While soft tissue sarcomas can occasionally form in bones, they usually develop in fat, muscle, nerves, tendons, joint linings, or blood or lymph vessels—the tissues that connect, support, and surround other body parts and organs.

These rare tumors grow almost anywhere in the body, but in adults are usually found in the head, neck, arms, legs, trunk, or abdomen. The most common occurrences in children are found in the arms, legs, chest or abdomen.

Types

More than 50 types of soft tissue sarcomas have been identified. These include:

  • Alveolar Soft Part Sarcoma are rare, slow-growing tumors mainly affect teens and often grow deep in the thigh, though they can show up in the heads and necks of younger children.
  • Clear Cell Sarcoma mainly affects younger adults ages 20 to 40, growing just beneath the skin and affecting tendons and muscle sheaths—usually in the foot and ankle.
  • Epithelioid Sarcoma mainly develop in the forearm or hand, most frequently in male patients in their teens or 20s.
  • Fibrosarcoma develops from the tissues keeping bones, organs and muscles secure. One type of the disease mainly affects middle-age adults, usually in the legs, arms or trunk. The other type affects children—most frequently at birth and almost never past age 2. Outcomes are better for children, with a lower chance the cancer will spread or return.
  • Gastrointestinal Stromal Tumor (GIST) is a rare tumor that tends to affect adults. It develops in the abdomen, where it can grow quite large before it’s noticed and is harder to remove.
  • Leiomyosarcoma develops from blood vessels in the trunk and limbs and mainly affects adults starting in middle age.
  • Liposarcoma develops from fatty tissues and usually appears in the arms, legs, abdomen, or buttocks—most frequently in men and women over 50.
  • Rhabdomyosarcoma are made of cells that normally develop into the skeletal muscles we use to control our body and are found mainly in the head and neck, urinary and reproductive organs, arms and legs, and trunk. While the tumors can affect all ages, they’re mainly found in children under 5 and are the most common childhood soft tissue sarcoma. They can grow quite large in a short period of time.
  • Synovial Sarcoma develop deep in the muscles of the arms and legs and mainly affect young adults, particularly men in their 30s.

Risk Factors

Soft tissue sarcoma is not a common type of cancer - it accounts for about 10,000 cases per year. While we don’t know what causes most soft tissue sarcomas, several risk factors exist:

  • Inherited disorders
    • Retinoblastoma
    • Neurofibromatosis (von Recklinghausen disease)
    • Tuberous sclerosis (Bourneville disease)
    • Gardner syndrome
    • Li-Fraumeni syndrome
    • Werner syndrome
    • Gorlin syndrome (Nevoid basal cell carcinoma syndrome)
  • Past radiation therapy for certain cancers
  • Chronic swelling in the arms or legs (tied to some lymph vessel cancers in adults)
  • Extensive exposure to certain chemicals including Thorotrast, vinyl chloride or arsenic

Soft Tissue Sarcoma Symptoms

Soft tissue sarcomas may cause a painless lump under the skin (usually on the arms, legs or trunk), but often no other symptoms exist at first. As the sarcomas grow and press on organs, nerves, muscles, or blood vessels, they can cause:

  • Pain
  • Breathing trouble
  • Abdominal swellings (or distension)

These symptoms can indicate a variety of different problems. Make sure to discuss with your doctor if you experience any of them, especially if you also have risk factors for soft tissue sarcoma. 

Diagnosis and Treatment

Successful soft tissue sarcoma treatment depends on a complete and accurate diagnosis. Each of our patients receives a thorough evaluation so we can create an individualized plan. We diagnose bone sarcomas with:

  • Medical history: We will discuss your symptoms and medical history, including whether you suffer from any of the soft tissue sarcoma risk factors. 
  • Physical examination: We will examine you thoroughly, including any lumps you have. 

A variety of tests can help determine whether you have soft tissue sarcoma. These tests include: 

  • Imaging Scans: A variety of imaging scans can help identify the presence, type and size of a soft tissue sarcoma, including: 
    • X-ray
    • CT (CAT) Scan: This imaging test uses x-ray technology and computers to create a detailed picture of your affected tissues. 
    • MRI: This imaging test uses high powered magnets to create a picture of the affected area. 
    • PET Scan: For this imaging test, your doctor injects you with radioactive glucose. The PET scan follows the glucose's path, which identifies the presence of cancerous cells. 
    • Bone Scan
  • Blood work
  • Biopsy: Your surgical oncologist will determine whether you need this procedure. If you do, your radiologist will remove tissue from your affected area for closer examination under a microscope. This will help determine whether you have cancerous cells and how quickly the cells are spreading. 

Soft Tissue Sarcoma Treatment

Treatment for soft tissue sarcoma depends on what type of disease you have, whether it has spread to other parts of your body, and where the tumor is located. Treatment options include: 

  • Surgery: This treatment removes as much of the tumor as possible, as well as some surrounding healthy tissue. This is called complete margin-negative resection. 
  • Chemotherapy: This treatment can destroy some, but not all types of soft tissue sarcomas. It uses powerful drugs delivered either orally or through your veins (IV).  
  • Radiation: This treatment uses high energy radiation beams to destroy the tumor and cancerous cells. Your sarcoma team will discuss with you whether this option is appropriate for your disease. If it is, make sure you understand the pros and cons of receiving radiation before or after surgery. 

We give each patient a thorough evaluation to determine the best recommendation. Children and teens may respond to treatment differently than adults and often have a better prognosis. We’re also working to safely lower the amount of radiation and chemotherapy younger patients receive while still providing successful cancer treatment.

Soft Tissue Sarcoma Follow Up Care

Following treatment, you will meet with our surgical oncologists on a regular basis to monitor your progress. This may include repeating some of the diagnostic tests and imaging scans. Soft tissue sarcoma can recur, or reappear, even after treatment. We will work with you to make sure we catch any recurrence as early as possible. Factors that can affect recurrences include: 

  • Sarcoma size: a large sarcoma size has a higher chance of recurring. 
  • Sarcoma grade: low grade sarcomas are less likely to recur. 
  • Location of sarcoma: Sarcomas that affect the inside of the abdomen and the organs around it are more likely to recur that those that affect the limbs. 
  • How much of the sarcoma was surgically removed: If your surgical oncologist was unable to completely remove the sarcoma, you may be more likely to have a recurrence. Sometimes the location of the sarcoma does not allow for a complete removal. In those cases, we will follow up surgery with radiotherapy. 
  • Previous Recurrence: A sarcoma that has already recurred is more likely to recur again. 
  • Type of sarcoma histology: The specific details of the tissue that make up your sarcoma. 
  • Your age: If you are older, it is more likely for the sarcoma to recur. 

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For more information or to schedule an appointment with a cancer care specialist, call 855-546-1944.

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