Soft Tissue Sarcomas
Soft tissue sarcomas are cancerous. They grow in areas that support and connect parts of the body, like tendons and muscles.
What Are Soft Tissue Sarcomas?
Soft tissue sarcomas are cancerous (malignant) tumors that affect children and adults. They grow in soft tissues, which support and connect parts of the body. These areas can include tendons, muscle, fat, blood vessels, nerves and linings of the joints.
These tumors are rare in children, affecting less than 1,000 children in the United States annually. They occur most commonly in the arms, legs, chest and abdomen in children. Soft tissue sarcomas can metastasize (spread) throughout the body.
Causes of soft tissue sarcoma remain unknown, but certain factors increase a child’s risk of developing this tumor:
- AIDS
- Previous radiation therapy
- Epstein-Barr virus infection
- Inherited genetic conditions, such as Li-Fraumeni syndrome
Soft tissue sarcomas are classified as rhabdomyosarcomas or non-rhabdomyosarcomas. Rhabdomyosarcomas form in the muscle and are the most common pediatric soft tissue sarcoma.
There are over 50 types of non-rhabdomyosarcomas, which are named according to their location. For example, soft tissue sarcomas in the fat are called liposarcomas.
What Are the Signs and Symptoms of Soft Tissue Sarcomas?
Initially, soft tissue sarcomas are painless lumps or swellings. Other symptoms appear as the tumor grows and compresses nearby structures, such as nerves. These symptoms include pain or weakness at the tumor site and limping (if the tumor is in the leg or foot).
How Are Soft Tissue Sarcomas Diagnosed?
Your doctor will first take a detailed history and perform a physical examination which includes vascular, neurologic and musculoskeletal testing.
Diagnostic testing is used to assess overall health and closely examine the tumor. Bloodwork and urinalysis are basic laboratory tests for evaluating overall health and organ function.
Imaging tests are used to look at the tumor. Imaging tests include X-rays, ultrasound, magnetic resonance imaging (MRI) and computed tomography (CT).
A biopsy confirms that the tumor is cancerous. There are several biopsy techniques, but all involve analyzing a small tumor tissue sample with a microscope by a pathologist.
How Are Soft Tissue Sarcomas Treated?
Treatment for soft tissue sarcomas involves doctors from different specialties, such as Oncology, Radiology, Radiation Oncology and Orthopedic Surgery. Developing a treatment plan depends on the tumor’s size, location and growth rate.
The three primary treatment options for soft tissue sarcomas are surgery, radiation therapy and chemotherapy. A combination of these treatments may be needed to get rid of the tumor.
- Surgery is the standard treatment for these tumors. Various surgical techniques are available. Your child’s care team will determine the best surgical approach for your child.
- Chemotherapy. Chemotherapy uses drugs to kill cancer cells and is used before and after surgery. Most of the time, chemotherapy is the first treatment for osteosarcoma and is done before and after surgery. The type, amount and how often the chemotherapy is done is decided by the oncologist (cancer doctor). In addition to killing the cancer cells, chemotherapy drugs sometimes also affect healthy cells (such as the cells that make hair and line the stomach and intestines) causing side effects. The goal of chemotherapy is to reduce the spread of the tumor.
- Radiation therapy is sometimes used to shrink the tumor before surgery or destroy any remaining tumor cells after surgery. Options include high-energy X-rays, injection of a radioactive substance into the tumor, or proton therapy. Proton therapy uses protons (positively charged particles) instead of X-rays.
Late side effects, like mood changes and the development of other cancers, can occur with cancer treatment. These side effects can last long after cancer treatment has started.
Your child will need follow-up testing to assess their health and determine whether the sarcoma has returned. Recurring soft tissue sarcomas will require additional treatment.
Survival rates for soft tissue sarcomas in children have improved significantly over the past several decades.
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