Adult soft tissue sarcoma is a disease in which cancer (malignant) cells are found in the soft tissue of part of the body. The soft tissues of the body include the muscles, connective tissues (tendons), vessels that carry blood or lymph, joints, and fat.

A lump or swelling in part of the body may appear if a person has a soft tissue sarcoma. The lump may not be painful. If there are symptoms, a doctor may cut out a piece of tissue from the swollen area. This is called a biopsy. The tissue will be looked at under a microscope to see if there are any cancer cells. A patient may need to go to the hospital for this test.

The chance of recovery (prognosis) and choice of treatment depend on the size and stage of the cancer (how far the cancer has spread), and the patient’s age and general health.

Stages of adult soft tissue sarcoma
Once adult soft tissue sarcoma is found, more tests will be done to find out if cancer cells have spread to other parts of the body. This testing is called staging. A doctor needs to know the stage of the disease to plan treatment. Unlike most other cancers, the size of a soft tissue sarcoma is not as important as how the cancer cells look under a microscope. The more different the cancer cells look from normal cells, the higher the stage. The following stages are used for adult soft tissue sarcoma:

Stage IA
The cancer cells look either very much like or somewhat different from normal cells (well-differentiated or moderately well-differentiated). The cancer is either near the surface or deep and is less than 5 centimetres in size (about 2 inches), but it has not spread to lymph nodes or other parts of the body (lymph nodes are small bean-shaped structures that are found throughout the body; they produce and store infection-fighting cells).

Stage IB
The cancer cells look either very much like or somewhat different from normal cells (well-differentiated or moderately well-differentiated). The cancer is near the surface and more than 5 centimetres in size, but it has not spread to lymph nodes or other parts of the body.

Stage IIA
The cancer cells look either very much like or somewhat different from normal cells (well-differentiated or moderately well-differentiated). The cancer is deep and more than 5 centimetres in size, but it has not spread to lymph nodes or other parts of the body.

Stage IIB
The cancer cells look very different from normal cells (poorly differentiated or undifferentiated). The cancer is either near the surface or deep and is less than 5 centimetres in size, but it has not spread to lymph nodes or other parts of the body.

Stage IIC
The cancer cells look very different from normal cells (poorly differentiated or undifferentiated). The cancer is near the surface and is more than 5 centimetres in size, but it has not spread to lymph nodes or other parts of the body.

Stage III
The cancer cells look very different from normal cells (poorly differentiated or undifferentiated). The cancer is deep and is more than 5 centimetres in size, but it has not spread to lymph nodes or other parts of the body.

Stage IV
The cancer may have spread to lymph nodes in the area or may have spread to other parts of the body, such as the lungs, head, or neck.

Recurrent
Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the tissues where it first started, or it may come back in another part of the body.

How adult soft tissue sarcoma is treated
There are treatments for all patients with adult soft tissue sarcoma. Three kinds of treatment are used:

• Surgery (taking out the cancer in an operation).
• Radiation therapy (using high-dose x-rays to kill cancer cells).
• Chemotherapy (using drugs to kill cancer cells).

Surgery is the most common treatment of adult soft tissue sarcoma. A doctor may remove the cancer and some of the healthy tissue around the cancer. Sometimes all or part of an arm or leg may have to be removed (amputated) to make sure that all of the cancer is taken out. If cancer has spread to lymph nodes, the lymph nodes will be removed (lymph node dissection).

Radiation therapy uses x-rays or other high-energy rays to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy) or from putting materials that produce radiation (radioisotopes) through thin plastic tubes in the area where the cancer cells are found (internal radiation therapy).

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in a vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the blood stream, travels through the body, and kills cancer cells throughout the body. Chemotherapy that is given after surgery when no cancer cells can be seen is called adjuvant chemotherapy. In soft tissue sarcoma, chemotherapy is sometimes injected directly into the blood vessels in the area where the cancer is found. This treatment is called regional chemotherapy.

Chemotherapy and/or radiation therapy may be used to shrink the cancer so it can be removed without taking off an entire arm or leg.

Stage IA, IB, and IIA Adult Soft Tissue Sarcoma
Treatment may be one of the following:

1. Surgery to remove the cancer.
2. Surgery with radiation therapy, before or after the surgery.
3. High-dose radiation therapy followed by surgery and radiation therapy.

If cancer is found in the head or neck or in the abdomen or chest, treatment may be one of the following:

1. Surgery to remove the cancer possibly followed by radiation therapy.
2. Radiation therapy followed by surgery.
3. Radiation therapy.

Stage IIB, IIC, and III Adult Soft Tissue Sarcoma
Treatment may be one of the following:

1. Surgery to remove the cancer.
2. Surgery to remove the cancer followed by radiation therapy.
3. Radiation therapy alone.
4. Radiation therapy and/or chemotherapy before surgery, possibly followed by radiation therapy.

Stage IV Adult Soft Tissue Sarcoma
If the cancer has spread to the lymph nodes, treatment may be one of the following:

1. Surgery to remove the cancer and removal of the lymph nodes where the cancer has spread (lymph node dissection), possibly followed by radiation therapy.
2. Radiation therapy before and after surgery to remove the cancer and lymph node dissection.
3. A clinical trial of surgery and/or radiation therapy followed by chemotherapy.

If the cancer has spread to the lungs, treatment may be one of the following:

1. Surgery to remove the primary cancer followed by radiation therapy followed by surgery to remove the cancer from the lungs.
2. Surgery to remove the primary cancer.
3. Surgery to remove the primary cancer followed by radiation therapy.
4. Radiation therapy, possibly followed by chemotherapy.

If the cancer has spread to other parts of the body, treatment may be one of the following:

1. Surgery to remove the cancer with radiation therapy before or after the surgery, possibly followed by chemotherapy.
2. Chemotherapy to reduce the pain and discomfort caused by the cancer.

Recurrent Adult Soft Tissue Sarcoma
Treatment depends on the kind of treatment the patient had before. Treatment may be one of the following:

1. Surgery to remove the cancer.
2. Surgery to remove the cancer followed by radiation therapy.
3. Chemotherapy alone.

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The Ewing’s family of tumours include: Ewing’s tumour of bone; extraosseus Ewing’s (tumour growing outside of the bone); primitive neuroectodermal tumour (PNET), also known as peripheral neuroepithelioma; and Askin’s tumor (PNET of the chest wall). These tumours are rare diseases in which cancer (malignant) cells are found in the bone and soft tissues. Ewing’s family of tumours most frequently occurs in teenagers.

If a patient has symptoms (such as pain, stiffness, or tenderness in the bone) the doctor may order x-rays and other tests. The doctor may also cut out a piece of tissue from the affected area. This is called a biopsy. The tissue will be looked at under a microscope to see if there are any cancer cells. This test may be done in the hospital.

The chance of recovery (prognosis) and choice of treatment depend on the location, size, and stage of the cancer (how far the cancer has spread), how the cancer cells react to the treatment, and the patient’s age and general health.

Stages of the Ewing’s family of tumours
Once one of the Ewing’s family of tumours has been found, more tests will be done to find out if cancer cells have spread to other parts of the body. This is called staging. At present, there is no formal staging system for the Ewing’s family of tumours. Instead, most patients are grouped depending on whether cancer is found in only one part of the body (localized disease) or whether cancer has spread from one part of the body to another (metastatic disease). Extraosseous Ewing’s has been grouped using the rhabdomyosarcoma staging system because they are both soft tissue tumours. Your doctor needs to know where the cancer is located and how far the disease has spread to plan treatment. The following groups are used for the Ewing’s family of tumours.

Localized
The cancer cells have not been shown to have spread beyond the bone in which the cancer began or are found only in the bone and nearby tissues.

Metastatic
The cancer cells have spread from the bone in which the cancer began to other parts of the body. The cancer most often spreads to the lung, other bones, and bone marrow (the spongy tissue inside of the large bones of your body that makes red blood cells). Spread of cancer to the lymph nodes (small bean-shaped structures found throughout your body which produce and store infection-fighting cells) or the central nervous system (brain and spinal cord) is less common.

Recurrent
Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the tissues where it first started or it may come back in another part of the body.

Localized Tumours of the Ewing’s Family
Treatment for localized tumours of the Ewing’s family may be one of the following:

1. A clinical trial of chemotherapy followed by radiation therapy
2. Combination chemotherapy followed by surgery with or without radiation therapy
3. A clinical trial of intensified chemotherapy
4. A randomized trial of post-surgical chemotherapy with or without stem cell transplant

Metastatic Tumours of the Ewing’s Family
Treatment for metastatic tumours of the Ewing’s family may be one of the following:

1. Combination chemotherapy followed by radiation therapy and/or surgery
2. High-dose chemotherapy with or without radiation therapy plus additional stem cell support
3. A clinical trial of intensive chemotherapy with multiple chemotherapy drug combinations

Recurrent Tumours of the Ewing’s Family
Treatment depends on where the cancer recurred, how the cancer was treated before, as well as individual patient factors. Chemotherapy may be used for patients who did not receive previous chemotherapy. Radiation treatment may be given to reduce symptoms. Surgery may be used to remove tumours that have spread to the lungs or other organs. Clinical trials are testing new treatments.

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Kaposi’s sarcoma (KS) is a disease in which malignant cells are found in the tissues under the skin or mucous membranes that line the mouth, nose, and anus. KS causes red or purple patches (lesions) on the skin and/or mucous membranes and spreads to other organs in the body, such as the lungs, liver, or intestinal tract.

Until the early 1980’s, Kaposi’s sarcoma was a very rare disease that was found mainly in older men, patients who had organ transplants, or African men. With the Acquired Immunodeficiency Syndrome (AIDS) epidemic in the early 1980’s, doctors began to notice more cases of Kaposi’s sarcoma in Africa and in gay men with AIDS. Kaposi’s sarcoma usually spreads more quickly in these patients.

If there are signs of KS, a doctor will examine the skin and lymph nodes carefully (lymph nodes are small bean-shaped structures that are found throughout the body; they produce and store infection-fighting cells). The doctor also may order other tests to see if the patient has other diseases.

The chance of recovery (prognosis) depends on what type of Kaposi’s sarcoma the patient has, the patient’s age and general health, and whether or not the patient has AIDS.

Stages of Kaposi’s sarcoma
There is no accepted staging system for Kaposi’s sarcoma. Patients are grouped depending on which type of Kaposi’s sarcoma they have. There are three types of Kaposi’s sarcoma:

Classic
Classic Kaposi’s sarcoma usually occurs in older men of Jewish, Italian, or Mediterranean heritage. This type of Kaposi’s sarcoma progresses slowly, sometimes over 10 to 15 years. As the disease gets worse, the lower legs may swell and the blood may not be able to flow properly. After some time, the disease may spread to other organs. Many patients with classic Kaposi’s sarcoma may develop another type of cancer later on in their lives.

Immunosuppressive treatment related
Kaposi’s sarcoma may occur in people who are taking drugs to make their immune systems weaker (immuno-suppressants). The immune system helps the body fight off infection. People who have had an organ transplant (such as a liver or kidney transplant) have to take drugs to prevent their immune system from attacking the new organ.

Epidemic
Kaposi’s sarcoma in patients who have Acquired Immunodeficiency Syndrome (AIDS) is called epidemic Kaposi’s sarcoma. AIDS is caused by a virus called the Human Immunodeficiency Virus (HIV), which attacks and weakens the immune system. Infections and other diseases can then invade the body, and the immune system cannot fight against them. Kaposi’s sarcoma in people with AIDS usually spreads more quickly than other kinds of Kaposi’s sarcoma and often is found in many parts of the body.

Recurrent
Recurrent disease means that the KS has come back (recurred) after it has been treated. It may come back in the area where it first started or in another part of the body.

How Kaposi’s sarcoma is treated
There are treatments for all patients with Kaposi’s sarcoma. Four kinds of treatment are used:

• Surgery (taking out the cancer).
• Chemotherapy (using drugs to kill cancer cells).
• Radiation therapy (using high-dose x-rays to kill cancer cells).
• Biological therapy (using the body’s immune system to fight cancer).

Radiation therapy is a common treatment of Kaposi’s sarcoma. Radiation therapy uses high-dose x-rays or other high-energy rays to kill cancer cells and shrink tumours. Radiation for Kaposi’s sarcoma comes from a machine outside the body (external beam radiation therapy).

Surgery means taking out the cancer. A doctor may remove the cancer using one of the following:

• Local excision cuts out the lesion and some of the tissue around it.
• Electro-desiccation and curettage burns the lesion and removes it with a sharp instrument.
• Cryotherapy freezes the tumour and kills it.

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in a vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells outside the original site. Chemotherapy for Kaposi’s sarcoma also may be injected into the lesion (intra-lesional chemotherapy).

Biological therapy tries to get the body to fight the cancer. It uses materials made by the body or made in a laboratory to boost, direct, or restore the body’s natural defences against disease. Biological therapy is sometimes called biological response modifier (BRM) therapy or immunotherapy.

Treatment by stage
Treatment of Kaposi’s sarcoma depends on the type of Kaposi’s sarcoma the patient has, and the patient’s age and general health.

Classic Kaposi’s Sarcoma
Treatment may be one of the following:

1. Radiation therapy.
2. Local excision.
3. Systemic or intra-lesional chemotherapy.
4. Chemotherapy plus radiation therapy.

Immunosuppressive Treatment Related Kaposi’s Sarcoma
Depending on the patient’s condition, the cancer may be controlled if immunosuppressive drugs are stopped. If the patient cannot stop taking these drugs or if this does not work, treatment may be one of the following:

1. Radiation therapy.
2. A clinical trial of chemotherapy.

Epidemic Kaposi’s Sarcoma
Treatment may be one of the following:

1. Surgery (local excision, electro-desiccation and curettage, or cryotherapy) with or without radiation therapy.
2. Systemic chemotherapy. Clinical trials are testing new drugs and drug combinations.
3. Biological therapy.
4. A clinical trial evaluating new treatments.

Recurrent Kaposi’s Sarcoma
Treatment of recurrent Kaposi’s sarcoma depends on the type of Kaposi’s sarcoma, and the patient’s general health and response to earlier treatments. The patient may want to take part in a clinical trial.

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Osteosarcoma is a disease in which malignant cells are found in the bone. It is the most common type of bone cancer. In children, it occurs most commonly in the bones around the knee. Osteosarcoma most often occurs in adolescents and young adults.

Malignant fibrous histiocytoma of bone (MFH) is a rare tumour of the bone. It may occur following radiation treatments. MFH is generally treated the same as osteosarcoma and appears to have a similar response to treatment.

If a patient has symptoms (such as pain and swelling of a bone or a bone region), a doctor may order x-rays and blood tests. If it is suspected that the problem is osteosarcoma, your doctor may recommend seeing a specialist called an orthopaedic oncologist. The orthopaedic oncologist may cut out a piece of tissue from the affected area. This is called a biopsy. The tissue will be looked at under a microscope to see if there are any cancer cells. This test may be done in the hospital.

The chance of recovery (prognosis) and choice of treatment depend on the size, location, type, and stage of the cancer (how far the cancer has spread), how long the patient had symptoms, how much of the cancer is taken out by surgery and/or killed by chemotherapy, and the patient’s age, blood and other test results, and general health.

Stages of osteosarcoma
Once osteosarcoma has been found, more tests may be done to find out if cancer cells have spread to other parts of the body. This is called staging. At present, there is no staging system for osteosarcoma. Instead, most patients are grouped depending on whether cancer is found in only one part of the body (localized disease) or whether the cancer has spread from one part of the body to another (metastatic disease). Your doctor needs to know where the cancer is located and how far the disease has spread to plan treatment. The following groups are used for osteosarcoma:

Localized osteosarcoma
The cancer cells have not spread beyond the bone or nearby tissue in which the cancer began. In young patients, most tumours occur around the knee.

Metastatic osteosarcoma
The cancer cells have spread from the bone in which the cancer began to other parts of the body. The cancer most often spreads to the lungs. It may also spread to other bones.

Recurrent
Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the tissues where it first started or it may come back in another part of the body.

How osteosarcoma is treated
If it is suspected that the problem is osteosarcoma, before the first biopsy, your doctor may recommend a specialist called an orthopaedic oncologist.

There are treatments for all patients with osteosarcoma. Three kinds of treatment are used:

• Surgery (taking out the cancer in an operation)
• Chemotherapy (using drugs to kill cancer cells)
• Radiation therapy (using high-dose x-rays to kill cancer cells)

Surgery is a common treatment for osteosarcoma. The doctor may remove the cancer and some of the healthy tissue around the cancer. Sometimes all or part of an arm or leg may have to be removed (amputated) to make sure that all of the cancer is taken out. If cancer has spread to lymph nodes, the lymph nodes will be removed (lymph node dissection).

In patients with osteosarcoma that has not spread beyond the bone, researchers are studying whether surgery without amputation of the arm or leg (limb-sparing procedures) can be done without the cancer coming back. Sometimes the cancer can be taken out without amputation, and artificial devices or bones from other places in the body can be used to replace the bone that was removed.

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill or put into the body by a needle in a vein or muscle. Chemotherapy is called systemic treatment because the drug enters the blood stream, travels through the body, and can kill cancer cells throughout the body. Chemotherapy with more than one drug is called combination chemotherapy.

Sometimes chemotherapy is injected directly into the area where the cancer is found (regional chemotherapy). In osteosarcoma, surgery is often used to remove the local tumour and chemotherapy is then given to kill any cancer cells that remain in the body. Chemotherapy given after surgery has removed the cancer is called adjuvant chemotherapy. Chemotherapy can also be given before surgery to shrink the cancer so that it can be removed during surgery; this is called neo-adjuvant chemotherapy.

Radiation therapy uses x-rays or other high-energy rays to kill cancer cells and shrink tumours. Radiation for osteosarcoma usually comes from a machine outside the body (external radiation therapy).

Treatment for osteosarcoma depends on the stage of the disease, where the cancer is found, and the patient’s age and general health

Localized Osteosarcoma/Malignant Fibrous Histiocytoma of Bone
Treatment of osteosarcoma may be the following:

• Chemotherapy followed by surgery followed by adjuvant chemotherapy
• Clinical trials are evaluating new methods of giving chemotherapy and new schedules of treatment. The use of radiation therapy is also under study

Treatment of malignant fibrous histiocytoma of bone may be the following:

• Neoadjuvant chemotherapy followed by wide local excision of the tumour

Metastatic/Unresectable Disease
Metastatic disease is cancer that has spread from the place in which it started to other parts of the body. Unresectable disease is cancer that cannot be removed with surgery.

Osteosarcoma
Treatment may be one of the following:

• Chemotherapy followed by surgery to remove the cancer followed by adjuvant chemotherapy
• Surgery to remove the cancer followed by adjuvant chemotherapy.

Surgery often includes removal of cancer that has spread to the lungs.

Malignant fibrous histiocytoma of bone
The standard treatments for patients with unresectable or metastatic malignant fibrous histiocytoma of bone have not yet been determined.

Recurrent Osteosarcoma
Treatment depends on where the cancer recurred, what kind of treatment was given before, as well as other factors. A clinical trial may be a reasonable treatment option.

If the cancer has come back only in the lungs, treatment may be surgery to remove the cancer in the lungs with or without chemotherapy. If the cancer has come back in other places besides the lungs, treatment may be combination chemotherapy. Clinical trials are evaluating new chemotherapy drugs.

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