Uterine sarcoma is a disease in which malignant (cancer) cells form inthe muscles of the uterus or other tissues that support the uterus.

The uterus is part of the female reproductive system. The uterus is the hollow, pear-shaped organ in the pelvis, where a fetus grows. The cervix is at the lower,narrow end of the uterus, and leads to the vagina.

Uterine sarcoma is a very rare kind of cancer that forms in the uterine muscles or in tissues that support the uterus. Uterine sarcoma is different from cancer of the endometrium, a disease in which cancer cells start growing inside the lining of the uterus.

Past treatment with radiation therapy to the pelvis can increase the risk of uterine sarcoma.

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 uterine sarcoma include the following:

• Past treatment with radiation therapy to the pelvis.
• Treatment with tamoxifen for breast cancer. If you are taking this drug, have a pelvic exam every year and reportany vaginal bleeding (other thanmenstrual bleeding) as soon aspossible.

Signs of uterine sarcoma include abnormal bleeding.

Abnormal bleeding from the vagina and other signs and symptoms may be caused by uterine sarcoma or by other conditions.Check with your doctor if you have any of the following:

• Bleeding that is not part of menstrual periods.
• Bleeding after menopause.
• A mass in the vagina.
• Pain or a feeling of fullness in the abdomen.
• Frequent urination.

Tests that examine the uterus are used to detect (find) and diagnose uterine sarcoma.

The following tests and procedures may be used:

• Physical exam and history: An exam of the body to check general signs of health, including checking 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.
• 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.

• 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. This procedure is also called a Pap smear. Because uterine sarcoma begins inside the uterus, this cancer may not show up on the Pap test.

• Transvaginal ultrasound exam: A procedure used to examine the vagina, uterus, fallopian tubes, and bladder. An ultrasound transducer (probe) is inserted into the vagina and used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The doctor can identify tumors by looking at the sonogram.

• Dilatation and curettage: A procedure to remove samples of tissue from the inner lining of the uterus. The cervix is dilated and a curette (spoon-shaped instrument) is inserted into the uterus to remove tissue. The tissue samples are checked under a microscope for signs of disease. This procedure is also called a D&C.

• Endometrial biopsy: The removal of tissue from the endometrium (inner lining of the uterus) by inserting a thin, flexible tube through the cervix and into the uterus. The tube is used to gently scrape a small amount of tissue from the endometrium and then remove the tissue samples. A pathologist views the tissue under a microscope to look for cancer cells.

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

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

• The stage of the cancer.
• The type and size of the tumor.
• The patient's general health.
• Whether the cancer has just been diagnosed or has recurred (come back).

There is no way to know for sure if you will get uterine cancer. Some women get it without being at high risk. However, several factors may increase the chance that you will get uterine cancer, including if you—

• Are older than 50.
• Are obese (have an abnormally high, unhealthy amount of body fat).
• Take estrogen by itself (without progesterone) for hormone replacement during menopause.
• Have had trouble getting pregnant, or have had fewer than five periods in a year before starting menopause.
• Take tamoxifen, a drug used to treat certain types of breast cancer.
• Have close family members who have had uterine, colon, or ovarian cancer.

If one or more of these things is true for you, it does not mean you will get uterine cancer. But you should speak with your doctor to see if he or she recommends more frequent exams.

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

The process used to find out if cancer has spread within the uterus 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 procedures may be used in the staging process:

• 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.
• CA 125 assay: A test that measures the level of CA 125 in the blood. CA 125 is a substance released by cells into the bloodstream. An increased CA 125 level is sometimes a sign of cancer or other condition.
• 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.
• Transvaginal ultrasound exam: A procedure used to examine the vagina, uterus, fallopian tubes, and bladder. An ultrasound transducer (probe) is inserted into the vagina and used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The doctor can identify tumors by looking at the sonogram.

• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen and pelvis, 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 to show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
• 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.

Uterine sarcoma may be diagnosed, staged, and treated in the same surgery.

Surgery is used to diagnose, stage, and treat uterine sarcoma. During this surgery, the doctor removes as much of the cancer as possible. The following procedures may be used to diagnose, stage, and treat uterine sarcoma:

• Laparotomy: A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease.
• Abdominal and pelvic washings: A procedure in which a saline solution is placed into the abdominal and pelvic body cavities. After a short time, the fluid is removed and viewed under a microscope to check for cancer cells.
• Total abdominal hysterectomy: A surgical procedure to remove the uterus and cervix through a large incision (cut) in the abdomen.

• Bilateral salpingo-oophorectomy: Surgery to remove both ovaries and both fallopian tubes.
• Lymphadenectomy: A surgical procedure in which lymph nodes are removed and checked under a microscope for signs of cancer. For a regional lymphadenectomy, some of the lymph nodes in the tumor area are removed. For a radical lymphadenectomy, most or all of the lymph nodes in the tumor area are removed. This procedure is also called lymph node dissection.

Treatment in addition to surgery may be given, as described in the Treatment Option Overview section of this summary.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

• Tissue. The cancer spreads from where it began by growing into nearby areas.
• Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
• Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

• Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
• Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if uterine sarcoma spreads to the lung, the cancer cells in the lung are actually uterine sarcoma cells. The disease is metastatic uterine sarcoma, not lung cancer.

The following stages are used for uterine sarcoma:

Stage I

In stage I, cancer is found in the uterus only. Stage I is divided into stages IA and IB, based on how far the cancer has spread.

• Stage IA: Cancer is in the endometrium only or less than halfway through the myometrium (muscle layer of the uterus).
• Stage IB: Cancer has spread halfway or more into the myometrium.

Stage II

In stage II, cancer has spread into connective tissue of the cervix, but has not spread outside the uterus.

Stage III

In stage III, cancer has spread beyond the uterus and cervix, but has not spread beyond the pelvis. Stage III is divided into stages IIIA, IIIB, and IIIC, based on how far the cancer has spread within the pelvis.

• Stage IIIA: Cancer has spread to the outer layer of the uterus and/or to the fallopian tubes, ovaries, and ligaments of the uterus.
• Stage IIIB: Cancer has spread to the vagina or to the parametrium (connective tissue and fat around the uterus).
• Stage IIIC: Cancer has spread to lymph nodes in the pelvis and/or around the aorta (largest artery in the body, which carries blood away from the heart).

Stage IV

In stage IV, cancer has spread beyond the pelvis. Stage IV is divided into stages IVA and IVB, based on how far the cancer has spread.

• Stage IVA: Cancer has spread to the bladder and/or bowel wall.
• Stage IVB: Cancer has spread to other parts of the body beyond the pelvis, including the abdomen and/or lymph nodes in the groin.

Recurrent uterine sarcoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the uterus, the pelvis, or in other parts of the body.

There are different types of treatment for patients with uterine sarcoma.

Different types of treatments are available for patients with uterine sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Four types of standard treatment are used:

Surgery

Surgery is the most common treatment for uterine sarcoma, as described in the Stages of Uterine Sarcoma section of this summary.

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

Radiation therapy is a cancer treatment that uses high energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:

• External radiation therapy uses a machine outside the body to send radiation toward the cancer.
• Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat uterine sarcoma, and may also be used aspalliative therapy to relievesymptoms and improve quality of life.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping 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). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Hormone therapy

Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances produced by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show 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.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment for uterine sarcoma may cause side effects.

For information about side effects caused by treatment for cancer, see our Side Effects page.

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.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Stage I Uterine Sarcoma

Treatment of stage I uterine sarcoma may include the following:

• Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and lymphadenectomy).
• Surgery followed by radiation therapy to the pelvis.
• Surgery followed by chemotherapy.

Stage II Uterine Sarcoma

Treatment of stage II uterine sarcoma may include the following:

• Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and lymphadenectomy).
• Surgery followed by radiation therapy to the pelvis.
• Surgery followed by chemotherapy.

Stage III Uterine Sarcoma

Treatment of stage III uterine sarcoma may include the following:

• Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and lymphadenectomy).
• A clinical trial of surgery followed by radiation therapy to the pelvis.
• A clinical trial of surgery followed by chemotherapy.

Stage IV Uterine Sarcoma

There is no standard treatment for patients with stage IV uterine sarcoma. Treatment may include a clinical trial using chemotherapy.

There is no standard treatment for recurrent uterine sarcoma. Treatment may include a clinical trial using chemotherapy.

For patients with recurrent carcinosarcoma (a certain type of tumor), treatment may include the following:

• Radiation therapy as palliative therapy to relieve symptoms (such as pain, nausea, or bowel problems) and improve the quality of life.
• Hormone therapy.
• A clinical trial of a new treatment.