Soft tissue sarcoma

A soft tissue sarcoma usually produces no signs and symptoms in its early stages. As the tumor grows, it may cause:

A noticeable lump or swelling
Pain, if it presses on nerves or muscles
A blockage in the stomach or intestines or gastrointestinal bleeding if the tumor is located in the abdomen or digestive tract

Soft tissue sarcomas can occur anywhere in your body, but the largest number — about 60 percent — occur in the arms, legs, hands or feet. Another 20 percent occur in the chest and abdomen. About 10 percent are found in the head and neck.

Soft tissue sarcomas go by a variety of names, depending on the tissue in which they originate. Examples of some sarcomas and their locations include:

Rhabdomyosarcoma	More common in children, this sarcoma occurs in the skeletal muscles.
Leiomyosarcoma	Occurs in the smooth muscles — muscles not under voluntary control. Found most commonly in the uterus, gastrointestinal tract or lining of blood vessels.
Hemangiosarcoma	Affects blood vessels, especially those in your arms, legs and trunk.
Kaposi's sarcoma	A malignancy that occurs in blood vessel walls. Often affects people with immune deficiencies, such as HIV/AIDS.
Lymphangiosarcoma	Affects the lymph vessels and sometimes seen in a limb with chronic swelling (lymphedema). This can be from an area of prior radiation therapy or certain rare chronic infections.
Synovial sarcoma	Tissue around joints such as knees and ankles are affected. Typically occurs in children and young adults.
Neurofibrosarcoma	Occurs in the peripheral nerves.
Liposarcoma	Fatty tissue, often in your legs and trunk, is affected.
Fibrosarcoma	Fibrous tissue in your arms, legs or trunk may be affected.
Malignant fibrous histiocytoma	A fibrous tissue tumor more likely to occur in the legs.
Dermatofibrosarcoma	Grows in the tissue beneath your skin, and often develops in your trunk or limbs.

When to see a doctor
Talk to your doctor if you discover a lump that persists or if you notice signs or symptoms that may indicate a soft tissue sarcoma, such as worsening abdominal pain or blood in your stool.

Generally, the cause of most soft tissue sarcomas is unknown. One of the few soft tissue sarcomas that has a known cause is Kaposi's sarcoma. It occurs in people with defective immune systems and is caused by a virus known as human herpes virus 8 (HHV8).

Additionally, in some cases, sarcomas may be hereditary, such as in:

Basal cell nevus syndrome. In addition to an increased risk of basal cell skin cancer, people with this disorder are more likely to develop rhabdomyosarcoma or fibrosarcoma. It's caused by a genetic defect.
Inherited retinoblastoma. This rare form of childhood eye cancer may increase a child's risk of soft tissue sarcoma and is due to inheritance of a mutated retinoblastoma gene.
Li-Fraumeni syndrome. This condition is characterized by an increased risk of many malignancies, including sarcomas, breast cancer, brain cancer and others. People with this syndrome appear to be particularly susceptible to the side effects of radiation therapy.
Gardner syndrome. This hereditary disease leads to precancerous and cancerous growths in the intestines and abdomen.
Neurofibromatosis. This condition results in developmental changes in the nervous system, causing nerve sheath tumors. About 1 in 20 people with neurofibromatosis develops malignant tumors.
Tuberous sclerosis. Benign tumors, seizures and learning problems are common with this disease, as is an increased risk of rhabdomyosarcoma.
Werner syndrome. A defect in the gene RECQL2 causes numerous health problems, including an increased risk of soft tissue sarcomas.

Radiation exposure
Radiation is occasionally associated with sarcomas. These have usually occurred as a side effect of radiation therapy given for other cancers, such as breast cancer or lymphoma. But radiation therapy is becoming more and more sophisticated, which may lead to fewer such side effects. For example, doctors today are better able to regulate doses of radiation and precisely target the tumor being treated.

Chemical exposure
Another factor that may increase your risk of soft tissue sarcoma is exposure to high doses of chemicals such as:

Vinyl chloride, used in making plastics
Dioxin, an unwanted byproduct of incineration
Herbicides that contain the chemical phenoxyacetic acid

With soft tissue sarcomas, tumors can grow large, press on normal tissue, and cause soreness or pain. If the sarcoma spreads to other organs, complications include dysfunction of the affected organ, such as shortness of breath if it spreads to your lungs.

If your family doctor suspects you have soft tissue sarcoma, you'll likely be referred to a cancer doctor (oncologist) who specializes in sarcomas. Soft tissue sarcoma is fairly rare and is best addressed by someone who has experience with it, often at an academic or specialized cancer center.

Because appointments can be brief, and there's often a lot of ground to cover, it's a good idea to arrive well prepared. Here's some information to help you get ready for your appointment, and what to expect from your doctor.

What you can do

Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
Make a list of all medications, as well as any vitamins or supplements, that you're taking.
Ask a family member or friend to come with you, if possible. Sometimes it can be difficult to remember all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions will help you make the most of your time together. List your questions from most important to least important in case time runs out. For a soft tissue sarcoma, some basic questions to ask your doctor include:

Do I have cancer?
Are there other possible causes for my symptoms?
What kinds of tests do I need to confirm the diagnosis? Do these tests require any special preparation?
What type of sarcoma do I have?
What stage is it?
What treatments are available, and which do you recommend?
What types of side effects can I expect from treatment?
Are there any alternatives to the primary approach that you're suggesting?
I have other health conditions. How can I best manage them together?
Are there any dietary or activity restrictions that I need to follow?
What's my prognosis?
Are there any brochures or other printed material that I can take with me? What Web sites do you recommend?
Can the cancer be removed?
Should I get additional treatments such as chemotherapy or radiation therapy either before or after an operation?
Is the surgeon experienced in this specific type of cancer operation?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time that you don't understand something.

What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may make time to cover other points you want to discuss. Your doctor may ask:

When did you first notice your signs and symptoms?
Are you experiencing pain?
Does anything seem to improve your symptoms?
What, if anything, appears to worsen your symptoms?
Do you have any family history of cancer? If so, do you know what type of cancer?

If it's suspected that you have sarcoma, your doctor will take a medical history, perform a physical exam and may also remove a sample of tissue (biopsy) from the tumor for examination. Techniques for biopsy of a suspected soft tissue sarcoma include:

Needle biopsy. Your doctor uses a thin needle to remove small pieces of tissue from the tumor. There are two types of needle biopsy — fine-needle aspiration and core biopsy. A core biopsy involves using a slightly larger needle to remove a small, solid core of tissue.
Surgical biopsy. Your doctor makes an incision through your skin and removes either the entire tumor (excisional biopsy) or a portion of the tumor (incisional biopsy). In some cases you may need only local anesthesia. For a larger or deep-seated tumor, you'll likely require general anesthesia for the procedure. It's important that a doctor experienced in the treatment of sarcomas perform the excisional biopsy.

Imaging tests, such as X-rays, computerized tomography (CT) scans, ultrasound, magnetic resonance imaging (MRI) and positron emission tomography (PET), also may be done to enable your doctor to see and evaluate the area of concern.

Grading and staging
Besides determining whether it is malignant, examining tissue can determine how aggressive the cancer is (its grade). Further testing, such as with imaging scans and blood tests, can help determine if the cancer has spread and, if so, how far (its stage).

Stage I. These tumors are generally small, but what's important is that the tissue cells appear normal. Additionally, the sarcoma hasn't spread to the lymph nodes or to distant sites.
Stage II. In more advanced soft tissue sarcomas, the cells begin to look abnormal and they may be dividing rapidly. At this stage, however, it still hasn't spread to the lymph nodes or other areas of the body.
Stage III. Not only do the cells look abnormal and divide rapidly, malignant cells are found in one or more lymph nodes.
Stage IV. At this stage, the cells are abnormal and rapidly dividing, and the cancer can be found in the lymph nodes and in other parts of the body.

As with other cancers, treatment for sarcomas depends on the size, type, location and stage of the sarcoma, including whether it has spread to the lymph nodes or other parts of your body, and your overall health.

Surgery
Surgery is the most common treatment for soft tissue sarcomas, especially if malignant cells haven't spread to other parts of the body. Surgery generally involves removing the cancer and some healthy tissue surrounding it. If the sarcoma has spread, surgical removal of the primary and secondary tumors may be possible.

Previously, amputation was a common treatment for soft tissue sarcomas in an arm or leg. Today, advances in surgical techniques and chemotherapy before surgery (neoadjuvant chemotherapy) or after surgery (adjuvant chemotherapy) and radiation therapy make limb-sparing surgery possible in most cases. However, amputation may be necessary to remove all of the malignant cells from soft tissue sarcoma in an arm or leg that has invaded nerves, arteries or muscles.

Radiation therapy
Radiation therapy — also called radiotherapy or X-ray therapy — involves treating cancer with beams of high-energy particles, or waves (radiation), such as gamma rays or X-rays. Although radiation can affect healthy cells as well as cancer cells, it's much more harmful to cancer cells. In addition, normal cells can recover from the effects of radiation more easily than cancer cells can.

Radiation therapy may be recommended to treat your soft tissue sarcoma. Your doctor may suggest using radiation therapy before surgery to shrink a cancerous tumor or after surgery to stop the growth of any remaining cancer cells. You may also receive chemotherapy at the same time as radiation in an effort to make the radiation more effective.

Chemotherapy
Chemotherapy uses medications to kill rapidly dividing cells. These cells include cancer cells, which continuously divide to form more cells, and healthy cells that divide quickly, such as those in your bone marrow, gastrointestinal tract, reproductive system and hair follicles. Unlike radiation therapy, which treats only the part of your body exposed to the radiation, chemotherapy treats your body as a whole (systemically). As a result, it treats cells that may have spread beyond where the cancer originated.

The role of chemotherapy in the treatment of soft tissue sarcoma isn't clear, because not all studies have had positive results. However, chemotherapy has been found effective in treating rhabdomyosarcomas.

In addition to chemotherapy, some drugs inhibit certain enzymes that are critical to the development and growth of a tumor. One of these drugs, imatinib (Gleevec), is used to treat a type of sarcoma called gastrointestinal stromal tumor. Other types of cancer-fighting drugs and new combinations of treatments are being explored in clinical trials.

A diagnosis of cancer, whether yours or your child's, presents numerous challenges. Remember that no matter what your concerns or the prognosis, there are resources and strategies that may make dealing with cancer easier. Here are some suggestions for coping:

Learn all you can. Find out everything you can about your or your child's cancer. The more you know, the more you can participate in treatment decisions. In addition to talking with your doctor, look for information in your local library and from reliable Internet sources.

The National Cancer Institute answers questions from the public over the phone at 800-4-CANCER (800-422-6237). Or contact the American Cancer Society at 800-ACS-2345 (800-227-2345).
Be proactive. Although you may feel tired and discouraged, don't let others — including your family and your doctor — make important decisions for you. It's vital that you take an active role in your or your child's treatment.
Maintain a strong support system. Having a support system and a positive attitude can help you cope with the challenges cancer brings. Although friends and family can be your best allies, they sometimes may have trouble dealing with your illness. If so, the concern and understanding of a formal support group or of others coping with their own cancer or that of their child can be especially helpful.
Take time for yourself. Eating well, relaxing and getting enough rest can help combat the stress and fatigue of cancer. Also, plan ahead for the times when you may need to rest more or limit what you do. If your child has cancer, one of the most important things you can do is take care of yourself. As a caregiver, you need to have the strength and emotional reserves to meet your child's needs.
Stay active. Having cancer doesn't mean you have to stop doing the things you enjoy or normally do. For the most part, if you feel well enough to do something, go ahead and do it. It's important to stay involved as much as you can. If your child has cancer, try to keep his or her life as normal as possible.

ASK AN EXPERT

Share this on:

Soft tissue sarcoma