Wednesday, September 14, 2016

Uterine Sarcomas

What is Uterine Sarcomas?
Cancer starts when cells in the body begin to grow out of control. Cells in nearly any part of the body can become cancer, and can spread to other areas of the body. To learn more about how cancers start and spread, see What Is Cancer?
Uterine sarcoma is a cancer of the muscle and supporting tissues of the uterus (womb).

About the uterus

The uterus is a hollow organ, about the size and shape of a medium-sized pear. It has two main parts:
  • The lower end of the uterus, which extends into the vagina, is called the cervix.
  • The upper part of the uterus is called the body, and is also known as the corpus.
The body of the uterus has 3 layers. The inner layer or lining is called the endometrium. The serosa is the layer of tissue coating the outside of the uterus. In the middle is a thick layer of muscle that is also known as the myometrium. This muscle layer is needed to push a baby out during childbirth.

Cancers of the uterus and endometrium

Sarcomas are cancers that start from tissues such as muscle, fat, bone, and fibrous tissue (the material that forms tendons and ligaments). Cancers that start in epithelial cells, the cells that line or cover most organs, are called carcinomas.
More than 95% of cancers of the uterus are carcinomas. If a carcinoma starts in the cervix, it is called a cervical carcinoma. Carcinomas starting in the endometrium, the lining of the uterus, are called endometrial carcinomas. These cancers are discussed in our other documents Cervical Cancer and Endometrial (Uterine) Cancer. This document is only about uterine sarcomas.
Most uterine sarcomas are put into categories, based on the type of cell they developed from:
  • Endometrial stromal sarcomas develop in the supporting connective tissue (stroma) of the endometrium. These cancers are rare, representing less than 1% (1 in 100) of all uterine cancers. These tumors are low grade -- the cancer cells do not look very abnormal and they tend to grow slowly. Patients with these tumors have a better outlook than those with other uterine sarcomas .
  • Undifferentiated sarcomas used to be considered a type of endometrial stromal sarcoma, but since they are more aggressive and are treated differently from low-grade tumors, they are now considered separately. These cancers make up less than 1% of all uterine cancers and tend to have a poor outlook.
  • Uterine leiomyosarcomas start in the muscular wall of the uterus known as the myometrium. These tumors make up about 2% of cancers that start in the uterus.
Another type of cancer that starts in the uterus is called carcinosarcoma. These cancers start in the endometrium and have features of both sarcomas and carcinomas. They can be classified with uterine sarcomas, but many doctors now believe they are more closely related to carcinomas. These cancers are also known as malignant mixed mesodermal tumors or malignant mixed mullerian tumors. Uterine carcinosarcomas are discussed in detail in our document Endometrial (Uterine) Cancer.

Benign uterine tumors

Several types of benign (non-cancerous) tumors can also develop in the connective tissues of the uterus. These tumors, such as leiomyomas, adenofibromas, and adenomyomas, are also known as types of fibroid tumors. Most of the time, these tumors require no treatment. Treatment may be needed, however, if they start causing problems--- such as pelvic pain, heavy bleeding, frequent urination, or constipation. In some cases, the tumor is removed, leaving the rest of the uterus in place. This surgery is called a myomectomy. Some treatments destroy these benign tumors without surgery, by blocking the blood vessels that feed them, by killing the tumor cells with electric current, or by freezing them with liquid nitrogen. Another option is to remove the entire uterus. This surgery is called a hysterectomy

What are the key statistics about uterine sarcoma?

The American Cancer Society's estimates for cancer of the uterine corpus (body of the uterus) in the United States for 2016 are:
  • About 60,050 new cases of cancer of the uterine corpus will be diagnosed, but only about 1,600 of these cases will be uterine sarcomas.
  • About 10,470 women in the United States will die from cancer of the uterine corpus.
 
 symptoms:

In most cases, the possibility of uterine sarcoma is suggested by certain symptoms. These symptoms do not always mean that a woman has a uterine sarcoma. In fact, they are more often caused by something else, such as non-cancerous changes in the uterus, pre-cancerous overgrowth of the endometrium, or endometrial carcinoma. Still, if you are having these problems, you should see a doctor to see find the cause and get any needed treatment.

Abnormal bleeding or spotting

If you have gone through menopause, any vaginal bleeding or spotting is abnormal, and it should be reported to your health care professional right away. About 85% of patients diagnosed with uterine sarcomas have irregular vaginal bleeding (between periods) or bleeding after menopause. This symptom is more often caused by something other than cancer, but it is important to have a medical evaluation of any irregular bleeding right away. Of the uterine sarcomas, leiomyosarcomas are less likely to cause abnormal bleeding than endometrial stromal sarcomas and undifferentiated sarcomas.

Vaginal discharge

About 10% of women with uterine sarcomas have a vaginal discharge that does not have any visible blood. A discharge is most often a sign of infection or another benign condition, but it also can be a sign of cancer. Any abnormal discharge should be investigated by your health care professional.

Pelvic pain and/or a mass

When they are first diagnosed, about 10% of women with uterine sarcomas have pelvic pain and/or a mass (tumor) that can be felt. You or your doctor may be able to feel the mass in your uterus, or you might have a feeling of fullness in your pelvis.

Staging is the process of analyzing information about a tumor to find out how far the cancer has spread. The stage of a uterine sarcoma is an important factor in choosing treatment. Ask your health care team to explain your cancer's stage so that you can make fully informed choices about your treatment.
The systems used for staging uterine sarcoma, the FIGO (International Federation of Gynecology and Obstetrics) system and the American Joint Committee on Cancer TNM staging system, are the same.
Staging is based on: the size or extent of the tumor (T), whether the cancer has spread to lymph nodes (N) and whether it has spread to distant sites (M). Uterine sarcoma is staged based on examination of tissue removed during an operation. This is known as surgical staging and means that doctors can't tell for sure what stage the cancer is in until after surgery is done (in most cases). The staging system classifies the cancer in stages I through IV, with each of these stages being further divided by letters (for example, stages IIA and IIB).
The staging system looks at how far the cancer has spread:
  • It can spread locally to other parts of the uterus and to tissues of the pelvis, including the fallopian tubes, ovaries, and tissue near the uterus.
  • It can also spread regionally to nearby lymph nodes (bean-sized organs that are part of the immune system) and other parts of the pelvis. The regional lymph nodes are found in the pelvis and farther away along the aorta (the main artery that runs from the heart down along the back of the abdomen and pelvis). The lymph nodes along the aorta are called the para-aortic nodes.
  • Finally, the cancer can spread to distant lymph nodes or organs such as the lungs, liver, bone, brain, and others.

Stages:

Tumor extent (T)

T0: No signs of a tumor in the uterus can be found.
T1: The tumor is growing in the uterus, but has not started growing outside the uterus.
  • T1a: The tumor is only in the uterus and is no larger than 5 cm across (5 cm is about 2 inches).
  • T1b: The tumor is only in the uterus and is larger than 5 cm across.
T2: The tumor is growing outside the uterus but is not growing outside of the pelvis.
  • T2a: The tumor is growing into the adnexa (the ovaries and fallopian tubes).
  • T2b: The tumor is growing into tissues of the pelvis other than the adnexa.
T3: The tumor is growing into tissues of the abdomen.
  • T3a: The tumor is growing into tissues of the abdomen in one place only.
  • T3b: The tumor is growing into tissues of the abdomen in 2 or more places.
T4: The tumor is growing into the bladder or rectum.

Lymph node spread (N)

NX: Spread to nearby lymph nodes cannot be assessed.
N0: The cancer has not spread to nearby lymph nodes.
N1: Cancer has spread to nearby lymph nodes.

Distant spread (M)

M0: The cancer has not spread to distant lymph nodes, organs, or tissues.
M1: The cancer has spread to distant organs (such as the lungs or liver).

Stage grouping

Information about the tumor, lymph nodes, and any cancer spread is combined to assign the stage of disease. This process is called stage grouping. The stages are described using Roman numerals from I to IV. Some stages are divided into sub-stages indicated by letters.
Stage I (T1, N0, M0): The cancer is only in the uterus (T1). It has not spread to lymph nodes (N0) or distant sites (M0).
  • Stage IA (T1a, N0, M0): The cancer is only in the uterus and is no larger than 5 cm across (T1a). It has not spread to nearby lymph nodes (N0) or distant sites (M0).
  • Stage IB (T1b, N0, M0): The cancer is only in the uterus and is larger than 5 cm across (T1b). It has not spread to nearby lymph nodes (N0) or distant sites (M0).
Stage II (T2, N0, M0): The cancer is growing outside the uterus but is not growing outside of the pelvis (T2). The cancer has not spread to nearby lymph nodes (N0) or distant sites (M0).
  • Stage IIA (T2a, N0, M0): The cancer is growing into the adnexa (the ovaries and fallopian tubes) (T2a). It has not spread to nearby lymph nodes (N0) or distant sites (M0).
  • Stage IIB (T2b, N0, M0): The cancer is growing into tissues of the pelvis other than the adnexa (T2b). It has not spread to nearby lymph nodes (N0) or distant sites (M0).
Stage III: Any of the following:
  • Stage IIIA (T3a, N0, M0): The cancer is growing into tissues of the abdomen in one place only (T3a). It has not spread to nearby lymph nodes (N0) or distant sites (M0).
  • Stage IIIB (T3b, N0, M0): The cancer is growing into tissues of the abdomen in 2 or more places (T3b). It has not spread to lymph nodes (N0) or distant sites (M0).
  • Stage IIIC (T1 to T3, N1, M0): The cancer in the uterus can be any size and may have grown into tissues in the pelvis and/or abdomen. It has not spread to the bladder or rectum (T1 to T3). The cancer has spread to lymph nodes near the uterus (pelvic and/or para-aortic lymph nodes) (N1). It has not spread to distant sites (M0).
Stage IV: The cancer has spread to the urinary bladder or the rectum (lower part of the large intestine), and/or to distant organs, such as the bones or lungs.
  • Stage IVA (T4, any N, M0): The cancer has spread to the rectum or urinary bladder (T4). It may also be in the lymph nodes (any N) but has not spread to distant sites (M0).
  • Stage IVB (any T, any N, M1): The cancer in the uterus can be any size and may or may not have grown into tissues in the pelvis and/or abdomen (including the bladder or rectum) (any T). The cancer may or may not have spread to lymph nodes near the uterus (any N). The cancer has spread to organs that are not next to the uterus, such as the bones or lungs, or it has spread to distant lymph nodes, such as those in the groin area (M1). 

Treatment options for uterine sarcoma, by stage

Surgery to remove the uterus, fallopian tubes, and ovaries and sample the lymph nodes is the main treatment for uterine sarcomas. Sometimes this is followed by treatment with radiation, chemotherapy (chemo), or hormone therapy. Treatments given after the cancer has been completely removed with surgery are called adjuvant treatments. Adjuvant therapy is given to help keep the cancer from coming back. This approach has helped patients with certain cancers like colon and breast cancer live longer. So far, though, adjuvant treatments for uterine sarcoma have not helped patients live longer. Since uterine sarcoma is rare, it has been hard to study it well.
Women who can't have surgery because they have other health problems are treated with radiation, chemo, or hormone therapy. Often some combination of these other treatments is used.

Leiomyosarcoma and undifferentiated sarcoma

Stages I and II: Most women have surgery to remove the uterus, fallopian tubes and ovaries (hysterectomy and bilateral salpingo-oophorectomy). Pelvic and para-aortic lymph node dissection or laparoscopic lymph node sampling may also be done. During surgery, organs near the uterus and the thin membrane that lines the pelvic and abdominal cavities (peritoneum) are carefully examined to determine if the cancer has spread beyond the uterus.
In young women with low-grade leiomyosarcomas (LMS) that have not spread beyond the uterus, the surgeon may—rarely--be able to leave the uterus, fallopian tubes, and ovaries in place, and instead remove only the tumor along with a rim of the normal tissue around it. This approach is not standard treatment, so it is not often offered. It may rarely be a choice for some women who want to still be able to have children after their cancer has been treated. This option has risks, however, so women considering this surgery need to discuss the possible risks and benefits with their gynecologic oncologist before making a decision. It may also be possible to leave a young woman’s ovaries in place (but remove the uterus and fallopian tubes), since it isn’t clear that this will lead to worse outcomes. Still, this is not a standard treatment, and you should discuss the possible risks and benefits with your doctor. In either case, close follow-up is important, and additional surgery may be needed if the cancer comes back.
After surgery, treatment with radiation (or sometimes chemo) may be recommended. This is called adjuvant treatment and may lower the chance that the cancer will come back in the pelvis. The goal of surgery is to remove all of the cancer, but the surgeon can only remove what can be seen. Tiny clumps of cancer cells that are too small to be seen can be left behind. Treatments given after surgery are meant to kill those cancer cells so that they don't get the chance to grow into larger tumors.
For LMS of the uterus, adjuvant radiation may lower the chance of the cancer growing back in the pelvis (called local recurrence), but it doesn't seem to help women live longer.
Since the cancer can still come back in the lungs or other distant organs, some experts recommend giving chemo after surgery (adjuvant chemotherapy) for stage II cancers. Chemo is sometimes recommended for stage I LMS as well, but it is less clear that it is really helpful. So far, results from studies of adjuvant chemotherapy have been promising in early stage LMS, but long-term follow-up is still needed to see if this treatment really helps women live longer. Studies of adjuvant therapy are still in progress. For anyone being treated for uterine LMS, entering a clinical trial is always a good option.
Stage III: Surgery is done to remove all of the cancer. This includes removing the uterus (a hysterectomy), removing both fallopian tubes and ovaries (bilateral salpingo-oophorectomy), and lymph node dissection or sampling. If the tumor has spread to the vagina (stage IIIB), part (or even all) of the vagina will need to be removed as well.
After surgery, treatment with radiation (with or without chemo) may be offered to lower the chance that the cancer will come back.
Patients who are too sick (from other medical conditions) to have surgery may be treated with radiation and/or chemo.
Stage IV is divided into stage IVA and stage IVb.
Stage IVA cancers have spread to nearby organs and tissues, such as the bladder or rectum. These cancers may be able to be completely removed with surgery, and this is usually done if possible. If the cancer cannot be removed completely, radiation may be given, either alone or with chemo.
Stage IVB cancers have spread outside of the pelvis, most often to the lungs, liver, or bone. There is currently no standard treatment for these cancers. Chemo may be able to shrink the tumors for a time, but is not thought to be able to cure the cancer. Radiation therapy may also be an option.
Women with stage IV uterine sarcomas might consider taking part in clinical trials (scientific studies of promising treatments) testing new chemo or other treatments.

Endometrial stromal sarcoma

Stages I and II: Early stage endometrial stromal sarcoma is treated with surgery: hysterectomy and bilateral salpingo-oophorectomy (removal of the uterus, both fallopian tubes and both ovaries). Some young women may be given the option of keeping their ovaries, but this is not the standard treatment. Pelvic lymph nodes may be removed as well. After surgery, some women do not get further treatment. These women are watched closely for signs that the cancer has returned. Others may be treated with hormone therapy and sometimes radiation to the pelvis. These can lower the chances of the cancer coming back, but they have not been shown to help patients live longer.
Patients who are too sick (from other medical conditions) to have surgery may be treated with radiation and/or hormone therapy.
Stage III: Surgery is done to remove all of the cancer. This includes removing the uterus (a hysterectomy), removing both fallopian tubes and ovaries (bilateral salpingo-oophorectomy), and lymph node dissection or sampling. If the tumor has spread to the vagina (stage IIIB), part (or even all) of the vagina will need to be removed as well. Treatment after surgery depends on the type of sarcoma.
Women with endometrial stromal sarcomas might receive radiation, hormone therapy, or both after surgery.
Patients who are too sick (from other medical conditions) to have surgery may be treated with radiation, chemo, and/or hormone therapy.
Stage IV is divided into stage IVA and stage IVb.
Stage IVA cancers have spread to nearby organs and tissues, such as the bladder or rectum. These cancers may be able to be completely removed with surgery, and this is is usually done if possible. If the cancer cannot be removed completely, radiation may be given, either alone or with chemo. Hormone therapy is also an option.
Stage IVB cancers have spread outside of the pelvis, most often to the lungs, liver, or bone. Hormone therapy can help for a time. Chemo and radiation are also options.
Women with stage IV uterine sarcomas might consider taking part in clinical trials (scientific studies of promising treatments) testing new chemo or other treatments.

Recurrent uterine sarcoma

If a cancer comes back after treatment, it is called recurrent. If the cancer comes back in the same area as it was in the first place, it is called a local recurrence. For uterine sarcoma, the cancer growing back as a tumor in the pelvis would be a local recurrence. If it comes back in another area like the liver or lungs, it is called a distant recurrence.
Unfortunately, uterine sarcoma often comes back in the first few years after treatment. Treatment options are the same as those for stage IV. If the cancer can be removed, surgery may be done. Radiation may be used to reduce the size of the tumor and relieve the symptoms of large pelvic tumors. Sarcoma often comes back as spread to the lungs. If there are only 1 or 2 small tumors, these may be able to be removed with surgery. Some patients have been cured by this treatment.

What will happen after treatment for uterine sarcoma?

For some people with cancer, treatment may remove or destroy the cancer. Completing treatment can be both stressful and exciting. You may be relieved to finish treatment, but find it hard not to worry about cancer coming back. (When cancer comes back after treatment, it is called recurrence.) This is a very common concern in people who have had cancer.
It may take a while before your fears lessen. But it may help to know that many cancer survivors have learned to live with this uncertainty and are living full lives. Our document Living With Uncertainty: The Fear of Cancer Recurrence gives more detailed information on this.
For other people, the cancer may never go away completely. These people may get regular treatments with chemotherapy, radiation therapy, or other therapies to try to help keep the cancer in check. Learning to live with cancer that does not go away can be difficult and very stressful. It has its own type of uncertainty. Our document When Cancer Doesn’t Go Away talks more about this.

Follow-up care

When treatment ends, your doctors will still want to watch you closely. It is very important to go to all of your follow-up appointments. During these visits, your doctors will ask questions about any problems you may have and may do exams and lab tests or x-rays and scans to look for signs of cancer or treatment side effects. Almost any cancer treatment can have side effects. Some may last for a few weeks to months, but others can last the rest of your life. This is the time for you to talk to your cancer care team about any changes or problems you notice and any questions or concerns you have.
It is important to keep health insurance. Tests and doctor visits cost a lot, and even though no one wants to think of their cancer coming back, this could happen.
Should your cancer come back, our document When Your Cancer Comes Back: Cancer Recurrence can give you information on how to manage and cope with this phase of your treatment.

Seeing a new doctor

At some point after your cancer diagnosis and treatment, you may find yourself seeing a new doctor who does not know anything about your medical history. It is important that you be able to give your new doctor the details of your diagnosis and treatment. Gathering these details soon after treatment may be easier than trying to get them at some point in the future. Make sure you have this information handy:
  • A copy of your pathology report(s) from any biopsies or surgeries
  • If you had surgery, a copy of your operative report
  • If you had radiation, a copy of your treatment summary
  • If you were hospitalized, a copy of the discharge summary that doctors prepare when patients are sent home from the hospital
  • If you had chemotherapy (including hormone therapy), a list of the drugs, drug doses, and when you took them
  • Copies of any imaging tests (such as CTs or MRIs) – these can often be put on a CD or DVD
  • How does having a uterine sarcoma affect your emotional health?

    When treatment ends, you may find yourself overcome with many different emotions. This happens to a lot of people. You may have been going through so much during treatment that you could only focus on getting through each day. Now it may feel like a lot of other issues are catching up with you.
    You may find yourself thinking about death and dying. Or maybe you're more aware of the effect the cancer has on your family, friends, and career. You may take a new look at your relationship with those around you. Unexpected issues may also cause concern. For instance, as you feel better and have fewer doctor visits, you will see your health care team less often and have more time on your hands. These changes can make some people anxious.
    Almost everyone who has been through cancer can benefit from getting some type of support. You need people you can turn to for strength and comfort. Support can come in many forms: family, friends, cancer support groups, church or spiritual groups, online support communities, or one-on-one counselors. What's best for you depends on your situation and personality. Some people feel safe in peer-support groups or education groups. Others would rather talk in an informal setting, such as church. Others may feel more at ease talking one-on-one with a trusted friend or counselor. Whatever your source of strength or comfort, make sure you have a place to go with your concerns.
    The cancer journey can feel very lonely. It is not necessary or good for you to try to deal with everything on your own. And your friends and family may feel shut out if you do not include them. Let them in, and let in anyone else who you feel may help. If you aren’t sure who can help, call your American Cancer Society at 1-800-227-2345 and we can put you in touch with a group or resource that may work for you.
     
 Now imagine sitting in a room and getting this information... it's a little daunting to say the least

Hope is what guides me it's what gets me through the day and especially the night...
 

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