Soft Tissue Sarcomas

Soft tissue sarcomas are malignant tumors that begin in soft tissue, such as muscle, fibrous tissues, tendons, fat, nerves, joint tissues and blood vessels.

These tumors are rare and can form anywhere in the body. Often painless, soft tissue sarcomas can be present in the body for a long period of time before they are discovered.

There are several types of soft tissue sarcomas that involve joints, bones and nerves. They include:

A few soft tissue sarcomas do not involve bones or joints. These include:

• Leiomyosarcoma — usually found in the stomach, bowels, uterus and blood vessels
• Liposarcoma — found in the fat tissue of the stomach.

Studies have identified a connection between soft tissue sarcomas and other cancers. Certain conditions, such as inherited diseases like Li-Fraumeni syndrome or neurofibromatosis, as well as the infectious virus Epstein-Barr, have been linked to an increased risk of developing soft tissue sarcomas.

Cause of soft tissue sarcomas

The cause of soft tissue sarcomas is unknown, but there may be a genetic reason for their formation.

Symptoms of soft tissue sarcomas

Symptoms of soft tissue sarcomas include:

• Painless swelling or a lump
• Pain or soreness due to the tumor compressing nerves or muscles
• A limp, or difficulties moving legs or arms 

Diagnosing soft tissue sarcomas

Symptoms of soft tissue sarcomas can be difficult to identify in children, so regular check-ups are extremely important. Prompt diagnosis and identification of cancer as early as possible is crucial to overall treatment.

Diagnostic evaluation begins with a thorough medical history and physical examination of your child. A detailed neurological assessment may also be performed.

• X-rays, which produce images of bones.
• Magnetic resonance imaging (MRI), which uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body.
• Computed tomography (CT) scan, which uses a combination of X-rays and computer technology to produce cross-sectional images ("slices") of the body.
• Radioisotope bone scan, which can help locate areas of abnormal growth.
• Blood tests, which can help determine drug usage and effectiveness, biochemical diseases and organ function.
• Biopsy, which is a surgical procedure to take a sample of the growth, may be necessary to confirm any findings.
• EOS imaging, a newly FDA-approved imaging technology which creates 3-dimensional models from two planar images. Unlike a CT scan, EOS images are taken while upright or standing position, enabling improved diagnosis due to weight-bearing positioning.

Treatment for soft tissue sarcomas

Treatment for soft tissue sarcomas varies, depending on the location and size of the tumor. 

Surgery

Surgical treatment for soft tissue tumors may be used alone or in conjunction with chemotherapy or radiation therapy. 

Surgeries performed are typically:

• Wide resection with margins
• Resection and reconstruction

In a wide resection with margins, the tumor is surgically removed, as well as some of the healthy tissue that surrounded the tumor — the margins of the tumor. In some cases, the child will need reconstructive surgery after the tumor has been cut out.