineoplastic agents (i.e., cyclophosphamide and methotrexate), when given systemically, may occur in high levels in breast milk and would affect the nursing baby. Women receiving chemotherapy should not breastfeed.[1]
Fetal Consequences of Maternal Breast Cancer
No damaging effects on the fetus from maternal breast cancer have been demonstrated,[2] and there are no reported cases of maternal-fetal transfer of breast cancer cells.
Pregnancy in Patients With a History of Breast Cancer
Based on limited retrospective data, pregnancy does not appear to compromise the survival of women with a previous history of breast cancer, and no deleterious effects have been demonstrated in the fetus.[3–11] Some physicians recommend that patients wait 2 years after diagnosis before attempting to conceive. This allows early recurrence to become manifest, which may influence the decision to become a parent.
Little is known about pregnancy after bone marrow transplant and high-dose chemotherapy with or without total-body irradiation. In one report of pregnancies after bone marrow transplant for hematologic disorders, a 25% incidence of preterm labor and low birth weight for gestational-age infants was noted.[12]
References
Helewa M, Lévesque P, Provencher D, et al.: Breast cancer, pregnancy, and breastfeeding. J Obstet Gynaecol Can 24 (2): 164-80; quiz 181-4, 2002. [PUBMED Abstract]
Amant F, Vandenbroucke T, Verheecke M, et al.: Pediatric Outcome after Maternal Cancer Diagnosed during Pregnancy. N Engl J Med 373 (19): 1824-34, 2015. [PUBMED Abstract]
Clark RM, Chua T: Breast cancer and pregnancy: the ultimate challenge. Clin Oncol (R Coll Radiol) 1 (1): 11-8, 1989. [PUBMED Abstract]
Harvey JC, Rosen PP, Ashikari R, et al.: The effect of pregnancy on the prognosis of carcinoma of the breast following radical mastectomy. Surg Gynecol Obstet 153 (5): 723-5, 1981. [PUBMED Abstract]
Petrek JA: Pregnancy safety after breast cancer. Cancer 74 (1 Suppl): 528-31, 1994. [PUBMED Abstract]
von Schoultz E, Johansson H, Wilking N, et al.: Influence of prior and subsequent pregnancy on breast cancer prognosis. J Clin Oncol 13 (2): 430-4, 1995. [PUBMED Abstract]
Kroman N, Mouridsen HT: Prognostic influence of pregnancy before, around, and after diagnosis of breast cancer. Breast 12 (6): 516-21, 2003. [PUBMED Abstract]
Malamos NA, Stathopoulos GP, Keramopoulos A, et al.: Pregnancy and offspring after the appearance of breast cancer. Oncology 53 (6): 471-5, 1996 Nov-Dec. [PUBMED Abstract]
Gelber S, Coates AS, Goldhirsch A, et al.: Effect of pregnancy on overall survival after the diagnosis of early-stage breast cancer. J Clin Oncol 19 (6): 1671-5, 2001. [PUBMED Abstract]
Gwyn K, Theriault R: Breast cancer during pregnancy. Oncology (Huntingt) 15 (1): 39-46; discussion 46, 49-51, 2001. [PUBMED Abstract]
Rugo HS: Management of breast cancer diagnosed during pregnancy. Curr Treat Options Oncol 4 (2): 165-73, 2003. [PUBMED Abstract]
Sanders JE, Hawley J, Levy W, et al.: Pregnancies following high-dose cyclophosphamide with or without high-dose busulfan or total-body irradiation and bone marrow transplantation. Blood 87 (7): 3045-52, 1996. [PUBMED Abstract]
Latest Updates to This Summary (12/04/2024)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary.
This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® Cancer Information for Health Professionals pages.
About This PDQ Summary
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of breast cancer during pregnancy. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.
Reviewers and Updates
This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
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be cited with text, or
replace or update an existing article that is already cited.
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewers for Breast Cancer Treatment During Pregnancy are:
Fumiko Chino, MD (MD Anderson Cancer Center)
Tarek Hijal, MD (McGill University Health Centre)
Joseph L. Pater, MD (NCIC-Clinical Trials Group)
Carol Tweed, MD (Maryland Oncology Hematology)
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website’s Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.
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Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.
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The preferred citation for this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Breast Cancer Treatment During Pregnancy. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/breast/hp/pregnancy-breast-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389427]
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Screening is looking for signs of disease, such as breast cancer, before a person has symptoms. The goal of screening tests is to find cancer at an early stage when it can be treated and may be cured. Sometimes a screening test finds cancer that is very small or very slow growing. These cancers are unlikely to cause death or illness during a person’s lifetime.
Scientists are trying to better understand which people are more likely to get certain types of cancer. For example, they look at a person’s age, their family history, and certain exposures during their lifetime. This information helps doctors recommend who should be screened for cancer, which screening tests should be used, and how often the tests should be done.
It is important to remember that your doctor does not necessarily think you have cancer if he or she suggests a screening test. Screening tests are done when you have no cancer symptoms. Women who have a strong family history or a personal history of cancer or other risk factors may also be offered genetic testing.
If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests, rather than screening tests.
Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast.
Breast cancer is the second leading cause of death from cancer in American women.
Different factors increase or decrease the risk of getting breast cancer.
Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast.
The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called lobes, which have many smaller sections called lobules. Lobules end in dozens of tiny bulbs that can produce milk. The lobes, lobules, and bulbs are linked by thin tubes called ducts.
EnlargeThe female breast contains lobes, lobules, and ducts that produce and transport milk to the nipple. Fatty tissue gives the breast its shape, while muscles and the chest wall provide support. The lymphatic system, including lymph nodes, filter lymph and store white blood cells that help fight infection and disease.
Each breast also has blood vessels and lymph vessels. The lymph vessels carry an almost colorless, watery fluid called lymph. Lymph vessels carry lymph between lymph nodes. Lymph nodes are small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Groups of lymph nodes are found near the breast in the axilla (under the arm), above the collarbone, and in the chest.
Other PDQ summaries containing information related to breast cancer include:
Breast cancer is the second leading cause of death from cancer in American women.
Women in the United States get breast cancer more than any other type of cancer except for skin cancer.
Breast cancer is more likely to occur as a woman ages. It occurs more often in White women than in Black women, but Black women die from breast cancer more often than White women. However, this difference could be due to factors, such as quality of the screening test, how long women wait to follow up after getting an abnormal test result, the quality of treatment, and the type of tumor.
Breast cancer rarely occurs in men. Because men with breast cancer usually have a lump that can be felt, screening tests are not likely to be helpful.
Different factors increase or decrease the risk of getting breast cancer.
Anything that increases your chance of getting a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor. Talk to your doctor if you think you may be at risk for breast cancer.
To learn more about risk factors and protective factors for breast cancer, visit Breast Cancer Prevention.
Breast Cancer Screening
Key Points
Tests are used to screen for different types of cancer when a person does not have symptoms.
Mammography is the most common screening test for breast cancer.
Magnetic resonance imaging (MRI) may be used to screen women who have a high risk of breast cancer.
Whether a woman should be screened for breast cancer and the screening test to use depends on certain factors.
Other screening tests have been or are being studied in clinical trials.
Breast exam
Thermography
Tissue sampling
Ultrasound exam
Screening tests for breast cancer are being studied in clinical trials.
Tests are used to screen for different types of cancer when a person does not have symptoms.
Scientists study screening tests to find those with the fewest harms and most benefits. Cancer screening trials also are meant to show whether early detection (finding cancer before it causes symptoms) helps a person live longer or decreases a person’s chance of dying from the disease. For some types of cancer, the chance of recovery is better if the disease is found and treated at an early stage.
Mammography is the most common screening test for breast cancer.
Digital breast tomosynthesis (DBT) uses x-rays to take a series of pictures of the inside of the breast from many different angles. A computer is used to make 3-D pictures of the breast from these x-rays.
Synthetic 2-dimensional mammography (S2D) uses x-rays to take pictures of the inside of the breast, usually from two different angles. A computer or x-ray film is used to make 2-D pictures of the breast.
Digital breast tomosynthesis (DBT) was approved by the U.S. Food and Drug Administration (FDA) in 2018 and is now used in 3 out of 4 facilities. One recent study found that synthetic 2-dimensional mammography (S2D) combined with DBT improved tumor detection rates and lowered mammogram callbacks, radiationdose, and overall costs. More studies are being done to compare different types of breast cancer screening.
Mammography is less likely to find breast tumors in women with dense breast tissue. Because both tumors and dense breast tissue appear white on a mammogram, it can be harder to find a tumor when there is dense breast tissue. Younger women are more likely to have dense breast tissue. To learn more, visit Dense Breasts: Answers to Commonly Asked Questions.
EnlargeMammography is an imaging test used to screen for and diagnose breast cancer. It can detect abnormal breast tissue, including cancer, sometimes before symptoms appear.
Many factors affect whether mammography is able to detect (find) breast cancer:
The timing of the mammography within the woman’s menstrual cycle.
The quality of the mammogram picture.
The skill of the radiologist in reading the mammogram.
Women aged 50 to 69 years who have screening mammograms have a lower chance of dying from breast cancer than women who do not have screening mammograms.
Fewer women are dying of breast cancer in the United States, but it is not known whether the lower risk of dying is because the cancer was found early by screening or whether the treatments were better.
Magnetic resonance imaging (MRI) may be used to screen women who have a high risk of breast cancer.
MRI is 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). MRI does not use any x-rays, and the woman is not exposed to radiation.
EnlargeAn MRI of the breast is a procedure that uses radio waves, a strong magnet, and a computer to create detailed pictures of the inside of the breast. A contrast dye may be injected into a vein (not shown) to make the breast tissues easier to see on the MRI pictures. An MRI may be used with other breast imaging tests to detect breast cancer or other abnormal changes in the breast. It may also be used to screen for breast cancer in some people who have a high risk of the disease. Note: The inset shows an MRI image of the insides of both breasts. Credit for inset: The Cancer Imaging Archive (TCIA).
MRI may be used as a screening test for women who have a high risk of breast cancer. Factors that put women at high risk include:
Certain gene changes, such as changes in the BRCA1 or BRCA2 gene.
An MRI is more likely than mammography to find a breast mass (lump) that is not cancer.
Women with dense breasts who have supplemental screening (for example, an MRI) show higher rates of breast cancer detection, but there is limited evidence about whether this leads to better health outcomes.
Whether a woman should be screened for breast cancer and the screening test to use depends on certain factors.
Women with risk factors for breast cancer, such as certain changes in the BRCA1 or BRCA2 gene or certain genetic syndromes, may be screened at a younger age and more often.
Women who have had radiation treatment to the chest, especially at a young age, may start routine breast cancer screening at an earlier age. The benefits and risks of mammograms and MRIs for these women have not been studied.
Breast cancer screening has not been shown to benefit the following women:
Older women who, if diagnosed with breast cancer through screening, will usually die of other causes. Screening mammograms for those aged 66 to 79 years may find cancer in a very small percentage of women, but most of these cancers are low risk.
In women with an average risk of developing breast cancer, screening mammography before age 40 has not shown any benefit.
In women who are not expected to live for a long time and have other diseases or conditions, finding and treating early-stage breast cancer may reduce their quality of life without helping them live longer.
Other screening tests have been or are being studied in clinical trials.
Studies have been done to find out if the following breast cancer screening tests are useful in finding breast cancer or helping women with breast cancer live longer.
Breast exam
A clinical breast exam is an exam of the breast by a doctor or other health professional. He or she will carefully feel the breasts and under the arms for lumps or anything else that seems unusual. It is not known if having clinical breast exams decreases the chance of dying from breast cancer.
Breast self-exams may be done by women or men to check their breasts for lumps or other changes. If you feel any lumps or notice any other changes in your breasts, talk to your doctor. Doing regular breast self-exams has not been shown to decrease the chance of dying from breast cancer.
Thermography
Thermography is a procedure in which a special camera that senses heat is used to record the temperature of the skin that covers the breasts. Tumors can cause temperature changes that may show up on the thermogram.
There have been no randomized clinical trials of thermography to find out how well it detects breast cancer or the harms of the procedure.
Tissue sampling
Breast tissue sampling is taking cells from breast tissue to check under a microscope. Breast tissue sampling as a screening test has not been shown to decrease the risk of dying from breast cancer.
Ultrasound exam
A procedure in which high-energy sound waves are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
An ultrasound is sometimes used as an additional screening test for women who are at increased risk of developing breast cancer (such as women with dense breasts). It is not known if supplemental screening with ultrasound leads to better health outcomes.
Screening tests for breast cancer are being studied in clinical trials.
False-positive results can lead to extra testing and cause anxiety.
False-negative test results can delay diagnosis and treatment.
Finding breast cancer may lead to breast cancer treatment and side effects, but it may not improve a woman’s health or help her live longer.
Mammography exposes the breast to low doses of radiation.
There may be pain or x-ray discomfort during a mammogram.
Talk to your doctor about your risk of breast cancer and your need for screening tests.
Screening tests can have harms.
Not all breast cancers will cause death or illness in a woman’s lifetime, so they may not need to be found or treated.
Decisions about screening tests can be difficult. Not all screening tests are helpful and most have harms. Before having any screening test, you may want to discuss the test with your doctor. It is important to know the harms of the test and whether it has been proven to reduce the risk of dying from cancer.
The harms of mammography include:
False-positive test results can occur.
Screening test results may appear to be abnormal even though no cancer is present. A false-positive test result (one that shows there is cancer when there really isn’t) is usually followed by more tests (such as biopsy), which also have risks.
False-positive results are more likely the first time screening mammography is done than with later screenings. For every 10 women who have a single mammogram, 1 will have a false-positive result. The chance of having a false-positive result goes up the more mammograms a woman has. Comparing a current mammogram with a past mammogram lowers the risk of a false-positive result.
The skill of the radiologist also can affect the chance of a false-positive result.
False-positive results can lead to extra testing and cause anxiety.
If a mammogram is abnormal, more tests may be done to diagnose cancer. Women can become anxious during the diagnostic testing. Even if it is a false-positive test and cancer is not diagnosed, the result can lead to anxiety anywhere from a few days to years later.
Several studies show that women who feel anxiety after false-positive test results are more likely to schedule regular breast screening exams in the future.
False-negative test results can delay diagnosis and treatment.
Screening test results may appear to be normal even though breast cancer is present. This is called a false-negative test result. A woman who has a false-negative test result may delay seeking medical care even if she has symptoms. About 1 in 5 cancers are missed by mammography.
The chance of a false-negative test result is more common in women who:
Finding breast cancer may lead to breast cancer treatment and side effects, but it may not improve a woman’s health or help her live longer.
Some breast cancers found only by screening mammography may never cause health problems or become life-threatening. Finding these cancers is called overdiagnosis. When these cancers are found, having treatment may cause serious side effects and may not lead to a longer, healthier life.
Mammography exposes the breast to low doses of radiation.
Being exposed to high doses of radiation is a risk factor for breast cancer. The radiation dose used with a mammogram is very low. Women who start getting mammograms at age 50 and continue getting them every 2 years have a lower risk of developing breast cancer from radiation exposure during a mammogram than women who start getting mammograms at age 40 and continue getting them every year. Women with large breasts or with breast implants may be exposed to slightly higher radiation doses during screening mammography.
There may be pain or x-ray discomfort during a mammogram.
During a mammogram, the breast is placed between two plates that are pressed together. Pressing the breast helps to get a better image of the breast. Some women have pain or discomfort during a mammogram. The amount of pain may also depend on:
Talk to your doctor about your risk of breast cancer and your need for screening tests.
Talk to your doctor or other health care provider about your risk of breast cancer, whether a screening test is right for you, and the benefits and harms of the screening test. You should take part in the decision about whether you want to have a screening test, based on what is best for you. To learn more, visit Cancer Screening Overview.
About This PDQ Summary
About PDQ
Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about breast cancer screening. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” 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.
Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is:
PDQ® Screening and Prevention Editorial Board. PDQ Breast Cancer Screening. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/breast/patient/breast-screening-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389160]
Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
Disclaimer
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
Contact Us
More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.
Cancer prevention is action taken to lower the chance of getting cancer. By preventing cancer, the number of new cases of cancer in a group or population is lowered. Hopefully, this will reduce the burden of cancer and lower the number of deaths caused by cancer.
Cancer is not a single disease but a group of related diseases. Our genes, lifestyle, and the environment around us work together to increase or decrease our risk of getting cancer. Each person’s cancer risk is made up of a combination of these factors.
To prevent new cancers from starting, scientists look at risk factors and protective factors. Anything that increases your chance of developing cancer is called a cancer risk factor; anything that decreases your chance of developing cancer is called a cancer protective factor.
Some risk factors for cancer can be avoided, but many cannot. For example, both smoking and inheriting certain genes are risk factors for some types of cancer, but only smoking can be avoided. Regular exercise and a healthy diet may be protective factors for some types of cancer. Avoiding risk factors and increasing protective factors may lower your risk, but it does not mean that you will not get cancer.
Different ways to prevent cancer are being studied, including:
changing lifestyle or eating habits
avoiding things known to cause cancer
taking medicines to treat a precancerous conditions or to keep cancer from starting
having risk-reducing surgery.
General Information About Breast Cancer
Key Points
Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast.
Breast cancer is the second leading cause of death from cancer in American women.
Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast.
The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called lobes, which have many smaller sections called lobules. Lobules end in dozens of tiny bulbs that can make milk. The lobes, lobules, and bulbs are linked by thin tubes called ducts.
EnlargeThe female breast contains lobes, lobules, and ducts that produce and transport milk to the nipple. Fatty tissue gives the breast its shape, while muscles and the chest wall provide support. The lymphatic system, including lymph nodes, filter lymph and store white blood cells that help fight infection and disease.
Each breast also has blood vessels and lymph vessels. The lymph vessels carry an almost colorless, watery fluid called lymph. Lymph vessels carry lymph between lymph nodes. Lymph nodes are small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Groups of lymph nodes are found near the breast in the axilla (under the arm), above the collarbone, and in the chest.
Other PDQ summaries containing information related to breast cancer include:
Breast cancer is the second leading cause of death from cancer in American women.
Women in the United States get breast cancer more than any other type of cancer except for skin cancer. Breast cancer is second to lung cancer as a cause of cancer death in American women. Breast cancer rates in women increased gradually for many years until the early 2000s and then decreased rapidly, coinciding with a drop in postmenopausalhormone therapy use. However, since 2005, there has been a small but steady increase in breast cancer rates in women. Deaths from breast cancer declined by 44% between 1989 and 2022. However, breast cancer deaths in Black women remain about 38% higher than in White women. Breast cancer also occurs in men, but the number of new cases is small.
Breast Cancer Prevention
Key Points
Avoiding risk factors and increasing protective factors may help prevent cancer.
The following are risk factors for breast cancer:
Older age
A personal history of breast cancer or benign (noncancer) breast disease
Inherited risk of breast cancer
Dense breast tissue
Reproductive history resulting in greater exposure to estrogen
Taking hormone therapy for symptoms of menopause
Radiation therapy to the breast or chest
Obesity
Drinking alcohol
The following are protective factors for breast cancer:
Reproductive history resulting in less exposure to estrogen
Taking selective estrogen receptor modulators or aromatase inhibitors and inactivators
Selective estrogen receptor modulators
Aromatase inhibitors and inactivators
Risk-reducing or prophylactic mastectomy
Ovarian ablation
Getting enough exercise
It is not clear whether the following affect the risk of breast cancer:
Hormonal contraceptives
Chemicals in the environment
Studies have shown that some factors have little or no effect on the risk of breast cancer.
Cancer prevention clinical trials are used to study ways to prevent cancer.
New ways to prevent breast cancer are being studied in clinical trials.
Avoiding risk factors and increasing protective factors may help prevent cancer.
Avoiding cancerrisk factors may help prevent certain cancers. Risk factors include smoking, having overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer.
Besides being a woman, older age is the main risk factor for breast cancer. The chance of getting breast cancer increases as a woman gets older. A 30-year-old woman has about a 1 in 175 chance of being diagnosed with breast cancer in the next 10 years, while a 70-year-old woman has a 1 in 9 chance of getting the disease during the same time period.
Women aged 50 to 69 years who have screening mammograms have a lower chance of dying from breast cancer than women who do not have screening mammograms. Screening by mammography decreases breast cancer deaths by identifying cases for treatment at an earlier stage.
A personal history of breast cancer or benign (noncancer) breast disease
Women with any of the following have an increased risk of breast cancer:
Women with a family history of breast cancer in a first-degree relative (mother, sister, or daughter) have an increased risk of breast cancer.
Women who have inherited changes in the BRCA1 or BRCA2gene or in certain other genes have a higher risk of breast cancer. The risk of breast cancer caused by inherited gene changes depends on the type of gene mutation, family history of cancer, and other factors.
Dense breast tissue
Having breast tissue that is dense on a mammogram is a factor in breast cancer risk. The level of risk depends on how dense the breast tissue is. Women with very dense breasts have a higher risk of breast cancer than women with low breast density.
Reproductive history resulting in greater exposure to estrogen
Estrogen is a hormone made by the body. It helps the body develop and maintain female sex characteristics. Being exposed to estrogen over a long time may increase the risk of breast cancer. Estrogen levels are highest during the years a woman is menstruating.
The following factors in a woman’s reproductive history increase the length of time her breast tissue is exposed to estrogen and may increase the risk of breast cancer:
Early menstruation: Beginning to have menstrual periods before age 12 increases the number of years the breast tissue is exposed to estrogen.
Starting menopause at a later age: The more years a woman menstruates, the longer her breast tissue is exposed to estrogen.
Older age at birth of first child or never having given birth: Pregnancy lowers a woman’s lifetime number of menstrual cycles. Breast tissue is exposed to more estrogen for longer periods of time in women who become pregnant for the first time after age 35 or who never become pregnant.
Taking hormone therapy for symptoms of menopause
Hormones, such as estrogen and progesterone, can be made into a pill form in a laboratory. Estrogen, progestin, or both may be given to replace the estrogen no longer made by the ovaries in postmenopausal women or women who have had their ovaries removed. This is called menopausal hormone therapy (MHT) or hormone replacement therapy (HRT). Estrogen therapy that began close to the time of menopause is associated with an increased risk of developing breast cancer. Estrogen therapy that began at or after menopause is associated with an increased risk of developing endometrial cancer and total cardiovascular disease, especially stroke. The risk of breast cancer does not decrease after women stop taking estrogen.
Combination hormone therapy (HT) is estrogen combined with progestin. This type of HT increases the risk of breast cancer. Studies show that when women stop taking estrogen combined with progestin, the risk of breast cancer decreases.
Radiation therapy to the breast or chest
Radiation therapy to the chest for the treatment of cancer increases the risk of breast cancer, starting 10 years after treatment. The risk of breast cancer depends on the dose of radiation and the age at which it was given. The risk is highest if radiation treatment was used during puberty, when breasts are forming.
Radiation therapy to treat cancer in one breast does not appear to increase the risk of cancer in the other breast.
For women who have inherited changes in the BRCA1 or BRCA2 gene, exposure to radiation, such as that from chest x-rays, may further increase the risk of breast cancer, especially in women who had x-rays before age 20.
Obesity
Obesity increases the risk of breast cancer, especially in postmenopausal women who have not used HT.
Drinking alcohol
Drinking alcohol increases the risk of breast cancer. The level of risk rises as the amount of alcohol consumed rises.
The following are protective factors for breast cancer:
Reproductive history resulting in less exposure to estrogen
A woman’s reproductive history can affect the length of time her breast tissue is exposed to estrogen. Early onset of menstruation, late onset of menopause, later age at first pregnancy, and never having given birth have been linked to an increase in estrogen exposure and breast cancer risk. The following reproductive factors decrease the length of time a woman’s breast tissue is exposed to estrogen and may help prevent breast cancer:
Early pregnancy: Estrogen levels are lower during pregnancy. In one study, women who had a full-term pregnancy before age 20 had a lower risk of breast cancer than women who did not have children or who gave birth to their first child after age 35.
Breast-feeding: Estrogen levels may remain lower while a woman is breast-feeding. Women who breastfed have a lower risk of breast cancer than women who have had children but did not breastfeed.
Taking selective estrogen receptor modulators or aromatase inhibitors and inactivators
Treatment with tamoxifen lowers the risk of estrogen receptor-positive (ER-positive) breast cancer and DCIS in premenopausal and postmenopausal women at high risk. Tamoxifen is also used to treat metastatic breast cancer and to prevent cancer from recurring after surgery to remove breast tumors. Treatment with raloxifene also lowers the risk of breast cancer in postmenopausal women. With either drug, the reduced risk lasts for several years or longer after treatment is stopped. Lower rates of broken bones have been noted in patients taking raloxifene.
Taking tamoxifen increases the risk of hot flashes, endometrial cancer, stroke, cataracts, and blood clots (especially in the lungs and legs). The risk of having these problems increases markedly in women older than 50 years compared with younger women. Premenopausal women who have a high risk of breast cancer may benefit the most from taking a low dose of tamoxifen, which may decrease breast cancer risk while also reducing some side effects of the drug. The risk of endometrial cancer lasts for 5 years after tamoxifen is stopped, but the risk of cataracts or blood clots does not last long. Talk with your doctor about the risks and benefits of taking this drug.
Taking raloxifene increases the risk of blood clots in the lungs and legs but does not appear to increase the risk of endometrial cancer. In postmenopausal women with osteoporosis (decreased bone density), raloxifene lowers the risk of breast cancer for women who have a high or low risk of breast cancer. It is not known if raloxifene would have the same effect in women who do not have osteoporosis. Talk with your doctor about the risks and benefits of taking this drug.
Other SERMs are being studied in clinical trials.
Aromatase inhibitors and inactivators
Aromatase inhibitors (anastrozole, letrozole) and inactivators (exemestane) lower the risk of recurrence and of new breast cancers in women who have a history of breast cancer. Aromatase inhibitors also decrease the risk of breast cancer in women with the following conditions:
postmenopausal women with a personal history of breast cancer
women with no personal history of breast cancer who are 60 years and older, have a history of DCIS with mastectomy, or have a high risk of breast cancer based on the Gail model tool (a tool used to estimate the risk of breast cancer)
In women with an increased risk of breast cancer, taking aromatase inhibitors decreases the amount of estrogen made by the body. Before menopause, estrogen is made by the ovaries and other tissues in a woman’s body, including the brain, fat tissue, and skin. After menopause, the ovaries stop making estrogen, but the other tissues do not. Aromatase inhibitors block the action of an enzyme called aromatase, which is used to make all of the body’s estrogen. Aromatase inactivators stop the enzyme from working.
Possible harms from taking aromatase inhibitors include muscle and joint pain, osteoporosis, hot flashes, and feeling very tired.
Risk-reducing or prophylactic mastectomy
Some women who have a high risk of breast cancer may choose to have a risk-reducing or prophylactic mastectomy (the removal of one or both breasts when there are no signs of cancer). After surgery, the risk of breast cancer becomes much lower in these women, and most feel less anxious about their risk of breast cancer. Some women diagnosed with breast cancer may decide to have a healthy breast removed at the same time the breast with cancer is removed. This is called contralateral prophylactic mastectomy. However, it is very important to have a cancer risk assessment and counseling about the different ways to prevent breast cancer before making any decision about surgery.
Ovarian ablation
The ovaries make most of the estrogen that is made by the body. Treatments that stop or lower the amount of estrogen made by the ovaries include surgery to remove the ovaries, radiation therapy, or taking certain drugs. This is called ovarian ablation.
Premenopausal women who have a high risk of breast cancer due to certain changes in the BRCA1 or BRCA2 gene may choose to have a risk-reducing oophorectomy (the removal of both ovaries when there are no signs of cancer). This decreases the amount of estrogen made by the body and lowers the risk of breast cancer. Risk-reducing oophorectomy also lowers the risk of breast cancer in average-risk premenopausal women and in women with an increased risk of breast cancer due to radiation to the chest. However, it is very important to have a cancer risk assessment and counseling before making this decision. The sudden drop in estrogen levels may cause the symptoms of menopause to begin. These include hot flashes, trouble sleeping, anxiety, and depression. Long-term effects include decreased sex drive, vaginal dryness, and decreased bone density.
Getting enough exercise
Women who take part in physical exercise have a lower risk of breast cancer.
It is not clear whether the following affect the risk of breast cancer:
Hormonal contraceptives
Hormonal contraceptives contain estrogen or estrogen and progestin. Some studies have shown that women who are current or recent users of hormonal contraceptives may have a slight increase in breast cancer risk. Other studies have not shown an increased risk of breast cancer in women using hormonal contraceptives.
In one study, the risk of breast cancer slightly increased the longer a woman used hormonal contraceptives. Another study showed that the slight increase in breast cancer risk decreased over time when women stopped using hormonal contraceptives.
More studies are needed to know whether hormonal contraceptives affect a woman’s risk of breast cancer.
Chemicals in the environment
Scientists are studying whether exposure to chemicals in the environment may increase a woman’s risk of breast cancer. Studies of this kind can be difficult to conduct and interpret for many reasons:
It is hard to determine the specific chemicals people have been exposed to in the past. It can take decades after a potential exposure before cancer develops, and a person may not be aware of or remember the past exposure.
Even if a chemical is shown in a laboratory test to cause cancer, this does not necessarily mean it will cause cancer in people exposed to that chemical in the environment. A chemical may cause cancer when tested at high levels in laboratory studies but not at the lower levels seen in the environment.
Individual chemicals are likely to cause only a small increase in risk, and it can be difficult to detect that increase in the context of the other factors that may influence a woman’s risk of breast cancer.
These reasons make it hard to know which chemicals, if any, may increase the risk of breast cancer. More studies are needed to know whether chemicals in the environment affect a woman’s risk of breast cancer. To learn more, visit Environmental Carcinogens and Cancer Risk.
Studies have shown that some factors have little or no effect on the risk of breast cancer.
The following have little or no effect on the risk of breast cancer:
having an abortion
making diet changes such as eating less fat or more fruits and vegetables
changes in your circadian rhythm (physical, mental, and behavioral changes that are mainly affected by darkness and light in 24 hour cycles), which may be affected by working night shifts or the amount of light in your bedroom at night
Cancer prevention clinical trials are used to study ways to prevent cancer.
Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials include healthy people who may or may not have an increased risk of cancer. Other prevention trials include people who have had cancer and are trying to prevent recurrence or a second cancer.
The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements.
New ways to prevent breast cancer are being studied in clinical trials.
Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about breast cancer prevention. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” 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.
Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is:
PDQ® Screening and Prevention Editorial Board. PDQ Breast Cancer Prevention. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/breast/patient/breast-prevention-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389410]
Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
Disclaimer
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
Contact Us
More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.
We offer evidence-based supportive and palliative care information for health professionals on the assessment and management of cancer-related symptoms and conditions.
Soft tissue sarcoma may occur anywhere in the body. In children, the tumors form most often in the arms, legs, chest, or abdomen.
EnlargeSoft tissue sarcoma forms in the soft tissues of the body, including the muscles, tendons, ligaments, cartilage, fat, blood vessels, lymph vessels, nerves, and tissues around joints.
Soft tissue sarcoma occurs in children and adults. Soft tissue sarcoma in children may respond differently to treatment and may have a better prognosis than soft tissue sarcoma in adults. Learn more about soft tissue sarcoma in adults at Soft Tissue Sarcoma Treatment.
Types of soft tissue sarcoma
There are many types of soft tissue sarcoma. They are grouped based on the type of soft tissue cell where they first formed.
This summary is about the following types of soft tissue sarcoma:
Blood vessel tumors
Blood vessel tumors include the following types:
Epithelioid hemangioendothelioma. Epithelioid hemangioendothelioma can occur in children but is most common in adults between 30 and 50 years. It may occur in the liver, lung, bone, skin, or soft tissue. Epithelioid hemangioendothelioma may be fast growing or slow growing. In about a third of patients with a tumor in the soft tissue, the tumor spreads to other parts of the body very quickly. Learn more at Childhood Vascular Tumors.
Angiosarcoma. Angiosarcoma is a fast-growing cancer that forms in the blood vessels or lymph vessels in any part of the body, usually in the soft tissue. Most angiosarcomas are in the skin or in the soft tissue near the skin. Those in deeper soft tissue can form in the liver, spleen, and lung. This cancer is very rare in children. Children sometimes have more than one tumor in the skin, liver, or both. Rarely, infantile hemangioma may become angiosarcoma. Learn more at Childhood Vascular Tumors.
Bone tumors
Extraskeletal osteosarcoma. This type of bone and cartilage cancer is very rare in children and adolescents. It is likely to come back after treatment and may spread to the lungs. Osteosarcoma occurs more often in bone.
Cartilage tumors
Extraskeletal mesenchymal chondrosarcoma. This type of cartilage cancer often affects young adults and occurs in the head and neck. It is usually high grade (likely to grow quickly) and may spread to other parts of the body. It may also come back many years after treatment.
Fat tissue tumors
Liposarcoma. This is a cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). Liposarcoma may spread to the lungs and rarely to the lymph nodes. There are several different types of liposarcoma, including:
Myxoid pleomorphic liposarcoma. This is a rare cancer that most often forms in the middle of the chest in children, adolescents, and young adults. This cancer is high grade and can grow quickly and spread to other areas in the body.
Pleomorphic liposarcoma. This is usually a high-grade cancer that is less likely to respond well to treatment. Pleomorphic liposarcoma often forms in older adults.
Dermatofibrosarcoma protuberans. This is a cancer of the deep layers of the skin that most often forms in the trunk, arms, or legs. Dermatofibrosarcoma protuberans usually does not spread to the lymph nodes or other parts of the body.
Some cancers have cells with a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In dermatofibrosarcoma protuberans, an abnormal gene is formed when the COL1A1 gene switches places with part of the PDGFRB gene. To diagnose dermatofibrosarcoma protuberans, the cancer cells are checked for this genetic change.
Fibrosarcoma. There are two types of fibrosarcoma in children and adolescents:
Infantile fibrosarcoma (also called congenital fibrosarcoma). This type of fibrosarcoma usually occurs in children 1 year and younger and may be seen in a prenatal ultrasound exam. This cancer is fast growing and is often large at diagnosis. It rarely spreads to distant parts of the body.
Some cancers have cells with a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In infantile fibrosarcoma, an abnormal gene is formed when the ETV6 gene on chromosome 12 switches places with the NTRK3 gene on another chromosome. To diagnose infantile fibrosarcoma, the cancer cells are checked for this genetic change. A similar tumor has been seen in older children, but it does not have the translocation that is often seen in younger children. Other abnormal genes involved in infantile fibrosarcoma are BRAF, ALK, RAF1, GOLGA4, LRRFIP2, and CLIP1.
Adult-type fibrosarcoma. This is the same fibrosarcoma found in adults. The cells of this cancer do not have the genetic change found in infantile fibrosarcoma. Learn more about soft tissue sarcoma in adults at Soft Tissue Sarcoma Treatment.
Myxofibrosarcoma. This is a rare fibrous tissue cancer that occurs less often in children than in adults.
Low-grade fibromyxoid sarcoma. This is a slow-growing cancer that forms deep in the arms or legs and mostly affects young and middle-aged adults. The cancer may come back many years after treatment and spread to the lungs and the lining of the chest wall. Lifelong follow-up is needed.
Pericytic tumors form in cells that wrap around blood vessels. There are different types of pericytic tumors, including:
Myopericytoma. Infantile hemangiopericytoma is a type of myopericytoma. Children younger than 1 year at the time of diagnosis may have a better prognosis. In children older than 1 year, infantile hemangiopericytoma is more likely to spread to other parts of the body, including the lymph nodes and lungs.
Plexiform fibrohistiocytic tumor. This is a rare tumor that usually affects children and young adults. The tumor usually starts as a painless growth on or just under the skin on the arm, hand, or wrist. It may rarely spread to nearby lymph nodes or to the lungs.
Tumors of unknown cell origin
Tumors of unknown cell origin (the type of cell the tumor first formed in is not known) have many subtypes, including:
Myxoma. Myxoma is a tumor that can occur in the heart or skin. This tumor is frequently seen in children with Carney complex. Carney complex is a rare condition marked by spots on the skin, and tumors in the heart, endocrine glands, skin, and nerves. Carney complex may be linked to gene changes in the PRKAR1A gene.
Synovial sarcoma. Synovial sarcoma is a common type of soft tissue sarcoma in children and adolescents. Learn about Synovial Sarcoma.
Epithelioid sarcoma. This is a rare sarcoma that usually starts deep in soft tissue as a slow growing, firm lump and may spread to the lymph nodes. If cancer formed in the arms, legs, or buttocks, a sentinel lymph node biopsy may be done to check for cancer in the lymph nodes. Epithelioid sarcoma is linked to a change in a tumor suppressor gene called SMARCB1. This type of gene makes a protein that helps control cell growth.
Extraskeletal myxoid chondrosarcoma. This type of soft tissue sarcoma may occur in children and adolescents. Over time, it tends to spread to other parts of the body, including the lymph nodes and lungs. The tumor may come back many years after treatment.
Undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma). This type of soft tissue tumor may form in parts of the body where people have received radiation therapy in the past, or as a second cancer in children with retinoblastoma. The cancer usually forms in the arms or legs and may spread to other parts of the body. Learn more about Osteosarcoma Treatment.
Intracranialmesenchymal tumor. This type of malignant tumor forms in the brain of children, adolescents, and young adults.
Causes and risk factors for childhood soft tissue sarcoma
Childhood soft tissue sarcoma is caused by certain changes to the way soft tissue cells function, especially how they grow and divide into new cells. Often, the exact cause of these changes is unknown. Learn more about how cancer develops at What Is Cancer?
A risk factor is anything that increases the chance of getting a disease. Not every child with one or more of these risk factors will develop a soft tissue sarcoma. And it will develop in some children who don’t have a known risk factor.
The risk of soft tissue sarcoma may be increased if your child has one of the following inheriteddisorders:
Talk with your child’s doctor if you think your child may be at risk.
Symptoms of childhood soft tissue sarcoma
Soft tissue sarcoma may appear as a painless lump under the skin, often on an arm, a leg, the chest, or the abdomen. There may be no other symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause symptoms, such as pain or weakness. Fever, weight loss, night sweats, and low or high blood sugar levels are rare symptoms.
Other conditions may cause the same symptoms. Check with your child’s doctor if your child has any of these problems.
Tests to diagnose childhood soft tissue sarcoma
If your child has symptoms that suggest a soft tissue sarcoma, the doctor will need to find out if these are due to cancer or another problem. The doctor will ask when the symptoms started and how often your child has been having them. They will also ask about your child’s personal and family medical history and do a physical exam. Depending on these results, they may recommend tests to find out if your child has a soft tissue sarcoma, and if so, its extent (stage).
The following tests may be used to diagnose soft tissue sarcomas. The results of these tests will also help you and your child’s doctor plan treatment.
X-ray
X-ray is a type of radiation that can go through the body and make pictures of areas inside the body.
Magnetic resonance imaging (MRI)
MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas in the body, such as the chest, abdomen, arms, or legs. This procedure is also called nuclear magnetic resonance imaging (NMRI).
EnlargeMagnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.
CT scan
CT scan (CAT scan) uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, such as the chest or abdomen. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. 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. Learn more about Computed Tomography (CT) Scans and Cancer.
EnlargeComputed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
Ultrasound
Ultrasound uses high-energy sound waves (ultrasound) that bounce off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
Biopsy
Biopsy is the removal of a sample of cells or tissue from the tumor so that a pathologist can view it under a microscope to check for cancer. The type of biopsy depends, in part, on the size of the mass and whether it is close to the surface of the skin or deeper in the tissue. The following types of biopsies may be used to check for soft tissue sarcoma:
Core needle biopsy removes tissue using a wide needle. Multiple tissue samples are taken. This procedure may be guided using ultrasound, CT scan, or MRI.
Incisional biopsy removes part of a lump or a sample of tissue.
Excisional biopsy removes an entire lump or area of tissue that doesn’t look normal. A pathologist views the tissue under a microscope to look for cancer cells. An excisional biopsy may be used to completely remove smaller tumors that are near the surface of the skin. This type of biopsy is rarely used because cancer cells may remain after the biopsy. If cancer cells remain, the cancer may come back, or it may spread to other parts of the body.
An MRI of the tumor is done before the excisional biopsy. This is done to make a picture of where the original tumor formed and may be used to guide future surgery or radiation therapy.
If possible, it is important to have the same surgeon perform the biopsy and the surgery to remove the tumor. The placement of needles or incisions for the biopsy can affect whether the tumor can be completely removed during a future surgery.
To plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. A pathologist views the tissue under a microscope to look for cancer cells and to find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors.
One or more of the following laboratory tests may be done to study the tissue samples:
Light and electron microscopy checks cells in a sample of tissue under regular and high-powered microscopes to look for certain changes.
Immunocytochemistry uses antibodies to check for certain antigens (markers) in a sample of a patient’s cells or tissues. The antibodies are usually linked to an enzyme or fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test may be used to tell one type of soft tissue sarcoma from another type.
Reverse transcription–polymerase chain reaction (RT-PCR) is a laboratory method used to make many copies of a specific genetic sequence for analysis. It may be used to look for certain changes in a gene or chromosome or for activation of certain genes, which may help diagnose a disease, such as cancer.
Cytogenetic analysis checks the chromosomes of cells in a sample of tumor tissue for broken, missing, rearranged, or extra chromosomes. Changes in certain chromosomes may be a sign of cancer. Cytogenetic analysis is used to help diagnose cancer, plan treatment, or find out how well treatment is working. Fluorescence in situ hybridization (FISH) is a type of cytogenetic analysis.
Molecular test checks for certain genes, proteins, or other molecules in a sample of tissue, blood, or bone marrow. Molecular tests also check for certain changes in a gene or chromosome that may cause or affect the chance of developing soft tissue sarcoma. A molecular test will often help determine the type of soft tissue sarcoma.
The Molecular Characterization Initiative offers free molecular testing to children, adolescents, and young adults with certain types of newly diagnosed cancer. The program is offered through NCI’s Childhood Cancer Data Initiative. To learn more, visit About the Molecular Characterization Initiative.
The following tests may be used to find out if cancer has spread:
Sentinel lymph node biopsy
Sentinel lymph node biopsy removes the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node in a group of lymph nodes to receive lymphatic drainage from the primary tumor. It is therefore the first lymph node the cancer is likely to spread to from the primary tumor. To identify the sentinel lymph node, a radioactive substance, blue dye, or both 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 found, more lymph nodes will be removed through a separate incision (cut). This is called a lymph node dissection. Sometimes, a sentinel lymph node is found in more than one group of nodes. This procedure is used for epithelioid sarcoma.
For the PET scan, a small amount of radioactive sugar (also called radioactive glucose) is injected into a vein. The PET scanner rotates around the body and makes pictures of where sugar is being used in the body. Cancer cells show up brighter in the picture because they are more active and take up more sugar than normal cells do.
For the CT scan (CAT scan), a series of detailed x-ray pictures are taken from different angles and are used to create 3-D views of tissues and organs. 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. Learn more about Computed Tomography (CT) Scans and Cancer.
Getting a second opinion
You may want to get a second opinion to confirm your child’s cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the genetic test results, pathology report, slides, and scans. This doctor may agree with the first doctor, suggest changes or another approach, or provide more information about your child’s tumor.
To learn more about choosing a doctor and getting a second opinion, visit Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your appointments, visit Questions to Ask Your Doctor about Cancer.
Types of treatment for childhood soft tissue sarcoma
Who treats children with soft tissue sarcoma?
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment for soft tissue sarcoma. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. Other specialists may include:
There are different types of treatment for children and adolescents with soft tissue sarcoma. You and your child’s cancer care team will work together to decide treatment. Often, the treatments depend on the type of soft tissue sarcoma. Many other factors will be considered, such as your child’s overall health and whether the cancer is newly diagnosed or has come back.
Your child’s treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s cancer care team before treatment begins about what to expect. For help every step of the way, visit our booklet, Children with Cancer: A Guide for Parents.
Types of treatment your child might have include:
Surgery
Surgery to completely remove the soft tissue sarcoma is done when possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. This is called neoadjuvant (preoperative) therapy.
The following types of surgery may be used:
Wide local excision removes the tumor along with some normal tissue around it.
Amputation removes all or part of the arm or leg with cancer.
Mohs surgery is used to treat cancer in the skin. Individual layers of cancer tissue are removed and checked under a microscope one at a time until all cancer tissue has been removed. This type of surgery is used to treat dermatofibrosarcoma protuberans. It is also called Mohs micrographic surgery.
check the area around where the tumor was removed for cancer cells and then remove more tissue, if needed
After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given 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 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 different types of radiation therapy:
External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. This type of radiation therapy may include:
Stereotactic body radiation therapy uses special equipment to place the patient in the same position for each radiation treatment. Once a day for several days, a radiation machine aims a larger than usual dose of radiation directly at the tumor. By having the patient in the same position for each treatment, there is less damage to nearby healthy tissue. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.
Conformal radiation therapy uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to nearby healthy tissue.
Intensity-modulated radiation therapy (IMRT) is a type of 3-D radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. This type of external radiation therapy causes less damage to nearby healthy tissue.
Proton beam radiation therapy is a type of high-energy, external radiation therapy that uses streams of protons (tiny particles with a positive charge) to kill tumor cells. This type of treatment can lower the amount of radiation damage to healthy tissue near a tumor.
The way radiation therapy is given depends on the type and stage of the cancer being treated, if any cancer cells remain after surgery, and the expected side effects of treatment. External and internal radiation therapy are used to treat childhood soft tissue sarcoma.
Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells. Chemotherapy either kills the cancer cells or stops them from dividing. Chemotherapy may be given alone or with other types of treatment, such as radiation therapy.
For children with soft tissue sarcoma, chemotherapy is taken by mouth or injected into a vein. When given this way, the drugs enter the bloodstream to reach cancer cells throughout the body.
The way the chemotherapy is given depends on the type of soft tissue sarcoma being treated. Some types of soft tissue sarcoma may respond to treatment with chemotherapy.
Chemotherapy that may be used alone or in combination includes:
Other chemotherapy drugs not listed here may also be used.
Learn more about how chemotherapy works, how it is given, common side effects, and more at Chemotherapy to Treat Cancer.
Observation
Observation is closely monitoring a person’s condition without giving any treatment until signs or symptoms appear or change. Observation may be done when:
complete removal of the tumor is not possible
no other treatments are available
the tumor is not likely to damage any vital organs
Targeted therapy uses drugs or other substances to block the action of specific enzymes, proteins, and other molecules involved in the growth and spread of cancer cells. Targeted therapies used to treat soft tissue sarcoma include:
Atezolizumab is used to treat children older than 2 years with alveolar soft part sarcoma that can’t be removed with surgery or has spread to other places in the body.
Larotrectinib is used to treat infantile fibrosarcoma.
Immunotherapy is a treatment that uses the person’s immune system to fight cancer. Pembrolizumab may be used to treat undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma) and progressive and recurrent soft tissue sarcoma.
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child’s age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Treatment of newly diagnosed undifferentiated pleomorphic sarcoma may include surgery. Treatment of recurrent or refractory undifferentiated pleomorphic sarcoma may include immunotherapy (pembrolizumab).
Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis is chemotherapy and radiation therapy. Surgery may be done to remove tumors that have spread to the lung.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment of progressive or recurrent childhood soft tissue sarcoma
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Side effects and late effects of treatment
Cancer treatments can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child’s treatment team about which side effects to look for and ways to manage them.
To learn more about side effects that begin during treatment for cancer, visit Side Effects.
Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:
physical problems
changes in mood, feelings, thinking, learning, or memory
second cancers (new types of cancer)
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the effects cancer treatment can have on your child. Learn more about Late Effects of Treatment for Childhood Cancer.
Prognostic factors for childhood soft tissue sarcoma
If your child has been diagnosed with a soft tissue sarcoma, you may have questions about how serious the cancer is and your child’s chances of survival. The likely outcome or course of a disease is called prognosis. The prognosis depends on:
the part of the body where the tumor first formed
the size and grade of the tumor
the type of soft tissue sarcoma
how deep the tumor is under the skin
whether the tumor has spread to other places in the body and where it has spread
the amount of tumor remaining after surgery to remove it
whether radiation therapy was used to treat the tumor
whether the cancer has just been diagnosed or has recurred (come back)
No two people are alike, and responses to treatment can vary greatly. Your child’s cancer care team is in the best position to talk with you about your child’s prognosis.
Follow-up care
As your child goes through treatment, they will have follow-up tests or check-ups. Some of the tests that were done to diagnose the cancer may be repeated 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 child’s condition has changed or if the cancer has recurred (come back).
When your child has cancer, every member of the family needs support. Taking care of yourself during this difficult time is important. Reach out to your child’s treatment team and to people in your family and community for support. To learn more, visit Support for Families: Childhood Cancer and the booklet Children with Cancer: A Guide for Parents.
Related resources
For more childhood cancer information and other general cancer resources, visit:
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Vulvar cancer is a rare cancer that starts in the tissues of the vulva.
Having vulvar intraepithelial neoplasia or HPV infection can increase the risk of vulvar cancer.
Signs of vulvar cancer include bleeding or itching in the vulvar area.
Tests that examine the vulva are used to diagnose vulvar cancer.
Certain factors affect prognosis (chance of recovery) and treatment options.
Vulvar cancer is a rare cancer that starts in the tissues of the vulva.
Vulvar cancer forms in a woman’s external genitalia, called the vulva. It occurs when cells in the vulva start to grow out of control. The vulva includes:
the mons pubis (the rounded area in front of the pubic bones that becomes covered with hair at puberty)
EnlargeAnatomy of the vulva. The vulva includes the mons pubis, clitoris, inner and outer lips of the vagina, and the openings of the urethra and vagina.
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 many 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.
Having vulvar intraepithelial neoplasia or HPV infection can increase the risk of vulvar cancer.
Anything that increases a person’s chance of getting a disease is called a risk factor. Not every person with one or more of these risk factors will develop vulvar cancer, and it will develop in some people who don’t have any known risk factors. Talk with your doctor if you think you may be at risk. Risk factors for vulvar cancer include:
having a history of abnormal Pap tests (Pap smears)
Signs of vulvar cancer include bleeding or itching in the vulvar area.
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 that looks like a wart or ulcer
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. EnlargePelvic 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.
Pap test: A procedure to collect cells from the surface of the cervix and vagina. A piece of cotton, a brush, or a small wooden stick is used to gently scrape cells from the cervix and vagina. The cells are viewed under a microscope to find out if they are abnormal.
Human papillomavirus (HPV) test: A laboratory test used to check DNA or RNA for certain types of HPV infection. Cells are collected from the vulva and DNA or RNA from the cells is checked to find out if an infection is caused by a type of human papillomavirus that is linked to vulvar cancer. This test may be done using the sample of cells removed during a Pap test. This test may also be done if the results of a Pap test show certain abnormal vulvar cells.
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.
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.
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).
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.
PET scan (positron emission tomography scan): A procedure to find malignanttumor 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.
Certain factors affect prognosis (chance of recovery) and treatment options.
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
Vulvar cancer can recur (come back) after it has been treated.
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:
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, using a proctoscope. A proctoscope is a thin, tube-like instrument with a light and a lens for viewing the inside of the rectum and anus. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
Chest x-ray: 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.
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.
Biopsy: The removal of cells or tissues from the bladder or rectum so they can be viewed under a microscope by a pathologist to check for signs of cancer, if it is suspected that cancer has spread there.
There are three ways that cancer spreads in the body.
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.
Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.
In vulvar intraepithelial neoplasia (VIN), abnormal cells are found on the surface of the vulvar skin.
In stage I, cancer has formed. The tumor is found only in the vulva. Stage I is divided into stages IA and IB.
EnlargeTumor sizes are often measured in centimeters (cm) or inches. Common food items that can be used to show tumor size in cm include: a pea (1 cm), a peanut (2 cm), a grape (3 cm), a walnut (4 cm), a lime (5 cm or 2 inches), an egg (6 cm), a peach (7 cm), and a grapefruit (10 cm or 4 inches).
In stage IA, the tumor is 2 centimeters or smaller and has spread 1 millimeter or less into the tissue of the vulva. Cancer has not spread to the lymph nodes.
In stage IB, the tumor is larger than 2 centimeters or has spread more than 1 millimeter into the tissue of the vulva. Cancer has not spread to the lymph nodes. EnlargeMillimeters (mm). A sharp pencil point is about 1 mm, a new crayon point is about 2 mm, and a new pencil eraser is about 5 mm.
Stage II
In stage II, the tumor is any size and has spread to the lower one-third of the urethra, the lower one-third of the vagina, or the lower one-third of the anus. Cancer has not spread to the lymph nodes.
Stage III
In stage III, the tumor is any size and has spread to the upper two-thirds of the urethra, the upper two-thirds of the vagina, the inner lining of the bladder or rectum, or to any number of lymph nodes. Stage III is divided into stages IIIA, IIIB, and IIIC.
In stage IIIA, cancer is found in lymph nodes in the groin that are not larger than 5 millimeters.
In stage IIIB, cancer is found in lymph nodes in the groin that are larger than 5 millimeters.
In stage IIIC, cancer is found in lymph nodes in the groin and has extended through the outer covering of the lymph nodes.
Stage IV
In stage IV, the tumor is any size and has become attached to the bone, or cancer has spread to lymph nodes that are not movable or have become ulcerated, or there is distant spread. Stage IV is divided into stages IVA and IVB.
In stage IVA, the cancer is attached to the pelvic bone or has spread to lymph nodes in the groin that are not movable or have become ulcerated.
In stage IVB, the cancer has spread beyond the pelvis to distant parts of the body.
Vulvar cancer can recur (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.
The following types of treatment are used:
Surgery
Radiation therapy
Chemotherapy
Immunotherapy
New types of treatment are being tested in clinical trials.
Treatment for vulvar cancer may cause side effects.
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.
One of the following types of surgery may be done to treat VIN:
Separate excision of a lesion: A surgical procedure to remove a lesion of concern.
Wide local excision: A surgical procedure to remove the area of skin affected by VIN and some of the normal tissue around it.
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.
Ultrasound surgical aspiration: 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.
Skinning vulvectomy: The top layer of vulvar skin where the VIN is found is removed. Skin grafts from other parts of the body may be needed to cover the area where the skin was removed.
The goal of surgery for vulvar cancer 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 to treat vulvar cancer:
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:
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.
After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy and/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. External radiation therapy uses a machine outside the body to send radiation toward area of the body with cancer.
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). Topical chemotherapy for vulvar cancer may be applied to the skin in a cream or lotion. The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Immunotherapy 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.
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. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Follow-up tests may be needed.
As you go through treatment, you will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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).
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
radiation therapy or chemotherapy and radiation therapy followed by surgery
radiation therapy with or without chemotherapy
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
radiation therapy or chemotherapy and radiation therapy followed by surgery
radiation therapy with or without chemotherapy
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of vulvar cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” 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.
Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Vulvar Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/vulvar/patient/vulvar-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389324]
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This summary addresses squamous cell cancer of the vulva and vulvar intraepithelial neoplasia (VIN). VIN may be a precursor to invasive squamous cell cancer.
About 50% of vulvar carcinomas arise in the labia majora, the most common site. The labia minora are the site of 15% to 20% of vulvar carcinoma cases. The clitoris and Bartholin glands are less frequently involved.[1] Lesions are multifocal in about 5% of cases. More than 90% of invasive vulvar cancers are squamous cell carcinomas.[2]
Incidence and Mortality
Vulvar cancer accounts for about 6% of cancers of the female genital system in the United States.[3]
Estimated new cases and deaths from vulvar cancer in the United States in 2025:[3]
New cases: 7,480.
Deaths: 1,770.
Anatomy
The vulva is the area immediately external to the vagina, including the mons pubis, labia, clitoris, and Bartholin glands.
EnlargeAnatomy of the vulva. The vulva includes the mons pubis, clitoris, inner and outer lips of the vagina, and the openings of the urethra and vagina.
Risk Factors
Increasing age is the most important risk factor for most cancers. Other risk factors associated with vulvar cancer include:
Human papillomavirus (HPV) infection: In many cases, the development of vulvar cancer is preceded by condyloma or squamous dysplasia. The prevailing evidence favors HPV infection as a causative factor in many genital tract carcinomas.[4]
HPV-associated VIN, termed usual-type VIN when high grades 2 and 3, is most common in women younger than 50 years, whereas non–HPV-associated VIN, termed differentiated-type VIN when high grade 3, is most common in older women.[5,6]
The former lesion-type VIN grade 1 is no longer classified as a true VIN.[5,6] The HPV-related basaloid and warty types are associated with VIN. About 75% to 100% of basaloid and warty carcinomas harbor HPV infection. In addition to the much higher prevalence of HPV in these subtypes than in the keratinizing subtypes, the basaloid and warty subtypes also share many common risk factors with cervical cancers, including:
Prognosis depends on the pathological status of the inguinal lymph nodes and whether spread to adjacent structures has occurred.[8] In patients with operable disease without lymph node involvement, the overall survival (OS) rate is 90%. However, in patients with nodal involvement, the 5-year OS rate is approximately 50% to 60%.[9]
The size of the primary tumor is less important in defining prognosis.[8]
Follow-Up After Treatment
Invasive and preinvasive neoplasms of the vulva may be HPV-induced, and the carcinogenic effect may be widespread in the vulvar epithelium. As a result, patients are monitored regularly for signs or symptoms of recurrence.
References
Macnab JC, Walkinshaw SA, Cordiner JW, et al.: Human papillomavirus in clinically and histologically normal tissue of patients with genital cancer. N Engl J Med 315 (17): 1052-8, 1986. [PUBMED Abstract]
Eifel PJ, Klopp AH, Berek JS, et al.: Cancer of the cervix, vagina, and vulva. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 11th ed. Wolters Kluwer, 2019, pp 1171-1210.
American Cancer Society: Cancer Facts and Figures 2025. American Cancer Society, 2025. Available online. Last accessed January 16, 2025.
Hampl M, Sarajuuri H, Wentzensen N, et al.: Effect of human papillomavirus vaccines on vulvar, vaginal, and anal intraepithelial lesions and vulvar cancer. Obstet Gynecol 108 (6): 1361-8, 2006. [PUBMED Abstract]
Pepas L, Kaushik S, Bryant A, et al.: Medical interventions for high grade vulval intraepithelial neoplasia. Cochrane Database Syst Rev (4): CD007924, 2011. [PUBMED Abstract]
Sideri M, Jones RW, Wilkinson EJ, et al.: Squamous vulvar intraepithelial neoplasia: 2004 modified terminology, ISSVD Vulvar Oncology Subcommittee. J Reprod Med 50 (11): 807-10, 2005. [PUBMED Abstract]
Schiffman M, Kjaer SK: Chapter 2: Natural history of anogenital human papillomavirus infection and neoplasia. J Natl Cancer Inst Monogr (31): 14-9, 2003. [PUBMED Abstract]
Olawaiye AB, Hagemann I, Bhoshale P, et al.: Vulva. In: Goodman KA, Gollub M, Eng C, et al.: AJCC Cancer Staging System. Version 9. American Joint Committee on Cancer; American College of Surgeons, 2023.
Homesley HD, Bundy BN, Sedlis A, et al.: Assessment of current International Federation of Gynecology and Obstetrics staging of vulvar carcinoma relative to prognostic factors for survival (a Gynecologic Oncology Group study). Am J Obstet Gynecol 164 (4): 997-1003; discussion 1003-4, 1991. [PUBMED Abstract]
Cellular Classification of Vulvar Cancer
The histological classification of vulvar disease and precursor lesions of cancer of the vulva was developed by the International Society for the Study of Vulvovaginal Disease (ISSVD).[1]
Nonneoplastic epithelial disorders of vulvar skin and mucosa
Lichen sclerosus (lichen sclerosus et atrophicus).
Low-grade squamous intraepithelial lesion (SIL) of the vulva (vulvar LSIL) encompasses flat condyloma or human papillomavirus effect.
High-grade SIL (vulvar HSIL) was termed VIN, usual type in the 2004 ISSVD terminology.
VIN, differentiated type.
Paget disease of the vulva
Characteristic large pale cells in the epithelium and skin adnexa.
Other histologies
Basal cell carcinoma.
Langerhans cell histiocytosis.
Malignant melanoma.
Sarcoma.
Verrucous carcinoma.
References
Bornstein J, Bogliatto F, Haefner HK, et al.: The 2015 International Society for the Study of Vulvovaginal Disease (ISSVD) Terminology of Vulvar Squamous Intraepithelial Lesions. J Low Genit Tract Dis 20 (1): 11-4, 2016. [PUBMED Abstract]
Stage Information for Vulvar Cancer
The staging evaluation for vulvar cancer may include the following procedures:
Cystoscopy.
Proctoscopy.
X-ray examination of the lungs.
Intravenous (IV) urography (also known as IV pyelography).
Suspected bladder or rectal involvement must be confirmed by biopsy.[1]
The Fédération Internationale de Gynécologie et d’Obstétrique (FIGO) Staging
FIGO and the American Joint Committee on Cancer have designated staging to define vulvar cancer; the FIGO system is most commonly used.[1,2] Stage is based on pathological staging at the time of surgery or before any radiation or chemotherapy, if they are the initial treatment modalities.[3]
The staging system does not apply to malignant melanoma of the vulva, which is staged like melanoma of the skin.[1] For more information, see the Stage Information for Melanoma section in Melanoma Treatment.
Table 1. Definitions of FIGO Stage I, IA, and IBa
Stage
Definition
FIGO = Fédération Internationale de Gynécologie et d’Obstétrique.
aAdapted from FIGO Committee on Gynecologic Oncology.[2]
bDepth of invasion is measured from the basement membrane of the deepest, adjacent, dysplastic, tumor-free rete ridge (or nearest dysplastic rete peg) to the deepest point of invasion.
I
Tumor confined to the vulva.
IA
Tumor size ≤2 cm and stromal invasion ≤1 mmb.
IB
Tumor size >2 cm or stromal invasion >1 mmb.
Table 2. Definition of FIGO Stage IIa
Stage
Definition
FIGO = Fédération Internationale de Gynécologie et d’Obstétrique.
aAdapted from FIGO Committee on Gynecologic Oncology.[2]
II
Tumor of any size with extension to lower one-third of the urethra, lower one-third of the vagina, lower one-third of the anus with negative nodes.
Table 3. Definitions of FIGO Stage III, IIIA, IIIB, and IIICa
Stage
Definition
FIGO = Fédération Internationale de Gynécologie et d’Obstétrique.
aAdapted from FIGO Committee on Gynecologic Oncology.[2]
bRegional refers to inguinal and femoral lymph nodes.
III
Tumor of any size with extension to upper part of adjacent perineal structures, or with any number of nonfixed, nonulcerated lymph nodes.
IIIA
Tumor of any size with disease extension to upper two-thirds of the urethra, upper two-thirds of the vagina, bladder mucosa, rectal mucosa, or regional lymph node metastases ≤5 mm.
IIIB
Regionalb lymph node metastases >5 mm.
IIIC
Regionalb lymph node metastases with extracapsular spread.
Table 4. Definitions of FIGO Stage IV, IVA, and IVBa
Stage
Definition
FIGO = Fédération Internationale de Gynécologie et d’Obstétrique.
aAdapted from FIGO Committee on Gynecologic Oncology.[2]
bRegional refers to inguinal and femoral lymph nodes.
IV
Tumor of any size fixed to bone, or fixed, ulcerated lymph node metastases, or distant metastases.
IVA
Disease fixed to pelvic bone, or fixed or ulcerated regionalb lymph node metastases.
IVB
Distant metastases.
Grade is reported in registry systems and may differ between systems; a two-, three-, or four-grade system may be applied. If not specified, the following system is generally used:[1]
GX: Grade cannot be assessed.
G1: Well differentiated.
G2: Moderately differentiated.
G3: Poorly differentiated.
Overall, about 30% of patients with operable disease have lymph node spread. The pattern of spread is influenced by the histology. Risk factors for lymph node metastasis include:[4–8]
Clinical node status.
Age.
Degree of tumor differentiation.
Tumor stage.
Tumor thickness.
Depth of stromal invasion.
Presence of capillary-lymphatic space invasion.
Well-differentiated lesions tend to spread along the surface with minimal invasion, whereas anaplastic lesions are more likely to be deeply invasive. Spread beyond the vulva is either to adjacent organs such as the vagina, urethra, and anus, or via the lymphatics to the inguinal and femoral lymph nodes, followed by the deep pelvic nodes. Hematogenous spread appears to be uncommon.
References
Olawaiye AB, Hagemann I, Bhoshale P, et al.: Vulva. In: Goodman KA, Gollub M, Eng C, et al.: AJCC Cancer Staging System. Version 9. American Joint Committee on Cancer; American College of Surgeons, 2023.
Olawaiye AB, Cuello MA, Rogers LJ: Cancer of the vulva: 2021 update. Int J Gynaecol Obstet 155 (Suppl 1): 7-18, 2021. [PUBMED Abstract]
Hopkins MP, Reid GC, Johnston CM, et al.: A comparison of staging systems for squamous cell carcinoma of the vulva. Gynecol Oncol 47 (1): 34-7, 1992. [PUBMED Abstract]
Homesley HD, Bundy BN, Sedlis A, et al.: Assessment of current International Federation of Gynecology and Obstetrics staging of vulvar carcinoma relative to prognostic factors for survival (a Gynecologic Oncology Group study). Am J Obstet Gynecol 164 (4): 997-1003; discussion 1003-4, 1991. [PUBMED Abstract]
Boyce J, Fruchter RG, Kasambilides E, et al.: Prognostic factors in carcinoma of the vulva. Gynecol Oncol 20 (3): 364-77, 1985. [PUBMED Abstract]
Sedlis A, Homesley H, Bundy BN, et al.: Positive groin lymph nodes in superficial squamous cell vulvar cancer. A Gynecologic Oncology Group Study. Am J Obstet Gynecol 156 (5): 1159-64, 1987. [PUBMED Abstract]
Binder SW, Huang I, Fu YS, et al.: Risk factors for the development of lymph node metastasis in vulvar squamous cell carcinoma. Gynecol Oncol 37 (1): 9-16, 1990. [PUBMED Abstract]
Homesley HD, Bundy BN, Sedlis A, et al.: Prognostic factors for groin node metastasis in squamous cell carcinoma of the vulva (a Gynecologic Oncology Group study) Gynecol Oncol 49 (3): 279-83, 1993. [PUBMED Abstract]
Treatment Option Overview for Vulvar Cancer
The primary treatment for vulvar cancer is surgery. Radiation therapy is also given to patients with stage III or IV disease.[1–3] Newer strategies have integrated surgery, radiation therapy, and chemotherapy and tailor the treatment to the extent of clinical and pathological disease. Patterns of practice in combining these treatments vary.[4]
Because there are few patients with advanced disease (stages III and IV), only limited data are available on treatment efficacy in this setting, and there is no standard chemotherapy regimen for these patients. Physicians may offer eligible patients with stage III or IV disease participation in clinical trials.
Information about ongoing clinical trials is available from the NCI website.
Wide local excision with or without radiation therapy
Radical vulvectomy and pelvic exenteration
Synchronous radiation therapy and cytotoxic chemotherapy with or without surgery
Surgery
Surgical resection
Since the 1980s, the trend of surgical resection in patients with vulvar cancer has been toward more limited surgery, often combined with radiation therapy to minimize morbidity.[5] In tumors clinically confined to the vulva or perineum, radical local excision with a margin of at least 1 cm has generally replaced radical vulvectomy; separate incision has replaced en bloc inguinal lymph node dissection; ipsilateral inguinal node dissection has replaced bilateral dissection for laterally localized tumors; and femoral lymph node dissection has been omitted in many cases.[2,5–7] However, the different surgical techniques have not been directly compared in randomized controlled trials. In addition, nonrandomized studies lack uniform staging definitions and clear descriptions of lymph node dissection or ancillary radiation.[8][Levels of evidence C2 and C3] The evidence base is, therefore, limited.
Sentinel lymph node dissection (SLND)
Another strategy to minimize the morbidity incurred by groin lymph node dissection in patients with early clinical-stage disease is SLND, reserving groin dissection for those with metastases to the sentinel node(s).
Evidence (SLND):
In a multicenter case series, 403 patients with primary vulvar squamous cell cancers smaller than 4 cm and clinically negative groin lymph nodes underwent 623 SLNDs (using radioactive tracer and blue dye for sentinel node identification).[9] All patients had radical resection of the primary tumor. Node metastases were identified in 26% of SLND procedures, and these patients went on to full inguinofemoral lymphadenectomy. The patients with negative sentinel nodes received no further therapy. After two local recurrences in 17 patients with multifocal primary tumors, the protocol was amended to allow only patients with unifocal tumors into the study.[9][Level of evidence C3]
Local morbidity was much lower in patients who underwent SLND than in patients with positive sentinel nodes who also underwent inguinofemoral lymphadenectomy (see Table 6).
Table 6. Comparisona of Local Morbidity in Patients Treated With SLND Versus SLND and Inguinofemoral Lymphadenectomy
Complications
Local Morbidity From SLND (%)
Local Morbidity From SLND and Inguinofemoral Lymphadenectomy (%)
SLND = Sentinel lymph node dissection.
aP < .0001 for all comparisons.
Wound breakdown
11.7
34
Cellulitis
4.5
21.3
Chronic lymphedema
1.9
25.2
The mean hospital stay was 8.4 days for patients who underwent SLND and 13.7 days for patients who underwent SLND and inguinofemoral lymphadenectomy (P < .0001).
The actuarial groin recurrence rate for all patients with negative SLND results at 2 years was 3% (95% confidence interval [CI], 1%–6%) and 2% (95% CI, 1%–5%) for those with unifocal primary tumors.
SLND may be useful when performed by a surgeon experienced in the procedure, and it may avoid the need for full groin lymph node dissection or radiation therapy in patients with clinically nonsuspicious lymph nodes.
Radiation Therapy
Radical radiation therapy can be used for patients unable to tolerate surgery or when surgery is not an option because of the site or extent of disease.[10–13]
Groin lymph node metastases are present in approximately 20% to 35% of patients with tumors clinically confined to the vulva and with clinically negative nodes.[9,14] Lymph node dissection is traditionally part of the primary surgical therapy in all but the smallest tumors. Some investigators recommend radiation therapy as a means to avoid the morbidity of lymph node dissection, but it is not clear whether radiation therapy can achieve the same local control rates or survival rates as lymph node dissection in early-stage disease.
Localized node-negative disease
A randomized trial to address the efficacy of radiation therapy in patients with clinically localized vulvar cancer has been reported.[14,15] In that study, women with disease clinically confined to the vulva, who did not have clinically suspicious groin lymph node metastases, underwent radical vulvectomy followed by either groin radiation (50 Gy at 2 Gy per fraction) or groin dissection (plus groin radiation if nodes were pathologically involved). Although the planned accrual was 300 patients, the study was stopped after 58 women were randomly assigned because of worse outcomes in the radiation therapy arm.
Five of 27 (18.5%) women in the radiation therapy arm and 0 of 25 women in the groin dissection arm had a groin recurrence, but this difference was not statistically significant (relative risk [RR], 10.21; 95% CI, 0.59–175.78).
There were ten deaths in the radiation therapy arm and three deaths in the groin dissection arm (RR, 4.31; 95% CI, 1.03–18.15).
Disease-specific mortality was not statistically significantly different between the two arms. However, there were eight vulvar cancer–related deaths in the radiation therapy arm versus two vulvar cancer–related deaths in the groin dissection arm (including one related to the groin dissection surgery) (RR, 3.70; 95% CI, 0.87–15.80).[14,15][Level of evidence A1]
There were fewer cases of lymphedema (none in the radiation therapy arm vs. seven in the groin dissection arm) and shorter hospital stays in the radiation therapy arm. The dose penetration of the radiation (3 cm for full dose) has been criticized as inadequate.[14]
In summary, the trial was stopped prematurely, and little can be said about the relative efficacy of the two treatment approaches.[14]
Pelvic node–positive disease
Pelvic radiation therapy has been compared with pelvic node resection in patients with documented groin node–positive disease.
Evidence (pelvic node resection vs. pelvic radiation therapy):
Patients with clinical stage I to stage IV primary squamous cell carcinoma of the vulva in whom groin node metastases were found at radical vulvectomy and bilateral groin lymph node dissection were randomly assigned (during the surgical procedure) to receive either ipsilateral pelvic node resection or pelvic radiation therapy (45 Gy–50 Gy at 1.8 Gy–2.0 Gy per fraction).[16] Because of a perceived emerging benefit of radiation therapy, the planned accrual of 152 patients was stopped after 114 patients were randomly assigned. However, the apparent benefit of radiation was subsequently attenuated with further follow-up.[16][Level of evidence A1]
After a median follow-up of 74 months, the 6-year overall survival (OS) rate was 51% in the pelvic radiation therapy arm versus 41% in the pelvic node resection arm (hazard ratio [HR], 0.61; 95% CI, 0.3–1.3; P = .18).
Vulvar cancer–specific mortality was statistically significantly lower in the pelvic radiation therapy arm (29% in the pelvic radiation therapy arm vs. 51% in the pelvic node resection arm) (HR, 0.49; 95% CI, 0.28–0.87; P = .015). However, there were 14 intercurrent deaths in the pelvic radiation therapy arm versus two deaths in the pelvic node resection arm.
Late chronic lymphedema was similar in both trial arms with 16% in the pelvic radiation therapy arm and 22% in the pelvic node resection arm.
Chemotherapy
There is no standard chemotherapy for vulvar cancer, and reports describing the use of this modality in the setting of metastatic or recurrent disease are anecdotal.[5]
Extrapolating from regimens used for anal or cervical squamous cell cancers, chemotherapy has been studied in combination with radiation in the neoadjuvant setting or as primary therapy in advanced disease. Chemotherapy regimens have included various combinations of fluorouracil (5-FU), cisplatin, mitomycin, or bleomycin.[5]
There is no clear evidence of improvement in survival or palliation. Given the advanced age and comorbidities of many patients with advanced or recurrent vulvar cancer, patient tolerance is a major consideration in the use of these agents.
Systemic treatment for inoperable patients
A systematic review of the use of neoadjuvant chemoradiation therapy in patients who were considered inoperable or who would have required extensive surgery, such as pelvic exenteration, colostomy, or urinary diversion, revealed no randomized trials.[17] Five nonrandomized studies that met the inclusion criteria of neoadjuvant chemoradiation therapy administered in this population with an intent to permit curative surgery were reviewed.[18–22] The five studies used four different chemoradiation therapy schedules and different radiation therapy dose-fractionation techniques. In the four studies using 5-FU with either cisplatin or mitomycin, the operability rate after chemoradiation therapy ranged from 63% to 92%.[18–21] In the one study using bleomycin, the operability rate was only 20%.[22]
In summary, there is evidence that neoadjuvant chemoradiation therapy with 5-FU plus either cisplatin or mitomycin may convert patients to a more operable status, but the evidence base is limited by study design. In addition to a paucity of randomized trials, interpretation of these studies is complicated by the lack of a standard definition of operability.[4][Level of evidence C3] Treatment-related toxicity is substantial.
Systemic treatment for operable patients
There is limited evidence about the use of neoadjuvant chemoradiation therapy in advanced operable vulvar cancer, and the available data do not suggest an advantage to this approach. A systematic review found only one randomized trial that addressed this issue, and it was published only in abstract form.[4,23] In that trial, 68 patients with advanced vulvar cancer (T2* >4 cm, T3*, any case with positive lymph nodes) were randomly assigned to either receive preoperative neoadjuvant radiation therapy (50 Gy) concomitantly with 5-FU and mitomycin or primary surgery. Neoadjuvant therapy–related serious toxicity was high (13 of 24 patients; 10 patients had wound diastasis). After a mean follow-up of 42 months, the 5-year OS rate was 30% in the neoadjuvant chemoradiation therapy arm and 49% in the primary surgery arm (RRdeath, 1.39; 95% CI, 0.94–2.06; P = .19).[4,23][Level of evidence A1] [Note: *T2 is defined as tumor confined to the vulva and/or perineum, more than 2 cm in greatest dimension, and T3 is defined as tumor that invades any of the following: the lower urethra, vagina, or anus.]
Fluorouracil dosing
The DPYD gene encodes an enzyme that catabolizes pyrimidines and fluoropyrimidines, like capecitabine and fluorouracil. An estimated 1% to 2% of the population has germline pathogenic variants in DPYD, which lead to reduced DPD protein function and an accumulation of pyrimidines and fluoropyrimidines in the body.[24,25] Patients with the DPYD*2A variant who receive fluoropyrimidines may experience severe, life-threatening toxicities that are sometimes fatal. Many other DPYD variants have been identified, with a range of clinical effects.[24–26] Fluoropyrimidine avoidance or a dose reduction of 50% may be recommended based on the patient’s DPYD genotype and number of functioning DPYD alleles.[27–29] DPYD genetic testing costs less than $200, but insurance coverage varies due to a lack of national guidelines.[30] In addition, testing may delay therapy by 2 weeks, which would not be advisable in urgent situations. This controversial issue requires further evaluation.[31]
References
Hacker NF, Van der Velden J: Conservative management of early vulvar cancer. Cancer 71 (4 Suppl): 1673-7, 1993. [PUBMED Abstract]
Thomas GM, Dembo AJ, Bryson SC, et al.: Changing concepts in the management of vulvar cancer. Gynecol Oncol 42 (1): 9-21, 1991. [PUBMED Abstract]
Homesley HD, Bundy BN, Sedlis A, et al.: Radiation therapy versus pelvic node resection for carcinoma of the vulva with positive groin nodes. Obstet Gynecol 68 (6): 733-40, 1986. [PUBMED Abstract]
Eifel PJ, Klopp AH, Berek JS, et al.: Cancer of the cervix, vagina, and vulva. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 11th ed. Wolters Kluwer, 2019, pp 1171-1210.
Hoffman MS, Roberts WS, Lapolla JP, et al.: Recent modifications in the treatment of invasive squamous cell carcinoma of the vulva. Obstet Gynecol Surv 44 (4): 227-33, 1989. [PUBMED Abstract]
Heaps JM, Fu YS, Montz FJ, et al.: Surgical-pathologic variables predictive of local recurrence in squamous cell carcinoma of the vulva. Gynecol Oncol 38 (3): 309-14, 1990. [PUBMED Abstract]
Ansink A, van der Velden J: Surgical interventions for early squamous cell carcinoma of the vulva. Cochrane Database Syst Rev (2): CD002036, 2000. [PUBMED Abstract]
Van der Zee AG, Oonk MH, De Hullu JA, et al.: Sentinel node dissection is safe in the treatment of early-stage vulvar cancer. J Clin Oncol 26 (6): 884-9, 2008. [PUBMED Abstract]
Petereit DG, Mehta MP, Buchler DA, et al.: Inguinofemoral radiation of N0,N1 vulvar cancer may be equivalent to lymphadenectomy if proper radiation technique is used. Int J Radiat Oncol Biol Phys 27 (4): 963-7, 1993. [PUBMED Abstract]
Slevin NJ, Pointon RC: Radical radiotherapy for carcinoma of the vulva. Br J Radiol 62 (734): 145-7, 1989. [PUBMED Abstract]
Perez CA, Grigsby PW, Galakatos A, et al.: Radiation therapy in management of carcinoma of the vulva with emphasis on conservation therapy. Cancer 71 (11): 3707-16, 1993. [PUBMED Abstract]
Kumar PP, Good RR, Scott JC: Techniques for management of vulvar cancer by irradiation alone. Radiat Med 6 (4): 185-91, 1988 Jul-Aug. [PUBMED Abstract]
Stehman FB, Bundy BN, Thomas G, et al.: Groin dissection versus groin radiation in carcinoma of the vulva: a Gynecologic Oncology Group study. Int J Radiat Oncol Biol Phys 24 (2): 389-96, 1992. [PUBMED Abstract]
van der Velden J, Fons G, Lawrie TA: Primary groin irradiation versus primary groin surgery for early vulvar cancer. Cochrane Database Syst Rev (5): CD002224, 2011. [PUBMED Abstract]
Kunos C, Simpkins F, Gibbons H, et al.: Radiation therapy compared with pelvic node resection for node-positive vulvar cancer: a randomized controlled trial. Obstet Gynecol 114 (3): 537-46, 2009. [PUBMED Abstract]
van Doorn HC, Ansink A, Verhaar-Langereis M, et al.: Neoadjuvant chemoradiation for advanced primary vulvar cancer. Cochrane Database Syst Rev 3: CD003752, 2006. [PUBMED Abstract]
Eifel PJ, Morris M, Burke TW, et al.: Prolonged continuous infusion cisplatin and 5-fluorouracil with radiation for locally advanced carcinoma of the vulva. Gynecol Oncol 59 (1): 51-6, 1995. [PUBMED Abstract]
Landoni F, Maneo A, Zanetta G, et al.: Concurrent preoperative chemotherapy with 5-fluorouracil and mitomycin C and radiotherapy (FUMIR) followed by limited surgery in locally advanced and recurrent vulvar carcinoma. Gynecol Oncol 61 (3): 321-7, 1996. [PUBMED Abstract]
Montana GS, Thomas GM, Moore DH, et al.: Preoperative chemo-radiation for carcinoma of the vulva with N2/N3 nodes: a gynecologic oncology group study. Int J Radiat Oncol Biol Phys 48 (4): 1007-13, 2000. [PUBMED Abstract]
Moore DH, Thomas GM, Montana GS, et al.: Preoperative chemoradiation for advanced vulvar cancer: a phase II study of the Gynecologic Oncology Group. Int J Radiat Oncol Biol Phys 42 (1): 79-85, 1998. [PUBMED Abstract]
Scheiströen M, Tropé C: Combined bleomycin and irradiation in preoperative treatment of advanced squamous cell carcinoma of the vulva. Acta Oncol 32 (6): 657-61, 1993. [PUBMED Abstract]
Maneo A, Landoni F, Colombo A, et al.: Randomised study between neoadjuvant chemoradiotherapy and primary surgery for the treatment of advanced vulvar cancer. [Abstract] Int J Gynecol Cancer 13 (Suppl 1): A-PL19, 6, 2003.
Sharma BB, Rai K, Blunt H, et al.: Pathogenic DPYD Variants and Treatment-Related Mortality in Patients Receiving Fluoropyrimidine Chemotherapy: A Systematic Review and Meta-Analysis. Oncologist 26 (12): 1008-1016, 2021. [PUBMED Abstract]
Lam SW, Guchelaar HJ, Boven E: The role of pharmacogenetics in capecitabine efficacy and toxicity. Cancer Treat Rev 50: 9-22, 2016. [PUBMED Abstract]
Shakeel F, Fang F, Kwon JW, et al.: Patients carrying DPYD variant alleles have increased risk of severe toxicity and related treatment modifications during fluoropyrimidine chemotherapy. Pharmacogenomics 22 (3): 145-155, 2021. [PUBMED Abstract]
Amstutz U, Henricks LM, Offer SM, et al.: Clinical Pharmacogenetics Implementation Consortium (CPIC) Guideline for Dihydropyrimidine Dehydrogenase Genotype and Fluoropyrimidine Dosing: 2017 Update. Clin Pharmacol Ther 103 (2): 210-216, 2018. [PUBMED Abstract]
Henricks LM, Lunenburg CATC, de Man FM, et al.: DPYD genotype-guided dose individualisation of fluoropyrimidine therapy in patients with cancer: a prospective safety analysis. Lancet Oncol 19 (11): 1459-1467, 2018. [PUBMED Abstract]
Lau-Min KS, Varughese LA, Nelson MN, et al.: Preemptive pharmacogenetic testing to guide chemotherapy dosing in patients with gastrointestinal malignancies: a qualitative study of barriers to implementation. BMC Cancer 22 (1): 47, 2022. [PUBMED Abstract]
Brooks GA, Tapp S, Daly AT, et al.: Cost-effectiveness of DPYD Genotyping Prior to Fluoropyrimidine-based Adjuvant Chemotherapy for Colon Cancer. Clin Colorectal Cancer 21 (3): e189-e195, 2022. [PUBMED Abstract]
Baker SD, Bates SE, Brooks GA, et al.: DPYD Testing: Time to Put Patient Safety First. J Clin Oncol 41 (15): 2701-2705, 2023. [PUBMED Abstract]
Treatment of Vulvar Intraepithelial Neoplasia
Treatment Options for Vulvar Intraepithelial Neoplasia (VIN)
Carbon dioxide (CO2) laser surgery and vaporization.[2,3] A disadvantage of vaporization is that it does not provide tissue for histological examination to confirm complete removal of the lesion and the absence of invasive disease.
Traditionally, there were three grades of VIN, however, there is little evidence that all three grades are part of the same biological continuum or that grade 1 is even a cancer precursor. In 2004, the International Society for the Study of Vulvovaginal Disease (ISSVD) changed its terminology, reserving the designation VIN for two categories of lesions based on morphological appearance.[9] In 2015, the ISSVD developed terminology for vulvar squamous intraepithelial lesions (SIL), which includes:[10]
Low-grade SIL of the vulva (vulvar LSIL) encompasses flat condyloma or human papillomavirus effect.
High-grade SIL (vulvar HSIL) was termed VIN, usual type in the 2004 ISSVD terminology.
VIN, differentiated type.
High-grade VIN is usually managed with active therapy because of a higher risk for progression to invasive disease.[2] Estimates of progression rates are imprecise. A systematic literature review that included 88 untreated patients with VIN 3 reported a 9% progression rate (8 of 88 patients) to invasive vulvar cancer during 12 to 96 months of observation. In the same review, the spontaneous regression rate was 1.2%, all of which occurred in women younger than 35 years.[1] However, in a single-center study, 10 of 63 (16%) untreated women with VIN 2 or VIN 3 progressed to invasive cancer after a mean of 3.9 years.[11]
VIN lesions may be multifocal or confluent and extensive. It is important to perform multiple biopsies in treatment planning to exclude occult invasive disease. VIN located in nonhairy areas can be considered an epithelial disease; however, VIN found in hairy sites usually involves the pilosebaceous apparatus and requires a greater depth of excision because it can track along hair roots.
Surgery
The principal treatment approach is surgery, but there is no consensus on the optimal surgical procedure. The goal is to remove or destroy the entire VIN lesion while preserving vulvar anatomy and function. Simple vulvectomy yields a 5-year survival rate of nearly 100% but is seldom indicated. Other more limited surgical procedures, including separate excision of multiple lesions, are less deforming.[12] The choice of treatment depends on the extent of the disease and the experience of the treating physician. There are no reliable data comparing the efficacy and safety of the various surgical approaches.
A systematic literature review identified only a single randomized trial comparing any of the surgical approaches.[2] In that trial, 30 women with high-grade VIN were randomly assigned to either receive CO2 laser ablation or ultrasound surgical aspiration.[3] There were no statistically significant differences in disease recurrence, painful dysuria or burning, adhesions, or eschar formation between the two treatments after 1 year of follow-up. Scarring was observed in 5 of 16 women treated with laser ablation and 0 of 14 women treated with ultrasound surgical aspiration (P < .01), but consequences of the scarring on sexual function or quality of life were not reported.[3][Level of evidence B1] The trial was too small to draw reliable conclusions about the relative efficacy of these surgical techniques. The remainder of the surgical literature is derived from case series and is prone to important study biases.[Level of evidence C2]
Whatever procedure is used, patients are at substantial risk of recurrence, particularly when the lesions are high grade or multifocal.[13] The most common sites of recurrence are the perianal skin, presacral area, and clitoral hood. About 4% of patients treated for VIN subsequently develop invasive cancer.[14,15]
Nonsurgical interventions
Topical imiquimod
Among women with high-grade VIN, substantial response rates and acceptable tolerability were reported for topical imiquimod 5%, an immune-response modifier with activity in human papillomavirus types 6- and 11-associated vulvar condylomata.
Evidence (imiquimod):
Three randomized placebo-controlled trials (including a total of 104 patients) with clinical response as the primary end points have been reported in either peer-reviewed-journals or in abstract format.[7];[4–6][Level of evidence B3] The results of these trials were summarized in a systematic review.[8]
At 5 to 6 months, the complete response rates in patients were 36 of 62 in the combined imiquimod arm versus 0 of 42 in the combined placebo arm, and the partial response rates were 18 of 62 in the combined imiquimod arm versus 1 of 42 in the combined placebo arm (relative risk, 11.95; 95% confidence interval, 3.21–44.51).
In the only trial reporting progression to cancer at 12 months, there was no difference in progression rate, but the trial was severely underpowered because only 3 of the total 52 women developed invasive disease by 12 months.[6]
The only trial reporting quality of life [6] showed no difference between imiquimod and placebo.
Local side effects of imiquimod included pain, edema, erythema, and a single case of erosion. However, no patients had to discontinue treatment as a result of toxicity.
Other nonsurgical interventions
Nonsurgical approaches have been studied because of the physical and psychosexual morbidity associated with many vulvar surgical procedures. Some of these approaches, including topical fluorouracil, recombinant interferon gamma, bleomycin, and trinitrochlorobenzene, have been largely abandoned because of high recurrence rates or intolerable local side effects, such as pain, irritation, and ulceration.[8,16]
Photodynamic therapy, using topically applied 5-aminolevulinic acid as the sensitizing agent for 635 nm laser light, has also been studied. However, data are limited to small case series with variable response rates.[17,18][Level of evidence C3]
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
References
van Seters M, van Beurden M, de Craen AJ: Is the assumed natural history of vulvar intraepithelial neoplasia III based on enough evidence? A systematic review of 3322 published patients. Gynecol Oncol 97 (2): 645-51, 2005. [PUBMED Abstract]
Kaushik S, Pepas L, Nordin A, et al.: Surgical interventions for high grade vulval intraepithelial neoplasia. Cochrane Database Syst Rev (1): CD007928, 2011. [PUBMED Abstract]
von Gruenigen VE, Gibbons HE, Gibbins K, et al.: Surgical treatments for vulvar and vaginal dysplasia: a randomized controlled trial. Obstet Gynecol 109 (4): 942-7, 2007. [PUBMED Abstract]
Sterling JC, Smith NA, Loo WJ, et al.: Randomized, doubleblind, placebo-controlled trial for treatment of high grade vulval intraepithelial neoplasia with imiquimod. [Abstract] J Eur Acad Derm Venereol 19 (Suppl 2): A-FC06.1, 22, 2005.
Mathiesen O, Buus SK, Cramers M: Topical imiquimod can reverse vulvar intraepithelial neoplasia: a randomised, double-blinded study. Gynecol Oncol 107 (2): 219-22, 2007. [PUBMED Abstract]
van Seters M, van Beurden M, ten Kate FJ, et al.: Treatment of vulvar intraepithelial neoplasia with topical imiquimod. N Engl J Med 358 (14): 1465-73, 2008. [PUBMED Abstract]
Terlou A, van Seters M, Ewing PC, et al.: Treatment of vulvar intraepithelial neoplasia with topical imiquimod: seven years median follow-up of a randomized clinical trial. Gynecol Oncol 121 (1): 157-62, 2011. [PUBMED Abstract]
Pepas L, Kaushik S, Bryant A, et al.: Medical interventions for high grade vulval intraepithelial neoplasia. Cochrane Database Syst Rev (4): CD007924, 2011. [PUBMED Abstract]
Sideri M, Jones RW, Wilkinson EJ, et al.: Squamous vulvar intraepithelial neoplasia: 2004 modified terminology, ISSVD Vulvar Oncology Subcommittee. J Reprod Med 50 (11): 807-10, 2005. [PUBMED Abstract]
Bornstein J, Bogliatto F, Haefner HK, et al.: The 2015 International Society for the Study of Vulvovaginal Disease (ISSVD) Terminology of Vulvar Squamous Intraepithelial Lesions. J Low Genit Tract Dis 20 (1): 11-4, 2016. [PUBMED Abstract]
Jones RW, Rowan DM, Stewart AW: Vulvar intraepithelial neoplasia: aspects of the natural history and outcome in 405 women. Obstet Gynecol 106 (6): 1319-26, 2005. [PUBMED Abstract]
Eifel PJ, Klopp AH, Berek JS, et al.: Cancer of the cervix, vagina, and vulva. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 11th ed. Wolters Kluwer, 2019, pp 1171-1210.
Küppers V, Stiller M, Somville T, et al.: Risk factors for recurrent VIN. Role of multifocality and grade of disease. J Reprod Med 42 (3): 140-4, 1997. [PUBMED Abstract]
Buscema J, Woodruff JD, Parmley TH, et al.: Carcinoma in situ of the vulva. Obstet Gynecol 55 (2): 225-30, 1980. [PUBMED Abstract]
Jones RW, Rowan DM: Vulvar intraepithelial neoplasia III: a clinical study of the outcome in 113 cases with relation to the later development of invasive vulvar carcinoma. Obstet Gynecol 84 (5): 741-5, 1994. [PUBMED Abstract]
Sillman FH, Sedlis A, Boyce JG: A review of lower genital intraepithelial neoplasia and the use of topical 5-fluorouracil. Obstet Gynecol Surv 40 (4): 190-220, 1985. [PUBMED Abstract]
Hillemanns P, Untch M, Dannecker C, et al.: Photodynamic therapy of vulvar intraepithelial neoplasia using 5-aminolevulinic acid. Int J Cancer 85 (5): 649-53, 2000. [PUBMED Abstract]
Fehr MK, Hornung R, Schwarz VA, et al.: Photodynamic therapy of vulvar intraepithelial neoplasia III using topically applied 5-aminolevulinic acid. Gynecol Oncol 80 (1): 62-6, 2001. [PUBMED Abstract]
Treatment of Stages I and II Vulvar Cancer
Treatment Options for Stages I and II Vulvar Cancer
Radical local excision with ipsilateral or bilateral inguinal and femoral lymph node dissection may be indicated. For stage I microinvasive lesions (<1 mm invasion) with no associated severe vulvar dystrophy, a wide (1 cm margin) excision (without lymph node dissection) may be done. For all other stage I lesions, if well lateralized, without diffuse severe dystrophy, and with clinically negative nodes, a radical local excision with complete unilateral lymphadenectomy may be done.[1] Candidates for this procedure should have lesions 2 cm or smaller in diameter with 5 mm or less invasion, no capillary-lymphatic space invasion, and clinically uninvolved nodes.[2,3]
For stage II disease, large T2* tumors may require modified radical vulvectomy or radical vulvectomy.[4] [Note: *T2 is defined as tumor confined to the vulva and/or perineum, more than 2 cm in greatest dimension.]
For both stage I and stage II disease, radical local excision and sentinel node dissection is indicated and groin dissection is reserved for those with metastasis to the sentinel node(s).[5]
Surgery and radiation therapy
Some investigators recommend radical excision and groin nodal radiation therapy as a means to avoid the morbidity of lymph node dissection. However, it is not clear whether radiation therapy can achieve the same local control rates or survival rates as lymph node dissection in early-stage disease. A randomized trial to address this issue in patients with clinically localized vulvar disease was stopped early as a result of early emergence of worse outcomes in the radiation therapy arm.[6,7] For stage II disease, adjuvant local radiation therapy may be indicated for surgical margins smaller than 8 mm, capillary-lymphatic space invasion, and thickness greater than 5 mm.[8,9]
Radiation therapy alone
For patients unable to tolerate radical surgery or deemed ineligible for surgery because of the site or extent of disease, radical radiation therapy may be associated with favorable survival.[10–13]
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
References
Malfetano JH, Piver MS, Tsukada Y, et al.: Univariate and multivariate analyses of 5-year survival, recurrence, and inguinal node metastases in stage I and II vulvar carcinoma. J Surg Oncol 30 (2): 124-31, 1985. [PUBMED Abstract]
Stehman FB, Bundy BN, Dvoretsky PM, et al.: Early stage I carcinoma of the vulva treated with ipsilateral superficial inguinal lymphadenectomy and modified radical hemivulvectomy: a prospective study of the Gynecologic Oncology Group. Obstet Gynecol 79 (4): 490-7, 1992. [PUBMED Abstract]
Hacker NF, Van der Velden J: Conservative management of early vulvar cancer. Cancer 71 (4 Suppl): 1673-7, 1993. [PUBMED Abstract]
Eifel PJ, Klopp AH, Berek JS, et al.: Cancer of the cervix, vagina, and vulva. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 11th ed. Wolters Kluwer, 2019, pp 1171-1210.
Van der Zee AG, Oonk MH, De Hullu JA, et al.: Sentinel node dissection is safe in the treatment of early-stage vulvar cancer. J Clin Oncol 26 (6): 884-9, 2008. [PUBMED Abstract]
Stehman FB, Bundy BN, Thomas G, et al.: Groin dissection versus groin radiation in carcinoma of the vulva: a Gynecologic Oncology Group study. Int J Radiat Oncol Biol Phys 24 (2): 389-96, 1992. [PUBMED Abstract]
van der Velden J, Fons G, Lawrie TA: Primary groin irradiation versus primary groin surgery for early vulvar cancer. Cochrane Database Syst Rev (5): CD002224, 2011. [PUBMED Abstract]
Thomas GM, Dembo AJ, Bryson SC, et al.: Changing concepts in the management of vulvar cancer. Gynecol Oncol 42 (1): 9-21, 1991. [PUBMED Abstract]
Faul CM, Mirmow D, Huang Q, et al.: Adjuvant radiation for vulvar carcinoma: improved local control. Int J Radiat Oncol Biol Phys 38 (2): 381-9, 1997. [PUBMED Abstract]
Petereit DG, Mehta MP, Buchler DA, et al.: Inguinofemoral radiation of N0,N1 vulvar cancer may be equivalent to lymphadenectomy if proper radiation technique is used. Int J Radiat Oncol Biol Phys 27 (4): 963-7, 1993. [PUBMED Abstract]
Slevin NJ, Pointon RC: Radical radiotherapy for carcinoma of the vulva. Br J Radiol 62 (734): 145-7, 1989. [PUBMED Abstract]
Perez CA, Grigsby PW, Galakatos A, et al.: Radiation therapy in management of carcinoma of the vulva with emphasis on conservation therapy. Cancer 71 (11): 3707-16, 1993. [PUBMED Abstract]
Kumar PP, Good RR, Scott JC: Techniques for management of vulvar cancer by irradiation alone. Radiat Med 6 (4): 185-91, 1988 Jul-Aug. [PUBMED Abstract]
Modified radical or radical vulvectomy with inguinal and femoral lymphadenectomy is the standard therapy.[1] Nodal involvement is a key determinant of survival. Radiation therapy is given to patients with large primary lesions and narrow margins. Radiation therapy to the pelvis and groin is given if inguinal lymph nodes are positive.[2] Radiation therapy to the pelvis and groin is usually given if two or more groin nodes are involved.[2,3]
Localized adjuvant radiation therapy consisting of 45 Gy to 50 Gy may also be indicated when there is capillary-lymphatic space invasion and a thickness of greater than 5 mm, particularly if the nodes are involved.[1]
Radiation therapy or chemoradiation therapy followed by surgery
Preoperative neoadjuvant radiation therapy or chemoradiation therapy may be used to improve operability and even decrease the extent of surgery required.[4–10]
Radiation therapy with or without chemotherapy
For patients unable to tolerate radical surgery or deemed ineligible for surgery because of the site or extent of disease, radical radiation therapy may be associated with long-term survival.[11,12] Some physicians prefer to add concurrent fluorouracil (5-FU) or 5-FU and cisplatin.[1,13]
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
References
Thomas GM, Dembo AJ, Bryson SC, et al.: Changing concepts in the management of vulvar cancer. Gynecol Oncol 42 (1): 9-21, 1991. [PUBMED Abstract]
Kunos C, Simpkins F, Gibbons H, et al.: Radiation therapy compared with pelvic node resection for node-positive vulvar cancer: a randomized controlled trial. Obstet Gynecol 114 (3): 537-46, 2009. [PUBMED Abstract]
Homesley HD, Bundy BN, Sedlis A, et al.: Prognostic factors for groin node metastasis in squamous cell carcinoma of the vulva (a Gynecologic Oncology Group study) Gynecol Oncol 49 (3): 279-83, 1993. [PUBMED Abstract]
Boronow RC, Hickman BT, Reagan MT, et al.: Combined therapy as an alternative to exenteration for locally advanced vulvovaginal cancer. II. Results, complications, and dosimetric and surgical considerations. Am J Clin Oncol 10 (2): 171-81, 1987. [PUBMED Abstract]
Anderson JM, Cassady JR, Shimm DS, et al.: Vulvar carcinoma. Int J Radiat Oncol Biol Phys 32 (5): 1351-7, 1995. [PUBMED Abstract]
van Doorn HC, Ansink A, Verhaar-Langereis M, et al.: Neoadjuvant chemoradiation for advanced primary vulvar cancer. Cochrane Database Syst Rev 3: CD003752, 2006. [PUBMED Abstract]
Eifel PJ, Morris M, Burke TW, et al.: Prolonged continuous infusion cisplatin and 5-fluorouracil with radiation for locally advanced carcinoma of the vulva. Gynecol Oncol 59 (1): 51-6, 1995. [PUBMED Abstract]
Landoni F, Maneo A, Zanetta G, et al.: Concurrent preoperative chemotherapy with 5-fluorouracil and mitomycin C and radiotherapy (FUMIR) followed by limited surgery in locally advanced and recurrent vulvar carcinoma. Gynecol Oncol 61 (3): 321-7, 1996. [PUBMED Abstract]
Montana GS, Thomas GM, Moore DH, et al.: Preoperative chemo-radiation for carcinoma of the vulva with N2/N3 nodes: a gynecologic oncology group study. Int J Radiat Oncol Biol Phys 48 (4): 1007-13, 2000. [PUBMED Abstract]
Moore DH, Thomas GM, Montana GS, et al.: Preoperative chemoradiation for advanced vulvar cancer: a phase II study of the Gynecologic Oncology Group. Int J Radiat Oncol Biol Phys 42 (1): 79-85, 1998. [PUBMED Abstract]
Perez CA, Grigsby PW, Galakatos A, et al.: Radiation therapy in management of carcinoma of the vulva with emphasis on conservation therapy. Cancer 71 (11): 3707-16, 1993. [PUBMED Abstract]
Slevin NJ, Pointon RC: Radical radiotherapy for carcinoma of the vulva. Br J Radiol 62 (734): 145-7, 1989. [PUBMED Abstract]
Eifel PJ, Klopp AH, Berek JS, et al.: Cancer of the cervix, vagina, and vulva. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 11th ed. Wolters Kluwer, 2019, pp 1171-1210.
Radical vulvectomy and pelvic exenteration may be indicated for patients with stage IVA vulvar cancer.
Surgery and radiation therapy
Surgery followed by radiation therapy may be done for large resected lesions with narrow margins. Localized adjuvant radiation therapy consisting of 45 Gy to 50 Gy may also be indicated when there is capillary-lymphatic space invasion and thickness greater than 5 mm.[1] Radiation therapy to the pelvis and groin is given if two or more groin lymph nodes are involved.[2,3]
Radiation therapy or chemoradiation therapy followed by surgery
Neoadjuvant radiation therapy or chemoradiation therapy of large primary lesions (to improve operability) may be done, followed by radical surgery.[4–10]
Radiation therapy with or without chemotherapy
For patients unable to tolerate radical vulvectomy or who are deemed ineligible for surgery because of the site or extent of disease, radical radiation therapy may be associated with long-term survival.[11,12] When radiation therapy is used for primary definitive treatment of vulvar cancer, some physicians prefer to add concurrent fluorouracil (5-FU) or 5-FU and cisplatin.[1,13–17]
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
References
Thomas GM, Dembo AJ, Bryson SC, et al.: Changing concepts in the management of vulvar cancer. Gynecol Oncol 42 (1): 9-21, 1991. [PUBMED Abstract]
Homesley HD, Bundy BN, Sedlis A, et al.: Radiation therapy versus pelvic node resection for carcinoma of the vulva with positive groin nodes. Obstet Gynecol 68 (6): 733-40, 1986. [PUBMED Abstract]
Kunos C, Simpkins F, Gibbons H, et al.: Radiation therapy compared with pelvic node resection for node-positive vulvar cancer: a randomized controlled trial. Obstet Gynecol 114 (3): 537-46, 2009. [PUBMED Abstract]
Boronow RC, Hickman BT, Reagan MT, et al.: Combined therapy as an alternative to exenteration for locally advanced vulvovaginal cancer. II. Results, complications, and dosimetric and surgical considerations. Am J Clin Oncol 10 (2): 171-81, 1987. [PUBMED Abstract]
Anderson JM, Cassady JR, Shimm DS, et al.: Vulvar carcinoma. Int J Radiat Oncol Biol Phys 32 (5): 1351-7, 1995. [PUBMED Abstract]
van Doorn HC, Ansink A, Verhaar-Langereis M, et al.: Neoadjuvant chemoradiation for advanced primary vulvar cancer. Cochrane Database Syst Rev 3: CD003752, 2006. [PUBMED Abstract]
Eifel PJ, Morris M, Burke TW, et al.: Prolonged continuous infusion cisplatin and 5-fluorouracil with radiation for locally advanced carcinoma of the vulva. Gynecol Oncol 59 (1): 51-6, 1995. [PUBMED Abstract]
Landoni F, Maneo A, Zanetta G, et al.: Concurrent preoperative chemotherapy with 5-fluorouracil and mitomycin C and radiotherapy (FUMIR) followed by limited surgery in locally advanced and recurrent vulvar carcinoma. Gynecol Oncol 61 (3): 321-7, 1996. [PUBMED Abstract]
Montana GS, Thomas GM, Moore DH, et al.: Preoperative chemo-radiation for carcinoma of the vulva with N2/N3 nodes: a gynecologic oncology group study. Int J Radiat Oncol Biol Phys 48 (4): 1007-13, 2000. [PUBMED Abstract]
Moore DH, Thomas GM, Montana GS, et al.: Preoperative chemoradiation for advanced vulvar cancer: a phase II study of the Gynecologic Oncology Group. Int J Radiat Oncol Biol Phys 42 (1): 79-85, 1998. [PUBMED Abstract]
Slevin NJ, Pointon RC: Radical radiotherapy for carcinoma of the vulva. Br J Radiol 62 (734): 145-7, 1989. [PUBMED Abstract]
Perez CA, Grigsby PW, Galakatos A, et al.: Radiation therapy in management of carcinoma of the vulva with emphasis on conservation therapy. Cancer 71 (11): 3707-16, 1993. [PUBMED Abstract]
Russell AH, Mesic JB, Scudder SA, et al.: Synchronous radiation and cytotoxic chemotherapy for locally advanced or recurrent squamous cancer of the vulva. Gynecol Oncol 47 (1): 14-20, 1992. [PUBMED Abstract]
Berek JS, Heaps JM, Fu YS, et al.: Concurrent cisplatin and 5-fluorouracil chemotherapy and radiation therapy for advanced-stage squamous carcinoma of the vulva. Gynecol Oncol 42 (3): 197-201, 1991. [PUBMED Abstract]
Koh WJ, Wallace HJ, Greer BE, et al.: Combined radiotherapy and chemotherapy in the management of local-regionally advanced vulvar cancer. Int J Radiat Oncol Biol Phys 26 (5): 809-16, 1993. [PUBMED Abstract]
Thomas G, Dembo A, DePetrillo A, et al.: Concurrent radiation and chemotherapy in vulvar carcinoma. Gynecol Oncol 34 (3): 263-7, 1989. [PUBMED Abstract]
Eifel PJ, Klopp AH, Berek JS, et al.: Cancer of the cervix, vagina, and vulva. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 11th ed. Wolters Kluwer, 2019, pp 1171-1210.
Local therapy must be individualized depending on the extent of local and metastatic disease.
There is no standard chemotherapy for metastatic disease, and reports describing the use of this modality are anecdotal.[1] However, by largely extrapolating from regimens used for anal or cervical cancer, chemotherapy has been studied. Regimens have included various combinations of fluorouracil, cisplatin, mitomycin, or bleomycin.[1–3] Given the advanced age and comorbidity of many patients with advanced or recurrent vulvar cancer, patient tolerance is a major consideration in the use of these agents.
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
References
Eifel PJ, Klopp AH, Berek JS, et al.: Cancer of the cervix, vagina, and vulva. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 11th ed. Wolters Kluwer, 2019, pp 1171-1210.
van Doorn HC, Ansink A, Verhaar-Langereis M, et al.: Neoadjuvant chemoradiation for advanced primary vulvar cancer. Cochrane Database Syst Rev 3: CD003752, 2006. [PUBMED Abstract]
Cormio G, Loizzi V, Gissi F, et al.: Cisplatin and vinorelbine chemotherapy in recurrent vulvar carcinoma. Oncology 77 (5): 281-4, 2009. [PUBMED Abstract]
Treatment of Recurrent Vulvar Cancer
Treatment Options for Recurrent Vulvar Cancer
Treatment options for recurrent vulvar cancer include:
Wide local excision with or without radiation therapy in patients with local recurrence.
Radical vulvectomy and pelvic exenteration in patients with local recurrence.
Synchronous radiation therapy and cytotoxic chemotherapy with or without surgery.[1]
Treatment and outcome depend on the site and extent of recurrence.[2] Radical excision of localized recurrence may be considered if technically feasible.[3] Palliative radiation therapy is used for some patients. Radiation therapy with or without chemotherapy may be associated with substantial disease-free periods in some patients with a small local recurrence.[1,4,5] When local recurrence occurs more than 2 years after primary treatment, a combination of radiation therapy and surgery may result in a 5-year survival rate of greater than 50%.[6,7]
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
References
Russell AH, Mesic JB, Scudder SA, et al.: Synchronous radiation and cytotoxic chemotherapy for locally advanced or recurrent squamous cancer of the vulva. Gynecol Oncol 47 (1): 14-20, 1992. [PUBMED Abstract]
Piura B, Masotina A, Murdoch J, et al.: Recurrent squamous cell carcinoma of the vulva: a study of 73 cases. Gynecol Oncol 48 (2): 189-95, 1993. [PUBMED Abstract]
Hopkins MP, Reid GC, Morley GW: The surgical management of recurrent squamous cell carcinoma of the vulva. Obstet Gynecol 75 (6): 1001-5, 1990. [PUBMED Abstract]
Miyazawa K, Nori D, Hilaris BS, et al.: Role of radiation therapy in the treatment of advanced vulvar carcinoma. J Reprod Med 28 (8): 539-41, 1983. [PUBMED Abstract]
Thomas G, Dembo A, DePetrillo A, et al.: Concurrent radiation and chemotherapy in vulvar carcinoma. Gynecol Oncol 34 (3): 263-7, 1989. [PUBMED Abstract]
Podratz KC, Symmonds RE, Taylor WF, et al.: Carcinoma of the vulva: analysis of treatment and survival. Obstet Gynecol 61 (1): 63-74, 1983. [PUBMED Abstract]
Shimm DS, Fuller AF, Orlow EL, et al.: Prognostic variables in the treatment of squamous cell carcinoma of the vulva. Gynecol Oncol 24 (3): 343-58, 1986. [PUBMED Abstract]
Latest Updates to This Summary (05/14/2025)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary.
This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® Cancer Information for Health Professionals pages.
About This PDQ Summary
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of vulvar cancer. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.
Reviewers and Updates
This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
be discussed at a meeting,
be cited with text, or
replace or update an existing article that is already cited.
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewers for Vulvar Cancer Treatment are:
Olga T. Filippova, MD (Lenox Hill Hospital)
Marina Stasenko, MD (New York University Medical Center)
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website’s Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.
Levels of Evidence
Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.
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PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”
The preferred citation for this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Vulvar Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/vulvar/hp/vulvar-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389203]
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Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
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