Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcoma

Non-rhabdomyosarcoma soft tissue sarcoma is a broad category of tumors that affect about 600 children in the U.S. each year. Children’s Health℠ offers care from pediatric cancer experts who are faculty members at UT Southwestern Medical Center. We use the latest therapies to give kids the best opportunity to overcome these cancers and get back to healthy, active childhoods.

What is Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcoma?

Soft tissue sarcomas are tumors that grow in muscles, organs and other soft tissues. Rhabdomyosarcomas are a type of soft tissue sarcoma that typically grow in muscles. All other soft tissue tumors are called non-rhabdomyosarcoma soft tissue sarcomas. They can grow in any part of the body with soft tissue and can spread to other organs.

What are the different types of Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcoma?

Blood vessel tumors

These tumors form in the lining of blood vessels or lymph vessels (part of the immune system). Children's Health has a special team dedicated to tumors and other problems related to blood vessels.

Bone and cartilage tumors

These grow in the cells of bones or cartilage (the kind of tissue that forms the structure of our noses).

Connective tissue tumors

Connective tissues exist throughout your body. They support and connect other tissues and organs. Soft tissue sarcomas that form in this tissue include:

Desmoid tumors

Desmoid tumors, which are also known as aggressive fibromatosis. These tumors are unlikely to spread to other areas of the body. Children with a genetic condition called familial adenomatous polyposis (FAP) are at higher risk for desmoid tumors.


Fibrosarcoma, which is typically found in children under age 4, especially infants. These tumors tend to be large and grow quickly, but they usually do not spread to other areas of the body.

Infantile myofibromatosis

Infantile myofibromatosis, which is a rare condition that is most often identified in newborn babies. This tumor can spread to multiple places in the body.

Fat tissue tumors (liposarcoma)

These tumors can grow in fat cells anywhere in the body. They are often found in the arms, legs or abdomen. These tumors often grow slowly and respond well to treatment.

Nerve tumors

These include a type of tumor that grows in tissue around nerves near the brain and spinal cord. Children with a genetic condition called neurofibromatosis Type 1 (NF1) are at higher risk for this type of nerve tumor.

What are the signs and symptoms of Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcoma?

Children or parents often notice a lump or swelling in the area where the tumor is growing. Sometimes these areas hurt, but often they don’t.

How is Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcoma diagnosed?

We identify the type of cancer by taking a sample of the tumor, looking at it under the microscope and studying its DNA. We also get detailed images of the tumor, through tests like an MRI, CT scan and PET-CT scan. These images show us how big the tumor is and whether it has spread to other places in the body.

We use the type, size and location of the tumor to decide what treatment will work best.

What causes Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcoma?

In most cases, there is no known cause, but some people may have an increased risk of developing sarcomas due to genetics. We know that patients with Li-Fraumeni syndrome, neurofibromatosis or hereditary retinoblastoma are at increased risk of developing soft tissue sarcomas.

How is Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcoma treated?

We use different methods to treat different types of tumors. Often we use a combination of treatments, at the same time or in stages, to treat the tumor and keep it from coming back. These include:


Surgery, to remove as much of the tumor as we can.


Radiation, which we can focus on specific parts of the body to kill cancer cells.


Chemotherapy, which kills cancer cells by sending chemicals throughout the body.

Targeted therapy

Targeted therapy, which includes drugs designed to attack specific types of cancers. For example, we can now treat fibrosarcoma with a new drug that blocks the specific way those cancer cells grow. Children’s Health does a DNA test on all tumors, to see if a targeted therapy will work for them.

Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcoma Doctors and Specialists

Children’s Health is home to some of the nation’s top pediatric cancer specialists and where physicians are also faculty members at UT Southwestern Medical Center. We use a team approach and work together with surgeons, radiation oncologists, pathologists, radiologists, physical therapists, psychologists and social workers to provide integrated, multidisciplinary care.

Frequently Asked Questions

  • Are non-rhabdomyosarcoma soft tissue sarcomas curable?

    They can be. If the tumor is small, grows in one area instead of spreading, and is easy to remove surgically, the child has a very good chance of making a full recovery. It’s harder to treat these tumors when they spread. Our After the Cancer Experience (ACE) survivorship program monitors patients for the long-term effects of cancer and cancer treatment throughout their lifetime.

  • Why should I choose Children’s Health for my child’s care?

    We always look for new and better treatments. Our academic affiliation with UT Southwestern Medical Center allows our providers to participate in ground-breaking research and provide innovative care. Our patients have access to clinical trials that might only be available at a handful of hospitals. We provide access to the newest treatment options for patients with relapsed cancers (cancer that has come back after initial treatment) through clinical trials offered in our Experimental Therapeutics Program. We also use genetic testing through our Precision Medicine Program. In the program, we identify genetic mutations that can be targeted with medications, providing a more effective treatment with decreased side effects.