About Sarcoma

The world knows Terry Fox. But few know that the disease which claimed Fox’s life was osteosarcoma, a form of sarcoma cancer. Since Fox's heroic battle with cancer in 1981, much progress has been made in treating and curing this insidious disease.

What is Sarcoma?

A sarcoma is a malignant tumor that develops from particular tissues, such as muscle or bone. There are two types of sarcoma: osteosarcoma and soft tissue sarcoma.

Soft tissue sarcoma affects nerves, muscles, joints, fat and blood vessels - the body’s “connective tissues.” There are more than 50 kinds of soft tissue sarcomas, but more than half start in the arms or legs.

Due to the fact that the tissues affected can be found anywhere on the body, soft tissue sarcoma does not target one particular area of the body. Depending on the type of tissue from where it originated, the sarcoma has a different name. For instance, leiomyosarcoma develops in smooth muscle, osteosarcoma originates in the bone, and liposarcoma originates in fat cells.

Bone cancers can be divided into the following three groups:

Osteosarcoma - often seen in children and young adults (ages 10-25). Treatment usually involves chemotherapy and surgery. Chemotherapy is given in the sarcoma unit on an inpatient basis. Surgery usually involves a limb salvage/sparing procedure. Occasionally amputation is required but this is very rare nowadays, approximately 5% of the time. 

Chondrosarcoma - most frequently seen in adults over the age of 50. This type of tumor arises in the cartilage. Treatment usually involves surgical removal. 

Ewings Sarcoma - most often found in the middle part of the bones such as the pelvis and lower extremities. Seen primarily in children and young adults (age 10-25). Treatment usually involves chemotherapy and surgery. Occasionally radiation may be included in the treatment.

Frequently Asked Questions

1. Where does sarcoma commonly form?2. What are the different types of sarcoma?3. Who gets sarcoma?4. What are the common symptoms of sarcoma?5. Are there any risk factors associated with sarcoma?6. How will my doctor diagnose sarcoma?7. What should I ask my doctor when diagnosed with sarcoma?8. Where is the best place to get treated?9. What determines whether I have “good” or “bad” sarcoma?10. What kinds of treatments are available?

1. Where does sarcoma commonly form?

About 43 percent of sarcoma cancers occur in the extremities (e.g., arms, legs); 34 percent occur in and around the internal organs (e.g., uterus, heart); 10 percent occur in the trunk (e.g., chest, back); 13 percent occur in other locations. In very rare cases, these tumors develop in the gastrointestinal tract.

2. What are the different types of sarcoma?


Smooth muscle, deep soft tissue – Lungs, brain, lower & upper extremities


Blood or lymph vessels – Skin, breast, liver, spleen, and deep tissue


Bones and joints – Arms, legs, pelvis

Clear Cell Sarcoma

Fibrous tissue (tendons and ligaments) in the limbs – Feet, hands, and gastrointestinal tract


Cells that wrap and grow into fat, muscle, and bone – Trunk

*Desmoid Fibromatosis

No obvious related normal tissue – Arms, legs, trunk

Desmoplastic Small Round Cell Tumor (DSRCT)

Localized round tumor of cells – Abdomen


Deep soft tissue – Hands, forearms

Ewing Family of sarcomas
(peripheral primitive neuroectodermal tumor, PNET)

No obvious related normal tissue; may be a tumor of stem cells – Pelvis & large, long bones; thigh, knee, shoulder


Smooth muscle, deep soft tissue – Arms, legs

Gastrointestinal stromal tumor (GIST)

Smooth muscle, deep soft tissue – Gastrointestinal tract

Kaposi sarcoma

Smooth muscle, deep soft tissue – Skin, mouth


Smooth muscle, deep soft tissue – Uterus, stomach, small intestine


Smooth muscle, deep soft tissue – Arms, legs, trunk

Myxofibrosarcoma (myxoid malignant fibrous histiocytoma, MFH)

Smooth muscle, deep soft tissue – Arms, legs

Mesenchymal Chondrosarcoma

Smooth muscle, deep soft tissue – Lungs, brain, lower & upper extremities


Smooth muscle, deep soft tissue – Thigh, knee, shoulder

Pleomorphic sarcoma, not otherwise specified
(NOS; often referred to in the past as malignant fibrous histiocytoma, MFH)

Smooth muscle, deep soft tissue – Arms, legs, trunk, neck


Smooth muscle, deep soft tissue – Arms, legs

Synovial sarcoma

Smooth muscle, deep soft tissue – Legs

*Desmoid tumors are not considered sarcomas; however, they have been classified as aggressive fibromatosis, and the optimal treatment plan usually requires multidisciplinary teams only found in sarcoma centers.

The list above is not complete, but several of the most common types are listed. Experts have found many types and subtypes of sarcomas. Pathologists (doctors who specialize in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease) are now trying to find new ways to quickly determine a tumor's subtype, as this helps determine treatment. Looking at a tumor's abnormal genetics may help determine its characteristics and predict which treatments will be most effective.

Source: http://www.cancer.net/cancer-types/sarcoma

3. Who gets sarcoma?

Sarcomas are rare and account for less than 1% of all new cancer cases.  Sarcomas affect people of all ages, although they are most often observed in children and young adults.  In addition, certain types of sarcomas are most common for specific age groups. For example, soft-tissue sarcomas account for about 7% of all cancers in children, and liposarcomas are most often found in adults between 30 and 60 years old.

4. What are the common symptoms of Sarcoma?

In their early stages, soft tissue and bone sarcomas rarely display any symptoms. Because soft tissue is very elastic, tumors can grow quite large before they are felt. Common symptoms include:

Lump or mass: The first symptom is usually a lump or mass. The lump will form in the area in which the tumor is growing, and there may be some pain if it is pressing on a nerve or muscle. Even if the lump isn’t painful, if it continues to grow in size, or if it is located deep within an extremity or body cavity, consult your doctor.

Uncomfortable swelling: This is another sign of a sarcoma, especially when it is located in the arms and legs. Some tumors can restrict motion, such as those found in the hip, knee, shoulder or hands. Some sarcomas, such as osteosarcoma, may first present itself as joint pain that gets noticeably worse.

Skin lesions: can occur when a sarcoma tumor breaks through the skin.

Due to the nature of sarcoma and its ability to form almost anywhere in the body, other symptoms may be a sign, and can therefore affect a variety of organs. For example, sarcomas in the abdomen may cause abdominal pain, vomiting or constipation, while sarcomas in the uterus may cause vaginal bleeding and/or abdominal pain. With gastrointestinal stromal tumors (GISTs), you may feel full after eating only very small meals, or you may vomit blood or have dark bowel movements.

5. Are there any risk factors associated with sarcoma?

Most sarcomas develop in people with no known risk factors, however identified risk factors include genetic conditions such as absence of the retinoblastoma. Other risk factors include chromosomal alterations and, rarely, radiation exposure, especially in childhood. According to the American Cancer Society, radiation exposure accounts for less than 5% of sarcomas. Exposure to vinyl chloride and arsenic are linked to development of liver sarcoma.

There are currently no screening tests for sarcoma, so it is important to know if you have a family history of sarcoma or any of the conditions listed above.  If so, you may want to ask your healthcare provider about genetic testing.

6. How will my doctor diagnose sarcoma?

If you have symptoms or other reasons that suggest you may have a sarcoma, your doctor will use one or more methods to find out if the disease is present, including reviewing your complete medical history to check for risk factors, and to ask you about symptoms, as well as any other medical conditions. They will also examine you for signs of sarcomas and other health problems.

A biopsy is a sample of tissue removed from a tumor and tested in order to determine if cancer is present. A physical exam may suggest that a tumor is a sarcoma, but a biopsy is the only way to be certain that it is a sarcoma and not another type of cancer or a benign disease. Several types of biopsies are used to diagnose sarcomas. Doctors with experience in these tumors will choose one, based on the size and location of the tumor. Most experts prefer a fine needle aspiration or a core needle biopsy as the first step.

Chest x-ray
This test may be done to determine if the sarcoma has spread to your lungs.

CT Scans and MRIs
The computed tomography (CT) scan is an x-ray procedure that produces detailed, cross-sectional images of your body. Instead of taking one picture like a conventional x-ray, a CT scanner takes many pictures as it rotates around you. A computer then combines these pictures into an image of a slice of your body. The machine will create multiple images of the part of your body that is being studied. A CT scan is often done if the doctor suspects a soft tissue sarcoma in the chest, abdomen, or the retroperitoneum. This test is also used to see if the sarcoma has spread into the liver or other organs.

MRI scans are often part of the work-up of any tumor that could be a sarcoma. They are often better than CT scans in evaluating sarcomas in the arms or legs. They provide a good picture of the extent of the tumor. They can tell your health care team many things about the tumor, including location, size, and sometimes even the type of tissue it comes from (like fat or muscle). This makes MRI scans useful in planning a biopsy.

Ultrasound uses sound waves and their echoes to produce pictures of parts of the body. A small instrument called a transducer emits sound waves and picks up the echoes as they bounce off the organs. The sound wave echoes are converted by a computer into an image that is displayed on a computer screen. This test may be done before a biopsy to see if the lump is a cyst, meaning it contains fluid and is likely benign, or if it is solid and more likely a tumor. This test is often not needed if an MRI was done.

7. What should I ask my doctor when diagnosed with soft tissue sarcoma?

Here are some questions you may want to ask your doctor: 

- How can this disease be treated?
- What are my treatment choices?
- How successful is the treatment likely to be?
- Would a clinical trial be appropriate for me?
- What are the expected benefits of treatment?
- What are the risks and possible side effects of treatment?
- Will I have to change my normal activities?
- Can I keep working during treatment?
- How often will I need checkups?

Your doctor is the best person to give advice about your ability to work or to answer questions about other activities. Many patients find it also helps to talk with others who are facing similar problems. This kind of help is available through cancer-related support groups. Social services, clergy, nursing personnel or your doctor can offer suggestions as to where to seek assistance with emotional support.

8. Where is the best place to get treated?

Because sarcomas are rare, it is important to find physicians and multidisciplinary treatment centers that have experience with this disease.

9. What determines whether I have a “good” or “bad” sarcoma?

You will hear a lot about the risk factors for survival from sarcoma. These are mainly:

- The location of the tumor.
- The size of the tumor.
- Whether it has spread to other parts of the body.
- The special characteristics of the tumor that can only be determined after biopsy.
- The “grade,” which is an important term you will hear about during the pathological analysis.

Source: http://www.mskcc.org/cancer-care/adult/soft-tissue-sarcoma/faqs