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What Is Sarcoma?

by Jeffrey Toretsky, M.D.
June 30, 2021
what is sarcoma

Dr. Jeffrey Toretsky is a St. Baldrick’s researcher at the Georgetown Lombardi Comprehensive Cancer Center in Washington, D.C. He explains what sarcoma is, how it’s diagnosed and treated, and how research is helping kids and adults with this type of cancer.

What is sarcoma?

A sarcoma is a bumpy tumor that occurs in the connective tissues (nerves, muscles and bones) anywhere in the body.

Sarcomas are rare, especially in young children. In kids between 10 and 20, sarcomas make up about 20-25% of childhood cancer diagnoses.

Sarcomas can start off being tiny lumps that you can’t feel. They can spread through the body, or metastasize, before they grow big enough to be seen.

There are many different types of sarcomas. Some sarcomas are more likely to occur in children, adolescents and young adults compared to older adults.

With children and adolescents, the most common sarcomas are Ewing sarcoma and osteosarcoma (bone tumors), and embryonal rhabdomyosarcoma and alveolar rhabdomyosarcoma (muscle tumors). About 80-90% of childhood sarcomas are Ewing sarcoma or osteosarcoma.

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What is the average age of diagnosis in children?

The average age at diagnosis varies depending on the type of sarcoma.

Rhabdomyosarcomas often occur in younger children. Osteosarcoma diagnoses peak in the late teens and early 20s. Ewing sarcoma diagnoses have a much larger plateau, spanning from puberty to the mid-20s.

What are the signs and symptoms of sarcoma?

The number one sign of sarcoma is a physical lump without any source of injury. If there is an unexplained pain or swelling in a joint or bone and it hasn’t gone away in a couple of weeks, it should be checked by a doctor.

How are sarcomas diagnosed?

Sarcomas can usually be spotted in an x-ray. If nothing shows up, doctors will follow up with a CT scan or MRI, depending on where the pain is located. The diagnosis will be confirmed with a biopsy.

Read how 9-year-old Tori is overcoming osteosarcoma >

What treatment options are available for sarcoma patients?

There are two aspects of treatment.

First, you have to treat the disease where it is present. This is called “local management.” This is done by surgery, radiation therapy or a combination of both.

The second aspect is to treat the disease before it spreads. In fact, many sarcomas have likely spread even though the metastases are not yet visible on imaging. Chemotherapy is used to get rid of these metastases.

How is childhood cancer research helping kids with sarcoma?

Research has given us insight into different therapies that target the tumor specifically, without damaging the other healthy cells. We call these “targeted therapies.”

Over time, we have figured out what the best chemotherapy is to use for each type of sarcoma. This is very helpful because some tumors respond better to certain therapies compared to others.

There is a wide spectrum of survival rates with sarcomas. Survival is affected by the tumor’s location, its type, and the age of the patient.

For kids younger than 14, long-term survival is almost 70%.

Ewing sarcoma and osteosarcoma are easier to treat. A diagnosis with only a localized tumor can bring 70-80% long-term survival. With a metastatic tumor, survival can be 15-20%.

Research is helping to improve these statistics, but there is still more to learn about the biology of sarcomas in order to find better therapies.

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