Facts

What Is Sarcoma?

by St. Baldrick's Foundation
July 5, 2023
what is sarcoma

What are childhood sarcomas?

A sarcoma is a tumor that grows in the bone or soft tissue (muscles, nerves and fat) anywhere in the body. Sarcomas are rare, especially in young children, accounting for less than 15% of childhood cancers.

Pediatric sarcomas are classified into two major categories: bone sarcomas and soft tissue sarcomas.

Bone sarcomas can appear in any bone, but are most often seen in the leg, arm, pelvis or rib. The most common bone sarcomas are Ewing sarcoma and osteosarcoma.

Soft-tissue sarcomas can occur in any soft tissue, anywhere in the body. The most common soft-tissue sarcomas are embryonal rhabdomyosarcoma and alveolar rhabdomyosarcoma.

Within these two umbrella categories, there are many different types of sarcomas that can develop in children.

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.

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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 is the most common childhood soft tissue sarcoma in children 14 years and younger. 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 most common sign of childhood sarcoma is a painless 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?

Treatment is tailored to each patient’s needs and typically combines surgery, chemotherapy and radiation. In addition to treating the tumor itself, treatment will also focus on preventing or managing the spread of the tumor to other parts of the body.

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 20, 5-year survival is about 70%.

5-year survival rates are higher for children with localized tumors compared to kids with metastatic disease. Unfortunately, children with relapsed disease have poor prognosis.

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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