Non-rhabdomyosarcoma soft tissue sarcoma in children

What is non-rhabdomyosarcoma soft tissue sarcoma in children?

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 non-rhabdomyosarcoma soft tissue sarcoma in children?

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

• Connective tissue tumors exist throughout your body. They support and connect other tissues and organs.

Soft tissue sarcomas that form in this tissue include:

• Desmoid tumors 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 are 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 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) 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 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 non-rhabdomyosarcoma soft tissue sarcoma in children?

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 non-rhabdomyosarcoma soft tissue sarcoma in children 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 non-rhabdomyosarcoma soft tissue sarcoma in children?

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 non-rhabdomyosarcoma soft tissue sarcoma in children 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 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.