By: Trish Adkins
When Declan Roberts was 3 years old, he told his mom, Megan:
“There are three things I can call stupid: Stupid cancer! Stupid medicine (YUCK)! Stupid shots!”
It was 2011 and Declan was already battling primary central nervous system (CNS) rhabdomyosarcoma, a type of pediatric sarcoma. Rhabdomyosarcoma arises in the soft tissues of the body. While it accounts for about 3% of all pediatric cancer diagnoses, Declan’s rhabdomyosarcoma was found in his CNS, which is extremely rare.
Declan began treatment — three brain surgeries, 30 weeks of intense chemotherapy and other procedures, which would keep him in the hospital and separated him for weeks from his two brothers, Brendan and Tommy. Still, his positive spirit and his drive to have no bad days, always won out over the negative.
While the cancer ultimately took Declan’s life, it never took his spirit or won any battles.
His family continues to fight for cures for pediatric sarcoma, as fundraisers, volunteers and lemonade stand hosts. Their work, together with the work of researchers and supporters everywhere, is helping to push the world closer to safer treatments and cures for pediatric sarcoma.
This year, more than 1,600 children will be diagnosed with one of the 100 different subtypes of pediatric sarcoma. Because pediatric sarcomas are so diverse, research continues to focus on finding targeted treatments that attack the mutations and oncogenes that drive sarcoma growth.
Here are 5 things you need to know about pediatric sarcomas:
1. Pediatric sarcomas are typically found in the connective and supportive tissues of the body.
These tumors are classified as sarcomas because they are of mesenchymal stem cell origin. Mesenchymal stem cells can differentiate into bone, cartilage and muscle cells which is why sarcomas typically arise in the connective and supportive tissues of the body.
2. While all sarcomas share some similarities, there are two major categories: bone and soft tissue sarcomas, based on where they typically arise.
Ewing sarcoma and osteosarcoma are types of bone cancers, while rhabdomyosarcomas and synovial sarcoma arise in the soft tissue of the body.
3. Sarcoma cure rates lag behind many other types of pediatric cancer.
The five-year survival rate for all types of pediatric cancer is approximately 86%. For sarcomas, the five-year survival rate is much lower. The three most common pediatric sarcomas — Ewing sarcoma, osteosarcoma and rhabdomyosarcoma have five-year survival rates ranging from 65 to 75%. For some sarcomas, the survival rates haven’t changed in decades.
4. On a molecular level, sarcomas can be fusion-positive or fusion-negative and understanding fusion status can guide therapeutic decision-making.
Fusion-positive tumors are characterized by chromosomal abnormalities that lead to the fusion of two disparate genes, whereas fusion-negative tumors are not driven by known gene fusions. For example, Ewing sarcoma is fusion-positive, while osteosarcoma is fusion-negative. Rhabdomyosarcoma can be fusion-positive or fusion-negative. More research is needed to understand the molecular underpinnings of sarcoma subtypes, so that sarcomas can be accurately diagnosed and better treatments can be made and tested.
5. Because sarcoma research is so important, Alex’s Lemonade Stand Foundation (ALSF) launched the Crazy 8 Initiative to empower collaborative research teams to tackle some of the most pressing problems in childhood cancer research today.
Through the Crazy 8 Initiative, ALSF committed $18.5 million to four research projects. One of those projects, led by Dr. Heinrich Kovar, is working to discover the origins of bone sarcomas and develop tumor models that can be used to test targeted drugs to treat this deadly pediatric cancer.
“You cannot just neglect these problems — and say okay, we’ve tried for such a long time and we can’t do anything about it. That’s not helping the kids. You have to take the risk,” said Dr. Kovar.
July is Sarcoma Awareness Month. You can help Alex’s Lemonade Stand Foundation fund cutting-edge sarcoma research by joining our monthly giving One Cup At a Time club or making a one-time donation. One Cup at a Time members get exclusive beside-the-scenes access to our funded projects, exclusive ALSF swag and to be part of the sustained fight to find cures for all children battling cancer.