Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS)

This group of tumors, called non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), include clear cell sarcoma, dermatofibrosarcoma, epithelioid sarcoma, fibrosarcoma, hemangiopericytoma, leiomyosarcoma, liposarcoma, malignant fibrous histiocytoma, malignant peripheral nerve sheath tumor, non-synovial cell sarcoma and undifferentiated sarcoma.

What is NRSTS?

NRSTS tumors develop in the soft tissues of the body:

• The most common sites are the arms and legs, but the tumors can occur anywhere. Other sites include the head and neck region, chest, abdomen and pelvis.
• Some types of NRSTS cancers are more likely to develop in children, including:
  • Fibrohistiocytic tumors (tumors in the body’s connective tissues including dermatofibrosarcoma)
  • Synovial sarcoma (common in the legs, especially the knees)
  • Malignant peripheral nerve sheath tumors, affecting nerve sheaths (coverings)
• Some NRSTS tumors may be inherited (passed down in families).
  • Li-Fraumeni syndrome, retinoblastoma and type 1 neurofibromatosis (NF-1) are genetic disorders that increase the possibility of developing a soft tissue sarcoma.
  • Children and teens with NF-1 are more likely to develop malignant peripheral nerve sheath tumors.

How common is NRSTS?

• NRSTS tumors account for about 4 percent of all childhood cancers.
• Fibrosarcomas are the most common of all childhood NRSTSs: 23.9 percent.
• NRSTS is most common in teens and infants younger than 1 year.
• In people younger than age 20, about 500 to 600 cases are diagnosed each year in the United States.
• Synovial sarcoma is rare. Only about 1 to 3 people in a million are diagnosed yearly in the United States.

What are the symptoms of NRSTS?

Often there are no symptoms for NRSTS. However, an injury or other trauma can make the tumor noticeable. Symptoms may include:

• Pain
• Swelling
• Numbness

Symptoms of advanced NRSTS include:

• Fever
• Sweating
• Weight loss
• Hyperglycemia (high blood sugar) in children with fibrosarcoma in the lungs

How is NRSTS treated?

• Surgery—is used to remove the tumor. This is the most effective NRSTS treatment.
• If surgeons cannot remove the tumor, radiation and/or chemotherapy may be used.
• Patients whose tumors cannot be surgically removed “as is” may receive chemotherapy and/or radiation therapy to shrink tumors before they are removed.

Surgery for NRSTS may involve several steps:

• A biopsy (removal of a portion of the affected tissue for examination)
• Removal of the tumor completely or as much as possible
• Removal, if needed and possible, from places where the tumor has spread

Patients with tumor remaining after surgery may benefit from radiation therapy.

• Radiation therapy—uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing.
  • External radiation uses machines outside the body to deliver the X-ray dose.
  • Internal radiation uses needles, seeds, wires or catheters (tubes) to deliver the radiation directly into or close to the cancer.
  • In children, the dosage must be chosen with care to reduce the amount of radiation growing tissues receive near the tumor site.
• Proton therapy—delivers high radiation doses directly to tumors while sparing normal tissues and reducing the side effects of traditional X-ray therapy. Proton therapy’s chief advantage is the ability to control its depth and intensity. The more precise the beam, the more targeted the therapy.

Certain patients with high-risk NRSTS may benefit from chemotherapy.

• Chemotherapy (“chemo”)—uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells.
  • Chemo may be injected into the bloodstream, so that it can travel throughout the body.
  • Some chemo may be given by mouth.
  • Combination therapy uses more than one type of chemo at a time.

What are the survival rates for NRSTS?

Survival rates for NRSTS vary widely due to the variety and numbers of cancers grouped under this name. Overall, the outlook for survival in patients treated for NRSTS depends on several factors:

• The size of the tumor
• Its grade (stage) of growth and spread
• The amount of tumor that can be removed surgically
• The extent of its spread, if any, to other parts of the body

Why choose St. Jude for your child’s NRSTS treatment?

• St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.
• St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
• The nurse-to-patient ratio at St. Jude is unmatched— averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
• Current treatment guidelines for these tumors were explored in a clinical trial that was developed at St. Jude.