Soft Tissue Sarcoma

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What is a soft tissue sarcoma?

A soft tissue sarcoma is a type of musculoskeletal cancer. They develop in soft tissues, which includes muscle, cartilage, connective tissue, fat, nerves, and blood and lymph vessels. There are also non-soft tissue sarcomas that develop in bone called osteosarcomas.

Soft tissue sarcomas are relatively rare in the United States. A little over 13,000 Americans are diagnosed with a soft tissue sarcoma each year. They can occur anywhere in the body, but are most common in the limbs (60%). About 30% develop in the torso or abdomen and 10% affect the head and neck.

There are more than 50 different types of soft tissue sarcomas. Some are more common in adults, while others tend to strike children more often. Soft tissue sarcomas in adults are very rare, accounting for about 1% of cancers. In contrast, soft tissue sarcomas account for about 15% of childhood cancers. Rhabdomyosarcoma is the most common soft tissue sarcoma in children. It typically affects children 10 years of age or younger.

Many soft tissue sarcomas have been linked to having certain inherited disorders, such as Li-Fraumeni syndrome or familial adenomatous polyposis (FAP). Other risk factors may include previous radiation treatment and certain chemical exposures.

Soft tissue sarcoma symptoms vary depending on the specific type of sarcoma and where it occurs. Often, there are no symptoms early in the disease. Some tumors are quite large by the time symptoms develop. In the limbs, people commonly notice a lump that grows over time. In the abdomen, digestive symptoms are often present.

Soft tissue sarcoma treatment depends on the stage and location of the cancer. In general, surgery is the most common treatment for most types and stages of soft tissue sarcomas. Doctors may also recommend radiation therapy, chemotherapy, and targeted therapy. Success in treating soft tissue sarcomas has increased tremendously over the past few decades.

See your doctor promptly if you notice lumps or bumps that persist or get bigger. Don’t ignore symptoms like pain or digestive problems that don’t go away. Diagnosing cancers early offers the best chance for successful treatment.

What are the symptoms of a soft tissue sarcoma?

Soft tissue sarcoma symptoms depend on the specific type and location of the cancer. These tumors don’t always cause symptoms in the early stages. Soft tissues can move and flex to accommodate the growing tumor. As a result, some tumors can be quite large before they are noticeable.

Most soft tissue sarcomas occur in the limbs where a lump is the most common symptom. The lump may or may not be painful, but tends to get larger with time.

Soft tissue sarcomas in the abdomen grow near organs and can cause problems with them. Symptoms can include abdominal pain that worsens, bloody stool or vomit, and black, tarry stools.

If you have symptoms that persist or worsen, it’s important to see your doctor promptly. In general, the earlier you find cancer, the better the prognosis.

What causes a soft tissue sarcoma?

Cancers, including soft tissue sarcomas, are the result of DNA mutations in genes that control cell growth. These changes allow cells to grow in an unrestrained manner. In some cases, these mutations are hereditary. In other cases, small changes accumulate over a person’s lifetime until the point the cells become very abnormal. The exact triggers for these changes are often unclear.

In childhood cancers, the genetic mutations most often happen randomly. Children don’t have decades of life to accumulate DNA damage. So, cancers are generally classified as inherited or spontaneous/sporadic.

What are the risk factors for a soft tissue sarcoma?

Researchers have linked certain factors to the risk of developing a soft tissue sarcoma. One of the main ones is having certain inherited disorders including:

  • Familial adenomatous polyposis (FAP or Gardner syndrome)
  • Li-Fraumeni syndrome
  • Neurofibromatosis type 1 (NF1 or Recklinghausen disease)
  • Retinoblastoma
  • Tuberous sclerosis
  • Werner syndrome

Other possible risk factors include previous radiation therapy, lymph system damage, immune system problems, and exposure to certain chemicals, including vinyl chloride, arsenic and dioxin.

Reducing your risk of soft tissue sarcoma

Cancer prevention and risk reduction strategies focus on changing risk factors you can control. Unfortunately, it isn’t possible to modify the main risk factors for soft tissue sarcoma. If you have a family history of soft tissue sarcoma or the inherited disorders that can lead to it, talk with your doctor. Find out about your risks and see if genetic testing is an option for you.

How is a soft tissue sarcoma treated?

Soft tissue sarcoma treatment options depend on the specific cancer, its stage, and its location. In early-stage cancer, the goal is often eliminating the cancer and preventing it from returning. In later stages, this may not be possible. Instead, the goal may be to control symptoms, improve quality of life, and extend life expectancy.

Surgery is the main treatment for most types and stages of soft tissue sarcoma. In the limbs, doctors remove the tumor and some surrounding healthy tissue. The goal is to spare as much of the arm or leg as possible. This helps preserve normal or near-normal function and appearance. However, in some cases, it isn’t possible to avoid amputation. Doctors may recommend chemotherapy, radiation therapy, or both before surgery. These therapies can be useful after surgery as well.

There are several targeted therapies available for soft tissue sarcoma. In general, doctors use these medicines for late-stage and recurrent sarcomas.

When you are considering treatment options, it’s important to find a doctor with plenty of experience treating your specific type of sarcoma. Ask about the goals, benefits and risks of the recommended treatment options. When the sarcoma affects a child, there are many special considerations when choosing a treatment. This includes late effects of cancer treatment. A team of healthcare providers will help you plan the right course of action for your child and your family.

What are the potential complications of a soft tissue sarcoma?

For many people, their soft tissue sarcoma prognosis is good. Most (60%) of sarcomas are localized at diagnosis. This means they haven’t spread. The 5-year relative survival rate for these localized tumors is 81%. However, complications are possible. Children and young people may have to deal with late effects of cancer treatment including:

  • Growth and development problems, which may include sexual development and fertility problems
  • Heart, lung or hearing problems after chemotherapy or radiation therapy
  • Learning problems

Your doctor will discuss guidelines for follow-up testing to monitor for these and other late effects. Discuss these potential problems with your team and ask about plans for monitoring and treating late effects.

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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2020 Jul 14
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