What is Leiomyosarcoma?

| June 2, 2012 | 1 Comment

Leiomyosarcoma

Leiomyosarcoma belongs to a group of cancers called soft tissue sarcomas. Sarcomas are cancers that develop in the supporting or connective tissues of the body, such as muscle, fat, nerves, blood vessels, bone and cartilage. Soft tissue sarcomas are rare. Approximately 1,200-2,000 people will be diagnosed with a sarcoma each year in the UK. Most people with leiomyosarcoma will be over the age of 50.

Leiomyosarcomas are one of the more common types of sarcoma to occur in adults. They start from cells in a type of muscle tissue called smooth muscle.

Smooth muscles are involuntary muscles that we have no control over. They are found in the walls of muscular organs like the heart and stomach as well as the walls of blood vessels throughout the body.

This means that leiomyosarcomas can start anywhere in the body. Common places are the walls of the womb (uterus), the limbs and the digestive system, particularly the stomach.

Causes of leiomyosarcoma

The exact causes of leiomyosarcoma are unknown, and research is ongoing to try to find out as much as possible about them.

Very rarely, soft tissue sarcomas may occur in an area that has previously been treated with radiotherapy for another type of cancer. The sarcoma will not usually develop until at least 10 years after the radiotherapy treatment. Improvements in targeted radiotherapy mean that the risk of developing a sarcoma after radiotherapy treatment is very small.

Exposure to some types of chemicals may increase the risk of developing some sarcomas. The chemicals include vinyl chloride (used for making plastics), some types of herbicides (weedkillers) and dioxins.

Signs and symptoms

People with early leiomyosarcoma often do not have any symptoms. Most leiomyosarcomas are diagnosed after a person develops symptoms. These may include:

  • a lump or swelling
  • abdominal discomfort or bloatedness
  • swelling or pain in any area of the body
  • bleeding from the vagina in women who have      had the menopause or a change in periods for women who have not yet had      the menopause.

If you notice any of the above, contact your GP, but remember that these symptoms can also be caused by many other things.

How it is diagnosed

Usually you begin by seeing your family doctor (GP), who will examine you. You will be referred to a hospital specialist for any tests that may be necessary and for expert advice and treatment. The doctor at the hospital will take your full medical history, do a physical examination and take blood samples to check your general health.

The following tests are commonly used to diagnose a leiomyosarcoma. The tests you have will depend on the part of the body being investigated. You may have had some of these tests already. If you are having investigations other than those listed, our cancer support specialists| can give you further information.

Endoscopy

This is the most common test used to diagnose problems in the stomach and oesophagus (gullet). Before an endoscopy, your stomach has to be empty so you will be asked not to eat or drink anything for at least four hours beforehand. Once you’re lying comfortably on the couch you will usually be given a sedative. This is generally injected into a vein in your arm, to make you feel sleepy and reduce any discomfort during the test. A local anaesthetic is then sprayed onto the back of your throat and the doctor passes an endoscope (a flexible tube with a light and lens at the end) down the gullet into the stomach.

Photographs are taken of the stomach, and a small sample of cells (a biopsy) can be taken for examination under a microscope. Sometimes the endoscopy tube has an ultrasound probe at the end, which allows an ultrasound scan to be taken of the stomach and surrounding structures. This is known as endoscopic ultrasound.

An endoscopy can be uncomfortable, but it is not painful. After a few hours, the effects of the sedative should have worn off and you will be able to go home. You should not drive for several hours afterwards, and it’s a good idea to arrange for someone to travel home with you. Some people have a sore throat after their endoscopy. This should disappear after a couple of days.

Hysteroscopy

This test is used to diagnose problems in the womb. The doctor uses a small, thin tube with a light and camera at the end (hysteroscope), to look into the womb and take tissue samples to be looked at under a microscope. The hysteroscope is passed through your vagina and into your womb. You may have this test as an outpatient under local anaesthetic, but sometimes a general anaesthetic is needed.

A hysteroscopy may be uncomfortable but should not be painful. Some women may have mild cramping during the procedure and for a few days afterwards.

Ultrasound scan

This test uses sound waves to make up a picture of the abdomen and surrounding organs. It is done in the hospital’s scanning department. You will be asked not to eat and to only drink clear fluids (nothing fizzy or milky) for 4-6 hours before the scan. Once you are lying comfortably on your back, a gel is spread over your abdomen. A small device like a microphone (called a probe) is then rubbed over the area. The sound waves are converted into a picture using a computer. Sometimes the probe is inserted gently into the vagina to examine the womb more closely. The test should not be painful and takes about 15–20 minutes.

CT (computerised tomography) scan

A CT scan takes a series of x-rays that build up a three-dimensional picture of the inside of the body. The scan is painless and takes 10-30 minutes. CT scans use small amounts of radiation, which is very unlikely to harm you or anyone you come into contact with. You will be asked not to eat or drink for at least four hours before the scan.

You may be given a drink or an injection of dye that allows particular areas to be seen more clearly. For a few minutes this may make you feel hot all over. If you are allergic to iodine or have asthma, you could have a more serious reaction to the injection, so it is important to let your doctor know beforehand.

You will probably be able to go home as soon as the scan is over.

MRI (magnetic resonance imaging) scan

This test is similar to a CT scan but uses magnetism instead of x-rays to build up cross-sectional pictures of your body. During the test you will be asked to lie very still on a couch inside a large metal cylinder that is open at both ends. The whole test may take up to an hour. It can be slightly uncomfortable and some people feel a bit claustrophobic during the scan. It is also very noisy, but you will be given earplugs or headphones to wear. You will be able to hear and speak to the person operating the scanner.

If you have any metal implants (such as certain types of surgical clips, pacemakers or metal in the eye from previous accidents or trauma) it will not be possible for you to have this test.

Biopsy

The results of the previous tests may make your doctor strongly suspect you have cancer. The only way to be sure is to take some cells or a small piece of tissue from the affected area to look at under a microscope. This is called a biopsy. A fine needle is passed into the tumour through the skin after the area has been numbed using a local anaesthetic injection. CT or ultrasound may be used at the same time to make sure that the biopsy is taken from the right place.

When the cells are looked at under a microscope, the pathologist will be able to tell whether they are benign (not cancerous) or malignant (cancerous). If a sarcoma is diagnosed, further tests may be done on the sample to try to find out exactly what type of sarcoma it is.

Grading and staging

Grading

Grading refers to the appearance of cancer cells under a microscope. The grade gives an idea of how quickly a cancer may develop.

Grading of soft tissue sarcomas can sometimes be difficult, especially for the less common types.

Low-grade means that the cancer cells look very much like the normal cells of the soft tissues. They are usually slow-growing and are less likely to spread. In high-grade tumours the cells look very abnormal. They are likely to grow more quickly and are more likely to spread.

Staging

The stage of a cancer is a term used to describe its size and whether it has spread beyond its original site. Knowing the particular type and the stage of the cancer helps the doctors decide on the most appropriate treatment.

The following is a commonly used staging system for non-gynaecological leiomyosarcoma. (A different system is used for leiomyosarcoma arising in the gynecological organs (see below). Your specialist can explain more if you have this type of leiomyosarcoma.)

Stage 1A

The tumour is low-grade and small (less than 5cm [2in]). It can be near the surface of the body (superficial) or deep within the body, but with no sign that it has spread to the lymph nodes or other parts of the body.

Stage 1B

The tumour is low-grade and large (more than 5cm [2in]). It is superficial with no sign it has spread to the lymph nodes or other parts of the body.

Stage 2A

The tumour is low-grade and large (more than 5cm [2in]). It is deep within the body but has not spread to lymph nodes or other parts of the body.

Stage 2B

The tumour is high-grade and small (less than 5cm [2in]). It can be near the surface of the body or deep within the body, but has not spread to lymph nodes or other parts of the body.

Stage 2C

The tumour is high-grade, large, (more than 5cm [2in]) and superficial, but has not spread to lymph nodes or other parts of the body.

Stage 3

The tumour is high-grade, large (more than 5cm [2in]) and deep, but has not spread.

Stage 4

The tumour has spread to lymph nodes in the area or to any other part of the body. This is known as secondary or metastatic soft tissue sarcoma.

Recurrence

This means that a soft tissue sarcoma has come back after it was first treated. It may come back in the tissues where it first started (local recurrence) or in another part of the body (metastasis).

Treatment

The treatment for leiomyosarcoma| depends on a number of things, including your general health and the size and position of the tumour within the body. The results of your tests will help your doctor decide on the best type of treatment for you. They can then discuss this with you.

The usual treatment for a leiomyosarcoma is surgery|, wherever possible, to remove the tumour. This may be followed by radiotherapy| to reduce the chance of the cancer coming back.

Chemotherapy| is also used for some leiomyosarcomas. This may be to try to reduce the chances of the leiomyosarcoma coming back or to treat a leiomyosarcoma that has spread.

Clinical trials

Research into treatments for leiomyosarcoma is ongoing and advances are being made. Cancer doctors use clinical trials| to assess new treatments.

Before any trial is allowed to take place, it must be approved by an ethics committee, which protects the interests of the patients taking part.

You may be asked to take part in a clinical trial. If you decide to take part, your doctor will discuss the treatment with you so that you have a full understanding of the trial and what it means to take part. You may decide not to take part or withdraw from a trial at any stage. You’ll then receive the best standard treatment available.

Your feelings

The need for practical and emotional support will vary from person to person and may depend on the treatment you receive and any side effects the treatment may cause. Your specialist will inform you of any potential side effects and how to deal with them before you agree to treatment.

You may experience many different emotions|. Anger|, guilt, anxiety| and fear are some of the most common feelings people have. You may find yourself tearful, restless and unable to sleep|. You may have feelings of hopelessness and depression|. These are all normal reactions but it is often difficult and distressing to admit to them.

You don’t have to cope with these feelings on your own and there are people available to help you. Some hospitals have their own emotional support services with specially trained staff, and some of the nurses on the ward will have received training in counselling. Some people feel more comfortable talking to a counsellor outside the hospital environment or to a member of their religious faith. We can put you in touch with counselling services| in your area.

You may also find it helpful to talk to your family and friends about how you feel.

 Source:

http://www.macmillan.org.uk/Cancerinformation/Cancertypes/Softtissuesarcomas/Typesofsofttissuesarcomas/Leiomyosarcoma.aspx

 

Gynaecological Leiomyosarcomas Staging – FIGO 2009

Stage I  Tumour limited to uterus

IA  <5 cm

IB  >5 cm

Stage II  Tumour extends to the pelvis

IIA  Adnexal involvement

IIB  Tumour extends to extrauterine pelvic tissue

Stage III  Tumour invades abdominal tissues (not just protruding into the abdomen)

IIIA  One site

IIIB  > one site

IIIC  Metastasis to pelvic and/or para-aortic lymph nodes

Stage IV 

IVA  Tumour invades bladder and/or rectum

IVB  Distant metastasis

 

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