Sarcoma Awareness Month is every July, and at Alex’s Lemonade Stand Foundation, we’re shining a spotlight on pediatric sarcomas — a group of rare and often aggressive cancers that affect bones and soft tissues in children, adolescents and young adults.

While sarcomas make up just 15% of childhood cancers, their impact is anything but small. Understanding what sarcomas are — and why more research is urgently needed — is an important part of supporting children and families facing these diagnoses.

What is sarcoma?
The origins of the word sarcoma comes from two Greek words — “sarx,” which means “flesh” and “oma” which means tumor.  Sarcomas arise from irregular or mutated connective tissue cells of the body — the bones, tendons, muscles, cartilage, and fat. While standard treatments for a sarcoma diagnosis can seem nearly the same regardless of specific diagnosis, types of sarcomas are vast and varied. 

Understanding the types of sarcoma
Sarcomas can show up anywhere in the body. They are divided into two categories: bone and soft tissue. Within those two categories there are types and subtypes defined by the pathology of the tumor (i.e. what it looks like under a microscope), the biology of tumor (what its genetic characteristics are), and its location in the body. 

Bone sarcomas most commonly include osteosarcoma and Ewing sarcoma, both which can metastasize to the lungs. Children facing bone tumors not only face a terrifying, potentially deadly diagnosis, but also face surgeries that can jeopardize their mobility.

Soft tissue sarcomas like rhabdomyosarcoma and other rare cancers are often inoperable at diagnosis.

Research changing the future of pediatric sarcoma treatment
Treatment for sarcomas has been largely unchanged in 40 years and includes chemotherapy, radiation, and surgery. However, there have been some notable shifts in treatments for some types of sarcomas. An FDA approved drug, called larotrectinib, is now an upfront treatment option for infantile fibrosarcoma, a rare soft tissue sarcoma that is diagnosed in infancy. Larotrectinib carries a very low risk of side effects versus chemotherapy .

Through a large ALSF Crazy 8 grant, a research team led by Heinrich Kovar, PhD is working to understand the origins of Ewing sarcoma and osteosarcoma. Dr. Kovar and his team have focused on a gene called EWS/FLI1, which plays a role in Ewing sarcoma. They’ve produced a map of human stem cells — special cells that develop into many different cell types — to track when EWS/FLI1 causes bone and other connective tissue cells to transform into tumors. Two other Crazy 8 teams are also studying osteosarcoma and focusing their work to better understand how to treat disease that spreads to the lungs. 

Ultimately, this innovative work could lead to cures for children with bone sarcomas.

You can help cure pediatric sarcoma
Research is making a difference. You can be part of what happens next. Help make more breakthroughs like Dr. Kovar’s possible by joining our monthly giving community, the One Cup at a Time Club. As a thank you for becoming a member, we’ll send you a travel cup in your choice of blue or yellow. If you can’t give monthly, consider a one-time donation today to support cutting-edge sarcoma research and support for childhood sarcoma families.