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Soft Tissue Sarcoma

What is Soft Tissue Sarcoma?

Soft tissue sarcoma is a type of cancer that grows in the body’s soft connective tissue including muscle, nerves, tendons, fat, and walls of blood and lymph vessels. Soft tissue sarcomas can be divided into two main types:

  • Rhabdomyosarcoma
  • Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS)

Soft tissue sarcomas account for about 7% of all childhood cancers. Rhabdomyosarcoma is more common in younger children, and NRSTS is more common in adolescents. The clinical behavior of soft tissue sarcomas can range from locally aggressive to highly metastatic.

Soft tissue sarcomas are named for the tissue which they most resemble:

  • Skeletal muscle: rhabdomyosarcoma
  • Smooth muscle: leiomyosarcoma
  • Cartilage: chondrosarcoma
  • Synovium: synovial sarcoma
  • Fat: liposarcoma
  • Blood and lymph vessels: angiosarcoma 
  • Peripheral nerves: malignant peripheral nerve sheath tumor
  • Fibrous tissue: fibrosarcoma

Certain inherited conditions can increase risk for soft tissue sarcomas. Children who have been exposed to ionizing radiation are also at higher risk. Treatment for soft tissue sarcoma usually includes surgerychemotherapy, and/or radiation therapy.

Young rhabdomyosarcoma patient kneels, talking with a golfer at a tournament

"Two years off therapy, we are cancer free but still not at the 5-year mark. As time passes, I find that my worries change. The stress of cancer is still there. 'Scanxiety' hits as our 6-month checkup time gets closer. But what I look for now are more post-therapy effects, especially related to proton beam radiation because it just hasn’t been around long enough to know what to expect."

– Michelle, mom to Reid

Reid was treated for two rare cancers behind his eye: rhabdomyosarcoma and ectomesenchymoma. His treatments included surgery, chemotherapy, and proton beam radiation.

Life after Soft Tissue Sarcoma

Monitoring for Recurrence

Soft tissue sarcomas can recur years into survivorship. The tumor can come back in the same place (local recurrence) or in another part of the body (distant recurrence).

Patients will receive follow-up care to screen for recurrence after treatment ends. The medical team will make specific recommendations for the types of tests needed and how often patients should be seen for checkups.

Health after Cancer

Children treated for soft tissue sarcoma are at risk for late effects related to therapy. Specific problems depend on the location of the tumor and the type of therapy received.

Long-term effects of surgery can include loss of function and changes in appearance.

Survivors treated with radiation therapy may have impaired growth, bone disease, and other problems due to treatment as well as increased risk of second cancers.

Survivors treated with systemic chemotherapy should be monitored for medication-specific late-effects which may include heart problems, kidney damage, infertility, endocrine dysfunction, and risk of second cancers. 

All survivors should continue to have regular physical checkups and screenings by a primary physician. For general health and disease prevention, survivors should adopt healthy lifestyle and eating habits.

Reviewed: June 2018