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General Information About Uterine Sarcoma

Uterine sarcoma is a disease in which malignant (cancer) cells form in the 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. (Information about other types of sarcomas can be found in the PDQ summary on Adult Soft Tissue Sarcoma Treatment.) Uterine sarcoma is different from cancer of the endometrium, a disease in which cancer cells start growing inside the lining of the uterus. (See the PDQ summary on Endometrial Cancer Treatment for information).

Being exposed to x-rays can affect the risk of developing uterine sarcoma.

Past treatment with radiation therapy to the pelvis is a risk factor for uterine sarcoma.

Possible signs of uterine sarcoma include abnormal bleeding.

Abnormal bleeding from the vagina and other symptoms may be caused by uterine sarcoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

• 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. The doctor or nurse inserts one or two lubricated, gloved fingers of one hand into the vagina and the other hand is placed over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. A speculum is also inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test or Pap smear of the cervix is usually done. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas.


• 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.


• Dilatation and curettage: A surgical procedure to remove samples of tissue or the inner lining of the uterus. The cervix is dilated and a curette (spoon-shaped instrument) is inserted into the uterus to remove tissue. Tissue samples may be taken for biopsy. 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).

Stages of Uterine Sarcoma

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:

• 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.


• 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.


• 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 in the organ or tissue that produces it.


• 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.


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


• Sigmoidoscopy: A procedure to look inside the rectum and sigmoid (lower) colon for polyps, abnormal areas, or cancer. A sigmoidoscope (a thin, lighted tube) is inserted through the rectum into the sigmoid colon. Polyps or tissue samples may be taken for biopsy.


• Barium enema: A series of x-rays of the lower gastrointestinal tract. A liquid that contains barium (a silver-white metallic compound) is put into the rectum. The barium coats the lower gastrointestinal tract and x-rays are taken. This procedure is also called a lower GI series.

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 for biopsy.


• 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: A surgical procedure to remove both ovaries and both fallopian tubes.


• Lymphadenectomy: A surgical procedure in which lymph nodes are removed and examined to see whether they contain cancer. This procedure is also called lymph node dissection. For a regional lymph node dissection, some of the lymph nodes in the tumor area are removed. For a radical lymph node dissection, most or all of the lymph nodes in the tumor area are removed.

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

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 stage IA, stage IB, and stage IC, based on how far the cancer has spread.

• Stage IA: Cancer is in the endometrium only.


• Stage IB: Cancer has spread into the inner half of the myometrium (muscle layer of the uterus).


• Stage IC: Cancer has spread into the outer half of the myometrium.

Stage II

In stage II, cancer has spread from the uterus to the cervix. Stage II is divided into stage IIA and stage IIB, based on how far the cancer has spread.

• Stage IIA: Cancer has spread to the glands where the cervix and uterus meet.


• Stage IIB: Cancer has spread into the connective tissue of the cervix.

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 stage IIIA and stage IIIB, based on how far the cancer has spread within the pelvis.

• Stage IIIA: Cancer has spread to one or more of the following:
  
  - the outermost layer of the uterus; and/or
  - tissues just beyond the uterus; and/or
  - the peritoneum.
  


• Stage IIIB: Cancer has spread to lymph nodes in the pelvis and/or near the uterus.

Stage IV

In stage IV, cancer has spread beyond the pelvis. Stage IV is divided into stage IVA and stage 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 lymph nodes in the abdomen and/or groin.

Recurrent Uterine Sarcoma

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

Treatment Option Overview

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. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

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.

Even if 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 increase the chances of a cure, is called adjuvant therapy.

Radiation therapy

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

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, 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 are used to reduce the production of hormones or block them from working.

New types of treatment are being tested in clinical trials.

Information about ongoing clinical trials is available from the NCI Web site.

Treatment Options by Stage

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).


• Surgery followed by radiation therapy to the pelvis.


• 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.

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.

Treatment Options for Recurrent Uterine Sarcoma

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.

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.

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